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

[Guest guest]

" Could Mad Cow Disease Already be Killing Thousands of

Americans Every Year?

January 7, 2004 by Michael Greger, M.D. for the

Organic Consumers Association

 

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

spongiformencephalopathies, 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.

 

For updates on this evolving crisis, visit the OCA Mad

Cow page or send a blank email to

DrGregerMadCowUpdates-@l...

 

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 report U.S. Violates WHO Guidelines for Mad Cow

Disease " .

 

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@c...

 

 

Any part of this report may be reproduced subject to

acknowledgment.

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80 The Wall Street Journal. 30 November 2001.

81 Beacon Journal (Akron). 5 June 2001.

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82 New York Times 30 January 2001.

83

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130.html

 

84 Altman, Lawrence K. " Four States Watching for Brain

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85

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87 Case Western Reserve University Magazine - Summer

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88 Case Western Reserve University Magazine - Summer

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89 USA Today. 7 January 1999.

90 Philip Yam. The Pathological Protein: Mad Cow,

Chronic Wasting, and Other Deadly Prion Diseases. New

York: Springer-Verlag Press, 2003.

91 Rampton, S and J. Stauber. Mad Cow USA: Could the

Nightmare Happen 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 "

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"

 

 

 

 

karl theis jr

 

 

http://groups.msn.com/exposureofthetruth

 

 

 

 

 

 

 

 

 

 

 

 

 

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