<DOC>
[105th Congress House Hearings]
[From the U.S. Government Printing Office via GPO Access]
[DOCID: f:39522.wais]
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
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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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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:]
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Mr. Shays. Let me just get some housekeeping out of the
way: I ask unanimous consent that all members of the
subcommittee be permitted to place any opening statements in
the record and that the record remain open for 3 days for that
purpose, and without objection, so ordered.
Also, I would ask unanimous consent that witnesses be
permitted to include written statements in the record if they
choose to summarize them. Without objection, so ordered.
At this time, as is the practice, we will swear in all our
witnesses, including Members of Congress, and I would invite
you to rise and raise your right hand. I am assuming, since I
see others standing up and raising their right hand, that you
are backup staff. If they say something for the record, they
will have to be sworn in.
[Witnesses sworn.]
Mr. Shays. For the record, our main witnesses have answered
in the affirmative and as well as others who have accompanied
them.
I would like to apologize to the people in the audience.
Next time we will have the witness table up closer and have a
few more rows of chairs. It may be some won't stay too long and
seats will become available.
At this time, let me just recognize our witnesses. We have
Michael Friedman, who is the Deputy Commissioner for the Food
and Drug Administration, FDA. He will kind of give us the human
health care element here. We have Linda Detwiler, chairman of
the TSE working group from the U.S. Department of Agriculture.
She will give us the animal health perspective. We have, as
well, Lawrence Schonberger, who is the assistant director for
Public Health, National Center for Infectious Diseases, Centers
for Disease Control and Prevention, CDC, and he will give us a
sense of disease detection. Then we have Clarence J. Gibbs,
acting chief, National Institutes of Neurological Disorders and
Stroke, National Institutes of Health, for the focus on
research into this disease.
Let me just say, all of you are doctors, so I didn't
introduce you as doctors. You are all experts and we are eager
to hear your testimony and welcome you. And if we could go in
the order I called you, that would be helpful. Dr. Friedman.
STATEMENTS OF MICHAEL FRIEDMAN, M.D., DEPUTY COMMISSIONER, FOOD
AND DRUG ADMINISTRATION; LINDA DETWILER, D.V.M., U.S.
DEPARTMENT OF AGRICULTURE, ANIMAL AND PLANT HEALTH INSPECTION
SERVICE, ACCOMPANIED BY GLENN MORRIS, FOOD SAFETY INSPECTION
SERVICE, USDA; LAWRENCE SCHONBERGER, M.D., CENTERS FOR DISEASE
CONTROL AND PREVENTION; AND CLARENCE J. GIBBS, JR., PH.D.,
NATIONAL INSTITUTES OF HEALTH
Dr. Friedman. Thank you very much, Mr. Chairman, and
members of the committee. We appreciate this opportunity to
participate in today's discussions on measures to prevent the
transmission of spongiform encephalopathies. I am Michael
Friedman, and I am the Deputy Commissioner for Operations in
the Food and Drug Administration, and with me are a number of
relevant staff to aid you in your considerations.
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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