Two young men, ages 26 and 28, died last fall in the same Michigan hospital of a rare brain disease that occurs mainly in elderly people. The incident, which raises fears that the human form of mad cow disease is here in the USA, prompted a swift investigation by federal health officials, but doctors familiar with the cases say there is no evidence to support that fear. They say autopsies and other tests indicate the victims died from so-called ”classic” forms of Creutzfeldt-Jakob disease (CJD).
While the cases are ”highly unusual and disturbing,” says University of Michigan neurologist Norman Foster, the data show that the forms of CJD suffered by the young men are ones seen previously in older individuals. CJD occurs at the rate of about 1 person per million per year, almost always in people over age 60. What doctors fear is that a new form of CJD, possibly similar to a variant that emerged in the mid-1990s in the U.K. and linked to consumption of mad-cow-infected beef, will strike here.
Anita Manning writes in USA Today that unlike classic CJD, the new Mad Cow variant, vCJD, strikes mainly young adults. It has killed more than 100 people. The only known case of vCJD in the U.S. was diagnosed recently in a 22-year-old British woman living in Florida, who is thought to have contracted the disease in England.
Mad cow disease has not been detected in cattle in the U.S., but a similar disease in deer and elk is spreading in the Midwest. Chronic wasting disease (CWD) is fatal to deer and elk but is not known to cause illness in humans.
Lawrence Schoenberger of the Centers for Disease Control and Prevention in Atlanta says the agency sent investigators to Michigan in late August, when the victims were still alive. ”The key thing here is the two were right together. We were worried that there was maybe a common exposure, but our investigation revealed that was not the case.” The men lived in adjacent counties but did not know each other, he says.
In the rare cases when CJD strikes before age 30, it is often caused by a hereditary form of the disease, says Foster, and ”tests are continuing to see if that may be a factor in these cases.” But extensive family interviews determined that neither man had a family history of dementia, nor had they eaten venison or elk meat or visited countries where mad cow disease has been detected. ”We feel as comfortable as anyone can that this is not related to either CWD or [mad cow disease],” says Foster, who treated the patients at the University of Michigan Medical Center in Ann Arbor.
”I discount the statement that these two young people, dying at the same time in the same hospital in southeast Michigan, did not eat venison, after living their entire lives in that state,” says John Stauber of the Center for Media & Democracy. He suspects a new American variant of CJD, perhaps related to chronic wasting disease, may be emerging. ”Any attempt to portray these CJD deaths as some sort of ‘normal’ occurrence that has simply, to date, gone unobserved is absurd,” he says.
”The fact that they both occurred at the same time in a relatively small population suggests that [CJD in younger people] may be more common than previously suspected,” Foster says. Doctors don’t expect to see it in young people, so misdiagnosis may occur. ”Any young individual with progressive neurologic disease should be considered for CJD.” He says the cases also underscore the need for a national system to seek out and report all cases of CJD. ”There certainly is the possibility that other cases have been seen and not diagnosed, or even if diagnosed, not reported.”
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Hunter David Peil has wondered whether the venison stowed in his freezer is safe to eat ever since the Wisconsin Department of Natural Resources said earlier this year that three deer shot by hunters last fall tested positive for chronic wasting disease (CWD), a fatal neurological illness related to mad cow disease. The cases were the first ever found east of the Mississippi River.
A state-organized cull this spring of 516 deer from the same region of southwest Wisconsin turned up 11 more cases of CWD, prompting officials to plan a much larger de-population effort to halt the spread of the disease. Starting next week, Wisconsin will call on farmers and other land owners to help shoot as many as 15,000 deer in the area.
Whatever the outcome, the disease outbreak has raised fears about the safety of meat left over from hunting season last fall. “The deer that we’ve shot here didn’t appear to be sick. But I really don’t know if I’ll eat more or not,” says Peil. “You think about it — is it really worth having a piece of venison?”
Wisconsin DNR spokesman Bob Manwell says, ?It’s similar to a lot of health advice that is given out, where it really comes down to a personal decision. There is a small risk, and it’s the individual’s decision as to how they feel about that.” For now, the state plans to go ahead this fall with a deer donation program that provided meat to food pantries and needy families. Hunters donated 3,921 deer to the program last year, resulting in about 176,000 pounds of venison. “Even after the realization that we have this disease in the state, there has still been demand for the meat,” Manwell says. “It’s really going to be up to individual food pantry organizations what they want to do about it.”
Will hunters still hunt if they do not plan to eat what they kill? “We’re going to be watching our [hunting] license sales very closely,” Manwell says. “We have a chance right now, if we take this action, of possibly eradicating this disease and maintaining the health of the rest of the herd. While it’s distasteful, it’s one of those things that we feel the need to do.”
“In Wisconsin, deer hunting is a kind of religion,” Peil says. “I think [hunters] will pitch in to see if they can slow the disease or stop it. But if they can’t, and it spreads, I think it’s going to be tough to hunt.”
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