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Regulators Debate U.S. Response to Mad Cow Disease

By Ori Twersky
WebMD Health News

May 26, 2000 (Washington) -- In April 1989, Joseph Sardo of Miami, Fla., was assigned to a new project. Soon afterward, he became withdrawn. At first, his family blamed it on the project. But by June, the data process administrator who once was able to add columns of 30 numbers in his head, could not even count back from seven. By August, he was dead at age 66.

Sardo suffered from Creutzfeldt-Jakob Disease, or CJD, a rare brain disorder characterized by sponge-like microscopic spaces in the nerve cells, called neurons, that cause progressive dementia and muscle spasms. It is thought to afflict about one person in 1 million. It can strike anyone, there is no treatment, it cannot be slowed, and it usually is fatal within one year.

Approximately 10 to 15% of CJD cases are inherited, yet how Sardo and the others contracted the disease remains unknown, which worries U.S. health officials. However, with a new, more infectious variation of CJD, better known as "mad cow disease," now posing an additional threat, they are taking no chances. In early June, FDA officials will once again meet with an "ad hoc" advisory committee created in 1995 to discuss what would be an appropriate response.

On the agenda is a blood donor deferral policy that has been credited in part with creating a national blood shortage, and the merits of a method that might free up that blood for transfusions as well as the manufacture of plasma derivatives. Adopted in August 1999, the deferral policy bans the use of blood donated from people who spent six or more months in the U.K. between January 1980 and December 1996.

The stakes could be high. Mistakes have been made in the past with regards to CJD and other infectious diseases. Between 1963 and 1985, the National Institutes of Health (NIH) supplied free of charge a human growth hormone that infected some recipients with CJD. And in the 1980s, the failure of government officials to form an appropriate blood donor policy with regards to HIV/AIDS literally led to an epidemic, during which thousands of Americans were infected while regulatory officials wrestled with the question of how to respond.

In this case, there are some striking similarities. "We simply don't know if there is the potential for CJD to be in the blood of donors," explains Steve Petteway, director of pathogen safety and research at Bayer Corp., where groundbreaking work has been done on that subject. But studies have shown that the infectious agent may reside in the blood of animals, which lends credibility to the FDA's concern, he says.

To find the answer, scientists around the world are working on a test to detect an unconventional protein called a prion, a previously unknown infectious agent believed to be behind CJD. Prions are thought to transform healthy molecules to a dangerous conformation, causing not only CJD but also mink encephalopathy, scarpie in goats and sheep, chronic wasting disease of mule deer and elk, feline spongiform encephalopathy, and bovine spongiform encephalopathy -- or mad cow disease.

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