Blood donors feared to have spread CJD
Mother passes on CJD to unborn baby
Inquiry report due out in two weeks
Youngest case ever: 12
BSE in cow born after feed ban: detailed report
CJD and the mortuary profession
Australia set to ban blood donors with British links
Crohn's disease now blamed on milk
Japan admits to bad dura mater: lot numbers published
17 Sep 00 Sunday Times article by Jonathan Leake, Science EditorBLOOD donated by seven people who subsequently developed variant CJD, the human form of mad cow disease, may have infected transfusion products given to many more patients, it was revealed this weekend. The National Blood Authority has confirmed that some of the blood taken >from the seven was pooled - mixed with the blood or blood products of many other donors - before it was distributed to hospitals.
The number of people who may have been affected is unknown. The authority has objected to suggestions that thousands are at risk, but has no estimates of its own.
The revelations follow experiments which show that blood from sheep that are incubating BSE but have no symptoms can transmit the disease to other sheep. Humans are equally vulnerable: blood taken from anybody incubating nvCJD could theoretically spread the disease to people given blood products.
Such a case has not yet been seen with blood, although dozens of people contracted a slightly different form of CJD after being given a human growth hormone that had been extracted from corpses.
Blood from donors is usually split into its components, such as red cells, which carry oxygen; plasma, with white cells that help fight infection; and platelets, which aid clotting.
This weekend the authority confirmed that it is now considering a complete ban on the use of plasma from British donors. Most plasma is already imported, but about 100,000 units come from within Britain. The possibility that recipients of transfusions have been infected may also disqualify them from becoming subsequent blood donors.
Carlene Dyas, a spokeswoman for the authority, confirmed that the blood donated by the seven nvCJD victims was taken before 1999, when the authority introduced new safety procedures designed to remove prions, the infectious proteins thought to transmit nvCJD. She said: "It is very worrying, but there is nothing to show that what happens in sheep does apply to humans." [Sheep could also understate blood risks, as do rodents. -- webmaster]
Dyas said most of the blood recipients could not be traced because records were not sufficiently detailed. More than a dozen patients known to have received blood products from people who later developed nvCJD have been identified, but will not be told of the danger.
The problem is reminiscent of the spread of HIV in the 1980s when blood products transmitted the Aids virus to more than 1,200 haemophiliacs. The mistake cost the government Ŗ80m in compensation payments.
Professor Peter Smith, acting chairman of the government's spongiform encephalopathy advisory committee, confirmed there was a risk: "The concern with pooled blood products is that, as with growth hormone, an infection in one person could be spread."
Smith also acknowledged that the measures taken so far to prevent donated blood infecting others may be insufficient. Since last year, all British donated blood has been stripped of its white cells, which are thought most likely to carry the infectious prion particles. Recent research indicates, however, that prions may also be carried by red cells. [Leucodepletion is also incomplete. -- webmaster]
Comment (webmaster): The response to BSE in the sheep blood from the Dept of Health was 'no problem, we are already doing leucodepletion' neglecting to mention that this didn't kick in until 14 years into the epidemic, its extent is incomplete, that red blood cells themselves could carry infectivity, and indeed that its effectiveness is untested.
One of the early victims of nvCJD, was Maurice Callaghan, an engineer from West Belfast who died in 1995. After his death, it was confirmed that he had been a regular blood donor. Now we have seven of 82 known victims also likely to have given blood.
There has been no overall official disclosure of blood donation records, though we can be sure it is asked early on in every family interview. Taking both the new acceleration of the caseload and the preclinical infectious sheep blood into consideration, blood donations by the currently diagnosed victim set is likely only a very minor portion of total donated and injected infectivity.
It is like setting out a mousetrap, baiting it with coumarin, and putting out the cat as well -- nvCJD is coming at the UK in so many different ways.that nearly everyone has been exposed mutiple times by multiple routes. What benefit now are tracking programs for recipients of known tainted blood, expensive imports of plasma (whole blood is probaly not feasible or would have other risks), and disposal of pricey surgical tools?
It is time to consider a philosophy of triage: not to waste resources on ineffectual and costly containment half-measures, instead put everything into molecular diagnostics, therapy, and normal function. Or rather, get the US (with its much larger biomedical reseaerch capabilities) to do it, which won't happen until the first confirmed case emerges (which is only a matter of time).
Sun, 17 Sep 2000 Telegraph By Rajeev Syal, Jenny Booth and Chris HastingsScientists shocked as disease reveals new deadly traits Tragic inheritance of baby 'born with CJD'
DOCTORS believe that a baby girl has been born with new variant Creutzfeldt-Jakob disease, the human form of mad cow disease. Her mother died of the illness earlier this year.
Four specialists who have examined the 11-month-old girl believe that she is exhibiting the symptoms of nvCJD and that she contracted the condition in the womb. The Telegraph knows the identity of the child, but cannot name her for legal reasons.
The specialists have passed on their findings to the child's grandmother after tests failed to detect any other ailment in the girl. Only a post-mortem examination, however, can offer conclusive proof of nvCJD.
If confirmed, this would be the first known example of nvCJD being transmitted from mother to child, and will heighten fears that the disease can be transmitted through blood. One leading microbiologist believes that some of the 67 people who have already died of nvCJD may have inherited it from their mothers, rather than contracting it from eating infected meat. [A case with onset at 10.5 years has recently been acknowledged. -- webmaster]
The baby's 50-year-old grandmother, who is now her legal guardian, said the doctors suspected that prions - the infectious agents believed to cause the disease - had been passed on to the baby in the womb and had given her brain damage.
She said: "They don't know if it's gone into incubation. If so, it could be years before we can finally confirm the disease." The health of the child has been the subject of speculation since her mother died of nvCJD in May, seven months after giving birth. The girl was found to have brain damage and has been suffering from fits and convulsions.
Doctors have said that she is growing at half the normal rate for a child of her age and suffers from poor sight and abnormally stiff limbs. Her appendix has been examined by doctors looking for signs of nvCJD, but the tests proved inconclusive. She will undergo further brain scans later this year. [The tests have established that the baby is heterozygous for methionine and valine at codon 129 of the prion gene. That places things in uncharted waters, this confiuration would delay onset of nvCJD over met/met. The lymph nodes are negative. All previous cases have ben met/met and lymph positive. -- webmaster]
On Friday, The Lancet reported research by scientists at the Institute for Animal Health confirming for the first time that BSE can be transmitted in sheep by infected blood transfusions. The finding increases the likelihood that a nvCJD-infected mother could pass on the disease to her baby.
Richard Lacey, the emeritus professor of medical microbiology at Leeds University, said that it was "inevitable" that infected mothers would pass on nvCJD through the placenta. He said: "The only thing that is uncertain is the scale on which it is happening." New variant CJD, a fatal disease of the brain and nervous system, is believed to have been transmitted to humans through eating beef infected with bovine spongiform encephalopathy.
The Ministry of Agriculture admitted in 1996 that a pregnant cow could pass BSE to its unborn calf. Maternal transmission also occurs in sheep, rats and mice. Scientists have observed that offspring often develop the disease more virulently than the parent and after a much shorter incubation period.
Until now, researchers have been baffled at the youth of the 67 people known to have died of nvCJD. Their average age is 27. Most victims were aged 18 to 40. Dr Rob Will, of the National CJD Surveillance Unit in Edinburgh, has suggested that the victims caught the disease through eating cheap mechanically recovered meat used in school meals or even baby food. Dr Lacey however suspects that some may have contracted the illness from their mothers.
The Telegraph has also learnt that the Department of Health is now considering the use of disposable surgical instruments throughout the NHS because of growing concern that blood and tissue from nvCJD carriers could remain infectious even after sterilisation.
As this newspaper revealed four years ago, tissue from such patients is capable of passing ordinary CJD to healthy patients, yet current standards for sterilising equipment are not adequate to destroy the prions.
Sunday 17 September 2000 Telegraph By Rajeev Syal, Chris Hastings and Jenny BoothTHE dark-haired baby attracts admiring glances wherever she goes. She has her mother's striking blue eyes, says her adoring grandmother.
Medical experts believe, however, that she is the first child to inherit the disease which killed her mother, variant Creutzfeldt-Jakob disease, while still in the womb.
The child's 50-year-old grandmother, who has to feed the 11-month-old girl through a tube, said: "Every time I look at her, I see the agony that my daughter endured in her last days. Seeing my own child die in agony nearly killed me and now I am terrified that I will also see my grandchild die in the same way."
For months she suspected that her granddaughter was suffering from the symptoms of nvCJD. Over recent weeks, her worst fears have been confirmed by doctors from the leading London hospital that is treating her grandchild.
The tragedy began in July 1998, when the woman's 22-year-old daughter - who ran her own catering business - became moody, tired and constantly tearful. Her daughter's frequent outbursts of temper were untypical; the two normally lived harmoniously together in a semi-detatched home in a Warwickshire village. By February last year, the young woman had become pregnant.
She developed severe back trouble and, five months into her pregnancy, had to give up work. She could hardly move her arms and legs and had to be helped around the house. Other symptoms included pins and needles in her legs and swollen and sore lips.
Doctors were mystified as to the cause of the illness. In October, to try to protect mother and child, the baby girl was delivered by Caesarean section weighing 6lb 4oz. Immediately, however, the doctors were aware that the little girl had difficulties swallowing and she was placed in a special baby care unit. The family was told by doctors two days after the birth that the baby probably had brain damage. The specialists decided to conduct a series of tests on the child.
The family had begun to guess the truth. The dead woman's mother recalled: "I had spoken to someone who told me that their relative had died of CJD, and I had seen the news reports on television about cows. Then it dawned on me; my daughter's moods and the jerky movements she had begun to suffer were similar. It was an awful moment."
By January, nvCJD was confirmed in the mother by a biopsy on her tonsils - a procedure that is 98 per cent accurate.After this she was warned that her baby may also have contracted the disease.The young woman was virtually confined to a wheelchair and her memory was so bad that she sometimes failed to recognise her mother and her child. The baby's father had moved away from the area.
Doctors tried to discover if her baby also had nvCJD, but because the case was unique, and hampered by the poor health of the child, they were not sure how to reach a diagnosis.
In May, the child's mother died after months of suffering. In the same month, doctors removed the little girl's appendix and took samples of her lymph tissue in the hope that an analysis would show whether she was carrying the prions - aberrant proteins - that caused the disease that killed her mother.
According to the baby's grandmother, the tests carried out by the doctors were necessarily inconclusive because nvCJD infection can only be finally confirmed after death through a post-mortem. They nevertheless believe that her granddaughter has been suffering from the effects of vCJD from the time of her conception.
Caring for her dead daughter's child has now become the focus of the woman's life, and it is proving to be a daunting task. Her granddaughter's eyesight has been affected, and it is impossible to know how much she can see. Her limbs are stiff and she needs physiotherapy. She sleeps a lot of the time, as her mother did when she was ill. Last week she was suffering fits and convulsions, and doctors have said that she is growing at half the normal rate. She is to undergo further examinations by nvCJD specialists later this year.
Her grandmother said: "The appalling thing is that I am watching my granddaughter die while the Government fails to warn others that they may be passing the disease on. I want this baby's case highlighted because no other family should go through what we have been through."
Sunday 17 September 2000 By Robert MatthewsTHE growing concern over the health of the baby born to a mother with variant CJD and the new evidence, announced last week, that the disease may have spread through blood transfusions, highlight the disturbing ability of nvCJD to surprise the experts.
A conclusive diagnosis of nvCJD in the 11-month-old child would imply a far shorter incubation period than many scientists thought possible. It would also mean that the disease can be passed down the generations, not merely acquired through contact with infected tissue.
Scientists have yet to pin down the likely size of the nvCJD epidemic, with estimates for Britain ranging from about 100 cases to more than 100,000; so far there have been only 82 probable or confirmed cases. The case of the baby girl means that scientists are now admitting that many predictions may have to be rethought.
According to Dr Neil Ferguson of the University of Oxford, a leading expert on the mathematics of epidemics, so-called vertical transmission down the generations has, at least until now, been thought to be relatively unimportant.
Dr Ferguson said: "It very much depends on the probability of it taking place. If a woman has to be very sick and symptomatic in order to give it to her baby, then the number of cases it creates are going to be very small, because she is unlikely to get pregnant."
The mother of the baby at the centre of the current concern is, however, understood to have been showing only minor symptoms of the disease at the time of conception; only later in her pregnancy did she develop the classic symptoms of nvCJD from which she died in May this year.
Richard Lacey, an emeritus professor of medical microbiology at Leeds University, said that the Telegraph story had wider implications than its obvious medical significance. Prof Lacey said: "The only thing that is uncertain is the scale on which it is happening. That will take decades to find out. I am aware of other cases where maternal transmission could be an issue; this isn't the only one.
"This poses difficult ethical problems: to what extent should individuals be told of the risk? Should such a person be told not to give blood when he or she is old enough to do so? What effect will this knowledge have on that person's way of life, on their emotions? There needs to be some sort of discussion about how we cope with it, but at the moment there is nothing."
Epidemiologists are puzzled by the fact that nvCJD is predominantly affecting young people, while classic CJD is a disease of the old. Prof Lacey suggested that one possible explanation is that the teenagers and young people who have died from nvCJD may have contracted the disease from their mothers while in the womb. He added that it is a recognised clinical syndrome that infectivity accumulates when it is passed on from one generation to the next.
Dr Stephen Dealler, an expert on BSE and nvCJD at Burnley Hospital, said that it had been believed that mother-to-offspring infection could take place in cows, but only with a considerable incubation period. "The thinking is that it may take place in cows, but the incubation period is still three to five years," he said. "This would lead us to expect an incubation period of around seven years in humans. If the baby does have vCJD, that's a very fast incubation period."
Last week's revelation resulting from animal experiments that the disease may be transferred through blood transfusions from infected people with no symptoms was being played down by government scientists, who said that all British blood is screened to remove cells that may harbour nvCJD [beginning 14 years into the epidemic, that is. -- webmaster]
The Telegraph has learnt, however, that the Department of Health is seriously considering the use of disposable surgical instruments throughout the National Health Service. This follows growing concern that blood and tissue from asymptomatic carriers of the disease could remain infectious even after sterilisations.
Comment (webmaster): This unfortunate case, while moving towards final confirmation, raises questions about novel symptoms that nvCJD might display upon second passage by a different route (human-to-human, placental) and whether previous ascertainment criteria, based on dietary cow-to-human transmission, would have picked them up. By declaring nvCJD to be a disease of the twenty-somethings, both older (post-55) and younger cases don't get needed referral to expert groups.
It is not at all clear whether other known cases resulted from placental transmission. As no mothers have gone on to develop nvCJD, this would require 10-15 years of subclinical or preclinical disease in the mother. On the other hand, it does not seem obligatory that the mother develop clinical sign during pregnancy, as the placenta would likely be contaminated during earlier disease stages.
Telegraph Sunday 17 September 2000 By Joe Murphy, Political EditorTHE definitive report into the BSE crisis - due in two weeks, after almost three years of preparation - will accuse former ministers of playing down the risks that variants of the disease could be passed on to humans.
Among others it will criticise John Gummer, the Conservative agriculture minister who fed his daughter a beefburger in front of cameras in an attempt to reassure the public that beef products were safe.
The report is expected to conclude that Ministry of Agriculture officials were over-influenced by concerns that the farming industry would be plunged into deeper crisis if there was a beef boycott. Although there was no firm evidence at the time of a clear danger, it will say that ministers should have insisted on a more cautious approach.
Former ministers - including Mr Gummer, Kenneth Clarke, the then health secretary, Lord Freeman, a former health minister, and Douglas Hogg, a former agriculture minister - have been sent official letters notifying them that they were criticised in the draft report. Few amendments have been allowed by the chairman of the inquiry, Lord Philips, the incoming Master of the Rolls.
Labour commissioned the inquiry, which cost Ŗ25 million, and is planning to exploit its findings to disrupt the Conservative Party conference.
Opinion (webmaster): The Master of the Rolls has been Lord Woolf who was appointed in June 1996, in succession to Lord Bingham of Cornhill (now Lord Chief Justice of England and Wales). Master of the Rolls, one of many titles of Pooh-Bah, ridiculed by Gilbert and Sullivan in the Mikado was Sir William Brett, who held the position from 1883-1897.
Master of the Rolls: historically, the most important of the lord chancellor's assistants in the court of Chancery, so called because he was responsible for the records, orginally on rolls of parchment. In the 18th century he became a second judge in Chancery. Since 1881 he has been a judge of the Court of Appeal.
Judge Phillips has been given numerous promotions during the Inquiry The question here is, who exactly makes these promotion decisions, can Labor be hoping for stronger criticism as a reward:
In January 1999, it was announced that the judge who is overseeing the proceedings, Sir Nicholas Phillips, now 62, is to be a Law Lord. He has been appointed Lord of Appeal in Ordinary and is to be known as The Rt. Hon. The Lord Phillips of Worth Matravers. http://www.open.gov.uk/lcd/judicial/senjud.htm#part2
Phillips was then promoted during the inquiry to the Lords Justices; as the Inquiry neared the end, he was promoted again, in June this year, to Master of Rolls in the Court of Appeal and Head of Civil Justice, with Lord Justice Nourse filling in until Phillips assumes his new duties on October 1, with Sir Richard Scott, Vice-Chancellor of the Supreme Court, to be a Lord of Appeal in Ordinary from October 1, in succession to Phillips' old slot.
The Right Honourable Sir Nicholas Addison Phillips, Knight, a Lord Justice of Appeal, having been appointed a Lord of Appeal in Ordinary and created Baron Phillips of Worth Matravers, of Belsize Park in the London Borough of Camden, for life--was, in his robes, introduced between the Lord Alexander of Weedon and the Lord Mustill. [House of Lords Tuesday, 12th January 1999].
Phillips became a judge 28 February 1985 at the High Court: Queen's Bench Division and wasmoved to the more prestigious better Court of Appeal on 2 October 1995.
In short, heading up this inquiry really put Phillips' career on the fast track.
The BSE Inquiry Report will be handed to Ministers on Monday 2 October 2000 [Minister of Agriculture, Fisheries and Food, the Secretary of State for Health and the Secretaries of State for Scotland, Wales and Northern Ireland] . An extension until the end of September 2000 was agreed by the Government on 11 February 2000.
Counsel and the Inquiry team sent out letters to all witnesses facing potential criticism and evaluated the responses to these letters. What are these letters? They are called 'Salmon letters' and are sent by a public inquiry to a witness facing 'potential criticisms'.
Why are they called Salmon letters? A royal commission into public inquiries (1966) was chaired by Lord Justice Salmon, who recommended that anyone who might face criticism in the proceedings and report of a public inquiry should first be given details of potential criticisms and an opportunity to respond to them. [Eleven million pounds were spent on lawyers for the witnesses.] Will these letters be published? It is not normal practice to publish the Salmon letters.
There will be no direct legal consequences to anyone from the Inquiry. A few long-retired politicians will be mildly rebuked. In particular, the Civil Service, which orchestrated the whole BSE cover-up, will excape criticism.
Saturday 9 September 2000 Telegraph feature by Allison"...Dr Will never stopped eating beef. Along with many colleagues, he took the view that if brain and spinal-cord tissue was removed from the food chain as per the guidelines laid down by the Southwood Report in February 1989, then there would be little risk to anyone after that. 'Families were possibly exposed in '84 or '85, before BSE was isolated; well, maybe there was nothing to be done about that. But the minute it was isolated and safety measures introduced, public health should have been guaranteed.
Now we know that those measures were not fully implemented or enforced, which is a national tragedy, and I hope that tragedy will be addressed by the Phillips Inquiry [set up in 1997 to look into the previous government's response to BSE and nvCJD].'
Dr Will offers me a tour of the laboratories. As we are getting up to go, I broach something that has been bothering me. Does he think the victims will get any younger?
'Well, we now have a 12-year-old.'
'That's what I said.' He looks almost ashamed.
Girl or boy?
'I can't say.'
But if the incubation period is at least 10 years, then the child was barely eating solid food when it contracted the infection. 'I'm not saying anything,' the neurologist says wearily. 'You've got small children of your own, Allison. You do the maths.'
Along the corridor, I am introduced to James Ironside, the erect, bone-dry Scot who first identified variant CJD when he was looking down a microscope in 1996. The name is familiar, anyway. Dr Ironside is the pathologist who examined tonsil and brain samples from Claire McVey and whose written evidence, submitted to her inquest in Barnstaple, enabled the coroner to record a verdict of death by misadventure. (Earlier nvCJD victims were deemed to have died of natural causes, to the disgust of relatives.) 'Now, this is grey matter,' says Dr Ironside, inviting me to join him at a microscope. 'You see those flowery shapes? We call them florid plaques - that is an aggregate of prion protein which is a hallmark of variant CJD.'
The cross-section of brain looks like a lunar landscape, with the florid plaques forming the craters. Is that nvCJD itself? 'No, I think of those as the fingerprints it leaves. We don't know why it forms them.'
Suffused by a pink dye, the section of brain looks enchanting, as if the moon were made of marshmallow. 'That's memory and personality, basically,' says Dr Ironside. The prion protein got into that person's spleen, probably incubated there for 10 years, then entered the central nervous system and travelled to the brain where it started to throw its weight around like a chimp at an embroidery circle. The pretty eruptions I have been admiring are the nerve cell endings - they have exploded, taking memory and personality with them.
Does Dr Ironside ever think about the patients? 'Never.' Why not? 'I have teenage children of my own. They ate beefburgers. I can't think about it, it would be too' - he searches for the technical term - 'distressing.'
The Phillips Inquiry is due to hand its report to ministers at the end of this month, but as yet no date has been set for publication. (The families' case for compensation will start four months later.) The inquiry has been remarkably leak-proof, although some of the evidence Lord Phillips has heard was described to me as, 'like Yes, Minister without the laughs'.
The same Whitehall source says, 'If you were being darkly cynical, you could say the wait-and-see policy over BSE was a qualified success. The dead still under three figures - a bit of a narrow squeak, admittedly, but hardly the calamity it might have been.'
Comment (webmaster): Given the average length from onset to diagnosis, this child would have been about 10.5 years old at onset. The wait-and-see policy was a disaster for this child. The death toll will reach 3 figures by the end of the year and reach an estimated victim-a-day by mid-2002, with no end in sight.
This would however be a record early onset. Previous earliest age at onsets were 12.0, 14.2,14.2. Here neither the age of the child to the month nor the age at onset is disclosed. Durations have averaged 15.8 months, assuming current age of 12.5, onset here was at 10.7, well into new territory.
Here is another version of the narrow squeek quote:
"It has been suggested that Ministers took a chance on the number of people who would be infected and decided to wait and see, rather than spark a catastrophic impact on the British beef industry. Insiders now say that, behind closed doors, ministers consider the death toll of 75 people to be an acceptable risk."
Having the toll stand today at 88 won't change that assessment, especially since they have all been villagers. Nor will 188 or 1888. The greater good will be brought up at 18888, reminders of the necessary sacrifices in WWII at 188888, the need for foreign partners to conduct research at 1888888, and opportunities for a truly large wildlife park after that.
The story below has such a large number of mistakes and obsolete date for a 10 Sep 00 story that it is beyond correction, but it is the only other account available:
09/10/2000 CHARLES LAVERY Sunday MailA 12-YEAR-OLD has become the latest victim of the new CJD outbreak, we can reveal.
But Scottish experts working flat-out to come up with a cure for the disease are keeping details of the case a closely- guarded secret. The youngster is the 76th person to contract the new, more powerful strain of the human form of Mad Cow Disease in Britain. Experts have warned that reported cases are on the rise.
Professor Robert Will, head of the CJD Surveillance Unit at Edinburgh's Western Infirmary, last night refused to say whether the 12-year-old was male, female, Scottish or English. He would only say: "I am not prepared to make any comment on individual cases."
But he believes CJD infection is on the rise and youngsters are especially at risk. He said: "I think it's a very anxious period. "For the first time, we believe cases are occurring with a higher incidence. "The previous highest total of deaths per annum was 18 in 1998 but, in the first six months of this year, we have already had 14."
Scientists working on the disease have always believed the incubation rate to be at least 10 years. That means the 12-year-old must have eaten contaminated food almost as soon as he or she was able to eat solids.
The chances of that happening are extremely rare, which means that the incubation period could be shorter than believed, or that the 12- year-old was given soft food to eat as a baby which contained the killer strain of CJD, first discovered in 1996.
The new strain is rearing its ugly head amid claims that the Government didn't take the proper steps to make sure British beef was safe.The Phillips Inquiry, set up in 1997 to look into the then Government's handling of the crisis, is due to hand itsreport to Ministers this month [on Monday 2 Oct 00].
But no date has been set for the papers to be released to the general public.
It has been suggested that Ministers took a chance on the number of people who would be infected and decided to wait and see, rather than spark a catastrophic impact on the British beef industry. Insiders now say that, behind closed doors, ministers consider the death toll of 75 people to be an acceptable risk.
Professor Will added: "All we can say is that a risk was taken, but we still can't tell how many are incubating the disease."
By THOMAS H. MAUGH II, LA Times Medical Writer See also: NIH evasive fact sheet, a backgrounder web site, or Paratuberculosis Awareness & Research Association victims' site.If, as some scientists are now convinced, Crohn's disease is caused by a microorganism, the question becomes: How is it transmitted? The shocking answer, they say, is through that most sacrosanct of beverages--milk. The microorganism under suspicion, Mycobacterium avium subspecies paratuberculosis, or MAP, is common in U.S. dairy herds, activists argue, and it is not killed by conventional pasteurization.
Transmission of MAP from infected cattle to humans through milk could explain much about the occurrence of Crohn's, including its geographical distribution and rising incidence.
The purported spread of MAP through milk "constitutes a public health disaster of tragic proportions," said Dr. John Hermon-Taylor of St. George's Hospital Medical Center in London.
Both the U.S. dairy industry and the Food and Drug Administration argue vehemently that the U.S. milk supply is safe and that pasteurization is effective at removing any potential threats. [This sounds familiar -- webmaster]
But several large milk distributors in Britain have already changed their pasteurization procedures to make it more likely that the microorganism will be killed. The suspected links between MAP, milk and Crohn's have received a great deal of attention in that country, but none in the United States.
Some facts seem indisputable. MAP causes Johne's disease in cattle, a debilitating disorder whose symptoms are identical to those of Crohn's in humans. Large numbers of cattle in the United States are infected by the organism. According to a National Animal Health Monitoring System study conducted in 1996, 22% of U.S. dairy herds have infected cows. The cows secrete the mycobacterium in their milk.
And there the two sides part company. The dairy industry argues that the link between MAP and Crohn's is unproved and that, even if there were a link, pasteurization kills the microorganism. [This sounds familiar -- webmaster]
"It is the position of the Food and Drug Administration that the latest research shows conclusively that commerical pasteurization does indeed eliminate this hazard," Joseph Smucker, FDA's milk safety team leader, wrote to activists concerned about the risk. [How did so many people get ill? How is possible to still culture the bacterium from milk?-- webmaster]
But the activists have compiled a growing dossier of evidence. Dr. Walter Thayer of Rhode Island Hospital notes that Crohn's is not distributed evenly around the world, but is seen only in milk-drinking areas--Australia, southern Africa, Europe, the United States, Canada and New Zealand. It is rare in India, where they drink milk but boil it first.
Work by Hermon-Taylor and Dr. Irene Grant of Queen's University in Belfast, Ireland, has shown that DNA from MAP was present in about 20% of milk samples collected throughout the country. Living bacteria could be grown from many of the samples.
An as-yet-unpublished study by the Ministry of Agriculture in Britain found that researchers could grow MAP from at least 3% of samples of commercial pasteurized milk, Hermon-Taylor said. "It confirms, as sure as God made little green apples, that retail milk in Britain is a definite source of human exposure to these bugs," he added.
MAP is extremely difficult to kill, and commercial pasteurization--which involves heating milk to 161 degrees Fahrenheit for 15 seconds--is not sufficient, according to studies by Grant. Heating to higher temperatures--up to 194 degrees for the same period of time--is also not effective, she said, but increasing the pasteurization time to 25 seconds, even at 161 degrees, is.
Last year, several large milk distributors in Britain told their suppliers to increase pasteurization time, and that has been accomplished. Activists want to see the same steps taken here.
"There comes a point in time where consumer health takes precedence over commerical concerns," says Karen Meyer, president of the Paratuberculosis Awareness and Research Assn. "If a human pathogen is entering the food chain, that is a major concern. We need to ensure the protection of the public health."
Kyodo World Service Wed, Sep 20, 2000Opinion (webmaster): This is hot, they actually published the bad lot numbers on the Lyodura in the newspaper. NIH has fought off US residents for years. on the growth hormone lot numbers, to protect a doctor in North Carolina who insisted on injecting crude pituitary extracts. Some recipients do want to know whether they got an injection from a bad lot; some don't -- but all want to make their own decision.
There is a staggering amount of this Lyodura implanted in people: Japan alone imported "12,545 boxes of the dura mater, manufactured in 1982 by B. Braun Melsungen AG." It doesn't say how many uses per box or how many boxes comprise a serial number, or how many individuals were pooled per serial number.
If some serial number 2105 or so still is available, it could be tested to see what species it came from. One worry is that they used sheep-- their contract surgeon, who died of CJD a few years thereafter, removed dura mater sourced in sheep brain.
Kazuo Maruta, a senior official at the Health and Welfare Ministry, said the German-made dried dura mater used for brain surgery produced in 1982 was likely to have been imported before 1997.
The U.S. Food and Drug Administration (FDA) in 1987 issued a domestic advisory not to use such dura mater made in that year, after learning about a report that it could cause CJD. Symptoms of the disease include rapidly progressive dementia, loss of cerebral functions, and paralysis of limbs, with parts of the brain becoming sponge-like.
Maruta, chief of the ministry's Pharmaceutical and Medical Safety Bureau, told members of the Health and Welfare Committee at the House of Representatives that it is "likely" that the product, Lyodura with serial numbers 2000 to 2999, was imported into Japan. He cited a report by Tokyo importer Nihon B.S.S. which said 12,545 boxes of the dura mater, manufactured in 1982 by B. Braun Melsungen AG, were imported that year.
Health and Welfare Minister Yuji Tsushima also told the committee, "It was impossible (for the government) to foresee the dangers of the product." Previously, the ministry has denied the possibility of any imports, insisting that Lyodura with the serial number 2105, which the U.S. government said caused the first-discovered case of CJD, was not imported into Japan.
The German firm recalled the 2105 Lyodura in early 1987 and the FDA advised domestic medical facilities to abandon the product with serial numbers from 2000 to 2999.
It was only
10 years laterthat the Japanese ministry prohibited use of the product. More than 40 people, including CJD victims and their relatives, have filed two damages suits with the Tokyo District Court and the Otsu District Court, Shiga Prefecture, accusing the Japanese companies involved and the German firm of negligence, as well as the Japanese government.
Mon, Sep 18, 2000 By Andrea Hopkins Reuters World ReportOpinion (webmaster): It was only last week that the authorities were literally "urging" these people to keep donating. Now they are to be banned in February or if they can't get to it by then, March 2001. The question is, will donors still be urged, or merely encouraged, or grudgingly allowed, to donate in the intervening 6 months, now that they are acknowledged to be a risk?
Australia is expected to ban thousands of potential donors from giving blood if they have had extended visits to Britain, after research showed that mad cow disease can be transmitted through blood transfusions.
Federal and state medical officers were meeting on Monday to decide on the specifics of a ban, which would trim blood supply by at least five percent, a spokeswoman for federal Health Minister Michael Wooldridge told Reuters. But she said the ban would not be enforced until February or March to give blood banks time to recruit new members or otherwise ensure a consistent blood supply.
"It will take time, because it is five percent of blood supply, at least -- in a nation where we only have voluntary donation. So it is a very big chunk," spokeswoman Serena Williams said.
The ban, which would likely affect potential donors who have lived in Britain for more than six months, is similar to bans imposed recently in the United States, New Zealand and Canada. It follows the release on Friday of Scottish research which showed mad cow disease and its deadly human equivalent, known as new variant Creutzfeldt-Jakob disease (vCJD), can be transmitted through blood transfusions.
Dozens of people have died in Britain from nvCJD, with their deaths linked to the consumption of beef from cattle suffering from bovine spongiform encephalopathy (BSE), or mad cow disease. Australian health officials are debating whether the ban should apply only to those who ate British beef products during their visit, or to anyone who had spent at least six months in Britain between 1980 and 1996.
Williams said the ban could eventually be lifted if new research showed the disease could not be transmitted through blood transfusions after all.
"The (federal) view has been, since July when the issue was first raised, to defer people who had been in Britain during those times from donating blood," spokeswoman Williams said. "But we need to all agree on this issue. So we're really just waiting to see what the states are saying." Officials at the Australian Red Cross, which manages much of Australia's blood donation system, were not immediately available for comment. Mad cow disease, causes dementia, loss of muscle control and eventual coma. It can lie unnoticed for up to 20 years but usually kills within 12 to 18 months once symptoms begin. While there has never been a reported case of transmission through blood transfusions or the use of blood products, Scottish research published in The Lancet medical journal showed transmission is possible. Mad cow disease first broke out in British herds in 1986 and peaked in 1992. Four years later scientists identified vCJD and suggested it can be contracted by eating contaminated beef.
11 September 2000 Ministry of Agriculture, Fisheries and Food report [pdf]1. On 27 June 2000, BSE was confirmed in a Holstein/Friesian dairy cow born on 25 August 1996. Birth records and the animalšs passport confirmed its identity and date of birth. An official investigation to identify the possible source of infection was undertaken. The investigation has failed to identify any particular likely source of infection in this case. Case History
2. On 8 May 2000, a private veterinary surgeon reported a suspect case of BSE to the Ministry of Agriculture, Fisheries and Food (MAFF) Animal Health Divisional Office (AHDO) in Exeter. The animal was a Holstein/Friesian dairy cow born on 25 August 1996 and aged approximately 44 months.
3. A MAFF Veterinary Officer from the Exeter AHDO visited the farm the same day (8 May 2000), placed the animal under official movement restriction and euthanased the animal on-farm as a suspect case of BSE. The animalšs head was removed and submitted to the Veterinary Laboratories Agency (VLA) Regional Laboratory at Starcross for examination and the carcase was despatched, under official control, to an incinerator for destruction.
4. At the VLA Regional Laboratory, the brain was removed and after fixation, forwarded to the VLAšs Laboratory at Weybridge. Samples of brain tissue from the animal were subject to examination by histopathology, immunocytochemistry and western blot. All three tests were positive for BSE. Disease was officially confirmed on 27 June 2000. Case History
5. The animalšs owner believed, in retrospect, that the onset of clinical signs may have been 15 March 2000 when the animal was 42 months of age. The animal was observed kicking in the parlour for no apparent reason. One month later it was treated for lameness but subsequently lost weight and its condition deteriorated.
6. The animal had been born on a different farm to that where it resided at the time disease was confirmed. The BSE casešs natal herd was a dairy herd comprising around 80 milking cows and followers. Bull calves were reared to 7 days of age before being sold for fattening. Female calves were reared as herd replacements or for sale as breeding cattle.
7. She was sold from her natal herd at 19 months of age on 31 March 1998 when the farm and the natal herd were sold. She was then sold again to a dealer as an in-calf heifer on 17 June 1999 at 34 months of age. She subsequently calved and was then sold to her final owner on 6 July 1999 and remained on this farm until killed as a suspect BSE case on 8 May.The date of birth and all movements since birth have been confirmed by an examination of the animalšs passport and farm records. 8. Standard procedures exist for dealing with BSE cases born on or after 1 January 1996. These include identification and tracing of all animals which may have been exposed to the same feed. Animals traced which are under 30 months of age are placed under official movement restrictions to prevent their slaughter for human consumption. Animals over thirty months of age cannot be slaughtered for human consumption by law but are placed under official supervision. When these animals die on-farm or are killed under the OTMS, they are examined for evidence of BSE. They are also blood sampled annually and samples archived so that they may be examined should a suitable test be developed in future for the diagnosis of BSE.
9. Other animals in the same birth cohort as the BSE case were traced. They were born in a well demarcated calving season from August 1996 to February 1997 and all animals born in that season were considered. All male calves were sold within 7 days of birth before receiving solid food. The remaining 23 females (not including the index case) had been sold along with the herd, via a dealer, and were subsequently dispersed. Eight of the 23 female animals were confirmed as already dead and fifteen remain alive. None of these animals is under thirty months of age. All are currently under observation and will be blood sampled annually in accordance with standard procedures. The owners of these animals will be asked to inform the authorities when they are to be killed or if they die so that brain tissue can be sampled and examined for BSE.
10. The BSE case has had only one calf which was born in June 1999. This animal was identified and placed under official movement restrictions when its dam was reported as a suspect BSE case on 8 May 2000. Following confirmation of BSE in its dam, the calf was slaughtered on 6 July 2000 and its carcase incinerated under official supervision.
11. The dam of the BSE case died on 14 November 1996 less than three months after the birth of the BSE case. According to the farmer and his son, the cow died suddenly having become trapped in a feeding trough. She appeared to be healthy and not exhibiting any signs suggestive of BSE or any other disease prior to her death.
12. The BSE casešs natal herd comprised approximately 80 milking cows and followers when the farm and herd were sold in 1998. When the herd and farm were sold, some animals remained on-farm but others were dispersed.
13. Three cases of BSE had previously been confirmed on the farm, one in a home bred animal in 1993 and two cases in purchased animals in 1990. Two cases of BSE in cattle bearing the farmšs herd mark were confirmed in 1991 and 1994 on other farms. On the basis of their dates of birth, all five animals are considered to have been exposed to infection in 1986/87 before the BSE casešs dam was born.
14. Prior to the birth of the BSE case, its dam had given birth to two calves, one in 1994 and one in 1995. Both were bull calves and were sold at 7 days of age. They did not receive solid food before being sold on.
15. The carcase of the dam of the BSE case was sent to a knackers yard which has subsequently closed. Consequently it has not been possible to determine the exact fate of the dam. Her carcase would have been excluded from the human and animal feed chains.
16. On the natal farm, calf feed was purchased in bags and collected by the farmer from a local merchant as required. The feed was not delivered with other feed. The bags were held in a dedicated store on pallets over a concrete floor.
17. An examination of the records of the feed mill that manufactured the calf feed do not indicate that mammalian protein was included in the calf feed. The mill also produced pig rations (which were not used on this farm) until April 1997. It is unlikely that there would have been cross contamination of the calf feed with mammalian protein in the feed mill because the feed manufacturer ceased to use mammalian protein in its pig feed in April 1994 replacing this material with vegetable protein. Furthermore, no pet food was produced in the mill.
18. Feed for the adult cattle was delivered in bulk and stored in a hopper close to the dairy. This was on the other side of the yard from the calf pens and calf feed store. There was no deliberate feeding of adult feed to the calves and accidental access by calves to the adult cattle feed is considered to have been highly unlikely.
19. Four hundred and twenty five fattening pigs were kept on the natal farm in 17 pens. The pigs were fattened under contract to a company which supplied both the pigs and the feed. The pens were emptied and refilled continuously.
20. Feed for the pigs came from a different mill to that producing the calf feed. It was delivered in bulk and stored in a feed hopper. The hopper was filled weekly and because of the continuous throughput of pigs it was never cleaned out. There were around 21 weekly deliveries of fresh food from the date of the March 1996 feed ban and the birth of this case. The feed hopper was situated approximately 12 feet from the open fronted calf pens. No pig feed was fed to the calves either deliberately or accidentally since all spillages were fed to the pigs.
21. The pigs were fattened for a retailer which had specified that the feed used should not contain meat and bone meal.
22. There were no livestock species on the farm other than pigs and cattle. The farm had a severe rat infestation from a grain store but this is not considered relevant to the epidemiology of BSE.
23. No animal by-products, e.g. blood meal, abattoir waste etc were used on the farm either as feed or fertiliser. No embryo transfer had been carried out on the natal farm. The BSE case had not been subject to any synchronisation using pituitary extract collected from abattoirs nor had it been subject to any surgical interference.
24. There was no use of organophosphate products on the natal farm at this time, nor were vaccines used.
25. The significance of this BSE case lies with the fact that it was born after 1 August 1996 from which date the UK had effectively enforced a ban on the feeding of mammalian meat and bone meal to all farmed livestock. On the basis that the consumption of feed containing infective material was the main route by which the disease was transmitted, maternal transmission or an as yet unknown third route of transmission (for which there is, as yet, no evidence) must be considered as possibilities in investigations into the cause of infection in animals born after 1 August 1996.
26. Investigations carried out on the farm where this BSE case was born and in the feed mills supplying feed for the cattle and pigs reared on those premises, have failed to reveal any evidence that mammalian meat and bone meal (MBM) was incorporated in any of the rations supplied to the premises. MBM was not incorporated into any of the rations used for calf feed and as the company ceased to use mammalian protein in pig feed from April 1994, there is no evidence to indicate that cross contamination of calf feed with MBM destined for other rations could have occurred at the point of production. Neither was there any pet food produced in the plant which might have contained MBM.
27. Although the farm contained adult dairy cattle, there was no evidence of deliberate feeding of rations destined for dairy cattle to the calves and the layout of the premises is such that accidental access to adult feed rations by the calves is considered highly unlikely.
28. Although the calves were reared in open fronted pens some twelve feet from the feed hoppers in which pig feed was stored, the farmer was adamant that pig feed had not deliberately or accidentally been fed to the calves. Moreover, the pigs were reared under contract to a company which supplied both the pigs and the feed. This company were themselves under contract to a retailer who had specified that the feed should not contain MBM.
29. The feeding of farmed livestock with MBM was banned on 29 March 1996, (although the feed ban is not generally recognised to have been complete until August 1996 when a feed recall scheme was complete and subsequent legislation prohibiting the possession of MBM on premises where livestock were kept, and requiring disposal and recall of MBM and the cleansing and disinfection of premises, vehicles and equipment had been produced, stored or used came into effect). Even if there had been some carry over of infected MBM prior to the 29 March legislation, there would have been 21 weekly deliveries of fresh food from the time of that ban to the birth of this case. This would reduce the risk of carry over of contaminated feed in the storage hopper.
30. Although it cannot be positively proved that the calves were fed MBM free feed or that there was no cross contamination of the feed they received with MBM, the investigations both on the farm and in the feedmill suggest that feed is an unlikely source of infection for this case.
31. If feed was not the source of infection, then what were the possibilities of maternal transmission? If it occurs, it is thought likely that maternal transmission takes place in the two years prior to a dam succumbing to BSE. The risk is greatest in the period six months before to six months after an animal exhibits clinical signs of disease and even then the risk of its occurring is of the order of 10%.
32. In this case, there is no evidence that the dam was affected by BSE. The owner found her dead after she had become trapped in a feeding trough. There is no evidence to suggest that she was exhibiting clinical signs of any disorder, let alone BSE, at or around the time she died. Although five BSE cases were associated with the natal herd, all five animals are considered to have been exposed to infection in 1986/87, i.e. before the BSE casešs dam was born and ten years before the BSE case was born. There is, therefore, no firm evidence to indicate that this was a case of maternal transmission. It is not now possible to determine whether the BSE casešs dam was incubating disease and might have exhibited clinical signs of infection had it survived beyond November 1996.
33. The farmer indicated that there had been no use of animal by-products on the premises (whether in feed or as fertiliser) and that the animal in question had not been injected with hormonal extracts or vaccines. Neither had here been any use of organophosphate products on the farm at the time when the affected animal was present.
34. The feeding history on the farm and the manufacturing practices employed in the mills producing the feed suggests it is unlikely that infected feed was a source of infection in this case. Nor is there any evidence that cross contamination of calf feed either in the manufacturing process or by the accidental feeding of rations destined for another species, might have occurred.
35. The dam of the BSE case was found dead without exhibiting any signs of disease due to BSE or any other disease. Although maternal transmission cannot be ruled out, there is no clear evidence that the animal became infected by this route.
36. In summary, therefore, no firm conclusions can be drawn as to the source of infection. We will continue to monitor the animalšs birth cohort, but we may never learn what actually caused the disease in this case.
The Director March 2000 by Dr. Paul Brown & George LambCreutzfeldt-Jakob disease (CJD) occurs worldwide at a yearly rate of about one person per million population, and causes nearly 300 deaths each year in the United States. The disease typically affects older adults, who fall victim to a progressive dementia associated with incoordination, abnormal movements (especially myoclonus, or muscle twitching) and mutism, and is usually fatal within 3 to 6 months of onset.
Although most cases are sporadic, with no evident cause, the disease can be transmitted experimentally and, during the last 25 years, has been responsible for more than 250 human deaths as a result of accidental CJD contamination of cadaveric pituitary hormones, neurosurgical instruments, and dura mater and corneal grafts. This feature of transmissibillty sets it apart from all other degenerative brain diseases (such as Alzheimer's disease), and is the reason for concern about as yet unproven risks to people who may in some way find themselves in contact with potentially infectious patients, living or dead.
Three characteristics of CJD are relevant to the risk of infection. The first is that infectivity is almost always found in high concentration in the brain and spinal cord, but may also be present in lower concentrations in the cerebrospinal fluid, the eyes and many other organs and tissues of the body, including blood. CJD has never been detected in external secretions and excretions.
The second characteristic is that infectivity is highly resistant to customary methods of decontamination, and is unusually durable in the environment. Most of the usual physical and chemical disinfection techniques such as boiling, irradiation and a variety of chemicals including alcohols, phenolics and formaldehyde, are ineffective. Inactivation studies conducted over half a century have led to the realization that only the harshest methods can be relied upon consistently to inactivate the agent. These methods include physical exposure to dry heat (incineration), steam under pressure (autoclaving), or chemical exposure to either sodium hydroxide or bleach. Durability of the agent has been documented by the finding of residual infectivity in tissue that had been exposed to ambient weather conditions for three years.
The third important characteristic is that the infectious agent is not easily transmitted from person to person: it does not cross the barrier posed by intact skin or adjacent mucous membranes (although it can cross the intestinal lining). Even after a penetrating injury (for example, a cut from a contaminated scalpel blade), the likelihood of infection is orders of magnitude less than after direct inoculation into the brain.
In March 1999, a meeting of experts was convoked by the World Health Organization to address the issue of pre- and post-mortem care and the precautions to be exercised by attendant personnel. One section of the document provides instructions for morticians, with particular attention to embalming procedures on autopsied bodies, and a slightly modified text of this section is reproduced here in order to serve as a practical reference manual for morticians.
Mortuary procedures may be performed on the bodies of patients who have died from CJD with a minimum of inconvenience to ensure the safety of personnel and avoid contamination of the workplace. Transportation of the unembalmed body to the mortuary should be in an enclosable, impermeable plastic pouch. Ordinary contact or handling of an intact, unautopsied body does not pose a risk, and cosmetic work may be undertaken without any special precautions.
If the body has undergone autopsy, care should be taken to limit contamination of the workplace by any leaking bodily fluids (especially >from the cranium) when transferring the body from its transport bag to the mortuary table that has been covered with an impermeable sheet. No other precautions are required.
Embalming: An intact (unautopsied) body can be safely managed with only minor adjustments to the usual procedures. The body should be placed on an impermeable sheet or body pouch to avoid surface contamination from perfusion drain sites, drainage fluids collected into a stainless steel container, and perfusion sites closed with cyanoacrylates (super glue) and then wiped with bleach, which should be used undiluted from a fresh, unopened container.
Embalming an autopsied or traumatized body is not encouraged, but may be safely performed when the following precautions are observed. Disposable masks, gowns and gloves should be worn, just as pathologists do when performing an autopsy. The body is placed on an impermeable sheet or body pouch so that suture-site leakage can be contained, and perfusion drain sites should be similarly arranged to avoid surface contamination. All drainage fluids are collected into a stainless steel container. Perfusion and autopsy incision sites are closed with cyanoacrylates (super glue), and the entire body wiped down with bleach, with special care taken to ensure contact of bleach with perfusion sites and closed autopsy incisions.
At the conclusion of the perfusion procedure, the container of drainage fluids is decontaminated by adding sodium hydroxide pellets at the rate of 40 g per liter of fluid (approximately 1 lb. per gallon). The mixture should be stirred after a few minutes and care should be taken to avoid spillage, as the fluid will be hot. It should then be left undisturbed for at least one hour, after which it can be disposed of like any other mortuary waste. Plastic sheets and other disposable items that have come into contact with bodily fluids should be incinerated. Mortuary working surfaces that have become contaminated accidentally should be flooded with sodium hydroxide or bleach, left undisturbed for at least one hour, and then (using gloves) mopped up with absorbent disposable rags, and the surface swabbed with water sufficient to remove any residual disinfectant solution.
Ideally, non-disposable instruments and tools should be immersed in sodium hydroxide and autoclaved at 134°C for at least 15 minutes. If an autoclave is not available, the immersed instruments can be boiled for 15 minutes (allowing the hot, caustic solution to cool before removing the instruments). If for some reason boiling is impractical, effective decontamination can be obtained by immersing instruments in sodium hydroxide or bleach for several hours at room temperature. At the conclusion of the chosen decontamination procedure, the instruments are removed from the container and washed with water to remove residual disinfectant fluid before drying and reusing. The sodium hydroxide or bleach can be disposed of as uninfectious (but corrosive) waste fluid.
Table 1. Frequency with which infectivity has been detected in organs, secretions and excretions of patients with Creutzfeldt-Jakob disease.
Brain +++ Heart muscle 0 Spinal cord ++ Skeletal muscle 0 Cerebrospinal fluid ++ Adipose tissue (0) Pituitary gland ++ Testis (0) Eye +++ Prostate (0) Lung + Tears 0 Liver + Nasal mucous 0 Kidney + Saliva 0 Spleen + Sputum 0 Lymph nodes + Urine 0 Blood + Feces 0
Presence of infectivity: +++ usual, ++ frequent, + irregular, 0 absent. Parentheses indicate that very few specimens have been tested.
Table 2. Effectiveness of various chemical and physical decontamination methods for inactivation of the infectious agent of Creutzfeldt-Jakob disease.
Incineration (1000°C) Autoclaving (134°C) for >_ 15 minutes NaOH (lye)1: 15 minutes in a boiling solution, or >_ 1 hour at room temperature NaCIO (bleach)2: >_ 1 hour at room temperature NaCIO (bleacj)2: >_ 1 hour at room temperaturePARTIALLY EFFECTIVE
Autoclaving (121°C) for >_ 15 min Chlorine dioxide Glutaraldehyde Iodophores Urea
Boiling (100°C) Dry heat (< 300°C) Alcohol Ammonia B-propiolactone Formaldehyde Hydrochloric acid Hydrogen peroxide Phenolics Ethylene oxide vapor Ionizing, ultraviolet, or microwave radiation
Sodium hydroxide (NaOH, lye): 1 N NaOH is a solution of 40 g NaOH in 1 liter of water (approximately 1 lb. per gallon). 1 N NaOH readily reacts with CO2 in air to form carbonates that neutralize NaOH and diminish its disinfective propelties. Therefore, 1 N NaOH working solutions should be prepared fresh for each use from solid NaOH pellets. At room temperature, NaOH is caustic but relatively slow acting and can be removed from skin or clothing by thoroughly rinsing with water. Hot NaOH is aggressively caustic and should not be handled until cool.
Sodium hypochlorite (NaClO, bleach): Household or industrial-strength bleach is sold at different concentrations in different countries, so that a standard dilution cannot be specified. Efficacy depends upon the concentration of available chlorine (which should be at least 20,000 ppm). The usual formulation sold in the United States is a 5.25-percent solution, which can be used either undiluted, or up to a 1:5 dilution in water. Working solutions should be prepared fresh for each use. Because chlorine is continuously evolved by bleach solutions, they must be kept tightly sealed and away from light.
Funerals and Cremations: Relatives of the deceased may wish to view or have some final contact with the body. Superficial centach such as touching or kissing the face, need not be discouraged. Interment of bodies in closed coffins does not present any significant risk of environmental contamination, and cremated remains can be considered to be sterile, since the infectious agent does not survive incineration-range temperatures (1000°C). Transport and interment are subject to local and national guidelines, and transport overseas is governed by international regulations.
Exhumations are conducted according to state and national guidelines. The body should be considered as having the same infectivity as at the time of burial and the precautions used for management of the autopsy should be followed.
The two golden rules for anyone working with deceased patients or their tissues are:
1) avoid penetrating injuries by possibly contaminated tissues or fluids, and
2) limit environmental contamination by rigorous attention to the containment of potentially infectious materials.
Accidental splashes of contaminated blood or spinal fluid onto the skin, lips or eyes, although preferably avoided, can be washed away with water, and contaminated abrasions or cuts can be treated like venomous bites - encouraged to bleed, chemically disinfected and then washed with large amounts of water.
Morticians should never refuse their services to the families of deceased patients for fear of personal safety, as attention to recommended procedures and precautions will ensure a risk-free workplace. After nearly 30 years of searching, we can say with confidence that the frequency of CJD among morticians is no higher than in the general population: only one definite case has been identified among the thousands of patients studied during this period of time. Thus, the disease cannot be said to pose a serious hazard to the mortuary profession.*
Dr. Paul Brown was educated at Harvard College and the Johns Hopkins Medical Center, and is the author of more than 200 articles on various aspects of Creutzfeldt-Jakob disease. Brown is a career investigator at the National Institutes of Health in Bethesda, Maryland, where he presently holds the title of senior research scientist.
George Lamb graduated from Rutgers University, and is a licensed funeral director who owned and operated a large funeral corporation. His experience includes positions as Post Mortuary Officer and Graves Registration Officer for the U.S. Army in Vietnam. Lamb is currently the director of Mortician Services at Hahnemann University Hospital where he is responsible for decedent affairs and the autopsy suite.
1. Brown R Gibbs CJ Jr., Rodgers-Johnson P, et al. Human spongiform encephalopathy: the National Institutes of Health series of 300 cases of experimentally transmitted disease. Ann Neurol 1994; 35: 513-29. 2. Brown P, Preece MA, Will RG. "Friendly fire" in medicine: hormones, homografts, and Creutzfeldt-Jakob disease. Lancet 1992; 340: 24-27. 3. Nakamura Y, Aso E, Yanagawa H. Relative risk of Creutzfeldt-Jakob disease with cadaveric dura transplantation in Japan. Neurology 1999; 53: 218-220. 4. d'Aignaux JH, Costaglioa D, Maccarlo L et al. Incubation period of Creutzfeldt-Jakob disease in human growth hormone recipients in France. Neurology 1999; 53: 1197-1201. S.Taylor DM. Inactivation of transmissible degenerative encephalopothy agents: a review. Vet 1 2000; 159: 10-17. 6. Brown P, Gajdusek DC. Survival of scrapie virus after 3 years' interment. Lancet 1991; 16: 98-99. 7. Kimberlin RH, Walker CA. Pathogenesis of experimental scrapie. In: Bock T, Marsh J, eds. Novel infectious agents and the central nervous system, Ciba Foundation Symposium 135. chichester, UK; John Wiley & Sons, 1988: 37-54. 8. Report on the WHO Consultation on Patient Care and Hospital Infection Control. Geneva, March 24-26, 1999.
The Director July 2000 by James F. Burnside III, CFSP, Curtis Rostad, CFSP, & Kurt Soffe, CFSPBy now, hopefully every funeral director and embalmer is aware of the existence of a disease known as Creutzfeldt-Jakob Disease (CJD). Concern has risen sharply and is wide spread according to funeral directors that have experienced confirmed and unconfirmed cases of CJD. National statistics indicate that the average CJD occurrence is .95 to 1.5 cases per year per one million people.
If you talk with your colleagues in funeral service, however, you will find that those with direct CJD experience may question the accuracy of this statistic, based on personal experience. Conversations with funeral directors across the country indicate the increasing presence of this disease. Funeral homes now see CJD listed as a cause of death in more cases then are reflected in the current available public statistics.
One concern held by funeral service practitioners lies in the possible misdiagnosis of current Alzheimer's or dementia patients. For example, if a family relates during the arrangement conference that their loved one died from Alzheimer's or dementia in a short period of time, this should signal a red flag. Alzheimer's, dementia and CJD share some common symptoms. CJD is short in its duration, however, and victims generally last six months from onset to death, compared to those afflicted with Alzheimer's and dententia. This may be the only clue that funeral service personnel should take extreme precautions.
This article will provide funeral service practitioners with an overview of the information and knowledge available about CJD and offer suggestions on working with the families experiencing the loss of a loved one infected with this disease.
Unfortunately, much of the current information available about this disease appears conflicting and confusing. There are probably several reasons for this, including:
* Much is still not known about this disease.
* Disagreement within the medical community and medical research fields about the risk CJD poses.
* Much of the information about handling CJD is written for healthcare workers and does not address the unique issues in the embalming room.
What is Creutzfeldt-Jakob disease?
CJD is one of a family of diseases known as Transmissible Spongiform Encephalopathies (TSE). It was first identified and described by both Creutzfeldt and Jakob in the 1920s. It is a fatal neurological disease for which there is no treamtent, no cure and no vaccine. Other TSEs include Kuru (a disease associated with cannibalism in New Guinea) and Gerstmann-Straussler-Scheinker syndrome.
There are also similar diseases among animals. In sheep and goats, it is called scrapie, and in cows, Bovine Spongiform Encephalopathy (BSE), or "Mad Cow Disease." In 1995, an outbreak of this disease in Great Britain caused worldwide concern about whether this disease could be transferred >from animals to humans by eating contaminated meat or other animal products. As a result, shipment of cattle or meat products into the United States was halted. No cases of BSE have been found in the United States.
A new variant of CJD, called nvCJD, has been identified in Britain and has been proven to be associated with BSE. The new variant kills younger people (the average age is 28) and unlike classic CJD, the incubation period appears to be seven to 24 months rather than years. Again, none of these cases have occurred in the United States.
Who gets CJD?
Creutzfeldt-Jakob disease affects both men and women worldwide, usually between the ages of 50 and 75.
What causes it?
CJD results when abnormal protein accumulates in the brain cells. Scientists do not know what triggers the conversion of protein from normal to abnormal form. This abnormal protein, believed to be the causative agent of CJD, is known as a prion (pronounced "pry-on"), a protein-based molecule with no RNA or DNA that is smaller than a virus.
What are the symptoms of CJD?
The initial symptoms are subtle and can include insomnia, depression, confusion, personality/behavioral changes, strange physical sensations, coordination and balance disorders, loss of memory and visual problems. The patient rapidly deteriorates with progressive dementia and usually myoclonus (involuntary irregular jerking motions) as the disease progresses. Language, sight, muscular weakness and coordination problems worsen. The patient finally loses all mental and physical functions. Coma follows and death is usually due to pneumonia precipitated by a bedridden, unconscious state. The duration of CJD from the onset of symptoms to death is usually two to six months.
How common is CJD?
The official mortality rate of CJD is approximately one death per million population worldwide per year. CJD is still classified as a rare disease, although the National Center for Infectious Diseases considers it an "emerging infectious disease." This figure may be understated because CJD is often misdiagnosed because of the dementia it causes, which is often confused with Alzheimer's. One study conducted by Yale University showed that 13 percent of Alzheimer's patients were found upon autopsy to actually have CJD. Approximately 80 percent of these deaths were among people age 60 or older.
How is it diagnosed?
There is no definitive diagnostic test. The disease is suspected when a patient develops a rapid dementia and myoclonus. Although a spinal test is 95-percent effective in supporting a clinical diagnosis of CJD, the only 100-percent effective diagnostic tool available is an autopsy with appropriate tests. The post-mortem finding of a coarse, spongy-like surface of the brain (and the underlying microscopic cellular changes) is characteristic of CJD.
How long before symptoms develop?
A person can become infected with CJD but exhibit no symptoms for 12 to 25 years, although the disease can run its course and result in death in less than three years from the time of infection.
How do you get it?
Three epidemiologic forms of CJD are well recognized. The familiar (genetic) form (found in five to 10 percent of cases) results from a genetic mutation. Approximately one percent of cases are iatrogenic (resulting from a medical procedure), including neurological procedures using contaminated instruments, corneal transplant, from electroencephalograph electrodes, cadaveric dura-mater grafts, and pituitary hormone administration. Sporadic CJD accounts for 80 to 85 percent of all cases. These cases result from the mutation of the protein by some unknown cause.
Is CJD infectious?
Although CJD was first described in the 1920s, it was not considered a transmissible disease until 1966. Since this is a neurological disease, the cerebro-spinal fluid is highly infectious. The transmissible agent has also been shown to be present in the brain, spleen, liver, lymph nodes, lungs, spinal cord, kidneys, cornea and lens and bone, and to a much lesser degree, blood.
CJD is hard to kill since the organism is not destroyed by formaldehyde, phenol, glureraldehyde, alcohol, dry heat, boiling, hydrogen peroxide, ultraviolet radiation, lye or standard gravity sterilization. The only effective sterilization technique is steam pressure sterilization (a process not commonly available in funeral homes) and incineration, which destroys the body or instrument along with the organism.
The use of sodium hypochlorite (household bleach) is often mentioned as a potential disinfectant, but its results are inconsistent. It is highly corrosive to metal instruments, gives off irritating fumes and can not be used as an embalming agent. In addition, it has been shown that the disease organism has long-term survivability and can still remain viable and can be transmitted after an inactive period of a year or more.
Is CJD a danger to healthcare and mortuary staff?
Today, more than two dozen cases of CJD exist among health-care workers, including physicians, neurologists, pathologists and laboratory technicians exposed to CJD. Presently, there are no documented cases of transfer of the disease from a deceased patient to mortuary staff. The cause 80 to 85 percent of the cases of CJD is unknown, however, and because it can take up to 25 years for symptoms of CJD to develop, it could be decades before the true number of CJD infections in health-care workers is known.
Can you get CJD from blood?
This question is presently being debated and researched. Although there are no documented cases of CJD resulting from blood or blood transfusion, evidence of animal transmission suggests that the disease has the potential to be transmitted through blood. Human epidemiological evidence only indicates that if blood transmission occurs, it is likely rare.
CJD cases have been found among people that received blood transfusions, but the link between the disease and the transfusion has not been proven. Several cases of CJD following a blood component (albumin) transfusion have been reported, and two cases were confirmed to come >from a person that died of CJD. The members of the Special Emphasis Panel on Creutzfeldt-Jakob Disease at the National Heart, Lung and Blood Institute have agreed that, "an unqualified and irreducible risk of exposure to CJD through blood and blood products does exist..."
Is it airborne? Can you get CJD by breathing it?
The risk of infection From aerosols, droplets and exposure to intact skin, gastric and mucous membranes is unknown. Nevertheless, the National Institutes of Health strongly cautions laboratory workers to avoid the generation of aerosols and droplets during the manipulation of tissues or fluids known or suspected to be contaminated with CJD.
What are health-care workers telling funeral service practitioners about CJD?
While health-care providers routinely take extreme precautions to avoid exposure to CJD during invasive procedures or autopsy examination, there seems to be little knowledge within health care on the procedures followed during a routine embalming. Many instances have occurred when a health-care provider did not think it necessary to notify a funeral home about the presence of CJD. When these health-care providers were questioned, it was clear that they did not know how preparation rooms are designed and equipped, what disinfection procedures funeral homes practice, what personal protective equipment is available to funeral home personnel, and what level of knowledge funeral service practitioners possess about this disease.
Health-care facilities often take extreme precautions when performing procedures in the presence of CJD. These include, but are not limited to, use of special equipment (such as cocoons), minimizing the number of employees allowed in contact with the patient, and routinely notifying all departments in the healthcare facility to use special precautions when performing procedures on this patient.
A person that dies from CJD will not present any particular challenges to preservation. The selection of embalming fluid, fluid strength and fluid volume will be the same as a similar body deceased from any other cause.
Similarly, the appearance of the body will be no different than that of a body deceased from any other cause. No special restorative techniques will be necessary. CJD is resistant to formaldehyde, however, as well as every other embalming-fluid component or disinfecting chemical, which includes gluteraldehyde, phenol and alcohol. Nothing you can do with the body will render it disinfected and, therefore, you can not technically embalm a person who is deceased from CJD. You can produce tissue firmness and preservation, and you can produce a pleasant cosmetic effect, but you have not really embalmed the body.
Personal Protection Measures
While there are no industry-specific standards of protection for mortuary staff relative to CJD, funeral service practitioners can emulate procedures used in the medical field. Most pathology departments have additional guidelines for handling patients deceased from CJD. These typically include wearing two or three pairs of disposable gloves (rubber or latex, never vinyl), protective eye covering and face shield, mask, cap, jumpsuit, waterproof apron and shoe coverings. (Hospital guidelines go beyond what is normally considered "standard" universal precautions when dealing with CJD.)
Avoid causing aerosol distribution of contaminants, and avoid contact with all tissues and body fluids. All solid waste should be placed in a leak-proof container and disposed of by incineration. Instruments should be disinfected by autoclave, incinerated or containerized and disposed of as medical waste.
All surfaces should be wiped with sodium hypochlorite. Despite its shortcomings, the use of ordinary household bleach is recommended for general disinfective use in the embalming room and especially when dealing with CJD. Surfaces that may be contaminated should be wiped with a .5-percent solution (1:10 dilution) of bleach.
Since the organism has also been isolated in several internal organs, aspiration of the body should not be attempted. Minute pieces of the internal organs can be aspirated into the trocar, but this exposes the embalmer to the organism and the trocar will remain contaminated after the process is completed.
Finally, consult with local publlc-health authorities before attempting preparation to see if special handling of the case is required. Most states and counties have regulations concerning the handling of infectious disease cases and, whether or not it is specifically named in the regulations, CJD is an infectious disease. There may be restrictions on shipping, public viewing or other exposure to the public.
Concerns Following Autopsy
The CJD organism is concentrated in the brain and spinal column. While we might assume that exposure to the CJD organism is rather limited when handling a "normal" case, an embalmer is fully exposed to the organism when an autopsy has been performed, when death follows neurosurgery, or when death is due to head trauma.
Therefore, embalmers should request that the body be placed in a body pouch following autopsy to minimize contamination of the cot and removal vehicle, and to prevent exposure to the removal personnel.
If restoration of the remains is attempted, all instruments, the embalming table, the embalming room, and embalming personnel are exposed to the organism and there are no procedures available that will guarantee rendering the organism harmless. The organism can remain viable for more than a year.
Limit the number of people who are exposed to the body by limiting admission to the preparation room during the preparation process. Exposure to others after the body has been repaired should also be limited. This includes other embalmers, hairdressers, cosmetologists, etc.
Should Embalmers Attempt Preparation?
Whether or not an embalmer agrees to prepare the body is a personal decision that should be weighed carefully. With CJD, the question of preparation can only be answered after carefully considering the facts and weighing the consequences. Universal precautions coupled with cautious, deliberate work practices and a dose of plain common sense will do much to reduce the exposure risk, but the risk can not be eliminated and the full extent of that risk is unknown.
Service to Families
Funeral homes around the country have worked with families that experienced the death of someone diagnosed with CJD. They have arranged alternative services, including visitations with photographs and casket present, graveside services, and religious services in churches and synagogues.
For example, one funeral home offered to a family a visitation at the mortuary, a visitation at the church along with church services, and graveside services with burial, but without embalming and viewing because a cranial autopsy was performed. It was suggested that the family have a private viewing at the home following the death. Before the death, the funeral home offered to transport the remains to a university hospital for the cranial autopsy without additional charge. The decision not to embalm was very difficult, but this funeral home had the opportunity to present to the spouse, prior to the death, information regarding the potential hazard that embalming an autopsied CJD victim could present to a funeral director. Concerns ranged from exposing employees to agents they knowingly could not disinfect to placing a body in state for family and friends to approach and possibly contract the agent through direct contact. Because of the cranial autopsy, touching the area of the forehead or hairline might possibly transfer any residual prion agent to that individual.
This was a very dclicate time for the funeral home and for the family, for their desire was to have the body embalmed and dressed for burial without an open-casket viewing. Upon learning of the severity of this infectious prion based on their research, and after receiving additional information regarding the inability to disinfect the agent with embalming chemicals, the family elected not to possibly expose someone else to this disease.
The family noted, however, that other funeral homes they contacted in the area would proceed with the embalming without reservation. Since this death, information, articles and seminars have been disseminated to Utah funeral homes regarding CJD and the challenge it presents to funeral service.
Incidentally, the husband who lost his spouse to CJD in this case now champions the cause for education and further studies regarding this wasting disease. He currently participates in a network of Internet communication around the country regarding issues involving CJD, titled cjdvoice. His message has been presented in several media formats, including CNN, Canadian Broadcasting Radio, NBC, ABC and CBS affiliates in Salt Lake City, along with the New York Times, Ottawa Citizen, USA Today and Utah's statewide daily papers. As a tribute to his wife, he is committed to promoting education and continued research involving all forms of transmissible spongiform encephalopathies.
Funeral service practitioners will continue to face the challenge of communicable diseases into thc foreseeable future. As each challenge appears, funeral service practitioners must make every effort to identify them, work safely with them, and never forget the impact of the challenges these diseases present to the families they serve.*
Until the many questions about CJD are answered, the authors of this article strongly encourage a funeral home working with a suspected or confirmed case of CJD to seek the most timely, current and up-to-date information available. Funeral service practitioners must never take this disease casually or as part of a "routine" service. Information is changing rapidly, and new information is available all of the time and at all levels of health care. It is the responsibility of every funeral service practitioner to obtain the most current information available and then make a responsible decision that will protect their staff, the families they serve and the community in which they live.
THE DIRECTOR August 2000 T. Scott Gilligan partner: law firm Kepley, Gilligan & Eyrich, Cincinnati, OH.In last month's issue, James Burnside, Curtis Rostad and Kurt Soffe provided a detailed analysis of Creutzfeldt-Jakob disease (CJD) and the practical risks it presents for funeral home and embalming personnel.
This follow-up article examines the legal responsibilities of funeral homes to families of CJD victims and to the public. Specifically, the issues addressed include the funeral home's obligations to the family under the Americans with Disabilities Act (ADA) and its common-law duty not to expose members of the public to health risks.
ADA and CJD Cases
Under the Americans with Disabilities Act (ADA), a public accommodation, such as a funeral home, must provide all of its services to a disabled individual or a family associated with a disabled individual unless doing so would present a direct threat to the health and safety of others. According to the ADA regulations issued by the Department of Justice, a "direct threat" is a significant risk to the health and safety of others that cannot be eliminated by a reasonable modification of policies, practices or procedures. The determination of whether a threat exists must be based on scientific evidence of actual risk and not on speculation or stereotypes.
Funeral directors are aware that since the enactment of the ADA in 1992, they cannot refuse to embalm a body infected with the AIDS virus, hepatitis C virus or other communicable diseases, nor can they impose any surcharge for embalming. In all of these cases, the universal precautions required by applicable OSHA regulations protect embalming personnel from the transmission of the disease. Therefore, since the significant health risk posed by these communicable diseases can be effectively eliminated by a policy of employing universal precautions (which are already required by law), the funeral home must offer embalming services to those communicable disease cases.
It is clear that an invasive embalming procedure on a CJD case does present a direct threat to the health and safety of embalming personnel. The issue, however, is whether that direct threat can be eliminated by reasonable modifications of the embalming practices and procedures employed by the funeral home. Both last month's article in The Director and the article by Dr. Paul Brown and George Lamb on embalming CJD cases in the March 2000 edition explain the extraordinary measures embalmers must follow when preparing CJD cases. Are these precautions "reasonable modifications" that the ADA requires all funeral homes to take?
Neither the Department of Justice nor the courts have addressed this particular question. Nonetheless, it is an issue that every funeral home may have to answer when suddenly presented with a CJD case and a family who requests embalming and public visitation. At that point, the funeral home will need to determine whether it has the equipment and training to safely embalm a CJD case. Furthermore, if it decides that it does not, it may be called upon to defend that decision in an enforcement action brought under the ADA.
For those funeral homes that decide they are unable to safely embalm a CJD case, it is strongly recommended that they now seek out other funeral homes, mortuary colleges or trade embalmers that will embalm CJD cases. If they can provide embalming services for CJD cases through other sources, they will avoid disappointing the family and encountering a possible ADA claim. Of course, this alternate embalming service should be identified in advance so that funeral homes are not left scrambling at the last minute when confronted with a CJD case.
Protecting the Public and CJD Cases
If a funeral home has embalmed a CJD case, what precautions, if any, should be taken to protect members of the public or family members who may come in contact with the body? Does the funeral home have any potential liability to the public by permitting an open-casket visitation?
According to the Fact Sheet on CJD published by the National Institution of Neurological Disorders and Strokes (NINDS), a division of the National Institutes of Health and the U.S. Public Health Service, transmission of CJD is fairly rare. As noted in last month's article, it has occurred with autopsy personnel who handled CJD-infected brain tissue or spinal-cord fluid and were accidentally self-inoculated. CJD also has been transmitted to individuals receiving infected grafts of dura mater, transplanted corneas, or injections of contaminated pituitary growth hormone. Additionally, it is possible that some cases in Great Britain may have resulted from the consumption of infected beef.
According to the NINDS' Fact Sheet, however, there have been no reported cases of transmission through exposure to blood or blood products. In addition, spouses and other household members who care for CJD patients have no higher risk of contacting the disease than members of the general population.
Given the above findings, there does not appear to he any known risk in permitting superficial contact with the intact, embalmed body of a CJD victim. Therefore, liability concerns in these eases should not arise since the available scientific literature indicates that there is no known risk. Indeed, because there is no scientific basis to assume a risk, it may indeed be a violation of the ADA to deny a family contact with the body of a CJD victim that is intact and embalmed. Health concerns will arise in those cases where there are exposed incisions on the head of the remains due to cranial autopsies or other reasons. In those cases, funeral home personnel should advise the family that they cannot be assured that even superficial contact with the body is risk-free.