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1 COMMONWEALTH OF MASSACHUSETTS

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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.

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19 \_\_\_\_\_\_\_\_\_\_\_\_\_\_\_\_\_\_\_\_\_\_\_\_\_\_\_\_\_

Notary

20 My commission expires: 9-17-10

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