British government warns variant CJD deaths may rise to 250,000

By Julie Hyland
3 November 2000

The Blair government has warned that variant Creutzfeldt Jacobs Disease (vCJD), caused by eating beef infected with "mad cow disease" (BSE-Bovine Spongiform Encephalopathy) could claim as many as 250,000 lives. This is double the previous estimate of 136,000 possible deaths and means that the government is now working on a "worst case scenario" of one in every 250 people in Britain dying from the disease.

Variant CJD is a fatal brain wasting disease beginning typically with depressive-type symptoms, lack of coordination and unspecified pains, before progressing to complete helplessness, blindness and certain death. As yet there is no proven means of arresting the disease's progress, let alone curing it.

The revised estimate was made public just days after Judge Lord Phillips published the final report of the government-convened inquiry into BSE. After a two-year investigation, Phillips' report did not make any criticisms of the food industry, whose practices lie at the heart of the scandal, or of former government ministers, despite acknowledging their efforts to cover-up the crisis.

Phillips conclusion that no one could be held responsible for the worst food health disaster in Britain was not surprising. The incoming Labour government, which convened the Inquiry in 1997, intended it mainly as a means of defusing public anger over a crisis that had played a significant role in eroding support for the previous Conservative government.

The official BSE report was followed by the announcement that the Labour government would ensure a care and compensation package to the families of those who died. Agriculture Secretary Nick Brown, speaking on BBC TV's Breakfast with Frost programme, admitted that the number of people who will die from vCJD could grow "much, much larger". He summed up official indifference to the terrible fate that could befall many families by claiming the numbers were "just predictions", whilst taking the opportunity to promote the British beef industry. "I eat British beef, I know British beef is amongst the safest in the world," Brown stated.

Also speaking to the BBC, Professor John Collinge, of the BSE Advisory Committee, took issue with the "false optimism and wishful thinking, which has bedevilled", the BSE investigation "for too long." "We might be seeing an epidemic that involves hundreds of thousands of people. Let's hope that's not the case, but it's still possible", he said.

Putting the risks into context, microbiologist and leading CJD expert Dr Stephen Dealler said on average people in the UK had eaten 50 meals made from the tissue of an infected animal. "At the moment the number of cases of CJD we are seeing are doubling every year. If they double for a long time then the numbers are in millions, if they double for just a few years then the numbers are in thousands. At the moment it is very difficult to know," Dealler said. The Report from the official BSE Inquiry found that a cow could be infected with BSE by eating contaminated material the size of a peppercorn.

Government adviser Professor Roy Anderson said that news that a 74-year old man had died from vCJD last year—most known victims have been younger—necessitated a major re-evaluation of the possible scale of the crisis. Anderson's earlier computer predictions had forecast that up to 6,000 people had been infected between 1980 and 1996. That figure could now rise as high as 130,000 as there is concern that many elderly people with vCJD could have been wrongly diagnosed as suffering from Alzheimer's disease, which has similar symptoms.

Fears of a vCJD epidemic have also been heightened by news that a cluster pattern of cases may be occurring in a former South Yorkshire mining village. Accountant Sarah Roberts, 28, of Armthorpe, Doncaster, died in September only nine weeks after she was diagnosed with vCJD. Her former neighbour and friend Matthew Parker, 19, who attended the same school, died of vCJD in 1997.

It has now been revealed that a third victim of vCJD, former RAF policeman Adrian Hodgkinson, 25, had made regular visits to Armthorpe to see his grandmother every weekend between 1972 and 1986. If a link is proven it would indicate that the three victims may have been infected by the same source. The CJD surveillance unit at Edinburgh University is exploring the possible link. If Doncaster does reveal a cluster it will be the second such grouping in Britain. Last month, a fifth person in the Leicestershire village of Queniborough died from suspected vCJD, following the deaths of four others who had lived there or had connections with the village.

Despite this, Prime Minister Blair continued to claim that the issue was one of "finding the balance between risk and public protection measures". This is a "cost-effective" approach, which argues that any regulatory measures that may interfere with profits are only justifiable when a certain death toll has been reached. This was the argument utilised by the Conservative government when BSE first emerged in order to reject intervention into the food industry on the grounds that the risk to public health was "minimal". So for example, when the government was working previously on a "central figure" of 6,000 deaths from vCJD it was not considered cost-effective to extend the ban on feeding cannibalised remains to livestock or introduce further safety measures, for fear of outraging major land and food interests. These had already reacted angrily to even banning the sale of beef on the bone, claiming it represented a serious threat to civil liberties.

Even now, faced with a mountain of evidence showing the link between BSE in cattle and vCJD in humans, there are those who still view the BSE crisis as a virtual conspiracy against the British beef industry. An editorial in the Daily Telegraph last week fulminated, “While it is still unclear whether 77 horrible vCJD deaths are connected with the BSE crisis, other disasters can be directly relate to the whole affair, chief among them the extremist ban of beef on the bone and the collapse of the British beef industry.”

It is a sign of how reluctant the government has been to enforce the necessary safety measures in the food industry that only now is it considering introducing a complete ban on feeding animal remains to other animals—a major factor in the rapid spread of BSE in cattle. As yet the current ban on feeding recycled meat and bone meal does not cover many animals including pigs, poultry and fish or the use of cows' blood in feed manufacture because blood was deemed free of the infective prions associated with BSE.

The Food Standards Agency (FSA) made the recommendation to extend the ban after scientists proved in laboratory tests that BSE could be transferred to sheep. Although there is yet no evidence of BSE infecting sheep in the field, there are concerns that it could be masked by scrapie, a disease that has similar symptoms and has been present in the national flock for 200 years without apparently proving any risk to humans. Many scientists believe that BSE originated as a mutation of scrapie after it crossed the species barrier between sheep and cows through the ingestion of cannibalised remains in feed or shared grazing land. An FSA spokesman said urgent screening was required to establish any risk, but this would take years to reach any conclusion. In the meantime, the Ministry of Agriculture is to draw up contingency plans for dealing with any future discovery of BSE in sheep, including a blanket ban on consumption and the slaughter of millions of animals.

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