- October 2001, 34-year-old Washington State native Peter
Putnam started losing his mind. One month he was delivering a keynote business
address, the next he couldn't form a complete sentence. Once athletic,
soon he couldn't walk. Then he couldn't eat. After a brain biopsy showed
it was Creutzfeldt-Jakob disease, his doctor could no longer offer any
hope. "Just take him home and love him," the doctor counseled
his family.[1,2,3] Peter's tragic death, October 2002, may have been caused
by Mad Cow disease.
-
- Seven years earlier and 5000 miles away, Stephen Churchill
was the first in England 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.[4] 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.[5] 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 were the first five named victims of Britain's Mad Cow epidemic.
They died from what the British Secretary of Health called the worst form
of death imaginable, Creutzfeldt-Jakob disease, a relentlessly progressive
and invariably fatal human dementia.[6] The announcement of their deaths,
released on March 20, 1996 (ironically, Meatout Day[7]), reversed the British
government's decade-old stance that British beef was safe to eat.[8]
-
- It is now considered an "incontestable fact"
that these human deaths in Britain were caused by Bovine Spongiform Encephalopathy
(BSE), or Mad Cow disease.[9] Bovine means "cow or cattle," spongiform
means "sponge-like," and encephalopathy means "brain disease."
Mad Cow disease is caused by unconventional pathogens called prions--literally
infectious proteins--which, because of their unique structure, are practically
invulnerable, surviving even incineration[10] at temperatures hot enough
to melt lead.[11] The leading theory as to how cows got Mad Cow disease
in the first place is by eating diseased sheep infected with a sheep spongiform
encephalopathy called scrapie.[12]
-
- 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 cannibals who ate
the brains of their dead.[13] Scientists called this new form of the disease
"variant" CJD.
-
- 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.[14] Hundreds of confirmed
cases of the sporadic form of Creutzfeldt-Jakob disease, however, arise
in the United States every year,[15] 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.[16] Of course, no one knows what causes sporadic
CJD. New research, discussed below, suggests that not hundreds but thousands
of Americans die of sporadic CJD every year, and that some of these CJD
deaths may be caused by eating infected meat after all.
-
- 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 the "humanized"
mice with infected cow brains, they came down with the same brain damage
seen in human variant CJD, as expected. But when they tried this in a different
strain of transgenic "humanized" mice, those mice got sick too,
but most got sick from what looked exactly like sporadic CJD! The Mad Cow
prions caused a disease that had a molecular signature indistinguishable
from sporadic CJD. To the extent that animal experiments can simulate human
results, their shocking conclusion was that eating infected meat might
be responsible for some cases of sporadic CJD in addition to the expected
variant CJD. The researchers concluded that "it is therefore possible
that some patients with [what looks like]... sporadic CJD may have a disease
arising from BSE exposure."[17] Laura Manuelidis, section chief of
surgery in the neuropathology department at Yale University comments, "Now
people are beginning to realize that because something looks like sporadic
CJD they can't necessarily conclude that it's not linked to [Mad Cow disease]..."[18]
-
- 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.[19] "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.[20]
-
- 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.[21] Hundreds
of "mad sheep" were found in the U.S. in 2003.[22] Scrapie remains
such a problem in the United States that the USDA has issued a scrapie
"declaration of emergency."[23] Maybe some cases of sporadic
CJD in the U.S. are caused by sheep meat as well.[24]
-
- Pork is also a potential source of infection. Cattle
remains are still boiled down and legally fed to pigs (as well as chickens)
in this country. The FDA allows this exemption because no "naturally
occurring" porcine (pig) spongiform encephalopathy has ever been found.
But American farmers typically kill pigs at just five months of age, long
before the disease is expected to show symptoms. And, because pigs are
packed so tightly together, it would be difficult to spot neurological
conditions like spongiform encephalopathies, whose most obvious symptoms
are movement and gait disturbances. We do know, however, that pigs are
susceptible to the disease--laboratory experiments show that pigs can indeed
be infected by Mad Cow brains[25]--and hundreds of thousands of downer
pigs, too sick or crippled by injury to even walk, arrive at U.S. slaughterhouses
every year.[26]
-
- 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 concluded, "The
present study indicated 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." Compared to people that
didn't eat ham, for example, those who included ham in their diet seemed
ten times more likely to develop CJD.[27] In fact, the USDA may have actually
recorded an outbreak of "mad pig" disease in New York 25 years
ago, but still refuses to reopen the investigation despite petitions from
the Consumer's Union (the publishers of Consumer Reports magazine).[28]
-
- Sporadic CJD has also been associated with weekly beef
consumption,[29] as well as the consumption of roast lamb,[30] veal, venison,
brains in general,[31] and, in North America, seafood.[32,33] The development
of CJD has also, surprisingly, been significantly linked to exposure to
animal products in fertilizer,[34] sport fishing and deer hunting in the
U.S.,[35] and frequent exposure to leather products.[36]
-
- 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.[37]
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.[38] Dateline NBC quoted D. Carleton Gajdusek,
the first to be awarded a Nobel Prize in Medicine for his work on prion
diseases,[39] as saying, "it's got to be in the pigs as well as the
cattle. It's got to be passing through the chickens."[40] Dr. Paul
Brown, medical director for the US Public Health Service, believes that
pigs and poultry could indeed be harboring Mad Cow disease and passing
it on to humans, adding that pigs are especially sensitive to the disease.
"It's speculation," he says, "but I am perfectly serious."[41]
-
- 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[42]). 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 BSE-related.
Switzerland, which has had a substantial BSE epidemic, has noted a sharp
recent increase in sporadic CJD."[43] In the Nineties, Switzerland
had the highest rate of Mad Cow disease in continental Europe, and their
rate of sporadic CJD doubled.[44]
-
- 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.[45] 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,[46] a rise similar to the one in Europe could be missed.[47]
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,[48] five
times the expected rate was found to be associated with cheese consumption
in Pennsylvania's Lehigh Valley.[49] A striking increase in CJD over expected
levels was also reported in Florida[50] and New York (Nassau County)[51]
with anecdotal reports of clusters of deaths in Oregon[52] and New Jersey.[53]
-
- Perhaps particularly 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 to one young
case in the previous 18 years. The true prevalence of CJD among any age
group in this country remains a mystery, though, in part because it is
so commonly misdiagnosed.[54]
-
- The most frequent misdiagnosis of CJD among the elderly
is Alzheimer's disease.[55] Neither CJD nor Alzheimer's can be conclusively
diagnosed without a brain biopsy,[56] and the symptoms and pathology of
both diseases overlap. There can be spongy changes in Alzheimer's, for
example, and senile Alzheimer's plaques in CJD.[57] Stanley Prusiner, the
scientist who won the Nobel Prize for his discovery of prions, speculates
that Alzheimer's may even turn out to be a prion disease as well.[58] In
younger victims, CJD is more often misdiagnosed as multiple sclerosis or
as a severe viral infection.[59]
-
- Over the last 20 years the rates of Alzheimer's disease
in the United States have skyrocketed.[60] According to the CDC, Alzheimer's
Disease is now the eighth leading cause of death in the United States,[61]
afflicting an estimated 4 million Americans.[62] 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.[63] 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.[64]
-
- Nobel Laureate Gajdusek, for example, estimates that
1% of people showing up in Alzheimer clinics actually have CJD.[65] At
Yale, out of a series of 46 patients clinically diagnosed with Alzheimer's,
six were proven to have CJD at autopsy.[66] 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.[67] An informal survey
of neuropathologists registered a suspicion that CJD accounts for 2-12%
of all dementias in general.[68] Two autopsy studies showed a CJD rate
among dementia deaths of about 3%.[69,70] A third study, at the University
of Pennsylvania, showed that 5% of patients diagnosed with dementia had
CJD.[71] Although only a few hundred cases of sporadic CJD are officially
reported in the U.S. annually,[72] hundreds of thousands of Americans die
with dementia every year.[73] Thousands of these deaths may actually be
from CJD caused by eating infected meat.
-
- The incubation period for human spongiform encephalopathies
such as CJD can be decades.[74] 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.[75]
-
- Five years ago this week, the Center for Food Safety,
the Humane Farming Association, the Center for Media & Democracy, and
ten families of CJD victims petitioned the FDA and the CDC to immediately
enact a national CJD monitoring system, including the mandatory reporting
of CJD in all 50 states.[76] The petition was denied.[77] The CDC argued
that their passive surveillance system tracking death certificate diagnoses
was adequate. Their analysis of death certificates in three states and
two cities, for example, showed an overall stable and typical one in a
million CJD incidence rate from 1979 to 1993.[78] But CJD is so often misdiagnosed,
and autopsies are so infrequently done, that this system may not provide
an accurate assessment.[79]
-
- 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."[80] "The chance of losing
an important case is high."[81]
-
- 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.[82]
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 U.S. aren't even picked up in the first
place.
-
- Autopsy rates have dropped in the U.S. from 50% in the
Sixties to less than 10% at present.[83] Although one reason autopsies
are rarely performed on atypical dementia cases is that medical professionals
are afraid of catching the disease,[84] 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.[85]
-
- Another problem is that the National Prion Disease Pathology
Surveillance Center itself remains underfunded. Paul Brown, medical director
for the National Institutes of Health, has described the Center's budget
as "pitiful," complaining that "there isn't any budget for
CJD surveillance."[86] To adequately survey America's 290 million
residents, "you need a lot of money." UK CJD expert Robert Will
explains, "There was a CJD meeting of families in America in which...
[the CDC] got attacked fairly vigorously because there wasn't proper surveillance.
You could only do proper surveillance if you have adequate resources."[87]
"I compare this to the early days of AIDS," says protein chemist
Shu Chen, who directs the Center's lab, "when no one wanted to deal
with the crisis."[88]
-
- 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."[89] 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."[90]
-
- 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 explained that the "disadvantage"
of these measures was that "the cost to the livestock and rendering
industries would be substantial."[91]
-
- Plant sources of protein for farm animals can cost up
to 30% more than cattle remains.[92] 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."[93]
-
- 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?[94] Or Alison Harrison, former director of public
relations for the Cattlemen's Association, as her official spokeswoman?[95]
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"?[96] 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,[97] millions of Americans
will remain at risk.
-
- Michael Greger, M.D., has been the Chief BSE Investigator
for Farm Sanctuary since 1993 and the Mad Cow Coordinator for the Organic
Consumers Association since 2001.
-
- For periodic updates on the Mad Cow crisis send a blank
email to DrGregerMadCowUpdates-subscribe@lists.riseup.net
-
-
-
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