Mad Deer Disease Spreads Across the USA
--Hunters Are Starting to Worry

This is from Outdoor Life on line at:
October 1999

Mad Deer Disease: Can Venison Kill You?
by Frank Miniter

A fatal brain disease is spreading in deer and elk... and hunters
dying from a very similar illness.

The disease that struck the three hunters is ominously similar to
another disease that's
spreading in deer and elk in Colorado and Wyoming. This possible
connection has
some people pointing to deer meat and crying killer.

The accusation could prove true. The disease found in deer and elk
is called Chronic
Wasting Disease (CWD), and it's closely related to Creutzfeldt-Jakob
Disease (CJD),
which is what killed the hunters. Both CJD and CWD are classified as
spongiform encephalopathies" (TSE). Diseases don't always make the
leap from one
species to another, but there is a connection between chronic
wasting disease and
Creutzfeldt-Jacob that has many scientists concerned: Another
spongiform chronic encephalopathy (BSE)-spread from cattle to humans
in the
United Kingdom, where it was dubbed "Mad Cow Disease."

Mad Cow Disease exploded in U.K. cattle herds in the late 1980s and
early '90s. But
it was not until 1996 that it was found to have crossed over to
humans. Since then, 43
people are known to have died in the U.K. from Mad Cow Disease, but
because of its
long incubation period-possibly up to 20 years-it may yet kill many
more. The
disease resulted in European bans on British beef and forced the
destruction of more
than half of the cattle in the U.K.

Because of the similarity, CWD has already been nicknamed "Mad Deer
Disease," but
it hasn't yet proved as sinister. In fact, another TSE called
"scrapie" has afflicted
sheep for at least 250 years and has never been found to cross over
to humans. Still,
because of Mad Cow Disease, CWD is hitting the hunting world like a
horror movie
monster, lurking unseen in the shadows.

Scientists, however, are on the monster's trail. More than a dozen
states and the
federal Centers for Disease Control (CDC) in cooperation with the
Animal Plant
Health Inspection Service (APHIS) are pouring money and resources
into a
thus-far-quiet, but nevertheless massive, investigation. They're
trying to answer two
questions: Is CWD killing people? And where is CWD?

To answer the first question, the CDC is taking a street cop
approach by chasing
down every lead that comes into its Atlanta headquarters. And with
what they've
found so far, this horror movie is starting to feel more like an
X-File. Larry
Schonberger, a medical epidemiologist for the CDC, says, "I've sent
one of our
epidemiologists out to investigate to see if there's a link between
CWD and the [three]
hunters' deaths. I did this because two of the victims were young
[one was 27 years
old, the other 30]. That's very unusual. CJD normally shows up in
people well over
30. But our scientist's initial report is that the deer eaten by the
victims had not come
from [known] infected areas. But we're taking no chances. In fact, a
young girl died
in a southern state from CJD who had reportedly eaten venison that
her father had
shot in Maine, so this year 300 deer will be checked for the disease
in Maine.

"At this point we can't rule out a link between Chronic Wasting
Disease and the
hunters' deaths, but I think it's unlikely. CJD occurs all over the
world at a ratio of
about one in 1 million people. Each year in the U.S. 250 to 300
people die from CJD.
So it's understandable that a few of the victims happened to have
eaten venison," says

Meanwhile, APHIS and many state agencies are doing a broader search
to discover if
CWD can be found outside known infected areas, and to find out how
fast it's
spreading from the infected area. Since there is no accurate test
that can be
performed on living animals, scientists are checking brain samples.
The samples are
collected at deer check stations and meat processors. From there
they are sent to a
number of labs for analysis. Colorado and Wyoming each check
thousands of samples
annually at their own research facilities, but other states, such as
Nebraska, South
Dakota, Montana and Nevada, send their samples to the APHIS lab in
Ames, Iowa. So
far no wild deer or elk outside of Wyoming and Colorado have shown
up with the
disease. CWD has been found in elk produced in the game-farm
industry, however.

Both CDC and APHIS efforts are designed simply to get a fix on the
situation. Right
now not much can be done to stop or prevent Mad Deer Disease because
know so little about it. To get answers, however, labs worldwide are
studying all spongiform encephalopathies. The CDC, for instance, is
investigating this
group of pathogens at its Prion Disease Pathology Surveillance
Center at Case
Western Reserve University in Cleveland. But despite the intensive
research efforts,
answers are slow to come.

In fact, scientists don't even agree on what causes TSEs, although
there is wide
support for a theory put forth by Stanley Prusiner, M.D., a
professor of neurology at
the University of California at San Francisco, whose research won
him the Nobel
Prize in Medicine in 1997. Prusiner theorizes that "prions"
(proteinaceous infectious
particles) cause this family of diseases, and not a virus, as was
previously assumed.
His chief reason for fingering "rogue proteins" is that the chemical
and physical
procedures that destroy most viruses don't affect TSEs, whereas
procedures that have
been found to degrade proteins seem to inactivate them. Prusiner's
hypothesis is that
prions kill by turning normal proteins in nerve cells into
infectious ones by forcing
them to alter their shape.

A Doomsday for Deer?
In light of the recent conjecture about CWD killing humans, it's
easy to overlook one
certainty about this disease: It kills deer. The ramifications of
that indisputable fact
are almost as unsettling. Before we go on, keep three things in
mind: CWD is
spreading, CWD is always fatal and CWD has no known cure.

In the infected areas of Wyoming and Colorado, about 4 to 8 percent
of deer and 1
percent of elk have the disease, according to Beth Williams, a
professor of veterinary
services with the University of Wyoming, and Mike Miller, a
biologist with the
Colorado Division of Wildlife.

"It's been spreading slowly since it was first found in the wild in
1981," Williams
says. "We think [CWD] passes from animal to animal through bodily
fluids. So it
probably takes physical contact of some kind to pass the disease."

That said, the spread of CWD may soon pick up speed because
whitetails may be next
on its hit list. Thus far the disease has mostly spread in mule deer
up the South Platte
drainage in northeastern Colorado. Mule deer have fairly thin
population densities
along the river, but whitetails have heavy population densities in
this area. This
dilemma has Miller worried because the whitetail's heavier
population density means
there is a greater chance of physical contact, which means CWD could
move more
rapidly. "Based on some computer models that we did at the Division
of Wildlife, this
scenario could prove devastating," Miller says.

"The only preventive measure we can take is to cut down the deer
population in the
infected areas. But these areas are popular with hunters, so that
would be
controversial. As a result, right now we're just checking for the
disease, but unless
something is done, CWD could spread farther and farther east. I'm
not saying that
it'll be in New Jersey next year, but in 10 or 15 years, who knows?"

So this article ends not with a climax, but with a "to be
continued." Many questions
still surround what we hope is erroneously named Mad Deer Disease.
we're left in the dark, speculating as to what it means to deer, to
hunting and to