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CJD risk set to cost blood service 70 million pounds
Human form of mad cow disease traced to Spain?
New book "Human BSE: Anatomy of a health disaster"
Blood supply threatened by mad cow: Canadian agency
The CDC's CJD Program: $100 million spent on blood recalls
Gov't bailout on gamma globulin shortage?
6th Annual Report of CJD Surveillance Unit
Ulster woman feared to be CJD victim
Belgian woman says husband died from human BSE
I daren't have children, says CJD fear woman
Hospital staffs in 'breakdown' over CJD woman's eyes
British beef ban challenge fails in EU Court
Judge Tosses Christie Brinkley's Mad Cow Libel Suit
Oprah and the Beef: Washington Post editorial

CJD risk set to cost blood service 70 million pounds

S Dealler web site  16 May 98

"The Committee on the Safey of Medicines of the Department of Health has decided to ban the use of UK derived plasma for the manufacture of blood products. This should take place in 'a few months time' after the cleaning of the plasma fractionation equipment at BPL and the Scottish plasma fractionation centre. The DofH admitted that up to 3000 people in 100 hospitals could have been treated with products made from plasma donated by a person who died of nvCJD. (No data given concerning the amount of blood from an infected person already used for manufacture of plasma products and the number of people that would have already received this (around 2 million), No data given on when the plama would start to be imported from the USA (September), No data given on transmission risk levels - SD)"

Committee On Safety Of Medicines Completes Review Of Blood Products

UK Department Of Health Wednesday 13th May 1998

The Committee on Safety of Medicines (CSM) has completed its review of the sourcing of blood products, as announced on 26 February 1998.

A number of precautionary measures to protect patients against the theoretical risk of contracting new variant CJD were announced at that time, including permission if necessary for the importation of plasma for manufacture, and a CSM review of the use of UK-sourced plasma, a component of blood used in the manufacture of a variety of blood products.

The CSM has reviewed all products individually and has advised that manufactured blood products should not be sourced from UK plasma for the present time (advice attached). The Secretary of State for Health has accepted this advice.

The reasons for moving to non-UK sourced plasma for the time being are that, although there is currently no evidence that nvCJD can be transmitted by blood, there is nevertheless a theoretical risk. Currently there is no test that can be applied to donors to detect the presence of the prion associated with nvCJD.

It is possible that manufacturing processes used to produce blood products may destroy the agent thought to cause nvCJD infections. However, no test is available to confirm this.

As a precautionary measure, therefore, the Government is allowing the NHS's Bio Products Laboratory and the Scottish National Blood Transfusion Service's Protein Fractionation Centre to import plasma from outside the UK until such time as a test is developed to screen for the possibility of infection, or it is proven that nvCJD cannot be transmitted through blood products, or that it can be proven that the manufacturing process destroys any infective agent.

Only when quality inspectors are assured that the stringent safety standards applied to the new sources of plasma are equivalent to those currently available in the UK will plasma be imported.

Professor Michael Rawlins, Chairman of the CSM, said: "No new evidence has been reported indicating that nvCJD can be trannsmitted via blood products. However, while the risk remains only hypothetical, it cannot be fully discounted.

"The NHS Bio Products Laboratory and the SNBTS' Protein Fractionation Centre have been advised to take steps to source products from plasma derived from outside the UK, while giving due regard to the supply of vital products to patients. This will not happen overnight, as suitable collection centres abroad will have to be identified and inspected by the UK authorities. After this, the plants manufacturing the products will have to be cleaned before overseas plasma can be used for manufacture. The CSM recognises that all this will take some months.

"Some of the less common products such as antibodies used to treat and prevent rare diseases may take longer to replace, but these are often life-saving products and doctors should continue to use them in the short term.

"It is important to note that the use of blood for transfusion, platelets and fresh frozen plasma is not affected by this advice. These products are produced from single donations and patients would not be exposed to the same large number of donors as when the manufactured products are used."

"There is absolutely no question of any risk to blood donors of contracting nvCJD. With the NHS treating more patients than ever before, blood stocks need urgent replenishment. It is vital that blood donors support the NHS and continue their life-saving work."


1. The group considered that manufactured blood products should not be sourced from UK plasma. Although it was accepted that some parts of the manufacturing process for blood products may separate prion proteins, the present state of the art means that these processes cannot be validated. Therefore the theoretical risk that nvCJD could be transmitted by blood products cannot be discounted.

2. BPL and PFC should move to sourcing products from plasma derived from outside the UK in a time frame to be agreed with the CSM and giving regard to the effects on the supply of all products, but especially of vital but less readily obtainable products such as some rare and life saving specific immunoglobulins. The latter may have to stay on the market for a longer period of time if replacement products could not easily be found. BPL and PFC should be asked to give a date by which non UK products could be made available. In the future when a test is available to identify the agent of ncCKD in blood donors or when a validated inactivation process is developed it is hoped that there will be a return to the use of UK donor plasma.

3. In the interim period clinicians who do not wish to use products derived from UK plasma have a choice of licensed products from other source plasma to replace all the commonly used products.

4. A date after which no products from UK plasma could be released for use should be agreed. It is not intended that products should be recalled. The group recognised that a period of several months would be required to establish satisfactory sources of plasma, to clean equipment and to produce products from the new source plasma.

5. The group advised that all CTXs for blood products derived from UK donors should be suspended.

6. The group also asked that the BPL and PFC should be encouraged to undertake research into validating processes for the identification and removal of nvCJD agents in source plasma, so that the use of UK plasma may be re-established safely in the future.

7. The group suggested that there was a need for clinicians to be educated in minimising the use of blood products where alternative treatments were available, for example the use of steroids in children with ITP and the use of Hepatitis A vaccine for travellers. The CSM was asked to consider how to take this forward.

Human form of mad cow disease traced to Spain

Thu, 30 Apr 1998 UPI US & World By ED SUSMAN
MINNEAPOLIS -- The human form of mad cow disease -- a deadly brain disorder -- that affects families in North Africa, Italy, Greece and Chile, had its origin in Spain more than 500 years ago.

Researchers at the National Institute of Neurological Disorders and Stroke in Bethesda, Md., say Wednesday that when authorities in 1492 expelled Spain's Jewish population, it set in motion the spread of Creutzfeldt-Jakob ("CROYTZ-FELD YAH-CUB") Disease, or CJD -- the human form of mad cow Disease -- around the world.

At the annual meeting of the American Academy of Neurology in Minneapolis, Dr. Lev Goldfarb of NINDS says genetic clues links diverse populations to a common ancestor in Spain at least 14 generations ago. Goldfarb says a final piece of the puzzle may be a CJD case recently reported in Spain. But, he says attempts to get tissue from that patient to do genetic tests have not yet been successful.

He says one genetic thread of CJD -- a degenerative brain disease which usually has its onset when the patient is in his or her fifth decade and is fatal within six months of diagnosis -- is found among 15 families of Libyan Jews, 7 Tunisian Jewish families, one family of Greek Jews and 5 non-Jewish families in Italy and 6 non-Jewish families in Chile.

Jews who were expelled from Spain -- the same year Columbus discovered the New World -- settled in North Africa and Italy, and later travelled west to South America.

Goldfarb says, "It seems that one mutation was responsible for this family's CJD disease." He and his colleagues also have discovered five other mutations responsible for clusters of CJD among residents of 23 families in Slovakia, one family in North America, one family in Germany, one family in Sicily and one family in Japan.

He says, "These six mutations represent more than half of the worldwide cases of CJD." Goldfarb says that in some villages in Slovakia as many as one in 1, 000 people carry the flawed gene which causes CJD.

He says CJD can also be spread through contaminated blood products. About 60 such cases have been reported. [Note corrections below]

Clarification and corrections to this news story

Lev G. Goldfarb, M.D.
Clinical Neurogenetics Unit, Medical Neurology Branch
National Institute of Neurological Disorders and Stroke
Bldg 36, Room 4D03, 36 Convent Drive, MSC 4129
Bethesda, MD 20892-4129, Voice 301-402 1480; Fax 301-496 6341 

"As part of the program of the American Academy of Neurology meeting in Minneapolis last week, I was presenting a poster on genetic studies of hereditary forms of Creutzfeldt-Jakob disease (CJD). Specifically, we identified ancestral origins of the 200K mutation that is the cause of hereditary CJD in about 80% of families known to be affected with this disease. We analyzed 62 families representing 11 world populations and came to a conclusion that the Jewish populations from Libya, Tunisia and Greece, and non-Jewish populations of Italy and Chile share the same haplotype suggesting a common origin for the 200K mutation. In contrast, six other populations did not share these characteristics indicating an independent mutation."

"Ed Susman, a UPI reporter, has written an article about our work that is available from the Local Headlines section of Daily news yahoo-UPI, the Wednesday April 29 issue. It is titled "Human form of mad cow disease traced to Spain". To a reader, such a title implies some connection between the hereditary CJD and mad cow disease that does not exist. These are two completely different forms.

In another paragraph, the reporter quotes me as saying that the "six mutations" (actually, six mutational events) "represent" more than half of the worldwide cases of CJD. This is also absolutely wrong. Hereditary CJD cases comprise only 10% of all known CJD cases, and what I actually said was that the hereditary CJD cases associated with the 200K mutation comprise about 80% of all known HEREDITARY CJD cases. "

"Worse, the very last statement of the article "CJD can also be spread through contaminated blood products" and "about 60 such cases have been reported" is completely untrue. In fact, not a single proven case of blood transmission has been reported.

Answering someone's question, I mentioned 60 cases reported in recipients of growth hormone derived from human pituitaries. But not in recipients of blood products. This was the worse mistake anybody could have done. Millions of patients who received blood and blood products and the industry that is producing these products would be very much concerned if such a news reaches them. Let me repeat: so far, CJD transmission to humans through blood or blood products has not been demonstrated."

I daren't have children, says CJD fear woman

Wed, Apr 29, 1998 by Cathy Gordon, PA News
. A young woman living in fear of contracting CJD today broke down in tears as she told a court of her heartbreaking decision not to have a family. Flower shop manageress Claire Johnston said she would love to have children, but she was not prepared to take the risk that the fatal disease could be passed on to them.

Ms Johnston, 29, who lives near Cheadle, Staffs, is one of six people involved in a test case at the High Court in London to claim damages for the psychiatric illness they say they have developed as a result of being told they may be incubating the CJD (Creutzfeld Jakob Disease) agent. They were all treated as children with human growth hormone taken from corpses to combat dwarfism and now fear that they may develop the incurable and horrific disease.

Twenty-five people have died following treatment on the human growth hormone programme and two others are currently ill with CJD, the human equivalent of "mad cow" disease.

Referring to her decision not to have children because she felt it would be "unfair", a tearful Miss Johnston told the court: "Why should I put them through what I am going through now?" Ms Johnston, whose brother Philip was also treated with human growth hormone and is another of the six plaintiffs, said she felt "sick and scared" when the reality sank in that she risked contracting the disease. She said she thought about developing the disease "literally every day" and added: "It's very hard for me. I know it's very hard for other people as well."

Ms Johnston said she used to be a happy-go-lucky person: "I'm totally different now. "Now I worry about what people think about me. Are they staring? Do they know who I am? It's like paranoia. What are they thinking about me?"

She and Philip, from Stoke-on-Trent, Staffs, and the four other plaintiffs -- Paul Andrews, from Putney, London; Neil Scanlon from Ebbw Vale, south Wales; David Lockhart from Newmarket, Suffolk and Justin Parkes >from Essex -- are representative cases for a group of 35 people treated with growth hormone from corpses after July 1, 1977, and who now fear they will contract CJD.

Their counsel Stephen Irwin QC has told the judge, Mr Justice Morland, that in the cases of Philip Johnston, 25, Mr Andrews, 32, Mr Scanlon, 36, and Mr Lockhart, 27, it was accepted by the defendant, the Secretary of State for Health, that the psychiatric illness had been caused by learning of the risk of CJD.

In those cases the dispute between the parties was about the nature and severity of the illness and the extent of damages which may be recoverable. In the cases of Ms Johnston Mr Parkes the defendant considered that no psychiatric illness has been sustained.

Between 1959 and 1985 nearly two thousand children in the UK whose growth was stunted because of a deficiency in the secretion of growth hormone in their pituitaries were treated with hormone from the pituitary glands of cadavers. The programme was ended in May 1985 after several children who had been treated in the US died of CJD.

In July 1996, Mr Justice Morland ruled that the Department of Health was negligent in not heeding the warning of Dr Alan Dickinson, who in 1977 told the Medical Research Council about the risk of contracting CJD from the hormone treatment. Last year people who were given the hormone treatment and claim they have suffered a psychiatric illness brought on by fear of contracting the disease were given the go-ahead by the same judge to seek compensation.

Blood supply threatened by mad cow: Canadian agency

Wednesday, April 29, 1998  Toronto Sun
TORONTO - The human form of mad cow disease may be a threat to Canada's blood supply, a spokesman for Hemophilia Ontario warned this week. Tom Smith said there is a possibility the rare but fatal brain disease is currently in blood products and the blood supply. Smith is urging a temporary ban on blood donations from people who have been in Britain during the past 30 years. He also wants screening done for Creutzfeld-Jacob Disease.

But Dr. Graham Sher of the Canadian Red Cross says such a donation ban would likely affect up to 40 per cent of all blood donors and would destroy the Canadian blood supply. However, there's no test available or any conclusive proof the disease is carried in the blood.

Last fall, the Red Cross alerted hospitals that about 200,000 vials of blood products, given to about 50,000 people, could pose a risk of triggering the degenerative brain disease. The blood products came from a single donor who carried a gene linked to the hereditary form of the disease.

At the time, the Red Cross said there was no known case of anyone getting the disease from a blood donation. But the presence of the gene in the donor could pose a risk that required the agency to notify hospitals.

EU steps up controls on blood donations, diseases

Reuters World Report Thu, Apr 30, 1998
BRUSSELS - European Union health ministers agreed on Thursday to step up controls on donated blood and plasma throughout the 15-nation bloc. The move comes in the wake of a series of tainted blood scares and a warning from European scientists that it might be possible to transmit Creutzfeldt-Jakob disease (CJD), the fatal human equivalent of mad cow disease, through blood transfusions.

The ministers also agreed to spend over 10 million Ecus ($11 million) between 1999 and 2003 on combating rare and pollution-related diseases. They are expected later on this year to agree to fund a series of measures to prevent domestic and workplace injuries. These three programmes are part of the EU's eight-pronged public health strategy, launched in 1993. The strategy -- which ministers agreed needed updating to cope with the threat of new or resurgent diseases -- also covers AIDS, cancer, drug dependence, health promotion and health monitoring.

Some of the health problems now facing Europe do not fit into these categories, leaving the bloc without the tools to tackle them effectively, European Social Affairs Commissioner Padraig Flynn told ministers. These include new diseases like CJD, for which there is no reliable diagnostic test, resurgent contagious diseases like tuberculosis and ageing diseases like Alzheimers among Europe's increasingly large elderly population.

As far back as 1995, EU health ministers stressed the need for a worldwide early warning system for contagious diseases. Discussions are continuing with the United States on creating such a system but no concrete proposals are expected soon, a prospect which Flynn finds alarming.

"If tomorrow there were to be an outbreak of a new and dangerous epidemic, we are poorly equipped to respond," Flynn warned in a discussion paper released on April 15. The paper suggested the EU should overhaul its health policy, focusing particularly on speeding up the bloc's response to potential epidemics, putting greater emphasis on disease prevention and improving information on health problems.

This reform will take time. In the meantime, ministers said they would plough ahead with disease prevention measures. These could include making health warnings on cigarette packets more visible and hard-hitting, and warning consumers of the dangers of "low tar" cigarettes. British doctors said earlier this month low-tar cigarettes were just as dangerous as standard cigarettes and might be responsible for an increase in a rare form of lung cancer.

6th Annual Report of the UK CJD SURVEILLANCE UNIT

Wednesday 22nd April 1998 Department of Health press release
Fulltext and tables available, graphics will be fixed on about May 10
The Department of Health today published the sixth annual report of the National Creutzfeldt-Jakob Disease Surveillance Unit which is based in Edinburgh.

The report covers the period from May 1990, when the Unit was set up, to the end of April 1997 in relation to sporadic, familial and iatrogenic CJD. It covers up to 1 January 1998 in relation to new variant CJD (nvCJD). The report also provides analyses of data collected by the Unit on the dietary and occupational histories of patients.

The main findings of the report are:

* the overall incidence of CJD in the UK remains comparable to other countries in Europe and elsewhere in the world, including countries which are free of BSE;

* there has been an increase in the number of cases of classic sporadic CJD recorded in England and Wales for the period 1970 - April 1997. Similar increases have also been reported in other countries which monitor the disease including those where there is little or no BSE. Most of the increase is amongst people over 70 years old and probably reflects better ascertainment rather than a real rise in incidence;

* analyses, which take account of delays in reporting and confirmation, have found no evidence that the incidence rate of nvCJD has increased over the period 1994 -1997;

* a study of the occupations of people with CJD shows no conclusive evidence of a link between occupation and CJD, including nvCJD;

* ongoing analysis of the data shows no convincing evidence of clustering of cases;

* analysis of the dietary histories of people with classic sporadic CJD has revealed apparent associations between various meats and animal products and risk of classic sporadic CJD. The report's authors stress that these apparent associations should be treated with great caution because of the difficulties in obtaining accurate dietary histories.

Belgian woman says husband died from human BSE

 Reuters World Report Thu, Apr 30, 1998
BRUSSELS - A Belgian woman whose husband died in 1994 said on Thursday she suspects he died from the human equivalent of mad cow disease. But Katleen Derycke told Belgian radio the truth about whether Belgium has had its first case of the "new variant" of the deadly brain-wasting disease in her husband Marc might never be known.

Repeated requests over two years for information were stonewalled by the Western Flanders hospital which treated him. And when the British centre monitoring the new strain of Creutzfeld-Jacob Disease asked earlier this year to see samples of his brain tissue none could be found, and even the autopsy report had disappeared.

"I'm angry. I will never know," Derycke told French-language RTBF radio. "It seems strange. I have the impression that they are trying to hide everything." Her husband Marc died of Creutzfeld-Jacob Disease in 1994 but she noticed similarities with descriptions of the mad cow disease-linked "new variant" first identified in Britain in 1996.

She told the radio she had recorded details of her husband's illness daily in a diary and earlier this year, having made no progress with the hospital in the Flemish-speaking town of Ghent, sent a translation to the British monitoring centre. "They telephoned and said that the symptoms were exactly those of new variant, but they had absolutely to see the tissues of my husband," she said. "They did not get them."

A spokeswoman for Belgium's public health ministry said it was seeking more information into the allegations. Belgium has reported four cases of mad cow disease, or bovine spongiform encephalopathy (BSE), since the first case was announced in October last year. Two of them were in the part of the country where Derycke lived.

Mad cow disease has led to the slaughter of thousands of cattle in Britain and in other countries. The crisis prompted the European Union to impose a beef export ban on Britain in March 1996 after scientists identified nvCJD which they said could be contracted by eating beef infected with BSE. So far 24 people have died of nvCJD and this would be the first reported case in Belgium.

Ulster woman feared to be CJD victim

Thu, Apr 30, 1998 By Ian Graham, PA News
A Northern Ireland woman is feared to have become the latest victim of CJD - the human form of mad cow disease. The County Down mother, thought to be in her early 40s, has been treated in Belfast's Royal Victoria Hospital and then transferred elsewhere for palliative care.

A spokeswoman for the RVH confirmed they had treated a woman for suspected CJD - the disease can only be confirmed by post mortem. Family sources said they were "devastated" and struggling to come to terms with the illness. Her husband has been keeping a daily bedside vigil. CJD is extremely rare, estimated to affect just one person in a million each year.

The woman is the latest in only a handful of cases of the disease recorded in Northern Ireland. West Belfast man Maurice Callaghan, who died in 1995 at the age of 30, was one of the first confirmed victims in the UK.

British beef ban challenge fails

PA News Tue, May 5, 1998  By Geoff Meade, European Editor 
A Government attempt to have the worldwide ban on British beef exports declared illegal were dashed today by European court judges in Luxembourg. They said the Brussels Commission did not exceed its powers when it slapped a ban on exports of British beef, not just to the rest of the European Union but throughout the world. The ban was imposed in March, 1996, in response to growing consumer fears over the spread of BSE.

The Government immediately complained that Eurocrats and other EU governments were acting politically and economically, to fend off a public backlash which could hit beef sales in the other members states. London also argued that the Commission had no right to interfere in Britain's trade with countries beyond the EU.

But the judges today backed the Commission's claim that it was only taking steps sufficient to contain the spread of mad cow disease - by preventing British beef which might be exported to non-EU countries re-entering the EU. The judgment comes more than two years after the ban was imposed and only shortly after an agreement between EU governments to start easing the trade blockade.

Today's ruling said the Commission can use its powers to intervene rapidly to prevent a disease which affects animals or threatens human health.

The ban was triggered by fresh information provided on March 20, 1996 by an independent scientific body set up to advise the British Government, the Spongiform Encephalopathy Advisory Committee (SEAC). The Committee confirmed that "the most likely explanation" for the appearance of a new variant of Creutzfeldt-Jakob Disease - an ecephalopathy affecting humans - was exposure to BSE.

The judges said: "The new information provided by SEAC significantly altered the perception of the risk which BSE represented for human health, and thus authorised the Commission to adopt safeguard measures." The European Court explained that the Commission was empowered to immobilise and contain animals and animal products within a specified territory as "an appropriate measure" in the event of disease threatening a serious hazard to animals or humans.

"In order for such containment to be effective, it is necessary to impose a total ban on the movement of animals and products outside the frontiers of the member state concerned, thereby affecting exports to third countries, furthermore those directives do not expressly preclude the Commission from banning exports to third countries."
The judges said that bearing in mind the probable link between BSE and a fatal disease affecting humans for which no known cure yet exists, and also uncertainty about the adequacy and effectiveness of anti-BSE measures adopted by the UK and the rest of the EU, the court considers that the Commission did not clearly exceed the bounds of its discretion in seeking to contain the disease within the territory of the United Kingdom by banning the export from that territory to other members states and to third countries of bovine animals and derived products.

Today's case was lodged by the Conservative Government and adopted by Labour when it took office a year ago. The complaint was backed by the National Farmers' Union which argued in court that the ban amounted to "a triumph of expediency over legality". But the judges today effectively confirmed the Commission's sweeping powers to shut off trade flows across the world from any of the 15 members states where such drastic action is the only effective remedy to guarantee eradication of disease.

Before the ban was imposed Britain exported beef worth more than 520 million a year, with about 450 million going to other EU member states. In addition more than 400,000 live calves a year were exported for veal production on the Continent. The ban remains firmly in place except for certain types of beef from Northern Ireland.

EU agriculture ministers agreed earlier this year that most beef exports could resume from Ulster, the only part of the UK which has a comprehensive computer-based monitoring system for the movement of cattle. Similar systems acceptable to Brussels are expected to be in place in the rest of this country within the next 12 months.

The NFU said it was disappointed by the ruling but pledged to maintain vigorous efforts to ensure the ban was lifted soon. NFU president Ben Gill said: "The Government and our industry have taken enormous strides towards the lifting of the ban over the last few months.

"The EU Agriculture Council's decision to accept the Northern Ireland Export Certified Herds Scheme in March is a key first step. "We will now redouble our efforts in working with the Government to ensure that there is a speedy approval of the Date Based Export scheme, which is also being considered by Brussels. "The UK now has the toughest anti-BSE controls anywhere in the world -- it is only a matter of time before Europe must lift this ban."

The Date Based Export scheme proposed by the UK Government is to allow the export of cattle born after August 1 1996 -- the date after which it became illegal for any meat and bonemeal to be held on farms or feed mills.

Judge Tosses Christie Brinkley's Mad Cow Libel Suit

Wed, 6 May 1998 Associated Press
NEW YORK - A judge on Wednesday threw out Christie Brinkley's $42 million defamation lawsuit alleging the National Enquirer published lies about her, including that she has an irrational fear of cows.

State Supreme Court Justice Emily Jane Goodman said some of the stories last year were ``truly obnoxious,'' but they were not defamatory or subject to legal action.

Brinkley said the Enquirer falsely reported that she had a nervous breakdown, delusions and paranoia and was a ``wild shopaholic'' who spent $30,000 in one day on clothes.

Brinkley denied another Enquirer report that after a cow named Molly mooed at her during a tennis match, she asked police to shoot the animal because she feared it had mad cow disease.

"For the judge to say that the Enquirer's lies are merely `obnoxious' and not defamtory is incomprehensible,'' Brinkley said through spokeswoman Fran Curtis. Her judgment (ruling) doesn't protect individuals from being attacked, exploited, or damaged by such vicious lies, which I find shocking.'"

Dori Ann Hanswirth, a lawyer for the tabloid, said: ``The National Enquirer is delighted and believes the judge made the right decision. Celebrities ought to be a little more thick-skinned about these things.''

The CDC's CJD Program

A Congressional Mandate ... online at National Hemophilia Foundation site
In response to concerns that CJD may be transmitted through blood or blood products, the U.S. Congress requested that the CDC conduct a study to assess whether CJD is a threat to the safety of the nation's blood supply. Researchers believe that the risk of transmission to humans through blood products is very small since there have been no known cases of CJD contracted by humans in this manner. However, precautions are being taken to assure that the blood supply is safe from this infectious agent.

Current blood safety policy requires that any blood products made from blood donated by a person who later develops CJD, or is found to have risk factors for CJD, must be withdrawn. Until the question of transmissibility is resolved, the availability and the price of blood products will continue to be adversely impacted by shortages caused by recalls and the destruction of blood products which, to date, has cost over $100 million.

The Program: How You Can Help

Because the signs and symptoms of CJD may not develop for up to 30 years, a person could be infected and not show any symptoms during his or her lifetime. Furthermore, there is no screening test available for CJD and the only sure way to test for CJD is by analyzing brain tissue after death.

The CJD program asks families of individuals who have received blood products to donate brain tissue after their death. The brain tissue will be shipped to Stephen J. Armond, M.D., Ph.D., Professor of Neuropathology, University of California, San Francisco to test for evidence of CJD. Other brain tissue will be stored at CDC in Atlanta for analysis in the future when more is known about the cause of CJD.

Participation in the CJD program is voluntary. The CDC is aware that the death of a family member is a difficult time to make important decisions. Therefore, the CDC is working through your regional coordinator, physician, and treatment center staff to provide support to you and your family as you discuss brain tissue donation. To help with making an informed and rational decision, the CDC has developed information packets which answer commonly asked questions about the program including: how to authorize the donation; how confidentiality will be assured; the ability to retrieve the brain tissue without disfigurement and without affecting funeral arrangements; and the time frame for receiving test results.

Why Should I Participate?

Participation in this project is the only current way to help determine whether CJD can be or has been transmitted by blood products. If results show that CJD is not transmitted through blood products, needless recalls and shortages could be avoided. On the other hand, if this study shows that CJD may be spread through blood, research could then focus on the development of screening tests in an effort to maintain a safe blood supply. This project is the response of Congress and the CDC to avoid a potential threat to the current members of our community who use blood products and future generations who will rely on a safe blood supply.

If you would like more information or would like to participate in the CJD program, please contact Tami Wood-Lively, HFM's Regional Coordinator, in Michigan at (800)482-3041, ext.29 or if you are outside Michigan, at (734)761-2535, ext.29.

Oprah and the Beef

Washington Post editorial Friday, May 1, 1998; Page A14 
WHEN TEXAS cattlemen first sought to punish Oprah Winfrey for airing a show about mad cow disease in 1996, the Texas attorney general declined to file suit on their behalf because, he was quoted as saying, it would only draw further attention to the show's "outrageous claims" about the meat supply. Though this piece of eminent good sense didn't convince the cattlemen, several of whom went ahead and sued Ms. Winfrey themselves, you might have thought that their decisive trouncing in federal court last month would pound in the message. No such luck.

Not only have the cattlemen appealed in federal court; last week they assembled a slightly different group of 130 plaintiffs and filed virtually the same suit again in state court, charging, as before, that the broadcast drove down beef prices and lost the industry millions of dollars. If there are still three or four people in the nation who haven't heard about Humane Society activist Howard Lyman's speculations about how mad cow disease might spread through U.S. beef stocks, and Ms. Winfrey's sympathetic exclamation that she had eaten her last hamburger, their state of ignorance is unlikely to outlast yet another round of argument over Ms. Winfrey's right to broadcast discussions of food safety.

Like the first case, this one invokes Texas's ill-advised agricultural defamation law, perhaps on the assumption that a state court in a heavily beef-industry town will treat a state law more tenderly than did federal judge Mary Lou Robinson, who promptly declared it irrelevant to the case.

But there's far more wrong with the "veggie defamation" laws than their apparent weakness when applied to a case like the Winfrey one -- in which doubt was cast on even the threshold issue of whether beef on the hoof counts as a protected "perishable food product." Passed in 13 states under pressure from agricultural producers weary of sometimes alarmist discussion of food-borne disease, the laws drastically lower free-speech protections; some would even punish claims that a speaker believes to be true. Retrying the Winfrey case under this wrongheaded law reopens the possibility that it will be overturned as unconstitutional. That would be a fitting backfire to what looks more like simple bullying.

New book "Human BSE: Anatomy of a health disaster"

Mon, 04 May 1998  contact: Trevor Johnson
Book Launch and Public Meeting organised by the Socialist Equality Party: full details
Sunday 10th May  3pm
University of London Union
Malet Street, London WC1E 7HY
Nearest Tube - Euston Square
"I am writing to you as a commissioner of the Workers' Inquiry into BSE and CJD, which was organised by the Socialist Equality Party in March 1997 to uncover the truth behind the BSE crisis. The book "Human BSE:Anatomy of a health disaster" summarises the proceedings of the investigation and hearing. We are holding an event in Central London on Sunday the 3rd of May to launch the book and publicise its findings.

As at the hearing itself, we will be supported by families of the victims. Stan Mellowship, father of human BSE victim Donna Mellowship, will speak from the platform, and others will be taking part.

You will also have seen the review of the book in New Scientist, to which we are currently writing a reply, but which admits that "it did give a platform to the relatives of those who have died. Their anger, pain and legitimate concerns are cast at the feet of Lord Justice Phillips as he wrestles with the fraught questions raised by Britain's mad cow crisis."

History of MBM

4 May 98 From the BSE Inquiry
Meal and bone meal has been used for cattle feed was first recommended by Justus von Liebig in the nineteenth century. Here we see the government ordering it to be used in the World War II era.
17             Another Order that is of interest, and perhaps we
18       might look at this briefly, is the Amendment Order of
19       1946, just after the war.  If you could be provided with
20       bundle L3, and if we could look at it at tab 1B?  What
21       we have here is an Amendment Order made under the
22       wartime legislation, suppliers and services transitional
23       powers.  In this amendment regulation, the schedule of
24       conditions as to the content of various national
25       compound feeds are revised.Day 17 - 27 April 1998page 14
1             If we turn to "National Cattle Feed", number 3, on
2       page 2 of the Order, then we find that the ration there
3       for rearing young stock is required by paragraph 1:
4             "The compound shall ..." 5             Then (b):
6             "... contain the following ..."
7             And if we look down to the list, we see:
8             "Any animal-protein-rich substances ..."
9             The requirement there is that the
10       percentage-by-weight minimum should be 5 per cent.
11   SIR NICHOLAS PHILLIPS:   With no upper limit.
12   MR WALKER:   With no upper limit.  So we find there that
13       during the war, the ration for rearing young stock was a
14       ration which, by legislative compulsion, included 5 per
15       cent of animal protein, so that these herbivores were,
16       by force of law, being fed a meat derived product.

Hospital staffs in 'breakdown' over CJD woman's eyes

By Celia Hall, Medical Editor Telegraph ... Friday 3 April 1998
A breakdown in communication between two Stirlingshire hospitals was a major factor when the eyes of a CJD victim were used in three transplant operations, a Government report stated yesterday.

Marion Hamilton, 53, of Mayfield Street, Stirling, had been suffering from cancer and died in Strathcarron Hospice, Denny, in February last year. The official cause of death was given as brochopneumonia and inoperable cancer of the lung. Her eyes were offered for donation with the family's agreement.

Her husband, Thomas Hamilton, 61, a retired Ministry of Defence engineer, suspected that she had been suffering from CJD, the human form of mad cow Disease, for at least 15 years.

The Scottish health minister, Sam Galbraith, ordered a review, headed by Sir William Stewart, chairman of the Dundee teaching hospitals NHS trust, after the mistake became public in December. Three patients received parts of Mrs Hamilton's eyes .

Sir William praised staff at the Strathcarron Hospice who had sought to ensure that the necessary was done to comply with the protocols for eye donation. The hospice properly asked for a post-mortem examination as a possible means of explaining Mrs Hamilton's long-standing neurological symptoms and behaviour changes, which had become much worse in the terminal stages of her illness.

While the Strathcarron staff did not know that a neurological disease of unknown cause was a reason for not recommending eye donation, they emphasised Mrs Hamilton's abnormal behaviour in medical notes which went with her body when it left the hospice.Sir William said that the breakdown in communication that followed was "a major factor" in the events.

Pathologists who carried out the post-mortem at Stirling Royal Infirmary were unaware Mrs Hamilton's eyes were to be used for transplants. At Falkirk and District Royal Infirmary an opthalmologist who removed them was unaware that there was to be an investigation into Mrs Hamiliton's undiagnosed neurological condition.

Sir William said: "There was clearly a breakdown in communication between staff at Stirling Royal Infirmary and Falkirk and District Royal Infirmary". The Falkirk opthalmologist did not see Mrs Hamilton's case notes, or the post- mortem request form which emphasised Mrs Hamilton's abnormal behaviour. "The result was that the eyes were retrieved without the retrieving officer having access to all the available information."

Mrs Hamilton's eyes were passed to the Manchester Eye Bank with no mention of any need to quarantine them, and with a unsigned form recording the cause of death as breast, not lung, cancer. Her eyes were used by the time the post- mortem was complete. Sir William's team concluded that the benefits of eye tissue transplantation greatly outweighed the risks despite the Marion Hamilton affair.

Mr Hamilton said in an earlier interview that he felt strongly for the recipients of the eye tissue particularly two younger people involved who for 20 years could suddenly be struck down with the disease. "To live with that hanging over them is horrific," Mr Hamilton said.


Gov't May Help in Globulin Shortage

Fri, 8 May 1998 The Associated Press By LAURAN NEERGAARD
WASHINGTON (AP) - The pharmacy director of Children's National Medical Center spends hours each week searching the country for a few doses of a medicine called immune globulin that is vital to sick youngsters at the Washington hospital.

He often cannot get the blood-based drug from its makers, which, because of a national shortage, are selling supplies as fast as they are brewed. So he turns to the medical world's version of ticket scalpers, paying $85 a gram to middlemen for a drug he usually buys for $25.

``We simply have no other options,'' pharmacist Douglas Scheckelhoff told Congress Thursday.

The frustration is worse for patients whose lives depend on regular infusions, said Donna Hobson of the Nebraska Immune Deficiency Foundation. ``My biggest fear is that one day there will be none available and ... I will succumb to some type of infection that will place me back in the hospital - or worse.''

A national shortage of immune globulin - vital for tens of thousands of Americans battling immune-system diseases, including children with AIDS - developed in late November. Even though the Food and Drug Administration urged manufacturers to establish emergency stockpiles for the sickest patients, the shortage promises to continue through this year.

Lawmakers scolded manufacturers Thursday for not taking steps to prevent the crisis, saying with demand rising 10 percent a year, they should have planned ahead. Questioning why companies continue to export immune globulin and how middlemen manage to buy up scarce doses, they said the federal government may have to intervene.

``Since you make a lifesaving product, companies have an obligation to meet'' demand, Rep. Christopher Shays, R-Conn., told major manufacturers of the drug. If not, he added, ``government's going to have to step in.''

``You are pointing at more government intervention,'' responded John Bacich of Baxter Healthcare Corp., who argued that the sudden shortage of immune globulin was a surprise. Instead, ``challenge us to fix it.''

Manufacturers denied raising immune globulin prices because of the crisis or knowingly dealing with middlemen who inflate prices. They said they are quickly attempting to upgrade their factories to produce more immune globulin, but that the changes cost millions of dollars and will take time.

``We have never stockpiled'' immune globulin, said Gail Gaumer Schulze of Centeon, whose production stoppage last year while the company struggled to meet FDA quality requirements is blamed for much of the shortage. ``We have never price-gouged. To our knowledge we have never entered relationships with people who have.''

Experts blame the shortage on several factors: Doctors prescribe immune globulin too often. Half the supplies are used against diseases it is not proved to help, Surgeon General David Satcher said Thursday.

Some companies slowed production last year to correct violations of FDA manufacturing rules. Several batches are quarantined against a theoretical threat, brain-destroying Creutzfeld-Jakob disease. Although that disease has never been found to spread in blood, some donors have been discovered at risk for the disease and the quarantine is a precaution. About 20 percent of immune globulin is exported.

``If there are shortages in this country and blood being donated by Americans is being sent overseas, we have to address it,'' Shays said.

He asked if patients would take stocks of the drug that have been linked to the theoretical brain risk, something the FDA sometimes allows as long as doctors first fully explain the choice. Hobson said she was offered that choice and took it because ``I don't believe I could live without'' the drug.

The FDA cannot force manufacturers to make more immune globulin, control middlemen's pricing or exports, or ensure that doctors prescribe it only to immune-deficient patients, acting Commissioner Michael Friedman testified.

Asked if Congress should consider such intervention. Surgeon General Satcher urged finding a way to curb doctors' overprescribing, and the Centers for Disease Control and Prevention suggested creating a national emergency stockpile.

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