[December 2002]

What state officials aren't telling you about chronic wasting disease -- the politics and blunders behind its spread and the true dangers.

When I spoke to Wisconsin Division of Public Health epidemiologist James Kazmierczak on September 13, he had the cheeriness of a man about to leave for a three-week vacation. A day earlier, his department had dispatched a news release intended to quell the "paranoia" haunting the state since a front-page Milwaukee Journal Sentinel story suggested that the chronic wasting disease in the state deer herd might infect humans. It seemed a bit hasty to publicize part of the Centers for Disease Control and Prevention's yet-to-be-completed investigation, but Kazmierczak, his agency's CWD point man, was eager to allay fears before he left town. In fact, he almost delighted in pointing out that one of three men who, according to the newspaper, shared wild game feasts in Wisconsin's Barron County, had died of a totally unrelated disease.

"You could live on a diet of deer brains and never get sick. There is either no, or very low, potential for infection in humans," he told me. Brains, eyes, spinal cords, spleen, tonsils and lymph nodes are the most infectious parts of a deer with CWD, making Kazmierczak's statement shocking, to say the least. But from the moment Gov. Scott McCallum ordered the state's agriculture, natural resources and health departments to "find a way to work together" to fight CWD, Kazmierczak had a job to do.

Even before the news story, officials feared that the deer herd, growing in some parts of Wisconsin at 50 percent per year, might explode if hunters stayed away. A statewide poll suggested just that in May: Thirty-six percent were considering sitting out the hunt; 42 percent were concerned about eating venison. "I could make a statement on the safety of venison, or [Department of Natural Resources Secretary] Darrell Bazzell could, but we're not health personnel and everything we said would be viewed as, 'Oh, he's just worried about license sales,' " says Tom Hauge, director of DNR's Bureau of Wildlife Management. So that task fell to Kazmierczak, who, though he works in public health, is a veterinarian, not a physician. From the very beginning, says an Ag Department source, "Our greatest fear was that the media would link CWD with mad cow disease in Britain."

Despite the fact that 32 Wisconsin deer had already tested positive for CWD (with 10 more added in mid-October) and that the state was about to launch a Herculean effort to test up to 60,000 more, Kazmierczak was not alone in dismissing the danger. State veterinarian Clarence Siroky, Wisconsin's highest-ranking animal health official, was traveling the state telling hunters, "CWD is like scrapie in sheep [which has never been shown to infect humans]. It's not mad cow disease in deer. There is nothing to fear about CWD, other than its spread within the deer herd." When someone in an audience near Appleton asked Siroky whether he would feed venison to his granddaughter, he answered, "Yes."

Even the chancellor of the University of Wisconsin-Madison lent his voice to the "don't worry, keep hunting" chorus. But while all the reassurances were going on in public, in the back rooms of state government, a very different scenario was playing out. State health and safety experts were trying to force the DNR to make volunteers "wear decontamination outfits, moon suits" when they collected deer brain stem samples. "Can you imagine what kind of message that would send, the panic it would cause around the state?" asks Carl A. Batha, DNR wildlife management supervisor. "The paranoia that's gripped the populace seems to have infiltrated the DNR at the highest levels."

Some DNR officials traced the paranoia all the way to the governor's office. The DNR had spent thousands of hours planning to build a landfill on state property north of Dodgeville to dispose of 25,000 to 30,000 deer carcasses from its CWD eradication zone, a more than 400-square-mile area in southwestern Wisconsin. Engineering work was complete, the heavy equipment about to roll in when the governor ordered the carcasses cremated instead. "We have a $2 billion budget crisis in this state, but we're treating deer carcasses like nuclear waste," DNR Western Area Wildlife Supervisor Tom Howard complained in September. "Cremating a carcass costs $124; landfilling $5." Ultimately, the DNR retained a La Crosse fur company that will skin, shrink-wrap, freeze and store the carcasses, allowing DNR to cremate only deer testing positive for CWD. The whole thing will cost about $2.5 million.

If you ask the country's highest-ranking experts on prion diseases like CWD, mad cow and human Creutzfeldt-Jakob disease (CJD), they will tell you that only incineration, bleach and chemical tissue digestors destroy the infectious agents that cause the diseases and that serious public concern is warranted. "We have repeatedly underestimated these diseases, and we've been wrong. People have died because of it," says Judd Aiken, UW animal health and biomedical sciences researcher. "I'm not an alarmist and I don't like having people mad at me, but it's the truth when I say don't consume venison from an area where CWD exists."

Seeing the course Kazmierczak and Siroky were taking, Dr. G. Richard Olds, chairman of medicine at the Medical College of Wisconsin, asked his Medical Society colleagues last summer, "Are we going to sit here and let the politicians bury this? Or are we going to speak up?" In October, the Medical Society of Milwaukee County's Public Health Committee chose the latter, saying there was a very small but real concern that humans might contract CJD from eating infected venison. "There is no direct evidence to show CWD is like mad cow disease, but it is reasonable to expect it will be," says Olds.

The group urged the state to adopt the steps Britain has taken to stop mad cow disease and immediately ban venison handling by meat processors who also prepare beef, chicken and other meats. The nearly indestructible CWD disease prions, it said, could contaminate equipment and pass into the rest of the food chain. "People have the right to decide for themselves whether they want to eat venison," says Olds. "They shouldn't have to worry that other meat they eat is contaminated."

Meanwhile, worried nurses asked MCW neurologist Piero G. Antuono, who has conducted autopsies on the brains of six CJD victims, whether their husbands should go hunting. "For God's sake," he told them, "don't you dare give venison to your kids... Forty percent of people in this state eat venison, but I would not eat deer meat from anywhere in Wisconsin... It's like not wearing a seatbelt," he says. "The chances are you'll still get home safely, but why would you put yourself at risk?"

A Killer Spreads
When Bill Mytton, DNR's now retired big-game specialist, learned in late February that CWD had crossed the Mississippi for the first time and been discovered in Wisconsin, his first words were, "Oh, shit!" "I can't even describe how I felt," he says. "It was this sickening fear." For two years, the department had been more concerned about TB spreading from lower Michigan, and it had begun testing a few deer killed during the gun-hunting season. DNR added a test for CWD almost as an afterthought. CWD had ravaged free-ranging deer in Colorado and Wyoming for decades but remained there until 1997, when it started spreading through game farms in South Dakota, Nebraska, Colorado, Oklahoma, Montana and Kansas. Infected elk exported from a single South Dakota ranch carried CWD to Saskatchewan, Canada, and Korea, says Mytton. Canada destroyed more than 7,800 elk trying to stop the disease; Korea banned elk farms entirely.

Like elk, deer can carry the disease for years without symptoms, infecting other animals. Until just recently, there was no live-animal CWD test, and by 1999, at least 24 elk from infected herds had been shipped to a handful of Wisconsin game farms. Steven W. Miller, administrator for DNR's Division of Land, so feared CWD that in 1998, he had tried to stop importation of deer and elk from the infected area, but the legislation necessary kept dying in the state Senate. Still, no one had expected to see CWD in Wisconsin anytime soon. Game farmers had a self-interest in avoiding the disease, and it would take decades to travel 1,000 miles, breech the Mississippi River and turn up here.

To be safe, in 1999, the DNR began testing deer shot near the game farms involved with the 24 elk in Fond du Lac, Dodge, Jefferson, Sheboygan and Washington counties. There was no trace of CWD in 630 deer brains examined in 1999 and 2000. In 2001, it set out to sample more deer, this time in Viroqua, Crivitz, Black River Falls, Spooner and the upper Michigan border. At the last minute, Mt. Horeb was added to the list because a previously unassigned DNR worker lived nearby. The Mt. Horeb site contributed 82 of the 345 tissue samples tested for CWD that year and three tested positive. The DNR was lucky to have detected CWD at all. "It was a fluke," says Mytton. "The number of samples was so small, you wouldn't even really expect to find it."

Three DNR game wardens were immediately dispatched to tell the hunters involved that their deer, all bucks 2 to 3 years old, had CWD. One had already turned his deer over to DNR because it looked sickly, but the others had been healthy specimens and the families had already consumed some of the venison.

It takes an average of 15 months for a deer infected with CWD to exhibit symptoms, but they can occur in as little as six months or not show up for as long as eight years, says Hauge. Then it will display abnormal behavior, stupor, head tremors, staggering, lack of coordination, trouble judging distance and difficulty swallowing. CWD-infected deer drink lots of water, increasing urination, and slobber excess saliva. They "waste away" until paralysis or pneumonia set in. "It is not a pretty death," says Hauge.

Scientists believe nose-to-nose contact spreads CWD, as well as environment and food contaminated by saliva, urine and feces and by does who infect their offspring. At first, they also believed that less than 5 percent of deer were susceptible and that CWD would remain confined to parts of Colorado and Wyoming. But then it destroyed 15 percent of the mule deer in northeastern Colorado, killed 52 percent on a ranch in Nebraska and crossed the Rocky Mountains.

Gaining speed, the killer turned up in a Nebraska mule deer in 2001. Officials there proclaimed a "wildlife disease emergency," predicting that CWD would "explode" if it got into the whitetail population and "devastate" that state's "entire deer population in 50 years." The USDA declared a national "CWD emergency," pledging to focus on game farms and wipe it out. Still, by August 2002, CWD-infected deer had been found in Montana, Kansas, South Dakota, New Mexico and Saskatchewan and Alberta, Canada, besides Wisconsin.

Even the DNR itself could become a casualty. Last year, it sold 730,000 gun and 258,000 bow hunting licenses in what has become a $1.5 billion-a-year state deer hunting and processing industry. Revenue from those licenses underwrote about a third of the conservation and wildlife management programs the state operates.

CWD also threatens to wipe out a way of life. The deer hunting culture may be stronger in Wisconsin than anywhere else in America. Here, residents talk about gun-hunting season, the third week of November, as "Holy Week," and spouses left behind hold Hunting Widows' Balls. Over the past five years, Wisconsin has ranked first in the average number of deer killed annually by hunters, with 465,000, followed by Michigan (455,000) and Pennsylvania (428,000), says Dan Schmidt, editor of Deer and Deer Hunting Magazine. That's despite having one of the shortest gun-hunting deer seasons in the country, nine frenetic days. In spite of the large harvest, mild winters and widespread feeding have pushed reproduction up, so that 30 percent to 50 percent more deer are born each year. Biologists say the state can sustain 1.1 million wild deer. Going into the fall hunt, the state's official estimate was 1.6 million, but the U.S. Geological Service National Wildlife Health Center put the total closer to 2 million. The DNR has tried in vain to expand the hunt for 10 years, running into opposition from the Wisconsin Conservation Congress and snowmobile industry.

The burgeoning herd has already driven up damage to agricultural crops, devastated wild habitat on which other animals depend and devoured ornamental plantings around suburban homes. In 2001, the state reimbursed farmers $1.5 million for crop damage, money from a $1 hunting license surcharge. Since 1997, DNR's crop damage fund has paid out more money than it has collected. And last year, Wisconsin had 90,000 deer-vehicle collisions, more than any other state in the country, according to The Wall Street Journal, forcing taxpayers to pay higher insurance premiums and at least $600,000 to renderers and landfills that dispose of road kill.

"If CWD scares hunters away," says Batha, "if the count is way down, we will throw up our hands. There was a special legislative session to give us the power to kill the deer if hunters don't, but I don't see how we will. I'm still looking for ardent hunters to help us, unless fear or their wives keep them away."

The DNR will provide "disposal options" for hunters in its eradication zone and the surrounding 10-county management zone, but in the rest of the state, hunters are on their own. The DNR quietly explored putting dumpsters at deer registration sites statewide, but "our fear is that if we build it, they will come," says Hauge, "and then how can we afford to pay the cost of disposing all of those deer?" The DNR is already spending more than $12 million on its CWD offensive.

CWD's Family Tree
When Richard Race, a leading researcher on CWD and related diseases with the National Institutes of Health's Rocky Mountain Laboratories, heard Kazmierczak's comment about eating deer brains, he was appalled. "That's irresponsible. Do we want to have a repeat of what happened in Britain, where for 10 years the government said BSE [mad cow disease] was safe to eat, and so far we have 133 people dead because of it?" asks Race. "It's not fair to say people either are or aren't susceptible to CWD at this point. Not enough people have eaten enough infected venison and lived long enough for it to incubate."

Researchers who saw the first cases of CWD in Colorado deer in 1967 thought it was a nutritional problem. By 1980, they realized it was part of a family of deadly neurological disorders called transmissible spongiform encephalopathies (TSEs), affecting humans, sheep, mink, cats and other animals. The most famous TSE of all, bovine spongiform encephalopathy (BSE), appeared in Britain in 1985. It killed nearly 200,000 cattle, then spread to humans who consumed infected beef. Unlike most diseases, which are caused by viruses or bacteria, the infectious agent in TSEs is believed to be a normal protein that mutates, becoming an infectious protein, or prion, and then causes the misfolding of other proteins. The process creates sponge-like brain lesions leading to physical and mental decline. Although human cases are rare, TSEs are so significant that two Nobel prizes for medicine have been awarded for prion disease research.

When mad cow disease first appeared, officials said it was "just like scrapie in sheep," the oldest known TSE, never known to have infected humans. The disease is called scrapie because infected sheep become so deranged that they rub themselves raw attempting to scrape off their fur. There is unproven speculation that CWD originated when environmental contamination passed scrapie from sheep to deer housed at Colorado State University's Foothills Research Station, then to wild deer and elk. That's one reason Wisconsin officials say CWD is like scrapie. Of course, that's what the British government thought, too, while nearly two million contaminated cattle slipped into the human food chain.

For more than a decade -- even after cats fed infected beef meat and bone meal came down with a mad cow-like disease -- the British government insisted that human beef eaters were protected by a "species barrier." Five years after some physicians and scientists started sounding alarms about eating infected beef, then British Prime Minister John Major was still reassuring the public: "There is no scientific evidence that BSE can be transmitted to humans." In January 1996, British Agriculture Minister John Gummer appeared on TV to feed his young daughter, Cordelia, a hamburger to demonstrate that beef was safe.

Two months later, everyone knew it wasn't true. Two dairy farmers, a butcher, a meat pie maker and eight young people who had eaten contaminated beef were dead from an Alzheimer's-like disease called new variant Creutzfeldt-Jakob disease (nvCJD). It looked similar to the rare sporadic CJD that affects only one person per million, but when their brains were autopsied, they looked just like the brains of mad cows. The government finally admitted that the "species barrier" -- if it existed at all -- had holes in it.

The World Health Organization now says mad cow disease passed to humans when they ate infected beef. Hamburgers and processed meats like sausage that included bovine offal (by-products) -- brains, spinal chords, eyes, lymph nodes, thymus and intestines -- areas with the highest concentration of disease prions, are the chief suspects, although laboratory scientists have since found TSE disease agents in blood and muscle, too. (Dr. Olds says Wisconsinites should forgo venison sausage even if deer offal is left out and their deer tests negative for CWD because there is no guarantee their deer won't be mixed with others in the sausage-making process.)

No one knows how much infected meat you'd have to eat to get new variant CJD or even whether a little now and then will add up to infection. But as little as one-half gram of BSE-infected brain -- the size of a good vitamin pill -- taken orally will infect a cow, NIH senior prion disease researcher Paul Brown told the FDA's TSE Advisory Committee on January 19, 2001.

Cooking at temperatures above 600 degrees F doesn't kill the infectious agent in TSEs, nor do detergents and enzymes known to kill most viruses. Radiation doesn't faze it, and even after being buried in the soil for three years, enough prions remain to spread the disease. Chlorine bleach is one of the better disinfectants, in concentrations of 50 percent and higher, but sometimes even that has diminished effectiveness. It's difficult to find a neurologist like Antuono willing to autopsy the brain of a patient suspected of dying of a TSE. A splash in the eye of bodily fluid can carry the disease straight to the brain.

Beef by-products are now banned from human consumption in Europe (though not in the United States), but since human TSEs incubate for decades, no one knows what the final human death toll will be. Those who have already died shared one of three genetic variations for the human prion protein, a genetic make-up seen in 40 percent of the population. But "that doesn't mean others are immune," says UW's Aiken. The disease may merely incubate longer in people with other genes.

The DNR's CWD Risk Assessment, written by former DNR veterinarian Doris Olander, dismisses the likelihood that CWD will infect humans. Says Olander: "Researchers and hunters have been exposed to CWD in Colorado for decades without becoming infected" and "there is no scientific evidence that CWD is transmissible through consumption of meat from an infected animal."

But "people overemphasize the lack of evidence of transmission of CWD," says University of Colorado neurologist Patrick Bosque, who, with Nobel laureate Stanley Prusiner, found disease prions in rather high levels in the muscle tissue (meat) of TSE-infected mice, research published in the prestigious Proceedings of the National Academy of Sciences. "And a lack of evidence is not evidence. In Colorado, the number of hunters in the endemic area was very small, and if it did transmit to humans, we wouldn't necessarily be able to see that yet. It's more reasonable that if you feed enough CWD-infected meat to enough people and wait long enough, some people will get it. But is it 1 in 10,000? Or 1 in 10 million? We don't know."

Research published in May 2000 in The European Molecular Biology Organization Journal states: "CWD transmissions would be similar to transmissions of mad cow disease," adding that, "it would seem prudent to take reasonable measures to limit exposure of humans (and cows and sheep) to CWD infectivity." But Olander, who has since gone to work for the USDA, looked at the same report and concluded that CWD is "less likely" to affect humans. She becomes vehement when asked about it now: "I wouldn't go hanging your hat on one paper. This isn't the be-all and end-all study," she says.

The study she discredits was authored by some of the most respected CWD research scientists in the country, including one the DNR flew in as a CWD consultant. The Milwaukee County Medical Society's Public Health Committee cited the same research in calling for new precautions in processing venison and cited "studies dating back to 1986" that "suggest an association" between hunting and CJD. One, conducted by Temple University and the NIH in 1996, found that exposure to deer through a hobby such as hunting resulted in a ninefold increased risk for CJD.

In a way, Wisconsin has become a giant petri dish for a grand experiment. It is not the first time the state has been on the frontier of TSE research, nor the first time a public official like Olander has tried to downplay unsettling research on TSEs. Only then, she was on the other side.

Wisconsin's TSE History
The original human TSE, classic Creutzfeldt-Jakob disease, is often mistaken for Alzheimer's, but it is a rare nervous system disease that results in rapidly progressive dementia, a nightmare of madness and terrifying hallucinations, loss of motor control, paralysis and death. Discovered in the 1920s, it seems to occur spontaneously across the globe (300 per year in the United States), attacking victims with a mean age of 68. Scientists refer to it as "sporadic CJD." Another extremely rare type of CJD occurs in families.

But in the 1950s, a human TSE called kuru was discovered among the primitive Fore people of New Guinea, and it was epidemic. American researcher Carleton Gajdusek received the Nobel Prize in 1976 for showing that kuru was spread by the Fore's ritual cannibalism of deceased relatives, including eating the brains of those who died of kuru. From kuru, scientists learned that a human TSE can incubate 10 to 40 years.

Transmissible Mink Encephalopathy, a related disease, has a long Wisconsin history. Four of the five known U.S. outbreaks since 1947 have occurred here. The first killed every mink on the affected Wisconsin ranch. In 1969, the late UW-Madison researcher Dick Marsh discovered that TME could be transmitted to raccoons and monkeys in the lab. By the mid-1980s, both Marsh and Nobel laureate Gajdusek believed all TSEs were really one disease altered by the passage from one species to another, and that a single TSE could have multiple strains that act differently. (The CWD in Wisconsin and the CWD in Western states may turn out to be distinct strains, but at present, scientists are applying the little they know about the Western disease to Wisconsin.)

When mink TSE wiped out 60 percent of the 7,300 breeding mink on a Stetsonville, Wisconsin, ranch in 1985, Marsh traced the disease to the mink's feed, downer dairy cows (cows unable to walk and considered unfit for human consumption). Marsh injected some of the infected mink tissue into the brains of two calves and waited. In less than two years, the calves' rear legs collapsed under them just like downer cattle. He injected some of the calves' brain tissue back into healthy mink. After an incubation period identical to that in the original infected mink, the new mink developed the same disease.

Concluded Marsh: "If spontaneous cases of prion diseases can occur in humans, they likely also occur in animals. Not naturally transmitted to other members of the species, these spontaneous incidents can still pose a danger because of the unnatural act of cannibalism, as seen in kuru in humans or... the feeding of animal protein produced by rendering ruminants" back to other ruminants (sheep, cows, elk, goats and deer). In effect, the then common practice made herbivore cattle into cannibals. In 1995 alone, DNR records show that 26,488 road-killed Wisconsin deer were rendered into feed.

No case of mad cow disease has ever been confirmed in the United States, but Marsh urged the USDA to ban the practice of feeding processed bone and blood meal made from rendered sheep, cows and deer to other ruminants. His suggestion would have cost the agricultural industry dearly in substitute protein, and the USDA took no action. Frustrated, in 1993, Marsh repeated his concern in the state Ag Review, warning Wisconsin dairy farmers they were feeding cattle to cattle. He also talked to The New York Times. Marsh's published comments ignited such a torrent of complaints from the state's agri-business industry, which underwrites much of the UW Agriculture School's research, that the college's dean tried to silence Marsh. Marsh was harassed and threatened with lawsuits, and the university sponsored a symposium "whose only purpose seemed to be arguing there was no need to change animal feeding patterns," recalls Aiken, then a Marsh colleague, as was Olander. (Both joined Marsh in pushing for a broad ruminant-to-ruminant feeding ban.)

Marsh was "not allowed to speak, while everyone discredited his work," says John Stauber, executive director of the Madison-based Center for Media and Democracy, who dedicated his book, Mad Cow, USA, to Marsh. Despite the humiliation inflicted by the university, Marsh would be vindicated. When protein feed from rendered downer cows and scrapie sheep was identified as the cause of mad cow disease in Britain in 1996, the university lionized Marsh in its Wisconsin Alumni Magazine as the scientist who'd predicted the disaster and tried to stop it.

"We were inundated. We had over 200 phone messages from CBS, NBC and other people in the media who wanted to talk to Dick," remembers professor Bruce Christenson, Marsh's successor as chair of the department of animal health and biomedical sciences. But by then, Marsh, 58, had cancer, recalls Christenson. "He was a warrior even when he knew he was dying."

Expert Advice
Battered by public criticism over his use of state planes, Gov. McCallum still arranged for one to fly Mytton and one of the DNR's wildlife veterinarians to Nebraska, where a meeting was under way on CWD. McCallum wanted Wisconsin to tap into the nation's best CWD expertise. To their dismay, the two soon discovered Wisconsin's situation was vastly different, and much worse.

CWD in the West was found in desolate rural areas with fewer than six deer per square mile. In Wisconsin, it was just outside the state's second-largest city, an area where deer density is more than 100 per square mile. Wisconsin's annual deer harvest is more than 200 times Colorado's, 10 times Wyoming's. Worse still, Wisconsin deer are highly social whitetails, not solitary mule deer. A mule deer might infect three or four others in a year; a single whitetail could infect 16, says UW-Madison wildlife ecologist John Cary.

The experts looked grim when they heard these things, recalls Mytton. "They said, 'You people in Wisconsin are in a shitload of trouble... There is no way you are going to stop this thing.' " Undaunted, the three agencies summoned by the governor -- the DNR, Department of Health and Family Services and Department of Agriculture, Trade and Consumer Protection -- along with experts from UW-Madison, the UW-Extension and the USDA, became the "brains or planning arm" of the state's CWD operation, to use Hauge's words.

On March 20, the DNR held its first CWD public information meeting in Mt. Horeb, drawing a crowd of 1,200. "It was probably the largest CWD meeting ever held in the United States," Hauge says, and a measure of "the close bond people in Wisconsin have to the land and deer." Colorado CWD expert Mike Miller told the crowd his state had made a big mistake. It decided to wait until it had more research, he said. "If Colorado did what you're doing here, 20 or 30 years ago, you wouldn't have this problem now."

There were 1,500 at the next public CWD meeting, but it was no longer just state residents who had a stake in Wisconsin's war on CWD. All of the country's 14 million deer hunters do. "If Wisconsin doesn't stop CWD, it will move all the way across the country. This will be a national story," warns Allen Boynton, wildlife biology manager for the Virginia Department of Game and Inland Fisheries. "Wisconsin is doing the right thing. There is no other responsible thing they could have done given current knowledge of this thing," he adds, explaining that Virginia has already outlawed deer and elk farms and will begin testing for CWD this fall, as will most Southern and Northeastern states, because, "It scares us to death."

How the Epidemic Started
The DNR set out to learn how CWD got to Wisconsin to prevent a possible repetition of events. It had three suppositions. One was that a hunter had brought a CWD-infected deer back to Wisconsin from Colorado. The disease could have spread when he dumped the carcass. Deer are herbivores, but "more than we ever thought, deer do chew on the bones of other deer, maybe as a source of minerals," says Hauge, citing the work of Canadian animal behaviorist Valerius Geist.

A second potential culprit was an unidentified wealthy landowner who unwittingly imported an infected deer, then released it into the wild to improve the local breeding stock. This seemed preposterous to DNR's Batha the first time he heard it, but when he met a well-heeled landowner who had paid $60,000 to have red pines planted along a roadside because he didn't like passersby looking at "his" wild deer, Batha knew anything was possible.

The third DNR theory pointed a finger at the state's deer and elk farmers and at the Ag Department regulating them. It blamed some Wisconsin deer farms for importing infected animals. Despite their high fences (7 feet, 10 inches required by law), more than 100 have escaped. DNR found a decayed deer along a Mt. Horeb highway with a game farm tag in its ear. But an infected animal wouldn't even have to break away from a farm to infect wild deer, says Mytton, offering as proof a photograph of a captive and a wild deer touching noses through a fence.

When Ag officials read the DNR's three theories in the newspaper, neck hairs bristled. Neither side, of course, endorsed any notion that incriminated itself, and internally, some Ag personnel pointed back at the DNR, blaming its "mismanagement" of the herd. They called CWD "Mother Nature's way of downsizing." And even some DNR officials agree the herd was out of control. Says Don Bates, who runs DNR's pheasant farm: "There have been 9- and 10-year-old does killed in the Mt. Horeb area, even one 12-year-old. That tells me this area was not managed as well as it should be, for a very long time."

Another Ag Department hypothesis says CWD has always been here. "How many deer are supposedly starving to death each year? Well, maybe it's been CWD," says an Ag source. "Minnesota was so proud it didn't have any CWD, but they'd only checked 50 deer. And how come Wisconsin is the only state east of the Mississippi that's found CWD? Because we're the only one that's looked for it." The problem with that theory, says NIH researcher Race, is that "given how rapidly CWD spreads, you'd find it all over." And, adds Hauge, "the herd would never have grown like it did." UW ecologist Cary suggests that given the number of cases found so far, and their distribution, CWD has been here for four or five years. In 25years, he says, it will wipe out all of the deer in a 5,184-square-mile area.

The most troubling explanation for CWD's appearance came from neither state agency but from veteran agricultural and environmental writer Mike Irwin, freelancing in Madison's Capital Times. Irwin's groundbreaking reporting linked CWD to a group of landowners in western Dane County who, in 1990, began a concentrated effort at deer management in order to raise "super" bucks. The landowners, who controlled 12 abutting square-mile sections in the northwestern part of the town of Vermont, agreed to give young bucks six years to grow so they'd develop the imposing antlers and muscular bodies that would get them into the record books. Then they began long-term feeding of nutritional supplements to wild deer. Their effort succeeded: Between 1990 and 2000, Dane County recorded the third-highest number of trophy bucks in North America.

Up until August 1997, when the FDA, reacting to Britain's mad cow epidemic, banned all ruminant-to-ruminant feeding (sheep, cattle, goats, elk, deer, antelope and buffalo) in the United States, Midwestern rendering plants routinely processed Wisconsin deer carcasses into meat and bone meal that went into feed mill products fed back to ruminants, including deer. (Cows, sheep and deer can still legally be processed into bone and blood meal feed for pigs, pets and chickens; then they can be rendered and fed back to cows, deer and other ruminants.)

The feeding practice may have amplified the disease in the same way feeding spread TSEs among the Fore people, Britain's cows and the Wisconsin mink, something further suggested by the fact that 11 of the first 18 cases of CWD found in Wisconsin came from the "super buck" area. The connection is especially vexing because "that kind of feeding has been going on all over the state," says author Stauber, "more evidence that CWD is spread all over Wisconsin." The DNR did ban feeding of deer statewide once CWD was discovered, but by then, much of the damage had been done.

How the State Blew It
Politics played a hand in CWD's spread as well. By the mid-1990s, state deer and elk farmers were bridled under DNR's control and, together with their lobbyists, they pressured legislators to move them to the friendlier regulatory climate of the Department of Agriculture, Trade and Consumer Protection. On June 1, 1996, all but the state's whitetail-only deer farms got their wish (whitetail farms move to DATCP control January 1, 2003).

"The Legislature took control away from an agency with 150 wardens and gave it to an agency that has four people statewide, and they have to regulate beef and hogs, too," says retired DNR big-game specialist Mytton. "It would be like having four cops to enforce the 55 mph speed limit statewide. You just had a totally unregulated industry."

The 931 Wisconsin deer and elk farms now manage approximately 35,000 animals, making this one of the leading states in the multibillion dollar U.S. deer and elk trade, which provides trophy hunts and sells antlers, the velvet from them (the so-called "velvet Viagra") and scent used to lure deer. Since 1996, Wisconsin farms have imported 3,000 animals, some from infected herds in Colorado, Nebraska and Saskatchewan, and since 2000, more than 900 have moved within the state from farm to farm.

That was just the kind of movement the DNR sought to stop. When DNR officers looked into the game farms after CWD was discovered, Mytton says, they were still importing animals. "And when we reviewed their books, they'd have fewer animals than their records said, but they'd just say, 'Oh, they escaped,' and there's no penalty." Mytton and others suspected that some died from CWD, but there was no way to prove it.

One of the DNR's biggest opponents every time it tried to get tighter regulations on elk and deer farm imports or add rules against baiting and feeding wild deer was state Sen. Kevin Shibilski (D-Stevens Point), says Mytton, who now lives in Montana. "He'd be lobbying until the middle of the night to kill it. Shibilski threatened the department numerous times." (Shibilski is now demanding that the DNR provide inexpensive CWD tests to hunters, the same tests researchers like UW's Aiken say "are not meat safety tests because they will miss some infected animals" and give hunters false assurances.)

Seriously limited by a lack of enforcement staff, DATCP tried friendly persuasion to convince deer and elk farmers to voluntarily test for CWD. It didn't work. More than 80 percent did no voluntary testing at all, and there were no deer farms at all among those that did.

As early as 1998, word of CWD's threat was spreading nationally. "The recent rash of cases in captive elk has created a strong possibility that things are going to get worse with CWD," warned the University of Georgia quarterly newsletter, typical of what others were saying. On April 17, 1998, Nebraska officials cautioned DATCP's Siroky (the man who would feed venison to his granddaughter) that a Bloomer, Wisconsin, farm had imported an elk from a CWD-infected herd. In May, Colorado tipped off DATCP that a West Bend farm had imported animals from another CWD-infected herd. Did DATCP investigate to see whether state herds had become infected? It barely seemed interested. The May 27 letter from Siroky's deputy, veterinarian Robert Ehlenfeldt, sent to the second farmer, reflects the regulator's attitude. It says, "The state of Wisconsin currently has no rules covering CWD and is taking no action... No restrictions are being placed on your herd."

Meanwhile, says Mytton, "We had Department of Ag people yelling at us because we wanted to address the problem with an actual quarantine." When Mytton suggested requiring double fencing and health certificates showing a source herd had tested CWD-free for five years, he says Siroky told him, "That would be implementing Gestapo tactics."

Fearing CWD, Montana Fish and Wildlife had already imposed a moratorium on deer and elk imports when, on September 15, 1998, Hauge's boss, Steven W. Miller, administrator for DNR's Division of Land, wrote a letter to then DNR Secretary George Meyer recommending Wisconsin do the same. DATCP immediately alerted deer and elk farmers. In a letter dated September 23, 1998, Mike Monson, president of the Wisconsin Commercial Deer & Elk Association, wrote Siroky: "Some people in the DNR are out to get us," adding that he saw "no reason they should impose a moratorium when not enough is even known about the disease."

In early October 1998, Siroky attended the U.S. Animal Health Association's national meeting in Minneapolis, where the CWD threat was again a major topic, but he apparently remained unconcerned. The next month, DATCP turned over the question of whether there should be new rules to address CWD to an advisory committee stacked heavily in the industry's favor. It included 10 elk and deer farm representatives and one member from both DNR and DATCP.

Not surprisingly, the committee failed to recommend any additional regulation. The January 21,1999 meeting minutes show Siroky (or his replacement, Ehlenfeldt) telling the group no new rules would be enacted without its approval, then saying, "how CWD is transmitted is unknown" but that "feed containing tainted animal by-products and genetic susceptibility have not been ruled out" and CWD "is not an infectious or contagious disease." Clearly, that last comment is inaccurate. CWD is highly contagious, something already documented in animal health literature and the reason for Montana's import ban.

The minutes explain that most of Wisconsin's deer and elk farms are primarily breeders of trophy animals (not meat producers) and "don't necessarily want to know" if they have CWD. So instead of testing animals that died or were killed on their farms, potential threats to the state's free-ranging deer, all of the state's deer farmers and most of its elk farmers ignored the problem -- with the state regulator's blessing.

More than 80 percent "hadn't been doing any testing. Well, what does it suggest when one of the first captive deer tested after the new mandatory CWD testing law passed in late August tests positive for CWD?" asks UW ecologist Cary. Perhaps fittingly, the CWD-infected buck shot September 4 by an out-of-state hunter who paid $4,000 to hunt on an Almond, Wisconsin, game farm, belonged to Stan Hall, a member of DATCP's do-nothing advisory committee.

No one knows yet how far CWD has spread across Wisconsin, but because Hall's CWD-infected Portage County deer had contact with animals on farms in Marathon and Walworth counties, the number of Wisconsin counties suspected of having CWD doubled to six. "In areas outside the region where there's CWD," says Kazmierczak, "it's business as usual" for hunters. The question is, just how far has it spread?

A Battle Plan
There was no internal dissent when the CWD science and health team voted on its plan: Kill and test all 25,000 to 30,000 deer in what, by late October, had grown 43 percent into a 411-square-mile eradication zone near Mt. Horeb, including parts of Dane, Sauk and Iowa counties. In the 10-county area surrounding the eradication zone, half to three-fourths of the deer will be killed (up to another 70,000). In each of the remaining 59 counties in the state, 500 will be tested. If none of the 500 deer test positive in any county, hunters will know "with a 99 percent degree of certainty" there is no CWD there, say officials. Hunters worried about eating infected venison can freeze their meat until the results come back.

The trouble is, with DNR testing tens of thousands of deer, it will take months to get the results. "But even if your deer tests negative for CWD, that doesn't say your venison is good to eat," cautions DNR Western Area Wildlife Supervisor Tom Howard. Using a lymph node and tonsil CWD test like the one commercially available for $60, the DNR found seven positive cases of CWD in July that were missed by brain stem tests because they don't detect CWD until it has incubated for 15 months. Lymph tissue tests can detect CWD 30 to 40 days after infection, but it takes an experienced pathologist to spot the one or two small foci where CWD appears, and it's easy to miss cases. "There is a great deal of pressure from the top Ag and DNR people not to debunk the tests," says UW researcher Aiken. "These tests are fine tools for surveillance, but we should not be using them as a food safety tool."

No part of dealing with CWD is going to be fast or easy. Cary's computer model predicts that it will take six years to eliminate all of the deer from the eradication zone, then 18 or more to return the deer population to normal. "What we are trying to do," says Hauge, "is like trying to change the tire on a vehicle cruising 60 miles an hour." Once all the deer are killed, "you will have to keep eliminating deer... until there are five years with no disease."

But the imminent problems are with initial eradication and waste disposal. By fall, more than 70,000 acres, almost 30 percent of the target area, had been closed to the eradication effort because of landowner opposition. The groups behind it -- Citizens Against Irrational Deer Slaughter (CAIDS) and Landowners for a Rational Response -- want the DNR to proceed slowly, do more research.

This opposition believes that only a small percent of the herd is susceptible to CWD. "They are absolutely wrong," says Aiken. "What we're seeing in the lab is much, much higher than that. In deer, CWD is very contagious. No other TSE is like this. It's not like mad cow disease. We can't just stop feeding protein and have the disease go away."

Although the state will cremate any CWD-positive deer killed in the eradication zone, there is concern that prions from infected deer killed elsewhere could con-taminate the environment. On September 11, Hauge and a cadre of support staff drove to Berlin, Wisconsin, to convince the city's sewage treatment plant directors that it is safe to continue to accept liquid residue from the local National By-Products animal rendering facility. The plant handles waste from 260 butchering operations that will process venison this fall. The city fathers feared infectious prions concentrated in the liquid would remain in the water their sewage plant discharges deep into the earth. National By-Products District Manager Charlie Beard told the group his plant won't accept the remains of deer from the eradication zone or its surrounding counties. But his company's Iowa plant is doing exactly that. This summer, Iowa DNR officials intercepted a National By-Products truck hauling road-killed deer from Wisconsin's CWD management area to its Ames rendering plant. "Those deer very likely had CWD because it makes them go into a stupor, and they're more likely to get hit," says Iowa DNR biologist Bob Sheets.

"Our concern is that CWD could spread country-wide through feed. Oh, they'll stamp it, 'Don't feed to ruminants,' but some poor peanut farmer in Arkansas is going to look at that $3 bag of animal protein feed and a $10 bag of soy protein, and he'll use the first." But when Iowa asked Wisconsin DNR to help stop the practice, says Sheets, "They told me, 'Well, that's the cheapest cost for us to get rid of it. If you want us to do something else, you'll have to pay the difference between a couple dollars and $125 a deer." Sheets was stunned. "There's no interagency cooperation going on at all," he says. (National By-Products also bid on processing all deer from the eradication zone. Asked how it would dispose of the deer, Beard said it was "a trade secret.")

In Berlin, DNR vet Olander reassured city fathers that "prions are hydrophobic -- they stick to solids," and the board agreed to continue taking the renderer's effluent. Meanwhile, other Wisconsin cities threatened to fine hunters who put deer waste in their garbage. DNR's Howard advised at least one hunter to just "dig a hole and bury it... it's a scientifically sound option."

But at the NIH, leading prion scientist Race doesn't agree. "We don't think landfilling is a great idea" because the disease agent can survive for years, surface and infect other animals. But then, "treating rendering plant effluent and injecting it into the soil doesn't sound too good either. The way to handle this stuff," says Race, "is by incineration and putting the ash in a closed container or in alkaline pressure cookers."

Death By Venison?

Mary Riley
Mary Riley didn't like the smell of venison cooking, so when her husband, Glenn, got a deer in Wisconsin, or in Colorado, where they lived from 1989 to 1999, he'd have the whole thing made into sausage and jerky. Now and then, Mary would eat it, Glenn says, because her friends often served it.

Once when Mary's best friend, Dawn, invited them for a chili supper, Mary raved about it, saying it was the best she'd ever tasted. As Mary took seconds, Glenn, Dawn and her husband, Steve, all chuckled to themselves, but no one told Mary it contained venison. More than a decade later, Glenn can't stop thinking about that night. "I really wish we'd respected Mary's desire not to eat venison because maybe then she'd be alive today," he says. Mary Riley, 43, died January 6, 2001 of sporadic CJD, a disease that rarely strikes people as young as she was. Asks Glenn Riley: "Did the venison kill Mary?"

When Riley began showing symptoms -- forgetting things, getting irritable -- she and her husband both thought she was going through the "change of life." They'd just opened a tavern on a lake near Shawano, something they had long dreamed of, when Mary became withdrawn. She wanted Glenn to stay home with her all the time. She was afraid and clung to him. The second spinal tap suggested Mary had CJD; the autopsy confirmed it.

"It took Mary real quick. Pretty soon, you could talk right in front of her and she didn't even know it," says Glenn. He says he came home one day and found Mary screaming at their son, the teen pinned against the wall. "She was yelling and he was crying," says Glenn. "That wasn't Mary. She didn't even know what she was doing." For a while, after Mary started going mad, she'd be "in and out, like Alzheimer's," says Glenn. But in a few months, they had to put her in a nursing home. "In the beginning, Mary knew something was wrong, but then she didn't even know what hit her," he says.

When Mary died, the "coroner refused to pronounce her dead. The local funeral home wouldn't touch her," says Glenn. "I had to go through Marshfield Clinic to get someone to come and pick her up. They told me, 'You have to have her cremated.' " Glenn listed the cause of death in Mary's obituary. Not long afterward, customers came into the tavern telling Glenn a state health official on the radio had said Mary didn't really die of CJD. But Glenn had the autopsy report from Marshfield Clinic.

"Hearing that just devastated the guy," recalls his brother-in-law, Rob Zaleski, a columnist at Madison's Capital Times. Glenn asked Zaleski to find out what was going on. Zaleski got a tape of Waupaca radio station WDUX's Jack Barry interviewing Mary Proctor, then chief of the state Department of Health's communicable disease epidemiology section. Proctor did seem to ridicule the belief that Mary Riley had died of CJD. Zaleski says when he called Proctor, she denied she'd been skeptical about Mary's case. Proctor told him that the state didn't even monitor cases of CJD, so she had no idea why Mary Riley died, but that "anyone can write anything on a death notice, even that someone died of an ingrown toenail."

Hearing that, Zaleski said, "You do sound skeptical." And, he says, "Proctor snapped: 'This conversation is over, and if you quote me, I'll sue.' " Zaleski wrote a column describing what had occurred, even Proctor's threat and, he says, she tried to get him fired. Zaleski played a tape of his Proctor interview for his boss, who contacted the state and got an apology. Marshfield Clinic, whose reputation Proctor had sullied, got one, too.

Like Kazmierczak, the man who says you can eat deer brains without ever getting sick, Proctor was part of the state office responsible for investigating possible links between CJD and chronic wasting disease. It is up to the same office to refer cases to the Atlanta-based CDC's special CJD investigation unit.

So is the CDC investigating Mary's case? "No," says Ermias Belay, M.D., the CDC's top prion disease epidemiologist, because the state hasn't asked it to. While many Wisconsinites assume that the CDC is a pit bull looking for any suspicious cases of CJD, the agency waits for people like Kazmierczak and Proctor to call. Mary Riley's physician, Marshfield clinic neurologist Susan Mikel, says she would never feed Wisconsin venison to her family and she's ready to call CDC herself, particularly now that Steve, Mary's chili dinner companion, seems to be developing the same symptoms.
 

Jeff Schwan
July's Milwaukee Journal Sentinel article did prod state officials to ask CDC to investigate the cases of the three men who shared wild game feasts. The two men the CDC is still investigating were 55 and 66 years old. But there's also Kevin Bass, a Minnesota hunter who ate Barron County venison and died of CJD at 41. And there's Jeff Schwan, whose Michigan Tech fraternity brothers used to bring venison sausage back to the frat house. His mother, Terry, says that in May 2001, Jeff, 26, began complaining about his vision. A friend noticed misspellings in his e-mail, which was totally unlike him. Jeff began losing weight. He became irritable and withdrawn. By the end of June, he couldn't remember the four-digit code to open the garage door or when and how to feed his parents' cats. At a family gathering in July, he stuck to his parents and girlfriend, barely talking. "On the night we took him to the hospital, he was speaking like he was drunk or high and I noticed his pupils were so dilated I couldn't see the irises," his mother says. By then, Jeff was no longer able to do even simple things on his computer at work, and "in the hospital, he couldn't drink enough water." When he died on September 27, 2001, an autopsy confirmed he had sporadic CJD.

In 2000, Belay looked into three CJD cases reported by The Denver Post, two hunters who ate meat from animals killed in Wyoming and the daughter of a hunter who ate venison from a plant that processed Colorado elk. All three died of CJD before they were 30 years old. The CDC asked the USDA to kill 1,000 deer and elk in the area where the men hunted. Belay and others reported their findings in the Archives of Neurology, writing that although "circumstances suggested a link between the three cases and chronic wasting disease, they could find no 'causal' link." Which means, says Belay, "not a single one of those 1,000 deer tested positive for CWD. For all we know, these cases may be CWD. What we have now doesn't indicate a connection. That's reassuring, but it would be wrong to say it will never happen."

So far, says NIH researcher Race, the two Wisconsin cases pinpointed by the newspaper look like spontaneous CJD. "But we don't know how CWD would look in human brains. It probably would look like some garden-variety sporadic CJD." What the CDC will do with these cases and four others (three from Colorado and Schwan from Upper Michigan), Race says, is "sequence the prion protein from these people, inject it into mice and wait to see what the disease looks like in their brains. That will take two years."

CJD is so rare in people under age 30, one case in a billion (leaving out medical mishaps), that four cases under 30 is "very high," says Colorado neurologist Bosque. "Then, if you add these other two from Wisconsin [cases in the newspaper], six cases of CJD in people associated with venison is very, very high." Only now, with Mary Riley, there are at least seven, and possibly eight, with Steve, her dining companion. "It's not critical mass that matters," however, Belay says. "One case would do it for me." The chance that two people who know each other would both contact CJD, like the two Wisconsin sportsmen, is so unlikely, experts say, it would happen only once in 140 years.

Given the incubation period for TSEs in humans, it may require another generation to write the final chapter on CWD in Wisconsin. "Does chronic wasting disease pass into humans? We'll be able to answer that in 2022," says Race. Meanwhile, the state has become part of an immense experiment.

"I would be absolutely shocked if this thing isn't all over the state, and I think it's all over Iowa, Minnesota, Michigan and northern Illinois, too," says author Stauber, "because that's how widespread the feeding has been. It's just no one has tested enough to find it." Inside state government, they're trying to remain hopeful. Says Ruth E. Heike, DATCP assistant counsel and co-chair of the CWD planning team: "Let me tell you, everyone on the science team who believes in God is praying that we don't find it somewhere else."

Mary Van de Kamp Nohl is a senior editor of Milwaukee Magazine.

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