nvCJD confirmed in newborn
Should they be told: other exposed British mothers
Girl, 15, becomes latest 'mad cow' victim
US blood ban kicks in
Red Cross: UK Travel/Residence Deferral: Questions and Answers
Monthly CJD statistics
Japan pulls Danish thrombin
5 March 2000 Jonathan Leake Sunday Times
| Mother and her four-month-old
daughter. Photograph: Simon Townsley Can CJD, the fatal brain disease linked to BSE, be passed from mother to child ? Doctors suspect this baby is the first case... They took her baby away before dawn. It was less than 24 hours after the mother - let us call her Janet [names have been changed to protect identities] - had given birth, but already the medical staff could see her baby was failing to feed and in urgent need of help. The nurses were not too surprised. Janet, who cannot be identified for legal reasons, had spent two weeks of her pregnancy in the hospital, diagnosed with severe depression. She had also undergone the trauma of a caesarean section. For a few days, they thought, they would have to help her and the baby get used to each other, and assist with feeding. But that night turned out to be the last Janet would spend with her little girl, Amanda. The depression grew worse; most worryingly, not all was well with the baby, which reacted strangely to tests. |
It was another two months before the pieces of the jigsaw finally fell into place. By then doctors, realising Janet's condition was unlike any usual psychiatric illness, had put her through an intense set of brain scans: to their dismay, they found degenerative changes indicative of new-variant Creutzfeldt-Jakob disease (nvCJD). Further tests confirmed the presence of abnormal "prions" - the agent found in cattle suffering from BSE, known colloquially as mad cow disease.
New variant CJD has claimed the lives of 51 people in Britain. The only thing they had in common was youth - most were between 18 and 40 years of age - and a genetic make-up that is shared by about 40% of the population. Though the disease can only be diagnosed with complete certainty after death, more than a dozen other people are believed to have the symptoms . But up to now there has been no suggestion that the devastating condition might pass from mother to child. Baby Amanda may be about to change all that.
Even after several weeks, Amanda was failing to swallow and had to be fed through a tube. She also seemed unable to gain significant weight. Doctors, filled with foreboding, tried a brain scan. They saw what the experts had prayed would never happen: the lesions and plaques indicative of nvCJD in a child that had never eaten meat. Nor does the damage seem static. It is getting worse.
The diagnosis, which is likely to be confirmed by other tests within the next few weeks, means the threat already hanging over everyone who ate infected beef products in the 1980s and early 1990s, when BSE was at its height, could extend to future generations. The seemingly long incubation period of vCJD means that Amanda may be the first, but not the last, child born with the disease.
It is four months after Amanda's birth and Janet is getting ready to see her baby. Her mother, Sara, and a nurse wash and dress Janet, and support her as she staggers downstairs, head rolling, arms waving. Home is a suburban, three-bedroom semi in a quiet cul-de-sac; a peaceful setting at odds with the drama within.
Amanda arrives in the morning, brought from the nearby hospital where she is cared for at night. Janet can still speak, but the mental deterioration she has suffered makes her speech and mannerisms childlike. Her balance has become so unsteady she must drink from a toddler's beaker.
Repeatedly, she says she is waiting for her baby to arrive. "She's gorgeous, she's so lovely," she says, over and over again. After a few minutes the baby does arrive, in the arms of the nurse who accompanies her wherever she goes, helping to feed her through a tube and dealing with emergencies.
Today, however, Amanda is fine. She and Janet sit together on the sofa, the proud mother trying to control her own drooling and involuntary shaking while also trying to wipe away the saliva that trickles from her daughter's mouth. Amanda, however, hardly responds. At four months old she weighs less than 10lb, is unable to see and makes little more than yelping noises.
Sara builds the day around the time her daughter and granddaughter spend together - six hours. She knows that, probably in just a few months, Janet is unlikely even to remember she has a baby - let alone recognise Amanda.
The doctors have warned her that the abnormal prions that have rampaged through the part of Janet's brain that control short-term memory and motor function are now attacking the rest of her mind. She has been told there is no hope of a cure. "I was so proud of my daughter and pleased to be having a grandchild. I could never have imagined that I would be nursing both of them to their deaths," says Sara.
"We are just an ordinary family, but we're being destroyed by a man-made disease that should never have happened." Just two years ago Janet was preparing to be married. She was a happy, talkative and affectionate young woman, full of life. "She was always laughing, telling jokes and making friends wherever she went," says her mother. "She was out most weekends, dancing or meeting people."
Born in the south, Janet was raised in the Midlands by her mother after she separated from Janet's father. The women were not just mother and daughter, but friends. When she was 18, Janet joined Sara in a catering business they ran with success for some years. Janet's bubbly character hid a sharp business edge.
"It was hard, but Janet made it work," recalls Sara. "She was so cheerful and friendly that the lads all loved her. I hired the staff and organised the cooking and she looked after the customers. Thanks to her, we did good business." The same cheerful nature and good looks attracted would-be boyfriends. In her early twenties, Janet was engaged to a young man called Andrew with a wedding planned for mid-1998.
Looking back, Sara realises that the fraught months before the wedding were the first sign something was seriously wrong . Just as the ceremony and reception had been booked, Janet called it off. She never gave a reason and Andrew was devastated. "He was distraught," said Sara. "He begged her to come back, but she wouldn't have any of it."
The next few months saw Janet plunged into frequent depressions and moodiness , which Sara had never seen before. They were just the start. Later she also began suffering from pains in her back and legs, which made her mental state even worse.
It was during this time that Janet struck up a new relationship with another young man. It was a troubled match and the couple parted in late 1998, only to reunite shortly afterwards. When Janet became pregnant she was delighted at first - but the disease was stealthily taking its toll.
The pregnancy that followed was, says Sara, a nightmare. Janet's personality was deteriorating as the disease got more of a grip and, as with many nvCJD victims, she went through a phase of being nasty to everyone around her. "One of the midwives told me they had never met anyone as evil as Janet," says Sara. "We all still thought she was depressed at her relationship breaking up. Nobody spotted how ill she really was."
Twice during her pregnancy Janet's mood became so black, the pains so severe and her ability to carry out simple tasks so poor that she was admitted to hospital. Doctors could find nothing wrong and she was not allowed to stay more than a few days.
Despite her extreme suffering, the doctors had not progressed beyond their diagnosis of acute depression by the time Janet came to give birth. Only some weeks later, in November last year, did she undergo the brain scan that indicated she was suffering from something worse.
Many people with vCJD have been diagnosed so late in the course of the disease that they are no longer capable of understanding what is happening. Not so with Janet.
"She has twice told me she knows she has got mad cow disease and that she is going to die," said her mother. "I hated to think what must have gone through her mind when she lay in bed at night." Janet now uses sedatives to help her sleep.
All known cases of vCJD have proved fatal, which is why the implication of her daughter's preliminary diagnosis - that transmission of the disease from mother to child is possible - is so worrying. Experts, however, have expected it.
While emphasising that he cannot comment on individual cases, John Collinge, the Medical Research Council's professor in charge of prion research, said: "It was something that was always on the cards. In sheep scrapie, a similar prion disease, the disease passes from ewes to their lambs. There is good evidence that in cattle about one in 10 infected animals transmits the disease to a calf.
"The prion that causes BSE is identical to the one found in humans with nvCJD, so it is logical that there would be a risk of nvCJD jumping from mothers to children."
If Amanda's diagnosis is confirmed, scientists will want to find out just how she got it. One possibility is that the nvCJD prions can pass across the placenta - something that has already been observed in sheep.
Fred Cohen, professor of pharmacology at the University of California and co-author of the Nobel prize-winning research that identified prions as the agent behind BSE and nvCJD, said the diagnosis on Amanda was "very scary" .
"For her to be showing the symptoms of nvCJD so early she would probably have had to contract it in the womb, perhaps very early on in her foetal development," he said. "It means any woman unknowingly incubating the disease could pass it to their children, too. If that potential is proven it makes this whole situation much more serious ."
What happens to Amanda will be of concern to one small group - the children of other women who have died of nvCJD. There are believed to be at least three such cases , all currently healthy. Anthony Bowen, whose son was born three weeks before his wife Michelle died of the disease, has told interviewers how he lives with the prospect of his son also becoming ill.
Such concerns are, however, already way beyond Janet's understanding. All she knows is that, for now, she has a beautiful baby to try to look after. As Sara said: "They haven't got much time left and all I want now is to make sure they spend as much of it together while they can still enjoy each other."
Saturday 29 January 2000 By Samantha Savage, The IndependentAn unnamed 24-year-old woman is in a serious condition with a suspected case of new variant Creutzfeldt Jakob Disease, the human form of mad cow disease. She is being cared for at Walsgrave Hospital, Coventry, where she is being examined by specialists in the the brain disease. The Hospital's chief executive David Loughton said that doctors were still awaiting the result of tests to establish the diagnosis.
Mr Loughton said the woman, who has not been named, was admitted to the hospital earlier this month. She was being cared for on a neurology ward, he added.
The woman's three-month-old daughter is also seriously ill in hospital, but the child is not thought to have the same illness. The woman will be taken to St Mary's Hospital in Paddington, west London, for tests next week, but her baby will not undergo further examination for the brain disease, which is believed to have an incubation period of at least four years.
Mon, Mar 6, 2000 Reuters World ReportSeven women in western England may have been infected with the human form of mad cow disease while giving birth, but it is wrong to warn them because they would only "worry," a regional health authority said on Monday.
Instruments used last year in a Caesarean section on a woman who was later found to be suffering from a new variant of Creutzfeld-Jakob Disease (CJD), were repeatedly reused after the October birth. It is now believed the the germ can survive the sterilisation of surgical instruments. The woman's baby has since been found to suffer from a neurological condition, sparking fears she may be the first British child to inherit the illness from its mother.
"They (the instruments) had been used seven times," West Midlands Director of Health Dr Rod Griffiths told BBC radio. "We know who the patients are, but no we haven't got in touch with them because ethically it's not at all clear whether that's the right thing to do," he said. Any risk was "vanishingly small."
Griffiths said the fact there was as yet no clear proof CJD could survive sterilisation on the instruments -- used between October 26 last year and January 29 this year -- meant it would be irresponsible to warn the women who may potentially have contracted it.
"If...I say to you, you may have been exposed to a germ, we don't know whether there was any left on the kit or not, it might be enough to give you the disease it might not, because we've no idea what the effective dose is," he said, "It doesn't seem to me to be the sensible thing to do." "Once I've told you, you can forget getting life insurance and you can worry about it for the rest of your life. Of course it may never affect you at all," he added.
While the hospital has not been named for legal reasons [George Eliot Hospital in Nuneaton, Warwickshire, with transfer to Walsgrave Hospital, Coventry -- see 29 Jan 00 and 14 Mar 00 newspaper stories -- webmaster], Griffiths said General Practitioners (GPs) in the West Midlands had been told they can tell patients who fear they have been infected as long as they ask first. "I don't want to tell people unless they ask, because if they ask it's a quite different game. But if I go out and give the bad news it ruins their lives, when it need not," he said.
Scientists say CJD is caused by eating meat from cows infected with the fatal brain-wasting illness bovine spongiform encephalopathy (BSE), or mad cow disease. Statistics show that nine people died last year from the new variant of CJD, which occurs when a brain protein called a prion changes and folds in an unusual way.
Sun, Mar 5, 2000 By Matthew Cooper, PA NewsA Department of Health investigation was under way today after surgical instruments used to deliver a baby whose mother was suffering from the human form of mad cow disease were unwittingly used by maternity ward staff for two months after the birth. The instruments, which may have been contaminated with the agent which causes new variant Creutzfeld Jakob Disease, were re-used in a small number of Caesarean sections before the mother was diagnosed as having the disease.
Dr Rod Griffiths, the West Midlands' Director of Public Health, said the risk that the women, thought to number less than 10, had been infected with CJD during the subsequent births was "vanishingly small". Dr Griffiths added that staff at the hospital where the baby was born had followed proper procedure, removing the surgical instruments from use as soon as the mother's condition was known.
The doctor added that it was theoretically possible for the prion which causes CJD to remain on instruments after operations, but it was usually removed simply by washing prior to sterilisation. The equipment which was re-used was properly washed and sterilised between operations, Dr Griffiths added.
The mother diagnosed as having CJD gave birth last autumn at a Midlands hospital. Neither the mother nor the hospital can be named for legal reasons. Her daughter is also thought to be suffering from a neurological condition and doctors are awaiting the result of tests to see if she also has CJD.
Asked to quantify the risk to those mothers exposed to the instruments, Dr Griffiths added: "When something has never happened, you don't know whether it's one in a million or one in a thousand." ....
Comment (Rachel Shepherd, New Zealand): "In my opinion the mothers and children who have been exposed should be told. It is their right to know and act appropriately. It is not enough to tell the family doctor, many people to not keep in touch with one doctor and although records can be forwarded to a new doctor they frequently are not forwarded.
What happens when one of these mothers gets pregnant again? What happens if either mother or child need surgery? Are we going to contaminate yet more unsuspecting people and say "it was an accident, it could not have been avoided"?
What happens when the mother or child show symptoms? There is the delay in treatment and the damage to a family in which a loved one is going insane. The only reason I can see for this "paternalistic" attitude is fear of legal action or damage to reputations."
Comment (J Ralph Blanchfield, Institute of Food Science & Technology, UK): "The reported decision not to tell the women is morally wrong, and dangerous if effective. Actually it is almost certainly ineffective, because the CJD victim gave birth in November 1999 and the instruments were used for two months after that; and (as was inevitably going to happen)at least one newspaper has named the hospital. So the women at risk (plus some others not at risk) will already know.
Why did it take two months after the delivery to diagnose in the mother? One can only speculate, but in other cases of vCJD (sorry, Roland, but vCJD is a convenient "shorthand") the early symptoms were assumed to be psychiatric. Assuming that in due course the disease is confirmed in the infant, it suggests an extraordinarily short incubation period, almost certainly beginning while a foetus."
Comment (Roland Heynkes, Germany's TSE expert): " Is it really ok not to tell them, that they can live without this fear? But perhaps there may be many more patients with developing CJD who all contaminate British surgical instruments. Indeed contaminated surgical instruments may be not be a problem for many British women, because they are already infected directly from cattle. What a depressing situation of uncertainity!"
COMTEX Newswire Mon, Mar 6, 2000 See also CJD Blood Recall for individual stories"Due to new blood donor deferral guidance issued by the U.S. Food and Drug Administration (FDA), beginning today the American Red Cross Blood Services will no longer be able to accept blood donors who have spent six months cumulative time or more in the United Kingdom (England, Northern Ireland, Scotland, Wales, Isle of Man or the Channel Islands) between 1980 and 1996.
Blood banking organizations in the United States are implementing this precautionary deferral under the FDA's guidance, which is intended to reduce the theoretical risk of transmitting new variant Creutzfeldt Jakob disease (nvCJD) through blood transfusion. The FDA regulates blood collection, processing, testing and distribution within the United States.
The Red Cross is working to help donors understand the deferral criteria and to lessen the possibility of donors self-deferring unnecessarily. The new deferral criteria are expected to decrease blood donations nationwide by 2.2 percent or approximately 285,000 units annually. To help ensure the blood supply remains stable, the Red Cross encourages all eligible blood donors -- especially those who have not donated before -- to call 800-GIVE-LIFE and schedule an appointment to donate blood.
"The Red Cross is committed to the safety of the blood supply and to helping our donors understand how this new deferral criteria affects them," said Dr. Richard Davey, chief medical officer for the American Red Cross. "We expect that many of our donors will have questions and we want them to understand that this is a precautionary measure by the FDA. The Red Cross and other organizations continue to research this disease. There has never been a case of nvCJD documented in the United States, and this disease has never been found to be transmitted through blood." [Absence of evidence is not evidence of absence x 2 -- webmaster]
The Red Cross has invested more resources than any other private organization in the United States to understand the relationship, if any, between CJD/nvCJD and blood transfusion. The Red Cross will share its data with the FDA for use when evaluating these deferral criteria in the future.
Blood centers within the United States have until April 17 to adopt the deferral criteria. The deferral criteria also calls for the indefinite deferral of donors who received bovine insulin (made from cattle) since 1980, unless the donor is certain that the product was not manufactured from cattle in the United Kingdom between 1980 and 1996.
New variant CJD, a fatal neurological disease, has been linked to the consumption of beef from cattle infected with bovine spongiform encephalopathy (BSE), commonly referred to as "mad cow disease." While there is no evidence that nvCJD is transmissible by blood and there have been no cases of nvCJD documented in the United States, the FDA, as a precautionary measure, has issued guidance for blood centers to indefinitely defer individuals from donating blood who fit the new criteria.
"Any deferral criteria has an impact on our donors and on the availability of the blood supply," Davey continued. "We are concerned about the anticipated impact this deferral will have on blood collections and will continue our efforts to recruit new, eligible blood donors into the Red Cross family to ensure the adequacy of our nation's blood supply for patients in need."
On any given day, the nation's blood supply is fragile. More blood donors are needed to counter the anticipated loss of annual donations caused by these deferral criteria. Donors should call 800-GIVE-LIFE to schedule an appointment to donate blood at the American Red Cross. To be eligible to donate blood, you must be at least 17 years or older, weigh at least 110 pounds and be in good general health. Donors with questions about the new deferral are encouraged to visit the Red Cross's Web site.
For more than 50 years, the American Red Cross has been the primary supplier of lifesaving blood and blood products in the United States. The Red Cross collects blood voluntarily donated by approximately 4.5 million donors, annually providing more than 6 million units of blood for patients nationwide. The Red Cross also enhances and saves the lives of thousands of patients each year by supplying one-quarter of the nation's tissue for transplantation, through its network of 15 tissue centers nationwide, and conducts advanced, highly sophisticated biomedical research that focuses on improving blood safety and developing potentially lifesaving products."
Opinion (webmaster): Indeed, the ban raises the question of how one helps donors understand why they can no longer give blood. Perhaps something like this:
"You can't give blood because you probably are infected with mad cow disease but not to worry because you probably aren't infected with mad cow disease."
If the donor asks to be tested for nvCJD, explain that there is no test and no point in testing because the disease is always fatal, it is like the early days of hepatitis and AIDS.
If the donor asks for the scientific basis for setting the visitation ban at 6 months and for many months of delays in implementation, say that time period was chosen because this gives an acceptable impact to the blood industry.
If the donor asks whether blood products from earlier unsuitable donors will be recalled or used up, say inventories will continue to be used until they can all be sold because this gives an acceptable impact to the blood industry.
If a donor who received bovine insulin injections for diabetes asks how to find out whether the product was manufactured from cattle in the United Kingdom between 1980 and 1996, say this is not possible but not too worry, the 100,000 higher efficiency of injection relative to dietary route might be offset by lower infectivity levels.
If the donor asks why FDA is instituting this ban 15 years into the BSE epidemic and 6 years into nvCJD, after millions of units of blood from British residents have already gone through the system, say it is all about risk reduction.
If the donor asks about funding of research into CJD, say it is such a rare disease that it is best to wait until a large number of cases shows up.
6 Mar 00 American Red Cross Press Release See also CJD Blood Recall for individual storiesUnited Kingdom Travel/Residence Deferral: Questions and Answers
The American Red Cross is working to help donors understand the deferral criteria and to lessen the possibility of donors self-deferring unnecessarily. The new deferral criteria are expected to decrease blood donations nationwide by 2.2 percent or approximately 285,000 units annually. On any given day, the nation's blood supply is fragile. More blood donors are needed to counter the anticipated loss of annual donations caused by these deferral criteria.
To help ensure the blood supply remains stable, the Red Cross encourages all eligible blood donors--especially those who have not donated before--to call 1-800-GIVE-LIFE and schedule an appointment to donate blood. To be eligible to donate blood, you must be at least 17 years or older, weigh at least 110 pounds, and be in good general health.
Q. What is this new deferral? A. The deferral implemented on March 6 by the Red Cross is the result of guidance issued by the U.S. Food and Drug Administration (FDA). The guidance calls for the indefinite deferral of all blood donors who have spent a cumulative time of six months or more in the United Kingdom between the years 1980 and 1996. The United Kingdom includes England, Northern Ireland, Scotland, Whales, Isle of Man, or the Channel Islands.
Q. Why is there a deferral for travel to or residence specifically in the United Kingdom? A. The deferral is being required because the U.S. Department of Health and Human Services, which has oversight over the FDA, believes that individuals who have visited or resided in the United Kingdom may be at risk for exposure to new variant Creutzfeldt Jakob disease (nvCJD). The deferral is intended to reduce the theoretical risk of transmitting nvCJD through blood transfusion.
Q. What is nvCJD? A. This disease is a rapidly fatal neurological syndrome. The new variant of CJD is similar to what is called "classic" CJD, but it has less specific symptoms and is harder to diagnose. In addition, nvCJD has primarily been found in a population much younger (19- to 50-years old) than that of classic CJD and runs its course in about 18 months. There have been more than 50 deaths as a result of nvCJD, all but three of which occurred in Britain.
The cause of nvCJD is unknown, but it has been linked to eating beef infected with bovine spongiform encephalopathy (BSE), commonly referred to as "mad cow disease." The time considered most relevant to the BSE epidemic in the United Kingdom was between the years 1980 and 1996.
It is important that donors deferred as a result of this policy understand two things--
1.The deferral is a precautionary measure only. There have been no reported cases of travelers to the United Kingdom becoming infected with nvCJD, and there is no evidence that this disease is transmitted through blood. Additionally, there have been no reported cases of BSE or nvCJD in the United States.
2. The Red Cross is required by the FDA to follow the agency's guidance and regulations. Failure to comply with guidance and regulations could result in a Red Cross blood center being shut down.
Q. Is nvCJD transmissible? A. There is no evidence that nvCJD is transmissible through blood or other means. NvCJD has been associated with eating beef infected with BSE, but other modes of transmission have not been proven.
Q. Why is the cutoff for the deferral six months? A. The FDA selected the six months or more travel/residence cutoff based in part on the estimated decrease in blood donations the deferral would cause. As a result of this deferral, it is anticipated that U.S. blood centers will lose 2.2 percent of annual blood donations. Had the FDA selected three months or even one month as the cutoff, the estimated annual loss of donations would have been 3.4 percent or 6.4 percent, respectively.
Q. Will this deferral cause a blood shortage? A. The blood supply in the United States is always fragile, and the Red Cross relies on the generosity of volunteer blood donors to give blood to help patients in need. The Red Cross is concerned over the anticipated impact this deferral will have on blood collections and we will continue to recruit new, eligible blood donors to help maintain a stable blood supply. The Red Cross is also actively researching nvCJD and any relationship it may have with blood transmission. We will continue our research efforts and provide our data to the FDA as it evaluates the deferral criteria.
We are very thankful for all donors and encourage repeat donors and those who have never donated to call 1-800-GIVE-LIFE to schedule an appointment to donate blood.
Q. Will potential donors who didn't eat meat while they were in the United Kingdom be deferred? Why can't they donate? A. Implementing these deferral criteria is a precautionary measure only, intended to reduce the theoretical risk of transmitting nvCJD. The Red Cross has implemented these deferral criteria based on guidance issued by the FDA, which regulates all blood centers. The FDA's guidance calls for the indefinite deferral of all blood donors who spent six months or more in the United Kingdom between 1980 and 1996.
Q. Will those deferred under this guidance be able to give blood again? A. This deferral is indefinite. Deferred donors will only be able to donate again if the FDA decides to revise or revoke this guidance.
Q. Do those who are deferred need to be examined by a doctor? A. This deferral is a precautionary measure only. There have been no reported cases of travelers to the United Kingdom becoming infected with nvCJD, and there is no conclusive evidence that this disease is transmitted through blood. Although the exact cause for nvCJD is unknown, it has been associated with eating beef infected with BSE, or "mad cow disease." There is no screening test for the disease; however, your doctor may be able to give you more information about it.
Q. Are people who are deferred under this new criteria sick? Do they have "mad cow disease?" A. According to the FDA guidance, people who have spent six months cumulative time or more in the United Kingdom between 1980 and 1996 may be at risk for exposure to nvCJD. However, because a person may have been exposed to nvCJD does not mean he or she has the disease. There have been no reported cases of travelers to the United Kingdom becoming infected with nvCJD, and there is no evidence that this disease is transmitted through blood.
Q. Why are these people deferred from donating now, if they weren't in the past? A. Deferral criteria do not apply to donors until the criteria is implemented. This deferral criteria is new and did not have an impact on potential donors until the date of implementation, which was March 6. This deferral is based on a theoretical risk only. There is no conclusive data that suggests nvCJD may be transmissible through blood.
Q. How can deferred donors support the blood supply? A. Deferred donors can encourage friends, family, neighbors, co-workers, and others to give the gift of life. The Red Cross also welcomes help as a blood drive volunteer or sponsor. Those interested in learning more about these opportunities should contact their local Red Cross Blood Services region or chapter.
Q. How does the United Kingdom meet its own needs for blood? A. The deferral adopted by the Red Cross and other U.S. blood centers was based on guidance issued by the FDA and applies only to U.S. blood centers. Some other countries, such as Canada and Australia, have adopted similar deferral criteria as precautionary measures.
The United Kingdom still collects blood from its residents for patient use there; however, Great Britain imports its plasma derivative products from other countries. Blood components such as red blood cells and platelets used for transfusion come from a single donor. Plasma derivatives, on the other hand, are made from large pools of donated plasma. However, the separation of plasma proteins from donated plasma for further manufacture into derivative products, referred to as "fractionation," has been shown to significantly reduce the amount of prion proteins. Regardless, Great Britain decided to ban the use of plasma from British donors in its derivative products because the government felt the risk of prion-infected units in a plasma pool contaminating large amounts of finished product was too great.
The American Red Cross does not ship blood or blood components to other countries, nor does it import blood from other countries.
Opinion (webmaster opinion): The Red Cross has came up with an extensive set of questions and answers to help visitors to England understand why they can't give blood. The webmaster had earlier proposed:
"You can't give blood because you probably are infected with mad cow disease but not to worry because you probably aren't infected with mad cow disease."
The Red Cross proposal says you are not exactly at risk for nvCJD but rather at risk for exposure and the ban is all the fault of kooks at FDA, roughly translating, in the webmaster's opinion, to this parody of Red Cross press release:
"The deferral is being required because certain hysterical individuals at the U.S. Department of Health and Human Services, which has oversight over the FDA, believes that individuals who have visited or resided in the United Kingdom may be at risk for exposure to nvCJD. The deferral is a precautionary measure only and not really needed. The Red Cross would never do this on its own but the FDA is holding a gun to our head. Failure to comply with regulations could result in the Red Cross being shut down which would mean the end to medicine in the United States indefinitely, so we are reluctantly complying, even though if it were up to us we would wait for a bunch of nvCJD cases to appear in the US."
One can appreciate the genuine dilemma with the blood supply but still not be of the opinion that melodramatic short-term benefits should be traded off against a very real future risk of another AIDS or hepatitis. Perhaps conserving blood through less elective and terminal-elderly major surgery is an option.
Some statements in the press release are difficult to reconcile with the research literature:
"Q. Is nvCJD transmissible? A. There is no evidence that nvCJD is transmissible through blood or other means."The press release could go into the Guinness Book of Records for most claims within a single document that 'absence of evidence is evidence of absence'. At least it ended on a note of humor:
"There is no screening test for the disease; however, your doctor may be able to give you more information about it."
American families affected by CJD have extensively documented the rarity of good medical information (1, 2, 3).
UK GOVERNMENT: Monthly Creutzfeldt-Jakob disease ...
Mon, Mar 6, 2000
The Department of Health is today issuing the latest monthly table, giving the numbers of deaths of definite and probable cases of Creutzfeldt-Jakob disease in the UK.
Year Referrals Deaths of definite and probable cases in the UK
Sporadic latrogenic familial GSS nvCJD Total
1985 - 26 1 1 0 - 28
1986 - 26 0 0 0 - 26
1987 - 23 0 0 1 - 24
1988 - 22 1 1 0 - 24
1989 - 28 2 2 0 - 32
1990 53 28 5 0 0 - 33
1991 75 32 1 3 0 - 36
1992 96 43 2 5 1 - 51
1993 78 38 4 2 2 - 46
1994 116 51 1 4 3 - 59
1995 87 35 4 2 3 3 47
1996 134 40 4 2 4 10 60
1997 161 59 6 4 1 10 80
1998 152 61 3 3 1 17 85
1999 159 53 5 0 0 11 69
2000 15 1 0 0 0 1 2
To 31 January 2000. Total number of definite and probable cases of nvCJD = 52
The next table will be published on Monday 3 April 2000.
Comment (webmaster): another 14 nvCJD cases are still alive according to the Sunday London Times of 5 Mar 00. There has been an additional confirmed death in Ireland and two further in France. This makes for a total of 69 confirmed cases, giving the UK Dept of Health a under-reporting level of 75.4%.
Mon, Mar 6, 2000 Kyodo World ServiceItoham Foods Inc. said Monday it has recalled a solution imported from Denmark and shipped to pharmaceutical manufacturers for use in hemostatic products, which stem bleeding, because it could have become a source of mad-cow disease.
The company said that hemostatic products made with the solution did not circulate in the market because the manufacturers have stopped processing it and that there is thus no risk of infection. Recalled were two bottles of the solution, weighing a total of 160 grams. The solution is used in the manufacture of hemostatic product "thrombin" for use in surgery, according to the company.
The material, extracted from the blood of cows in Denmark, was imported from the Scandinavian country Feb. 4 and shipped to pharmaceutical makers Feb. 21, Itoham said.
Itoham decided to voluntarily recall the material after an employee discovered on the Internet on Feb. 29 that a cow was found in Denmark to have suffered from mad-cow disease, or Creutzfeldt Jakob disease (CJD), and that all cows in the farm were immediately killed.
According to Itoham, the exporter told the company that the cows whose blood was extracted for the solution had no connection with the farm where the infected cow was found. However, the company notified drugmakers last Friday of its decision to recall the solution because of the possible contamination risk. CJD is a rare, fatal brain disorder which causes a rapid, progressive dementia and associated neuromuscular disturbances.
COMTEX Newswire Fri, Mar 3, 2000The message confirming that Denmark was hit by a case of mad cow disease, BSE, was delayed for three days. The Central Veterinary Laboratory in the UK sent a fax message last Friday (25 February) to Statens Veterinaere Serumlaboratorium in Denmark, confirming signs of BSE in the brain of the cow that was suspected to have the disease.
At this time, the people at the Danish laboratory had already left for the weekend and the message was not found until Sunday (27 February), reported CNN Danmark. The error was not very serious, according to the head of the Danish laboratory said that the results were not expected until the Monday (28 February).
Wed, Mar 1, 2000 Reuters World ReportNorway banned imports of some types of meat from Denmark from Wednesday after an outbreak of mad cow disease in that country. The Norwegian state food safety authority SNT said in a statement that the ban covered beef, mutton and goat meat. It did not say how long the measures would last.
Denmark's government reported on Monday that health authorities had found one milk cow afflicted with bovine spongiform encephalopathy (BSE), the scientific name for the deadly brain-wasting disorder known as mad cow disease. It was the first case of BSE since 1992 in Denmark.
The SNT said Danish meat that did not include any spinal cord, brain or eyes was believed to be safe and was not covered by the ban. The ban applies to animals younger than one year old due to the long incubation period for BSE.
Many Norwegians travel to Denmark on weekend ferry trips and stock up on everything from alcohol to meat, which is far cheaper than in Norway.
Danish consumers have been advised to throw away beef from fridges and freezers and a number of products have been removed from the Danish shops. New slaughter procedures have also been introduced in the country -- the spinal cord, eyes, brain, the spleen and parts of the intestines will be removed from all cows slaughtered, as these are the parts that are most dangerous if the animal is infected.
The Finnish ministry of forestry and agriculture is not worried about the mad cow disease discovered in Denmark, although about 3.8m kilos of beef was imported from Denmark to Finland last year. The ministry is currently awaiting the investigation results from the Danish authorities.
In Sweden, all imports of Danish beef have been stopped and grocery stores have removed the meat from the counters. It appears that the storeowners want to be sure that the meat is safe and imports have been stopped until further information is provided. There have been suspected cases of BSE in Sweden, but when the animals have been tested none were infected...
In January-November 1999, Danish beef exports totalled 90,000 tonnes, of which three-quarters went to other European Union countries, primarily Italy, Germany and Spain. Outside the EU, Russia was the biggest export market for Danish beef.
Ireland with 350 registered incidents of BSE continued to ship beef abroad while Portugal with almost 200 cases had seen beef exports at standstill for some 18 months, Meland said. Britain, the European country worst hit by BSE, has registered some 180,000 cases of mad cow disease.
Denmark's only previous known incident of BSE was in August 1992 when one single animal -- an imported Scottish highland cow -- was found to have contracted the disease. At that time, the whole herd was put down and cremated immediately. The one cow now found with BSE was of pure Danish origin. It was slaughtered immediately, the ministry said. The 73-animal herd it belonged to was put under observation.... Denmark has some two million cattle, 700,000 of which are cows.
Wed, Mar 1, 2000 By Rachael Crofts, Consumer Affairs Correspondent, PA NewsA meat inspector has been suspended after a piece of spinal cord was found hanging from a health-marked beef carcass at an abattoir, the Ministry of Agriculture disclosed tonight. An investigation into events surrounding the discovery has now been launched, MAFF said.
The discovery was made during an unannounced check by the State Veterinary Service at an abattoir in the North West, as part of ongoing MAFF audits of Meat Hygiene Service controls. The two-and-a-half-inch-long piece of material was removed and disposed of in accordance with the regulations, MAFF said. A MAFF spokesman added: "There was therefore no risk to public health."
Removal of spinal cord is one of the most important of the Specified Risk Material (SRM) controls introduced in the wake of the BSE inquiry. Meat hygiene inspectors are instructed to ensure all traces of it are removed from carcasses. The regulations demand the removal of all SRM, which consists of tissue in cattle and sheep which might harbour BSE infection.
Tissue which has to be disposed in a controlled way includes the head, spinal cord, tonsils and spleen of animals aged over six months and the thymus and intestines of animals of all ages slaughtered for human consumption.
A spokesman for MAFF said: "An investigation has been mounted into events surrounding the discovery. "The meat inspector responsible for the final inspection of beef carcasses has been suspended pending its results.
"No further breaches were found by an unannounced follow-up visit to the abattoir by the State Veterinary Service." The Meat Hygiene Service is liaising with MAFF's investigation branch with a view to possibly prosecuting the plant's owner for breaches of regulations.
Times of London 3/9/00 BY SIMON DE BRUXELLESA 15-YEAR-OLD girl from north Devon is believed to have become the youngest victim of the human form of "mad cow" disease. The girl, who has not been named [given elsewhere as Claire McVey -- webmaster], died at the end of February from new variant CJD, six months after falling ill. Health experts said yesterday that she may have contracted the incurable disease from eating a beefburger contaminated with infected meat more than ten years ago.
The teenager, who was a pupil at Ilfracombe Community College, is the 52nd victim of the disease linked to BSE, according to Dr James Ironside, of the CJD surveillance unit in Edinburgh. Although doctors are confident of the diagnosis, the cause of death will not be officially confirmed until tests have been carried out on brain tissue from the girl.
Dr Ironside said: "Since the first group of ten patients reported in 1996, there have been 52 patients who have died from new variant CJD. There is a smaller number of patients still alive who are suspected of having the disorder. The girl, who is one of the 52, is one of the youngest cases. The average age of people who die from variant CJD is 29."
Last September, a 17-year-old girl from Lanarkshire in Scotland was reported to have become the youngest known victim of the illness.
Ilfracombe Community College was one of the first Devon schools to introduce a ban on beef products in school meals after confirmation that BSE in cattle could be passed on to humans. The ban was lifted 18 months ago.
The school's headteacher, David Humphrey, said in a statement issued last night: "A pupil died tragically earlier in the year following a long and serious illness. The exact cause of death cannot be confirmed until a full inquest is held in the near future.
"Whatever the cause, the death of a young person with their whole life ahead of them is always hard and this will come as a great shock to the local community. Our sympathy and our thoughts go out to the family and friends who are trying hard to come to terms with their tragic loss.
"We are currently seeking advice from the Health Authorities, and if the cause of death is confirmed as CJD, we will be sharing information with parents and pupils at the school to allay fears, although my understanding is that other pupils are not at risk."
Devon County Council said the authority had been notified of the schoolgirl's death. A spokesman for the North Devon coroner said an inquest will be held at a later date.
Thu, Mar 9, 2000 By Damien Brook, PA NewsA patient was being tested for new variant Creutzfeldt-Jakob Disease in a Midlands hospital today. Leicester Royal Infirmary confirmed the investigations were taking place to establish whether the person had developed the human form of BSE but refused to give any details about the patient.
Dr Philip Monk, an expert in communicable diseases at the hospital, said: "It is a difficult disease to identify because it mimics a number of brain diseases. So, unfortunately it is not always possible to confirm the diagnosis." Dr Monk confirmed X-ray and pathology samples from the patient were being sent for examination.
There have been three reported deaths in Leicestershire attributed to new variant CJD and nationally 52 suspected cases were reported by January this year. In September 1998, Stacey Robinson, 18, from Thurmaston died at Coalville Community Hospital. The following month, 24-year-old Pamela Beyless from Glenfield died after an 18-month battle against the disease. A man, believed to be in his 30s, was also reported to have died of the disease in the same year.
A 15-year-old girl who has died after a long illness is thought to be the youngest victim of the disease, which is linked to consumption of BSE-infected beef. Claire McVey, a pupil at Ilfracombe Community College in Devon, died in January at Derriford Hospital in Plymouth. An inquest has been opened and adjourned pending scientific investigation of tissue samples.
The CJD surveillance unit in Edinburgh has been notified of her death and she has been included in the latest statistics recording deaths from the illness. If nvCJD is confirmed as the cause of her death, she may have contracted it in infancy, according to experts.
Wed, Mar 8, 2000 By Padraic Flanagan and Nick Allen, PA NewsA 15-year-old girl who has died after a long illness is thought to be the youngest victim of the human form of mad cow disease. Claire McVey died in January from suspected new variant Creutzfeldt-Jakob disease, which is linked to consumption of BSE-infected beef. The teenager was a pupil at Ilfracombe Community College in Devon. She died at Derriford Hospital in Plymouth where a post-mortem was carried out.
Later it emerged that Claire was determined to continue with her studies as she battled illness and had school work sent to her at her home. Family and friends rallied round Claire, a Spice Girls fan and keen dancer, and last September local firefighters raised money for her to go on a dream trip to Disneyworld in Florida. The firefighters, including her uncle Bob Wilson, walked 10 miles in full kit raising 2,600 pounds.
The teenager died at the end of January this year after months of illness and an inquest has been opened and adjourned. Today Claire's mother Sharon was too upset to talk about the tragedy at her rural cottage near Ilfracombe.
David Humphries, principal of the 1,200-pupil Ilfracombe Community College confirmed a pupil had died following a long illness but he said he did not know the nature of the illness. He said: "The pupil who died was a lovely, bright, vivacious kid, open, honest and forthright. "She would tell people what she thought but always with charm. She was a good dancer and liked the Spice Girl. She was very, very sociable.
"She had very, very loyal friends and pupils supporting her who kept in touch with her. It is desperate when someone dies but particularly when it is a young person who had so much of life before her. "There were ups and downs and I think at times she was better than at others."
"But for a long period of time she made clear her interest in school, her wish to remain at school and it was very important we sent work to her so she would continue to be part of things," said Mr Humphries.
An inquest into her death has been opened and adjourned pending scientific investigation of tissue samples. The CJD surveillance unit in Edinburgh has been notified of her death and she has been included in the latest statistics recording deaths from the illness. If nvCJD is confirmed as the cause of her death, she may have contracted it in infancy, according to experts.
The North Devon healthy authority, who confirmed the teenager's death, said there was no danger of the disease having spread to her family or classmates. Since nvCJD was identified in 1992 there have been 52 deaths from the disease in Britain. The average age of victims is 29.
Thursday 9 March 2000 By Sean O'Neill ISSUE 1749 TELEGRAPH UKA 15-YEAR-OLD girl who has died after a long illness is believed to be the youngest victim of the human form of mad cow disease. Claire McVey died in January from suspected variant Creutzfeldt-Jakob disease, which is linked to consumption of BSE-infected beef.... She died at Derriford Hospital in Plymouth, where a post mortem examination was carried out. A spokesman said: "We can confirm that we conducted an autopsy on a teenager which confirmed a diagnosis of variant CJD."
Dr Mark Kealy, consultant in communicable diseases at North Devon health authority, said there was no danger of the disease having spread to her family or classmates. He said: "One assumes that she must have had links with bovine offal and consumed it in the past before the controls were put in place and properly enforced.
"What we are seeing at present are people who were presumably infected in the mid to late Eighties. It is possible this girl may have contracted the illness when she was a toddler."
A man who lived for years with the fear that he might develop CJD died 10 months after noticing the symptoms, an inquest heard yesterday.
Andrew Charles, 32, was given growth hormones as a child and was told there was a high risk that it could lead to CJD. He began suffering dizzy spells and died in October at his mother's home in Stanwell, Middlesex. Verdict: misadventure.
Acta Med Croatica 1999;53(2):93-6 Liscic RM, Brinar V, Miklic P, Barsic B, Himbele JA 37-year-old patient with Creutzfeldt-Jakob disease (CJD) is presented, who had received a cadaveric dura matter graft 12 year before the onset of neurologic symptoms. Initial clinical presentation included cerebellar symptoms, with dementia and myoclonus developing in later stages of the disease. EEG showed diffuse slowing with sporadic triphasic periodic activity. CT was normal in the early stage but pronounced cerebral and cerebellar atrophy with widened sulci were seen on MRI in the late stage of the disease. The prion protein (PrP) gene was homozygous for valin at the polymorphic codon 129. Cerebrospinal fluid analysis for 14-3-3 protein was positive. We believe that this patient is the first Croatian to acquire CJD by dural implant.
Based on this case and a review of 66 cases from the literature, it is manifest that the awareness of iatrogenic transmission of CJD and adoption of preventive measures are the only effective way to stop the spread of CJD among surgically treated patients.
Opinion (webmaster): Pediatric CJD as of November, 1999 is reviewed elsewhere on site;there is a graph of kuru onset ages, the earliest being 4 years of age. and an account of a 13 year old reportedly with nvCJD.
"Since vCJD was identified in 1996 there have been 52 deaths from the disease in Britain..."
It has never really been clarified when the first cases of nvCJD were identified. There are immense diplomatic ramifications to non-notification, not to mention beef exports. In Mar 96, authorities announced the first 10 cases.
In retrospect, that represented a lot of cases that had built up and been studied for years. By the date of autopsy of the third under-30 case (or perhaps even by late symptoms), they knew there was a problem, say by 1994. Others would argue that the special surveillance unit, set up to look exactly for transmission from cow to human, was an ex post facto committee, that transmission was suspected very early on.
It is very difficult to track the number of cases known to be in progress. The disease can in fact be reliably diagnosed while the patient is still alive and increasingly this is done. Indeed, other kinds of CJD are hardly ever seen in persons under 30 worldwide and so could not distort statistics. However, because of beef exports, the government prefers to wait several months after death, which can be two years after accurate diagnosis. The effect is that official statistics very much lag the number of reliably known cases.
However appalling individual stories may be, the bigger issue is the ultimate scope of the epidemic. This is likely known by now from large-scale tonsil surveys. These have not been released.