- 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
- "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
- 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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