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39–522 CC 1997

POTENTIAL TRANSMISSION OF SPONGIFORM

ENCEPHALOPATHIES TO HUMANS: THE FOOD

AND DRUG ADMINISTRATION’S [FDA] RUMINANT TO RUMINANT FEED BAN AND THE

SAFETY OF OTHER PRODUCTS

HEARING

BEFORE THE

COMMITTEE ON

GOVERNMENT REFORM

AND OVERSIGHT

HOUSE OF REPRESENTATIVES

ONE HUNDRED FIFTH CONGRESS

FIRST SESSION

JANUARY 29, 1997

Serial No. 105–3

Printed for the use of the Committee on Government Reform and Oversight

(

(II)

COMMITTEE ON GOVERNMENT REFORM AND OVERSIGHT

DAN BURTON, Indiana, Chairman

BENJAMIN A. GILMAN, New York

J. DENNIS HASTERT, Illinois

CONSTANCE A. MORELLA, Maryland

CHRISTOPHER SHAYS, Connecticut

STEVEN H. SCHIFF, New Mexico

CHRISTOPHER COX, California

ILEANA ROS-LEHTINEN, Florida

JOHN M. MCHUGH, New York

STEPHEN HORN, California

JOHN L. MICA, Florida

THOMAS M. DAVIS, Virginia

DAVID M. MCINTOSH, Indiana

MARK E. SOUDER, Indiana

JOE SCARBOROUGH, Florida

JOHN SHADEGG, Arizona

STEVEN C. LATOURETTE, Ohio

MARSHALL ‘‘MARK’’ SANFORD, South

Carolina

JOHN E. SUNUNU, New Hampshire

PETE SESSIONS, Texas

MIKE PAPPAS, New Jersey

VINCE SNOWBARGER, Kansas

BOB BARR, Georgia

——— ———

HENRY A. WAXMAN, California

TOM LANTOS, California

ROBERT E. WISE, JR., West Virginia

MAJOR R. OWENS, New York

EDOLPHUS TOWNS, New York

PAUL E. KANJORSKI, Pennsylvania

GARY A. CONDIT, California

CAROLYN B. MALONEY, New York

THOMAS M. BARRETT, Wisconsin

ELEANOR HOLMES NORTON, Washington,

DC

CHAKA FATTAH, Pennsylvania

TIM HOLDEN, Pennsylvania

ELIJAH E. CUMMINGS, Maryland

DENNIS KUCINICH, Ohio

ROD R. BLAGOJEVICH, Illinois

DANNY K. DAVIS, Illinois

JOHN F. TIERNEY, Massachusetts

JIM TURNER, Texas

THOMAS H. ALLEN, Maine

———

BERNARD SANDERS, Vermont

(Independent)

KEVIN BINGER, Staff Director

DANIEL R. MOLL, Deputy Staff Director

JUDITH MCCOY, Chief Clerk

PHIL SCHILIRO, Minority Staff Director

(III)

C O N T E N T S

Page

Hearing held on January 29, 1997 ......................................................................... 1

Statement of:

Friedman, Michael, M.D., Deputy Commissioner, Food and Drug Administration; Linda Detwiler, D.V.M., U.S. Department of Agriculture, Animal and Plant Health Inspection Service, accompanied by Glenn Morris, Food Safety Inspection Service, USDA; Lawrence Schonberger,

M.D., Centers for Disease Control and Prevention; and Clarence J.

Gibbs, Jr., Ph.D., National Institutes of Health ......................................... 11

Hueston, Will, D.V.M., Virginia-Maryland College of Veterinary Medicine;

and Frank O. Bastian, M.D., University of Southern Alabama, School

of Medicine .................................................................................................... 99

Letters, statements, etc., submitted for the record by:

Bastian, Frank O., M.D., University of Southern Alabama, School of

Medicine, prepared statement of ................................................................. 111

Detwiler, Linda, D.V.M., U.S. Department of Agriculture, Animal and

Plant Health Inspection Service, prepared statement of ........................... 47

Friedman, Michael, M.D., Deputy Commissioner, Food and Drug Administration, prepared statement of .................................................................. 15

Gibbs, Clarence J., Jr., Ph.D., National Institutes of Health, prepared

statement of ................................................................................................... 75

Hueston, Will, D.V.M., Virginia-Maryland College of Veterinary Medicine,

prepared statement of ................................................................................... 103

Schonberger, Lawrence, M.D., Centers for Disease Control and Prevention, prepared statement of .......................................................................... 54

Shays, Hon. Christopher, a Representative in Congress from the State

of Connecticut:

Information concerning holding hearings ................................................ 6

Prepared statement of ............................................................................... 4

Waxman, Hon. Henry A., a Representative in Congress from the State

of California, prepared statement of ........................................................... 10

(1)

POTENTIAL TRANSMISSION OF SPONGIFORM

ENCEPHALOPATHIES TO HUMANS: THE

FOOD AND DRUG ADMINISTRATION’S [FDA]

RUMINANT TO RUMINANT FEED BAN AND

THE SAFETY OF OTHER PRODUCTS

WEDNESDAY, JANUARY 29, 1997

HOUSE OF REPRESENTATIVES, COMMITTEE ON GOVERNMENT REFORM AND OVERSIGHT, Washington, D.C.

The committee met, pursuant to notice, at 1:10 p.m., in room

2247, Rayburn House Office Building, Hon. Christopher Shays

(chairman of the Subcommittee on Human Resources) presiding.

Present: Representatives Shays, Pappas, Waxman, and Towns.

Staff present: Lawrence J. Halloran, staff director and counsel;

Anne Marie Finley and Robert Newman, professional staff members; R. Jared Carpenter, clerk; Phil Barnett, minority chief counsel; Agnieszka Fryszman, minority counsel; and Ellen Rayner, minority chief clerk.

Mr. SHAYS. I would like to call this hearing to order and acknowledge that this is the Human Resources Subcommittee of the

Government Reform and Oversight Committee, but given that the

committee has not officially established its subcommittees, we are

operating at the permission of the chairman and ranking member,

who have authorized this committee to proceed.

I would like to welcome our witnesses and our guests. I have a

statement, as does Mr. Towns, and Mr. Waxman has a statement

as well.

In the last Congress, this subcommittee devoted considerable

time to an examination of the Federal approach to emerging infectious agents, particularly foodborne pathogens. The central question then, and now, is: What is the appropriate regulatory response

to a public health threat about which there is little conclusive scientific information, small known risk, but theoretical risks of serious, even calamitous, spread of infection?

Transmissible spongiform encephalopathies, TSEs, constitute a

class of degenerative, fatal diseases that attack the brain. TSEs infect numerous mammal species including sheep, cows, deer, elk,

goats, minks, and humans. The causative agent is not known.

There is no diagnostic test to detect the presence of a TSE, only

a postmortem dissection.

The TSE in sheep, called scrapie, has been known for more than

200 years, with the disease posing no known threat to human

health or the safety of the human food supply. TSEs emerged as

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a public health issue only in the late 1980’s when an epidemic of

bovine spongiform encephalopathy, BSE, or ‘‘mad cow disease,’’

struck British dairy and beef cattle.

The source of that outbreak is not known, but it is believed the

incidence and virulence of the disease were amplified by what is

called ruminant-to-ruminant feeding—the use of ruminant animals,

sheep, cows and goats, in feeds for ruminant animals. In Great

Britain, sick cows and sheep were ground into feed for healthy

cows, which then became infected.

In tragic proof of the adage ‘‘you are what you eat,’’ it now appears that consumption of BSE-infected beef was responsible for

the emergence in Britain of a variant form of the human TSE,

Creutzfeldt-Jakob disease.

While no BSE has been reported in the United States, the U.S.

Department of Agriculture, USDA, and the public health agencies

of the Department of Health and Human Services, HHS, have

taken steps to prevent its emergence here. In 1989, USDA banned

the importation of meat and other potentially infected products

from countries in which BSE exists.

Last year, the FDA testified before this subcommittee that regulatory action was imminent on a ruminant-to-ruminant feed ban as

a preemptive safeguard against the appearance or the amplification

of TSEs in meat animals entering the U.S. food supply.

Today, 8 months later, we will discuss the timing and substance

of the FDA proposal to prohibit certain ruminant-to-ruminant feeding practices. In the weeks ahead, we will hear from producers and

consumers about other steps that might afford additional public

health protections against TSEs.

Other steps may be necessary because the food chain is not the

only possible vector for TSEs to emerge as a public health problem.

There is a theoretical danger that CJD can be transmitted through

blood and blood products. There is some concern the suspected infective agent, the prion, survives the processing of cow remnants

into the oils and gelatins used in making cosmetics, drug capsules,

and a variety of other products.

Therefore, we ask: How should these risks be evaluated in the

absence of definitive scientific evidence? What practical and

proactive public health policies will minimize those risks? What research will clarify the causes, courses and cures of TSE diseases?

We learned the hard way with hepatitis and AIDS that emerging

infectious agents can slip past our public health defenses unless we

vigilantly maintain an early warning system sensitive to probability as well as proof. Better to protect against unproven risks

than wait for proof that may only emerge in increased mortality

statistics.

Some say ‘‘mad cow disease’’ is a misnomer because the afflicted

animals appear more worried than mad. They are not the only

ones, frankly, that are worried, but our worry should not be misconstrued. Valid public health concerns should not be sensationalized into unsubstantiated fears about the U.S. food supply, which

is, without question, among the safest in the world.

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Our goal is the proactive protection of the public health, and in

that regard we welcome our witnesses today in that effort. And in

this, I would now like to call on Mr. Towns, who has been, frankly,

while I am chairman, he is ranking member, and a copartner in

this committee and the good work we are doing.

[The prepared statement of Hon. Christopher Shays and the information referred to follow:]

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Mr. TOWNS. Thank you. I would like to yield to the ranking member of the committee to go first.

Mr. WAXMAN. You want me to go first?

Mr. SHAYS. I would be happy—— Mr. TOWNS. It is different protocol. You are going to have to organize the committee.

Mr. SHAYS. Let me keep protocol and call on Mr. Waxman to

yield to Mr. Towns.

Mr. WAXMAN. Mr. Towns is the ranking member of the subcommittee from last year but hasn’t officially been made ranking

member this year. I have full expectation that he will be, and I

would like to yield to him.

Mr. TOWNS. Thank you very much.

First, let me thank you, Mr. Chairman, for calling this hearing

this afternoon. We will hear from the agencies charged with protecting the health and safety of the American public, and from

members of the scientific community; I also believe we need to hear

from consumer groups and representatives of the industry that will

be impacted by what decisions we make here. I am glad the chairman plans to have another hearing on this topic so that all voices

can be heard and so we can ensure we are all working cooperatively to make certain that our food supply is safe.

BSE has had a devastating impact in Great Britain where hundreds of thousands of cattle have been destroyed to prevent the

spread of the disease. We are fortunate that no cases of BSE have

been reported in the United States.

Since 1989, the U.S. Department of Agriculture has banned the

importation of live ruminants of cattle, sheep, and goats, and ruminant products from countries where BSE exists. British beef has

not been imported to the United States since 1985. The FDA is now

taking an additional step, banning the use of ruminants, tissues,

and ruminant feed, which will cutoff the primary means of transmission of BSE.

The jury is still out on what causes BSE and whether BSE in

cattle is transmissible to humans. Research in this area is ongoing.

But let me add, I agree with the chairman that more research is

necessary and we should move aggressively to make certain that

we get information. Given the lack of concrete scientific data that

is currently available, I am interested in hearing from the witnesses as to what, if any, additional steps are necessary at this

time.

Thank you again, Mr. Chairman, and I look forward to hearing

from the witnesses and working with you to make certain that our

food supply continues to be safe. Thank you.

Mr. SHAYS. Thank you, Mr. Towns.

Before calling on Mr. Waxman, I will invite Michael Pappas, who

is a new member of this committee and a welcomed addition.

Mr. PAPPAS. Thank you, Mr. Chairman.

I would like to thank you and the members of this committee

and these witnesses and all of those interested for taking time to

share their concerns with us today and in any subsequent hearings. The world’s greatest enemies seem to be getting stronger, yet

tinier and harder to control, every year. These tiny dangers in the

form of bacteria, viruses, parasites, and prions continue to chalVerDate 11-MAY-2000 09:36 Jun 28, 2002 Jkt 078738 PO 00000 Frm 00011 Fmt 6633 Sfmt 6602 W:\DISC\39522 pfrm09 PsN: 39522
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lenge our scientific knowledge and force the scientists to work

harder each year.

Hollywood fears of a great disease wiping out humans and/or animals are only exacerbated by the real-life horrors in the news of

the Ebola virus and now ‘‘mad cow disease.’’ As a public official, I

believe it is my duty to assist in placating any fears of the public

and the agricultural community by ensuring that adequate steps

are in place to assure the continued safety of our citizens and our

unparalleled agricultural industry. However, it is my duty to ensure that the cure fits the problem, and that government does not

overreact to a problem that may not exist, or impose a cure that

could be considered, for lack of a better term, overkill or recklessly

trample over the rights of individuals in government’s desire to do

right. In such, I am hopeful we will maintain a balanced, reasoned

approach to this serious issue and propose rules based on facts, not

fears.

Finally, I would like to welcome Dr. Linda Detwiler, who is based

in New Jersey, as a witness before this panel. When I talk about

agriculture in New Jersey, it is good to know that the witness will

have had firsthand experience with it, and I look forward to hearing from her and other witnesses.

Thank you, Mr. Chairman.

Mr. SHAYS. Thank you, Mr. Pappas.

Mr. Waxman, who I might point out is the ranking member of

the full committee and also was chairman on the Commerce Committee, the subcommittee that dealt with all environmental and

health issues, so it is wonderful to have you here today.

Mr. WAXMAN. Thank you, Mr. Chairman, and I am pleased that

you have called this hearing.

This country has the safest food supply in the world and we want

to keep it that way. We also want to ensure that American consumers do not lose confidence in the safety of the products that

they and their children use every day, so I am glad we are here

to determine whether more needs to be done to protect against the

possible transmission of BSE. In particular, I want to commend

you, Mr. Chairman, for your leadership in this area. Your continued interest has been essential in prompting FDA regulation.

But I must say that I find it very ironic that in this committee

we are talking about what the FDA needs to be doing while down

the hall other committees are trying to reduce the authority of the

Food and Drug Administration.

We will continue to face threats to our food supply, threats that

we know about and threats that are real. We have enough scientific information to know that they are serious threats, like the

E. coli outbreak we suffered last year, and potential threats to our

blood supply through new and emerging diseases. That is why we

need a strong and effective FDA as well as a strong and effective

line of defense at USDA and the Centers for Disease Control and

Prevention.

In the last Congress, many Republican Members, and some

Democrats, were pressing for a reduction in the FDA’s regulatory

abilities and a weakening of the agency’s ability to enforce the law.

Some in Congress, also tried to legislate away the ability of agenVerDate 11-MAY-2000 09:36 Jun 28, 2002 Jkt 078738 PO 00000 Frm 00012 Fmt 6633 Sfmt 6602 W:\DISC\39522 pfrm09 PsN: 39522
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cies to make sound, science-based decisions in a reasonable period

of time.

I am particularly concerned about how Congress has restricted

the FDA’s authority to regulate dietary supplements. We recently

enacted the Dietary Supplement Health and Education Act under

a great deal of pressure from the industry. Some of these dietary

supplements are produced from animal tissues. Now, we don’t

know if that is a reason to be concerned, but under the provisions

of this new law, the manufacturer of these products is subject to

very little regulatory oversight. In fact, Congress went so far as to

block FDA from acting until FDA can prove the dietary supplements are harming people. As a result, FDA can do very little to

reduce any BSE threat in these products, should one develop.

I think the point has been made by the other members of this

panel. We don’t want to scare people into thinking that there is a

crisis. We don’t want to overreact. We want to act in a balanced,

reasonable manner. We have agencies like the FDA, the USDA,

and the Centers for Disease Control, to give us that appropriate

balance. But as they design the appropriate balance, we have got

to give them the regulatory tools to act when it is necessary and

not hamper them from acting when it is in their best judgment,

based on the facts and the science, that there is a reason to act.

So I am pleased we are holding this hearing, Mr. Chairman, and

I want to commend you for your leadership. This is an important

issue and deserves an airing in the Congress.

Mr. SHAYS. I thank the gentleman.

[The prepared statement of Hon. Henry A. Waxman follows:]

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Mr. SHAYS. Let me just get some housekeeping out of the way:

I ask unanimous consent that all members of the subcommittee be

permitted to place any opening statements in the record and that

the record remain open for 3 days for that purpose, and without objection, so ordered.

Also, I would ask unanimous consent that witnesses be permitted

to include written statements in the record if they choose to summarize them. Without objection, so ordered.

At this time, as is the practice, we will swear in all our witnesses, including Members of Congress, and I would invite you to

rise and raise your right hand. I am assuming, since I see others

standing up and raising their right hand, that you are backup

staff. If they say something for the record, they will have to be

sworn in.

[Witnesses sworn.]

Mr. SHAYS. For the record, our main witnesses have answered in

the affirmative and as well as others who have accompanied them.

I would like to apologize to the people in the audience. Next time

we will have the witness table up closer and have a few more rows

of chairs. It may be some won’t stay too long and seats will become

available.

At this time, let me just recognize our witnesses. We have Michael Friedman, who is the Deputy Commissioner for the Food and

Drug Administration, FDA. He will kind of give us the human

health care element here. We have Linda Detwiler, chairman of the

TSE working group from the U.S. Department of Agriculture. She

will give us the animal health perspective. We have, as well, Lawrence Schonberger, who is the assistant director for Public Health,

National Center for Infectious Diseases, Centers for Disease Control and Prevention, CDC, and he will give us a sense of disease

detection. Then we have Clarence J. Gibbs, acting chief, National

Institutes of Neurological Disorders and Stroke, National Institutes

of Health, for the focus on research into this disease.

Let me just say, all of you are doctors, so I didn’t introduce you

as doctors. You are all experts and we are eager to hear your testimony and welcome you. And if we could go in the order I called

you, that would be helpful. Dr. Friedman.

STATEMENTS OF MICHAEL FRIEDMAN, M.D., DEPUTY COMMISSIONER, FOOD AND DRUG ADMINISTRATION; LINDA

DETWILER, D.V.M., U.S. DEPARTMENT OF AGRICULTURE,

ANIMAL AND PLANT HEALTH INSPECTION SERVICE, ACCOMPANIED BY GLENN MORRIS, FOOD SAFETY INSPECTION

SERVICE, USDA; LAWRENCE SCHONBERGER, M.D., CENTERS

FOR DISEASE CONTROL AND PREVENTION; AND CLARENCE

J. GIBBS, JR., PH.D., NATIONAL INSTITUTES OF HEALTH

Dr. FRIEDMAN. Thank you very much, Mr. Chairman, and members of the committee. We appreciate this opportunity to participate in today’s discussions on measures to prevent the transmission of spongiform encephalopathies. I am Michael Friedman,

and I am the Deputy Commissioner for Operations in the Food and

Drug Administration, and with me are a number of relevant staff

to aid you in your considerations.

12

TSEs, as you know, are transmissible, slowly progressive, uniformly fatal, degenerative diseases of the central nervous system,

not only of humans but several species of animals as well. Examples of TSEs that we will be discussing today include scrapie in

sheep and goats, bovine spongiform encephalopathy, or BSE, in cattle, transmissible mink encephalopathy, and a chronic wasting disease of deer and elk in the wild.

In humans, there are a number of TSEs, but of note today especially is Creutzfeldt-Jakob disease, which will be referred to as CJD

for short.

Mr. SHAYS. Thank goodness.

Dr. FRIEDMAN. A rare disease, CJD effects roughly one to two

persons per million each year worldwide. This rate appears not to

have been increasing despite much more intensified monitoring of

the disease since the mid-1980’s.

As you pointed out, Mr. Chairman, a major concern for this committee has been BSE, and I’d like to reiterate the point that was

made earlier, this disease which was so destructive in Great Britain has not been detected in this country, and since 1989, no cattle

have been imported from BSE countries as designated by USDA.

Now, in recent years, FDA has made an effort to better understand and prevent the possible spread of TSEs. We have acted

alone but also in concert with the Centers for Disease Control, the

National Institutes of Health, and the U.S. Department of Agriculture, as well as relevant industries and consumer groups. The

seating arrangement at this table is symbolic of that real cooperation and collaboration in this regard. Our activities and our formal

internal and external linkages in this framework are described

much more fully in my written statement.

I would like to briefly summarize two major efforts that we’ve

undertaken and your committee has expressed specific interest in.

The most recent major measure is FDA’s proposed rulemaking to

prohibit the use of nearly all tissues from ruminants, animals such

as cows and sheep and goats, and from mink as well, in feed intended for other ruminants. However, earlier, since November

1992, FDA has been asking manufacturers of regulated products to

ensure that they do not use materials from countries where BSEinfected cattle may reside.

Our first warning in this regard was sent to manufacturers of dietary supplements, but we eventually sent similar requests to all

industries in our purview that use animal tissues or animal-derived materials. FDA-regulated products that could contain bovine

tissues are many, but include animal feed, human and animal

drugs and biologics, dietary supplements, medical devices, and cosmetics.

The recent reports of a possible linkage between BSE and a new

variant of CJD and humans has prompted us to take a more comprehensive look at and to take more comprehensive steps to assure

the safety of ruminant feed, which as you noted earlier, seems to

have been the main source of the infection in the United Kingdom.

Our notice of proposed rulemaking in this regard, which is supported by last year’s recommendation from the World Health Organization and other agencies and industry groups, will help assure

that animal protein derived from ruminant and mink tissues is not

13

marketed as a food additive in ruminant feed until FDA is presented with scientific data demonstrating it to be safe. Such data

do not exist at this time.

This precaution is based on evidence that TSE-infected sheep

and cattle tissue in cattle feed seems to have caused and to have

amplified the BSE epidemic in the U.K. We are currently seeking

public comments on our proposal as well as six alternative measures that we also have stated in our proposal, and which are summarized in my written statement, and we plan to discuss these

measures with interested parties at two public fora over the next

month. We believe that the proposed step would be significant help

in preventing the spread of disease in the unlikely event the disease should occur in this country, and we regard the concerns as

fully justifying this proposal.

Another major set of actions that we’ve taken in this area is to

address the otherwise CJD bloodborne transmission and reduce

such risk, if it exists at all. Our blood supply is amongst the safest

in the world, and we know of no reported instances of CJD transmission through blood. In fact, scientists have been unable to

transmit CJD to subhuman primates by infusing them with blood

from a CJD patient. The scientists think the data are not complete

in this area.

There are some studies that suggest that there may be reason for

concern, but while there is much we do not know about CJD, we

recognize the disease has a long incubation period during which it

is currently undetectable, and there is no serum test available for

us to detect it.

Aware of these problems and limitations, FDA has been working

very closely with CDC and NIH as well as blood and plasma recipients, medical professionals, academicians, and blood product industry scientists to determine the most appropriate protective actions

to be taken. Nine years ago, our agency issued a memorandum to

all blood establishments recommending that persons who had received human pituitary-derived growth hormone, a substance

which has been linked to the development of CJD in human beings,

be barred from donating blood.

Three years ago, FDA issued recommendations for more complete

reporting by blood establishments of post-donation information.

This improved identification of blood products in donors subsequently diagnosed with CJD.

In 1994, at FDA’s request, the manufacturers placed the identified end-date licensed injectable derivatives of blood and plasma,

and their intermediates, into quarantine, and in June 1995, the

agency discussed their disposition at a meeting of our special advisory committee on CJD. Acting on the advice of that panel, FDA

in August 1995 issued an interim policy that called for blood products from donors later diagnosed with CJD to be discarded.

Since then, FDA has consulted extensively with experts in this

field, and last month we revised and refined our policy further in

making the following recommendations in order to best utilize

medically valuable products while still protecting the public health.

In particular, we emphasized the importance of donor deferral, the

need for a careful investigation of a family history of CJD, which

14

could be then confirmed by a physician on the basis of diagnostic

and history taking procedures currently available.

Mr. Chairman, we are making every effort to improve the safety

of our food and our blood supply. We will continue to evaluate new

information, recognizing how much we yet do not know about practical aspects of the TSEs, and consider adopting appropriate public

health actions and policies. We do so collaboratively from both

within and outside of government, and I look forward to an opportunity to answer questions that may arise. With me are staff who

will help in that regard.

Thank you for this opportunity.

Mr. SHAYS. Thank you, Dr. Friedman.

[The prepared statement of Dr. Friedman follows:]

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Mr. SHAYS. Dr. Detwiler.

Dr. DETWILER. Good afternoon, Mr. Chairman, Members. Thank

you very much for giving me this opportunity to appear before the

subcommittee to discuss the Animal and Plant Health Inspection

Service’s, or APHIS, efforts to prevent our Nation’s cattle from becoming infected with bovine spongiform encephalopathy, BSE.

As you’ve announced, my name is Linda Detwiler. In addition to

serving as APHIS’s Area Veterinarian in Charge for New Jersey,

I also chair the APHIS TSE working group.

Mr. Pappas, I just want you to know that even though New Jersey, we have the nickname the Garden State, most people don’t

think of us as that, but I grew up on a farm. My dad still has a

farm, and I keep a couple of Jersey cows that are big, fat, and

happy.

Mr. PAPPAS. If I could, Mr. Chairman, just to mention, I come

from the 12th District of New Jersey, which I am told from the

New Jersey Farm Bureau is the largest cattle producing district in

the State of New Jersey; 26 head. I know that is relative compared

to other States.

Dr. DETWILER. With me today is Dr. Glenn Morris of the public

health division of USDA’s Food Safety and Inspection Service to assist me with questions that might pertain to FSIS.

APHIS is a part of the U.S. Department of Agriculture’s Marketing and Regulatory Programs mission area. Our primary responsibility is to protect the health of U.S. agriculture from foreign

animal and plant diseases and pests that could adversely impact

production and hamper the health of our Nation’s livestock. This

ensures that our Nation’s crops, poultry, and livestock are marketable both domestically and overseas.

In carrying out this mission, APHIS closely monitors the agricultural health situations of our trading partners; regulates the importation of animals and animal products based on the potential

risk of agricultural disease or pest introduction; and conducts ongoing surveillance programs to ensure that no diseases or pests of

concern have slipped past our defenses. In the event of an outbreak, APHIS is poised to immediately implement emergency response efforts. Working together with the industry and other State

and Federal agencies, we provide a nationwide agricultural health

infrastructure.

To reiterate, BSE has not been detected in the United States,

and USDA has worked aggressively and proactively to keep it that

way. The measures APHIS has taken in this regard include prohibitions and/or restrictions on certain animal and product imports;

ongoing surveillance for signs of the disease in the United States;

preparation of an emergency response plan in the unlikely event an

introduction were to occur; and ongoing educational efforts.

APHIS has formed a TSE working group, which is composed of

an agency pathologist; an epidemiologist; veterinarians from our

import-export; emergency programs; and international services

staffs; three of our field veterinarians, including myself; and a public affairs specialist.

Our group continually monitors and assesses all ongoing events

and research findings regarding spongiform encephalopathies, as

new information and knowledge may lead to revised conclusions

43

about risk, pathology, and improved diagnostic and prevention

measures.

The working group also disseminates information about TSEs

and serves as a reference source for questions about these diseases.

In doing this, we have actively shared information and coordinated

closely with each of the Federal agencies represented here today,

as well as the States, the livestock and affiliated industries, veterinary and research communities, and consumer groups. Together,

all of us are working to ensure that the Federal approach to TSEs

is based on the most up-to-date and sound scientific data available.

Before I begin to discuss our program to exclude BSE from the

United States, I would like to begin with some background on the

status of TSEs in this country.

The primary TSE known in this country is scrapie. It was first

diagnosed in the United States in sheep and goats in 1947, and

since 1952 the United States has had some form of eradication and

control for the disease. Since 1992, these efforts have taken the

form of a nationwide scrapie flock certification program and interstate movement restrictions on sheep and goats from infected and

source scrapie flocks.

The intent of the program is to monitor flocks over a period of

5 years or more and certify for health and marketing purposes

those that have not displayed evidence of scrapie, and another aspect of the program is to prohibit the movement of high risk animals from scrapie flocks in interstate commerce. Scrapie has existed in some countries, most notably Great Britain, for centuries,

and sheep with the disease have never been shown to pose a direct

risk to human health.

Currently, APHIS is working with the sheep industry to reexamine our program and make adjustments as needed to both the regulations and certification programs. We’re also working to develop a

national effort of active scrapie surveillance using the most recent

diagnostic techniques. If this effort is successful, we will be the

first Nation in the world to achieve this end.

I can also speak on a personal nature. APHIS provides a lot of

samples for the research community. Like Dr. Joe Gibbs, I’ve selected cerebrospinal fluid samples, I’ve collected tonsil biopsies.

That’s a little more difficult, as the sheep don’t want to open their

mouths and say ‘‘ahh’’ too easily.

In 1989, APHIS banned all live cattle and other ruminants and

restricted the importation of most cattle products from Great Britain, which at that time was the only country known to have BSE.

As other countries have reported BSE in native cattle, they have

become subject to these same restrictions.

In 1991, APHIS formalized these restrictions with regulations.

Under these regulations, certain products cannot be imported into

the United States, except under special permit for scientific, educational or research purposes or under certain conditions. These

products include serum, meat-and-bone meal, bone meal, blood

meal, offal, fat, glands, and collagen. Gelatin derived from

ruminants from BSE countries is currently prohibited entry into

the United States for use in animal feeds or for any purpose that

would result in contact with ruminants. All these were enacted to

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protect the health of U.S. livestock and safeguard our human population as well.

APHIS has a comprehensive surveillance program in the United

States to ensure swift detection and control in the unlikely event

BSE introduction occurred.

To ensure that we would be able to identify BSE readily if it

were to appear in the United States domestic cattle herd, we sent

USDA pathologists to Great Britain after the disease was first

identified in 1986. Our goal was to learn directly from our British

counterparts about the pathology of the disease and diagnostic

techniques. In addition, USDA has trained over 250 Federal and

State field veterinarians throughout the United States and several

of our diagnosticians have spent time in Great Britain in an effort

to learn from that country’s experience in the disease.

USDA’s Food Safety and Inspection Service performs pre-slaughter inspections at all federally inspected slaughter establishments,

and their inspectors are on the alert for animals that appear to

have central nervous system disorders. Any animals exhibiting

neurological signs similar to those seen with BSE are condemned,

and their brains are submitted to APHIS’s National Veterinary

Services Laboratories for analysis. In addition, private veterinarians refer neurologic cases to us directly from the farm or from veterinary schools.

Since 1990, more than 60 diagnostic labs throughout the United

States and USDA’s National Veterinary Services Laboratories have

examined thousands of cattle brains submitted from adult cattle

displaying neurologic disease signs either at slaughter or on the

farm. I provided in my written testimony the number of brains submitted. I’ve updated that. As of January 23d, we’ve examined 5,342

brains with no evidence of BSE.

We’ve also provided veterinary practitioners, lab diagnosticians,

and inspectors with information to assist them in recognizing the

clinical signs of BSE, and I really want to emphasize this, the importance is we educate producers on what to look for and where to

report it. That is one of the best methods also of surveillance, and

I think APHIS has really tried to concentrate our efforts in this

education.

In the unlikely event we have a BSE occurrence, we have developed an aggressive emergency action plan to deal with the animal

health and public health issues. The plan includes immediately informing Congress, concerned State and Federal agencies, the livestock industry, consumer groups, and the general public about the

implications of such an outbreak and what we would be doing to

respond in terms of handling the animals and animal products, and

in the area of surveillance, if this is committed, to continually work

with researchers both in the United States and abroad to update

our diagnostic techniques, which is a key to us for surveillance. The

education, as I stated earlier, is critical. We have developed training materials, video we have obtained from the United Kingdom.

I’ve submitted those to your committee for information, video on

scrapie, BSE, fact sheets on those two diseases and chronic wasting

disease.

Although BSE has not been diagnosed in the United States, we

support the Food & Drug Administration’s effort to provide an adVerDate 11-MAY-2000 09:36 Jun 28, 2002 Jkt 078738 PO 00000 Frm 00048 Fmt 6633 Sfmt 6602 W:\DISC\39522 pfrm09 PsN: 39522
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ditional safety net by banning certain products in ruminant feed.

We are currently continuing to review that proposal carefully and

we will submit formal comments on its specific provisions as part

of the rulemaking record.

Mr. Chairman, members of the committee, thank you for providing us the opportunity to alleviate public concern about any risk

of BSE introduction into the United States. By taking the necessary precautions to prevent known risks such as importing infected cattle or cattle products, as well as other potential risks such

as introduction and amplification of the agent in the cattle food

chain, we are protecting the cattle population. And a BSE-free cattle population safeguards all of us as consumers against the possibility of a human health risk.

And may I ask, I brought a simple diagram—— Mr. SHAYS. Sure. This will be concluding your comments?

Dr. DETWILER. Yes.

Mr. SHAYS. You know, what I am going to ask you to do is maybe

just turn that mike that is up there and see if you could speak

somewhat toward there so it is part of the record. At least kind of

project your voice that way.

Dr. DETWILER. This diagram will—— Mr. SHAYS. I am asking you to do something impossible, I am

sorry.

Dr. DETWILER. Yes, sorry.

Dr. FRIEDMAN. Would you like me to point and you can just

speak?

Mr. WAXMAN. Mr. Chairman, there is a court reporter, so we

don’t have to get a recording.

Mr. SHAYS. Are you picking it up?

The REPORTER. Yes.

Dr. DETWILER. The known risks of BSE would be the foreign

sources of BSE. In 1989, APHIS took the precautions to shut that

off. So we have a protection against the cattle population. That’s

a known risk against an introduction.

Now, the unknown or the unquantified risks and the potential

risks would be a spontaneous case occurring in cattle or some link

with sheep, and the two theories for the origin of BSE is it came

from scrapie infected sheep incorporated into the rendering chain,

and by some change in the rendering, got into a cattle feed ration,

and then the feed fed back to the general population. The other is

from a spontaneous case occurring in a cow, through the feed chain

with rendering, changes in rendering process incorporated into the

general population through feed into the feed supply in a country

and through into the U.S. cattle population. And with the FDA’s

proposed regulation on the ruminant feed, that would prohibit this

into the United States, thereby shutting off both the known risks

of BSE as well as the unquantified or possible risks. And then by

protecting the U.S. cattle population, that would protect the human

population for the use of cattle products.

And I would just like to say, too, one thing on a personal note.

In 1985, I took the job—I left private practice to take the job with

the government and I had some hesitation to do that because of

perceptions of family, you know, government employees, and

friends and colleagues, and because of personal reasons I said I will

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do this for a year. I got involved with scrapie early on and I worked

in the agency to control and eradication and surveillance of scrapie.

Early on with BSE I got involved with the agency’s preventive actions. All along with these efforts, I am here to tell you I am not

high up in the department, I am not high up in APHIS. I’ve been

involved in the day-to-day dog fights with these programs and the

disease, and the thing that made me stay these number of years

are the people I worked with, not only in the agency, the people

like my colleagues in APHIS and like Dr. Gibbs, that give me their

phone number and say call me any time day or night, or if there’s

something we need to know on a day off will go into the office and

fax me some research. People in the industry that are willing, saying what do we need to do. The sheep industry, people that sat at

their table and cried because of the loss of their flocks, said we’ll

donate our flock to research. Those people. International colleagues

that we share frustrations, and I have family in all these places.

So from my 1985 to 1986 game plan, I am here to tell you in

1997 I am still here because I am proud now to say that I am a

Federal employee, I am proud really to say that I work for APHIS,

who is an agency that is not complacent, and I work with a lot of

good colleagues. So hopefully when you call me back in 20 years

when I am ready to retire, I will say the same thing.

[The prepared statement of Dr. Detwiler follows:]

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Mr. SHAYS. Well, we’re proud to have you here and hopefully

when we do that, maybe you will be running one of those agencies

in 20 years.

We have now Dr. Schonberger, and we welcome your testimony.

Thank you, Dr. Detwiler.

Dr. SCHONBERGER. Good afternoon. I am Lawrence Schonberger,

from the Centers for Disease Control and Prevention. I coordinate

CDC’s surveillance on Creutzfeldt-Jakob disease, CJD. I am accompanied by Dr. Rima Khabbaz and Dr. Bruce Evatt. We are pleased

to discuss CDC’s role in two public health issues about CJD: First,

whether a possibly new variant form of CJD reported in the United

Kingdom may represent food-borne spread to humans of bovine

spongiform encephalopathy; and second, whether CJD may pose a

risk to blood safety. To help in the assessment of both these issues,

CDC gathers and interprets surveillance data about CJD.

Bovine spongiform encephalopathy was first diagnosed in 1986

as part of an ongoing outbreak in cattle in the United Kingdom.

Although there is no general agreement among investigators about

the original source of this outbreak, or epizootic, there is general

agreement that feeding rendered bovine meat and bone meal to

young calves amplified the spread of this disease. Indeed, the key

control measures which were directed in eliminating the use of ruminant protein for ruminant feed, what we’re discussing today had

a marked beneficial effect.

Based on 10 persons with onset of an apparently new variant

form of CJD in 1994 and 1995, an advisory committee in the

United Kingdom announced its concern just last March that these

patients could represent the beginning of an epidemic in humans

that might parallel the course of the epizootic of the bovine

spongiform encephalopathy, but delayed a few years. Shortly thereafter, consultants called for the establishment of worldwide surveillance programs for both bovine spongiform encephalopathy and the

newly recognized form CJD.

In the United States, as you’ve just heard, the USDA has reported no evidence of the cattle disease and CDC has found no evidence for the occurrence of the human disease. CDC’s surveillance

efforts for the new variant CJD have included ongoing reviews of

national mortality data, an active surveillance effort in CDC’s

emerging infections programs, ongoing reviews of hospital records

of patients under 55 years of age identified through national mortality data in collaboration with State health departments, and a

new collaboration with the American Association of Neuropathologists to obtain reports of suspected cases of the new variant

CJD regardless of age or initial clinical diagnosis.

In my written testimony I explained why I believe the evidence

now is strong that the newly described variant represents a novel

form of CJD. Whether this novel variant is causally linked to bovine spongiform encephalopathy, however, is less clear. Although

the accumulating evidence for such a link is increasing, continuing

surveillance of CJD and bovine spongiform encephalopathy in

many countries, including the United States, and especially in the

United Kingdom, will be critical for determining whether and to

what extent the agent of bovine spongiform encephalopathy may be

causing disease in humans.

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In the meantime, because of the general acceptance that ruminant-to-ruminant feed played a role in amplifying bovine

spongiform encephalopathy in the United Kingdom and because of

the risk of the possible transmission of this cattle disease to humans, CDC continues to support FDA’s proposal to modify or end

this cattle feeding practice in the United States.

CDC surveillance data have also been used to examine where

CJD may pose a risk to blood safety. Although some laboratory experimental studies support concern about such a risk, epidemiologic data indicate that this risk, if present, must be low. Published

case control studies and limited followup data on patients who received blood units from a CJD donor, for example, have not indicated an increased risk of CJD in blood recipients. The 3,642 cases

of CJD in the United States reported through CDC’s mortality system, 1979 through 1994, demonstrated stable annual rates of this

disease. Thus, despite regular blood donations by donors who subsequently developed CJD, blood transfusions do not appear to be

amplifying CJD infections in the population.

In addition, none of these several thousands cases of CJD were

reported also to have had hemophilia, thalassemia or sickle cell diseases, diseases with increased exposure to blood or blood products.

Because clotting factor concentrates used by hemophilia patients to

control bleeding are commonly derived from 4,000 to 30,000 blood

donors, CDC has also sought cases of CJD specifically among persons with hemophilia. None have been found.

CDC and the American Red Cross have initiated a study of recipients of transfusible blood components derived from CJD donors.

At last report, of the 23 investigated recipients who survived 5 or

more years after their transfusion, none had died of CJD. So despite some experimental evidence suggesting a potential for bloodborne transmission of CJD, the accumulating epidemiologic data

have strengthened CDC’s previous conclusions that the risk, if any,

for transmission of CJD by blood products is extremely small and

theoretical.

Thank you for the opportunity to discuss these public health

issues concerning CJD, and I will be happy to answer questions

you or other members of the subcommittee may have.

[The prepared statement of Dr. Schonberger follows:]

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Mr. SHAYS. Thank you, Dr. Schonberger.

Dr. Gibbs.

Mr. GIBBS. Thank you. I would like to thank the subcommittee

for inviting me to participate in the hearing. My name is Clarence

Joseph Gibbs, Jr. I am a Ph.D., and I received my undergraduate

and graduate degrees from the Catholic University of America here

in Washington, DC. For more than 30 years I have served as a research scientist and currently as the Acting Chief of the Laboratory

of Central Nervous System Studies, Division of Intramural Research, National Institute of Neurological Disorders and Stroke at

the National Institutes of Health.

I also hold appointments as teaching and research Associate Professor, Department of Neurology and Department of Pathobiology

of the Johns Hopkins University Medical Center, Baltimore.

I also serve on numerous interagency task forces, including the

Public Health Service Interagency Coordinating Committee on

Human Growth Hormone and Creutzfeldt-Jakob disease, Interagency Committee on Bovine Spongiform Encephalopathy and the

Interagency Animal Model Committee.

I also serve as senior scientist and consultant chairman on the

transmissible spongiform encephalopathies, to the Division on

Emerging Diseases of the World Health Organization in Geneva

and to the Division of Neurosciences of the Pan American Health

Organization.

Today, I will provide a brief overview of the transmissible

spongiform encephalopathies and discuss the implications for

human use of animal products and the safety of the blood supply.

Mr. Chairman, as I prepared this testimony which addresses

rather recent health concerns, I was struck by the fact that much

of our understanding of these topics stems from a study of child

growth and disease patterns in primitive cultures, first initiated in

our Neurology Institute by Carleton Gajdusek in 1959.

Forty years ago the study of the spreading epidemic of kuru, a

fatal neurological disease in children and adults in the remote

highland interior of New Guinea, led to the first recognition and

demonstration in our laboratory of slow virus infections of man.

Kuru occurred in Stone Age cultures where it was spread by contamination of infants, children, and adult females with brain tissue

in a mourning ritual of cannibalistic respect for the dead. Discovery

of such slow infections led our laboratory to demonstrate that

Creutzfeldt-Jakob disease and Gerstmann-Straussler-Scheinker

syndrome were caused by infectious agents that were related to the

agent causing scrapie in sheep and goats. The kuru discovery also

led us to recognize that fatal subacute sclerosing panencephalitis is

a delayed and slow measles virus infection; that transverse myelitis and adult T-cell leukemia are the result of human lymphotropic

virus type-I human retrovirus infection; and that AIDS is a slow

infection with the HIV retrovirus.

Our kuru study led to the identification of a new group of subviral pathogens in which the infectious agent is not a nucleic acid,

but which are beta-pleated proteins or amyloids often called prions.

The diseases caused by these agents are characterized by brain tissue giving a ‘‘spongy’’ appearance upon examination under the microscope, hence the term spongiform encephalopathy. In more modVerDate 11-MAY-2000 09:36 Jun 28, 2002 Jkt 078738 PO 00000 Frm 00074 Fmt 6633 Sfmt 6602 W:\DISC\39522 pfrm09 PsN: 39522
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ern societies, the medically induced spread of Creutzfeldt-Jakob

disease has been shown to result from contaminated human growth

hormone, dura mater grafts, corneal transplants and brain electrodes which are viewed as the result of intended beneficial

invasive procedures.

The onset of the rapidly fatal central nervous system diseases

caused by these agents may occur many decades after primary infection by the peripheral route. On inoculation directly into the

brain or eye, incubation periods may be only 1 to 2 years.

Our recognition that the Gerstmann-Straussler-Scheinker syndrome was transmissible and thus belonged to the group of

spongiform encephalopathies demonstrated for the first time that a

human brain disease can arise in an autosomal dominant pattern

of inheritance, but at the same time can arise through infection.

This in turn led to our elucidation that familial forms of

Creutzfeldt-Jakob disease and related diseases are due to

mutations on the gene of the prion protein. This combination of genetic and infectious etiology had not been previously described in

human medicine.

We have demonstrated infection as the etiology of five human

diseases and five diseases affecting animals. These we have classified as the Transmissible Spongiform Encephalopathies, or more

correctly the Transmissible Cerebral Amyloidoses. In humans they

are: kuru, Creutzfeldt-Jakob disease, Gerstmann-StrausslerScheinker syndrome, Fatal Familial Insomnia, and the new variant

Creutzfeldt-Jakob disease first observed in Britain last year. In

animals these include scrapie, transmissible mink encephalopathy,

chronic wasting disease of deer and elk, and bovine spongiform

encephalopathy. All are experimentally transmissible to nonhuman

primates and laboratory rodents. These transmissions have permitted us to determine the pathogenesis of each of these diseases

and to demonstrate their unique physical, biological and biochemical properties. As a group, their infectivity is resistant to

treatment with most organic and inorganic chemicals, they are

thermostable, and high levels of ionizing radiation and ultraviolet

light have no effect. Moreover, we have tested literally hundreds of

drugs in infected animals and a number have been administered to

a few patients by non-NIH physicians without success.

The recent French report that the prion protein is not detectable

in material that transmits BSE to mice does not necessarily demonstrate that the infectious agent is something other than the betapleated protein. The transmission of an infectious amyloid disease

without detectable PrP, or prion protein, in the brain should not be

surprising. The assay for prion protein is not sufficiently sensitive

to detect it before infectious titers, that is, levels in the brain,

reach many thousands of infectious doses per gram. In the mid

1960’s, we demonstrated with our French and English collaborators

that during the early incubation of the transmissible spongiform

encephalopathies, when the virus titer in the brain was still very

low, there were already marked functional changes, even though no

pathology was yet detectable, even by electron microscopy. A month

or two later, polynucleation of neurons appeared in spider monkeys, incubating kuru, and somewhat later, microvacuolation and

membrane changes visible only by electron microscopy. This preVerDate 11-MAY-2000 09:36 Jun 28, 2002 Jkt 078738 PO 00000 Frm 00075 Fmt 6633 Sfmt 6602 W:\DISC\39522 pfrm09 PsN: 39522
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ceded the first appearance of astrogliosis and spongiform change.

It was only much later that the classical scrapie-TSE pathology appeared with virus titers in brain of 10 to the minus 5 or higher.

Thus, it is clear that early replication to only low infectivity titer,

far below that necessary to detect prion protein biochemically or

immunologically, can already lead to disease, including the cardinal

electroencephalographic change signs of extensive hypsarhythmia

of the Lennox-Gastaut syndrome in rhesus monkeys. It is no surprise that on further passage, especially into a different host, prion

protein appears at detectable levels. Thus, in my view, the recent

French work reported in Science does not indicate that an infectious amyloid is not responsible for the disease. Instead, it further

confirms that such a nucleating protein is present, since prion protein appears on passage into a host producing high titer of the

nucleating agent.

In Fatal Familial Insomnia, many patients have no detectable

prion protein, and presumably very low titer infectious amyloid.

Yet this early nucleation is sufficient to cause progressive fatal

neurophysiological derangement. Dr. Brown in our laboratory has

demonstrated that there is considerable variability in the presence

of prion protein in different brain areas in different cases of FFI

and CJD; in certain areas often none is found. Variation in the concentration and distribution of the infectious protein has also been

noted in bovine spongiform encephalopathy in infected cattle brain.

The committee has asked that I discuss the differences between

the transmissible spongiform encephalopathies in human immunodeficiency virus, another slow infectious agent. As noted earlier,

the so-called conventional viruses, including retroviruses such as

HIV, do cause slow infection. The differences, however, are that unlike the spongiform encephalopathies, conventional viruses contain

either DNA or RNA, induce specific antibodies, are inactivated by

most chemicals, heat and radiation, and can be identified by electron microscopy and immynological techniques.

Early in the course of our studies we sought to determine the

mode of transmission in these diseases, particularly in CreutzfeldtJakob disease, since 90 percent of the cases occur sporadically at

the rate of one to two deaths per million population wherever you

look for it. We had ample evidence that in kuru there is no vertical

transmission and no evidence of infectivity in blood or breast milk.

The same can be said about our inability to detect infectivity in

donor units of Creutzfeldt-Jakob disease human whole blood transfused to chimpanzees or packed lymphocytes from patients inoculated into small monkeys more than 20 years ago. In spite of these

early negative studies which are still in progress, concern about the

possibility of transmitting Creutzfeldt-Jakob disease through blood

or blood products has arisen in recent years as increasing numbers

of blood donors who later died from CJD have been identified. Substantial evidence from experimentally infected animals, and fragmentary evidence from humans with CJD, indicates that blood, and

particularly white blood cells, may sometimes contain low levels of

the infectious agents. We are conducting a study in collaboration

with the National Heart, Lung and Blood Institute, Food and Drug

Administration, the American Red Cross, and the Communicable

Disease Center to address two specific questions.

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First, we seek to determine the distribution of infectivity in components and plasma derivatives of normal human blood to which

had been added a large amount of the infectious agent; that is, to

see whether any blood component or plasma derivative might be

free of infectivity in spite of an unrealistically large infectious

input. For this study we added a suspension of high titer hamster

scrapie brain cells to normal whole blood and will assay them for

infectivity. Second, we will determine the distribution of infectivity,

if present at all, in components and derivatives in an experimental

model characterized by a low blood level of circulating pathogen.

For this study, we chose to analyze blood from terminally ill mice

that had been inoculated with a mouse-adapted strain of

Creutzfeldt-Jakob disease in order to look for infectivity.

In addition, we have initiated attempts to isolate the infectious

agent from the blood and blood products of humans with clinically

evident CJD, as well as mutation-positive but still healthy members of CJD families to examine the infectious status of blood during the preclinical phase of disease. These specimens will be inoculated in parallel into two types of assay animals: squirrel monkeys:

known susceptibility, but expensive, and with an extended period

of observations; and transgenic mice carrying a human prion protein gene insert: limited knowledge about susceptibility, but less

expensive, with a period of observation of less than 2 years.

It is important to note that there has never been a recorded case

of CJD in a hemophiliac patient.

In view of the fact that none of the transmissible spongiform

encephalopathies have proven susceptible to treatment, there is understandable concern about human exposure to food and other

products from infected animals.

Since only 2 of the 6,000 patients in the world have been under

20 years of age, and none under 14 years of age, we have pointed

out that the appearance at this time in Great Britain of CJD in

adolescents and prepubertal children could represent a possible

link with the bovine spongiform encephalopathy epidemic. This

would not mean that beef or sausage produced from mixtures including viscera of slaughtered cattle animals was the cause, nor

could it clearly implicate the milk and milk products.

Mr. SHAYS. I am going to ask you if you would bring your statement to a conclusion. I think you have been in this business so long

that I am afraid that you can keep us here a long time.

Mr. GIBBS. I could keep you forever.

Mr. SHAYS. I know you could.

Mr. GIBBS. I don’t mind talking.

Mr. SHAYS. I understand.

Mr. GIBBS. All right, I will wind it up, then, Mr. Chairman.

Mr. SHAYS. Thank you.

Mr. GIBBS. I would simply like to wind it up by saying that our

current research efforts continue to focus entirely on the transmissible spongiform encephalopathies.

In addition to our overall efforts on these diseases, we are concentrating on the following areas: The studies we have proposed for

blood and blood products; the isolation, purification, and characterization of the normal prion protein and the method of its converVerDate 11-MAY-2000 09:36 Jun 28, 2002 Jkt 078738 PO 00000 Frm 00077 Fmt 6633 Sfmt 6602 W:\DISC\39522 pfrm09 PsN: 39522
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sion into its pathological abnormal isoform; and the molecular biology of the spongiform encephalopathy.

And finally, Mr. Chairman, I would like to say that in the four

decades that I’ve been working in this field, all of our work has

been done in collaboration with Food and Drug Administration, Department of Agriculture, Centers for Disease Control, all the Federal agencies. But just as importantly, it has involved most of academia in the United States and, by and large, it is fully international in scope and in work.

Thank you, Mr. Chairman, for the opportunity for presenting this

testimony.

[The prepared statement of Mr. Gibbs follows:]

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Mr. SHAYS. I thank the gentleman. It has been very important

for us to hear your testimony. I am sorry we have had a little bit

of distraction.

I am going to invite any of the guests that are sitting up in the

front to move away from the table. Thank you. And again I apologize to those of you who have been trying to have a place to sit,

and we will try to make sure we deal with that next time.

At this time I would ask Mr. Towns. You have the floor, Mr.

Towns.

Mr. TOWNS. Thank you very much, Mr. Chairman.

Let me just sort of indicate that we do not want to frighten anyone, as was already indicated, but we want to make certain that

everyone is safe.

Dr. Friedman, what steps has the FDA taken to issue a warning

to hunters and other communities like Indian reservations where

there is a high consumption of venison and other wild game that

could be actually infected by BSE?

Dr. FRIEDMAN. Our Center for Food Safety has been in touch

with individuals associated with State wildlife commissions, especially for the State of Colorado and for the Department of Wildlife

Management for Wyoming.

There has been quite an active program on the State level to do

two things. One is to better assess the incidence of these chronic

degenerative diseases in the deer and elk that are being hunted

and to find out as much as they can about the incidence of these

infections in those populations.

A second effort that’s been carried out at the State level by these

individuals and by others has been to educate the hunter population to look out for animals acting unusually, to submit specimens from those animals that are killed for those specimens then

to be looked at to see if the disease exists, and then to warn those

hunters not to consume meat from those animals until such time

as they’ve been tested or, if there is any doubt, to be extra safe and

to not do it at all.

We recognize that assessing the wildlife population is a very difficult thing. We know of these two areas where this chronic disease

does exist, and we feel that this is a very good start toward educating those populations.

Mr. TOWNS. Do you feel there are other things that should be

done?

Dr. FRIEDMAN. I think we’re still at the point of gaining information about how widespread the penetrance is of this abnormality in

the deer and elk populations in the United States.

I think that educating the community of hunters to look out for

animals acting unusually is a prudent thing to do. I need more information, and we’re in the process of trying to gather that information before promulgating other steps, but I think this is something we’re going to pay attention to for the future.

Mr. TOWNS. Thank you.

Let me raise another issue. Is there anything to be worried about

in terms of cosmetics or even dietary supplements as well? Should

we have any concerns?

Dr. FRIEDMAN. I think that the—I’d give you the following answer. The short answer is, I don’t believe so. Now let me document

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why that is. It is not a simple assertion, and it is not one made

without careful consideration.

In a situation where you don’t have all the scientific information,

and we do not, we must be mindful and open minded of new information as it emerges. The second thing is that we should have a

threshold which is relatively low to protect the American public.

We know that for the last several years there has been an import

alert partially done by the Food and Drug Administration. There’s

been an import prohibition from the U.S. Department of Agriculture for those products coming into the United States from BSE

animals and BSE countries.

We have more recently received information from the European

Community that there is an absolute prohibition on using BSE animal parts in cosmetics not only for use within the European Community but it’s also a prohibition for export to other places, like the

United States we must presume.

Therefore, as we look at the wide variety of products that are

used in cosmetics, we see that the vast majority of those are coming from non-BSE countries and that, with new rules being promulgated by the European Community, we’re very comfortable with

products made in the United States. We know there is no BSE, we

know that those are—from U.S. animals do offer the American public the confidence that they need, and we see efforts being made by

foreign governments to try to do that as well.

Mr. TOWNS. Thank you.

Is there any evidence that blood products have been linked to

CJD?

Dr. FRIEDMAN. I’d be happy to let the Centers for Disease Control answer that as well. But our review of this says that we have

not been able to demonstrate convincingly any case of blood-borne

transmission of CJD.

Dr. SCHONBERGER. The evidence for some concern at all comes

from laboratory and experimental studies. There have been four

different reports in the literature where the researchers have said

that they’ve been able to isolate or to show infectivity of blood in

a sick CJD patient.

Of course we’re worried in the blood risks area about what happens before the donor with CJD gets sick because that’s when this

person donates the blood.

In that area, there’s some animal model studies that have demonstrated that in those animal models—and we’re talking about rodents now who have been injected with a high dose of the infectious material. In those rodents, indeed we can detect infectivity in

the blood throughout much of the incubation period. So that’s the

basis for the theoretical risk concern.

Now, at CDC we’re interested in looking at what does this mean

in terms of the human risk, and in that area we have not been able

to demonstrate or find any evidence, any convincing case resulting

from exposure to blood or blood products, including hemophilia patients, who are known, because of the clotting factor that they receive, to be statistically exposed to a CJD donor at some point because of the 10,000 to 30,000 different donors that contribute to the

concentrate that they receive.

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And if the person who is a hemophiliac gets treated as a youngster for many years, somewhere along the line one of those donors

is going to have been incubating CJD. And yet we still don’t have

an increased risk in the hemophilia population. We’re getting to

the point now where you’d almost expect a case by chance alone

given the size of their population, and we can’t even find that case.

So the bottom line from our perspective is that it is a theoretical

risk, for the reasons that I’ve cited, but it is not as yet really a real

risk, and so our control measures need to take that into consideration.

And what we want to make sure that we do—and we are talking

here about balance—is that we don’t institute control measures

that are more risky than the risk itself of the disease from the

product that we’re talking about; and that’s the tricky balance.

In this area, the newspapers and other public media can be helpful to us because they need to educate the recipients of blood products to know that there is this theoretical risk, OK, but it’s not a

real risk, not something to be scared about at this point. There is

nothing there now to indicate the real risk.

Mr. TOWNS. Dr. Friedman, let me ask you this, then: What are

you doing to monitor blood products?

Dr. FRIEDMAN. This is a joint effort between the Centers for Disease Control, ourselves, and organizations like the American Red

Cross.

The monitoring takes place in a couple of different ways. The

first is to try to identify those donors who, unbeknownst to themselves, already have CJD and may show the clinical symptoms at

some later date.

We need to identify those individuals, identify individuals who

are at high risk of having the disease for familial reasons, and then

to segregate off their blood products to make decisions. That’s one

sort of observation.

A second set of observations are for those individuals who receive

blood products from a CJD donor who didn’t know he or she had

CJD at the time they made the donation, and then to carefully

evaluate those individuals to look for the sort of long-term findings

that we’re talking about.

Clearly there is a theoretical risk, but we know that this is not

a highly infectious situation. It has been estimated that each day,

despite the very best efforts of the blood programs and in our own

efforts and other efforts at the State level, despite those best efforts, we know that there are CJD individuals donating blood unbeknownst to themselves and unbeknownst to the blood bank, and

yet we’re not seeing a rise and we’re not seeing cases of CJD resulting from that, so that we know that the risk may be very, very

small.

That doesn’t make us comfortable. It only makes us more vigilant.

Mr. TOWNS. It didn’t make me more comfortable either.

Dr. FRIEDMAN. No. And that’s absolutely correct, sir.

Mr. TOWNS. Thank you, Mr. Chairman.

Mr. SHAYS. Thank you, Mr. Towns.

The gentleman has the floor.

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Mr. PAPPAS. Could any of the panelists tell me or tell the rest

of us, as well, have there been any other recorded incidents of any

other species in any other country that may suffer from BSE or

scrapie or any other similar type of disorder?

Dr. DETWILER. I can address the animal area. These other

spongiform encephalopathies in animals that have been detected

are scrapie in sheep. Most of the world actually is thought to have

countries where scrapie is endemic.

Probably the two that might be recognized as scrapie free

throughout the world—and that’s not by everyone but commonly—

are New Zealand and Australia for scrapie.

Another disease called transmissible mink encephalopathy that’s

been diagnosed in ranch-raised minks, it has been diagnosed in the

United States. The last case was in 1985. Prior to that, we had

cases in 1947 and a few in early 1960’s. TME, or mink

encephalopathy, has also been diagnosed in Canada, Russia, Finland, and Germany. Chronic Wasting Disease, that’s of the captive

mule, deer, and elk in the United States; there’s been a spongiform

encephalopathy diagnosed in cats, both domestic and large cats.

That’s been in the domestic cats in the United Kingdom; 75 cases,

1 in Norway, 1 in Lichtenstein, that’s been associated with feed as

well, and in exotic ruminants in zoos also associated with feed in

the United Kingdom.

And when I say the exotics, I mean kudu or the gemsboks, things

that you normally see on the plains, in zoos.

So that’s the animal spongiform encephalopathies.

Mr. PAPPAS. And is there any reason to believe that these have

any reason to spread? I mean, many of those instances you’ve spoken about were decades ago, so it sounds as though the incidents

come less and less.

Dr. FRIEDMAN. If I might just offer one observation, there seems

to be for many of these diseases fairly solid species barriers between one species infecting another. Herdsmen have lived with

scrapie-infected sheep for hundreds of years and there hasn’t been

a disease easily identifiable with that.

So there has been sheep shearing and slaughtering and so forth,

and even under those sort of situations we haven’t seen a human

disease that we can easily point to. That doesn’t mean that it

couldn’t occur, but it means that for many of the most prevalent

diseases we haven’t seen that in humans.

The question of what’s happening with BSE and the new variant

Creutzfeldt-Jakob disease is an area of very intense investigation,

as has been described.

Dr. DETWILER. One other point to make with the animal

spongiform encephalopathies: There doesn’t seem to be between

species, like sheep and cattle, contagious spread. There’s no evidence of that at all, like if you house cattle with sheep versus one

sheep spread from one to the other.

Mr. PAPPAS. By each of your identifications here as to which Federal agency that you’re identified with, we have the Food and Drug

Administration, we have U.S. Department of Agriculture, Centers

for Disease Control and Prevention, and National Institutes of

Health.

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Are there other Federal agencies that are involved in researching

these issues?

Dr. DETWILER. The Agricultural Research Service, part of the

U.S. Department of Agriculture, is extremely involved. We work

with them, as does NIH and the others.

Mr. PAPPAS. Is there any—and this is not to suggest that I don’t

agree with the research that is ongoing, but is there any reason to

believe that there is any duplication of effort by you folks?

Dr. FRIEDMAN. Do you mean in terms of research?

Mr. PAPPAS. Any of the involvement that you and your peers and

colleagues in the various agencies, yes.

Mr. GIBBS. I’d like to address that. In my experience throughout

the years, there has been no duplication, it has been a collaborative

effort, and, by and large, it has been one agency covering one aspect, another agency covering a different aspect based on the discipline involved in that institute.

So, in fact, there’s been no duplication but certainly coordination

in all of our work.

Mr. PAPPAS. Would the rest of you agree with that?

Dr. DETWILER. I’d like to also address that. I serve on an ad hoc

group for an agency known as Office of International Epizootics to

represent the United States, and that agency also coordinates research efforts. We had a meeting in October in France to do that.

And you would find that, almost worldwide, that this community

of researchers does not seem to duplicate but to coordinate. And I

know with ARS and efforts we’ve done within the Department of

Agriculture, we’ve had even international coordination so that we

don’t do duplication.

Mr. PAPPAS. So even within the agencies of the U.S. Government,

is there a similar body where there is a coordinating body that periodically meets or consults with one another to ensure that this

concern for duplication doesn’t take place?

Dr. FRIEDMAN. Let me try and address a partial answer to that,

and then I certainly would welcome other comments as well.

If you look at this in several segments, there is a mosaic of regulatory activities depending upon the responsibilities of each of the

relevant areas. The U.S. Department of Agriculture has a defined

set of responsibilities, and to the extent that they integrate that

with the Food and Drug Administration, then our concerns about

animals and ultimately their concern about people are intermeshed. And so you have to look at this as a mosaic not just at

the Federal level but, I stress, at the State and other levels as well.

We couldn’t function adequately without the scientific input from

CDC and from NIH and from academic centers and from private

scientists as well. And the reason for that is that at a time when

we have incomplete information to make the best regulatory decision, we can’t be paralyzed waiting for the most complete information to come about; that wouldn’t be appropriate. We must make

decisions, but we must make them in the most thoughtful and most

appropriate way, and that has to be driven by science.

And so, by the very needs of that, there’s a huge amount of communication and sharing of information both domestically and

abroad, because all these communities in some sense interact.

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Dr. DETWILER. Also, Mr. Pappas, the Agriculture Research Service has a committee, the BSE Research Advisory Group, where they

do coordinate such efforts in the United States, and it is not only

intergovernment—NIH, Paul Brown, and Joe have been invited, as

well as FDA—but we also have coordinated efforts with private

labs, either university labs or private, like the Basic Institute for

Research in Staten Island, University of Wisconsin, Stan Prusner’s

lab in California, Rocky Mountain Laboratory, etc.

They recently sponsored a meeting in Ames, IA, in June to again

have some papers presented as well as to meet after the meeting

and to discuss further research efforts.

Mr. PAPPAS. So is it safe to say this is my last question. Is it safe

to say that there is no agency that is, quote/unquote, the lead agency, or is that not correct?

Dr. FRIEDMAN. I think a more proper way to say it is that each

of the agencies has a field of responsibility for which they are primarily responsible but that none of the agencies acts alone.

Dr. SCHONBERGER. For example, when the problem of CJD after

receipt of human growth hormone occurred, the agencies met; and,

basically, CDC wrote a protocol—an epidemiologic protocol for followup of this group of patients. And we’ve published on that risk.

We’ve had 16 cases of CJD in a group of about 8,000 human

growth hormone recipients.

Now, NIH tests the lots for infectivity, and they’ve reported, in

the New England Journal of Medicine, some of the results that

they have had from that type of study. So it is a collaborative effort.

Our work with the American Red Cross on following known recipients of CJD donor blood was in part a result of discussions that

went on between FDA and CDC on the type of new information

that would be useful and helpful in this area.

Mr. GIBBS. May I comment?

Mr. SHAYS. Sure. And then I’ll call on Mr. Waxman.

Mr. GIBBS. Yes. In regard to this, I would like you to envision

what it means for scientists to get together to discuss, to bare their

knuckles about their work and their findings, and then to walk out

of that room, each knowing he is going to do his thing or she is

going to do her thing, but it is a coordinated effort.

In that regard, I would like to submit these for the record, Mr.

Chairman.

Mr. SHAYS. Sure.

Mr. GIBBS. The seven different international workshops on bovine spongiform encephalopathy. Out of that has grown most of the

research that has been conducted in this country and a fair amount

of what’s been conducted in the European Community.

So it is a sharing of information, with the work being done in the

collaborative fashion that there is no duplication; rather, there is

complementation.

Mr. PAPPAS. Thank you, Mr. Chairman.

Mr. SHAYS. Mr. Waxman, you have the floor.

Mr. WAXMAN. Thank you, Mr. Chairman.

I want to commend the four of you for your testimony. I think

you have done an excellent job not only in your presentation to us

but dealing with this problem that may or may not be a big one

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in this country but we have seen to be quite horrible in Great Britain.

And people say they don’t like government. But when there is a

problem like this one, we sure want government to be involved and

we want the research to be done, we want the regulatory tools to

be exercised, because we want the public to be protected.

As I understand, what we know about this disease, we know that

if cows eat parts from other cows, that there is a danger that they

may get what is called mad cow disease. And so, therefore, you

have acted to stop the importation of any feed or cows from any

other country where there might be a problem. Is that right?

Dr. FRIEDMAN. Correct.

Mr. WAXMAN. And the second area where there is a potential

would be if our cows would ingest some feed or some dietary supplement that had animal parts in it. And as I understand, what

FDA is proposing is to make sure that animal feed will not have

other animal parts in it.

Is that a fair statement, Dr. Friedman?

Dr. FRIEDMAN. Yes. The danger is that one cow in the United

States could spontaneously develop this disease, and if we render

that cow’s part in other cow feed, you would amplify the infection

in a silent way until it was very large. That apparently is what

happened in the United Kingdom.

By making sure that those ruminant sheep and cows don’t get

recycled into ruminant feed even if one cow in the United States

were to have spontaneously BSE, even if it occurred genetically by

accident, it would be a dead end; that cow would not be recycled

into other cows; and so the chance of and epidemic occurring is

vanishingly small at that point.

Mr. WAXMAN. So we seem to know if it is a cow eating cow parts,

there is a danger, and Dr. Gibbs told us about cannibals eating the

brains of other people, and that was a way of transmitting the disease from person to person.

Dr. FRIEDMAN. Yes.

Mr. WAXMAN. Do we feel that we know that people can get this

disease, the human version of it, by eating beef?

Mr. GIBBS. There is no direct—— Dr. FRIEDMAN. We’re all anxious to answer.

Mr. GIBBS. In specifically answering your question, there is no

definitive proof that a human being has become infected with any

of the diseases from any animal affected with those diseases.

Mr. WAXMAN. So we want to close off the areas we know are either a real danger or potential danger. You want to act reasonably

and prudently, and we want to know all the scientific information.

But people shouldn’t fear eating a hamburger; people shouldn’t fear

a danger in the blood supply; and people shouldn’t fear that if they

need a dietary supplement that has animal parts in it, that it is

diseased, from what we know at this point. There’s a theoretical

danger, but we don’t know of any great danger that people, if they

are hearing about this hearing, getting up in the middle of the

night and worrying about it?

Dr. FRIEDMAN. That’s correct.

Mr. WAXMAN. Now let me just followup by saying you don’t have

complete science and these things evolve. So if you found out there

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was a danger, we want to be sure that you have the tools to act

and maybe act quickly even if you don’t have all the information.

For example, you already are acting to stop animal parts in feed

that animals will ingest, but what if there are animal parts in a

product that humans would ingest? We have no reason to think

there is a danger right now.

But if you found out there was a danger, Dr. Friedman, since the

FDA has regulatory control over food supply which would include

dietary supplements, many of which have animal parts in them,

what legal authority do you have to act, and maybe quickly, without all the full knowledge about the issue, so that we won’t have

to wait until there is a horror story before action is taken?

Dr. FRIEDMAN. With your indulgence I’ll answer in three different ways, that question, if I may.

The first is that, as a matter of fact, it is not a theoretical set

of actions that we’ve taken, but there was a time, I believe in 1992,

when an individual was diagnosed, a human was diagnosed with

CJD. That individual was taking a dietary supplement, and we

went to investigate to see whether that dietary supplement, which

had animal parts in it, came from a country which had BSE or we

have reason to be concerned.

In fact, we are prepared. We have acted in that capacity and

would be ready to do so again in the future.

The second point that I would like to make is that in 1992 and

again in 1994, I believe, we contacted the manufacturer of the dietary supplement to alert them to potential concerns about this matter, granted that we don’t have all the scientific information, but

informing them that selecting products from countries known to be

free of BSE was the prudent and appropriate thing to do, keeping

records and carefully tracking where materials came from was the

appropriate thing to do. And we continue that dialog.

The third is, as you’ve pointed out, we do have some regulatory

powers in this regard, and where we are, were we to find material

being imported that had—was dangerous, we certainly would act to

do something about that.

Mr. WAXMAN. Let me stop you right there and ask you this question, because we’re going to be looking at FDA reform in this Congress, and if we’re going to reform FDA, we want to be sure we’re

reforming it to be sure that we have an FDA to protect the public.

If you have a danger from animal parts in animal feed, you’re

able to tell the manufacturer, from what I heard you say in your

testimony, ‘‘Stop using animal parts until you can show that it is

safe.’’

If it came to a human supplement, dietary supplement, and it

had animal parts in it, as I read the law, based on the act that

we’ve just adopted, you have the burden to show that it is unsafe,

that it shows a significant or unreasonable risk of illness or injury,

and it is not the manufacturer’s burden but it is yours.

You would have to then go in and be able to make this case before you can act?

Dr. FRIEDMAN. That is correct. And what we have asked, and the

verb here is important, the dietary supplement manufacturers to

do is to restrict their access to BSE-free countries.

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Our ability to demand that or to require that is not existent now.

And so this was your urging, this was—we importune them based

upon the quality of the science.

Mr. WAXMAN. But you can’t enforce it?

Dr. FRIEDMAN. We can’t demand that. I may not be picking the

word exactly right.

Mr. WAXMAN. Well, you can write them a letter saying, ‘‘Don’t

use imported animal parts, and keep track of the animal parts you

use so we can monitor it.’’ But if they don’t want to bother to do

it, there’s no way you can go in there and force them to do it.

Dr. FRIEDMAN. I believe that is correct. But not having the counsel here who is the most expert in that, I would defer to that individual.

Mr. WAXMAN. Let me just say that what I’m trying to do is, as

we deal with these laws elsewhere—— Dr. FRIEDMAN. Yes.

Mr. WAXMAN [continuing]. Make sure you have the ability to act

when it is appropriate and needed, and not have such a high

threshold before you can take any action that it may well be too

late by the time you do act.

And I think we maybe went too far in the law, saying that you

have to prove a significant or unnecessary risk before anything can

be done. It is a higher standard than what you have to meet to act

to stop animals from being exposed to animal parts.

Dr. FRIEDMAN. That’s absolutely correct, sir. If I may mention

one other thing, though, and that’s to reiterate the point that we

made earlier, which is that Great Britain has voluntarily and the

European Community has enforced that animal parts from Great

Britain will not be exported.

So that, that is the highest risk country, and we have two means,

not ever—not just at our own borders, where we have, USDA and

FDA have various prohibitions in place, but also at their own borders not to export it.

Mr. WAXMAN. Well, you’ve made a good point that we have to

keep in mind. You are acting appropriately given the kinds of dangers we know about. I think the American public should be proud

of the work that all of you are doing, and feel comfortable that this

is not a risky issue right now, and all the other risks are theoretical, and you’re on top of it.

What I want to explore with you in the time I have available is,

as we look at other committees that have legislative jurisdiction,

when you have not the complete information but enough to cause

you concern as information evolves, I just want to be sure that we

don’t weaken the FDA by making the laws so tough that you cannot act as conscientiously and appropriately as you all have seemed

to be doing in your respective agencies to date.

Dr. FRIEDMAN. Thank you.

Dr. DETWILER. I just wanted to respond that USDA’s prohibitions

would actually prohibit organs and tissues from ruminants to come

in, which then in turn would not allow them for dietary supplementation.

Mr. WAXMAN. Of course, the danger would be if it is local, if you

have some domestic animal that develops—— Dr. FRIEDMAN. Right.

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Dr. DETWILER. Right, but you mentioned about import.

Mr. SHAYS. I’d like to affirm what Mr. Waxman said in terms of

our sense of your contribution, both in your work and also now before this committee. We’re very pleased that all four of you agreed

to come.

And the purpose of this hearing was really to followup on the

hearing we had in May. We knew that FDA in particular and

USDA were focused on this issue, and we’re determined to come

out with some rulemaking. And we’re happy to hear what that is

and we’re happy to get a sense of its impact.

Dr. Detwiler, when you mentioned New Zealand and Australia,

I was surprised that you said that they had basically a tremendously good track record, given that they have such a large sheep

population; and I thought you maybe could explain to me why. I

was thinking in one sense that they might have a more difficult

time, given they have such a large population.

Dr. DETWILER. I think being island countries helps some whenever you’re talking disease risk. But again, scrapie, it is hard to assess in any of these diseases risk of freedom of a disease, because

when you do actual prevalence or incidents in a country, you

should be able to survey your whole population with some type of

test and ascertain which animals have the disease and which don’t.

Up to this time we can only really confirm the animals that show

clinical evidence of the disease, so you can’t do the systematic approach to those that might be infected with the agent. There seems

to be no evidence, and it’s based upon animals that they sell out

of the country, surveillance that they’ve done within the country,

and the fact that their quarantines have taken place on an island.

They have imported animals in that have subsequently come down

with scrapie, but they have been before they were introduced into

their national flock. This was back in the fifties.

Mr. SHAYS. And they’ve been ruminant-to-ruminant feeding?

Dr. DETWILER. They have been talking about proposing to do

that. I don’t know if that’s under way. I can find out for you.

Mr. SHAYS. OK. Is it possible that—I guess this is for you, Dr.

Schonberger. Is it possible that CJD is under-reported because it

gets disguised as other diseases, like Alzheimer’s in particular?

Dr. SCHONBERGER. Right. There actually have been some studies

of Alzheimer’s disease in looking for the frequency of CJD mixed

into the Alzheimer’s diagnosis, and it’s extremely low, actually, in

the Alzheimer disease category.

The answer to your question is yes, there is some under-reporting. As a matter of fact, in the active surveillance that we instituted in the emerging infection programs last May, April and May,

we were able to document about a 10 to 15 percent under-reporting

based on death certificates alone and by the active surveillance

areas, including, by the way, Connecticut, where we do have an

emerging infection program. They contacted, as part of this surveillance, all of the neurologists and tried to identify all the cases that

they could come up with.

And when you compare that with what you get through our national mortality data, you end up, as I say, with about 10 percent,

15 percent more.

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I should tell you, by the way, that our surveillance for the new

variant CJD, one of the characteristics of the new variant CJD is

that it affects an unusually young group.

So that, as Dr. Clarence Gibbs was talking about, the median age

of the new variant cases in the U.K., and we’re talking about now,

what is it, 14 cases there, is about 30 years old. OK, that means

about they’ve had five cases who have died under the age of 30. We

don’t have those cases here.

Mr. SHAYS. One of the things that’s fairly clear to me—and Dr.

Detwiler, you kind of set it off in terms of the fact that you take

tremendous satisfaction in the cooperation that exists within the

U.S. Government, and the private sector as well, but as well within

the international community—is part of that cooperation based on

the fact that there is a long incubator status, and when there is

an indication of TSE that real alarm bells go off because it’s potentially the tip of the iceberg?

I’d open that up to anyone, but you were the one that triggered

the cooperation. Maybe I should open it up to someone else, whoever would like to respond. Did you hear the question? I just want

to understand—— Dr. FRIEDMAN. I think the answer is yes. But what you do is you

recognize that it may be a while until you appreciate the full magnitude of an infection. And I think everyone is very chastened by

what happened in the United Kingdom and how badly out of control that situation was and how difficult it was to get it under control. And therefore I think all the scientists approach this with

some caution, and when they see an early case or an early indication, there is vigorous action.

Mr. GIBBS. I can only answer by stating that in the case of variant Creutzfeldt-Jakob disease in the United Kingdom, the minute

the surveillance group in the U.K. detected a case, we knew about

it on the telephone from them. It’s that rapid communication.

Mr. SHAYS. Thank you. These are proposed regulations, and the

bottom line regulation is, ruminant-to-ruminant feeding in the

United States is banned.

Dr. FRIEDMAN. Yes, sir.

Mr. SHAYS. When will these take effect? And you know what I’d

also like you to do, and fairly briefly, describe to me what happened after the May hearing and how that system worked to the

point where on January 3d, I think you came out with your proposed rule.

Dr. FRIEDMAN. Certainly. As you recall from our previous hearing, we had the advanced notice of a proposed rule, and the number of comments that we received to that was very large, something

in excess of 650; and some of these were quite lengthy and thoughtful commentaries.

We worked very hard with our colleagues and with the scientific

community to try and craft the best proposal or set of proposals

that we could, and in that regard we tried to balance several

things. One was practicality, looking at ease, at economy, at enforceability. And always underlying this was the scientific—the imperfect scientific basis upon which we were building this proposal.

That was completed—that effort was completed in late summer,

late August, and was sent forward for more full review by the deVerDate 11-MAY-2000 09:36 Jun 28, 2002 Jkt 078738 PO 00000 Frm 00100 Fmt 6633 Sfmt 6602 W:\DISC\39522 pfrm09 PsN: 39522
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partment and other parts of the government, to assure that we had

paid proper attention to economic issues and other regulatory concerns that are necessary, that are mandated for a rule of this magnitude. That time was longer than I would have liked in toto. Our

comment period ends, I believe, in the next couple of weeks.

Mr. SHAYS. Sometime in February.

Dr. FRIEDMAN. Yes, it is early to mid-February. We’re in the

process of reviewing comments that we’re receiving now. It is my

utmost hope, and it is the commitment that I’ve given you personally previously, that I intend to honor, which is that I want this

completed just as quickly as we possibly can.

I think there has been a value in engaging as many different

people in this effort up to this point. If this is going to be truly enforceable, then having a proposal which makes sense to the largest

number of people means that their participation will result in a

more wholehearted way than if they didn’t understand the background of this or if we didn’t pay attention to practicality and economic issues that were important to them.

So what we think is ultimately what we care about is the protection of these herds and therefore the protection of the American

public, and the chance of assuring that is greater by having this

more broader participation at this time.

Mr. SHAYS. So when this takes effect in February, then there is

no more appeal process? Would there potentially be an appeal process?

Dr. FRIEDMAN. I should really ask someone from the Center. I

don’t know whether there would be a further appeals process, sir.

Mr. SHAYS. Come on up, sir. Just identify yourself. You were

sworn in, correct?

Dr. MITCHELL. Yes.

Mr. SHAYS. You can pick up the mike if you’d like.

Dr. MITCHELL. It’s Dr. Mitchell. The comment period will close

on February 18th, and that is the comment period to the proposed

rule. We are receiving comments to that proposal now and there

will be more coming in. We will be considering those comments and

then publishing a final rule. And there will be another separate period announced in the final rule, on when the final rule would be

implemented.

Mr. SHAYS. Give me a sense of how long that would happen.

Dr. MITCHELL. In this rule we’re proposing 60 days.

Mr. SHAYS. And then it would take effect in 60 days?

Dr. MITCHELL. Yes.

Mr. SHAYS. And obviously there’s a potential, particularly those

involved in ruminant-to-ruminant feeding wanting to contest it in

court, and that then that could stay it?

Dr. MITCHELL. Yes. This being a major rule, there are our review

processes.

Mr. SHAYS. I have 5 more minutes of questioning. But I’d be

happy to have Mr. Waxman speak, if you’d like a couple more minutes, then I’ll begin.

Mr. WAXMAN. Mr. Chairman, I’m not going to take 5 minutes. I

just want to say to this group, you’re giving bureaucrats a good

name. I think you’ve done an excellent job and I’m proud of the

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work you’ve done in trying to protect the public from all the various aspects in which you’re responding to this disease.

Dr. FRIEDMAN. Thank you. That’s very nice of you. I appreciate

that comment.

Mr. SHAYS. Dr. Friedman, just two basic questions. I’d like this

for the record. The USDA has banned importation of beef products

and cattle from countries that have BSE since 1989. I’d be interested to know why the FDA hasn’t taken similar steps to ban the

importation of bovine ingredients from BSE-affected countries in

dietary supplements and cosmetics.

Dr. FRIEDMAN. Those products, there has been an import alert.

There have been some shipments which have been stopped. That

depends upon the quality of labeling of those products. But from

the early 1990’s we have had standard operating procedures in

place and we have had import alerts to ban bringing those products

in.

Mr. SHAYS. OK. One other question. Gelatin from BSE countries

for animal use has been banned from the United States by USDA

regulations, also 1989. And the FDA has no such ban for gelatin

for use in human food and drugs. Is that the same response?

Dr. FRIEDMAN. I think it’s a similar response. If I may, I will

elaborate on that a little bit.

Again, the largest BSE population, the country most at risk, is

the United Kingdom, and they have a prohibition on exporting

gelatin made from BSE-infected native cows.

They are, however, taking bones and hides from BSE-free countries, making that into gelatin and then exporting that into a variety of places, including the United States. So even though that’s

called British gelatin, it is not from British cattle and therefore

doesn’t bear those risks that you might associate, unlikely or theoretical as those risks might be.

The World Health Organization, a number of other organizations, including USDA in their 1991 rule, based upon all the scientific information we had available, determined that gelatin was

not a risky means of transmitting BSE, and so it’s been sort of a

world scientific opinion in that regard.

We are, however, for this product and for all products, vigilant.

And should new information, new scientific information emerge, we

want to take advantage of that.

Mr. SHAYS. OK. Thank you. And Dr. Gibbs, I’m concerned about

the fact that the labs that do TSE are slated to close in 1998. Am

I hearing proper information or not?

Mr. GIBBS. Perhaps I used the wrong terminology of closure.

There’s certainly a downsizing of our laboratory, but mainly because a number of scientists who were involved have left for other

positions around the country.

I have been assured by the director of our institute that we will

continue to be in business for several more years. We’re currently

being funded very handsomely, and NINDS is funding this field of

transmissible encephalopathies to the tune of almost $7 million per

year.

So our lab is not the only part that’s working on this. Much of

that would be in the extramural grant program. But it is my intention and it has been the assurance I’ve gotten from my director

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that we will be in business for several more years, but not on the

grandiose scale that we had been previously through the many

years when we were developing this whole field.

Mr. SHAYS. Let me just ask, if any of you had wished that we

asked a particular question that you wanted on the public record,

tell us what the question was and answer it. But I’m not looking

for a long response because we’re going to get to our next panel,

but if there’s anything that needs to be part of the record.

Mr. GIBBS. One thing, Mr. Chairman, in your opening remarks

you talked about diagnostic tests not being available. I will submit

for the record a paper that we just published in September on the

development of a diagnostic test for the spongiform encephalopathy

agent, particularly in humans but also in cattle and in sheep, using

spinal fluid as a mark—there is a marker in spinal fluid. And this

test is now being put in the hands of our technology transfer organization at NIH.

Mr. SHAYS. Do any of you wish to make a closing comment, or

we’ll get on with our next panel.

Dr. FRIEDMAN. May I only thank you and the committee members for the thoughtful and courteous way that you’ve conducted

this hearing.

Mr. SHAYS. You’re not surprised, are you?

Dr. FRIEDMAN. No, sir. Pleased but not surprised. We just hope

it continues.

Mr. SHAYS. Thank you. It was wonderful to have all of you here.

And we will get on with our next panel.

Mr. SHAYS. Our second panel is William Hueston, who is from

the Virginia-Maryland College of Veterinary Medicine, and Frank

Bastian, who is from the University of Southern Alabama School

of Medicine.

I ask both individuals to come, and we will swear you in.

We will have a 5-minute recess so people can move back and

forth.

[Recess.]

Mr. SHAYS. We have William Hueston and Frank Bastian. I will

swear you in.

[Witnesses sworn.]

Mr. SHAYS. On administering the oath, both witnesses before the

committee have responded in the affirmative. And Dr. Hueston, I

will call you first.

You all have prepared statements, if in the process of hearing the

comments made before you want to amend your statement or add

to it, feel free. We like the witnesses who follow, both of you were

here, to comment on what was said if you think that’s necessary

so we don’t even have to ask it. OK?

Dr. Hueston.

STATEMENTS OF WILL HUESTON, D.V.M., VIRGINIA-MARYLAND

COLLEGE OF VETERINARY MEDICINE; AND FRANK O.

BASTIAN, M.D., UNIVERSITY OF SOUTHERN ALABAMA,

SCHOOL OF MEDICINE

Mr. HUESTON. Thank you. My name is Will Hueston. I am here

as a veterinary epidemiologist, and my background, I have been involved in the study of bovine spongiform encephalopathy now for

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7 years, beginning as a public servant, an employee of the U.S. Department of Agriculture doing risk analysis work on the likelihood

of us seeing bovine spongiform encephalophy in the United States.

I have also spent 6 months assigned to the epidemiology unit in

1991 investigating BSE in Great Britain. I have served on advisory

committees for the International Office of Epizootics and World

Health Organization, and then most recently was appointed by the

British Government as a member of their Spongiform

Encephalopathy Committee and still serve in that capacity.

I appreciate your opening remarks. This is a most challenging

disease. It has been identified as being a common source epidemic,

a feed-borne, an animal feed-borne epidemic traced to the incorporation of ruminant-derived animal proteins. And it is also an area

where there is a tremendous amount of ongoing scientific debate so

that on a weekly basis there is new information arriving.

Mr. SHAYS. I am going to ask you to move the microphone closer

to you. You can move the ice pitcher if you want. Thank you.

Mr. HUESTON. Thank you. So here we have a new and emerging

disease on which there is new information weekly, and the challenge for the agencies involved, animal and public health agencies

and the affected industries and producers is how does one make rational policy in the face of this ongoing, changing scientific information. And I would like to propose to the committee that risk analysis is the tool for reaching those rational decisions.

Essentially, risk analysis involves identifying hazards, what

could go wrong; assessing the likelihood that those things may go

wrong, and the magnitude of the impacts should they go wrong;

evaluating or elucidating risk management options, what are the

opportunities that we have to reduce the likelihood of something

going wrong or to minimize the impact should it go wrong; and

last, risk communication. And risk communication involves incorporating all the potentially affected parties in the entire process of

considering the evidence, evaluating options, and assessing our

strategies.

The options for the control of bovine spongiform encephalopathy

focus first and foremost on animal feeding. The source and hazards

you’ve identified and explained quite nicely. Certainly, we have the

imports of animals and potential materials going into feed from

Great Britain and we have the indigenous sources.

We have the opportunity of controlling and the second step

through inactivation of these materials. Unfortunately, the information to date says this agent is very, very difficult to inactivate.

Last, we have the opportunity to look at how we use the material

or the finished product to avoid exposure to susceptible animals.

Now, having said this, there is multiple different options in

which one can put together these risk management strategies to

achieve the end goal of managing risk. The proposed rule that’s

being discussed today, the proposed final rule is scientifically

sound.

From my experience, it focuses on use and looks on use to finish

product, the sourcing. If the material has ruminant-derived protein

or mink-derived protein, then those materials are limited in their

potential use, and that use restriction goes from both the renderer

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to the blender to the feed manufacturer to the establishment and

individuals feeding cattle.

I think the flexibility that is built into the rule is laudable, this

flexibility that says and allows that as new information becomes

available, it provides the flexibility to respond to that new information in a very prompt and expeditious manner. I would like, for the

benefit of keeping this short and to the point, to share with you a

few of the observations that I bring from my involvement with the

British experience.

First, the science and the art of effective disease prevention and

control must be practical and implementable. Disease prevention

and control cannot occur by regulation alone, and there exists no

enforcement authority large enough or effective enough to enforce

regulations that people don’t want or understand the implications.

So the challenge here is to come up with a consensus among all of

the affected parties on the ideal, scientifically sound management

protocols and to move ahead to implement them.

Again, we, as human beings, operate under two mutually exclusive paradigms. One being an ounce of prevention is worth a pound

of cure, contrasted with if it ain’t broke, why fix it. And that’s part

of the situation we face here today. As speaker after speaker reiterated, we do not have bovine spongiform encephalopathy in the

United States.

My second lesson or experience is that we absolutely need practical and applied research, as well as basic research. So while one

group of scientists debates the characteristics of the etiologic agent,

my focus as an epidemiologist is given the information we have,

how can we control, manage, minimize the risks to animal and

human health. And that means we need research dollars focused

on issues like surveillance and inactivation and alternative uses of

this material that’s generated, this ruminant-derived protein.

There is a tremendous opportunity to collaborate with researchers

in other countries. I think this is a golden opportunity to let drop

any limitations on that investigation.

The third is that I want to reiterate a comment made by some

of the other speakers. I applaud the coordination and collaboration

that’s in evidence here between the animal and public health agencies. I think this is unique. This was not the initial characteristic

in Great Britain. There was not an active communication between

the human and animal health agencies. It led to a lot of misunderstanding, a great deal of mistrust, and I think potentiated the challenge that they are currently facing.

Finally, a sobering note. If, in the end, our prevention is successful, it is effective and we never see BSE in the United States, then

all of the preventive measures that have been put in place will be

criticized as unnecessary. If, on the other hand, we see a case of

BSE in the United States, then obviously the prevention, it will be

too late to prevent its occurrence, and the same individuals will be

criticized, the same agencies will be criticized for not taking appropriate actions. And we will join Great Britain, France, Switzerland,

Ireland, and Portugal in trying to rebuild our national image and

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trying to recapture the trust through verification with our trading

partners, and last, in trying to reestablish our reputation as a

world leader in providing an abundant, high quality and affordable

safe food supply. Thank you.

[The prepared statement of Mr. Hueston follows:]

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Mr. SHAYS. Thank you, Doctor, for your observations.

Dr. Bastian.

Dr. BASTIAN. I appreciate the opportunity to participate in this

hearing of the Committee on Government Reform and Oversight

regarding Federal agencies’ response to the potential threat of

transmissible spongiform encephalopathy. I have been working for

over 20 years in this field.

I am a professor of pathology, M.D., and practice neuropathology

at the University of South Alabama where I have served as a consultant for the diagnosis of Creutzfeldt-Jakob disease from tissues

submitted to me from other institutions all over the United States.

I have been involved in research on the transmission of

spongiform encephalopathy regarding the nature of the transmissible agent. In 1984, I was visiting professor in the laboratory

of Tony Palmer at the University of Cambridge in England for the

purpose of studying scrapie. At that time I visited with Drs.

Dickenson, Somerville and Fraser at the Neuropathogenesis Unit

in Edinburgh where I presented my research data and reviewed

their experience with scrapie mouse models, and I presented lectures at seven institutions during my visit to the U.K.

In 1991, I published a book entitled, ‘‘Creutzfeldt-Jakob Disease

and Other Transmissible Spongiform Encephalopathies.’’ In 1992, I

held a symposium on bovine spongiform encephalopathy or mad

cow disease at the American Society of Microbiology general meeting in New Orleans. In May 1996, I presented at the Duma Foundation of Infectious Disease Symposium on Emerging Infections

held at the National Press Club in Washington, DC.

Subsequently, I was invited to present my findings at the USDA

advisory committee meeting in Ames, IA, in June, and in December

1996, I was an invited speaker for discussion of the state-of-the-art

of the science at the CERES international symposium on the transmissible spongiform encephalopathies.

Now, my assignment today is to deal with the effectiveness of the

agencies in their handling of research funding and control measures relating to the transmissible spongiform encephalopathies,

which I will refer to as the TSEs. The TSEs include scrapie in

sheep and goats, transmissible mink encephalopathy, bovine

spongiform encephalopathy, and Creutzfeldt-Jakob disease in humans, otherwise referred to as CJD.

I will begin by pointing out that the agencies have been stymied

by the fact that, one, the identity of the transmissible agent of the

TSE is not known; two, there is no preclinical test for TSE agent;

three, the epidemiology of TSE is not known; and four, the susceptibility to TSE is not known.

There are a limited number of theories regarding the nature of

the transmissible agent. First, the prion or replicating protein theory which suggests that abnormal folding of the host protein is the

cause, is not consistent with basic concepts in biology wherein DNA

or RNA is required for replication.

At a recent international symposium, researchers presented evidence that the folding of the protein as proposed by Dr. Prusner

does not occur. The numerous strains evident in TSE are more consistent with an agent possessing it’s own genome. The recent paper

in science is significant in that the authors found that the prion is

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not necessary for infection and instead is a product of the infection

rather than being the causal agent.

The concept I propose is that there is a wall-less bacteria involved in the pathogenesis of TSE. In 1979, I reported spiroplasmalike occlusions from the brain biopsy of a patient with CreutzfeldtJakob disease. Spiroplasmas are present in the hemolymph of most

insects and several strains are known to experimentally induce

spongiform encephalopathy in rodents.

We have demonstrated that spiroplasma proteins cross-react

with TSE antibodies. In fact, the unique fibril proteins within

spiroplasma are identical morphologically to fibrilproteins consistently seen in TSE tissue preparations and not in controls. Recently,

we have documented the presence of a molecute gene in

Creutzfeldt-Jakob brain tissues with a 97 percent homology to

spiroplasma. The spiroplasma concept fits the epidemiology chain

as evidenced for TSE and as no other theory does. This concept

should be further investigated.

The emphasis placed totally on the prion theory by the scientific

community over the past 15 years to the point of exclusion of all

other theories has frustrated any realistic attempt to develop a preclinical test for TSE, the lack of which has resulted in incomplete

knowledge of the epidemiology of TSE.

CJD has a worldwide occurrence with one to two cases seen per

year in a town the size of Mobile. Only 250 CJD cases occur each

year in the United States. I suspect that the incidence of the disease is much higher. Furthermore, research efforts have been concentrated on molecular biology studies without regard to our basic

lack of understanding of the pathogenic mechanisms involved in

TSE. The agencies have fallen short in the handling of these matters.

In an effort to search for the agent, they have placed almost all

of their funding in one basket. I’ve heard that at least $75 million

has been given to one research laboratory in the past 15 years. My

opinion is that this has not been money well spent since we appear

no closer to resolving the identity of a TSE agent from that effort.

This lack of progress has impaired efforts to develop a preclinical

test nucleic which is necessary to have a lead on either an agentspecific acid or a protein. The prion is now realized to be simply

a reaction product of the infection. In regard to epidemiological

studies that have settled on using death certificates, which are totally unreliable, the clinical diagnosis is wrong in at least 25 percent of cases. We have no idea of the extent of the disease in this

country, much less the distribution of the agent. I have pushed for

making CJD reportable, but the agencies are only interested in crises, particularly whether the new variant of CJD has arrived in

this country. I disagree with that approach.

The revelation of possible contamination of blood products by

CJD-infected professional blood donors has been handled by the

agencies by massive withdrawals of blood products. I question the

wisdom of this Band-Aid treatment alone. Since we are still 10

years away from recombinant DNA production of blood clotting factors, the current methodology of filtration of blood products is likely

inadequate to protect us from contamination and we are waiting

for the ax to fall again.

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Recommendations. I suggest that we do not try to blame the

prior handling of the TSE problem by the agencies, especially since

we are now enlightened by evidence indicating that the dogma is

wrong. Let’s move forward.

I would like to make the following recommendations: One, in regard to funding of research efforts, we should pursue all clues

available regarding the nature of the agent. The money should not

all be given to one or two laboratories, but should be spread out

to provide for some fresh approaches. The primary aim of the research should be to develop a preclinical test.

In addition, there should be funds for studying basic pathogenic

mechanisms in an animal model. I believe the immune system is

very important in the pathogenesis of TSE and should be investigated. Levels of funding must be increased to encourage other researchers to enter the field. The problem will be more likely to be

solved if we encourage participation by scientists from multiple disciplines. The rarity of the disease has hampered getting the attention of many scientists in the past, since most Ph.D.’s must search

out funding with a reasonable probability of getting it.

My second recommendation, in regard to epidemiological studies,

we must avoid the crisis management approach previously used by

the agencies, and instead try to get a handle on the prevalence of

CJD. I believe that CJD and the other TSEs should be reportable.

Identification of the patients early on in their illness would provide

researchers the opportunity to apply new diagnostic tests or therapeutic measures. The other approach would be to develop a clinical

center for CJD patients thereby concentrating clinical data on a

rare disease.

In conclusion, my request is that you pursue some new directives

with haste, since there is at least a dangerous theoretical threat of

TSE from our blood supply and food, particularly beef products.

Funding is necessary to search out the transmissible agent, which

could lead to development of a much needed preclinical test, even

an immunization program. New research avenues should be pursued in light of recent scientific revelations.

I thank you for your interest.

[The prepared statement of Dr. Bastian follows:]

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Mr. SHAYS. Thank you.

Congressman Pappas.

Mr. PAPPAS. Thank you, Mr. Chairman. Gentlemen, thank you

for being here and participating. You may have been here in the

room during the questioning of the first panel, and one of the lines

of questioning that I participated in was in the area of coordination

of the research that is ongoing by various Federal agencies and

maybe some others.

I am wondering if you both could comment about that. Dr.

Hueston, you mentioned that you feel that there is a high degree

of coordination and collaboration, and Dr. Bastian, I don’t recall

seeing what your comment was with regard to that, and if you

think that there is a need for a lead agency.

Dr. BASTIAN. I believe there is no question that there is need for

a lead agency, and personally, I believe that the NIH should take

the ball on this, in taking on the research. Unfortunately, the problem has been that the research has been going down a road of no

return, in a sense, and we have to have the research directed in

a more unbiased fashion.

Mr. PAPPAS. If I can interrupt, why do you think the NIH should

be the lead agency versus another? I am not saying I disagree, I

just——

Dr. BASTIAN. Let me say that in my experience so far, probably

the most reasonable approach has been so far from the USDA. The

USDA, and it seems like I am confusing the issue here, but the

USDA has essentially tried to put the science on the table, and

through a series of symposia, they, indeed, with inviting both sides

of the coin, they have tried to do that. However, the USDA right

now is not very well-funded.

The NIH, I believe, needs new direction. I think they have to

turn 180 degrees to deal with the problem. But they have the

money. My personal feeling is that there has to be a move to take

on certain aspects also of the epidemiology. I believe the CDC has

not really been interested in CJD until March 20, and as a result,

their efforts are, I don’t believe, are going in the right direction either. So what you need is a single agency to assume responsibility

for the epidemiology, the research, and then this would indeed aid

the other agencies in dealing with the problem.

For example, the FDA, they don’t have a preclinical test. It’s impossible to really be able to make wise decisions without a preclinical test. You’ve got to do the science. My point is that although

we can set up all these regulations, we have to get on the ball and

go after the science, and that’s why I suggested the NIH because

that’s been their prerogative up until now.

Mr. PAPPAS. Thank you, Dr. Bastian.

Dr. Hueston, would you answer the same question?

Mr. HUESTON. Yes, sir, the first question being should there be

coordination, correct?

Mr. PAPPAS. Yes, and should there be a lead agency.

Mr. HUESTON. I would argue, no, there shouldn’t be a lead agency. My experience in the United Kingdom in watching what happened over there is when they attempted to identify a lead agency,

that lead agency takes the direction of their major focus. So if you

identify a human health agency, they focus on the human health

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issues. You identify an animal health agency, they focus on the animal health issues and that is their expertise.

What we have is an excellent opportunity to take advantage of

the relative merits and the expertise of different agencies as they

apply to a complex problem such as this.

Mr. PAPPAS. And my second question for you both is how effectively do you gentlemen believe that this proposed rule would protect livestock and the citizens of our country for protection from

TSEs. I am having to get used to another group of acronyms. When

I was involved in county government, human services, they had oodles of acronyms and I used to carry a little card in my pocket. I

guess I am going to have to get another card. Go ahead.

Mr. HUESTON. I think it will depend entirely on the degree of

compliance, the degree to which they are accepted and implemented, and that brings me back to the proposed rule needs to be

understood, needs to be accepted, and the ideal situation would be

it comes out of a consensus of all of the effected parties, from the

consumer to the producer to the industries associated and the government agencies.

Dr. BASTIAN. I really, can’t really deal with that question because

I, I would say that the problem in England really resulted from an

adaptation or a mutation of the agent from cattle feeding cattle,

and surely it does increase the virulence of the breaking of the species barrier and then into humans. But as far as the value or the

efficiency of handling the situation in this country by putting these

measures in without pursuing the science, I just can’t answer that.

Mr. PAPPAS. Thank you, Mr. Chairman.

Mr. SHAYS. Thank you, gentlemen. I am getting a sense of some

differences between the two of you. Where would you define the

biggest disagreement that you would have, Dr. Hueston, with what

Dr. Bastian has said and vice versa.

Mr. HUESTON. I believe what you are seeing is the beauty of

science in action, that each of us is taking this from a different perspective.

Mr. SHAYS. Right, but tell me the perspective and why you disagree.

Mr. HUESTON. Dr. Bastian has been addressing the basic issues

and basic science questions of the nature of the agent and the development of the disease. I am at the other end of that spectrum

in that my field is very applied. Given the information that we

have now, what are the realistic measures we can put into place

to control the disease. So I come from the standpoint of saying

there is an awful lot of science available. There are a number of

units around the world working on this disease and more science

becomes available on a weekly basis.

So our challenge is to put that information together with some

mechanisms and tools to try to come up with an effective and justifiable approach to control the disease. Now, having said that, I

think there is a big difference here, that, in other words, I think

we can control the disease without a preclinical test.

Mr. SHAYS. Without what?

Mr. HUESTON. Without a preclinical test. A preclinical test would

be ideal, but we cannot postpone taking public health and animal

health measures until we have a preclinical test. Similarly, it

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would be ideal to be able to completely characterize the agent responsible for these diseases. However, we know sufficient information about the agent, I believe, to put into place effective management strategies.

Mr. SHAYS. Dr. Bastian, how would you define the differences?

Even if you agree, I would like you to put it in your own words.

Dr. BASTIAN. Well, I think it’s a mistake to consider that this is

an English problem.

Mr. SHAYS. This is a what problem?

Dr. BASTIAN. I said I think it’s a mistake for the agencies or the

industry to consider that this is an English problem or a UK problem.

Mr. SHAYS. Right.

Dr. BASTIAN. Because a virulent form of the agent has been unleashed. It clearly—there are patients dying in England from this

virulent form of the Creutzfeldt agent. I know Dr. Gibbs tried to

say there was no hookup between the agent, the TSE agent and

the clinical cases over there. I think that’s clearly wrong.

There are two very important papers that show that the pathology in the Macaque monkeys is identical following BSE inoculation

to that as seen in the new clinical cases of CJD. And two, Collinge,

who studied the changes in the PrP associated with the different

infections in England, has clearly shown a signature of the BSE

agent that’s present in the new cases of the CJD cases.

Mr. SHAYS. I have to tell you, and it’s not your fault, but you are

losing me a little bit here. I would like to, in more simple terms,

just understand the differences between the two of you, and then

you want to elaborate. But the purpose is just to help me get a

framework for pursuing some other questions.

Dr. BASTIAN. Basically, I agree with Dr. Hueston that some

measures have to be taken, and I have no problem with that. But

I—my only point is that we have to not—we have to pursue and

try to get this preclinical test, or look at an experimental model of

the disease in detail to look at the basics of that. If we just put

all our efforts into control measures, we may be missing the boat

because we just don’t have enough information.

Mr. SHAYS. OK. What I think I hear you saying is you need to

see a little more proof before you see action taken.

Dr. BASTIAN. No, I am not disagreeing with Dr. Hueston. I realize measures have to be taken in light of what data is available.

I don’t disagree with that, and I don’t disagree with his view of taking these measures at this time. Not at all. My point is that this

should not be the only thing we do.

Mr. SHAYS. OK. Well, I think we probably all agree.

Let me just understand. Your basic view is that, and I will quote

you in conclusion, ‘‘I do not favor a ban on ruminant-to-ruminant

feeding since the practice of feeding cattle their own kind in England—’’

Dr. BASTIAN. Oh, I do favor.

Mr. SHAYS. You do favor.

Dr. BASTIAN. Yes.

Mr. SHAYS. You do favor a ban on ruminant-to-ruminant feeding,

in spite of the fact that we don’t have a definitive sense.

Dr. BASTIAN. Correct.

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Mr. SHAYS. Why would you favor that?

Dr. BASTIAN. Again, I believe we have to act. We have to set up

some regulations. My point is in regard to getting more definitive

tests so that we can—in a sense, for example, in a sense possibly

have enough data down the road to be able to remove such bans.

Mr. SHAYS. It’s conceivable that this whole effort, banning ruminant-to-ruminant feeding, is not the problem. You are shaking your

head.

Dr. BASTIAN. I don’t see that—I am sorry.

Mr. SHAYS. No, I was looking at Dr. Hueston. You were shaking

your head so I would like to translate that.

Mr. HUESTON. Right, I believe there is overwhelming evidence to

suggest that it was the recycling of feeding of ruminant-derived

protein that led to the epidemic in the animals—— Mr. SHAYS. And that involves the prions.

Mr. HUESTON. Well, in my hypothesis, it could be the prions, it

could be another agent.

Mr. SHAYS. OK. The feeding process of ruminant-to-ruminant,

there is consensus, then the question is what is the cause with that

process of feeding—the prion bacteria, correct?

Mr. HUESTON. Right, the discussion of what is the agent, the actual ideological agent within that material.

Mr. SHAYS. So there is agreement on the process of transfer. We

just don’t know what the agent is.

Mr. HUESTON. Correct.

Dr. BASTIAN. We’ve essentially, by this mechanism, created a

pattern of serial passage, and serial passage where you would inoculate a bacterium into an animal and take it from one animal to

another, you can clearly increase the virulence of the organism,

and I believe that’s exactly what’s happened in the English experience.

Mr. SHAYS. It’s, in sense, a compounding.

Dr. BASTIAN. It’s a classic experiment with bacteria. You can increase the virulence of the organism by simply serial passage in an

animal model.

Mr. SHAYS. Dr. Hueston, what if Dr. Bastian is right?

Mr. HUESTON. What if he is right in terms of the spiroplasma?

Mr. SHAYS. Yes, when will it start to matter?

Mr. HUESTON. If we look at this recycling or process of incorporating animal-derived proteins into animal feeds, it involves a

process called rendering and that involves heating and treatment

of the material, and that heating and treatment of the material destroyed, we felt up until 10 years ago, destroyed all of the potential

agents that might cause disease and it was an ecologically sound

method of recycling a waste product, if you will, into a usable form.

Now, it even—in fact, the processes that are being discussed and

the processes that could be provided can inactivate some of the

agents like the one Dr. Bastian is discussing. I think as we first

take the control measures that are prudent, upon which we can get

a large degree of compliance, and then as more information becomes available, we modify, adjust, update those recommendations

to take advantage of the new information.

Mr. SHAYS. One of the things that was clear to me when Dr.

Gibbs spoke, and you as well, Dr. Bastian, both of you have been

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in this field a long period of time and you are expert witnesses. I

wasn’t sure we needed you to do that except for the fact that I get

the sense that I could probably count on my hands or hands and

toes the number of people who are in this field in the United

States. Is this a really small group?

Dr. BASTIAN. That is the problem. And one major problem has

been that you have not been able to attract what I consider the

true scientists, the Ph.D.’s that are slaving in the university settings. There is no money available, and so you’ve got to be able to

attract these people.

Mr. SHAYS. This is not meant to be a digression, but in these

hearings we have on the Gulf war illnesses syndrome, you know,

many potential causes and many effects from those causes, we have

found that there seem to be very few people who have gotten into

the whole issue of detecting chemical exposure and knowing how

to treat it, and we’re being told that—and it’s been really a surprise to me that there aren’t more in the FDA, or excuse me, the

VA or DOD or Pentagon who have this expertise. And I have to believe in the market process, but sometimes there becomes a disincentive to get in these fields, and I particularly feel in terms of

chemical exposure and detection and treatment, that we need

many more people in that field.

So what would guide us, then, because you obviously, Dr.

Bastian, are sensitive to the fact that institutes of health are one

primary way of responding to the lack of market focus, and so I

sense from you you are a little unhappy with the institutes of

health and how they have allocated funds in this area.

Dr. BASTIAN. The problem has been that all of the research has

gone in one direction, that’s correct, and basically the prion theory,

for example, it just expands to incorporate new data. It doesn’t

matter how much—I mentioned putting more money into the field,

it doesn’t matter how much more money you put into the field if

the research is not going in the right direction. My point being that

we’ve got to consider all the clues out there.

There is one fascinating study that was published in May wherein a group in New York inoculated hay mites into mice and produced scrapie. Now, these—this was based on the fact that in England at a very early time that fields that had scrapie-infected animals, if they removed the animals and put fresh animals in, the

new animals came down with the disease.

So they took the hay mites in those fields and inoculated them

and produced the disease. So there was something in the hay mites

that produced scrapie. They then took the mite preparation, did

immunological cross-reactivity studies with the scrapie antibodies

and indeed showed cross-reactivity. So there was something reacting in the hay mites to the scrapie antibody. However, they have

not been able to find the PrP gene, and I suspect that there is a

spiroplasma in those hay mites.

Mr. SHAYS. The prion.

Dr. BASTIAN. Yes, the prion, they have not been able to find the

prion gene. So what they are likely showing by immunological

cross-reactivity is antibodies developed to the scrapie from—how

you prepare the antibodies in scrapie is take the scrapie material,

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inoculate into rabbits, and the rabbit produces antibodies to these

proteins that are inoculated.

Now, what I suspect is that the—what’s been produced in those,

the scrapie antibodies, is an antibody to the agent, which I believe

is the spiroplasma, and I am sure that’s what’s reacting in those

hay mites is likely to be a spiroplasma. What’s fascinating is I was

told by personal communication that hay mites that are not infectious, that is those that do not produce the disease, also show

immunological cross-reactivity with the scrapie proteins, and in

that sense—— Mr. SHAYS. So what’s the bottom line to your point though?

Dr. BASTIAN. My point is that this is further evidence, the prion

not being the answer.

Mr. SHAYS. OK.

Dr. BASTIAN. And if you are going to put money into, if you are

going to solve this problem, you are going to have to check all possibilities.

Mr. HUESTON. Congressman Shays, may I try to put some of this

research into perspective?

Mr. SHAYS. I am going to conclude fairly soon. I am going to invite Dr. Gibbs and Dr. Detwiler to come back afterwards to make

a short comment or observation, if you like. You don’t have to, but

my philosophy is if people are willing to stay through the hearing

and hear other comments, would like further input. So we appreciate that both of you stayed.

Dr. Hueston.

Mr. HUESTON. Thank you. Prior to the identification of bovine

spongiform encephalopathy, the majority of the published research

about these transmissible spongiform encephalopathies actually

originated in the United States, and certainly that was readily the

case with the human forms of the disease and much of it came out

of the NIH lab. We have currently in the United States a number

of groups evaluating the human spongiform encephalopathies, looking at animal diseases such as chronic wasting disease, looking at

the scrapie and groups that study transmissible mink

encephalopathy. So there are groups and there is activity continuing in the United States.

I think that Dr. Bastian is making a very important point to say

that we should always maintain a healthy skepticism to make sure

that we aren’t tracking down the wrong path, and that there is a

need to encourage other approaches and other examinations of this

issue. As it might relate, just for your information——

Mr. SHAYS. I think he was saying a little more. I think he was

saying there is research that would suggest that, so I think he was

saying more than healthful.

Dr. BASTIAN. Right.

Mr. HUESTON. Recall there are people around the world who continue to put forth a whole range of theories for the origin, for the

etiological agent associated with these.

Mr. SHAYS. Right, but one of the questions would be, and I am

sorry to interrupt, is: are we putting too much in one area, or are

we putting enough there, but should we put more in another area?

That’s one of the points I am hearing and you probably wouldn’t

disagree with that.

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Mr. HUESTON. I wouldn’t disagree.

Mr. SHAYS. I am sorry. Continue.

Mr. HUESTON. I just want to make one other point. Essentially

all, the vast majority or essentially all the work right now on bovine spongiform encephalopathy is happening overseas. And we are

quite comfortable, in fact, encouraging that that be the case because we don’t want the agent coming into the United States even

for experimental work in laboratories. So that’s another reinforcement of why the collaboration is extremely important, so that we

can work with our collaborators in other countries where they experience disease to preclude as one more further protection from

that agent coming into the United States.

Mr. SHAYS. Is there any question you wish I had asked you or

the committee had asked you, any point you want to put on the

record? If there is, I would be happy for you to put that question

on the record yourselves.

Mr. HUESTON. I will address one. I think it is very easy—increasingly, I’ve been studying the animal health policy. How would you

implement policy and what is the most effective means for controlling and preventing disease with policies, and there is some interesting recognitions. One is the question of reportable diseases. One

thing that Dr. Bastian suggested or put forth is his opinion that

Creutzfeldt-Jakob should become a reportable disease.

From my experience with animals’ diseases and watching the

British situation, from the moment one makes a disease reportable,

the actual reporting of the disease decreases. So we have an interesting human phenomenon going on here. As an example, in Great

Britain, when they were recently mandated by the European Community to make scrapie reportable, the reported cases of scrapie in

sheep in Great Britain dropped over half. I do not believe that that

is because of the miraculous beneficial effects of making a disease

reportable.

Mr. SHAYS. I could make my observation that they wanted reportable because they were going to take some fairly drastic action,

and Dr. Bastian, I don’t mean to put words in your mouth, but I

wonder if that analogy would be appropriate. In other words, that

if we did it, it would have that same effect.

Mr. HUESTON. Well, in the discussions to make Creutzfeldt-Jakob

reportable in the United Kingdom, it is the consensus of the public

health authorities that making the disease reportable would reduce

the reporting and the likelihood of followup on the cases.

Mr. SHAYS. If that is true then we should not have any reportable diseases.

Mr. HUESTON. I think it depends a lot on the specific diseases.

May I take you for a second, having talked to families, I don’t know

if you’ve had the opportunity to work with families as Dr. Bastian

and I have visited with families that have cases of CJD. This is a

very, relatively rapid onset, it’s a degenerative disease that’s ultimately fatal. There are a lot of questions the families have that

can’t be answered and it leads to a tremendous amount of emotion,

grief, and concern, and families are understandably extremely apprehensive about being identified with a—— Mr. SHAYS. One second here. We’re getting an echo. I think it’s

one of the mikes. Take your time.

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Thank you for doing that.

Mr. HUESTON. So the challenge is that if the disease is made reportable and my concern was, as you can imagine, there is a great

deal of attention on the families where this disease is reported. The

families lose a lot of their privacy, so the feeling is if the disease

was made reportable, they would have less chance to trace back to

the families and ask the important questions that we need to further understand the disease.

Dr. BASTIAN. As a physician, I disagree with Dr. Hueston on

that, because the families are desperate for information. The families are willing to participate in any sort of effort. In fact, I’ve received several calls this past week from families that asked what

can we do, how can we help resolve some of the information regarding this. And my—a major problem in this whole field is the fear

that’s been placed amongst the medical profession about this. People are afraid to handle the patient.

I received a call from a physician, a neurologist in Florida, and

he said I have a patient I believe has Creutzfeldt-Jakob disease.

The hospital will not admit the patient. If I got the patient admitted, the neurosurgeon would not biopsy it, and the pathologist will

never autopsy the case. How on earth are you going to make a diagnosis?

Now, this clinical test that Dr. Gibbs put forth, my personal experience with that, it was based on a, on the finding of abnormal

proteins in the spinal fluid. It’s got nothing to do with PrP, but

there are abnormal proteins occurring in the spinal fluid in a significant number of these patients. But it’s also seen in other diseases, like herpes encephalitis and recent stroke.

So in the right clinical setting, the test is maybe useful, but in

a personal experience, I received a brain biopsy from a patient submitted to me from Tampa, FL, by the neuropathologist, and I

looked the biopsy and saw spongiform encephalopathy, CreutzfeldtJakob disease. And he said well, we sent some—the spinal fluid

test is negative. I said I don’t care, this is Creutzfeldt-Jake disease.

The patient died about a year later and I received the brain for

examination and clearly had Creutzfeldt-Jakob disease. But the

neuropathologist said, you know, we sent four specimens of CSF

over this time period, for examination of CSF for this unusual protein, and we finally got a positive. This test could be very important, except that from the recent data, it appears not to be positive

until the disease has occurred or is about to appear. And my point

is that before we offer this as a solution, let’s test this in an adequate model system, and right now there has not been a good

model system for this disease.

For example, with the poor FDA people dealing with the blood

products, they don’t—one, we don’t know if the blood is infectious.

Two, we don’t know if it is infectious, we don’t know at what phase

of the disease it is infectious. We have no basic information. The

point is in making all these decisions and control measures, which

I think have to be done, I am not saying don’t do it.

Mr. SHAYS. I understand.

Dr. BASTIAN. But you’ve got to go ahead and try to get some of

the basic information to try to make a common sense decision on

some of this. And we don’t have that as yet.

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Mr. SHAYS. OK. I think I am fairly clear and the committee is

fairly clear on that. Is there any other comment you want to make?

Dr. Gibbs, I’d be happy to have you come up and make a comment if you like. We’re not going to resolve all the world’s problems

today, but we’re just trying to get a focus for the committee.

Dr. GIBBS. Thank God we don’t have to solve the world’s problems. First of all, let me just say if there is anything we have

learned from the outbreak of BSE in the United Kingdom, we

should have learned it very strongly, and that is stop feeding ruminant to ruminant. I think the evidence is clear in that regard.

Mr. SHAYS. And let me just say, I am going to interrupt you to

say when we had our previous hearing, there was consensus among

a large number who testified except those who were involved in the

feeding process themselves, who wanted very much for the FDA to

take that action and we asked each one specifically. So there was

consensus at our hearing certainly that the FDA do exactly what

they have done.

Mr. GIBBS. The second point I would like to make was with regard to some of Dr. Bastian’s comments, and that is you may have

missed it in my testimony. Certainly, I will submit it to the written

testimony, and that is my laboratory is not the only laboratory at

the NIH working on these diseases. You have the Rocky Mountain

Laboratory in Hamilton, MT, part of the Allergy and Infectious

Disease Institute working on it.

The most important thing to remember is my budget is an intramural budget, far below the many millions of dollars that are given

in extramural programs by grants to academia and so forth. Our

grant program undergoes peer review and is rated on peer review,

not by NIH personnel, but by people from academia, and if you receive a high enough score on your proposal, you are approved. If

your score is really good, you are funded.

Mr. SHAYS. Let me ask you, isn’t there always the potential, obviously there is always potential, but more than potential here, particularly with orphan diseases, which this is, in fact, an orphan disease, correct, in the sense that there is not many have it, and

therefore the private factor is not going to be out there funding out

of market reasons. Isn’t there always the concern that you just

don’t have enough of your like-minded people on those peer reviews

to consider it, you know, your application?

Mr. GIBBS. That’s a possibility, but in this regard, I think this

field is what I put on the frontier of medicine, and is so important,

that I don’t think you would find that problem. I think if it is good

research, it’s going to be funded. And I don’t think there is a feeling of competitiveness in the sense of, well, we won’t fund this because he’s in this institution.

Mr. SHAYS. I don’t think it’s as obvious, but one of the things this

committee may do, is in fact,—we may not do it, but we may look

at the whole issue of how studies are done, research is done, and

who decides. Because we hear a number of people complain. Obviously, they tend to be people who didn’t get necessarily their

project funded and so on, and then there are people who seem to

be automatically in the system repeated without even having to

make applications, and it continues, and you just wonder if they

are no longer there if that project wouldn’t stop and then go.

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Mr. GIBBS. Well, certainly there is a point to be considered here,

and that is as you pointed out earlier, there are relatively few laboratories in the United States working on these diseases, that’s No.

1. And No. 2, those that are working on this disease, by and large,

form a community, and as I see it, a fair number of those, outside

of government, are well supported by not only NIH grants, but by

USDA grants, FDA grants, and by the private sector, foundations.

Mr. SHAYS. Dr. Bastian—thank you, Dr. Gibbs, I appreciate it.

Mr. GIBBS. I had one other thing, please. In regard to Dr.

Bastian’s comments about the tests that I submitted here, and that

is you will see that it has a 99 percent sensitivity and a 99 percent

specificity. Now, there is no problem clinically diagnosing herpes

encephalopathy from Creutzfeldt-Jakob disease. But we recognize

the test does pick up herpes encephalopathy.

Mr. SHAYS. Right.

Mr. GIBBS. But clinically you can separate those two, and our

test is also beneficial in testing the spinal fluid of cattle experimentally infected with scrapie and mink encephalopathy and sheep

with naturally occurring scrapie.

Mr. SHAYS. Dr. Hueston, any other comment you would like to

make?

Mr. HUESTON. No, sir, thank you.

Mr. SHAYS. Thank you.

Dr. BASTIAN. I think regarding the test, the question is is the

test positive in a certain period of the disease? It may be extremely

important, but we just don’t know, from my experience, my personal experience, we just don’t know how this will fit into the picture. And so in an animal model, you could test that.

Mr. GIBBS. Those studies are underway right now.

Mr. SHAYS. Let me just say to you, the last thing I want to do

is get into the specifics of a particular study, but Dr. Gibbs, I think

what I am hearing Dr. Bastian say is that, you know, we’re going

down one trail and he would argue, it seems to me, that we’re

going, you know, with a lot more energy down that trail and we

also should be going down this other trail. And I think I am hearing him say that we’re not doing that to the extent we should. And,

you know, that’s a judgment call. I mean, he’s telling the committee that’s his opinion, and it’s something we would—what I am

saying is I don’t care to resolve that issue today.

Mr. GIBBS. OK.

Mr. SHAYS. OK. Is that all right? I have a lot of respect for both

of you and all the others who have come and it’s been very helpful

and we’ll try to sort out of the some stuff.

Mr. GIBBS. If we can be of further assistance, we are standing

by.

Mr. SHAYS. I was thinking of the staff member, and Mary, where

does she get all these good panelists. She did it again.

Mr. GIBBS. Well, I live on the Hill, I don’t necessarily like to

travel to the Hill.

Mr. SHAYS. Thank you. This hearing is adjourned.

[Whereupon, at 4:10 p.m., the subcommittee was adjourned.]

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