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CJD tests after tragic coma teenager dies
Blood supply trade-off: less CJD, more AIDS and hepatitis
Mad Cow USA authors respond to London Times misquote
Parents win right to claim CJD damages
nvCJD: future trends not predictable yet
Fatal brain illness seen as long as 16 years after graft
Risks of vCJD blood transmission
Shakeup at EC after coverup scam
Does the CDC understand CJD?

CJD tests after tragic coma woman Vicky dies

 PA News  Fri, Nov 21, 1997        By Melanie Harvey and Brendan Berry
Another probable victim of CJD, the human form of mad cow disease, died in hospital today. Tragic Vicky Rimmer, 20, fell sick in 1993 and had lain in a deep coma for more than four years. Her grandmother Beryl Rimmer, of Connah's Quay, North Wales, was at her bedside at Deeside Community Hospital in North Wales when she died.

Vicky became ill when she was 16 and was the first teenager to be diagnosed as suffering from suspected CJD -- Creutzfeldt-Jakob's disease. Tonight Mrs Rimmer, who had visited Vicky every day, said she was "devastated". Speaking from her home in Heswall, Wirral, Mrs Rimmer told PA News: "The world is a sadder place today. "Before the illness Vicky was strong, full of energy and vibrant. She was always smiling."

Vicky had been moved to Deeside so she could be closer to her grandmother's home at the time where she had also lived. That is where she remained until her death. Mrs Rimmer said her fight to find out how her daughter became ill goes on. She hopes others will benefit from her family's tragedy.

"I was determined to find out what was wrong with her and how she got CJD, she had never been ill in her life, never even had a cold," she added. She said that Vicky, who worked weekends at a kennels before her illness, had been a keen meat eater.

"She loved beef burgers," Mrs Rimmer said. "All teenagers love them. It is frightening and it upsets me when I see experts now saying British beef is safe.

"You see other youngsters eating it and it worries me. I don't like to think of other people going through what we have been through. What I would like to see now is people admitting what has gone on and saying there was a problem with the meat in the 80s."

Since May Vicky had also been suffering static pneumonia. Her condition deteriorated earlier this week when paramedics twice had to be called in. Her body is to be moved for a post-mortem examination in Edinburgh where a specialist unit is investigating CJD cases.

Tests to confirm she had the newly-identified variant of the disease are not expected to be completed for at least six weeks. Latest Department of Health figures show 21 people have died from this strain of CJD since 1995.

Incidence of nvCJD in the UK

Commentary by JR Blanchfield, UK
20 November 1997
Eurosurveillance Weekly
Official data released in London on 3 November show that there have been 22 cases of definite and probable cases of nvCJD in the UK to the end of September 1997. Three, ten, and eight cases died in 1995, 1996, and 1997, respectively, and one case was still alive (1). One more case has been reported since the end of June 1997 (2).
References:

1. Department of Health press release, 3 November 1997.

2. Gill N. Incidence of new variant Creutzfeldt-Jakob disease in the UK.

Eurosurveillance Weekly
Reported by Caroline Akehurst  
PHLS Communicable Disease Surveillance Centre, England

"We have this at fifth-hand.
DoH --> PHLS --> Eurosurveillance Weekly --> FSNET --> BSE-L

At which stage the phrase " Three, ten, and eight cases died in 1995, 1996, and 1997" was inserted is not clear, but anyway its use implicitly suggests that it constitutes some kind of pattern from which an inference might be drawn.

It has repeatedly been stated and agreed here (a) that present data do not provide any means of foreseeing the future incidence (due to smallness of numbers, possible varying individual susceptibility, unknown dose levels, unknown incubation periods) and (b) of all the methods of arranging nvCJD events into any sort of pattern, year of death is of least value, both because a year is a crude time-interval and because the length of illness can vary considerably -- and we have just had sad but dramatic illustration of that, in the death of Vicky Rimmer on Thursday of this week after 4 years in a coma, compared with other victims dying only a few months after clinical diagnosis.

A few months ago Robert LaBudde made a more meaningful attempt to see a pattern so far, by relating it, not to the year of death, but to the best estimate of publicly available information as to date of clinical diagnosis (even that is imprecise and very "woolly" because in some cases the early clinical symptoms were first thought to be psychiatric, with varying periods of time before clinical diagnosis). CJDSU doubtless has better data from which to build a "date of clinical diagnosis" pattern so far, but has not issued such data or published a pattern based on it. In any event, that would not currently provide a basis for forecasting, but it would be of interest to see what pattern, so far, it would show."

Charles Arthur, The Independent" comments
"To add to that, some sources I have spoken to have been increasingly doubtful that Vicky Rimmer's case was nvCJD. The CJD Unit was doing a postmortem last Friday but results will take some weeks, and anyway they won't comment on individual cases.But the "probable/confirmed" case who was alive in the official DoH figures was not Rimmer, I heard. Let's wait and see."

Recall that Nature carried a commentary (385: 197-198, 16 Jan 1997) by Simon Cousens et al at the London School of Hygiene and Tropical Medicine which did have dates of onset for 14 new variant cases at that time. The figures then showed 7 onset in 1994, 6 in 1995, 1 in 1996. Of course, that will have moved on.

Parents win right to claim CJD damages

November 19 1997     Times   BY MICHAEL HORNSBY 
PARENTS of children who died from a lethal brain disease after being treated with growth hormone taken from human corpses won an appeal yesterday against being excluded from government compensation. The decision means that six families whose children developed Creutzfeldt-Jakob disease (CJD) after being given the hormone to combat dwarfism will now be eligible to seek damages.

Between 1959 and 1985 some 2,000 children were enabled to attain normal stature through implants of growth hormone taken from the pituitary glands of almost a million corpses. A small number of these children - 26 to date - developed CJD. It has been established that they were inadvertently given growth hormone from the bodies of people who had died of CJD and were infected by this route.

The Court of Appeal overturned a High Court ruling that parents of affected children could claim compensation only if their hormone treatment began after July 1, 1977. Lord Justice Kennedy, sitting with Lord Justice Judge and Lord Justice Chadwick, said the six families barred from compensation should be entitled to seek damages because their children's treatment had "straddled" the cut-off date.

Potential increased risk of virus transmission due to exclusion of older donors because of CJD

Transfusion 1997 Oct;37(10):996-1002 
Busch MP, Glynn SA, Schreiber GB
University of California, San Francisco, USA.
Concern over the theoretical possibility of disease transmission via blood from donors who develop Creutzfeldt-Jakob disease has led to proposals to exclude older individuals from donating plasma for further manufacture into pooled plasma donations. The impact of extending this age-deferral policy to blood donors was examined with respect to the risk for known transmissible viruses.

Demographic characteristics and confirmed prevalence rates first-time donations) and incidence rates person-years for repeat donors) for viral markers were compared for donors < 50 years old and > or = 50 years old and for donors < 60 years old and > or = 60 years old . Incidence rates were combined with infectious window-period estimates for each virus, to calculate the risk of virus transmission per 10(6) donations.

Unadjusted prevalence rates were significantly greater for younger than for older donor groups for human immunodeficiency virus (HIV), hepatitis B surface antigen (HBsAg), and hepatitis C virus (HCV). Incidence rates (and transmission risk estimates) for HBsAg were significantly higher in the < 50 donor group than in the > or = 50 group, and those for HIV, human T-lymphotropic virus, and HCV were not significantly higher.

Blanket removal of donors over the age of 50 would potentially lead to the following significant increases in the risk of infected units: HIV, 12 percent; HCV, 21 percent; and hepatitis B virus (HBsAg), 22 percent. CONCLUSION: Removal of donors over the age of 60 would not significantly affect the risk of infected units. Deferral of donors > or = 50 years of age from whole-blood donations for unfounded concerns about Creutzfeldt-Jakob disease could have adverse effects on both blood availability and safety.

Mad Cow USA authors respond to London Times

November 21, 1997 Correspondence
Dear Editor,

As authors of the new book, "Mad Cow U.S.A.: Could the Nightmare Happen Here?" we wish to correct erroneous information which was attributed to us in your November 14, 1997 story titled "U.S. Herds Carrying Mad Cow Disease."

Contrary to your reporting, our book does NOT claim that "thousands of American cattle carry a deadly strain of bovine spongiform encephalopathy."

There is indeed strong evidence suggesting that a form of transmissible spongiform encephalopathy (TSE) occurs in U.S. cattle, but nowhere do we attempt to offer a quantitative estimate of the number of affected animals. Our conclusions are identical to those of leading TSE researcher Clarence Gibbs of the U.S. National Institutes of Health, who argues that "All mammalian species thus far tested have the prion protein" which causes mad cow disease and the other TSEs. As a result, Gibbs explains,

"Every mammalian species in the world should have its own spongiform
encephalopathy, which means that the disease is endemic in all species. You
cannot escape it."
Gibbs expects that one would expect to find a TSE in U.S. cattle at a rate of one per million animals per year--considerably less than the "thousands" of cases claimed in your article. A disease this rare becomes a serious problem only through the practice of "animal cannibalism"--the feeding of rendered animal proteins back to their own species, which creates a closed amplification loop enabling the disease to multiply as happened in England.

Our motivation for writing "Mad Cow U.S.A." was to draw attention to the fact that this feeding practice, which has been banned in England since the late 1980s, continues on a massive scale in the United States. While this practice continues, the U.S. remains in danger of a health and economic nightmare like the one that England has endured.

The U.S. government did not take any action to restrict the practice of animal cannibalism even in cows until August 1997, and even then its actions remain too little, too late. Cows are still nourished here on fats and blood plasma derived from the rendered remains of their own species. The practice of feeding pigs to pigs and chickens to chickens also continues on a massive scale, and nothing but wishful thinking and industry PR can explain our government's continued insistence that pigs are somehow exempt from the possibility of contracting and spreading a fatal TSE.

The U.S. is also world headquarters to two forms of TSE which have almost never been seen in other countries. One of those is transmissible mink encephalopathy, which is probably caused by feeding downer dairy cows to mink, according to studies by Dr. Richard Marsh, a leading TSE researcher at the University of Wisconsin.

In Colorado and Wyoming, moreover, a TSE called chronic wasting disease occurs in free-ranging deer and elk, and no one yet knows how long it has been occurring or what caused it. A survey last year found the disease in more than 1 percent of all animals tested--a considerably higher rate of incidence than the "one in a million" scenario that one would naturally expect.

Given these facts, we believe that Europe is being very prudent in refusing to grant "BSE-free" certification to U.S.-sourced pharmaceuticals and cosmetics. England's experience with BSE has taught a painful lesson about the need for a precautionary principle when dealing with human and animal health issues. It is a lesson which, unfortunately, has still not been absorbed by the U.S. government and animal industry.

Sincerely,

Sheldon Rampton, John Stauber
3318 Gregory Street, Madison, WI 53711
Phone (608) 233-3346    Fax (608) 238-2236

Fatal brain illness seen as long as 16 years after graft

November 14, 1997 --By MIKE COOPER, Reuters 
ATLANTA - Patients who received contaminated grafts during surgery have developed fatal Creutzfeld-Jakob Disease as long as 16 years later, health officials said Thursday. The Centers for Disease Control and Prevention said 61 cases of Creutzfeld-Jakob Disease (CJD) have been linked worldwide to dura mater grafts manufactured by a German company since 1979.

One of 43 graft-related CJD cases in Japan involved a patient who developed the rapidly progressive fatal illness 16 years and one month after receiving a dura mater graft, the agency said. The average among the 43 patients was approximately seven years.

"The maximum latency period from the time of exposure to the time patients had onset of CJD was 16 years," CDC epidemiologist Dr. Ermias Belay said.
The German manufacturer, B. Braun Melsungen AG, began screening donors for the brain-destroying illness in 1987 and stopped mixing dura obtained from different cadavers in its product, called lyodura, Belay said. The product substitutes for the tough fibrous membrane covering the brain or spinal cord, but it has not been licensed for marketing or distribution in the United States.
"There is always this inherent risk that CJD could be transmitted through this kind of product, especially if the dura mater is obtained from a patient who has developed CJD or who subsequently develops CJD," Belay said.
Scientists have suggested an association between a variant form of CJD reported in the United Kingdom and bovine spongiform encephalopathy (BSE), the so-called "mad cow disease" that has infected about a million cows in Britain since 1985. Both illnesses belong to the same group of degenerative neurological diseases.

No cases of the variant CJD or BSE have been found in the United States. The CDC has said there is no direct evidence that BSE can spread to humans. Creutzfeldt-Jakob Disease afflicts only about one person per million and is most often found in patients 55 to 65 years of age. The CDC said 10 to 15 percent of CJD cases are inherited.

A University of Kentucky researcher suggested earlier this year that eating squirrel brains could cause CJD. Cases have also been linked to the use of contaminated corneal transplants, electrode implants and the receipt of human growth hormone, the CDC said.

British study will assess risks of vCJD blood transmission

Nando Times 15 Nov 97
The British government last week launched a study into the risk that the agent that causes nvCJ) might be transmitted by blood, and instructed the National Blood Authority to look at ways of reducing the potential risk.

The moves were announced by Frank Dobson, the health secretary, following advice from the government's Spongiform Encephalopathy Advisory Committee (SEAC). The committee says recent research suggests that the pathogenesis of vCJD differs from that of classical CJD in that the causative agent may occur in particular in lymphocytes, and that it would therefore be "logical" to consider removing such cells from blood products.

SEAC pointed out that while it is so far impossible to assess the risk of transmission of vCJD, risk assessments are urgently needed to obtain a clearer picture. Dobson said his department will carry out a full study immediately, and will consider introducing the leukodepletion of blood, depending on the outcome of the study.

Steve Dealler, a bovine spongiform encephalopathy (BSE) researcher at Burnley General Hospital, who has been campaigning vigorously about the potential risks of contaminated blood (see Transfusion Medicine, 6, 217; 1996), welcomed the government's action, but says that it should have been taken immediately after the risk of BSE passing to humans was acknowledged in March last year.

Dealler and others are sceptical about whether leukodepletion of blood will be sufficient to eliminate all risk, as plasma is likely to be contaminated by leakage from broken cells. The solution would be to screen donors for the disease, he points out, arguing that this makes development of a diagnostic test for nvCJD a priority.

Efforts to do so may be helped by the recent discovery of a monoclonal antibody specific for the abnormal version of the prion protein thought to cause the disease (see Nature 390, 74; 1997).

The government "is acting responsibly" in taking a precautionary stance, says Tony Wilson, chief executive officer of the UK Haemophilia Society. But he remains concerned that economic factors may be given excessive weight in the cost-benefit analysis of the safety of blood products. He points out that most UK haemophiliacs are still treated with clotting factors from natural blood, which are inherently more risky than the recombinant alternatives widely used elsewhere, because the natural products are cheaper in Britain.

Meanwhile, the French national bioethics committee last week concluded that while the risk of transmission of vCJD by blood remained "hypothetical", a body should be set up to monitor scientific knowledge of the risk. Dealler adds that the renewed attention on the risk of blood products suggests it is only a matter of time before European countries raise the question of a ban on exports of British blood products.

Shakeup at EC after coverup scam

13 November 1997 Nature
Paris. The European Parliament last week wrapped up its investigation of the bovine spongiform encephalopathy (BSE) crisis. The conclusion of the final report by the temporary committee on BSE is that the European Commission (EC) has made substantial progress in reforming its system of providing scientific advice. But the report "deplores" that although most of the EC officials responsible for handling BSE have been assigned to other duties, none has been otherwise disciplined.

The conclusion lifts the parliament's earlier threat to sack the EC's commissioners if its demands were not met. Some observers argue that the parliament has been highly successful in using the threat to obtain changes in the way the EC operates, in particular a radical reform of its scientific committees to distance them from economic interests and make their deliberations more open (see Nature 385, 664; 1997). Details of the committees and their proceedings are online.

EU official ordered to retire after claims of withholding data

 7 August 1997 Nature
Munich. The European Commission's deputy director-general for agriculture with responsibility for veterinary affairs, Fernando Mansito Caballero, has been ordered to take early retirement. The order comes five months after accusations that he deliberately withheld from the public information about the dangers of bovine spongiform encephalopathy (BSE) in 1990.

The move is widely believed to result from pressure from the European Parliament, which had accused the commission of mismanaging the BSE affair and demanded an investigation within the commission. Mansito allegedly said in 1990 that BSE should be discussed behind closed doors to avoid inciting public alarm.

But the commission is denying that Mansito's forced resignation is related to these accusations. "The retirement of SeŇor Mansito took place within an internal reorganization. It is not a sanction," says a commission spokesman. "He will receive financial compensation and will be entitled to his full pension." Indeed, one official's report of a meeting in June 1990 records that Mansito reprimanded the members of the commission's scientific veterinary committee for playing down health risks and for "only discussion."

The CDC refuses to make CJD a notifiable disease

Fri, 21 Nov 1997
Dear Ms. Armstrong:
Thank you for your letter of August 15, 1997 to David Satcher, M.D., Director, Centers for Disease Control and Prevention (CDC). He has forwarded it to me because of my role in conducting CJD surveillance in the United States.

First let me point out where I find myself in agreement with the points you have made in your letter about Creutzfeldt-Jakob disease (CJD) reporting practices. I agree that new variant CJD is an emerging disease and the CJD agent is notoriously resistant to many inactivating procedures that may be highly effective against the agents of other diseases.

Also, I agree that people who will develop CJD are donating blood on a regular basis and all cases of CJD are not ascertained in current nationwide statistics. Further, consistent with your observation, the incidence of CJD in all towns in the United States during specific 10 year periods will not always be the same as the overall national incidence. [clusters]

CDC conducts ongoing CJD surveillance through periodic review of the national multiple cause-of-death data compiled by the National Center for Health Statistics (NCHS). Mortality data analysis is an efficient way of conducting CJD surveillance because CJD is invariably fatal, over 85% of patients die within a year of onset, and its diagnosis is better ascertained at the time of death.[Very few dementia cases are ever investigated -- few pathologists are willing to do autopsies today-- webmaster]

Since new variant CJD was first reported, CDC has conducted active CJD sureveillance seeking evidence of the occurrence of the new variant CJD in its four established Emerging Infections Program sites and the Atlanta Metropolitan Active Surveillance Project areas.

In additions, to determine the presence or absence of new variant CJD in the United States, CDC, in collaboration with state health departments, is conducting a surveillance program aimed at reviewing clinical and neuropathologic records of decedents aged <55 years. CDC is also working with the American Association of Neuropathologists to search for possible cases of the new variant CJD in the United States, regardless of the patients age or clinical diagnosis.

My primary disagreement is with your belief that nationwide statistics on CJD are more inaccurate than statistics on "nationally notifiable diseases" and that this justifies reclassifying CJD as an internationally quarantineable and nationally notifiable disease. From my personal experience with notifiable diseases with current CJD reporting practices, I believe that the latter is better, although I recognize that there is plenty of room for improvement.

In my opinion, whereas increased epidemiologic and laboratory resources for conducting CJD surveillance would clearly be useful in improving the accuarcy of national statistics on CJD, making CJD nationally reportable would not. Routine mortality data combined with greater epidemiologic and laboratory resources to more actively investigate individual cases and focus on specific sub-populations would constitute a more effective way to reach our goal of improved monitoring of this disease.

Finally, let me convey my sincere condolences about your father's passing away in May 1996 from CJD and thank you again for your thoughtful letter.

Sincerely yours,
Ermias Belay, M.D.
Medical Epidemiologist
Division of Viral and Rickettsial Diseases

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