[House Hearing, 105 Congress]
[From the U.S. Government Publishing Office]



 
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





                     U.S. GOVERNMENT PRINTING OFFICE
39-522                       WASHINGTON : 1997
________________________________________________________________________
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              COMMITTEE ON GOVERNMENT REFORM AND OVERSIGHT

                     DAN BURTON, Indiana, Chairman
BENJAMIN A. GILMAN, New York         HENRY A. WAXMAN, California
J. DENNIS HASTERT, Illinois          TOM LANTOS, California
CONSTANCE A. MORELLA, Maryland       ROBERT E. WISE, Jr., West Virginia
CHRISTOPHER SHAYS, Connecticut       MAJOR R. OWENS, New York
STEVEN H. SCHIFF, New Mexico         EDOLPHUS TOWNS, New York
CHRISTOPHER COX, California          PAUL E. KANJORSKI, Pennsylvania
ILEANA ROS-LEHTINEN, Florida         GARY A. CONDIT, California
JOHN M. McHUGH, New York             CAROLYN B. MALONEY, New York
STEPHEN HORN, California             THOMAS M. BARRETT, Wisconsin
JOHN L. MICA, Florida                ELEANOR HOLMES NORTON, Washington, 
THOMAS M. DAVIS, Virginia                DC
DAVID M. McINTOSH, Indiana           CHAKA FATTAH, Pennsylvania
MARK E. SOUDER, Indiana              TIM HOLDEN, Pennsylvania
JOE SCARBOROUGH, Florida             ELIJAH E. CUMMINGS, Maryland
JOHN SHADEGG, Arizona                DENNIS KUCINICH, Ohio
STEVEN C. LaTOURETTE, Ohio           ROD R. BLAGOJEVICH, Illinois
MARSHALL ``MARK'' SANFORD, South     DANNY K. DAVIS, Illinois
    Carolina                         JOHN F. TIERNEY, Massachusetts
JOHN E. SUNUNU, New Hampshire        JIM TURNER, Texas
PETE SESSIONS, Texas                 THOMAS H. ALLEN, Maine
MIKE PAPPAS, New Jersey                          ------
VINCE SNOWBARGER, Kansas             BERNARD SANDERS, Vermont 
BOB BARR, Georgia                        (Independent)
------ ------
                      Kevin Binger, Staff Director
                 Daniel R. Moll, Deputy Staff Director
                       Judith McCoy, Chief Clerk
                 Phil Schiliro, Minority Staff Director










                            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









 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 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.
    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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[GRAPHIC] [TIFF OMITTED] T9522.002

[GRAPHIC] [TIFF OMITTED] T9522.003

    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 challenge 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 agencies 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:]
    [GRAPHIC] [TIFF OMITTED] T9522.004
    
    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.
    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 BSE-infected 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 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 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 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 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 additional 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 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 Neuro-
pathologists 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.
    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 blood-borne 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 modern 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-Straussler-Scheinker 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 beta-pleated 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 preceded 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 
Creutzfeldt-Jakob 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.
    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 conversion 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 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.
    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.
    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.
    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.
    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 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 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.
    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.
    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.
    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 department 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 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 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 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 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
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 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 
spiroplasma-like occlusions from the brain biopsy of a patient 
with Creutzfeldt-Jakob 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 agent-specific 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.
    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 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 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.
    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 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, 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.
    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.
    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, Creutzfeldt-Jakob 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.
    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.
    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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