Mad Cow U.S.A.

"What do I care?" replies the second cow. "I'm a 'refrigerator'."

No? How about this one, from a British cartoon: A large heifer sits on a nearly empty bus. Next to her, a male passenger laments silently: "Just my luck. All these seats, and I get the one next to the crazy!"

But seriously, folks: The more you learn about so-called mad cow disease, the less it looks like a laughing matter. Aside from the more than 1 million cattle slaughtered as a result of the fatal brain malady, at least 21 Europeans have been diagnosed with a human variant of the disease. One-third of the herds in the United Kingdom have been affected, decimating the once-proud British beef industry.

That may be the good news. The bad news, as Sheldon Rampton and John Stauber postulate in the just-released "Mad Cow U.S.A.: Could the Nightmare Happen Here?" is that it may be only the beginning. It could happen here. There's even a chance that it has already begun.

Stauber, who grew up in rural Wisconsin, and Rampton, a reformed Mormon missionary from Las Vegas, run the not-for-profit Center for Media & Democracy in Madison, Wis. "Mad Cow" is the second book from the unlikely pair: "Toxic Sludge is Good for You: Lies, Damn Lies and the Public Relations Industry" debuted last year. The two became interested in mad cow while investigating recombinant bovine growth hormone, a genetically engineered drug designed to increase milk production. The drug also increases cows' need for protein-heavy, animal-based foods that can spread the brain disease.

The daring duo from the dairy state are not the first to be touched by the mad cow muse. Reporter Nichols Fox and noted nonfiction writer Richard Rhodes examined the epidemic in their recent books on food safety, "Spoiled" and "Deadly Feasts." The disease also inspired science-fiction writer Ronald Wright's apocalyptic novel "A Scientific Romance." Potboiler-maker Michael Crichton found in mad cow a convenient -- if implausible -- ending for "The Lost World." Last but not lightest, two weeks ago "60 Minutes" featured a British artist's installation, "Mad Cow Disease," an unwieldy series of Plexiglas boxes housing cross-sections of genuine, life-size bovines, suspended in clear liquid.

The relatively obscure ailment, which so far has claimed but a handful of human victims, has captured more than its share of scholarly concern, artistic endeavor and morbid humor, albeit of the nervous-titter variety. One Internet wag started a Web site for mad cow jokes. And talk show host David Letterman reportedly included the disease in one of his nightly lists.

Letterman's quip is lost to history, but here is an alternative Top 10 list of reasons to stand in awe of mad cow disease and its many relatives: They occur spontaneously around the world, perhaps in every species; they cross species boundaries, from hamster to human; they apparently have no DNA yet replicate themselves; they mutate to adapt to new species, becoming more virulent; they have a latency period of between four and 40 years; they cannot be seen, even through an electron microscope; they can remain buried with no host organism indefinitely and still remain infectious; they can survive immersion in alcohol, formaldehyde and household bleach, not to mention irradiation; they can endure incineration for an hour at 685 degrees, a temperature that will melt lead; they cannot be positively detected by any practical test -- except postmortem examination.

Oh, and did we mention they are all untreatable?

Diagnosis: unknown, then. Only after epidemiologist John Wilesmith of the Central Veterinary Laboratory at Weybridge in Surrey had examined the brains of several similar cases did Jonquil get her epitaph -- the first known victim of mad cow disease. The ailment is known to the scientific community as bovine spongiform encephalopathy: spongiform, because the disease transforms the brain into a spongelike mass; encephalopathy, because it affects the brain, as well as the rest of the central nervous system. BSE destroys the brain's neurons, gradually reducing the organ to a hunk of Swiss cheese.

Jonquil was only the first victim. As Stauber and Rampton recount, Victoria Rimmer, a 15-year-old who loved horseback riding and hamburgers, began displaying signs of failing memory and fading eyesight, plus weight loss and disorientation, in 1993. She was hospitalized within four months. During the time between Jonquil's diagnosis and Rimmer's illness some 120,000 U.K. cattle had contracted BSE, and the public had become more and more restive, despite reassurances from the government that the new disease was not transmissible to humans.

"I knew it had to be something she had eaten," Rimmer's grandmother told the press.

Grandma knows best. Three years of government denials later, while Victoria Rimmer lay in a hospital unable to speak, lift her hand or control the spittle that fell continuously onto her nightgown, British Health Secretary Stephen Dorrell finally announced to the House of Commons that BSE was "the most likely explanation at present" for 10 cases of degenerative brain disease in patients under 42 -- disease transmitted, quite possibly, in the oh-so-British roast joint, meat pies and other beefy treats the victims had consumed. The malady soon spread to a few exotic animals in British zoos as well as several housecats.

Both Jonquil and Rimmer had succumbed to a little-known group of diseases known as transmissible spongiform encephalopathies. Most are transmitted through contact, especially with heavily infected body parts such as the brain, eye, spinal cord and certain glands. At least one such disease, human familial insomnia, infects through contact and inheritance. The sheep disease scrapie, the most widespread TSE, can be transmitted maternally as well. The diseases also are believed to occur as spontaneous genetic mutations at a constant rate of one per million individuals in mammalian species around the globe.

In some ways, the scariest part of the new epidemic -- which U.S. authorities insist has not appeared in this country -- is the uncertainty that surrounds it within the scientific community. No one really knows what the disease is, what agent causes it.

"I keep thinking of this [illness] as a catalyst," muses John Huntley, New York's official state veterinarian. "It causes other things to react without being changed itself. No wonder it's so hard to denature."

Perhaps TSEs are caused by some previously unknown type of virus that manages to elude our testing devices. Current scientific sentiment, however, leans toward new Nobel laureate Stanley B. Prusiner's prion theory. Prusiner believes that TSEs are caused by rogue protein molecules, identical in composition to regular proteins known as prions but folded the wrong way. The misshapen molecules impersonate normal prions, joining them on the walls of neural and white blood cells. There they gradually induce other prions to change their shapes, destroying the structure of the central nervous system. They reproduce and mutate by some unknown means.

If Prusiner is right, the disease agent has no name. In fact, it requires no less than the redefinition of what we call an organism -- even how we define life itself.

Two mad cows waiting at the feeding trough. Cow No. 1 asks cow No. 2, "Did you check the menu?"

"I don't have to," replies the second cow. "Beef: It's what's for dinner."

Scientist Michael Hansen of the Consumer Policy Institute at Consumers Union in Yonkers, couldn't agree more: "It's so obvious what is behind this."

What Hansen and most every other scientist see lurking in the BSE background is a practice known crudely as animal cannibalism: feeding animal parts to other animals of the same or similar species -- even when, as in the case of cows, the animals are herbivores.

In the beef and dairy industries, the product was promoted as "meat and bone meal." In reality, according to numerous industry and media reports, the end product might consist of a potent grind of sewage sludge; newspaper waste; treated manure; heads, hooves and entrails; sawdust; road kill and "downer" animals unfit for human consumption; dirty poultry litter and feathers encrusted with excrement; factory waste; and leftover grease and blood from restaurants and supermarkets.

In other words, we had turned our food animals into the ultimate recycling machines.

In chickens, the practice of feeding dead chickens, litter and fecal matter has been blamed in part for the explosive spread of diseases such as salmonella and campylobacter. In cows, in the United Kingdom at least, it's to blame for the development and spread of BSE. Whether the infected British cattle contracted the disease through ingestion of scrapie-infected sheep or by eating the remains of other cows infected with a spontaneously occurring TSE, three factors have been pinpointed as probable causes of the epidemic:

• The centralization of the animal food supply, which enabled what would have been a localized flare-up to spread and amplify in herds throughout the country and abroad.

• The greatly increased use of rendered animal protein -- including scrapie-infected sheep and cow remains -- in cow and calf feed.

• Changes in rendering technology, which switched to continuous, lower-temperature processing from processing in batches.

Not so fast, responds Linda Detweiler of the U.S. Department of Agriculture. The agency's official risk assessment on BSE found that the use of hydrocarbon solvents in this country was abandoned during the '70s oil crisis and that the process used in most plants in this country was virtually the same as that in Britain.

We hope.

"It's something I'm very aware of," says Michael Scannell, a Columbia County farmer. "I have a whole file on [BSE] here. It's hard to understand. Between the U.K. and Europe there's been maybe a million cows killed. You'd think people would sit up and notice -- a million cows. It just baffles me."

Scannell's meat and dairy cattle are fed almost exclusively on grain he's raised on his small organic farm in Schodack Landing. He still worries, however, when he buys day-old calves from other farmers.

"It's the milk replacer," he says, speaking of the dry, supplemented mix farmers use to raise motherless calves. Previously, it was possible to buy mixes certified by the National Organic Farmers Association -- but no more, since all milk replacers today contain some animal protein. "Maybe it's just [harmless] fats in there, like they say, but who knows?" he says. "You can't trust anyone these days."

You may not even be able to trust organically raised beef such as Scannell's, according to Pat Kane, head of the New York state chapter of NOFA. She confirms that NOFA-certified farms can't use feed containing animal products. "But you don't want to encourage people to assume it's safe," Kane adds quickly. "I don't think enough is known about the disease to say anything's safe."

Take my cow. Please.

It's no joke: The European Union has imposed a ban on importation of cattle, cattle-derived animal feed and meat from countries where native cows have been diagnosed with BSE. So has the United States, starting in 1989. After the 1996 announcement that people were dying from BSE, the FDA finally enacted regulations that forbade the feeding of any ruminant -- goat, sheep or cow -- or any mink to any other ruminant. The regulations took effect Oct. 4, 12 years after the first BSE case was reported.

The agency had proposed similar feeding restrictions in 1993 but withdrew them in the face of an outcry from powerful cattle, sheep and rendering interests. In exchange, the industry promised its own voluntary ban. However, an FDA survey of the industry's largest rendering firms later proved that 11 of the 15 were ignoring the "ban." All the farmers interviewed for this article thought it possible that economic reasons could tempt some less scrupulous members of their industry to undermine the new mandatory ban, feeding cheaper pig feed, for example, to cattle.

"Who's gonna check?" wonders one dairy farmer.

But Michael Scannell reports that the area's largest auction house has stopped taking "downer" cows, a generic term for cattle that founder and die for a wide variety of reasons, often undiagnosed: "Now, if it can't walk onto the truck, they won't take it. They tell you to take it home and bury it in the backyard. Of course, they won't tell you it's because of BSE." That's a far cry from the days when, for instance, a government investigation of one slaughterhouse involved in the Jack-in-the-Box "E. coli" outbreak determined that fully 8 percent of the cattle processed were downer cows.

More serious disposal problems would result, of course, if the United States ever did see a case of BSE. There are scientists who wonder whether we already have.

"I personally hope we have no BSE," says Dr. Masuo Doi of Utica, who supervises USDA meat-processing plant inspections in part of upstate New York, including the Capital Region. In May, Doi observed a Hereford cow sent to a upstate slaughterhouse exhibiting signs of BSE and videotaped the animal. He then sent brain samples to a department laboratory. The brain was, indeed, spongiform, but several experts eventually attributed its condition to a rare ailment previously unknown in Herefords. According to Doi, there had not been another reported case of that disease since the mid-'70s.

Doi is no stranger to TSEs. Back in 1979, he noticed a group of 106 pigs behaving oddly at the Tobin Packing Co. plant in Albany; in his report, Doi described the animals as "excitable" and unsteady on their hind legs. Brain sample slides were made, which Karl Langheinrich, chief pathologist at the USDA's Eastern Laboratory in Athens, Ga., compared to those of scrapie-infected sheep and mink with TSEs. The findings didn't cause a stir at the time, but when Doi heard about BSE in Europe, he again brought the pigs to the attention of USDA officials. Unfortunately, the brain samples Doi had stored were lost when Tobin closed in 1991. Retired TSE expert William Hadlow examined some of the remaining slides this year but in his final report wrote that he could draw no definitive conclusions based on "the limited microscopic findings, however suggestive of a TSE they may be." (Hadlow refused comment for this story, stating he had "said all [he was] going to say" about Doi's troublesome pigs.)

Luckily for local meat-lovers, the inspector believes he caught all the affected swine before they were transformed into pork chops. Personally, he's not much of a carnivore. "You can't live on tofu alone," Doi laughs, "but we try to eat as little meat as possible."

The case for an existing porcine TSE is bolstered, says Michael Hansen of Consumer's Union, by studies in 1973 and 1985 of Creutzfeldt-Jakob victims. Both found links between the disease and consumption of pork, particularly pig brains. Pigs injected with BSE experimentally do develop the disease. Although pigs fed BSE-infected rations in a U.K. experiment have so far -- more than six years later -- failed to sicken, Hansen criticizes the experimentors for feeding the animals infected food a total of just three times.

"There's good circumstantial evidence" that some form of BSE may exist in the United States, says Dr. Doris Olander, one of the university researchers continuing Marsh's work. "Is there proof? No."

Olander and others worry that inspectors trained to identify BSE by watching videotapes of British cows are looking for the wrong disease if, in fact, a different, so-called "drowsy" strain is infecting cattle on this continent. For years, Marsh vainly petitioned the USDA to include downer cows in its BSE surveillance program, which examines brains from suspect cattle deaths. Although the agency complied last year, as of January 1997 only about 200 such cases had been tested, according to Hansen.

The presence of an existing TSE in the U.S. food chain would be particularly significant, say safe-food advocates, because of holes in the FDA feeding ban. Unlike in Britain, where mammalian protein cannot be fed to any farm animal, here no restrictions exist on feed for pigs or poultry, which can even be given TSE-infected meat. That means scrapie or some other TSE could be fed to pigs, which are then fed to cattle. Or a cow infected with BSE could be fed to pigs, which, in turn, could end up in cattle feed. Even the USDA estimates that based on the known rate of spontaneously occurring TSEs, up to 600 U.S. cows could be expected to get the disease annually.

Some scientists fear TSE-causing prions fed to chickens could be transferred to animal feed or even vegetable crops through poultry manure; feather meal, made from manure-encrusted poultry feathers, is an ingredient in some prepared calf foods. In addition, blood and gelatin products, including pill casings, are exempt from federal feeding and import bans -- embargoes the FDA itself admitted in a 1997 report were "unlikely to be 100 percent effective." (Meanwhile, Stauber reports that at a conference last week, National Institutes of Health scientists admitted that Creutzfeldt-Jakob disease has been detected in human blood and plasma.)

"We have all these scientists arguing about [experimental] models," frets Stauber, "and we're still feeding pigs to pigs. We know what causes this disease -- how clear does it have to be?"

How do you know your cow has mad cow disease? When she starts giving milk of amnesia.

After the link between BSE and nvCJD was made public last year, state and federal officials promptly rounded up and destroyed what was left of the 496 cows imported from Britain to this country between 1985 and '89 -- but most had died or been slaughtered. New York's John Huntley says 13 cows were incinerated in this state, but he admits that the total number imported was higher. Nobody knows how many went into human and animal food supplies. Life expectancy for a dairy cow is two to five years in North America, compared with five to eight in Britain; hogs are lucky to last a year, far less than the incubation period for most TSEs. Are we rushing to shut the barn door too late?

The authors of "Mad Cow U.S.A." argue, pragmatically, that all we can do now is apply a "precautionary principle."

"The truth is that risks come from so many directions and are so unpredictable that consumers can't and shouldn't be expected to cope with those risks by selectively boycotting products," they conclude. ""When risks become known" -- even hypothetical, unproven risks -- "action should be taken to avoid them." ... If we let industry set the rules, however, there will literally be no limit to what we'll swallow, and the nightmare of mad cow disease -- or something just as bad, or worse -- not only "can" happen here, but almost certainly will."

BSE, they maintain, is just the cow in the coal mine, a warning that the globalization of our food supply has introduced hazards impossible to quantify. When four companies control 86 percent of our farm produce and one of the four, ConAgra, is the nation's leading pesticide distributor, the No. 1 flour miller and top manufacturer of frozen food as well as one of the top three meat processors in the country, and when the package of hamburger sitting in your refrigerator may have started out as several dozen cows from different states or even other countries, the vast potential for disease organisms to spread is obvious.

Remember that bumper sticker, "God is coming, and boy, is she pissed"? At least one researcher has described the nearly indestructible agent that causes TSE as "godlike." (Another sarcastically labeled it "the virus from Mars.") As Nichols Fox notes in "Spoiled," British scientist and "Gaia Hypothesis" author James Lovelock would view the new pathogens as attempts by the earth to heal itself, reactions to environmental changes made by humans.

"What Lovelock and others remind us," Fox writes ominously, "is that the result of the earth's self-regulating may not be to our advantage, nor is the process likely to be pleasant." The cows, in other words, may not be the only things feeling a little mad.