How Now Mad Cow?

The unfolding story of mad cow disease follows an all-too-familiar and damning pattern. A threat to public health is discovered, the affected industries and their allies in government respond with a public relations campaign, the evidence mounts and some reforms are implemented. This is followed by more evidence and more reforms. Yet nowhere in this scenario have the federal agencies charged with protecting public health—the Food and Drug Administration (FDA), the U.S. Department of Agriculture (USDA), and the Centers for Disease Control and Prevention—taken proactive steps to remedy the situation. Rather, they have operated in the interests of huge agriculture and food lobbies. Since 1993, I have devoted numerous “First Stones” to mad cow and related diseases. Nearly every prediction—and warning—from scientists who are experts in this field has come to pass. Yet, by and large, the mainstream media have chosen to listen to the palliative pronouncements of government officials and industry flacks. With mad cow disease now established in the United States that may be changing. Mad cow, first discovered in Great Britain in 1985, is a type of malady known as transmissible spongiform encephalopathy (TSE). The disease gets its name from the sponge-like formations that occur in the brains of infected mammals. The sheep form of the disease, which has been recognized since 1755, is known as scrapie. In Britain, cattle contracted mad cow disease, known as Bovine Spongiform Encephalopathy (BSE), by eating protein feed supplements that contained scrapie-infected sheep. The human strain of TSE comes in several forms, including Creutzfeldt-Jacob disease (CJD), Kuru (a TSE that several decades ago plagued a population of New Guinea cannibals before changes in dietary laws), and new variant CJD (nvCJD), the form of the disease that comes from eating infected cattle. The USDA has long known that mad cow disease posed a threat. However, department officials were worried about danger to the industry—not the public. In 1991, the USDA prepared contingency plans to deal with the possibility that mad cow disease could rear its ugly head in the United States. To wit, it drew up a strategy paper titled “BSE Public Relations.” That plan reads in part, “The mere perception that BSE might exist in the United States could have devastating effects on our domestic markets for beef and dairy.” And it noted that the agricultural industry is “vulnerable to media scrutiny” regarding “the practice of feeding rendered ruminant products to ruminants and the risk to human health” that might stem from this practice. Mad cows in America To all indications, and contrary to recent news reports, an American strain of BSE has long been circulating through the food chain. In 1985, a Stentsonville, Wisconsin, mink ranch was wiped out by transmissible mink encephalopathy. The diet of the mink consisted of 5 percent horsemeat and 95 percent “downer cows”—cows so lame they fall down and are unable to get up. Could one of those downer cows fed to the mink have been infected with an American strain of BSE? In December 1992, the late Richard Marsh, a veterinary scientist at the University of Wisconsin, reported on experiments in Mission, Texas, and Ames, Iowa, where brain matter from scrapie-infected American sheep was injected into the brains of cows. The infected cows developed BSE, but their symptoms differed from the mad cow disease that was plaguing Europe. In May 1993, Marsh told me, “The signs that these cattle showed were not the widely recognized signs of BSE—not signs of mad cow disease. What they showed was what you might expect from a downer cow.” In other words, BSE-infected cattle in Europe went mad before dying, but BSE-infected cows in the United States simply fell down and died. Each year in the United States about 150,000 cattle suffer from downer cow syndrome. Those downer cows that made it out of the pasture alive ended up in the slaughterhouse and into the food chain. Until 1996, when the practice was banned by the USDA, the slaughterhouse remains of at least 14 percent of all cattle, including downer cows, were rendered into protein and fed back to other cows as feed supplements. What’s more, the meat from these tough and old downer cows usually ended up in fast-food hamburgers and other highly processed meat products—that is until the slaughter of downer cows was halted by Agriculture Secretary Ann Veneman on December 30, 2003. “The USDA tends to respond to commodity groups rather than the consumer. And the government hasn’t taken any measures to restrict what goes into animal feed,” Marsh said in 1993. “The Center for Veterinary Medicine at the FDA would have to make the recommendation not to feed ruminant animals to cattle, but we can’t get them to do this.” In 1996, the USDA belatedly decided it was time to stop feeding the rendered protein from ruminants (cows, sheep and deer) to other ruminants. The department had considered implementing such policies in 1991 but decided not to because such regulations “could pose major problems for the U.S. livestock, feed and rendering industry,” according to “BSE Rendering Policies,” an internal 1991 USDA report. The impetus for this ban was the breaking news in Britain that some people, mostly young, were beginning to die agonizing deaths from a new kind of CJD (nvCJD), the cause of which, as the British government acknowledged, was consumption of mad cow meat. The British Ministry of Health discovered this new form of the disease because it had set up a registry for CJD. However in the United States, the USDA, in its “BSE Public Relations” plan, advised the department to “avoid the public relations problems such as have occurred in the U.K,” such as setting up a registry of CJD cases that “appeared to legitimize concern about a link between BSE and human health.” Variable symptoms In the last three years, studies have suggested that classical CJD might be caused by eating BSE-infected cattle, according to a recent report by Todd Hartman in the Rocky Mountain News. In Great Britain, scientists injected BSE into mice whose brains were genetically engineered with human genes. While some of the mice developed nvCJD, the kind people get from eating mad cows, other mice came down with classical CJD. In their November 2002 report, the scientists wrote, “This finding has important potential implications as it raises the possibility that some humans infected with [mad cow disease] may develop a clinical diseases indistinguishable from classical CJD.” And in 2003, French scientists discovered that scrapie, the sheep TSE, caused brain damage in mice similar to that of classical CJD. Hartman writes, “The two studies suggest that at least some of the hundreds of Americans who contract classical CJD each year could have been infected by BSE-contaminated meat, and not simply by biological bad luck.” How common CJD in its various forms is in the United States is unclear. Some medical experts believe that the incidence of CJD in the U.S. population is much higher than the commonly assumed 1 per million. A 1989 study at the University of Pittsburgh examined the case histories of 54 demented patients who, upon their death, were autopsied at the University of Pittsburgh. The study discovered that 39 (72 percent) of the patients had Alzheimer’s; 15 (27.7 percent) had central nervous system disorders; and three (5.5 percent) had CJD. The researchers concluded that the three cases of CJD turned up in their study “had a much longer course than is usually seen with that condition and failed [when the patient was alive] to show the usual EEG abnormalities.” In other words, the CJD cases discovered in Pittsburgh exhibited symptoms that were more compatible with Alzheimer’s disease than classical CJD. A 1989 Yale University study reported similar findings. Postmortem examination of 46 patients diagnosed with Alzheimer’s revealed that six (13 percent) actually had CJD. The Pittsburgh and Yale studies point to the possibility that some of the 4 million people in the United States suffering form Alzheimer’s may actually be infected with the agent that causes CJD. And that raises this question: Has an unrecognized from of BSE infected U.S. cattle and entered the human food chain? Bolstering this concern is a report from the Philadelphia Inquirer that seven people in New Jersey have died from classical CJD, all of whom ate at the same racetrack in Cherry Hill, New Jersey. The scope of CJD incidence in humans is further complicated by the fact that a deer form of mad cow disease, known as chronic wasting disease, is endemic in parts of Wyoming, Colorado and Wisconsin. At least two young hunters who ate and dressed deer have come down with CJD. And a third, a young woman who ate venison from a deer shot in Maine, also contracted the disease. Because CJD is overwhelmingly a disease of the old, the young age of the current victims raises the strong possibility that they contracted the disease through eating deer suffering from chronic wasting disease. In 1999, Paul Brown, an expert on TSE at the National Institutes of Health, 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 health warning, however, has not been given 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 said at the time. Why the silence? One reason could be that state wildlife departments are heavily dependent on income derived from licenses for big game. The Colorado Wildlife Division maintains that chronic wasting disease does not affect humans. Yet at the same time it advises hunters to “wear rubber gloves when field dressing carcasses, minimize handling of brain and spinal column and wash hands afterwards”—and then go home and feast on venison, though not the “brain, spinal cord, eyes, spleen and lymph nodes of harvested animals.”