The Killer Among Us

[Guest guest]

The establishment will try and not make connections between cross species

transference.

 

Mad cow is called scrapie in sheep. Chronic wasting disease in deer and some

other animals and CJ in humans. They should call it the same disease whatever

animal it is affecting, but that would scare people if they were aware of the

extent of the problem.

 

It is affecting skunks, deer, minks, sheep, and no telling what else and these

idiots in Washington are saying they have got a handle on it and not to worry.

I am sure tired of elected officials who are nothing but liars and scam artists.

 

Frank

 

 

 

 

 

 

 

 

 

The Killer Among Us

 

By Michael Greger, AlterNet

January 7, 2004

 

In 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. Peter's death in 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. 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 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. The announcement

of their deaths, released on March 20, 1996 (ironically, Meatout Day), reversed

the British government's decade-old stance that British beef

was safe to eat.

 

 

 

It is now considered an incontestable fact that these human deaths in Britain

were caused by Bovine Spongiform Encephalopathy (BSE), or Mad Cow disease.

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 at

temperatures hot enough to melt lead. 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. "

 

 

 

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.

Scientists called this new form of the disease " variant " CJD.

 

 

 

Other than Charlene, a 24 year old woman now 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 knows what causes sporadic

CJD. New research 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.

 

 

 

This is not the first time meat was linked to sporadic CJD. In 2001, a team of

French researchers found 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 some cases of sporadic CJD in the U.S.

are caused by sheep meat as well.

 

 

 

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 – and hundreds of thousands of downer pigs, too sick

or crippled by injury to even walk, arrive at U.S. slaughterhouses every year.

 

 

 

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. 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).

 

 

 

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 Prize

in Medicine for his work on prion diseases, as saying, " it's got to be in the

pigs as well as the cattle. It's got to be passing through the chickens. " 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. "

 

 

 

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 BSE-related. Switzerland, which has had a substantial BSE epidemic,

has noted a sharp recent increase in sporadic CJD. " In the Nineties, Switzerland

had the highest rate of Mad Cow disease in continental Europe, and their rate of

sporadic CJD doubled.

 

 

 

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.

 

 

 

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. The true prevalence of CJD

among any age group in this country remains a mystery, though, in part because

it is so commonly misdiagnosed.

 

 

 

The most frequent misdiagnosis of CJD among the elderly is Alzheimer's disease.

Neither CJD nor Alzheimer's can be conclusively diagnosed without a brain

biopsy, 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. 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. In younger victims, CJD is more often misdiagnosed as multiple

sclerosis or as a severe viral infection.

 

 

 

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.

 

 

 

Prion disease expert 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 deaths of about 3%. A third study, at the

University of Pennsylvania, showed that 5% of patients diagnosed with dementia

had CJD. Although only a few hundred cases of sporadic CJD are officially

reported in the U.S. annually, hundreds of thousands of Americans die with

dementia every year. 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. This means it can be years between eating infected meat and being

diagnosed with 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.

 

 

 

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. The petition

was denied. 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. But CJD is so often

misdiagnosed, and autopsies are so infrequently done, that this system may not

provide an accurate assessment.

 

 

 

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 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. 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.

 

 

 

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. " 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. "

 

 

 

" 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. "

 

 

 

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

explained that the " disadvantage " of these measures was that " the cost to the

livestock and rendering industries would be substantial. "

 

 

 

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, 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.

 

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