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The Nov. 11 edition of the Bangor Daily News carried an Associated Press article describing a near panic sweeping Europe about “mad cow disease.” French chefs are removing beef dishes from their menus, schools have stopped serving beef in their cafeterias and officials of the European Union are calling for stringent new safety controls and the testing of thousands of animals.
British beef is banned from France while Switzerland is threatening to do the same to French exports. In spite of the furor, only two deaths have occurred on the continent from variant Creutzfeldt-Jakob disease, the malady that has been associated with bovine spongiform encephalopathy, better known as mad cow disease. By contrast, Britain, where BSE first arose, has recorded more than 80 deaths from VCJD.
In late October, the British government released a 16-volume report on its handling of the crisis. As a series of articles in recent issues of New Scientist and the journal Nature tell it, the British government’s response was inept and deliberately misleading. What happened in Britain should provide both the Europeans and the rest of the world guidelines in how to guard against this terrible disease.
The first confirmed cases of BSE appeared in 1984 in cattle whose behavior became increasingly erratic, who developed uncontrollable staggers, and whose brains, at death, were found to have developed holes making them spongelike. Experts at the time had no real idea of how BSE originated. Today, New Scientist’s Claire Ainsworth writes that the best guess is that it arose from a chance mutation in the prion gene of cattle or sheep in the early 1970s.
Prions are small proteins simpler in structure than viruses. But this was not known in the mid-1980s, and the panel set up to study the BSE outbreak made what turned out to be a fatal mistake. They believed BSE arose from a disease known as scrapie in sheep, through the use of meat and bone-meal renderings from infected sheep as cattle feed. Since scrapie was not known to have infected humans, it was decided that BSE-infected meat was equally harmless.
In 1989, this became the official line of the British government, which again, assured the public that the country’s beef was perfectly safe. Others had their doubts, saying diseases such as scrapie, upon jumping to another species, could become pathogenic. This became evident in 1994, when several domestic cats were found to be infected with BSE that only could have been acquired from eating infected beef.
Despite this wakeup call, the British government continued to rely on its initial committee finding that BSE-contaminated beef posed only a “remote” risk to human health. Farmers continued to feed meat and bone meal to cattle, and slaughterhouses were not required to alter their methods to prevent infected spinal tissue from contaminating their products.
Moreover, the government withheld publication of research on BSE for six months and refused research funds to a scientist who was doing a mathematical analysis of the link between BSE and VCJD.
In May 1995, the first human death from VCJD occurred and, in March of 1996, the British government announced a probable link between BSE and VCJD. Reaction was immediate and draconian. The European Union banned British beef from the continent, beef was removed from schools and markets, and thousands of infected cattle had to be destroyed. The British beef industry was in chaos, but the damage already had been done. Fatalities from VCJD have gone from one in 1995, to 24 to date.
Debora Mackenzie in New Scientist estimates the ultimate death toll could exceed 20,000 from a disease once so rare that a doctor could go his entire career and never encounter one. And it is not an easy passing, as numbness, mood swings and hallucinations are followed by the characteristic staggering gait. Finally, the victim loses thoughts, sight, memory and personality before death offers a merciful release.
There has been no proven case of VCJD from eating domestic beef reported in the United States. But the connection between scrapie in sheep, BSE in cattle, and VCJD in humans seems to have been proven beyond question, and our own government will do well to study the mistakes made by their British counterparts.
Clair Wood taught physics and chemistry for more than a decade at Eastern Maine Technical College in Bangor.
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