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"Good Cow, Bad Cow"

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Call it "mad farmer" disease. Or "mad butcher" disease. Or "mad consumer" disease. All three groups are increasingly furious about the inaction, vacillation and confusion of their political leaders over the spread of Bovine Spongiform Encephalopathy — bse, or "mad cow" disease — across the Continent and into the cattle herds, slaughterhouses and psyches of Europe. Since the disease was diagnosed in Britain in 1986 BSE, which can be spread to humans to cause the incurable and invariably fatal variant of Creutzfeldt-Jakob disease, or CJD, has officially turned up in all but five E.U. countries. The result: scores of bereaved families of CJD victims, collapsing markets for beef throughout Europe and a rash of finger pointing about who is to blame.

The cows, of course, should be mad — the threat of BSE infection could result in the mass slaughter of millions of them. Within the past month some of those millions went into furnaces as officials ordered whole herds in France and Germany slaughtered and burned after the appearence of BSE-positive animals among them. By week's end the crisis of confidence had become so great that the European Commission recommended banning all cattle aged 30 months or older from the food chain unless they test negative for BSE. That could exclude as many as 8 million animals per year from the already crippled beef market. The Commission also called for a total ban from all animal feed of so-called meat and bone meal (MBM) — the ground-up bones and offal of slaughtered cattle. It is that animal matter, especially brains, spinal cords and other nerve tissue, that is believed to carry the BSE contagion to healthy cattle. From there it spreads to humans as the CJD variant, a wasting brain disease. The killing of older cattle is recommended because 99.9% of Britain's cases of BSE occurred in animals aged 30 months or more. But the destruction of cattle even younger than 30 months may be necessary anyway. Beef sales are down by as much as 50% in parts of the E.U., and farmers have little choice but to destroy cattle that consumers won't eat at any price. "We are considered public poisoners," says Raphaλl Simonin, 27, whose family maintains a herd of 350 cattle near Vittel, 345 km southeast of Paris. "We are not responsible for this crisis. Some of my colleagues are going to have to give up everything if their banks and creditors don't help them find solutions."

Well might Simonin be mad. But families of victims are angriest of all. "For a long time the people in the beef industry were saying, it's the journalists that are crazy and there is no real problem," says Jean Duhamel, whose sister Laurence died of CJD in February at the age of 36. "They have to take responsibility for what they've done." Also angry should be taxpayers, who will pay farmers for animals destroyed — a best-case estimate of $1.1 billion per year, which could triple if beef consumption falls 30% over time. And somebody will pay for the MBM ban, up to $2.6 billion a year to destroy waste meat and bone plus more millions to buy vegetable protein needed to replace it. That bill will be paid in higher meat prices when — and if — people begin eating beef again.

The crisis might never have occurred had the Continent's politicians heeded lessons learned in Britain. After months of indecision, politicians in 1996 finally responded to growing consumer outrage by banning MBM to all farm animals — not just sheep and cattle as they had done in 1988 — and ordering mass slaughter of older cattle. Since then more than 4.5 million animals, both tested for BSE and untested, have gone to the furnaces. After peaking at 36,000 cases in 1992 the incidence of BSE has fallen to 1,150 so far this year and beef consumption is back to pre-1996 levels — although prices remain depressed.

The response to Britain's social and economic agony should have been to follow its example: ban MBM from the entire feed chain, order extensive testing and ruthlessly slaughter suspect herds or even all cattle older than 30 months. Instead, politicians on the Continent often banned imports of British beef, even after it could be considered safe, and assured consumers that they were being protected. Even after the affliction turned up across the English Channel, politicians struggled to stem consumer panic by declaring beef to be BSE-free. German Agriculture Minister Karl-Heinz Funke proclaimed on Nov. 20 that "German beef is safe." But by last week Funke was telling consumers to buy only from "butchers they can trust."

But can consumers trust anybody? Officials speak of frequent "cross-contamination" of MBM in supposedly safe feedstocks and suspect carcasses can still sneak into butcher shops, even under Britain's well-established regulations. "It is possible there is beef getting into the food chain which has not been subject to strict BSE controls because it is killed privately or in unlicensed abattoirs," said John Krebs, chairman of the British Food Standards Agency. In Italy, consumers are so worried about French imports that beef sales are collapsing. Following government guidelines, butchers post detailed certificates to show their beef is safe, but customers stay away. "People don't take a scrap of notice," says Rome butcher Antonio Gelonese. "We're already snowed under with official bits of paper."

So little is known about the disease that consumers can feel justified in their doubts. BSE and CJD are caused by malformed proteins call prions that have the ability to infect other proteins — especially in the brain, which becomes riddled with sponge-like holes. In humans, the incubation period may be 10 years or longer. But once the disease manifests itself, death is agonizing, inevitable and swift, usually occurring within a year. So far, 85 people in Britain have been diagnosed with the BSE-linked variant of CJD, and all but four of them have died. Experts predict anywhere between 1,000 and 136,000 cases in the future, but one member of the government's advisory board in August suggested the most likely number was around 6,000.

Even if high-end estimates prove correct, CJD will remain a relatively rare disease, and measures taken this week in the E.U. should help limit its spread. Vegetarianism may be the surest preventive, but if consumers avoid eating neural tissue such as brains they should be reasonably confident of not contracting the disease. Says Martin Hirsch, director of the French Food Safety Agency: "Muscle tissue and milk are products in which no infection has ever been found in cattle, either experimentally or naturally." In the past, that kind of rational argument was sufficient to reassure consumers. But today, they're no longer buying it.

Reported by Helen Gibson/ London, James Graff/Brussels, Martin Penner/Rome, Thomas Sancton/Paris and Regine Wosnitza/Berlin, with other bureaus
 
By HELEN GIBSON

Queniborough, in central Leicestershire, is a quintessential old English village with thatch-roofed cottages and elegant 18th century houses lining the main street. But the tranquillity is deceptive. In July, health authorities officially confirmed that Queniborough is the site of a "cluster" of cases of CJD. Five of the 81 deaths in Britain from the fatal brain disease have occurred among residents who lived within a 5-km radius of each other in the 1980s. Three men and two women, aged between 18 and 35, died over the last two years. "Of course we are all worried," says Ian Clarke, who works in his family's butcher shop. "But we are still waiting to hear some hard facts."

The authorities are working hard to provide them, using the tragedy of the Queniborough cluster to find common features among the victims. Interviews with their families, two sets of questionnaires and meetings between health authorities and residents have so far turned up only one common link. "All the victims ate beef between 1980 and 1991 — but they didn't buy it from the same butcher," says Dr. Philip Monk of the Leicestershire Health Authority. There was no occupational link — victims included a van driver, a farm worker and a bank employee — and baby food, school meals, contaminated vaccines or drinking water were all ruled out as unlikely links or causes. Says Arthur Beyless, 54, who with his wife nursed his daughter for 26 months before she died: "Pamela lived away from home for years — she could have eaten infected beef anywhere."

Though worried, many villagers believe the cluster is a coincidence and seem to blame neither their current butcher nor his predecessor. "Do I buy beef? I have never wavered," said Dorothy Stretton as she purchased two steaks last week. In fact, local people seem more distressed — at least on the surface — over the way the media have besieged the village since July. They complained of some German reporters hanging around the school offering candy in exchange for interviews. A British newspaper headline, "Village of the Damned," also annoyed villagers even though the description was attributed to residents.

The victims' families, except for Beyless who feels it is best to air the issue, generally avoid talking to outsiders. The deaths have added sadness to the quiet life of Queniborough, where most prefer to keep their feelings private.
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