AR-News: The case of the Cerry Hill cluster

[Andrew Gach]

Excerpts from the article "The Case of the Cherry Hill Cluster" in the March 28 issue of the New York Times.

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In January 2000, a friend of Skarbek's named Carrie Mahan became ill. One evening, Mahan, 29, went with her boyfriend to a party and came home unusually tired. The next morning she started hearing songs in her head and had trouble using her key to unlock her car door. At an emergency room in Philadelphia, doctors gave her medicine and suggested rest. But she was back the next day, complaining of anxiety, nausea and hallucinations. She was admitted, then transferred to the University of Pennsylvania Medical Center. Things got worse quickly. She faded in and out and began to suffer body twitches called myoclonus jerks. Soon she fell into a coma and was put on life support. About a month later, on Feb. 24, 2000, she was allowed to die. 

Early on in Mahan's illness, one of her doctors, Peter Crino, wrote on her chart, ''Could this be C.J.D.?'' -- by which he meant Creutzfeldt-Jakob disease, a fatal brain disorder often characterized by myoclonus jerks, loss of coordination and sometimes dementia. C.J.D. is extremely rare; the University of Pennsylvania Medical Center, which is an unofficial referring hospital for such cases in its region, typically sees only about one or two victims of C.J.D. a year. So the working diagnosis was some sort of viral infection of the brain. But when Crino saw the autopsy and brain biopsy results, he suspected that his first thought had been right. ''She had holes all over the place,'' he said. ''She clearly had a devastating neurologic injury. Her brain was just gone.'' 

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Mahan's doctors were not sure which of these versions of C.J.D. she may have had. Her prion gene was found to be normal. And since no one was known to have gotten mad cow disease in the United States, the possibility of infection seemed remote. Her C.J.D. was most likely the third kind. It had no direct cause. In medical parlance, the disease was sporadic C.J.D. 

It was not a perfect diagnosis. For one thing, Mahan was black, and very few black C.J.D. victims had been reported. She was also extremely young to have sporadic C.J.D.: it usually strikes people in their 60's or older. According to Pierluigi Gambetti, who runs the national surveillance center for C.J.D. at Case Western Reserve University Medical School, only 3 to 5 percent of the cases his center sees are in people in her age group or younger. Gambetti was sent some of Mahan's brain tissue to look at, and when he subjected it to a test for the presence of harmful prions, he got a negative result. But this itself was not especially troubling to Mahan's doctors. Prion pathology is an emerging science. Odd things occur all the time. What Mahan undeniably had were innumerable holes in her brain, and sporadic C.J.D. fit the pathology best. So C.J.D. went on her death certificate. 

Skarbek did not concern herself at the time with the complexities of her friend's diagnosis. What mattered was that her friend died. Pat Hammond, Janet's mother, who hired Mahan at the Garden State Race Track in Cherry Hill, N.J., had also been close to her. Mother and daughter absorbed the bad news together. ''We were told it was C.J.D.,'' Skarbek said. ''You know, the human form of the mad cow but not related to eating mad cow.'' Sporadic C.J.D. affects about one person in a million in the United States, where there are about 250 to 300 cases a year. Mahan had had terrible luck and nothing more. Skarbek was an accountant; she knew how to look at numbers without emotion. She could accept that and move on. ''We were told she was just one of those who got it,'' she said. ''It was a tragedy.'' 

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 One day, in June 2003, she happened to be reading The Burlington County Times, a local newspaper. She scanned the obituaries. ''I was just looking in the newspaper for Cinnaminson residents because I live in Cinnaminson,'' she said. She came across one for a woman named Carol Olive. ''The first paragraph said she died of C.J.D.,'' Skarbek remembered. ''I'm thinking, Oh my gosh, that's what Carrie died of. The second paragraph said she worked at the Garden State Race Track. That's when I almost fell over.'' She asked her mother if she remembered Olive from the racetrack. She did: Olive had worked as a media representative, an administrative employee, alongside her and Mahan. Could their deaths have been a result of mad cow, rather than of chance? The staff had been small. ''Suddenly we had two victims out of a hundred administrative employees,'' Skarbek said. 

Skarbek couldn't sleep the night after she discovered Olive's death. The next day she went immediately into her home office and searched for victims of C.J.D. in Lexis-Nexis, the online database that includes newspaper and magazine articles. She came upon an obituary for John Weber, who lived in Pennsauken, a neighboring town, and died of C.J.D. in 2000. She decided to call his family, even though she said she knew they'd think she was ''nuts.'' ''This way I'd just put it away,'' she explained. ''I'd make the call, and they'd say there was no relation to the track, and I'd forget about it.'' Weber's brother William answered the phone. He said his brother had a season pass to the Garden State Race Track and ''ate there at least once a week.'' 

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There is a lot of anxiety over mad cow disease in America right now. The fear has its origins in 1987, when British media began to report that for two years something strange had been happening on British farms: dairy cows repeatedly falling over, returning to their feet and charging. After finding the first sick cow, British veterinarians quickly found a lot of others. The daily papers could hardly keep pace with the rising toll. (It is now at more than 180,000 and has involved cows from nearly 36,000 farms.) Photographs of pyres of burning British cows appeared on front pages across the world. 

The disease was eventually traced to infected feed. To keep the cows growing fast, farmers had put rendered animal parts into their food. Some of the animal parts were infected with prions. It had occurred to people that mad cows might in turn infect humans -- humans do eat a lot of cows. But public officials in Britain counseled calm. British beef is safe, they said again and again. In 1990, John Gummer, the minister of agriculture, coaxed his 4-year-old daughter, Cordelia, into eating a hamburger on television. 

The assurances were made in vain. Mad cow in England had a second act. The first human to die was Stephen Churchill, a 19-year-old who fell victim to mad cow disease in 1994 and died in May the next year. He was followed by others. In March 1996, European officials banned British beef exports. As of February, 156 cases of the disease, formally called variant C.J.D., have been identified. 

The epidemic seemed unlikely to strike in America. The United States imported little British feed or beef. And the United States Department of Agriculture tracked the 300 or so British cattle that had been imported for breeding, and though it eventually lost track of half of them, they were past the usual age of onset of the disease. The U.S.D.A. declared America's meat safe. But food safety is an emotional issue. Many American consumers assume that whatever England gets, we will get worse. In England there are farms around every turn in the road. They look idyllic. They can be watched. In America farms are isolated in vast tracts of the Midwest and West. The logic of the market compels American ranchers to fatten their cattle with the help of hormones. Yet no cases of mad cow were reported here. 

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Many public health advocates and some scientists say they suspect that mad cow does in fact exist in America. According to an international panel that reported in February to the U.S.D.A., it is ''probable that other infected animals have been imported from Canada and possibly also from Europe.'' The panel added that since the animals haven't been detected, their infected tissue has probably been rendered and fed to other animals, ''so that cattle in the U.S.A. have also been indigenously infected.'' And humans? ''I hope that there are no undiagnosed cases of variant C.J.D.,'' said Gambetti of Case Western Reserve. ''However, my hopes are meaningless.'' He wants more testing and surveillance. Peter Crino, the neurologist who treated Carrie Mahan, said: ''I've got to think there's something more to the story of sporadic C.J.D. We're getting past the lightning strikes idea'' -- the idea that prion diseases just happen. 

Some organic food and vegetarian Web sites track and publicize suspicious-looking aggregations of cases of C.J.D., like a cluster of cases in Lehigh, Pa., in the late 80's, or a more recent group in Washington State reported to involve 35 deaths. And they never miss a chance to point out that the U.S.D.A., like the Ministry of Agriculture, Fisheries and Food, the agency that botched mad cow in England, is charged with a conflicting mission: protecting both the food supply and the business interests of food suppliers. Working on a book on prion diseases, I often run into people who tell me, ''My mother died of mad cow'' or ''I have a friend whose aunt died of mad cow.'' In such cases, it turns out that sporadic C.J.D. was diagnosed in the relative in question but that the family remains skeptical. The victim ate a lot of meat or spent a week in England in the 80's -- and the death was so awful. Always the awfulness of the death comes up. It's something many families can never let go of. It fuels the debate and the fear. 

Full story

http://www.nytimes.com/2004/03/28/magazine/28MADCOW.html?pagewanted=print&position=

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