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BSE-The Untold Story

Richard Uridge investigates reports that a new version of the brain disease CJD is taking hold in young people across the Atlantic,and asks whether American Scientists are right to question Britain's strategy for dealing with Mad Cow Disease,and it's equivalent in humans.

Richard Uridge : Mad Cow Disease.Three simple words with an almost humorous ring,but there's nothing remotely funny about Bovine Spungiform Encephelopathy,so far it's claimed 40 lives in Britain,and no one's sure how many more people will die.
[Compare the 40 dead people to the thousands who die of lung cancer from smoking,or the number involved in drink driving accidents or from alcohol damage to the brain or liver and, purely in terms of numbers and risk,one wonders why.Perhaps because it was perceived that it would permeate the human food chain? BSE is such a scare story -LB]

"I think we were in real difficulty with these diseases,they're biologically obscure,they have very long incubation periods and we simply didn't have some of the information that people would have liked us to have and we still don't."

Richard Uridge : BSE has cost British tax payers a staggering 4 billion pounds.More than 3 million cattle have been slaughtered,and British farming years to recover.Yet there's the possibility that our whole response to the disease is will take many based on an incorrect hypothesis.This programme investigates the challenges to the pillars of conventional wisdom on BSE,and its human equivalent new variant Creuzfeld-Jacob disease.

"If we're wrong,and the route of transmission is not through eating beef,then we have decimated an industry,and we have frightened millions of people totally unnecessarily"

Richard Uridge : In fact,there's a serious body of scientific opinion that believes BSE got into people through the blood stream,not the stomach.Right or wrong,last week the British government started importing one blood product,plasma from the US,where officially there is no BSE,and no new variant of CJD.But American experts have told us that Britain could be making a big mistake because there's mounting concern that a new strain of CJD is taking hold on the other side of the Atlantic.
"We have our own BSE problem here.You know we're facing what the UK faced a few years ago."

"Is it a time bomb,is it just something that a few people seem susceptible to,it makes for a very bad science-fiction movie."

Richard Uridge : So our story begins in America.Older people have been sporadically and apparently spontaneously contracting CJD for may years,but now younger Americans are being affected,and bearing in mind that it was young people getting CJD here that alerted Britain to a possible link with mad cows,the trend is worrying an increasing number of people across the Atlantic.Andrew Kimbrel is a lawyer in Washington DC,and Director of the Centre for Food Safety.His particular concern is that the picture is confused,because no one is monitoring the disease properly,as the case of Douglas McKewan suggests.

Andrew Kimbrel : He was one of 7 cases in a cluster in Utah,in the past year.Now when I spoke to Tracy McKewan,his wonderful wife,the story that she told,what it took for her to get this disease diagnosed was extraordinary,and they put them through every test you can imagine.He was a man in his 30s,young kids,good job,just a regular guy. Comes home feeling sick,feeling disorientated,not sure what's going on with him.They decide that he's mentally ill.
Well she doesn't believe it.She thinks something's terribly wrong.So she happens to see a guy on TV talking about one of the other CJD cases in their area,and she says "That's what my husband has,that's what he has".So she calls her doctor,and he says,you know,"Darling if I had to listen to everybody who sees things on TV,and says "That's what I have" or "My husband has"...".She says "No,that's what he has",and she then has to go to another hospital, they won't do it,she then has to go to Washington here,where she finally gets some NIH people to do the proper tests,and of course she has the variant CJD,and he died.
For all we know there could be 50 or 100,but 7 who had enough courage,enough,to get the special test,to go the next step,to figure out what's going on.This is a very fast .....people are dying in months,they waste away in months.
[It sounds like Roy Neary in Close Encounters -LB]

Richard Uridge : Jay Whitlock is wasting away from CJD in Oklahoma.doctors say it's a sporadic case.His wife Julie is not so sure.One - because he's just 27 years old.Two - because the symptoms she describes are almost identical to those who've suffered from new variant CJD in this country.It's a critical distinction,and one which she outlined for us in this, her first ever media interview.

Julie Whitlock : Right now,he's in a field nursing facility.He's uncomprehensive [I think she means incomprehensible -LB],having some hallucinations,incontinent.He still does,know me,he will hug me and hold me,use names.He started having memory trouble in January of this year 1999.He was forgetting phone numbers,and then when we would be heading somewhere to go eat,he'd go past the restaurant,and I'd say "What are you doing?" and he'd say "Well where are we going?",and I'd tell him again,and we'd be heading home and he'd take different routes to get home,and I'd say "What are you doing?" and he'd say,"If you want to drive,you get over here and drive".So you know,the next time he'd take different directions somewhere,I would think "Okay,he's testing me",you know? I'd think it was just a man thing,he know,just doing that on purpose,and then...that was through January,and it was February 1st,his employer talked to me,and said,"You know,he's forgetting...are you having trouble?",Jay said,"Well he's forgetting some stuff",he said "Well he's doing it at work too",and I didn't have any idea about that.So that's when I got him to the doctor the next day.
Most times, I feel like we're living a nightmare,and,you know,the children,Zachary,he's...the youngest is 3,and he knows daddy's sick,he doesn't understand any more.But the oldest,he knows there's no medicine to make daddy better,and he will pray to God to find the medicine [A lot of good that will do -LB] to make his daddy better.You know,so the kids know it's just continuous stress,worry and heartache.

Richard Uridge : Sadly there is no cure for CJD,and proof of whether Mr Whitlock does indeed have the new variant will have to wait until after his death.Only then can tests be conclusive.But if the tests are positive,how might he and the other American victims have contracted the disease? One theory is that it comes from eating deer or elk meat infected with a Spungiform Encephelopathy,similar to Mad Cow Disease,but it is only a theory.Even in Britain there is still no absolute proof,that eating mad cows gave humans CJD,and that's something that bothers June Goodfield,a zoologist,and campaigner for the public understanding of science.

June Goodfield : It isn't enough just to say,"In the absence of any other viable hypothesis,this is the best one".It isn't enough,because we have a number of things,including the fact that we have not reproduced it experimentally in non-hum,an primates,weighing against it.It is vitally important we find out why.It's important because,if we're wrong,and the route of transmission is not through eating beef,then we have decimated an industry,and we have frightened millions of people totally unnecessarily,and I believe we could nail it down.

Richard Uridge : She says that there are experiments that could establish this one way or another,and she argues that if eating meat was the cause,we should have seen many more cases by now.

June Goodfield : If you think of the tons of BSE infected hamburgers that have been fed, probably,to British children,if eating BSE infected meat was the cause of new variant CJD in human beings,we would would be expecting to see evidence of this in that age group and we are not seeing it.[Not if it has very long incubation periods-LB]
That is a very significant epidemiological fact.

Richard Uridge : The mainstream consensus among British experts that is despite the lack of absolute proof,the government had to act on what remains a very probable explanation.Alan Dickinson,carried out much of the ground breaking work on Scrapie,the sheep brain disease, which many people believe was the cause of BSE in cows.He thinks eating infected material from mad cows is almost certainly the way humans got new variant CJD.His careful not to blame per se,he says the more likely culprit is infected brains,spinal chord or offal,which then got into pies,sausages and burgers.
[If this is true why aren't dogs and cats suffering from CJD or BSE as,those products are more likely to end up in dog and cat food? -LB]

Alan Dickinson : The fact that 20-30 people have got BSE,I think is indisputable.The fact that they have got it from something that you would be likely to buy in a supermarket or a butchers shop,I think is a very,very high, probability. Alternative probabilities,I see no reasonable ones for accepting.If you have a hit-and-run accident,and the car is never found,you are in a reasonable position to conclude that it was a car that did it,for a variety of reasons.I think we're in this same position,of saying,a bovine product of some sort.....a bovine food product of some sort,has been the origin of these cases in humans.

Richard Uridge : Our search for clues now takes us back to the late 1950s,when the American Nobel Prize winner Carlton Gadusek,was working with the Foray people in Papua New Guinea.He was intrigued by a brain disease called "kuru".Why,he wanted to know,did only the women and children of the tribe suffer from it.June Goodfield is one of Dr Gadusek's contemporaries.

June Goodfield : The men of the Foray were the ones who were responsible for going hunting, and they went off,and they would sort of kill a few wild pig or what have you.But they kept the spoils of the hunting for themselves.On the whole,the women of the Foray ate vegetables,roots and tubers and what have you,the occasional rodent.But often had very little meat,and they would from time to time,as they prepared the bodies for burial,put slivers of the meat from the flesh,and eat them and give them to the children,or they would put the brain tissue into a little bamboo and steam it and eat it.But anybody who says that Carlton Gadusek believes that cannibalism was the route,is misrepresenting what Carlton Gadusek thinks,and I know because I've asked him,and I've quoted it in my book,and I know because I've asked Joe Gibbs,and I've quoted it in my book.
[There was a TV programme that claimed that a tribe,maybe the same one,had the women and children eat the brains of the dead males,maybe this was the misrepresentation -LB]

Richard Uridge : Gibbs worked with Dr Gadusek,so we spoke to him in his office in Maryland,and asked him about his experiments with chimpanzees to test the theory that eating brains spread kuru in the Foray tribe.

Joe Gibbs : The first experiments we did were to put stomach tubes into the animals,as you would for a gastric analysis,and through those tubes,we introduced copious amounts of highly infectious,known to be transmissible kuru,CJD,and nothing has ever happened to those animals.

June Goodfield : Faced with the fact that experimentally they could not reproduce this route of transmission,that is eating,in the laboratory,they were faced with the fact,"How on Earth did the young children and the women of the Foray get the infected agent?".The only way that Gadusek and Gibbs could experimentally reproduce this,was by inoculation in some form.

Joe Gibbs : Somewhat later,in the course of our studies,I designed an experiment,in which I took squirrel monkeys and starved them for two or three days,then allowed them to handle the infected tissues,from other primates that had developed the diseases,kuru,CJD and Scrapie and in doing so,they consumed the infected tissues.I think in most cases it was upwards of around 80 grams of tissue and all of these squirrel monkeys,both,for kuru,CJD and Scrapie, all developed disease.They have positive pathology and we extracted the prion protein from their brain.So it was,yes we transmitted orally,but letting the animals handle the tissue and consume the tissue,rather than putting it directly into the stomach of chimpanzees and other small monkeys.

June Goodfield : And they finally came to the conclusion that if you looked at the kids and the women,you know they're not living in antiseptic,sterile conditions,they're living in close contact with the Earth,where they have cuts and sores on their hands,where if they are scrabbling with their hands in the bodies or the brain tissue,their inoculating themselves through open wounds or they've been handling a bit of material and they rub their eyes,and it's coming in through the mucus membrane,or they're picking their noses and and it's going in through the mucus membrane there.They believe it was self's inoculation through some way getting into the blood stream.

Joe Gibbs : And they had multiple,multiple,multiple,routes of inoculation.Lesions on their skin, in their eyes,in their mouths,and other lesions caused by puni grass cuts and by leeches on their bodies and so forth,mosquito bites.All these provided a ample means of infectivity.

Richard Uridge : Put simply,these experiments suggest that primates are infected while eating,but not by eating.In other words by inoculation rather than by ingestion.But "so what if it does?",says Dr Ros Ridley,of the School of Vetinary Medicine in Cambridge.

Ros Ridley : Whether the infectious material got in through cuts in the skin,cuts in the mouth, perhaps with bad teeth or whatever,or whether it was because they swallowed it,is,in a sense, immaterial.They contaminated each other with the brains of their dead relatives by handling that material.Now if we look across now to the new variant CJD and we look at those people who have become infected,they are absolutely,perfectly normal people leading perfectly normal lives in Britain.They have not inoculated themselves with some strange material.They don't have strange professions.They don't work in abattoirs,or they don't dissect animals up,or anything of that sort.They're just ordinary people.What they have done,of course,is to eat beef in many forms,not just the recognisable meat form,but prepared foods of various types,everything from sausages onwards,potentially contains beef from various parts of the carcass,and so in their case,the likelihood that it is eating,rather than some other means of introducing bovine material into the body,seems overwhelming,because they are such ordinary people.

Richard Uridge : In the course of making this programme,we discovered that despite the proclaimed ordinariness of its victims,an abattoir worker,Jason Keat,has died of new variant CJD,and if transmission is through blood,as we have already heard that some scientists believe,could vaccines or or other medicines containing things like calf serum,have played a part?
The year long BSE enquiry which was due to have reported its findings next month,has looked into that possibility,and sections of the food industry have perhaps naturally,been keen to cast doubt on the infected meat theory.So could it have been vaccines? Professor Robert Will heads Britain's CJD Surveillance unit based in Edinburgh.

Robert Will : I personally believe that's highly unlikely,because if there was any infectivity in calf serum,it will have been at a very,very low level.
[People are worried about the very very low level of microwaves in cell phones which may be producing brain tumours.Level is not the question.In principle is the question.As Chaos theory shows,small things can have big effects.So how low the level is may not be relevant.Although longer time exposure and power level may be relevant in cell phones,so maybe level is important - LB]
Whereas I think that we know there probably was a high level of exposure orally to BSE infection through the food chain.The other issue is that when vaccines are given,they are only given in a very tiny dosage,once,and so the level of exposure,as far as I'm concerned is likely to be minute,compared to other types of exposure that almost certainly did take place.
There is further concern,however,about the issue of blood and perhaps surgical instruments. As far as I'm concerned from the evidence we have on classical CJD,I don't think there is any good evidence that this form of CJD has been transmitted through blood or blood products.We can't be absolutely certain about that,which is why more work is being done, epidemiologically,and also to do with transmission studies.However,new variant CJD might be different,and the reason for that is that I believe it's due to a different infectious agent,and we have evidence from Dr Ironside's work that the tissue distribution of infectivity in the body foe example,in the lymph nodes and spleen,is likely to be much higher in new variant CJD than it is in the ordinary form of CJD,and that does raise legitimate concerns about the possibility of blood or blood products resulting in on ward transmission of new variant CJD from an individual who is incubating it to someone who receives the product,and for that reason,although there are enormous uncertainties about the type of risk [Ref:Video:BB14:RI 3; N30:The Numbers Game;OB4 Equinox {Living Dangerously};Protext Files;Green File Mindfld.wri;Red File: Reith992.wri],that's involved,or the level of risk,a number of actions have been taken,for example in the UK plasma products,that are derived from blood, will be sourced from plasma...derived from outside the UK,from the middle of this year,and there's also plans to remove the white cells from all blood donations throughout the UK. So,I think,although this is a theoretical risk of onward transmission,it's something that has been taken very seriously,and action has been taken.

Room for a Moo?

David Gould : A farmer with a heart?
Rural crisis: Broadbottom farmer David Gould says life is near to impossible for dairy farmers in the wake of the BSE problems. Economics dictate he can't keep calfs like this one.

A FARMER hit hard by the rural crisis is giving away his cows to anyone who can offer them a good home. Broadbottom farmer David Gould says plummeting market prices are making life near impossible for dairy farmers. Prices have dropped so low that some farmers routinely shoot calves dead just after birth in an attempt to stem spiralling losses.
Farmer Gould refuses to do this but says such a 'hand to mouth existence' cannot go on without the help of some Good Samaritans. So he's now calling on anyone with enough spare land to give him a helping hand and adopt 'a farm-yard friend'.
"The economics of the situation leads to some farmers shooting them - but I can't do that," he said. "If there are any readers out there who can take one or more off my hands and give them a good home I would be willing to do it. "You don't necessarily have to be a farmer to look after a cow but someone with a smallholding with a couple of acres of land."
The Little Hill Farm owner says the number of farms with cows has dropped to a paltry 10 locally. But such a situation - which sees a farmer collect less than nine pence per pint of milk - has arisen due to the strength of the pound and the BSE crisis. "The powers that be say the prices are to do with the strength of the pound," he said. "But as far as I know we don't export milk, or if we do it must be very little."
He added: "The BSE crisis has been the main factor though and we do feel betrayed by the way the government handled the whole issue. "It's the government's fault in the first place for relaxing the sterilisation process. "Through all the years we were punished but the farmers themselves never let their standards slip." If you have the resources to 'home a cow', contact The Advertiser on 0161 339 7611 and we will pass on your details.
Mark Travis
[It's nice to know that some of our "countryside guardians" look after their animals so well that they don't shoot them in cold blood because they don't turn a profit. Well done farmer Gould for putting life before money -LB]

A FARMER who will give away bull calves to a caring home has had several offers to" help - including sponsorship from the Hindu community. The Advertiser reported how Broadbottom farmer David Gould is offering to take a novel approach to falling market prices.
Farmers countrywide face prices which have dropped so low that some consider shooting calves dead just after birth in attempt to stem spiralling losses Farmer Gould won't do that and instead is proposing to hand over any bull calf to someone who can offer a suitable home.
We have already had a number of offers for help from a few unlikely sources. A proposed urban farm and a land owner have said they wish to help by taking them off David's hands. David is especially grateful to those offering help because another imminent calving spell is set to signal new additions in Broadbottom. "We have none left now but we will start calving next week and if it's practical for them we hope to do something soon in the coming weeks," he said. "We will have to think through what is best for the cows but we are definitely keen for others to come forward if they wish."
Ashton man Vishnu Mohandas proposed sponsoring the calves, a scheme which he and his friends have already done at a Sanctuary in Watford. "I read this in the paper and was very pleased with how Mr Gould is handling the situation Mr Mohandas said . "I have sent money to India for a similar scheme while I, and many others nearby, have contributed money to a sponsorship scheme in Watford. Anyone interested in adopting a bull calf must have plenty of land. [The Advertiser 27/9/2000]

MP speaks out

MP Andrew Bennett has slammed farmers involved in recent petrol protests - saying "they've got a cheek". Mr Bennett described last week's crisis as very sad, adding that farmers got their fuel virtually for free.
The MP went on to reveal his thoughts about the haulage industry and how, in order for the majority of lorry drivers to he satisfied, there would have to he cuts. "We've got a third more lorry drivers than we need - there should be some restructuring,"he said. "I appreciate many are self-employed and buy vehicles by mortgaging their houses, but they shouldn't take it out on the general public,it doesn't help." Mr Bennett says motorists should consider smaller cars and recommended working and shopping locally.
[Whilst I agree with Andrew,and the Farmers and hauliers have no right to break the rule of law "for the benefit of the country" we've probably got a third too many politicians too,and they're probably paid a third too much -LB]

Richard Uridge : The process of leuko -depletion - filtering out white cells,in which the infective agent is thought to be more concentrated,won't start until November.But the Department of Health told us that the decision to source plasma from countries without BSE came into effect just last week.Then they told us that the imports are coming from the US, where as we've been hearing,there are growing concerns that CJD is spreading to much younger people than normal,27 in at least one case.but Professor Will says there's no evidence of a new variant of CJD that can't be traced to Britain,and anyway these are only precautions against a theoretical risk.Alan Dickinson agrees,although he did find the infective agent of the sheep brain disease Scrapie,in blood during his research.

Alan Dickinson : You're not dealing with solid tissues,you're dealing with serum and blood which can have small amounts of infectivity.I think I was the first person to show that.I regarded finding it on a small proportion of occasions ,in heart,blood,as really an index that my crude method of extracting blood from a heart,was sufficient to incorporate quite a lot of heart tissue and blood cell lining tissue.I'm sure I was doing it far more crudely,and one would hope that more is known now about can hear me perhaps doubting this,more is recognised and appreciated about the ease of cross contamination of these kinds of products when people think they are dealing in clean room situations with safety cabinets.These words are dangerous in their own right.

Richard Uridge : There are other doubts about the official British position on CJD,and again they arise from the investigations into kuru,in the Foray tribe of Papua New Guinea,and later work by Joe Gibbs.Essentially the question boils down to how new is new variant CJD? Twenty years ago a neurosurgeon died in Boston,apparently of sporadic CJD,but as Joe Gibbs explains,later examination of his brain,revealed the classic symptoms of the new variant.

Joe Gibbs : I maintain that the so-called florid or daisy plaques that they refer to in new variant, occurs outside of the UK in cases,and I've shown them in kuru,chronic wasting disease of deer and elk are massive plaques with vaccules around them,and then the paediatric neurosurgeon in Boston who died.he was first diagnosed has having a very rare skin disease called Almeyer Dago's Disease,and that disease he did have,but then he started to develop neurological signs,and ultimately died and we received the autopsy tissues on him.We demonstrated that he had the prion protein in brain and in lung tissue and it turned out to be the very clear sporadic type of CJD.Now my argument here is that,I'm not sure what are the restrictive clinical and pathological characteristics of so-called new variant,because I can show a number of these things in sporadic cases.I can show people that have gone for psychiatric evaluation,then they developed ataxia and had an extended period of clinical progressive disease over a period of 18 months - 2 years,but they turned out not to have new variant,according to the UK description.

Richard Uridge : British CJD experts have also examined the Boston neurosurgeon's brain tissue.Professor Robert Will believes Joe Gibb's theory's based on a misunderstanding.

Robert Will : Well I think there's a whole wealth of evidence now,that suggest that new variant CJD is a new disease.For example,when the original paper was published in April 1996,one of the things we put in that paper,was the importance of reviewing other neuropathological information from the past,in other countries,to see whether this disease had been seen before elsewhere,and since then we have not been informed or been able to identify,any other case which fulfils the neuropathological appearances of new variant CJD.Now I think there's a slight misunderstanding here,because although florid plaques are a very important component of the diagnosis,neuropathologically,the fact that there is a single or one or two, florid plaques,is not the issue.The issue is that these plaques are widely distributed throughout the brain.So it is true that occasionally,a type of florid plaque has been seen other cases,you know single or one or two florid plaques,for example,in the case you mentioned,of the neurosurgeon,and also in another case,with dura mater related CJD,but these cases have been reviewed by Dr Ironside and by other neuropathologists,and the view I think,is clearly that the occasional florid plaque does happen sometimes,but the overall neuropathological appearances do not look at all like new variant CJD,and the general consensus among the neuropathological community is,according to my understanding,that new variant CJD is indeed a new disease.

Richard Uridge : Another of the commonly held beliefs about BSE concerns the role played by the animal feed industry.However unsavoury it sounds,renderers turned animals carcasses into animal feed.Sheep were fed to cows,and cows were fed to cows.

[Then if the farming community was decimated they have only their greedy exploitative selves to blame -LB]
Everything went into the pot and was cooked to produce meat and bone meal,then the temperature of the whole process was reduced,ostensibly to save on fuel,and the assumption is it was no longer hot enough to kill off the infective agent.More recent experiments have shown that's probably not true,because even the higher temperature doesn't work.Only one condition does.It's called "pressure cooking",and is now widely used in mainland Europe.Paul Foxcroft is Sales Director of the rendering company Prospa de Molder. He doesn't dispute that BSE was spread by infected feed,but now thinks a combination of things caused BSE to explode in Britain and only in Britain.

Paul Foxcroft : You've got more,unknown to anybody,more active Scrapie agent in the meat and bone meal,year upon year,as more and more sheep are slaughtered.Perhaps a sudden boost of active Scrapie agents within the meat and bone meal again as if there was an increase in the incidence of Scrapie within the sheep flock.You have more meat and bone meal,more then of that meat and bone meal going into the dairy feed,you then have more of that dairy feed being put into the cow.Now I don't believe that combination of circumstances has existed anywhere else in the world,at that same time.We could be perhaps a little mischievous and raise the possibility of organophosphates as maybe a predisposing factor.There certainly could will be genetic factors in terms of genetic susceptibility,as the gene pool is narrowed if you like,with artificial insemination over the last several decades, where we've perhaps used bulls with a susceptibility,or lack of resistance,whichever way you want to term it,into the herd...national herd.Therefore,it's almost a lottery,we've hit this horrendous jackpot,at least we got all the numbers.There are plenty of other countries out there that have perhaps only got 1,2,or 3,or 4 of the numbers and for that they get nothing.

Richard Uridge : So why are the numbers coming up in America now? What special combination of circumstances exists across the Atlantic? Michael Hanson of the US Consumers Association believes his governments rules about what goes into animal feed are far too lax,and that they just aren't looking hard enough into the problem of CJD.

Michael Hanson : There is the problem of "if you don't look,you don't find".They tend to be concentrating on supposedly looking for a British style BSE case,they're not focusing on intensively sampling animals that might be at risk.So the brain chemistry,the neuropathology that they're looking at,they're looking for the very obvious signs,like you see in BSE cattle in Britain.They're not looking for more subtle signs,and even though there is a quotes "surveillance system",we think it's highly flawed,and while there's no direct evidence,I think there's indirect evidence that there might be a TSE existing in food animals,in the US.

Richard Uridge : Andrew Kimbrel,the lawyer from the Centre for Food Safety,who told us that America was having its own BSE crisis has a shopping list for his government.

Andrew Kimbrel : We just filed a formal legal petition saying "make CJD a reportable disease" which it is not in this country,even though we've seen explosions of cases,for goodness sake "stop feeding ruminants to ruminants".We're asking exactly for the UK regulations on feeding animals to animals,which we don't have in this country."Blood Supply",and an investigation on why in the world our wildlife is coming down with TSEs,at such an alarming rate.

Richard Uridge : So what are we to make of the developments in America? And what can they tell Britain about its pillars of conventional wisdom on mad cows and CJD? I went to see the man who advises the British government on these issues,the chairman of the Spungiform Encephelopathy Advisory Committee or CEAC,Professor Sir John Patterson.I asked him first about the cases of CJD in younger people,and the claims that America now has a new variant of the disease.

John Patterson : It's very difficult to comment on that usefully.All my experience on the UK CEAC tells me that you really need to know all the details of any cases or any problems that are being posed before commenting on them.But if it's true that there are some cases of human Spungiform Encephelopathy in unusually young people,then I think now we have some clear definitions of the clinical state of those patients,we have some modern imaging techniques,for doing MRI scans,and most particularly we have some laboratory based biochemical tests that can be applied to material from these patients which should be able to define the situation much more clearly,and I think you need to get all those details first,before judging what the significance might be.

Richard Uridge : But it wouldn't surprise you,or perhaps it would,if there were 40 cases eventually, in America over the next 2-3 years?

John Patterson : Oh I think it would surprise me enormously if that were the case,and that's why one would want to know all the details of any such case,let alone 40,before commenting on it.

Richard Uridge : One of the conventions,with BSE is that it was caused by people ingesting the infected agent...infected meat,yet some experiments carried out by Carlton Gadusek in he 1950 showed that it might actually be through blood contact.Has his work been disregarded in building the current hypothesis?

John Patterson : No,not at all,I mean it's clearly recognised that there are a number of ways of transmitting Spungiform Encephelopathies.You refer of course,to the peripheral route of inoculation,blood to blood,and you would have to say that in that context,the most vulnerable people would be those that are occupationally exposed because they work in abattoirs,and to date,there have been no cases of variant CJD in abattoir workers which would link the occupation with the exposure.

Richard Uridge : What about the death of Jason Keat? He was an abattoir worker in his 20s, on his death certificate it says that he died from new variant CJD?

John Patterson : You,you...I mean I ....It may surprise you to know of course that I can't relate individual names to individual cases.It is true that there are one or two people that who've classical CJD who've worked in abattoirs,and if you tell me that Jason Keat was one of those with variant CJD who also worked in an abattoir,then I accept that,but then there 39 other cases,who didn't and it's very difficult to unify those 39 cases with a hypothesis about blood to blood transmission.Whatever his occupation he probably,like the other 39 cases,was a consumer of beef products,which would be the most likely hypothesis for the way in which BSE was contracted.

Richard Uridge : You don't think 1 in 40 or 1 in 41 is significant then?

John Patterson : Er no,I don't think it is,in the same way that one of the cases we know had been a vegetarian for many years,but if you trace back the history of that individual,to the point at which she became a very strict vegetarian,then it's most likely we think,that she was exposed to beef products before she became a strict vegetarian and the occurrence of one case we don't feel necessitates altering our proposal about the most likely route of transmission.

Richard Uridge : But it is just a hypothesis,at this stage.Is it possible that another hypothesis would explain transmission and that is that is that vaccines have somehow played a role?

John Patterson : I think that the CJD surveillance unit and Professor Will in quite a long and extensive questionnaire to the families of each of these cases,and looking all the time for occupational exposure,geographic clustering,medical histories,histories of operations which might unify all these 40 cases,then you cannot find such evidence.

Richard Uridge : And in the absence of any other evidence,the current hypothesis is the best one,in your view?

John Patterson : I think it is,yes.

Richard Uridge : If I can turn to the use of blood and blood products.Is there a concern that BSE or new variant CJD should I say,could be spread through blood products?

John Patterson : Yes,and this is going back a little while now,but of course CEAC made some recommendations to the government about that,particularly in relation to removing the white cells from blood transfusion,and the technique which is called leuko-depletion is being extended to become comprehensive in the UK blood transfusion service as soon as possible.

Richard Uridge : Because the white cells actually carry a greater proportion of the infective agent?

John Patterson : Again the evidence is very incomplete in this,but if you think about it intuitively,and you say "we must apply the precautionary principle",then it seems logical to remove the white cells,as the most likely component of blood which would transmit the infection.

Richard Uridge : Why is it that precautionary measures are being taken only now?

John Patterson : Well it takes quite a long time of course to set some of these processes in train....

Richard Uridge : That's 3 years potentially in which people could have been infected person to person.

John Patterson : There is a possibility that if variant CJD is transmissible by blood or blood products,and that some of the cases we know have been blood donors,that it might have been transfused from one individual to another,that's exactly the sort of eventuality that one is trying to prevent,from people who are currently incubating variant CJD,but haven't actually manifested it,yet.That's why those precautions are being taken.

Richard Uridge : Might it not have been more sensible to take that precautionary measure at the outset? It didn't seem that it was a problem taking precautionary measures as far as the beef industry was concerned quickly,so why not the medical industry?

John Patterson : I think that's exactly right,because of course the proposition was that the source of cases of variant CJD was oral exposure to beef products which contained some of the specified offals,most notably brain and spinal chord.So it was important really to shut that door as quickly as possible.There was then a need to look at the evidence about the transmission of classical CJD via blood transfusion,and there the evidence is conflicting,and by and large there,the conclusion has to be that classical sporadic CJD is only very rarely transmitted by blood transfusion, if at all.
However,it then became clear that variant CJD was behaving differently,particularly in relation to the phase of the infection in humans,in the lymphoid tissues.You can find the agent of variant CJD in the spleen and in the tonsils and in the lymphoid tissue in the appendix,and so one had to recognise that it was possible that circulating lymphocytes also contained this agent.There was also a developing body of knowledge about animal experimentation using mice,which led one, taking all this into account,to recommend to the government that leuko-depletion would be a sensible precautionary measure.Now that all took time to develop,time to think about and then of course,once you've made the decision,time to implement.So that's where you get the apparent delay from.

Richard Uridge : Finally,are we now any nearer to breaking the deafening silence on the crucial question? How many more people will die from variant CJD in Britain? We've seen estimates prepared for the Royal Society,based on the number of cases so far.Depending on how you interpret them,the projections are either good news or chilling reading.They indicate there could be as few as a dozen or so more deaths,or as many as 13 million.

John Patterson : That was one of the major questions that was asked immediately on March 20th 1996,and the answer then was that there was a very broad range of possibilities.Three years later and fortunately the range of possibilities still remains very wide,and so,to be honest I think the only that we will tell,is to wait for another 2 or 3 or 5 years before we can answer...we can narrow down that range usefully for you.

Channel 4 Report





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