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Mad, bad, and difficult to cover

IN KENT, ENGLAND, in 1985, some cows were staggering because of a new disease. In 1996, the British government, economy, and press were staggering from the same thing: bovine spongiform encephalopathy (BSE, a.k.a. mad cow disease). After years of the British government insisting people were not at risk from BSE, a new strain of a similar disease in humans, Creutzfeldt-Jakob disease (CJD), was reported in March. The result was what the British Medical Journal and others called a media meltdown.

BSE is a neurodegenerative disease caused by a prion (a transmissible protein). BSE appears to be a form of scrapie, another prion-induced neurologic disease seen in sheep for centuries, which jumped from sheep to cows when British cattle farmers started using animal feed containing prion-infected sheep offal. CJD is also a prion-induced neurodegenerative disease; however, it usually occurs in people over age 60, and the latency period between infection and onset of symptoms is decades. The new cases in Britain (along with one in France) have occurred in people under age 42. This led the Spongiform Encephalopathy Advisory Committee of the British government to conclude in March that a new strain of CJD had arisen. The committee also said the new cases were probably linked to exposure to BSE before the ban on offal in animal feed took effect.

In the years since cows started staggering, the British government has taken steps that critics said were too little, too late. Farmers were first offered only half the going price for each infected animal, giving them little incentive to detect BSE. It took two years to ban animal feeds with offal once the connection was made and to change slaughtering techniques to reduce contamination of brain and offal into meat. In the meantime, Europe banned importation of British beef and cattle, McDonald's restaurants and schools in the United Kingdom stopped serving British beef, and the British cattle industry ($6.2 billion in sales) was brought to its knees.

Press coverage of BSE has ebbed and flowed since 1985, with stories ranging from screaming--"Mad Cow Disease Killed My Mother" (South Oxfordshire Courier , 1990)--to calm dismissal--"Risk from BSE is slight" (Yorkshire Post , 1990). But in March, when the new strain of CJD was announced, television and newspaper coverage went into overdrive, jumping from interviewing young CJD victims and their families to speaking to angry British farmers. In March, the headlines read "Mad Cow Can Kill You: Government to Admit it Today" and the scarier "We've Already Eaten 1,000,000 Mad Cows" (Daily Mirror , March 20, 1996).

"This was absolutely a political story rather than a science story," said Steven Sander Ross, associate professor of professional practice in the Graduate School of Journalism at Columbia. "This sort of thing happens often when a major science story breaks." A major medical story often becomes a political one and outgrows its technical base as government officials make pronouncements and issue reports, he said.

The BSE story shook the Conservative government of Prime Minister John Major. In Britain, Ross noted, the news led to finger-pointing at Tory policies that had cut funding to food safety and health care programs. Britain has a successful agricultural system less subsidized than the European system and a strong quarantine program to keep out animal diseases and parasites. When British beef was suddenly suspect, European countries seized the opportunity to ban a British product. European news outlets, which tend to fall along political party lines, relished complaining about British agricultural practices, and British newspapers, which also toe party lines, defended British policies from foreign attack.

The only balanced article on BSE that Ross saw appeared in the March 30, 1996, issue of The Economist. An unsigned article, along with one in the March 23 issue, gave an overview of BSE, CJD, prions, and the shortcomings of various government actions. The March 30 article also discussed possible continued prion infectivity in cattle fields and vertical transmission from cow to calf. The tentative wording of the Spongiform Encephalopathy Advisory Committee's report, which added to public distress, did not help matters. Medical research in Britain is underfunded, Ross pointed out, and epidemiologic studies of rare diseases like CJD cost money. "The report was written in such a tone that any reporter would have said 'They needed more research and had no money,'" he said. There is also the question of whether the slightly increased rate of CJD seen in Great Britain in the past few years and the 11 cases in younger people are not a new phenomenon but were diagnosed because the British medical system is now looking for CJD.

That is unlikely, said W. Allen Hauser, M.D., associate director of the Sergievsky Center and Columbia professor of neurology and public health. It is possible that patients who would have been diagnosed with Alzheimer's disease or senile dementia in the past are now being correctly diagnosed with CJD, he said, but the pathology in younger patients can not be confused with anything else, and the new cases are unlikely to be artifacts of increased surveillance.

The epidemiology of BSE and CJD deserves further study. Scrapie has existed for centuries in Britain, where lamb and mutton are popular meats, but a link between CJD and eating lamb has never been noted, said Dr. Hauser. But then, sheep brain and eyeball--which carry the most prion in infected animals--have never been popular dishes in England. There is some suggestion of a connection in Libyan Jews, who eat sheep brain and who also have a high rate of CJD, he noted.

This story is still developing, Ross pointed out. Reports in the British press that the first cases of the new strain of CJD are the start of a large epidemic may not be unfounded. The link between eating BSE-infected beef and developing CJD seems strong to at least one group. "British neurologists that I know have not been eating beef for many years," said Dr. Hauser.

--Valerie DeBenedette

Related links...

  • BSE page, Institute of Food Science and Technology

  • BSE page, National Institute of Animal Health (Japan)

  • BSE article by Dorothy B. Preslar, Federation of American Scientists' Program for Monitoring Emerging Diseases (ProMED); update, August 12, 1996

  • BSE information, British Ministry of Agriculture, Fisheries and Food

  • Transmissible spongiform encephalopathies page, Institute of Animal Health, UK

  • Outline of British government's BSE eradication program

  • Creutzfeldt-Jakob disease page, "Outbreak" emerging-diseases site

    VALERIE DeBENEDETTE writes frequently on medical topics. Her work has appeared in National Cancer Bulletin, Cosmetic Dermatology, Drug Topics, and The Physician and Sportsmedicine. She is also a part-time staff writer for Gannett Suburban Newspapers. Her first book, Caffeine (Springfield, N.J.: Enslow, 1996), a guide for teen-agers, has just been published.

    ILLUSTRATION: Howard Roberts

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