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            1               COMMONWEALTH OF MASSACHUSETTS
                         
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            4                                   *    
                 VERBATIM MINUTES OF THE        *
            5    SOCIAL SECURITY ADMINISTRATION *
                 POLICY CONFERENCE ON           *
            6    CHRONIC LIVER DISEASE          *
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           10                        The Radisson Hotel
                                     700 Memorial Drive
           11                     Cambridge, Massachusetts
                 
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                                Wednesday, November 17th 2004
           14                       Commencing at 9 a.m.
                 
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            1    PANELISTS: 
                 
            2    Leonard B. Seeff, MD
                 
            3    Barry Zevin, MD
                 
            4    Averell Sherker, MD
                 
            5    Philip Rosenthal, MD
                 
            6    Normal Grace, MD
                 
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            1                            MR. JULIAN:  My name is James 
            2    Julian, Acting Director, Office of Medical Policy.  I 
            3    would like to take care of a couple of housekeeping items 
            4    and go over the agenda.  First, if you could take a moment 
            5    and identify the three exits in the room.  We have one 
            6    over here (indicating), one to everybody's right 
            7    (indicating) and also one behind you (indicating); 
            8    restrooms are located in the hallway outside. 
            9      This morning's presentation and this afternoon's 
           10    presentation will be transcribed, so if you have comments 
           11    or questions, could you, please, identify yourself to the 
           12    court reporter; she's here in pink (indicating).  If she 
           13    doesn't get your name, she will signal you, so say your 
           14    name, and she'll be able to take that down.  Also, there 
           15    will be an evaluation form at the end; could you, please, 
           16    leave that with the registration desk before you leave at 
           17    the end of the day?  Also Rona and Alaria outside will 
           18    assist you with any logistical questions or concerns. 
           19      We have a couple of brief changes to the agenda today.  
           20    We have a break scheduled from 10:30 to 10:45.  Because we 
           21    have a Dr. Rosenthal will be on the phone, we will push 
           22    through the break.  If anybody needs a break, feel free to 
           23    get up and get coffee in the back and make use of the 
           24    restrooms.  We apologize for that, but because we'll have 
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            1    Dr. Rosenthal on the phone we wanted to go ahead and go 
            2    forward with that. 
            3      I would like to go over the agenda.  Speaking first will 
            4    be Dr. Leonard Seeff, speaking from 9:15 to 9:35.  Dr. 
            5    Seeff is at the National Institute of Health he's a 
            6    special expert on viral hepatitis and National Institute 
            7    of Diabetes and Digestive and Kidney Diseases.   Speaking 
            8    second will be Molli Conti.  Ms. Conti is an advocate 
            9    participant.  She's Vice President of Outreach with 
           10    Hepatitis B Foundation.  She will be speaking from 9:35 to 
           11    9:55.  Speaking next will be Mr. Rodolfo Porro, 9:55 to 
           12    10:10.  Mr. Porro is a patient who can offer firsthand 
           13    testimony of a person suffering from chronic liver 
           14    disease. 
           15      Speaking fourth by phone will be Dr. Rosenthal.  Dr. 
           16    Rosenthal is the Medical Director of Pediatric Liver 
           17    Transplants, Director of Pediatric Hepatology and 
           18    Professor of Pediatrics and Surgery at University of 
           19    California at San Francisco. 
           20      At this time, it's my pleasure to introduce Mr. Martin 
           21    Gerry.  Mr. Martin Gerry is the top disability policy 
           22    person at Social Security if not the federal government. 
           23                            MR. GERRY:  I want to thank you 
           24    all for coming today and welcoming me to this event.  
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            1    (Inaudible.)  I would like to take a few moments to talk 
            2    about the process that led up to this kind of meeting.  
            3    Historically, we have tended to develop our rule, what we 
            4    call "listings" for the most part, as a technical process, 
            5    Periodically issue new revisions.  We ask for public 
            6    comments, but usually reaction to the changes are 
            7    proposed.  The approach that we have moved to has been 
            8    extremely interesting. 
            9      We really want to have an open dialogue with people who 
           10    are very familiar with our beneficiaries and particularly 
           11    beneficiaries -- in this case, chronic liver disease -- 
           12    help us to figure out what is the best way to go about the 
           13    adjudication processes and also the best way to help 
           14    people with these conditions who may be eligible for these 
           15    benefits, but we want to have equal opportunities for 
           16    work. 
           17      I've been asked by several people about, what do you 
           18    think is going to come out of the meeting.  One of the 
           19    greatest things, I don't know what's going to come out of 
           20    this meeting.  We'll get a lot of insight.  I know we're 
           21    going to learn a lot, I suspect, that I will hear about 
           22    and think about.  I found these to be extremely 
           23    stimulating, but the key is everyone's participation in 
           24    this process; that's what makes it work. 
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            1      So thank you all for being here and participating in the 
            2    process, and I'll introduce Glenn Sklar, Associate 
            3    Commissioner for Disability Programs.  
            4                            MR. SKLAR:  Good morning.  Again, 
            5    I welcome everybody and appreciate the fact that some of 
            6    you folks have come in out of town.   I'm the last person 
            7    standing between you and the panel, so I'll be brief.  By 
            8    way of background, SSA's medical listings are our 
            9    screening tool, a quick step way we can resolve a lot of 
           10    cases and adjudicate cases at Social Security.  Keep in 
           11    mind that each year, 2.5 million new claims and 2.5 
           12    million people show up at our door asking for disability 
           13    benefits.  We make hard decisions, who gets and who does 
           14    not.  What SSA's medical listings are about, it's a quick 
           15    medical screening tool to help make that decision. 
           16      Sometimes it's possible to come up with a good screening 
           17    tool.  If you meet these medical listings, you have paid 
           18    benefits.  Sometimes it's not.  The question here is 
           19    whether you can look at the medical criteria on chronic 
           20    liver disease and make that type of assessment. 
           21      We're going to be looking to the panel experts and 
           22    experts on treatment to help us with that issue.  Keep in 
           23    mind, too, at SSA, Social Security, if you don't meet the 
           24    medical listing which is medical criteria, there are other 
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            1    ways to getting onto Social Security disability.  The next 
            2    step to consider is your functional impact and your 
            3    functional abilities and limitations.   So you're not 
            4    automatically disqualified from benefits if you don't meet 
            5    the medical listing.  
            6      However, keep in mind, it's a wonderful screening tool 
            7    used for the last 40 years, but we need expert input and 
            8    welcome your ideas on how we can do a better job with 
            9    chronic liver disease. 
           10      One other quick piece of background, we did publish a 
           11    rule making on digestive disorders about two years ago.  
           12    Part of that is also involving chronic liver disease, and 
           13    we'll be talking a lot about that today.  We'll be 
           14    focusing exclusively on chronic liver disease and will not 
           15    be entertaining comments on anything else in that package 
           16    either because we're on the record, and we have opened up 
           17    only the piece on chronic liver disease.  With that 
           18    background, I would like to welcome our panel and welcome 
           19    all of you to the conference this morning.
           20                            DR. SEEFF:  My name is Leonard 
           21    Seeff; I'm at National Institutes of Health where my focus 
           22    is actually on hepatitis and all the studies related to 
           23    hepatitis.  Our focus has been on Hepatitis C, but we're 
           24    moving to Hepatitis B now with some conferences coming up 
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            1    and more work focusing on that.  I'm not exactly sure how 
            2    to address this panel, and I wasn't sure to whom I was 
            3    speaking, physicians or non-physicians, so I was asked to 
            4    make a few comments, and I was asked to address one, How 
            5    is chronic liver disease diagnosed?  Secondly, what does a 
            6    liver biopsy indicate?  And this section, pediatric liver 
            7    disease, and Dr. Rosenthal will fill in on the difference 
            8    between liver disease in the pediatric population and 
            9    adults.  Do we need a listing for chronic liver disease?  
           10    So I thought what I would do is start simply because I 
           11    assume there are a lot of people here who are not 
           12    physicians and try to address what I've been asked to 
           13    address. 
           14      The first issue is, How do we diagnose chronic liver 
           15    decease?  And what we have to do is, first of all, when we 
           16    as physicians, hepatologists, gastroenterologists, 
           17    internists and someone taking care of patients, is to 
           18    identify the disease exists, and then the next thing to do 
           19    is to define what the causes of chronic liver disease 
           20    because there are numerous entities that may cause chronic 
           21    liver disease, some more common than others. 
           22      I'm sorry this is a little busy, but the question is, 
           23    What are the clues that we as physicians have to existence 
           24    of chronic liver disease, and you can divide it up into 
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            1    four categories.  One is there would be liver tests by 
            2    chemical abnormalities of the liver test that would bring 
            3    the disease to one's attention or raising it separately.  
            4    Second of all, somebody may in fact appear because they 
            5    have symptoms associated with liver disease that may be 
            6    the first evidence that there's chronic liver disease as 
            7    it exists. 
            8      Thirdly, we may see people toward the end of their 
            9    disease who have severe symptoms of bleeding, swelling of 
           10    the abdomen, swelling of the ankles or encephalopathy.  
           11    Fourthly, there may have been radiologic findings; for 
           12    example, an ultrasound, CT scan, that hadn't been done for 
           13    one or other reason, and you identify the presence of 
           14    features that lead one to consider the possibility of 
           15    chronic liver disease; that is, the presence of ascites, 
           16    which is fluid in the abdomen, and enlarged liver or 
           17    spleen. 
           18      Depending upon the cause for the chronic liver disease 
           19    and at the moment, the most common cause that we're 
           20    dealing with is Hepatitis C of all of the forms.  I'll 
           21    show you some figures on this in a moment.  The most 
           22    common chronic liver disease is Hepatitis C and Hepatitis 
           23    B is also quite common, not in this country, although 
           24    worldwide is the most common of the Hepatitis B viruses 
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            1    because it's found to be endemic in parts of the world 
            2    that are the most populace nations; that is, Southeast 
            3    Asia -- Asia and Africa and so on where there's more 
            4    Hepatitis B than there is Hepatitis C. 
            5      What we find or what I've found in my experience, I used 
            6    to be at the VA Hospital for many years taking care of 
            7    patients with all forms of chronic liver disease, the VA 
            8    in Washington, and then I came to NIH, and I see patients 
            9    at the NIH, but these are people who come for a specific 
           10    reason.  So usually what happens is that people have been 
           11    found to have an abnormal liver test, and the two tests 
           12    that are most commonly to be found abnormal are the ALT 
           13    and AST otherwise known as the SGOT and SGPT.  Often this 
           14    is found in individuals unexpectedly, people who don't 
           15    have a physical exam for one reason or another.  They may 
           16    be looking for insurance policy, starting a new job or 
           17    they may be tested for something else or they have gone to 
           18    donate blood and have an abnormal ALT or an AST. 
           19      This leads one to the consideration of disease of the 
           20    liver which is inflammatory in type.  There's an 
           21    inflammation that we think is responsible for that. 
           22      The other one, which is the alkaline phosphatase, which 
           23    leads one to consider the possibility of another form of 
           24    chronic liver disease, the obstructive forms of liver 
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            1    disease, things like primary biliary cirrhosis, primary 
            2    cirrhosis and so on. 
            3      Occasionally, somebody is found to have this the 
            4    Hepatitis B or C virus with normal liver enzymes; for 
            5    example, blood donors are screened before they donate 
            6    blood, and they may be be found to be Hepatitis C positive 
            7    although their liver enzymes are normal.  Generally, by 
            8    definition, we have concluded that a period of six months 
            9    of abnormality defines the probability of chronic liver 
           10    disease, incipient chronic liver disease.  This came from 
           11    studies done 25 years ago at the Mayo Clinic where they 
           12    were looking at chronic liver disease, and they 
           13    concluded -- they tried to distinguish between acute liver 
           14    disease and chronic liver disease, and they concluded if 
           15    the liver enzymes were abnormal became normal by six 
           16    months, this was acute disease and consistent with chronic 
           17    disease.  That's the timing that we have continued to 
           18    have. 
           19      I think there are some instances which are next 
           20    determined before the six month period that chronic liver 
           21    disease exists.  That's the definition.  So patients may 
           22    be identified or at least the clues to the existence of 
           23    the disease, the findings of abnormal enzymes, or they may 
           24    come in, as I say, with symptoms of extreme fatigue or 
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            1    they may come in with end stage liver disease for the 
            2    first time with upper intestinal bleeding from viroses and 
            3    swelling of the abdomen, ascites, swelling of the ankles, 
            4    edema or mental confusion or encephalopathy, or rarely 
            5    they may be identified for the first time by radiologic 
            6    findings. 
            7      So that establishes the likelihood, and we can get by 
            8    with this mostly, but if we want to be absolutely certain 
            9    of the severity of the disease, a liver biopsy is 
           10    extremely helpful.  It should be noted -- and this is 
           11    important -- that many persons with chronic liver disease 
           12    may have no symptoms whatsoever particularly early in the 
           13    course of the disease.  That's true of Hepatitis C and 
           14    even B.  Even the development of the disease in most cases 
           15    is never known.  Most people have no symptoms when they 
           16    have acute Hepatitis C and often less frequently acute 
           17    Hepatitis B.  But that doesn't mean to say that this isn't 
           18    a severe problem despite the fact they have no symptoms.  
           19    Some people have no symptoms early on and it's true to say 
           20    as the diseases progresses from acute to chronic liver 
           21    disease -- and I will show you a slide  -- brief sequence 
           22    of events and evolve into cirrhosis and even then in many 
           23    instances, there are no symptoms. 
           24      Once cirrhosis develops, this is a severe problem.  So 
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            1    the lack of symptoms does not in fact exclude an 
            2    underlying disease of great importance.  The overt signs 
            3    and symptoms of chronic liver disease are usually late 
            4    manifestations of advancing cirrhosis.  It doesn't mean 
            5    that the early course that symptoms such as fatigue 
            6    exists, and in some instances devastating fatigue.  Other 
            7    people have no fatigue or no symptoms whatsoever, and 
            8    often we see patients identified by liver enzymes that 
            9    have chronic hepatitis, and we think they were infected 20 
           10    years earlier, and we say, "You've got Hepatitis C because 
           11    we screened you and they found this"; and they say, "I 
           12    have never been sick.  I don't feel sick."  There are some 
           13    people with no symptoms.  Other people have profound, 
           14    devastating fatigue. 
           15      So here is a quick, brief summary of the sequence of 
           16    events.  This probably is true for most forms of chronic 
           17    liver diseases, some more than others, but it starts off 
           18    with an initial acute injury to the liver, and that is an 
           19    inflammatory condition where there's inflammation.  You 
           20    look at the liver and do a biopsy, you see lots of 
           21    inflamed cells and death of some of these cells, and in 
           22    those diseases that are destined to progress to chronic 
           23    liver disease, what happens is you begin to develop early 
           24    scarring of the liver, called "fibrosis."  That scarring 
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            1    is first confined to the liver and begins to expand and 
            2    extend, and then you end up with advanced fibrosis of the 
            3    liver.  Now, this is not inevitable, but this is the 
            4    course that some people take, many people take, depending 
            5    on what the cause of the disease is. 
            6      They can go on to develop cirrhosis which we call 
            7    "compensated" first which is they have -- to do the liver 
            8    biopsy, you will see cirrhosis, but the liver functions 
            9    seem to be fairly good, and they don't have ascites or 
           10    peripheral edema, but if you do a liver biopsy, there's 
           11    cirrhosis.  This may over time progress to decompensated 
           12    cirrhosis; by that we mean, the liver is not properly 
           13    functioning.  The two major components of not functioning 
           14    properly, one is that the function of the liver, the 
           15    ability to make proteins to get rid of bilirubin so on is 
           16    not working very well.  They can become jaundiced.  Their 
           17    (inaudible) becomes abnormal, their albumen drops, and 
           18    there's an obstruction to blood flow through the liver 
           19    because of the scarring to the liver and therefore you get 
           20    dilatation of vessels, or you may get bleeding from 
           21    viroses, or you may have back up of fluid. 
           22      The next and greater complication is the development of 
           23    liver cancer.  I can tell you, and this is not to frighten 
           24    people but to be certain that people recognize that liver 
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            1    cancer is now an emerging problem in the United States.  
            2    It is among  -- we have just had a major meeting that's 
            3    been published in the Journal of Gastroenterology, which 
            4    covers an enormous and important conference at the NIH 
            5    showing that this is one of the cancers that's increasing 
            6    while many other cancers are decreasing.  It's now the 
            7    eighth cause of cancer in the United States, and we think 
            8    it's going to get worse before it gets better, and much of 
            9    this is a consequence of Hepatitis C that was acquired 20, 
           10    25 years ago. 
           11      The acute Hepatitis C is coming down.  The instance of 
           12    acute liver disease is coming down.  What we're dealing 
           13    with is the type that took place in the '70s and '80s of 
           14    chronic liver disease and the incidence of liver cancer is 
           15    going up dramatically, and we think it will peak in 2015 
           16    and 2020.  This is a problem that we're really quite 
           17    concerned about.  It's a very -- most cancers are fatal.  
           18    Life is fatal, but cancers are more fatal.  This is one 
           19    that, unfortunately, has a very high mortality within a 
           20    very short period of time, and we're trying to do 
           21    something about that.  
           22      As you can see, the problem is it's a long leg period, 
           23    from 20 to 40 years from the time of acute infection.  So 
           24    what are the causes?  We define the existence of chronic 
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            1    liver disease patients that have had abnormal enzymes or 
            2    they come in with profound fatigue which leads us to do 
            3    the testing, and we find that liver enzymes are normal.  
            4    What do we consider?  There are a whole bunch of things 
            5    that we consider. 
            6      So chronic hep infection with the hepatitis viruses B 
            7    plus D or delta hepatitis or Hepatitis C are among the 
            8    most common.  Hepatitis C is the most common.  People used 
            9    to think about chronic liver disease as being a 
           10    consequence of alcohol.  Alcohol is far less common as a 
           11    basis for chronic liver decease but it still is. 
           12      We're now moving into a new era, and the era is obesity 
           13    and its impact is widespread, and every sub specialist 
           14    will tell you how important obesity to them for the heart, 
           15    for the muscular skeletal system and so on.  From the 
           16    point of the liver, we're facing a new epidemic, 
           17    non-alcoholic, fatty liver disease or non-alcoholic sciata 
           18    hepatitis which is the emerging disease.  We think that   
           19    20 years from now, we will not being dealing with 
           20    Hepatitis C, but we will be dealing with mesh as the 
           21    predominant cause for chronic liver disease. 
           22      Then there's autoimmune hepatitis which is part of an 
           23    immunologic manifestation that affects many other organs 
           24    and may include the liver.  We used to believe that all of 
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            1    chronic liver disease was autoimmune hepatitis.  When I 
            2    first began, I used to think chronic active hepatitis was 
            3    all autoimmune.  Turns out that it's responsible for only 
            4    about five percent of all cases. 
            5      Then there's the genetic disease of human chromatosis, 
            6    Wilson's Disease, copper overload, iron overload.  There's  
            7    an entity called "Alpha 1 Antitrypsin Deficiency."  This 
            8    is an enzyme that the liver is unable to extrude, 
            9    interferes with the function, and the emerging problem, 
           10    too, is drug-induced chronic liver disease.  So happens, 
           11    we are at the NIH have a big study called "Dillon 
           12    Drug-induced Liver Injury" trying to understood why many 
           13    people who take drugs will develop injury and most other 
           14    people who take the same drugs don't, and one of the 
           15    potential outcomes of this diseases of this entity is 
           16    chronic liver disease, and then you get the cholestatic 
           17    forms of chronic liver disease; that is, obstructive forms 
           18    of liver disease, primary bilary schlerosis, primary 
           19    schlerosis cholangitis; and ultimately you end up with, I 
           20    can't make a diagnosis but the person has schlerosis.  
           21    It's what we call cryptogenic.  It's a general term for, 
           22    We don't know the cause and someday we will, and we'll be 
           23    able to put them into slots as we go along. 
           24      Next slide, please.  This is personal communication, not 
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            1    yet been published, but to give you a sense of the causes 
            2    of chronic liver disease and a big study done in 2000 with 
            3    this and there's a study that NHI did together with the 
            4    Centers for Disease Control and surveillance they did, and 
            5    it turns out when we look at the causes of chronic liver 
            6    disease, 43.3 percent of this was a consequence of 
            7    Hepatitis C alone, 23.8 percent was the consequence of the 
            8    HC virus, plus alcohol C; non-alcoholic fatty liver 
            9    disease was 11 percent.  Other things in combination of 
           10    autoimmune hepatitis, primary bilary cirrhosis, primary 
           11    cholangitis, hemachromatosis and drug-induced cryptogenic 
           12    was 9.9., and alcohol was 6.7 percent on its own.  That's 
           13    interesting because somehow in the general population, the 
           14    view is that cirrhosis must mean alcohol, and here you see 
           15    alcohol on its own have been represented in 7 percent, and 
           16    then Hepatitis B in this country, 2.4  percent.  It's low 
           17    on the totem pole, but it's an emerging problem 
           18    particularly as immigration from other parts of the world 
           19    comes about in this country. 
           20      Next slide.  How do we diagnose these?  I think I don't 
           21    have to go into detail, but just as a brief summary, 
           22    chronic viral hepatitis, you diagnose by identification of  
           23    the abnormal liver tests and do various assays for 
           24    Hepatitis B, C and D as needed.  As you know, Hepatitis A 
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            1    doesn't cause chronic liver decease.  It's largely B and C 
            2    in this country with a little bit of D, and D almost never 
            3    occurs on its own.  It always, sort of, piggy-backs itself 
            4    on Hepatitis B. 
            5      Chronic alcoholism, we have to take a history, and we 
            6    have to do certain blood tests.  Non-alcoholic sciatic 
            7    hepatitis, we have to do biopsy and imagining.  Autoimmune 
            8    hepatitis, there are autoimmune markers.  There are a 
            9    whole series of things.  I'm not sure we need to go 
           10    through that here.  I'm not sure the rest of my slides are 
           11    meaningful, but let me see what the next one was.
           12      There's another entity that I should also bring to your 
           13    attention, and maybe this will be discussed.  This is the 
           14    so-called extra hepatic manifestations of Hepatitis C. 
           15    Hepatitis C not only has an impact on the liver, but may 
           16    cause other entities, an entity called "mixed cryoglobulin 
           17    anemia" which is more common in Europe, but we see it 
           18    here.  Kidney disease may be an association with a B cell 
           19    lymphoma and a couple of skin diseases, cero negative 
           20    arthritis, arthritis without the usual tests, and the next 
           21    slide shows you the essential (inaudible) anemia is 
           22    associated with a palpable kind of rash of the skin, 
           23    arthralgias, kidney disease, some neuropathies, Renaut's 
           24    phenomenon, fatigue and weakness.  So just to remind you 
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            1    there may be other manifestations that may be specific to 
            2    the liver. 
            3      Next slide, please.  What I did was to show a sequence 
            4    of events.  Maybe the next slide shows you how we go about 
            5    trying to make this diagnosis, and I'm not sure whether 
            6    this is relevant.  If it is, I'll go through.  If it's not 
            7    I'll skip it.  We go through a series of steps.  You think 
            8    the patient has chronic hepatitis.  You do your physical 
            9    examination looking for risk factors to see if they have 
           10    taken any drugs.  Let me tell you and remind you that the 
           11    drugs that they're talking about are not only conventional 
           12    drugs but more and more the case of complimentary, 
           13    alternative medicines the herbal products which are very, 
           14    very common, and we're seeing instances of drug-induced 
           15    liver disease from herbal products.  It's not a major 
           16    problem but it does occur. 
           17      Then you test for Hepatitis B, and if the person is 
           18    positive, you then test with delta hepatitis to make sure 
           19    they don't have delta.  You go through a series of tests 
           20    to decide what kind of Hepatitis B we're talking about.  
           21    If that's negative, you then go and test for Hepatitis C 
           22    and if they're C positive  -- and there are various tests 
           23    for that -- you come up with a diagnosis of chronic 
           24    hepatitis.
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            1      Once you have excluded Hepatitis B and Hepatitis C and 
            2    alcohol, then you have to go through all the other tests 
            3    looking for hemachromatosis and Wilson's Disease, 
            4    autoimmune hepatitis and so on and liver biopsy.  Let me 
            5    quickly run through the liver biopsy.  What does a liver 
            6    biopsy indicate?  It is necessary in some instances and 
            7    helpful in most when diagnosing some of the conditions.  
            8    We can diagnose many of the conditions without a liver 
            9    biopsy, but what do we use if for? 
           10      We evaluate the extent of the chronic liver disease.  We 
           11    grade it, we score it, and what we do is to look to see 
           12    how much inflammation and how much scarring there is, and 
           13    there's a whole various scoring systems that we use.  
           14    Sometimes when we look for Hepatitis C, we find other 
           15    things that may have an impact such as presence of fat or 
           16    iron. 
           17      Next slide, please.  Is a liver biopsy mandatory, and I 
           18    think the answer to that is, in general, liver biopsy is 
           19    not mandatory, but the findings are very helpful for the 
           20    both the patient and the health care provider for 
           21    predicting the natural history of the disease and the 
           22    relative urgency of treatment.  Coming back to Hepatitis 
           23    C, there are some instances where we do a biopsy, with 
           24    minimal inflammation.  The liver enzymes are minor.  If we 
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            1    had a treatment that completely cured everybody, we would 
            2    treat without the liver biopsy because this is not the 
            3    case with Hepatitis C.  We try to determine who really 
            4    needs it, so it's useful in that instance.
            5      The liver biopsy is especially useful for the treatment 
            6    and partially effective and associated with unpleasant 
            7    side effects and liver biopsy is unnecessary if the 
            8    treatment is highly effective, and there are some kinds of 
            9    Hepatitis C, some genetic forms.  One is the Geno types 1, 
           10    2, 3, 4, 5, 6, etcetera.  In Geno types 2 and 3, the 
           11    response rate in treatment is now about 80 percent.  We 
           12    think you can treat without a liver biopsy. 
           13      There are complications of liver biopsy.  People hate to 
           14    have to have it.  There's pain.  There could be bleeding 
           15    at the site.  There can be peritonitis from bile, and 
           16    there's a rare instance of death from bleeding.  So that 
           17    is a problem, and everyone is looking for alternate ways 
           18    of trying to get the information, and what we're looking 
           19    for are so-called "markers" of chronic liver disease and 
           20    of fibrosis and this is -- my center has just put together 
           21    at the request of government an action plans on liver 
           22    diseases.  It will be published next month which will 
           23    define the liver disease and research needed in the next 
           24    10 years and the thread that runs through all of these 
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            1    whether alcoholic liver disease or PVC or Hepatitis B and 
            2    C is how do we define the progression of the disease -- 
            3    that is fibrosis -- because in essence the problem with 
            4    liver disease and chronic liver disease and fibrosis and 
            5    cirrhosis. 
            6      I think with that, I'll stop there.  If there are any 
            7    questions, I will be happy to answer.  The big issue will 
            8    be about the treatment this afternoon.  Thank you.  Any 
            9    questions about as part of the presentation?  I'm not sure 
           10    whether this was what you wanted.  I'm afraid this is what 
           11    you got. 
           12                            AUDIENCE:  I have a question.  If 
           13    you had a patient who really objects to having a liver 
           14    biopsy, what other testing would you recommend for that 
           15    patient?  Would you consider ultrasound and DNA blood 
           16    test?  As a patient advocate, we have a lot of patients 
           17    who are not so enthusiastic about a biopsy. 
           18                            DR. SEEFF:  If we could treat the 
           19    patient without the inflammation that we get from liver 
           20    biopsy, we will do that.  Liver biopsy has gone through 
           21    various phases.  There was a point in time when I began 40 
           22    years ago everybody had liver disease alcoholic hepatitis, 
           23    and we didn't know about it.  There was a phase where it 
           24    disappeared and then it returned with Hepatitis C because 
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            1    of the fact that we have a treatment which is only 
            2    partially effective that has very unpleasant side effects, 
            3    so we felt that we needed to define the form of liver 
            4    diseases that really warrants treatment.  For example, if 
            5    there was minimal fibrosis, and the enzymes were normal or 
            6    slightly elevated and all factors taken into account, we 
            7    say we won't treat until something better comes along. 
            8      What are the alternatives?   Well, ultrasound imaging is 
            9    getting better, and we learn much more.  There's no doubt 
           10    at the time moment in my mind view and other hematologists 
           11    might comment on it.  One specific information that comes 
           12    from liver biopsy.  We call the liver biopsy the "gold 
           13    standard," but it has its own problem.  For example, 
           14    there's something called som (inaudible) where putting a 
           15    little needle into the liver which is a massive organ and 
           16    taking out this tiny piece, and we're hoping that's 
           17    representative of what the liver problems are, and that's 
           18    not always the case.  There are problems with liver 
           19    biopsy.  
           20      By and large in many instances, we can get by physical 
           21    examination, looking at the liver function tests by a 
           22    finding of coming up with a clue.  There are some new 
           23    modalities developed in Europe, something called 
           24    "fibroscan" and "fibrotests."  It's a a series of markers 
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            1    that when you put it together, it gives you a clue as to 
            2    where you are along the markers.  There's this new thing 
            3    that has just come out that was presented at the liver 
            4    meeting a couple of weeks ago which is called to test 
            5    liver stiffness.  It's an instrument that costs $60,000 to 
            6    purchase, but you apply it to the side where the liver is, 
            7    and press a button, and this thing bounces against your 
            8    liver, and it measures the resistance.  They think it will 
            9    tell you whether there's fibrosis or not.  This has to be 
           10    proven, but we're moving in that direction.  Okay, thank 
           11    you very much. 
           12                            (Applause.) 
           13                            MS. CONTI:  Great news, we finally 
           14    got the expert.  Good morning, I'm Molli Conti.  I'm from 
           15    Hepatitis B Foundation.  I know a lot about Hepatitis B, 
           16    and I'm going to be impartial to include Hepatitis C as 
           17    well.  The most important thing to understand about both 
           18    the hepatitis have similar  -- even though there are 
           19    different compositions; one is an RNA virus; and one is a 
           20    DNA virus; but the end result of fibrosis and liver cancer 
           21    is the same for all these patients. 
           22      Just a little commercial about the foundation, the 
           23    Hepatitis B Foundation is a national, non-profit, and 
           24    we're dedicated to finding a cure and bringing hope to 
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            1    those affected worldwide, and we will share those 
            2    statistics with you today.  They're pretty impressive. 
            3      The foundation began in 1991.  There was a family who 
            4    had a child who was affected by Hepatitis B, and it's 
            5    actually a physician with a very strong research 
            6    background, and he was startled to discover that there 
            7    were no resources for his family to turn to find out more 
            8    about this disease, and so the Hepatitis B Foundation 
            9    began in 1991.  This is actually just the home of the 
           10    foundation, and we're lucky enough to be in a research 
           11    center doing research on Hepatitis B and C. 
           12      It's actually a wonderful situation because we give the 
           13    researchers the opportunity to have a patient connection.  
           14    They hear us talking on the help line to the patient, to 
           15    the newly diagnosed patient, who is, kind of, bewildered 
           16    about what to do, and the whole dynamics gives a unique 
           17    perspective to the center, to all of us who work at the 
           18    center. 
           19      This is a couple of statistics on Hepatitis B in the 
           20    United States.  Twelve million Americans have been 
           21    infected.  More than one million people are chronically 
           22    infected.  The 12 million Americans that have been 
           23    infected, that means some of those folks are  -- doesn't 
           24    mean all of those folks are -- chronically infected.  In 
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            1    the adult population they go through the acute phase and 
            2    recover. 
            3      We still have one million people in the United States 
            4    chronically infected and up to 100,000 new infections 
            5    every year.  That's the number that sticks with me 
            6    considering we've had a vaccine for Hepatitis B for over 
            7    20 years.  There's also a vaccine for Hepatitis A, not for 
            8    C.  Five thousand people do die each year from Hepatitis 
            9    B, and we lose one health care worker everyday from 
           10    Hepatitis B.  Hepatitis B is a very hardy virus and can 
           11    live outside the body as well as a very highly sexually 
           12    transmitted disease, so it's easy to get. 
           13      Hepatitis C in the United States  -- and Dr. Seeff  
           14    talked about the large number, 3.9 million infected with 
           15    Hepatitis C; that's 1.8 percent of the Americans; 25,000 
           16    new infections annually 2.7 million, and most of those are 
           17    accredited to IV drug users.  I apologize for this small 
           18    print.  But it is impressive that with the vaccine around 
           19    for as long as Hepatitis B has been around, that we still 
           20    have this many risk groups of folks. 
           21      I would like to talk about the second group a little 
           22    bit, infants born to mothers who are infected at the time 
           23    of the delivery.  You heard Dr. Seeff speak about people 
           24    who give blood for the very first time, and the blood 
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            1    banks screen for hepatitis.  In the United States, 
            2    pregnant woman are screened for Hepatitis B.  This is 
            3    another way people find out they have been infected. 
            4      The reason we screen pregnant women is that we have a 
            5    wonderful protocol in place.  If the infant is given the 
            6    first shot of the vaccine along with the H for the first 
            7    12 hours after the delivery, it will block the 
            8    transmission of the virus.  The important thing to note 
            9    about Hepatitis B is that the younger you are when you're 
           10    exposed to the virus, the more likely to be chronic as an 
           11    adult.  That's why we have really effective mandates for 
           12    Hepatitis B vaccine in the United States for school age 
           13    children in the United States.  This is just a list of all 
           14    the different ways you can get Hepatitis B. 
           15      Hepatitis C is a silent infection.  Most people don't 
           16    show symptoms.  The common symptoms are fever, fatigue, 
           17    muscle and joint pain, loss of appetite, nausea and 
           18    vomiting.  The more serious symptoms are the jaundice and 
           19    the bloating.  When it doesn't address in the common 
           20    symptoms is the chronic fatigue that we hear most patients 
           21    talk about.  We need medical experts to talk about the 
           22    level of chronic fatigue that these patients experience. 
           23      So what we really need to do is make the connection for 
           24    Hepatitis B and C that ultimately leads to liver cancer. 
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            1    We are marketing people that have done a great job in 
            2    letting us understand high blood pressure can lead to 
            3    heart attacks, smoking to lung cancer.  We need to make 
            4    the connection that both Hepatitis B and C cause liver 
            5    cancer.  I think there's an misunderstanding of what it is 
            6    among the men on the street. 
            7      The next two slides -- I have tell you when I looked at 
            8    the homework eye assignment for this presentation when you 
            9    talked about, you know, your listings.  I had one of my 
           10    medical advisors, Dr. Tom London, read over the listings, 
           11    and of course his comment was, "Boy, the criteria was 
           12    rather narrow to qualify for disability."  So I'm going to 
           13    plant the seeds among the medical people here.  This Child 
           14    Pugh Turcotte score, judging these five variables which 
           15    Dr. Seeff could identify better than I.  Based on these 
           16    five variables, the way it was explained to me, was that 
           17    the patients could be scored A, B or C.  Someone depending 
           18    on the severity with a score of C would get severe 
           19    cirrhosis already were labeled with disability and 
           20    limited.  The B score with moderate abnormal evaluations A 
           21    scored it may look similar on the slides than it is in 
           22    reality, I would guess.  But I wanted to put that up there 
           23    because that was his thought in the scoring system; that 
           24    may be a value for this screen. 
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            1      Next slide.  I want to take a moment and talk about the 
            2    API population, which is the Asian Pacific Island 
            3    population.  Dr. Seeff talked a bit in his presentation.  
            4    These folks are disproportionately affected by Hepatitis 
            5    B.  They would get ratings of about 15 percent of Asian 
            6    Pacific Islanders.  We're a global community, and we talk 
            7    about the immigration factor coming to the United States.  
            8    We're go to hear more, but 15 percent is a very high rate 
            9    of Hepatitis B.  They make up more than half of the 1.3 to 
           10    1.5 million known Hepatitis B carriers in the United 
           11    States, and 80 percent of the liver cancer in Asian 
           12    Americans is caused by chronic Hepatitis B. 
           13      The liver cancer rates among males are 13 times higher 
           14    in American Viet Namese, eight times higher in Korean and 
           15    six times higher in Chinese Americans than in the 
           16    caucasian Americans.  This is a sensor slide.  California 
           17    has the highest rate.  This is the distribution of the 
           18    Asian Pacific Islanders in the United States.  They say 
           19    with you our experts are.  I thought it was important for 
           20    you to know that because it's certainly an issue going 
           21    forward.  We're going to see more and more of. 
           22      The demand for information this is the Hepatitis B 
           23    Foundation web site.  We have over a million visitors to 
           24    our site about every 18 months, people looking for 
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            1    information.  We also have a help line as well as we have 
            2    developed a newsletter that tracks all of the drugs that 
            3    are not only approved for Hepatitis B but are currently in 
            4    a pipeline in a clinical trial.  On the right-hand side, 
            5    you will notice the language factors, what we have 
            6    developed is a Q and A format where people could come and 
            7    find answers to the most commonly requested information 
            8    and questions on Hepatitis B and translated into those 
            9    languages, and we have huge traffic on those sites since 
           10    we put them up.  So people are out there looking for 
           11    health information. 
           12      Next slide, please.  This is just a little bit and this 
           13    is the part of what I do, is manage outreach and education 
           14    programs for the Hepatitis B Foundation. 
           15      Next slide, please.  Worldwide, these are what the 
           16    numbers look like.   Two billion people have been infected 
           17    with Hepatitis B, between 350 and 400 million chronically 
           18    infected people.  This is a big problem that's not going 
           19    to go away in the near future. 
           20      Next slide, please.  This is a little  -- I would like 
           21    to leave you with this.  What it really is is all about 
           22    the patients.  For the last five years, the Hepatitis B 
           23    Foundation has hosted a patient conference for patients  
           24    and family infected by Hepatitis B.  We did it with the 
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            1    Asian Liver Center from Stanford last year in order to 
            2    take it from the Philadelphia area out to the west coast 
            3    where you saw that map that has the greatest population.  
            4    We're going to continue to do that and support these 
            5    families in any way we can.  If there's any question, I 
            6    would be glad to take any question you have. 
            7                            AUDIENCE:  Do you have a statistic 
            8    in the US on infants that are born with Hepatitis B from 
            9    mothers who have that?
           10                            MS. CONTI:  I don't, but I could 
           11    get it for you.  If you would send me an E-mail.  I have 
           12    that stat.  It's not in the memory bank today. 
           13                            MS. CONTI:  Chris, do you have it?
           14                            AUDIENCE:  Yes.
           15                            MR. JULIAN:  If you just would 
           16    just say your name for the court reporter, please.
           17                            MR. MUNEZ:  Glen Munez, National 
           18    (inaudible) Foundation.  What are some of the challenges 
           19    or difficulties that you face in addressing messages to 
           20    culturally diverse communities?  Of course, language is 
           21    one and some of the others, how do you address them?  
           22                            MS. CONTI:  It's challenging to go 
           23    into those specialties populations.  I don't have the 
           24    right face to do that.  We had a really strong success 
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            1    rate of finding partners that work in the community.  
            2    Korean population is a very, very religious community.  
            3    They look to the faith-based initiative to find answers to 
            4    their questions.   We could talk for a really long time 
            5    about the challenges, and translation is certainly one of 
            6    the answers if not all of the answers. 
            7      Over this past weekend, there was a conference held for 
            8    the Asian population and liver disease in New York, and 
            9    they're going to come up with a paper to address all of 
           10    this.  If you give me your information, I will make sure 
           11    you get it.  I think we need to look at these ethnic 
           12    groups together and no one easy answer.  There's something 
           13    like 50 or 60 groups lumped into that API population, so 
           14    it's really very unfair to lump them, but at the same 
           15    time, it's a learning experience that we're all going to 
           16    be embarking on even more in the future.  Thank you very 
           17    much. 
           18                            (Applause.) 
           19                            MR. PORRO:  Hello, my name is 
           20    Rodolfo Porro, and I have Hepatitis B. 
           21                            AUDIENCE:  Could you speak more 
           22    into the mike, please? 
           23                            MR. PORRO:  I would like to thank 
           24    Paul Kryglik. I would like to thank Paul Kryglik and Rona 
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            1    Harper for getting me here.  I'm grateful for the 
            2    opportunity to represent the patients' perspective on this 
            3    disease.  As I'm following some very knowledgeable folks, 
            4    I'm going to introduce what I can and learn a lot more. 
            5      Let me start by saying it would not be an overstatement 
            6    for me to say that the Hepatitis B support group has given 
            7    me hope for my future.   Four years ago, I was living in 
            8    New York City enjoying a slightly delusional acting career 
            9    which I found satisfying, but the running New York joke at 
           10    that time was, What do you do for a living?  Oh, I'm an 
           11    actor.  Really, what restaurant?  My restaurant was 
           12    located in the heart of Greenwich Village, and one day in 
           13    this restaurant on the stage of the restaurant, I was 
           14    serving beer.  It was a sports bar, Yankee hang out, and 
           15    as I was setting down the beer, and the table was covered 
           16    in a cloak of second-hand smoke, I suddenly felt dizzy; 
           17    and after that, I started to forget little things that 
           18    waiters need, like, your pen, your menu list, different 
           19    things about the menu.  So at the end of that day, I said 
           20    to myself, this is probably just a flu or something that's 
           21    going around.  But I was causing a heavy line in waiters 
           22    at the computer when I was trying to input the orders. 
           23      At the end of the day, I tried to get over this 
           24    situation to go to a bar and started to relax and one of  
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            1    my friends looked at me and said, "Why are your eyes 
            2    yellow?"  And it took me by surprise.  I guess he had had 
            3    experience with this before.  So he pointed in the 
            4    direction of the nearest sliding scale health center at 
            5    the time.  Indeed, I had chronic Hepatitis B, and that's 
            6    how I found out I had the disease, and four years since 
            7    then have been the worst years of my life and the best 
            8    years of my life at the same time. 
            9      The bargaining of chronic illness that's expounded on by 
           10    so many people is no less a new and a different 
           11    perspective everytime you wake up.  Sometimes the glass is 
           12    half full and sometimes it's half empty.  It's like a 
           13    roller coaster.  But when I went back to Miami where my 
           14    family was, I made a decision to leave New York. 
           15      The completely empty glass with the crusty bottom came 
           16    when they told me I had cirrhosis.  The first time I went 
           17    in for a biopsy  -- and I know Dr. Seeff spoke about the 
           18    biopsies.  There are complications sometimes that arise, 
           19    and in this particular instance, they made the mistake of 
           20    confusing my liver for my gallbladder, and they punctured 
           21    my gallbladder instead.  So I went slightly vasovagal 
           22    after that, a violent reaction when you're scared. 
           23      The second time, they did get it right, and the news was 
           24    wrong.  The news was advanced cirrhosis.  So then that was 
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            1    near the time of September 11th as I was recovering from 
            2    biopsy on the morning of September 11th, my little sister 
            3    called me and said, "Turn on your TV," and I did and saw 
            4    what we all saw that morning.  And so through this prism 
            5    of advanced cirrhosis and the turns out chance and 
            6    everything that happened in New York which is where I 
            7    lived, and it was my adopted hometown, I decided I needed 
            8    to ask for further help; and I decided that I was ready 
            9    for the most valuable tool, that chronic patients have 
           10    which is knowledge; and that's where the Hepatitis B 
           11    support group comes in, Steve Bingham and Sherry Martin; 
           12    and I would leak to give them kudos for everything that 
           13    they do in the group.  They provide a forum where I can 
           14    help other people and take the focus away from me. 
           15      After almost a year of highs and lows on the internet 
           16    with them and my adopted internet family where we 
           17    celebrated good lab results, and at the same time, they 
           18    picked me up after the diagnosis of advanced cirrhosis 
           19    which is a label that carries a lot of psychological 
           20    baggage to it.  
           21      I met people at the first reunion.  I met mothers who 
           22    had become virtual pioneers in research by themselves in 
           23    the field of Hepatitis B.  I met activitists from the HIV 
           24    communicaty who had been there before and were eager to 
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            1    show us the way.  I met transplant recipients who in turn 
            2    give us the gift of hope once again, and I met others like 
            3    myself who were learning the art of living at the moment 
            4    and enjoying the power of helping others at the same time. 
            5      I'm going to share a bit about one of these people that 
            6    I met.  His name is James Smith.  He's got a morbid sense 
            7    of humor.  He's a transplant recipient now.  When he wrote 
            8    some of these entries, he was waiting for his liver. 
            9      One of the things in my first experience with the 
           10    Hepatitis B Foundation, we were, sort of, subconsciously 
           11    telling each other, Hey, If you take this too seriously, 
           12    this stuff can kill you.  So a lot of us have a sense of 
           13    humor about our condition. 
           14      Jamie was among the list that I will call Binghams and 
           15    (inaudible), Steve Bingham.  He had Hepatitis B and C, 
           16    liver cancer lesions on his two lobes, the right and left, 
           17    coronary complications.  He had sleep apnea and 
           18    Parkinsonism and neurological issues from a car wreck in 
           19    '98.  In a letter to his family, Jamie wrote this, 
           20    "(Reading)" I have been pretty tired lately and have been 
           21    sleeping two hours for every one that I'm awake.  I have 
           22    trouble with thought and memory which is non-existent.  
           23    That's, pretty much, my life until I get a liver 
           24    transplant.  It's heartbreaking because I used to be such 
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            1    a hard worker.  I wish I hadn't had that last car wreck so 
            2    I could go back into construction. 
            3      "(Reading)" I see at least one doctor a week and take a 
            4    pharmacy load orally each day, including allergy pills 
            5    since I'm allergic to everything that grows and most 
            6    things that walk or fly.  It's a pity I didn't go into 
            7    pharmaceuticals like my sister and brother. 
            8      April 30th 2003, he writes, "(Reading)" Good news, they 
            9    haven't thrown dirt in my face yet.   I hope we can get 
           10    together before it becomes too late for some things.   I 
           11    just bought a violin.  I'm going to start lessons to keep 
           12    me occupied.  Since my surgery is in Nashville, I may get 
           13    to play with someone who can help me out musically. 
           14      April 10, '03, "(Reading)" I like Dr. Gordon, and I hope 
           15    he will remain as my surgeon but I may have to move to the 
           16    VA Transplant Program.  My neurological problems are 
           17    getting in the way of my job.  It's hard to do tech work 
           18    over the phone when you lose the ability to communicate. 
           19      May 10th '03, "(Reading)" I'm anticipating having my 
           20    transplant surgery.  They just told me (inaudible).  
           21    September 15th 2003, I stutter like crazy now.  All kinds 
           22    of trouble getting words out.  I'm still working on my 
           23    long-term disability.  I heard nothing so far.  I have to 
           24    call them, but that would curtail talking on the phone.  
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            1    At the present, it's hard to make myself understood.  It 
            2    took me 30 minutes to write this.  That is a daunting 
            3    task.
            4      January 8th '04, "(Reading)" I just got hope from 
            5    Vanderbilt University Medical Center.  I talked to the 
            6    lead surgeon on my transplant, and I'm on the list.  I'm 
            7    at the top of the list, too.  How long it will take is 
            8    anybody's guess.  It all depends on when somebody who is a 
            9    doner dies and has my blood type.  A geno type that has a 
           10    usable liver.  It could be five minutes, or it could be 
           11    five years. 
           12      March 11th, "(Reading)" It feels like I've been released 
           13    from stirrer, the pokey, Kusgau, the joint, take your 
           14    pick.  I'm finally home after spending most of this week 
           15    in the hospital with abdominal pain.  My stomach was 
           16    violently rejecting anything I put into it.  Even with all 
           17    the tests they performed, they could find no reason for 
           18    it.   The funniest was after they had admitted me, and I 
           19    have been vomiting for three days and had the runs for 14 
           20    hours, and I had dropped 20 pounds in the span of four 
           21    days.  They kept harping on me about giving them a stool 
           22    sample. 
           23      Jamie got his liver this year and he's doing fine.  One 
           24    of the issues we must consider is the difficulty patients 
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            1    have in going through the protracted process of qualifying 
            2    for disability while they were interned.  My lab results 
            3    right now consistently reflect an impaired liver, but 
            4    beyond that, I still lead a fairly active life.  However, 
            5    my energy levels are never predictable, and the peaks and 
            6    valleys in this chronic fatigue syndrome  -- as a matter 
            7    of fact, the majority of patients run from normal to not 
            8    being able to get out of the bed. 
            9      Chronic fatigue syndrome and depression are common 
           10    symptoms in the majority of the people that suffer chronic 
           11    hepatitis B.  They suffer from one or the other.   In my 
           12    opinion, they should be represented much more specifically 
           13    in the listings.  I believe that these functional 
           14    limitations are debilitating enough and also prevalent 
           15    enough that they merit conclusion or (phone ringing, 
           16    inaudible) Hepatitis B under recurrent or persistent 
           17    syndromes. 
           18      My proposed additions are symptoms that are in many 
           19    cases present many years before the currently proposed 
           20    listings of ascites can be documented.   To what extent 
           21    these functional limitations such as cognitive impairment 
           22    fatigue and depression, debilitating and recurring enough 
           23    to modify the burden of operational proof, if you will, 
           24    from patients who may feel like Jamie but whose life 
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            1    values do not reflect it.  That's the question that we 
            2    should be asking here today.  
            3      I do not think this is a slippery slope.  It is, 
            4    however, proactive and sensitive to the predictive course 
            5    of the disease and to the patient.  There are also 
            6    tangential issues that affect the outcome of narrowing the 
            7    listings.  We have learned in the HIV community what a 
            8    powerful role depression and fatigue play in predicting 
            9    the future course of disease.  By narrowing the listings 
           10    with the goal of proficiency, we may be compounding the 
           11    problem. 
           12      Hepatitis B patient and a writer  -- somebody I met at 
           13    the first conference I went to whose name is Will Green -- 
           14    described this very thing in his book Hepatitis B, The 
           15    First Year.  He wrote, "(Reading)" Many people with HBV 
           16    experience depression.  We can't ignore HBV's effect on 
           17    our ability to think clearly and positively.  Putting up 
           18    with daily fatigue can put a real damper on enjoying life.  
           19    Many of us with HBV experience the feelings of loneliness 
           20    and estrangement, and that isolation can render to 
           21    depression.  It's not an admissible process.  We simply 
           22    may not have the energy to keep up.  Our livers don't 
           23    allow our minds to fully function, so sometimes we can't 
           24    tell what's emotional and what's psychological and what's 
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            1    physical.  
            2      Symptoms like the one on the Hep B support group that is 
            3    called Rampon, another transplant patient referred to as 
            4    liver madness, wags and wain.  Dr. Gregory Everson, 
            5    Director of Hepatology at Colorado and Hedi Weinberg also 
            6    wrote in their book, "(Reading)" In my experience, these 
            7    symptoms rarely, if at all, correspond with the severity 
            8    of the disease as Dr. Seeff mentioned this morning. 
            9      Some of my patients suffer from extreme fatigue, but 
           10    have little injury to their liver.  Others with  
           11    aggressive hepatitis may not have any symptoms at all.  So 
           12    getting to the bottom of what a patient can engage in, 
           13    what kind of substantial gainful activity he can engage 
           14    in, should be a question.  Fatigue and depression should 
           15    be occupying center stage in your consideration for your 
           16    new listings. 
           17      In addition, the concept of a healthy carrier has now 
           18    been, pretty much, debunked.  So we regularly see patients 
           19    who are being told that carriers with similar connotation 
           20    to the healthy HIV patient.  However, these patients may 
           21    suffer sporadically from debilitating migraines, dizziness 
           22    and fatigue, or what we call in laymen's terms "brain 
           23    fog," upper right quadrant pain and undetected, elevated 
           24    markers for liver disease. 
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            1      So in dealing proactively with Hepatitis B, it's one of 
            2    the main viral causes globally.  It's only two percent so 
            3    far nationally in the United States, but it is a great 
            4    global challenge that we face.  Because of its many modes 
            5    of infectivity, its assault on the Asian community that is 
            6    disproportionate in comparison to the United States 
            7    general population, its potential for carriers to transmit 
            8    the disease for many years.  The insidious childhood 
            9    infections where the disease is often asymptomatic and 
           10    much more often leads to the chronic carrier state. 
           11      Three-quarters of the world's live in hot zones for 
           12    Hepatitis B, high infection rates.  So I've been able to 
           13    establish personally for myself that when I get 10 to 12 
           14    hours of sleep, I can only tell you what my personal 
           15    experience is.  Chances are good that my energy will 
           16    remain stable for most of the day.  Stress of any kind 
           17    throws that equation out the window.  My goal is to live 
           18    as much as possible in a stress-free bubble, desirable for 
           19    most but essential for me in order to keep my energy level 
           20    predictable and stable.
           21      Having Hep B gives me a clear focus as to what's 
           22    important in life, and the things I used to dismiss are 
           23    now invading my senses everyday almost.  I'm lucky about 
           24    that.  That's one of the good things about having a 
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            1    chronic illness, the challenging part is having that clear 
            2    vision and losing sight of it because of quality of life 
            3    issues.  That could be alleviated with a little help at 
            4    the right time, and I guess that's it. 
            5      If you have any questions for me.  Okay, thank you. 
            6                            (Applause.) 
            7                            MR. JULIAN:  At this point in 
            8    time, we would like to welcome Dr. Rosenthal, and he will 
            9    be delivering remarks by way of telephone conference.  Dr.  
           10    Rosenthal, are you on the line?
           11                            DR. ROSENTHAL:   Yes I am.  I can 
           12    barely hear you, though. 
           13                            MR. JULIAN:  We'll proceed.  We 
           14    can hear you extremely well, and you are miked into the 
           15    room. 
           16                            DR. ROSENTHAL:  Thank you very 
           17    much for allowing me the opportunity to make this 
           18    presentation to your policy conference, and I'm going to 
           19    be directing my remarks about chronic liver disease in 
           20    children.  I'm Phil Rosenthal, and I'm Professor of 
           21    Pediatrics and Surgery, University of California, San 
           22    Francisco. 
           23                            MR. JULIAN:  Your slides are now 
           24    on the screen.
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            1                            DR. ROSENTHAL:  Great.  On my 
            2    second slide which has an outline of what I hope to cover 
            3    with you in the next few minutes.  What I thought I would 
            4    do is give you the indications for liver transplant in 
            5    children, give you an idea of some of the chronic liver 
            6    diseases that I see as a practicing hepatologist.  I also 
            7    would like to discuss with you the criteria that we 
            8    utilize for liver transplants prioritization. 
            9      There are not enough organs to go around.  We actually 
           10    have to make decisions upon who is the sickest, and who 
           11    should get transplants.  I hope to focus on comments and 
           12    statements for new criteria that were sent to me and 
           13    answer to the post questions that I was asked to comment 
           14    on. 
           15      Next slide.  So what are the clinical indications for 
           16    liver transplantation in children?   Some of these 
           17    disorders are very straightforward, as liver failure which 
           18    could be from drug overdose or from mushroom eating.  We 
           19    also see metabolic diseases in children.  Some of those 
           20    are genetically based, urea cycle defects, Wilson's 
           21    Disease.  These patients present often with persistent 
           22    coagulopathy.  They have prolonged INRs, and they can 
           23    bleed profusely.  They can have recurrent GI bleeding as a 
           24    result of the scarring and fibrosis in the liver that goes 
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            1    on to cirrhosis, results in esophageal varices which can 
            2    hemorrhage at anytime and be life threatening. 
            3      Many of the patients can develop recurrent cholangitis, 
            4    inflammation, failure to treat itself.  This is often seen 
            5    in children in particular in disease biliary atresia where 
            6    there's a destruction of the connection between the liver 
            7    and the intestines, and a procedure called a (inaudible) 
            8    Procedure which is a portal enteroscopy where a piece of 
            9    bowel is used to make a conduit between the liver and the 
           10    intestines, and in that case infection can get up into the 
           11    liver causing inflammation, fever and abscesses of the 
           12    liver.  We also see patients with intractable ascites.  In 
           13    particular in children -- I want to make a point -- that 
           14    many of these children do not grow well.  We refer to them 
           15    as "values to thrive."  Also the quality of life in many 
           16    of these children is severely influenced by their chronic 
           17    liver disease. 
           18      Next slide.  Where are some of the specific indications?  
           19    Primary liver disease with life threatening complications, 
           20    I would consider the decompensated cirrhosis, GI bleeding, 
           21    ascites, liver synthetic dysfunction going so far as 
           22    chronic hepatic encephalopathy.  Indications for liver 
           23    transplant as I mentioned are acute liver failure, 
           24    metabolic liver disease. 
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            1      Next slide.  There are also secondary liver diseases as 
            2    patients live longer; for example, patients with cystic 
            3    fibrosis are able to have their pulmonary problems managed 
            4    appropriately, but they then develop secondary liver 
            5    disease, and we have transplanted several children as a 
            6    result of severe liver disease and as a result of cystic 
            7    fibrosis.  There's also a group of children that get liver 
            8    tumors and in particular hepatoblastoma is childhood of 
            9    liver tumor.  The preferred method of treatment is 
           10    resection, but not always is the tumor amenable to a 
           11    resection of part of the liver.  Sometimes we have to 
           12    remove the whole liver to cure these patients.  Also we 
           13    see in these children occasionally hepatocellular 
           14    carcinoma. 
           15      There are also non-progressive liver diseases that have 
           16    intolerable complications.  For example, we may have some 
           17    patients with progressive (inaudible) hepatocholestasis or 
           18    a disease (inaudible) syndrome where there's a lack of 
           19    enough bile ducts to get the bile flowing out.  I've seen 
           20    some children having a miserable life because of chronic 
           21    cholestasis; they just can't stop itching. 
           22      Next slide.  This is just a recap here of some of the 
           23    liver disorders that cause liver transplantation.  I'm not  
           24    going to go through all of this list.  The big groups that 
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            1    children primarily fall into are metabolic disorders which 
            2    are hereditary and cholestatic disorders.  There are some 
            3    patients even in children with Hepatitis B and C that go 
            4    on to require transplantation.  Other groups are neonatal 
            5    hepatitis which occurs in newborns and then autoimmune 
            6    hepatitis. 
            7      Next slide.  I also want to mention that some of the 
            8    liver transplants for metabolic diseases, we've made some 
            9    progress with these metabolic diseases, and one in 
           10    particular has to do with tyrosinemia where we actually 
           11    have drug therapy, a pesticide actually, and the name is 
           12    very long so I'll just refer to it as NTBC.  But this 
           13    therapy has revolutionized for pediatric hepatologists the 
           14    treatment of tyrosinemia because it can stabilize and 
           15    improve many of these patients, and the indications for 
           16    transplant in many of these patients is the risk for 
           17    adenoma/carcinoma developing. 
           18      Also bilirubin conjugation effect such as Crigler-Najjar 
           19    Sydrome Type 1 where patients lack the enzyme required for 
           20    the conjugation of bilirubin.  Urea cycle defects, we have 
           21    now discovered fatty oxidation defects meaning changes to 
           22    dehydrogenate (inaudible) dehydrogenation deficiencies and 
           23    then patients with glycogen storage disease where sugar 
           24    gets stored in their liver and can't get it out of their 
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            1    liver, and this causes injury to their liver, and patients 
            2    may result with -- depending on their type  -- with 
            3    hypoglycemia and seizures. 
            4      Next slide.  So what are some of the things that I do on 
            5    a daily basis for taking care of these chronic liver 
            6    disease patients who are awaiting transplant?  We need to 
            7    manage their complicated portal hypertension, episodes of 
            8    bleeding.  They may be developing ascites that needs to be 
            9    controlled.  We need to be focused on their nutritional 
           10    support because children have a small window when they 
           11    need to grow and they need to develop.  We also need to 
           12    aggressively manage any infections they may develop, as I 
           13    mentioned, cholangitis, peritonitis where their fluid in 
           14    the ascites becomes infected, and of course, I would be 
           15    loathed as a pediatrician to be sure, they need to 
           16    immunized.
           17      Next slide.  Moving ahead, I want to talk about liver 
           18    transplantation listing and waiting, and this UNOS which 
           19    is the united network for organ sharing which is the 
           20    national consortium that we utilize for allocation of 
           21    organs within the United States.   Next slide.  So all we 
           22    base the timing of when is it the right time to 
           23    transplant, and this is an issue that I face on a daily 
           24    basis.  Unfortunately, there are not enough organs to go 
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            1    around for children and the adults that need transplants, 
            2    so we have to prioritize.
            3      Next slide.  So the prioritization scheme is that 
            4    forwarded patients with liver failure get the highest 
            5    priority followed by patients with acute liver failure, 
            6    and then chronic liver disease is the next category. 
            7      In 1998 a minimal listing criteria was first first 
            8    defined by determining who was sick enough to require a 
            9    transplant and this utilized the scoring called "CPT" or 
           10    "Child Pugh Turcotte Score."  Next slide.  This score 
           11    takes into account several objective and subjective 
           12    issues, and these include serum albumin, serum bilirubin, 
           13    prothrombin time, on physical exam, the presence of 
           14    ascites and whether or not the patient is in a hepatic 
           15    coma if they are encephalopathic or not. 
           16      Next slide.  It became clear that this was not 
           17    necessarily serving the needs of all patients, and 
           18    decision was made to deemphasize waiting times because the 
           19    longer the person was put on the waiting list, the more 
           20    brownie points or the more priority they got to get 
           21    transplanted, and it was decided that another measure to 
           22    predict the risk of dying focusing on this illness 
           23    severity scale was the way to go, and therefore, the 
           24    patients were going to be prioritized with this system 
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            1    rather than how long they had been listed on the waiting 
            2    list to get an organ, and this was the development of a 
            3    current system, MELD and PELD. 
            4      MELD stands for model end stage liver disease and PELD 
            5    stands for pediatric end stage liver decease scoring.  So 
            6    the the MELD PELD listing criteria, this is a listing of 
            7    severity scale, and it's been proven to show that this is 
            8    a highly predicted the risk of dying from liver disease 
            9    for patients awaiting transplant.  Next slide.  MELD PELD 
           10    model variables as opposed to CPT score also includes 
           11    bilirubin and albumin.  For adult score, creatinine is 
           12    utilized.  This is not utilized in the pediatric scoring 
           13    system.  NINR is utilized. 
           14      The etiology of liver disease becomes important because 
           15    there are exceptions that indicate priority, gives 
           16    patients priority in the MELD PELD system.  In children, 
           17    the growth is factored into the PELD scoring system.  Next 
           18    slide.  There are some exceptions because certain diseases 
           19    are considered to be more life threatening for patients, 
           20    and therefore, patients with tumors especially in children 
           21    such as hepatocellular carcinoma and hepatoblastoma get 
           22    higher priority because the tumor metastasizes or spreads 
           23    out of the liver, and those patients will no longer be 
           24    candidates for transplantation. 
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            1      The patients with hepatopulmonary syndrome, this is a 
            2    term where fistulae develop in the lungs as a direct 
            3    result of portal hypertension and cirrhosis.  These 
            4    patients get priority.  In adult usually familial 
            5    amyloidosis gets the priority.  In children, patients with 
            6    urea defects and other metabolic disorders such as primary 
            7    hyperoxaluria where these patients often require liver and 
            8    kidney transplant get priority, and then patients with 
            9    Crigler-Najjar Type 1 where they can't conjugate bilirubin 
           10    and are severe risks for development of injury to their 
           11    brain disorder known as (inaudible), and they have 
           12    permanent brain damage, and they get priority exclusion 
           13    for transplantation. 
           14      Next slide.  I wanted to move on to make specific 
           15    comments regarding the actual introductory text for 
           16    childhood criteria.  Instead of reading these all over to 
           17    you, I would just mention them by number and may make my 
           18    comments about those pertinent sections.  If we start with 
           19    Section 2, I think this statement does not take into 
           20    consideration disorders such as storage disease where 
           21    cells may be enlarged and filled with products such as 
           22    Alpha 1 antitrypsin deficiency and glycogen storage 
           23    disease, and these patients can have chronic liver disease 
           24    and yet not have necessarily at any particular time liver 
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            1    cell necrosis or inflammation.  They may actually have  
            2    normal transaminase and still have injury occurring to 
            3    their liver, same with cystic fibrosis (inaudible) early 
            4    intervention.
            5      Also in this statement, there's a statement regarding 
            6    clinical manifestations may vary from asymptomatic to 
            7    incapacitation to liver failure.  I think it might be 
            8    important to define precisely what one means by liver 
            9    failure.  One should refer to synthetic dysfunction or INR 
           10    or encephalopathy as part of that definition.  Next slide.  
           11    The Section 2 continuing on, I would suggest removal of 
           12    the term, especially in childhood criteria, "alcoholic 
           13    hepatitis" and replace it with "toxin-induced hepatitis," 
           14    and here I would refer to poisoned mushrooms which can 
           15    cause liver failure and injury.  The next statement, I did 
           16    not have any comment on.
           17      Next slide, Section 2a, there's no mention at all in 
           18    these childhood criteria about poor school performance or 
           19    developmental delay which is, I think, important in their 
           20    assessment of children and their disability.   I also 
           21    think that instead of lower extremity edema, wording would 
           22    be peripheral edema.  
           23      Next slide, Section 2a, I would recommend that we change 
           24    abnormalities of liver enzymes to abnormalities of serum 
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            1    transaminase to be more specific, and that would replace 
            2    abnormal liver biopsy to abnormal liver histology on 
            3    biopsy; and I think we also should define abnormal 
            4    coagulation studies; and you may want to include prolonged 
            5    INR and thrombocytopenia among platelets which can be with 
            6    chronic liver disease as a result of hypersplenism 
            7    secondary to the cirrhosis in portal hypertension.
            8      Next slide, Section 2b, I think it might be worthwhile 
            9    to add in cholangitis and peritonitis to give an idea of 
           10    the severity and disability of these patients' impairment.  
           11    Next slide, 2c, I would again add cholangitis and 
           12    peritonitis, and I want to point out that not all patients 
           13    that have portal hypertension necessarily have chronic 
           14    liver disease.  In fact, the majority of patients that 
           15    have portal hypertension are secondary to a disorder known 
           16    as portal vein thrombosis where they get a clot in their 
           17    portal vein which results in the blood flow to the 
           18    intestines being unable to get into the liver, and 
           19    actually the liver histology on biopsy is normal.  So they 
           20    could have portal hypertension.  This is not due to 
           21    chronic liver disease. 
           22      Next slide, Section 2d, when you talk about massive 
           23    hemorrhage, you may want to consider documentation of the 
           24    hematocrit or a hemoglobin drop as part of that criteria, 
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            1    how many units of blood, for example, a patient required 
            2    to be transfused, how many cc per kilogram in a young 
            3    child.  Section 2e, I have no suggested changes on the 
            4    next line.  Moving on to the next slide, Section 2f, I 
            5    have no suggested changes.  Moving on to the next slide, 
            6    Section 2g, I have no suggested changes.  Moving on to 2h, 
            7    I would suggest that we remove crises for rejection and 
            8    replace this with the words of rejection episodes.  We may 
            9    want to consider a statement regarding degree of 
           10    rejection.  This is based on liver biopsy, and this could 
           11    be mild, moderate or severe, or you may want to include 
           12    consideration of the number of rejection episodes that are 
           13    documented by liver biopsy in this section. 
           14      Moving on to the next slide, this has to do with 
           15    listings, the chronic liver disease childhood criteria, 
           16    and 105.05.  I think here to demonstrate esophageal 
           17    varices besides endoscopy, you may want to change x-ray to 
           18    just imaging studies.  If you want to be more specific, 
           19    you could put in ultrasound or MR, CT or x-ray. 
           20      Moving on to the next slide regarding the ascites, I 
           21    suggest that you add medically prescribed treatment 
           22    instead of despite prescribed treatments.  Moving on to 
           23    the next slide, the question regarding treatment 
           24    modalities for chronic liver disease in children, many of 
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            1    them are similar to adults.  Some of them are different 
            2    because of the size of the children, for example, variceal 
            3    bleeding.  While banding has been shown to be the most 
            4    favored method of controlling variceal bleeding in 
            5    patients, for very small children, it becomes impossible 
            6    to do mainly because the instrument, itself, is too big to 
            7    get down into the esophagus to actually do banding, and in 
            8    smaller children we have to rely on the use of 
            9    sclerotherapy needles in order to control the bleeding 
           10    because the instruments are more amenable to be able to 
           11    get -- using the pediatric scope and needles to be able to 
           12    perform the procedure. 
           13      As with adults, some patients benefit from surgical 
           14    shunts to relieve the portal hypertension.  Occasionally, 
           15    in children, we do (inaudible) shunts or text procedures.  
           16    Some patients with massive variceal bleeding who are in 
           17    the intensive care unit may require octreotide infusion to 
           18    help control the bleeding, and in larger patients 
           19    sometimes as in adult use propranalol or other beta 
           20    blockers to control hypertension.  I'm loathed to use beta 
           21    blocker in small children because a small child who bleeds 
           22    as opposed to an adult, their response to bleed is to 
           23    actually increase their heart rate in an attempt to 
           24    circulate profusion of their blood.  By using beta 
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            1    blockers, the propranalol, the way that works is to lower 
            2    the hear rate.  You're taking away the sensory mechanism 
            3    in the ill child should they have a variceal bleed, and 
            4    therefore, in smaller children I'm loathed to use beta 
            5    blockers. 
            6      For encephalopathy or hepatic coma, we also use 
            7    lactulose or neomycin, and we'll restrict protein in the 
            8    diet.  Obviously besides decreasing the bone degenerating 
            9    lows from this diet, it also has effects on growth so 
           10    there has to be the balance of protein in the diet to 
           11    control encephalopathy and allowing the patient to be 
           12    anabolic, to grow.  Pruritis or itching in children can be 
           13    a huge problem.  No therapy is ideal.  We do use drugs 
           14    such as ursodeoxycholic acid, occasionally phenobarbital, 
           15    and an antibiotic, rifampin, to control this.  But usually 
           16    this is temporizing. 
           17      For the Ascites, we utilize diuretics and salt and 
           18    sodium restriction.  Sometimes we need to do albumin 
           19    infusions and diuretic therapy to control the ascites.  
           20    Severe ascites will have an effect on the development of 
           21    the child.  For example, a six-month old with massive 
           22    ascites besides the ascites pushing up on the lung making 
           23    it more difficult to breathe, it may not allow the child 
           24    to learn or sit up or roll over as a result of the 
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            1    ascites.  For the cholestasis in our patients, we utilize 
            2    vitamin supplementation A, D, E and K and triglyceride oil 
            3    is added to the diet for calories.  The MCT oil, the MCT 
            4    triglycerides do not require bile salts, for example, for 
            5    their absorption, and I want to reiterate that nutritional 
            6    support is critical in children to allow growth and 
            7    development. 
            8      There are patients that can have cirrhosis that can be 
            9    stable and go for years with cirrhosis and not 
           10    deteriorate.   Also there may be times when we can't do a 
           11    liver biopsy; for example, patients with massive ascites 
           12    with encephalopathy, coma and coagulopathy may be too sick 
           13    to obtain a liver biopsy.  There may be times when a 
           14    biopsy demonstrated metabolic disorder such as Wilson's 
           15    Disease, which copper therapy may make a patient 
           16    stabilize. 
           17      The patients with urea cycle defect which we need to do 
           18    a metabolic enzyme determination; otherwise, 
           19    histologically the biopsy may look perfectly normal.  
           20    Patients with PFIC, I mentioned Alagille Syndrome or, as I 
           21    mentioned previously, tyrosinemia. 
           22      Next slide.  Do our proposed listings accurately 
           23    describe most individuals with liver disease unable to 
           24    sustain work activity?  I think my suggested changes for 
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            1    children, their work is school or play or growth and 
            2    development.  I think these listings should satisfy those 
            3    requirements.   I also think consideration in some way to 
            4    incorporating the PELD score into the pre-transplant 
            5    evaluation, that these children should be considered.  
            6      The next slide I just put down model variables from a 
            7    previously slide I had shown earlier reminding you that's 
            8    it's bilirubin, albumin, INR, the etiology of liver 
            9    disease and the children growth and age that are important 
           10    in the PELD scoring system. 
           11      Next slide.  Are there disparities in access to 
           12    treatment for children which impact the severity of 
           13    treatment?  Well, answering this question, I'd say the 
           14    answer to this is, No, in general, most states and the 
           15    federal government do have programs in place to help 
           16    children with their chronic liver diseases who do not have 
           17    private insurance.  However, I would also mention that I 
           18    often see children that come in in severely bad shape and 
           19    this sometimes may be the result of both patient and their 
           20    families being unaware of the resources available in their 
           21    community or within their state.  So in general there are 
           22    things in place to help these patients not always, though, 
           23    do patients and their families take use of these 
           24    resources, and often times these patients come into the 
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            1    hospital severely debilitated states. 
            2      And I believe that was my last slide, and I will be 
            3    happy to answer any questions that might be pose.  Thank 
            4    you. 
            5                            (Applause.) 
            6                            MS. GORDOBIAN:  This is Judy 
            7    Gordobian, I'm with the federal government with Health 
            8    Resources and Services Administration, and thank you for 
            9    your presentation.  The question I had as we heard this 
           10    morning about adults with hepatitis and children, do 
           11    adolescents -- where do adolescents fall in terms of 
           12    diagnosis and treatment? 
           13                            DR. ROSENTHAL:  There are -- 
           14    Hepatitis B, I assume? 
           15                            MS. GORDOBIAN:  Yes.
           16                            DR. ROSENTHAL:  Chronic hepatitis 
           17    B in children, there are currently two approved drugs as 
           18    opposed to adults, where there's three approved drugs.  
           19    The children with Hepatitis B can be treated with standard 
           20    interferon therapy which requires three shot a week for 
           21    about six months of therapy which is no simple task for a 
           22    child or the use of oral (inaudible) therapy which is 
           23    daily pills or liquid.  The problem there is that most of 
           24    these therapies are not very effective in actually 
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            1    eliminating the disease.  It may keep it under control but 
            2    not eliminating it. 
            3      In adults, there's a third drug called Adeprivar which 
            4    is has been FDA approved for the use in adults.  It's 
            5    currently undergoing clinical trials for its use in 
            6    children.  So that's for B.  And as long as I'm on this 
            7    topic here, I will mention that children with chronic 
            8    Hepatitis C which are much less numbers than the adult 
            9    problem which is currently the leading indication for 
           10    liver transplantation in the United States, but it's not 
           11    the leading indication for children.  It's biliary 
           12    atresia. 
           13      Children with chronic Hepatitis C only have available to 
           14    them the old three time a week interferon in combination 
           15    with oral ribovar therapy.  Interferon is not currently  
           16    for use in children.  There's an NIH study that, 
           17    parenthetically, I happen to be involved with which will 
           18    be looking at the use of interferon and riboviron by for 
           19    children with chronic Hepatitis C. 
           20                            AUDIENCE:  (Inaudible.)  
           21    Washington, DC.  Dr. Rosenthal, you mentioned about the 
           22    availability of the federal and state programs to assist 
           23    kids and their families.  Do you know -- are they 
           24    available uniformly across the country; is it more 
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            1    difficult in rural areas or on reservations to find access 
            2    to those services?  What's the structure in place for 
            3    those types of services? 
            4                            DR. ROSENTHAL:  I think that it's 
            5    beyond the scope of what I personally do everyday.  To 
            6    answer that, I can tell you from my own experience from 
            7    California where I do get referrals from many of the 
            8    states around, and obviously there huge differences from 
            9    state to state.  The federal programs are for all the 
           10    states but actually how the states administer these 
           11    programs differs, and I would agree that access may be 
           12    very variable between whether or not one was in a rural 
           13    community or was one in a suburban or urban community,  
           14    also depending on the state, itself. 
           15      There are still disparities, obviously, but once 
           16    patients gets referred to a center as my own, then usually 
           17    they can access the resources that are available to them.   
           18    But resources do vary from state to state; that's the best 
           19    I can answer.
           20                            AUDIENCE:  Could you also address 
           21    what in terms of types of things that you're looking at in 
           22    terms of school setting, playground, what are the types of 
           23    problems you would expect children with chronic liver 
           24    disease to have?
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            1                            DR. ROSENTHAL:  Well, for example, 
            2    a child going to school who is yellow is going to be 
            3    ostracized.  I've had patients with severe cholestasis and 
            4    Alagille Syndrome who are itchy all the time.  They can't 
            5    sit still in their class.  They're shorter and smaller 
            6    than other kids, getting ostracized because of that.  They 
            7    may be scarred because of itching.  They may have a big 
            8    spleen because of portal hypertension which doesn't allow 
            9    them to participate in activities because their platelet 
           10    counts may be low, and there's concern about getting 
           11    trauma to their spleen which may cause a bleed or banging 
           12    their head, so they may be limited in their physical 
           13    activities.  Those are some of the issues that I've seen 
           14    in children that are school age. 
           15                            AUDIENCE:  Would there be mental 
           16    concentration type of issues, too?  
           17                            DR. ROSENTHAL:  If they have 
           18    encephalopathy, absolutely.  This may be demonstrated by 
           19    the fact that they're not able to concentrate very well.   
           20    They may not do math processing very well.  For example, 
           21    in adults, the classic story is is an accountant who can 
           22    no longer add his numbers when they get encephalopathic?   
           23    The same occurs in children. 
           24      In fact, there's been very little study in children with 
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            1    liver transplants and quality of life, and there's now 
            2    some studies through NIH sponsored group called Blitter 
            3    (taken phonetically) Studies in Pediatric Liver 
            4    Transplantation that I'm involved with which are going to 
            5    be looking at the quality of life of children with liver 
            6    transplants.  One of the problems there is that previously 
            7    everyone is focused on the fact of just getting these 
            8    children to live.  Whether they have continued disability 
            9    or not hasn't been previously focused upon.  One more 
           10    question. 
           11                            AUDIENCE:  Are there drugs that 
           12    children take either before transplants or after; what 
           13    kind of side effects do they have?
           14                            DR. ROSENTHAL:  Well, there's a 
           15    lot of drugs, so therefore, they have lots of side 
           16    effects.  I'll try to batch them together.   Prior to 
           17    transplant, many of the drugs that we utilize to try to 
           18    keep things in control; for example, steroids which is one 
           19    of the biggies used before and after transplant causes 
           20    weight gain; causes one to eat a lot; can cause hairiness; 
           21    can cause buffalo hump; can cause diabetes; can cause 
           22    streaks on the abdomen; can cause acne.  So those drugs 
           23    have major side effects. 
           24      After transplant, many of the drugs, depending on the 
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            1    unit of suppressant we need to use because we need to keep 
            2    the children from rejecting their organ.  Some of the side 
            3    effects that we're seeing now is children who have 
            4    long-term transplants have kidney injury.  Some of my 
            5    children are not only now requiring -- having had a liver 
            6    transplant, they now require kidney transplants.  Some of 
            7    the drugs can affect your bone marrow.  You have more risk 
            8    of infection after transplant. 
            9      With liver transplant, we're always trying to balance 
           10    the issue of too much immunosuppression causing rejection, 
           11    causing infection, as opposed to not allowing the patients 
           12    to have rejection and potential loss of their grasp. 
           13                            AUDIENCE:  Thank you. 
           14                            MS. TORES:  This is Nancy Tores 
           15    with the Office of Medical Policy. 
           16                            DR. ROSENTHAL:  Hi, Nancy.
           17                            MS. TORES:  Hi, and your 
           18    presentation was wonderful.  I want to thank you.  On one 
           19    of your suggestions on esophageal varices, you said you 
           20    might consider the amount of blood transfused.  In 
           21    children given the different sizes of children, how would 
           22    you suggest that we do that?
           23                            DR. ROSENTHAL:  I guess you didn't 
           24    hear my quick comment because I was rushing to make sure I 
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            1    got it done.  Actually, the way we transfuse little 
            2    children is by the number of MLs or CCs per kilogram.  So 
            3    a major bleed may be 10 to 20 CCs per kilo of blood 
            4    transfused, so we can quantitate the number of CCs per 
            5    weight of the child, an indication on how much bleeding 
            6    was going on. 
            7                            MS. TORES:  Thank you.  I have a 
            8    second question.  Can a laboratory finding alone such as 
            9    serum bilirubin be an accurate measure of functional 
           10    ability. 
           11                            DR. ROSENTHAL:  Absolutely not.  
           12    One of the reasons why bilirubin is in the criteria for 
           13    PELD scores for children is the fact that bilirubin is a 
           14    very good discriminator, but there are hereditary 
           15    disorders, bilirubin metabolism.  There are several 
           16    syndromes known as Crigler-Najjar Syndrome, Type 1, where 
           17    I mentioned they lack the enzyme to conjugate bilirubin.  
           18    There are also unconjugated hyperbilirubin (inaudible) 
           19    Crigler-Najjar, Type 2.  There's a syndrome called 
           20    "Gilbert's Syndrome" which actually five percent of the 
           21    population have, normal population, where they do not 
           22    conjugate bilirubin very well where they would have 
           23    normally elevated serum bilirubin, but they may not be 
           24    apparent with jaundice.  They may not be yellow, but their 
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            1    bilirubin will be above the normal range.
            2      There are also hereditary disorders.  They're very rare, 
            3    conjugated hyperbilirubin such as Dubin-Johnson Syndrome 
            4    and Rotor's Syndrome, so just having an elevated 
            5    bilirubin, just that criteria alone for determination of 
            6    liver disease would be very wrong.  In fact, I've had many 
            7    patients with cirrhosis and severe disease who have normal 
            8    bilirubin.  It's not bilirubin alone used for scoring 
            9    system. 
           10                            DR. HETLAND:  This Dr. Hetland, 
           11    I'm a pediatrician in Social Security.  Thank you, Dr. 
           12    Rosenthal, for your presentation.
           13                            DR. ROSENTHAL:  Sure.
           14                            MR. HETLAND:  I saw some quizzical 
           15    looks when you were talking about banding of esophageal 
           16    varices in the audience.  Could you explain what that is, 
           17    and why you can't do it in small children, and then what 
           18    happens to these kids? 
           19                            DR. ROSENTHAL:  Banding is 
           20    basically using rubber bands.  Again, I realize that many 
           21    of the people in the audience are not all medically 
           22    sophisticated.  You may have heard that sometimes adults 
           23    that have hemorrhoids get rubber band ligation or rubber 
           24    bands put on for their hemorrhoids.  The same idea is for 
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            1    patients that have been esophageal varices.  Those are two 
            2    big veins in the portal system.  Again, to review anatomy, 
            3    all of the feed that we eat goes from our intestines to 
            4    the liver to the portal system.  If that venous pressure 
            5    is too high, those veins are going to pop, and what you 
            6    see is varices ballooning out of the veins usually in the 
            7    lower part of the esophagus. 
            8      What we can do is put an endoscope down which is a tube 
            9    with a flashlight and through a channel use an instrument 
           10    that has a needle so we can inject the vein with a 
           11    sclerosant which would make it harden up and keep it from 
           12    bleeding, and if a patient is big enough, we place a six 
           13    shooter over the scope which has rubber bands on it, and 
           14    we can actually suck up the vein and place a rubber band 
           15    around that vein which would cause a clot to form and 
           16    falls off and passes out into the stool, and that's one of 
           17    the ways we can control the bleeding from the esophagus. 
           18      The deal here is the way the instruments are made, you 
           19    have to have an esophagus wide enough to be able to pass a 
           20    scope with a six shooter at the end of it so you can do 
           21    the band ligation, and in very small children, less than 
           22    two or three years of age, it's just technically 
           23    impossible to get the instrument in to do the band.  
           24    Therefore, we have to rely on sclera therapy which is the 
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            1    older way before the band ligation instruments came along 
            2    to actually inject with a needle into those vein to get 
            3    them to sclerose, to clot, to stop bleeding. 
            4                            DR. HETLAND:  Thank you.  You had 
            5    a suggestion about that we should consider the degree of 
            6    rejection on liver biopsy, mild, moderate and severe.  Are 
            7    there, like, national standards for what those terms mean?
            8                            DR. ROSENTHAL:  There are 
            9    histologic standards.  The diagnosis of rejection on a 
           10    biopsy is based upon very specific criteria that a 
           11    pathologist will read.  Just like on a biopsy, there are 
           12    scoring systems for the amount of inflammation or scarring 
           13    based upon a person with Hepatitis C.  There are several 
           14    different scoring systems. 
           15      Pathologists have a standardized way of grading the 
           16    amount of rejection depending on how many bile ducts are 
           17    affected on the biopsy.  If there are a few affected, are 
           18    they all infected, or are there other findings such as 
           19    endotheliitis and injury to blood vessels themselves.  
           20    That could be utilized in determining  -- and in fact is 
           21    utilized in determining the severity of the rejection 
           22    episodes. 
           23                            DR. HETLAND:  This is a request.  
           24    We already have Dr. Rosenthal's slides, but I'm going to 
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            1    request that we also receive the power point presentations 
            2    for those of you that did.  And also, Mr. Porro, I would 
            3    like to have a copy of what you spoke about because I 
            4    would like to read about what your friend wrote about in 
            5    his diary, thank you. 
            6                            DR. SCHUSTER:  This is Dr. Frank 
            7    Schuster; I'm a child neurologist who works with Social 
            8    Security, one of the medical offices at the Social 
            9    Security Administrations, and I wonder if Dr. Rosenthal 
           10    can comment on the -- well, we used to call it years 
           11    ago -- and I go back a few years  -- Reye's Syndrome where 
           12    we used to lose most of our patients.  They would present 
           13    with elevated ammonia levels and so forth, and I don't 
           14    know where that stands at the present time.  Could you 
           15    comment on that for me?  
           16                            DR. ROSENTHAL:  Sure, I'd be happy 
           17    to.  Reye's Syndrome is a fatty liver.  It's 
           18    microventricular fat with encephalopathy, and in the past, 
           19    this was seen much more common after a flu-like illness or 
           20    chicken pox in a child and could be devestating.  Usually 
           21    the liver would recover over time, but the neurologic 
           22    injury would be so severe, that often these patients would 
           23    die.   There seems to be a link -- though it's never been 
           24    unequivocally established -- between the use of aspirin in 
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            1    patients during chicken pox or flu, and in fact, you're 
            2    probably quite aware that there are lots of 
            3    recommendations by pediatricians that children get a 
            4    fever, that we not use aspirin but instead use 
            5    acetaminophin or ibuprofen for fevers. 
            6      With that switch in the US from the use of aspirin to 
            7    other medications for fever control in children, there's 
            8    been a drastic decline in Reye's Syndrome.  I don't think 
            9    I've seen a Reye's case in the last five to 10 years.  So 
           10    Reye's has pretty much gone away; though I would suggest 
           11    it's not completely gone. 
           12                            DR. HETLAND: Are there other 
           13    questions from the audience?  Okay.  I have one more for 
           14    Dr. Rosenthal.  Dr. Seeff introduced me to a new syndrome 
           15    that I'm not familiar with called "NASH"; is that correct?
           16                            DR. ROSENTHAL:  Ah hah.
           17                            DR. HETLAND:  Are you seeing that 
           18    in overweight adolescents?
           19                            DR. ROSENTHAL:  Absolutely, I have 
           20    one of about seven or eight centers in the United States 
           21    that are NIH funded where we're studying NASH in both 
           22    adult and children.  With the obesity epidemic in the 
           23    United States, I can tell you that NASH has become a major 
           24    problem.  NASH stands for Non-alcoholic Sciatal Hepatitis.  
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            1    It's part of a spectrum of non-alcoholic fatty liver 
            2    disease which was first discovered in the '80s by an adult 
            3    hepato pathologist at the Mayo Clinic who noticed that 
            4    many patients had very similar findings as alcoholic 
            5    hepatitis patients did where they would get fat in their 
            6    liver, but these patients were clearly not abusing 
            7    alcohol.  That's where the term came in, non-alcoholic 
            8    hepatitis came in. 
            9      We learned that one can have fat in their liver, can get 
           10    fat in their liver and inflammation in their liver all the 
           11    way up to cirrhosis, and it's believed that many patients 
           12    with NASH may actually go on to require liver 
           13    transplantation.  I'm seeing many, many patients was NASH 
           14    weekly, and hopefully we're going to be able to develop 
           15    some good medical therapies besides exercise and diet 
           16    which I'm sure you're all aware, any of you who have 
           17    attempted to doing that; that that's not a good, long-term 
           18    solution to this problem.  NASH is becoming a major public 
           19    health epidemic in the United States at the present time. 
           20                            MR. SKLAR:  Dr. Rosenthal, this is 
           21    Glenn Sklar from the Social Security Administration in 
           22    Baltimore.  Let me thank you for braving the wideworld of 
           23    technology.  It worked wonderfully.  
           24                            DR. ROSENTHAL:  Great.
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            1                            MR. SKLAR:  At this point in time, 
            2    we're going to segway to the full panel, mainly because 
            3    there are a lot of crossover issues, and also this can 
            4    certainly be addressed by other members of the panel as 
            5    well.  You mentioned the MELD PELD and CPT scores 
            6    particularly in children; would those be relevant at all 
            7    for adults as well?
            8                            DR. ROSENTHAL:  I can answer you 
            9    quickly MELD is specifically for adults and PELD is 
           10    specifically for children.  CPT scoring was the old method 
           11    we used for allocation of organs.  It's good criteria for 
           12    assessing severity of liver disease, but I don't think 
           13    it's good enough to determine the severity of who should 
           14    get transplanted.
           15                            MR. SKLAR:  Now in our particular 
           16    situation, we have an unusual standard.  We have an 
           17    inability to perform what we call substantial gainful 
           18    activity, essentially to function in the work environment, 
           19    without worrying with the legal terminology; to what 
           20    extant would it be relevant for us as we determine 
           21    medically whether someone can actually perform substantial 
           22    gainful activity from just a medical perspective.
           23                            DR. ROSENTHAL:  To answer that 
           24    from my standpoint as a pediatric hepatologist, a child 
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            1    can't go to school and be a student.  It has to be  home 
            2    bound and home school as a result of that; that's 
            3    disabling because they don't have the intersection of 
            4    their peers; they don't have their whole school 
            5    environment which I think is critical for the development 
            6    of that child; and I think that will be one of the 
            7    important things to document. 
            8      My purpose in transplanting children is getting them 
            9    back to having a perfectly normal life.  The children who 
           10    get transplanted can go to school, can do most anything, 
           11    win gold medals, be in the Olympics.  Children with 
           12    chronic liver disease don't have the opportunity to do 
           13    those kinds of activities, and that's what makes them 
           14    disabled. 
           15                            DR. SEEFF:  Phil, can you hear me? 
           16                            DR. ROSENTHAL:  Yes.
           17                            DR. SEEFF:  This is Leonard.
           18                            DR. ROSENTHAL:  Yes, hi, Leonard.
           19                            DR. SEEFF:  I actually wanted -- 
           20    you did a much better job than I did.  I didn't sit down 
           21    and go through those listings and you did, and I think 
           22    that's terrific, and I would like to go through these 
           23    listings word by word and see what you put in here; and 
           24    why you put them in here; and why you have taken some 
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            1    things out; and I don't know whether we should do that now 
            2    and I'm now referring to the adult rather than pediatric 
            3    set of listings. 
            4                            DR. ROSENTHAL:  I personally 
            5    didn't do that to the adult.
            6                            DR. SEEFF:  Sorry, I didn't follow 
            7    up on this.  It was sent to me but I mislaid it.  I wonder 
            8    whether we can read through this and see whether.
            9                            AUDIENCE:  I would like to point 
           10    out that there's a comment period that's expiring in 
           11    January of '05.  We would all like to go through this, 
           12    too, if we don't go through it all, you have an 
           13    opportunity.
           14                            DR. SEEFF:  I'm looking at the 
           15    proposed criteria on chronic liver disease on the 
           16    right-hand side of the blue sheet, and there's not a heck 
           17    of a lot there.   Sounds as if we need a break.  We have 
           18    to take a break.  The court reporter needs a little 
           19    sustenance. 
           20                            DR. ROSENTHAL:  My one limited is 
           21    that I have to leave in another 20 minutes.  I have two 
           22    liver biopsies I have to do this morning, so I've got 20 
           23    minutes.  However long your break is, I've got to leave at 
           24    8:30.
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            1                            DR. SEEFF:  Have you completed 
            2    your assessment of the listing for the pediatrics?
            3                            DR. ROSENTHAL:  I believe I have.
            4                            DR. SEEFF:  I was hoping to focus 
            5    on the adult, and so while you would be a tremendous 
            6    support, we have some other hepatologists who are much 
            7    smarter than I am who will help out, I'm sure.
            8                            DR. ROSENTHAL:  Can I sign off?
            9                            DR. SEEFF:  I don't want to 
           10    control this.  Let me hand it over.
           11                            MR. SKLAR:  Dr. Rosenthal, thank 
           12    you, it was a terrific presentation.  In light of the 
           13    technology, we're stretching the criteria.  I think we 
           14    have one final comment now as well.  We'll close out your 
           15    piece and take it back to the full panel after a short 
           16    break.
           17                            MS. KUKKA:  Hi, this is Christine 
           18    Kukka.  Hi, Phil.
           19                            DR. ROSENTHAL:  Hi, Chris.
           20                            MS. KUKKA:  I have a question 
           21    which is rather theoretical.  I know your task at hand was 
           22    more on the liver transplant, but if a child did not 
           23    present with cirrhosis but yet had sustained elevated 
           24    ALTs, required treatment, received interferon and 
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            1    experienced some pretty tough side effects from 
            2    interferon, that treatment was very much needed, had to be 
            3    home schooled, could not develop, even though it's not a 
            4    liver transplant scenario, could that not qualify the 
            5    child for some disability given the quality of life, 
            6    social development, school experience is basically robbed 
            7    by virtue of the treatment?
            8                            DR. ROSENTHAL:  What I would say 
            9    to you, having read over the criteria very carefully, the 
           10    definition of chronic disease -- I would say this is a 
           11    disability, but I would say this is acute disability to 
           12    respond to your question and not the issue of chronic 
           13    disability which is what SSA folks are tying to define.  
           14    It wouldn't be treatment alone that would qualify one for 
           15    chronic disability.  It would have to be long-term side 
           16    effects of all of these things, and usually most patients 
           17    that get interferon, for example, while they can have 
           18    disabling side effects, they are acute and go away when 
           19    interferon is stopped, and nobody is going to be on 
           20    long-term interferon therapy, certainly not a child. 
           21                            MR. SKLAR:  I think at this point, 
           22    we'll take a 15 minute break.  Thank you, Dr. Rosenthal, 
           23    we appreciate your time to be with us today, and we'll 
           24    reconvene at 11:30.  It would be a wonderful thing if Dr. 
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            1    Seeff needs a discussion through the adult listings, we 
            2    appreciate that, and it will be very helpful to us.
            3                            (A short recess was taken.)
            4                            DR. SEEFF:  We're going back on 
            5    the record.  We can get started.  I don't want to be 
            6    presumptuous because obviously this was written by other 
            7    people, and I was invited as a guest to make comments.  
            8    What I would like to do, if this is acceptable, is to read 
            9    through the set of criteria that we have here.  I worked 
           10    for Dr. Jay Huffnagle whose name everybody knows.  You may 
           11    not know it, but Jay was my fellow.  He's now my boss, and 
           12    if I have learned anything from him over the years I've 
           13    worked with him is that he's meticulous. 
           14      If there are going to be things that are written down, 
           15    that people are going to have to read, we did this with 
           16    the consensus conference, with this liver cancer meeting.  
           17    Every word has meaning, and my sense is there are things 
           18    that I don't fully understand, and as long as I'm not 
           19    stepping on toes, I'd like to read through it and see how 
           20    you feel about it and see how these statements came about, 
           21    and what's in and what's out. 
           22      I didn't read this document, but I think I just, sort 
           23    of, glanced through it.  You might want to pay attention 
           24    to Page 57012, and under proposed 500D, two lines down, 
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            1    you see the word "site" S-I-T-E; it should be C-I-T-E.  
            2    For example, we cited encephalophathy; that's one comment 
            3    I wanted to make. 
            4      As Phil was going through this, I was thinking, Should I 
            5    have gone through each of the proposals that now exist and 
            6    I should have.  When I started looking at it, I began to 
            7    wonder if we can start with this blue sheet which is 
            8    proposed adult digestive listings and as I understand it, 
            9    the left is what used to exist, and the right is the plan 
           10    for the future.  If that's correct, I would like to start 
           11    from the beginning. 
           12      I would like to begin with the heading "Chronic liver 
           13    disease and cirrhosis of any kind."   I assume in order to 
           14    get to that, you have to go through this green document to 
           15    see what you mean by chronic liver disease.  What 
           16    precisely is chronic liver disease?  So you have to go to 
           17    this blue to see what you mean by chronic liver disease.  
           18    Let me suggest the following, and perhaps the panel might 
           19    want to comment on it. 
           20      There are two things discussed here when you talk about 
           21    chronic liver disease.  One is chronic liver, which 
           22    incorporates cirrhosis, and the other one is before 
           23    cirrhosis develops.  The hepatologic definition is 
           24    persistence of abnormal enzymes, and these can be ASTs or 
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            1    a ALTs, alamine amino transferase, the aspartame amino 
            2    transferase or the SGLT or SGPT for six or more months 
            3    which would define the necro inflammatory forms of disease 
            4    or the persistence of alkaphosphatase for six or more 
            5    months, and that's chronic liver disease. 
            6      Then we get to cirrhosis and cirrhosis is different from 
            7    chronic liver disease.  It's the end stage of chronic 
            8    liver disease.  The question is, What are the criteria, 
            9    and do we have criteria for listing cirrhosis, and how do 
           10    you we get to cirrhosis?  And I don't know that's the 
           11    case.  Maybe it is. 
           12      I also believe that we should be talking about two forms 
           13    forms of cirrhosis.  One is compensated cirrhosis, and the 
           14    other is decompensated cirrhosis.  There these are two 
           15    different entities.  Compensated is something that you can 
           16    live with forever.  You can die of heart disease with 
           17    cirrhosis, and you can die of an automobile accident with 
           18    compensated cirrhosis and not even know about it.  When it 
           19    becomes decompensated, that the problem of liver disease 
           20    arises.  
           21      How do we define decompensated?  There are three ways 
           22    maybe.  One is that there's biochemical dysfunction; that 
           23    is, persistent hyperbilirubin, increase in bilirubin 
           24    values in a patient with cirrhosis that's not accounted 
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            1    for by two issues.  One is hemolysis, destruction of red 
            2    cells that may lead to increase in bilirubin or a genetic 
            3    condition called "Gilbert's Syndrome." So if you have a 
            4    bilirubin greater than 2.5 and you have cirrhosis, this is 
            5    one way of defining decompensated liver disease. 
            6      The other one is low albumin or low platelet count.  So 
            7    I think, to me, if I had done this, I would try to define 
            8    and distinguish between compensated and decompensated.  
            9    They may be weighted different, and they may have 
           10    different connotations.  Certainly somebody with 
           11    decompensated cirrhosis is much closer to death than 
           12    somebody that has compensated cirrhosis. 
           13      When it comes to the heading, the question is, the 
           14    question of chronic liver disease, how we define it, and I 
           15    would hope that it's easier than it is for me to be able 
           16    to look at that heading and say, I know what we mean when 
           17    we talk about chronic liver disease.  I know what we mean 
           18    when we talk about cirrhosis. 
           19      My suggestion, I would separate these into two 
           20    manifestations, the earlier and the later manifestation.  
           21    If we have agreed on what we call chronic liver disease 
           22    and how we define cirrhosis, then the listing is chronic 
           23    liver disease and cirrhosis of any kind with (a) 
           24    esophageal varices demonstrated by x-ray, endoscopy or 
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            1    other appropriate medically extensive imaging.  Is that 
            2    enough; is there anything else to use to describe varices?  
            3    So that's okay. 
            4      With massive hemorrhage attributable varices which 
            5    requires a transfusion of at least five units of the blood 
            6    in 48 hours.  That's the issue that we hepatologists are a 
            7    little uncomfortable about.  We're not sure where that 
            8    number came from, and in fact Dr. Norman Grace,  who is on 
            9    my left representing the ASLU and ALS, is one of the 
           10    world's experts in portal hypertension and has written 
           11    guidelines about this, and I have asked him where this 
           12    information of five units of blood in 48 hours came from, 
           13    and his view is a little different.  And perhaps you would 
           14    like to comment about that. 
           15                            DR. GRACE:  Thanks Leonard.  Can 
           16    you here me okay?  There's a consensus conference that 
           17    occurs every five years called Boveno Conference held in a 
           18    very nice part of Italy, and every five years, we get 
           19    together and define criteria for portal hypertension which 
           20    is decompensated cirrhosis, and a criteria we use for a 
           21    significant variceal bleed is two units or greater.  So 
           22    I'm no not sure where the five came from, but it's 
           23    certainly not anything internationally that we use. 
           24                            DR. SEEFF:   Is it a time 
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            1    interval?  It's associated with a bleed.
            2                            DR. GRACE:  Usually within a 
            3    five-day interval. 
            4                            DR. SEEFF:   Do you have any 
            5    comments.
            6                            DR. SHERKER:  Not all portal 
            7    hypertensive bleeding has varices.  One thing that is not 
            8    accounted for here is borderline hypertensive gastropathy 
            9    which may have a significant morbidity even recurrent 
           10    disability more so than acute variceal bleeding.
           11                            DR. GRACE:  That's a good point.  
           12    It says esophageal varices when five percent of patients 
           13    bleed from gastro varices where they may have small or no 
           14    esophageal varices, and that's just as life threatening as 
           15    esophageal variceal bleeding.  Secondly, with the current 
           16    treatment particularly esophageal banding or variceal 
           17    ligation, esophageal varices can be obliterated and later 
           18    on because the portal hypertension persists, patients can 
           19    bleed from other sites.  So to confine it to esophageal is 
           20    not correct.
           21                            DR. SHERKER:  The condition is 
           22    really portal hypertension, and esophageal varices and 
           23    esophageal variceal bleeding is a manifestation of portal 
           24    hypertension. 
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            1                            DR. SEEFF:   Do you think we need 
            2    different wording and if there's a general agreement that 
            3    the words "esophageal varices" be struck or that we use 
            4    esophageal varices or gastro  --
            5                            DR. GRACE:  I think the term that 
            6    I would like to see is portal hypertensive bleeding.
            7                            DR. SHERKER:  And one of those for 
            8    hypertensive bleeding could be esophageal bleeding.
            9                            DR. SEEFF:  When you say portal 
           10    hypertensive bleeding, for example, I think you need  --
           11                            DR. GRACE:  That would include 
           12    esophageal varices, gastro varices, portal hypertensive 
           13    gastrophathy.  Some patients can bleed from the duodenum 
           14    varices, rectal varices, renal varices, involves the whole 
           15    GI tract, and particularly, as I say, with the current 
           16    therapies, endoscopic therapies, that are aimed at the 
           17    esophagus, we're seeing more and more bleeding from other 
           18    sites. 
           19                            DR. SEEFF:   Does this overstep 
           20    our bounds?  Keep going, okay.  We won't change it to say 
           21    portal hypertension --
           22                            DR. GRACE:  Including esophageal 
           23    varices and gastro varices.
           24                            DR. SEEFF:  Identified by x-ray, 
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            1    endoscopy or other --
            2                            DR. GRACE:  I would put endoscopy 
            3    first because that's by and large the method we most 
            4    commonly use.  X-ray is really  -- barium studies are not 
            5    helpful.  Angiography is sometimes used, but 95 percent of 
            6    diagnoses are made by endoscopy.   Can I go back a little 
            7    bit? 
            8      I think in terms of compensated and decompensated, we 
            9    have a system that Dr. Rosenthal referred to the child's Q 
           10    classification which has been validated for years as 
           11    predictive of outcome.  It's been refined with a scoring 
           12    system assigning points for various criteria, one to three 
           13    points for each of the five components.  A child's A would 
           14    be five to six points; child's B would be seven to nine; 
           15    and child C, 10 to 15.  This is very validated system, and 
           16    certainly I think could better define cirrhosis if you 
           17    want to have a specific quantitative system for doing it.
           18                            DR. SHERKER:  The one issue that I 
           19    think is probably underscored, the one process 
           20    underscored, in the CPT score is cholestatic liver 
           21    (inaudible).  It's appropriate to use the CPT score or 
           22    MELD or both as indicators.  I think we should resist 
           23    being exclusive in tying people to that because there are 
           24    disabilities and won't be picked up by that. 
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            1                            DR. SEEFF:   As things stand at 
            2    the moment, the MELD and PELD scores are not in the system 
            3    at all.  They have not been referred to our even CPT 
            4    score.  I don't know whether you wanted to include that.  
            5    As I heard, this is not to be completed until a month or 
            6    so from now, so the other possibility if you would be 
            7    willing, perhaps the three of us could craft this and see 
            8    whether you would like to use it if that's not 
            9    overstepping the boundaries.  That might be helpful.  I 
           10    don't know. 
           11                            MR. SKLAR:  That would be 
           12    incredibly helpful.  The comment period is open 'til 
           13    January, and we would welcome that type of expertise.  
           14                            DR. SEEFF:   Having then defined 
           15    the entities and defined the fact that bleeding could come 
           16    from one or several sources, the next issue is of five 
           17    units within 48 hours.  The two possibilities, upper GI 
           18    bleeding identified that requires transfusion, or do we 
           19    need two units?  How do you come to two units?  
           20                            MR. GRACE:  Well, the criteria is 
           21    more than just the two units.  Hemodynamically significant 
           22    bleed is the way it's defined.  One of the criteria used 
           23    to find a hemodynamically bleed is at least a two-minute 
           24    bleed.  There are other criteria such as drop in blood 
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            1    pressure and increase in pulse rates, so forth.
            2                            DR. SEEFF:   So, I mean --
            3                            DR. GRACE:  If you wanted to be 
            4    accurate, I would put down a hemodynamically significant 
            5    bleed is more accurate than a specific number of units of 
            6    blood because we like to keep these patients somewhat 
            7    undertransfused.  The amount of blood that the patient is 
            8    actually given is somewhat subjective depending on the 
            9    physician taking care of it.  To say that someone has four 
           10    units of blood is not hemodynamically significant.  
           11    Somebody who has five is significant.
           12                            DR. SEEFF:   So, Norm, the 
           13    question is, What's the significance of bleeding if 
           14    someone has portal hypertension and bleeds, and they are 
           15    required transfusion because they were hemodynamically 
           16    unstable, what's the significance of morbidity and 
           17    mortality; does that identify the fact that these people 
           18    are dying within a particular period of time?
           19                            DR. GRACE:  The mortality for a 
           20    variceal bleed has improved over the last several years 
           21    with advances in therapy.  The mortality of a variceal 
           22    bleed is 15 to 20 percent range where it used to be closer 
           23    to 40 to 50 percent.  If nothing is done after an acute 
           24    bleeding is controlled, if nothing is done to prevent the 
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            1    re-bleeding, the risk of re-bleeding approaches 60 to 70 
            2    percent, so we need to get into therapy to prevent a 
            3    current variceal bleeding. 
            4      The variceal bleeding per se is not a liver 
            5    transplantation, but it's significant for serious liver 
            6    disease. 
            7                            DR. SEEFF:   Are there any 
            8    questions at this point if anyone want to ask?  The next 
            9    instance -- I'm not smart enough to fully understand what 
           10    this is saying -- consider under a disability for one year 
           11    following the lost documented massive hemorrhage so that 
           12    disability is  -- does that mean that the disability 
           13    persists for a year after the hemorrhage, after which if 
           14    they haven't bled, then they're back to square one?
           15                            DR. GRACE:  The data, that if you 
           16    go more than a year without recurrent bleeding, you are 
           17    back at a base line risk for bleeding of about 25 percent. 
           18                            DR. SEEFF:  If the person has not 
           19    bled for one year, does that mean that the disability has 
           20    now decreased, and then we go to the next component.
           21                            DR. GRACE:   Well, I think the 
           22    disability probably is going to be determined more by the 
           23    state of decompensation than the risk of bleeding.
           24                            DR. SEEFF:   That's the next area.  
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            1    Then we come to the issue of chronicity, and the two 
            2    components or the only component that I see here now is 
            3    ascites, and what is being removed from the previous 
            4    listings are the shunt operation, the fact that a shunt 
            5    operation was needed, the serum bilirubin of 2.5 or 
            6    greater and encephalopathy.  If a patient has chronic 
            7    liver disease, ie, cirrhosis, doesn't the presence of 
            8    encephalopathy in fact define serious entity?  Doesn't the 
            9    fact that persistent hyper bilirubinemia define the 
           10    seriously progressive disease?  What about things like 
           11    spontaneous bacterial peritonitis for example?  Because 
           12    what we're talking about is that the only thing that will 
           13    define the future disability presence of ascites of six 
           14    month-period or more demonstrated on at least evaluations 
           15    occurring at least two months apart either by paracentesis 
           16    or on physical examination associated with a low albumin 
           17    and a prolongation prothrombin count. 
           18                            DR. GRACE:   Let me comment as to 
           19    where this criteria lets us down.  The patient comes in 
           20    with a variceal bleed, hemodynamically significant bleed;  
           21    it's the second or third bleed; and the physician decides 
           22    to do a procedure called "TIPPS" which is a radiologically 
           23    placed shunt where you're connecting the hepatic vein and 
           24    portal vein to decompress the system. 
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            1      Now, one of the things that that procedure accomplishes  
            2    is in most patients, it gets rid of ascites.  But the 
            3    downside of the procedure is, it increases the risk of 
            4    hepatic encephalopathy.  So you can have a patient who is 
            5    chronically disabled with hepatic encephalopathy with no 
            6    ascites after one of these procedures and will not meet 
            7    these criteria. 
            8                            DR. ZEVIN:   The other problem 
            9    with these criteria is there's no sense of quantity of 
           10    ascites.  A good ultrastenographer can find a trace of 
           11    ascites in almost anybody.   So depending on what the 
           12    local culture is of reporting the ascites, it may or may 
           13    not appear on a report.  What we're looking at is 
           14    significant ascites.
           15                            DR. SEEFF:   It's stated here, 
           16    it's documented by paracentesis, and on two occasions 
           17    separated by these two months.  Paracentesis or  --
           18                            DR. ZEVIN:  Or examination --
           19                            DR. SEEFF:  Low albumin and 
           20    prolonged prothrombin times. 
           21                            DR. ZEVIN:  My clinical skills may 
           22    not be as good as yours, but there are lots of people 
           23    particularly obese people in whom it's extremely difficult 
           24    to demonstrate ascites on physical examination.  I would 
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            1    really -- I generally would (inaudible) using these 
            2    procedures.
            3                            DR. SHERKER:   One of the 
            4    questions for the listings is, Are there things that are 
            5    going to be markers for functional disability?  We know 
            6    that standard is related to functional disability and 
            7    listings are to help to establish functional disability.  
            8    One of the things that would have been problematic with 
            9    the previous listings as they were is that for patients 
           10    where cirrhosis, we had a fair number of patients who died 
           11    before they got their benefits.  The period of time, if we 
           12    look at a child's Q score for somebody in the previous 
           13    listings, there's some hints that that's where they were 
           14    going.  This is somebody who meets these listings.  We 
           15    were looking at a very poor prognosis, and a lot of people 
           16    who are dying required transplantation before their 
           17    benefits kicked in. 
           18      So I think that one of the things as far as markers go, 
           19    certainly if we can have markers and the markers and 
           20    listings are markers that predict a mortality or fair 
           21    highly likelihood of mortality in six to 12 months, that 
           22    would speak to those being obviously things that should be 
           23    there.  I don't want people to die before they get their 
           24    benefits. 
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            1      As far as the level of functional disability, I think 
            2    it's somebody who has had a bleed; they're going to 
            3    need -- it's predictable that they're going to need quite 
            4    of a bit of medical interventions.  Someone who has had 
            5    encephalopathy, they're going to need to have quite a bit 
            6    of medical intervention.  As far as the other things in 
            7    terms of symptomatology, the amount of ascites that 
            8    interferes with people's mobility is actually significant 
            9    to the functional ability.  The amount of edema that 
           10    interferes with their mobility is going to be significant 
           11    for their edema. 
           12      I haven't heard anybody mention pain today, but that's 
           13    something I will talk about more later.  I'll also pose a 
           14    question to my hepatology colleagues.  Low platelet counts 
           15    are one of the things that we're commonly finding in 
           16    cirrhotic patients and certainly an indicator of portal 
           17    hypertension, and something that when patients come to me 
           18    and ask about their physical activities and have low 
           19    platelets and abnormal coagulation, and ask, What should I 
           20    do; can I go back to my work as a construction worker; I'm 
           21    very concerned about that; and I'm interested in hearing 
           22    their concerns, too; and the possibility of putting a 
           23    platelet count onto these basic listings of something that 
           24    would indicate a high level of functioning ability.
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            1                            DR. SEEFF:   I'm not sufficiently 
            2    familiar with the meaning of the Social Security rulings 
            3    because this relates to functional disability as a 
            4    consequence of a particular finding or the predictability 
            5    of that finding with respect to long-term outcome.  
            6    Because, yes, ascites that's massive, clearly has a 
            7    functional -- if it's massive, it needs to function in a 
            8    capacity of the people who can't walk around and move 
            9    around breathe because the diaphragm is left out and 
           10    they're having consequence as a result of that. 
           11      Even the ascites that's not massive, predicts outcome 
           12    and tells you that this person is now decompensated, has a 
           13    much greater chance of dying in a confined period of time 
           14    than the person who has cirrhosis without ascites.  So I 
           15    guess that's the question:  Is the disability defined by 
           16    the fact that it predicts outcome or that it has a 
           17    functioning impairment at that moment in time?  I think 
           18    they are two separate issues.
           19      Whatever it is, it seems to me that we need to -- I 
           20    guess I would ask the question, Why was encephalopathy 
           21    removed?  If somebody has cirrhosis and hepatic 
           22    encephalopathy, is this not a disability --
           23                            AUDIENCE:   Can I address this?
           24                            DR. SEEFF:  Sure.
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            1                            AUDIENCE:  It's not necessarily 
            2    removed.  It's removed because all it is is a reference 
            3    listing to the mental listings, 1202.  So that's all that 
            4    occurred is the evaluation would be done under the mental 
            5    listing.  It's not removed.  We would still evaluate  
            6    those cases under mental criteria.
            7                            DR. SEEFF:   So two sorts of 
            8    evaluations.  One is liver disease, and the other one is 
            9    mental, and you tie these two together and say these two 
           10    are associated. 
           11                            AUDIENCE:  Whatever the 
           12    consequences are from the hepatic encephalopathy it would 
           13    be evaluated under the criteria for the organic mental 
           14    disorders. 
           15                            MR. SKLAR:  What's the standard 
           16    we're trying to get out here?  What we're trying to put 
           17    together here is a medical standard that would indicate 
           18    inability to work for at least 12 months from a disability 
           19    in 12 months that likely would result in death.  It's a 
           20    fairly rigorous standard to meet, and it would be strictly 
           21    on a medical criteria although I will say we've had to 
           22    (did not speak into the microphone) impossible to do it 
           23    alone.  So that's, sort of, framework we're operating.  
           24    Laws are fairly difficult standard, but what we tried to 
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            1    do is instruct medical criteria as to that outcome.
            2                            DR. SEEFF:  So a person with 
            3    cirrhosis who has ascites which is not massive but modest 
            4    and to the extent everything else is okay, they can do 
            5    limited work.  Does that represent a disability they can 
            6    do it within the next 12 or six months down the road, you 
            7    treat the ascites, and it goes away or it lessens, gets 
            8    better, but it's still defined as serious liver disease, 
            9    the consequences of which are going to be due to death 
           10    unless they have an auto accident or something 
           11    intervening.  Does that represent a disability that Social 
           12    Security Administration will accept?
           13                            MR. SKLAR:  I wish I could give 
           14    you a clear answer.  Think of it in terms of the 
           15    individual would be unable to return to their prior job, 
           16    and they will be essentially unable to work at any job for 
           17    12 months or more. 
           18                            DR. SEEFF:  Okay.  As far as the 
           19    listing --
           20                            AUDIENCE:   With relation to your 
           21    last question, the answer is maybe.  It depends on the 
           22    patient's or the claimant's age, and it depends on the 
           23    past work experience as a very younger person, and if they 
           24    can do sedentary work with the liver condition, it would 
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            1    prevent him to doing it for 12 continuous months, they 
            2    would not be (inaudible).  If you take that same person 
            3    and advance their age, and you can move their work into if 
            4    they're bricklayer, a person with that same medical 
            5    condition might become disabled under the regulation.  So 
            6    you have to consider the physical, the medical condition 
            7    impairment, together with the person's vocational factor 
            8    and age and past work to get to the alternate 
            9    determination of whether or not they're disabled. 
           10      Disability is a legal term and not a medical term.  
           11                            DR. GRACE:   How much of the 
           12    criteria is the ability to perform their current job? 
           13                            AUDIENCE:  That's significant 
           14    because if a person can perform their past job, then they 
           15    are considered not disabled regardless of the medical 
           16    condition. 
           17                            DR. GRACE:   Let me give you a 
           18    case that might -- I'm curious to see how it fits into 
           19    your criteria.  The patient comes in with a variceal 
           20    bleed.  The bleed is controlled, and the patient is put on 
           21    what is now standard therapy, a non-selected beta blocker 
           22    to prevent bleeding.  He doesn't have ascites.  He doesn't 
           23    have what we define as hepatic encephalopathy, but the 
           24    beta blocker has side effects that causes lethargy and 
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            1    depression in some people.  If this person was an airplane 
            2    pilot, I don't think you would want him flying your plane.  
            3    Is this disability or not?
            4                            AUDIENCE:  In that the case, if 
            5    the effect of the treatment is such that it would prevent 
            6    him or her from doing her best work, that would not be 
            7    applicable as far as flying that airplane.  If the person 
            8    was an elevator operator, maybe they could do their past 
            9    work.  So their vocational factors, as I indicated, are 
           10    quite important in making this legal determination.  And 
           11    just a heart condition is another -- even though we're on 
           12    liver  -- it's similar to a heart attack or heart 
           13    condition. 
           14      Chemotherapy is a good example where the effects of 
           15    chemo can be such that it can render you unable to perform 
           16    your past work, and if it continues for a period of 12 
           17    continuous months, and if you look at the other criteria, 
           18    you're considered disabled.  It's not just the medical 
           19    factors alone which make this a complicated process. 
           20                            MS. SIEGEL:  I'm a staff attorney 
           21    at the Disability Law Center.  Just to put another -- put 
           22    that in perspective.  When you get into that kind of 
           23    vocational analysis, you're not dealing with the listings 
           24    anymore.  They're, kind of, two separate routes to a 
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            1    disability finding, so I think to incorporate -- you would 
            2    not necessarily incorporate those factors into the 
            3    listing.  It's separate legal analysis.
            4                            AUDIENCE:  That's true, that's a 
            5    very important point.  You need the listings disabled as a 
            6    matter of law.
            7                            DR. SEEFF:   I'm uncertain what to 
            8    do at this point.   It seems to me that I'm now learning 
            9    something new.  One, we're talking about chronic liver 
           10    disease.  How do we define this this chronic liver 
           11    diseases?  And as I showed you, there's a progression.  
           12    Early on, there's no disability whatsoever when you first 
           13    develop chronic hepatitis.  You have nothing.  You don't 
           14    even know you have it. 
           15      When you see these patients have had chronic Hepatitis C 
           16    particulary Hepatitis C for 15 or 20 years, and they're 
           17    discovered to have this because they have a blood test 
           18    done, and they're absolutely shocked when we tell them.  
           19    There's no symptoms.  There's no disability.  As this 
           20    disease begins to be progress, and more and more scarring 
           21    occurs, to me that's the whole issue of chronic liver 
           22    disease regardless what the cause is whether it's alcohol 
           23    or Hepatitis C or B or any of those that it causes.  It's 
           24    the progression of the fibrosis, and that has two major 
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            1    components. 
            2      One, it interferes with the function of the liver.  The 
            3    ability of the liver to make the proteins that are 
            4    necessary for clotting and so on, and the second thing is 
            5    the portal hypertension.  The fact that there's an 
            6    interference and progression of blood and that leads 
            7    ascites, large spleen.  The spleen begins to trap the 
            8    platelets, and that count goes down. 
            9      So the question is when you define it here and you have 
           10    chronic liver disease, then the next thing is to get the 
           11    functional side of it.  What is the functional disability 
           12    that goes with it?  I actually thought what was happening 
           13    over here, the only thing that was being identified of the 
           14    fact was ascites.  I'm now told that encephalopathy exists 
           15    but it's a different place. 
           16      As a hepatologist and not as a Social Security expert or 
           17    legal expert, I would like to define how severe the 
           18    disease is.  The disease can be defined by the CPT score 
           19    or MELD score or simply by saying that there are certain 
           20    biochemical abnormalities that are part of the CPT score 
           21    and MELD score that in fact define the fact that this 
           22    person has a serious disease.  If they have cirrhosis and 
           23    persistent elevation of bilirubin of greater than 2.5, 
           24    these people are at risk of dying. 
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            1      Now, that doesn't make them disabled, the fact that the 
            2    bilirubin or spleen doesn't make them disabled, but they 
            3    are decompensated cirrhosis, and often that doesn't occur 
            4    on its own.  At the same time, you may have a low albumin, 
            5    low platelet, physical findings of ascites.  You may get 
            6    infected and get spontaneous bacterial peritonitis.  You 
            7    may develop encephalopathy, so all of these are the 
            8    factors that define the severity of the disease. 
            9      So I guess the question here is identifying what the 
           10    factors are that lead you to believe that this is serious 
           11    liver disease and which of those are actually disabling 
           12    over a period of six to 12 months for which some type of 
           13    compensation is needed.  That summarizes what needs to be 
           14    done here?  Do you agree with that or what? 
           15                            AUDIENCE:   I agree to an extent.  
           16    I think if in the listings in the area of cirrhosis we can 
           17    at least have the items that are likely to predict death 
           18    in a period of time or need for a liver transplant IN a 
           19    period of time, that would be helpful because I think we 
           20    have a pretty good idea that those are people who are not 
           21    going to be able to work after their death. 
           22      The other area which I will talk about a little bit more 
           23    is in the area of extra hepatic syndromes, and in that 
           24    area I will really look toward the model that is listed in 
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            1    the immunological diseases in the HIV section that really 
            2    reviews the fact that these are -- particularly Hepatitis 
            3    C and B to a lesser extent -- are infectious diseases that 
            4    have protein manifestations and very frequently much prior 
            5    to somebody being in a preterminal phase or terminal phase 
            6    cause a severe amount of functional disability, and I will 
            7    have some more word to say about that. 
            8      But I think on the question of cirrhosis, and when does 
            9    it become cirrhosis itself defined you would be disabled.  
           10    I think again it will be very helpful to use the CPT 
           11    score, to use the MELD score in some way and at least to 
           12    have that in the listings because those are very strong 
           13    predictors of very poor outcomes, and I'll say medically 
           14    when we say very poor outcomes, that may not mean the same 
           15    thing to non-medical people as it means to medical people.  
           16    Very poor outcome means death and progressive illness 
           17    before death, so I think having those in there would be 
           18    appropriate and would parallel what's present in the 
           19    cardiovascular listings and some of the other listings. 
           20                            AUDIENCE:  If I can add something 
           21    with respect to functional disabilities.  One of the major 
           22    functional disabilities that I suspect you people are 
           23    being assessing on a regular basis is fatigue related to 
           24    liver disease which runs a course totally independent of 
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            1    histologic or functional disease.  There are patients who 
            2    have something short of decompensated liver disease often 
            3    with minimal fibrosis on the liver biopsies who are 
            4    disabled by fatigue.  Whether that's truly Hepatitis C 
            5    related it's not clear. 
            6      The question I have is, How do you approach patients who 
            7    are coming out for disability on the basis of chronic 
            8    fatigue syndrome, and would those criteria be applicable 
            9    in this situation? 
           10                            MR. SKLAR:  I know that our 
           11    standard -- there's a standard in place for chronic 
           12    fatigue syndrome.  I believe it's separate and 
           13    independent, and we'll check on that.  It's another area 
           14    one of these areas that's bifurcated from this particular 
           15    listing, but we're certainly considering these types of 
           16    assessments.
           17                            AUDIENCE:  But Hepatitis C doesn't 
           18    exclude someone from having chronic fatigue syndrome under 
           19    you definition, does it?  That's a process that occur --
           20                            MR. SKLAR:  I'm not the best 
           21    person to comment.  Jim Julian. 
           22                            MR. JULIAN:  The chronic fatigue 
           23    can be -- there are ways to take that into account, not 
           24    necessarily at listing level.  The listing level being 
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            1    something that in and of itself, we're looking for the 
            2    criteria that if you have this, per se, you are disabled.  
            3    If somebody didn't necessarily meet that level, then you 
            4    could then go down to the functional level where you would 
            5    look at all of the claimant's impairments and how they 
            6    affect them and say, Given these medical conditions, there 
            7    are certain things they can and can't do.  If that would 
            8    preclude them from doing their past relevant work or other 
            9    work, they will be considered to be disabled. 
           10                            AUDIENCE:  Would it be appropriate 
           11    to reference that the same way that encephalopathy is 
           12    referenced in those documents?  
           13                            MR. JULIAN:  There are listings 
           14    that deal with -- basically we have taken the 
           15    encephalopathy out in the proposed because it references 
           16    it into a different criteria, and the decision maker would 
           17    know to go to the other criteria associated with chronic 
           18    fatigue. 
           19                            AUDIENCE:  Another symptom that 
           20    again doesn't necessarily correlate with severe liver 
           21    disease, particularly cholestatic disease, primary 
           22    bilirubin cirrhosis, is pruritus which can be disabling, 
           23    and patients occasionally who have had transplants solely 
           24    for pruritus.  Is there a way that that can be evaluated 
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            1    or discussed as well?
            2                            DR. SEEFF:  That's an important 
            3    point.  I have patients who become nearly suicidal because 
            4    of profound pruritus.  They are unable to sleep.  It is a 
            5    devastating symptom, and in those who have it.  We see it, 
            6    not only in cholestatic liver disease, but we see it in 
            7    Hepatitis C as well.  About 10 percent of people that I 
            8    have seen when I was at the VA came in with profound 
            9    pruritus, and this was the most devastating thing that 
           10    they had to deal with, and I'm sure that Barry will refer 
           11    to that as well.   There are other such things.  Somebody 
           12    else was going to make a comment.
           13                            AUDIENCE:  When we started out the 
           14    program initially, the first speaker indicated that the 
           15    listings are just the screening tool to take out certain 
           16    obvious cases; that if you meet this test, you are 
           17    disabled as a matter of law.  Just because you do not meet 
           18    the listing, does not mean that you are not disabled.  
           19    There are other criteria.  Part of the regulations that a 
           20    person can meet and become disabled by following those 
           21    others or subsequent or lesser standards.  So I think this 
           22    is one of the things that you're getting confused with.  
           23    Don't get off on the reason process that if you don't meet 
           24    the listing, you are not disabled.  That's not part of 
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            1    Social Security law.   So there are other things, fatigue 
            2    for instance, may be are not part of this listing as such 
            3    designated as a specific factor, but that does not mean a 
            4    person with extreme fatigue would not be entitled to 
            5    disability or Social Security right now. 
            6                            AUDIENCE:   This may be a matter 
            7    of philosophy in the listings, and I'm sure that there's 
            8    been much more discussions of this thing than occurred 
            9    just here.  The statement was made that  -- well the 
           10    evaluator will know to look in another section and that 
           11    unfortunately, commonly has not been how that has been  -- 
           12    how I have seen that happen in terms of my patients.  I 
           13    think that very frequently in the way the previous 
           14    listings were written, when we recorded Hepatitis C as the 
           15    major condition for example, a quick look at the listings 
           16    would be made by that evaluator.  Often the medical 
           17    evidence may be spotty in many of the patients that I see, 
           18    and no consideration would go further than that as far as 
           19    as do they have other manifestations beyond what was in 
           20    the listings.  I think for that reason, it's actually 
           21    helpful to have the references to the other sections in 
           22    the actual listings.  And I'll preview what I'm going to 
           23    say later, particularly in Hepatitis C which is a 
           24    tremendously prevalent condition as the most common of the 
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            1    common liver diseases that we really do need to be able to 
            2    refocus on some of the other things that are going on in 
            3    those Hepatitis C patients, and definitely at least point 
            4    evaluators to the other areas that might be significant 
            5    that might need to be evaluated.  I'll talk about that 
            6    more later. 
            7                            AUDIENCE:  Just a small point.  I 
            8    think what's being missed is that there's a provision with 
            9    within the listings of equal of the severity.  All of 
           10    these very real items that have been raised could be used 
           11    as a substitute for any of the other criteria in the 
           12    listings, and we're still talking about projected 
           13    assurance more or less of either death or total inability 
           14    to work for 12 months.  It's not a firm listing as written 
           15    here but a substitution of all these other things we 
           16    talked about; that they are of equal severity to the one 
           17    of the things listed. 
           18      The program permits that kind of combination of items 
           19    that will cause a functionally equal severity as this 
           20    does, and meets really is defined legally as the listings 
           21    that are written here.  I don't know that I've confused 
           22    you or not. 
           23                            DR. SEEFF:  What I'm beginning to 
           24    realize is that there's a slight disconnect between we 
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            1    physicians and the Social Security Administration's legal 
            2    needs, and the question is how to get these two to merge, 
            3    and I'm not sure quite how.  I know that Dr. Jones wants 
            4    to make a point, and then I will let her do so.  The 
            5    question is where to go from here?  If you would like us, 
            6    the four of us could try to work on this section based on 
            7    what we said and give it to you and for you to use as you 
            8    think necessary and we obviously our charge is going to 
            9    have to be not only what are we concerned about and what 
           10    defines chronic liver disease and what defines components 
           11    that predict outcome but also what specifically relating 
           12    to disability from that particular item, and that will 
           13    have to be separated out. 
           14      So if that is what you would like us to do, I have just 
           15    said so, perhaps we could work on it over the next couple 
           16    of weeks to put something together, send it to you, and 
           17    you use it as you see fit. 
           18                            MR. JULIAN:  There's a mechanism, 
           19    and we can provide you with the web site.  We will ensure 
           20    that you have that.
           21                            MR. PORRO:  I'm just wondering 
           22    hypothetically, the distinction between prognosis of six 
           23    to 12 months and substantial gainful activity, are they 
           24    mutually exclusive?  Or can you take a look at somebody, 
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            1    for example, who doesn't have a severe prognosis but who 
            2    cannot for some of the liver markers symptoms of elevated 
            3    platelet count, portal hypertension, things of that 
            4    nature.
            5                            DR. SEEFF:  Certainly that can be 
            6    the case.  There could be some abnormality within a given 
            7    period of time but need not necessarily to find imminent 
            8    mortality but can be disabling, and we will try to work on 
            9    that.
           10                            DR. GRACE:  I think one of the 
           11    things that we just have peripherally referred to, but I 
           12    think is very important, is that over the last 20 to 25 
           13    years we have made major advances in pharmacologic therapy 
           14    of liver disease.  Many of the drugs we use have side 
           15    effect.  These patients are going to be on the drugs 
           16    sometimes for life, and I'm not sure how these side 
           17    effects of treatment are factored into disability.  They 
           18    may not be decompensated, but they may be disabled because 
           19    of the side effects of treatment at least being disabled 
           20    from currently performing their current occupation. 
           21                            MR. JULIAN:  Hopefully, we can 
           22    have a final question and wrap for lunch shortly. 
           23                            MS. ST. JOHN:  I'm Tina St. John, 
           24    I'm the medical director of the Hepatitis C program.  I 
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            1    don't want to steal your thunder.  But in general, sort 
            2    of, what we've been dancing around is that there are 
            3    several classifications really to be considered here.  
            4    There's the cirrhotic category which appears to be the 
            5    main thing addressed in terms of listings and that it's 
            6    important to distinguish between decompensated and 
            7    compensated.  But it's also important to recognize there 
            8    are severe and chronic liver diseases C B one where the 
            9    patients may not be cirrhotic but may be disabled, and I 
           10    think in terms of what the Social Security Administration 
           11    is seeking in part is for us to help define criteria that 
           12    be help them evaluate in an evidenced based way how to 
           13    look at patients that present with perhaps non cirrhotic 
           14    and are there criteria that we can look at that helps 
           15    determine what the level of function or dysfunction is 
           16    from a Social Security Administration standpoint, and 
           17    maybe we can talk this afternoon about neuropsychiatric 
           18    listings in terms of cognitive disability which is 
           19    certainly very significant and does not necessarily 
           20    correlate with liver histology.  Let's see where the 
           21    discussion goes this afternoon. 
           22                            DR. SEEFF:   Thank you, and we 
           23    will continue this afternoon. 
           24                            (Lunch break was taken.)
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            1                            MR. JULIAN:  Good afternoon, we're 
            2    going to go ahead and get started with the afternoon 
            3    session.  I just want to thank everybody for this morning.  
            4    It was very interesting information.  One of the things 
            5    that we would like to do is  -- the public comment period 
            6    is open.  What I'm going to do is pass around the list, 
            7    and if anybody would like us to send you the link where 
            8    you can post public comments, put your web address here 
            9    and/or your mailing addresses, and we'll get you a copy of 
           10    that information.  We welcome comments from anybody and 
           11    everybody. 
           12      What I would like to do this afternoon is just take a 
           13    brief moment and, kind of, explain how Social Security 
           14    finds somebody disabled, and then introduce the panel for 
           15    this afternoon's session.  Basically, with Social Security 
           16    Disability, we're dealing with individuals who come to us 
           17    alleging that they are disabled either through a physical 
           18    or mental disability or a combination of the two.  Social 
           19    Security has established a five-step sequential evaluation 
           20    process to determine whether or not somebody is disabled.  
           21    What I would like to do is briefly explain that five-step 
           22    process. 
           23      The first step in the process is to determine whether or 
           24    not somebody is still engaging in work.  We call it 
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            1    "substantial gainful activity."  It's a fairly low 
            2    threshold.  It's about $800 a month.  But if somebody is 
            3    working, we don't go any further because we find they are 
            4    not disabled because they are currently engaging in work.  
            5    If somebody isn't working, the next question we're going 
            6    to ask is, Do they have a severe impairment?   Basically, 
            7    a severe impairment is going to interfere with their 
            8    ability to engage in work. 
            9      It's a fairly low threshold in a sense that the folks 
           10    coming to Social Security are alleging an impairment and 
           11    they're seeking treatment for it.  It's going to be deemed 
           12    a severe impairment.   Assuming somebody has a severe 
           13    impairment, we then ask ourselves, given the impairment, 
           14    is it at the listing level of impairment.  That's what 
           15    we're talking about today.  That's a level of disability 
           16    that we say if you have an impairment, and it's at such a 
           17    level, then you are disabled.  Whether you're 20 or 60 
           18    years' old if you have this condition at this level, you 
           19    are found disabled. 
           20      Assuming people don't meet that criteria, we would then 
           21    go on to our vocational considerations, and we would say, 
           22    you have a severe impairment, but it's not at a listing 
           23    level; but given your condition, can you return to your 
           24    past relevant.  That's work that you've done in the last 
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            1    15 years.  Can you return to that work at the substantial 
            2    gainful activity level?  So we're asking, Can the claimant 
            3    do the work they have done in the past? 
            4      If they can't, then what we're going to  -- if they can 
            5    return to their past work, then we deem them not to be 
            6    disabled.  If they can't do their past relevant work, and 
            7    I'll give you an example.  If a person is older, and their 
            8    past relevant work was a bricklayer or working in a steel 
            9    foundry which is hard, physical labor, and they're 
           10    suffering from a bad back, bad knees; where they can't do 
           11    it; where they're suffering from fatigue; what we then do 
           12    is go on and ask, they can't do their past relevant work; 
           13    they're suffering from a medical condition; are there any 
           14    other jobs out there in the national economy which the 
           15    claimant could make an adjustment.  So we're saying, is 
           16    there any other work the claimant can do given their 
           17    limitations, and that's looking at all the limitations, 
           18    their pain, their fatigue, as well as the limitations 
           19    imposed by the physical condition, itself. 
           20      Assuming the claimant can't make an adjustment to any 
           21    other work in the economy, then we would find the claimant 
           22    disabled.  At the vocational factors, we employ medical 
           23    experts and vocational experts.  So that's a brief 
           24    explanation of disability, Social Security.  Yes, sir.
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            1                            DR. GRACE:  Does the work have to 
            2    be at the patient's educational level?  Let's say somebody 
            3    was an accountant and you say, Well, you can clear dishes 
            4    from a table.
            5                            MR. JULIAN:  No, sir, you can 
            6    clear dishes from a table.  We're looking to see given 
            7    those impairments.   An example might be, you've got a 
            8    younger individual, typically someone in their 40s.  If 
            9    they had a bad back, and they had a history of being a 
           10    carpenter or physical laborer.  They have a high school 
           11    education.  We might say, You can't go back to being a 
           12    carpenter or that type of heavy labor, but perhaps you 
           13    could be a security guard or a checker or cashier at a 
           14    grocery store; that's the determination that the 
           15    administrative law judges are making and also the state 
           16    disability determination service. 
           17      It's a balancing act.  I'm saying you have these 
           18    conditions, and we think you can do other types of work.  
           19    And I just want to show  -- we can make this available for 
           20    people to look at.  This is the disability evaluation 
           21    under Social Security, so this is the book.  This is our 
           22    listing book which basically lists the physical and mental 
           23    impairments that a person can be suffering from.  It's not 
           24    an all inclusive book.  Sometimes we have to work 
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            1    impairments and disabilities in by analogy.  This shows 
            2    the criteria that Social Security is currently using to 
            3    find somebody disable at Step 3 of the special evaluation 
            4    process.  So this has everything from organic mental 
            5    disorder down to amputation of your feet, so it's called 
            6    "Head and Toes." 
            7      There are times when people need more than one type of 
            8    listing.  There are times they don't need the listing, and 
            9    we found them disabled because of a residual functional 
           10    capacity.
           11                            AUDIENCE:  How is the rate of pay 
           12    for benefits someone receives?  Is it all the same?  I 
           13    could be very naive. 
           14                            MR. JULIAN:  There are two types 
           15    of payments for Social Security.  There is supplemental 
           16    security income payment, which is a needs based system and 
           17    disability insurance benefits, and that's based where you 
           18    pay into the system.  That's your FICA.  Depending on what 
           19    paid in is what you would or the claimant would receive 
           20    out on the back side.   If you get your Social Security 
           21    statements, usually about two months before your 
           22    birthdate, it's going to explain to you if you were found 
           23    disabled, you would receive X number of dollars for 
           24    disability.  You can also get that, not just two months 
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            1    before your birthday; you can go to your local Social 
            2    Security office and request one on line at any time and 
            3    that would be a current rate of pay. 
            4                            AUDIENCE:  There's got to be a 
            5    point you run.  Some people haven't paid into  --
            6                            MR. JULIAN:  Some people are not 
            7    insured status.  You have to have to quarters of work in 
            8    your last 40 quarters.  So there's a computation there.  
            9    It's like payment for your insurance.  If you haven't paid 
           10    your insurance, you are not qualified for disability 
           11    insurance.  The supplemental security income is a need pay 
           12    system.  If you make too much money, or you family makes 
           13    too much money, you wouldn't be eligible for that.
           14                            AUDIENCE:  Would that level be 
           15    enough to make you want to get gainful employment?  Or is 
           16    it like unemployment, there's a job you wouldn't take 
           17    because you have more money on disability?  
           18                            MR. JULIAN:    Disability is 
           19    not --
           20                            AUDIENCE:  Works the same as 
           21    unemployment as far as those levels?
           22                            MR. JULIAN:  It's going to vary by 
           23    person to person.   I will say this, you will not get rich 
           24    on disability payments.  I think for a lot of people, it's 
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            1    more for medical care is what they really need.
            2                            AUDIENCE:  SSI, it's actually 
            3    below the poverty level. 
            4                            MR. JULIAN:  SSI, is at a much 
            5    lower rate than disability benefits.  The disability 
            6    insurance is based on what you paid into the system. 
            7                            AUDIENCE:  Could you clarify the 
            8    statement about access to medical care if you are an SSDI?   
            9    I just want to get a clarification on access to medical 
           10    care if you're deemed disabled under the SSDI program and 
           11    SSI program.
           12                            MR. JULIAN:  Under the SSI 
           13    program, there's a five-month wait if you're found 
           14    disabled under disability insurance benefits, SSDI.  As 
           15    opposed to supplemental Social Security income, you are 
           16    immediately put on the disability rolls. 
           17                            AUDIENCE:  He's talking about the 
           18    24 months' waiting period for Medicare.  On the DI program 
           19    once you are found eligible, it almost amounts to 25 
           20    months, but it's a 24 month waiting period before you are 
           21    eligible for Medicare.  Congress has in a couple of cases 
           22    eliminated a 24-month waiting period, Lou Gehrig's Disease 
           23    (inaudible) and I think there's one other.  In almost 
           24    every other case, it's 24 months.  That's where you're 
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            1    determined to be eligible for cash benefits.  SSI Medicaid 
            2    coverage is immediate, so it starts at the same time as 
            3    the cash coverage.
            4                            MR. JULIAN:  That's a brief 
            5    overview of the disability system.  What I would like to 
            6    do is introduce the panel for this afternoon and times 
            7    they'll be speaking.  First is Dr. Averell Sherker.  Dr. 
            8    Sherker is the Director of Hepatology at the Washington 
            9    Hospital Center, Faculty Section of Gastroenterology.  
           10    Also Dr. Barry Zevin, he's the medical director of the Tom 
           11    Waddell Health Center and assistant clinical professor of 
           12    medicine at the University of California, San Francisco 
           13    School of Medicine.  You also have Miss Barbara Siegel, 
           14    senior attorney, Disability Law Center in Boston, 
           15    Massachusetts.  Finally, we have Christine Kukka who is 
           16    the parent of the child who's suffering from Hepatitis B, 
           17    and she's a patient advocate. 
           18      Dr. Sherker will speaking from 1:30 to 1:50.  I'll 
           19    adjust the times, but we will just go 20-minute intervals 
           20    and see where it leads us.  So, please, welcome the 
           21    panelists. 
           22                            DR. SHERKER:   Thank you for the 
           23    invitation to be here at this very instructive process.  
           24    What I am going to do, and Dr. Zevin and I have 
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            1    coordinated, we're going to talk about the treatment of 
            2    chronic liver disease.  Dr. Zevin is going to cover 
            3    Hepatitis C, and I'm going to cover everything else.  This 
            4    is basically the syllabus is.  We're going to talk about 
            5    the major chronic liver diseases that affects adults 
            6    particularly viral hepatitis, autoimmune, alcoholic liver 
            7    disease, non-alcoholic fatty liver disease and NASH, 
            8    cholestatic liver diseases, metabolic liver diseases, 
            9    malignancies, and at the end, I have a couple of slides 
           10    about the complications of end stage liver disease, and 
           11    your comments to me of these conditions. 
           12      Hepatitis B virus, as we heard this morning, in most 
           13    adults acquired infections are self-limited.  It's unusual 
           14    but Hepatitis B infection in adults (inaudible) for 
           15    chronicity which as we learned this morning persists for 
           16    more than six months.  So that most of what we're dealing 
           17    with are individuals who were infected either at birth or 
           18    in childhood.  Chronic Hepatitis B goes to the different 
           19    phase where it may be replicated, the virus is replicating 
           20    itself, or it can be non-replicating or in a dormant 
           21    state. 
           22      Those individuals who have replicating virus may benefit 
           23    from anti-viral therapy, and we have two major classes of 
           24    therapy available.  One is interferon compounds and the 
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            1    other is oral nucleoside and nucleotide agents.  It's 
            2    important to emphasize that many of the patients who have 
            3    chronic Hepatitis B do not need treatment specific to 
            4    their infection mostly because they're in non-replicating 
            5    phase.
            6      As far as interferon treatment goes -- and Dr. Zevin is 
            7    going to have more to say about interferon  -- interferon 
            8    comes in two flavors.  There's the standard interferon 
            9    given three times a week for Hepatitis C and either higher 
           10    dose three times a week or daily for Hepatitis B infection 
           11    or the neuropeglated interferons which have the advantage 
           12    of a longer half life or longer duration of action given 
           13    once a week.  It should be noted that pegylated 
           14    interferons are not yet FDA approved for use in Hepatitis 
           15    B infection although they are commonly used in practice. 
           16      The advantage of interferons is that they are associated 
           17    with a finite duration of therapy, classically 16 weeks 
           18    and gradual extension to 24 weeks, and in some virus, they 
           19    may actually be a benefit of 12 months, 48 weeks of 
           20    therapy.  Taking the literature that's out there, 
           21    approximately a third, possibly somewhat less than a third 
           22    of those people treated with interferon will achieve a 
           23    non-replicated state, and in most situations when that's 
           24    induced by interferon, that effect is durable and 
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            1    persists. 
            2      The patients who have decompensated liver disease are at 
            3    high risk for severe decompensation and even death related 
            4    to interferon treatment because the way interferon works, 
            5    it actually stimulates the immune system to react against 
            6    the virus, and someone without adequate reserve of liver 
            7    to tolerate that, that may be enough to cause serious 
            8    harm.  So patients with decompensated liver disease are 
            9    generally excluded from interferon treatment. 
           10      Interferon is associated with a large number of adverse 
           11    effects, most common, almost universal, are flu like 
           12    symptoms.  These are variable in severity.  Some people 
           13    experience symptoms in the beginning of treatment which 
           14    gradually ease off, and others have severe debilitating 
           15    symptoms which prevent us from using therapy or force us 
           16    to stop.  Very common, patients will experience altered 
           17    sleep patterns, fatigue, poor concentration, depression is 
           18    common.  It can be either an exacerbation of preexisting 
           19    condition, and certainly one is concerned when one hears a 
           20    prior history of suicide attempt of severe depression or a 
           21    hospitalization for depression.  That has to be taken into 
           22    consideration if you're considering a treatment with 
           23    interferon either for Hepatitis B or Hepatitis C.  But 
           24    depression (inaudible) in someone with no prior history. 
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            1      Myelo suppression is common, typically manifests as 
            2    neutropenia, low white cells, with infection risk or 
            3    thrombocytopenia, low platelets with a bleeding risk.  
            4    Interferons work by stimulating the immune system and can 
            5    either unmask or exacerbate preexisting or de novo 
            6    autoimmune diseases, and very commonly we'll see patients 
            7    who end with chronic thyroid disease requiring thyroid 
            8    replacement after a course of interferon therapy or during 
            9    the course. 
           10      Nucleosides and nucleotides are generally well tolerated 
           11    oral medications; however, they rarely result in a durable 
           12    suppression of viral replication.  For that reason, they 
           13    are generally used as a long-term treatment to suppress 
           14    virus.  But it's double edged because the longer they are 
           15    used, the more likely they are to induce resistance in 
           16    therapy.  There are two licensed agents.  There are a 
           17    number that are going to be available in coming years.  As 
           18    I said, these medications generally are very well 
           19    tolerated with minimal side effects. 
           20      One thing that's important about these agents, they 
           21    offer some hope for clinical improvement in patients with 
           22    decompensated liver disease.   In contrast to interferon 
           23    which can actually cause chronic decompensated liver 
           24    disease to worsen.  These agents in small studies show a 
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            1    remarkable improvement in some patients even resulting in 
            2    people listed for transplantation being removed from 
            3    transplantation lists because they no longer need 
            4    transplantation as long as the virus remains sensitive to 
            5    the agent. 
            6      A Brief discussion on alcoholic liver disease, Dr. Seeff  
            7    showed you that the importance of alcohol liver disease is 
            8    a factor by itself, applies only to a minority of patients 
            9    with chronic liver disease.  What's important about 
           10    alcoholic liver disease is that it responds very well to 
           11    abstinence.  Patients who stop drinking have a remarkable 
           12    potential for improvement.  It's not universal, but that's 
           13    the best hope.  In patients who have persisting liver 
           14    dysfunction, liver failure despite abstinence, liver 
           15    transplantation may be a consideration. 
           16      Autoimmune hepatitis is a disorder where the immune 
           17    system is abnormally triggered to react against the normal 
           18    liver and is treated with long-term immunosuppressive 
           19    therapy.  Typically, patients who are given moderate doses 
           20    of corticosteroids, Prednisone, to achieve a remission, 
           21    and then the medication is tapered, but the medication has 
           22    to be tapered slowly, and often use of thyoprin will allow 
           23    us to get the Prednisone dose down to a lower level. 
           24      For the most part, these patients require life long 
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            1    immunosuppression.  Very few will be able to completely 
            2    stop immunosuppression, and many of these patients will 
            3    require long-term possibly life long Prednisone albeit at 
            4    low dose, but that is associated with severe potential 
            5    adverse effects, including diabetes, hypertension, bone 
            6    disease, eye disease, glaucoma, cataracts and other risks 
            7    associated with long-term Prednisone.  So we can obviously 
            8    achieve remission and obtain remission, but there's a high 
            9    price to be paid. 
           10      There are other agents which have been used in small 
           11    pilot studies in individuals.  Largely these are drugs 
           12    that have been developed for use in organ transplantation.  
           13    They can be used sometimes with success, (inaudible) and 
           14    intolerant patients need to be used.  Patients with 
           15    autoimmune hepatitis who come to medical attention late or 
           16    who are poorly compliant or intolerant or resistant to 
           17    treatment can be treated successfully with liver 
           18    transplantation. 
           19      As we heard about non-alcoholic fatty liver disease of 
           20    which the more severe end of the spectrum is NASH or 
           21    non-alcoholic steatohepatitis is commonly associated with 
           22    obesity or metabolic syndrome, including obesity, 
           23    diabetes, hypertension and hyperlipidemia and can result 
           24    in progressive liver disease.   Some of these patients 
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            1    have had fat only, and some will have fat and inflammation 
            2    and the most severely affected patients can progress to 
            3    end state liver disease and cirrhosis. 
            4      Just a point related to one of the slides Dr. Seeff 
            5    showed you talking about cryptogenic cirrhosis, if you 
            6    look at patients with a diagnosis cryptogenic cirrhosis 
            7    who have been transplanted and compare them to the patient 
            8    profile of the typical patient with non-alcoholic fatty 
            9    liver disease, there's a tremendous amount of overlap.  
           10    The belief is that these patients with cryptogenic 
           11    cirrhosis are burnt out NASH cases.  What makes it 
           12    difficult is as the fatty liver disease progresses to end 
           13    stage liver disease, the fat tends to disappear from the 
           14    liver biopsy so these people may not be obvious when they 
           15    come to transplantation. 
           16      The treatment is generally aimed at the underlying 
           17    conditions.  Small studies suggest that improvement 
           18    particularly of glycemic control or diabetes can result in 
           19    improvements of liver histology, but what's really lacking 
           20    are long-term longitudinal studies to prove the benefit of 
           21    any treatment for the liver disease. 
           22      Cholestatic liver diseases, I'm going to limit myself to 
           23    two of the more common ones.  The primary biliary 
           24    cirrhosis is an autoimmune disease affecting the cells of 
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            1    the bile ducts, and it tends to occur predominantly in 
            2    women, nine to one, and can be associated as I mentioned 
            3    this morning with severe pruritis and other symptoms.  
            4    There's a medication called (inaudible) acid which has 
            5    been shown in large study to improve biochemistry with 
            6    possible histology and likely survival with long-term use, 
            7    generally a well-tolerated medication.  The only 
            8    significant adverse effect is diarrhea and tends to be 
            9    limited and resolves with continued use.  The use of this 
           10    medication has had a tremendous impact on the outcome of 
           11    this disease so that primary biliary cirrhosis has gone 
           12    from being a leading indication for liver transplantation 
           13    in the '80s to where now it's fairly uncommon that 
           14    requires transplants. 
           15      The primary sclerosing cholangitis is a condition mostly 
           16    related to inflammatory bowel disease with narrowing and 
           17    scarring of the bile ducts with blockage and potential for 
           18    infections in the bile, so called cholangitis.  
           19    Individuals who have dominant strictures benefit from 
           20    endoscopic therapy where the strictures are dilated or 
           21    otherwise stented.  These patients run a significant 
           22    lifelong risk of developing particularly malignancy 
           23    cholangio carcinoma, and the problem with these folks, the 
           24    cancer risk may be there despite the fact these patients 
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            1    are asymptomatic and totally functional.  So it's very 
            2    challenging to decide who and when liver transplant is 
            3    required and obviously done in this situation to prevent 
            4    the development of cancer. 
            5      There are a large number of metabolic diseases, most of 
            6    which are within the treatment realm of the pediatrician 
            7    as we heard this morning, but two diseases that were seen 
            8    common in adults hereditary hemochromatosis.  This is a 
            9    disorder, very common genetic disease, which ultimately 
           10    causes multi-system involvement related to iron overload 
           11    particularly involving liver, heart, endocrine organs, 
           12    diabetes, pituitary, skin disease and arthritis.  These 
           13    patients, once diagnosed, are treated fairly simply by 
           14    just removing iron by removing blood, but the process is 
           15    usually a fairly prolonged process, and a typical patient 
           16    presenting with hemochromatosis will have 10 and 30 or 
           17    more grams of iron in excess of each blood phlebotomy, 
           18    each blood unit that's removed removes about 250 
           19    milligrams.  It's not unusual for these patients to 
           20    require every two week phlebotomies.  
           21      Once they achieve the state of iron deficiency, it has 
           22    to be repeated once or twice a year, just to maintain the 
           23    iron deficient state.  These folks have a very high risk 
           24    of liver cancer if the condition is diagnosed after 
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            1    cirrhosis is established.   The other metabolic liver 
            2    disease of adults, albeit rare, is Wilson Disease.  This 
            3    results in abnormal copper accumulation which affects the 
            4    liver and central nervous system.  It can cause acute or 
            5    chronic liver disease.  In fact, acute liver disease is an 
            6    acute exacerbation of recognized chronic liver condition, 
            7    or it may present as a neurological or psychiatric 
            8    disease.  These people are treated with copper chelation 
            9    therapy.  The medication is Dipenicilamine (taken 
           10    phonetically) which is an antivenereal that chelates 
           11    copper, has a large number of potential adverse effects 
           12    frequently associated with hypersensitivity reactions, 
           13    skin disease, kidney disease. 
           14      Trientine is another agent that can be used in patients 
           15    who are intolerate to penicillamine, and there's evidence 
           16    that supplementing the diet with zinc actually impairs 
           17    copper absorption and decreases copper accumulation. 
           18      Patients who present with the fulminant presentation of 
           19    Wilson Disease or fulminant liver failure with Wilson 
           20    Disease will not recover with chelation therapy.  It takes 
           21    took long.  These people need to be transplanted as their 
           22    therapy. 
           23      Liver malignancies, hepatic carcinoma, is growing in 
           24    importance and in frequency.  One of the most common 
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            1    malignancies worldwide and increasing in frequency in 
            2    North America.  It's mostly associated with cirrhosis, but 
            3    Hepatitis B, different from Hepatitis C, can be associated 
            4    with liver cancer and carcinoma even in the presclerotic 
            5    stages while Hepatitis B patients with hepatomas do have 
            6    cirrhosis.  Small tumors can be diagnosed on periodic 
            7    screening of at risk patients and in this way can be cured 
            8    by resection or transplantation.  Other modalities such as  
            9    injecting with alcohol using radio frequency to basically 
           10    heat the tumor or chemo hemolization may be a benefit but 
           11    not long-term benefits.  These are used as therapeutic 
           12    modalities while the patient is awaiting liver 
           13    transplantation. 
           14      Patients who present with large symptomatic tumors 
           15    generally have vascular involvement.  They may have spread 
           16    of the tumors outside the liver, and unfortunately, we 
           17    really don't have any helpful therapies to offer this 
           18    patient.  Cholangio carcinoma is a malignant tumor arising 
           19    from bilirubin epithelium, highly associated with 
           20    sclerosing cholangitis and other cholangiopathies, 
           21    parasitic infections of the bile ducts as seen in Asian 
           22    countries.  These tumors generally present late and 
           23    metastasize early.  Unfortunately, they frequently occur 
           24    following transplantation, and the therapeutic options are 
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            1    limited. 
            2      Briefly talking about the common complications of end 
            3    stage liver disease.  As we talked about in the panel 
            4    discussion, portal hypertension may present with massive 
            5    variceal hemorrhage or other hemorrhage from other 
            6    abnormalities blood vessels throughout the gut.  It's 
            7    managed acutely with vasoconstrictors which is 
            8    somatistatin and its analogues, and it's treated with 
            9    endoscopic therapies.  The treatment of choice is band 
           10    ligation but in some situations it's not technically 
           11    possible, and sclerotic therapy is done as discussed this 
           12    morning. 
           13      Some patients who have recurrent or resistant bleeds may 
           14    benefit from insertion of a metal shunt within the liver 
           15    that basically connects the portal bloodstream -- portal 
           16    blood systemic circulation and by doing so reduces the 
           17    pressure and reduces the risk of bleeding.  It used to be 
           18    before these shunts were available that formal shunt 
           19    surgery was commonly done, but that is associated with 
           20    significant morbidity and mortality.  There's a limit role 
           21    for some surgery in very select patients who have very 
           22    well preserved liver function (inaudible). 
           23      As Dr. Grace discussed this morning, non-selected beta 
           24    blockers can be used in patients with varices both for 
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            1    primary and secondary phophylaxis bleeding, but again, 
            2    these agents in some patients are not particularly well 
            3    tolerated and may cause fatigue and disability issues.  
            4    Ascites, first line of therapy is diuretics and sodium 
            5    restriction; however, the dose may be limited by effects 
            6    of kidney and electrolytes disturbances.  Patients with 
            7    massive ascites may be treated by intermittent large 
            8    volume paracentesis where four to six liters of fluid is 
            9    removed through a needle at one time, sometimes done with 
           10    albumin infusion at the same time, and when this becomes 
           11    required frequently, then there may be a role for using 
           12    the TIPPS, the shunt I talked about, to control ascites.  
           13    These patients are at risk for serious complications, 
           14    particularly infection which is spontaneous bacterial  
           15    peritonitis which has a high mortality in hospital and a 
           16    marker of severe liver impairment with a significantly 
           17    limited survival and renal syndrome where the kidneys are 
           18    affected by the liver disease and ascites. 
           19      Encephalopathy was alluded to this morning as well.  It 
           20    may be sub clinical in which case can only be identified 
           21    on batteries of neuropsychiatric tests.  Clinically, we 
           22    recognize four stages.  Stage 1 is mild, personality 
           23    changes, day and night reversal, and Stage 4 is coma.  
           24    Exacerbations of encephalopathy are frequently exacerbated 
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            1    by other medical conditions, infections, bleeding, renal 
            2    failure, electrolyte disturbances, dehydration, dietary 
            3    proteins suppression.  Patients with encephalopathy are 
            4    put on protein restriction frequently or other 
            5    comorbidities.  These patients are treated with lactulose 
            6    which is a non-absorbing osmotic agent that affects 
            7    ammonia absorption and causes a change in the bacterial 
            8    floor of the gut, and there is also a role for 
            9    antibiotics, non-absorbing antibiotics, mycin. 
           10      That's all I have to say. 
           11                            (Applause.)
           12                            MR. WASHINGTON:  Dave Washington 
           13    from the office of Social Security.  You mentioned under 
           14    Wilson the medical metabolic disease, that manifestation 
           15    can be neurological or psychiatric.  What type of 
           16    psychiatric problems might a person manifest?  
           17                            DR. SHERKER:  The copper 
           18    accumulates particularly in the vasoganglia so that these 
           19    patients end up with movement disorders, but very common 
           20    manifestations psychiatric disease as well. 
           21                            MS. SIEGEL:  I'm curious about 
           22    what you said about abstinence can lead to recovery.  At 
           23    what point is the liver damage not reversible, and how can 
           24    you tell? 
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            1                            DR. SHERKER:  Basically, it's a 
            2    matter of following patients over time.   Typically, we 
            3    expect an improvement in a six-month timeframe, but 
            4    anecdotally -- this is Dr. Grace's opinion as well -- we 
            5    see patients who've been abstinent for long periods of 
            6    time, and you see recovery six months, nine months, 12 
            7    months, 18 months after stopping.  Patients who may have 
            8    significant coagulopathies, patients come in with variceal 
            9    bleeds who stop drinking, if you measure their portal 
           10    pressures and pressure readings, they actually improve 
           11    even beyond a year.  But we generally will see some sort 
           12    of significant improvement after six months' abstinence. 
           13                            DR. SEEFF:  We had a meeting, as I 
           14    mentioned to you about liver cancer, and the person who 
           15    reviewed it, Dr. Tim Morgan from the West Coast, pointed 
           16    out that stopping alcohol in people who develop cirrhosis 
           17    may not produce evolution to develop the cancer.  If 
           18    anything, it may even go up, and the reason for that is 
           19    not because it goes up, but because people who have died 
           20    as a result of alcoholic hepatitis no longer have it 
           21    because they're no longer drinking.  They live a little 
           22    longer, and therefore, the likelihood is that they may go 
           23    on to develop cancer at a higher rate than they had 
           24    continued to drink. 
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            1      The question is where the cut off is and I think Norman 
            2    can comment. 
            3                            DR. GRACE:  Well, I think Averell 
            4    summed it up very nicely.  The only thing I would add that 
            5    that (inaudible) cirrhosis of the liver.  Now we know that 
            6    there's a dynamic component of cirrhosis; that is, 
            7    continuously laying down the fibrous tissue and 
            8    reabsorbing it.  In early stages cirrhosis there's 
            9    potential of significant improvement, if you have advanced 
           10    cirrhosis, probably not. 
           11                            DR. SHERKER:  There's a common 
           12    theme throughout these diseases that if you can somehow 
           13    eliminate whatever the inciting agent be it Hepatitis C 
           14    virus or iron or alcohol, there's a significant potential 
           15    for recovery histologically which, as Dr. Grace says, has 
           16    only come to the medical consciousness in the last five or 
           17    10 years. 
           18                            DR. ZEVIN:  I'll make one other 
           19    comment related to the alcoholic situation which is to 
           20    remind people that getting people with chronic 
           21    alcoholicism to stop drinking and abstain is not 
           22    necessarily the typical experience of us physicians, and 
           23    the continued injury to the liver will continue, and the  
           24    person may not be disabled because of their drinking 
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            1    directly, but they will continue to have the liver injury, 
            2    and often the end stage liver disease will remain. 
            3                            DR. SHERKER:   The funny thing is 
            4    as a hepatologist, I see relatively few alcoholics.  There 
            5    seems to be a referral bias.  A lot of people don't refer 
            6    alcoholics. 
            7                            DR. ZEVIN:  My name is Barry Zevin 
            8    from San Francisco.  I may or may not be in the same time 
            9    zone as everybody in the room so excuse me if I'm not.  
           10    I'm coming to speak on liver disease, not as a 
           11    hepatologist or liver specialist, but as a primary care 
           12    internal medical physician.   I work at a community health 
           13    center, a large health care for homeless program, and my 
           14    interest is liver disease is primarily in Hepatitis C and 
           15    also Hepatitis B strictly from the point of view that a 
           16    very large portion of my patients are infected with these 
           17    viruses, and I'm going to speak about treatment, and it 
           18    may surprise some people that it takes me a very long time 
           19    to get to the liver as I speak about treatment here. 
           20      We're also working on a handbook documenting disability 
           21    simple strategies for medical providers primarily for the 
           22    health care for the homeless community.  I think that's 
           23    funded through the Social Security Administration so look 
           24    for that in 2005, maybe by the end of this year which 
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            1    would help the basic understanding process.  So I'm going 
            2    to start by talking about everything about the liver. 
            3      Is chronic Hepatitis C a usually asymptomatic condition 
            4    which results in long-term harm to the liver?  My answer 
            5    to that is no, and I hope other people's answer to that 
            6    will also be no as we go along. 
            7      Next slide, generalized symptoms, fatigue is the most 
            8    common.  I will also talk about pain and other somatic 
            9    symptoms and decreased quality of life in general.  The 
           10    number of people who suffer with these symptoms who have 
           11    Hepatitis C is always hard to track down.  If you look at 
           12    good study across populations in specialty liver clinics 
           13    referral liver clinics, hepatology centers, consistently 
           14    more than 50 percent will have some other kinds of 
           15    symptoms primary fatigue and muscular-skeletal pain.  If 
           16    you look at community-based populations, it becomes 
           17    somewhat more confusing.  It's hard to track down 
           18    community-based populations, and I will have some 
           19    observations about that. 
           20      Next slide.  So what about fatigue?  There are many 
           21    causes of fatigue, most of the fatigue in Hepatitis C 
           22    patients appears directly attributable to the Hepatitis C.  
           23    It appears the fatigue of Hepatitis C may be more severe 
           24    than fatigue in other chronic medical conditions and other 
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            1    liver diseases.  When I see patients who complain of 
            2    fatigue which is very common in all populations, we need 
            3    to really address the treatable causes of fatigue.  We are 
            4    said to have a chronic gigantic sleep debt in our culture 
            5    in the United States, and certainly among homeless people 
            6    who I see, not enough sleep is very common.  Certainly 
            7    people who are abusers of stimulants, which is common in 
            8    my population, will be common across many Hepatitis C 
            9    populations because of the linkage to injection drug use.  
           10    People who go on a run of smoking crack for three days 
           11    will feel fatigued at the end of it. 
           12      It's amazing when I talk to patients about this, and 
           13    say, you know, You're fatigued, and you might be less 
           14    fatigued if you weren't using that way, and on occasion, 
           15    people come back and say, I feel less fatigued.  Certainly 
           16    depression, thyroid dysfunction and the full range of 
           17    medical problems that cause fatigue need to be addressed.  
           18    I'm going to talk about extrahepatic syndromes and 
           19    disorders, and the more we can treat those, the more we 
           20    may be able to get traction on the fatigue but for some 
           21    portion of the patients with Hepatitis C and fatigue, it's 
           22    severe profound fatigue, and they're not going to feel 
           23    better.  
           24      Interestingly, successful treatment of Hepatitis C with 
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            1    antiviral agents does improve the fatigue in many, but not 
            2    all of these patients.  I'm also a HIV treater and 
            3    specialist, around long enough that I heard the statements 
            4    made from some experts early on in the HIV epidemic, Well, 
            5    at least HIV isn't a painful disease, and it's come to be 
            6    recognized much more that HIV certainly can be a painful 
            7    disease, and the same is true of Hepatitis C.  The typical 
            8    pain they have is myalgias and arthralgia, muscle and 
            9    joint pains, are often similar to the type of joint pain 
           10    that patients with rheumatoid arthritis might get.  They 
           11    might complain of morning stiffness, of pain multiple 
           12    joints symmetrically or few joints. 
           13      The muscle pain is deep, aching pain, very typical, and 
           14    across populations may present in 25 percent of people 
           15    with chronic Hepatitis C.  Neuropathy, headache, abdominal 
           16    pain, I'll have something to say about all this.  Just to 
           17    touch on the other somatic symptoms, itching  -- you heard 
           18    that a few times here -- Well, somebody may say, Why would 
           19    itching be disabling?  Those of us who have not had a 
           20    severe problem with itching just seems like  -- everybody 
           21    is going to start to itch now  -- you start to realize 
           22    that this is a continuous problem night and day.  You can 
           23    really get into trouble with not being able to function. 
           24      Cold chills, hot flashes, again, I don't know what to 
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            1    make of that, except that patients very frequently 
            2    complain of it.  Sleeping problems, sleep disturbances are 
            3    very common altogether, but there seems to be something 
            4    about sleep disturbances and the effects of Hepatitis C in 
            5    the central nervous system, and I will have more to say 
            6    about that.  I'll come to GI symptoms.  This talk used to 
            7    be called "Hepatitis C from Head to Toe."  I'll start with 
            8    some things above the neck and move on from there. 
            9      Next slide.  Depression and Hepatitis C, depression 
           10    anxiety, somatization are all increased in Hepatitis C 
           11    appear to be increasing compared to other liver diseases, 
           12    possibly increased compared to other chronic diseases, and 
           13    again, these symptoms and syndromes may appear to be 
           14    common independent of the route of acquisition of the 
           15    Hepatitis C, and there's been some nice work done on 
           16    depression and common finding of depression and Hepatitis 
           17    C independent of somebody who was a former active or never 
           18    drug user. 
           19      Treatment, antiviral treatment for Hepatitis C can be 
           20    effective in treating psychiatric symptoms, but certainly 
           21    to be aware that that treatment particularly with 
           22    interferon is very likely to exacerbate the psychiatric 
           23    symptoms before it makes them any better.  Neuro-cognitive 
           24    impairment, somebody mentioned brain fog earlier.  What is 
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            1    this?  Patients complain of memory loss, can't 
            2    concentrate, can't think straight.  Sometimes it's quite 
            3    diffuse.  It can occur with both mild liver impairment and 
            4    severe liver impairment. 
            5      You heard a little bit about clinical and subclinical 
            6    hepatic encephalopathy.  Again from a disability and 
            7    functional point of view, the hepatic encephalopathy in 
            8    somebody with cirrhosis that can be demonstrated on 
            9    neuropsychiatric testing may still have severe effects on 
           10    people's daily and work function.  You lose a step of 
           11    concentration, that's going to be a very significant 
           12    factor in a lot of work.  Why even in the patients who 
           13    don't have cirrhosis of the liver do we see this cognitive 
           14    dysfunction?  It's interesting because it can be 
           15    demonstrated that there's something going on in the brain 
           16    in these patients.  You can do proton MRS, invoke 
           17    potentials.  There's abnormalities in the Hepatitis C 
           18    patients that are not present in patients with other liver 
           19    disease or patients without Hepatitis C. 
           20      No one really understands this well.  I can give you my 
           21    theory which is that it's an immunological phenomenon.  
           22    There's a high degree of activation of the immune system, 
           23    millions of particles of virus, probably some Hepatitis C 
           24    in the central nervous system, and all of that activation 
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            1    of the immune system is releasing cytokines, finding 
            2    things locally that is having some effect on the central 
            3    nervous system.  Somebody tomorrow may have a better 
            4    explanation than that, and I would welcome it. 
            5      Next slide, the patients will complain of brain fog.  We 
            6    can often understand this by taking a careful history, 
            7    talk to people about their work.  Somebody mentioned the 
            8    accountant who can no longer add up their column of 
            9    numbers.  This is true in many areas across the spectrum.  
           10    People who say, I never used to forget my keys.  I never 
           11    used to be in this situation, and now these things are 
           12    happening to me.  I'll say, the first thing I think, Does 
           13    this patient have depression?  Can I treat the depression, 
           14    and will they get better?   Even if they don't have 
           15    depression, can I treat them for depression, and will they 
           16    get better?   It's a tough thing to deal with.  Certainly 
           17    neuro-psych testing is helpful.  It needs to be done in a 
           18    comprehensive manner. 
           19      The very simple kinds of neuro-psych testing often will 
           20    not uncover this, but a battery of  test appropriately 
           21    administered will get at what's the degree of impairment 
           22    and it's in the range of concentration and memory usually.  
           23    Sticking with the head and neck, dry mouth, dry eyes, very 
           24    common, dental problems from the dry mouth are common.  
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            1    Lots of reasons the patients get dry mouth.  The 
            2    medication cause them to have dry mouth.  Sometimes 
            3    patients smoking cigarettes or smoking cannibis which will 
            4    cause dry mouth.  I always laugh at my patients when they 
            5    say, I need some medical marijuana for the symptoms, and 
            6    by the way, my mouth is very dry.  This may be a problem.  
            7    Is this is a disabling problem?  Perhaps it's not a 
            8    disabling problem on its own, but taken together with a 
            9    number of other things that people may have, this 
           10    contributes to poor nutrition and poor functioning. 
           11      The good news about thyroid disease is that most 
           12    patients with Hepatitis C don't have overt thyroid 
           13    disease.  The bad news is when we start to treat them, a 
           14    lot of them are going to have overt thyroid disease, so we 
           15    ought to pay attention to it. 
           16      The Hepatitis C and the skin, I thought I would take a 
           17    minute to talk about this.  This is something that I see 
           18    fairly frequently.  I mentioned itching already.  
           19    Porphyria cutanea tardus which is a position I always 
           20    figured is something that I needed to know about for board 
           21    exams and not in real life.  In a population that has a 
           22    high prevalence of Hepatitis C, I now see porphyria 
           23    cutanea tardus quite frequently.  I give a talk and show 
           24    some pictures of it.  I spared you the gory pictures, but 
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            1    as soon as I give the talk, I get calls from physicians 
            2    and nurse practitioners, "I diagnosed it; I saw it."  This 
            3    is a condition that is associated with sun exposure, light 
            4    exposure.  People have developed blisters and vesicles, 
            5    and large blisters can be very unsightly and deforming.  
            6    In a few cases of people who have it pretty bad, it can 
            7    entirely prevent them from going out.  Certainly 
            8    vasculitis runs a spectrum from mild rash to severe skin 
            9    ulcers to systemic diseases, and a whole range of other 
           10    skin problems associated with Hepatitis C.  Again, I 
           11    mention it mainly to put it on the table as these things 
           12    happen, and if all we think about is Hepatitis C and the 
           13    liver, we're going to miss the fact that all these other 
           14    things are associated. 
           15      Endocrine conditions connected with Hepatitis C and 
           16    diabetes from a disability point of view, these things all 
           17    add up.  Endocrine effects of cirrhosis, certainly the 
           18    decrease testosterone in men is going to affect energy 
           19    levels as well as sexual functioning, and there may be 
           20    other issues when a patient has cirrhosis that are not 
           21    directly attributable to the liver disease. 
           22      Coming closer to the liver, Hepatitis C and the 
           23    digestive tract, abdominal pain and nausea are common 
           24    symptoms at all stages of Hepatitis C.  This thing with 
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            1    nausea, kind of, took me by surprise because I kept 
            2    hearing people complain about nausea, and I said, you 
            3    know, that's just angst.  But careful reviews in large 
            4    Hepatitis C populations show very substantial amounts of 
            5    patients complaining of nausea that doesn't seem very 
            6    amenable to usual treatments.   A little good news, 
            7    there's a whole bunch of gastrointestinal problems that 
            8    are not necessarily related to Hepatitis C directly and 
            9    ought to trigger us to looking at some of the other 
           10    problems.  So what about this joint pain, arthralgia and 
           11    muscle pain?  There's a long list of rheumatological 
           12    symptoms and syndromes probably related to autoimmune 
           13    phenomenon triggered by the general immune activation from 
           14    having this chronic infection. 
           15      I'm going to concentrate my remarks on the joint pain 
           16    and muscle pain and on the mixed cryoglobulinemia.  But 
           17    just to say it, just in case somebody knows somebody with 
           18    Hepatitis C, the confusion with Hepatitis C joint pain and 
           19    rheumatoid arthritis can really be disastrous.  A lot of 
           20    Hepatitis C have positive rheumatoid factor.  Positive 
           21    rheumatoid factor plus joint pain will trigger some 
           22    unskilled doctors to make a diagnosis of rheumatoid 
           23    arthritis, put patients on potentially toxic drugs.  I've 
           24    seen patients come in to me on methotrexate for Hepatitis 
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            1    C and rheumatoid arthritis, and their livers are rapidly 
            2    deteriorating when they never had rheumatoid arthritis and 
            3    shouldn't have been on that medication. 
            4      Next slide, the joint pain and muscle pain is usually 
            5    manageable.  Sometimes a little bit of a question how to 
            6    manage it since the one thing our Hepatitis C patients 
            7    know -- of course the one thing that's wrong is, I'm never 
            8    supposed to take Tylenol.  That's probably our best 
            9    medicine in reasonable doses for Hepatitis C.  We try and 
           10    keep patients away from many of the non-steroidal 
           11    antiinflammatories, motrin, which are safe in most of our 
           12    Hepatitis C patients, but we can't predict who it might be 
           13    hepatoxic in.  We may need to use opiate medications.  
           14    Again, this is a population of a heavy background in drug 
           15    abuse, and using opiate medications may be problematic but 
           16    may be the only way of addressing the pain.  In some 
           17    cases, this pain can be severe and disabling. 
           18      True arthritis inflammation of the joints with swelling 
           19    is unusual in Hepatitis C and should make us think of 
           20    something else, but morning stiffness which we often 
           21    associate with rheumatoid arthritis is quite common.  The 
           22    muscle pain people often describe as aching or sharp 
           23    pains.  I think I said most of that. 
           24      What about this weird thing called "cryoglobulinemia?" 
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            1    Depending on where you are in the world, it's either very 
            2    common in Hepatitis C patients or less common in Hepatitis 
            3    C patients.   We can diagnosis it with blood tests, but 
            4    blood tests are tricky to do particularly those who don't 
            5    work in a hospital with a lab at hand, so it needs to be 
            6    done right, and thankfully, the severe syndrome of 
            7    cryoglobulinemia which might fall into systemic autoimmune 
            8    disease phenomenon is not that common.  On the other hand, 
            9    moderate symptoms or subacute syndrome with neuropathy and 
           10    fatigue and joint pain and cryoglobulins is fairly common.   
           11    On the bad end of the spectrum, severe vasculitis, 
           12    sometimes systemic; on the less severe end of the spectrum 
           13    peripheral neuropathy, usually a sensory painful 
           14    neuropathy parastasis but sometimes motor as well and the 
           15    joint pain, Reynaud's Phenomenon, and we fear kidney 
           16    involvement, and kidney disease from Hepatitis C is 
           17    rapidly coming to be in some settings the most common 
           18    reason why patients are on dialysis in the areas that have 
           19    a lot of injection drug users and a lot of Hepatitis C. 
           20      The next slide, what about Hepatitis C and the nervous 
           21    system, and we'll talk more about peripheral neuropathy 
           22    because this is something that's frequently problematic 
           23    for our patients.  Patients complain of pain.  They 
           24    complain of pain, numbness, burning, needles and pins 
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            1    sensations in the hands and feet, sometimes weakness in a 
            2    particular area, possibly carpal tunnel syndrome may be 
            3    more common in Hepatitis C and often gets to the point of 
            4    affecting people's ambulation and affecting people's 
            5    mobility.  Certainly, people who either have jobs that 
            6    involve manual labor and a lot of physical exertion and 
            7    walking around, this is very problematic, too, and it's 
            8    very problematic to many of our patients who have chronic 
            9    pain because of this and can't concentrate and do their 
           10    regular work.  
           11      You can diagnosis this by doing electrophysiological 
           12    studies or ceryl nerve biopsies.  I don't recommend that.  
           13    You can usually get down to it with a history and physical 
           14    exam and realizing that in fact this is a real cause of 
           15    neuropathy and then trying to treat this the way we treat 
           16    other neuropathic pain.  If the neuropathy is very severe, 
           17    we ought to be going look for blood clots and anemia. 
           18      This was covered well several times.  I'm going to do it 
           19    again just because I think it's important.  What about 
           20    patients with cirrhosis?  What amounts  -- why would 
           21    patients with cirrhosis be disabled?  The clinical and 
           22    subclinical encephalopathy, again, our patients 
           23    significant others know, our patients co-workers know, our 
           24    patients probably know themselves, our patients' doctors 
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            1    don't always know that this is what's going on.  It 
            2    changes from day to day.  Patients who come to the doctor 
            3    and say, I'm having a good day today, but yesterday I just 
            4    couldn't think straight.  What's that?  And the physicians 
            5    don't realize this is going to wax and wain. 
            6      Abdominal pain, again pain as a whole, why is pain 
            7    disabling?  This is one of the difficulties in just paying 
            8    attention to the listings. 
            9      Pain is disabling because of inability to concentrate.  
           10    Pain is disabling because the medications that we use to 
           11    treat pain may have other side effects.  Pain can be 
           12    disabling because of the direct effects on ambulation on 
           13    movement.  But a pain is subjective, and sometimes it's 
           14    hard to get at.  Why are patients who have right upper 
           15    quadrant pain when the liver doesn't have any nerve 
           16    endings as I'm constantly reminded by my hepatology 
           17    colleagues?  I'm not sure, but our patients do complain of 
           18    that. 
           19      Ascites, when you have a lot of ascites, it's hard to 
           20    move.  It's hard to breathe.  Chronic anemia, low blood 
           21    proteins, patients will have weakness and severe fatigue 
           22    in those situations.  Finally, the frequency of medical 
           23    visits and hospitalizatons.  When patients need frequent 
           24    paracentesis for massive ascites, that may mean once a 
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            1    week for some of these patients.  That may be something 
            2    that is very problematic, certainly development of 
            3    infections and hemorrhage when people are in and out of 
            4    the doctor's office and in and out of the hospital and may 
            5    not have time for (inaudible, intercom system). 
            6      HCV in all stages has been linked to decreased quality 
            7    of life.  Primarily related to extrahepatic syndromes, we 
            8    know when we treat some of these extrahepatic syndromes, 
            9    particularly depression and painful syndromes, quality of 
           10    life will improve.  We need to pay attention to the fact 
           11    that when we treat Hepatitis C successfully, the quality 
           12    of life usually will improve.  During the course of  
           13    treatment, the quality of life may not. 
           14      Next slide, with the question of quality of life, we do 
           15    have some questions.  There are some population surveys 
           16    that show that people who don't know that they have 
           17    Hepatitis C appear to have less fatigue and less 
           18    complaints, and that's been attributed to a labeling 
           19    effect and also attributed to what's called a lack of 
           20    knowledge and incorrect knowledge effect.  When our 
           21    patients, all they know is they have Hepatitis C, and this 
           22    is a potentially fatal disease, but haven't had a lot of 
           23    education about what else is there with it, they may 
           24    definitely have fear of depression and symptoms. 
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            1      There's other very good work showing a global effect on 
            2    quality of life that doesn't seem attributable strictly to 
            3    depression and labeling, and I think that that's very 
            4    real.  It's certainly very real in my patients' lives.  
            5    The other thing that's very interesting is that people 
            6    with Hepatitis C are underrepresented in the workforce.  
            7    This is shown in wide population data and as well as some 
            8    data from looking at welfare work data in Texas.  Perhaps 
            9    this is related to the underlying problems of how people 
           10    got their Hepatitis C in the first place, involvement with 
           11    drugs, but it seems to go beyond that, and it seems to 
           12    speak to the global decrease in quality of life and 
           13    fatigue and other symptoms that are keeping people out of 
           14    work.  It appears to be a fact that patients with 
           15    Hepatitis C are not in the workforce as much as others. 
           16      In summary of this section, Hepatitis C is very 
           17    prevalent.  It affects the liver and multiple body 
           18    symptoms.  Disabling symptomatology is common, and the 
           19    current listings don't recognize that. 
           20      I'm going to take just a minute to talk about treatment.  
           21    Standard of care treatment for Hepatitis C now is 
           22    (inaudible) interferon plus ribavirin.  A little bit about 
           23    the side effects of interferon, generally when we talk 
           24    about flu like symptoms, it's a funny thing to say.  It  
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            1    means something to doctors.  I'm not sure it means 
            2    something to everyone else.  But when we talk about 
            3    feeling feverish, feeling headachy, feeling generally 
            4    lousy and that goes on for weeks to months, those may be 
            5    the flu like symptoms that some of our patients are 
            6    experiencing. 
            7      Generally, we're recommending for the average patient 
            8    and the average patient definitely has Geno type 1 
            9    Hepatitis C, a year of therapy.  For patients who are 
           10    lucky enough not to have Geno type 2 and 3, six months' 
           11    therapy may be adequate.  For patients who are unlucky 
           12    enough to be co-infected with HIV and Hepatitis C, 18 
           13    months of therapy may becoming the standard.   That's a 
           14    long-term therapy.  
           15      The other piece of therapy in addition to interferon is 
           16    the ribavirin.  Main problem with ribavirin is related to 
           17    anemia, and that's treatable during the course of 
           18    treatment but adds extras injections, adds extra 
           19    possibility of side effects, and people may  -- partly 
           20    because the treatment for anemia is very expensive -- 
           21    people may spend a lot of time anemic before they actually 
           22    are receiving erythropoietin to treat their anemia, and 
           23    they may be fatigued because of that. 
           24      I'm going to take a detour, next slide, and talk about 
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            1    homelessness and what I do because the weight of the 
            2    pathology in Hepatitis C does seem to fall into the 
            3    populations that are homeless that are at risk of homeless 
            4    that struggle with severe poverty.  There's at least a 
            5    million people homeless in the United States.  They have 
            6    worse overall health status in terms of medical status, 
            7    mental status and substance abuse than non-homeless 
            8    populations, including non-homeless poor people. 
            9      There are multiples barriers to medical care for 
           10    homeless people.  For many homeless people, the scope of 
           11    living is narrowed to mere survival, and their existence 
           12    is marked by multiple traumas and losses.  This is what I 
           13    see everyday, and it's relevant to asking me about 
           14    treatment for Hepatitis C.   Homeless people may not trust 
           15    institutions, including medical institutions, and 
           16    homelessness and its associated issues are stigmatizing 
           17    and shame is very important.  Hepatitis C is stigmatizing, 
           18    and substance abuse is stigmatizing, and mental health 
           19    disorders are stigmatizing.  Looks like the whole thing is 
           20    stigmatizing.  
           21      People with past or current injection drug use make up 
           22    the largest group of people infected with Hepatitis C.  
           23    For a variety of reasons, people who use or have used 
           24    injection drugs face severe poverty, disenfranchisement, 
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            1    barriers to health care and homelessness.  I mentioned 
            2    that homeless people have overall worse health than other 
            3    groups. 
            4      So why am I talking about this because 20 to 50 percent 
            5    homeless individuals across various populations are 
            6    positive for Hepatitis C.  In terms of homeless 
            7    populations, African Americans, alcoholics, Geno type 1 
            8    patients are overrepresented.  Those are the patients who 
            9    are the hardest to treat for Hepatitis C, and have the 
           10    worst outcomes and have the least ability to resolve their 
           11    Hepatitis C infection.  Finally, treatment is infrequently 
           12    offered to homeless people. 
           13      I would mention that 20 years ago, widespread 
           14    homelessness was considered an emergency.  We seem to be 
           15    stuck with it today.  We need to develop systems to be 
           16    able to deliver adequate, in fact excellent, home health 
           17    care to homeless people.  We need to develop systems in 
           18    which we can house the sickest homeless people who are 
           19    homeless people who are the most likely to be sick that 
           20    includes people who are candidates for being treated with 
           21    interferon, ribavirin for Hepatitis C, but homelessness 
           22    will interfere with their ability to adhere to that, and 
           23    adhereness is one of the strongest predictors of success. 
           24    Certainly the extent of medical problems in homeless 
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            1    people and the range that it goes across different disease 
            2    systems is very significant, and we need to recognize 
            3    that. 
            4      Just to talk about Hepatitis C for a moment, the 
            5    treatment can be effective.  We know that it's worth 
            6    doing.  It's also clear that it's very arduous to the 
            7    individual being treated and the provider of treatment.  
            8    We need to have systems in place that can work.  Oddly in 
            9    many instances, the treatment which is very costly may be 
           10    available to patients, may be available through drug 
           11    companies, through charitable programs, may be available 
           12    through state Medicaid programs.  But the other 
           13    information that patients need to benefit from treatment, 
           14    including housing, is not available.  Those are things 
           15    that may be less costly, but they are not paid for. 
           16      Let me talk about my experience in our health center 
           17    where a large health care for homeless program and primary 
           18    care health center, part of the DPH in San Francisco, 
           19    typical for people who take care of poor people, we're 
           20    undergoing budget cuts at the moment.  I don't know what 
           21    I'm going to find when I get back.  Inner city San 
           22    Francisco, we review 3,000 charts of primary care 
           23    patients.  Over a 1,000 have Hepatitis C antibody, and 
           24    that's about a third of our patients.  We were able to do 
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            1    some confirmatory HCV viral load testing fairly randomly 
            2    on those patients, and 25 percent of those patients had a 
            3    negative viral load, meaning that they resolved their 
            4    Hepatitis C infection.  This is important because that's 
            5    not a test that's widely available to people practicing 
            6    with uncertain people.  We may be able to tell 25 percent 
            7    who are fatigued and sick because they think they have a 
            8    severe disease; that good news, you don't have that 
            9    diseases.  Despite that, we haven't been able to get that 
           10    to our patients.  
           11      In San Francisco in the public sector, we have two 
           12    part-time hepatologists available to treat an estimate of 
           13    somewhere in the range of five to 10,000 Hepatitis C 
           14    patients.  As you might imagine, that's not going to go 
           15    very far, and very few patients, maybe one to 200 a year, 
           16    get treated by our hepatologists at San Francisco General 
           17    Hospital; therefore, we decided in our health center, 
           18    we've got to do something about this.  Can we treat 
           19    Hepatitis C in a general medical setting with a very 
           20    strapped resources, and we have started treating patients.  
           21    We have four doctors and one nurse with special interest 
           22    in Hepatitis C who are doing this.  The only extra thing 
           23    that we've been able to get is about four hours of extra 
           24    nursing time to try and track these patients. 
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            1      We've treated 17 patients, three were infected, six of 
            2    them with Geno type 1 which may speak to some selection 
            3    bias as to who we decided to treat because the spectrum of 
            4    those patients with Geno type 1 in our population is no 
            5    different than in any other population with injected drug 
            6    users; fourteen completed their treatment; three dropped 
            7    out; and eight of the 14 had a sustained viral response 
            8    which is what we're looking for. 
            9      So I have some recommendations.  First of all, Hepatitis 
           10    C is currently the most common liver disease, one of the 
           11    most common chronic infectious diseases that are prevalent 
           12    in America, and that ought to merit some special 
           13    discussion in the listings.  This is something across the 
           14    spectrum that affects a huge number of people.  I would 
           15    recommend an introductory text similar to that used for 
           16    the HIV in Section 14 be implemented to direct the 
           17    evaluators to the complexity of the condition.  The text 
           18    could also cover discussion of issues of treatment-related 
           19    effects.  In terms of the treatment, the one good thing 
           20    from a disability point of view of the treatment, although 
           21    the treatment can be disabling to many patients because 
           22    only a fraction of patients will respond to the treatment 
           23    and because we know that if people haven't responded in 
           24    the first three months, we can stop treatment after three 
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            1    months in many of patients who have not responded, so they 
            2    are not going to have a long-term disability from 
            3    treatment, itself, in those we have stopped. 
            4      A specific section on Hepatitis C be included in the 
            5    listings to include reference to hepatic and extrahepatic 
            6    manifestations and to direct evaluators to the appropriate 
            7    sections, for example, with hepatic encephalopathy and 
            8    directing people to the sections on organic mental 
            9    disorders. 
           10      Finally, a specific text regarding functional criteria 
           11    be included in Hepatitis C listings because this can have 
           12    profound effects on functioning that's very similar 
           13    probably for the same reasons in terms of a chronic 
           14    infectious disease with a high degree of immune system 
           15    activation as HIV and a section similar to what's in 
           16    Section 1408 and for HIV be included and that this is 
           17    technically important in patients I see who are homeless 
           18    and whose functional disabilities may be very profound and 
           19    easy to document than their medical conditions because 
           20    their medical care  -- expert medical care may not be 
           21    available to those people. 
           22      That's the end, thank you.
           23                            (Applause.)
           24                            DR. SEEFF:   Thank you for that; 
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            1    it gave another perspective looking at the other important 
            2    components.  All I can tell you is that I spent two years 
            3    writing the guidelines for treatment, and you managed to 
            4    get it in a few slides of your presentation is something 
            5    else.  The focus that you have is an important focus, and 
            6    it is something that we need to spend time on.  
            7    (Inaudible) I would like to just remind people that 
            8    treatment is significantly less effective in African 
            9    Americans.  I think many people may not be aware of that, 
           10    and for reasons that are  not fully clear, the response 
           11    rate in African Americans who predominantly have geno type 
           12    1 -- 97, 98 percent of African Americans have got geno 
           13    type 1 which is the more difficult to treat as compared to 
           14    about 75 percent Caucasian; that's not the only reason.  
           15    We have a big study called "Viral Hep C Trial" trying to 
           16    look at this issue of why there's non response comparing 
           17    African Americans.  So this is a very important component 
           18    and something that we need to consider when we talk about 
           19    treatment of African Americans. 
           20      We're not sure of other ethnic populations, Native 
           21    American Indians for example, because little has been done 
           22    in looking at the disease in that group of individuals, 
           23    and there again, there are problems of our acceptance of 
           24    medicine; and there are tribal issues that need to be 
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            1    discussed as their there are initially in African 
            2    Americans who were concerned about being involved in the 
            3    study because of their past history having been involved, 
            4    in retrospect, in grievous errors.  But this is an 
            5    important issue that we can reside about the difference in 
            6    response rate based on ethnic origins.
            7                            MR. JULIAN:  We're going to jump 
            8    back in if we could.  We've got two more panel speakers.  
            9    Questions and answers from anybody, and we hope that we 
           10    get good comments from everybody and appreciate the 
           11    comments everybody has made so far.  Ms. Siegel from the 
           12    Disability Law Center will be speaking, and then Ms. Kukka 
           13    will be following.  Thank you. 
           14                            (A short recess was taken.)
           15                            MS. SIEGEL:  Hi, good afternoon, 
           16    I'm Barbara Siegel from the Disability Law Center, and 
           17    first of all, I just want to thank Social Security for 
           18    having this conference.  It's really been fascinating for 
           19    me, not being a medical person, but being interested in 
           20    medical things and thank you for inviting me.  So I took 
           21    down the palm tree slide, just imagine it's still there.  
           22    I'm going to pick up on some of the threads from Dr. 
           23    Zevin's talk but from the perspective of an advocate, not 
           24    a clinician, and I'm going to talk about access to 
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            1    treatment issues and the relationship of those issues to 
            2    proving disability and coming up with evidence, that kind 
            3    of thing, and then I'm going to be flag some advocacy 
            4    issues that come in up in chronic liver disease cases. 
            5      My background is as a legal services lawyer for almost 
            6    10 years representing people who are trying to get on 
            7    disability benefits, and now I mostly represent people who 
            8    are on disability benefits and who are trying to go back 
            9    to work, so I've seen both ends as well. 
           10      First thing I want to talk about is really a big 
           11    disconnect between the evidentiary requirements in the 
           12    proposed listings and the reality of a lot of people's 
           13    lives who have chronic liver disease.  Just to start off, 
           14    this is the requirement in both the adult and kids' 
           15    listings about the need for longitudinal evidence covering 
           16    a period of at least six months with observations and 
           17    treatment unless they can make a favorable determination 
           18    without it.  I understand why that's there, and why that's 
           19    good practice.  But the reality, it just doesn't work in a 
           20    lot of cases with the reality of some people's lives, and 
           21    Dr. Zevin described at least part of the population with 
           22    chronic liver disease in a lot of detail, so I'm not going 
           23    to go into it real deeply. 
           24      At the risk of sounding like John Kerry in the debates, 
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            1    I'm going to throw out a few statistics just to add to the 
            2    picture of it.  IV drug use accounts for 60 percent of 
            3    Hepatitis C transmission, and Hepatitis C, the prevalence 
            4    in the homeless community is at 10 to 20 times higher than 
            5    that in the non-homeless community.  This is according to 
            6    a book that came out a couple of months ago from the 
            7    Boston Health Care for the Homeless Program in conjunction 
            8    with the health care for the homeless program. 
            9      The other reality is just lack of health insurance 
           10    generally, and what that means for the treatment, and 
           11    these are some numbers that came out of the Kaiser Family 
           12    Foundation report.  The 45 million number we have heard ad 
           13    nauseam, so we won't go into that.  Even here in 
           14    Massachusetts, roughly 500,000 non-elderly uninsured, and 
           15    there are huge disparities between different ethnic and 
           16    racial groups about who's uninsured.  The highest rate of 
           17    lack of health insurance is among the Latino population, 
           18    and folks who are uninsured tend to be low income people, 
           19    not necessarily the lowest income because Medicaid picks 
           20    up a lot of that group, and people in poor and fair 
           21    health, and these folks tend to be uninsured for a longer 
           22    period of time which has implications for the kind of 
           23    treatment they get. 
           24      So when you put numbers like this and the things that 
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            1    Dr. Zevin talked about next to the requirement of, sort 
            2    of, six months of regular treatment with observations, 
            3    it's hard to make that work.  So that's a big problem, and 
            4    what I want to talk about is some ways to deal with that. 
            5      Now, somebody this morning mentioned this whole idea of 
            6    equaling a listing as opposed to meeting the listing, and 
            7    I think for advocates, this is one handle that people can 
            8    use if they're working with people with chronic liver 
            9    disease trying to get them access to these benefits.  What 
           10    equaling a listing means is that the person's medical 
           11    findings don't match exactly what's in those listings, but 
           12    the severity is equivalent, and for this, you look at the 
           13    whole picture.  You look at, certainly, the medical 
           14    findings, but you also look at other evidence about the 
           15    person's functioning, and this can be a way to deal with 
           16    the narrowness of the listings. 
           17      The other thing, just to put it in perspective -- I 
           18    don't know the numbers on this -- but in my experience and 
           19    that of others I have talked with, most Social Security 
           20    Disability and SSI cases aren't decided at the listing 
           21    level.  The listings are meant to be a, kind of, shorthand 
           22    for impairments that are so severe, that if you meet one, 
           23    you're disabled, no more questions asked.  But a lot of -- 
           24    I think the majority of people who get on benefits end up 
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            1    getting on at Steps 4 and 5 of the sequential evaluation.  
            2    Step 5 has to do with residual functional capacity and 
            3    ability to do other work, and for people who have not 
            4    dealt with this concept of residual functional capacity, 
            5    what it means in Social Security terms is what you can do 
            6    despite your impairments, medical and physical; and so for 
            7    people who -- for people who aren't at a listing level or 
            8    even at an equals a listing level, this is where the work 
            9    really happens; and where you need to develop the 
           10    evidence; and for this, you really need to look at things, 
           11    like symptoms, like pain, fatigue, weakness, nausea, all 
           12    of the non-hepatic type symptoms; and how those affect 
           13    functioning; and Social Security actually has a lot of 
           14    good regulations and rules about how you evaluate evidence 
           15    of these things; and the core of it is that you do need to 
           16    establish some medically determinable impairment that 
           17    could be expected to cause some symptoms; but beyond that, 
           18    you look at the person's daily activities.  I can give you 
           19    the list. 
           20      You look at the location, duration, frequency and 
           21    intensity of pain and other symptoms, precipitating and 
           22    aggravating factors, the type and dosage, effectiveness 
           23    and side effects of medication, the treatment other than 
           24    medication that you receive, other measures you take to 
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            1    alleviate the symptoms and other factors concerning 
            2    functional limitations.  So there's a lot to work with 
            3    there for people with chronic liver disease. 
            4      I just want to mention one other thing for kids.  When 
            5    you talk about equaling a listing, there's an equivalent 
            6    of that for kids.  It's called "functional equivalence" 
            7    where kids don't have a listing level of impairment, you 
            8    do a similar analysis.  There are six domains of 
            9    functioning, and the child has to have marked impairments 
           10    in two of them that's disabling level that's functionally 
           11    equal to a listing. 
           12      One other thing I want to mention about the residual 
           13    functional capacity analysis is that it can be important 
           14    to bring in non-medical evidence as well as medical 
           15    evidence.  Once you've got your diagnosis there, if what 
           16    you're talking about is, how does a person function, you 
           17    can bring in evidence of co-workers of a person who tried 
           18    to work and had problems because of their liver disease on 
           19    the job.  You can bring in somebody who lives with the 
           20    person who can see the kinds of difficulties they have on 
           21    a day-to-day basis.  Even if somebody is in a shelter, you 
           22    can have a statement from shelter staff about how this 
           23    person functions.   So all that kind of thing is real 
           24    evidence in these cases, so it does go beyond just the 
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            1    medical evidence. 
            2      One issue I want to touch on is this issue of compliance 
            3    with treatment.  This comes up in a lot of Social Security 
            4    disability cases, not just liver disease cases, but 
            5    there's a regulation, Social Security regulation, that 
            6    says if a physician prescribes a treatment for you, and 
            7    the treatment is likely to make you able to go back to 
            8    work; and you don't follow the treatment; and you don't 
            9    have a good reason for not following the treatment; then 
           10    you can be found not disabled for that reason.  I haven't 
           11    done many of these cases, but I think this is going to be 
           12    a big issue and has been a big issue especially people, 
           13    with substance abuses, and I just wanted to point out one 
           14    of the Social Security rulings, and these are rulings that 
           15    are, sort of, sub regulatory body of provisions that they 
           16    do have the force of law, and there's one about what this 
           17    rule means about failure to follow prescribed treatment.
           18      Two things I want to point out.  One is that the 
           19    treatment really does have to be expected to restore the 
           20    ability to work and, you know, if you're at a point of 
           21    irreversible damage or condition that may improve 
           22    somewhat, but you're going to be at a disabling level and 
           23    not able to work, then failure to follow that treatment 
           24    isn't a legal reason to not get benefits.  The other issue 
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            1    is why some people don't get treatment.  One is lack of 
            2    insurance, lack of access to care, and it goes beyond 
            3    that, really, things like if you have ascites, and one of 
            4    the parts of your treatment regimen is a low salt diet, 
            5    and you're living on the street eating in soup kitchens, 
            6    there's no way you're going to be able to do that.  So 
            7    there may be situational reasons why you're not complying 
            8    with treatment. 
            9      The other issue I wanted to flag is related but also 
           10    this issue of substance abuse and the rules on this 
           11    actually changed in '95, '96.  It used to be there was a 
           12    listing -- there still is a listing for substance abuse, 
           13    but if you met the listing just for substance abuse, you 
           14    could get benefits, and that was politically unpopular and 
           15    changed in '95 and then in '96; and now what the rule is 
           16    is that you can't get benefits if -- what they call 
           17    "DINA," drug induced and alcoholism is material to a 
           18    finding of disability. 
           19      What that means, first you look at, Does the person meet 
           20    the definition of disability considering everything, 
           21    including the substance abuse.  If they do, you look at 
           22    what limitations would be left if the substance abuse 
           23    didn't exist, if it stopped, and if the person would still 
           24    be limited at a disabling level in the absence of 
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            1    substance abuse, then it's not material, and the person 
            2    should be able to get benefits.  But there's another 
            3    nuance to that, too, which is that if you can't tell, if 
            4    it's just unclear, maybe there hasn't been a period of 
            5    sobriety; or it's just that you just don't know what's 
            6    going to happen; then there's  -- this is referring to 
            7    Social Security and other sub regulatory provision called 
            8    an "emergency message," which basically says if you can't 
            9    tell what the limitation would be without the substance 
           10    abuse, then it's still not material, and, you know, this 
           11    is just an important point to keep in mind when 
           12    representing people in this population, and that's really 
           13    all I wanted to flag. 
           14                            (Applause.) 
           15                            DR. GRACE:  How do you define 
           16    compliance?  The reason I ask is that a lot of studies 
           17    that have been done, very few patients are 100 percent 
           18    compliant different than an ambulatory basis with taking 
           19    medication as prescribed.  As a rule of thumb in studies 
           20    taking 75 to 80 percent prescribed medication, that isn't 
           21    compliant. 
           22                            MS. SIEGEL:  I don't know of a 
           23    legal definition of compliance other than following what 
           24    your doctor tells you.  It doesn't really say you have to 
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            1    comply with the majority of it or 75 or 80 percent of it, 
            2    so it's an interesting question.  But it's not something 
            3    that I think is really addressed in the regulations.  So I 
            4    think we would just look at, would what's being prescribed 
            5    make a difference in terms of the person's ability to 
            6    perform substantial gainful activity.
            7                            DR. SHERKER:  When it comes to 
            8    determining whether alcohol or drugs would have an effect, 
            9    what level is required; is it likelihood; or is it more 
           10    likely than not; what's the shade of gray?
           11                            MS. SIEGEL:  Again, it's  -- they 
           12    don't -- there's not really a standard, like, you know, 
           13    preponderance of the evidence.  I suppose the default 
           14    standard would be more likely than not based on medical 
           15    opinion. 
           16                            MS. SWEENEY:  One thing you said 
           17    about -- sorry, Eileen Sweeney.  The point made about how 
           18    most people don't get on listings level.  They get on 
           19    later.  I think that might be true at the ALJ stage or 
           20    higher.  I'm not so sure that's the case.  I think the 
           21    listings are really essential to how the State Disability 
           22    Determination Services who see the bulk of the cases, that 
           23    they're what they do, and they don't mess around with the 
           24    steps; and so the bulk of the people get on early levels.  
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            1    So even though everything you said is right about 
            2    important later levels once you're up in the process and 
            3    advocates and put in all the pieces in, as a practical 
            4    matter, if you can meet that listing at the first level 
            5    with the state agency, you're going to be a lot better off 
            6    healthwise and cash and everything like that.
            7                            MS. SIEGEL:  Absolutely.
            8                            MS. SWEENEY:  For people who don't 
            9    know the system very well, it's essential to get the 
           10    listings right for so many people.
           11                            MS. SIEGEL:  Yeah, I couldn't 
           12    agree with you more.
           13                            MR. GERRY:  One of the great 
           14    problems we have is what I call the problem of "unappealed 
           15    denials." Of the people we deny, 60 percent at the PBS 
           16    level, and 50 percent of the people who are denied do not 
           17    appeal.  If you don't get it right at the PBS level for 
           18    half the people who could have appealed but didn't, you 
           19    may  never get it right.  The waterfall steps really cut 
           20    the number of people down.
           21                            MS. SIEGEL:  That's true.  What's 
           22    also interesting, at least in Massachusetts, is that the 
           23    percentages of people who get approved are flipped at the 
           24    initial level and if you go to the ALJ hearing.  Here it's 
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            1    between 35 and 40 percent of the people who apply get 
            2    approved either on application or on the first appeal 
            3    which is a paper review, and that 50 percent that don't 
            4    get approved that go on to appeal, of these in 
            5    Massachusetts, it's close to 70 percent who get approved. 
            6      I know Social Security has been working for a long time 
            7    to make those levels of review more consistent, but it 
            8    seems it's persisted.  But I absolutely agree; it's 
            9    important to get the listings right, and if you can meet a 
           10    listing at the initial listing or in front of the ALJ, you 
           11    can meet a listing, that's preferable because you don't 
           12    get into vocational issues at that point. 
           13      I think, for me, that raises the importance of the whole 
           14    idea of equaling the listing as well as meeting the 
           15    listing. 
           16                            MR. SKLAR:  For what it's worth, 
           17    before coming over, I did look at the data on disability 
           18    listings.  I noted that back in the '80s, 80 percent of 
           19    the cases were tied to listing steps; in other words, 80 
           20    percent of the cases made initially were listings case.  
           21    The phenomenon is that if the downward trend continues, 
           22    and it's really continued for two decades to the point 
           23    where less than 50 percent of the cases are being paid 
           24    under the listings.  That's one of the great mysteries 
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            1    that we have.  In fact, we recently contracted with the 
            2    Academy of Sciences to help us figure out why is that.  
            3      Listings can be such a great screening tool for us, and 
            4    done right, they could provide better services to the 
            5    claimant and certainly reduce the burden on the agency, 
            6    but that's a continuing ball.  The fewer cases decided at 
            7    the listings level (inaudible) in the vocational factors 
            8    where the bulk of opinion is that it's much more arbitrary 
            9    and it's more difficult to come up with uniform type of 
           10    decisions.
           11                            MS. SIEGEL:  And it takes longer.  
           12    If you don't get approved early on and you have to go to 
           13    an administrative law judge, and around here it can take a 
           14    couple of years to get through the process.  If the case 
           15    here will sit at the office of hearing and appeals for 
           16    nine, 10, 11, 12 months. 
           17                            MR. GERRY:  The other thing is if 
           18    you get a lawyer, the payment of lawyer comes out of your 
           19    benefit award.  It isn't paid by the agency.  So that 
           20    every time you make someone, in effect, get a lawyer in 
           21    order to appeal, not only do you detract the time, you may 
           22    take away the substantial amount of the benefit of the 
           23    ecomonic recovery, itself.
           24                            MS. SIEGEL:  Except for legal 
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            1    services.
            2                            MS. SWEENEY:  If you don't get a 
            3    lawyer, you may never get your benefits. 
            4                            MS. SIEGEL:  That's true.  The 
            5    approval rate for people who are represented either by a 
            6    lawyer or a non-legal advocate are much much higher. 
            7                            AUDIENCE:  What's the cost to your 
            8    security administration, Social Security Administration, 
            9    for having to have those initial applicants not approved 
           10    when they go to the appeal process?  Have you done a study 
           11    to see whether or not the agency is benefitting or maybe 
           12    not benefitting from having that kind of a situation?
           13                            MR. GERRY:  I can't give you exact 
           14    numbers, but to put it into perspective, my best estimate 
           15    is for 2005, we will spend just under $7 billion to 
           16    administer the disability program.  Start with that amount 
           17    of money which is a lot of money.  Of that $7 billion, we 
           18    will give less than half to the state determination 
           19    agencies.  It isn't fair to say that all that money is 
           20    necessarily spent on appeals.  There are 650,000 of those 
           21    per year.  But we also spend a lot of money on medical and 
           22    vocational experts to support that process. 
           23      I do believe that we could well invest significantly 
           24    more money in the front end if we got significantly 
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            1    better, more accurate and comprehensive set of results.   
            2    It's costing us definitely in the billions to shift as 
            3    much work down the line as we can. 
            4      It's not going down.  Both our rate of receipts went up 
            5    to almost 8.2 million this year, and the hearing rate is 
            6    going up simply because there are more cases coming 
            7    through the system.  That will keep going as the baby 
            8    boomers hit. 
            9      If you look at statistics of who files the claims -- 
           10    obviously age is a major factor -- Social Security 
           11    retirement age has gone up for some people, not of me, but 
           12    for some people.  That will simply add more people to the 
           13    number of people who are filing because there will be that 
           14    many more years of retirement.  So you can just anticipate 
           15    a continued growth, and I don't think three million claims 
           16    a year at all is a crazy number when you're at 2.8 
           17    million.
           18      So I think my point is -- you make a good point.  It's 
           19    one that the commissioner has made which is.  If you're 
           20    ever going to get on top of that process for a variety of 
           21    reasons, you really want to make the best possible 
           22    decisions as early as possible, and I think underlying 
           23    that has to be the willingness to pay the amount of money 
           24    to get the best expertise; and that's something she has 
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            1    been talking about quite a bit. 
            2      There's no question it'll cost us more money, but we, in 
            3    many cases  -- I'm looking at Ron Bernosky going to 
            4    hearing; you've had somebody who has been paid as an 
            5    expert at the state level; you've had consultative 
            6    examinations that either didn't answer the questions they 
            7    were asked or answered questions that didn't matter; you 
            8    have experts who have never seen the particular patient; 
            9    trying to give opinions sometimes in bulk forms, sometimes 
           10    not; all that adds up to a lot of money. 
           11      So it's not that we're not spending, ultimately, a lot 
           12    of money.  The question is, When you finally get to the 
           13    adjudicator, the relevant information is there. 
           14                            AUDIENCE:  I commend you for 
           15    holding meetings like this.  I'm in the front end.  I 
           16    think it's been terrific to hold meetings like this.
           17                            MS. KUKKA:  Thank you very much 
           18    for your endurance.  I know it's been a long afternoon.  
           19    My name is Christine Kukka, I would like to thank the 
           20    Social Security Administration for having me.  I'm from 
           21    Scarborough, Maine.  I would like to offer my thoughts 
           22    about the proposed rules, how they pertain to children 
           23    with viral hepatitis.  I will also delve into the Asian 
           24    American community, but it really pertained to a lot of 
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            1    refugee communities in my comments. 
            2      I come to you wearing three hats; one as a parent of a 
            3    child with chronic Hepatitis B; another as a patient 
            4    advocate; thirdly, as a medical writer.  I'm left my 
            5    journalism job many years ago and became very active on 
            6    the hepatitis front, and I was the primary writer and 
            7    editor of the CBC-funded P Kids Pediatric Hepatitis 
            8    Report.  It explores Hepatitis B and C in children, the 
            9    available treatment options and the emotional and civil 
           10    rights issues that arise when children blood born 
           11    infection attend day cares centers and schools and 
           12    participate in school sports.  
           13      The experience of children with viral hepatitis in this 
           14    country is an untold story of avoidance and ignorance.  As 
           15    you know, nearly one-half of the 1.25 million with chronic 
           16    hepatitis are of Asian American descent as is my daughter.  
           17    Like 20 to 30 percent of those chronically infected, she 
           18    acquired her infection at birth or during early childhood. 
           19    She was born in China and either infected by her birth 
           20    mother or by an unsafe injection during her months in the 
           21    orphanage. 
           22      Do not be lulled into thinking this happens only in 
           23    developing countries.  In 1999 -- I know someone had asked 
           24    about looking at the rate of perinatal infection here in 
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            1    the US.  One CDC study followed 19,000 Hepatitis B women, 
            2    and that was the infection rate in their offspring.  
            3    Either the vaccine was not administered in time, or the 
            4    mother's viral for whatever reason, it was not effective.  
            5    I'm not sure HV may also have been administered there.
            6      Today, only 22 states currently screen pregnant women 
            7    for Hepatitis B.  I'm amazed that the E-mail lists which 
            8    you, Rodolfo, and I participated in several Asian American 
            9    mothers who say, "You know, I never knew I had this until 
           10    I was pregnant," and half of them happen to be lucky 
           11    enough to live in a state where they screened pregnant 
           12    women.  While all the medical associations recommend that 
           13    pregnant women be screened, sometimes it comes down to 
           14    state requirements. 
           15      As we all know, unless a HB or hepatitis B vaccine is 
           16    immediately administered, 90 percent of babies born to 
           17    infected women will contract the infection.  In contrast, 
           18    six percent of Hepatitis C infected moms will infect their 
           19    children.  Despite the CDC's recommendation for universal 
           20    B immunization during infancy, Asian American Children 
           21    have a low rate vaccination, only 50 percent.  Today, only 
           22    20 percent of Asian American teens have been vaccinated 
           23    against Hepatitis B. 
           24      So you see, though my daughter was born in China, her 
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            1    experience is not that different from other Asian 
            2    Americans or first generation children in this country, 
            3    and just a depressing aside, five states still don't 
            4    require Hepatitis B vaccine for public school entry.  I 
            5    live in Maine; I live in one of them. 
            6      Because Hepatitis B often not diagnosed or treated until 
            7    after decades of infection and serious liver damage, the 
            8    incidence of liver cancer -- we've been told  -- is very 
            9    high in very older Asian Americans. 
           10      So do we really know -- let me go back.  If we're 
           11    failing to identify infected adults, how do we fear in 
           12    children with Hepatitis B?  We don't know.  There are no 
           13    accurate reports of how many children and teens are 
           14    chronically infected with Hepatitis B.  Immigrants, for 
           15    example, are not screened when they come to this country.  
           16    Viral hepatitis infections are usually silent in children 
           17    and young adults, causing them symptoms.  Obviously, this 
           18    is why 30 percent of adults with Hepatitis C know they're 
           19    infected. 
           20      Frequently, the identified or diagnosed children are 
           21    those adopted overseas and screened for blood-born 
           22    infections as recommended by the American Academy in 
           23    Pediatrics.  Their parents have the health insurance, the 
           24    resources to monitor their child's health carefully 
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            1    through regular doctor visits.  What happens with the 
            2    children who are diagnosed?  What happens when their 
            3    infectious disease status is shared with day care 
            4    providers and schools, often disaster.  
            5      My daughter was dismissed from a daycare because of her 
            6    infection.  Her teacher asked she be moved to another 
            7    classroom because she thought her hepatitis might pose a 
            8    threat to a child who was recovering from leukemia.  We're 
            9    straight, white, middle class, college educated.  Think of 
           10    the fears that immigrants or first generation families 
           11    must face.  Are they going to ask to be tested for 
           12    Hepatitis B?  Adding to that are the cultural practices 
           13    that discourage the political activism that has gained 
           14    medical coverage, public awareness and civil rights 
           15    protection and disability coverage for the HIV-AIDS 
           16    community.
           17      I have raised these social, political, cultural, 
           18    economic, emotional issues because I believe it impacts 
           19    the medical care of the Hepatitis B community in the 
           20    United States.  It affects their access to health care  
           21    whether infected pregnant woman are screened, whether 
           22    children are immunized at birth; whether their parents ask 
           23    for children to be screened; whether children are 
           24    monitored; and also it affects the sexual transmission of 
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            1    Hepatitis B especially among sexually active teens and 
            2    young adults who have missed early childhood immunization. 
            3      As you heard today, most infected children are 
            4    asymptomatic, those that happen to be identified and 
            5    monitored.  A child infected at birth has an immune system 
            6    that fails to notice the presence of the virus.  As a 
            7    result, the immune system doesn't attack the infected 
            8    liver cells, so their ALT levels never rise.  They may 
            9    have a sky high viral load, but in the absence of  -- 
           10    where you have normal ALTs, treatment has tended not to be 
           11    effective. 
           12      The current AFLD guidelines recommending treating 
           13    children only if their ALT levels are twice normal over 
           14    six months.  A diagnosis of cirrhosis is not currently 
           15    required even though your proposed guidelines, Section 
           16    105.05 requires cirrhosis to be present to be qualified 
           17    for disability.  Cirrhosis can take years to develop.  
           18    Children may qualify for treatment with elevated ALTs over 
           19    six months and have advanced fibrosis, and treatment may 
           20    be invaluable in preventing the progression from fibrosis 
           21    to cirrhosis. 
           22      Treatment, unfortunately, what is available to children, 
           23    is limited and fairly ineffective.  Today, as you have 
           24    heard, only Lamivudine which quickly causes viral 
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            1    resistance in 19 percent in children treated for 12 
            2    months.  It's like the HIV experience.  Why would you put 
            3    children on an antiviral that they're going to begin to 
            4    resist?  It will then preclude that option for the later 
            5    in life. 
            6      The other option is conventional interferon which has 
            7    had a very lackluster performance.  Think of class, 
            8    accessibility to medical care.  When a child is treated 
            9    with standard interferon, the parent gives these 
           10    injections a week, no easy fete for anyone. 
           11      There are two promising drugs, Adefovir which does not 
           12    cause such a quick viral mutation rate.  The other is 
           13    pegylated interferon which has been used on adults with 
           14    Hepatitis C for several years.  Only this month is the 
           15    first NIH partially funded pegylated interferon trials 
           16    starting for kids with Hepatitis C.  There's not one on 
           17    the board yet for using this drug for children with 
           18    Hepatitis B even though it looks as promising as anything 
           19    has looked in a long time, but these two treatments are 
           20    what we have for children who need them. 
           21      The side effects from interferon, which I want to talk 
           22    about, while Phil Rosenthal definitely talked about kids 
           23    in bad, serious shape, who almost need liver transplants, 
           24    I want to disagree with him a little and say that six- to 
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            1    12-month course of interferon can be fairly debilitating 
            2    and can certainly cause functional disability that might 
            3    require or validate a claim for disability.   In some of 
            4    the these children, if they're working a nine to five job, 
            5    they would be out on the street after a course of 
            6    interferon instead of attending school and daycare.  They 
            7    suffer depression, joint and muscle pain, fever, stunted 
            8    growth, and this is from kids who unlike us cannot 
            9    articulate all the symptoms and all that they're feeling.  
           10      I think it's imperative that quality of life issue can 
           11    be addressed in some way, shape and form in these 
           12    guidelines and that there be a cross-threading or an easy 
           13    reference between mental issues -- I'm probably saying 
           14    this all wrong -- issues for whatever your adult 
           15    counterpart is for depression, chronic fatigue syndrome 
           16    that that be an easily referenced for kids undergoing 
           17    treatment. 
           18      Remember, if they're treated successfully, infected 
           19    children, unless they're treated successfully, infected 
           20    children become infected adults who will face an 
           21    inevitable decline in their health due to the continued 
           22    infection in their livers. 
           23      In summary, I would strongly recommend that we remove 
           24    cirrhosis as a required medical condition in children.  
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            1    Serious liver disease that requires treatment can occur 
            2    before cirrhosis develops in children.  I also recommend 
            3    that your system be expanded to acknowledge and articulate 
            4    the impact treatment will have on the physical and 
            5    emotional and social development of the children and be 
            6    specific about how -- put in some yardsticks for measuring 
            7    social development and school performance, integrate the 
            8    whole functional development aspects into the criteria. 
            9      I also ask that the listing be expanded to reflect what 
           10    you have now for HIV-AIDS.  As I believe Hepatitis B 
           11    becomes better diagnosed, specifically in Asian American 
           12    and immigrant communities, you will have a similar 
           13    generational pattern where parents will be affected as are 
           14    children.  Maybe they have symptoms at the statement, 
           15    maybe they don't, but it exerts tremendous pressure on the 
           16    family unit. 
           17      Again, keeping in mind the immigrant and first 
           18    generation community, it should be easier for people to 
           19    who deserve it to apply for Social Security.  The road 
           20    blocks facing them right now are tremendous.  So, please, 
           21    take into consideration the unique health and cultural 
           22    issues and risks that affect the Asian American and 
           23    immigrant community; and please use, where possible, 
           24    HIV-AIDS experience as a model, thank you. 
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            1                            (Applause.) 
            2                            MR. JULIAN:  We're going to open 
            3    the panel up to questions.  Open to questions, comments 
            4    for the panel, and, panelists from this morning, if you 
            5    wouldn't mind jumping in if there are questions and 
            6    concerns. 
            7                            MS. SWEENEY:  Can I just ask about 
            8    people who have comorbidities who can't get the treatment 
            9    they need because of their liver conditions, I mean, could 
           10    somebody talk about that?  Is there a problem for those 
           11    people who have health problems that don't get treated 
           12    properly? 
           13                            DR. ZEVIN:  I think I know what 
           14    your question is.  For example, patients with diabetes who 
           15    also have liver disease.   I get a lot of questions from 
           16    other physicians asking me, This medicine that we want to 
           17    use for diabetes is listed for toxic to the liver.  Can we 
           18    use it for Hepatitis C patients, and the answer is a lot 
           19    of times, we don't know.  Sometimes we know you need to 
           20    use medicine.  We the answer is often it may need more 
           21    monitoring, may have more side effects, so it increases 
           22    the complexity of treating many of the co-conditions.
           23      I would go as far as to say there are things that we 
           24    can't do.  Most of the time, if we do it, it makes it more 
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            1    complex.  It may increase the amount of medical visits, 
            2    the amount of lab monitoring, that sort of thing.
            3                            DR. SHERKER:   There's a lot of 
            4    misinformation among the medical community 
            5    particularly  -- I have patients who have been told that 
            6    they can't go on oral contraceptives.  They can't go on 
            7    cholesterol agents, all of which is  -- there's no basis 
            8    and medical fact.  So there's a lot of  -- I think  -- 
            9    this is occurring probably.
           10                            MS. SWEENEY:  You mentioned 
           11    earlier about people because of their joint pain may 
           12    actually be misdiagnosed as having rheumatoid arthritis, 
           13    and the treatment for that is?
           14                            DR. ZEVIN:  That would be one 
           15    example where a common treatment for rheumatoid arthritis 
           16    is Methotrexate, well known as a toxic substance to the 
           17    liver.  Again, you might give it to somebody with 
           18    Hepatitis C if you absolutely had to, but you would surely 
           19    look for other alternatives, and if it's being given based 
           20    on the wrong diagnosis in the first place, you wouldn't 
           21    want to do that. 
           22                            DR. SEEFF:  There's also the issue 
           23    that some patients with Hepatitis C have got autoimmune 
           24    markers.  Where this important is that the use of 
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            1    interferon in people with autoimmune hepatitis is a 
            2    serious problem and causes serious disease.  You have to 
            3    be very cautious about that.  So we have to distinguish 
            4    between people who have Hepatitis C with autoimmune marker 
            5    from people who have autoimmune hepatitis because the 
            6    treatment may be different. 
            7      Also, for example, if we use steroids to treat 
            8    autoimmune hepatitis, if the patient has underlying 
            9    Hepatitis B, this can lead to a problem when you withdraw 
           10    the steroids.  You can have a flare in hepatitis as a 
           11    consequence when withdrawing corticosteroids.  So one has 
           12    to be very, very carefully evaluate such patients to see 
           13    what kind of disease you're dealing with and what the 
           14    treatment should be.  The treatment that may be good for 
           15    one disease may be bad for another disease. 
           16                            DR. GRACE:  Just a following up on 
           17    that line, based on your initial question, there are 
           18    certain diseases, comorbid diseases, where you would not 
           19    want to treat patients if they have other types of 
           20    autoimmune disease or something like sarcoid where 
           21    interferon can cause a real flare of that disease.  You 
           22    have to look at the total patient, not just the liver. 
           23                            MS. SWEENEY:  On that point, not 
           24    everybody should be treated.  A lot of those are the 
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            1    homeless folks.  Is there a different reason that people 
            2    don't get treatment, extreme poverty?
            3                            DR. ZEVIN:  Lack of access to 
            4    treatment is one of the reasons.  That's not a reason why 
            5    a patients shouldn't be treated.  That's a social failing.  
            6    In terms of interferon treatment, there's a fairly long 
            7    set of contraindications to treatment, severe depression 
            8    being one of them, but what  -- and even that if we manage 
            9    it appropriately, we know that we can treat those 
           10    patients. 
           11      The issue becomes -- this is a potentially extremely 
           12    taxing treatment, something that really is going to be 
           13    very, very difficult to tolerate for a period of time, 
           14    number 1.  Number 2, it's a treatment that is very clearly 
           15    a very high degree of adherence, increases the chance of 
           16    response.  So if we're dealing with homeless populations, 
           17    we want that person to be in as stable a situation as 
           18    possible.  Somebody who's homeless, it's going to be very 
           19    hard for them to survive when they're undergoing this very 
           20    taxing type of treatment similar to chemotherapy. 
           21      The other part of it is the aspects of being able to 
           22    have enough follow-up and enough stability to try and 
           23    ensure adherence.  Doctors are very bad at predicting who 
           24    is going to be able to adhere to treatment or not.  So we 
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            1    try hard not to say, Okay, you're excluded from this 
            2    because you're homeless because you'll never be able to 
            3    adhere; that's incorrect.  But we have some pretty strong 
            4    suspicions that once we know people, who has been able to 
            5    follow up.
            6                            DR. SEEFF:  Let me make another 
            7    comment about this as Barry made; that is, the issue of 
            8    drug addicts.  The NIH had a consensus conference in 1997, 
            9    and the consensus conference agreed at that point that 
           10    patients -- people who are infected and were drug addicts 
           11    should not be treated until they have been off drugs for 
           12    at least six months, maybe six or one month.  The concern 
           13    was several things.  One was if they continue to use 
           14    drugs, they may reinfect themselves, and the decision was 
           15    then made that that was the case.  There has to be a 
           16    six-month delay.  
           17      We had another conference, 2002, in which we decided 
           18    that that was not correct, and the reason for this, in 
           19    fact a majority of people with chronic Hepatitis C 
           20    currently have acquired it as a result of the use of 
           21    injection drug use.  In the past, it was blood 
           22    transfusions, partly.  That is no longer and issue because 
           23    we screen blood donors, and by and large, transfusion 
           24    associated with Hepatitis C has disappeared.  The 
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            1    Hepatitis C in the rate of infection has dropped 
            2    dramatically, but it still occurs, and it occurs 
            3    predominantly in the drug population. 
            4      We wrote a paper which we published three or four months 
            5    ago supporting -- this came out of the National Institute 
            6    on Drug Abuse together with one of the co-workers saying 
            7    that it is possible to treat active drug users, but it 
            8    takes multiple people involved in this.  It's not only the 
            9    hepatologist.  This includes the psychiatrist.  It 
           10    includes the drug counselor.  It includes the support 
           11    group. 
           12      The fact is that if we deny drug users treatment, we're 
           13    dening a lot of people treatment who really warrant 
           14    treatment, and many of them are going to run into further 
           15    troubles if they're not treated.   We know it's more 
           16    difficult, and we know the response rate is not as good as 
           17    among individuals who are not active drug users, but 
           18    there's studies done in the very area where Barry is from, 
           19    Dr. Silvestri has been working on this; and she has very 
           20    good information to indicate that she's getting quite good 
           21    results from treatment. 
           22      We have to remember, also, treatment is not confined to 
           23    the hepatologist or the gastroenterologist.  This is true 
           24    in the VA system where there are enormous numbers of 
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            1    Hepatitis C.  There's a huge problem in the VA, and they 
            2    recognize this, and they've developed a wonderful system 
            3    of screening and a data base that is nonpareil; in other 
            4    words, nowhere in the world is there information available 
            5    than there is in the Veteran's Administration.  They are 
            6    now treating drug users.  Obviously the first thing is to 
            7    get people off drugs, but it may not always be possible, 
            8    and if you have the right kind of support system, it's 
            9    conceivable that you can treat these people with some 
           10    results. 
           11                            DR. SHERKER:  Not everybody with 
           12    Hepatitis C necessarily needs to be treated.  You know, 
           13    particularly in Geno type 1 disease where the response 
           14    rates are significantly less than 50 percent, and the 
           15    African American population when they're in the single 
           16    digits and teens, I think one needs to take the whole 
           17    picture into acount.  You have to balance risk factor.  
           18    You have to balance the degree of disease, the duration of 
           19    the infection. 
           20      If I see somebody whose likely exposure was 30 years 
           21    ago, has minimal fibrosis on a liver biopsy, who's African 
           22    American infected with Geno type 1 with low response rate, 
           23    I will  -- again, I'll talk to people and let them make 
           24    their own decisions -- but I'm very much disinclined to 
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            1    treat those people because the benefit of treatment is 
            2    small, relative to the very small potential benefit, 
            3    higher risk. 
            4      On the other hand, somebody who's more recently 
            5    infected, somebody who has a more favorable Geno type, 
            6    significant fibrosis at a relatively young age, I'm more 
            7    aggressive and more inclined to treat.   The rationale for 
            8    liver biopsy is largely used in my practice to decide who 
            9    not to treat, who doesn't necessarily need treatment, and 
           10    we're all used to treating with HIV paradigms; and 
           11    Hepatitis C viral load really means nothing. 
           12      My viral load is the amount of inflammation in the liver 
           13    biopsy.  My CD 4 count, the amount of fibrosis in the 
           14    liver biopsy, and really, the liver biopsy particularly in 
           15    Geno type 1 disease decides treatment. 
           16                            MR. GERRY:  I don't want to 
           17    interrupt the flow of this. 
           18                            DR. GRACE:  We've talked about the 
           19    homeless which is a very important population.  There's 
           20    another population we haven't mentioned, and that's the 
           21    prison population where Hepatitis C is (inaudible) or 
           22    otherwise (inaudible).  One of the problems that we have 
           23    is that it varies tremendously from state to state.  It's 
           24    certainly important these patients are treated because 
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            1    it's a very costly treatment. 
            2                            MR. GERRY:  What I wanted to ask 
            3    the panel and people from the audience is a question about 
            4    qualifications and process.  We're looking across the 
            5    board at the whole question of expertise in making 
            6    adjudication decisions, and as you know, we both, right 
            7    now, employ both full-time and certainly the vast majority 
            8    of the situation part-time medical consultants to help 
            9    give good decisions.  
           10      In cases where medical evidence isn't complete, we 
           11    request consultative examinations.  We have a whole 
           12    process and spent quite a bit of money doing that.   We do 
           13    something very similar later on in the process at the 
           14    hearing level where we give medical expertise and 
           15    qualifications. 
           16      I guess my question is, In terms of chronic liver 
           17    disease, what kinds of people should we be asking in terms 
           18    of advice?  Who's qualified to help us if we just go with 
           19    the listings that we were talking about earlier, you can 
           20    see a sense of the criteria being applied to this level?  
           21    What are the qualifications in terms of formal 
           22    qualifications and also in terms of actual experience, 
           23    just address that and maybe what we're trying to do at 
           24    this point get a sense of how we pole the right expertise 
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            1    to match up with a claim.  So that's, kind of, my 
            2    question. 
            3                            AUDIENCE:  Representing HERSA, one 
            4    of our main missions is to provide access to quality 
            5    health care for the underserved.  One of the ways we do 
            6    that, we bring in the consumers, even to review grant 
            7    applications, and I think it would be great if you could 
            8    tap the consumer population.  What better critic is  --
            9                            MR. GERRY:  I was thinking about 
           10    individual adjudication decisions.  Somebody who ends up 
           11    with the right listings and right rules, and we have non 
           12    medically trained adjudicators, and they're supposed to 
           13    work for somebody who can understand the medical evidence 
           14    and help them arrive at the decision; if you were going to 
           15    describe who is that person; that is, the medical person; 
           16    who is in the best position to give that advice; who would 
           17    those people be? 
           18      Is it a question of board certification?  Ongoing 
           19    experience?  Those are the kinds of questions. 
           20                            MS. KUKKA:  I would encourage 
           21    employing doctors who deal with a lot of patients with 
           22    that infection.  Coming from Maine or other states, I'm 
           23    recalling the map that she about where Asian American 
           24    communities are.  You couldn't find a hepatologist in 
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            1    Maine to save your life who has worked with people with 
            2    Hepatitis B and probably not a lot with Hepatitis C yet.  
            3    Again, I would much more prefer him to be evaluating my 
            4    disability claim than a doctor in Maine who has dealt 
            5    with, maybe, alcoholic liver disease and that's it.
            6                            DR. SEEFF:  I think there are two 
            7    answers to that question.  For one -- and I don't want to 
            8    be self-serving about it because I've been involved in one 
            9    of these -- the American Association for the Study of 
           10    Liver Disease, AASLD, contracts out to mentors to write 
           11    guidelines, and there are guidelines that have been 
           12    written on Hepatitis B, Dr. Anna Locke and Dr. Brian 
           13    McMahon, published in the Journal of Hepatology and 
           14    updated a short time ago. 
           15      I happened to be responsible for three other people 
           16    writing the guidelines for treatment for Hepatitis C, and 
           17    there are specific points at which we think it's important 
           18    to consider treatment.   We take into account Geno type.  
           19    We take into account the age.  We do take into account, if 
           20    we can, get a liver biopsy.  As Dr. Sherker said, if they 
           21    have degree of fibrosis, this would put them in the 
           22    category in which treatment should be considered.  That's 
           23    the one part. 
           24      The other part are the physicians.  You are completely 
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            1    right.  Unfortunately, this takes a lot of experience, and 
            2    experience has been defined by not that many people.  In 
            3    each city, there's several hepatologists who did have 
            4    experience in taking care of patients, but even the 
            5    enterologist who rarely has seen one or two or three 
            6    cases, doesn't really have the expertise to be able to 
            7    respond to this.  You have to be able to identify within 
            8    each state, and that listing can come from the American 
            9    Association for the Study of Liver Disease which has the 
           10    list of people in their area. 
           11      There's the other issue that we talked about the people 
           12    who just don't fall into the categories in which treatment 
           13    is even available.  The homeless is a huge concern, and 
           14    there are many reasons why they have a higher rate of 
           15    infection, and yet they have poor access to medical care.  
           16    The incarcerated, anywhere from 20 to 40 percent in 
           17    prisons are infected with the Hepatitis C virus.  Of 
           18    course the concern is that many of these people go out to 
           19    the community, and often given a period of time, they 
           20    infect others. 
           21      I believe there's a law that the prison system is 
           22    supposed to take care of individuals who have diseases.  
           23    The problem with Hepatitis C or Hepatitis B is that 
           24    there's a duration of treatment that's required, and 
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            1    people who are going to be in prison for six months, for 
            2    example, you can't start a treatment that's going to last 
            3    for less than a year unless you have someone in the 
            4    community who's going to pick it up and treat it.  So this 
            5    is a big issue being dealt with.
            6      We actually have a paper in press regarding a huge 
            7    meeting that's been held involved with CDC, the NIH, the 
            8    prison authorities and so on, got together to try to deal 
            9    with this, and unfortunately, the issues come down to 
           10    money.  The old story, there's not enough funds to treat 
           11    individuals with the disease.  So, you know, this is an 
           12    issue that's beyond our capability. 
           13      I know who should be treated.  I know that there's not 
           14    enough funds to treat the people who should be treated.   
           15    I know who probably shouldn't be treated.  There are  
           16    studies that have been done, particularly in the VA in 
           17    that field or studies that have reported the fact that 
           18    only a given proportion of people in fact qualify for 
           19    treatment.  The others don't qualify because they're 
           20    severely depressed, or they have other diseases that make 
           21    it impossible for them to be treated.  There are other 
           22    reasons they will not be treated even those who are in 
           23    treatment, I think, unfortunately there's not enough 
           24    funding to be able to treat such people. 
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            1                            DR. ZEVIN:  I think I want to 
            2    answer that question in a slightly different way.   When 
            3    we look at Hepatitis C and some of the other common 
            4    conditions which people are applying to disability for, 
            5    there are really often issues of social class and other 
            6    reasons why there are other disparities involved. 
            7      From my experience, as most of the experts who are 
            8    currently used, are people whose experience has been 
            9    primarily in private practice setting.  Sometimes in 
           10    academic settings, but for the most part, private practice 
           11    settings.  Because of the nature of the way our health 
           12    service system is organized in the United States -- and 
           13    this is particularly true in California -- the people in 
           14    private practice rarely see people who are poor and people 
           15    who have the poor people type of medical problems and 
           16    people who are seen. 
           17      But there's a whole other part of the medical system 
           18    that includes the VA system.  It includes the system of 
           19    community health centers or the Medical Association of 
           20    Community Health Centers.  It includes people working in 
           21    the Department of Public Health who have a lot more 
           22    experience, just out of necessity, dealing with some of 
           23    the type of problems that certainly in the range of 
           24    Hepatitis C and Hepatitis B, and where those are the 
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            1    physicians who are likely to have had the clinical 
            2    experience. 
            3      Number 1, I would confirm, yes, you want people who have 
            4    ongoing, current, today clinical experience, not retired 
            5    five years ago, but I used to know something about that.  
            6    It really does help all of us to keep on seeing patients; 
            7    and then I would look at some unconventional ways of doing 
            8    things; maybe contracting with community health centers 
            9    for their services or other public entities where you 
           10    might find a little more expertise. 
           11                            MR. GERRY:  Maybe if I could 
           12    expand the question to you and others.  If you look at our 
           13    process, if you talk about people who come to us for SSI 
           14    applicants which is part of the population I used to work 
           15    with, BI applications.  You've got a significant number of 
           16    the people who are uninsured when they come to us.  You 
           17    also have people who may be partially insured; that is, 
           18    partly covered by Medicaid.  Whether they have a home 
           19    where they receive treatment is a question whether they 
           20    are technically eligible. 
           21      We have a model which, kind of, assumes that in addition 
           22    to this expertise that if you don't have a treating 
           23    physician or if you do have a treating physician.  If you 
           24    don't have a treating physician who doesn't have any kind 
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            1    of chronological involvement with you, then we use the 
            2    consultative examination process.  We try to ask 
            3    questions. 
            4      Almost by definition, we get someone who has never seen 
            5    you before and probably will never see you again, and we 
            6    expect that within a reasonably short  -- I think in some 
            7    cases, not too expensive process -- we're going to get an 
            8    answer to questions raised in these listings; not whether 
            9    someone has Hepatitis C or B; but what the implications of 
           10    that -- as we've gone through this all day -- what the 
           11    implications of that would be in terms of requirement. 
           12      I guess what I'm wondering, Is that feasible here?  Are 
           13    we seeing -- is that model which assumes that can be done 
           14    actually, technically possible?  And that's the first 
           15    question, and if not, if we don't have a treating 
           16    physician, someone with some kind of longitudinal contact 
           17    with a patient, do we need to create a situation where 
           18    that occurs before we render some kind of decision about 
           19    eligibility? 
           20      What I'm trying to figure out in fairness to the 
           21    process, if the observation were that you need some time 
           22    with a patient and to try out or to understand the actual 
           23    implications of this particular patient -- and I've heard 
           24    all day people vary a lot.  You can't say too many things 
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            1    that are absolutely true of everybody.  It's not just 
            2    getting a test result. 
            3      Then what I'm trying to figure out is whether what we 
            4    ought to do is think about it, a strategy, where we might 
            5    put somebody in a treating physicial model where we would, 
            6    first, have to provide access to health insurance and care 
            7    in order to get the information that would be necessary 
            8    for anybody that you're talking about.  Even if we got the 
            9    person that you're talking about, would they be able in a 
           10    couple of hours to come to this conclusion, or is it a 
           11    question of experience, expertise and some connection with 
           12    this particular patient over some period of time. 
           13                            DR. SHERKER:   I think a lot of it 
           14    depends on what information you're providing this 
           15    physician with.  Given a liver biopsy and given history of 
           16    variceal bleed, I can render a more accurate opinion than 
           17    somebody who comes in who has an ALT of 47, HCV, RNA 
           18    positive, Geno type 1, who used needles 30 years ago.  I 
           19    can't tell you anything about that patient's natural 
           20    history with respect to complications other than just 
           21    quote you statistics and say there's a 10 percent chance 
           22    that this person has cirrhosis; and, if so, then this. 
           23      You know, with that information, you're going to have a 
           24    hard time getting meaningful results out of your 
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            1    consultation process.
            2                            MR. PORRO:  I wanted to mention 
            3    there's a step before -- as Dr. Zevin was saying  -- about 
            4    the community health centers and all this professional 
            5    relationship with the patient, you could establish a track 
            6    record and have documentation.  But there's the population 
            7    that you were talking about, and Ms. Siegel referred to 
            8    also, which is the drug infected population, people with 
            9    those kinds of problems.  Then you have third party 
           10    corroboration, and I don't really see where you can go 
           11    except to perhaps a shelter worker or something of that 
           12    nature. 
           13      You know, someone that in a professional position to 
           14    actually be responsible for the information they're 
           15    offering, it's difficult. 
           16                            DR. GRACE:  If you just rely on 
           17    medical records, they vary tremendously in their quality.  
           18    Some medical records give you a very detailed social 
           19    history, work history and so forth.  Other medical records 
           20    will be one line.  If you're just dealing with a record, 
           21    and you have never seen a patient, sometimes rendering a 
           22    decision is going to be very difficult. 
           23                            MR. GERRY:  Somebody with medical 
           24    records would be emergency room reports collected at 
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            1    different times would know comprehensive assessment.  I 
            2    have seen some of those files, and I don't know how -- I 
            3    guess my question is, No matter how experienced you are, 
            4    you wouldn't be able to do much with that.
            5                            DR. ZEVIN:  I think some of it is 
            6    really dependent if you have an interested, somewhat 
            7    knowledgeable physician, who has a sufficient period of 
            8    time to take a full history, to do a physical exam, to 
            9    gather some of that other information, who has the 
           10    knowledge to say -- I'll just pull out one thing which is 
           11    very common  -- the knowledge to say to somebody with 
           12    Hepatitis C, Do you have problems with numbness and 
           13    burning and tingling in your feet? 
           14      Well, many of the people I see  -- putting an editorial 
           15    comment here -- you can save a lot of money right now 
           16    doing a better job finding out who's got a treating 
           17    physician before sending them to a CEE which a lot of 
           18    patients seem to be at the CEE before they ever ask me for 
           19    any records.  
           20      If that CEE is actually given the time, has the 
           21    knowledge, I happen to think that you can find out a whole 
           22    lot about what's going on with somebody given the luxury 
           23    of 90 minutes to really take down all the things that's 
           24    going on with that person and what that person's medical 
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            1    history has been and doing the medical examination and 
            2    having time to review old records that have been 
            3    previously gathered.  
            4      On the other hand if you're in a situation where this is 
            5    a CEE that being run as, sort of, a mill and people are 
            6    going through and having 20 minutes, saying, you don't -- 
            7    have you gone for any blood?  No, I have the lab here.  
            8    You don't know the listings.  Here's the report.  You're 
            9    not going to get anywhere with that.  I would be inclined 
           10    to say that you would have more luck with having just the 
           11    time and, frankly, the remuneration to attract interested 
           12    enough physicians who if they didn't know would be 
           13    motivated to know and perhaps could be paid even to say, I 
           14    spent this time with this syndrome because I wasn't 
           15    familiar with it.  Now, I'm familiar with it.  Now, you're 
           16    going to have to prove it by writing something that you do 
           17    know what's happening. 
           18                            MS. ST. JAMES:  I think that it 
           19    may be beneficial.  One thing I heard that was consistent, 
           20    most everyone in the room, is that perhaps in the listings 
           21    for to get more specific to include more specific 
           22    parameters so that people can be evaluated with very 
           23    specific parameters right now.  I certainly mean no 
           24    disrespect, but what's been proposed is a little vague and 
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            1    perhaps too narrow. 
            2      I think one of the things that we talked about is, 
            3    there's a whole lot more to it than just variceal bleeding 
            4    and how many transfusions you've had.  If we can get it 
            5    nailed down in the listings, and again in the way 
            6    analogous to what's down in the HIV section, that would 
            7    eliminate the need for all these consultations.  So that 
            8    may cut on the front end, but I think there will be some 
            9    people that you may need to follow for a period of time 
           10    and, boy, you would be doing them a tremendous favor; not 
           11    only them, but in the long run in the health care system 
           12    to have a care provider in somebody's life that has 
           13    chronic liver disease;  what you spend on doing that, will 
           14    save you in the long run. 
           15                            MS. SIEGEL:  The Massachusetts 
           16    Welfare Department used to for their General Relief 
           17    Program which is, sort of, a mini version of SSI, they 
           18    used to give people temporary Medicaid cards when they 
           19    applied to help them get treatment to get their medical 
           20    reports filled out.  I'm not sure they still do that.  
           21    They also send people for CE.  It seems to me that given 
           22    the kind of longitudinal evidence that you're looking for 
           23    in the listings, it's going to be hard to get that in a 
           24    one shot CE no matter how detailed. 
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            1                            DR. SEEFF:  I love what you're 
            2    trying to do which is to try to get the best possible 
            3    opinion about what should be done with the individual 
            4    patients.  This is a daunting treatment, using interferon 
            5    Ribavirin.  If I could find any other way to treat any 
            6    other patients, I would get rid of those drugs.  I don't 
            7    see that happening. 
            8      We've got new drugs five years down the line.  We've 
            9    been saying it for the last five years.  We're not there 
           10    yet, so it's a daunting treatment.  I think that this is 
           11    something that one doesn't enter into lightly, and I think 
           12    it takes a caring physician or a caring support group to 
           13    work with that patient. 
           14      I'm not evening talking about the people who are 
           15    necessarily in the community health system.  I'm talking 
           16    about people who are being cared for by well-known 
           17    physicians who do studies, and I when I talk to these 
           18    patients, they say, "I've never seen the doctor.  I've got 
           19    a nurse taking care of me, and I don't feel I've got any 
           20    communications." 
           21      I think treating patients for Hepatitis B and Hepatitis 
           22    C, they're two parts to it.  One is to make the decision, 
           23    does this treatment warrant it?  And there, there are, 
           24    sort of, guidelines that these change as we learn more.  
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            1    But there were some guidelines, and these come out of the 
            2    guidelines that we're using.  The second part is helping 
            3    the patient through the next year. 
            4      So it takes more than just the initial decision.  It 
            5    takes the care and support and understanding of what's 
            6    happening because there are side effects that come from 
            7    these drugs.  Interferon has many and Ribavirin has many, 
            8    the combination has many.  There's depression, there are 
            9    all kinds of things.  So it takes a support system that 
           10    takes the person through that. 
           11      I think you can get experts if the data are available if 
           12    they are not available, they should be obtained in order 
           13    to be able to make that decision.  Does this person 
           14    warrant it?  If the answer is yes, that could be done.  
           15      Next I think the patient and doctor have to talk about 
           16    this.  They all talk about chemotherapy.  They can lose 
           17    their hair.  They become depressed.  They can develop all 
           18    kinds of side effects, and this is not lightly 
           19    understaken.  It takes a caring physician or health 
           20    provider to be part of the whole system of helping to get 
           21    this person through. 
           22      Remember, this is not penicillin where 100 percent of 
           23    people will respond, maybe not in pneumonia.  This is a 50 
           24    percent response rate in the most common Geno type, Geno 
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            1    type 1.  When you talk about Geno types 2 and 3, there's 
            2    80 percent.  So I think this would be helpful to have 
            3    someone if this becomes necessary, to say, yes, looking at 
            4    the data, that treatment should be undertaken. 
            5      The next part is between the patient and doctor or a 
            6    support team.  There are models.  I was invited to give a 
            7    talk in New Mexico where there's a large rural area of 
            8    people and some reservations, and the way the university 
            9    has decided to deal with this is to have teleconferences.  
           10    They have an expert at the university who, once a week, 
           11    ties into these peripheral institutions and actually has 
           12    cases presented and discussed and helps the providers make 
           13    a recommendation about treatment. 
           14      The interesting thing, not all the people who are 
           15    administering the treatment are physicians.   There's 
           16    pharmacist, nurses, doctors who are not expert who are 
           17    involved. But there's an expert who is helping to monitor 
           18    there.  
           19      I'm going to California on this weekend where apparently 
           20    there's another such system that's being considered to see 
           21    whether they can in fact run this kind of thing so they 
           22    can help support and understand what's going on.   I say 
           23    this now from the point of view of a physician taking care 
           24    of patients.  It may not have relevance to Social Security 
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            1    Administration, but I think it's a very difficult task.  
            2    It takes expertise, both in making the decision and 
            3    continuing to follow the patients while they're being 
            4    treated. 
            5                            MR. GERRY:  I was going to say 
            6    that I was interested to hear your comments about HIV.  We 
            7    all know the cities of public immune disorders, one in 
            8    Philadelphia and one in San Francisco.  Actually people 
            9    were a lot more satisfied with the system than you might 
           10    think, how well it's working with people with HIV.  
           11    Ironically, from that standpoing, we're involved right now 
           12    in trying to and beginning a large demonstration in 
           13    California of people who have immune disorders in which we 
           14    want to create a network of medical expertise but also a 
           15    network of the kind of -- I don't want to say CE.  A 
           16    network of people who at the local level who could provide 
           17    ongoing care and treatment. 
           18      I think most of us who went to those meetings were 
           19    overwhelmingly impressed that, with autoimmune disorders, 
           20    many of the same diagnostic problems -- in fact, even more 
           21    severe diagnostic problems and the understanding -- what 
           22    I'm trying to say is that there were quite persuasive 
           23    arguments made there.  If that's the case, it makes the 
           24    point that much more.  
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            1      What I'm trying to figure out, and what I'm looking at 
            2    is just this question of whether -- right now, we're the 
            3    only industrialized country in the world that makes access 
            4    to health insurance dependent on access to (inaudible).  
            5    The reason we're the only country to do that is it's 
            6    idiotic to do that.  It's completely contrary to any kind 
            7    of common sense or proper management of the program.  It's 
            8    an historical anomaly.  It represents nothing more. 
            9      But it is very instructive in that what happens is in 
           10    the case of some of the patients Barry talked about who 
           11    don't come with health insurance and don't have a medical 
           12    home and don't have much of a record; what people are 
           13    asking for is both cash benefits but also health 
           14    insurance.  So that the paradox, they need a treating 
           15    physician and some longitudinal record and some ability to 
           16    gauge this; that's the question that you're being asked to 
           17    permit to happen. 
           18      You can say, Yes, they can get a medical doctor.  That 
           19    passes the medical home point which is a serious problem 
           20    for homeless and other people.  So I think what we're 
           21    looking at, and I was convinced we ought to pursue with 
           22    the idea that maybe that for people that don't have a 
           23    treating physician, the likelihood  --  if we could do a 
           24    consultative examination of the kind that you're talking 
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            1    about, which is what we're talking about there, that would 
            2    solve some of the problems. 
            3      You could get  -- certainly people would devote the 
            4    amount of time that's appropriate to what they have on the 
            5    record, and they would get to see the patient and for some 
            6    subset -- maybe it's all subsets.  The other thing that 
            7    I'm thinking about is whether we ought to have the ability 
            8    which we don't have right now to allow people to be 
            9    treated for awhile, so view that eligibility issue before 
           10    we get to the other eligibility issue, and then based on 
           11    information that's collected during that period of ongoing 
           12    treatment, whether they will ultimately get cash benefits 
           13    or not, then we can make a better assessment so we have 
           14    some answer to the question, better.  That's why I was 
           15    asking the questions about.
           16      As far as the CE, no problem.  I have heard, as I'm sure 
           17    you have, and Eileen has, that major function consultative 
           18    examinations in some places is to support denials.  When 
           19    you look at bold purchasing of consultative examinations 
           20    it would tend to feed the argument that it certainly isn't 
           21    the place for investing much resources.  I'm thinking 
           22    about trying to reverse that idea and say that, if 
           23    anything, we ought to have the flexibility to allow people 
           24    to be treated until the point comes in which maybe we 
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            1    could have a fair assessment of the information. 
            2      Right now, the burden is on the claimant, and the burden 
            3    of an untreated  -- or the claimant who doesn't have a 
            4    medical home and doesn't have access to prove the kind of 
            5    complex factors that we were talking about seems 
            6    overwhelming but that's what happens. 
            7                            MS. ST. JAMES:  I know this 
            8    probably sounds extreme, and I know it's beyond, probably, 
            9    the scope of the Social Security Administration, but 
           10    certainly when you're talking about somebody who may have 
           11    a long-term disability, and again my expertise is where we 
           12    know that folks that clear with all the caveats of who's 
           13    eligible and how difficult the treatment is and all of 
           14    those things.  If you find someone who is a good candidate 
           15    for treatment, that you provide the possibility to get 
           16    that care for a year, and that person clears and recovers, 
           17    and is then able to reenter the workforce; and has their 
           18    life back; socially, from philosophically, that certainly 
           19    appeals. 
           20      Even from an economic standpoint in terms of somebody 
           21    that's on long-term disability and unable to be in the 
           22    workforce versus somebody that you invest in trying to get 
           23    them well, and they recover is also economically -- it's a 
           24    win win. 
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            1                            AUDIENCE:  Dr. St. John had 
            2    pointed out that the proposed listings were narrow, and if 
            3    we were able to get a good screening criteria up front, we 
            4    could hopefully save funds in the long run.  With the 
            5    expertise that's right here, can you give me the  
            6    consensus on what standard should be used to determine 
            7    whether someone who has chronic liver disease for 12 
            8    months -- this is a long-term disability program  -- from 
            9    a medical standpoint alone?
           10                            MS. ST. JAMES:  You want to herd 
           11    cats, don't you?  My comment was that you want to herd 
           12    cats.  Whenever you get a bunch of medical types in one 
           13    room, it's like trying to herd cats.  It doesn't work very 
           14    well. 
           15      I think that there are parameters.  It probably isn't 
           16    going to happen today.  You could get together a panel of 
           17    experts to hash this out and come to a consensus about 
           18    what are the evidence-based parameters that we can use. 
           19      You know, we've talked a bit about MELD for adults and 
           20    PELD for children.  Each one of these has certain 
           21    limitations, and I think -- I'm not going to be 
           22    presumptuous enough to tell you ABC.  Do I think it's 
           23    worthwhile to perhaps have a panel of experts in this 
           24    field, discuss this and present a consensus of that group?  
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            1    I think it's well worthwhile it to do so that.  
            2      I think we have reasonably good information to be able 
            3    to share on some of these issues.  Some of it, we're still 
            4    collecting a lot of information especially with C.  This 
            5    is relatively discovered, C, in 1992, and a lot of 
            6    long-term, follow-up.  We're just starting to collect that 
            7    data now.
            8      Can we help provide you with information to help make 
            9    those listings?  I think so.  What do some of the other 
           10    folks think?  
           11                            DR. SHERKER:  I think one of the 
           12    problems is that we're pretty good at predicting the short 
           13    outcome of somebody who has liver disease with 
           14    decompensation.  The problem is the vast majority of 
           15    patients that you're dialing with are not in that 
           16    category.  Until you can come up with hard criteria and 
           17    listings for such problems as fatigue, which is a daunting 
           18    problem, I think it's a little naive to suspect that you 
           19    will be able to come up with listings to pick up the 
           20    majority of patients who are looking for disability 
           21    benefits. 
           22      The natural history of Hepatitis C is that only a small 
           23    minority, 20 percent, advance to cirrhosis.  Of those who 
           24    advance to cirrhosis, not all will end up dying of end 
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            1    stage liver disease.  So you're dealing with a relatively 
            2    small piece of the pie that we're  fairly good at 
            3    predicting outcomes.  The vast majority of patients even 
            4    with biopsy, I see Stage 2 fibrosis with biopsy, that 
            5    patient can still be measured in decades. 
            6      I think you're going to have a hard time getting 
            7    listings that are going to easily categorize those 
            8    patients with the available data.
            9                            DR. GRACE:  I think it's I don't 
           10    know if the problem within that some things securities 
           11    instruction you are history is replete with experts saying 
           12    this is the best treatment.  When it's put to the test, it 
           13    doesn't pan out.  No matter what, we should come up with 
           14    criteria, we should test and validate what we think is 
           15    right, is really right.  What I'm getting at, where we 
           16    randomly test criteria and see what the differences are.
           17                            MR. JULIAN:  We're getting close 
           18    to the end of the day, so I just want to take a moment and 
           19    ask everybody, in your packet, the black packet, that was 
           20    on the table, on left-hand side there's an evaluation.  If 
           21    you could just take a moment before you leave today to 
           22    complete the evaluation.  There's a box outside the 
           23    registration table, if you could just leave it there, and 
           24    what we'll do now is turn the mike over to Glenn Sklar for 
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            1    closing remarks. 
            2                            MR. SKLAR:  I want to thank the 
            3    advocates.  This has been a great session for us.  We will 
            4    absolutely consider your input.  Please keep in mind our 
            5    record is open until January, and we urge you to, please, 
            6    submit your comments.  To the extent they are in conflict 
            7    with the notices of proposed rule making, we don't care; 
            8    that's the point of this whole session.  Please feel free 
            9    to be creative as you need to be.  There's no right 
           10    answer, and we're certainly engaging you in this process 
           11    to help get to the right answer. 
           12      Other than that, I would say there's a session tomorrow 
           13    unrelated to chronic liver disease on hemophilia and 
           14    sickle cell anemia.  For those of you who won't be here we 
           15    wish you a safe trip home.  Thank you very much.
           16                            (The conference concluded at 4:21 
           17    p.m.)
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            1                  C E R T I F I C A T E
            2    COMMONWEALTH OF MASSACHUSETTS
            3    ESSEX, SS.
            4      I, Deborah Battista, Professional Stenographic Reporter 
            5    and Notary in and for the Commonwealth of Massachusetts, 
            6    do hereby certify that:
            7      This transcript is a true and accurate record to the 
            8    best of my knowledge, skill and ability of the testimony 
            9    taken. 
           10      I further certify that I am neither attorney for nor 
           11    related to or employed by any of the parties to the action 
           12    in which this conference is taken; and further, that I am 
           13    not a relative or employee of any attorney employed by the 
           14    parties hereto or financially interested in the action.
           15      IN WITNESS WHEREOF, I HAVE HEREUNTO SET MY HAND AND SEAL 
           16    THIS 20TH DAY OF DECEMBER, 2004. 
           17    
                 
           18    
                 
           19                         _____________________________
                                      Notary
           20                         My commission expires: 9-17-10
           21    
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                             EYAL COURT REPORTING SERVICE, INC
                                      (617) 964 - 4317