Prion Disease: US Declares Scrapie Emergency
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BSE in sheep: huge cull planned
'Declaration of Emergency because of Scrapie in the United States'
USDA to slash meat inspectors
Scrapie eradication: the speculation continues
"We need to get prions out of the food chain."
EU blood donors face travel check; albumen use pushed
France announces mad cow testing program
French cattle herds destroyed after mad cow finds
EU plans tougher controls on animal feed
UK Insurers to DNA test for genetic illnesses
Bovine brain and pituitaries: how wholesome are they?

Declaration Of Emergency because of Scrapie in the United States

March 17, 2000 Federal Register (Volume 65, Number 53)[Notices] [Page 14521]
Declaration of Emergency Because of Scrapie in the United States

Scrapie, a degenerative and eventually fatal disease affecting the central nervous systems of sheep and goats, is present in the United States. Scrapie is a complicated disease because it often has an extremely long incubation period without clinical signs of disease.

Currently, scrapie-free countries have an enormous competitive advantage over U.S. sheep producers, who are unable to certify that their flocks originated from a scrapie-free country or region. Because importing countries are demanding that imported sheep come from scrapie-free regions and sheep producers in the United States are unable to make this certification, U.S. producers are finding themselves locked out of the international market, a situation that is taking a serious financial toll on the U.S. sheep industry.

We estimate that scrapie costs the U.S. sheep industry $20 million per year in direct losses, and millions of dollars more in lost potential markets and flock productivity. Therefore, the Animal and Plant Health Inspection Service (APHIS) has determined it is necessary to accelerate the eradication of scrapie from the United States, an accomplishment that would allow the U.S. sheep industry to once again become competitive in the global market. We estimate this plan will cost a total of $100 million over 7 years.

However, APHIS resources are insufficient to carry out this accelerated scrapie eradication program, which requires $10 million for FY 2000. This $10 million includes approximately $3.6 million for diagnostic support; approximately $2.6 million for animal identification and regulatory enforcement; $1.2 million to indemnify owners of animals that are found through surveillance to be high risk, suspect, or test positive (although no regulations currently exist to provide for the payment of indemnity for sheep and goats, APHIS expects to have such regulations in effect in 2000); $625,000 for the purchase of animals for diagnostic purposes; $692,000 for activities such as necropsy, disposal of animal carcasses, coordination, and training; $1 million for slaughter surveillance activities; and $250,000 to update the generic database for scrapie (making it possible for field and laboratory personnel to enter test and other data efficiently and to generate required reports) and for data analysis at APHIS' Center for Epidemiology and Animal Health.

Therefore, in accordance with the provisions of the Act of September 25, 1981, 95 Stat. 953 (7 U.S.C. 147b), I declare that there is an emergency that threatens the sheep and goat industry of this country, and I authorize the transfer and use of such funds as may be necessary from appropriations or other funds available to the United States Department of Agriculture to conduct a program to accelerate the eradication of scrapie from the United States.

EFFECTIVE DATE: This declaration of emergency shall become effective February 1, 2000.
Dan Glickman, Secretary of Agriculture.
[FR Doc. 00-6638 Filed 3-16-00; 8:45 am]

Opinion (webmaster): Scrapie eradication has been attempted many times in the past by various countries to little effect. The technology does not exist today to eradicate it. First, although pre-clinical sheep can now be detected through the third eyelid test, by the time this is possible, these animals may have already spread the disease to others through saliva, feces, placenta, insect transmission, shed skin and other proposed mechanisms. Second, while some sheep prion alleles give high susceptibility to scrapie, it is premature to attempt to breed out bad prion genes because this may only drive the disease underground. Knockouts of the prion gene may lead to paralysis as it does in mice.

It seems very likely that pre-clinical sheep are regularly introduced into the human food supply even today. The risk of this is unknown. Some percentage of sporadic CJD may be due to scrapie. There is published evidence from in vitro conversion experiments that some strains of scrapie is just as efficient as BSE in converting human prion to a rogue conformation. There is a further worry [see below] that BSE has crossed over into sheep and could affect consumers in this way.

Following the money, one sees that very little if any goes to basic research. One wonders how much a pound of lamb really would cost at the supermarket if these huge govenment expenditures were factored into the price. Dr. Richard Lacey, as well as the National Cattlemen's Beef Association, have even proposed getting rid of sheep altogether as a better solution. After a decade, they could be reintroduced from safer countries such as New Zealand. This would probably be more cost effective and more likely to actually result in eradication.

On the hunt for a Wolf in Sheep's Clothing

Science Volume 287, Number 5460 Issue of 17 Mar 2000, pp. 1906 - 1908 Michael Balter
The revelation that scrapie probably spawned "mad cow disease" and its human variant has renewed a campaign to eradicate scrapie before it or the mad cow pathogen--perhaps masquerading as scrapie--threatens people

As Welsh farmer David Jones opens a creaky iron gate, his black sheepdog, Zack, bolts up a hill toward a dozen ewes grazing near the top. The sheep take flight, disappearing over the crest with Zack in hot pursuit. They reappear a few seconds later, running down the hill with Zack nipping at their hindlegs. "Don't bite them, Zack!" yells Jones, eager to make a good impression on his visitors.

As the sheep cower next to the gate, Zack glaring at any woolly thing that dares to move, Jones leaps into the middle of the flock and collars a ewe with his blue shepherd's staff. "See these brown patches on top of her head?" he asks, pointing to where the animal has lost some wool. "That's the first sign of scrapie."

If Jones's diagnosis is confirmed, it would be the third case of this fatal neurodegenerative disease among his 700 ewes during the past year. Scrapie, which is the ovine counterpart to bovine spongiform encephalopathy (BSE), or "mad cow disease," is a scourge that British sheep farmers--as well as their nation's health and agriculture officials--are treating with new respect.

Like BSE in cattle and Creutzfeldt-Jakob disease (CJD) in humans, scrapie is linked to aberrant proteins called prions, which many researchers believe act alone to infect and destroy nervous tissues. BSE has apparently jumped the species barrier and caused more than 50 deaths in the United Kingdom and France from a new form of CJD in humans.

Although there is no evidence that scrapie, which has afflicted British flocks for more than 250 years, can also infect people, the devastating experience with BSE provides little room for comfort: It demonstrated that animal prions can harm humans, which means that scrapie should not be disregarded as a potential health threat.

Even more worrisome, prion researchers have been able to infect sheep with BSE; the resulting disease, which causes the animals to tremble and stagger and eventually die from a progressive loss of brain cells, closely resembles scrapie. This has raised serious concerns among public health officials that if a BSE epidemic did break out among sheep, it might not be recognized immediately.

For these reasons, in 1996 the Spongiform Encephalopathy Advisory Committee (SEAC), an expert panel that advises the British government on transmissible prion diseases, recommended beefing up the low-profile scrapie research then under way, with the ultimate aim of eradicating the disease. A major scrapie initiative is now in progress at the Ministry of Agriculture, Fisheries, and Food's (MAFF's) Veterinary Laboratories Agency in Weybridge.

And for the past 2 years, scientists at the Compton headquarters of the U.K.'s Institute for Animal Health (IAH) have been harnessing the tools of epidemiology, genetics, and mathematical modeling to lay the scientific groundwork for the task. Much of their initial work has meant studying scrapie at its source. IAH scientists attend sheep shows and slog through muck on sheep farms like the one Jones runs in Trecastle, looking for clues to how this poorly understood disease spreads and how it might someday be brought to heel.

Although the research is in its infancy, the Compton team's preliminary findings are providing new clues to risk factors for scrapie infection. They have also bolstered previous studies showing that some sheep are endowed with genes that help them resist a scrapie infection, while others have genes that make them highly susceptible--a finding that might allow farmers to breed in resistant genotypes and breed out susceptible ones. The scientists are also on the lookout for BSE in sheep masquerading as scrapie.

As they gather knowledge about this ancient killer, researchers are building bridges to farmers, whose cooperation will be essential to any eradication effort. Although the fractious dispute over BSE and British beef often sowed distrust between the two communities, the fight against scrapie is uniting scientists and farmers in a common cause. After all, says IAH mathematical biologist Angela McLean, who leads the Compton project, the primary goal is one that everyone agrees on: "We need to get prions out of the food chain."

A resilient enemy: Despite scrapie's long history in the British Isles, surprisingly little is known about how it is transmitted from sheep to sheep and flock to flock, or what puts individual animals at risk, aside from evidence that ewes may infect lambs during pregnancy or just after birth.

"You can't just dive in and try to eradicate scrapie instantly," says Richard Cawthorne, MAFF's deputy chief veterinary officer. "We need to understand a lot more about the disease's basic mechanisms." Indeed, the most obvious strategy, killing off infected sheep in hopes of purging the pathogen, has failed.

For example, soon after the first scrapie cases appeared in the United States in 1947--apparently a result of imports of British sheep--the U.S. Department of Agriculture (USDA) declared a state of emergency and ordered the slaughter of whole flocks each time a single sheep fell ill. Yet this scorched-earth policy, which continued until the early 1980s, hardly made a dent in the U.S. scrapie toll.

The mass slaughters discouraged farmers from reporting the disease and "drove it underground," says veterinarian Linda Detwiler, coordinator of the USDA Animal and Plant Health Inspection Service's working group on transmissible prion diseases. The agency now relies on voluntary programs to identify and isolate infected flocks, and--as is current practice in the United Kingdom--only infected animals are killed.

The average number of [reported] cases has dropped by about half in the past decade, but several dozen new cases are still reported each year among the 11.5 million sheep in the United States. "We have tried every scrapie control and eradication program known to mankind, but they did not work," says Detwiler.

On the hoof: Since notification became mandatory in 1993, yearly numbers of reported cases have edged up. Although scrapie flourished, the disease intrigued few researchers--until it became clear in the mid-1990s that people had become infected with BSE, apparently from eating meat or other products >from infected cattle.

"If the BSE epidemic had not occurred, I suspect scrapie would still be a scientific backwater," says molecular biologist Chris Bostock, IAH's director.

But there was more at play than merely a missed research opportunity. The persistence of scrapie may well be responsible for the rise of BSE in cattle. Many researchers believe that the BSE epidemic began when scrapie-infected meat, bones, and nervous tissue from sheep were added to cattle feed during the decade leading up to July 1988, when the practice was banned.

Support for this idea emerged last year, when IAH researchers reported in the January 1999 issue of the Journal of General Virology that one "strain" of scrapie prion isolated during the 1970s--called CH1641--is strikingly similar [but not identical according to the paper itself, leaving this whole concept of a sheep origin in doubt. -- webmaster] to the BSE prion.

Although the concept that protein-based prions come in strains like viruses or bacteria is controversial, researchers agree that certain characteristics--such as how many sugar molecules are bound to the prion's amino acids--generally remain stable even when the prions infect other species. The key evidence that humans have been infected with BSE, for example, came from the fact that prions isolated from the brains of variant CJD (vCJD) victims are nearly identical to BSE prions.

Yet even if the BSE prions that jumped from cattle to humans originated in sheep, this does not necessarily mean that scrapie prions can infect humans. The most persuasive evidence that scrapie prions can't--for now--harm people is that decades of exposure to scrapie-infected sheep has not caused a single known infection in humans. [This says very little since scientists are unable to determine the cause of the vast majority of CJD cases -- webmaster].

Indirect support also comes from experiments demonstrating species barriers against prion infection: Mice fed BSE prions become infected, while hamsters do not. "There appears to be an absolute block between cows and hamsters," says Bostock. However, if brain extracts from BSE-infected mice are fed to hamsters, they do become infected.

Some researchers suggest that a similar block may exist between sheep and humans, but not between sheep and cattle: Scrapie prions must be modified somehow in cattle before they are capable of infecting people. Because cattle are no longer being fed ground sheep, the presumed BSE link between scrapie and vCJD should now be severed. Therefore, says Byron Caughey, a virologist at the U.S. National Institutes of Health's Rocky Mountain Laboratories in Hamilton, Montana, "there are no grounds for hysteria."

Lurking in the madding crowd? The scarcer BSE is in the sheep population, the harder it will be to flush it out. The bars indicate how many samples are required to be 95% certain of detecting at least one BSE case hidden among 5000 suspected scrapie cases.

A far more serious concern is that sheep might harbor BSE prions. Until the practice was banned in 1988, the remains of both cows and sheep often wound up in ruminant feed. And sheep certainly are capable of being infected. In 1993, researchers at the IAH's branch in Edinburgh, Scotland, first reported that BSE could be transmitted to sheep via infected cattle brain extracts--results that have been confirmed by many other groups.

There's no evidence yet that BSE is lurking in sheep, but if these prions do find their way into a new animal reservoir, they will be easy to miss. Although the number of reported cases of scrapie is relatively low--averaging about 500 per year among the U.K.'s 40 million sheep--an anonymous survey of more than 11,000 sheep farmers, carried out by the Compton and MAFF teams, indicates that the actual occurrence may be eight times the official numbers.

Underreporting of scrapie cases, combined with the ability of BSE symptoms to masquerade as scrapie, makes it unlikely that BSE in sheep would be detected unless it amounted to more than 5% of scrapie cases, according to SEAC calculations. For these reasons, says McLean, "deep down" the Compton project "is about BSE in sheep." Eradicating scrapie, she says, would be the best insurance against an undetected BSE epidemic in these animals.

Ewegenics for rams? The key to defeating scrapie--and defusing the BSE threat--may lie in figuring out which sheep should be allowed to reproduce. Over the past decade, geneticist Nora Hunter of the IAH's Neuropathogenesis Unit in Edinburgh and other researchers have established that sheep vary in their susceptibility to scrapie depending on variations--called polymorphisms--in the nucleotide sequence of the gene coding for PrP, the normal protein that apparently causes disease if it converts to the abnormal, or prion, form. Some polymorphisms make sheep nearly impervious to scrapie, while others make them highly susceptible. Testing for these polymorphisms, and only allowing sheep with beneficial ones to breed, might help eradicate scrapie

Both supporting and complicating this picture are findings from a pilot study last year of four British flocks--two of which were scrapie-infected and two scrapie-free--led by IAH field epidemiologist Matthew Baylis. The surprising news is that the scrapie-free flocks had only slightly lower proportions of susceptible genotypes than did the scrapie-infected flocks. But the number of older animals with susceptible genotypes in the infected flocks was much lower than expected.

This implied, the team concluded, that the real scrapie toll in these flocks had been much higher than thought--either due to underreporting or because farmers were not recognizing scrapie. If so, the two infected flocks must have started out with significantly higher proportions of younger susceptible sheep, which then succumbed to scrapie.

These findings support the idea that selective breeding should at least cut the toll from scrapie, although researchers caution that there are many uncertainties. "Breeding for resistance might remove clinical signs of scrapie," says Hunter, "but there is a slight worry that the resistant animals might still be carriers of infection."

And McLean says that although breeding programs might eradicate today's strains of scrapie, it's unknown whether the resistant sheep could withstand altered or "mutant" prions that might arise later. Still, says veterinary epidemiologist Linda Hoinville, who leads the scrapie epidemiology group at the Veterinary Laboratories Agency, "this is the most promising strategy at the moment."

McLean and her team have begun asking farmers like David Jones--who has spray-painted his sheep to mark those with susceptible or resistant genotypes--to breed lambs only from scrapie-resistant ewes and rams. And Hoinville's team believes it has evidence that this effort may pay off: In preliminary, unpublished work on one flock in which scrapie was rampant (carrying a whopping 6% infection rate at the outset), the Weybridge group found that only allowing resistant rams to breed reduced scrapie incidence in later generations to negligible levels [subject to the threshold of detectibility; as these sheep age conclusion could change drastically -- webmaster]. If these results are confirmed, it could mean that researchers and farmers may soon be able to write the final chapter on this deadly disease's 250-year history and the human health concerns that have become an unsettling subtext to the story.

Writing Scrapie's Coda, Codon by Codon?

Science Volume 287, Number 5460 Issue of 17 Mar 2000, p 1907  Michael Balter
Over the past decade, geneticists have begun to unravel why some sheep are more vulnerable than others to scrapie. They have found that different variations, or polymorphisms, in the gene coding for PrP--a cellular protein that many scientists believe becomes infectious when it converts to an abnormal form called a prion--seem to confer varying degrees of susceptibility. This correlation raises the possibility that genetically susceptible sheep could be bred out of the population, leaving only scrapie-resistant animals (see main text).

Studies of sheep experimentally infected with scrapie have shown that three codons, or positions, in the PrP gene--codons 136, 154, and 171--are critical in determining whether the animal comes down with the disease. Each codon gets translated into one of the 256 amino acids of the sheep PrP protein. Individuals most vulnerable to scrapie have the amino acids valine, arginine, and glutamine at the respective positions dictated by the three codons. Using the single-letter code for amino acids, this polymorphism is referred to as VRQ. At the other extreme, sheep with the polymorphism alanine-arginine-arginine (ARR) are the most resistant.

Indeed, out of hundreds of scrapie-infected sheep tested worldwide, only one, in Japan, has turned out to be ARR. Three other polymorphisms (shown at below) apparently lead to intermediate levels of vulnerability to the disease.

Muddling this neat picture, however, are some bizarre differences in the effect of polymorphisms in different sheep breeds. For example, Suffolk sheep with the genotype ARQ are susceptible to scrapie, whereas ARQ Cheviot sheep are resistant.

"We really don't understand this," says Nora Hunter, a geneticist at the Institute for Animal Health's Neuropathogenesis Unit in Edinburgh. Hunter and her colleagues are currently testing several hypotheses, including the possibility that the two breeds are being infected by different prion strains, or that Suffolk sheep may produce higher levels of PrP and thus have more protein available for conversion to the prion form.

More clear, however, is why PrP polymorphisms correlate with scrapie susceptibility in the first place. Findings reported in the 17 July 1997 issue of Nature and in the 13 May 1997 Proceedings of the National Academy of Sciences show that the VRQ version of normal PrP protein is easily converted into the prion form when mixed with other prions in the test tube. (Most researchers studying prion diseases believe this mechanism is responsible for the creation of new prions in infected animals.)

The ARR polymorphism, on the other hand, strongly resists this conversion, while polymorphisms corresponding to intermediate scrapie susceptibility fall in between. This biochemical confirmation of the importance of PrP polymorphisms has bolstered the view that breeding VRQ and other susceptible genotypes out of the sheep population might be the best course toward eradication [Again, this may simply push scrapie below the limits of detectibility or favor another strain of scrapie with possibly worse properties -- webmaster]. Says Hunter: "At the moment, there really isn't any good alternative."

French cattle herds destroyed after mad cow finds

18 Mar00  Reuters World Report 
Two more herds of cattle totalling 174 animals were destroyed in France after the discovery of two fresh cases of mad cow disease, local officials said on Monday.

The prefecture in the Deux-Sevres region of west central France said mad cow disease, or bovine spongiform encephalopathy (BSE), had been detected in a beef cow born in 1994. It said it was the region's first case.

The prefecture in the Manche region of northwest France reported a new case involving a dairy cow born after 1990. The two animals and their respective herds were destroyed.

France's farm ministry could not immediately confirm the new cases but did confirm a BSE case reported on Friday, which brought the total number reported in France this year to nine. BSE has been linked to a form of the human Creutzfeldt-Jakob Disease, a fatal disorder for which there is no known cure.

France is locked in a legal battle with the European Commission over its refusal to lift a ban on British beef because of fears it is not entirely safe from BSE. It said on Saturday it planned to set up a nationwide testing programme to gather data on the spread of the disease.

Herd destroyed after BSE case confirmed in France

Wed, Mar 22, 2000  Reuters World Report
France's farm ministry on Wednesday said a herd of 72 cattle had been destroyed after the confirmation of the latest case of mad cow disease (BSE). The ministry said a dairy cow born in 1994 was found to be suffering from the deadly brain-wasting disease in the first case of BSE to appear in the central Rhone district and the eleventh case of the illness this year in France.

A farming group representing the herd's owner had alerted the ministry but was waiting for official confirmation to destroy the animals. BSE has been linked to the new-variant Creutzfeld-Jakob Disease, a similar illness in humans for which there is no known cure.

BSE Belgium: new case born after bam

ProMED  14 Mar 2000
A new case of mad cow disease, the second this year and the 12th since the disease first appeared, has been detected at a small farm in northwest Belgium. The five year-old cow, whose symptoms of BSE disease were confirmed by three laboratory tests.

It was killed and immediately incinerated. The remaining 300 over cows from the farm will also be killed and incinerated and the brains of all those older than one year will be analysed. An inquiry is currently underway and its results will be important, because the disease-ridden cow was born on July 21 1995, after the government banned the feeding of cows with animal by-products.

EU plans tougher controls on animal feed

Wed, Mar 22, 2000  Reuters World Report
The European Commission on Wednesday proposed tighter controls over animal feed to strengthen consumer protection following Belgium's dioxin-in-food crisis last year.

"In an emergency situation time is the critical factor. Immediate intervention to contain the risk and stop it spreading is vital," EU Consumer Safety Commissioner David Byrne said.

The planned legislation closes loopholes in existing law and extends powers that already apply in the food sector to animal feed, giving the Commission powers to intervene quickly if a serious risk to public health is discovered. Last year's dioxin alert was traced to animal feed in Belgium contaminated with motor oil. The oil entered the food chain after being mixed with vegetable oils used in feed manufacture.

The Commission said the crisis had highlighted deficiencies in the current system "in particular due to the poor management and co-ordination between different (national) authorities, and to the late transmission of information." Consumer concern over what is fed to animals was heightened by reports of sewage sludge being fed to French farm animals. The French authorities have said the practice has now stopped.

Under the proposed legislation, which is subject to joint approval from the European Parliament and EU farm ministers, the Commission will be able to intervene and suspend or impose conditions on the export of suspect feed. It also requires animal feed producers, importers and operators to inform the authorities if they have any knowledge of potential contamination, and establishes a programme of inspections and annual reports. The measures are part of the wide-ranging policy paper on food safety unveiled by Byrne earlier this year.

Sheep flocks to be tagged and tested for BSE

March 26 2000 Sunday London Times Jonathan Leake, Science Editor
Government scientists have become so concerned about the threat of BSE appearing in sheep that they are setting up a national programme to test thousands of flocks. It coincides with a decision by ministers to order civil servants to draw up a scheme to tag all the nation's 40 million sheep.

The moves follow new laboratory evidence suggesting that sheep diagnosed with scrapie, a related disease, could actually have been suffering from BSE. There is no hard evidence of any sheep having contracted BSE, but scientists have warned that the risk of such a diagnosis is getting higher. They have also asked civil servants to draw up provisional plans for a cull. This would, however, be enacted only if BSE were confirmed in sheep.

Professor Peter Smith, acting head of the Spongiform Encepalopathy Committee (SEAC), the independent scientific group that advises the government on BSE, CJD, scrapie and other so-called prion diseases, said the consequences of finding BSE in sheep would be "very profound". He added: "We could be very close to getting the bad news. If we do get BSE in sheep, then the implications will perhaps be more serious than the discovery of BSE in cattle."

In cattle, BSE infects only certain parts of the body. In sheep, it affects the whole animal, meaning that more infected material may enter the human food chain. Scientists have known for some years that sheep can catch BSE from eating infected material. The difficulty in spotting the disease has been that it has identical symptoms to scrapie, which is believed to be harmless to humans.

New research, carried out at the Institute for Animal Health (IAH), has now suggested some cases diagnosed as scrapie may, in fact, be BSE. The findings have been backed by further research showing that animals immune or resistant to scrapie are indeed vulnerable to BSE.

Professor Chris Bostock, director of the IAH, who is also a member of SEAC, said: "We have found sheep that should be resistant to scrapie that have been diagnosed with it. This raises a suspicion they could have had BSE. Samples taken from 200 infected animals are being tested to determine the strain of prion. We have had 30 results so far, none of which showed BSE. The results for the remainder are still awaited."

Despite the fact that infective feed is now banned, if BSE had once entered the sheep population, it could still persist. In cattle BSE passes from cows to calves and in sheep scrapie is renowned for its ability to transmit between generations and across flocks.

It was confirmed last week that the agriculture, fisheries and food ministry (Maff) is putting on trial a test developed by the American government that can detect prions - the agents believed responsible for both BSE and scrapie - in sheep from two weeks old. It has also established a dedicated laboratory in Weybridge, which is preparing the protocol for a national programme to screen every flock in which scrapie or any other prion disease is diagnosed. Britain has 78,000 sheep farmers.

Maff officials say the scheme for tagging sheep will be announced within weeks and be operating by October. A spokesman for the National Farmers Union said the impact on sheep farmers of BSE would be "very serious", but stressed that Britain already had regulations that made its lamb among the safest in the world. [Let's turn this around and ask what nation has regulations that made its lamb among the most dangerous in the world. Surely Britain. Lamb from New Zealand or Australia would be far safer, with neither scrapie or BSE reported in the flocks though high-risk genotypes have been demonstrated. -- webmaster]

The SEAC has advised Maff it needs to make contingency plans for a possible cull. Ian McConnell, professor of veterinary sciences at Cambridge University and a member of SEAC, said: "We have told Maff it would be wise to have an operational plan in case BSE is found. Having been caught short over culling cattle, they do not want to be caught again."

The ministry would not discuss plans for a cull, although it confirmed it wanted to eradicate all prion disease in sheep. "For practical and commercial reasons, it is not possible to discuss the government's response if BSE is found in sheep, but clearly it would take account of the risk to public health," a spokesman said.

Comment (webmaster): This very disturbing story rather goes back and forth -- do they have mad sheep yet or not? The situation has become chaotic -- look at the contradictory quotes given out by the same experts in the 17 Mar 00 Science article.

It sounds like some ARR genotype sheep were unexpectedly found with scrapie and that this genotype has little resistance to experimental BSE. This could simply mean the ARR genotype was never all that resistant to scrapie to begin with, only to a few common strains tested.

The story also muddies the water in regards to the scrapie strain found that was put forward as a possible source of BSE -- was it the original source of BSE or simply BSE secondarily transmitted to sheep? How are these issues to be differentiated experimentally? Do scrapie strains differ in their risk factor to humans?

It is important to keep in mind that sheep were first exposed to large amounts of BSE 15 years ago. This means that any BSE found in sheep today has been passaged through many generations and genotypes of sheep. The dogma that every strain of TSE is faithfully propagated is being increasingly recognized as an oversimplification. This means that there would be dozens of BSE strains in sheep by now, with unpredicable properties.

It is hard to get too excited over "in cattle, BSE infects only certain parts of the body. In sheep, it affects the whole animal" because this was complete rubbish to begin with: MAFF simply wanted to keep selling the contaminated meat while discarding the low value spinal cord and brain. But they painted themselves into a corner by saying earlier that sheep were totally contaminated.

It looks as though further half-measures are planned, pinning hopes on testing and tagging their way out of this mess. However, it is premature to contemplate eradication -- the scientific foundation for it is just not there.

The thinking must be that 'a few more years of eating BSE lamb and cows can't do too much further damage as everyone has been massively dosed already.' That is not quite right because new strains could be far more virulent than the one leading to nvCJD and additional millions at home and abroad continue to receive exposure.

At some point, it all comes round to Prof. Lacey's idea of depopulating the British Isles of livestock for a decade and going all-out on research. Rather than pumping further tens of billions into the sheep, pig, and cow industries, perhaps a few hundred million could go into research that would lead to a therapy. Better to get an early start on that, the postdocs and ministers are just as exposed as the nvCJD victims class.

France announces mad cow testing program

Sun 19 Mar 00 
France said on Saturday it planned to set up a nationwide testing program to gather data on the spread of mad cow disease. France, which has come under fire from the European Union over its safety record on the disease, said tests would begin in April or May and be conducted on 40,000 cows that had died or were killed after they showed signs of the disease. [This is an impressive survey especially in conjunction with the pithing and sausage casing bans. France is one of the few countries taking adequate measures. -- webmaster.]

``The French program, particularly demanding, will go beyond the action plan now being discussed by the European Union,'' the Health and Farm ministries said in a joint statement. They said they would seek tenders from across Europe for a suitable large-scale testing method for BSE -- bovine spongiform encephalopathy -- also known as mad cow disease. The Farm Ministry confirmed in February an eighth case this year of the disease and a farming group reported another case on Friday.

An EU report in February said traces of meat and bone meal continued to be found in French animal feed despite a ban on cows being fed on animal proteins imposed in July 1996. Scientists have blamed BSE for human cases of a new variant of Creutzfeldt-Jakob Disease or nvCJD, a deadly, brain-wasting disorder for which there is no known cure.

The announcement of the tests followed moves by France's food safety agency to ban a slaughterhouse method involving piercing cow skulls with a metal bolt that experts fear may increase the risk of the disease spreading to humans. The EU's executive Commission took France to court in January over its refusal to lift a ban on British beef although EU states agreed in August to end an embargo imposed in 1996.

Comment (Francois Moutou, Unite d'Epidemiologie, AFSSA Alfort ProMED): " It is correct the plan is to test 40 000 cows, but it is not right to say that these will be 40 000 animals that had died or are killed after they showed signs of the disease.

The idea is to look with the test for BSE on fallen stock, emergency slaughtered animals, or any animal found dead on the farm without sign of BSE. These animals represent a higher risk sub-population where the test could be usefully "tested". The clinical surveillance (passive surveillance as it is called) of BSE, starting with the recognition of suspect clinical signs on live animals will continue, as started in February 1991 in this country."

USDA Seeks 2 Percent Reduction in Processing Plant Inspection Force

3/22/2000 By: Philip Brasher, AP Farm Writer
Despite recent scares over contaminated hot dogs and other meat products, the government wants to cut back inspectors visits to processing plants, saving on overtime pay and eliminating 150 jobs, or 2 percent of its inspection force.

Inspections would be reduced only at plants, such as soup canneries, where the risk of contamination is small and where doing so would allow money to be shifted to other meat and egg safety programs, Agriculture Department officials said Tuesday

``By having our resources allocated based on risk, the public is well served," said Margaret Glavin, associate administrator of USDA's Food Safety and Inspection Service. Critics said the move could imperil public health.

Inspectors currently are required to visit each processing plant once a shift, including overtime runs. The department wants to switch to daily, random checks, starting in a year. Inspections would concentrate on plants that have a history of problems or are considered to be riskier because of what they process, such as ground beef, the most common source of the deadly E. coli O157:H7.

In addition to cutting USDA's 7,500-employee inspection force by 150 jobs, the change would save the industry, which must cover the inspectors overtime pay, an estimated $9 million a year. The department would save $4 million in personnel costs. Slaughtering plants, which must have USDA inspectors on site at all times, would not be affected.

Critics, including the inspectors union, say the change will benefit the industry while possibly endangering public health. ``This idea comes out of left field with no data to support it," said Caroline Smith DeWaal of the Center for Science in the Public Interest. ``Its just saving money for the industry."

Several major incidences of food poisoning have been traced to processed meat products in recent years, including a 1998 outbreak at a Michigan hot dog plant that killed 15 people and sickened at least 100 others. However, overall foodborne illnesses have been dropping, and health officials say the decline is due in part to a new science-based inspection system that is now being used in processing plants nationwide. It requires plants to identify and control for possible contamination rather than relying on federal inspectors to catch problems.

USDA officials say that means their inspectors can be used more effectively in an oversight role. Switching to a random inspection system for processors will make it harder for facilities to hide problems from the government, Glavin said.

``If a plant does not know when an inspector might show up we think there's some deterrent there," she said. She acknowledged the reduced inspections will save the plants money but said that was not the purpose. [Note once a day inspection is replacing once a shift, meaning only 1/3 as many inspections. -- webmast er]

The inspectors union is fighting the reduction as well as a pilot project at several slaughtering plants where inspectors have been pulled off production lines and used to oversee plant employees. Internet resources:

USDA's Food Safety and Inspection Service site:
National Joint Committee of Food Inspection Locals:

Comment ( Alvin Sewell ): "I had the opportunity to participate in consultations with FSIS officials and the National Joint Council of Food Inspectors in February. The budget draft proposal did identify 150 positions in second shift processing plants that were to be eliminated. But when challenged on the number, management admitted that the actual number was 350 by the end of fiscal 2001. That's 150 by April 2001, then another 200 by the end of fiscal 2001 in September. These cuts are in addition to the cuts that are already planned under implementation of HACCP Based Slaughter Inspection, which is expected to be in full swing by July of 2001. It would seem that USDA continues to have problems giving the public the whole story."

Government Study: E. Coli Levels in Cattle Highest in Summer

3/21/2000 By: M2 Communications WASHINGTON, D.C.
Up to 28 percent of cattle entering processing plants in late summer may carry with them strains of the E. coli bacterium that cause food poisoning in humans, according to an U.S. Agricultural Research Service study.

Improved laboratory methods allowed the scientists to ferret out the microbe and detect this prevalence level, which is higher than previously reported. But the research also showed that intervening measures at processing plants can reduce the incidence of E. coli 0157:H7 on beef carcasses to less than 2 percent, even in the peak contamination season of July and August. The study will be published in the Proceedings of the National Academy of Sciences on March 28 [as "Correlation of enterohemorrhagic Escherichia coli O157 prevalence in feces, hides, and carcasses of beef cattle during processing"] . ARS is the U.S. Department of Agriculture's chief scientific agency.

The study, conducted by scientists at the ARS Meat Animal Research Center (MARC), Clay Center, Neb., included examination of E. coli shed in the feces of live cattle as well as microbes on beef carcasses in commercial processing plants.

During the summer E. coli peak, 28 percent of the live cattle entering the processing plants were actively shedding E. coli 0157:H7 in their feces and 43 percent of 341 carcasses were initially contaminated with the bacterium. Eleven percent of hide surfaces were also contaminated with the bacterium.

After processing was complete, only six of 330 carcasses, or 1.8 percent, showed some level of contamination. [1.8 percent of 34,000,000 cattle slaughtered every year translates to 612,000 contaminated carcasses or about 1.2 billion quarter pound hamburgers. -- webmaster]

EU blood donors face travel check

Wednesday March 22, 2000 James Meikle The Guardian
What's wrong with our food? - special report.
Blood donors throughout the European Union are to be asked if they have visited Britain or other countries with BSE so that scientists can assess the risk of them spreading the human form of the disease, CJD.

Medical advisers to the European commission say checks should be made on all British meat exports for 16 years up to 1996 and on the dangers of transmission of other diseases through blood transfusions before deciding whether to ban donations from people who have stayed in BSE-affected countries.

EU-wide blood controls may be introduced, including the filtering of white blood cells from donations, since these are thought most likely to carry the infective agent of CJD. EU scientists accept there is no proof this will help cut the risk of transmission of CJD, despite the fact Britain already does it as a precaution. The Department of Health last night expressed scepticism over whether EU-wide bans would make blood any safer.

The latest recommendations from the commission's medical products and medical devices committee reflect the difficulty in balancing the potential risks of transmitting the incurable CJD with the consequences bans on potential donors could have on blood supplies.

There currently is a world shortage of blood. There is no proven or even probable case of CJD having been passed on through blood transfusions. There is as yet no test for identifying CJD in people's blood and no ethical code on whether people who might be identified as having it before symptoms occur should be told of the diagnosis.

Albumin industry launches global promotion

BMJ 2000;320:533 26 February Gavin Yamey
< The plasma products industry has launched a($2.2million international programme to promote albumin despite recent official advice to restrict its use to very limited indications. Albumin use has declined substantially following publication of a systematic review by the Cochrane Group, which raised serious concerns about the product's use in critically ill patients (BMJ 1998;317:235-40).

One year after publication of the review, the Committee on Safety of Medicines advised doctors to restrict its use to the treatment of hypovolaemia (BMJ 1999;318:1643). Internal industry documents passed to the BMJ outline an "albumin support" programme aimed at countering the "threats to allowable indications and the competition from alternative products."

The global albumin market was worth 900,000 pounds in 1996. Documents from the Inter-national Plasma Products Industry Association (IPPIA) and the European Association of the Plasma Products Industry (EAPPI) outline two kinds of strategy data collection and "promotional." The documents show that the associations have completed their own literature review and compiled a "Cochrane critique dossier."

They have bought a large database to "identify/provide health economics data that supports use of albumin both alone and versus substitutes." They have also established a medical advisory panel. Promotional activities will be "primarily centred around scientific conferences." The documents acknowledge the need for a marketing campaign: "Certainly in the US, IPPIA will have, in comparison with the European program, a strong marketing component."

Other recommendations include creating a website and holding workshops to develop monographs on fluid resuscitation of critically ill people. Members of the European Plasma Fractionation Association (EPFA), another organisation representing the industry, may be joining the support programme.

In a fax to its members dated 16 November 1999, Richard Walker, president of the association, wrote: "I believe this is a very good program and therefore recommend EPFA members to participate ... Participation in this program will be very cost effective with respect to our needs to support the use of albumin (the financial contribution per member would be about the same as the EPFA annual membership fee)."

Mr Walker is also the chief executive of Bio Products Laboratory, which is part of the National Blood Authority, a special NHS health authority. Asked about the possibility of a conflict of interest between his advice to EPFA members and his NHS position, he commented:

"I don't see a conflict of interest. At [Bio Products Laboratory] we are supplying products to meet patient needs. We are very clear that we will only promote products on the basis of scientific information." His company, he said, would not be joining the albumin support programme, although individual EPFA members might still do so. The agreed funding for the programme includes 1.6 million pounds from US pharmaceutical companies, including Alpha, Baxter, Bayer, and Centeon (now Aventis Behring). A clinical study in Italy is already under way, with funding of 87,500 pounds from local companies.

Insurers to DNA test for genetic illnesses

Telegraph  Monday 20 March 2000  By Marie Woolf, Political Correspondent
Opinion (webmaster): This is a very poor idea that will interfere with medicine -- it would be better to get rid of the insurance companies altogether and have the government fund health care from a fixed budget, as is done in Oregon or Canada. Familial CJD kindreds would certainly be affected. Perhaps also people who are met/met at codon 129 and at higher risk of contracting nvCJD would also be affected -- some 24 million people in the British population.

MINISTERS are to allow insurance companies to use genetic testing to assess a person's risk of inheriting serious illness. People with a genetic tendency to chronic conditions will face significantly higher premiums for medical, life and travel insurance.

In the past, ministers have considered banning genetic testing because of fears that it would create an under-class unable to obtain insurance cover. But after a consultation completed last week, the Government is to sanction a series of reliable genetic tests that insurance firms can use.

It plans to allow the companies to use single gene tests, which show whether a person with a family history of an illness such as Huntington's disease - jerky, involuntary movements accompanied by progressive dementia - is likely to develop it later in life.

Worried consumer and civil liberties groups say that sections of society with serious medical complaints may be left without cover. Anna Bradley, director of the National Consumer Council, said: "We would be very concerned about measures that could stop people getting insurance."

Dr Evan Harris, Liberal Democrat MP for Oxford West and Abingdon, said that genetic testing should be carried out for medical purposes only. "By allowing insurers to test for even single gene disorders you are going to get a genetic under-class. There will be enormous public opposition to this. People should not take these tests without adequate counselling."

The Department of Health has set up a genetics and insurance committee of experts to monitor and regulate insurers' use of genetic tests and to agree on a series of tests that can accurately predict a life-threatening illness. The committee will regulate which laboratories are allowed to carry out the tests.

Government advisers feared that unless testing was permitted, insurers would carry out the tests with no regulation. The new policy will start by September when up to seven tests will be presented to the experts for Government approval.

Insurers believe that within five years, as technology develops, genetic tests will be widespread and could cover such inherited illnesses as breast cancer, cystic fibrosis, Alzheimer's and sickle cell disease. They say that the use of tests could help people with a family history of a disease gain lower premiums because they will be able to rule out the prospect of developing the disease.

The Association of British Insurers said: "Because genetic testing is in its infancy, the number of people who could take such a test is small. But the numbers could well be more of an issue in the future, in five years or less." Under the plans, insurers will not have the power to force people to take genetic tests. But they will be able to ask those with a family history of a disease to take one. They will also have the right to ask whether people have taken a genetic test and to demand to see the results.

A spokesman for the Department of Health said: "The genetics and insurance committee has been set up to receive applications to demonstrate the validity of tests, but the industry will have to prove that such tests are reliable. If the insurance company thinks you have a genetic condition, it can load a policy." Insurance premiums are traditionally weighted according to risk. Smokers and people with a history of chronic illness pay more. A positive test for a life-threatening illness could increase premiums five-fold. About four per cent of people applying for life assurance have to pay between 50 per cent and five times more than ordinary applicants. Around one per cent are not accepted because the risks are considered too high.

Bovine brain and pituitaries: how wholesome are they?

Medline 22 Mar 00
Opinion (webmaster): We have to wonder why the FDA is looking into detecting beef in ingredients at this time. Is this about companies breaking the ruminant-to-ruminant feed ban on a massive scale, as was done with diethylstilbesterol? Perhaps they should also be testing 'nutriceuticals' in addition to feed. Some of these actually brag about containing bovine brain and bovine pituitary. Human pituitaries have responsible for a large number of growth hormone CJD cases all over the world.

A rapid method for PCR detection of bovine materials in animal feedstuffs.

Mol Cell Probes 2000 Mar;14(1):1-5
Wang RF, Myers MJ, Campbell W, Cao WW, Paine D, Cerniglia CE
Rapid identification of bovine materials in animal feedstuffs is essential for effective control of a potential source of bovine spongiform encephalopathy. We have developed a rapid method for the detection of the presence of bovine materials in animal feeds. Animal feed samples were prepared by a Chelex-100 treatment method, then subjected to polymerase chain reaction (PCR) detection. The assay can be completed in 2 h including 30 min for sample preparation, 35-65 min for PCR cycling and 30 min for gel electrophoresis. This method is not only rapid, simple and consistent, but also avoids a hazardous waste disposal issue associated with a previously described guanidine thiocyanate (GuSCN) extraction-PCR method. Typical American pep pill: "

Brain Nutrition[tm]

"The brain has a remarkable capacity for memory and activity, but it needs a constant flow of oxygen and specific nutrients to keep it functioning for an entire lifetime. The brain depends on glucose as an energy source. The nutrients in Brain Nutrition are essential to the glands and organs involved in glucose metabolism. Brain Nutrition also provides essential vitamins and minerals that must be present for brain function. 60 tablets.

Recommendations: One to three tablets daily, preferably at mealtime, as an addition to the everyday diet.

Each three tablets contain
Essential vitamins and minerals:
Vitamin C (Ascorbic Acid)       300 mg
Magnesium Aspartate     100 mg
Potassium Aspartate     100 mg
Zinc Aspartate  30 mg
Manganese (Chelate)     15 mg
Folic Acid      800 mcg
Vitamin B12 (Cyanocobalamin)    500 mcg

Other ingredients:
L-Glutamine     300 mg
L-Phenylalanine         300 mg
Brain Extract (Freeze-Dried)    300 mg
Choline (Bitartrate)        200 mg
Gamma-Aminobutyric Acid (GABA)  100 mg
Alpha-Ketoglutaric Acid         100 mg
Glycine         100 mg
L-Tyrosine      100 mg
Pituitary Extract (Freeze-Dried)        65 mg
L-Methionine    50 mg
RNA/DNA Complex         50 mg
L-Cysteine      50 mg
Pyridoxal Phosphate     15 mg
Contains no sugar, salt, yeast, wheat, corn, soy, dairy products, coloring, flavoring or preservatives. All organs and glands derived from bovine sources."

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