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Subject: Spreading 'Mad Deer' Plague Leaves U.S. Scientists Baffled ANTONIO REGALADO / Wall Street Journal 24may02
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<H2 style=3D"TEXT-INDENT: 0px; LINE-HEIGHT: 115%" =
align=3Dcenter>Spreading 'Mad=20
Deer' Plague Leaves U.S. Scientists Baffled </H2>
<H4 style=3D"TEXT-INDENT: 0px; LINE-HEIGHT: 115%" align=3Dcenter>ANTONIO =
REGALADO /=20
Wall Street Journal 24may02</H4>
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  <TBODY>
  <TR>
    <TD width=3D24 rowSpan=3D3></TD>
    <TD width=3D24 bgColor=3D#bdaa72>&nbsp;</TD>
    <TD><FONT size=3D2>&nbsp; States with CWD in captive animal=20
      populations</FONT></TD></TR>
  <TR>
    <TD width=3D24 bgColor=3D#009cbb>&nbsp;</TD>
    <TD><FONT size=3D2>&nbsp; Areas with CWD-infected wild=20
  populations</FONT></TD></TR>
  <TR>
    <TD colSpan=3D2><FONT size=3D2><I>Source: Wisconsin DNR</I>.</FONT>=20
      <P><IMG height=3D263=20
      =
src=3D"http://www.mindfully.org/Heritage/Mad-Deer-Leaves-US-WSJ24may02.GI=
F"=20
      width=3D243 border=3D0></P></TD></TR></TBODY></TABLE>
<P>FORT COLLINS, Colo. -- There's trouble on the other side of the =
chain-link=20
fences surrounding the state government's Foothills Wildlife Research =
Facility.=20
The dusty pastures are ground zero for a deer-killing plague that is=20
frighteningly similar to mad-cow disease.</P>
<P>It was here that the condition, known as chronic wasting disease, or =
CWD, was=20
first identified decades ago, and where animal-management practices may =
have=20
fueled an epidemic. Now, scientists are conducting research in the pens =
and=20
laboratories here in their search for ways to stop it.</P>
<P>They are racing against a disease that has rapidly spread across =
eight states=20
and parts of Canada. The U.S. Department of Agriculture has called CWD =
an=20
"emergency." In Colorado, sharpshooters, some working from helicopters, =
have=20
slaughtered 10,000 deer in the past year trying to contain its spread.=20
Identified first only in captive animals, it has leapt the fences, =
infecting a=20
small but growing number of the nation's more than 20 million =
free-ranging=20
deer.</P>
<P>Fears that CWD could devastate regional hunting and tourist economies =
have=20
sent states scurrying to Washington for financial aid. Last week, in=20
congressional hearings on the outbreak, Wisconsin Gov. Scott McCallum =
pleaded=20
for financial assistance and declared that CWD "is threatening our way =
of=20
life."</P>
<P>Brain tests following last fall's hunting season in Wisconsin =
suggested that=20
about 3% of white-tail deer are infected in a large region straddling =
two=20
counties. Those findings have generated shock waves in a state where =
hunters=20
killed 446,000 deer last year, generating more than $1 billion in =
economic=20
activity and countless venison dinners.</P>
<P>Much about the illness remains a mystery. A little-understood protein =
known=20
as a "prion," a form of which is behind mad-cow disease, causes chronic =
wasting=20
disease. In ways scientists don't yet fully grasp, prions enter the =
brain and=20
set off a chain reaction, causing some of the brain's own proteins to =
assume an=20
aberrant form. In humans, such rogue prion proteins are blamed for a =
rare,=20
naturally occurring human illness called Creutzfeldt-Jakob disease, and =
a=20
related disease linked to eating contaminated beef from "mad cows."</P>
<P>There's no evidence that meat from deer or elk has infected humans. =
But=20
alarms have been raised recently over five unusual cases of =
brain-wasting=20
disease in young people living in the U.S., and researchers are probing =
the=20
cases for possible links.</P>
<P>One involves Doug McEwen, a 30-year-old hunter from Kaysville, Utah, =
who died=20
in 1999 of Creutzfeldt-Jakob disease. The condition is extremely rare, =
and, when=20
it occurs naturally, almost always strikes late in life. An early onset =
has been=20
one of the hallmarks of the disease linked to cattle. The McEwen case =
caught the=20
attention of activist groups that were lobbying for stricter =
surveillance in the=20
U.S. of mad-cow disease. The Center for Food Safety, a consumer watchdog =
group=20
in Washington, called Mr. McEwen's case the first possible case of "mad =
deer"=20
disease.</P>
<P>Researchers haven't been able to document a link. The Centers for =
Disease=20
Control and Prevention in Atlanta studied Mr. McEwen's case and those of =
two=20
other people 30 years old or younger who died of Creutzfeldt-Jakob =
disease=20
between 1997 and 2000. An analysis of their brain tissue ruled out =
mad-cow=20
disease as the cause. But two were hunters and one was the daughter of a =
hunter,=20
and all had regularly consumed elk or deer meat. Intrigued by the =
possibility=20
that wild game caused their disease, epidemiologists quizzed family =
members=20
about the victims' lifestyles and eating habits. But researchers said =
they found=20
"no strong evidence for a causal link" to CWD.</P>
<P>Mysterious Moves</P>
<P>Meanwhile, scientists in Fort Collins and elsewhere are conducting =
federally=20
funded tests to figure out how the animals are becoming infected. In =
mad-cow,=20
the disease source was traced to cattle feed containing bone meal from=20
contaminated livestock. But no one knows how CWD moves from animal to =
animal.=20
One thing is clear: The infectious agents are hard to kill off. The =
grounds at=20
the 35-acre Foothills facility have been dug up, disinfected and even=20
temporarily closed. Still, when the animals return, they invariably lose =
weight,=20
salivate copiously, behave oddly, and die.</P>
<P>"We know very, very little. We don't understand the transmission, we =
don't=20
understand the origin, we don't understand any of this," says Stanley =
Prusiner,=20
the University of California, San Francisco neurologist who won a Nobel =
Prize=20
for developing the prion hypothesis.</P>
<P>Chronic wasting disease has been around for at least 35 years, but =
until the=20
mad-cow outbreak, it was of interest to only a handful of wildlife =
biologists.=20
Elizabeth Williams was one of them. Just out of veterinary school in =
1977, she=20
learned about the trouble researchers had been having since the mid-60s =
at the=20
animal research pens on the edge of Fort Collins. The deer there were =
doing=20
poorly in captivity, mysteriously losing weight and dying. Biologists =
suspected=20
a nutritional problem, or perhaps poisoning, and termed the syndrome =
"chronic=20
wasting."</P>
<P>Dr. Williams soon found the problem. Peering at thin slices of the =
animals'=20
brains through a microscope, she recalls seeing "a lot of lesions." The=20
sponge-like patterns of decay she found reminded her of a video she'd =
seen as a=20
student about the human cannibals of Papua New Guinea. An adventurous =
scientist=20
named D. Carleton Gajdusek had won a Nobel Prize for showing that the =
ritual=20
acts of eating human brains were causing a rare condition, called =
spongiform=20
encephalopathy, that was killing the islanders.</P>
<P>The deer were dying of something similar. Dr. William's =
classification of CWD=20
as a spongiform disease in 1977 was a career-making finding, although =
CWD=20
remained an obscure wildlife problem.</P>
<P>An early sign that CWD was infectious came two years later, when=20
veterinarians detected a case at another research facility in Sybille =
Canyon,=20
Wyo., about 120 miles northwest of Fort Collins. That site, a jumble of =
open=20
paddocks on a steep hillside, sometimes exchanged animals with the Fort =
Collins=20
pens.</P>
<P>Two years after the Sybille case turned up, biologists diagnosed the =
first=20
case in a wild animal -- a sick elk found in Rocky Mountain National =
Park. It=20
may never be known for sure if the disease actually started in the Fort =
Collins=20
pens, or was simply first identified there. "The pens are like =
microscopes for=20
mule deer," says N.T. Hobbs, an ecologist at the Colorado State =
University, who=20
now leads a major study of CWD funded by the federal government. "Just =
because=20
you see something, that doesn't mean you created it."</P>
<P>The Foothills Wildlife Research Facility, Colorado's main center for =
studying=20
diseases of wild animals, is surrounded by a double ring of =
eight-foot-high=20
fencing, and none of the animals now being studied there is ever allowed =
to=20
leave alive. But security wasn't always so tight. For years after Dr. =
Williams's=20
findings about CWD, deer were often brought into the pens to breed, then =

released back into the wild. The pens only had a single fence line then, =
and=20
especially during rutting season wild bucks would come to the perimeter =
to nose=20
with the females inside.</P>
<P>By 1985, however, it became clear that the Fort Collins pens and =
Sybille=20
Canyon had become deadly reservoirs of sickness. Whatever was causing =
CWD was=20
somehow loose in the dirt enclosures. That led to what Dr. Hobbs calls a =

"grisly" clean-up effort at the Foothills facility. The deer, big horn =
sheep and=20
other animals being studied on site were all destroyed, and six inches =
of=20
topsoil hauled away. The out buildings were washed with bleach, then =
left vacant=20
for over a year. But when new deer were brought to the Foothills pens, =
the=20
disease came back too.</P>
<P>Today, both Fort Collins and Sybille Canyon are considered hopelessly =

contaminated. Terry Kreeger, the Wyoming state veterinarian who lives at =
Sybille=20
with his family, says when an elk or deer dies of CWD, he hauls the =
carcass in=20
his pick-up truck to Dr. Williams's laboratory near Laramie. The corpse =
is=20
dissected and incinerated, but few other precautions are taken. Dr. =
Kreeger, for=20
instance, says he washes his truck out with a hose without worrying =
about=20
putting more prions into the environment. "This horse is long out of the =
barn,"=20
he shrugs.</P>
<P>New Way to Spread</P>
<P>Initially, CWD had been spreading slowly, since wildlife in the main=20
infection area of about 20,000 square miles between Fort Collins and =
Laramie=20
were contained by natural barriers, such as the slopes of the Rocky =
Mountains.=20
But CWD's jump from deer into local elk gave the disease an =
unpredictable new=20
way to spread: via man-made transport as part of the trade in elk. In =
1996, CWD=20
turned up on a commercial elk farm on Saskatchewan, Canada. Three years =
later,=20
elk-breeding operations in four U.S. states had found sick animals as =
well.</P>
<P>At the time, commercial elk farming was booming among small ranchers =
looking=20
for new income. Elk require little food or space to thrive, and ranchers =
can=20
make money from their meat and antlers, which are sawed off in the =
spring, then=20
ground and sold as nutritional supplements. So-called velvet antler is =
exported=20
to Asia where it is considered an aphrodisiac, and is also sold in U.S. =
chains=20
such as General Nutrition Centers, where it retails for about $17 an =
ounce.</P>
<P>Since 2000, only 259 farmed elk have died or been diagnosed with CWD, =

according to the North American Elk Breeders Association. This is just a =
small=20
percentage of the 160,000 elk it estimates are in captivity on 2,300 =
U.S. and=20
Canadian elk ranches. But the disease has an incubation period of two to =
three=20
years. Since 1998, elk farmers have destroyed 4,432 elk known to be =
exposed to=20
the sickness.</P>
<P>Some researchers speculate that the elk trade brought the disease to =
wild=20
deer in Wisconsin. Discovery of those infected animals sharply escalated =
CWD=20
concerns nationwide, because the new region of infection is so far from =
where=20
the disease was first identified. But the truth is, nobody knows.</P>
<P>Scientists in Colorado and Wyoming are now urgently trying to =
determine just=20
how CWD is spread, partly funded by a $2.2 million grant from the =
National=20
Science Foundation. At Sybille Canyon, researchers have infected three =
young=20
deer with CWD by feeding them the brains of deer that died of the =
disease.=20
Blood, saliva, feces and urine collected from the animals every six =
months will=20
be injected into the brains of laboratory mice to see if they cause=20
infection.</P>
<P>A separate set of experiments will confirm whether prions can lurk in =
the=20
environment, as the team suspects. At the Fort Collins pens, Michael =
Miller, a=20
Colorado Division of Wildlife biologist, has overseen the construction =
of=20
isolation rooms to test different theories. In one room, two fawns are =
living=20
alongside the decomposed carcass of a CWD deer. Others are being reared =
in rooms=20
that previously housed animals with CWD.</P>
<P>The unknowns worry some consumer advocates. "I think that we have to =
assume=20
the worst of CWD -- that it could be even more dangerous and costly than =
mad cow=20
because of its unique ability to spread through the environment and =
animal to=20
animal," says John Stauber, the author of "Mad Cow U.S.A.," a book =
arguing the=20
U.S. hasn't done enough to keep bovine spongiform encephalopathy, as the =
disease=20
is known formally, out of the country. "With BSE there was a feeding =
loop that=20
could be shut down. Here, it seems to spread like a cold or the =
flu."</P>
<P>Some laboratory studies suggest CWD could theoretically infect =
people. Byron=20
Caughey, a prion researcher at the National Institutes of Health's Rocky =

Mountain Laboratory in Hamilton, Mont., found that CWD prions could =
convert=20
human prion proteins to their deadly form in a lab dish. However, the =
efficiency=20
of such "conversion" was extremely low, evidence of a substantial =
species=20
barrier.</P>
<P>So far, few steps have been taken to reduce people's exposure to CWD =
prions.=20
In Colorado, where hunting and wildlife sightseeing generate nearly as =
much=20
economic activity as skiing, there are no special regulations governing =
how deer=20
carcasses are handled. Hunters in Fort Collins have been asked to sever =
the head=20
of any deer they bag and deposit it in a steel drum outside the Division =
of=20
Wildlife's offices across from the Holiday Inn. A couple of weeks later, =
a state=20
lab reports whether the kill was infected.</P>
<P>Margy Constantino, a Brooklyn native who makes her own bullets in the =

basement of her ranch house near Fort Collins, says some hunters haven't =
waited=20
for test results before eating their kills. Ms. Constantino, who lives =
in an=20
area with rates of CWD as high as 15%, shot a buck through the heart =
last year=20
and waited about three weeks for results.</P>
<P>Though the results came back negative, she'd already had the animal =
butchered=20
in the meantime. Since the local butchers tend to give customers ground =
meat=20
made from pooled scraps, often there's no guarantee a hunter's kill =
won't be=20
mixed with that of an animal that turned up positive for CWD.</P>
<P>"The processor takes scraps and turns it into hamburgers," says Jim =
Widmier,=20
the proprietor of Arrow Dynamics, a shop in Fort Collins that caters to =
bow=20
hunters. "That is a scary situation. The hamburger and sausage you get =
is going=20
to be ground up with other people's."</P>
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