Mad Deer Disease Spreads in US--Hunters Are in Danger

New York Times, Tuesday, October 31, 2000
Biologists Say Hunters Should Beware of Brain Disease
By Sandra Blakeslee
The Associated Press

Some scientists worry that chronic wasting disease, which has appeared
in some elk and deer, could be transmitted to hunters, but no cases in
humans have been directly tied to the meat. Elk forage in Wyoming.
Likening the situation to the early days of the mad cow epidemic in
Britain, some biologists say American hunters should be warned about a
similar malady that has infected wild deer and elk in parts of Colorado
and Wyoming.

The malady is called chronic wasting disease. While no cases of human
disease have been directly traced to deer or elk meat, there is a
growing body of evidence to suggest that it could happen. And with the
hunting season in full swing, a number of scientists are calling for
more action to warn hunters about the potential problem.

Both mad cow disease and chronic wasting disease are believed to be
caused by aberrant proteins, called infectious prions, which affect the
brain, destroying tissue and leaving it with sponge-like holes. When the
cow disease first appeared in Britain in the mid-1980's, government
agriculture and health officials initially offered assurances that it
could not spread to people.

By now, though, 77 Britons have died of a related brain disorder that
they are believed to have contracted from consuming affected meat. It is
not known how many others may ultimately fall ill and die.

Eventually, the British government destroyed almost four million cattle
to stem the spread of the disease. But last week a three-year
investigation into the causes of the epidemic severely criticized the
government's "culture of secrecy" in not being more honest with the
public, and for using "an approach whose object was sedation."

Wildlife officials in Colorado and Wyoming, where the chronic wasting
disease is firmly entrenched along their shared border and is estimated
to affect 1 percent of elk and from 6 percent to 15 percent of deer,
insist that not enough is known about the problem to cancel hunting

Dr. Mike Williams, a veterinarian at the Colorado Department of Wildlife
in Fort Collins said, "We don't think the problem is a big deal." Areas
where the disease is endemic are not closed to hunting, nor is there a
need to close them, he said. "If people choose to hunt there, it is
their choice," he said.

Instead, hunters are advised by state wildlife officials to avoid
obviously sick animals and to use rubber gloves in cutting up all
carcasses, particularly brain and nervous tissues where the infectious
prions apparently concentrate. But people familiar with hunting
practices in those areas say hunters are not taking even these

"Around here, people are not knowledgeable about the disease or just
don't care," Arnold Hale, a retired hunting outfitter from Livermore,
Colo., said in a telephone interview. "When you talk to hunters, most
don't trust the government. I don't know anyone taking precautions."

Kurt Zunker, 28, a probation officer from Cheyenne, Wyo., who is an avid
hunter, said: "I'm aware of the situation but not really abreast to the
complete ramifications of it. It won't stop me from hunting."

Mad cow disease and chronic wasting disease are among a bizarre class of
prion-caused disorders known as transmissible spongiform
encephalopathies or T.S.E.'s. The aberrant protein molecules are folded
abnormally, which seems to endow them with their ability to cause
disease. Sometimes they seem to misfold all on their own. In other
cases, the misfolded proteins are transmitted via food, blood
transfusions or surgical instruments.

Transmission is difficult to track, however, because people or animals
typically develop the disease a long time after they have been exposed
to the misfolded prions. Until a few years ago, for example, it was
widely believed that each animal, including humans, had its own unique
form of T.S.E. and that the diseases rarely passed between species.

But in 1996, when young Britons began dying from a particularly rapid
human form of the disease, Creutzfeldt-Jakob disease, or C.J.D., the
species barrier began to crumble. Until then C.J.D. was diagnosed
exclusively in much older people. Tests soon revealed that the disease
was a new variant of C.J.D. that could be traced directly to eating
cattle infected with mad cow disease.

In recent months, researchers have discovered that a prion from
hamsters, thought never to infect mice, not only replicates profusely in
mice but it does so without producing disease symptoms. In other words,
mice can be silent carriers of a disease that originated in hamsters.
When subsequent generations of mice are exposed to blood or nervous
tissues from silent carrier mice, they get the disease. Other
experiments carried out in the United States show that the deer or elk
prion in chronic wasting disease can convert healthy human prions into
infectious prions in test tubes. The conversion rate is slow but

The best available science shows that chronic wasting disease can infect
human tissue but wildlife officials are carrying on with "business as
usual," said Dr. Tom Pringle, a biologist in Eugene, Ore., who closely
follows T.S.E. worldwide and independently studies the disease.

"Who'd want hamburger from a cow where 15 percent of the herd had mad
cow disease?" Dr. Pringle asked. "Who'd want mutton from a sheep where
15 percent of the sheep had scrapie? To me it looks like Russian
roulette for hunters."

Most Americans don't understand how dangerous these diseases really are,
Dr. Pringle said. It is extremely difficult to kill infectious prions
that have come into contact with surgical instruments, yet some hunter
who is "tired, cold, hungry and drunk" will cut off a deer head, handle
the animal's spinal cord and "stick his hunting knife back into his
scabbard," he said. That knife could spread disease.

Dr. Pringle strongly criticizes wildlife officials as downplaying the
seriousness of chronic wasting disease. Just as British agricultural
officials initially acted to protect the image of the cattle industry -
one even fed hamburger to his 4-year-old daughter on television to prove
beef was safe - American wildlife officials are acting to shield hunting
and fishing, he said. Fishing and hunting license fees provide a
substantial portion of their agencies' income.

But Dr. Elizabeth Williams, a veterinarian at the University of Wyoming
in Laramie and a leading researcher on chronic wasting disease, said
that hunters are not going to be deterred until there is much better
evidence that chronic wasting disease in deer and elk is a danger to
people. The prevalence and incidence of chronic wasting disease has not
changed much over the past several years, she said. And it is still not
known how one animal passes the disease to another, much less if humans
face more than a theoretical risk.

Hunters, many of whom come from other states, are given a 54- page
brochure that describes chronic wasting disease, Dr. Miller said, and on
page 9 they are advised to avoid coming into contact with brain, spinal
cord, eyes, spleen or lymph nodes of any deer or elk they might kill.
The brochure states that "it is unlikely that chronic wasting disease is
naturally transmissible to humans."

Nevertheless, some Colorado wildlife officials have suggested killing 50
percent of the deer in the north- central part of the state where more
of the deer are infected, Mr. Hale said. "They want to kill them and
start over," he said. But he added that they do not think the disease
threatens hunters. "It would have shown up by now" in people, he said.

On Saturday, Mr. Zunker went elk hunting about 15 miles from his home
not far from the state border, an area where rates of chronic wasting
disease are high.

"I talked to a game warden the other day and he didn't mention anything
to me about a problem in that area," he said. "I don't get overly
concerned. I don't know if Game and Fish have done a good job of
informing the public. But if it's a true threat, how come they haven't
reduced hunting quotas?"

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