© 2002 Gabe Kirchheimer
The perfect pathogen has arrived, millions 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-has many nations on high alert. The US continues to deny Mad Cows exist in America, but the government's own data show they do.
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, the national media have been the targets of such incidents, and extreme fear and interest in terrorist activity have pushed many other issues to the margins of reportage. Of course, many unpopular concerns rarely make it to CNN regardless of the political climate, but ignorance is not always bliss.
Right under the noses of America, a fatal, untreatable and deeply hidden biological threat appears to be spreading virtually unchecked in the USA. 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 contains no DNA. It is not even alive.
The Perfect Pathogen-which causes Mad Cow disease 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. 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 officials of the World Health Organization and British authorities were forced to admit that 10 human deaths from the apparently rare Creutzfeldt-Jakob brain-wasting disease (CJD) were "likely" directly related to eating tainted beef.
The increase in TSEs 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 Third World, a million tons to Asia alone. Japan has confirmed the presence of Mad Cow disease, it's domestic beef market has been devastated almost overnight, and the world has 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 when they will be available.
A Clever Protein
Infectious prions represent truth stranger than science fiction. Virtually indestructible, they represent an entirely new class of pathogen-and medicine's worst nightmare. 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 the AIDS virus 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 long-term implications for the planet and its human and animal inhabitants are staggering. The number of vehicles which may harbor this hidden killer runs 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 "pure vegetable oil," are seasoned-like many products with "natural flavors"-with beef fat.
U.S. Cows: Sacred or Mad?
The US Department of Agriculture, which annually allowed a billion pounds of "rendered" protein to be fed to cattle before instituting a simple feed labeling regulation in 1997, adamantly denies mad cows exist in America, but leading private and government researchers, several key studies and even statistical probability contradict these assertions. The agency itself has admitted that "the potential risk of amplification of the BSE agent is much greater in the United States" than in Britain. With 100 million head of cattle, America could soon be revealed as the planet's biggest mad-cow sanctuary.
Meanwhile, chronic wasting disease (CWD), a similar condition which affects deer and elk, is epidemic in parts of the Southwest, and scrapie, the mad-sheep analogue suspected of infecting British cattle with BSE, has spread unchecked to 45 states. In addition to maternal transmission there is limited evidence that CWD, scrapie and BSE-all "prion diseases"-can be horizontally transmitted from one live animal to the next through close contact, perpetuating infection even in the absence of external agents. (Confusingly, Scrapie and BSE strains are capable of jumping between bovines and sheep, and a strain described as scrapie in sheep may be referred to as BSE when found in cattle, or the other way around, although such strain may have originated in the other species.)
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 Declaration of Emergency Because of Scrapie in the United States, 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 U.S. 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, U.S. producers are finding themselves locked out of the international market, a situation that is taking a serious financial toll on the U.S. 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 Declaration of Emergency Because of Chronic Wasting Disease issued by Secretary of Agriculture Ann Veneman, effective Sept. 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."
And on October 25, 2001 Reuters reported, "Companies that make amino acids used in pharmaceuticals and vaccines should not use cattle and sheep from mad cow-infected countries as a source, a U.S. 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."
A Reuters article the next day, "FDA Urged to Consider Ban on Cow Brain Products" by Ori Twersky, 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 'mad cow' 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. . . ."
WSJ Checks In
With the government issuing emergency decrees for sheep, goats, deer and elk in response to widespread TSE infection among domestic and wild animals, and the FDA considering a ban of products including those containing domestic bovine nervous system tissue, it seems inevitable 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, "Moo Over, Mad Cow Cometh" by Holman W. Jenkins Jr., admitting the futility of postponing reality. " 'Not a single case of mad cow' has been the proud mantra of the U.S. 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 U.S. 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-the human form of mad-cow disease-and BSE are both TSEs, which are invariably fatal. Naturally occurring or "sporadic" TSEs afflicts humans, bovines and many other animals at the rate of one in a million victims. 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-have been mostly younger people between 13-40 years of age. "Health officials say they've got mad cow under control, but millions of unaware people may be infected," warned a Newsweek cover story on March 12, 2001. "[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." The stakes are extremely high. One infected animal, whose remains are rendered, powdered and mixed into feed, can infect thousands of other animals, and the thousands of people who eat them.
All the British nvCJD victims express a genetic trait shared by 38% of the British population and all bovines. Jun Tateishi, professor emeritus of Japan's Kyushu University and an authority on prion study, explains, "Basically, 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," which apparently enables the effective transmission of BSE prions to humans carrying the same methionine pairing. "What we should note is that 91.6% 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." No test for this genetic trait is available; the other two variants (for pairs of amino acids at Codon 129 on the short arm of human Chromosome 20) might simply effect longer incubation periods in the rest of the population.
An Indestructible Protein
Not a virus, not a bacterium, the abnormal version of a protein known as a
prion is able to withstand conditions which kill any known pathogen, and represents
a biological threat never before seen on Earth.
The 1997 Nobel Prize in Physiology or Medicine was awarded to San Francisco scientist Stanley Prusiner for his discovery of "Prions-a new biological principle of infection," 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 "fold" improperly and mutate their neighbors in a domino pyramid 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.
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 continues to claim that not a single BSE-infected cow has been found. The US Centers for Disease Control (CDC), which has refused to mandate CJD as a reportable disease in the face of many petitions, similarly asserts that only about 280-300 people a year die from it (about one for each million Americans), 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? "I don't expect the British strain of mad-cow disease to be much of a problem here," says expert Dr. Tom Pringle, a molecular biologist and founder of the astonishingly extensive Official Mad Cow Website (mad-cow.org), and whose comments have appeared in mad-cow articles in the New York Times. "The main fear is that our own cattle may carry a different strain of the disease that is distinct from the British strain." TSEs are known to exist in numerous strains within a single species; sheep scrapie has at least 20 variants.
In Britain, speculates Pringle, "The top level of government itself does not know-nor want to know-the scope of the epidemic. This is to establish 'plausible deniability.' " It would appear the US is also burying its head in the sand.
The evidence for epidemics of both BSE and CJD 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 "downer" cows-animals too sick to stand. (100,000 downer cows die each year in the US, a fact first revealed on national television by Howard Lyman, a cattle rancher turned animal activist, on the Oprah Winfrey Show, April 15th, 1996, resulting in a widely publicized unsuccessful libel suit-a public relations disaster for the beef industry-against Oprah Winfrey by the Texas Cattleman's Association.)
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 "the presence of a previously unrecognized scrapie-like infection in cattle in the United States."
The disease was different from that seen in Britain. Significantly, rather than exhibiting overt mad-cow symptoms, the U.S. animals simply collapsed; European cattle with BSE usually act skittish and "crazy" before death. In 1990 cows in Texas experimentally inoculated with American scrapie developed BSE and became lethargic and staggered to their death, just like downer cows. Some states, such as New York, don't send downer cows for USDA 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, "A study performed with Prionics-Check reveals that fallen stock represent BSE high-risk categories."
2) The USDA describes their BSE testing program as "aggressive." However, leading scientists concur that mad cows surely exist in the U.S. Dr. Clarence Gibbs, a preeminent TSE researcher who ran the laboratory of the National Institute of Neurological Disorders and Stroke until his death on Feb. 16, 2001, and chaired a World Health Organization investigation into BSE, had no doubts about domestic infection: "Do I believe BSE is here? Of course I do." 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. A June 1996 article in Food Chemical News stated, "After 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 [Marsh] who believes mad-cow disease was linked to US cows in the mid-1980s."
"Thirty-seven million animals are slaughtered a year for consumption and less than 1,000 are tested a year-it's too low," says Pierluigi Gambetti, the director of the CDC's National Prion Disease Pathology Surveillance Center. "If you don't look, you don't find it. Our testing is not on the cutting edge." Nearly one million animals are tested by both France and Germany every year.
What would the USDA do upon discovering a case of BSE? "Their first impulse would be to suppress it," 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, "Their strategy might be, act like you're looking, but really do a don't look, don't find." However, as Tom Pringle points out, "Absence of evidence is not evidence of absence."
3) In spite of the USDA's categorical denials, one in a million cows naturally develop 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 CJD studies. Hanson has repeatedly pointed to the evidence: "A 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%) of CJD among the 54 studied. A Yale study found that of 46 patients diagnosed with Alzheimer's, six (or 13%) 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."
These shocking figures indicate 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.
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 post-mortem tests for BSE are now widely used in Europe, ante-mortem 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 June 21, 2001 in the Journal of Biological Chemistry, promises to fulfill the need for a simple TSE test for live human and animal subjects. (The bad news is the researchers note that the presence of infectious prions in urine indicates 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 as 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 "worried well"-persons convinced they were developing CJD-have already 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 ruined in mid-2001 by cross-contamination with bovine material, and in the latest cover-up, according to BBC News, October 21 2001, the costly error wasn't announced until three months after its discovery.
The slow responses of Britain, the USA and other nations to the AIDS crises 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 USA and other nations 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.
Surgical instruments are at high risk of transmitting the infection, as autoclave steam sterilization does not neutralize infectious prions. Blood, blood products, bovine extracts and transplant organs such as brain dura matter and corneas are not usually screened for CJD, although in Britain and around the world infected organ recipients, who developed symptoms sometimes decades after treatment, have been traced to unwitting donors later found to have CJD, sometimes years after surgery. Effective prion sterilization protocols are not in wide use, and 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.
A Rendered Disaster
The common practice of feeding domestic animals rendered protein supplements-the
boiled-down, powdered remains of slaughterhouse and other animal waste-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 was authoritatively banned in the UK in 1989. In August 1997 the US FDA tardily issued weak regulations addressing this common practice. Consumers Union's Hanson explained the US ban: "All they said is that you've got to label it 'Do not feed to cattle and other ruminants.' Farmers can walk in a feed store and still buy it. Nobody asks 'Are 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." USDA feed rule compliance among America's thousands of livestock farmers is virtually impossible to effectively monitor or enforce.
Incredibly, Hanson noted in 1999, "The 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."
But calves are not the only hapless recipients; Hanson believes the industry is likely feeding cows "a 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." 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-animals too sick to stand-which are most suspect for harboring BSE.
Perhaps most repugnant, thousands of tons of fermented chicken manure are fed back to millions of U.S. cows each year, in a bizarre loop of inexpensive husbandry. Hanson and Pringle believe that "cow to chicken manure to cow" 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 actually contract TSEs from livestock, the issue has "not really ever been investigated," says Pringle. "No 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."
It remains possible that all domestic animals may indeed be susceptible to TSE infection. According to Hanson, the USDA has "functionally ignored the potential TSE in pigs." Their very short factory-farm life span 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, has also indicated that poultry and especially pigs could harbor TSEs and pass them on to humans. "It's speculation," Brown has acknowledged, "but I am perfectly serious."
Pigs experimentally inoculated have developed BSE, and a suspected outbreak of porcine spongiform encephalopathy occurred near Albany, NY in 1997. A 1973 study published in the American Journal of Epidemiology discovered that 10 of 38 CJD patients ate hog brains.
Big Beef and 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% of cattle farmers reporting profitability in 2000, up from only 15% in 1996. Ironically, Europe's crisis has been a huge boon to "BSE-free" American beef exports, which shot up 34% in 2000, with shipments to the Russian Federation increasing twenty-fivefold. Mad cow has clearly been great for business, although McDonalds 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 the Webmaster of the successor to Pringle's Mad-Cow site (www.purefood.org/madcow.htm), points out the "USDA 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." 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 Feb. 11, 2001 the New York Times reported, "Although 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."
The US has failed to close gaping loopholes in the firewall against mad cow,
and the feeding of potentially infectious cow parts back to cattle continues
largely unmonitored. On Jan. 10, 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 feed-labeling and mixing
The next day, the New York Times followed with a front-page article describing the lapses: "Large 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, 'Do not feed to ruminants .' Manufacturers must also have a system to prevent ruminant products from being commingled with other rendered material."
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 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 both 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 "meat" 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 Food and 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. U.S. 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."
"Although the department [of Agriculture] classifies the tissues as being 'not meat,' their presence in a meat product is not a violation of food safety laws," notes Lance Gay ironically in the Sun Herald, August 7, 2001. "Much of the mechanically separated meat is sold to the school-lunch program, which the department also administers."
The Zoo Loop
TSEs have been observed in numerous rodent, primate and ungulate 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. "Large number of monkeys and lemurs in French zoos appear to be infected with the agent that causes 'mad cow disease,' according to a provocative study published today in Proceedings of the National Academy of Sciences," reported the New York Times' mad-cow reporter, Sandra Blakeslee, on March 30, 1999.
"The 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 C.J.D., may have similar underpinnings."
"This is a huge scandal because it potentially affects the survival of many of the world's primate species," commented Tom Pringle on the PNAS study. "It also suggests very strongly that the nvCJD epidemic will indeed be a 'plague of biblical proportions,' " quoting a warning given by prominent neurogeneticist John Collinge, a member of the British government's Spongiform Encephalopathy Advisory Committee.
Got Mad Milk?
Although it is rarely mentioned, 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. Many have chosen to avoid cheese from the UK as a precautionary measure; many hard cheeses contain rennet, an enzyme extracted from the stomach of calves.
Supplements, Vaccines and Blood
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 on bovine calf serum, are still being made from suspect materials. On Feb. 8, 2001 the New York Times finally picked up the story (curiously placed in the business section) under the headline "5 Drug Makers Use Material With Possible Mad-Cow Link." "For 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." The list also includes flu shots and hepatitis vaccine.
Numerous dietary supplements containing glandular material, brains and other bovine ingredients are also at high risk. "Velvet Antler" capsules from General Nutrition Centers and many other retailers "come from the growing antlers of elk and can contain infectious agents," says Hanson. "They're filled with nerve tissue and blood. I wouldn't want to be the one to be experimented on." "It's just insane not to have greater safeguards" for supplements, Dr. Paul Brown, chairman of the FDA's advisory committee on mad-cow disease, told the Times. "The FDA is toothless."
The American Red Cross, which collects half the U.S. blood supply without testing for CJD, now bans Americans who have spent three months in Britain or one year elsewhere in Europe from giving blood. The strict ban has created a predicted national shortage of blood, especially in New York City, where 25% 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%.
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 have reached recipients. Over the past 10 years, at least $100 million worth of plasma products have 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.
"The thing that worries me is the immunization of the children," says Pringle. "Every 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. Every schoolchild in the UK has already been immunized with vaccine made from serum from infected bovines."
Mad Deer, Sad People
In the Southwest US, an outbreak of chronic wasting disease (CWD), the TSE affecting deer, elk and other ungulates, is now raging, with 5-15% of elk in areas of Colorado and Wyoming reportedly infected. The case of Doug McEwen, 30, a 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% of classic CJD patients develop symptoms at his age. Inexplicably, blood plasma donated by McEwen was cleared by the authorities and distributed during and after his death. 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. 'It's OK. Doug, it's OK.'
"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. . . .
" 'This is the worst thing I have seen,' [Tracie McEwen] says. 'I wouldn't wish it on my worst enemy.' "
For nearly two years McEwen had donated blood plasma, which was processed by Bayer into fractionated blood products in Clayton, NC, then shipped to 46 countries around the globe. "The scope of this is breathtaking," Tom Pringle says of the decision to release McEwen's blood. "You've got a time bomb ticking in millions and millions of people. And as they become donors, it spreads further." Of the infected deer which almost certainly led to McEwen's death, Pringle is unequivocal: "I think they have scrapie. Most cases trace back to Ft. Collins, Colorado at the Foothills Research Station, an experimental facility which was contaminated," a contention shared by several other CWD researchers. Wild animals might also contract the disease by raiding contaminated feed meant for livestock.
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's BSE Red Book-Emergency Operations handbook states, "Cleaning and disinfection is not necessary to prevent the spread of BSE."
Pringle is not optimistic. In the US, "it 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."
In the absence of a CJD test, the world can only guess the extent of the problem. Quoted by the CBS Evening News on Jan. 31, Prusiner, "when asked if, in his darkest moment he thought that this is the plague of the 21st Century, said, 'I don't need a dark moment to wonder if that's the case, because everybody's wondering that, not just me.' "
Interviews with Dr. Tom Pringle and Dr. Michael Hanson were conducted on several occasions between 1998-2001.