December 1998 Mad Cow News
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CJD Watch goes operational
Pentosan: new drug hope for CJD?
MAFF Goes to the Dogs ... a Play in Three Acts
BSE fibrils found in 19 hunting dogs
Threats to meat inspectors
Surgeon killed by CJD-infected growth hormone
Beef-on-the-bone ban may be lifted
Beef dishes making their way back to British menu
BSE fears lead to ban on British spleens
Surge in Irish BSE cases

CJD Watch goes operational

Contact CJD Watch or visit Web site 30 Nov 98
CJD Watch --  a Public Awareness Project. 
Are there really only 250 cases per year of CJD in the US? A lot of people doubt this. Now families affected by CJD have begun collecting their own data and posting it on the Web. Individual coordinators for each state collect case reports in newspapers and contact affected families to gather statistics about the age of the victim, duration of illness, occupation, age at death, state of residence, how diagnosis was made and so on. These are assembled by the national coordinator, Barbara Mathews. CJD Watch accepts international cases as well.

New drug hope for at risk CJD groups

 Wed, Nov 25, 1998 By Suzanna Chambers, PA News
A common drug to protect those thought to be at risk of developing CJD, the human form of mad cow disease, is being considered by the Government. Proposals to prescribe Pentosan have been discussed by the Department of Health and scientists are hopeful that it could benefit people who ate infected beef at the end of the 1980s.

Dr Stephen Dealler, a microbiological at Burnley General Hospital, Lancashire, told BBC 2's Newsnight that he believed it was worth trying the drug in high risk groups, including the children of mothers who died from new variant Creutzfeldt-Jakob (CJD). The disclosure comes on the third anniversary of the death of mother of three Michelle Bowen, who died of nvCJD at the age of 29.

Dr Dealler, whose research has been funded by the Government, said the drug could also be given to people who need blood transfusions. He said: "It's an amazing drug ... there are some obvious groups that would benefit from this kind of drug ... Michelle Bowen's child -- she had a Caesarean section -- she died shortly after of nvCJD.

"I would expect her child to have been exposed to the disease, just like people who may have been inoculated with the disease through blood transfusions, gammaglobulin or anti-D. It is very difficult now ethically to avoid it."

Pentosan has been tested successfully on mice infected with scrapie in the early 1980s. "The fantastic thing was you could actually inject just a single injection of the drug into a mouse that was already infected with scrapie, in this case, and you'd find that some of those mice did not go down with the disease ... the infectivity in the mouse had completely gone, it was as if the mice had been cured," Dr Dealler said.

"I think we just have to say the evidence is good enough, now we must go ahead ... that sounds like a very good opportunity I doubt we should miss this one...", he told Newsnight. It is not the first time a proposal to carry out research into Pentosan has been submitted to the Department of Health, the programme disclosed.

Chris Bostock, director of the Government's Institute of Animal Health and a member of SEAC, the committee that advises the Government on BSE and nvCJD, would like to see research into whether Pentosan has the same effects on mice if given orally instead of injected. He is also keen to see at what dose the licensed version of the chemical might work.

"In the 80s ... it was fundamentally a disease of sheep ... I think when one is now faced with the prospect -- the hypothetical prospect -- of quite a large number of humans infected with these diseases then that if you like changes the balance of where you might put your research effort," he told Newsnight. "This is a very clear biological effect that might be translatable into some form of prophylaxis or some form of treatment."

But he thinks it is too early to prescribe the drug to people. "I don't believe that we yet have a good enough idea of how these drugs work to know how you set about designing a proper clinical trial."

Philip Comer, of Det Norske Veritas, risk assessment advisors to the Government, said it was definitely worth investigating further into the effects of the drug. "It appears that Pentosan could be a useful risk reduction measure and certainly is worthwhile investigating further, but I think there's quite a lot of work that needs to be done to demonstrate that," he said.

Summaries from Dealler's web site:

Independent 26.11.98

Cystitis drug may help to prevent CJD. Explanation that pentosan had large amounts of clinical trials in humans because of its use in interstitial cystitis. From this is was known that the risks were minimal. It explained the underlying mode of action of the drug but little else.

Guardian 26.11.98

Common drug may be shield against CJD. Dr. Dealler is largely quoted as explaining that pentosan polysulphate, because of its amazingly low toxicity should be considered as a prophylactic against nvCJD in blood and blood products. None of the details as to why the toxicity is so low are included but the problems in proving that it actually works in humans are discussed. This basically shows that we cannot carry out trials in humans until it is too late.

Newsnight 25.11.98

Pentosan polysulphate as potential prophylactic against nvCJD. This was as a series of interviews with people that have either been put at risk or might be. Then Dr. Dealler was interviewed about the drug, only the major factors of 'it worked in mice' were included greatly. Chris Bostock indicated that he felt that further research should take place over the next few years and then we should consider using it.

Comment (webmaster):

This story has gotten way ahead of fairly meagre data. Why the rush of interest now in an old compound? I get almost daily letters from concerned individuals who are ready to try any drug mentioned favorably on the Internet. Someone could start a very large web site that merely listed all the compounds which have back-fired seriously over the years. This compound cannot even be tested in humans without early diagnosis. Let's not trade one catastrophe for two.

Comment (Roland Heynkes):

"This is true and already in:

Ehlers,B.; Diringer,H.
Dextran sulphate 500 delays and prevents mouse scrapie by impairment of agent replication in spleen
Journal of General Virology 1984 Aug; 65 (Pt 8): 1325-30.
The authors wrote that Pentosansulphate is reliably helpful only if given at the time of infection."

MAFF Goes to the Dogs ... a Play in Three Acts

30 Nov 98 webmaster [based on actual historical accounts of MAFF research into BSE in dogs]
Act 1, Scene 1

Rural England. Men milling about on horseback in red coats and riding boots holding ale tankards. Hounds baying in distance.

Servant: "My Lord, the hounds are baying at imaginary game, but they are still in the kennel. Methinks it is the BSE cows that we have thrown them."

Master: "Very well, then. Cull a few dozen of our maddest dogs and toss the heads in the back of that lorry. They are wanted at the lab at Weybridge."

Servant: "But sire, this be Friday and we cannot get the hounds to Weybridge before Monday. Their brains will be pure mush."

Master: "That is precisely the point and exactly how they want them."

Act 2, Scene 1

Central Veterinary Laboratory in Weybridge, England. Men and women milling about in white lab coats and mud-caked boots holding beakers. BBC radio in background going on about 7th anniversary of BSE epidemic.

Lab Tech: "Doctor, these hound heads have gone rancid. Their brains are pure mush. They did not follow the cow and sheep brain protocol we gave them. All they needed to do was put them on ice."

Junior Veterinarian: "Indeed, whatever shall we do with 444 hound brains that are not fit to study? I don't suppose my supervisor will want to lose a week and start afresh. Carry on, then -- prepare all 444 brains for electron microscopy. Perhaps some fibrils survived; the prion protein is almost indestructible."

Act 2, Scene 2

Central Veterinary Laboratory senior staff lounge. Men milling about in tweed jackets and brown jogging shoes holding cups of tea.

Junior Veterinarian: "Sir, we have found scrapie fibrils in 19 hounds. The fibrils are just like those we have seen in thousands of cows. It would be an easy matter to confirm our findings with prion antibody."

Senior Veterinarian: "I simply cannot find the funding to carry out immunohistochemistry. Nor do we have room to store any slides. Indeed, we don't even have the postage to send them to Collinge in London. You really must get back to your work on swine flu."

Junior Veterinarian: "Shall we be publishing our result? There were no fibrils in control hounds. And the appearance of the fibrils was quite characteristic."

Senior Veterinarian: "No. Quite frankly the results are inconclusive. Because the brains were mush, you could not correlate fibrils with spongiform change. But there is no need to destroy the lab notebooks because England will never have a Freedom of Information Act."

Act 3, Scene 1

London ministry. Men milling about in tweed jackets and black wingtips holding large tumblers of single malt scotch. Flames shoot up from heavily marbled steaks on the grill.

Minister of Agriculture: "My lords, the very hounds have gone mad. 19 of 444 confirmed though we had them all go to mush. The junior veterinarian has been dismissed."

Minister of Health: "My good chap, what does it matter, we British do not eat dogs. A fallen hound goes to pet food. Only the elderly are put at risk. And the waiting list is so long they will never come up for diagnosis."

Minister of Agriculture: "My point is that the junior veterinarian took copies of the lab notebooks. And there is email everywhere. There is potential for unfavorable press and delays in resuming exports."

Minister of Information: "No one dares breach the Official Secrets Act. To speak openly about BSE in dogs borders on treason. I daresay another press release about a species barrier is sufficient."

Minister of Agriculture: "No. That fellow in America will bring up the mink in Wisconsin eating downer cows again. Mink prion sequence is too close to dog. We shall look like fools -- cannot the Internet be shut down?"

Minister of Health: "Very well. We shall do nothing. That strategy has served us well. And don't send a memo round -- this meeting never happened."

Minister of Agriculture: "But what about the BSE Inquiry? Their people have been all over the ministry, photocopying. They surely know about the hounds."

Minister of Information: "Admit to 19 hounds on the MAFF web site. Stress that it is too late to repeat the experiment, that no material was saved, that there are now ethical issues involved in deliberately exposing dogs to the BSE agent though there weren't back then. No health precautions for cats were recommended by unnamed experts. SEAC was duly mis-informed after it was too late. Etc., etc., you know the drill by now. Dogs protected in 1990 by the exclusion from all animal food, including pet food, of specified risk materials."

Minister of Agriculture: "But these dogs are from 1992."

Minister of Information: "My dear fellow, have another scotch and tuck into your steak. There is nothing quite like a sizzler from the Pampas."

Curtains.

BSE fibrils in 19 hunting dogs

30 Nov 98 MAFF chronology of their mad dog research

"DOGS

Q. What was MAFF's Hounds Survey?

A. Following the confirmation of BSE in a cat in 1990 the Government decided, on advice from the predecessor to SEAC, to undertake a survey of hunting hounds. This was not, as recently reported by the BBC, because of reports of abnormal behaviour in hounds, but a random survey to see if there was any evidence at all that a TSE could be found in dogs.

Q. Why were hunting hounds chosen?

A. Because they are fed on raw material from fallen farm animals which could before 1988 have included animals with BSE. Also before 1990 the material could have included the brain and other parts of the cow which contain infectivity. Hounds were therefore a group at potentially much greater risk than ordinary dogs.

Q. What were the results?

A. The survey was concluded in 1992 and looked at the brains from 444 hounds. These were not hounds which had died of natural causes but hounds which had been culled from hunting packs for other reasons. The results were disappointing partly because of difficulties in ensuring that the brains did not start to deteriorate between the death of the hound and its examination. In 19 cases fibrils were found which were somewhat similar to fibrils found in other species of animal with TSEs. But this did not correlate with spongiform change in the brains of those hounds and the conclusion was that it could not be taken as firm proof of a TSE.

Q. Why did the Government not publish the survey?

A. Quite frankly the results were so inconclusive that it would not have added anything to the sum total of human knowledge.

Q. Why was no further work undertaken?

A. Dogs are covered by the ongoing free service which the Veterinary Investigation Service of MAFF offers to veterinary surgeons. Animals, including pet animals of any species, which the private veterinary surgeon suspects might have symptoms of a progressive neurological disease can be examined for evidence of a spongiform encephalopathy without charge. Laboratory diagnosis of a spongiform encephalopathy in any species has been notifiable since November 1994, thus ensuring that cases with atypical symptoms are not missed. That is the service which identified the 85 cases in cats and has examined 15 dogs since 1990. No TSEs were found in the dogs.

There are several reasons for doing further scientific research on dogs. Against this the ethical issues of deliberately exposing dogs to the BSE agent have to be considered.

For example:

1. To protect animal health

New work was not necessary because in 1990 when FSE was found in cats action was taken to protect both cats and dogs from potential exposure by extending the ban on the use of specified offals in human food to all animal food. The Pet Food Manufacturers Association had in fact introduced a ban on this material in commercial petfood since 1989. As the Government was not seriously considering relaxing the ban on dog food whilst maintaining it on cat food there was no animal health reason to conduct special experiments to see whether dogs were susceptible.

2. To protect public health

There could be 3 possible reasons doing such research.

(a) Because the species concerned is normally eaten - clearly this is not the case with dogs.

(b) Whether special precautions would be necessary because dogs were kept as pets - the same issues would arise in relation to cats where disease was known and where no special precautions have been advised at any time by the experts.

(c) Because it would significantly extend our scientific knowledge of the disease leading eventually to a better understanding and potential benefits to human and animal health. It is known that spongiform encephalopathies can be established in a range of species and is not confined to the cow. There would be no significant new insights into the disease if we could establish experimental disease in the dog. The main species used for studying the disease is the mouse and most progress is made by studying in increasingly greater detail the disease in special strains of inbred mice. The main argument for looking at other species is whether they could tell us more about the potential risk to man and that involves looking at species which are closely related to man, such as primates. Some work has been done on this and other work is being considered by overseas scientists. MAFF's preferred route is to use genetically engineered mice with human genes which may well teach us more about how the disease might behave in people without the ethical problems raised by working in higher primates.

Q. Will MAFF reconsider and do further work on dogs?

A. We have no plans to do so.

Q. Were SEAC aware of these results?

A. Yes. They considered this in June 1995 and agreed with the conclusion that no further action was justified at that stage to resolve the issue.

Q. Should not more action have been taken to warn dog owners?

A. Action was taken both in relation to cats, where disease has been established, and dogs where it has not, to ensure that they are not exposed through food. That action was taken in 1990 and we do not believe that most dog owners would feel it right that Government should conduct experiments on dogs unless there was a very strong justification to protect either public health, animal health or to make significant new scientific advances in the studies of these diseases.

Q. Are dogs at risk?

A. Dogs have been protected since 1990 by the exclusion from all animal food, including pet food of specified risk materials (SRMs).

Q. What about the Norwegian case?

A. The case which has been reported from Norway (which has not been confirmed as BSE) is an 11 year old dog. We have been informed about this by the Norwegian authorities and have offered whatever help they need. Our understanding is that, although they have found evidence of spongiform change in the brain the distribution of vacuoles and pattern of staining when antibodies against PrP were applied suggest that the dog was not affected with a transmissible spongiform encephalopathy. Further material has been provided to experts at the VLA who are in agreement.

The Norwegian authorities have not been able to secure the release of the data for publication."

Curb on threats to meat inspectors

November 30 1998  London Times BY MICHAEL HORNSBY
THE Government promised yesterday to crack down on violence and intimidation against meat inspectors by slaughterhouse managers and their staff. More than 50 per cent of inspectors who took part in a survey by Unison, the public sector union, and the Meat Hygiene Service (MHS) complained of being verbally abused, threatened with knives and shotguns and even physically attacked.

"I am deeply disturbed to hear continued reports of intimidation of Meat Hygiene Service staff," Nick Brown, the Minister of Agriculture, said. "It is totally unacceptable that public servants employed to ensure the safety of meat should be treated in this way. The Government will take firm steps against this."

The release of the survey results comes as Britain's farmers try to recapture foreign markets for their beef. Government assurances that abattoir controls are now being properly enforced played a key role in getting the EU to ease the beef embargo.

Ben Priestley, Unison's national officer responsible for negotiations with the MHS, said the 1,000 inspectors who were members of the union faced abuse and threats on an almost daily basis. In the worst cases people have been threatened with shotguns and knives," he said.

Eric Hoyle, who spent 15 years as a meat inspector, believes the stress of the job was a factor in the onset of ME (myalgic encephalomyelitis), or "yuppie flu", which forced him to retire two years ago at the age of 48. "On one occasion I was physically attacked," he said.

Mr Hoyle said he believed the threat of intimidation meant that many inspectors were inclined to turn a blind eye when they saw a problem so as to avoid a confrontation with the abattoir manager. Of the 1,540 questionnaires sent out to inspectors 770 came back and of those 409 reported incidents of violence or intimidation. The Federation of Fresh Meat Wholesalers, representing most of the big abattoirs, dismissed the reports of intimidation as highly exaggerated.

Surgeon killed by CJD-infected growth hormone

Wednesday 11 November 1998 Electronic Telegraph By Paul Stokes
A promising surgeon died from Creutzfeldt-Jakob disease which he contracted through contaminated growth hormone injections he received as a teenager, an inquest heard yesterday. Described as a meticulous consultant orthopaedic surgeon destined for international recognition, Neil Kreibich died at the age of 37 unable to control his hands. His widow, Liz, said after the hearing how he died nine days after the first birthday of his youngest son Robert, but he had never been able to hold him because of the debilitating disease. He leaves two other children - Anna, now six, and William, four.

The Department of Health which has been found liable for his death and Mrs Kreibich, a former nurse, is awaiting compensation. So far, 27 people are believed to have been affected by CJD from contaminated human growth hormones.

Dr James Ironside, an expert in CJD, told the Newcastle inquest that Mr Kreibich died last year from a different type of the disease than that linked to the human equivalent of Mad Cow Disease which causes dementia. He said: "This is a completely separate matter . . . the damage was particularly in the part of the brain called the cerebellum, which involves balance and co-ordination." Mr Kreibich eventually reached a height of 5ft 6ins after the hormone treatment, but the practice was stopped in the mid-1980s when the dangers from contamination began to emerge.

Recording a verdict of misadventure, David Mitford, the coroner, said the treatment had been done with good intentions. He said: "I'm quite satisfied that the cause of Mr Kreibich's death was CJD that was directly associated with the administration of the growth hormone." Mrs Kreibich, of Newcastle, said her husband's illness began with a slight unsteadiness when walking. Within a year he had to leave his job. She said: "His hands which had been operating and doing fine surgery just over a year before were now useless to him."

Ian Pinder, senior orthopaedic surgeon at the Freeman Hospital, Newcastle, where Mr Kreibich worked, described him as having "outstanding clinical acumen and meticulous surgical technique". He said: "I believe he would have achieved an international reputation."

Brown hints beef-on-the-bone ban may be lifted

 Fri, Nov 27, 1998 By Helen William, PA News
Agriculture Minister Nick Brown today hinted that the beef-on-the-bone ban may be lifted by Christmas. In an interview with The Times he said: "I will have something to say very soon. It's clearly right to consult others first then make the statement rather than say what I want the statement to be then consult others."

A lifting of the ban, a year after it was imposed, would mean that T-bone steaks, beef ribs and oxtail could be back on sale by early 1999 but first Mr Brown has to consult the European Commission and government chiefs. Expectation that the ban could be lifted has been prompted by a report by the Spongiform Encephalopathy Advisory Committee, the Government's scientific advisers, due to be issued on Monday by Mr Brown.

It is expected to say the chances of humans contracting CJD, the human variant of mad cow disease is small. Mr Brown also said the Government will not be carrying out a proposal to stop butchers buying turkeys from farmers which still have their head, feet and giblets in place.

Beef smuggling made easier

 Listserve opinion 29 Nov 98
"One further reason for me not to lift the ban against british exports now is that it makes smuggling much easier. Remember the fiasco last year about a unit of the British army helping load the boats? It was never entirely clear that this was a rogue operation as said at the time.

What happened before is the beef got shipped to Russia or eastern Europe, than trans-shipped to Italy, then exported to Germany (though it could have ended up anywhere). I would guess that some creative paperwork accompanied each movement. Now it might just be mixed in with 'legitimate' exports as this would bring a better price.

The concern is thus only partly with current beef. The real issue may be warehoused beef from the bad old days diverted from the incinerator queue. In business, they call this inventory shrinkage and of course a business would track this with periodic outside audits. Where is the government data pertaining to tonnes of beef officially in storage minus tonnes of beef actually in storage. The attraction of warehoused beef is of course its bargain basement price (might get paid to take it even).

Exports won't be resuming until early summer of 1999. A lot could happen between now and then. Let's hope a mountain or two of beef gets incinerated."

Beef dishes making their way back to British menu

November 30, 1998 By GERRARD RAVEN, Reuters
LONDON - Agriculture Minister Nick Brown said Monday he hoped to put beef ribs back on British dinner tables now that scientists reported the risk from mad cow disease was down more than 50 percent. Ribs of beef have been denied to millions of Britons since Dec. 16 last year when Brown's predecessor, Jack Cunningham, banned sales of beef on the bone.

Cunningham's ban followed a report by the government's Spongiform Encephalopathy Advisory Committee (SEAC) which said there was a slight risk of catching brain-wasting Creutzfeldt-Jakob Disease (CJD) by eating in-bone beef. The ban angered farmers and consumers alike.

In a new report sent to Brown last week, SEAC said the number of British cattle infected with mad cow disease which are slaughtered for human consumption next year was likely to be about 43 against around 99 in 1998. Only one or two of these beasts -- against the 2-1/4 million slaughtered every year -- were likely to have high levels of infectivity in their dorsal root ganglia, posing a possible risk to anyone eating their meat on the bone.

"Clearly the time is coming when we can lift the domestic ban on beef on the bone," Brown said at London's annual Smithfield livestock show. "I hope to have something to say reasonably soon." He said the government would first consult its senior medical advisers and the European Union.

Earlier, Stephen Dorrell, Health Secretary in the Conservative government from 1995 until its election defeat last year, appeared before the official inquiry into mad cow disease set up by the new government. Chairman Sir Nicholas Phillips told Dorrell he felt he and fellow ministers had failed to stress the importance of rules designed to keep infected beef out of the human food chain.

The Conservatives introduced the rule in 1989 that specified material, including the spinal cord, must be removed from cattle carcasses by abattoirs before they were sold to butchers. But in November 1995, the State Veterinary Service reported that it had found 17 occasions when spinal cord had been found attached to carcasses after dressing. This was just five months before scientists announced evidence that people could develop CJD by eating beef from animals suffering from Bovine Spongiform Encephalopathy.

The announcement led to a worldwide ban on British beef exports by the European Union which the bloc's farm ministers only agreed earlier this month to lift. The European Commissioner for Consumer Affairs Emma Bonino on Monday told a European Parliament conference on food safety that Britain should be free of mad cow disease by 2003 and criticism of a decision to lift the export ban on British beef was entirely without foundation.

Some 32 Britons have so far been identified as suffering from the new variant of CJD. Most have died.

Phillips told Dorrell that the abattoir regulations were at first said to be "ultra-precautionary." "As time went by, those who knew about the subject attached increasing importance to these regulations as evidence raised question marks over the thesis that this is not transmissible," Phillips said. "We have not found anywhere a stage at which this message was passed to the trade -- 'Looking at it now, the following pointers suggest that the risk of transmissibility may be higher. In these circumstances, it is absolutely crucial that the bits which may be infected are removed'." Dorrell said the Conservative government had regarded the rules as important and expected abattoirs to follow it to the letter.

Public anxiety over bse a `government priority'

PA News Sun, Nov 29, 1998
Trying to quell public anxiety over the escalating BSE crisis was a Government priority, former Conservative Health Secretary Stephen Dorrell is set to tell the BSE inquiry today. Attempts to allay public fears were led by scientists who were continually carrying out tests to discover the extent of the problem, says written evidence from Mr Dorrell, who was Health Secretary between July 1995 and May 1997. Mr Dorrell was Health Secretary during several major developments in the crisis.

These included the development of CJD in a fourth dairy farmer which "obviously raised fears that BSE could be passed on from cattle to man" and the discovery of failures in slaughter houses to remove spinal cord. In October 1995 two teenagers were diagnosed as having contracted CJD, which was later confirmed by scientists as a new variant of the disease.

In his written statement to the inquiry Mr Dorrell says: "At that time, I believe I was unaware of the significance of the age of the two young people and was guided by SEAC's (Spongiform Encephalopathy Advisory Committee) advice. "I believe that it was later, after the SEAC meeting in March 1996, that the significance of the new development became apparent to me."

By November 1995, unannounced State Veterinary Service visits to slaughterhouses had unearthed 17 occasions when spinal cord had been found attached to the carcass after dressing. This was "potentially serious", Mr Dorrell's evidence says. Within days he a total ban on the use of vertebrae from cattle aged over six months in the production of mechanically recovered meat was announced.

Knowing the potential for mass public anxiety about the issue Mr Dorrell says he urged scientists to use ministers as a filter for information on the crisis. He says: "I emphasised that SEAC should give their advice to Ministers, rather than directly to the public. Ministers were under an obligation to ensure that the findings, whatever they were, were presented in a balanced manner. I wanted to avoid unnecessary public concern."

By March 1996 there were nine confirmed and three possible cases of CJD in young people. They considered that the cases could represent a new form of CJD which prompted Mr Dorrell to make a House of Commons statement. Mr Dorrell says he believes that ministers were wary to provide the public recommendations on the crisis without having had expert advice. "My own view was that Ministers were not in a position to make decisions without such advice."

CJD is `worst form of death' - Dorrell tells BSE probe

PA News Mon, Nov 30, 1998  By Jeanette Pearson, PA News
Former Conservative Health Secretary Stephen Dorrell today told the BSE inquiry that it was hard to imagine a worse form of death than the brain disease CJD. Mr Dorrell was Health Secretary between July 1995 and May 1997 when there were several major developments in the crisis.

He told the inquiry: "What we are talking about is a terrible disease, an untreatable disease, it's very hard to imagine a worse form of death than CJD. So it's a very unpleasant risk we are guarding against." He added: "Even now the SEAC (Spongiform Encephalopathy Advisory Committee) finding is still that any trasmitability of BSE to man is the result of beef eating before 1989 before controls were put in place."

The government's position from the start has been that specified bovine offal should be removed and tested outside the human good chain, Mr Dorrell said. Trying to quell public anxiety over the escalating BSE crisis was also a government priority and attempts to allay public fears were led by scientists who were continually carrying out tests to discover the extent of the problem, Mr Dorrell told the inquiry.

During Mr Dorrell's time in office major developments in the crisis included the development of CJD in a fourth dairy farmer raising fears that BSE could be passed on from cattle to man and the discovery of failures in slaughterhouses to remove spinal cord. In October 1995 two teenagers were diagnosed as having contracted CJD, which was later confirmed by scientists as a new variant of the disease.

Mr Dorrell told the inquiry: "At that time, I believe I was unaware of the significance of the age of the two young people and was guided by SEAC's advice. "I believe that it was later, after the SEAC meeting in March 1996, that the significance of the new development became apparent to me."

By November 1995, unannounced visits to slaughterhouses had unearthed 17 occasions when spinal cord had been found attached to the carcass after dressing. This was "potentially serious", Mr Dorrell said. Under cross-examination, Mr Dorrell said: "There were cases of 17 lapses which are 17 too many but I do not think this means that the law was unenforceable."

He added: "The only basis which I felt free to say beef is safe is on the basis that these safeguards were in place and being enforced. Clearly if the safeguards were not being enforced we could not have felt that beef was in the normal meaning of the word safe." He added: "These were people under a statutory obligation to perform a duty and under those circumstances I am not sure what more ministers can do." ....

UK probe chairman attacks BSE advice to abattoirs

Reuters World Report Mon, Nov 30, 1998 By Gerrard Raven
LONDON, Nov 30 (Reuters) - The chairman of the official British inquiry into mad cow disease said on Monday ministers in the former Conservative government failed to stress the importance of rules designed to keep infected beef out of the human food chain.

Judge Sir Nicholas Phillips clashed on the issue with Stephen Dorrell, Conservative Health Secretary from 1995 to 1997. Dorrell told the long-running inquiry that ministers had assumed abattoirs were obeying instructions introduced in 1989 to remove specified material, including the spinal cord, from cattle carcases before selling them to butchers.

But in November 1995, the State Veterinary Service reported that it had found 17 occasions when spinal cord had been found attached to carcases after dressing. This was just five months before British scientists announced they had found evidence that it was possible for people to develop the brain-wasting Creutzfeldt-Jakob Disease by eating beef from animals suffering from Bovine Spongiform Encephalopathy.

The announcement led to a worldwide ban on British beef exports by the European Union which the bloc's farm ministers only agreed earlier this month to lift. Some 30 Britons have so far died of the new variant of CJD.

Phillips told Dorrell that when the abattoir regulations were first introduced, they were said to be "ultra-precautionary." "As time went by, those who knew about the subject attached increasing importance to these regulations as evidence raised question marks over the thesis that this is not transmissible," Phillips said. "We have not found anywhere a stage at which this message was passed to the trade -- 'Looking at it now, the following pointers suggest that the risk of transmissibility may be higher. In these circumstances, it is absolutely crucial that the bits which may be infected are removed'."

Another inquiry member, former senior civil servant June Bridgeman, said other witnesses to it had said they regarded the abattoir regulations as a mere precaution because ministers had been assuring people that beef was safe to eat.

However, Dorrell said the Conservative government had regarded the rule as important and would not have been able to say beef was safe if they had known they were being flouted. Dorrell was the first of a string of former Conservative cabinet ministers, who will give evidence to the inquiry, set up by Britain's new Labour government.

BSE fears lead to ban on British spleens

 Mon, Nov 30, 1998 By John von Radowitz, Medical Correspondent, PA News
Concern over the possibility that BSE-contaminated beef might infect the spleen today led to a ban on using organs from British patients for a diagnostic test. Spleen tissue is used to test for a rare inflammatory disease, sarcoidosis. Specially prepared spleen tissue from someone with the disease is injected under the skin of the recipient patient to see if it produces a reaction. From now on, the Kveim Skin Test will have to rely on spleens taken from donors from countries other than the UK that have a low BSE risk.

Announcing the move today, Health Secretary Frank Dobson stressed it was a purely precautionary measure. He pointed out that the Kveim Skin Test was very rarely used. In the past 12 years only two spleens had been used in the production of the test tissue, called Kveim Skin Test Antigen (KSTA), in Britain. Both donors were still alive and had no signs of neurological illness.

The spleen, a plum-coloured organ lying behind the stomach, produces white blood cells and acts as an emergency reservoir of red blood cells. Experts believe organs such as the spleen and lymph nodes may carry new variant CJD -- the human version of BSE -- as well as the brain and spinal cord. The Government was advised to take action over the possible risk from spleen tissue by the Spongiform Encephalopathy Advisory Committee, which helps shape policy on BSE and CJD.

Mr Dobson said: "The Government has said it will take whatever steps advised are necessary to protect the public from the risk of exposure to nvCJD. Although there is no evidence of transmission via the Kveim Skin Test it is right that we should act immediately on the advice of our experts."

Sarcoidosis, which most often appears in the lungs or lymph nodes, can easily escape diagnosis or be mistaken for several other illnesses. The Kveim Skin Test is one method used to diagnose sarcoidosis in a minority of cases where it is considered clinically appropriate.

Surge in Irish BSE cases

Mon, 30 Nov 1998 The Irish Times, 28 Nov 1998
A surge in the number of reported cases of BSE in Ireland produced the highest monthly total this year. 14 cases were reported, nine more than November of last year. The number of cows found with BSE has increased sharply, moving close to the highest levels detected since the disease was first identified in the State in 1989.

While there was no official comment from the Department of Agriculture and Food, the figure, which brings the total number of BSE cases this year to 73, represents a setback in the fight to eradicate the disease. Cases for the year look set to equal, if not exceed, last year's 80 - the highest annual total on record since 1989.

Detected BSE cases remained under 20 annually until 1996 when it was announced in the House of Commons in London there might be a link between it and nvCJD, a similar type of disease found in humans. Cases reported to the Irish authorities from August 1996 doubled and 18 cases were recorded that November.

Since then, the monthly totals have remained below this level until this month when a sudden surge was recorded. There is no known scientific reason for this jump.

Four of the diseased cows were found in herds in Monaghan, two were in herds in Cavan, Waterford, Limerick and Clare and there were cases in single herds in Meath and Cork. The oldest victim of the disease detected during the month was eight years old and the youngest were dairy cows found in herds in Limerick and Waterford.

CWD actually not in Alberta

1 Dec 98 submitted by Jane Pritchard
"An item which appeared on the BSE listserve on November 13, 1998 - a clipping from an http site - that stated that "There is also evidence of CWD in southwest Wyoming, in South Dakota and among captive herds in Alberta, Canada".

In fact, CWD has never been diagnosed in Alberta, Canada .

Alberta has an active surveillance program of both captive and wild cervids. The case definition includes any animals greater than one year of age. To date, the Alberta program has sampled a total 184 animals, primarily elk and deer. No positive or suspicious cases have been found

The methodology Alberta uses in specimen selection and testing makes the data from this surveillance program valid under the OIE (Office Internationale des Epizootic) protocol. The OIE is the international organization that oversees animal health requirements for negotiation of international trade agreements

For more information, please contact Dr. Cornelia Kreplin, Chief Provincial Veterinarian."

Comment (webmaster):

My recollection is that the captive elk CWD was from Saskatchewan, that that animal was imported from South Dakota, and its antecedents were a captive animal in Ft. Collins, Colorado. All known CWD can be traced to the research facilities in Ft. Collins. CWD has not been seen in the wild in Canada nor South Dakota, only in NE Colorado, Wyoming and possibly western Nebraska. Canada generally has a strong pro-active program. Elk breeders are also reported strong advocates of eradication (unlike laissez-faire sheep producers).

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