Two months later Jim, 55, was dead. Specialists had diagnosed him with Creutzfeldt-Jakob disease, a fatal affliction in which rogue proteins called prions riddle the brain with spongelike holes. In humans, CJD is known to be acquired either sporadically (striking one person in a million), genetically or by infection. (About 300 people in the U.S. have died from it in the past year.)
It is well-known that humans can contract CJD by eating cattle stricken with mad cow disease, which has killed 120 people (mostly in Great Britain) since 1995. But the deaths of Jim Botts and two other men raise a troubling new question: whether game animals infected with chronic wasting disease, a mad cowlike ailment, pose similar dangers.
Judy Botts first began to wonder whether there was a connection between venison consumption and her husband's illness days after Jim's death. That was when she learned from her mother that 66-year-old Wayne Waterhouse, a bait-company owner and acquaintance of Jim's from their old hometown of Chetek, Wis., had also died of CJD in 1993. For years Waterhouse, an avid hunter, had held periodic feasts for as many as 100 friends, serving deer, elk and other wild game at his Brule River cabin about two hours from Chetek. Jim Botts, who fished the Brule, attended about a dozen times. In 2000 Water-house's son Gary, 55, called Judy to discuss the coincidence; he later pointed it out to the press, along with the fact that a third regular at the feasts, 66-year-old Roger Marten, had died with symptoms similar to CJD. The resulting flurry of articles prompted officials at the Centers for Disease Control to test tissue from Botts, Waterhouse and Marten. The studies will determine if Marten actually died of CJD and will analyze the tissue of all three men for markers linked to chronic wasting disease.
Up to now that illness has never been known to leap the so-called "species barrier" from game animals to humans. Even Gary Waterhouse says he's confident that the deaths were "totally coincidental." That may change soon. CDC spokesman Thomas Skinner expects the agency will have test results back to the Wisconsin Department of Health & Family Services "within a few weeks." The health department will then share those results with the men's relatives and the public. The findings will touch the lives of hunters, venison eaters and their families across the U.S.–especially in towns like Chetek, where the shooting season is an important part of the culture and economy. Dr. Fred Bannister, 63, a local physician, points out that statistics show no rise in CJD cases in areas with ailing animals. Still, he says, panic has already set in. "It's devastating," he continues. "Think of what will become of the tourism industry."
Whether or not the dead men's tissues show signs of chronic wasting disease, officials will continue to eliminate infected herds. The eradication of animals has begun in several states, not only to limit the scourge among deer and elk, but to prevent it from spreading to cattle. Without such measures, says Dr. Dennis Maki, director of the University of Wisconsin's infectious diseases department, "I'd be surprised if we didn't see a disease–CJD–in humans."
Judy Botts is betting we already have. "When Jim died," she says, "it was like my soul had been ripped out." The high school sweethearts wed in 1966. After Jim took a job at Honeywell, where he would become an engineer, they moved to the St. Paul suburb of Blaine. There, they raised Steve, now 33 and an environmental consultant; Chad, 31, a salesman; and 29-year-old twins Melissa, a physical therapist, and Melanie, a nanny. "Jim never did anything halfway," Judy recalls. "If he fished, he built the fishing pole and made the flies."
That made his last days all the more agonizing. "I was hand-feeding him," Judy says. "At times he'd have this look of absolute fear." Now she hopes Jim's death serves a purpose. "I want people to make wise decisions about what they eat. And I want them to know about Creutzfeldt-Jakob. I want them to know it's out there and what to expect."
Margaret Nelson in Chetek