This article appeared in the Boston Globe yesterday. Thought it might interest.
>RE: Globe article by Terry Allen: Rare, animalborne disease a
>Boston Globe 12/12/99
> Rare, animalborne disease a medical mystery
> Officials examine Maine deer in hunt for clues
> By Terry J. Allen, Globe Correspondent,
> New England is entwined in a medical mystery that stretches across
>species and the country. The clues raise more questions than
>answers: Three young people, one with links to Maine, have
>contracted an extremely rare, infectious disease that leads to
>dementia and death. A strikingly similar disease is killing
>thousands of deer and elk in the western United States. Elk farms in
>New England, which cater to an Asian market for aphrodisiacs, are
>importing animals that officials warn could spread the infection.
> And in the background looms the specter of mad cow disease, the
>British epidemic responsible for the slaughter of hundreds of
>thousands of cattle, the devastation of the British beef industry,
>and the death, so far, of more than 40 people.
> Researchers are struggling to determine if these factors are part
>of the same trail of evidence or if they are connected only by
>coincidence and fear.
> Either way, there is no denying that federal researchers are
>worried. How else to explain why a US Department of Agriculture
>official came to Maine last month to supervise a ''harvest'' of 299
>heads from deer shot during the fall hunting season? ''They used a
>fancy spoon to scoop out a portion near the brain stem for
>analysis,'' said Mark Caron, a Maine wildlife biologist.
> The reasoning behind the examination of deer brains? One of the
>young victims of the rare disease who lived in the South had, years
>before her death, eaten meat from deer her father had shot in Maine.
> The Maine samples are part of a nationwide investigation into a
>family of diseases called transmissible spongiform encephalopathies,
>or TSE, that do similar damage to the brains of different mammals
>deer, elk, sheep, cattle, cats, mink, and humans. But they are
>subtly different illnesses. And until mad cow disease - the bovine
>TSE - hit Britain and apparently spread to people who ate infected
>beef, most scientists believed TSEs did not jump from one species to
> The possibility that chronic wasting disease the TSE that affects
>deer and elk has made the leap to humans is what had federal
>researchers concerned enough to survey Maine.
> The 28yearold woman who ate Maine deer died of CreutzfeldtJakob
>disease the human TSE. There are only five reported CreutzfeldtJakob
>cases per billion worldwide in people 30 years old or under,
>according to Lawrence Schonberger of the federal Centers for Disease
> Yet, since 1996, CreutzfeldtJakob disease has been detected in
>three Americans in that age group; two are dead and one is dying.
>All three, it turns out, had hunted extensively or eaten venison.
> ''It may be a coincidence,'' said Dr. Pierluigi Gambetti, professor
>of pathology and director of the National Prion Disease Pathology
>Surveillance Center at Case Western University in Cleveland, where
>the Centers for Disease Control sends its human brain samples. ''It
>would be imprudent to say there is a danger of an epidemic. But,
>yes, it's something that has to be studied.''
> Said Thomas Pringle, a biochemist who runs a Web site on TSEs:
>''What has CDC worried is that the original tipoff in Britain that
>something was wrong was the upsurge in cases of CreutzfeldtJakob
>disease in young people.''
> The CDC medical epidemiologist, Ermias Belay, citing a Red Cross
>survey, emphasized that since 40 percent of Americans have eaten
>wild venison at least once, the three cases could be explained by
>chance alone. ''If there had been one more,'' he said, ''it might
>tip the balance.''
> But, countered Michael Hansen, a research associate at Consumer's
>Union, ''Given how rare the disease is in young people and how
>difficult it is to make a diagnosis, the possibility that some cases
>go undetected cannot be ruled out.''
> That is what nearly happened last year in Utah to Tracie McEwan.
>The young wife and mother of two watched desperately as, over a few
>months, her 28yearold husband, Doug, lost motor control and memory,
>became disoriented, and suffered dramatic mood swings.
> ''After doctors performed hundreds of tests,'' Tracie recalled, ''I
>saw a television show about mad cow disease.'' She demanded the
>doctors test for CreutzfeldtJakob disease. A brain biopsy confirmed
> ''We have decided to get pretty aggressive, primarily because of
>the situation in Britain,'' said the CDC's Schonberger. He
>emphasized, however, that ''none of the three cases has been linked
>to chronic wasting disease in deer and elk.
> While the threat to humans remains theoretical, the danger of the
>disease within deer and elk populations is real and growing. ''It's
>been spreading slowly since it was first found in the wild in
>1981,'' said Beth Williams, a professor of veterinary services with
>the University of Wyoming. Chronic wasting disease is now a major
>problem in Colorado and Wyoming, infecting 4 to 8 percent of the
>62,000 deer there and 1 percent of elk, she said.
> In states other than Wyoming and Colorado, tests on 4,500 seemingly
>healthy wild animals have found no evidence of disease, but that
>sampling does not fully allay scientists' concerns. TSEs are caused
>by prions, a puzzling proteinlike substance that is extremely
>difficult to detect or kill. Prions can remain infectious on
>correctly sterilized surgical instruments and have been transmitted
>through corneal transplants, growth hormones, and meat that has been
> They are also apparently passed among deer and elk by routine kinds
>of contact. Outbreaks of chronic wasting disease have spread among
>farmed elk not only in Colorado and Wyoming, but in other parts of
>the West, forcing the destruction of herds and the quarantining of
>farms. Montana saw its first confirmed case last month.
> So far New England appears to be free of the disease, but the
>chance of infection rises with the number of western elk shipped
>east to supply the region's growing elk farming industry. The
>animals are prized for the velvet on their antlers, believed in some
>parts of Asia to be aphrodisiacs.
> ''Elk farming is the recipe for introducing CWD into areas where it
>has not existed before,'' said Dr. Victor Nettles, director of the
>Southeastern Cooperative Wildlife Disease Study, referring to
>chronic wasting disease.
> ''We are at the extremely initial stage of awareness and
>monitoring,'' warned Robert Deblinger, assistant director of the
>Massachusetts Wildlife Division. ''But states need to be aggressive.
>It's only going to get worse, so it's important to be vigilant. That
>is the only way you keep the diseases out.''
> Of immediate concern to New England's wildlife managers is the
>health of native deer, which support the region's multibilliondollar
>hunting industry. Since there is a two to fiveyear incubation period
>and no reliable test for live animals, apparently healthy elk can
>harbor and spread the disease.
> The elktrading network is extensive and loosely regulated.
>Paperwork that accompanies each elk to Vermont, for example, tells
>only where the animal was bought, not where it was a year or even a
>month before. And if the herd it passed through later has an
>outbreak of chronic wasting disease, there are no procedures to
>trace exposed elk or issue warnings.
> Vermont, Maine, and New Hampshire have not formally notified elk
>farmers to be on the lookout for neurological symptoms, nor do they
>require reporting of suspicious symptoms. In Vermont, when one of
>the state's 250 captive elk dies, the owner may comply with a
>voluntary program for testing.
> ''From the standpoint of protecting wildlife,'' said Steve Weber,
>chief of New Hampshire's Wildlife Division, ''a ban on elk farming
>is the prudent thing to do, but when balanced against existing
>industry, there are tradeoffs you make.'' New Hampshire has a
>handful of elk farms and a few hundred elk, Weber estimated.
> So far, Massachusetts is the only New England state to ban elk
>farming. ''Anytime you want to fence in a wild animal it's going to
>get out,'' warned Robert Deblinger, assistant director of the
>Massachusetts Wildlife Division. ''We worry about interbreeding and
>genetic pollution as well as the introduction of chronic wasting
> That infected western elk can transmit TSE to native New England
>deer is established science. The more troubling and as yet unsolved
>mystery is whether, like its bovine counterpart, chronic wasting
>disease can, or has, jumped species to infect humans.
>C Copyright 1999 Globe Newspaper Company.
>Terry J. Allens e-mail is <firstname.lastname@example.org>
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