The first symptoms don't look like much. People get listless, moody, sometimes scruffy. Then they start to walk strangely, stumble a lot as if drunk, and finally end up in a wheelchair. Soon they can't remember words, faces, loved ones. By the end--which usually comes after a few years--patients are demented and mute. No cure is known.

           Until recently, Creutzfeldt-Jakob Disease (CJD) was one of those rare conditions known mostly to medical specialists; one in a million people, the statistics said, would get it, and generally in old age. That's why doctors in England couldn't figure out what was going on over the last 10 years, when patients in their teens, 20s, and 30s started showing up. Their symptoms weren't quite like the classical form of the disease; they died faster, too, often within a year. And their brains, when cut open after death--the only sure way to diagnose CJD--showed an unusual pattern. They had turned into a kind of sponge, full of holes surrounded by soft masses of dead cells. What they looked like, oddly enough, were the brains of cows killed by "mad cow disease," a new condition that was giving English beef farmers no end of trouble.

           You've probably heard the next part of the story. For 10 years, government officials and most scientists in Britain and elsewhere denied any connection between mad cow and the new type of Creutzfeldt-Jakob, and they set up a scientific commission to prove the point. But when the commission delivered its report this spring, it instead dropped a bombshell. "On current data and in the absence of any credible alternative," it announced, "the most likely explanation at present is that these cases [of the new form of CJD] are linked to exposure to" mad cow disease.

           So far, it might all have been an interesting tale from the other side of the Atlantic. No British beef has been imported to the U.S. since 1985, and U.S. officials have always insisted that there is no mad cow disease in this country. Beef producers and processors--who see the fate of a $60-billion-plus industry hanging in the balance--said the same.

           But while the headlines have dutifully faded, some disturbing evidence continues to trickle in. There have been more of the strange CJD cases in Britain, and one in France. There is now stronger evidence of a potential link between human and cow disease. And it's become clear that while the U.S. doesn't have the British version of mad cow, something very odd is going on with American cattle.

           The best-case scenario, according to the experts, is that the whole thing will blow over as another food scare. The worst is an epidemic that, as one advocate put it, "makes AIDS look like the common cold." For now, U.S. policy is to bet on the former, and to reject the kinds of precautions Britain reluctantly put in place years ago. But behind the scenes there's an increasingly heated argument with high stakes in science, money, and politics. How it turns out will depend in part on what researchers, especially at large agriculture universities like Minnesota and Wisconsin, find in the next few years--and, for that matter, whether they even look.

           The scientific name for mad cow disease is Bovine Spongiform Encephalopathy (BSE), which roughly translates as "cow's spongy brain disease." It's part of a larger group of "transmissible spongiform encephalopathies" (TSEs) that show up in various species--cats, cows, humans, and so on. One of the most common is scrapie, which has been killing sheep in Europe and North America for decades.

           BSE is a more recent arrival. Few if any cases were documented until 1985, when vets were called to Ashford, England to check on some cows that had taken to leaving the herd, stumbling a lot, and occasionally running around like maniacs. By the time the vets and the press had put a name to the new disease, several hundred new cases were being diagnosed each week. By early this year, close to 200,000 BSE cows had been diagnosed, killed, and incinerated in the U.K.

           To this day, no one knows exactly how spongiform encephalopathies are transmitted, or for that matter by what; from what scientists have learned so far, the "agent" in question may be so radically new they're not even sure it exists. It is, research suggests, not a bacteria, virus, or parasite, but a protein molecule run amok. The warped chain of amino acids known as a "prion" can wrap itself around other proteins, preferably in the brain and central nervous system, causing them to also warp and attack other proteins, ad infinitum. Eventually whole clusters of cells die, leaving the spongy pattern typical of TSEs.

           Prions have a couple of other interesting properties. They appear resistant to sterilization, heating, freezing, drying, even toxic chemicals. And they're infectious: When you inject brain tissue from a TSE animal into another, you usually create a form of the disease. That's how thousands of dwarves, who for several decades received growth hormone from dead people's pituitary glands, came down with the human spongiform encephalopathy, Creutzfeldt-Jakob. Today, human growth hormone is made in the lab, but plenty of other uses of cadaver brain parts remain: Neurosurgeons sometimes use outer-brain membranes from dead people, and pituitary hormones have been used as a treatment for female infertility.  

           It's also tissue from dead animals that's thought to be responsible for the BSE epidemic; to understand why, you have to know a little bit about the people who call themselves "the silent industry." U.S. farmers, ranchers, and vets have some 40 billion tons' worth of carcasses and slaughterhouse waste on their hands each year. Landfilling or burning it all would be a hassle and expensive. So instead, most of the dead meat goes to places like Greg Vanhoven's family business in South St. Paul.

           Vanhoven Co. is a rendering--or, as Greg Vanhoven prefers to put it, food waste recycling--plant, one of perhaps a dozen in Minnesota and several hundred nationwide in a business that melds blood and gore with high tech. The trade press describes "spray- dry" machines that turn blood into a fine, brown powder (gardeners know it as blood meal); gigantic kettles that boil fat to make tallow; grinders that crush bones into minuscule fragments. Vanhoven Co., which processes mostly waste from the nearby slaughterhouses, sells by-products to paint and paint thinner manufacturers and cosmetics companies. But his, and most renderers', biggest customer is the feed industry: It buys "animal meal" that is added to the diets of everything from cows to turkeys and pets as a high-powered nutritional supplement.

           Vanhoven, an amiable man who likes to explain what he considers a vastly misunderstood business, says it was clear to him almost from the start that BSE was "a bad situation." The British epidemic is now widely thought to have come from cattle eating the processed leftovers of scrapie-infected sheep. Vanhoven says his company has had a policy for several years now of not accepting sheep and other animals that have been found to have TSEs in the U.S.; he says it's just not a risk worth taking.

           There is, it's worth noting, no proof that it was sheep parts that started the epidemic in cows--or, for that matter, that humans can get CJD from eating cows. But there is evidence that TSEs can be transmitted orally, and that they can jump from one species to another. In England, mad cow panic first hit in 1989, when domestic cats and zoo felines started dropping dead with their own form of spongiform encephalopathy; their food, too, contained material from rendered sheep and cows. More recently, French researchers have found that monkeys can get a CJD-like version of TSE when they are fed meat from BSE cows. And, perhaps most intriguingly, researchers recently announced that humans and cows have a striking similarity in their genetic sequences right at the spot that's thought to be implicated in prion production.

           If BSE can be passed to humans, the scale of the outbreak is anyone's guess. The British scientific advisory commission reviewed 10 cases of the new, "variant" CJD, and 10 more have since been diagnosed in Britain. In the U.S. there are no known cases, though doctors suspect CJD in a 24-year-old upstate New York woman whose family says they ate some British cutlets sent as a gift when she was a teen.

           In the long term, one researcher has estimated up to 500,000 people could die from CJD in Britain as a generation exposed to BSE grows up. Others put the figure closer to 5,000. Either way, the cases would only begin showing up now: CJD has about a 10-year incubation period, and it's been about that long since the beginning of the BSE epidemic.

           Thus far, the key tenet of the BSE debate in the U.S. has been that the disease does not exist in this country--or, as they note when questioned, only exists at a very low level. USDA officials have acknowledged that American cows like any others would be expected to develop BSE "sporadically" (without being infected), at a rate of about one in a million; in a herd of 100 million, give or take a few, those cases are unlikely to be discovered, especially since there is not an approved test to check for the disease in live animals. USDA's 10-year-old BSE surveillance program consists largely of asking slaughterhouse inspectors to watch for cows with suspicious symptoms and send in their brains for autopsy. Several thousand brains have been thus examined, and none showed the typical spongy pattern.  

           There is, however, something mysterious killing U.S. cattle. Upton Sinclair first described it in The Jungle--animals that would simply lay down and never get up, no matter how much you pushed or prodded them. Some "downers" are looked at by vets, some aren't; some, but not all, are diagnosed with a known disease. Most go to rendering while a few become hamburger. And some are picked up by dead-animal haulers--a fascinating little subset of the rendering industry consisting, as one expert puts it, "basically of guys with pickup trucks who have their territories, and when they step on each other's toes they meet in back of a bar."

           Among the haulers' customers are people who raise carnivores on a large scale, such as mink farms. Mink, as it happens, have their own form of TSE; a couple of times a decade there's an outbreak that wipes out a farm or two. How the mink get the disease is another mystery, one UW-Madison veterinary science professor Richard Marsh has been studying for several decades.

           In 1985, the year the first BSE cases were diagnosed in England, Marsh heard of a TSE epidemic at a mink farm in Stetsonville, Wisconsin. He went to check things out, curious among other things whether sheep meat might be to blame--scrapie cases had been diagnosed not too far away. But the farm's coolers and freezers contained nothing but cow carcasses, and the farmer swore he'd fed nothing but downer cows. "At that point, a lightbulb went on in the heads of the investigators," says Doris Olander, who works on the TSE research with Marsh (who was unavailable for comment due to illness).

           Marsh decided to check. He injected some of the infected mink brain tissue into U.S. cattle; they died with spongy brains, though without the typical BSE symptoms. When he fed the brains of those cows back to healthy mink, they in turn developed a spongiform encephalopathy and died. The infection, whatever it was, seemed to work both ways; species-jumping at its best.

           A few years later, USDA researchers conducted a series of related experiments, injecting scrapie-infected sheep brains into the brains of healthy cows. Sure enough the heifers got sick, but they didn't act like Britain's mad cows. Instead they simply fell down and died--just like downer cows, and like the cows Marsh had inoculated with mink brains. "This evidence suggests," Marsh wrote in a dairy trade magazine, "that a BSE-like disease is present, but not recognized in the U.S."

           Marsh's research remains controversial; several University of Minnesota scientists contacted for this story said the evidence seems too thin to form a conclusion. Olander, for her part, is quick to caution that the UW team doesn't think all downer cows are victims of an American strain of BSE. But some, she says, may be, and to find out for sure would take more study. The team applied for research funding several times, but no one was interested.

           Downer cows, it appears, are not something either government or the industry cares to look into. The subject is so obscure no one even knows how many there are, though rough estimates run around 100,000 a year. University of Minnesota professor Victor Cox, who's widely acknowledged as having done more research on the subject than anyone else, says he was able to pretty well describe what killed "the vast majority" of the animals--injuries, and in particular nerve compression after calving--but adds that that leaves a lot of downers about which very little is known. Still, he goes on, "While there are many cases which are not diagnosed, I have no reason to suspect that their cause is any different from the large sample I have observed." He, too, has run out of funding and has taken to putting his personal money into studying treatments. "It comes down to a cost-benefit thing," he says: Even though the dead animals amount to a loss to the industry, it's cheaper and less hassle to replace them than to worry about the cause. And, adds Olander, ag specialists seem leery about attracting attention to the problem, period. "It's something of an Achilles' heel," she says, "something the animal-rights people might get ahold of."

           Which leaves the UW team, and a few other researchers, with one suspicion: If a new strain of BSE is active in American herds, and if it's not diagnosed because the symptoms don't fit the expected pattern, the animals affected are most likely being fed straight back to other animals--and humans? (Government regulations prohibit sick cattle from being slaughtered for food, though they can be rendered; animals not showing symptoms can be used for any purpose.)  

           For years now, Marsh's team as well as consumer organizations like the Foundation on Economic Trends have been calling on the U.S. government to ban feeding ruminants--cud- chewing animals, such as cows and sheep--to other ruminants. That's what Britain has done since 1989, and what the World Health Organization recommends for all countries.

           So far, U.S. officials have resisted the idea, though not always comfortably. In 1991, the federal Animal and Plant Health Inspection Service (APHIS) produced an internal document on U.S. rendering policy that said staff members were split on the matter: Some believed that "a spongiform encephalopathy is present in the U.S. cattle population," and that ruminant-to-ruminant feeding should be outlawed. But those staff members, the document said, were a minority. Besides, such a measure would create a disposal nightmare and encounter "considerable industry opposition."

           Sure enough, the industry's main trade group, the National Cattlemen's Beef Association (NCBA), adamantly opposed a ban until very recently--though not necessarily for economic reasons. In 1993, association spokesman Gary Weber told the trade paper Food Chemical News that ranchers could probably get by without protein from rendered cows and sheep. But, he said, the NCBA didn't want to appear to be ruled by "activists."

           Three years later, Weber stood before a bank of microphones and announced that his group had changed its mind: They would voluntarily ban ruminant-to-ruminant feeding. The announcement may have been designed, in part, to steal the thunder from the Food and Drug Administration, which a few weeks later came out with its own feed ban proposal. But more than anything, it was a way, as Weber all but acknowledged, to slow down what seemed like a runaway public-relations train in the wake of the British announcement. "We cannot afford to have what is a $160 billion industry in the U.S. [including beef and allied trades like veterinary medicine and parts of the pharmaceutical industry] be played Russian roulette with," he said. "There is simply no margin of error here if we are wrong."

           Just what will become of the proposed feed ban remains an open question. An FDA decision is not expected for several months; if the agency goes with the ban, it's not clear it would stand a chance at preventing the disease. In England, cows that were born years after ruminant-to-ruminant feeding was banned are still getting sick at a rate of several hundred a month. Recently, scientists in Iceland caused a stir when they announced that they'd found hay mites--tiny bugs related to the ones that show up in house dust--carrying the agent that causes scrapie. And there's some indication that infected animals can pass the disease on to their calves. Any of those possibilities would mean that BSE could continue cycling through the cattle population, in Britain or elsewhere, no matter what the animals eat.

           Feed ban or not, it's easy to see why cattle folks have been feeling defensive lately. Between stories about hormone injections, fecal matter on steaks, and deadly e-coli bacteria, beef has taken its share of public-relations lumps lately, and enemies--health gurus, vegetarians, animal-rights advocates--seem to lurk everywhere. Prices have been low for years, and many ranchers are worried about competition from Mexican and Canadian imports. BSE could not have come at a worse time. Four weeks after the British science task force's announcement, according to a survey commissioned by the NCBA, some 86 percent of consumers had "heard, read or seen something about 'mad cow disease' in the past two weeks," and almost one-third were eating less beef as a result. (On the bright side, the NCBA told its members, "There was an offsetting decline in the number who listed diet/health reasons for giving up beef.")

           Worries about PR aren't confined to the beef industry proper. Agriculture departments, in England as in the U.S., are set up with the dual responsibility of both monitoring and promoting agribusiness, and there's a busy revolving door between the public and private sector (the NCBA's Weber spent 10 years working for the government). In England, it was a government minister who came up with the most memorable PR stunt of the whole saga so far, back in 1990: In front of the world's television cameras, then-Minister of Agriculture John Selwyn Gummer grinningly fed his 4-year-old daughter a burger. His boss, Prime Minister John Major, didn't sound entirely convinced when he told Parliament that "I am advised that British beef is a safe and wholesome food."

           U.S. officials watched the U.K. debacle carefully. In 1991, APHIS--the same agency that had come up with the divided opinion on ruminant feeding--prepared a PR plan for a BSE crisis. "The mere perception that BSE might exist in the United States could have devastating effects on our domestic markets for beef and dairy products," the document--a copy of which was obtained by the newsletter PR Watch through the Freedom of Information Act--warned. "A number of articles already published could potentially create alarm among U.S. customers."  

           By way of contravention, the paper recommended avoiding the term mad cow disease (even though, it noted, reporters and the public might have "difficulty pronouncing 'bovine spongiform encephalopathy'"). Officials were advised to refrain from guaranteeing beef's safety "when, in fact, absolute safety cannot be proven." They should appear "open and honest" rather than "involved in a cover-up." And finally, "close cooperation with industry groups and other agencies of Government" was crucial.

           That, more or less, was the script followed after the British commission's announcement March 20. Within days, the USDA assembled a meeting of 70 top "health and meat experts" including representatives from various embassies, industry reps such as Weber, and a bevy of government officials. After a closed-door meeting, members told the press that everyone agreed the chances of BSE in the U.S. were slim, that enough had been done to protect the public, and that the U.S. would even give technical assistance to the ravaged Brits. The same message was repeated in scores of news conferences, press releases, and one-on-one briefings over the next weeks.

           In cases where all that didn't work, the gloves came off. On April 16, Oprah Winfrey did a show on mad cow disease featuring NCBA spokesman Gary Weber and rancher-turned-food activist Howard Lyman, who ended up doing most of the talking. Cattle futures dropped 20 percent in the next couple of hours; one analyst averred how traders were particularly worried about all the "homemakers who are at home and... watching Oprah."

           The response was swift. Texas Commissioner of Agriculture Rick Perry--the same guy who two weeks earlier had staged a barbecue/press conference where reporters munched on smoked brisket--asked his state's attorney general to prosecute Lyman, Oprah, and her production firm under a new "food disparagement" law. (Texas and 11 other states have such laws, and more-- including Minnesota--have been considering them. They generally forbid speaking ill of foods unless you have "sound scientific inquiry, facts or data.") A Texas rancher also filed suit, and on April 23, Oprah announced a follow-up show featuring only Gary Weber. "You all need to know, you cattle people, that we're just dependent on y'all out there," Reuters quoted her as saying.

           Interestingly, Oprah had devoted only a fraction of her original show to BSE/CJD; she bypassed the official topic to seize instead on the fact that "cows are eating other cows... they're cannibals." That in itself had "just stopped me cold from eating another hamburger," she declared. It was an industry promoter's worst nightmare: Even if the BSE scare could be managed, the things it stirred up might be worse. Two months later, feed and rendering industry spokespeople still go out of their way to point out that "people shouldn't think the cows are eating raw meat."

           For now, the crisis seems to have been defused. Beef sales are pretty well back to normal--McDonald's, for one, told the Wall Street Journal it never felt an impact--and the European Union has lifted a ban on British beef in return for a promise to kill and burn some 3,000 cows a week for several years. In the U.S., government agencies have announced a series of efforts designed to increase public confidence, such as increased slaughterhouse inspections and the FDA's proposed ruminant-to-ruminant feed ban.

           Whether any of those things will make much difference is unclear: So much remains unknown about BSE it's hard to say what, if anything, is safe or dangerous. Science has barely begun to probe the nature of the disease, and researchers are well aware of the political and economic stakes. In Britain, one of the most vocal scientific critics of government policy, Richard Lacey of Leeds University, was deemed in need of a "psychiatric evaluation" by one member of Parliament. And a scientist named Harash Narang, who for years had researched BSE for the British government, was fired after he went public with claims that his work on a simple test for the disease had been suppressed. Part of the controversy had apparently come when Narang announced that in trying out his test at a slaughterhouse, he found one in three animals headed for the human food supply to be positive for BSE.

           Some of the other big unknowns about BSE are less sensational, but equally far-reaching. So far, the infection has been found mostly in brains, spinal cords, and other organs that are supposed to be kept away from meat; but, as the British government found out last year, that may not mean much in modern slaughterhouses where meat and bones are separated by giant saws and sometimes ground to a pulp in the same crusher. Some researchers even speculate that the infectious agent could enter the bloodstream, and thus the meat itself, which would make all the other precautions pointless.  

           Similarly, no one is sure which cow products are most likely to carry the disease. Speculation in England has focused on hamburger and sausage, which are most likely to contain meat from older cows who have had time to develop full-blown infections. But there's little information on whether cows without symptoms, such as beef cattle slaughtered at a young age, may transmit the infection too. Similar uncertainty applies to many of the other things made from cows--gelatin for yogurt and medication capsules, dried liver for nutritional supplements, collagen for wrinkle creams, and so on. Right now the FDA says those products are all safe; it has suggested manufacturers not use materials from countries where there is BSE, but it hasn't banned them.

           And finally, though officials say there's no evidence of any unusual CJD cases in the U.S., they don't have much data to go on so far. For one thing, studies suggest that a fair number of CJD cases may be misdiagnosed as having Alzheimer's disease; for another, though British doctors have been required to report CJD cases to the government since 1989, the U.S. has specifically resisted opening a registry for the disease. As the 1991 government PR plan noted, that would "appear to legitimize concern about a link between BSE and human health."

           Five years later, and just weeks after the British science committee's announcement, the Centers for Disease Control announced that it would take a little closer look at CJD incidence after all. Four states, including Minnesota, were selected to participate in a surveillance pilot project, and state epidemiologist Mike Osterholm says so far the evidence is reassuring. Two months' worth of surveying death certificates and interviewing neurologists, he says, didn't turn up any unusual cases of CJD or increased frequency of the disease. But he does acknowledge that that doesn't guarantee nothing's going on: Because of the disease's long incubation period, "if we find it we've already failed." There is, of course, an argument to be made over whether it's worth worrying about any of this. There are plenty of other potential risks in food, meat and otherwise; in contrast with some of them, BSE seems reassuringly theoretical. And the word "safe" itself gets slippery when it's caught up in regulations and statistics designed to assess everything from pesticides to nuclear plants. The British government found itself debating critics over whether safety meant "an absence of risk" or "not a very big risk."

           What's really annoying about the BSE/CJD story, as the British magazine New Statesman and Society noted, isn't so much a clear and present danger, but the fact that so much effort has been spent denying the possibility of risk. Listening to the official pronouncements, in Britain as in the U.S., you'd never have guessed that experts were at least considering the chances that an infectious agent of unknown power, transferred through food and possibly other means (blood and breast milk are being studied), was causing a disease that resulted in dementia and death. BSE may not turn out to be another AIDS disaster, or even anything beyond a cow disease. But to claim certainty one way or the other, scientists are beginning to admit, is hypocritical at best. As Joe Gibbs, deputy chief of the National Institutes of Health's central-nervous-system diseases lab, told Newsday's Laurie Garrett, "There is sound reason to suspect a strong link [between human illness and mad cow disease] in Britain. Are we sitting on a time bomb? There's no way of knowing. But I wouldn't be honest if I told you there was nothing to worry about.

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