Heparin anti-clotting agent made from banned bovine mucosa
Canadians Notify 525 families that kids have received tainted blood
New Zealand quarantines blood supply
Please help locate vicitms of CJD who were blood donors
Leucocytes contaminated in woman with CJD
Transmission by Milk
CJD as Fast-Acting Alzheimer's
U.S. Disease present inDowner Cows
U.S. Variant of BSE
Inadequate British Measures

Heparin anti-clotting agent made from banned bovine mucosa

Bruna Parma R.& D. Dept.
Opocrin S.p.A.
25 Jun 1996

I am a biologist working in the R&D Dept. of a pharmaceutical plant producing extractive drugs from animal organs,in particular heparin. In the last years we had a lot of troubles with BSE because in the past our heparin was from bovine mucosa.

When the BSE problem arose, we performed (according to International guidelines) a very expensive validation study (made by a qualified institute in Scotland) in which our heparin production method completely purified completely raw material known to have scrapie agent.

The method of extraction and purification of heparin is formed by three steps: the first step resulted in a clearance of 2.9 log, the second step and the third completely removed/inactivated the input Scrapie spike which was 7.7 logs.

To be sure of our results we asked one of the most important experts of BSE for comment; in a detailed Scientific Report he declared, on the basis of the validation study, raw material from a BSE-free country [Brazil], and of a lot of other important reasons that our heparin was free of BSE.

Despite of this, we were obliged by Health Authorities to change raw material source (pork intestinal mucosa instead of bovine intestinal mucosa).

With an enormous lost of money and consequent lost of jobs in Italy and in Brazil, we had to convert our production into pork heparin with the consequent revision of the production, purification, analytical methods. Incidentally, we were studing a very interesting fraction of heparin active against heavy cardiovascular pathologies.

Speaking about things less tragical then death but very important: How much is the "only" economical damage caused by this "hysteria" originated by misinformation? How many people lost and are losing their jobs (farmers, workers of food and pharmaceutical industry, etc.), not only in UK but in all Europe and, my Brazilian collegues are an example, in the world?



NATO calls blood safety conference in The Hague

THE HAGUE, Netherlands (May 3, 1996 3:30 p.m. EDT) -- NATO said Friday it will host a major international conference here next week to discuss the safety of blood transfusions in battle zones in the light of infections such as HIV/AIDS and Creutzfeldt-Jakob Disease of the brain.

The NATO Military and Civil Blood Conference, the fourth in a series, will be held in The Hague from May 6 to 8. It has been organized by NATO's Joint Medical Committees in collaboration with the Dutch Defense Ministry and blood transfusion services.

"The main aim is to improve the interchangeability of blood and blood products between NATO forces," Joost Ruitenberg, chairman of NATO's Scientific Committee told Reuters.

The program comprises around 50 speakers from 25 nations, with an audience of 150 military and civil doctors, scientists and technicians all related to blood and disaster medicines.

In a timely contribution, one lecture from Paul Brown of the U.S. National Institute of Health Monday will be on whether blood and blood products can transmit Creutzfeldt-Jakob Disease, the human brain-wasting disorder at the heart of the British mad cow scare.

Another from Cees van der Poel of the Dutch Blood Bank will be on the harmonization of ways to detect infectious diseases, including Human Immunodeficiency Virus (HIV), which causes AIDS.

Further discussions will focus on deep-frozen blood for use during NATO and UN peace keeping and enforcing operations, plans for the exchange of blood and blood products among allied forces and storage logistics in extreme climates.

Copyright © 1996 Nando.net
Copyright © 1996 Reuter Information Service

New Zealand CJD Blood Quarantine

WELLINGTON, New Zealand (May 4, 1996 7:05 p.m. EDT) - New Zealand health officials said Saturday they had quarantined blood products from a donor who died of Creutzfeldt-Jakob Disease and played down any risk that other people could be infected.

Copyright CNN news



Notice to Physicians

Please Help Locate Patients with Creutzfeldt-Jakob Disease who may have been blood donors


The transmission of Creutzfeldt-Jakob disease (CJD) by dura mater, growth hormone, gonadotrophin and cornea has been documented, but the possibility of its transmission by blood transfusion remains controversial. However, a number of manufacturers and blood programs, including the Canadian Red Cross, have taken precautionary measures to prevent the theoretical risk of blood-borne transmission.

The Canadian Red Cross seeks physicians' cooperation to identify, with their consent, patients who have been diagnosed with CJD and who have donated blood. We will then be able to find and remove blood components and products that were derived from blood donated by these patients.

We would also like to know if any such patients received blood transfusions. This information will help us further study the relationship of CJD with transfusion. Your cooperation will be most appreciated.

The blood supply in Canada is safe, and with your help we will be able to make it even safer.

For notification and further information please contact Dr. M.T. Aye, National Director, Blood Services, Canadian Red Cross Society, 1800 Alta Vista Dr., Ottawa ON K1G 4J5; tel. 613 739-2220, fax 613 739-2505.

July 26, 1995.


Canadian hospital warns parents of dangers in blood supply

[[Biological errors were corrected in the story below, e.g., CJD is prion-caused, not viral -- webmaster]]

TORONTO (May 5, 1996 10:05 p.m. EDT) -- In the latest blow to Canada's blood supply, a top hospital has warned hundreds of parents that their children may have received blood products infected with a lethal brain prion.

Officials at Toronto's Hospital for Sick Children say 525 families have received letters about the incident, in which a man who donated blood died last year of Creutzfeldt-Jakob disease -- the human equivalent of mad cow disease.

Though the risk of getting the disease through transfusion is almost nil, chief pediatrician Dr. Robert Haslam said the trust of the public was at stake.

"We debated the pros and cons of telling the parents," he said during a news conference Friday. "Let me tell you, there was a lot of debate."

The hospital decided to warn parents because "it was the ethical thing to do," Haslam said, adding that other hospitals should follow Toronto's example.

Already, 80 parents who received letters have called on the hospital's hotline. "They're very angry and they are very anxious," Haslam said.

The blood products were used at the hospital between 1989 and July 1995, the same month the Red Cross learned of the donor. That prompted the largest blood recall in the agency's history in Canada.

The Red Cross has been deluged with calls about the virus, as it struggled to cope with fallout from a continuing judicial inquiry into how thousands of Canadians were infected with AIDS and hepatitis C in the 1980s.

Many Canadians -- mostly hemophiliacs who must use blood products -- have already received warning letters from their doctors. In Alberta, for example, the Calgary Regional Health Authority sent letters last year to 1,000 people who received blood products.

A Calgary man who received one of those letters later died of Creutzfeldt-Jakob disease, but officials stressed they could not link the death with tainted blood products. [[If the odds are 6 in 1,000,000 of getting CJD in 6 years, actually the odds of 1 in 1,000 getting the disease is very remote -- webmaster]]

Hospital officials say there has never been a documented case of someone getting the disease through a blood transfusion. [[No surprise -- there is no conceivable way that this could be documented -- webmaster]]

Creutzfeldt Jakob is very rare, afflicting just one in a million people. Symptoms, which can take up to 30 years to develop, include loss of co-ordination and dementia. Death occurs about seven months after symptoms appear. No one knows what causes it, and there is no test to detect it. The only way to tell whether someone has the disease is to examine their brain after they die.

In March, the British government admitted that people may risk contracting the disease if they eat beef from cattle infected with bovine spongiform encephalopathy, or mad cow disease.

Britain has started to slaughter thousands of cattle to restore confidence in its beef.

Copyright © 1996 Nando.net
Copyright © 1996 The Associated Press


Listserve Item of 8 May 1996: A letter in the New England Journal [one page letter, about 1992 or 1993 and occurred in the second volume for that year, about page 460] of Medicine which indirectly suggests to me that blood from someone with CJD could be infectious. In that letter, the correspondent reported on the death of a pregnant woman who died of CJD. If my memory serves me correctly, one of the substances from the woman that was found to be infectious were leukocytes from cord blood. Although most blood transfused in the US is given as packed RBCs, there are always a few leukocytes mixed in.

The colostrum (the milk produced early in the post partum period) from this woman was desecribed as containing the infectious agent.

Bob Lamparter [pathologist]
Lewisburg, PA


MAD COW DISEASE: A SOBERING "WAKE UP CALL"?

The WellnessWise Journal ... April 14, 1996

Copyright 1996 by David J. DeRose, MD, MPH

"Mad Cow Disease" grabbed headlines in late March 1996 when a group of prestigious British Scientists were constrained to admit that the fatal disease could possibly be transmitted to humans. The news brought the British meat industry to a virtual standstill. English folk avoided the meat markets. The European Union and a cascade of other countries banned British beef.

Referred to as Bovine Spongiform Encephalopathy (BSE) by the medical community, the illness is aptly named. The designation makes it clear that we are talking about a brain disease (encephalopathy) that affects cows (bovine). Furthermore, the disease riddles the afflicted cow's brain with tiny holes--causing it to look like a sponge under a microscope (thus rendering the brain "spongiform"). The lay term, "mad cow disease," in addition to being much easier to pronounce, also conveys the fact that infected cows may develop mental deterioration and behavioral abnormalities.

First diagnosed in Britain in 1986, BSE has powerful emotional appeal. It is one of a group of fatal brain diseases that can be transmitted from one living creature to another. These diseases are technically called "transmissible encephalopathies." Many British consumers had apparently comforted themselves for years with the thought that it was unlikely for such a disease to spread from cows to humans. However, even before March 1996, I would guess that scientists who were aware of this fatal cattle disease would--if honest--have to admit to some level of discomfort. After all, transmissible encephalopathies affect many other creatures besides cattle. Some 16 or18 animal species have been documented to come down with diseases that resemble BSE. Many or all of those species can apparently get a BSE-like disease from eating infected cattle meat. Furthermore, cattle themselves likely contracted the disease from a different species. Most researchers believe that BSE was introduced into British cattle herds by feeding them dead sheep carcasses which were infected with a similar illness called "scrapie." (The sheep disease gets its name from the fact that afflicted sheep can become mentally unstable and literally "scrape" the wool off their hide.)

HUMANS CAN GET TRANSMISSIBLE ENCEPHALOPATHIES

However, it is not only animals that can contract transmissible encephalopathies. Humans can also get them. A disease called Creutzfeldt-Jakob disease (CJD) can be thought of as a type of fast acting Alzheimer's disease. This is an extremely rare illness that has typically occurred in older individuals. I have personally only seen one patient with this rare and fatal disease.

We know from cannibalistic tribes in New Guinea, that a disease like CJD is transmissible to humans. There, at one time, the dead were honored through ritual cannibalistic rites. Children typically ate the brains of their deceased parents during these rituals. Many of them did get a disease called "kuru" which is a transmissible spongiform encephalopathy similar (if not the same as) CJD. The disease sometimes took up to 30 years to develop. Young children who engaged in these cannibalistic practices tended to develop the illness sooner than their older siblings or peers who also consumed infected brains.

Many scientists had speculated that if sheep could transmit the disease to beef, beef could transmit the disease to humans. In fact even in the United States, a country which claims to have no evidence of BSE, there have been reports that are less than comforting. For example, an outbreak of a related illness, Transmissible Mink Encephalopathy occurred on a Wisconsin mink farm. It was discovered that the mink illness was likely related to that farmer's practice of feeding them local "downer" dairy cows. This is a designation for cows that either are so ill that they fall down and can not get up, or actually are found dead on the farm. Deemed unfit for human consumption, such cows have been used for animal feed among other things. In this case, the carcasses found their way into the minks' diets.

The mink outbreak provided another example of probable BSE infecting other species. (The link could not be made definitely since the "downer cow or cows" that were fed to the mink were not autopsied.) It also raised another concern: countries like the US who think they have nothing to worry about, may in fact have diseased animals in their herds.

Despite assurances from the USDA that BSE has not crossed the Atlantic, the mink story leaves us with an uncomfortable feeling. However, there is even more disturbing news. Laboratory research has demonstrated that different strains of prions do exist. For example, a British researcher found that when he injected the prions from an infected group of goats into mice, those mice developed an encephalopathy (brain disease) characterized by drowsiness. The prions from a different group of infected goats also caused brain disease when injected into mice; however, instead of becoming lethargic, the diseased mice turned hyperactive! The message was clear: there were apparently at least two different strains of scrapie-like illness in goats. When transmitted to another species (in this case the mice) the infected rodents came down with one or the other of these two very different looking diseases.

Such research has raised an important question. If scrapie in goats could give rise to different appearing diseases in mice, could sheep scrapie result in more than one type of cow disease? There is some suggestion that this could be the case. There may well be more than one strain or type of BSE. The different types may cause different looking diseases. Some who have been studying links between cattle and mink disease in the US, have raised the concern that BSE is present in America, but "looks different" than the British variant due to a different BSE strain. Specifically, the BSE in the US may be one cause of "downer cow" disease rather than its "mad cow" cousin in England.

FINALLY TAKING NOTICE

It was Creutzfeldt-Jakob statistics that really got the medical community's attention in March 1996. By that time some eight young Britons had succumbed to this devastating illness. The unusual occurrence of the disease in young people combined with a different pattern to the sponge-like brain destruction, prompted leading British scientists to raise the concern that BSE may be the source of a new variant of CJD. Further thickening the plot was the occurrence of a worrisome cluster of CJD in British dairy farmers.

The biggest concern, of course, is that we are just glimpsing the tip of that proverbial iceberg. One prominent researcher is putting estimates of BSE-related CJD well into the thousands. And the problem does not appear to be confined to Britain. There are worrisome reports now from France and Italy. Still others are raising concerns that some cases of "Alzheimer's" may actually be spongiform encephalopathy. Granted, all of these conjectures may not prove true; but what if just some of them are? Even if only in part?

PRIONS: FRIGHTENING CARRIERS OF DISEASE

Adding to the fear of this relatively newly appreciated disease is the nature of the BSE infectious agent. Many animal borne diseases are caused by bacteria or viruses. Risk from these illnesses can often be decreased or eliminated by a combination of careful washing, refrigeration and thorough cooking. Bovine spongiform encephalopathy, however, appears to be caused by an unusual infectious agent known as a "prion" (pronounced "pree-on"). Prions are truly very unusual infectious agents. They have no genetic material and consist entirely of protein. Research suggests that these prion proteins are similar in structure to proteins that occur naturally in the brains of humans and other animals. The prions differ from those similar normal proteins, however, in their three dimensional shape. Prions seem to cause disease by coming into contact with those normal proteins and then stimulating them to change their shape to mimic the prion protein. This change in shape appears to set up a chain reaction. Normal proteins change their shape to look like the prion proteins and then later influence neighboring proteins to do the same. The end result is a progressively devastating and ultimately fatal disease. No treatment has yet been discovered.

Making the picture even bleaker is the resiliency of prions. They are not destroyed by the usual means used to kill infectious agents. They are resistant even to boiling at temperatures as high as 250 degrees Celsius (well over 400 degrees Fahrenheit). They are also resistant to ionizing radiation.

Prion related diseases are also extremely difficult to diagnose. There is no way to do a blood test for BSE and an infected animal does not mount any immune response to the infection. Tell-tale signs of diseases like BSE are usually only possible to diagnose at autopsy. However, not all infected animals necessarily have the characteristic sponge-like brain changes. Of particular concern, animals who carry the disease and have not yet developed signs of BSE, may not show the characteristic brain involvement.

WHAT ANIMAL PRODUCTS ARE FREE OF PRIONS?

Of even more concern is the fact that we do not know which tissues could carry the infection. Most of the focus of late has been on beef. This is warranted. Although organ tissue carries the greatest risk, animal studies do suggest that meat itself can transmit prion-related diseases. However, we cannot exclude the possibility that milk could also carry disease-inducing prions. Many may recall how some years ago assurances were given that a mother could not pass HIV to her child by nursing. Of course, we now know that HIV can be transmitted in breast milk. Granted, prion diseases are vastly different than HIV. The risk from milk (if it does exist) does appear to be much smaller than from eating beef or cattle organ tissues. Nonetheless, a British BSE expert has pointed out that there has been at least one human case that suggests passage of prions in milk. This was the case of a woman dying with CJD who demonstrated the infectious agent in her colostrum (the type of breast milk made in the initial days following delivery).

Eating the flesh of other animals (besides cows) or drinking their milk may not be safe either. These other animals may also be affected with a prion-related diseases. As we have already mentioned, sheep as well as goats can become infected with such illnesses. Even if some of these illnesses do not seem to be easily transmitted to humans (because of what is often called, "the species barrier"), the possibility exists that different strains of prions could present more danger.

OTHER CONSIDERATIONS

There is one other very important aspect that needs to be reemphasized. These transmissible spongiform encephalopathies have extremely long incubation periods. (This is the technical term for how long it takes for a person with the infection to actually show signs of the disease) As already mentioned, in humans, there is evidence that kuru can take up to 30 years to manifest itself. This is likely to be the same with CJD. With BSE, it may take up to 6-8 years or more before infected cows show signs of the disease. The important implication is that thousands of animals and humans may now be infected yet not develop symptoms for many years.

The current British proposal to slaughter all older cows may not go far enough. If younger cows are infected they may not yet show any signs of the disease. However, an infected cow can theoretically transmit the illness whether or not it has yet developed symptomatic disease. The widely publicized 1988 ban on feeding dead sheep parts to British cattle did not stop the epidemic. When the statistics were reviewed in a 1995 report, over half of all the British cattle that had developed BSE were born after the ban was implemented. The important message is that young cows may be infected, and may be able to transmit disease to humans.

CONCLUSIONS

The Consumers' Association , an independent British "watchdog group", has been quoted as saying that the only way to avoid BSE risk would be to stop eating beef all together. Others are saying even more. Some are suggesting that the time has come to recognize that disease in animals has become so rampant that we all need to seriously consider becoming vegetarians. They point out that BSE is just one of many fatal diseases linked to animals. Literally hundreds of deaths have been documented in the US alone from a host of animal related infections. Some of the most significant causative agents may not yet be household words, however most will recognize at least one of them: salmonella, listeria, campylobacter, and yersinia. Of course, we can't forget that the risk of dying prematurely from the Western World's two leading killers, cancer and heart disease, generally rises with increasing consumption of animal products.

If there is even a small chance of getting a severe fatal disease, some are suggesting it would be prudent to avoid all animal products. But maybe a few other questions are more important. What is the next transmissible disease in animals that will be recognized? Do I want to wait for tragedy to strike me or someone close to me--or have I already had warning enough?

APPENDIX: IS IT ALREADY TOO LATE?

If you have been eating beef and using other animal products for years is it already too late? The answer is clearly, "no." In the case of human prion related diseases, there is evidence that the total amount of prion exposure affects the incubation period of the disease. In other words, someone who eats a large amount of prion containing foods may develop the disease in 10 years or less, while someone with moderate, yet significantly less, exposure may develop the condition in 15-20 years. Another with still smaller exposure may not come down with the illness for 30 or more years.

Thus, even if someone was doomed to come down with a condition like CJD (because of previous exposure to prions), it may be possible to delay the onset of the disease significantly. And don't forget: a diet that moves away from animal products can also dramatically help you decrease your risk of other diseases like heart disease and cancer.

FURTHER INFORMATION

Those interested in additional information on BSE and/or other animal related diseases are encouraged to ask to be placed on the mailing list for prepublication information on Dr. Neil Nedley's book when it becomes available. In addition to the written text, an accompanying CD-ROM with color visuals suitable for public presentations will be available. Send e-mail to "docderose@aol.com" with the request "Nedley list" (leave off the quotes, of course.) You will receive further information when it becomes available.

SELECTED REFERENCES:

Almond JW, Brown P, Gore SM, Hofman A, Wientjens DPWM, Ridley RM, et al. Will bovine spongiform encephalopathy transmit to humans? . British Medical Journal 1995;311:1415-21 (25 November)

Brown, P. Bovine spongiform encephalopathy and Creutzfeldt-Jakob disease: The link is unproved, but no better explanation is presently forthcoming. British Medical Journal, 30 March 1996

Dealler SF, Lacey RW Transmissible spongiform encephalopathies: the threat of BSE to man. Food Microbiology 1990;7: 253-79.

Dealler, S & Kent, J. BSE: an update on the statistical evidence; British Food Journal 1995, volume 97, p3-18;

Gore SM. More than happenstance: Creutzfeldt-Jakob disease in farmers and young adults. British Medical Journal 1995;311:1416-8.

Gore, SM Bovine Creutzfeldt-Jakob disease? Failures of epidemiology must be remedied. British Medical Journal, 30 March 1996

Gruzen, T; U.S. Never Banned Suspect Feed Fda, Keeping Watch On British Beef Crisis, Says Safeguards Exist; Chicago Tribune, Thursday, March 28, 1996

Hoinville LJ, Wilesmith JW, Richards MS. An investigation of risk factors for cases of bovine spongiform encephalopathy born after the introduction of the "feed ban." Vet Rec 1995;136:312-8.

Lacey RW, Bovine spongiform encephalopathy is being maintained by vertical and horizontal transmission, British Medical Journal 20 January 1996;312:180-1.

Lacey RW. Creutzfeldt-Jakob disease and bovine spongiform encephalopathy. British Medical Journal 1996;312:180-1. (20 January)

Patterson, WJ & Dealler, S. Bovine spongiform encephalopathy and public health. J. Public Health Medicine. Volume 17 number 3, 1995, pages 261-268.

Prusiner SB, Molecular Biology Of Prion Diseases. Science (Magazine) Vol. 252, pages 1515-1522; June 14, 1991.

Prusiner SB, The Prion Diseases. Scientific American. January 1995 Volume 272 Number 1 Pages 48-57.

Wells GAH, Wilesmith JW. The neuropathology and epidemiology of bovine spongiform encephalopathy. Brain Pathol 1995;5:91-103.


Send all correspondence to:

David J. DeRose, MD, MPH
Email:
docderose@aol.com (preferred) 74532.622@compuserve.com