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US citizen groups initiate 'Mad Cow' legal actions
Shocking quotes from CDC
American groups file petition at FDA 6 Jan 98
Second petition to CDC filed 6 Jan 1998
Texas cattlemen make top 10 PR blunder list
Another BSE case in Holland
Early diagnosis of Alzheimer
Pentosan stops scrapie, may help with CJD-research

US 'Mad Cow' Legal Action

7 Jan 1999  Press Release
Andrew Kimbrell, D'arcy Kemnitz, 
Attorneys At Center For Food Safety
202-547-9359 

Legal Actions Filed To Force FDA, CDC, To Combat 'Mad Cow' Type Diseases In People, Wildlife And Livestock In The United States.

Terminally Ill Utah Hunter, Age 30, Could Be The First Victim Of U.S. 'Mad Deer' Disease.

'Mad Cow' Type Diseases Are Already Killing People, Deer, Elk And Sheep In The U.S.

Washington, DC -- Today, Thursday, January 7, 1999, two formal legal petitions were filed demanding that the Food and Drug Administration (FDA) and the Centers for Disease Control (CDC) act immediately to monitor, regulate and prevent 'mad cow' type diseases in the United States. These diseases, known as Transmissible Spongiform Encephalopathies, or TSEs, are killing people, deer, elk and sheep in the United States. Petitioners include the Humane Farming Association, the Center for Food Safety, a group of United States CJD victims and their families and consumer, farm and animal protectionists.

The demand for immediate government action is given added urgency by the extremely unusual case of a thirty-year old Utah man, R. Douglas McEwen, who is now terminally ill with CJD, a 'mad cow' type disease in humans. Mr. McEwen hunted deer and elk; it is feared he may have contracted CJD by eating or handling deer or elk infected with 'mad deer' disease. Additionally, there is industry and governmental concern that as a frequent donor Mr. McEwen may have contaminated blood products internationally.

Mr. McEwen and his wife Tracie are petitioners in today's legal actions. His fatal disease underscores concerns that current federal regulations are grossly inadequate to prevent and monitor potential animal and human epidemics in the United States.

The family of 'mad cow' type disease, TSEs, include numerous strains and have different names in different species. The best known strain is British Bovine Spongiform Encephalopathy (BSE), dubbed 'mad cow disease,' which has now spread into the British human population as 'new variant Creutzfeldt-Jakob Disease' or nvCJD. So far 33 teenagers or young adults are confirmed dead or dying of nvCJD. Since the disease has a virtually invisible incubation period that might last decades, it won't be known for many years whether the final toll will be in the dozens, hundreds or thousands of human lives.

In the United States efforts to identify, monitor and prevent human and animal deaths from TSE diseases have been grossly inadequate, despite the fact that people, deer, elk and sheep in the U.S. are dying from these diseases, known as Creutzfeldt-Jakob Disease (CJD) in people, Chronic Wasting Disease (CWD) in deer and elk, and scrapie in sheep.

CJD in humans in the U.S. appears to be often misdiagnosed as Alzheimer's or other types of fatal dementia, and seems much more prevalent than admitted by government agencies. Given the very long invisible incubation period of this 100% fatal disease, and that fact that it can be spread by infected medical instruments and is almost impossible to kill by heat and disinfection, and the consequences of failing to identify the disease are extremely serious.

The first legal petition demands that the U.S. Centers for Disease Control (CDC) aggressively look for CJD in humans and make CJD a reportable and monitored disease. This petition is also being filed in all fifty states with the appropriate state health officials.

The second legal petition demands that the Food and Drug Administration (FDA) close serious loopholes in U.S. animal feed regulations which currently allow types of cannibalistic feeding practices known to cause and spread 'mad cow' type diseases in animals and humans. For instance, current U.S. regulations allow calves to be fed milk replacer containing cattle blood protein, and pigs to be fed back to pigs and cattle. U.S. sheep infected with scrapie, a 'mad cow' type disease, can be used for pet and pig feed in the U.S.

Commenting on today's legal actions Bradley Miller, National Director of the Humane Farming Association stated, "TSEs represent a potentially devastating threat to both human and animal health. Our government's response to date has been shamefully inadequate. These legal actions provide a blueprint by which federal and state agencies can act decisively to prevent a TSE epidemic in this country."

Dr. Michael Hansen, Research Associate of Consumer's Union commented, "The current increase of TSEs in wildlife and humans shows that the time for effective prevention may be running out. The federal agencies must immediately take action to avert what could become a very significant public health problem."

Andrew Kimbrell, public interest attorney and Director of the Center for Food Safety stated, "Given what we know now, it is unconscionable that the CDC is not strictly monitoring this disease, and that the FDA is still allowing the feeding of blood and other animal by-products to animals. The federal agencies are obviously putting the interests of agribusiness companies ahead of their duty to protect the public from this terrible and fatal group of diseases. We will go to court if necessary to ensure that the agencies do their job in protecting human health and animal welfare."

The Center for Food Safety
A project of the International Center for Technology Assessment 310 D Street, NorthEast Washington, D.C. 20002 (202) 547-9359 telephone (202) 547-9429 facsimile

U.S. urged to track human ``mad cow''-like malady

Reuters North America Thu, Jan 7, 1999
WASHINGTON - The U.S. government should set up a system to monitor deaths from the human equivalent of "mad cow" disease because the toll could be much higher than thought, three groups said in petitions filed on Thursday.

The groups also asked for federal action to reduce the chance that livestock would consume feed carrying agents of the disease. The human variant is Creutzfeldt-Jakob Disease.

About 200 people a year die from CJD in the United States, according to estimates by the Centers for Disease Control and Prevention. CJD usually strikes people over 50 years old.

More stringent U.S. action was needed, the petitioners said, because of the possibility people could contract a new strain of CJD by eating meat from infected animals. Officials say no case of "mad cow" disease has been detected in U.S. cattle. The petitioners said studies of deaths attributed to dementia "suggest much higher levels of CJD than commonly suspected" and requested the CDC require health officials to report deaths from CJD-like diseases. The petitioning groups asked the Food and Drug Administration for tough limits on the inclusion of ingredients from dead carcasses in feed for live animals. FDA introduced rules in 1997 to restrict such usage but exempted material from the remains of hogs.

The petitions were filed by the Humane Farming Association, the Center for Food Safety and the Center for Media and Democracy, as well as relatives of some CJD victims. Spokesmen for the CDC and FDA were not immediately available for comment.

Stronger action needed to prevent cases of mad-cow disease

UPI Science News by Ed Susman Fri, Jan 8, 1999
WASHINGTON -- Lawyers for health activists say they want federal agencies to take stronger action to prevent cases of mad-cow disease. Attorneys at the Center for Food Safety in Washington say today they have petitioned the Food and Drug Administration to ban the use of cattle blood in cattle feed -- a practice linked to the spread of mad-cow disease among cattle and CJD, Creuzfeldt-Jakob Disease, among humans.

Lawyer D'Arcy Kemnitz says, "The U.S. lags behind Britain and Europe in implementing safeguards in the feeding of animals and allows practices that should be banned, such as feeding pigs to pigs, pigs to cattle and cattle blood products to calves."

An outbreak of mad-cow disease in Britain is associated with at least 35 cases of CJD, a similar disease, in humans, states one petition that would prohibit such practices. The petition would require meat suppliers to maintain paperwork on meat supply sources for 10 years.

"Now they only have to keep that paperwork for a year," Kemnitz said, which makes it useless to track supplies, because CJD infection can remain dormant for years before symptoms occur. CJD and mad-cow disease are brain illnesses known as Transmissible Spongiform Encephalopathies (TSE). These diseases cause dementia-like symptoms and are almost universally fatal. Confirmation of the disease can usually be made only by autopsy.

Another petition calls for the Centers for Disease Control to make TSE illnesses reportable, mandating that doctors and health officials file reports when these diseases are encountered. Kemnitz said such reporting would give health officials an idea of how frequently these diseases occur and would also give them a better chance to track how humans and animals were infected. Such diseases are known to occur in deer, elk, sheep, squirrels and mink, as well as cattle and humans. A similar petition is being filed in all 50 states with the appropriate state health officials, Kemnitz said.

One of the petitioners is Utah deer hunter R. Douglas McEwen, 30, who has been diagnosed with CJD. McEwen hunted deer and elk, and doctors believe he may have contracted CJD by eating or handling deer or elk infected with "mad-deer" disease.

Industry and government are concerned that McEwen, a frequent blood donor, may have contaminated blood products internationally. Kemnitz said,

"In the United States, efforts to identify, monitor and prevent human and animal deaths from TSE diseases have been grossly inadequate, despite the fact that people, deer, elk and sheep in the U. S. are dying from these diseases, known as CJD in people, chronic wasting disease in deer and elk, and scrapie in sheep.

Andrew Kimbrell, director of the Center for Food Safety, said, "Given what we know now, it is unconscionable that the CDC is not strictly monitoring this disease, and that the FDA is still allowing the feeding of blood and other animal byproducts to animals."

Shocking quotes from CDC

7 Jan 1998 USA Today 'Life' section 
Here's the quote:

"Lawrence Schonberger of the CDC counters that the agency 's surveillance system now captures 86% of CJD case in the USA, a record that he calls 'superb.' He also notes that the Utqh case was promptly reported and investigated -- revealing that the case apparently had nothing to do with exposure to deer, elk, or beef."

Comment (webmaster): The CDC has a long track record of ill-informed comments and opposition to monitoring and research into the disease, sometimes attributed to pressure from drug companies, the beef industry, or the Alzheimer lobby. In my opinion, they need to get better informed or relinquish their role to another agency.

I will never forget sitting at lunch with a top CDC honcho in Washington, DC, and being told firmly that CJD had never been confused with Alzheimer's in the medical literature. The individual was adamantly opposed to this concept and become extremely agitated when I began to go through the 50+ articles in reputable medical journals that document mis-diagnosis of these two diseases. The agency, despite immense financial resources, has to this day never even compiled a simple bibliography of CJD cases mis-diagnosed as Alzheimer. CDC simply has a policy that no Alzheimer is CJD.

The above statement has two gross errors.

1. The 86% figure is circular reasoning; it refers to an oxymoronic in-house audit of a flawed survey. It has been extensively documented that the average American neurologist cannot reliably recognize CJD; little is learned by mailing out questionaires. The agency has no idea how many cases of Alzheimer are really attributable to CJD. Even if this number is as low as 1%, the rate of CJD suddenly triples. However, the agency lacks any data to set even this lower bound. Almost all published CJD series, even for familial CJD, have a much higher reported conflation of these diseases than 1%, more like 10%.

A good case in point is the brilliant work on the McEwen case in Salt Lake City. The victim's wife, after an appalling series of run-arounds with psychologists who said her husband was just stressed and seeking to get off work, finally saw a television show on CJD and told the neurologist the diagnosis she suspected. The response: he is too young and the disease is too rare. It is ridiculous that the patient's family has to diagnose the disease -- and this has happened over and over. Would this case ever had been diagnosed correctly if Mel Steiger had not been on TV the night before the doctor appointment?

Schonberger may calls this 'superb' surveillance and reporting but what about the 121 plasma products that went out to 20 countries and could not be effectively recalled [R. Rouseau, Bayer Corp., unconfirmed]?

2. The CDC makes a very common error concerning sporadic CJD that is inexcusable at this level of government. True, the Utah case did not strain-type out to nvCJD [Gambetti lab, unconfirmed]. Of course, the victim had no exposure to the British strain of BSE so there was never a motivation for this diagnosis, other than the early age of onset. US strains of BSE, such as the Stetsonville strain [taken as experimentally back-infected cows], cannot be ruled out at this time by strain-typing.

Familial CJD had not been ruled in or out at the time of the CDC statement. The open reading frame -- but not promoters or poly A sites -- of the prion gene is being studied at this moment by the Lev Goldfarb lab at NIH [unconfirmed]. There need not exist a prior family history; numerous cases exist of confirmed prion mutations where no prion family history of dementia could be documented.

Assuming the correct diagnois is "sporadic CJD," it is important to remember that in sporadic CJD, by definition, the cause of CJD is unknown. No one has the slightest idea how easily CWD (mad deer and elk disease) transmits to humans and if so, what its strain type(s) would be. The victim's diet was most unusual: elk and deer twice a week. So the CDC statement goes way beyond the facts. It reflects instead a purely political agenda that does not serve the public exposed to this disease.

What is the real issue here for CDC? Perceptions of the safety and adequacy of the blood supply. The argument, basically Big Brother knows best, is if we tell the public that the blood cannot be certified as free from CJD, indeed, that a CJD victim with a virulent strain has just donated extensively to the pool, the public will be alarmed. To explain the risks to each patient, to notify earlier recipients, to track exposed individuals over long periods of time, to put more money into research, to explain why more wasn't done earlier -- unthinkable. Better to issue press releases saying the blood has not been shown to be infectious, never mind that it has not been shown not to be infectious. And nobody knows better than the Schonberger what Rohwer and Brown have in press about infectivity of blood fractions.

Canada recently dropped a science-based policy because of costs and shortages. Because it wasn't practical to assure CJD-free blood, a policy decision was made to declare blood from CJD donors wasn't a risk after all:

Canada lifts ban on blood donors with Creutzfeldt-Jacob disease

Lancet 7 Jan 99 Wayne Kondro Saying that there is no scientific evidence that "classic" Creutzfeldt-Jakob disease is bloodborne, the Canadian government has lifted a 4-year policy obligating the quarantine of blood products made from plasma donated by people with CJD.

After ordering a mid-December quarantine of thousands of vials of fractionated blood products made from plasma donated by a Utah man who was thought to have had variant CJD, Health Canada's Bureau of Biologics late last month freed Canada's blood agencies to use albumin, immunoglobulins, and factor VIII made from the man's plasma, after it was determined the 29-year-old had had classic CJD. He had donated blood over 100 times in the past 2 years, including some after he had begun to show signs of CJD.

"In this case, we are absolutely sure that this is a case of classical CJD, and the expert advice that we have received in the past several months is that there is no evidence whatsoever that there has been any transmission or is any transmission of classical CJD through blood or blood products", Bureau of Biologics director Keith Bailey told reporters. "Our assurance would be that to the best of our knowledge, there isn't a problem with these products", Bailey said.

While acknowledging that there is still a theoretical possibility that classic CJD could be bloodborne, Health Canada officials said the preponderance of the evidence suggests it is not, so there is little rationale for maintaining the bureau's quarantine policy, which was introduced in 1995.

The new policy mirrors that of the USA and only obligates the withdrawal of plasma from donors with variant CJD.

Petition for the FDA (23 pages)

7 Jan 99 petition filed at FDA

To comment on the petitions: There are two of them.  The  one below has docket number FDA 7774 and the other  with HHS which has docket 99P-033/CP 1.   Use docket numbers when writing to the
agencies.  Addresses are below.  Anyone   can write to the agencies in support (or opposition) to the
petitions.  Comment  substance weighs more than emotion.  For example, letters could outline the writer's
experience with CJD and familiarity with the petitions' content, merging what they know
about CJD and what the petition asks for.  For instance:  "At first, CJD
was not diagnosed, just like it said in the petition, then we experienced the
frustration of misdiagnosis . . . " or whatever is appropriate and factually accurate.  
CITIZEN PETITION BEFORE THE UNITED STATES FOOD AND DRUG ADMINISTRATION
Dockets Management Branch Food and Drug Administration Department of Health and Human Services
Room 1-23, 12420 Parklawn Drive Rockville, Maryland 20857

HUMANE FARMING ASSOCIATION, Post Office Box 3577, San Rafael, CA 94912-8902, et al. Petitioners,
vs. Docket No _______
JANE HENNEY, in her official capacity as
Commissioner Food and Drug Administration
5600 Fishers Lane, Room 14-71 Rockville, MD 20857 Defendants.

PETITION SEEKING IMMEDIATE ACTION BY THE FDA TO COMBAT THE SPREAD OF TRANSMISSIBLE SPONGIFORM ENCEPHALOPATHY (TSE) IN THE UNITED STATES
Petition Requesting Immediate Action by the Food & Drug Administration to Combat Transmissible Spongiform Encephalopathies

TABLE OF CONTENTS

I. Introduction

II. Petitioners

III. Statement of the Facts

IV. Procedural History

V. Statement of the Law

VI. Argument

A. Inadequate Regulations Violates FDA's Mandate to Protect Public Health

B. Specific Regulatory Amendments Requested and Supporting Information

1. Blood and Blood Products Must Not Be Used in Animal Feed Since Blood and Blood Products Carries TSEs

2. Gelatin and Gelatin By-Products Must Not be Used in Animal Feed Since Gelatin and Gelatin By-Products Likely Carries TSEs

3. Record Keeping Requirements of One Year are Grossly Inadequate: Establishments and Individuals Responsible for Keeping Records Must Keep Files for Ten Years to Protect the Public Since BSE Incubation Periods Last from Two or Three to Eight Years.

4. Pure Porcine Protein Must Not be Used in Animal Feed Since Pigs are Susceptible to TSEs

...a. Case Studies Show that People who Eat Pig Brains Risk Infection of TSE

...b. FDA is Aware of the Evidence of Porcine TSEs

5. Animal Feed Labeling Requirements Must be Expanded to Include Other Species Beside Ruminant Species Since Other Species Get TSEs

...a. TSEs Infect Cats and Cat Food Must be Safe from TSE Contamination

...b. TSEs Likely Infects Dogs and Dog Food Must be Safe from TSE Contamination

...c. Some Animals Might Be Carrier Species

6. Animal Feeds Must Not Contain Any Part of Any Animal Showing Signs of a TSE; Nor May Any Such Contaminated Material Be Used as Fertilizer, Cosmetics, or Other Products

VII. Environmental Impact & Certification

VIII. Agency Action Requested

I. Introduction

Pursuant to the Right to Petition Government Clause contained in the First Amendment of the United States Constitution, the Administrative Procedure Act, and the Food and Drug Administration's (FDA) implementing regulations, the undersigned submits this citizen petition for rulemaking and collateral relief and respectfully requests the Commissioner to establish regulations that protect the public from Transmissible Spongiform Encephalopathies (TSEs) by amending animal feed regulations.

II. Petitioners

This petition is submitted on behalf of the following petitioners:

A. The Humane Farming Association, an I.R.S. Code 501(c)3 not for profit organization [with 125,000 members -- webmaster], with its principle offices located in the State of California and its mailing address at Post Office Box 3577, San Rafael, California 94912-8902, (415) 485-1495;

B. The Center for Food Safety, a project of the International Center for Technology Assessment, an I.R.S. Code 501(c)3 not for profit organization with its principle offices located in the District of Columbia at 310 D Street, N.E., Washington, D.C. 20002-5722; (202) 547-9359;

C. Center for Media & Democracy, an I.R.S. Code 501(c)3 not for profit organization, with its principle offices located in the State of Wisconsin at 3318 Gregory St., Madison, Wisconsin 53711-1725; (608) 233-3346;

D. R. Douglas and Tracie L. McEwen, 4 East 270 South, Kaysville, Utah, 84037; Mrs. McEwen's husband is R. Douglas McEwen, 30, a territory manager for a brokerage company until August of 1998, is presently terminally ill with CJD. It is an extremely agonizing decline for him, her, and their two young daughters, ages 3 and 8. He was diagnosed on Nov. 25, 1998 at the University of Utah Hospital in Salt Lake City, Utah, after a brain biopsy.

E. Patricia C. Ewanitz, 46-03 216th Street, Bayside, New York 11361; Ms. Ewanitz' husband, Andrew P. Ewanitz, Jr., a engineer and plant facilities manager, died of Creutzfeldt-Jacob Disease (CJD) at Jacob Perlow Hospice, 1st Avenue and 16th Street, New York City on April 9, 1997 after his initial diagnosis was a mild stroke;

F. Elizabeth A. Armstrong, 205 S. Cherry Street, Lebanon, Illinois, 62254; Ms. Armstrong's father, Charles O. Butcher, a law enforcement officer, died of CJD on May 6, 1996, at Veterans Administration Medical Center, Richmond, Virginia, after an initial diagnosis of COPD then Parkinson's Disease;

G. Melvin J. Steiger, 5582 Cora Way, Salt Lake City, Utah 84118-2316; Mr. Steiger's wife, Eleanor M. Steiger, 57, an accounting clerk, was initially diagnosed with depression. Symptoms began in 1996 with severe leg muscle cramps. Symptoms eventually included changes in personality where she become less patient and more argumentative, loss of coordination, memory and ability to function. Admitted to the hospital for a possible stroke, Mrs. Steiger was positively diagnosed with CJD on the 19th of February, 1998. On February 23, 1998, a biopsy, an EEG and examination confirmed that diagnosis. She passed away at home on March 19, 1998;

H. Beverly Wilson Goodman, 7308 Moody Court, Fort Worth, Texas 76180-6107; Ms. Goodman's father, Perry Marlin Wilson, died of CJD at Zale Lipshey a division of Parkland Hospital, Dallas, Texas, with a diagnosis of "unknown" on May 18, 1997;

I. Mildred B. Campbell, 2229 Hwy. 601-S, Mocksville, North Carolina; Ms. Campbell's husband, John D. Campbell, a retired law enforcement officer, died of CJD at Forsyth Memorial Hospital, Winston-Salem, North Carolina on March 18, 1997, with an original diagnosis of stoke and peripheral neuropathy; CJD was later confirmed by a brain biopsy;

J. Dorothy E. Kraemer, 901 S. Stanley Street, Stillwater, Oklahoma 74074; Ms. Kraemer's mother cause of death from CJD was confirmed through a brain autopsy after her death at Stillwater Nursing Home on May 16, 1996; the original diagnosis was rapid onset Alzheimer's Disease;

K. Cecile L. Sardo, 17065 N.W. 22nd Street, Pembrook Pines, Florida 33028; Ms. Sardo's husband, Joseph Sardo, died of CJD;

L. Jim and Rebecca Goodman, of E103 County Q, Wonewoc, Wisconsin 53968, who make their living by farming in the State of Wisconsin;

M. Bruce and Shelley Krug, of Box 84 West Road, Constanbleville, New York 13325; the Krugs are dairy farmers.

III. Statement of The Facts

Transmissible Spongiform Encephalopathy (TSE) is a mysterious class of diseases that are called by different names in different species. For instance some identified types of TSE are Creutzfeldt-Jakob Disease (CJD) in humans, scrapie in sheep, bovine spongiform encephalopathy (BSE) in British cattle, transmissible mink encephalopathy (TME) in mink in North America, and chronic wasting disease (CWD) in deer and elk in North America. There may be different strains of TSE within species, and new strains may be produced when TSEs move from one animal species to another.

There is vigorous scientific debate as to exactly what the TSE agent is, since it has never been completely isolated, but the leading theory seems to be that it is a mutant or infectious form of a naturally present protein probably present in all mammalian species called a prion protein; the name prion was created by Dr. Stanley Prusiner whose scientific work was awarded a Nobel Prize in 1997.

The common characteristics of TSE diseases are that they are invariably fatal. They can be transmitted through iatrogenic or physician induced exposure, such as corneal transplants, use of dura mater during surgery, contaminated human growth hormones, contaminated probes or possibly even blood. Additionally, infection may occur through exposure of infected material to mucous membranes or open cuts, and apparently in some cases, such as with British BSE-nvCJD, through the ingestion of the infectious agent. The agent does not trigger an immune response, and the infected animal or human appears perfectly normal until at some point in life (usually the sixth decade in humans who develop sporadic CJD) the disease emerges as holes and amyloid plaque material spreading in the brain, causing symptoms of dementia, similar to Alzheimer's disease, physical failure and death.

TSEs can be difficult to diagnose during life; confirmation of TSE disease is usually made post-mortem through autopsy analysis, or through a newly-developed spinal fluid test and case study, and can be confirmed by passaging the infectious agent from the deceased specimen into the brain of a laboratory animal. (For instance, Dr. Richard Marsh of the University of Wisconsin passaged TME from mink that had fed on 'downer' dairy cattle into two Holstein bull calves via injection in the mid 1980s. The disease that resulted killed the calves, but they did not develop the 'madness' symptoms which led British BSE to be labeled 'mad cow disease.' Instead, the two bull calves suffered 'downer' cattle syndrome and TSE was confirmed as the cause of death in post-mortem examination. This provided further evidence of a theory first expressed in 1964 at a conference organized by Gajdusek and Gibbs that the source of TME might actually be a BSE in U.S. cattle.)

Prior to the outbreak of BSE in British cattle in the mid 1980s, and its spread to the human population as nvCJD which has claimed to date 35 lives, the best known TSE was sheep scrapie. It appeared in Spanish sheep in the 18th century, and has since appeared in most countries that raise sheep. Sheep scrapie appeared in U.S. sheep in the late 1940s, via Canadian sheep of British origins. After the failure of U.S. eradication efforts, sheep scrapie has spread across the United States and its actual occurrence is unknown.

Prior to the appearance of nvCJD in Britain, the best known occurrence of a TSE in humans was the disease kuru, a TSE that appeared in the Fore tribe in New Guinea in the early 20th century, spread by cannibalistic rituals until the recognition of that fact in work by Dr. Daniel Carleton Gajdusek and Dr. Clarence Joseph Gibbs of the U.S. National Institutes of Health. For this work Gajdusek received the first Nobel Prize awarded for TSE research.

When British BSE, dubbed 'mad cow disease', appeared in the mid 1980s, it stood out because of the madness symptoms of the animals in a rabies-free nation. From just a few animals the disease rapidly spread to infect hundreds of thousands. By 1988 epidemiology demonstrated that the disease was in fact being spread by the feeding of rendered animal byproducts as a protein and fat feed supplement. British cattle were consuming the remains unfit for human consumption of sheep, cattle and other animals. While the high prevalence of sheep scrapie in Britain has led to presumptions that British BSE is sheep scrapie in cattle, there is no proof of this. Others suspect that BSE may have emerged from the feeding of a small number of BSE infected cattle back cannibalistically; in any case, the feeding of infected cattle back to healthy cattle clearly amplified and spread BSE within British cattle, whatever the origins of the TSE agent.

The practice of feeding rendered animal byproducts back to livestock began in very small ways in the 19th century, but became a large widespread practice involving billions of pounds of rendered animal fat and protein from the 1960s to the present. The practice continues today, although with some recent restrictions due to the spread of BSE and the emergence of nvCJD.

Despite the British outbreak, the practice of feeding rendered livestock meat and bonemeal back to livestock has been most widely practiced in the United States, where no meaningful restrictions were even attempted until August, 1997. Today, the U.S. lags behind Britain and Europe in implementing safeguards in the feeding of animals, and allows practices that should be banned, such as feeding pigs to pigs, pigs to cattle, and cattle blood protein to calves. These practices are allowed despite evidence that TSE disease may already infect pigs and cattle in the U.S., and despite the existence of proven TSEs in U.S. sheep, deer, elk and mink.

Indeed, Dr. Clarence Joseph Gibbs theorizes that every mammalian species is likely to have individuals with a TSE disease at some low level, perhaps one in a million. Therefore, any cannibalistic feeding practices might amplify and spread the disease, as in Britain, possibly creating a TSE that can jump from one species to another, as has apparently happened in the U.K. with the spread of BSE into humans as nvCJD. While 35 deaths to date seems a small number, the British government admits that the virtually invisible disease nvCJD may have an incubation period measured in decades, and therefore it will require the passage of many additional years to determine the extent of the disease and to predict the eventual death toll. Estimates have ranged from scores to hundreds of thousands eventual deaths from nvCJD in Britain.

CJD exists in the United States, but it is unclear at what level. Most CJD in the U.S. is considered 'sporadic' with an unknown cause. A smaller percentage appears to have a genetic factor.

The U.S. has a large population of persons with various dementia diseases, the most often diagnosed being Alzheimer's (four million cases). There is no routine postmortem diagnosis of dementia deaths, although autopsy is the only sure way to determine what type of dementia a patient had. It appears that one of four dementia diagnoses made during life are actually incorrect, with another type of dementia causing the death.

Studies of people in the U.S. who have died of dementia suggest much higher levels of CJD than commonly suspected, with proven levels of anywhere from 1 to 13% of dementia victims. Individuals whose family members have died of CJD report great difficulties in receiving a correct diagnosis. Their recent activism through grassroots organizations such as CJD Voice, and the recognition that CJD seems much more common than thought, has led some states (Texas, Utah, Kentucky and Ohio) to begin making mandatory the reporting of CJD. Without specific new programs to investigate and identify CJD cases, the true number cases and whether they are increasing will remain difficult to document.

Given what is now known about the ability of TSE diseases to move between species and infect humans, the existence of animal TSEs in the United States, and the long and invisible incubation period during which a human or animal CJD strain might become epidemic, it is imperative that state and federal agencies implement programs for accurately determining and monitoring the number of TSE cases in humans and animals in the United States.

On December 16, 1998, press in Utah reported the case of a thirty year old man who is terminally ill due to diagnosed CJD. R. Douglas McEwen, is now a petitioner in this action. Mr. McEwen's young age makes this an extremely unusual occurrence; British nvCJD came to public attention because of the similarly young age of its victims. Mr. McEwen was a reportedly avid consumer of deer and elk meat, and some areas of the western U.S., including the area where Mr. McEwen hunted, have been shown to have very high levels (1% to 6% in tested individuals) of chronic wasting disease (CWD) in deer and elk, a TSE apparently unique to North America.

It is possible that Mr. McEwen has CWD contracted through contact with or consumption of infectious deer or elk. Whether or not this is the case, his CJD emphasizes the critical importance of taking all prudent steps to prevent the spread of TSEs in animals and people, and in determining and monitoring the existence of these diseases in the United States.

Human health concerns are amplified by the many unknowns surrounding CJD, including the invisibility of the agent and its infectious routes. For instance, contaminated medical instruments are impossible to adequately disinfect and have spread TSEs to healthy patients, as have cadaver-source tissue and organs. In laboratory tests blood can transmit TSEs, and in Britain the use of British plasma has been curtailed because of concerns that it might spread nvCJD. Incidentally, Mr. McEwen, the Utah CJD patient, was reportedly a regular, dedicated plasma donor.

In summary, the emergence of infectious TSEs that can move between species is a new and unanticipated public and animal health threat that is being inadequately studied and addressed in the United States. This petition seeks to remedy those failures.

IV. Procedural History

In light of the new scientific understanding, new studies and new evidence presented in this petition, the petitioners now request the Food and Drug Administration (FDA) and the United States Department of Agriculture (USDA) take the following immediate actions to prevent the potential spread of transmissible spongiform encephalopathy including bovine spongiform encephalopathy (BSE), also known as "Mad Cow Disease," Chronic Wasting Disease (CWD), and Creutzfeldt-Jacob Disease (CJD):

1. Amend 21 C.F.R. ■ 589.2000 ("Substances Prohibited From Use in Animal Food or Feed; Animal Proteins Prohibited in Ruminant Feed" finalized at 62 Federal Register 30,936 (June 5, 1997)) as follows:

a. Amend the rule so that blood and blood products are not fed to animals;

b. Amend the rule so that gelatin and gelatin products are not fed to animals;

c. Amend the rule so that pig and porcine materials are not fed to animals;

d. Amend the rule so that animal feed labeling requirements include other species beside ruminant species, since TSE is not only a ruminant disease and other animals beside ruminants are infected and pass along TSEs;

e. Amend the rule to state that no part of any TSE contaminated animal can be fed to animals or humans and that such contaminated material cannot be used as fertilizer, cosmetics, or other products; and

f. Expand the record keeping requirements from one year to ten years.

2. 21 C.F.R. ■ 589.2000 "Substances Prohibited From Use in Animal Food or Feed; Animal Proteins Prohibited in Ruminant Feed" should read:

(i) Label the materials as follows: "Do not feed to any animal."

(h) Inspection; records retention.

(1) Records that are to be made available for inspection and copying, as required by this section, shall be kept for a minimum of 10 years.

(Requested changes indicated in bold.)

Petitioners incorporate by reference information submitted to the agency through previous petitions dated June 23, 1993, December 21, 1993, March 26, 1996, March 27, 1996, as well as a the petition to the Health and Human Services agency dated January 6, 1999 and copied to this agency which presented evidence and requested action to combat the development of TSEs in general, and BSEs in particular, in the United States. Petitioners also incorporate by reference agency actions requested in those previous petitions.

Petitioners also incorporate by reference information submitted to the agency in a previous petition dated March 27, 1997, by the Government Accountability Project which demanded that the laws and regulations that keeps pigs with nervous system disorders from entering the food supply be strengthened.

Petitioners further incorporate by reference information submitted to the agency in a previous petition dated March 4, 1998, by Farm Sanctuary and Michael Baur which requested that the FDA and the USDA immediately label all downed cattle as adulterated pursuant to 21 U.S.C. ■ 342 (a). Petitioners further incorporate by reference information submitted to the agency in a supplemental petition dated May 10, 1998, which amended the March 4, 1998, petition. That supplemental petition requested that the FDA and USDA immediately label all downed livestock, not just cattle, as adulterated pursuant to 21 U.S.C. ■ 342 (a) to protect the health of the nation by reducing the possibility of the spread of transmissible spongiform encephalopathy (TSE) and its derivations, bovine spongiform encephalopathy (BSE) also known as "Mad Cow Disease," and Creutzfeldt-Jakob Disease (CJD).

On June 23, 1993, petitioning attorneys submitted an "Amended Petition Requesting the Food and Drug Administration to Halt the Feeding of Ruminant Animal Protein to Ruminants" on behalf of several individuals and a non-profit organization. The petition requested the FDA and the USDA to undertake agency actions that would address the potential health threat posed to U.S. animal herds, especially cattle, from TSEs and the threat posed to U.S. meat consumers, especially beef consumers, through the zoonotic development of CJD as a result of eating TSE contaminated meat.

On December 21, 1993, petitioning attorneys submitted a "Supplemental Petition Requesting the Food and Drug Administration to Halt the Feeding of Ruminant Protein to Ruminants" on behalf of the same individuals and non-profit organization. The supplemental petition was filed as result of, inter alia, the FDA to respond to the June 23, 1993, petition within the one hundred and eighty (180) days mandated by 21 C.F.R. 10.30(e)(2).

In a much belated response to the two petitions, the FDA issued a proposed rule in the Federal Register proposing that specified offal from adult sheep and goats (of more than 12 months of age) is not generally recognized as safe for use in ruminant feed and is an unapproved food additive when added to ruminant feed. In June 5, 1997, a limited rule was promulgated by the FDA providing that a few certain types of animal proteins would not be used as raw materials in the rendering industry.

This petition serves to notify the FDA that it acted arbitrary and capriciously by failing to promulgate a rule preventing the establishment and amplification of TSE, and its particular strains of the disease BSE, CJD, and nvCJD through animal feed.

V. Statement of the Law

A. Food and Drug Administration, Compliance Policy Guide 7126.24 (10-1-80) states:

Rendered animal feed ingredients which contain harmful microorganisms, toxins or chemical substances may be considered adulterated under Sections 402(a)(1) or (2) of the Act. Where a rendering procedure itself raises a question of disease transmission, the ingredient made may be deemed adulterated under Section 402(a)(4).

B. Federal Food, Drug and Cosmetic Act, 21 U.S.C. Section 321(f) defines food as "articles used for food or drink for man or other animals" and "articles used for component of any such article."

C. Federal Food, Drug and Cosmetic Act, 21 U.S.C. Section 342, Adulterated Food. A food shall be deemed to be adulterated Ď (a) (1) If it bears or contains any poisonous or deleterious substance which may render it injurious to health; but in case the substance is not an added substance such food shall not be considered adulterated under this clause if the quantity of such substance in such food does not ordinarily render it injurious to health; or (2)(A) if it bears or contains any added poisonous or added deleterious substance ... or (4) if it has been prepared, packed, or held under insanitary [sic] conditions whereby it may have become contaminated with filth, or whereby it may have been rendered injurious to health.

D. Administrative Procedure Act, 5 U.S.C. Section 706, Scope of Review. To the extent necessary to decision and when presented, the reviewing court shall decide all relevant questions of law, interpret constitutional and statutory provisions, and determine the meaning and applicability of the terms of an agency action. The reviewing court shall --

(1) compel agency action unlawfully withheld or unreasonably delayed; and

(2) hold unlawful and set aside agency action, findings and conclusions found to be -- (A) arbitrary, capricious, an abuse of discretion, or otherwise not in accordance with law.

E. Food and Drug Administration, 21 C.F.R. ■ 589, Substances Prohibited From Use in Animal Food or Feed, Subpart A, General Provisions Sec. 589.1 Substances prohibited from use in animal food or feed.

(c) The Food and Drug Administration either on its own initiative or on behalf of any interested person who has submitted a petition, may publish a proposal to establish, amend, or repeal a regulation under this part on the basis of new scientific evaluation or information. Any such petition shall include an adequate scientific basis to support the petition, shall be the form set forth in Sec. 571.1 of this chapter, and will be published in the Federal Register for comment if it contains reasonable ground. [45 FR 28319, Apr. 29, 1980]

F. Food and Drug Administration, 21 C.F.R. ■ 10.30(e)(2). Citizen Petition.

The Commissioner shall furnish a response to each petitioner within 180 days of receipt of the petition.

VI. Argument

A. Inadequate Regulations Violates FDAs Mandate to Protect Public Health

According to the FDA, "[t]he Food and Drug Administration touches the lives of virtually every American every day." For it is FDA's job to see that the food we eat is safe and wholesome, [and] the cosmetics we use won't hurt us . . . Feed and drugs for pets and farm animals also come under FDA scrutiny. FDA also ensures that all of these products are labeled truthfully with the information that people need to use them properly.

As will be established infra, the FDA and the USDA have failed to adequately regulate the rendering industry in order to protect the public health. The agency's refusal to amend the regulation "Substances Prohibited from Use in Animal Food or Feed" on the basis of new scientific evaluation and information, as requested in this petition would be arbitrary and capricious. An agency's action is reviewed by the judiciary under the arbitrary and capricious standards of law. Under this standard, an agency must show a "rational connection between the fact found and the choices made." In determining whether an agency decision was "arbitrary and capricious," a court must consider whether the decision was based on a reasoned evaluation of the relevant factors and whether there has been a clear error of judgment.

An agency action is arbitrary and capricious when an agency: has relied on factors which Congress has not intended it to consider, entirely failed to consider an important aspect of the problem, offered an explanation for its decision that runs counter to the evidence before the agency or is so implausible that it could not be ascribed to a difference in view or the product of agency expertise.

Many different experts have warned of the possible epidemic proportion of the health threat to the United States' public from TSEs. One expert explained: [T]he British BSE disaster was not some throwback to antique conditions or mere bad luck. It followed in part from a pernicious anti-regulatory ideology that has taken hold in Britain and the United States in the last two decades. . . . [E]ggs and chicken meat contaminated with salmonella, hamburger and fresh cider poisoned with deadly E. Coli and beef infected with BSE all evidence a failure of government inspection, supervision and policy.

The FDA's promulgated rule has been described as "disturbing" in that "compromises [are] built into the FDA's . . . ban." As one expert on TSE states: [The regulation] . . . prohibit[s] the use of tissue from cattle, sheep and goats in feed for those ruminants but would permit the continued feeding of ruminant blood, milk and gelatin [This is the continued feeding of ruminants to ruminants]. Ruminant tissue would continue to be processed into feed for chickens, pigs and pets despite the known susceptibility of pigs and cats to spongiform encephalopathy and the possible passage of the disease agent through chickens into their manure, which organic farmers use to fertilize vegetables. Nor do the ubiquitous contamination of U.S. poultry and eggs with salmonella and the continuing outbreaks of human E. coli infection in government meat inspire confidence in government inspection.

From the rendering industry's perspective, the ban turns a valuable asset into an expensive liability. That reversal in Britain led to widespread cheating. Without rigorous enforcement, U.S. consumers have reason to fear the same result. Based upon the imminent human health hazard presented by TSEs, including BSE, the FDA must amend this regulations in order to protect human health. The FDA itself, by its very action in implementing animal feed regulations, demonstrates its understanding that animal feed practices that allow for high risk mammalian material to be fed to other mammals is a public health hazard. Allowing significant loopholes in the regulations, such as the feeding of cattle blood to cattle, shows a failure to consider important aspects of the health risk, and is clearly arbitrary and capricious.

B. Specific Regulatory Amendments Requested and Supporting Information

1. Blood and Blood Products Must Not Be Used In Animal Feed Since Blood and Blood Products Carries TSEs

Blood and blood products are listed as exceptions to the list of prohibited substances in the current regulations. Petitioners request that the regulations be amended as follows: (a) Definitions (1) Protein derived from mammalian tissues means any protein-containing portion of mammalian animals, excluding: Blood and blood products; gelatin; inspected meat products which have been cooked and offered for human food and further heat processed for feed (such as plate waste and used cellulosic food casings); milk products (milk and milk proteins); and any product whose only mammalian protein consists entirely of porcine or equine protein.

This current exception allows the producers and the rendering industry to continue the practice of feeding cattle blood and blood products to cows. This exception to the rule negates the very purpose of the rule. TSEs appear to be found in blood and blood products. Science shows that (beta)-leucocytes, a component of blood, may propagate prions in blood and are needed for the TSE agent to spread throughout the body. Further, CJD and scrapie have been shown to be transmissible through blood components in rodent models. Because the blood itself is likely infectious, petitioners demand that the agencies stop feeding mammal blood to other animals.

In addition to the blood itself being infected, blood poses another risk in the transmission of TSEs. Blood flows and moves within the body to conduct and transport materials from one site to another site. Thus, simply as a fluid transporter, blood carries potentially high risk neurological material. That high risk neurological material is already banned from use in animal feeds, and thus blood must be banned as well.

In cows, high risk neurological material gets in the blood through the slaughtering process. Many cows are slaughtered via the use of pneumatic stun guns which inject air into the cows' brains destroying them. A cow's brain is literally "blown out" of the cranial cavity. Such force is used in this process that parts of the brain may travel throughout the cow's body. For instance, scientists have discovered chunks of brain throughout the lungs and the liver. Such high risk materials will be found in the blood of the animals. Thus, merely as a transporter of high risk material, petitioner demand that blood no longer be allowed in animals' feed. Although there is a risk of TSE contamination from slaughterhouse blood, an even more dangerous practice involves feeding blood directly to animals by adding the blood directly into animal feed.

Blood and blood products are used as animal feed in two ways. In the first practice, blood serum is used to a colostrum supplement for newborn calves. Colostrum is the first milk a calf would receive from its mother which is rich in antibodies and disease prohibiting agents. In modern factory farming practices, calves may be removed from their mothers immediately after birth. A colostrum replacer which might contain blood serum is then fed to the newborns. This practice is especially risky since calves are likely to be more vulnerable to transmission of infectious agents.

The second method of using blood as animal feed is the practice of spraying blood directly onto calf feed for weaned calves. A farm management article states that, "Hog producers have known for years that adding spray-dried plasma made from pigs' blood to their post-weaning starter rations could make money. . . Now there's a growing body of research that suggests spray-dried cows' plasma can make money for dairy producers, too." Since "[t]he method of spray drying helps maintain plasma protein structure and integrity" any infectious agent in the blood will remain potently infectious. Whereas in other animal feed processes, such as rendering, where animals' parts are heated or cooked, the level or titre of TSE infectious agent might be significantly reduced, the practice of spray-drying allows the infectious agent remains unreduced by heat or other processing. Thus, the plasma's protein structure, including the structure of the TSE prion, likely remains intact. The practice of feeding blood to animals is a high risk practice that is a health risk to the public. Based upon this information, the petitioners request the regulatory changes proposed above including the elimination of blood and blood products from use in animal feed.

2. Gelatin and Gelatin By-Products Must Not Be Used In Animal Feed Since Gelatin and Gelatin By-Products Likely Carry TSEs

The current FDA regulation lists gelatin, inter alia, as an exception from prohibited animal feed substances. Petitioners therefore request the following regulatory changes: (a) Definitions (1) Protein derived from mammalian tissues means any protein-containing portion of mammalian animals, excluding: Blood and blood products; gelatin; inspected meat products which have been cooked and offered for human food and further heat processed for feed (such as plate waste and used cellulosic food casings); milk products (milk and milk proteins); and any product whose only mammalian protein consists entirely of porcine or equine protein.

Gelatine has been shown to be infected with TSE. During the European Commission controversial lifting of the embargo on gelatin in the UK in June, 1996, the Scientific Committee on Cosmetology, the Scientific Committee for Food and the European Medicine Evaluation Agency opposed the end of the embargo on gelatin as it might carry TSEs. Furthermore, the World Health Organization has specifically recommended that in order to minimize the risk of the transmission of TSEs there should be special regulations for brain, spinal cord or related tissue. Those special regulations will keep high risk neurological tissue out of the food supply. However, high risk material can get into animal feed through gelatin. Gelatin is made from animals' bones. For instance, whole vertebra, the nerve tissues of which is designated high risk neurological material, is used to make gelatin. Thus, petitioners request that gelatin itself be a prohibited substance in animal feed. While England has stopped this mechanical recovery of meat or deboning, the United States continues engaging in this high risk practice. Thus, gelatin is not safe to be added to animal feed and should be prohibited under the regulations. 3.

Record Keeping Requirements of One Year are Grossly Inadequate: Establishments and Individuals Responsible for Keeping Records Must Keep Files for Ten Years To Protect the Public Since BSE Incubation Periods Last from Two or Three to Eight Years

Currently the FDA requires cattle "establishments" and "individuals who are responsible for feeding ruminant animals to maintain records for one year of purchase invoices and labels of all their animal feed." Petitioners request that this regulation be amended as follows:

(f) Requirements for establishments and individuals that are responsible for feeding ruminant animals. Establishments and individuals that are responsible for feeding ruminant animals shall maintain copies of purchase invoices and labeling for all feeds containing animal protein products received, and make the copies available for inspection and copying by the Food and Drug Administration; and

(h) Inspection; records retention.

(1) Records that are to be made available for inspection any copying, as required by this section, shall be kept for a minimum of 1 year 10 years.

Evidence of TSE incubation periods indicates that the FDA's current record keeping requirement should be changed from one year to ten years for two reasons. First, a one-year record keeping requirement does not protect public health because animals do not show signs of TSE infection within one year. Second, the proposed changes will establish an adequate paper trail so that the agencies can trace back contaminated and infectious material.

The scientific evidence is clear that TSEs do not incubate in one year. According to APHIS' own publication, BSEs are known to incubate for periods of two to eight years. Additionally, another two scientists have suggested that BSE may have an incubation period of between three and eight years. Based on the long incubation period, a one year record keeping requirement will not be adequate to detect the source of TSE. For example, a six year incubation period would mean a five-year gap in record keeping and thus no trace-back is possible to the source of contamination. Therefore, a ten year record keeping period will ensure the agency is reasonably prepared to prevent TSE outbreaks.

4. Pork and Pure Porcine Protein Must Not Be Used in Animal Feed Since Pigs are Susceptible to TSEs

Porcine and equine protein are referenced in the regulations, inter alia, as an exception to the definition of protein and thus allowed in animal feed. Petitioners request the following amendments to the regulation: (a) Definitions (1) Protein derived from mammalian tissues means any protein-containing portion of mammalian animals, excluding: Blood and blood products; gelatin; inspected meat products which have been cooked and offered for human food and further heat processed for feed (such as plate waste and used cellulosic food casings); milk products (milk and milk proteins); and any product whose only mammalian protein consists entirely of porcine or equine protein.

The current rule allows renderers to continue the practice of feeding animals to animals as long as that material comes from single species slaughterhouses, dubbed pure porcine or pure equine materials. Although FDA permits pig products in animal feed, the science shows that pigs are susceptible to TSEs. Due to this public health danger, other countries have proposed regulations banning the use of the remains of any mammals in pig feeds and banning the use of pig slaughterhouse waste and petfood waste as animal feed. Thus, FDA should prohibit the use of pig products or pure porcine protein in animal feed.

a. Case Studies Show that People who Eat Pig Brains Risk Infection of TSE

The greatest concern to human health involving infection from pigs comes from the ingestion of pig products as food. Epidemiologic evidence indicates that TSE victims are greater-than-average consumers of pig products. This concern comes from three case control studies. In the most recent and largest study, evidence shows that TSE in pork causes CJD in humans. This case control epidemiological study indicated a possible link between brain consumption and sporadic CJD, not nvCJD. In this study, involving over four hundred CJD cases, researchers discovered a link between animal consumption and sporadic CJD. This link from animal consumption to sporadic CJD is worthy of notice because it has been presumed that only nvCJD, not sporadic CJD, was caused by animal consumption. The second study involved 38 CJD patients and showed that an unusually high number Ď a full one-third Ď ate brains as food. Indeed, those who ate brain tissue as food had a preference for hog brain. The third study involved 26 victims of CJD and showed that nine out of 45 food items were statistically related to the risk of CJD infection. Six of nine high risk food products came from pigs. The scientists who presented the evidence cautioned "[t]he present study indicate[s] that consumption of pork as well as its processed products (e.g., ham, scrapple) may be considered as risk factors in the development of Creutzfeldt-Jakob Disease." "An increased consumption among [CJD] patients was found for roast pork, ham, hot dogs . . ., roast lamb, pork chops, smoke pork and scrapple." In light of the risk of CJD from the consumption of pork and pig products, the agencies must protect the public from the risk of this disease by banning the use of all mammalian proteins, including swine, in the feed of all food animals.

b. FDA is Aware of the Evidence of Porcine TSEs

Evidence for the potential PSE (porcine spongiform encephalopathy) was gathered by an FSQS veterinarian, Dr. Masuo Doi. In 1979, Dr. Doi noticed some unusual central nervous system (CNS) symptoms in young (about 6 months old) hogs coming into a slaughter plant in Albany, New York. Dr. Doi decided to conduct a detailed study on central nervous system (CNS) symptoms/disease in young hogs which ran for 15 months (January 1979 to March 1980). The study consisted of extensive observations of animals, followed by pathological, histopathological, and microbiological work on tissues from various organs of particular animals after slaughter.

The brains of 60 animals were examined. Examination was performed by Dr. Karl Langheinrich, Pathologist-In-Charge at USDA's Eastern Laboratory in Athens, Georgia. According to the USDA FSQS laboratory report, dated early November, 1979, Dr. Langheinrich noted similar brain defects between the pigs' brains and the brains of other animals infected with TSEs. Indeed, the doctor's main diagnosis was encephalopathy and diffuse gliosis of undetermined etiology. Portions of the brains were sent for microbiological testing to a neurologist at the University of Georgia, where they came up negative for pseudo-rabies. The brain matter was unique enough that USDA scientists, Dr. Langheinrich and Dr. Doi, have mentioned it to students and scientific colleagues over the years.

Both the behavior of the pigs, as well as the histopathology on at least one pig, showed signs consistent with a porcine TSE. Behavioral changes can be seen in TSE-infected animals before any changes in brain morphology are visible. Dr. Clarence Joseph Gibbs, in testimony before a Congressional hearing on the TSE issue on January 29, 1997, made just this point In the mid-1960s, we demonstrated with our French and English collaborators that during the early incubation of the TSEs, when the virus titer in the brain was very low, there were already marked functional changes, even though no pathology was yet detectable, even ultrastructurally. 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 10-5 or higher."

In November, 1996, USDA sent the single histopathology slide to Dr. William Hadlow, one of the foremost spongiform encephalopathy pathologists in the world. Dr. Hadlow reported: I am impressed, though, with what seems to be an increase in the number of astrocytes in the section. Some astrocytes are in clusters, some are enlarged and vesicular. Where they are most numerous, a few rod cells (activated microglia) are seen. These findings suggest some perturbation of the nervous tissue. Although such a glial response occurs in the transmissible spongiform encephalopathies, I do not know its significance in this case without examining other parts of the brain for changes characteristic of these diseases. Thus, from looking at this one (1) section of brain, I cannot conclude that the pig was affected with a scrapie-like spongiform encephalopathy. (Emphasis added.)

In recounting the quality of the slide, Dr. Hadlow stated, "It was a bum slide. There was no evidence to make any diagnosis." Months later when Dr. Hadlow received additional slides he recalls that those slides were poorly prepared as well. Thus, Dr. Hadlow does not conclusively rule out a form of porcine spongiform encephalopathy, but rather this type of evidence suggests similarities to spongiform encephalopathies. England has developed more restrictions on what can be put into the rendering mix as science has learned more about how TSE can be transmitted. The United States however, continues to allow heads and backbones of cows, pigs and other animals. Considering the scientific evidence that pigs carry TSEs and further considering the risk to people who consume porcine products, Petitioners request that regulatory changes be made so that porcine protein material cannot be used in animal feed.

5. Animal Feed Labeling Requirements Must be Expanded to Include Other Species Beside Ruminant Species Since Other Species Get TSEs

Regulations addressing the labeling of animal feeds containing high risk TSE material should be amended as follows:

(I) Label the materials as follows: "Do not feed to cattle or other ruminants animals"

Labeling requirements must be changed to "Do not feed to animals" because the causative agent of TSE is unknown. The FDA's regulation contains a loophole for the rendering industry allowing the selling of TSE contaminated material to other animal food producers, such as pet food manufacturers. The theory behind a possible origin of TSEs is generally attributed to Dr. Clarence Joseph Gibbs, Jr. This "Gibbs Hypothesis" states that TSE occurs "spontaneously" as a natural mutation in mammals including humans. Usually, these odd, very rare, spontaneous cases would not be noticed or passed on. However, the ritual of butchering human bodies and cannibalism of the Fore People of New Guinea is a model for how a disease can spread when a species consumes its own members. This example shows why TSE infected material should not be fed to animals. The prion gene that causes TSEs has been found in all mammals studied so far. TSEs also have been found, or experimentally induced in many mammals including sheep, pigs, goats, cattle, deer, elk, mink, mice, hamsters, guinea pigs, domestic cats, puma, cheetah, eland, kudu, Arabian oryx, nyland, marmosets, macaques, chimpanzees and humans. Vulnerable species now include salmon and Drosophila (fruit flies). Considering the number of susceptible animals to TSEs, the agency should change its regulation to "Do not feed to animals" in order to ensure the TSEs do not affect public health and spread throughout all U.S. domesticated animals.

a. TSEs Infect Cats and Cat Food Must be Safe from TSE Contamination

In 1990, a cat named Max was the first feline to die of CJD in England. The chief veterinary officer stated that there was "╬[N]o cause for alarm at all. . . . This is only one cat death out of 7 million cats in the U.K.'" Over the course of only the next four years, sixty-six domestic cats would die before the government admitted that contaminated pet food probably killed them. "By 1 July 1998, 85 cats had succumbed to FSE in GB, 1 in Northern Ireland, 1 in Norway and 1 in Liechtenstein (both homebred cases). In light of the BSE epidemic in other countries, the FDA should change its rules to "Do not feed to animals."

b. TSEs Likely Infects Dogs and Dog Food Must be Safe from TSE Contamination

In England, the pet food manufacturers voluntarily introduced a ban on all specified offal thought to be high risk for TSE in 1989. Later, in 1990, subsequent to the death of the first feline victim, "Max," the government codified that ban. Since that time, 85 cases of cat TSE (Feline Spongiform Encephalopathy FSE) have been found. Over 400 hound dogs were examined in 1991, and although there were allegations that the British government covered up the findings, it appears that dogs do get TSE. Additionally, the Gibbs Hypothesis indicates that dogs' brains will spontaneously mutate the disease at a rate of about one-in-a-million, and they are thus susceptible under the hypothesis. Consequently, dogs should be protected from contaminated food.

c. Some Animals Might be Carrier Species

In order to adequately protect public health, agencies must approach the disease as a uniform TSE disease rather than addressing subgroups or different strains of TSE. This disease is so dangerous because of its likely tendency to produce new strains or new variants in different species just as BSE is presumed to have led to nvCJD. Out of all the variations of differing strains entering differing animals, no one knows just which ones will infect and kill humans. The agencies must also consider that some animals might merely be carrier species and not become ill themselves. When scientists injected scrapie into hamsters and then injected infectious agents from the hamsters' brains into mice, the mice did not get a TSE. Indeed, when those mice brains were injected into a second set of mice, the second set did not get scrapie, either. However, when the second set of mice brains were injected back into hamsters, the hamsters did indeed become infected with a TSE.

In an analogy, the "British BSE-nvCJD" strain of TSE could be carried by pigs without the pigs showing symptoms of infection, yet later if the pigs parts were fed to cows in an animal feed supplement, the cows could become infected with that TSE which has infected and killed humans. Scientific experts on medical properties of TSE have determined "that the results presented here would strongly favour a decision to stop feeding ruminant-derived products to all animal species" especially domestic animals, poultry and animals raised to be consumed by humans since the poultry or domestic animals put into animal feed supplements could be carriers.

Indeed, scientists have expressed their concern about this potential spread of the disease: [F]arm animals that do not contract overt disease after consuming ruminant-derived meat and bone meal may, perhaps, develop a subclinical carrier state. Pigs and chickens that have been fed with cattle-derived meat and bone meal are thought to be safe to eat with respect to BSE, because these animals do not develop disease after oral exposure to bovine prions. But, to the best of our knowledge, bovine prions from BSE-exposed pigs and poultry have never been assayed using calves as ╬indicator' animals.

The FDA has been put on notice by scientists that it is not just cows that present the risk of spreading infectious TSEs. The agencies must consider a broader view of prohibiting mammals from being fed to other mammals. Evidence shows that TSE has been experimentally induced in animals, and that "British BSE-nvCJD" has been likely transmitted from cows to people via ingestion of beef. Additionally, laboratory studies prove that some species can be carriers. Moreover, the "Gibbs Hypothesis" indicates that TSEs will occur at very low levels as a sporadic mutation rather than as an a result from an infectious agent. Thus, there are many avenues of contagion and infection. Any feeding of any animal to any other animal will exponentially increase the spread of this deadly disease. The scientific evidence shows that variety of risks the rendering industry is subject to and the agencies must change the regulations to stop the feeding of TSE contaminated mammals or mammals parts to any other mammal.

6. Animal Feeds Must Not Contain Any Part of Any Animal Showing Signs of a TSE and Nor May Any Such Contaminated Material Be Used as Fertilizer, Cosmetics, or Other Products

The rules promulgated by the agencies to date have not adequately addressed the risk to human and animal health. The rules arbitrarily define protein to exclude hundreds of types of animal parts and proteins from different species. To address loopholes, petitioners request that the current regulations be amended as follows: (a) Definitions (1) Protein derived from mammalian tissues means any protein-containing portion of mammalian animals, excluding: Blood and blood products; gelatin; inspected meat products which have been cooked and offered for human food and further heat processed for feed (such as plate waste and used cellulosic food casings); milk products (milk and milk proteins); and any product whose only mammalian protein consists entirely of porcine or equine protein.

The current rule excludes a few types of protein from rendering while allowing many types of proteins from many species into animal feed. However, this rule is expected to "establish . . . a flexible system of controls and is designed to ensure that ruminant feed does not contain animal protein derived from mammalian tissues." That goal is not being met by the current rules while animal parts which could carry TSEs are allowed to be used by the rendering industry. It only takes one contaminated animal part in a particular batch of bone meal or other animal food stuff to initiate a huge dispersal of infectious agent and spread the disease. This loophole must be closed by removing the exceptions to the definition of protein. It is imperative that the United States agencies address the scope of the problem at hand. The appropriate response is for the agency to promulgate a precautionary rule addressing all TSEs. The Congressional Research Service of the Library of Congress has stated: [A] case can be made that the scientific uncertainties suggest action to control feeding practices that may add to the possibility of cattle contracting BSE. Given the uncertainties, many believe it is safest to err on the side of caution until more of the questions on BSE and CJD are answered.

British beef laws have demanded that mammals no longer be fed to mammals. To comport its regulations with the those in other countries, the United States must acknowledge that animal parts which might carry TSE infection, not merely BSE infection, must not be used as fertilizer, cosmetics or other products. The WHO recommendations stated that no part or product of any animal which has shown signs of a TSE should enter the human or animal food chain. The WHO explains that many animals that are used as raw material for rendering have never been inspected and where inspected some parts of ruminants still make their way into animal feed. The recommendation requires all countries to ensure the killing and safe disposal of all parts of products of such animals so that TSE infectivity can not enter the food chain. Meanwhile, the U.S. agencies optimistically overemphasize that "[British style] BSE has NOT been found in the United States."

However, BSE is only one type of TSE. As noted, there are many other diseases under the classification TSE, including, sheep scrapie, mink encephalopathy and a deer and elk disease called Chronic Wasting Disease (CWD). These TSEs have been known to exist in the United States for decades. The agencies must expand their rulemaking beyond British BSE-nvCJD, which is merely one facet of a disease that does not acknowledge a species barrier.

Moreover, the USDA overstates its case when it alleges that there is no BSE in the United States. It should be noted that one particular BSE positive cow from England was reported in Canada. Additionally, the FDA is aware that mink TSE has been transmitted to bulls which then showed symptoms of "downer cow syndrome" and died of TSE. There is scientific evidence that BSE will exist in cows at the same sporadic rate that it exists in humans, at about one in a million animals. Therefore, it is fallacious and misleading to contend that there is no BSE in the United States. Certainly, some BSE exists, at least in its sporadic variation or perhaps as "downer cow syndrome," even if not as "British BSE-nvCJD".

Moreover, the question as to whether or not BSE exists in the United States is really a question of surveillance. We've had one reported case of BSE in North America -- in Canada, in a cow imported from the United Kingdom. That cow was found by a rancher who had gone out on the range to feed his herd because of a severe snowstorm. If there had been no storm, the rancher would have stayed home, the cow would have gone down, a coyote would have eaten it, and no one would have been any the wiser.

These facts have led Dr. Clarence Joseph Gibbs, a TSE researcher, to query, "Do we have BSE in the United States? The real question is, if we do, will we find it?" Furthermore, agencies' allegation that there is no BSE in the United States must be viewed in light of the fact that the number of BSE tests has been "phenomenally small." So far the USDA has only tested 2,000 animals out of 7,100 dead suspected cows.

Considering that other countries have banned feeding of any possible TSE carriers to other animals, and further considering that it is likely that at least sporadic BSE exists in the United States, as well as the fact that surveillance for "British BSE-nvCJD" is poor, the agencies must implement a rule keeping animals showing signs of TSEs from being used in products such as food additives, fertilizers, and cosmetics.

VII. Environmental Impact & Certification

The enforcement actions here requested will not cause the release of any substance into the environment. They are categorically excluded from the requirement of environmental documentation under 21 C.F.R. 25.33(g).

The undersigned certify that, to the best knowledge and belief of the undersigned, this petition includes all information and views on which the petitions relies, and that it includes representative data known to the petitions which are unfavorable to the petition. Except as described above, petitioners know of no other similar issue, act, or transaction to this petition currently begin considered or investigated by any HHS office, other federal agency, department, or instrumentality, state municipal agency, court or any law enforcement agency consistent with FDA regulation 21 C.F.R. ■ 10.30(e)(2).

VIII. Agency Action Requested

Therefore, for the reasons cited in this petition, the reasons incorporated by reference in attorneys' earlier petitions, and because of the extraordinary health threat posed to both cattle and humans by the feeding of rendered animal protein to animals, especially ruminant animals, petitioners request that the FDA and USDA immediately make the following regulatory changes:

1. Amend 21 C.F.R. ■ 589.2000 ("Substances Prohibited From Use in Animal Food or Feed; Animal Proteins Prohibited in Ruminant Feed" finalized at 62 Federal Register 30,936 (June 5, 1997)) as follows:

a. Amend the rule so that blood and blood products are not fed to animals; b. Amend the rule so that gelatin and gelatin products are not fed to animals; c. Amend the rule so that pig and porcine materials are not fed to animals; d. Amend the rule so that animal feed labeling requirements include other species beside ruminant species, since TSE is not only a ruminant disease and other animals beside ruminants are infected and pass along TSEs; and e. Amend the rule to state that no part of any TSE contaminated animal can be fed to animals or humans and that such contaminated material cannot be used as fertilizer, cosmetics, or other products and further that contaminated material must be destroyed; and f. Expand the record keeping requirements from one year to ten years. 2. 21 C.F.R. ■ 589.2000 "Substances Prohibited From use in Animal Food or Feed; Animal Proteins Prohibited in Ruminant Feed" should read:

(i) Label the materials as follows: "Do not feed to any animal."

(h) Inspection; records retention. (1) Records that are to be made available for inspection and copying, as required by this section, shall be kept for a minimum of 10 years.

Petitioners are requesting a response to this petition within one hundred eighty (180) calendar days. In the absence of an affirmative response, the Petitioners will be compelled to consider litigation in order to achieve the full and complete action required to address this violation of federal law.

Dated this 6th day of January, 1999.

On behalf of all petitioners,
Andrew Kimbrell Executive Director
Joseph Mendelson, III Legal Director

cc:  Daniel Glickman, Secretary 
U.S. Department of Agriculture Room 200-A, Admin. Bldg. 
14th Street & Independence Avenue, S.W. Washington, D.C.  20250

Donna Shalala, Secretary 
U.S. Dept. of Health and Human Services 
200 Independence Avenue, Southwest Washington, D.C.  20201

CDC Receives second petition

6 Jan 99 filing with CDC
CITIZEN PETITION BEFORE THE UNITED STATES
DEPARTMENT OF HEALTH AND HUMAN SERVICES
Department of Health and Human Services
200 Independence Avenue, Southwest
Washington, D.C.  20201
 
HUMANE FARMING ASSOCIATION,
    Post Office Box 3577,
    San Rafael, CA 94912-8902, et al.
Petitioners,

 vs.

Docket No._____________
DONNA SHALALA,
	in her official capacity as,
	Secretary
	Department of Health and Human Services
	200 Independence Avenue, Southwest
	Washington, D.C.  20201
Defendant

Petition Seeking Immediate Action By The HHS And CDC To Combat The Spread Of Transmissible Spongiform Encephalopathy (TSEs) In The United States

TABLE OF CONTENTS
I.   Introduction
II.  Petitioners
III. Statement of the Facts
IV.  Procedural History
V.   Statement of the Law
VI.  Argument

  A. The Department of Health And Human Services Should Implement
a National System for Monitoring All New Strains of TSE, Including "British
BSE-nvCJD" in Cattle because The Current Scientific Evidence Shows That TSEs
Are Transmissible to Humans

		1.The Current Scientific Evidence

  B.TSE is an Infectious Transmissible Disease

		1. Humans May Contract TSE from Wildlife
		2. Humans May Contract TSE from Pork
		3. Humans May Contract TSE from "Downer Cows"
		4. Humans May Contract TSE from Other Humans
		
  C. The Department of Health And Human Services Should Implement
a National System For Monitoring All New Strains of TSE, Including "British
BSE-nvCJD" in Humans Because it Is Necessary to Protect Human Health.
	
...1. Health and Human Services' Statutory Mandate
Requires the Monitoring of TSEs

...2. A National Monitoring Program is Necessary Since CJD
is Commonly Misdiagnosed
			a. The Yale University Study
			b. The University of Pittsburgh Study
			c. Other evidence

...3. New Lab Tests Are Available For Testing TSE

VII.  	Environmental Impact & Certification
VIII.	Agency Action Requested

I. Introduction

Pursuant to the Right to Petition Government Clause contained in the First Amendment of the United States Constitution, the Administrative Procedure Act, and the Health and Human Services (HHS) implementing regulations, the undersigned submits this citizen petition for rulemaking and collateral relief and respectfully requests the Secretary to protect the public from Transmissible Spongiform Encephalopathies (TSEs) by establishing a national monitoring system for testing, studying and reporting on TSEs.

II. Petitioners

1. The Humane Farming Association, an I.R.S. Code 501(c)3 not for profit organization [with 125,000 members -- webmaster], with its principle offices located in the State of California and its mailing address at Post Office Box 3577, San Rafael, California 94912-8902, (415) 485-1495;

2. The Center for Food Safety, a project of the International Center for Technology Assessment, an I.R.S. Code 501(c)3 not for profit organization with its principle offices located in the District of Columbia at 310 D Street, N.E., Washington, D.C. 20002-5722; 202- 547-9359;

3. Center for Media & Democracy, an I.R.S. Code 501(c)3 not for profit organization, with its principle offices located in the State of Wisconsin at 3318 Gregory St.; Madison, WI 53711-1725; 608-233-3346;

4. R. Douglas and Tracie L. McEwen, 4 East 270 South, Kaysville, Utah, 84037. Mrs. McEwen's husband is R. Douglas McEwen, 30, a territory manager for a brokerage until August of 1998, is terminally ill with CJD, in an extremely agonizing decline for him, her, and their two young daughters, ages 3 and 8. He was diagnosed on Nov. 25, 1998 at the University of Utah Hospital in Salt Lake City, Utah after a brain biopsy.

5. Patricia C. Ewanitz, 46-03 216th Street, Bayside, New York 11361; Ms. Ewanitz' husband, Andrew P. Ewanitz, Jr., a engineer and plant facilities manager, died of Creutzfeldt-Jacob Disease (CJD) at Jacob Perlow Hospice, 1st Avenue and 16th Street, New York City on April 9, 1997 after his initial diagnosis was mild stroke;

6. Elizabeth A. Armstrong, 205 S. Cherry Street, Lebanon, Illinois, 62254; Ms. Armstrong's father, Charles O. Butcher, a law enforcement officer, died of CJD on May 6, 1996, at Veterans Administration Medical Center, Richmond, Virginia, after an initial diagnosis of COPD then Parkinson's Disease;

7. Melvin J. Steiger, 5582 Cora Way, Salt Lake City, Utah 84118-2316. Mr. Steiger's wife, Eleanor M. Steiger, 57, an accounting clerk, was initially diagnosed with depression. Symptoms began in 1996 with severe leg muscle cramps. Symptoms eventually included changes in personality where she become less patient and more argumentative, loss of coordination, memory and ability to function. Admitted to the hospital for a possible stroke, Mrs. Steiger was positively diagnosed with CJD on the 19th of February, 1998. On February 23, 1998, a biopsy, an EEG and examination confirmed that diagnosis. She passed away at home on March 19, 1998

8. Beverly Wilson Goodman, 7308 Moody Court, Fort Worth, Texas 76180-6107; Ms. Goodman's father, Perry Marlin Wilson, died of CJD at Zale Lipshey a division of Parkland Hospital, Dallas, Texas, with a diagnosis of "unknown" on May 18, 1997;

9. Mildred B. Campbell, 2229 Hwy. 601-S, Mocksville, North Carolina; Ms. Campbell's husband, John D. Campbell, a retired law enforcement officer, died of CJD at Forsyth Memorial Hospital, Winston-Salem, North Carolina on March 18, 1997 with an original diagnosis of stoke and peripheral neuropathy. CJD was later confirmed by a brain biopsy;

10. Dorothy E. Kraemer, 901 S. Stanley Street, Stillwater, Oklahoma 74074; Ms. Kraemer's mother's cause of death from CJD was confirmed through a brain autopsy after her death at Stillwater Nursing Home on May 16, 1996. The original diagnosis was rapid onset Alzheimer's Disease;

11. Cecile L. Sardo, 17065 N.W. 22nd Street, Pembrook Pines, Florida 33028; Ms. Sardo's husband, Joseph Sardo died of CJD;

12. Jim and Rebecca Goodman, of E103 County Q, Wonewoc, Wisconsin 53968; The Goodman's make their living by farming in the State of Wisconsin;

13. Bruce and Shelley Krug, of Box 84 West Road, Constanbleville, New York 13325; The Krugs are dairy farmers.

III. Statement of the Facts

Transmissible Spongiform Encephalopathy (TSE) is a mysterious classification of diseases that are called by different names in different species. For instance some identified types of TSE are Creutzfeldt-Jakob Disease (CJD) in humans, scrapie in sheep, bovine spongiform encephalopathy (BSE) in British cattle, transmissible mink encephalopathy (TME) in mink in North America, and chronic wasting disease (CWD) in deer and elk in North America. There may be different strains of TSE within species, and new strains may be produced when TSEs move from one animal species to another.

There is vigorous scientific debate as to exactly what the TSE agent is, since it has never been completely isolated, but the leading theory seems to be that it is a mutant or infectious form of a naturally present protein probably present in all mammalian species called a prion protein; the name prion was created by Dr. Stanley Prusiner whose work was awarded a Nobel Prize in 1997.

The common characteristics of TSE diseases are that they are invariably fatal. They can be transmitted through iatrogenic or physician induced exposure, such as corneal transplants, use of dura mater during surgery, contaminated human growth hormone or contaminated probes. Additionally, infection may occur through exposure of infected material to mucous membranes or open cuts, and in some cases through ingestion of the infectious agent. The agent does not trigger an immune response, and the infected animal or human appears perfectly normal until at some point in life (usually the sixth decade in humans who develop sporadic CJD) the disease emerges as holes and amyloid plaque material spreading in the brain, causing symptoms of dementia, physical failure and death.

TSEs can be difficult to diagnose during life; confirmation of TSE disease is usually made post-mortem through autopsy analysis, or through a newly-developed spinal fluid test, and can be confirmed by passaging the infectious agent from the deceased specimen into the brain of a laboratory animal.

Prior to the outbreak of BSE in British cattle in the mid 1980s, and its spread to the human population as nvCJD which has claimed to date 35 lives, the best known TSE was sheep scrapie. It appeared in Spanish sheep in the 18th century, and has since appeared in most countries that raise sheep. Sheep scrapie appeared in U.S. sheep in the late 1940s, via Canadian sheep of British origins. After the failure of U.S. eradication efforts, sheep scrapie has spread across the United States and its actual occurrence is unknown.

Prior to the appearance of nvCJD in Britain, the best known occurrence of a TSE in humans was the disease kuru, a TSE that appeared in the Fore tribe in New Guinea in the early 20th century, spread by rituals involving cannibalism until the recognition of that fact in work by Dr. Daniel Carleton Gajdusek and Dr. Clarence Joseph Gibbs of the U.S. National Institutes of Health. For this work Gajdusek received the first Nobel Prize awarded for TSE research.

When British BSE, dubbed 'mad cow disease', appeared in the mid 1980s, it stood out because of the madness symptoms of the animals in a rabies-free nation. From just a few animals the disease rapidly spread to infect hundreds of thousands. By 1988 epidemiology demonstrated that the disease was in fact being spread by the feeding of rendered animal byproducts as a protein and fat feed supplement. British cattle were consuming the remains unfit for human consumption of sheep, cattle and other animals. While the high prevalence of sheep scrapie in Britain has led to presumptions that British BSE is sheep scrapie in cattle, there is no proof of this. Others suspect that BSE may have emerged from the feeding of a small number of BSE infected cattle to other cattle. In any case, the feeding of infected cattle back to healthy cattle clearly amplified and spread BSE within British cattle, whatever the origins of the TSE agent.

The practice of feeding rendered animal byproducts back to livestock began in the 19th century, and became a large widespread practice involving billions of pounds of rendered animal fat and protein from the 1960s to the present. The practice continues today, although with some recent restrictions due to the spread of BSE and the emergence of nvCJD.

Despite the British outbreak, the practice of feeding rendered livestock meat and bonemeal back to livestock has been most widely practiced in the United States, where no meaningful restrictions were even attempted until August, 1997. Today, the U.S. lags behind Britain and Europe in implementing safeguards in the feeding of animals, and allows practices that should be banned, such as feeding pigs to pigs, pigs to cattle, and cattle blood protein to calves. These practices are allowed despite evidence that TSE disease may already infect pigs and cattle in the U.S., and despite the existence of proven TSEs in U.S. sheep, deer, elk and mink.

Indeed, Dr. Clarence Joseph Gibbs theorizes that every mammalian species is likely to have individuals with a TSE disease at some low level, perhaps one in a million. Therefore, any cannibalistic feeding practices might amplify and spread the disease, as in Britain, possibly creating a TSE that can jump from one species to another, as has apparently happened in the U.K. with the spread of BSE into humans as nvCJD. While 35 diagnoses of nvCJD to date seems a small number, the British government admits that the virtually invisible disease nvCJD may have an incubation period measured in decades, and therefore it will require the passage of many additional years to determine the extent of the disease and to predict the eventual death toll. Estimates have ranged from scores to hundreds of thousands eventual deaths from nvCJD in Britain.

CJD exists in the United States, but it is unclear at what level. Most CJD in the U.S. is considered 'sporadic' with an unknown cause. A smaller percentage appears to have a genetic factor. The U.S. has a large population of persons with various dementia diseases, the most often diagnosed being Alzheimer's (four million cases). There is no routine postmortem diagnosis of dementia deaths, although autopsy is the only sure way to determine what type of dementia a patient had. It appears that one of four dementia diagnoses made during life are actually incorrect, with another type of dementia causing the death.

Studies of people in the U.S. who have died of dementia suggest much higher levels of CJD than commonly suspected, with proven levels of anywhere from 1 to 13% of dementia victims. Individuals whose family members have died of CJD report great difficulties in receiving a correct diagnosis. Their recent activism through grassroots organizations such as CJD Voice, and the recognition that CJD seems much more common than thought, has led some states (Texas, Utah, Ohio and Kentucky) to begin making reporting of CJD mandatory. Without specific new programs to investigate and identify CJD cases, the true number cases and whether they are increasing will remain extremely difficult to document.

Given what is now known about the ability of TSE diseases to move between species and infect humans, the existence of animal TSEs in the United States, and the long and invisible incubation period during which a human or animal CJD strain might become epidemic, it is imperative that state and federal agencies implement programs for accurately determining and monitoring the number of TSE cases in humans and animals in the United States.

One petitioner, R. Douglas McEwen, a thirty-year old Utah man is terminally ill with diagnosed CJD. Mr. McEwen's young age makes this an extremely unusual occurrence; British nvCJD came to public attention because of the similarly young age of its victims. Mr. McEwen was an avid consumer of deer and elk meat, and some areas of the western U.S., including the area where Mr. McEwen hunted, have been shown to have very high levels (1% to 6% in tested individuals) of chronic wasting disease (CWD) in deer and elk, a TSE apparently unique to North America.

It is possible that Mr. McEwen contracted CWD through contact with or consumption of infectious deer or elk. Whether or not this is the case, his CJD emphasizes the critical importance of taking all prudent steps to prevent the spread of TSEs in animals and people, and in determining and monitoring the existence of these diseases in the United States.

Human health concerns are amplified by the many unknowns surrounding CJD, including the invisibility of the agent and its infectious routes. For instance, contaminated medical instruments are impossible to adequately disinfect and have spread TSEs to healthy patients, as have cadaver-source tissue and organs. In laboratory tests blood can transmit TSEs, and in Britain the use of British plasma has been curtailed because of concerns that it might spread nvCJD.

Mr. McEwen, the Utah CJD patient, was reportedly a plasma donor. Even in the United States, the FDA has recently been advised by scientists to limit blood donors to those who have not been exposed to Mad Cow Disease in England. This suggestion would mean that anyone who has visited Britain since 1980 could no longer give blood. Further, Health Canada has recently been advised to quarantine blood which may be contaminated with infectious CJD from Mr. McEwen who was dedicated to giving plasma on a regular basis.

In summary, the emergence of infectious TSEs that can move between species is a new and unanticipated public and animal health threat that is being inadequately studied and addressed in the United States. This petition seeks to remedy those failures.

IV. Procedural History

Considering the new emerging science of TSE as well as the new evidence presented in this petition, the petitioners request the HHS, including the FDA and the CDC, to immediately undertake the following actions to prevent the potential spread of transmissible spongiform encephalopathy (TSE), including bovine spongiform encephalopathy (BSE, also known as "Mad Cow Disease"), (CWD) Chronic Wasting Disease, and Creutzfeldt-Jacob Disease (CJD):

(1) initiate a significant epidemiological investigation to determine the incidence of transmissible spongiform encephalopathies among the human population of the United States; and

(2) develop an ongoing national monitoring and registry program utilizing autopsy examinations to determine any changes in the incidence of CJD-like diseases among the human population of the United States; and

(3) direct all state medical officers to engage in a reporting process, similar to the reporting requirements of other infectious and transmittable diseases, to the CDC for the purposes of establishing a national cumulative database and reporting system.

Petitioners incorporate by reference information submitted to the FDA through previous petitions dated June 23, 1993, December 21, 1993, March 26, 1996, March 27, 1996, and a petition dated January 6, 1999 to the FDA, copied to this agency. Petitioners also incorporate by reference the agency actions requested in those previous petitions. Petitioners also incorporate by reference information submitted to the agency in a previous petition dated March 27, 1997, by the Government Accountability Project which demanded that the laws and regulations that keeps pigs with TSEs and nervous system disorders from entering the food supply are fully enforced.

Petitioners further incorporate by reference information submitted to the agency in a previous petition and supplemental petition dated March 4, 1998 by Farm Sanctuary and Michael Baur that requested that the Food and Drug Administration (FDA) and the United States Department of Agriculture (USDA) immediately label all downed cattle and other livestock as adulterated pursuant to 21 U.S.C. ■ 342(a) in order to protect the health of the nation by reducing the potential of Transmissible Spongiform Encephalopathies (TSEs), bovine spongiform encephalopathy (BSE), also know as "Mad Cow Disease," (CWD) Chronic Wasting Disease and Creutzfeldt-Jakob Disease (CJD).

On June 23, 1993, petitioning attorneys submitted an "Amended Petition Requesting the Food and Drug Administration to Halt the Feeding of Ruminant Animal Protein to Ruminants" on behalf of several individuals and a non-profit organization. The petition requested the Food and Drug Administration and the United States Department of Agriculture to undertake agency actions that would address the potential health threat posed to U.S. animal herds, especially cattle, from transmissible spongiform encephalopathies (TSEs) and the threat posed to U.S. meat consumers, especially beef consumers, through the potential zoonotic development of Creutzfeldt-Jakob Disease (CJD) as a result of eating TSE-contaminated meat.

On behalf of the same individuals and organizations, petitioning attorneys submitted a "Supplemental Petition Requesting the Food and Drug Administration to Halt the Feeding of Ruminant Protein to Ruminants." On December 21, 1993, the supplemental petition was filed as result of, inter alia, the FDA's failure to respond to the June 23, 1993 petition within the one hundred and eighty (180) days mandated by 21 C.F.R. ■ 10.30(e)(2).

In a much belated response to the two petitions, in 1994 the FDA issued a proposed rule in the Federal Register stating that specified offal from adult sheep and goats (of more than 12 months of age) is not generally recognized as safe for use in ruminant feed and is an unapproved food additive when added to ruminant feed. In June 5, 1997, a limited rule was promulgated by the FDA providing that a few certain types of animal proteins would not be used as raw materials in the rendering industry.

Despite petitioners continuing requests, the FDA has failed to promulgate a rule that will adequately protect animal and human health from TSEs in general and BSE in particular.

V. Statement of the Law

A. The Food and Drug Administration, Department of Health and Human Services, Subchapter L; Regulations Under Certain Other Acts Administered by the Food and Drug Administration, Part 1240, Control of Communicable Disease, Subpart B, Administrative Procedures states: Whenever the Commissioner of Food and Drugs determines that the measures taken by health authorities of any State or possession (including political subdivisions thereof) are insufficient to prevent the spread of any of the communicable diseases from such State or possession to any other State or possession, he [sic] may take such measures to prevent such spread of the diseases as he [sic] deems reasonably necessary, including inspection, fumigation, disinfection, sanitation, pet extermination, and destruction of animals or articles believed to be sources of infection.

21 C.F.R. ■ 1240.30 (1998).

B. Title 42. The Public Health And Welfare, Chapter 6a, The Public Health Service, General Powers And Duties, Research And Investigations, 42 U.S.C.S. ■ 241 (1998), Research and Investigations Generally:

(a) Authority of Secretary. The Secretary shall conduct in the Service, and encourage, cooperate with, and render assistance to other appropriate public authorities, scientific institutions, and scientists in the conduct of, and promote the coordination of, research, investigations, experiments, demonstrations, and studies relating to the causes, diagnosis, treatment, control, and prevention of physical and mental diseases and impairments of man, including water purification, sewage treatment, and pollution of lakes and streams. In carrying out the foregoing the Secretary is authorized to--

(1) collect and make available through publications and other appropriate means, information as to, and the practical application of, such research and other activities;

(2) make available research facilities of the Service to appropriate public authorities, and to health officials and scientists engaged in special study;

(3) make grants-in-aid to universities, hospitals, laboratories, and other public or private institutions, and to individuals for such research projects as are recommended by the advisory council to the entity of the Department supporting such projects and make, upon recommendation of the advisory council to the appropriate entity of the Department, grants-in-aid to public or non-profit universities, hospitals, laboratories, and other institutions for the general support of their research;

(4) secure from time to time and for such periods as he [sic] deems advisable, the assistance and advice of experts, scholars, and consultants from the United States or abroad;

(5) for purposes of study, admit and treat at institutions, hospitals, and stations of the Service, persons not otherwise eligible for such treatment;

(6) make available, to health officials, scientists, and appropriate public and other nonprofit institutions and organizations, technical advice and assistance on the application of statistical methods to experiments, studies, and surveys in health and medical fields;

(7) enter into contracts, including contracts for research in accordance with and subject to the provisions of law applicable to contracts entered into by the military departments under title 10, United States Code, sections 2353 and 2354, except that determination, approval, and certification required thereby shall be by the Secretary of Health, Education, and Welfare;

(8) adopt, upon recommendations of the advisory councils to the appropriate entities of the Department or, with respect to mental health, the National Advisory Mental Health Council, such additional means as the Secretary considers necessary or appropriate to carry out the purposes of this section.

The Secretary may make available to individuals and entities, for biomedical and behavioral research, substances and living organisms. Such substances and organisms shall be made available under such terms and conditions (including payment for them) as the Secretary determines appropriate.

(b) Testing for carcinogenicity, teratogenicity, mutagenicity, and other harmful biological effects; consultation.

(1) The Secretary shall conduct and may support through grants and contracts studies and testing of substances for carcinogenicity, teratogenicity, mutagenicity, and other harmful biological effects. In carrying out this paragraph, the Secretary shall consult with entities of the Federal Government, outside of the Department of Health, Education, and Welfare, engaged in comparable activities. The Secretary, upon request of such an entity and under appropriate arrangements for the payment of expenses, may conduct for such entity studies and testing of substances for carcinogenicity, teratogenicity, mutagenicity, and other harmful biological effects. (Italics and bold added for emphasis.)

C. Title 42. The Public Health And Welfare Chapter 6a. The Public Health Service General Powers And Duties Federal-State Cooperation, 42 U.S.C.S. ■ 243 (1998), General grant of authority for cooperation:

(a) Enforcement of quarantine regulations; prevention of communicable diseases. The Secretary is authorized to accept from State and local authorities any assistance in the enforcement of quarantine regulations made pursuant to this Act which such authorities may be able and willing to provide. The Secretary shall also assist States and their political subdivisions in the prevention and suppression of communicable diseases and with respect to other public health matters, shall cooperate with and aid State and local authorities in the enforcement of their quarantine and other health regulations, and shall advise the several States on matters relating to the preservation and improvement of the public health.

(b) Comprehensive and continuing planning; training of personnel for State and local health work; fees. The Secretary shall encourage cooperative activities between the States with respect to comprehensive and continuing planning as to their current and future health needs, the establishment and maintenance of adequate public health services, and otherwise carrying out public health activities. The Secretary is also authorized to train personnel for State and local health work. The Secretary may charge only private entities reasonable fees for the training of their personnel under the preceding sentence.

(c) Development of plan to control epidemics and meet emergencies or problems resulting from disasters; cooperative planning; temporary assistance; reimbursement of United States. (Italics and bold added for emphasis.)

D. Title 42. The Public Health And Welfare Chapter 6a. The Public Health Service General Powers And Duties Quarantine And Inspection, 42 U.S.C.S. ■ 264 (1998), Regulations to control communicable diseases:

(a) Promulgation and enforcement by Surgeon General. The Surgeon General, with the approval of the Administrator [Secretary], is authorized to make and enforce such regulations as in his judgment are necessary to prevent the introduction, transmission, or spread of communicable diseases from foreign countries into the States or possessions, or from one State or possession into any other State or possession. For purposes of carrying out and enforcing such regulations, the Surgeon General may provide for such inspection, fumigation, disinfection, sanitation, pest extermination, destruction of animals or articles found to be so infected or contaminated as to be sources of dangerous infection to human beings, and other measures, as in his judgment may be necessary. (Italics added for emphasis.)

E. The Public Health and Welfare regulations Chapter 6A, Public Health Service, General Powers and Duties Injury Control, 42 U.S.C.S. ■ 280b Research (1998), states

(b) The Secretary, through the Director of the Centers for Disease Control and Prevention, shall collect and disseminate, through publications and other appropriate means, information concerning the practical applications of research conducted or assisted under subsection (a). In carrying out the proceeding sentence, the Secretary shall disseminate such information to the public, including elementary and secondary schools. (Italics and bold added for emphasis.)

F. Administrative Procedure Act, 5 U.S.C. Section 706, Scope of Review. To the extent necessary to decision and when presented, the reviewing court shall decide all relevant questions of law, interpret constitutional and statutory provisions, and determine the meaning and applicability of the terms of an agency action. The reviewing court shall Ď

(1) compel agency action unlawfully withheld or unreasonably delayed; and

(2) hold unlawful and set aside agency action, findings and conclusions found to be -- (A) arbitrary, capricious, an abuse of discretion, or otherwise not in accordance with law.

VI. Argument

A. The Department of Health and Human Services Should Implement a National System for Monitoring all New Strains of TSE, Including "British BSE-nvCJD" in Humans Because the Current Scientific Evidence Shows That TSEs are Transmissible to Humans.

1. The Current Scientific Evidence TSE is not the first animal disease to cross the species barrier and affect humans. Many other human infectious diseases started in animals. For example, measles is related to canine distemper and influenza is related to a porcine disease. Usually, related diseases exist between humans and domesticated animals. The call for agency action to protect the United States public has come from science and policy experts who have studied TSEs around the world.

The number of hypothetical risks from these novel disease agents seems endless. They could pop up in medicines, organ transplants, in gelatine (which is used in everything from dessert mixes to medicine gel-caps), or in garden fertilizer made from rendered bone meal. Government and industry officials worry that public discussion of hypothetical risks could trigger unnecessary panic . . . What we need is good data, and in the meantime we need serious implementation of measure to prevent the disease from spreading Ď not just surveillance that will only alert us to tragedy after it has already arrived. We need the precautionary principle.

Just this year, the largest scale case control epidemiological study of sporadic CJD (not nvCJD) comprised of 405 CJD patients linked sporadic CJD to consumption of animal products, especially brains. Although this is a European study, there is no reason to suspect that the results would be different in the U.S., particularly in light of the small case control epidemiological studies done in the U.S. Those smaller U.S. studies also link sporadic CJD to the eating squirrel brains and pork products. This means the FDA policy that only "British BSE-nvCJD" poses a health risk to humans and animals may be incorrect. In fact, sporadic CJD may also be linked to consumption of American animal products as well.

Dozens of people have died in England because of this disease. In fact, several people died before the English government admitted the "mad cow's" disease and human nvCJD were related. To date, there is no other infectious disease agent like TSE; it is an entirely new contagion. Although viruses build up over hours, days, or months until the body's immune system is activated, TSE does not illicit an immune response or any outward symptoms of infection until the patient is terminal.

As the TSE causative agent is not conclusively known, the agency should use the precautionary principle to protect the public by putting into effect a cumulative TSE monitoring and reporting system until the scope of the disease is known and the extent of a United States epidemic is understood. The FDA has been advised by some of the world's leading TSE scientists, including Dr. Stanley Prusiner who won the Nobel Prize for his scientific discoveries involving TSEs, to bar blood donations from people who lived in or visited Britain. "The [scientific advisors] said blood banks should survey donors to find out if they lived or spent up to a year in Britain from 1980 to the present."

Health Canada has advised the Canadian Blood Services and Hema-Quebec to put a temporary hold or quarantine on blood products. At present, Canada is being told not to distribute any of the designated blood products on their shelves and it is possible that the blood that has not already been used on patients might be withdrawn and destroyed.

Public health agencies have been cautioned by scientists and have been "put on notice" that mere surveillance for one brain disease or pathology is not sufficient. Last summer the Yale researcher Laura Manuelidis showed the CJD can evolve into more virulent strains by being passed from human beings to mice, from there to hamsters, and finally to rats. Her paper, published in the July 4, 1997, issue of the journal Science, concluded that when the agent changed, it provoked a variant disease, and warned that public-health agencies should be on the watch for diverse signs of brain pathology in human beings Ď not only those symptoms known to be associated with the new variant of CJD. Manuelidis's concern is that if a new variant of CJD has appeared in the United States, we will be slow to realize it, because we are looking for the variant that appeared in Great Britain.

While "sporadic CJD" has been around for some time, a new variant dubbed, "nvCJD", has been linked to the consumption of TSE-infected cattle products in England ("British BSE-nvCJD"). The scientific evidence shows that agencies must be on the watch for different and new variations of CJD.

B. TSE is an Infectious Transmissible Disease

1. Humans May Contract TSE from Wildlife

TSEs are undisputably present in wildlife in the United States. A specific variation of wildlife TSE, called Chronic Wasting Disease (CWD), has been found in various deer and elk for at least the past 30 years.

CWD is insidious because the method of spread is unknown and it has a long incubation period. Furthermore, there is no test available to detect infection in a live animal; only long quarantines and postmortem tests can be used. The recent rash of cases in captive elk, coupled with the complex web of animal traffic that has been associated with the rapid growth of elk ranching, has created a strong possibility that things are going to get worse with CWD.

The United States Animal Health Association Wildlife Diseases Committee has recommended resolutions and recommendations for a Model Program for Surveillance, Control, and Eradication of CWD in Domestic Elk. However, the HHS has no such model recommendations for CJD in humans. In 1996, in portions of Colorado where hunters were required to turn over the heads of mule deer and elk, four- to six-percent of mule deer and one-percent of the elk population killed by hunters where shown to have indications of CWD. These are extremely high numbers for what was once considered a rare disease.

These numbers indicate that the disease is at an epidemic proportion in some areas of the Rocky Mountains. Since the disease can only be detected at the end of a long incubation period, the true incident of CWD in the mule deer and elk population is ultimately much higher. The Wyoming Game and Fish Department has produced a video titled "Chronic Wasting Disease of Deer and Elk" which cautions hunters against consuming visibly ill animals. Animals in the early stages of infection, not exhibiting symptoms, will test negative for signs of CWD; this does not mean they are not infected and infectious, it merely shows that the disease is not being detected by test. In June of this year, CWD was diagnosed in a captive elk in Oklahoma. In 1998 alone, three additional states have discovered CWD: Nebraska, Oklahoma and South Dakota.

One case control epidemiology study has suggested that exposure to deer might be associated with a health risk to humans. Specifically, exposure to deer via hunting leads to an increased risk of contracting CJD. This study indicates that those exposed to deer were nine times more likely to later develop CJD. Indeed, one petitioner from Utah, currently suffering from CJD, is 30-year old Utah man who has hunted elk in Southern Wyoming where CWD in elk is known to occur. This is an unusually young age for classic, sporadic CJD. This tragic situation must be addressed by the agencies through monitoring and reporting procedures as well as possible case studies. Petitioners plea for the agencies to recognized these problems of CWD in light of the larger issues of TSEs and begin a significant epidemiological investigation to determine the incidence of TSE in U.S. humans and animals.

Indirect evidence exits that TSEs occur in other wildlife as well. In a 1984 case control epidemiological study, four CJD victims had eaten wild animal brain: one patient has eaten wild goat and three other patients had consumed wild squirrel brain; all four developing CJD. More recent evidence exits that squirrel brains might be particularly risky. "In the last four years, 11 cases of a human form of transmissible spongiform encephalopathy, called Creutzfeldt Jakob disease, have been diagnosed in rural western Kentucky . . . 'All of them were squirrel-brain eaters.'" Another article indicates this squirrel disease is being linked as a possible cause to the infection of five different people who ate squirrels in different areas of Kentucky. "In a letter to The Lancet, [Dr. Joseph Berger, a neurologist at the University of Kentucky] . . . advise[d] that ╬caution might be exercised in the ingestion of this arboreal rodent.'" That doctor went on to bemoan the lack of clear data available on the disease. "CJD has been a reportable disease in Kentucky only for the last year and a half, so we do not have enough data to judge whether the incidence of CJD in the state is higher than elsewhere, though that is my impression." Petitioners request that the agencies begin a national reporting and monitoring system so that different parts of the country can begin to assess whether the incidence of the disease is higher in one region or another so that the infection can be traced.

CJD researchers have determined that as the disease-causing substance infects different species, the actual substance itself might change. Furthermore, some species may just be "carriers" for the disease, which may mutate into new variations. Due to the fact that there is so little certainty even amongst the experts in this field, it is imperative that the Federal agencies compile data on TSEs. The identification of the possible risk between CWD in cervids, such as elk and deer, and TSE in squirrels, and the relationship between human nvCJD to BSE indicates that the disease is of extreme importance to the public health community. Therefore, United States health agencies need to monitor not just "sporadic CJD" or "British BSE-nvCJD", but must monitor all TSEs.

2. Humans May Contract TSE from Pork

Epidemiological evidence exists that TSE in pork is linked to CJD in humans. This has been alleged by three studies, two from the U.S. and one from Europe. The first study involved 38 CJD patients and stated that one-third of those CJD patients had eaten animal brains. Of all patients who consumed brains, those who consumed brains frequently with a preference for hog brains were at a higher risk of CJD compared to a control group who also ate brains. The second study involved 26 victims of CJD. That study found that nine out of 45 food items were statistically linked to the risk of CJD infection. Of those nine, a total of six came from pigs. "An increased consumption among [CJD] patients was found for roast pork, ham, hot dogs . . ., roast lamb, pork chops, smoke pork and scrapple." The scientists cautioned "[t]he present study indicate[s] that consumption of pork as well as its processed products (e.g., ham, scrapple) may be considered as risk factors in the development of Creutzfeldt-Jakob Disease."

The third study indicated a possible link between brain consumption and sporadic CJD, not nvCJD. In this case control epidemiology study, over four hundred CJD cases were studied and researchers discovered a link between animal consumption and sporadic CJD. This link from animal consumption to sporadic CJD is worthy of notice because it has been presumed that only nvCJD, not sporadic CJD, was caused by animal consumption. In light of the risk of CJD from the consumption of pork and pig products, federal health agencies must begin a pro-active policy of protecting the public from this disease.

3. Humans May Contract TSE from "Downer Cows"

An undiagnosed TSE may occur in cows in the U.S. cattle population called "downer cows." In 1979, at the USDA field station in Mission, Texas, researchers inoculated cattle with scrapie to see if cattle were susceptible to it. Some of the affected animals developed a TSE different from British-style BSE. The symptoms were more similar to those seen in "downer cow" syndrome (a stiff-legged gait, incoordination, abnormal tail position, disorientation, and terminal recumbency). A leading expert in a TSE study stated that this evidence suggested that a bovine TSE was present at a low level in U.S. cattle and that "downer cows" should be tested. "Susceptibility of cattle to scrapie further suggests the possibility that sporadic cases of BSE may have occurred in the United States under the clinical picture of the downer cow syndrome." While there is a small BSE monitoring program begun in 1990, that program did not look at brains from downer cows until 1993. Although now included in the study, very few downer cows have been tested. As of January 23, 1997, only around six hundred brains of downers were studied out of some 5,342 cattle brains tested. There are approximately 100,000 downer cows each year in the U.S. If just one-half of one percent (0.5%) of these cows had a TSE, then there are 500 infected cows in the U.S. Even though the agencies are testing for TSEs in cattle, they are only testing for one strain and the program is ineffective. Even though the agencies are testing for BSE in cattle, they are only looking for British-style BSE. The petitioners demand that the agencies begin a national reporting and monitoring system to address this problem.

4. Humans May Contract TSE from Other Humans

Aside from the concern of humans being infected through consumption of meat, a threat now exists of human to human transmission. In September 1998, a forty-year-old Colorado woman died of CJD. Experts struggled to determine whether she died of the same disease which has now claimed 35 people in England, "British BSE-nvCJD". After the autopsy, experts decided that although she had many symptoms of nvCJD, she did not have nvCJD. Rather, this individual was infected with a TSE disease when doctors used a CJD contaminated medical product made of dura mater (the tissue that covers the brain). The Colorado woman was actually infected with a TSE during an otherwise successful surgery. While in England a special task force has been set up to address this problem, the United States has not undertaken any plan to address it. England is now assessing the costs and benefits of destroying all the equipment used in surgery, including operating tables and light switches. Utilizing a precautionary analysis, the chair of the English working group stated, "Even if it leads to a small reduction in the numbers of who contract CJD it will be worth it." Clearly, the U.S. public is not safe from this disease. In addition to possible infection through food vectors, growing evidence of iatrogenic TSE transmission warrants the establishment of studies to understand the scope of the problem. The State of Utah has decided to begin the process to make CJD a reportable disease after R. Douglas McEwen, a petitioner in this action and a 30-year old Kaysville man, has come down with the disease. Since Mr. McEwen was a regular plasma donor and that plasma was pooled, it is uncertain just how far this contagion has spread. Other governments continue to call for additional research while noting the inadequacy of current studies.Most of the laboratory and epidemiological studies of blood infectivity and disease transmissability have been made on the sporadic form of CJD, and clinical and neuropathological observations suggest that nvCJD may have distinctive biological features. Further studies of new variant cases are needed in order to determine whether or not the tissue distribution of infectivity in nvCJD differs from that of classical CJD, and, in particular, whether the infectious agent might be present in blood more frequently or in greater amounts than in blood of patients with other forms of CJD.

Such a study needs to be included within a larger system for discovering, reporting, and compiling information on TSEs to protect the public.

C. The Department of Health and Human Services Should Implement a National System for Monitoring all new Strains of TSE, Including "British BSE-nvCJD" in Humans because it is Necessary to Protect Human Health

1. Health and Human Services' Statutory Mandate Requires the Monitoring of TSEs

The petitioners request that the Department of Health and Human Services establish and implement a reporting procedure in concert with other agencies to report the incidences of CJD and combat this infectious disease in the United States. The scientific evidence shows that TSEs have been identified by scientists as a human health threat in the United States. Although TSEs are a grave risk to human health, neither state nor federal health agencies have any type of uniform or coordinated programs for testing, monitoring, reporting, compiling, or maintaining information on this disease. Thus, there is a scientific need for such a system to aid the public and other agencies in tracking this disease to protect human health.

The Health and Human Services Secretary, Donna Shalala, is directed by the Congress to protect the public health by controlling communicable or transmissible diseases. This congressional mandate, cited supra, under "Statement of the Law," directs the Secretary to use her power to research, investigate and work cooperatively with state and local health authorities to prevent infectious diseases from spreading as well as allowing her to prevent the introduction of transmissible diseases into the United States. Additionally, the Secretary is directed to collect and disseminate information through the Director of the Centers for Disease Control and Prevention. Petitioners demand the agencies start using this power to test, study and report on Transmissible Spongiform Encephalopathies (TSEs).

Petitioners have also shown that TSE is a communicable disease. Failure of the agencies to control TSE through a national monitoring program is contrary to the statutory mandate under Title 21, part 1240 directing the Secretary to control communicable diseases. The law mandates under Title 42, part 241 that the Secretary shall use her power to research and investigate diseases to protect the public from physical and mental impairments; all the scientific evidence indicates that the public needs protecting from a communicable disease such as TSE. Congress has allowed the Secretary the authority to cooperate with state and local health authorities for the prevention of communicable diseases under Title 42, part 243, in order for there to be an effective understanding of the current spread of the disease a uniform national reporting procedure is necessary. As it relates specifically to iatrogenic and matters involving "British BSE-nvCJD" the Secretary is authorized under Title 42 part 264 to use her authority to prevent the introduction of transmissible diseases into the United States. Further, the Secretary is directed to collect and disseminate information through the Director of the Center for Disease Control and Prevention, examples of which are the tests, studies and reports requested under Title 42 part 280b. Failure for the Secretary to execute a national monitoring program in light of the significant extent and depth of scientific information on TSEs is arbitrary and capricious.

An agency's action is reviewed by the judiciary under the arbitrary and capricious standards of law. Under this standard, an agency must show a "rational connection between the fact found and the choices made." An agency action is arbitrary and capricious when an agency has relied on factors which Congress has not intended it to consider, entirely failed to consider an important aspect of the problem, offered an explanation for its decision that runs counter to the evidence before the agency or is so implausible that it could not be ascribed to a difference in view or the product of agency expertise. In determining whether an agency decision was "arbitrary and capricious," a reviewing court considers whether the decision was based on a reasoned evaluation of the relevant factors and whether there has been a clear error of judgment.

Given the significant human health risk involved and the agencies knowledge of the extent and breadth of the scientific evidence agency failure to initiate a national TSE monitoring program would clearly be arbitrary and capricious under this standard.

2. A National Monitoring Program is Necessary Since CJD is Commonly Misdiagnosed

Alzheimer's disease is a neurological disease with symptoms similar to those of CJD. It is considered one type in the classifications of dementias. Dementias are difficult to clinically diagnose and there is typically a 25% error rate in diagnoses. The GAO has projected the prevalence of Alzheimer's in this country to be 5.7% (1.9 million) of Americans 65 years old and older by the Government Accounting Office.

a. The University of Pittsburgh Study: A University of Pittsburgh study indicates that CJD is commonly misdiagnosed as Alzheimer's disease or other senile dementia. In the study, autopsies were done on 54 demented patients who had been diagnosed with a non-CJD dementia or Alzheimer's disease. Of those 54 patients, scientists found 3 cases of CJD. This study represents a 5.5% misdiagnosis rate.

b. The Yale University Study: A Yale University study also indicates that CJD is commonly misdiagnosed as Alzheimer's disease. In the study, 46 patients diagnosed with Alzheimer's were autopsied. The autopsy showed that 6 patients actually had CJD. This study represents a 13% misdiagnosis rate.

c. Other Evidence: CJD Voice is a support group for people who have had a case of CJD in their families or among their friends. As of July, 1998, CJD Voice had 223 members, and 91 out of 99 members reported an initial misdiagnosis. Those false diagnoses were stroke (18), depression (13), dementia (10), unknown (6), and Alzheimer's disease (5) instead of the correct diagnosis of CJD.

Among the problems in tracking CJD and TSE diseases is that autopsies are rarely performed. In a recent report, the Congressional Research Service noted this problem:

An autopsy is required to confirm a CJD diagnosis, and the number of autopsies performed in the United States has declined over the past 25 years to less than 10% of non-homicide-related deaths. There probably is an under-reporting of CJD in the elderly due to the autopsy decline and the similarity with the symptoms of Alzheimer's disease. One study found that 13% of clinically diagnosed Alzheimer cases were proven to be CJD at autopsy. Younger persons with these neurological symptoms are more likely to be autopsied. Pathologists may be reluctant to perform an autopsy on a CJD victim because of the potential risk of contracting the disease.

While autopsies are very rare overall, they are even more rare in the case of suspected CJD. Medical workers are understandably reluctant to put themselves at risk of catching an infectious disease in order to correct a misdiagnosis. While it is generally believed that only one-in-a-million people suffer from sporadic CJD, scientists know this disease is being grossly under reported in clinical practice. One leading scientist expressed her concern with the lack of good empirical data on the disease. Fewer than 10% of all deaths are investigated with an autopsy, and even a smaller percentage of victims of dementia. Alzheimer's disease was rarely diagnosed prior to the 1940s, but now we diagnose all sorts of people with the disease. But Alzheimer's is a heterogeneous disease with many different causes. One cause for some people could be an infectious agent. And we really have no idea how much CJD there is. The one-in-a-million figure may be an underestimate.

Another scientist has suggested that monitoring CJD and TSE will play a key role in protecting the public from this health threat. Many were wrong about [BSE], including me. We didn't imagine that it could pass from cows into humans. But now we think it can, and it has the potential to be terrifying. Perhaps the best analogy is to the AIDS epidemic. Although it's almost certain that TSE doesn't transmit as readily as HIV, it's similar in a number of other ways. It can remain in the body for long periods without obvious symptoms, and it is fatal. But what I'm thinking about is how we regarded AIDS in the early days, before we really understood it. We underestimated the threat. Perhaps we should avoid making that mistake again.

The prevalence of misdiagnosed CJD infection and the scientific community's need for information on this health issue indicates that a national testing and monitoring system should be implemented immediately to protect public health.

3. New Lab Tests are Available for Testing TSE

In September of 1996, Drs. Kelvin H. Lee, Clarence Joseph Gibbs and others announced that they had discovered a way to determine whether a patient was suffering from CJD or from Alzheimer's disease or other dementia. This test along with a case study is a useful tool to determining ante-mortem whether a patient is suffering from CJD. Dr. Larry Schonberger, a medical epidemiologist at the Centers for Disease Control and Prevention, in Atlanta, Georgia, stated, "This is an important step forward. We've been hungry for a test for this disease. A lot of people have dementia, and it's unclear as to what it might be, and we're very hesitant to do a brain biopsy. This would be a much easier way to make a diagnosis while the patient is still alive."

A distinguished leading expert on this disease, Dr. Clarence Joseph Gibbs, reported to Congress that the accuracy of this cheap and simple spinal fluid test rates at 96%-99%. The New York Times reported that the test correctly identified 96 percent of the patients with TSE and correctly ruled the disease out in 96 percent of those patients with other types of dementia. This test is especially important as it will confirm for family members whether a patient is not expected to survive a year, as in the cases of TSEs, or whether a patient's suffering will be a "long, drawn-out affair" as in the case of Alzheimer's.

Aside from the benefits to the individual patients and their families, this test allows agencies to determine actual numbers of sufferers of TSEs among the public. Petitioners believe that this test can be used to assist in the establishment of a national reporting system by the Centers for Disease Control and Prevention. This test provides the methodology needed to get accurate data to the agencies, which can then inform the public about the risk of the disease.

Although these tests can be used while the human or animal is alive, these tests only detect the disease toward the end of a long incubation period. Before a human or animal exhibits symptoms there is no way to test for the disease even though the human or animal is infected and contagious. Given the long incubation period, and the fact that the disease can be transmitted and undetected and the epidemiological evidence that consumption of animal products especially brain consumption has been linked to sporadic CJD, a precautionary approach must be utilized by the agencies. Therefore, the petitioners demand action from the agencies to begin epidemiological tests with corresponding reporting and monitoring systems.

VII. Environmental Impact & Certification

The enforcement actions here requested will not cause the release of any substance into the environment. They are categorically excluded from the requirement of environmental documentation under 21 C.F.R. ■ 25.33(g). The undersigned certify that, to the best knowledge and belief of the undersigned, this petition includes all information and views on which the petitions relies, and that it includes representative data known to the petitions which are unfavorable to the petition. Except as described above, petitioners know of no other similar issue, act, or transaction to this petition currently being considered or investigated by any HHS office, other federal agency, department, or instrumentality, state municipal agency, court or any law enforcement agency consistent with FDA regulation 21 C.F.R. ■ 10.30(e)(2).

VIII. Agency Action Requested

The petitioners request the HHS and its departments, the FDA and the CDC, to take the following immediate action to prevent the potential spread of transmissible spongiform encephalopathy (TSEs):

(1) initiate a significant epidemiological investigation to determine the incidence of transmissible spongiform encephalopathies among the human population of the United States;

(2) develop an ongoing national monitoring and registry program utilizing autopsy examinations to determine any changes in the incidence of CJD-like diseases among the human population of the United States; and

(3) direct all state medical officers to engage in a reporting process, similar to the reporting requirements of other infectious and transmittable diseases, to the CDC for the purposes of establishing a national cumulative database and reporting system.

Petitioners are requesting a response to this petition within one hundred eighty (180) calendar days. In the absence of an affirmative response, the Petitioners will be compelled to consider litigation in order to achieve the full and complete action required to address this violation of federal law.

Dated this 6th day of January, 1999.

On behalf of all petitioners,

Andrew Kimbrell
Executive Director

Joseph Mendelson, III
Legal Director

CC: Jane Henney, Commissioner
United States Food and Drug Administration
c/o Dockets Management Branch
12420 Parklawn Drive, Room 1-23
Rockville, MD 20857

Top 10 PR blunder list released

UPI US & World Wed, Jan 6, 1999
SAN FRANCISCO -- Texas cattlemen came out No. 1 on a list of the top 10 PR blunders of 1998 for their unsuccessful $12 million libel lawsuit against talk show host Oprah Winfrey.

The list, released by San Francisco-based Fineman Associates Public Relations, says it took "Texas-sized, uh, chutzpah, to go head-to-head" with Winfrey, who "came out the victor."

Others on the list include:

-- the National Basketball Association for its seven-month lockout involving "a group of millionaires bickering over profits and astronomical salaries."

-- the Internal Revenue Service for pledging to impose a six-figure gift tax on any fan who caught a home run ball from Mark McGwire or Sammy Sosa, even if they gave the ball back. The IRS quickly relented, but the PR consultants say the damage was already done.

-- the Professional Golfers Association Tour for going up against young golfer Casey Martin, who needs a golf cart to compete in tournaments because of a painful leg condition.

-- Conway Truck Loading Services of Fort Worth, Texas, for firing a Palmdale, Calif., driver who jackknifed his truck in order to avoid a multi-truck pileup.

-- the Abercrombie & Fitch clothing company for welcoming college students back with a catalog/magazine that featured a two-page article called "Drinking 101" in a year in which binge drinking on campus has been a national story.

-- NationsBank for clashes with a trade journal and the exodus of leading female executives following the bank's merger with Bank of America.

-- the Parent Teacher Association for auctioning off "its brand cachet to the highest bidder" in connection with its deal with Office Depot.

-- United Paramount Network (UPN) for failing to allay concerns about its controversial new sitcom, "The Secret Diary of Desmond Pfeiffer."

-- Washington DC and politicians over the sex scandal involving President Clinton.

Early diagnosis of Alzheimer

AP-NY 01-Jan-99 
LONDON -- Researchers at New York University have developed a new brain- scanning method that may be able to detect Alzheimer's disease in its early stages, according to a new study.

People with Alzheimer's develop characteristic microscopic changes in the brain, but because they are deep within the brain and are not easily seen on a scan, doctors don't normally test for them. There is no single, comprehensive diagnostic test for Alzheimer's. Instead, doctors rule out other conditions through a process of elimination.

Autopsy is the only way to confirm a diagnosis of the disease, which slowly kills nerve cells in the brain and causes personality changes and memory loss that gets progressively worse, leading to dementia. But in preliminary research published in this week's issue of The Lancet, a British medical journal, scientists say they have found a way to measure brain shrinkage before death, and the method could help diagnose the disease early.

``This is quite exciting and very significant work,'' Dr. Zaven Khachaturian, senior consultant to the Chicago-based Alzheimer's Foundation was reported as saying in The Boston Globe.

There is no medical treatment currently available to cure or stop the progression of Alzheimer's. Some drugs have been shown to ease the symptoms of the disease, which is estimated to affect nearly 18 million people worldwide, or 3 percent of people over the age of 60.

In the study of 16 people, Dr. Mony de Leon of the Department of Psychiatry at New York University and his colleagues found that a part of the brain responsible for memory, the entorhinal cortex, was 27 percent smaller in people with mild Alzheimer's than in their healthy counterparts.

The scientists first examined the brains of 20 people who had died at about age 80, measuring the size of certain landmarks in the entorhinal cortex that could be seen on a magnetic resonance image, or scan.

They compared the landmarks in the brains of 16 people who they knew had moderate to severe Alzheimer's disease with those of four people of a who died at a similar age but who did not have the disease. They found that the size of that part of the brain was 45 percent smaller in the people who had Alzheimer's.

The researchers then tested the same technique on 16 live patients around the age 80 -- half of whom had mild or very mild Alzheimer's and the other half were healthy volunteers. The entorhinal cortex, as measured by the scanned landmarks, was 27 percent smaller in the Alzheimer's patients, the study said.

``At this point, there is no major advantage to knowing many decades in advance that someone is developing Alzheimer's because there is nothing close to a cure,'' Dr. Kirk Daffner, co-director of the memory disorder unit at Brigham and Women's Hospital in Boston, told The Globe.

Khachaturian was quoted by The Globe as saying the NYU study lends evidence to the argument that the onset of Alzheimer's comes earlier than old age and that it simply takes decades to manifest itself.

New brain cells possible

January 8, 1999
WASHINGTON - In a discovery that could offer new hope to Alzheimer's or Parkinson's disease patients, Swedish scientists have found rapidly multiplying stem cells in the brain and spinal cord of rats.

Stem cells are the unspecialized "parent" cells to all tissues in the body, which later grow into bone, blood, skin, nerves and muscles.

In Friday's edition of Cell magazine, Jonas Frisen and Ann Marie Janson of the Karolinska Institute in Stockholm report that under normal conditions, these neural stem cells, called ependymal cells, remain inactive.

But when the scientists wounded the rats' spinal cords, they found that these cells proliferate 50 times more quickly than normal to generate migratory cells that specialize to form scar tissue at the site of the injury.

People could also host such reserves of undifferentiated stem cells, and the ability to stimulate them could be used to treat patients with neurological diseases such as Parkinson's and Alzheimer's, the scientists say.

Another BSE case in Holland

7 Jan 99 Vincent Dedet DVM, free lance journalist "Netherlands begins 99 with its fifth case, declared today 7 of January. It is a six years old cow from a 80 cows farm near Utrecht. It is a BAB case, whose symptoms made a suspicion at the end of december and that has been slaughtered on the 30th of December."

"All animals on the farm (79 cows, 14 ewes, 1 ram, 1 cat) have been slaughtered today. The inquiries are : on farm for all the contemporary animals, plus tracing this animal and his mother's descendance over the country (what if one is grazing over the border ? Let's say in Germany for instance ?). These animals, once detected, will be slaughtered to look for BSE. Of cours, feedstuffs are being investigated."

Pentosan stops scrapie, may help with CJD-research

 Reuters World Report Jan 7, 1999
based on Lancet Volume 353, Number 9147  9 January 1999
 
LONDON - British scientists said on Friday that a chemical they have already successfully tested on mice could help in the fight against new variant CJD, the human brain-wasting malady linked to mad cow disease. The researchers, from Edinburgh's Institute for Animal Health, called for further animal experiments to establish whether pentosan polysulphate (PS) could reduce the risk of Creutzfeldt-Jakob disease.

Thirty-five people in Britain have been diagnosed as having the invariably fatal new variant CJD since scientists said in March 1996 that they had evidence that it could be contracted by eating beef from animals suffering from mad cow disease. But the likely extent of the future problem is still unclear because of the long incubation period associated with the disease.

The Edinburgh researchers injected mice with scrapie, the version of mad cow disease which afflicts sheep, and injected PS seven hours later. Treated with one milligram of PS, the mice injected with one strain of scrapie were completely protected from the disease, those injected with another strain had at least a hundredfold reduction in sensitivity to it, and some of those injected with a third survived. [The relevance of this to humans infected a decade or more ago is unclear. -- webmaster]

"Although direct testing of the efficacy of PS in reducing susceptibility to variant CJD is not possible, further animal studies are essential to examine the possible use of PS for risk reduction," the scientists wrote in the British medical journal The Lancet. They noted that PS is already licensed in the United States for the treatment of a form of cystitis.

European Union farm ministers agreed in November to lift an export ban on British beef imposed after the new evidence of transmissibility of mad cow disease to humans more than two years earlier. The agreement came after Britain slaughtered thousands of cattle judged at possible risk from the mad cow disease epidemic which was believed to have been caused by the incorporation of sheep remains in fodder.

Lancet summary:

'intraperitoneal pentosan given 7 h after scrapie prolonged survival significantly'

Variant Creutzfeldt-Jacob disease (CJD), the human form of transmissible spongiform encephalopathy, has no known cure and the diagnosis is usually made when patients are terminally ill. During the long incubation period--when there are no symptoms--there is a potential but unknown risk of transfer of infection by blood transfusion, treatment with blood products, transplantation, or reuse of surgical instruments. Christine Farquhar and colleagues report that a single intraperitoneal dose of pentosan polysulphate prolonged survival in mice injected with scrapie--the ovine form of the disease. Oral pentosan was ineffective. Further animal studies should assess the possible use of pentosan polysulphate in treatment of variant CJD

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