Mass screening for nvCJD
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Mass screening for nvCJD after death of appendectomy patient
Scientists to step up BSE tests on sheep
Cases of CJD in young women in the US
Mad cows and mother's milk the perils of poor risk communication
FDA to release blood products quarantined for CJD investigation
U.S. seen removing white cells from donated blood
CJD sufferer died before her mother's eyes
BSE case found on northern Dutch farm

Scientists to step up BSE tests on sheep

Aug. 21/98 The British Times MICHAEL HORNSBY
Scientists advising the U.K. Government are to step up checks on sheep flocks because of fears that sheep may have become infected with bovine spongiform encephalopathy. Infection in sheep, unlike in cattle, is likely to be found throughout the carcass, the story says, making it impossible to protect consumers simply by removing the most dangerous parts at the abattoir.

The implications of this for sheep farmers are potentially devastating, since the only way to deal with BSE in sheep might be to slaughter entire flocks, not just those animals that are visibly ill.

The scientists' concern is that some cases of scrapie, a fatal brain disease that has afflicted sheep for centuries without known harm to human beings, might now be BSE. Since 1995 a total of 27 people have died from a new variant of Creutzfeldt-Jakob disease.

Reporting on its latest meeting, the Spongiform Encephalopathy Advisory Committee said it had concluded that "additional work was required to determine the extent of scrapie in UK sheep and the strains involved" and had set up a sub-group to do research.

The committee was quoted as noting that limited evidence from laboratory tests showed that "the clinical disease caused by inoculating sheep with BSE appeared to be very similar to scrapie. As in scrapie, infectivity was found in the spleen of sheep experimentally infected with BSE, although no infectivity has been found in the spleen of BSE-infected cattle. To distinguish the BSE strain from scrapie strains requires lengthy and expensive post-mortem testing of sheep tissue. Such work must continue because ... finding the BSE strain in the national flock might have important implications for public health."

Jeff Almond, Professor of Microbiology at Reading University and a member of the committee, was cited as saying that little comfort could be drawn from the failure to find any BSE-like strains of scrapie, because so few sheep had been examined.

Scrapie in sheep in the U.K. has been a notifiable disease only since 1993. The compulsory slaughter of scrapie-infected sheep, coupled with compensation of 25.34 per animal, was introduced only three weeks ago. The heads and spleens of sheep of any age, and the spinal cords of sheep over one year old, are banned for human consumption, as is meat recovered from the vertebral column of any sheep.

Maff press release of 19 Aug 98:

The public health controls currently in place are: 

for affected animals:

       clinically affected sheep and goats are not permitted to be used for food or animal feed; 
       suspect cases of scrapie are slaughtered and disposed of by the Ministry of Agriculture and the farmer is paid

for all sheep and goats:

       the heads and spleens of sheep and goats of any age are removed from the food chain; 
       the spinal cord of older animals (>1 year) is removed from the food chain; 
       the vertebral column of sheep and goats of any age is not permitted to be used
          in the preparation of mechanically recovered meat. 

7. The three part strategy for improving the surveillance of scrapie began in August 1997 with the start of an abattoir survey of sheep brains. In June 1997 the Government consulted on measures for the compulsory slaughter of affected sheep and goats and
this legislation came into force on 29 July 1998. A pilot postal survey of farmers was conducted in May 1998 and the main
survey is scheduled for the Autumn. 

8. MAFF's research programme on TSEs in sheep was expanded in 1997 and more than 1000 scrapie free sheep were imported
from New Zealand at the beginning of this year to facilitate pathogenesis and transmission studies of BSE and scrapie in sheep. 

BSE case found on northern Dutch farm

26 Aug 98 Listserve and Reuters World Report
The third case of BSE has been diagnosed at a farm in Frieselo the Netherlands. All 90 cows from the farm will be culled and examined for TSE as well as all family lines. Cow was about 6 years of age. The village Vriescheloo is only about 10 km from the German border. Autopsy done by ID-DLO in Lelystad. The whole herd will be destroyed. Parents and descendants of the affected cow will be traced and removed. Cows born on the farm in the same year as the affected cow will also be traced. No information on what tests will be performed. Two prior cases of BSE occurred in the Netherlands in March and April 1997. It is unknown how the cow contracted the disease. Investigators will probe whether the cow was fed high-risk material, such as the eyes, brain, spinal cord of cattle and sheep, violating EU regulations.

The German government has declare a total of 5 cases of BSE. Liechtenstein (how many cows there ?) declared a BSE case during July or August. Liechtenstein is between Switzerland and Austria but does not border with Germany. Interestingly, there was one FSE case of obscure source reported there [H. Budka]

CJD show on 'Private Eye'

Correspondent 28 Aug 98
The CJD Voice website was real busy after the Private Eye show. The chatroom was real crowded and there was a woman there who has a friend who is 24 and has CJD. She got it at 23. Also, there was a posting from a mother on the CJD Foundation website who said her 25 year old daughter has CJD. Given the woman in Rochester who died a couple months ago and was in her 20s and the woman last year in Florida who was 28 it definitely seems like someting is going on. Odd the all the young CJD cases in the US are women -- could it be bovine CNS used as the lipid in face creams etc?

Mass screening after appendectemy result

Aug. 26/98 PA News  By Padraic Flanagan
The Department of Health  helpline is 01803 861854.
The U.K. Government has ordered a massive testing programme of surgical specimens to find out the spread of the deadly human form of BSE following the chance discovery of evidence of new-variant Creutzfeldt Jakob Disease in a patient who had his appendix removed in routine surgery three years ago, and died in June of this year.

A helpline was opened today to reassure former patients at the same hospital who may be worried of contracting the disease from its surgical equipment. tel 01803 861854.

The scare was prompted by the case of Tony Barrett, a coastguard who had his appendix removed at Torbay Hospital in Devon, in September 1995. The Guardian was cited as reporting that the operation took place eight months before he displayed any symptoms of nv-CJD and nearly three years before he died.

Ministers have approved a review of thousands of laboratory specimens of appendices and tonsils which are routinely kept in hospitals after removal. If they find signs of nv-CJD -- which has killed 27 people since May 1995 -- in just one more sample then voluntary mass screening of patients about to have their appendix or tonsils removed could take place.

Researchers are preparing procedures and ethical rules for the initial studies, which will be funded by the Medical Research Council. If tests on patients are authorised, it is probable they - or parents in the case of children - would be asked for permission. Guidance on whether to tell them the results has still to be considered because the disease is incurable.

The case behind the latest twist in the saga involved Tony Barrett, a coastguard. He had his appendix removed at Torbay hospital, in Devon, in September 1995, eight months before displaying any signs of nv-CJD and nearly three years before he died. Mr Barrett complained of numbness in his face and right hand in May 1996. In April 1997, he was treated for depression and later he became hyperactive and aggressive. This was followed by intermittent deafness, blurred speech and unsteadiness. He died in Derriford hospital, Plymouth, last June.

A research letter about his case, although he will not be named, and the suggested screening of specimens is expected to be published in The Lancet medical journal. After his death, doctors examined his appendix and found a rogue protein associated with nv-CJD. Samples from 44,000 appendectomies and 800,000 tonsillectomies carried out each year are routinely kept by hospital laboratories.

Last night the Government's chief medical officer, Sir Kenneth Calman, said there would be no immediate change of health or BSE controls. But officials are anxious for repeat tests on other appendices "to see what it means. Its significance is not clear. We cannot overstate that. "If we do a test on appendices and do not find anything, it is only partly reassuring. All this will do is give us a preliminary view of what is going on in the population as a whole."

The testing of previous samples would be done anonymously but if another positive case was discovered, the Government would switch to "pro-active mode" and ask permission to test people's appendices and tonsils before surgery. However, initial checks may not uncover further examples because of the small number of nv-CJD victims in a population of 50 million. At present there is no treatment to "modulate" the disease as there is with HIV and AIDS, for instance, although work on simple, reliable and effective tests for the disease and drugs to block its progress is under way.

Sir Kenneth said: "Ministers are fully aware of the background to this and all the consequences. They (support) the wish to pick up the opportunity to look at the prevalence in the population." Health chiefs in the south-west are also concerned that surgical equipment used to remove Mr Barrett's appendix was used in subsequent operations after cleaning and sterilisation. Surgical instruments used on known or suspected nv-CJD patients are meant to be destroyed.

Sir Kenneth said yesterday the risk of contamination was minimal, but a helpline will be opened today for patients seeking reassurance. "It is a very unlikely mode of transmission... Advisers consider the risk is minimal," he said. A further review of decontamination and disposal procedures was under way but "no action is anticipated at this stage". South and West Devon Health Authority said: "Our advice is that there is no evidence that other patients are at risk."

Commentary [Charles Arthur, The Independent]:

"Lots of misinformation flying around yesterday on this list, even though the Guardian in Britain had the scoop on it. The paper is in the Lancet of Aug 29. Appendectomy carried out on otherwise healthy Devon man. He later developed nv-CJD. Pathology department had bright idea, because they keep (routinely) samples or whole organs removed after surgery, of testing for PrP. Found it.

Appendectomies do mostly use disposable instruments, at least in terms of those most in contact- scalpels and thread. (Not so sure about things like retractors, though.) Not so surprising in retrospect that appendix should be PrP+ before CNS symptoms appear: it is lymphoid tissue, and we know that lymphoid - the whole gut, thymus, through to the anus - is involved in the early incubation. (John Collinge's comments, not mine, if anyone's wondering.)

Wider tests have NOT yet started, but 1,000+ tonsils and appendixes will be tested starting within the next few months to see what it tells about prevalence of the disease. Tests will be anonymous, rather like HIV testing in the early 80s. As a control when the original appendix was tested, 10 others were also tested. Showed negative.

Moral dilemma as government launches tests on appendix

 PA News  By John von Radowitz, Medical Correspondent, PA News
A key question remained unanswered tonight as an investigation to check thousands of appendix and tonsil specimens for signs of new variant CJD was launched by the Government. No decision has yet been taken over whether living patients found to be infected should be told of their condition.

Questioned about the subject today the Chief Medical Officer Sir Kenneth Calman said future policy would depend on the initial findings. "If individuals are identified, or samples are identified, that are positive, then I think we will need to change the nature of the study," he said. "We will need to re-think the whole process." The issue, which echoes the controversy that surrounded the early days of HIV testing, presents a potential ethical nightmare for doctors.

So many unknown factors are involved that finding the infective agent in a tissue sample is no guarantee that the person will contract the disease. What is more nothing can be done for victims of the human form of mad cow disease. Whether or not it would be right to inform individuals that they might in years to come suffer an incurable fatal illness is open to question. But the Government's decision to carry out the checks was broadly welcomed today.

One expert, microbiologist Dr Stephen Dealler, said it would mark a turning point by providing the first indication of how many people might eventually succumb to nvCJD.

But because the disease has such a long incubation time, possibly 30 years or more, no-one knows how many people might be infected. Microbiologist Dr Stephen Dealler said today's development could mark an important turning point, providing the first indication of how many people were likely to succumb to nvCJD in the future. Even if the news was bad enough to "scare the living daylights out of people", he expected the Government to make it public.

Dr Dealler, once an outspoken critic of Government policy on the human risk from BSE and now advising the Department of Health, said: "We already knew that you can find signs of nvCJD in the tonsils of people going down with the disease. The unanswered question has always been how early during the incubation period do you start to see these effects."

Taking account of the need for BSE to "jump" the species barrier and re-emerge in humans as nvCJD, the incubation period could be anything from 25 to 40 years, he said. This would mean the cases seen so far are probably the very first in an epidemic of as yet unknown dimensions.

Dr Dealler said: "We have all eaten, on average, 50 meals of meat from infected cattle, so we can't go around thinking we're not at risk. We have to put up with the fact that we have been exposed. "Here for the first time there's a way of getting a handle on how severe the epidemic is going to be, and full marks to the Government for that."

He pointed out bad news would have a silver lining, since it was likely to spur a so far complacent pharmaceutical industry into action. A disease was only seen as a worthwhile research investment by drug companies if it affected enough people. "It turned out, for example, that one person in 100 was infected with nvCJD and might need to take drugs to protect them for the rest of their lives, I can imagine the pharmaceutical companies getting very interested," said Dr Dealler.

The move followed the chance discovery of the infectious prion protein agent believed to cause the disease in a patient who had his appendix routinely removed three years ago and died in June. Until now it had been thought the disease could only be confirmed by examining brain tissue, usually after a patient was dead.

Scientists now have the chance to track the exposure of the population to BSE in the late 1980s and the risks they faced before potentially infective beef began to be removed from the food chain. Samples from 44,000 appendectomies and 800,000 tonsillectomies carried out each year are routinely kept by hospital laboratories.

Sir Kenneth said: "The death of any patient, particularly in these tragic circumstances, is a matter of great regret. However, this new finding provides us with an opportunity which it is only sensible to explore. "We are currently in discussion with the Medical Research Council and research workers as to how best to carry out anonymous studies on appendices and tonsils that have been previously removed during routine surgery, to investigate whether the nvCJD prion protein might be present in any of them and, if it is, to assess its significance. "These will be important studies, though their interpretation will require considerable caution. They could advance our understanding of the disease."

He was also taking action over worries about the possibility of surgical instruments being contaminated by nvCJD. Instruments used on known or suspected nvCJD patients are supposed to be destroyed. However, those used in this patient's appendix operation would have been employed again after cleaning and sterilisation. Experts have told the Government medical instruments are an unlikely transmission route, particularly as scalpel blades are always thrown away after surgery. But to be sure risks were minimised, Sir Kenneth arranged for an expert group to carry out a further assessment of decontamination procedures. "This will start work shortly," he said.

Today's decisions were prompted by the case of Tony Barrett, a coastguard who had his appendix removed at Torbay Hospital, Devon, in September 1995. His operation took place eight months before he displayed any symptoms of nvCJD and nearly three years before he died. After his death, doctors examined his appendix and found traces of the rogue prion protein that is thought to spread the disease by causing a "chain reaction" of infection, turning other normal proteins into the dangerous form.

Since May 1995, nvCJD -- a new strain of the brain disease whose existence was announced by the Government in March 1996 -- has killed 27 people. But because it has such a long incubation time, possibly 30 years or more, no one knows how many people might be infected.

Dr James Ironside, from the National CJD Surveillance Unit in Edinburgh, said the number of specimens to be checked would run into thousands. Many will have been removed some time before the mad cow scare began, possibly back in the 1970s. "We are taking advice from the London School of Hygiene and Tropical Medicine as to how many specimens we need to examine," said Dr Ironside. The findings would be collated, assessed and published, he said.

Sir Kenneth said: "This is the first foray into looking into prevalence. It won't be the last one." It has been suggested that if just one more specimen is shown to contain the nvCJD agent an "active" screening programme may be launched, involving everyone who has their tonsils or appendix removed in the future. But Sir Kenneth said all next step decisions would have to wait until the initial results were known.

Clive Evers, who chairs patient group the CJD Support Network, said the ethical issue was complex and difficult, but could not be avoided. "It will be important to have some form of protocol developed," he said. "There's going to be a lot more to it than this step-by-step approach. "Usually when anything like a diagnostic test emerges there has to be a protocol on how it will be used. I think it will have to be addressed in the same way as Alzheimer's Disease was, where consortium of organisations was set up to deal with the ethical questions raised."

People can be screened for genetic factors that predispose them to Alzheimer's. But as in nvCJD the disease is incurable, many experts say it is debatable whether there is any point in carrying out such tests. Mr Evers said he was encouraged by the openness shown by the Department of Health, but added: "It does have to be handled sensitively in relation to the public."

Dr Richard Nicholson, editor of the Bulletin of Medical Ethics, said: "I think what one needs to know is what exactly the test does and what a positive result really means. "If it doesn't mean you will necessarily get the disease that presents a problem. By applying the normal rule of informing patients, you may cause them to be worried and anxious for the rest of their lives. "To an extent many of the issues are similar to what occurred during the early days of Aids, and much the same rules need to apply."

Dr Dealler, once an outspoken critic of Government policy on the human risk from BSE, is now advising the Department of Health.

He said: "We already knew that you can find signs of nvCJD in the tonsils of people going down with the disease. The unanswered question has always been how early during the incubation period do you start to see these effects." Taking account of the need for BSE to "jump" the species barrier and re-emerge in humans as nvCJD, the incubation period could be anything from 25 to 40 years, he said. This would mean the cases seen so far are probably the very first in an epidemic of as yet unknown dimensions.

Dr Dealler said: "We have all eaten, on average, 50 meals of meat from infected cattle, so we can't go around thinking we're not at risk. We have to put up with the fact that we have been exposed. "Here for the first time there's a way of getting a handle on how severe the epidemic is going to be, and full marks to the Government for that." He pointed out that bad news would have a silver lining, since it was likely to spur the pharmaceutical industry into action.

Mad cows and mother's milk the perils of poor risk communication

Aug. 15/98 The Lancet.  Book Review
Douglas Powell, William Leiss. Montreal & Kingston: McGill-Queens
University Press. 1997. Pp 308. $55. ISBN 0-7735-1619.
"The communicability of cholera ought not to be disguised from the people, under the idea that the knowledge of it would cause panic, or occasion the sick to be deserted."
--John Snow (On the Mode of Communication of Cholera, 1854)

In March, 1996, British Secretary of State for Health Stephen Dorrell rose in the House of Commons to tell his colleagues that scientists had discovered a new variant of Creutzfeldt-Jakob disease (nvCJD) in 10 patients. More importantly, he said, a link between the illness and the consumption of beef from cattle with bovine spongiform encephalopathy (BSE) could not be ruled out.

The media began reporting doomsday predictions that by the year 2000 nvCJD would account for 5000 to 500 000 deaths. The Humane Society of the United States issued a press release stating that "some scientists speculate that this disease would become an epidemic as deadly as AIDS". Media maven Oprah Winfrey announced on her show that she would no longer eat hamburger because of her fear of catching what now had become "mad cow" disease. She later successfully defended herself in a lawsuit investigated by the National Cattlemen's Beef Association for violating a Texas law prohibiting the "defaming" of beef.

The frenzy over the subject, which had now caught the public's eye, led to the European Union banning the export of British beef, and, with the consumption of beef across Europe falling dramatically, Britain's beef market collapsed. It is estimated that the EU itself lost more than 6 billion in beef subsidies, and more than 700 million was paid out to compensate farmers and abattoir proprietors in Britain who had lost their herds and their livelihood.

Although mad-cow disease seemed to burst suddenly upon the scene, its ascendancy was not as rapid as most might think. The "crisis" actually played out over 8 years with numerous findings and reports warning of the incidence of BSE and the hazards of use of rendered offal (entrails, hooves, and whatever else is not fit for human consumption) as ruminant feed.

As far back as 1988, the British government had made BSE a reportable disease and issued an order to slaughter and burn all cows suspected of having BSE as well as destroying milk from any suspect cow. Despite those actions, by the end of 1996 more than 166 000 BSE-stricken cattle had been reported from 32 400 British farms. Doug Powell and William Leiss take this long-running episode as a prime example of the perils of poor risk communication. In particular to BSE, the problem they see stems from a decade of British government denial that there was any risk at all. It was this no-risk message that they say led to the devastating social and economic costs to a nation of beef eaters.

The authors, whose particular academic disciplines are food science and environmental policy, lay out several recent episodes (a chapter for each) of the maddening consequences of poor risk communication. Tackling some of the biggest headline grabbers in modern times, they tease apart the risk communication mistakes made with dioxin (Agent Orange), Escherichia coli O157:H7, silicone breast implants, bovine growth hormone, polychlorobenzenes, and genetic engineering in plants.

Interestingly, the undercurrent is that if good risk communication had been practised then none of these encounters would have become part of our lexicon today. While that may not be entirely accurate, it is true that the problems in communicating risks arise in the chasm between how scientists speak about risks and how the public intuitively understands them. One of the longstanding misconceptions of the scientific community is that if they could just explain the situation ("get the numbers right"), they would be well on their way to dealing with the public's perception of risk. What is lacking in this stratagem is an appreciation of relative incapacity of human beings to handle apprehension, suspicion, and a sense of personal dread.

The problematic aspects of poor risk communication always revolve around lack of trust, lack of credibility, and lack of timely information -- in the game of scientific communication, it is rarely the scientific part that gets you into trouble. And, although the authors say their aim was not to lay blame, it is clear that the lessons to be learned fall heavily on the side of the communicators. This text is filled with lessons on how to do it right, and not just when confronted with mad cows and Englishmen.

FDA to immediately release blood products quarantined for CJD investigation

August 28, 1998 Health News Daily via NewsEdge Corporation
Office of Blood Research and Review Director Jay Epstein, MD, told the HHS Advisory Committee on Blood Safety and Availability Aug. 27. Before the announcement, FDA required manufacturers to quarantine blood-derived products while the agency investigated whether a donor to the blood pool from which the product was made was at increased risk of developing Creutzfeldt-Jakob Disease.

"Following this announcement, we will rapidly communicate to the manufacturers the ability to release products currently held on quarantine under the prior policy. The decision should rapidly affect availability. The paperwork will follow," Epstein said. He added that the next step will be for FDA to "issue a revised guidance concerning the withdrawal recommendations for CJD."

Epstein clarified FDA's intentions after U.S. Surgeon General David Satcher, MD/PhD, told the committee that the Public Health Service had considered the committee's May recommendation to end the policy of withdrawal for CJD risk-implicated blood lots. A review of national mortality surveillance data "continues to show the absence of CJD occurring among patients" who have diseases that require blood transfusions and blood products.

Surveillance data from 1979 to 1996 were analyzed, "during which time a total of 4,164 cases of CJD were reported to CDC," Satcher said. Therefore, over 18 years the rate of CJD occurrence was about one person in one million per year. "The absence of CJD among the estimated total of 17,000 hemophilia patients in the U.S. has continued despite increased CJD surveillance, and I think an increase in publicity," which points towards no increased risk of CJD over the general population, he surmised. Autopsies of about 30 hemophiliacs who died with neurological symptoms showed no sign of CJD, he reported.

Satcher added that among hemophiliacs, "the absence of cases of CJD in children and adolescents aged 5-19...provides strong additional evidence that blood and blood products are very safe. FDA set forth the policy to withdraw products made from blood plasma lots determined to be at an increased risk for CJD contamination, following the recommendations of the Transmissible Spongiform Encephalopathies Advisory Committee. The guidelines called for withdrawal of products made from a plasma pool that was later found to have been donated into by a person who later died of autopsy-confirmed CJD, had a family history of CJD, or received a dura mater transplant from a pooled source.

In an October 1997 meeting, the TSE committee recommended some relaxations to the withdrawal guidelines, to exempt from withdrawal those products containing blood products with lower change of harboring CJD infectivity such as albumin. In response to a House inquiry into the IV immune globulin shortage, the General Accounting Office found that 38 withdrawals had been initiated because of CJD between December 1996 and April. GAO estimated that, because of the quick usage of the product, only about 6% of withdrawn IVIG actually was returned to the manufacturer and destroyed due to the shortage. Manufacturers pointed out that output was further decreased 5%-10% because of quarantine periods.

The IVIG shortage factored into the decision to change the CJD policy. " Even one CJD-related withdrawal can have a substantial effect on the supply of IVIG," Satcher said. PHS also considered "that despite the emergence of new variant CJD in Europe and the increased attention and concern for CJD in general, the emergence of this agent in countries in Europe, unlike the U.S., did not initiate a policy of withdrawal or quarantine...for the classic form of CJD," Satcher said.

While no cases of nvCJD have been observed in the U.S., if a case is discovered and that individual has donated blood, it will trigger a withdrawal of blood products from that lot. New variant CJD has infectivity detected in the tonsil and spleen, while infectivity for classic CJD has not been detected in these areas.

U.S. seen removing white cells from donated blood  Aug 28, 1998
NEW YORK Within several years, all blood donated in the United States will routinely be filtered to remove white blood cells, which carry viruses that often infect patients who receive transfusions, government and blood-collection officials told Reuters.

Their predictions follow Britain's decision on July 17 to remove white blood cells from all donated blood to protect against the "theoretical risk'' of the human-variant form of Creutzfeldt-Jakob disease (nvCJD), also known as Mad Cow disease. U.S. health officials say there is no proof that nvCJD is transmitted by blood, but they support removing white blood cells for other, compelling general health reasons. Several other European countries are also removing white blood cells from stored blood, either to guard against the new variant of CJD or as a general health safeguard. They include Ireland, France, Norway, Portugal, and Austria.

"I think within the next two years virtually all the blood in the United States will probably be treated to remove white blood cells,'' said Dr. Harvey Klein, chief of transfusion medicine at the National Institutes of Health.

"Blood is sacred to the American public, which is less concerned about the extra cost than it is having the safest possible unit of blood for themselves, their mothers and grandparents,'' Klein told Reuters. The special fine-mesh filters used to remove white blood cells from blood cost $15 to $20 each, he said. One filter is needed for each transfusion. On average, a pint of blood costs about $75 in the United States.

"Even if white blood cells harm only a small percentage of people, if it is logistically easy to remove them, we should,'' Klein said. Filtration is also advisable to prevent new pathogens from entering the blood supply, he said. All U.S. blood donations are already filtered to remove blood clots and other rough debris. But fewer than 20 percent are believed to be filtered to remove white blood cells, also known as leukocytes.

Three components of blood are useful to patients receiving transfusions: red blood cells, which carry oxygen; platelets, which enable blood to clot; and plasma, the clear fluid portion of circulating blood. Leukocytes, whose job is to rid the body of invading foreign organisms, provide no benefits to most transfused patients but expose them to numerous health risks, according to many health experts.

"White blood cells are a contaminant, not the actual product or cell you're trying to transfuse into patients,'' said Dr. Celso Bianco, president-elect of America's Blood Centres, a nonprofit organisation that collects and distributes donated blood. He said one of the most troublesome viruses carried by leukocytes is cytomegalovirus, which causes serious lung and brain disorders in premature babies and can cause pneumonia and retinitis in patients receiving organ transplants.

Transfused leukocytes also cause fevers in many transfused patients, Bianco said, and can stimulate alloimmunization or the development in blood recipients of antibodies that kill platelets received in future transfusions. In addition, leukocytes transmit the human T-cell lymphotropic virus, which has been linked to neurological disorders and a rare form of leukaemia.

Bianco said he expects all whole blood and separated units of red blood cells and platelets to be filtered within two to five years to remove leukocytes by a procedure formally known as leukocyte reduction. (Plasma does not need to be leukocyte-filtered because white blood cells are centrifuged away in the process of separating plasma from blood.) "It's a good thing for patients and I see no reason to delay, including additional costs,'' Bianco said. Two companies sell over 90 percent of the fine-mesh filters used for leukocyte reduction in the United States: Pall Corp. of East Hills, N.Y., and Baxter International of Deerfield, Ill.

Mark Weinstein, hematology director for the U.S. Food and Drug Administration, said Britain's decision to remove white blood cells from donated blood had brought the issue to the fore in the United States. He said government and industry representatives would meet September 18 in Bethesda, Md., to discuss whether, and how soon, the United States should step up its level of leukocyte filtration.

"Our blood is safe and could be made safer, and that's what we have to examine,'' Weinstein said, noting the FDA is the lead federal agency in regulating blood products. Dr. Richard Davey, chief of biomedical services for the American Red Cross, said he would like to see all blood products leukocyte-reduced without delay.

"We're concerned about the extra cost, but the American health system will bear it. The extra expense is well worth it to keep patients healthy and safe,'' Davey said.

The American Red Cross and America's Blood Centres each collect and distribute about 40 percent of blood donated in the United States. Hospitals collect most of the rest.

CJD sufferer died before her mother's eyes

Aug. 27/98 PA  News Jeanette Pearson
A mother described today how she looked on helplessly as her daughter died before her eyes as she battled against new variant Creutzfeldt-Jakob disease. Pat Mellowship, 58, nursed Donna for three years after she was reduced to a "five foot baby" as she died from the incurable disease.

"It was one of the hardest things that this family has had to go through ... having to watch our daughter die," Mrs Mellowship told Hornsey Coroners Court, north London. "We also had to watch the suffering of our other children and grandchildren. We felt helpless. "The pain of losing Donna will stay with us forever. All we can do now is to keep on going for the sake of her children. That's what Donna would have wanted us to do."

She added: "You do not expect your daughter to die before you. It's particularly hard to come to terms with the death of a young person, particularly of a disease that should never have been allowed to happen." Donna, 34, from Somerset Close, Lordship Lane, Tottenham, north London, died of new variant Creutzfeldt-Jakob disease (nv CJD) on December 31 last year. She left two children Natalie, 15 and Martin, 10, and separated from her husband Keith Boxhall partly as a result of her illness, the court heard. In a written statement to coroner William Dolman, Mrs Mellowship said: "One thing to note is that my daughter had always been a keen consumer of meat ... in particular meat pies and crispy pancakes. She never had much money and always shopped at cheap stores ... and bought brand name products which were cheapest at the time."

Mrs Mellowship told how she had a close relationship with her daughter who lived nearby before eventually moving in with her mother as her health deteriorated. She said "Donna was one of my four children, Stephen, Diane and Karen. She was a home-loving fully animated young woman and all she wanted to do in her life was to be a mother."

Mrs Mellowship told the court that during the late 1970s and 1980s Donna had worked at Woolworths "which involved food preparation and dealing with uncooked meats". She then described how her daughter's health gradually began to deteriorate.

"On or about January 1996 Donna was not herself. Before she was content to sit at home with a cup of tea and a cigarette, she suddenly became very withdrawn and depressed and we all thought she was having a nervous breakdown. She was very tearful, her speech began to slur and she complained of feeling tired."

On several occasions in 1995 she described how Donna fell asleep during the day, "as if she had no interest in life or living". Mrs Mellowship said Donna began to shake like a leaf and was forgetful and began to drag her foot as she walked which caused her to trip over. As her condition deteriorated, Donna went from the Royal London Hospital in Whitechapel to the North Middlesex Hospital and back to the Royal London Hospital before she moved in with her parents.

The court heard how doctors had branded her attention seeking and said Donna would "snap out of it".

Donna was finally diagnosed as suffering from nvCJD in the Royal London Hospital and after that the family nursed her in their own home. Mrs Mellowship said: "Donna continued to deteriorate, she was now forgetting to feed her children which was uncharacteristic of her as she was a natural mother whose life revolved around her children.

"She was crying and lashing out as if she was attempting to hit someone, Donna was not a violent person. "She couldn't remain still, she fell out of bed on various occasions, she couldn't remember to wash, clean her teeth or put on clean clothes. She did not know how to perform the simplest of tasks, we had to do everything for her." As Donna's condition got worse, Mrs Mellowship said: "It was just like nursing a five foot baby, it was not easy. "We were looking after her on our own, we were having a horrendous time. Donna deteriorated rapidly, she was dying before my eyes."

Coroner William Dolman ruled Donna's death was by misadventure. He said: "The evidence we have heard today gives a long and tragic story. It's a particularly sad and unremitting decline. "Donna died at the age of 34 from the disease. It's clear that certainly from the beginning of 1996 to December 1997 there was a long, painful decline of this very cruel disease, cruel but extremely rare."

In her statement, Mrs Mellowship described how Donna used to crawl towards the bathroom in an effort to indicate she wanted to use the toilet and her weight plummeted from around 13 stone until she looked anorexic. Finally in September 1996, Donna was diagnosed to have nvCJD at the Royal London Hospital. Mrs Mellowship said: "It was the most awful moment of my life, I then had to tell her father, sisters and her brother. Before we brought Donna home we had to tell Donna's children. It was one of the hardest things to do.

"The children went crazy, running around and screaming it wasn't true. It was terrible to have to tell them the truth. Our lives were turned upside down, we were in terrible shock." Mrs Mellowship described how the family had to fight to get things for Donna but they were given a hospital bed and a feeding pump from the Royal London Hospital but nothing else, she said. The family had an early Christmas in December 1996 as they thought she was going to die which Mrs Mellowship described as a happy and sad time.

Leading CJD expert Dr James Ironside, consultant neuropathologist from the CJD surveillance unit in Edinburgh confirmed the cause of death was CJD new variant. Giving evidence in court, he said "In the 1980s this disease was caused by prions, an abnormal form of protein. The normal is present in us all but in this group of diseases, protein changes shape and accumulates in the brain. What is thought to occur in nvCJD is that we believe the BSE prion is introduced into the brain and starts a chain reaction."

Dr Ironside said that the incubation period of the disease is unknown but said it's likely to be many years. Referring to the restrictions placed on the use of cattle and beef farming, the coroner asked Dr Ironside what the chances were of the public getting BSE. Dr Ironside said: "I think it's very unlikely for a number of reasons."

Dr Ironside explained that the removal of cattle organs including brains is now rigorously enforced and said BSE in cattle is much lower now than it was in the 1980s. He said "The chances of being exposed to BSE are very remote." Dr Ironside agreed with the coroner that it was near zero. He added: "I think that the risks from eating meat on the bone are negligent."

The coroner then asked if the public are safe to eat oxtail. Dr Ironside replied: "I think a number of lessons have arisen from this whole episode. I do not think it's a good idea to consume neurological tissue from any species because there are a lot of issues we do not know about. If you are aware of the risks the decision is up to you, I personally would not eat oxtail," he said.

Dr Ironside referred to the mass screening for the human form of BSE following the discovery of evidence of the disease in a patient who had his appendix removed. He added: "This is an important opportunity, we are planning to go ahead on that."

The coroner added during his summing up: "Twenty-seven deaths have been confirmed over all these years and maybe five more out of a 57-58 million population. We are dealing with something that's extremely rare. My verdict has to be one on the basis of probabilities. The cause of death is CJD new variant. She died as a result of misadventure. "Perhaps some reassuring information that the likely cause of this tragic illness seems to have been removed from the food chain, although perhaps we should not eat oxtail, we can now safely eat meat on the bone."

After the inquest the family's solicitor David Body said they were now considering a compensation claim and a letter was to be sent to the Government's Chief Medical Officer. He said the family were calling on the Chief Medical Officer to make better provision for "the care needs of the people who have this disease and better provision so that people with the disease can maintain some kind of dignity". Mr Body said the family wanted a central fund set up to provide cash for victims.

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