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Mad All Over

This family of killer diseases hits close to home. Too close.

Creutzfeldt-Jakob disease eats holes in your brain and causes you to stagger, twitch, cry out in your sleep, lose control of your bodily functions, go mad and die. The disease may have been hidden in a hamburger you ate in Europe ten years ago, a corneal transplant or your own genetic makeup. It can incubate for anywhere between five and thirty years and will make itself known only when you're within months of death.

Two Denver residents died of CJD--a cousin of mad-cow disease--this year.
And Colorado is the epicenter for another related malady--chronic wasting disease, or CWD--among mule deer and elk. In affected parts of the state, the incidence among deer is between 4 and 6 percent; it is 1 percent in elk. At the height of the mad-cow epidemic in England, the incidence of bovine spongiform encephalopathy, or BSE, in cattle in that country was less than 1 percent.

Last February, Chris Melani, a Longmont hunter, took the head of a deer he'd shot to the Colorado Division of Wildlife for testing. He was told the DOW would contact him within three weeks if the deer had chronic wasting disease. When he heard nothing, he and his family ate some of the meat. He took what was left to a packing plant, where, he says, "It got mixed up with a bunch of other people's deer and made into venison sausage"--ending up on who knows how many dinner plates. Six weeks after turning in the head, Melani learned his deer was among forty that had tested positive for CWD last season. "They said it was okay," he says. "They weren't going to tell anyone else about it."

Although scientists don't know if humans can become ill by eating tainted venison, the encephalopathies do transmit from species to species, and 26 people in England have succumbed to a frightening new variation of Creutzfeldt-Jakob disease that's linked to mad-cow disease.

One of the most common routes for CJD infection is tissue from human cadavers used in surgery. In September, a forty-year-old Denver woman died of CJD. Because the disease generally occurs spontaneously only in the elderly, autopsy tissues were collected and sent away for analysis.

Last month it was determined that this woman had not died of new-variant CJD--that is, that her illness was not connected to eating meat. "It appears that she acquired the disease when she had surgery in Denver in 1992," says Dr. Richard Hoffman, a state epidemiologist with the Colorado Department of Public Health and Environment. During surgery to correct a congenital malformation of the spinal column, a portion of dura mater, tissue taken from the outer covering of the brain, was used to protect part of the woman's central nervous system.

"The material came from a company in Germany," says Hoffman. "In 1992 such material was pooled from several donors--I don't know how many. It will never be possible to prove 100 percent that that was the cause, but the incubation period of six years is about the right length of time for this disease if it's brought on by contaminated tissue."

Between 1987 and 1992, exposure to infected dura mater killed seven people in New Zealand, Britain, Italy and Japan, according to Richard Rhodes's book, Deadly Feasts: The Prion Controversy and the Public's Health. Three Australian women died during the same period because they'd used a hormone called gonadotrophin to counteract infertility. And there have been eighty known deaths worldwide from the use of contaminated human growth hormone.

Creutzfeldt-Jakob claimed a second Denver victim this year, a 78-year-old man. "It's the worst thing there is," says Linda, one of his daughters. "It turns your brain tissue to sponge."

"He'd always been relatively healthy," says a second daughter, who asks to be identified only as Sue. "But six months before this came to a head, we noticed some things. He was becoming forgetful, unable to complete projects he'd done all of his life. We put it down to old age. In February, on a trip, he experienced some dizziness and light-headedness. He came back, and there were other problems. He had trouble walking; his speech was a little slurred; his balance was totally off. We thought perhaps he'd had a stroke, but the tests were negative."

There were other tests: for Lou Gehrig's disease, for Guillain-Barre. But their father's health continued to deteriorate, and when the hospital could offer nothing but custodial care, his family took him home. "In four months," Sue says, "he went from fairly healthy to unable to care for himself whatsoever. He was unable to swallow, and he contracted pneumonia. Less than a week later, he was dead."

At present, the only viable way of identifying Creutzfeldt-Jakob is a brain biopsy, a procedure difficult to perform on a living subject. CJD was diagnosed in this man only after death. On the death certificate, the cause of death is listed as pneumonia.

Because of misdiagnoses like this, health activists say, no one really knows how prevalent CJD is. Although federal officials say instances of CJD have not increased in this country, in most states, including Colorado, CJD is not a reportable disease--one that states are required to document for the Centers for Disease Control. Meanwhile, alternative studies indicate that perhaps 13 percent of patients diagnosed with Alzheimer's are in fact suffering from CJD.

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