Mad Deer Disease in Wisconsin?

Stop the madness
Malady threatens Wisconsin's elk, deer and, ultimately, people.

By Brian McCombie
Isthmus newspaper Madison, Wisconsin, 7/13/00

Imagine a disease worse than AIDS rippling through Wisconsin's deer herd.
One that's always fatal, cannot be tested for in live animals, and has the
chance of spreading to anyone who eats the infected venison. Sound like the
premise for Michael Crichton's next apocalyptic thriller?

Unfortunately, such a disease already exists in epidemic levels in
the wilds of Colorado and Wyoming. It's infected some game farms, too, and
Wisconsin game farmers have imported more than 350 elk with the potential
for this disease, including elk from farms known to be infected.

"If most people knew what kind of risk this disease poses to
free-ranging deer in the state, they'd be very concerned," says Dr. Sarah
Hurley, Lands Division administrator for the Department of Natural
Resources. The DNR is now testing free-ranging deer around these game farms
for the disease: "We're focusing our energies on those areas where we think
there's the greatest possibility of transmission."

The malady the DNR's looking for is chronic wasting disease
(CWD)--better known, to the extent it is known at all, as mad elk disease.
It's a form of the mad cow disease that devastated Britain's cattle
industry in the 1980s, scared the bejesus out of the populace, and is
believed to have killed at least 70 people to date. An elk or deer with CWD
can be listless, may walk in circles, will lose weight and interact
progressively less with fellow animals.

The corresponding human affliction is called Creutzfeldt-Jakob
disease (pronounced Croytz-feld Yawkob) or CJD. People with CJD experience
symptoms similar to Alzheimer's, including memory loss and depression,
followed by rapidly progressive dementia and death, usually within one
year. While CJD is rare (literally one in a million odds of getting it),
over the last few years at least three deer hunters have died of it. There
is no proof either way whether they contracted the disease from
CWD-infected venison, but new research says it is possible.

All three varieties--mad cow, mad elk and CJD--belong to a family
of diseases called transmissible spongiform encephalopathy. These diseases
alter the conformation of proteins in the brain called prions; after-death
brain samples usually show a series of microscopic holes in and around
brain cells.

No one is exactly sure how mad elk disease spreads. At first,
transmittal through blood seemed likely, as from mother to fawn. But CWD
has moved between adult animals at game farms, leading scientists to
conclude that it can be spread through saliva or simple contact. Also, the
rates of transmission are higher in areas where animals have the most
opportunities for contact. Wisconsin's concentrated population of 1.7
million deer interact freely with each other, and scientific modeling
suggests CWD could tear through our deer herd devastatingly fast.

Despite the danger, Wisconsin and other states are relying on only
sporadic testing and a system of voluntary compliance. It's a system that
some say has more holes in it than a CWD-infected brain.

At present, Wisconsin game farm owners-even those harboring elk and
deer brought in from farms with known cases of CWD-do not have to call a
veterinarian if a deer or elk suddenly dies or acts strange. They're also
not required to inform the state Department of Natural Resources (DNR) or
the Department of Agriculture, Trade and Consumer Protection (DATCP) if
animals escape into the wild.

"The lax attitude is pretty shocking," says John Stauber, a Madison
activist and co-author of Mad Cow U.S.A. To protect people and deer,
Stauber argues for an immediate importation ban for game farms, plus
programs of testing and surveillance. He suggests both DATCP and DNR aren't
taking such measures because, as the regulators in charge, they don't want
to find the CWD he thinks is likely already in state.

"It's in their bureaucratic interest to not [actively] look for CWD
in the game farms," says Stauber. "Because if they find it, who's to
blame?"

In the wild and especially out west, chronic wasting disease is
spreading fast. Northeastern Colorado documented its first case in 1981. By
the mid-1990s, samplings of mule deer brains showed 3% to 4% testing
positive for CWD. Within a few years, the rate was 8%, and now Larimer
County, the center of the endemic area, has a 15% rate of infection among
mule deer. It's also being found in deer and elk in Wyoming.

"Fifteen percent of a wild population of animals with this disease
is staggering," says Dr. Thomas Pringle, who tracks CWD-type diseases for
the Sperling Biomedical Foundation in Eugene, Ore. "It's basically unheard
of." Moreover, he adds, "this is an unusually virulent strain... with
highly efficient transmission mechanisms.

CWD could eventually spread to Wisconsin on its own, animal to
animal. But that would take decades. Game farms, though, provide a
mechanism to cut through all that time and distance and drop CWD smack in
the middle of the state.

An open-records search by Isthmus reveals that the first shipment
of farm elk from areas with CWD in the wild occurred in 1992, with 66
Colorado elk going to a game farm in Plymouth. In April 1998, DATCP was
informed that a Bloomer game farm had purchased one elk from a Nebraska
farm later found to be CWD-infected. This prompted a Sept. 15, 1998, memo
from Steven Miller, head of the DNR's Lands Division, to Secretary George
Meyer, with copies to DATCP chief Ben Brancel and Gov. Tommy Thompson. In
it, Miller recommends that Wisconsin follow the lead of Montana (which
found CWD on two game farms) and place "a moratorium on the importation of
all game farm animals.... At present it appears the only way to help assure
the disease does not spread into Wisconsin."

But the moratorium was never put in place, so it's possible that
even more elk potentially carrying CWD are now in state.

Instead of a moratorium, Wisconsin has opted for testing. It is
among 12 states and two Canadian provinces that currently test deer for
CWD. Last year, the Wisconsin DNR began testing road- and hunter-killed
deer in 1999 within a five-mile radius of game farms that have brought in
elk from CWD-infected areas. Test areas include all or part of Fond du Lac,
Dodge, Jefferson, Sheboygan and Washington counties. All of the
approximately 250 brains examined in 1999 came back negative; this year,
500 to 600 deer will be tested.

Meanwhile, DATCP is asking owners of game farms that have animals
from herds known to have cases of CWD infection to voluntarily enter a
surveillance program. The agency's top veterinarian, Dr. Clarence Siroky,
argues that voluntary compliance makes more sense than a moratorium
because, ban or no ban, game farm operators "are going to find a way to
bring these animals into the state. We don't have police patrols and
impregnable borders to keep anything in or out."

With voluntary compliance, Siroky says, at least there are records
of animals entering the state. So if CWD or other diseases are discovered,
the animals can be traced back to their original herds and other farms they
may have been at. "It's better to know where the animals are coming in
from," he insists.

Siroky may be right that an importation ban would result in some
game farms smuggling in animals. But currently, game farmers can bring in
any deer or elk, even those from known CWD-infected areas, so long as they
can produce a health certificate showing the animal's been tested. The
problem is that no test exists to find CWD in live animals. Animals can
carry CWD for years and still look healthy, so some of the 370 elk shipped
into Wisconsin between 1996 and 1999 from CWD areas could have the disease.
The odds are even higher for animals purchased from farms later found to
have CWD.

Wisconsin has approximately 100 deer or elk farms and they're big
business. On the Internet, prices for elk calves start at $1,500, and
breeding bulls go for up to $20,000. Some farms sell venison and the velvet
that peels from new elk antlers (considered an aphrodisiac in Asia). Others
offer "hunts" costing between $1,000 and $5,000 for trophy deer, to more
than $10,000 for bull elk with massive antlers.

Given these economics, it's reasonable to question why anyone with
a suspicion of CWD in his or her herd would call in state regulators or a
vet. A farm with a proven CWD case, confirms Dr. Robert Ehlenfeldt, DATCP's
director of Animal Disease Control, would be shut down indefinitely.

And if a problem develops on a Wisconsin game farm, there's no
guarantee that's where it will stay. Dr. Hurley says even fenced-in animals
have easy nose-to-nose contact with wild and other farmed animals. Besides,
as the DNR's chief of special operations Thomas Solin has documented, many
game farms are not secure. Gates are sometimes left open. Fences rust and
break, rot and topple, get crushed by fallen trees. Even if game farm
animals don't escape, such breaches allow wild deer to get in, mingle with
the farmed deer and elk, then leave.

Unlike other diseases, there's no test for CWD in living animals
because it doesn't create an immune system counter-response, detectable
through blood analysis. You can't kill CWD and related diseases by cooking
the meat. One test Stauber recounts in Mad Cow U.S.A. found that scrapie, a
sheep form of CWD, stayed viable after a full hour at 680 degrees
Fahrenheit. Most disinfectants don't kill these diseases, either, and they
can exist in the soil for years.

And while diseases like mad cow and mad elk do have some trouble
jumping from species to species, it can happen.

This May, Byron Caughey of the National Institutes of Health
announced that he had converted human brain materials with
mad-elk-contaminated brain matter at rates roughly equal to the transfer
between mad cow and humans. Says Dr. Pringle, referring to Caughey's work,
"CWD may not transmit that easily, but the rate isn't zero." Pringle notes
that the test Caughey used has been a very reliable proxy to determine
transmission possibilities for other diseases, including mad cow.

Once they jump the species barrier, transmissible spongiform
encephalopathy diseases mutate to fit the new host and are then passed on
rather easily within that species. Unfortunately, says Pringle, no one is
trying to determine if CWD has jumped into people as Creutzfeldt-Jakob
disease. Making matters more difficult is the fact that the disease can
incubate for decades before symptoms are seen.

In states with CWD-infected deer, thousands of people have
undoubtedly been exposed to CWD-infected venison. A February 1998 Denver
Post article tells of one hunter who's venison tested positive for CWD. By
the time he was notified, his meat had already been ground up and mixed
with meat from hundreds of other deer for venison sausage.

With AIDS, Pringle notes, there was a definite overreaction, with
people initially afraid to even shake hands with people infected with the
virus. Looking at the CWD situation in Colorado, he says there's been
complete underreaction.

"It's like, 'Oh, what the hell. Nobody's died yet--so keep eating
the venison!'" Pringle worries that if the disease is found in humans, it
will after years of spreading through the human community.

Looking over documents obtained by Isthmus through its open-records
request, Stauber says DATCP is behaving more like a lobbyist for the game
farm industry than an agency bent on protecting Wisconsin's people from
CWD. He points to DATCP's Cervidae Advisory Committee as a prime example.

In a Nov. 11, 1998, memo from Siroky to DATCP secretary Ben
Brancel, Siroky notes that the committee is needed to "obtain information
from the public concerning disease regulation" of farmed deer and elk, and
"to help formulate action plans for importation requirements, prevention
and control" of CWD. But of the 12 people Siroky nominates, one's a DNR
warden, one's a DATCP employee, and the other 10 are game farm owners. And
two of these owners were among those DATCP knew had purchased elk from
farms at high risk of having CWD.

"There's no significant input from anyone else," says Stauber.
"Farmers, deer hunters and consumers are all left out. Meanwhile, the
government's failing to take all necessary precautions to alert the public
to this potential health threat."

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