THE CHANGES IN KEN JOHNSTON were so minor that at first they were easy to brush away. Peggy Johnston first noticed last January that her husband was coming home unusually tired after working at the Sundance Golf Course they owned in Spokane. She also noticed he'd become uncharacteristically irritable and easily depressed, but she reasoned that the hard, physical work of maintaining the golf course through the winter's harsh ice storms had simply taken a toll on her husband of 38 years.
Then his golf game started to go. Ken had been a golf pro before they'd bought the Sundance and he was serious about his game. When they vacationed in Hawaii at the end of January, though, "he wasn't playing like he normally does," says Peggy. "He was all over the place."
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By May, the fatigue had deepened, and Ken abandoned his four-day-a-week swimming regimen. One day, after playing poorly at the Spokane Country Club, he told Peggy he was giving up the game he loved. "I can't hold onto the club," he complained. In fact, he was having trouble controlling his entire left arm. Ken's moods worsened. He became easily frightened, even paranoid. "I couldn't figure out what was going on," says Peggy, her voice raw with grief. "It was so subtle I didn't realize how bad it was." Figuring her husband would eventually snap out of his funk, she looked forward to a June trip with her church group for some much-needed relaxation. On the morning she was due to leave, Ken came to her, terrified and crying, and pleaded with her not to go. "There's something terribly wrong with me," he said. "I don't know what it is, but I think there's something eating my brain."
There was. He soon developed double vision that progressed to blindness. His body became wracked with tremors. He had trouble walking and even performing simple tasks like getting himself dressed. Dementia settled over him like a new skin. He saw snakes appear out of the air and believed himself to be a captain in command of a large boat. "He was lying in bed one night and he said, 'Can you see the stars?' It was the smoke detector," says Peggy. "I could still find him in there, but most of the time he was lost."
Finally, one day in August, he complained of a sore throat and simply stopped swallowing. At age 59, Ken Johnston was dead from Creutzfeldt-Jakob Disease (Croytz-felt Yawk-ohb), or CJD. This rare but inevitably fatal neurological disease riddles its victims' brains with microscopic holes and scar tissue, creating a spongy consistency in brain tissues. Undetectable until symptoms emerge, it is also incurable.
Tragic as Ken Johnston's death was, it was not extraordinary in a purely statistical sense. CJD kills at an annual rate of about one to two deaths per million people—more often, in fact than rabies. Washington state usually sees about five such deaths each year, according to the Department of Health. The condition is thought to arise from a genetic quirk, inherited in about 15 percent of the cases and occurring randomly in the rest.
In that respect, Creutzfeldt-Jakob is apparently a piece of the human condition. But unlike any other known genetic disease, CJD can also be infectious under the right circumstances. In the 1980s, British farmers unwittingly created those conditions by feeding diseased animals to dairy and beef cattle, creating an epidemic of bovine spongiform encephalopathy (BSE), or mad cow disease, a bovine version of CJD. The disease subsequently jumped to humans, killing 23 young Britons and a French citizen. Scientists have since raised the grim specter of a continent-wide brain-wasting plague whose victims could range into the millions.
In America, mad cow disease recently enjoyed publicity from the lawsuit Texas cattle ranchers pressed against talk show host Oprah Winfrey—who had the bad taste to badmouth burgers on a 1996 show—but it is generally not considered a domestic public-health threat. Aside from Oprah's tribulations, the media
ignore it. The government and livestock industry reassure us that mad cow disease isn't here.
If that turns out to be true, chalk it up to very good luck. For years, the US employed virtually the same rendering and feeding practices that precipitated the UK crisis. Although the federal government recently instituted new animal feed rules to prevent an American outbreak, the loopholes are large enough to drive through a herd of mad cows—or perhaps a pack of deranged pigs.
CREUTZFELDT-JAKOB DISEASE is a member of a family of mysterious diseases known as transmissible spongiform encephalopathies (TSEs). Spongiform disease has been recognized for centuries in sheep, as scrapie, while CJD was first diagnosed in humans in 1920. For decades, scientists considered CJD a hereditary condition and one that largely struck people over 60 years old. In the 1960s, however, that view began to change. Carleton Gajdusek, an American virologist who'd been studying a CJD epidemic among New Guinea cannibals, linked the outbreak to cannibalism. He later demonstrated that CJD could be experimentally transmitted to monkeys, and won the 1976 Nobel Prize in Medicine for his research.
