Could Mad Cow Disease Already be Killing Thousands of Americans Every Year?

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Could Mad Cow Disease Already be Killing Thousands of Americans Every Year?

by Michael Greger, M.D.

January 7, 2004

 

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.

 

 

PLEASE FEEL FREE TO FORWARD OR REPRINT ANY PART OF THIS REPORT.

 

For updates on this evolving crisis, visit

http://www.organicconsumers.org/madcow.htm or send a blank email to

DrGregerMadCowUpdates-

 

For background on this important issue, read the excellent book Mad

Cow U.S.A., the full text of which is available free online at

http://www.prwatch.org, or my article " U.S. Violates WHO Guidelines

for Mad Cow Disease " at

http://organicconsumers.org/madcow/GregerBSE.cfm.

 

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. Dr. Greger has debated the National

Cattlemen's Beef Association 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 runs the Mad Cow

disease website http://www.organicconsumers.org/madcow.htm. Dr.

Greger is a graduate of the Cornell University School of Agriculture

and the Tufts University School of Medicine. He can be reached for

media inquiries at (206) 312-8640 or mhg1.

 

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Here? Common Courage Press; (September 1997):149-50. Full text

available free online at http://prwatch.org/books/madcow.html

92 Food Chemical News 25 March 1996: 30.

93 Food Chemical News 5 July 1993: 57-59.

94 http://www.philly.com/mld/inquirer/5884855.htm

95 http://organicconsumers.org/madcow/usda1204.cfm

96 http://www.prwatch.org/prwissues/1998Q1/oprah.html

97 " World Health Organization says BSE is a major threat "

http://www.organicconsumers.org/madcow/BSE7601.cfm

 

 

--

Michael Greger, M.D.

Chief BSE Investigator for Farm Sanctuary

http://www.nodowners.org

Mad Cow Coordinator for the Organic Consumers Association

http://www.organicconsumers.org/madcow.htm

(617) 524-8064

(206) 312-8640

mhg1

185 South St #6

Boston, MA 02130

 

For periodic updates on the Mad Cow crisis send a blank email to

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