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Did wild game feasts lead to fatal brain disorders?

July 21, 2002 Milwaukee Journal Sentinel by John Fauber and Mark Johnson
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 deer.

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 trail cutting 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 one in 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's a 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 55 when 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.

It is "highly unusual" for a Pick's patient to die within a year of the onset of symptoms, Grafman said. The vast majority of patients die within two to 10 years.

Marten died slightly less than a year after he first displayed symptoms, according to his son, Randy.

It's also unusual for someone to get Pick's after age 60. Marten died at age 66.

More important, the gold standard for diagnosing Pick's disease is to do an autopsy and a molecular analysis of brain tissue.

According to a certificate of death obtained from the state of Nevada, where Marten died after being taken to a clinic in Reno for treatment, no autopsy was performed.

UW's Maki said if Marten actually had CJD and was misdiagnosed, the possibility of chronic wasting disease having infected the three men becomes even more likely.

But even if the case was accurately diagnosed as Pick's, "It's very intriguing," he said. "That's all we can say at this time."

Body of research

Maki said he believes that if chronic wasting disease spreads among Wisconsin's deer herd, it's only a matter of time before the disease spreads to people.

"We eventually will see cases (in humans)," he said.

However, he predicted the number of cases is likely to remain small and the risk low.

Two years ago, federal government researchers at the Rocky Mountain Laboratories in Hamilton, Mont., analyzed prions from deer infected with chronic wasting disease. The analysis showed a substantial molecular barrier that helped prevent chronic wasting prions from converting normal human protein to the mutant form. Despite the barrier, deer prions ultimately were able to convert human protein to the mutant form. They did so with about the same level of efficiency as mad cow prions.

Maki and others point to a growing body of evidence linking the consumption of deer or other wild game with Creutzfeldt-Jakob.

-- In 1984, neurologists at Baylor College of Medicine investigated four suspicious deaths involving unrelated patients, all with a history of eating the brains of wild animals. All the patients eventually died, and Creutzfeldt-Jakob was the suspected cause.

"Our patients ate many brains from many wild animals over the course of many years and therefore could have come in contact with infected material," the researchers wrote.

-- In 1986, a study involving 26 CJD patients by researchers at Temple University and the National Institutes of Health found that exposure to deer through a hobby such as hunting resulted in up to a ninefold increased risk for CJD.

-- A 1997 study in the journal Lancet linked five unrelated CJD patients from different towns in rural Kentucky to a history of eating squirrel brains.

"Culinary preparations include scrambling the brains with eggs or putting them in a meat and vegetable stew referred to as 'burgoo,' " the researchers wrote.

-- In October 2001, a study in the journal Archives of Neurology reported on the cases of three people who contracted CJD and who had been deer hunters or had regularly eaten venison.

The three individuals, who were from Maine, Oklahoma and Utah, all were under the age of 30 and came down with the disease between 1997 and 2000. The vast majority of CJD patients get the disease in their 60s and 70s.

The researchers, some from the U.S. Centers for Disease Control and Prevention, concluded that although circumstances suggested a connection with chronic wasting disease, they could find no "causal link."

Now, the cases of Botts, Marten and Waterhouse raise some of the same questions.

Fred Bannister, a small-town doctor who knew all three men and took part in the wild game feeds, said a thorough investigation of the case is needed.

"I'm just a little guy out here in the woods who has suspicions," said the semiretired Chetek family physician. "There is something unusual."

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