Mark A. DeSorbo
ATLANTA, GA-Officials at the Cen ters for Disease Control and Prevention (CDC) are investigating the first suspected case of Creutzfeldt-Jakob Disease (CJD), the human variant of mad cow disease.
The CDC, along with Florida Department of Health officials, believe the 22-year-old British woman, who lives in the sunshine state, contracted the disease overseas.
“I think the important thing is that she resided and was raised in the United Kingdom during the period when the [mad cow] epidemic was at its peak in the United Kingdom,” Dr. Steve Ostroff, acting director for the National Center for Infectious Diseases at the CDC said during an April teleconference with the media. “There is every reason to believe, and both we and the Florida Department of Health believe, that her exposure and her illness occurred within the United Kingdom and not in the United States.”
While the CDC and the U.S. Department of Agriculture (USDA) maintain it is “very confident that the United States does not have mad cow disease,” the reasoning behind reporting the case represents “the first recognized resident of the United States in which this particular diagnosis has been made.”
“She sought medical care in the United Kingdom and the diagnosis was made in the United Kingdom,” Ostroff says. “And the diagnosis at this point is not absolutely definitive because the information is still being reviewed by the National Surveillance Center in the UK, but so far all evidence points to this particular diagnosis.”
Challenging discoveries
The human variant, CJD is a rare, degenerative, fatal brain disorder that emerged in Britain in the mid-1990s and is believed to be caused by eating meat from cattle infected with mad cow disease, known as bovine spongiform encephalopathy (BSE).
The mind-drilling proteins of mad cow disease are now being found in muscle tissue, countering the belief among most scientists that the damaging proteins were contained within brain and nerve tissue.
In a study published in the Proceedings of the National Academy of Sciences, U.S. researchers explained that they had discovered for the first time that the abnormal proteins, or prions, can spread through muscle tissue in mice.
If the same goes for livestock, it may not only challenge existing philosophies about mad cow disease, but also refute current safeguards in place to protect beef consumers from the fatal human variant.
“Our findings indicate that a comprehensive and systematic effort to determine the distribution of prions in the skeletal muscle of animals with prion disease is urgently needed,” the study indicates.
Meanwhile, the USDA announced that it will continue to test a flock of sheep now that two of the 125 recently tested positive for a transmissible spongiform ecephalopathy (TSE), a derivative of mad cow disease.
Bobby Acord, administrator of the USDA's Animal and Plant Inspection Service, says the flock was confiscated in March 2001 from a Vermont farm after livestock from an associated flock tested positive for TSE in July 2000. The flock from Vermont, he says, consisted of offspring from animals that were imported from Belgium and the Netherlands in 1996. Two years later, the USDA learned that the sheep had been exposed to feed contaminated with mad cow disease.
“These results confirm that our previous conclusions were correct and that we took the appropriate preventative actions in confiscating these animals, Acord says. “None of these confiscated animals entered the animal or human food supply.”
So far, 117 people in Britain have died from the human variant, CJD. France has reported six deaths, while Italy and Ireland have each reported one.
The Associated Press, Reuters and several other mainstream media outlets have reported that no cases have been reported in the United States among humans. The Center for Food Safety (CFS; Washington, DC), however, claims the disease, or some form of it, is already here, and has been for some time.
On January 7, 1999, CFS filed two legal petitions demanding that the U.S. Food and Drug Administration (FDA) and the CDC act immediately to monitor, regulate and prevent “mad cow” type diseases in the U.S., saying people, deer, elk and sheep are dying.
The legal petitions, which charge the United States efforts have been “grossly inadequate,” were filed on behalf of a 30-year-old Utah man, R. Douglas McEwen, who is terminally ill with CJD.
“McEwen hunted deer and elk. It is feared that he may have contracted CJD by eating or handling deer or elk infected with mad deer disease,” a newsletter on the Web site said. “There is additional concern because McEwen was a frequent blood donor, and may have contaminated blood products internationally. McEwen is a petitioner in the local action along with the CFS.” [See “Mad cow disease: A shot heard around the world,” CleanRooms, March 2001.]
Aside from the sheep from Vermont, however, CFS claims that deer and elk are dying because of a mad cow-like ailment may now be substantiated.
In early April, health and wildlife officials in Denver confirmed that that four mule deer at a captive elk facility in northwest Colorado tested positive for a brain wasting illness similar to mad cow disease, leading to the slaughter of some 300 wild deer.