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Britain: Report links CJD cluster to local farming and butchery practices

An official investigation of a cluster of five deaths from variant Creutzfeldt Jacobs Disease (vCJD) in the village of Queniborough concludes that local farming and butchery practices were the most likely source of the infection.

In the UK there have been 95 confirmed or probable cases of vCJD, the fatal brain wasting disorder related to BSE (Bovine Spongiform Encephalopathy) or Mad Cow Disease.

Leicestershire Health Authority carried out the investigation into the vCJD cluster in Queniborough, a village about 100 miles north of London with a population of only 2,297. Three of the victims—Stacey Robinson (19), Glen Day (35) and Pamela Bayless (24)-all died within months of each other in 1998. In May 2000, a 19-year-old man who lived nearby died, followed by 24-year-old Christopher Reeve last September.

Pamela Bayless's father Arthur said when he noticed how “Glen, Stacey and Pam all died within months of each other, I spoke to Glen's dad and we discussed how strange it was that it was all in Queniborough. It's such a rare disease.” He campaigned for an inquest into his daughter's death, but without success. Then in October 1998, the local newspaper, the Leicester Mercury, reported that three people had died in Leicestershire, two in the same village, and contacted the Health Authority for comment. The official response in 1999 said the deaths were a coincidence and posed “no cause for alarm”. The investigation only came about due to the insistence of the families of the young victims.

The Health Authority's report concludes, “The people who had vCJD were exposed to the BSE agents through the consumption of beef which had been processed from butchers. There was a risk of cross-contamination of bovine brain material during the boning and cutting process in those premises where the skull was split to remove the brain”. It suggests a moderately high incidence of BSE in the area originated in the mid-1970s as a result of farmers giving their cattle meat and bonemeal feeds containing recycled animal tissues. In addition, the cattle incubated the disease longer because they ate bonemeal from 6 days old, rather than the more usual 6 months, and were slaughtered later because they were slower-growing Friesians.

It was also usual practice in the early 1980s before BSE was discovered for small abattoirs in the area to sell cattle heads to local butchers who would split the skulls, cut out the brain—the most infectious organ containing BSE-and remove the remaining meat using the same knives they employed to do their other butchery. Thus local farming and butchering practices created a higher than usual risk of meat being contaminated with the BSE agent, the report concludes.

There have been conflicting reactions from several leading scientists to the Leicestershire report.

Professor Roy Anderson, an expert in BSE epidemiology, says the investigation has come to "a very plausible explanation," and was important for establishing an incubation period for the disease in humans of 10-16 years. However, "It is important not to over-interpret this cluster. It is significant, but it is only five cases," Anderson said.

Professor John Collinge, a member of the committee advising the government on BSE warned: "For me, the main finding from this report is that the significant exposure appears to pre-date 1985. That sent a little chill down my spine, certainly. It fits with our estimates that we have been making of the likely incubation periods of BSE in humans.

"The cases we are seeing at the moment are by definition those with the shortest incubation periods and the average incubation period could well be in the region of 30 years, Collinge told the press. "The upper limits of the modelling at the moment are in the region of one to two hundred thousand—that is one extreme of the possibilities—but we may see thousands, or tens of thousands."

Professor Hugh Pennington, professor of microbiology at Aberdeen University investigated the E.coli bacteria deaths resulting from contaminated meat in Scotland two years ago. Pennington said the report provided "a very plausible story and underlines what we know already, but does not explain why there was a cluster, because I do not think what they were doing was unique. It also does not explain why the victims were so young. It is very important data and very useful to have, but it has not unlocked the secret of CJD."

The most critical response came from Professor Richard Lacey, who was vilified by the then Conservative government and the media when he first exposed the BSE crisis and its implications for human health. Lacey said the report has scapegoated local butchers without addressing the real causes. He told the BBC, “They have no idea, it is just guess work, speculation. The aim is to reassure, rather than get at the truth. This has been the whole basis of CJD over 15 years—not to get at the truth, but to reassure in the short term.” He pointed out that vCJD is difficult to catch through the oral route (i.e. by eating contaminated meat), "It is not clear exactly how it spreads, it could be more than one way."

This dissent and caution among experts in the field of BSE is in sharp contrast to the official “spin” that has been put on the Leicestershire report, which came across more as a public relations exercise designed to reassure the villagers of Queniborough. The presentation of the report gave an overall impression that the deaths of five young people in the village were due to a series of unlucky coincidences.

But the farming and butchery practices in Leicestershire were not unique. Farmer's wife Margaret Winterton who has lived in the village for 18 years said, “I don't think it is the answer. I think a lot more investigation is necessary. I think the butchering practices that they have explained have been carried out all over the country—so why is there the cluster here?”

In response to the report's finding that butchery methods had played a role, local butcher David Clarke said, “You are talking about something from the Eighties, not something related to the present time.”

But ever since the emergence of BSE in cattle, the main thrust of government policy has been to protect the profit interests of the meat industry, something that has not essentially changed even when a direct link with vCJD in humans was admitted.

It is reported that health officials are also investigating the death of three people with vCJD in the Yorkshire village of Armthorpe, and of two men who lived within 250 metres of each other in Greater Manchester.

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