Are Mad Cow-Like Diseases Developing in the USA?

In These Times Magazine
January 2000

A 21st Century Plague?
Joel Bleifuss, In These Times

The scale of the public health threat posed by Mad Cow disease and
other transmissible spongiform encephalopathies (TSEs) may not be
known for years to come. In Britain, the death toll resulting from Mad
Cow -- or, more precisely, its human manifestation, new-variant
Creutzfeldt-Jakob disease (nvCJD) -- could be catastrophic. Here in the
United States, chronic wasting disease, a TSE found in deer and elk,
may pose a threat to those who eat infected animals.

No one is sure to what extent the public is at risk from TSEs, which are
caused by little-understood proteins called prions. The prevalence of
the disease in livestock populations is still unknown. How humans are
infected with a TSE from other species is undetermined. And the
disease in humans is difficult to track, since it may take decades from
the time of initial infection to show any symptoms.

Mad Cow -- or bovine spongiform encephalopathy (BSE)-appears to
have originated from the common practice of feeding livestock
high-protein feed supplements derived from rendered slaughterhouse
waste, some of which was from TSE-infected animals. By recycling
TSEs -- which occur naturally in mammals but are extremely rare-into
the food supply, an epidemic was touched off. In Britain, nearly 50
people have been diagnosed with nvCJD, a disease whose associated
prion is nearly identical to the prion that causes Mad Cow. But those
numbers may be deceptively low due to the disease's long incubation
period and because the victims appear to have been exposed before
1986, when Mad Cow was discovered in British cattle.

To measure the extent of the problem, for the past two years British
government scientists have been performing biopsies on tonsils, one of
the body parts that contains the nvCJD prion. The results have not been
made public. But statements from those familiar with the studies are not
reassuring. In August, John Pattison, chairman of the government's
Spongiform Encephalopathy Advisory Committee, predicted that the
number of people infected with nvCJD could reach millions. In
December, Lord Justice Phillips, who has been heading an inquiry into
the government's mishandling of the crisis, said the current cases may
be the just "tip of the iceberg." And the BBC has reported that a study
by the Royal Society, Britain's premier scientific body, has estimated
the death toll might reach 13 million.

Making matters worse, theories have emerged that some people who
contract nvCJD may get it not from eating infected meat but from
secondary infections. Last July, John Collinge, a member of the
Spongiform Encephalopathy Advisory Committee, wrote in The Lancet
that he expected the human epidemic to "evolve over decades." He went
on to raise concerns that the infectious prions might be transmitted "via
blood transfusion, tissue donation, and, since prions resist routine
sterilization, contamination of surgical instruments." He also raised the
disturbing specter that other species of livestock may be infected. "The
theoretical possibility that BSE prions might have transferred to other
species and continue to present a risk to human health cannot be
excluded," he wrote.

Mad Cow prions or their TSE cousins have not been found in the U.S.
cattle population. (However, testing by the U.S. Department of
Agriculture is so inadequate that such a statement is meaningless.) But
a TSE specific to deer and elk, chronic wasting disease, is endemic in
parts of Colorado and Wyoming and has been found on game ranches
in Montana and Oklahoma. Chronic wasting disease was first observed
in 1967 in captive mule deer, which were subsequently released into
the wild. One theory has it that the captive deer were exposed to sheep
that carry an ovine form of TSE known as scrapie, which is relatively
commonplace. Another is that the deer contracted the disease by eating
feed that contained rendered protein from an infected animal. In parts of
Colorado and Wyoming it affects as many as 8 percent of the deer and
1 percent of the elk.

At least two young hunters, who ate and dressed deer, have come down
with CJD. And a third, a young woman who had eaten venison from a
deer shot in Maine, also contracted the disease. Because CJD is
normally a disease of the old, the youth of the current victims raises the
strong possibility that they contracted the disease through infected

Paul Brown, an expert on TSEs at the National Institutes of Health, has
told John Stauber, author of Mad Cow U.S.A., that deer hunters must
be out of their minds to be consuming deer in areas where chronic
wasting disease is prevalent. That message, however, has not gotten out
to the general public. "The failure of state and federal agencies to take
swift action and warn hunters about potential risks of chronic wasting
disease is inexcusable," Stauber says. "The best scientific minds on this
issue have failed to adequately warn the public, and in this instance
deer hunters, of the deadly risks of these types of diseases."

Why the silence? One reason could be that state wildlife departments
are heavily dependent on the income received from licenses for big
game. The Colorado Wildlife Division maintains that chronic wasting
disease does not affect humans, but advises hunters to "wear rubber
gloves when field dressing carcasses, minimize handling of brain and
spinal column and wash hands afterward" and to "avoid consuming
brain, spinal cord, eyes, spleen and lymph nodes of harvested animals."

"The best available science at this time does not indicate a significant
threat to human health as result of chronic wasting disease," Montana
state epidemiologist Tod Damrow told the Billings Gazette. The
problem is that there is no available science.

Vigorous preventive measures to stop the spread of TSEs could
threaten the bottom line of entire industries (meat and human blood
products to name two), which has led regulatory agencies to put the
health of the industry above the public's. The federal bodies that have
been entrusted with protecting the public health, the Centers for
Disease Control and the Food and Drug Administration, have taken
different approaches to this disease and the threat it poses. Meanwhile,
the USDA, which regulates animal feeding practices, continues to treat
the threat as a PR problem.

One of the hunters who was diagnosed with CJD had been a big blood
plasma donor, with his blood being pooled with others' and used in
121 products like clotting factor for hemophiliacs, which are sold in 20
countries. Beginning in 1993, products made from blood coming from
someone with CJD were recalled. That policy cost the blood industry,
including the American Red Cross, millions of dollars and was
abandoned in 1998, when the CDC determined that there was no
evidence that blood from CJD victims was unsafe. On the other hand,
there was no evidence that the blood was safe. In effect, the CDC was
"equating absence of evidence as evidence of absence" says Tom
Pringle, a molecular biologist and webmaster of,
which has become the definitive Web site on TSE. "The politics and
economics of it is that they don't want recalls, and if CJD is more
common that means more of the donors have it and the risk to the
blood supply is higher."

The CDC has concentrated its efforts on testing those stricken with TSE
symptoms for the presence of nvCJD, which is linked to the
consumption of British mad cows. But anyone who contracted a TSE
from exposure to either deer with chronic wasting disease or cattle
infected with an American strain of BSE would not test positive for

"The CDC is playing this public relations game, focusing on nvCJD
and ignoring the possibility that chronic wasting disease in humans
resembles the type of CJD we already have in the United States and not
nvCJD," Stauber says. "The federal government and the meat industry
have attempted to depict TSEs as foreign diseases, so that then
journalists and the public will assume it can't happen here. But we
already have TSE diseases in sheep, mink, deer, elk, humans and
possibly in cattle and pigs. And we continue to engage in risky
livestock feeding practices, like weaning calves on cattle blood protein,
that could put us in a similar situation as Great Britain."

The FDA is being more proactive than the CDC, worried that nvCJD
contracted in Britain by American visitors could contaminate the U.S.
blood supply. (An import ban on British beef is already in place here.)
In June, the FDA's TSE Advisory Committee voted 12 to 9 to prohibit
Americans who spent six months in Britain between January 1980 and
December 1986 from donating or selling blood. This policy will go
into effect April 17 and is expected to affect about 2 percent of U.S.
blood donors.

For its part, the USDA has failed to institute a complete ban on the
practice of feeding rendered animal protein to other animals (rather
than the current partial ban on feeding rendered ruminants to other
ruminants). Instead, the agency has spent time preparing a secret PR
strategy for when the first mad cow is found in the United States.
According to documents released to the Center for Food Safety under
the Freedom of Information Act, the department's Animal and Plant
Health Inspection Service has put together a 27-member BSE Response
Team, which will be flown to a "situation room" at BSE Headquarters
-- an underground bunker in Riverdale, Maryland -- in the event that
BSE is found in the U.S. cattle population. Once a case is confirmed, 24
hours will be spent giving special briefings to unnamed "select industry
and trading partners." Then the public will be told that something has
gone terribly wrong.

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