Posted on 03/29/2004 6:12:59 AM PST by Pharmboy
Janet Skarbek is 36 years old and lives in Cinnaminson, N.J. She is the author of ''Planning Your Future: A Guide for Professional Women,'' a book about managing the unknown. It was published in 2001 by the Professional Women's Institute, a small networking and support organization that Skarbek and three other women jointly ran out of their homes. ''Planning Your Future'' presents a world where exemplary order and control are possible. It urges working women to get ahead by thinking ahead: choosing a career with their children in mind, timing pregnancies so as not to lose traction at work. It tells them to fend for themselves in a society they may sometimes perceive as unsympathetic to their needs. Skarbek herself turned down a plum corporate job for the sake of her two kids. ''I knew that I wasn't willing to work the hours that a vice president of a Fortune 500 company would require to get the job done right,'' she said.
''Planning Your Future,'' with its emphasis on success through self-sufficiency, was a minor volley in the culture wars. It landed Skarbek an invitation to speak at a Department of Labor conference in 2001 on ''the 21st-century work force.'' Skarbek is forceful and credible, a former internal auditor for the Internal Revenue Service who started her own accounting practice. President Bush opened the conference. When Skarbek's turn came to speak, she repeated her message to professional women to buck up, take their careers into their own hands and watch as their incomes rose.
Skarbek resembles Lewis Carroll's Alice -- the same short stature, broad forehead, straight, long hair and grown-up gaze, as well as the same touching, plucky personality. And like Alice, she, too, was about to fall down a rabbit hole.
n 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.''
Unlike many diseases, C.J.D. is caused not by a virus or bacteria but by a misformed prion (a type of protein) that no longer performs its proper function in the body. Prions -- at least according to current scientific thinking -- cause several other diseases, most notably bovine spongiform encephalopathy, or mad cow disease. Prion diseases are unique because of the number of ways they can occur. They can result from a genetic mutation that generates the harmful prion or from an infection of prions from an outside source, most likely in meat that is consumed -- or simply by chance: a certain number of proteins in the body just happen to lose their shape over time and bring about the disease.
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.''
Three years passed. Skarbek published her book. She gave seminars for women in corporate life. She drove her two kids in the family minivan to Girl Scout meetings and karate class. ''Plan!'' she urged everyone. 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.''
Skarbek dropped the phone. Weber was her ''eureka moment,'' she said. ''To me, it was just sending up red flags everywhere.''
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.
In the years after mad cow struck in humans in Britain, the U.S.D.A. changed some of its regulations. Beginning in 1993, it started testing ''downers,'' or cattle that are too sick or injured to walk. But it did not test very many of the approximately 35 million cattle slaughtered each year in this country. The 40,000 or so downers that were tested over the last decade all tested negative -- until this December, when a cow in Washington State tested positive. Much to the relief of the U.S.D.A., the cow turned out to be from a Canadian herd; it probably ate infected feed in Alberta when it was a calf. But this discovery has not entirely reassured food-safety experts, let alone the public. Anxiety returned earlier this month, when the inspector general of the Agriculture Department announced a criminal investigation to determine if documents from the slaughterhouse in Washington were falsified to cover up the fact that the cow was not a downer and had been tested only by accident. If the diseased cow was walking, the entire logic of the testing system would unravel. And without a sound testing system, who could say with confidence that mad cow was not here in America?
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.
Until June 2003, Skarbek was not suspicious of meat. She loved steak, and her family often ate at McDonald's. But after finding the three deaths of people associated with the Garden State Race Track, she became more careful. She started feeding her family organic meat. She also contacted public health officials about her discovery. She called the Centers for Disease Control and Prevention in Atlanta and the New Jersey Department of Health and Senior Services and told them what she had found. ''You have to understand that there's only a thousand season-pass holders,'' she told them. ''And there's a hundred administrative employees. So at this time, out of the 1,100 who ate there, the most often we should have seen one sporadic C.J.D. case was every 909 years. Here we've got three!'' She sent the same information by e-mail and fax. She had testified about sales taxes before the House Ways and Means Committee and spoken about women and the work force to a Washington audience of 6,000, but that did not mean health experts would take her seriously. The New Jersey Department of Health, citing Gambetti's findings, responded that Mahan hadn't died of C.J.D. In the other two cases, it said it was waiting for more information. ''They all blew me off,'' she said.
But Skarbek was undeterred. She continued combing through Lexis-Nexis, looking for C.J.D. in the local obituaries. She kept finding people. In 1997, Jack Schott, a 59-year-old dispatcher for trucking companies, died of C.J.D. So did a jazz musician named Kenneth Shepherd, in 2003, and in the same year, a 71-year-old man named John LaPaglia Sr. Skarbek began to get tips from friends and families and found two more victims: Walter Z., an I.R.S. accountant, and Alfred P., both of whom died in 1997. (In these two cases, for privacy reasons, the families did not release their full names to the press.)
What excited Skarbek most was that all the victims had eaten at the Garden State Race Track: Alfred P. dined there with a New Jersey congressman, Robert Andrews. Both Walter Z. and John LaPaglia had been season-pass holders. Shepherd's wife remembered him eating at the track. Jack Schott went in the early 90's. ''He had the beef,'' Skarbek said. ''His wife had the fish.'' Carol Olive, her sister said, was ''a lover of hamburgers.''
Skarbek kept the C.D.C. and the New Jersey Department of Health up to date on her discoveries, despite what she saw as their lack of encouragement. She wrote them in January: ''The cluster of people that ate at the racetrack and then developed C.J.D. is now at eight victims.'' She expressed frustration at not being heard. ''My concern is that the New Jersey Department of Health is not taking this cluster seriously,'' she wrote. She explained that a quick look at a map revealed that each of four victims lived in a town that bordered on Cherry Hill, the location of the racetrack. (In 2001, the track was closed.) She looked up the population of these towns: Cherry Hill had 70,000 people, Cinnaminson 14,583, Merchantville 3,801, Pennsauken 35,737 -- for a total of 124,121. Since the natural occurrence of sporadic C.J.D. was supposed to be one person in a million, a population this size should be expected to see a case only once every eight years, she calculated. ''Yet,'' she pointed out, ''we had four people die of C.J.D. in just over three years. The idea that it was just sporadic C.J.D. and could be easily dismissed as just occurring spontaneously in nature doesn't ring true.''
karbek's case may have seemed compelling, but it had one obvious weakness. She was trying to prove that the cluster of victims associated with the racetrack died of variant C.J.D., the human form of mad cow disease -- not sporadic C.J.D. But in humans these two diseases look very different, even though both are caused by prions. The sporadic type tends to produce holes in the brain. The variant type is typically characterized by a daisylike formation of thick deposits of prions in the brain, as well as by holes. James Ironside, director of the National C.J.D. Surveillance Unit in Britain, who saw some of the earliest cases of mad cow in humans in England, said he was ''overwhelmed'' by the difference between the two forms.
Skarbek did not know how to surmount this objection. But she was a go-getter. She wasn't about to give up on her cluster so easily. Fortunately, she was in contact with Terry Singeltary. She had seen his name quoted often on the Web in articles on C.J.D. and mad cow. Singeltary lost his mother to an extremely rare strain of sporadic C.J.D. in 1997. Soon after, he learned that a year earlier to the day, the mother of his next-door neighbor died of the disease. Since that time, he has become convinced that these sporadic cases are not sporadic at all, that mad cow is now a disease of humans in America. He said he believes that his mother was accidentally infected during surgery and the mother of his neighbor from taking nutritional supplements made from high-risk bovine tissue, which he calls ''mad cow in a pill.''
Singeltary has a sloping face and slicked-back hair. He is nearsighted, with small blue eyes. He looks like Lewis Carroll's White Rabbit. From his living room in Bacliff, Tex., he dominates the listservs and message boards of an online debate over sporadic C.J.D. -- the scientists who say it exists; the heartbroken family members who doubt it. Early, deep in his grief, he would sign his e-mail messages to scientists, ''I am the madson of a deadmom who died of madcow.'' Singeltary turned out to be helpful for Skarbek. He pointed her to a paper that was published in 2002 in the journal of the European Molecular Biology Organization by John Collinge, the premier prion researcher in England. Collinge argued that experiments conducted in mice suggest that infections with mad cow can sometimes look like sporadic C.J.D. Collinge accepted the implications: he recommended that ''serious consideration should be given'' to the idea that some of the more recent sporadic C.J.D. cases in Europe were in fact related to mad cow disease.
Skarbek said she thought that the same was true in New Jersey. It is hard to guess what a disease will look like based on laboratory experiments in mice -- ''The fact that you saw it in animals does not necessarily mean that it happens in humans,'' said Ermias Belay, the coordinator of C.J.D. surveillance for the Centers for Disease Control and Prevention -- but Skarbek was convinced. A new strain of mad cow, she argued, possibly domestic in origin, had been present in the meat served to people who had gone to the Garden State Race Track. ''Everyone ate at the track between 1988 and 1992,'' she said. ''I think the mad cow was probably served maybe during a one-week period during those years is what I'm suspecting. As we get more people who ate at the track and died of this, I think we'll be able to narrow it down maybe to the exact month.''
Is it possible that meat from an infected cow got to the Garden State Race Track in the late 80's? There are a lot of assumptions built into Skarbek's theory. There has to be a strain of mad cow disease in America that is rare enough or different enough to have avoided detection. (Belay of the C.D.C. said he thought that if there were a new variant of mad cow, it would have already shown up in England, where there has been far greater exposure.) That strain has to infect humans in a way that mimics the symptoms of sporadic C.J.D. but also differs from the well-documented symptoms of variant C.J.D. in Britain. And it had to be present in no more than a few cows whose meat was consumed by only a small group of people who ate at a single venue over a short period of time more than a decade ago. It does not help Skarbek's theory that during those years the racetrack food was provided by more than two dozen suppliers.
There is also the matter of the cluster itself. Is it really what it appears to be? Skarbek is not trained in this line of work. She effectively made up the rules of her investigation as she went along. Originally she was interested in victims who ate at the track often. Eventually she was interested in victims who ate there even once. This increased her pool considerably. Attendance at the racetrack from 1988 to 1992 was at least four million people. It is true that eight cases of sporadic C.J.D. would still be a lot in a group this size, but even that is not necessarily meaningful. Averages are tricky. You can assume that a chance occurrence like sporadic C.J.D. will happen at a steady rate over vast periods of time and among large populations. For instance, the overall number of cases of C.J.D. in the country has held steady for more than 20 years. But as you focus in on a smaller part of the picture, local aberrations are common -- even likely. Flip a coin a hundred times and you should expect heads and tails to come up about even. But during those hundred flips you are likely to see a long run of all heads or all tails. If that's the only time you happen to be paying attention -- or if you happen to live near Cherry Hill, N.J. -- you may well think something strange is going on.
Peter Crino, Carrie Mahan's neurologist, said he admired Skarbek's effort but had reservations about her technique. ''She's not an epidemiologist,'' he said. ''She's not terribly sophisticated.''
One day in February, Skarbek traveled down to Washington to meet with aides to senators and congressmen, including those of Senator Frank Lautenberg of New Jersey and Senator Edward Kennedy of Massachusetts. She took documents that testified to what she saw as the failure to protect the public from mad cow. She had made poster-board versions of them at Kinko's. One quoted from an internal memo from the U.S.D.A. on how to avoid a public-relations debacle of the sort Britain had experienced over mad cow. Another excerpted a General Accounting Office report from 2002 noting that mad cow disease ''may be silently incubating somewhere in the United States'' as well as the international panel report to the U.S.D.A. from earlier this year warning that if that were true, those cattle would have been slaughtered, rendered into feed pellets and fed to other cattle, amplifying the infection.
Perhaps reluctantly, the New Jersey Department of Health has continued to look into Skarbek's concerns. The C.D.C. is advising the agency as it gathers the victims' autopsy results and medical and laboratory data on them. ''I'm not calling it a cluster,'' said Eddy Bresnitz, the chief epidemiologist for the state. ''The individual in South Jersey is calling it a cluster.''
''They are investigating trying to disprove it,'' Skarbek insisted. She is past being mollified, anyway. Recently, after she was bounced from a television talk show (for more urgent reports on Rush Limbaugh and Michael Jackson), she sent an e-mail message to a C.J.D. listserv, saying, ''I believe the national beef lobby put pressure on them, just like they have in the past.'' She has filed so many Freedom of Information Act requests that a C.D.C. doctor told her she's known among his colleagues as ''the witch.'' (A spokesman for the C.D.C. denied this, calling its contact with her ''quite cordial.'') She recognizes the transformation that her worldview has undergone. ''Before June and July, when I started looking into this,'' she said, ''I would have never believed these things went on. Maybe I was just naive to it, but I never knew.''
''Planning Your Future'' takes a dim view of impulsive job changes. ''Do your research ahead of time, so you aren't regretting your career decisions later on,'' it counsels. But after Skarbek became alarmed by the Cherry Hill cluster, she closed the Professional Women's Institute. When the infected cow was found in Washington State in December, she suspended her accounting practice to devote herself full time to the cluster. ''It's not that I think I'm special,'' she said. ''I just feel that God was getting me ready to do this: the book, speaking in front of groups, speaking to Congress. I was picked to do this. And when it's done, I'm going to go back to my job.''
Last month she found two more possible C.J.D. victims who ate at the track -- one a longtime employee at The Philadelphia Inquirer, the other a contracts manager who was 72 and a season-pass holder. She continues to pass the information on to Bresnitz at the New Jersey Department of Health. He remains skeptical. ''Even if it turned out that all the victims ate hamburger at the same time at the racetrack, now what?'' he asked. ''What do I do with that information?'' Tracing the meat further back than the racetrack would be almost impossible at this point.
Just over a week ago, Skarbek found yet another victim, a woman named Jodi Tharp, who died of C.J.D. in 2001 and had owned a horse that raced at the track. She had eaten there, too, according to her husband. ''It fills in a missing hole,'' Skarbek said. ''We had 1997, 2000, 2003 and 2004. Now we have 2001.'' Bresnitz responded that Skarbek's case still wasn't good science. Without ''a reasonable hypothesis,'' he said, any investigation is a ''dead end.''
Skarbek has a different ending in mind. She sees herself unraveling a conspiracy of government and business interests to hide an epidemic. The idea pleases her. She recited a line from ''The Pelican Brief,'' the movie about a female law student who uncovers a government conspiracy: ''So you're the little lady who started this great brouhaha?''
D.T. Max is a frequent contributor to the magazine. He is at work on ''The Dark Eye,'' a cultural and scientific history of mad cow and other prion diseases, to be published next year by Random House.
CJD has a much higher prevalance in Eastern European Jews. We'd have to see if there is an ethnic factor first - and also try to determine what percentage of the population of the region has eaten at the racetrack as well to get a sense of the probability of this happening.
I know of one recent case.....
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