The amateur sleuth, the CJD victims, and the link to a day at the races

Janet Skarbek is not a scientist but she reads the obituary columns. And her conclusions have alarmed America
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The Independent Online

Americans are once more being asked to confront the spectre that bovine spongiform encephalopathy (BSE), which ravaged the British beef industry and crossed over into the human population with devastating consequences in the past decade, may have invaded their own shores undetected - or under the veil of a government cover-up.

Americans are once more being asked to confront the spectre that bovine spongiform encephalopathy (BSE), which ravaged the British beef industry and crossed over into the human population with devastating consequences in the past decade, may have invaded their own shores undetected - or under the veil of a government cover-up.

The latest BSE scare, which is casting a shadow of fear and suspicion on a small town in southern New Jersey, has prompted two prominent members of the Senate this week to demand an official study by medical experts from the Centres for Disease Control (CDC) in Atlanta, Georgia.

The senators were responding to the possibility - as remote and, as yet, unproven as it may be - that America may indeed be on the brink of the same trauma suffered by Britain after the first cases of BSE were discovered in herds in 1986 and when, 10 years later, people began to die from variant Creutzfeldt-Jakob disease (vCJD), known to be caused by ingesting BSE-contaminated meat.

Never mind that the source is one crusading woman with no scientific credentials who has merely seen a string of coincidences, involving food served at a local racecourse and an undeniably high incidence of vCJD in her area. And never mind that all those cases were diagnosed as sporadic CJD, which is distinct from variant CJD, which is tied to BSE.

Aside from the intervention by the two senators representing New Jersey, Jon Corzine and Frank Lautenberg, her hypothesis has at least been convincing enough for The New York Times to run its own investigation in the pages of its Sunday magazine 10 days ago.

Janet Skarbek, 36, is getting attention for her theory because it chimes directly with one of America's most deep-seated fears - about gruesome death and the food chain. What she is saying is that she has stumbled upon proof at last that the United States - despite all the protestations of government officials to the contrary - has long been afflicted with the variant of CJD in humans caused by BSE.

She is not the first person in America to make this claim. Ever since images from Britain of pyres of animals stricken with BSE appeared in newspapers here - and reports followed in the Nineties of people dying in the United Kingdom from vCJD caused by eating infected beef - Americans have been counting down to the day it starts over here. If it happened in Britain, it is bound to happen in the US.

These fears were only rekindled last December when a so-called "downer" cow was diagnosed with BSE in Washington State. Described as the first instance of a cow in the US found to have the ailment, it set all kinds of alarm bells ringing. It helped slightly, however, that the animal had been imported to the US from Canada.

In February, an international panel of scientists delivered to the United States Department of Agriculture a report that warned that it is "probable that other infected animals have been imported from Canada and possibly also from Europe" into the US. The panel added that if such cows have slipped through the system undetected, their infected tissues may have been rendered and fed to other cows, which could mean "cattle in the USA have also been indigenously infected".

Ms Skarbek is neither a veterinarian nor a scientist. She is, however, an avid reader of the obituaries in her local newspaper. Here is her claim: that an unusually high number of CJD cases have occurred in recent years in and around her nearest town, Cherry Hill. She calls it a cluster, although that is really for the scientists to determine. But there is more and it has to do with a now-defunct horse-racing course.

Research done by Ms Skarbek - either by trolling through the obituaries or talking to surviving family members - indicates that there has been a startling number of the recent victims of CJD in her area. More than that, they either worked at or were visitors to the Garden State Race Track in Cherry Hill that was closed down a couple of years ago. Could it be, she began to ask, that they had all eaten in the cafeteria there and more importantly, had eaten beef contaminated with BSE? She is convinced this must be so.

Senators Corzine and Lautenberg asked the CDC for an "immediate and thorough investigation" into her alarming claims. Left little choice, the CDC has replied that its scientists, "will use the best available science to respond in timely and thorough way".

This does not mean that the CDC does not remain sceptical - as do many other neurological experts - about the so-called Cherry Hill cluster. But the politics of BSE are so delicate and the stakes for the beef industry and the safety of the food chain so high, that Ms Skarbek, privately referred to in the corridors of the CDC as 'the witch", is no longer being ignored. It certainly did her no harm when The New York Times ran its long profile of her and of her crusade under the headline, "The Case of the Cherry Hill Cluster".

So far, there have been no confirmed cases of vCJD, tied to the consumption of contaminated beef, in the US, except for a 22-year-old woman in Florida in 2002 who was a UK citizen and was thought to have contracted the disease while in England. But it is this blanket denial of any threat to humans by the government that Ms Skarbek finds so hard to believe.

It all began for her in January 2000 when a friend, Carrie Mahan, became ill. She had trouble thinking and even getting the key in her front door. Her deterioration was rapid - she was gone within a month - and doctors determined she had died of sporadic, or classic CJD, after finding tell-tale holes in her brain at a post-mortem examination. It is sporadic CJD that kills about one in a million Americans every year. In about 85 per cent of those cases the exact causes are never determined. Otherwise, deaths are attributed to genetic defects or contamination from instruments used in surgery. But what is proven, according to scientists, is that sporadic CJD has no link to BSE.

There was something odd, however, about Ms Mahan's case. CJD, which attacks the brain by opening tiny holes, was first identified in the 1920s by two German scientists after whom it was named. The sporadic version nearly always strikes people older than 60. Ms Skarbek's friend was 29 when she died. She was also black and there have been very few cases of blacks contracting the illness.

What did not concern Ms Skarbek at the time was that Ms Mahan had worked at the racecourse. Then, three years later, in June 2003, she was reading the obituaries in the local Burlington County Times and fell upon an entry for a Carol Olive, who had also died of CJD. Ms Olive had also worked at the racecourse. "That's when I almost fell over," Ms Skarbek told the NYT. "Suddenly we had two victims out of a hundred administrative employees."

From there on, there was no stopping her sleuthing. In the ensuing months, she has transformed herself into the BSE incarnation of Erin Brockovich, whose crusade to uncover industrial contamination of her community's water supply became a Hollywood film staring Julia Roberts. What she has found since that June day last year has only reinforced her conviction that she is on to something terrifying.

After finding the Olive case, Ms Skarbek began reading moreobituaries.She came upon John Webber, who had died in 2000. The medical examiners said he had been a victim of sporadic CJD. She decided she had to ring his family. What followed, she said, was her "eureka moment". The brother of the dead man confirmed he had held a season pass to the race track and that he "ate there at least once a week".

Ms Skarbek has given the CDC, as well as the New Jersey health department, documentation that allegedly demonstrates that as many as 13 people who have died in her corner of New Jersey and in nearby Pennsylvania and who were diagnosed with sporadic CJD, were all either workers at the closed race track or were patrons there and at its cafeteria. Another six deaths in the region seem to fit the same pattern, she says, although she is still trying to confirm whether at least two of those victims had connections with the track.

"There will be more," she insisted this week. As for the decision of the two senators to get involved, she could not be more pleased. "I think it's wonderful that they're getting involved."

The involvement of the government is vital, she said, in part because she cannot get access to important records on her own, including many of the death certificates. "I've gotten as far as I have just through word of mouth," she conceded. But she is worried that the CDC will approach it from the wrong angle. "Essentially they are trying to disprove the cluster theory," she said, "rather than going out and checking all the death certificates, which I don't have access to, and finding out if there are any more CJD deaths connected to the track."

There is a long period during which all the victims visited the track, from 1985 to 2004. This means that the supposed statistical anomaly - the argument that so many cases of CJD could not happen in such a small area in such a short time - becomes less convincing. However, she suggests that the time when they all ate at the track is much shorter. For now, she puts it between 1988 and 1992, but she hopes to be able to show eventually that in fact they may have eaten there during a much shorter time period, perhaps even a few weeks.

"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 ... As we get more people who ate at the track and died of this, I think we'll be able to narrow it down to the month."

It has not escaped Ms Skarbek that she may be emerging as the latest underdog heroine who uncovers what may be a government conspiracy of silence of unprecedented proportions. In talking to the NYT she fancied herself as the law student in the Pelican Brief who exposes just such a cover-up and even quoted a line from the John Grisham book, "So you're the little lady who started this great brouhaha?" The Cherry Hill cluster is starting to attain brouhaha dimensions. But with Ms Skarbek, we are still a long way off from knowing whether she is on to something real.