Health alert after eyes of CJD victim are transplanted
CJD case prompts organ transplant review
Test on eye donor six months after grafts
Hemophilia doctors fear 'mad cow' contamination in blood stocks
CJD Voices: US families organize
More cheetahs dead in France
New Yorker piece: mad cows and angry scientists
Emerging epidemics: Washington Post essay
This mad pig went to market
40 tonnes of beefburgers tipped into the sea
Irradiation of meats approved
December 1 1997 Times MARK HENDERSONHEALTH officials launched an urgent inquiry into transplant procedures yesterday after it emerged that three patients had received tissue from the eyes of a Scottish woman with Creutzfeldt-Jakob disease, the human form of "mad cow" disease. Screening guidelines may now be changed. The corneas and sclera of Marion Hamilton, who died of lung cancer in February, were cleared for transplant despite a post-mortem examination which showed she had CJD. The results were not passed to the United Kingdom Transplant Support Service, which arranged for three patients - two men and a woman in her 80s - to receive Mrs Hamilton's eye tissue. It is not yet clear whether the results were available when the operations took place. Stirling Royal Infirmary, which carried out the post-mortem examination, is to hold its own inquiry.
Organs and tissues for transplant are not routinely tested for CJD because the disease is so rare, though they are screened for other viruses and bacteria. Organs such as hearts and livers cannot be tested for the disease as they must be transplanted within six hours of death and the CJD test takes months. Corneas can be tested as they can be kept for two months before transplant. Experts believe CJD can be transmitted though transplants.
Sam Galbraith, the Scottish Office Health Minister, said his department would investigate how the Stirling incident took place and ways of improving checks on donated organs: "It is important when we explore the cause of death that we consider what possible concurrent diseases ... are there. We will try and tighten up these procedures."
The Department of Health will consider a review of transplant procedures nationally when the inquiry has established what happened in Stirling.
Mrs Hamilton, 53, who had three daughters and was separated from her husband, had not had CJD diagnosed before her death. One of her daughters said she had been behaving erratically in the weeks before she died. She was said to have been staggering and falling over, and had become a "senile old lady" in her early 50s.
Mrs Hamilton was cared for at Strathcarron Hospice in Stirlingshire after inoperable lung cancer was diagnosed. Before her death she signed a donor registration form allowing for her eyes, which were not affected by the cancer, to be used for transplants. None of her other organs was donated.
A spokesman for the United Kingdom Transplant Support Service said it was satisfied that the Stirling incident was a one-off and welcomed the Scottish Office's swift move to investigate it. "We are sure the transplant was carried out in good faith, but there are always lessons to be learnt when there is such a serious mix-up."
More than 6,000 cornea transplants take place every year, and this was the first case of CJD contamination the service had seen, she said.
Stirling Royal Infirmary is part of the national transplant network, under which available organs and tissues are matched with patients by computer and sent to hospitals for immediate surgery.
[Guardian 5.11.97: Tainted blood raises CJD fears for in-vitro fertilization babies. This was due to a recently withdrawn contaminated blood product from the USA being used in the process. World-wide there are 3 documented cases of women suffering from CJD while pregnant and none of the children now aged 3, 7 and 22 have any symptoms. (also a case in Dundee, UK and a case in nvCJD in Manchester. - S.Dealler]
Daily Telegraph, 1 December 1997 By Richard Savill and Celia HallTRANSPLANT surgery throughout Britain is to be reviewed after parts of the eyes of a woman with CJD, the human form of mad cow disease, were given to three people. An urgent inquiry was launched yesterday as the Health Department demanded full details.
It also emerged that Britain's leading transplant doctors are already preparing new guidelines on standards and, as a result of the case, will be taking advice from specialists in Creutzfeldt-Jakob disease.
two corneas and a sclera, the white part of the eye, from Marion Hamilton, 53, were given to two men and a woman in her 80s. Mrs Hamilton died from lung cancer at Strathcarron Hospice, near Stirling, last February.
A post mortem investigation was carried out and a source at Stirling Royal Infirmary, whose staff were present during the autopsy, said this had not discovered the CJD. Diagnosis and confirmation came in later tests carried out in Glasgow and the information had come through only in recent weeks.
It was not known why or at what stage the brain matter was sent for tests, or whether CJD was suspected at that time. The UK Transplant Support Service, which handles organ donation arrangements, said it would not have considered transplantation if it had known about the CJD.
Robert Johnson, president of the British Transplantation Society, which represents transplant specialists, said they were working on new national guidelines for transplation.
"Certainly we will be seeking expert advice on CJD," he said. "It is a difficult area with latent diseases which can take years to develop."A major problem is that CJD is only fully diagnosed when brain samples can be studied after death. Full post mortem examinations are not routinely carried out on all potential donors, he said.
Sam Galbraith, health minister at the Scottish Office, which has launched the inquiry, said the case emphasised the need to tighten procedures for the screening of tissues used in transplants.
"It is important when we explore the cause of death that we consider what possible concurrent diseases, which are not all that obvious at the time, are there. We will try to tighten up these procedures."Mr Galbraith sought to play down the chances of a patient developing CJD after a transplant. "The risk of developing CJD after transplant is one per cent," he said. "The risk of receiving a transplant from a donor suffering from the disease is infinitesimally small. But there is a hole in our defences we must close.
"We need to make sure in future that we explore more fully not just the primary cause of death but the probable hidden manifestations of all diseases."Mr Galbraith said Mrs Hamilton was not suffering from the new variant of CJD which is believed to act more quickly and has been linked with eating meat from animals infected with BSE.
Last night, Stirling Royal Infirmary declined to comment, saying only that it was "co-operating with the Scottish Office in its inquiries". Sources said the hospital had "acted within current procedures and had done what is expected of them". The sources denied reports that the hospital may have bungled in not relaying the results of the post mortem.
"The post mortem merely confirmed that the lady in question died of lung cancer. There was no question of CJD being uncovered, diagnosed or confirmed through the post mortem. Other tests were carried out elsewhere. The confirmation of CJD has only come through in recent weeks."One of Mrs Hamilton's three daughters, Jane, 33, said the family had been "shattered" by news of her mother's condition. "I cannot believe nobody told us she had this bug," she told the Sunday Mail in Glasgow. She said her mother's mental and physical behaviour had become erratic. "She couldn't walk in a straight line and kept staggering to one side," she said. "Mum was always falling over."
Dr. Roland Heynkes
"In my opinion, this case illuminates a growing problem, which will not become smaller even when BSE will be eradicated. The necessity to reduce costs in hospitals leads to a reduction of thorough pathological examinations. At the same time BSE infections in the past decade or two, multiplied the number of people with developing CJD and increases the importance of fast and early CJD diagnosis. In this situation it is unacceptable, that a patient with known, likely neurological symptomes becomes a organ donor without comprehensive examination. And it is curious that it took nearly a year to get the diagnosis.
Does anybody know a study, that demonstrated a one per cent risk of developing CJD after transplantions? Of course, the risk to get an organ from an already suffering CJD patient, is very small. But in my opinion the risk to get an organ from an infected and CJD developing donor without symptomes is increasingly high, especially in the UK.
Sure, these measures sound good, but will we have the money? This could be much more expensive than the BSE crisis.
This seems to be a scandal, but the BSE crisis also improved the policy of openness especially in the UK and in the EU since the responsibility changed from Franz Fischler to Emma Bonino. In Germany we are still waiting for something like the british MAFF internet site..."
December 2 1997 Times BY IAN MURRAYTESTS confirming that a woman eye donor had suffered from Creutzfeldt-Jakob disease were carried out two weeks ago, more than six months after three patients were given corneal implants from her.
Marion Hamilton died of lung cancer in a Stirlingshire hospice in February and her eyes were used in operations performed in March and April. It was only last month that tests were ordered on tissue from her brain to find out if the reason she became unsteady on her feet shortly before she died was that she was suffering from CJD.
Sam Galbraith, the Scottish Health Minister, said a comprehensive review of the case would be announced later this week and would report shortly. As a Health Minister, surgeon and a transplant recipient, he was "only too aware of the concerns" being raised over the eye tissue transplants. He was satisfied that there had been no question of information regarding the patient's infection with CJD being withheld before the transplants took place.
"I am determined that we get the full facts of this case so that we can learn from it for the future," Mr Galbraith said. The inquiry will concentrate initially on why it took so long for tests to be made to discover why Mrs Hamilton had suddenly become senile and unsteady, even though she was only 53.
Her eyes were removed shortly after her death, in keeping with her own wishes, and within 48 hours had been sent to an eye bank run by the UK Transplant Support Service Authority at the Royal Eye Infirmary in Manchester. After the hospital carried out routine tests to make sure that the corneal material was not a carrier of hepatitis B/C or of HIV, they were cleared for use. Two patients, one from Wolverhampton and the other from Liverpool, received corneal grafts; the other, from Manchester, received a sclera - the white of the eye.
Andrew Tullo, consultant eye surgeon at the hospital, said last night: "The surgeons who carried out the transplant procedures have been informed as to the identity of the patients and we understand that the patients in question have now been informed."
He said that the hospital had followed all the transplant authority procedures for accepting corneal tissue for donation. "There is a list of conditions which, if apparent at death, would mean that the eyes would not be accepted for transplant. None of these conditions was reported to the hospital in this case."
He said it would be difficult to speculate on the chances of one of the patients contracting CJD as a result of the transplant. The only reported case of CJD developing from a corneal transplant occurred in America in 1974, 18 months after the operation.
Officials have emphasised that the variant of CJD diagnosed in Mrs Hamilton was the so-called "classic" strain, not the new variant that has been linked to BSE. [There is no such thing as a "classic" strain -- webmaster]
A helpline for patients who have had eye transplant operations using material from the bank at Manchester has been set up on 0161 276 8500.
November 28, 1997 TimesLONDON - British experts on hemophilia are seriously concerned about the safety of blood transfusions amid fears national stocks could be contaminated by the fatal human form of "mad cow" disease.
The Hemophilia Center Directors' Organization called Thursday for patients to be treated either with blood products from foreign donors or artificial products to ensure they were not exposed to the new form of Creutzfeldt-Jakob's Disease (CJD).
The British government has acknowledged there is a probable link between recent occurences of CJD in unusually young victims and Bovine Spongiform Encephalopathy (BSE), or mad cow disease, which causes progressive brain degeneration in cows.
The Hemophilia Society said it was aware that a number of people with the variant of CJD had given blood before their condition was identified.
Clotting factor concentrates used in treating hemophiliacs are produced by pooling blood from large numbers of donors, making it "probable" that some concentrates would have been in contact with blood from donors with CJD, the society said. It said, however, that it was not aware of any cases of mad cow-linked CJD among Britain's population of hemophiliacs.
Peter Flanagan of the National Blood Service said "we are sympathetic to the predicament they (hemophiliacs) are in. The simple fact is we don't know whether there is a risk."
British authorities recently twice recalled blood plasma products after discovering two of 22 listed victims who have died from mad cow-linked CJD donated blood.
Fri, 28 Nov 1997 Liz Armstrong
"CJD Voice is comprised of over 50 individuals who have either lost a loved one to CJD or are currently battling the effects. CJD Voice is a support group for those going through CJD with a loved one or for anyone that just needs "a little input from someone that has been there". Some of the topics covered recently included....[This is an excellent group that provides a very valuable service to the families of affected people. They provide specialized and compassionate coverage of issues and open discussion forums that provide special insight on these issues. Highly recommended. If you are just getting started with CJD, Dr. Shaun Heaphy of the University of Leicester maintains an excellent and balanced introduction to the whole subject as well as BSE news-- webmaster]
Care in the End..... everyone gave their opinion as to what was the best in each situation during the final stages of CJD, i.e. hospital vs. nursing home; we also have discussed the various immunizations each individual had during specific years prior to CJD - nothing overall in common; one thing we have discovered that almost every (I say almost, but I'm 99% sure it is ALL) patients prior to being "symptomatic" suffered from a dry cough for an extended period of time and had some vision problems. As moderator, I maintain copies of ALL correspondence and will be compiling everything to put on our homepage."
Sat, 29 Nov 1997 correpondentFrance reported 1 BSE-cheetah (FSE) on Feb 1997, 2 more on 28 Nov 1997 (all imported from GB). Do the British succeed in exporting all infected cheetahs, or don't they simply report own FSE-cases? Does anybody know where the 6 British FSE-cats (1997 cases) lived? And where the other 78?
Switzerland has just reported the 32nd mad cow in 1997. If my calculation is correct, a total of 262 now.
[The latest data from the UK (MAFF Progress Report on BSE in Gt Britain, June 1997) show 5 cheetahs (an increase of 1 over the November 1996 Progress Report), no reports of more recent UK cases.]
The New Yorker 12/01/97
"Later this month, Stanley B. Prusiner will receive the 1997 Nobel Prize in Physiology or Medicine, an award that strikes some scientists as unusual because the work for which he is being honored is still hotly disputed. As Richard Rhodes argues in the December 1, 1997, issue of The New Yorker, the ongoing debate is more than just academic; at stake is the fight against mad-cow disease, a fatal epidemic.
In his article "Pathological Science," Rhodes explains that Prusiner is being honored for his discovery of prions, rods of protein which he believes cause the cruel, untreatable, and invariably fatal maladies known as transmissible spongiform encephalopathies (T.S.E.s). Unlike all other known infectious agents, prions have no nucleic acids--no DNA or RNA. However, Rhodes argues, Prusiner has yet to prove conclusively that T.S.E.s are caused by prions and not by a virus.
If, as some researchers still believe, T.S.E.s are caused by a virus, isolating it--and then finding a vaccine against it--will be slow and painstaking. But, Rhodes writes, "hardly anyone is looking. The lion's share of research money is now directed toward prions." The debate also has implications for the way we fight the spread of mad-cow disease from animals to humans. The steps taken so far by the British and American governments to keep livestock feed free from T.S.E.s make sense whether the infections agent is a protein or a virus. But our next moves toward preventing and treating T.S.E.s "will hinge on the scientific theory of infection," Rhodes writes.
Rhodes notes that "Prusiner's Nobel Prize will enlarge his influence on the future of T.S.E. research; if he chooses to encourage a full range of studies--including continuing to search for a virus--that work will go forward." In his conclusion, Rhodes suggests that prion theory may turn out to be a case of what the chemist Irving Langmuir called "pathological science"--sensational scientific "discoveries," like cold fusion and "N Rays," that turn out to be false. Three of Langmuir's criteria for pathological science "fit surprisingly well with the current state of prion theory," he argues, including the one that states, "The effect is barely detectable." As Rhodes notes, "Since no one has demonstrated that prions are infective--the Holy Grail test--Prusiner's theory has yet to cross this threshold. So far, his effect isn't detectable at all."
Richard Rhodes is the author of "Deadly Feasts" (Simon & Schuster), about the search for the causes of mad-cow and similar diseases. His previous books include "The Making of the Atomic Bomb," which won the 1988 Pulitzer Prize in Nonfiction and a National Book Award. "
*** The December 1 New Yorker has a rather churlish Pruisner-debunking article by Richard Rhodes entitiled "Pathological Science." The prion theory is compared with cold fusion and other ". ..sensational scientific 'discoveries' that turned out to be false--not fraudulent but merely self-deluded." -- Paul Michelsen, D.V.M.
*** "Someone faxed me the full text. In brief, it is Rhodes carrying a bucket of water for East Coast virologists. The issues seem to be:1. Personality conflicts and professional envy,Show me one good paper on prion viruses. This isn't about viruses, it's about hurt feelings.
2. Fear of losing grant money to competitor.
If Prusiner can now channel grant money to more productive purposes, I say more power to him. However, this is a double-dedged sword. We don't need another Microsoft situation. There need to be competing labs and diverse research agendas. I didn't see any 15B3 (anti-rogue prion antibody) or laminin receptor coming out of Prusiner's lab. There have been better dividends lately from the three Swiss labs.
The rumor mill has Prusiner shutting down both the NIH and Rocky Mountain labs, both to expand the empire and to shut down competitors. This is going too far and not supported by the record. And I see dangerous public health implications, given the extravagant brown-nosing with the meat and drugs industries." -- webmaster
The Washington Post November 30, 1997 Stephen S. MorseHumans, it seems, are under attack. New and bizarre diseases lurk around every corner. It is a hypochondriac's nightmare made real: Will I die from mad cow disease or the deadly Ebola virus? Does my partner have the AIDS virus? Was my hamburger cooked well enough to kill E. coli bacteria? Is my favorite fishing hole teeming with Pfiesteria piscicida? Though these threats of disease seem as random as drive-by shootings, the reasons for them lie at the heart of our interaction with nature. As Pogo said, the enemy is us.
Many of these diseases are neither new nor inexplicable. Often, they are the inevitable product of the way that humans have changed the environment, the unanticipated consequences of human activities. Diseases formerly present only in the animal world have transferred to the human population because of changing conditions that allow increased contact between the two. But many of these diseases are not nearly as frightening as they first seem, because they often do not spread rapidly within the human population.
Our vulnerability to infectious diseases is not going to go away. The issue, then, becomes one of monitoring and, where possible, controlling new outbreaks. Toward that end, in 1993, Barbara Hatch Rosenberg, a colleague, and I convened a group of scientists under the auspices of the Federation of American Scientists to form ProMED (the Program to Monitor Emerging Diseases) in the hope of encouraging international cooperation in disease surveillance. A year later, ProMED linked interested scientists by e-mail in order to make it easier to report and discuss outbreaks of infectious diseases. The system has rapidly evolved into a worldwide unofficial disease reporting system that now boasts about 10,000 subscribers in more than 120 countries.
Popular literature often identifies these emerging diseases with exotic locales and tropical forests, but they can occur anywhere. Take hantavirus pulmonary syndrome, first identified in the southwestern United States in 1993. Beginning in May, patients were admitted to hospitals in New Mexico, Arizona and Colorado complaining of fever and breathing difficulties; more than half the patients subsequently died. By mid-August, there were 23 cases in the three states.
An alert emergency room physician, who was based in New Mexico (where most of the cases occurred) noted that two of his patients seemed to be stricken by the same strange disease, prompting him to alert public health authorities. Many possible causes were investigated. None was confirmed until until a broad range of laboratory tests at the federal Centers for Disease Control and Prevention (CDC) showed a positive reaction with two different known forms of hantaviruses.
The natural history of almost all known hantaviruses involves a rodent host, so attention immediately turned to trapping local rodents and taking blood and tissue samples. The most common rodent host, the ubiquitous deer mouse, was soon identified. Infected rodents shed the virus in their urine and droppings, and people who inhaled it unwittingly became infected. The preceding winter had been unusually warm and wet, resulting in a bumper crop of nuts -- and environmental studies showed an exceptionally large rodent population. As a result, researchers theorized, people came into contact more frequently with infected rodents and, hence, the virus.
It is unlikely that the virus itself was new in the rodents. Further sleuthing revealed occasional sporadic cases of this disease as far back as 1959, as well as the existence of related viruses in a variety of rodent species throughout the Americas. In fact, the virus was long present in rodent populations, with the occasional (and often undiagnosed) human case, but in 1993 the rodent population explosion in the Southwest increased the chance of human contact and led to the distinctive cluster of cases.
Temporary natural changes in climate caused this particular outbreak. More often, though, the ecological changes that precipitate a new infection's introduction into a human population are simply the unexpected result of ways in which humans change the environment. Many are the result of changes in land use caused by agriculture. The first hantavirus, identified during the 1950s, was called Hantaan, which caused outbreaks of Korean hemorrhagic fever. Hantaan, which gave its name to the hantavirus group, is a distant relative of the virus that caused the U.S. outbreak in 1993. It, too, is carried by a common field mouse. Increased rice planting in Korea, China and other Asian nations has led to more rodents and, as a consequence, many more cases of human disease over the last few decades.
This pattern of change is also evident close to home. Agricultural runoff, perhaps from chicken farms along rivers in Maryland, Virginia and North Carolina, may have provided the stimulus for the sudden exuberant growth of an existing microbe, pfiesteria -- and the subsequent diagnosis of human illness.
As another example, consider Ebola. Its natural host is probably another mammal, perhaps a bat, with which people foraging in the forest may accidentally come in contact on rare occasions in certain parts of Africa. The disease is becoming more prevalent as economic pressures force more people to forage or to attempt to grow crops in previously unused forest. It is a quick killer that rapidly causes victims to bleed internally and go into shock. And it is, ironically, the very speed with which it kills that prevents it from spreading more rapidly within the human population.
The success of a newly introduced infection generally depends on its ability to spread. Fortunately for us, many newly introduced infections, like Ebola, have been unable to make this transition successfully, but human activities and behavior can sometimes allow the dissemination of a newly introduced infection, despite the fact that the virus has not adapted to the human host to allow it to be transmitted efficiently from person to person.
Human population movements, upheavals caused by migration or war, can greatly affect this process. Migration from rural areas to cities, occurring increasingly throughout the world, can introduce remote viruses to a larger population. HIV is the most notable example of an infection that has spread because of these recent social changes, but it is unlikely to be the last.
The United Nations has estimated that, largely as a result of continuing migration, 65 percent of the world population will live in cities by the year 2025. The phenomenon of rural to urban migration can allow infections arising in isolated rural areas, which may once have remained obscure and localized, to reach larger populations, a situation exacerbated by the high population density and poor sanitary conditions of these newly burgeoning cities in developing countries. Once in a city, a new infection may find additional opportunities to spread further locally and, via air travel, around the world. This was how HIV succeeded in dispersing throughout the world, and it is likely that other infections will benefit from similar opportunities.
Historically, "new " diseases have appeared and spread, often as byproducts of exploration, trade or warfare, when the movement of people, animals or goods brought geographically isolated infections to new grounds. Most historians believe that yellow fever, and the mosquito that carries it, were spread from west Africa in the 16th century by ships plying the slave trade. Rats carrying bubonic plague spread to Europe in the Middle Ages, first by the overland route from central Asia (through war or the silk trade, or probably both), and later on ships. Smallpox was brought to the New World by the European colonists. In the 19th century, steamships carried cholera to Europe and Africa.
Today, trucks and airplanes have largely replaced caravans and steamships. With improved methods of transportation, this movement is more rapid and widespread today than at any time in the past, allowing richer opportunities for infections to emerge and spread.
In industrialized countries, high-density settings can allow both common and newly introduced diseases to spread rapidly once they have gained a foothold. Modern high-intensity agriculture is one example. Hemolytic uremic syndrome, the disease associated with several contaminated batches of hamburger meat in recent years, is caused by a newly emerged strain of Escherichia coli. E. coli is a common intestinal bacterium in both humans and cattle. Strains capable of causing disease were probably present for some time in scattered herds of cattle. Now, as cattle from all over the country are brought together, with the meat from many original sources pooled and rapidly processed in large batches, this once-isolated organism has a chance to find its way into meat that may go anywhere in the country. Similarly, bovine spongiform encephalopathy ( mad cow disease) first appeared 12 years ago in the United Kingdom. It is likely that a change in rendering processes allowed scrapie, a disease found in sheep, to be introduced into cattle that were fed with contaminated sheep byproducts.
Despite evidence of increasing opportunities for the emergence and spread of novel infections, there is still cause for optimism. We possess many of the tools to anticipate and control these infections. We understand many of the factors responsible for the emergence of diseases, and can work to deepen our understanding of how this knowledge can be used to counteract them. We should work to make these same tools available throughout the rest of the world.
Dealing with emerging infections will also require the ability to recognize these pathogens when they first appear, and to act appropriately. Ultimately, taking the approriate actions will require strong international political will, and is likely to remain a stumbling block for some time to come. Nonetheless, an effective early warning system -- global infectious disease surveillance -- with rapid diagnosis, is indispensable to solving the first problem, that of immediate recognition. A worldwide network of early warning centers will be required. National and international capabilities for disease surveillance must be expanded and strengthened
Institute for Public Affairs In These Times June 8, 1997 JOEL BLEIFUSSİİİSome pigs in the United States may be infected with a porcine form of mad cow disease, according to an alarming study by U.S. Department of Agriculture (USDA) scientists that has recently come to light. This previously unrecognized form of the disease in swine may be infecting humans, according to epidemiological studies that link pork consumption with mad cow's human equivalent, Creutzfeldt-Jakob disease (CJD).
In late 1978, Dr. Masuo Doi, a veterinarian with the Food Safety and Quality Service, observed signs of a mysterious central nervous system (CNS) disorder in some young hogs that had arrived at the Tobin Packing Plant in Albany, N.Y., from several Midwestern states. For the next 15 months, Doi studied 106 of the afflicted pigs. He described their symptoms this way: "Excitable or nervous temperament to external stimuli such as touch to the skin, handling and menacing approach to the animals is a common characteristic sign among swine affected with the disease. " These symptoms, Doi now notes, are strikingly similar to those of British cattle infected with mad cow disease, which is scientifically known as bovine spongiform encephalapathy ( BSE) .
Doi sent brain material from these pigs to Karl Langheinrich, the head pathologist at the USDA's Eastern Laboratory in Athens, Ga. In a November 1979 report, Langheinrich noted that one pig's brain exhibited what the veterinary reference work Pathology of Domestic Animals defined as "the classical hallmarks of viral infection of the central nervous system. " Langheinrich went on to report that the damage in the pig's brain was similar to the damage observed in the brains of sheep afflicted with scrapie and of mink afflicted with transmissible mink encephalapathy, the two other variants of transmissible spongiform encephalapathy (TSE) known at the time.
In March of this year, Dr. William Hadlow, a retired veterinary pathologist who is one of the world's leading TSE researchers, examined the microscope slides of pig brain from Doi and Langheinrich's 1979 investigation. The pig "could have suffered from a scrapie-like disease, " he reports, but adds that such a conclusion cannot be "justified by the limited microscopic findings, however suggestive of a TSE they may be. "
The Government Accountability Project (GAP), a Washington-based organization that supports public-sector whistle-blowers, has been working with Doi to alert the public that a porcine form of mad cow disease may be circulating in the American pig population. In a March 27 letter to Secretary of Agriculture Dan Glickman, GAP points out that if we assume a similar incidence of central nervous system disorders in swine being slaughtered nationwide as that found among swine at the Tobin Packing Plant, "it is reasonable to question whether, since at least 1979, USDA has been allowing 99.5 percent of animals with encephalitis, meningitis and other CNS disorders into the human food supply. "
And what happens once those thousands of diseased pigs are eaten by the American public? Two epidemiological studies found pork to be a dietary risk factor in Creutzfeldt-Jakob disease (CJD). A 1973 study published in the American Journal of Epidemiology discovered that 14 of 38 CJD patients (36 percent) ate brains. Further, of those who ate brains, most (10 of the 14) preferred hog brains. Another study published in the American Journal of Epidemiology in 1989 looked at how frequently 26 CJD patients ate 45 separate food items. Nine of these foods were found to be statistically linked to increased risk of CJD. Of those nine, six came from pigs--roast pork, ham, hot dogs, pork chops, smoked pork and scrapple. (The three that were not pig-derived were roast lamb, raw oysters/clams and liver.) The authors of the study concluded: "The present study indicated that consumption of pork as well as its processed products (e.g. ham, scrapple) may be considered as risk factors in the development of Creutzfeldt-Jakob disease. While scrapie has not been reported in pigs, a subclinical form of the disease or a pig reservoir for the scrapie might conceivably exist. "
The number of Americans who develop CJD in a given year is in dispute. The Centers for Disease Control (CDC) claims that the human form of mad cow disease occurs at a rate of one in a million. Further, ignoring evidence of a new variant of CJD found in Britain, the CDC maintains that people who eat an infected animal cannot contract the disease. In January, CDC Assistant Director for Public Health Lawrence Schonberger told a congressional hearing, "The bottom line from our perspective is that it's a theoretical risk ... but it is not as yet really a real risk. "
But does the CDC really know how many Americans contract CJD? Evidence indicates that CJD may often be misdiagnosed, and thus go unreported. A 1989 study at the University of Pittsburgh autopsied the brains of 54 patients who had been diagnosed with Alzheimer's and discovered that three of the patients (5.5 percent of the sample) actually had CJD. A 1989 study at Yale University reported similar findings. Postmortem examination of 46 patients who had been diagnosed with Alzheimer's revealed that six (13 percent of the sample) actually had CJD. The New York-based Consumers Union, which publishes Consumer Reports, argued in a paper presented to the USDA, "Since there are over 4 million cases of Alzheimer's disease currently in the United States, if even a small percentage of them turned out to be CJD, there could be a hidden CJD epidemic. "
Which brings us to the issue of what the Food and Drug Administration (FDA) is doing to address this food-borne threat to public health. In the past several months, in response to questions about Doi's 1979 pig research, USDA officials have put out a good deal of misinformation to public-interest groups, the media and even the National Association of Federal Veterinarians. On repeated occasions, officials have said that the slides of the pig brains from the 1979 study were unavailable because they had been sent to scientists in England who were studying mad cow disease. But, it turns out, the USDA never sent any slides to England.
"Agency officials repeatedly misrepresented scientists' investigations and conclusions to consumer groups and government employees and neglected to keep other agencies also working on TSE issues informed, " says Felicia Nestor of GAP. "The USDA had to be pushed to investigate scientific evidence which only they had. "The USDA's lackluster response to this public health threat comes as no surprise. For years, the agency has done its best to ignore evidence that a distinct American strain of mad cow disease may already afflict the U.S. cattle population. (See "The First Stone, " May 31, 1993 and April 15, 1996.) Veterinary researchers in Mission, Texas, in 1979 and Ames, Iowa, in 1992 found that cattle injected with brain matter from scrapie-infected American sheep developed BSE. However, the brains of these infected cattle did not exhibit the spongy holes found in the brains of their BSE -plagued British cousins. Furthermore, cows afflicted with this American strain of scrapie- induced BSE do not go mad; they simply collapse and die.
The distinction is important because the American strain of the disease leads to symptoms that resemble what happens to the 100,000 American cattle that succumb to "downer cow syndrome " each year. Veterinary researchers fear that the widespread practice of feeding downer cows (in the form of rendered protein feed supplements) to other cattle, sheep and hogs could already be fueling a TSE epidemic in the United States like the one that plagued Britain. In fact, in 1979, before BSE was discovered in Britain, Doi pointed out in his study of deranged pigs that "many animals have been found to be 'downers' at first observation. "
On January 3, the FDA, which is part of the USDA, finally drafted a rule that would ban the fortifying of animal feeds with "any mammalian tissue. " USDA researchers, critical of the government's foot-dragging, have been calling for such a ban for seven years. But undercutting this important step, the FDA has played a taxonomic shell game and arbitrarily removed pigs from the class "mammalia. " Consequently, if the FDA's proposed rule is adopted, animals being fattened for slaughter will stop eating cow renderings and instead eat only pig remains. Since mad cow disease in Britain was spread by feeding mad cows to healthy cows, the FDA's pigs-are-not-mammals proposal gives any porcine form of mad cow disease a point of entry into the human food chain.
On April 28, Consumers Union filed comments with the FDA on the agency's proposed regulations. The group advocates a complete ban on the use of all mammalian protein in all feed intended for food animals, as is now the case in England. "The draft rule, " says Consumers Union, "is not adequate to protect public health, because it would continue to leave the door open for a porcine TSE to contaminate pork and other meat. "
It would be nice if the USDA were as concerned about protecting public health as it is about the financial health of the $ 30 billion- a-year pork industry and the $ 60 billion-a-year beef industry. Ditto for the Wall Street Journal, where editors have put on hold a story by a staff reporter on mad pig disease and the possible link between pork consumption and CJD.
ABC's World News Tonight has also sat on this information for a couple of weeks. On May 12, the network did air a story that examined the fact that CJD was being misdiagnosed as Alzheimer's. But the network failed to note that CJD is the human form of mad cow disease. The network also neglected to mention the possible connection to pork or the fact that the CJD patient featured in its story, Marie Farris, had been employed at a packing plant where she handled slaughtered pigs.
December 2 1997 Times BY RUSSELL JENKINS AND MICHAEL HORNSBYFARMERS' leaders yesterday demanded an urgent meeting with the Prime Minister as militants threatened to mount further quayside blockades of cheap Irish beef imports.
Livestock farmers say that a demonstration at the dockside at Holyhead, Anglesey, in which 40 tonnes of beefburgers were tipped into the sea, was just a "warm-up". Yesterday, as hundreds of packets of beefburgers lapped against the quayside, port authorities stepped up security in the face of an expected resumption of hostilities.
There are fears that a militant hardcore of farmers, who admire the direct action of their French counterparts, could resort to more aggressive tactics. They claim they are being robbed of their livelihoods by cheap imports and government inaction.
Bob Parry, president of the Farmers' Union of Wales, wants to meet Tony Blair to tell him that farming is facing its greatest crisis since the Second World War. He said that the union could not condone action such as throwing beefburgers into the sea. But he added: "Last night's events show that farmers have had enough and are prepared to take the law into their own hands. The situation facing British agriculture is dire."
The farmers' anger was brought to a head at the Gaerwen Smithfield market last Friday when prices for dairy cattle fell sharply to as little as 50p/kg - lower than in the early 1980s and less than the low point of the BSE crisis. Farmers were heading home from market with unsold livestock.
A convoy of farm vehicles carrying about 400 farmers converged on Holyhead after a rowdy meeting at Gaerwen. They were angry at the Government's failure to take up the offer of European Commission cash to compensate farmers for the damaging effects of successive revaluations of the green pound.
Farmers seized on statistics released by the Ministry of Agriculture showing that total income from farming has fallen by 37 per cent in real terms this year. The National Farmers' Union claimed that the true decline was closer to 47 per cent.
Jack Cunningham, the Agriculture Minister, condemned the farmers' action and told them that it would not do their cause any good. He said on BBC Radio 4:
"The idea that there is a cheque sitting in Brussels that I can bring back to British farmers cost-free is simply not the case. For every £100 that comes from Brussels, £71 has to be funded by the British taxpayer."
Dr. Michael Friedman, acting FDA commissioner, said that irradiation will become a useful tool in combating food-borne illness, but that ultimate responsibility still will rest with the food handler and preparer.
"We think it is safe and we think it is appropriate," Friedman said of the procedure. "But the consumer should not believe that he or she does not have to use good cooking and handling techniques."Some anti-nuclear activists have protested irradiation as unsafe, but Friedman said FDA scientists determined that the process does not change the fundamental properties of meat and does not make it radioactive in any way.
"There is no contact with a radioactive substance. There is nothing left on the meat," Friedman said.The FDA acted on a 3-year-old petition from Isomedix Inc., a New Jersey company with long experience in medical sterilization that wants to offer meat processors irradiation with cobalt-60 gamma rays. There are many other ways to safely irradiate meat and other companies in the market.
Such techniques would enable meat packers to kill bacteria at the end of the production line, after it is already sealed in packages and cannot be contaminated further. This is particularly important in ground beef, where bacteria can easily get beneath the surface during grinding.
Though irradiation has been available for years for poultry, pork, spices and some fresh produce, interest in the process for beef intensified after this summer's recall of 25 million pounds of Hudson Food Co. hamburger tainted with E. coli.
The meat industry lobbied vigorously for irradiation as an alternative to Clinton administration proposals for greater government authority to recall contaminated products and punish violators.
"I think there is a greater degree of interest," said Patrick Boyle, president of the American Meat Institute, a meatpacking industry organization.In this year's FDA spending bill, Congress ordered the agency to act within 60 days on the Isomedix petition. The bill also changed labeling requirements for all foods treated with irradiation so that the words need be no larger than those for the ingredients. The three years it took to act on the petition were necessary, Friedman said.
"There were some very complex scientific issues that had to be dealt with," he said. But, he added: "We believe the safety of food is so important that we will be focusing our efforts in a more effective way in the future."The FDA's action Tuesday approves safe irradiation dosage levels for various forms of meat, such as frozen, fresh and so on. It is now up to the Agriculture Department to issue regulations for processing plants that conform to those levels. Once that is done, Boyle said meat plants would have to figure out how to use irradiation, whether they can afford it and whether there is a consumer demand. It is uncertain how adaptable the process would be for hamburger that is ground in the grocery store. Most likely, consumers would see products marketed in the future that would offer them the choice of purchasing irradiated meat.
"I think it's going to take a little time for industry and consumers to move toward the adoption of irradiation as a purchasing option," he said.One reason irradiation is not widely used on other products is consumer wariness of the process and lack of education about it, said Brian Folkerts, vice president for governmental affairs at the National Food Processors Association. "We need to stop giving consumers the impression that the label is a warning when it has been found safe," Folkerts said.
2 Dec 97 webmasterLobbying of the FDA has focused on the size and distinctness of the warning label, the nuclear industry wanting smaller and abandonment of the familiar yellow and red warning triangle. The action by the FDA has converted hazardous waste expensive to dispose of into a valued industrial commodity. Noted irradiation advocate Richard Rhodes placed a prominent article in favor of radiation in the Washington Post; this same Rhodes placed a story about prion viruses in the New Yorker just ahead of the Nobel ceremony in Stockholm.
Meat irradiation would not be likely to affect any TSE titre at the dosages considered, though let's wait and see how the advertising is pitched. The FDA is being scientifically dishonest here to say the least -- the issue is how are lethal chemical changes induced in E.coli (which though no Micrococcus radiodurans still has awesome chromosomal repair machinery) deep within some meat crevice without also inducing very substantial chemical changes in the meat itself? The danger is that sanitation will slip in the rendering and slaughter industry as they gradually come to rely on a final blast of radiation to make the product wholesome. Better to hose off the cow before taking it to market.