Not a virus, not a bacterium, the abnormal version of a protein known as a "prion" represents a biological threat never before seen on Earth. Able to withstand conditions which kill any known pathogen, mutant prions easily jump the species barrier, fatally infecting populations of humans or animals with TSEs.

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 on contact, causing them to "fold" improperly and mutate their neighbors in a domino pyramid of devastation, until the host develops spongy holes in the brain, loses nervous system function and dies. Unlike normal prions, mutants do not break down when meat is ingested. The immune system does not attack the invader, because rogue and normal prions are chemically identical.

The brain and spinal cord 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 according to Michael Hanson, 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. Even the federal Food Safety and Inspection Service has 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 other, edible tissues.

The Evidence

The scientific evidence for numerous undiscovered cases of CJD in the US lies largely upon two university studies. In written comments to a Harvard/ USDA BSE risk-analysis project on Sept. 28, 1998, Hanson summarized, "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 (Boller et al., 1989). A Yale study found that of 46 patients diagnosed with Alzheimer's, six (or 13%) were CJD at autopsy (Manuelidis and Manuelidis, 1989). 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 studies indicate an exponential increase over the expected incidence of "sporadic" CJD, providing preliminary yet persuasive evidence of an unrecognized outbreak of CJD in the USA.

Another pivotal study was undertaken in 1991 by Dr. Richard Marsh, a TSE researcher at the University of Wisconsin. Marsh showed that US cows inoculated with tissues obtained from TSE-infected mink-whose diet consisted of 95% offal from downer cows-developed BSE.

The BSE strain was different from that seen in Europe, and proved that other strains might indeed exist in America.

(Significantly, rather than exhibiting classic "Mad Cow" symptoms, the animals simply collapsed, qualifying for the all-inclusive "Downer Cow Syndrome" which affects 100,000 of 100 million US bovines annually.) When the brains of the infected cattle were fed back to mink, they duly developed transmissible mink encephalopathy, suggesting "the presence of a previously unrecognized scrapie-like infection in cattle in the United States."

On Feb. 1, 2000 US Secretary of Agriculture Dan Glickman declared a scrapie "emergency that threatens the sheep and goat industry of this country," effectively admitting that the disease, with 20 known strains, is out of control. "The sheep thing in the US is demonstrably worse," says Pringle. "They know they've got scrapie in thirty-nine states, that there are a lot of really infected flocks, and they know those flocks are being eaten by people, and there's no effort to keep scrapie out of the human food chain. They've gotten away with murder."

Bad Blood

Firm experimental evidence that blood can contain infectious prions was produced last November. 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.

Surgical transmission of CJD is a very real problem. In Britain, it is now even illegal to reuse contact lenses for fear of spreading CJD contamination through the eyes. Many cases of transmission through transplants of corneas, brain-matter grafts and other organs have been documented. It is inevitable that worldwide sterilization procedures will undergo drastic modification in the face of the prion.

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."

On Feb. 8, the New York Times ran a groundbreaking article, headlined "5 Drug Makers Use Material With Possible Mad Cow Link," confirming allegations made by critics that many vaccines were likely produced using contaminated bovine serum, including "some regularly given to millions of American children, including common vaccines to prevent polio, diptheria and tetanus."

Mad Deer, Sad People

In the Southwest US, an outbreak of Chronic Wasting Disease (CWD), a TSE affecting deer and elk, is now raging, with 5-15% of wild 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....p>"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." Wild animals might also contract the disease by raiding contaminated feed meant for livestock.

It has recently 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."