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The coincidence of five cases of vCJD, a new, rare but fatal disease, associated with the small village of Queniborough might have provided further evidence about the source of this disease commonly associated with BSE. What it did illustrate was the challenge of handling complex and sensitive scientific information in a public forum. (750 words)

Variant Creutzfeld-Jakob disease (vCJD) was first recognized as a new disease in 1995 when the BSE (Bovine Spongiform Encephalopathy), or mad cow disease, was still prevalent in Britain. It is similar to the long know conventional CJD which affects older people. Victims of vCJD are, however, generally under 40.

In addition to the timing factor, BSE and vCJD are similar in nature. Both are believed to be caused by prion proteins, molecular organisms which are able to influence the growth of brain tissue and thereby cause it to misfunction.

Although vCJD has been subject to £140 million in research over six years, the link between BSE and vCJD is assumed rather than proven. A range of different animals has been infected with BSE using a variety of laboratory techniques. But the final link to humans is, of course, not something that can be the subject of research, in a lab or elsewhere.

When five people with connections with one small rural community died of the disease, it was hoped that some factor other than chance might be found.

By January 1999 when the fifth person associated with Queniborough developed vCJD symptoms, there had been just more than 40 cases of vCJD in UK's population of 60 odd million. The chances of contracting the disease were in the region those of being hit by lightening. But in the parish of Queniborough, population about 1,800, the odds were clearly significantly shorter.

The hope, or expectation, was that something unique or near unique about Queniborough implicating all five people would provide evidence of how the disease was spread.

The Leicestershire Health Authority having drawn blanks on a number of potential causes from blood transfusions to baby food, identified beef or more precisely the way beef was raised, butchered and sold as holding a potential answer. The study compared where the victims or rather their families purchased their beef in the 1980's when all five victims lived in the area, with a random selection of other residents, http://www.leics-ha.org.uk/cjd/cjdbrief.htm.

The study was able to provide "a biologically plausible explanation" for how four of the five could have been exposed to BSE infected beef. These four families purchased beef from local independent butchers' shops who sold beef slaughtered in local abattoirs.

These abattoirs, in common with most small abattoirs across the country at the time, wiped sides of beef rather sprayed them with water as the final process before shipping. This together with the pithing could have caused potentially infectious BSE tissue from the brain to be spread over the carcases.

The study further concluded that if the explanation was correct the incubation period for vCJD was from 10 to 16 years the time from earliest to latest exposure of the victims. This in turn suggests that the incidence of vCJD has several years to run.

All this sounds very plausible. But beyond the challenges of studying social events of more than 10 years ago recognized by the researchers, the study failed to identify why it was Queniborough rather than one of hundreds of other similar rural communities up and down the country that was afflicted. Or, indeed, why epidemiologist have nor identified rural residency as a factor in the incidence of vCJD.

But, at the end of the day the biggest challenge for the Leicestershire Health Authority was relating this information first to the people of Queniborough in a manner that would be understood and would not create unnecessary alarm. The secondary challenge was that of handling the press and media who could be expected to put various and sundry spins on the information provided and who the people of Queniborough were just as likely to believe.

In providing the village with two weeks notice of the release of the findings, the authority attempted to play down their findings by suggesting they were "extremely obvious" and "staring us in the face."

The authority did not really have the option of saying that they had not really found anything conclusive. The people of Queniborough were expecting more and elements of the press and media would have surely spun a believable if not legitimate cover up story.

March 22 2001