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Seven years earlier and
5000 miles away, Stephen Churchill was the first in
to die. His first symptoms of depression and dizziness gave way to a
living nightmare of terrifying hallucinations; he was dead in 12 months at
age 19. Next was Peter Hall, 20, who showed the first signs of
depression around Christmas, 1994. By the next Christmas, he couldn't
walk, talk, or do anything for himself. Then it was Anna's turn, then
Michelle's. Michelle Bowen, age 29, died in a coma three weeks after
giving birth to her son via emergency cesarean section. Then it was
Alison's turn. These
In humans, prions can cause Creutzfeldt-Jakob disease (CJD), a human spongiform encephalopathy whose clinical picture can involve weekly deterioration into blindness and epilepsy as one's brain becomes riddled with tiny holes.
We've known about Creutzfeldt-Jakob disease for decades, since well before the first mad cow was discovered in 1985. Some cases of CJD seemed to run in families; other cases seemed to just arise spontaneously in about one in a million people every year, and were hence dubbed "sporadic." The new form of CJD caused by eating beef from cows infected with Mad Cow disease, though, seemed to differ from the classic sporadic CJD.
The CJD caused by
infected meat has tended to strike younger people, has produced more
psychotic symptoms, and has often dragged on for a year or more. The most
defining characteristic, though, was found when their brains were sampled.
The brain pathology was vividly reminiscent of Kuru, a disease once found
in a New Guinea tribe of
Other than Charlene, a 24
year old woman now so tragically dying in Florida, who was probably
infected in Britain, there have been no reported cases of variant CJD in
the U.S. Hundreds of confirmed cases of the sporadic form of
Creutzfeldt-Jakob disease, however, arise in the United States every
year, but the beef industry is quick to point out these are cases of
sporadic CJD, not the new variant known to be caused by Mad Cow
disease. Of course, no one
Although the fact that
Mad Cow disease causes variant CJD had already been strongly established,
researchers at the University College of London nevertheless created
transgenic mice complete with "humanized" brains genetically engineered
with human genes to try to prove the link once and for all. When the
researchers injected one strain of
This is not the first
time meat was linked to sporadic CJD. In 2001, a team of French
researchers found, to their complete surprise, a strain of scrapie--"mad
sheep" disease--that caused the same brain damage in mice as sporadic
CJD. "This means we cannot rule out that at least some sporadic CJD
may be caused by some strains of scrapie," says team member Jean-Philippe
Deslys of the French Atomic Energy Commission's medical research
laboratory. Population studies had failed to show a link between CJD
and lamb chops, but this French research provided an explanation why.
There seem to be six types of sporadic CJD and there are more than 20
strains of scrapie. If only some sheep strains affect only some people,
studies of entire populations may not clearly show the relationship.
Monkeys fed infected sheep brains certainly come down with the
disease. Hundreds of "mad sheep" were found in the U.S. in 2003.
Scrapie remains such a problem in the United States that the USDA has
issued a scrapie "declaration of emergency." Maybe
A number of
epidemiological studies have suggested a link between pork consumption and
sporadic CJD. Analyzing peoples' diet histories, the development of CJD
was associated with eating roast pork, ham, hot dogs, pork chops, smoked
pork, and scrapple (a kind of pork pudding made from various hog carcass
scraps). The researchers
Sporadic CJD has also
been associated with weekly beef consumption, as well as the
consumption of roast lamb, veal,venison, brains in general, and,
in North America, seafood.[32,33] The development of CJD has also,
surprisingly, been significantly linked to exposure to animal products in
fertilizer, sport fishing and deer hunting in the U.S., and
We do not know at this
time whether chicken meat poses a risk. There was a preliminary report of
ostriches allegedly fed risky feed in German zoos who seemed to come down
with a spongiform encephalopathy. Even if chickens and turkeys
themselves are not susceptible, though, they may become so-called "silent
carriers" of Mad Cow prions and pass them on to human consumers.
Dateline NBC quoted D. Carleton Gajdusek, the first to be awarded a Nobel
The recent exclusion of most cow brains, eyes, spinal cords, and intestines from the human food supply may make beef safer, but where are those tissues going? These potentially infectious tissues continue to go into animal feed for chickens, other poultry, pigs, and pets (as well as being rendered into products like tallow for use in cosmetics, the safety of which is currently under review). Until the federal government stops the feeding of slaughterhouse waste, manure, and blood to all farm animals, the safety of meat in America cannot be guaranteed.
The hundreds of American families stricken by sporadic CJD every year have been told that it just occurs by random chance. Professor Collinge, the head of the University College of London lab, noted "When you counsel those who have the classical sporadic disease, you tell them that it arises spontaneously out of the blue. I guess we can no longer say that."
"We are not saying that all or even most cases of
sporadic CJD are as a result of BSE exposure," Professor Collinge
continued, "but some more recent cases may be--the incidence of sporadic
CJD has shown an upward trend in the UK over the last decade... serious
consideration should be given to a proportion of this rise being
We don't know exactly what's happening to the rate of CJD in this country, in part because CJD is not an officially notifiable illness. Currently only a few states have such a requirement. Because the Centers for Disease Control (CDC) does not actively monitor the disease on a national level, a rise similar to the one in Europe could be missed. In spite of this, a number of U.S. CJD clusters have already been found. In the largest known U.S. outbreak of sporadic cases to date, five times the expected rate was found to be associated with cheese consumption in Pennsylvania's Lehigh Valley. A striking increase in CJD over expected levels was also reported in Florida and New York (Nassau County) with anecdotal reports of clusters of deaths in Oregon and New Jersey.
worrisome is the seeming increase in CJD deaths among young people in this
country. In the 18 years between 1979 and 1996, only a single case of
sporadic CJD was found in someone under 30. Whereas between 1997 and 2001,
five people under 30 died of sporadic CJD. So five young Americans dying
in five years, as opposed
The most frequent
misdiagnosis of CJD among the elderly is Alzheimer's disease. Neither
CJD nor Alzheimer's can be
Over the last 20 years the rates of Alzheimer's disease in the United States have skyrocketed. According to the CDC, Alzheimer's Disease is now the eighth leading cause of death in the United States, afflicting an estimated 4 million Americans. Twenty percent or more of people clinically diagnosed with Alzheimer's disease, though, are found at autopsy not to have had Alzheimer's at all. A number of autopsy studies have shown that a few percent of Alzheimer's deaths may in fact be CJD. Given the new research showing that infected beef may be responsible for some sporadic CJD, thousands of Americans may already be dying because of Mad Cow disease every year.
Nobel Laureate Gajdusek,
for example, estimates that 1% of people showing up in Alzheimer clinics
actually have CJD. At Yale, out of a series of 46 patients clinically
diagnosed with Alzheimer's, six were proven to have CJD at autopsy. In
another study of brain biopsies, out of a dozen patients diagnosed with
Alzheimer's according to established criteria, three of them were actually
dying from CJD. An informal survey of neuropathologists registered a
suspicion that CJD accounts for 2-12% of all dementias in general. Two
autopsy studies showed a CJD rate among dementia
The incubation period for
human spongiform encephalopathies such as CJD can be decades. This
means it can be years between eating infected meat and getting diagnosed
with the death sentence of CJD. Although only about 150 people have so far
been diagnosed with variant CJD worldwide, it will be many years before
the final death toll is known. In the United States, an unknown number of
animals are infected with Mad Cow disease, causing an unknown number of
human deaths from CJD. The U.S. should immediately begin testing all cows
destined for human consumption, as is done in Japan, should stop feeding
slaughterhouse waste to all farm animals (see
http://organicconsumers.org/madcow/GregerBSE.cfm), and should
immediately enact an active national surveillance program for CJD.
In 1997, the CDC set up the National Prion Disease Pathology Surveillance Center at Case Western Reserve University to analyze brain tissue from CJD victims in the U.S. in hopes of tracking any new developments. In Europe, surveillance centers have been seeing most, if not all, cases of CJD. The U.S. center sees less than half. "I'm very unhappy with the numbers," laments Pierluigi Gambetti , the director of the Center. "The British and Germans politely smile when they see we examine 30% or 40% of the cases," he says. "They know unless you examine 80% or more, you are not in touch." "The chance of losing an important case is high."
One problem is that many
doctors don't even know the Center exists. And neither the CDC nor the
Center are evidently authorized to reach out to them directly to bolster
surveillance efforts, because it's currently up to each state individually
to determine how--or even whether--they will track the disease. In Europe,
in contrast, the national centers work directly with each affected family
and their physicians. In the U.S., most CJD cases--even the confirmed
ones--seem to just fall through the cracks. In fact, based on the autopsy
studies at Yale and elsewhere, it seems most CJD cases in the
Autopsy rates have
dropped in the U.S. from 50% in the Sixties to less than 10% at
present. Although one reason autopsies are rarely performed on
atypical dementia cases is that medical professionals are afraid of
catching the disease, the primary reason for the decline in autopsy
rates in general appears to be financial. There is currently no direct
reimbursement to doctors or hospitals for doing autopsies, which often
forces the family to absorb the cost of transporting the body to an
autopsy center and having the brain samples taken, a tab that can run
upwards of $1500.
Andrew Kimbrell, the director of the Center for Food Safety, a D.C.-based public interest group, writes, "Given what we know now, it is unconscionable that the CDC is not strictly monitoring these diseases." Given the presence of Mad Cow disease in the U.S., we need to immediately enact uniform active CJD surveillance on a national level, provide adequate funding not only for autopsies but also for the shipment of bodies, and require mandatory reporting of the disease in all 50 states. In Britain, even feline spongiform encephalopathy, the cat version of Mad Cow disease, is an officially notifiable illness. "No one has looked for CJD systematically in the U.S.," notes NIH medical director Paul Brown. "Ever."
The animal agriculture
industries continue to risk public safety, and the government seems to
protect the industries' narrow business interests more than it protects
its own citizens. Internal USDA documents retrieved through the Freedom of
Information Act show that our government did indeed consider a number of
precautionary measures as far back as 1991 to protect the American public
from Mad Cow disease. According to one such document, however, the USDA
Plant sources of protein for farm animals can cost up to 30% more than cattle remains. The Cattlemen's Association admitted a decade ago that animal agribusiness could indeed find economically feasible alternatives to feeding slaughterhouse waste to other animals, but that the they did not want to set a precedent of being ruled by "activists."
Is it a coincidence that USDA Secretary Veneman chose Dale Moore, former chief lobbyist for the National Cattlemen's Beef Association, as her chief of staff? Or Alison Harrison, former director of public relations for the Cattlemen's Association, as her official spokeswoman? Or that one of the new Mad Cow committee appointees is William Hueston, who was paid by the beef industry to testify against Oprah Winfrey in hopes of convicting her of beef "disparagement"? After a similar conflict of interest unfolded in Britain, their entire Ministry of Agriculture was dissolved and an independent Food Safety Agency was created, whose sole responsibility is to protect the public's health. Until we learn from Britain's lesson, and until the USDA stops treating this as a PR problem to be managed instead of a serious global threat, millions of Americans will remain at risk.
Michael Greger, MD, is a graduate of the Cornell University School of Agriculture and the Tufts University School of Medicine. Dr. Greger has been speaking publicly about Mad Cow disease since 1993. He debated National Cattlemen's Beef Association Director Gary Weber before the FDA and was invited as an expert witness at the infamous Oprah Winfrey "meat defamation" trial. He has contributed to many books and articles on the subject, continues to lecture extensively, and currently coordinates the Mad Cow disease website for the Organic Consumers Association. Dr. Greger can be reached for media inquiries at (617) 524-8064 or email@example.com.