# Who is to blame for Mad Deer?



## Tom Morang (Aug 14, 2001)

August 2002

Who is to Blame for Mad Deer?

http://www.progressive.org/August 2002/mcco0802.html

by Brian McCombie


The helicopter rises up over the ridge line, the noise of the rotors scattering the targets below. But the snipers in the doorway already have their scoped, high-powered rifles locked in, and the bullets fly until the targets pitch forward, kicking and writhing in their death throes.

The latest battlefield description from Afghanistan? No. It's the next battlefield from the rolling, wooded hills near Madison, Wisconsin. The snipers are employees of the Wisconsin Department of Natural Resources. The targets? White-tailed deer, potential carriers of a deadly disease that may also infect people. It's called Chronic Wasting Disease (CWD), and it's steadily spreading across North America.

"CWD clearly originated in northeastern Colorado and now has ended up spreading far and wide into many states and two Canadian provinces," writes John Stauber, a Madison, Wisconsin, activist and co-author of Mad Cow U.S.A. (Common Courage, 1997), which examines England's Mad Cow nightmare and whether it could happen here.

The disease, he claims, is traveling faster and more effectively than nature could ever accomplish. He suspects this is due to the interstate transportation of game farm animals. And he blames the expansion of the disease on the game farm industry and state agricultural agencies that act more as game farm patrons than as regulators.

The outbreak is causing near hysteria in rural Wisconsin. The state plans to kill as many as 50,000 deer in the south-central part of the state, and deer hunters everywhere are left to wonder whether their venison is safe to eat. Research and anecdotal evidence suggests it is not. And that's scary news for the fourteen million deer hunters around the country.

Both Mad Cow and Chronic Wasting Disease are kinds of Transmissible Spongiform Encephalopathy (TSE). These diseases aren't viral or bacterial, yet somehow they transform or "fold" proteins in brain cells called prions. When enough infected prions deposit themselves in the brain, microscopic ruptures form in the brain cells. Prior to death, behavioral changes become apparent.

As the disease progresses, infected cattle become very agitated, kicking violently with no provocation. They also have trouble eating and swallowing, and usually lose weight. Similarly, deer with Chronic Wasting Disease stop eating. Their resulting emaciated state gives the disease its name. They also shy away from fellow animals, begin to slobber uncontrollably, and walk in circles.

As with all TSEs, Chronic Wasting Disease has no cure and is always fatal. The only way to test for it in elk and cattle is to kill them and examine brain samples under a microscope. A live test for deer was recently developed using a tonsil biopsy, but it's not yet clear how accurate this is.

The human version of TSE is called Creutzfeldt-Jakob Disease (pronounced Croytz-feld Yawkob). People with Creutzfeldt-Jakob Disease experience symptoms similar to Alzheimer's, including memory loss and depression, followed by rapidly progressive dementia and death usually within a year. While Creutzfeldt-Jakob Disease is rare (humans literally have a one-in-a-million chance of getting it), over the last few years three young deer hunters (from Utah, Oklahoma, and Maine) died of the illness.

Those deaths sparked an investigation by the Centers for Disease Control and Prevention, largely because the three hunters were younger than thirty, which is extremely rare for Creutzfeldt-Jakob Disease (sixty-eight is the median age for deaths resulting from the illness). While it found no connection to Chronic Wasting Disease-infected venison, the Centers for Disease Control and Prevention also had no way to test deer these hunters had already consumed. The agency did kill and test some deer where the victims of the disease had hunted. All the animals tested negative. There was evidence, though, that all the hunters were exposed to elk from Colorado or Wyoming, possibly from areas where Chronic Wasting Disease is prevalent. However, it was impossible for center investigators to know if those particular elk were infected.

Dr. Thomas Pringle thinks it's very likely that Chronic Wasting Disease can harm people. A molecular biologist who for five years covered TSE diseases for Sperling Biomedical Foundation in Oregon, Pringle notes that game agencies in Colorado and Wyoming have spent the last two decades assuring hunters there was no scientific proof that anyone had ever died from eating Chronic Wasting Disease-tainted venison. Yet, Pringle says, the research on Chronic Wasting Disease's potential human health risks is virtually nonexistent. He contends these agencies took their position to protect a multibillion dollar industry that revolves around deer and elk hunting.

The research that does exist isn't encouraging. In September 2000, the European Molecular Biology Organization published a study that found that deer prion materials infected with Chronic Wasting Disease converted human prion materials in test tubes at very low rates. "Chronic Wasting Disease and [Mad Cow conversions happened] at about the same rate, in this proxy test, for humans," Pringle observes, and says similar tests alerted British scientists that Mad Cow beef could potentially infect people. To date, more than 100 people have died from a Mad Cow-derived form of Creutzfeldt-Jakob Disease.

In early April 2002, Byron Caughey, who directed the European Molecular Biology Organization research, told a Wisconsin newspaper that while the risk of people contracting infection from a Chronic Wasting Disease deer is probably low, "it's not a risk I'd want to take." The head of the Wisconsin Department of Natural Resources, Darrell Bazzell, publicly admitted his agency couldn't guarantee that meat from deer infected with Chronic Wasting Disease was 100 percent safe to eat, leading one Milwaukee food bank to stop accepting venison.

The epicenter of Chronic Wasting Disease is the Foothills Wildlife Research Facility in Fort Collins, Colorado, operated by the state's Department of Wildlife. In the mid-1960s, the Department of Wildlife ran a series of nutritional studies on wild deer and elk, releasing them when various projects were completed. Soon after the studies began, however, Foothills deer and elk began dying from a mysterious disease. It was not identified as Chronic Wasting Disease until 1980.

The Foothills facility also held a number of sheep with scrapie, the sheep form of TSE, which has existed in North America since 1947, and which Pringle thinks was transferred into the deer and elk from contact with the sheep. He believes Chronic Wasting Disease "must be an extremely virulent strain" to jump the species barrier.

"That's the theory," says Michael Miller, a veterinarian and Chronic Wasting Disease expert at the Foothills facility. Yet he also says it's possible the disease existed naturally in wild deer and elk, and infected animals were brought into Foothills for nutritional studies and began spreading the illness among the closely confined animals.

In 1981, the first wild animal (an elk) with Chronic Wasting Disease was found in Larimer County, Colorado, near the Foothills facility, and the disease moved out into northeastern Colorado and southeastern Wyoming. Today, the disease is found in more than 15,000 square miles of Colorado alone. However, testing by the Colorado Department of Wildlife in the 1980s found Chronic Wasting Disease at under 1 percent in elk and 2 percent or less for deer. But the rate of infection picked up speed in the mid-1990s. Pockets in Colorado today have deer at 7 to 8 percent infection rates, while 15 percent of the deer in Larimer County have tested positive for Chronic Wasting Disease.

In 1996, an elk at a Saskatchewan game farm was found to have the disease. By 2001, the province had twenty-nine game farms under quarantine, and eventually nearly 8,000 elk were slaughtered, with more than 100 testing positive for Chronic Wasting Disease.

"We traced back all the Chronic Wasting Disease exposures to a single elk from South Dakota," says Dr. George Luterbach, chief veterinarian for the Canadian Food Inspection Agency. That elk arrived in the province in 1989 and died in 1990. Chronic Wasting Disease was eventually found on the South Dakota farm, and Luterbach thinks an animal from there infected the Saskatchewan game farm, which then bought and sold elk, seeding the disease into other operations. Citing Canada's privacy act, Luterbach won't release the name of the South Dakota farm.

The year 2000 also saw Saskatchewan record its first wild deer with Chronic Wasting Disease, followed the next year by two more. Darrel Rowledge, director of the Alliance for Public Wildlife, a conservation group based in Calgary, says, given that Chronic Wasting Disease is virtually indestructible (disinfectants and ultra-high temperatures don't prevent transmission) and always fatal, historical and scientific records should reveal its presence in North America before the 1960s. They don't, so Rowledge, like Stauber, blames game farms for transporting the disease. "Scientists knew that privatization, domestication, and commercialization of wildlife was going to cause horrendous disease problems," he says. But in many state legislatures and agricultural agencies, "There was this presumption that [game farmers] should be allowed to exist until it was proven that they were doing something wrong."

Chronic Wasting Disease was also discovered on game farms in Alberta, Colorado, Kansas, Montana, Nebraska, Oklahoma, and South Dakota from 1997 to 2001. By the time Wisconsin announced its problem, Nebraska and South Dakota had infected wild deer, too.

But Wisconsin is arguably in the most dire straits. Elk appear the least susceptible to Chronic Wasting Disease, with mule deer (a western cousin of white-tails) next in line. All the evidence suggests that white-tailed deer most easily contract and spread the illness. The exact route of infection between animals isn't known, but Miller says casual contact passes the disease. This could include deer feeding together, touching noses, or stepping in each others' feces and urine.

Most deer in Colorado and Wyoming are mule deer, very thinly dispersed (usually fewer than ten animals per square mile), and much less sociable than white-tails. But Wisconsin has an estimated 1.6 million white-tails, often at seventy or more per square mile, and in frequent contact. Pringle thinks Chronic Wasting Disease could rip through the deer population east of the Mississippi with virtually nothing to stop it.

In February, Wisconsin reported that three deer killed by hunters the previous fall had Chronic Wasting Disease, its first appearance east of the Mississippi River. After further testing found another fifteen deer with Chronic Wasting Disease approximately twenty miles west of Madison, the Department of Natural Resources announced it would try to eradicate all the deer (estimated at more than 25,000) in the 360-square-mile area, figuring fewer deer will slow the spread of the disease. The Department of Natural Resources began giving away free hunting permits this June, vowing a near-continuous hunt in the fall. The state legislature and the governor also gave the agency the legal right to shoot deer from roads and, if necessary, from helicopters.

The Resources Committee of the U.S. House of Representatives held Chronic Wasting Disease hearings in mid-May, and Wisconsin Governor Scott McCallum, who had asked the federal government for $18.5 million to fight the disease, testified that Chronic Wasting Disease could destroy Wisconsin's wildlife and hunting heritage. While Wisconsin Congressmen chimed in supportively, not everyone was a booster.

Representative Jay Inslee, Democrat of Washington, asked McCallum about a 1998 Wisconsin Department of Natural Resources memo on Chronic Wasting Disease-exposed elk coming onto Wisconsin game farms. Why hadn't Wisconsin taken more precautions to keep out the disease? he asked. McCallum insisted state agencies had taken the appropriate steps, but Inslee doesn't buy it.

"There were at least two specific instances where other states had informed Wisconsin that Chronic Wasting Disease-infected [or exposed] herds had sent elk to Wisconsin," Inslee says. "Even in light of this, Wisconsin didn't require mandatory testing and inspection of game farms."

"It's important to note that there's never been a case in Wisconsin of Chronic Wasting Disease in an elk ranch or game farm," says Henry Kriegel of a Montana public relations firm that represents a large game farm association. Wisconsin's discovery of Chronic Wasting Disease in wild deer, he argues, has "become an opportunity for those who oppose game farming to get media attention and create leverage for their position against game farming."

The first part of Kriegel's statement is true. Yet he doesn't reveal the whole picture.

For example, the voluntary monitoring plan had only forty of the state's 272 elk farmers signed up by the summer of 2000, and just eighty by May 2002. Wisconsin's 570 deer farmers ignored the voluntary program almost entirely.

Flaws with no mandatory testing were apparent in October 2001, after Colorado discovered a Chronic Wasting Disease outbreak on a number of game farms. At that point, 450 elk had been shipped to game farms in other states, including nineteen to Wisconsin. The Department of Agriculture, Trade, and Consumer Protection either quarantined or killed and tested these elk, except for two elk which the department wasn't able to locate. They had died before the investigation, and no one is sure where the carcasses are. A third carcass was recovered, but it was so decomposed that a brain sample couldn't be taken.

Game farm regulations concerning Chronic Wasting Disease vary by state, but in the past someone could import nearly any animal as long as it had a health certificate. That process could find detectable diseases like bovine tuberculosis, but did little for the nontestable Chronic Wasting Disease.

Once a state finds Chronic Wasting Disease, though, the whole game changes. South Dakota and Nebraska, for example, now require game farms to import animals only from operations certified as Chronic Wasting Disease-free for at least five years. Wisconsin put such a regulation into effect following its discovery of the outbreak.

Many states recently closed their borders to elk or deer from states with Chronic Wasting Disease. But, as with much of the regulatory framework surrounding game farms, this was done only after years of interstate trade in game farm animals.

The U.S. Department of Agriculture, in September 2001, declared a Chronic Wasting Disease emergency nationwide and announced its intention to wipe out the disease. With agriculture its regulatory focus, though, the department's efforts are concentrated on the game farm industry, not the spread of the disease in the wild. Among its initiatives is to provide indemnity monies (about $3,000 per elk) to game farms found with Chronic Wasting Disease where the standard management procedure is euphemistically called "depopulation." That is, slaughtering all the animals.

The U.S. Department of Agriculture took a more proactive approach this spring, actually buying up the stock of fifteen game farms in Colorado, even though no Chronic Wasting Disease was ever found in these facilities. The department then "depopulated" them to the tune of approximately 1,200 elk.

No word yet if game farms in other places with Chronic Wasting Disease, like Wisconsin, will now be bought up, too, or if the Department of Agriculture will also try to eradicate Chronic Wasting Disease in the wild--or if it can.

In most states, game farms are regulated by agriculture departments, though that wasn't always the case. In Wisconsin, for example, the Department of Natural Resources oversaw game farms until the mid-1990s, when the state legislature and then-Governor Tommy Thompson shifted responsibility to the Department of Agriculture, Trade, and Consumer Protection, a move the game farmers applauded.

Rowledge says these regulatory shifts across the United States weren't accidental. In the 1970s, more and more potential game farmers wanted to set up operations so they could sell elk velvet (the soft material that peels off newly formed antlers, which is marketed as a nutritional supplement and aphrodisiac), host "canned" hunts where animals are shot inside these farms, and market elk meat.

Despite tall fences, game farms have a well-documented history of captive and wild animals intermingling. For state wildlife biologists, the big concern was game farms bringing in diseases. "Whenever you move an animal," Rowledge says, "you're moving all the diseases and parasites the animal has in it and on it. You have no choice."

So state wildlife agencies generally opposed these farms. "When there was resistance," Rowledge says, "the game farmers sought to put themselves under the jurisdiction of bureaucracies that were friendly to their ideas."

Stauber thinks the federal government must step in with an eradication program or Chronic Wasting Disease will expand even further across the continent.

"If I'm right, we've got a hell of a crisis on our hands," he says. "My hope is that growing public outrage over Chronic Wasting may light a fire under the feds to address a problem they've ignored for a decade and a half."


Brian McCombie is a freelance writer based in Marshfield, Wisconsin. He specializes in wildlife and environmental issues.


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## Tom Morang (Aug 14, 2001)

The following is a comment on the article I posted above. The way I see it the anti's are playing the game just as predicted......................tm

Take a look at the article below before reading the attached article. The article below was written in May. The attached article was written in July. The article below names the activists against hunting, meat eating, farming, and the methods used to generate hysteria in the public to further the cause. The attached article has those activists names and are used in the manner predicted in the article below. 


COMMENTARY: Mad deer disease cause for concern -- not crucifixion
by Dan Murphy on 5/31/02 for www.meatingplace.com

I'd like to talk about the threat of bovine spongiform encephalopathy for a moment.

Oh, wait. We don't have BSE in this country, do we?

But to listen to a slew of very vocal "experts," chronic wasting disease -- so-called "mad deer" -- which has affected hundreds of both wild and captive deer and elk, is practically a kissin' cousin to mad cow, making the latter's occurrence as predictable as the arrival of the TV networks' summer re-runs.

So they say.

Like those stale shows themselves, the rhetoric spewed out by this mad cow crew is equally tiresome.

Back in the Nixon era we had the silent majority; I call these people the strident minority.

Only they're even less savory than Tricky Dick.

That's because these self-appointed spokespeople and peddlers of misinformation, whom we'll meet in a moment, aren't working to solve some problem that might require the combined efforts of government and industry. No -- that would be commendable.

Instead, the folks fanning the flames surrounding both mad cow and mad deer disease are hard-core activists who are virulently anti-meat, anti-technology and pro-organic foods. That's their real agenda, and if using the specter of mad cow or mad deer helps fire up the media to start (or should I say, resume) questioning the safety of our food supply, I get the feeling most of these people would consider such tactics about as controversial as putting a collar on a cat.

A quick profile of this incestuous cadre of connivers, in part courtesy of research done by the Center for Consumer Freedom:

* John Stauber, author of the book, "Mad Cow U.S.A.: Could the Nightmare Happen Here?" -- who maintains it already has -- and one of the leading drumbeaters of the mad deer mania. Not a week goes by that Stauber doesn't fire off an e-mail that's practically gleeful over the latest developments related to the mad deer disease problem.
* Ronnie Cummins, head of the Organic Consumers Association, who says he hopes a "crisis of confidence" develops in the United States over mad cow fears, leading to a "new era of sustainable, organic agriculture."
* Howard Lyman, ex-rancher, vegetarian activist and The Humane Society of America front man, who got famous by stating that "mad cow disease would make AIDS look like the common cold" on The Oprah Winfrey Show, later soaking up even more pub during Oprah's Texas trial on charges she deliberately undermined the cattle industry (she was acquitted).
* Andrew Kimbrell, president of the pseudo-scientific Center for Food Safety, made up of mostly organic food activists eager to scare consumers away from buying beef.
* D'Arcy Kemnitz, former staffer with People for the Ethical Treatment of Animals, now a lawyer and lobbyist for the Center for Food Safety and a leader in the mad cow scare campaign to discredit meat production.
* Michael Hansen, a research associate with Consumers Union, who garnered substantial ink and airtime when a wave of mad cow media coverage swept the country in 2000. Hansen was quoted extensively questioning FDA's vigilance in enforcing the ban on mammalian proteins in ruminant feed and condemning USDA's surveillance program to examine brain tissue from suspect cattle for signs of BSE -- of which none have been found.
* Thomas Pringle, a Eugene, Ore-based Ph.D. (in mathematics) whose Web site (www.mad-cow.org) contains such assertions as, "mad deer disease may already be claiming human lives." Pringle touts his connection with the Sperling Biomedical Foundation, whose officers are Pringle, his brother John and Doug Heiken, regional director of the environmental activist Oregon Natural Resources Council.
* David Fenton, head of the public relations firm Fenton Communications, notorious for orchestrating scare campaigns, including alleged threats from mad cow disease, genetically modified foods, pesticides, sugar and caffeine and for promoting sustainable agriculture and animal rights. Fenton also runs the Environmental Media Services, which offers up to reporters a variety of left-wing experts eager to pontificate on the liberal agenda.
Thanks to gullible reporters, many of these crusaders are accorded the credibility of serious scientists. The Center for Consumer Freedom points out that even the New York Times has variously identified Pringle as:

"A biochemist in Eugene, Ore., and independent researcher on transmissible spongiform encephalopathies" (Jan. 14, 2001).
"A biologist in Eugene, Ore., who closely follows [Transmissible Spongiform Encephalopathy] worldwide and independently studies the disease" (Oct. 31, 2000).
"A national expert on mad cow disease" (July 26, 2000).
"A scientific consultant for the Sperling Foundation, a charitable public health organization" (Feb. 23, 1999).

Perhaps more telling are the connections among various "independent" activist groups. For example, check out one interlocking Internet pathway:

Lyman's Web site (www.madcowboy.org) links to the Organic Consumers Association (www.organicconsumers.org), which encourages visitors to buy John Stauber's "Mad Cow U.S.A." book for more information. From there, a link connects to Stauber's Center for Media and Democracy, which is linked to www.mad-cow.org and its proprietor, "biochemist/biologist/national expert/scientific consultant" Thomas Pringle.

Or how about this: The Organic Consumers Association maintains a Web site with links to Public Citizen against food irradiation, Greenpeace against GMOs and the Sierra Club against bovine growth hormone, a cause championed by Dr. Samuel Epstein, the Chicago-based ex-professor and full-time nut job, who authored a stinging article condemning GMO milk, which featured an introduction by those celebrated anti-capitalists Ben and Jerry, who cashed in on a nine-figure buyout deal with Unilever, one of the largest food corporations in the world, which is opposed by a coalition of anti-globalization activists also fighting against Nike, NAFTA, Wal-Mart, Starbucks, Coca-Cola, the World Bank, the World Trade Organization and just about every Fortune 500 firm on Earth and even some that aren't (my favorite "news" headline on the site: "Acquisition of Zacky Farms creates near-monopoly for Foster Farms"), a coalition that is linked to the "9-11 is America's fault for being affluent" crowd, which is linked to the Green Party, whose presidential candidate was Ralph Nader, who founded Public Citizen and we've come full circle.

Keep surfing, and eventually it all ties in with mad deer disease.

In fact, the fine folks listed above are the genesis of the saturation coverage of mad cow by the mainstream media the past three years. Now, they're running with unsubstantiated claims concerning the alleged connection between mad cow and chronic wasting disease in deer and elk, to the point of attempting to connect mad deer with the 1999 deaths of two huunters, one each in Utah and Oklahoma, who died from CJD, the rare, classical form that strikes less than one in a million people. The Centers for Disease Control concluded that the men's deaths were not mad deer-related.

As opposed to what you'd read in, say, The New York Times, here are the facts:
* There is no connection between chronic wasting disease in wildlife and the occurrence of Creutzfeldt-Jakob disease in humans. Dr. Paul Brown, chairman of an FDA advisory panel on CWD, said that "to date, there's no identified instance of disease in human beings attributed to chronic wasting disease, either through contact [with sick animals] or through consumption."
* There has never been a mad cow case in the United States, and USDA has examined more than 12,000 brains from suspect cattle (either downers or animals with neurological symptoms), without finding any evidence of BSE, according to Dr. Linda Detwiler, USDA senior staff veterinarian.
* Classical CJD is not caused by eating beef from mad cows. "To call Creutzfeldt-Jakob disease the human form of mad cow disease is incorrect, or at best, misleading," says Dr. Roger Brumback, a pathology professor at the University of Oklahoma. "Most people get CJD because they're born with a genetic defect or as a result of a spontaneous genetic mutation."
The bottom line is that the link between CJD and CWD remains unclear. What's really going on is an attack on modern agri-business, big corporations and biotechnology, which of course have collectively ruined the planet and we'd all be way better off if we could just turn back the clock to the glorious 19th century.

Minus the slavery and the oppression of women and the rise of robber barons and the legacy of colonialism and that nasty business of people dying in droves from typhoid and malaria and smallpox and similar stuff, all of which tended to put a bit of a damper on life back then.

So what does it all mean? Although the science surrounding BSE and CJD and CWD is less-than conclusive, one fact has become crystal clear:

Tomorrow, and every other day as well, the strident minority will be toiling away, trying to foster fears of a British-style epidemic of mad cow/mad deer any way they can, then capitalize on the resulting confusion and controversy.

And that's a sickness that rivals the very disease about which these hustlers claim to be so concerned.

(Be sure to logon to www.meatingplace.com every Friday morning for another Commentary from Dan Murphy, Editor of MMT magazine).


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## Mad (Jul 13, 2002)

While at the www.meatingplace.com (aka Marketing & Technology Group, Inc.) be sure to check the contacts & link pages to see who they represent. Be sure to register (It's Free!) & select the industry you represent. Whose best interests are they looking out for?

As far as I know the people they criticize in their article are non-profits. John Stauber's book Mad Cow USA is a free PDF download from his website - www.prwatch.org 

As far as "Oh, wait. We don't have BSE in this country, do we?" Richard Marsh published in
The Journal of General Virology back in 1991 states - "Epidemiological investigation of a new incident of transmissible mink encephalopathy (TME) in Stetsonville, Wisconsin, U.S.A. in 1985 revealed that the mink
rancher had never fed sheep products to his mink but did feed them large amounts of products from fallen or sick dairy cattle."

maddeer.org


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## rb1 (Jun 24, 2002)

Thought I would add this. RB1

Posts: 540
Did wild game feasts lead to fatal brain disorders?
Families left to ponder connection after deaths of three participants 

By JOHN FAUBER and MARK JOHNSON 
of the Journal Sentinel staff 

The wild game feasts were a fall ritual that drew outdoorsmen to the Waterhouse family cabin overlooking the Brule River, and filled the cedar-frame retreat with the aromas of partridge, Western elk, moose and Wisconsin white-tailed. 
Now, years later, the legacy of those hearty spreads of the late 1980s and early '90s is a medical mystery linking three of the diners - James Botts, Wayne Waterhouse and Roger Marten. 
One by one, the three have died from rare brain diseases, leaving their families and health officials wondering whether their deaths were an eerie coincidence or evidence that the deer and elk brain disorder known as chronic wasting disease has crossed the threshold from animals to people. 
Either way, their tale is one more warning sign on a cautionary trailcutting through the heart of one of Wisconsin's most popular and revered traditions: deer hunting. 
Waterhouse and Botts both died of Creutzfeldt-Jakob disease, an always-fatal brain ailment that occurs in only onein a million people. Marten was believed to have died of Pick's disease, a somewhat more common neurological disorder that can be diagnosed in error when the true culprit is Creutzfeldt-Jakob. 
Over the years, as many as 100 men may have taken part in the wild game feeds at the Waterhouse cabin. The odds are strongly against two men dying of Creutzfeldt-Jakob, according to Dennis Maki, a professor of medicine and an infectious disease expert at the University of Wisconsin-Madison. Three would increase those odds dramatically. 
"It's very suspicious," he said. 
The families of the three men were devastated and baffled by their deaths - Waterhouse and Marten in 1993 and Botts in 1999 - all before chronic wasting disease was known to exist in Wisconsin's deer herd. 
"Did hunting kill my dad? Did deer kill him?" asked Waterhouse's son, Gary. "If you'd have taken deer hunting away from him, that would have been the end of him. . . . Maybe the deer killed him. I don't know." 
Raising more suspicion, however, is the fact that some of the meat served at the wild game feasts was elk and deer from Western states - including Colorado, where chronic wasting disease has been endemic for decades. 
Presented last week with specifics of the cases, state public health officials expressed concern. 
"We've immediately decided to proceed with an investigation," said Jeffrey Davis, chief medical officer and state epidemiologist for communicable diseases at the Wisconsin Division of Public Health. 
He said the state will request death certificates and clinical and laboratory records for the three men. 
Suspicions rising 
So far, there has not been a documented case of a person contracting chronic wasting disease, but a handful of suspicious cases have surfaced in Wisconsin and around the country, all involving venison eaters who have contracted Creutzfeldt-Jakob disease, a deadly neurological disorder closely related to mad cow disease. 
One of those cases involved D. Kevin Boss, a Minneapolis resident who died of CJD in 1996 at the age of 41. Boss occasionally ate venison from Wisconsin, including deer killed in Barron County, provided by his brother-in-law. Wayne Waterhouse lived in Barron County; Botts lived in Minnesota but grew up in Barron; Marten lived in nearby Buffalo County. 
There have also been several documented cases of Creutzfeldt-Jakob among people who cooked and ate brains from squirrels and wild goats. 
The human version of mad cow disease, known as new variant Creutzfeldt-Jakob, has killed more than 130 people in Europe and is believed to be caused by eating contaminated beef. 
Scientists who watched mad cow disease jump from animals to humans are now deeply concerned that chronic wasting disease will make the same leap - if it hasn't already happened. 
"We're actively looking for human beings who have acquired chronic wasting disease," said G. Richard Olds, chairman and Linda and John Mellowes Professor of Medicine at the Medical College of Wisconsin. 
Today, research on chronic wasting, mad cow and Creutzfeldt-Jakob centers on an unusual infectious agent suspected of causing all three diseases. All three are so-called transmissible spongiform encephalopathies and are believed to be caused by prions, microscopic pathogens that have no DNA and are neither bacteria nor a virus. 
Prions are mutant proteins that get normal proteins to mimic their distorted shape, resulting in a buildup of spongelike holes in the brain. They are particularly adept at infecting nerve cells such as those found in the brain, spinal cord and eyes. 
One reason prions are so feared is that they are highly resistant to heat and other sanitizing methods. It is believed that they can exist in the soil and other locations for years and resurface to infect animals. 
In addition, prions may silently incubate for years or even decades in a person before producing symptoms of disease. 
"These things behave differently from any other infectious agents we've ever dealt with before," Olds said. "They're practically indestructible." 
Dreaded consequences 
If chronic wasting disease prions were going to infect people, the men who gathered for the feasts at the Waterhouse cabin in northwestern Wisconsin were as likely candidates as any. 
Several of the men were prolific hunters, bagging hoofed game in Wisconsin, the Western United States, Mexico and Canada. 
The trophy room in the Chetek home of Gary Waterhouse, Wayne's son, includes a full-size musk ox, polar bear, grizzly bear and bobcat. It also contains two full-size mountain goats and numerous mounted heads of deer, elk and moose. All the trophies were shot by Gary. 
The son inherited his love and dedication to hunting from his father. 
"My dad was an avid hunter, and he was a very good hunter," Gary said. 
Likewise, Marten, a native of nearby Mondovi, traveled North America hunting trophy animals. But he was equally happy to hunt in Wisconsin, even from home. 
"He hunted deer every year from a lawn chair with a six-pack of beer," said his son, Randy. 
Botts, who was raised in Chetek and later moved to the Minneapolis area, was not a hunter but regularly traveled to the Brule River to fish. 
His wife, Judy, said he mentioned being invited to the wild game feasts at the Waterhouse cabin. 
Initially, she said, she made no connection between the banquets and her husband's disease, but then news broke earlier this year about chronic wasting disease in Wisconsin. 
"It just came to me and it came to me very strong," she said. 
It'sa connection state officials dread having to make, given the grave health and economic consequences it could have for Wisconsin. The state Department of Natural Resources has gone so far as to say on its Web site that there is "no scientific evidence that CWD is transmissible through consumption of meat from an infected animal." 
Still, the department is taking unprecedented precautions, telling hunters to wear rubber gloves when field-dressing carcasses, minimize handling of brain and spinal tissues and avoid consuming brain, spinal cord, eyes, spleen, tonsils and lymph nodes. The department is also requesting that hunters process their animals individually without mixing meat from different animals. 
"Health experts advise that no part of any animal with evidence of CWD should be consumed by humans or other animals," the department mentions on its Web site. 
Meanwhile, state health officials are escalating their surveillance of Creutzfeldt-Jakob cases in Wisconsin. 
Earlier this year, the state division of public health urged doctors around the state to report probable cases of CJD, especially in patients under the age of 55. 
None of the three men was younger than 55. Botts was 55when he died; Waterhouse and Marten were 66. 
'It's very intriguing' 
Regardless of their age, UW's Maki said the likelihood of two people who know each other contracting CJD is very low. Three would be even more unlikely and would suggest a common exposure - such as contaminated meat. 
Maki posed these theoretical numbers: 
If each of the three men knew 5,000 people, they would have had a circle of acquaintances totaling 15,000. 
With a known incidence of one in 1 million people, you'd expect to find a CJD case among a group of 15,000 people roughly once every 70 years, he said. Two cases would take 140 years and three cases 210 years. 
One case could just be a sporadic occurrence, he said. With two cases, "You'd have to sit up and prick up your ears," he said. With three cases, "the statistical likelihood is extremely low." 
He said the northern Wisconsin scenario is complicated by one man being diagnosed with Pick's disease. 
Like Creutzfeldt-Jakob, Pick's is a fatal neurological disorder. While prions are suspected as the cause of CJD, the cause of Pick's remains unknown, although prions have not been ruled out as playing a role. 
The Creutzfeldt-Jakob Disease Foundation says that CJD can be mistaken for a variety of neurological disorders, including Alzheimer's and Pick's disease. Some doctors may not even consider CJD as a possible diagnosis since it is considered rare, the foundation says. In addition, the brain biopsy needed to make a definitive CJD diagnosis is invasive, costly and risky. 
Jordan Grafman, chief of the cognitive neuroscience section at the National Institutes of Health in Bethesda, Md., said it would be "very rare" today for a CJD case to be misdiagnosed as Pick's. Medical authorities in the U.S. now are fairly vigilant about looking for the disease because of the mad cow disease outbreak abroad. 
However, such a misdiagnosis would have been somewhat more possible in 1993, he said. And there may be other reasons to question Marten's diagnosis


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## Steve (Jan 15, 2000)

I don't know about anybody else but this is becoming very scary to me. If I get a deer this year, I will be leery of eating it, which in turn will make me leery of shooting one in the first place. I even wonder about my deer from last year. To me Wisconsin is a little too close for comfort and it sounds like CWD may have existed there for years before it was detected. Who's to say it's not in MI already? I don't think there is an affordable test people can run on the deer that they take to make sure it is not infected. It's not cut and dry like Bovine TB and the inspection of the lungs.


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## rb1 (Jun 24, 2002)

Steve it has me scared too. First we got to stop the import of deer going into our state. then we got to keep a close watch on 
the deer close to our borders. check all feed bone meal ect.I think this is the DNR's job and they should get to it instead of talking about. RB1


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## Benelli (Nov 8, 2001)

Steve and rb1...a little of the original subjetc but...

I / we have a little comfort knowing that where I hunt most in 452, its the only place where deer in MI have been tested for CWD (452 samples in 1998, not sure if thats a sufficient sample size, but at least it was good news that no CWD was found). I also have more comfort in knowing that baiting and feeding has been halted up there and the deer numbers reduced to a point where the are becoming more in balance with the habitat (to the dislike of many) and suspected routes of transmission of many diseases have been eliminated / lessened.

I suppose there is inherent risk in eating certain foods, drinking certain beverages, smoking, driving, etc. But these subjects have been studied closely over the years and you kind of know the risks and accept them. I know if I smoke Ill have about a 1 in 70 chance of getting cancer. I know if I eat a pound of bacon a day my heart will likely explode some day, etc. 

What I dont know yet is if I eat meat from a BSE and/or TSE infected animal and get CJD, is related to the meat consumption or other factors?

Ill hunt this year as usual and eat my venison as usual. If I were to hunt the western UP where I hear they have some ridiculously high deer numbers, I think I would pass an a venison dinner until more study was done so I could evaluate the risk with a little more data/education.

Again, It is scary.


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## Fierkej (Dec 21, 2001)

Rb1,

The MI Department of Agriculture, on April 26, 2002, did implement a one-year ban on the importation of all deer and elk. Nearly half of the states in the nation have implemented similar bans. The Dept. of Ag. requires mandatory CWD surveillance for captive cervids herds meaning that all death losses of cervids over 16 months of age must be submitted for testing. The DNR this year is increasing CWD surveillance, planning to test at least 2,000 free-ranging white-tailed deer and 50 free-ranging elk for CWD, testing approximately 50 deer from 40 counties. These counties were chosen based on the number of captive cervids and deer research facilities in the county. The counties in the western upper penninsula that border Wisconsin will also be tested this year. This surveillance is to take place for three years, and during that time all counties in the state will be tested.


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