April 23, 2002 Chapter from Everything You Know Is 
Wrong by Gabe Kirchheimer


The perfect pathogen has arrived: Millions of people may be infected, 
and planet earth will never be the same.
The advent of widespread mad cow disease--and the corresponding human 
epidemic of Creutzfeldt-Jakob disease (CJD)--
has many nations on high alert. The US Department of Agriculture 
adamantly denies mad cows exist in America.
However, leading private and government researchers, several key 
studies, and even statistical probability contradict
these assertions. The agency itself has admitted that ìthe potential 
risk of amplification of the BSE agent is much
greater in the United Statesî than in Britain. With 100 million head 
of cattle, America could soon be revealed as the
planet's biggest mad cow sanctuary.

In the wake of terrorist attacks on America, public health threats from within have been increasingly ignored. A new awareness of bioterrorism has been sparked by the discovery of anthrax spores sent around the country. Yet right under the nose of America, a fatal, untreatable, and deeply hidden biological threat appears to be spreading virtually unchecked in the US. Harder to detect than anthrax and far more stealthy, the perfect pathogen was not engineered or spread by a terrorist group. It did not escape from a top-secret military facility. The agent is not a virus, nor a bacterium, and it contains no DNA. It is not even alive.

The perfect pathogen--which causes mad cow disease in cattle and Creutzfeldt-Jakob disease in humans--is a malformed protein molecule known as an infectious prion, and until recently, the unprecedented mechanism of its awesome destructive power was disbelieved by many of the world's leading biologists.

The presence of the infectious agent in livestock is assured in perhaps half the countries of the world, although only a fraction have admitted it (including the UK, Ireland, France, Germany, Italy, and Japan). While desperately denying the existence of mad cow disease on its own soil, America continues to profit from the honesty of its affected trade partners. This arrangement is quietly destroying the health of the nation, but business is booming.

Are you familiar with CJD? Welcome to a living hell. Take a brief walk with me while I tell you of the most horrifying disease known to mankind. --Dolly Campbell, whose husband died of Creutzfeldt-Jakob disease

It's a Mad, Mad, Mad, Mad World

Every so often, a plague comes along with the power to shape nations. Such a plague is mad cow disease, or bovine spongiform encephalopathy (BSE), which first made international headlines in March 1996, when British authorities and the World Health Organization were forced to admit that ten human deaths from the apparently rare brain-wasting Creutzfeldt-Jakob disease (CJD) were ìlikelyî to be directly related to eating tainted beef.

The increase in transmissible spongiform encephalopathies (TSEs)--which includes BSE and CJD--among livestock and people is now recognized as an expanding worldwide plague. Tests in Europe, where most countries routinely fed millions of recycled cattle corpses back to cows until the crisis broke, have revealed many cases of BSE, in addition to the 177,000 confirmed in Britain, which has incinerated nearly five million cows as a result. Consumption of British beef has plummeted; financial losses have been catastrophic.

The disease vector--tainted cattle feed containing the ground-up remains of cows harboring infectious prions--has been shipped all over the world, a million tons to Asia alone. In September 2001, Japan confirmed the presence of mad cow disease within its borders, devastating its domestic beef market almost overnight, while the world reacted with another round of import bans.

Nobody knows how many people have contracted new-variant Creutzfeldt-Jakob disease (nvCJD) through contaminated beef and byproducts. Not only meat, but many processed foods, drugs, vaccines, surgical instruments, dietary supplements, and even cosmetics may carry this plague, spread mainly through the forced cannibalism of millions of bovines. In Britain and beyond, maternal transmission of nvCJD presages generations of victims. There is no treatment or cure. Experimental tests for the living recently have been developed, but there is no indication of when they'll be available.

A Clever, Indestructible Protein

Infectious prions represent truth stranger than science fiction. Virtually indestructible, they represent an entirely new class of pathogen. Not a living organism, the abnormal version of a protein known as a prion is able to withstand conditions which kill any other known pathogen, representing a biological threat never before seen on Earth. With unique abilities to survive temperatures upward of 1,100 F, jump species barriers, evade the immune system, and replicate themselves in victims whose very bodies remain infectious, these rogue proteins are sowing widespread devastation among animals and humans. Even HIV is neutralized by boiling water, but routine sterilization procedures are ineffective against this misfolded molecule, which destroys brain tissue by filling it with spongy holes.

The 1997 Nobel Prize in Physiology or Medicine was awarded to San Francisco scientist Stanley Prusiner for his discovery of ìprions--a new biological principle of infection,î even as others expressed incredulity at an infectious agent containing no genetic material whatsoever. Thought to replicate in the manner of crystals, abnormal prions malform neighboring prions upon contact, causing them to ìfoldî improperly and mutate their neighbors in a domino effect of devastation, until the host develops vacuoles in the brain, loses nervous system function, and dies. Unlike normal prions, mutants do not break down when meat is digested. The immune system is not provoked to attack the invader, because normal and rogue prions are almost chemically identical.

The long-term implications for the planet and its human and animal inhabitants are staggering. The number of vehicles which may harbor this hidden killer reads like a shopping list of common products. Not even vegetarians are immune: White sugar is bleached with cow bones, and McDonalds French fries, advertised as prepared in ìpure vegetable oil,î are seasoned--like many products with ìnatural flavorsî--with beef fat.

Mad Deer, Sad People

In the Southwest, an outbreak of chronic wasting disease, the TSE affecting deer, elk, and other ungulates, is now raging, with 5 to 15 percent of elk in areas of Colorado and Wyoming reportedly infected. The case of Doug McEwen--a 30-year-old hunter who died of CJD in Utah on March 28, 1999--starkly illustrates the tragedy surrounding the illness. McEwen, who regularly ate deer meat, was diagnosed with classic CJD although, like many of the British victims, his youth might seem to indicate another, more virulent strain, as only 1 percent of classic CJD patients develop symptoms at his age. McEwen's situation was graphically reported by Mark Kennedy in the Ottawa Citizen the day before he died:

Tracie McEwen reaches over to the dying man... As he moans softly, she strokes his arm and kisses his forehead. ìIt's OK. Doug, it's OK.î

Tracie married Doug exactly four years ago. She marked their anniversary by pouring sparkling cider into cups, making a toast, and lovingly dropping some into Doug's mouth....

It started slowly. First, there was the memory loss and the inability to do simple math, then the light tremors. Eventually came violent seizures as well as unexplainable outbursts of emotion--hysterical laughter, sometimes followed by uncontrollable crying. By late January, he could no longer speak in sentences....

ìThis is the worst thing I have seen,î [Tracie McEwen] says. ìI wouldn't wish it on my worst enemy.î

Inexplicably, blood plasma donated by McEwen was cleared by the authorities and distributed during his illness and after his death. For nearly two years McEwen had donated blood plasma, which was processed by Bayer into fractionated blood products in Clayton, North Carolina, then shipped to 46 countries around the globe. ìThe scope of this is breathtaking,î Dr. Tom Pringle says of the decision to release blood contaminated with CJD. ìYou've got a time bomb ticking in millions and millions of people. And as they become donors, it spreads further.î Pringle is a molecular biologist and founder of the astonishingly extensive Official Mad Cow Website , and his comments have appeared in mad cow articles in the New York Times.

Of the infected deer which almost certainly led to McEwen's death, Pringle is unequivocal: ìI think they have scrapie. Most cases trace back to Ft. Collins, Colorado, at the Foothills Research Station, an experimental facility which was contaminated,î a contention shared by several other CWD researchers. Wild animals might also contract the disease by raiding contaminated feed meant for livestock.

State of Emergency

Although the existence of mad cow disease is unconditionally denied by the American authorities, the prevalence of TSEs in other farmed livestock has been cause for two recent Declarations of Emergency by the USDA.

Effective February 1, 2000, then-Secretary of Agriculture Dan Glickman proclaimed a ìDeclaration of Emergency Because of Scrapie in the United States,î due to a clear epidemic:

Scrapie, a degenerative and eventually fatal disease affecting the central nervous systems of sheep and goats, is present in the United States. Scrapie is a complicated disease because it often has an extremely long incubation period without clinical signs of disease. Currently, scrapie-free countries have an enormous competitive advantage over US sheep producers, who are unable to certify that their flocks originated from a scrapie-free country or region. Because importing countries are demanding that imported sheep come from scrapie-free regions and sheep producers in the United States are unable to make this certification, US producers are finding themselves locked out of the international market, a situation that is taking a serious financial toll on the US sheep industry.... Therefore...I declare that there is an emergency that threatens the sheep and goat industry of this country, and I authorize the transfer and use of such funds as may be necessary from appropriations or other funds available to the United States Department of Agriculture to conduct a program to accelerate the eradication of scrapie from the United States.

This admission was followed by a ìDeclaration of Emergency Because of Chronic Wasting Diseaseî issued by Secretary of Agriculture Ann Veneman, effective September 21, 2001:

Chronic wasting disease (CWD), a disease of deer and elk, is part of a group of diseases known as transmissible spongiform encephalopathies (TSEs), a group that also includes scrapie and bovine spongiform encephalopathy (BSE). While considered rare, the incidence of CWD is on the rise among both wild and domestic cervids. The disease, which occurs mostly in adult animals, is progressive and always fatal. The origin and mode of transmission of CWD are unknown. The disease has become of particular concern due to its fatal nature, lack of known prevention or treatment, its impact on the farmed cervid industry, and its possible transmissibility to cattle or other domestic livestock and humans.

Scrapie, the mad-sheep analogue suspected of infecting British cattle with BSE, has spread unchecked to 45 states.

On October 25, 2001, Reuters reported: ìCompanies that make amino acids used in pharmaceuticals and vaccines should not use cattle and sheep from mad cow-infected countries as a source, a US advisory panel said Thursday.... Current manufacturing processes cannot guarantee that prions, the infectious material thought to cause mad cow disease, would not be transmitted from amino acids to the end product.î

A Reuters article published the next day, ìFDA Urged to Consider Ban on Cow Brain Products,î stated:

The US Food and Drug Administration (FDA) may soon consider banning the sale of any product containing cow brains or spinal tissue, whether made abroad or here in the US.

Expert advisors to the FDA voted 18 to 1 on Friday in favor of urging the federal agency to begin assessing the necessity and feasibility of passing regulations to either ban or restrict the use of products containing these tissues, due to the theoretical risk of ìmad cowî disease.

These products range from soup stock and sausage casings to cosmetics, drugs, medical devices and dietary supplements....

But if the FDA should follow its committee recommendation, there are unlikely to be any immediate consequences. The FDA's rule-making process could take months and even years to complete, while the agency reviews the available data and upcoming studies.

The WSJ Checks In

With the government issuing emergency decrees for sheep, goats, deer, and elk in response to widespread TSE infections among domestic and wild animals--and with the FDA considering a ban of products, including those containing domestic bovine nervous system tissue--it seems inevitable that mad cows will rear their spastic heads, even as big business desperately tries to bury the truth.

On August 29, 2001, none other than the Wall Street Journal ran an editorial, ìMoo Over, Mad Cow Comethî by Holman W. Jenkins, Jr., which admitted the futility of postponing the inevitable:

ìNot a single case of mad cowî has been the proud mantra of the US beef industry since the disease was discovered in Britain 15 years ago. Not finding a case, though, has been largely a function of not looking especially hard.... Looking is often finding, so this would seem to bode a consumer panic and economic disaster if mad cow is as widely spread as many experts believe. The US cattle industry long ago convinced itself that a single case would mean curtains for its $3.6 billion in annual beef exports, not to mention a bruising domestic whack as consumers defect to chicken, pork or--horrors--soy burgers....

Washington and the cattle lobby have spent a decade praying mad cow doesn't show up here, despite knowing it must sooner or later. Though 36 million head are slaughtered a year, the Agriculture Department has examined all of 12,000 brains since 1990. The time has come to gear up a real hunt for our first case, if only to get it over with.

CJD and nvCJD

CJD and BSE are both TSEs, which are invariably fatal. But not every case involves infection from contaminated material. Naturally occurring, or ìsporadic,î TSEs afflict humans, bovines, and many other animals at the rate of one in a million. Sporadic CJD, which primarily affects the elderly, can incubate for decades before leading to loss of coordination, horrific mental breakdown, and death.

The 100 British victims of nvCJD--which has a shorter incubation period than CJD--have been mostly younger people between 13 and 40 years of age. ìHealth officials say they've got mad cow under control, but millions of unaware people may be infected,î warned a Newsweek cover story on March 12, 2001. ì[O]nce a few cattle contracted it, 20th-century farming practices guaranteed that millions more would follow. For 11 years...British exporters shipped the remains of BSE-infected cows all over the world [to] more than 80 countries.î The stakes are extremely high. One infected animal, whose remains are rendered, powdered, and mixed into feed, can infect thousands of other animals, plus the thousands of people who eat them.

All the British nvCJD victims express a genetic trait shared by 38 percent of the British population and all bovines. Jun Tateishi, professor emeritus of Japan's Kyushu University and an authority on prion study, explains: ìBasically, there are differences in genes...between humans and animals. Humans have three types of [paired] gene structures: methionine, valine, and a combined type. On the other hand, a cow has only the methionine type,î which apparently enables the effective transmission of BSE prions to humans carrying the same methionine pairing. ìWhat we should note is that 91.6 percent of Japanese have the methionine gene type. Compared to British people, the rate is overwhelmingly high. I can't say so for sure yet, but my opinion is that Japanese are about 2.5 times more likely to get mad cow disease than British people.î No test for this genetic trait is available.

A Different US Strain?

Over the last decade the USDA has tested over 12,000 cow brains, looking for the pathology seen in infected British cattle, and it continues to claim that not a single BSE-infected cow has been found. The US Centers for Disease Control and Prevention (CDC), which has refused to mandate CJD as a reportable disease in the face of many petitions, similarly asserts that only about 280 to 300 people a year die from it (about one for each million Americans, the standard rate for the naturally occurring variety), with no nvCJD detected in the US.

But what if America has been harboring a different and stealthy strain of BSE all along, with a corresponding variant of CJD, and neither were being detected by current methodology? ìI don't expect the British strain of mad cow disease to be much of a problem here,î says Dr. Pringle. ìThe main fear is that our own cattle may carry a different strain of the disease that is distinct from the British strain.î TSEs are known to exist in numerous strains within a single species; sheep scrapie has at least 20 variants.

In Britain, speculates Pringle, ìthe top level of government itself does not know--nor want to know--the scope of the epidemic. This is to establish ëplausible deniability.'î It would appear the US is also burying its head in the sand.

America's Epidemics

The evidence for epidemics of both BSE and CJD in America is persuasive:

1. In 1985, Dr. Richard Marsh, a TSE researcher at the University of Wisconsin investigating a mysterious outbreak of transmissible mink encephalopathy (TME) in that state, found that the minks' diet consisted almost exclusively of ìdownerî cows--animals too sick to stand.

In 1994, Marsh showed that when the brains of infected cattle were fed to healthy mink, they developed TME; healthy cattle inoculated with tissues obtained from TME-infected mink duly developed BSE. These experiments showed ìthe presence of a previously unrecognized scrapie-like infection in cattle in the United States.î

The disease was different than that seen in Britain. Significantly, rather than exhibiting overt mad cow symptoms (European cattle with BSE usually act skittish and ìcrazyî before death), the US animals simply collapsed. In 1990, cows in Texas experimentally inoculated with American scrapie developed BSE, becoming lethargic and staggering to their deaths, just like downer cows. Some states, such as New York, don't send downer cows to the USDA for testing, leaving open the possibility that BSE in thousands of suspect animals is going undetected.

According to Prionics, which manufactures Europe's leading BSE test: ìA study performed with Prionics-Check reveals that fallen stock...represent BSE high-risk categories.î

2) Leading scientists aver that mad cows surely exist in the US. Dr. Clarence Gibbs--a preeminent TSE researcher who chaired a World Health Organization investigation into BSE and ran the laboratory of the National Institute of Neurological Disorders and Stroke until his death--had no doubts about domestic infection: ìDo I believe BSE is here? Of course I do.î

And Dr. Stanley Prusiner, who won the 1997 Nobel Prize in Medicine for his discovery of prions, expressed that contention to a congressional caucus in May 1996. That June, an article in Food Chemical News stated, ìAfter more than two decades of research on prions, Stanley Prusiner of the University of California at San Francisco suggested that mad-cow disease must be present in US cows.... He said he agreed with [Richard Marsh] who believes mad-cow disease was linked to US cows in the mid-1980s.î

ìThirty-seven million animals are slaughtered a year for consumption, and less than 1,000 are tested a year--it's too low,î says Pierluigi Gambetti, the director of the CDC's National Prion Disease Pathology Surveillance Center. ìIf you don't look, you don't find it. Our testing is not on the cutting edge.î Nearly one million animals are tested by both France and Germany every year.

What would the USDA do upon discovering a case of BSE? ìTheir first impulse would be to suppress it,î asserts Dr. Michael Hanson, a senior research scientist at the Consumer Policy Institute of Consumers Union (publisher of Consumer Reports) and one of the country's leading food-safety experts. Of the government's TSE-detection program, Hanson reiterates, ìTheir strategy might be, act like you're looking, but really do a ëdon't look, don't find.'î

As Pringle points out: ìAbsence of evidence is not evidence of absence.î

3) In spite of the USDA's categorical denials, it's a scientific fact that one in a million cows naturally develops BSE. With about 100 million cattle in the US, that would mean approximately 100 mad cows exist on American soil at any given time. Many likely collapse before scheduled slaughter and are rendered into feed, with the potential to infect thousands of other animals.

4) The best evidence for widespread, hidden CJD is contained in a pair of revelatory university studies. Hanson has repeatedly pointed to the evidence: ìA study at the University of Pittsburgh, in which autopsies were done on 54 demented patients diagnosed as having probable or possible Alzheimer's or some other dementia (but not CJD), found three cases (or 5.5 percent) of CJD among the 54 studied. A Yale study found that of 46 patients diagnosed with Alzheimer's, six (or 13 percent) were CJD at autopsy. Since there are over two million cases of Alzheimer's disease currently in the United States, if even a small percentage of them turned out to be CJD, there could be a hidden CJD epidemic.î

These shocking figures indicate that tens and perhaps hundreds of thousands of Americans are currently infected with a preventable variant of CJD. Since sporadic CJD occurs in only one in a million people, an infectious source must exist.

A Rendered Disaster

The common practice of feeding rendered protein supplements--the boiled-down, powdered remains of slaughterhouse and other animal waste--to domestic animals spread BSE in the UK. Surviving high heat and solvents, mutant prions from each BSE-infected cow infected thousands of other bovines, as huge batches of feed were mixed and fed back to cattle in a bovine version of Soylent Green's forced cannibalism.

Feeding mammalian protein to ruminants (cud-chewing animals) was authoritatively banned in the UK in 1989. Eight years later, in August 1997, the FDA tardily issued weak regulations addressing this common practice. Consumers Union's Hanson explained the US ban: ìAll they said is that you've got to label it, ëDo not feed to cattle and other ruminants.' Farmers can walk in a feed store and still buy it. Nobody asks, ëAre you feeding it to cattle or pigs?' They have to keep records of where the material came from for one year, for a disease with an average incubation period of five years. It's a joke. The way the rule is written, you can take scrapie-infested sheep, CWD-infested deer, and BSE-infested animals and legally put that in animal feed and give it to pigs, chickens--anything but ruminants, as long as it's labeled. That's outrageous.î On top of that, USDA feed-rule compliance among America's thousands of livestock farmers is virtually impossible to effectively monitor or enforce.

Incredibly, Hanson noted in 1999, ìThe new thing is to feed calves spray-dried bovine plasma. It's hardly processed, so you're not knocking down the infectivity--and you can put it right in the feed.î

But calves are not the only hapless recipients; Hanson believes the industry is likely feeding cows ìa huge amount of bovine blood products. Legally, you can take any blood product from cattle and feed it to cows. I've been told that cows won't eat feed with more than ten percent blood, because they can taste it, and that chickens will eat feed with up to thirty-five percent blood.î Blood has been shown capable of containing infectious prions.

What Goes Around, Comes Around

In spite of the successful initiative by the European Union to ban all animal products in livestock feed, American animal agribusiness continues to make widespread use of rendered protein and feed containing animal parts.

Under current feed regulations, livestock often eat one another's remains. After inedible pig parts are rendered, they are often fed back to pigs, cows, and chickens; cow parts are fed to chickens and pigs; and pigs and chickens are still routinely fed rendered protein that includes the remains of downer cows, which are most suspect for harboring BSE.

Perhaps most repugnant, thousands of tons of fermented chicken manure are fed to millions of US cows each year in a bizarre loop of inexpensive husbandry. Hanson and Pringle believe that ìcow to chicken manure to cowî could turn out to be a BSE vector path; infectious prions apparently survive ingestion and could plausibly make the round-trip on this perverse journey.

As for the question of whether fowl can contract TSEs from livestock, the issue has ìnot really ever been investigated,î says Pringle. ìNo one wanted to know, because so much cattle bone meal is fed to chickens. However, the chicken prion has a strong similarity to the mammalian amyloidogenic region, so it is theoretically possible.î

It remains possible that all domestic animals may indeed be susceptible to TSE infection. According to Hanson, the USDA has ìfunctionally ignored the potential TSE in pigs.î Their very short factory-farm lifespan of six to eight months might hide any symptoms of TSE, which usually spends several years incubating in mammals. Dr. Paul Brown, a senior investigator for the National Institutes of Health and the author and coauthor of numerous TSE studies, also has indicated that poultry and especially pigs could harbor TSEs and pass them on to humans. ìIt's speculation,î Brown has acknowledged, ìbut I am perfectly serious.î

Pigs that were experimentally inoculated have developed BSE, and a suspected outbreak of porcine spongiform encephalopathy occurred near Albany, New York, in 1997. A 1973 study published in the American Journal of Epidemiology discovered that ten of 38 CJD patients had eaten hog brains.

Big Beef and the USDA

Critics contend the $150 billion-a-year cattle industry is itself infected with agribusiness greed, preventing any possibility of truthful or timely disclosure of mad cows. Although American beef consumption has been cut nearly in half since 1980 (while chicken and pork have risen), the beef industry has rarely been as lucrative, with 85 percent of cattle farmers reporting profitability in 2000, up from only 15 percent in 1996. Ironically, Europe's crisis has been a huge boon to ìBSE-freeî American beef exports, which shot up 34 percent in 2000, with shipments to the Russian Federation increasing twenty-fivefold. Mad cow disease has clearly been great for business, although McDonald's has suffered large European and Japanese losses in the wake of widespread beef avoidance.

With America's sacred cow at stake, many doubt the USDA will voluntarily reveal the discovery of any BSE-infected cows--which would lead to certain market collapse and public panic. Dr. Michael Gregor--a physician who was one of the earliest critics of the US's handling of the BSE threat (and is the Webmaster of the successor to Pringle's mad cow site )--points out that the ìUSDA has a conflict of interest, as the agency is responsible both for consumer safety and the promotion of American agriculture, of which meat is the primary industry.î He notes that industry groups have successfully lobbied against changes in the USDA's research program to accommodate the possibility that BSE is already present in the US.

In the absence of sufficient inspectors and vigorous monitoring, the agency puts its trust in the beef industry to implement its rules. Allegations that the relationship between the two entities is overly cozy were fortified with the appointment of President Bush's USDA staff. On February 11, 2001, the New York Times reported: ìAlthough they have had a record year, cattle ranchers in the United States now face growing anxiety over mad-cow disease...which could drive down beef prices. But last week, they triumphed when Ann M. Veneman, the new agriculture secretary, named Dale Moore, a lobbyist for the National Cattlemen's Beef Association, as her chief of staff. Charles P. Schroeder, the association's chief executive, said the cattle industry was investing heavily in food safety and looking forward to working with its former advocate.î

Failed Regulations

The US has failed to close gaping loopholes in the firewall against mad cows, and the feeding of potentially infectious cow parts back to cattle continues largely unmonitored. In early 2001, the FDA charged livestock-feed producers and rendering plants--which powder a variety of animal waste for use as a cheap feed supplement--with widespread noncompliance with labeling and mixing regulations.

The next day, the New York Times followed with a front-page article describing the lapses: ìLarge numbers of companies involved in manufacturing animal feed are not complying with regulations meant to prevent the emergence and spread of mad-cow disease in the United States.... All products that contain rendered cattle or sheep must have a label that says, ëDo not feed to ruminants.'... Manufacturers must also have a system to prevent ruminant products from being commingled with other rendered material.î

The issue of monitoring America's thousands of cattle farmers, the end-users of rendered feed, has not been addressed by the Food and Drug Administration, which primarily monitors interstate commerce.

Brain and spinal cord tissue are the primary--but not the only--reservoirs of infectious material in humans and animals. Current USDA and FDA regulations are designed to prevent this material from ending up on the American dinner plate, but the automatic meat-recovery (AMR) systems in wide use at modern slaughterhouses, which mechanically strip the spine of flesh, routinely include banned material in the meat. The USDA and the federal Food Safety and Inspection Service have found spinal-cord fragments and nervous-system tissue in AMR meat samples. It has also been shown that, upon impact on the skull, pneumatic slaughterhouse stun guns can force bovine brain matter into the bloodstream and edible tissues.

On August 10, 2001, the Center for Science in the Public Interest petitioned the USDA to ban AMR ìmeatî from the human food supply. Warning that cattle are better protected from mad cow disease than people, CSPI Food Safety Director Caroline Smith DeWaal stated:

Machines that strip meat from bones provide the best pathway for BSE to get into human food. While the Foodand Drug Administration in 1997 banned the use of processed cattle parts in making cattle feed, USDA has not taken adequate precautions to protect the human food supply. US cattle aren't allowed to eat cattle spinal cord--and neither should people.

The CSPI press release notes:

AMR meat paste typically is used in the production of hundreds of millions of pounds of hot dogs, hamburgers, pizza toppings, and taco fillings, and although USDA has asked companies to remove spinal cord from the spinal column and neck bones before they enter the machines, the agency rarely checks the industry's compliance. Since 1998, USDA has tested approximately 100 samples of AMR meat for spinal cord. Of those, nine samples tested positive for this central nervous system tissue.

ìAlthough the department [of Agriculture] classifies the tissues as being ënot meat,' their presence in a meat product is not a violation of food safety laws,î notes reporter Lance Gay of the Scripps Howard News Service. ìMuch of the mechanically separated meat is sold to the school-lunch program, which the department also administers.î

The Zoo Loop

TSEs have been observed in numerous rodent, primate, and ungulate (hoofed) species, and in various felines such as cheetahs and domestic cats. During the late 1980s and the 1990s, numerous French zoo primates, felines, and hoofed animals were shown to have TSEs. ìLarge numbers of monkeys and lemurs in French zoos appear to be infected with the agent that causes ëmad cow disease,' according to a provocative study published today in Proceedings of the National Academy of Sciences,î wrote the New York Times' mad cow reporter, Sandra Blakeslee, in March 1999. ìThe finding is bad news for people living in Britain who fear that a human form of mad cow disease, called new variant Creutzfeldt-Jakob disease, or CJD, may have similar underpinnings.î

Tom Pringle commented on this study: ìThis is a huge scandal because it potentially affects the survival of many of the world's primate species. It also suggests very strongly that the nvCJD epidemic will indeed be a ëplague of biblical proportions'î (quoting a warning given by prominent neurogeneticist John Collinge, a member of the British government's Spongiform Encephalopathy Advisory Committee).

Got Mad Milk?

It's rarely mentioned, but infectious prions can be contained in milk, although it remains a remote vector. A 1992 Japanese study published in the New England Journal of Medicine showed that human breast-milk colostrum (the first milk a baby receives) is capable of transmitting prions, and the infection of lambs with scrapie through milk has also been demonstrated. It is not clear whether post-colostrum milk possesses this capacity. Some people have chosen to avoid cheese from the UK as a precautionary measure; many hard cheeses contain rennet, an enzyme extracted from the stomachs of calves.

Supplements, Vaccines, Blood, and Medical Instruments

Other routes of infection remain of grave concern. Direct inoculation presents the highest risk. Despite warnings from Pringle and others, US vaccines, which are often grown in bovine calf serum, are still being made from suspect materials. In February 2001, the New York Times finally picked up the story (curiously placed in the business section) under the headline, ì5 Drug Makers Use Material With Possible Mad-Cow Link.î The article stated, ìFor the last eight years the FDA has repeatedly asked pharmaceutical companies not to use materials from cattle raised in countries where there is a risk of mad-cow disease.... But regulators discovered last year that...some of the world's largest drug concerns were still using ingredients from those countries to make nine widely used vaccines...[which] include some regularly given to millions of American children, including common vaccines to prevent polio, diphtheria and tetanus.î The list also includes flu shots and the hepatitis vaccine.

Numerous dietary supplements containing glandular material, brains, and other bovine ingredients are also at high risk. ìVelvet Antlerî capsules from General Nutrition Centers and many other retailers ìcome from the growing antlers of elk and can contain infectious agents,î says Hanson. ìThey're filled with nerve tissue and blood. I wouldn't want to be the one to be experimented on.î

ìIt's just insane not to have greater safeguards [for supplements],î the chair of the FDA's Advisory Committee on Mad Cow Disease, Dr. Paul Brown, told the Times. ìThe FDA is toothless.î

The American Red Cross, which collects half the US blood supply but doesn't test for CJD, now bans blood donations from Americans who have spent three months in Britain or one year elsewhere in Europe. The strict ban has created a predicted national shortage of blood, especially in New York City, where 25 percent of the red-cell supply was until recently imported from FDA-approved European blood banks. The Red Cross estimates that the current ban will cut nationwide blood donations by 6 percent.

In the absence of strict government regulations, some medical organizations have voluntarily recalled large lots of fractionated blood products containing donations from people later found to have CJD, usually after some of the products have reached recipients. Over the past ten years, at least $100 million worth of plasma products has reportedly been destroyed.

Many drugs are derived from cattle, including growth hormones from pituitary glands, adrenaline products, cortisone, insulin for diabetics, and medications for the treatment of stomach ulcers. Thromboplastin, a common blood coagulant used in surgery, is derived from bovine brains. Pituitary extracts from mad cows (as well as human donors with CJD) have been traced as the cause of CJD infection in recipients.

ìThe thing that worries me is the immunization of the children,î says Pringle. ìEvery kid in the United States can't go to school without their shots... They're growing vaccines out of fetal-calf serum. Then you're injecting four-year-old children--which is much worse than eating, 100,000 times more effective [at spreading the disease]. Every schoolchild in the UK has already been immunized with vaccine made from serum from infected bovines.î

Surgical instruments are at high risk of transmitting the infection, as autoclave steam sterilization doesn't neutralize infectious prions. Blood, blood products, bovine extracts, and transplant organs--such as brain dura mater and corneas--are not usually screened for CJD, even though in Britain and around the world, infected organ recipients, who sometimes developed symptoms decades after treatment, have been traced to unwitting donors later found to have CJD. Effective prion sterilization protocols are not in wide use, but disposable surgical instruments are now used in many British procedures. It is inevitable that worldwide sterilization protocols will undergo drastic modification in the face of the prion.

When Will We Get the Test?

The spread of BSE has given birth to the emerging industry of prion diagnostics, which is rapidly growing to fill a demand for tests. Although postmortem tests for BSE are now widely used in Europe, antemortem (i.e., pre-death) tests for BSE and CJD are not yet commercially available.

A urine test developed by Israeli researchers at the Department of Neurology at Hadassah University Hospital, described in the Journal of Biological Chemistry (21 June 2001), promises to meet the need for a simple TSE test for live human and animal subjects. (The researchers note that the presence of infectious prions in urine indicates that they are being widely dispersed in soil, which has been experimentally shown to preserve prion infectivity over a period of years.) However, it is unclear how and when a viable CJD test will be released. In Britain, the expected demand from millions of panicked individuals--concerned they may have a horrible brain-wasting disease--may delay screening while public policy for dealing with the results is formulated. A finding of thousands or even hundreds of thousands of cases, as has been projected by some researchers, could drastically alter British society. Already, several cases of suicide by the ìworried wellî--persons convinced they were developing CJD--have been recorded in Britain.

British public health officials have been widely castigated for incompetence, delays, and cover-ups in dealing with the BSE/CJD crisis. A crucial five-year study into whether British sheep have BSE was admitted to be ruined in mid-2001 by cross-contamination with bovine material. In the latest chapter of the cover-up, according to BBC News, the costly error wasn't announced until three months after its discovery.

The slow responses of Britain, the US, and other nations to the AIDS crisis are recalled by relatives of CJD victims, who hope this legacy of statistical obfuscation, delays in test availability, and poor dissemination of prevention information will not be repeated. Although Britain may be the first nation where widespread CJD testing occurs, testing in Japan, the US, and elsewhere will surely follow. It remains unclear which governments will promote or downplay the importance of CJD screening. With CJD, as with BSE, not looking is not finding.

Time Will Tell

It has been proven experimentally that even fly larvae, after eating infected tissue, can transmit scrapie to hamsters; the larvae were still infectious after death. Nevertheless, the US government handbook BSE Red Book--Emergency Operations states: ìCleaning and disinfection is not necessary to prevent the spread of BSE.î

Pringle is not optimistic. In the US, ìit would be a wrenching experience to totally get away from the bovine economy, and realistically, they're only going to take half-measures. It's like a joke now to talk about containment. It's like locking the barn door after the horse is gone. WTO, NAFTA, has really helped globalize CJD. You don't know where your sutures are coming from, your shampoo, your sunscreen. The Pandora's box has been opened.î

In the absence of a CJD test, the world can only guess the extent of the problem. As CBS Evening News relayed: ìWhen asked if, in his darkest moment, he thought that this is the plague of the twenty-first century, [Dr. Prusiner] said, ëI don't need a dark moment to wonder if that's the case, because everybody's wondering that, not just me.' î