"Top regulatory agencies in two nations don't make a decision of this magnitude on the basis of the meager information that has been publicly disclosed," says Dr. Tom Pringle, a molecular biologist and the energetic administrator of the encyclopedic Official Mad Cow Disease Home Page, a project of the Sperling Biomedical Foundation. Pringle believes that such steps are "too little, too late. With this many millions of Americans and Canadians exposed and possibly incubating disease for fifteen years already, it is only a matter of time before nvCJD manifests itself in North America in a person who has already given blood. And a lot of people there for under six months will continue to donate." Pringle has been quoted in recent articles in the New York Times, although his printed comments have been considerably toned down, to his chagrin.

Considering that the British Mad Cow crisis destroyed the UK beef market, precipitated a worldwide ban on British imports, exposed an official cover-up, and continues to induce severe public anxiety (even suicide among the "worried well"), many critics doubt the US government is offering complete and accurate information concerning grave problems affecting the large and powerful domestic meat, rendering, bone, gelatin, blood and medical industries.

However, if one puts together all the pieces of the puzzle, gaping regulatory loopholes and evidence of widespread infection become apparent. In truth, America has probably been harboring many TSEs for decades. CJD's tremendous scientific complexity, long incubation period and symptomatic similarity to Alzheimer's has likely veiled the leading edge of a deadly epidemic in the United States.

How Now, Mad Cow

Mad Cow disease first gained the world's attention in March 1996, when officials of the World Health Organization and British authorities were forced to admit that 10 deaths from CJD were directly related to eating tainted beef. After widespread infection of British cattle was revealed, millions of cows were burned in high-security incinerators and the residue treated as biohazard waste. To date, nearly 100 people in Britain have been diagnosed with nvCJD, although the total number of cases will certainly run much higher.

How much higher is a matter of debate. Dr. Richard Lacey, a leading microbiologist whose early warnings were ignored by the British government, cautions that BSE "is much more serious than AIDS." On Dec. 17, 1999 Lord Phillips, the BSE Inquiry chairman, stated that the known cases of nvCJD may turn out to be just the "tip of the iceberg." On Jan. 7, 2000 the European Union's Scientific Steering Committee warned that millions of European consumers were at risk of developing CJD. And in July, 1999 neurogeneticist John Collinge, a member of the British government's Spongiform Encephalopathy Advisory Committee who has warned of "a disaster of Biblical proportions," stated in The Lancet, "Studies... suggest that the early variant CJD cases may have been exposed during the preclinical phase of the BSE epidemic. It must therefore be considered that many cases may follow from later exposure in an epidemic that would be expected to evolve over decades."

On Feb, 28, 2000 The Sunday Times of London carried a long-dreaded report, filed by Science Editor Jonathan Leake: "Doctors are investigating Britain's first suspected CJD baby... born to a woman who has since been found to have new variant CJD... The case has also endangered medical staff attending the baby's birth because the mother's placenta contains high levels of prions... The revelation coincides with a decision by the Department of Health to make formal preparations for a likely surge in CJD cases... It will mean that any woman incubating nvCJD who becomes pregnant could unknowingly transmit it to her children. Since almost everyone in Britain has been exposed to contaminated beef products and the disease is thought to take many years to develop, it could mean that any epidemic would last for generations." The mother has since died; the official diagnosis of the baby's condition is inconclusive.

All the known nvCJD victims express a genetic trait shared by 38% of the human population and all bovines. (No test for this gene variant is available; the second and third variants for prion protein at Codon 129 on Chromosome 20 might simply effect longer incubation periods in the rest of the population.) Deployment of a simple CJD blood test, for TSE infection or simply genetic susceptibility, in the UK and elsewhere has been delayed, deferring possible discovery of widespread human infection. 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.'"

A Clever Pathogen

The practice of feeding domestic animals "rendered" protein supplements-the boiled-down, powdered remains of slaughterhouse waste-spread BSE in the UK. Surviving temperatures of up to 600 C (in an experiment carried out by the US National Institutes of Heath), mutant prions from each BSE-infected cow infected thousands of other bovines, as huge batches of feed were mixed and fed back to cattle.

Feeding mammalian protein to ruminants was banned in the UK in 1989. In August 1997 the US FDA finally issued weak regulations addressing this common practice. Dr. Michael Hanson, chief scientist of Consumers Union (publisher of Consumer Reports) describes the rules: "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." Compliance is voluntary and virtually impossible to monitor or enforce. Incredibly, Hanson notes, "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."

Additionally, 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 necessarily hides any symptoms of TSE infection. Pigs and chickens are still routinely fed rendered protein, which often includes the remains of "downer" cows-animals too sick to stand-which are most suspected of harboring BSE. After inedible pig parts are rendered, they are often fed back to pigs, cows and chickens. Fermented chicken manure is routinely fed to millions of cows and pigs, in a perverse loop of inexpensive husbandry and forced cannibalism.

"Their first impulse would be to suppress it," Hanson says of the government's TSE detection program. "Their strategy might be, act like you're looking, but really do a don't look, don't find." However, as Pringle points out, "Absence of evidence is not evidence of absence."

A new Swiss test which detects TSEs in live animals has drastically increased estimates of infected cows in Europe, as many asymptomatic cattle with subclinical BSE have been discovered. BSE's incubation period is from three to 10 years, and cattle are usually slaughtered before symptoms might manifest. Humans consuming subclinical animals are still at tremendous risk of contracting CJD. John Collinge has told the official BSE Inquiry that some cows may be carrying a dangerous silent infection: "It may be that there is rather more infectivity in muscle or other tissues in those animals and that is why they do not have a brain disease." Last year, Dr. Mary Jo Schmerr, chief scientist for prion diseases at the US National Animal Disease Center in Ames, Iowa, devised a different TSE test, and the UK CJD Surveillance Unit in Edinburgh, Scotland has been working with her on a new blood test for humans.