From http://www.mad-cow.org/00/jan00_news_mid.html }}>Begin A 21st century plague? By Joel Bleifuss, In These Times Variations of Britain's infamous Mad Cow disease could kill up to 13 million people. But regulatory agencies are slow to act on this data, since precautions could threaten the bottom line of the meat industry. The scale of the public health threat posed by Mad Cow disease and other transmissible spongiform encephalopathies (TSEs) may not be known for years to come. In Britain, the death toll resulting from Mad Cow -- or, more precisely, its human manifestation, new-variant Creutzfeldt-Jakob disease (nvCJD) -- could be catastrophic. Here in the United States, chronic wasting disease, a TSE found in deer and elk, may pose a threat to those who eat infected animals. No one is sure to what extent the public is at risk from TSEs, which are caused by little-understood proteins called prions. The prevalence of the disease in livestock populations is still unknown. How humans are infected with a TSE from other species is undetermined. And the disease in humans is difficult to track, since it may take decades from the time of initial infection to show any symptoms. Mad Cow -- or bovine spongiform encephalopathy (BSE)-appears to have originated from the common practice of feeding livestock high-protein feed supplements derived from rendered slaughterhouse waste, some of which was from TSE-infected animals. By recycling TSEs -- which occur naturally in mammals but are extremely rare-into the food supply, an epidemic was touched off. In Britain, nearly 50 people have been diagnosed with nvCJD, a disease whose associated prion is nearly identical to the prion that causes Mad Cow. But those numbers may be deceptively low due to the disease's long incubation period and because the victims appear to have been exposed before 1986, when Mad Cow was discovered in British cattle. To measure the extent of the problem, for the past two years British government scientists have been performing biopsies on tonsils, one of the body parts that contains the nvCJD prion. The results have not been made public. But statements from those familiar with the studies are not reassuring. In August, John Pattison, chairman of the government's Spongiform Encephalopathy Advisory Committee, predicted that the number of people infected with nvCJD could reach millions. In December, Lord Justice Phillips, who has been heading an inquiry into the government's mishandling of the crisis, said the current cases may be the just "tip of the iceberg." And the BBC has reported that a study by the Royal Society, Britain's premier scientific body, has estimated the death toll might reach 13 million. Making matters worse, theories have emerged that some people who contract nvCJD may get it not from eating infected meat but from secondary infections. Last July, John Collinge, a member of the Spongiform Encephalopathy Advisory Committee, wrote in The Lancet that he expected the human epidemic to "evolve over decades." He went on to raise concerns that the infectious prions might be transmitted "via blood transfusion, tissue donation, and, since prions resist routine sterilization, contamination of surgical instruments." He also raised the disturbing specter that other species of livestock may be infected. "The theoretical possibility that BSE prions might have transferred to other species and continue to present a risk to human health cannot be excluded," he wrote. Mad Cow prions or their TSE cousins have not been found in the U.S. cattle population. (However, testing by the U.S. Department of Agriculture is so inadequate that such a statement is meaningless.) But a TSE specific to deer and elk, chronic wasting disease, is endemic in parts of Colorado and Wyoming and has been found on game ranches in Montana and Oklahoma. Chronic wasting disease was first observed in 1967 in captive mule deer, which were subsequently released into the wild. One theory has it that the captive deer were exposed to sheep that carry an ovine form of TSE known as scrapie, which is relatively commonplace. Another is that the deer contracted the disease by eating feed that contained rendered protein from an infected animal. In parts of Colorado and Wyoming it affects as many as 8 percent of the deer and 1 percent of the elk. At least two young hunters, who ate and dressed deer, have come down with CJD. And a third, a young woman who had eaten venison from a deer shot in Maine, also contracted the disease. Because CJD is normally a disease of the old, the youth of the current victims raises the strong possibility that they contracted the disease through infected deer. Paul Brown, an expert on TSEs at the National Institutes of Health, has told John Stauber, author of Mad Cow U.S.A., that deer hunters must be out of their minds to be consuming deer in areas where chronic wasting disease is prevalent. That message, however, has not gotten out to the general public. "The failure of state and federal agencies to take swift action and warn hunters about potential risks of chronic wasting disease is inexcusable," Stauber says. "The best scientific minds on this issue have failed to adequately warn the public, and in this instance deer hunters, of the deadly risks of these types of diseases." Why the silence? One reason could be that state wildlife departments are heavily dependent on the income received from licenses for big game. The Colorado Wildlife Division maintains that chronic wasting disease does not affect humans, but advises hunters to "wear rubber gloves when field dressing carcasses, minimize handling of brain and spinal column and wash hands afterward" and to "avoid consuming brain, spinal cord, eyes, spleen and lymph nodes of harvested animals." "The best available science at this time does not indicate a significant threat to human health as result of chronic wasting disease," Montana state epidemiologist Tod Damrow told the Billings Gazette. The problem is that there is no available science. Vigorous preventive measures to stop the spread of TSEs could threaten the bottom line of entire industries (meat and human blood products to name two), which has led regulatory agencies to put the health of the industry above the public's. The federal bodies that have been entrusted with protecting the public health, the Centers for Disease Control and the Food and Drug Administration, have taken different approaches to this disease and the threat it poses. Meanwhile, the USDA, which regulates animal feeding practices, continues to treat the threat as a PR problem. One of the hunters who was diagnosed with CJD had been a big blood plasma donor, with his blood being pooled with others' and used in 121 products like clotting factor for hemophiliacs, which are sold in 20 countries. Beginning in 1993, products made from blood coming from someone with CJD were recalled. That policy cost the blood industry, including the American Red Cross, millions of dollars and was abandoned in 1998, when the CDC determined that there was no evidence that blood from CJD victims was unsafe. On the other hand, there was no evidence that the blood was safe. In effect, the CDC was "equating absence of evidence as evidence of absence" says Tom Pringle, a molecular biologist and webmaster of www.mad-cow.org, which has become the definitive Web site on TSE. "The politics and economics of it is that they don't want recalls, and if CJD is more common that means more of the donors have it and the risk to the blood supply is higher." The CDC has concentrated its efforts on testing those stricken with TSE symptoms for the presence of nvCJD, which is linked to the consumption of British mad cows. But anyone who contracted a TSE from exposure to either deer with chronic wasting disease or cattle infected with an American strain of BSE would not test positive for nvCJD. "The CDC is playing this public relations game, focusing on nvCJD and ignoring the possibility that chronic wasting disease in humans resembles the type of CJD we already have in the United States and not nvCJD," Stauber says. "The federal government and the meat industry have attempted to depict TSEs as foreign diseases, so that then journalists and the public will assume it can't happen here. But we already have TSE diseases in sheep, mink, deer, elk, humans and possibly in cattle and pigs. And we continue to engage in risky livestock feeding practices, like weaning calves on cattle blood protein, that could put us in a similar situation as Great Britain." The FDA is being more proactive than the CDC, worried that nvCJD contracted in Britain by American visitors could contaminate the U.S. blood supply. (An import ban on British beef is already in place here.) In June, the FDA's TSE Advisory Committee voted 12 to 9 to prohibit Americans who spent six months in Britain between January 1980 and December 1986 from donating or selling blood. This policy will go into effect April 17 and is expected to affect about 2 percent of U.S. blood donors. For its part, the USDA has failed to institute a complete ban on the practice of feeding rendered animal protein to other animals (rather than the current partial ban on feeding rendered ruminants to other ruminants). Instead, the agency has spent time preparing a secret PR strategy for when the first mad cow is found in the United States. According to documents released to the Center for Food Safety under the Freedom of Information Act, the department's Animal and Plant Health Inspection Service has put together a 27-member BSE Response Team, which will be flown to a "situation room" at BSE Headquarters -- an underground bunker in Riverdale, Maryland -- in the event that BSE is found in the U.S. cattle population. Once a case is confirmed, 24 hours will be spent giving special briefings to unnamed "select industry and trading partners." Then the public will be told that something has gone terribly wrong. 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