Mad Deer and Elk Disease Spooks Hunters
and Health Officials in New England

Boston Globe 12/12/99

Rare, animal borne 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

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 another.

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 28 year-old woman who ate Maine deer died of Creutzfeldt Jakob disease
(CJD) 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 Control.

Yet, since 1996, Creutzfeldt Jakob 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 moods swings.

''After doctors performed hundreds of tests,'' Tracie recalled, ''I saw a
television show about mad cow disease.'' She demanded the doctors test for
Creutzfeldt Jakob disease. A brain biopsy confirmed her fears.

''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 thoroughly cooked.

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 disease.''

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