Mad All Over

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

"Sometime, somewhere, someone has to start taking reasonable surveillance seriously and establish a baseline," says Liz Armstrong, who founded CJD Voice, a Web-based support system for the friends and families of CJD victims. "It is only then that anyone can state whether there is an increase, a decrease or no change in the number of CJD cases in the U.S." CJD Voice's membership currently numbers 242, Armstrong says, and often grows by a member a day.

Gloria, a Californian who asked that her last name not be used, knows all about the family of diseases that includes CJD. Her husband died three years ago of the disease.

Ten years ago, right before a planned trip to Israel, her husband had reconstructive surgery on an injured eye. "Right before we left, in his sleep, he would babble and scream and laugh," remembers Gloria. "We went to the doctor, and the doctor kind of laughed and said, 'Don't watch the 11 o'clock news.'" But in Israel, the sleep disturbances continued. "He came to me one night with a flashlight," Gloria says, "and said there were men climbing down from the roof and to be very quiet."

After that, the hallucinations came in droves. "Every once in a while, he would cry and say, 'What's happening to me?'" Gloria says. "At the end of nine months he was in diapers, couldn't walk, didn't know who I was, kept looking for 'Gloria,'" she continues. "I had to pick him up out of bed, dress him. He shook continuously, and he kept rubbing his left blind eye--said it was so itchy. One morning I came out of the shower and he invited me to his bed. He couldn't do anything much. Afterward, he tried to throw me out of the apartment because his wife was going to be coming home. Finally, he went into a coma and died a few days later.

"I worked as a therapist in a day treatment program with schizophrenics and autistic kids," Gloria concludes. "But I have never seen anything like this. Never."

Despite the news from England of human deaths caused by consumption of contaminated beef, federal officials here say there's little cause for alarm. Bovine spongiform encephalopathy has never been detected in U.S. cows, they say, and there has been no confirmed case of new-variant CJD here.

But activists point out that testing has been done on only a tiny percentage of the 70 million or so cows in the United States and that the economic pressure to find nothing wrong is tremendous. In fact, there are indications that some form of BSE does exist in this country: In 1985, large numbers of mink on a Wisconsin ranch began dying of a spongiform encephalopathy; they had been fed meat from fallen dairy cattle. And Stanley Prusiner, who won a Nobel Prize for his work on the origins of CJD, said in 1996 that mad-cow disease had to be present at a low level in the U.S.

The cause of this family of diseases is generally believed to be an agent unlike anything previously known--an errant protein called a prion that manages to multiply uncontrollably in a process like that of crystallization. But although Prusiner won a Nobel Prize for this theory, there are credible researchers who believe the infectious agent will turn out to be viral. This is no abstract argument: Any hope of finding a cure depends on a clear understanding of cause.

The first case of CWD in a Colorado mule deer was discovered in 1967. In 1979 the first two elk succumbed; they had been held in the same area as the afflicted deer. Today the incidence among mule deer in the affected areas of the state is between 4 and 6 percent. In elk, CWD is at less than 1 percent, and the Division of Wildlife no longer requires that elk heads be tested. The disease has been found in northeastern Colorado, in and around Larimer County and concentrated in the area between Fort Collins and Estes Park, according to Todd Malmsbury, DOW spokesman. There is also evidence of CWD in southeast Wyoming, in South Dakota and among captive herds in Alberta, Canada.

CWD does not arise spontaneously in the wild, and no one is sure how the animals became infected. An anonymous Utah rancher offers his opinion on the mad-cow disease Web site ( Deer have always scavenged feeder bins, this rancher says, and have therefore eaten animal renderings in cattle feed, including "not only cow, sheep and all other animal bypass, but also themselves in highway kills and deer and elk dressings from hunter processings commercially done."

The rancher is skeptical about the alleged absence of the disease in U.S. cattle, noting that cows raised for food do not live long enough to manifest BSE. "Let's be real here," the rancher concludes. "If it is ever suspected, no rancher or feeder would lay himself open to having his entire herd culled for BSE at a potential loss of several hundreds to near $1,000 per animal, and neither would the livestock-dependent industries, including pharmaceuticals, cosmetics, soaps and beyond."

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