Prion disease: Girl, 13, youngest BSE case
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Girl, 13, could be youngest BSE
Eyewitness account of BSE Inquiry
TSEs in blood
Further French BSE, but no further UK imports
French are right in beef war, saY CJD families
US pushing livestock too hard
USDA seeks sheep slaughter over BSE
Genome of radiation-resistant bacterium probed
Another British lion dead from BSE
Blood donor ban in New Zealand

Girl, 13, could be youngest BSE

What's wrong with our food? - special report
James Meikle  Tuesday November 23, 1999   The Guardian (UK)
A girl of 13 may be the youngest person to have contracted the human form of BSE, the fatal disease linked to eating infected beef, in a case being anxiously monitored by doctors and government officials. The girl is showing possible signs of the condition. So far the youngest of 48 people to have died from variant Creutzfeldt-Jakob disease was 16.

She was under a year old when the cattle disease was first formally identified in 1986. Parts of cows that might be infective were banned from the human food chain in 1989.

Her case, if confirmed, would give a sharp new edge to questions about the infective dose of the bovine spongiform encephalopathy agent, the length of incubation in humans and the rigour of hygiene controls before March 1996, when the then Conservative government finally conceded a probable causal link between the disease in humans and eating beef in the mid to late-1980s - nearly a year after the first victim had died.

News of the girl comes at a bad time politically, given the row over beef exports to France and the proposed lifting of the ban on beef on the bone imposed by Labour two years ago. The inquiry into how the government handled the BSE crisis until 1996 has been examining whether ministers, officials and advisers underestimated the risks for years.

The health department and its unit monitoring progress of the disease refuse to discuss individual cases. They have previously pointed out that far from all suspected cases turn out to be variant CJD. But the disclosure will prompt questions as to whether the girl was infected from food she ate as a baby.

The Tories introduced food controls 10 years ago after a long argument about whether there was sufficient evidence to justify concerns that people may catch the condition from beef products. For years it was justified as purely a precautionary measure.

Mechanically recovered meat, machined off animal bones and often used in cheap beefburgers, sausages and pies, was banned only in late 1995, but scientists now believe it was particularly risky material.

The government last year introduced new filter treatments to reduce the risk of people catching the condition through blood transfusions. It recently banned reuse of equipment used in eye tests and is still considering whether it should enforce the use of disposable equipment in a range of other surgical procedures.

Human BSE victims confirmed since the death of Stephen Churchill, 19, in May 1995 have been aged between 16 and 52 when the disease became evident. Victims have continued living for between a few months and more than three years. The problem for doctors and the government is that so little is still known about BSE and how it is transmitted to man.

There is still no incontrovertible proof that it is, but even if the hypothesis is correct it could be three years before anyone can predict if hundreds or thousands will eventually die.

Research on cannibals in Papua New Guinea and the death rate among people infected with a similar condition contracted through growth hormone implants suggest incubation periods could run from 4.5 to 30 years, with an average of 13 years.

GIRL, 13, shows CJD symptoms

November 23, 1999 John von Radowitz, Medical Correspondent, PA News
A 13-year-old girl may be the youngest victim of the human form of mad cow disease. The girl, whose identity and whereabouts are being kept secret, is thought to be displaying symptoms of new variant Creutzfeldt Jakob Disease.

If the case is confirmed it has major implications. It raises the question of whether the girl was infected by baby food, and may shed light on the disease's incubation period. The girl was less than a year old in 1986 when BSE, the cattle disease thought to manifest itself in humans as the new variant form of CJD, was formally identified.

Three years later the Government banned parts of the cow most likely to be infected, such as the brain and spinal cord, from human food products. So far the youngest of the 48 people to have died from nvCJD has been 16.

David Churchill, chairman of the support group the Human BSE Foundation, was cited as saying he was aware of the case, adding, "I can confirm that the story is true - the girl is showing symptoms of the disease. This case raises a whole new spectre. There's no way anyone can say this child picked up nvCJD prior to knowledge about BSE.

Back in 1986 BSE was not only identified but becoming prevalent. It can only have been picked up after the emergence of BSE, and the likelihood is that it was through baby food. ... The ability to diagnose this illness from its symptoms is improving with each case, and there are some fairly clear diagnostic guidelines now. The chances of a misdiagnosis, or missed diagnosis, are less likely than they used to be."

Although the disease could only be confirmed for certain after death, the girl was showing signs and behaviour known to be linked with nvCJD.

Heather Paine, spokeswoman for the Infant and Dietetic Foods Association, was cited as saying that as far as she knew no high-risk beef material, such as mechanically recovered meat stripped off the spine, had ever been used in baby products, adding, "To my knowledge no MRM from cattle has ever been put in baby food. Manufacturers are very aware of what mothers want to feed their babies." At one-year-old the girl would probably have been weaned off commercial baby food and eating home-prepared meals which may have included mince and beef cuts, said Ms Paine.

Comment (webmaster):
There have been rumors going around for quite a while on this and other (?) very young cases. It is not clear why it is just in the newspaper now when the government is focusing all of its efforts on lifting the beef export ban.

There are many possible explanations: leaky feed ban, bovine brains or spleen in the baby food, childhood vaccination shots required nationwide, etc. It is very tacky for the Food Association to suggest that the mother is somehow to blame -- note the complete absence of disclosure of what they _do_ put in baby food.

The Inquiry record shows many internal memos from 1986 on expressing concern in particular about baby food and vaccines (which are made with bovine serum and injected). However, little was done and carelessly sourced vaccines continued to be used into the 90's; the food industry actively discouraged schools from taking beef off the menu.

Keeping nvCJD in perspective, one additional case brings the total to 50. It does not shed any light on how many other children will be affected nor on the ultimate scale of the epidemic.

Eyewitness account of BSE Inquiry

Down on the Farm
Private Eye, No.988 17 Nov 99
Comment (webmaster): This may or may not be an accurate account of a day at the Inquiry, but it raises some valid concerns and illustrates how little we really know about what happened and consequently where we are and what the lessons were.

SINCE evidence given to the BSE inquiry now runs into millions of words, it may not be surprising the media have long since lost interest. But the handful of insomniacs who have tried to follow the convolutions of the hearing through its website may have observed how, over the months, a fascinating alternative scenario for the BSE disaster has gradually emerged, punching ever larger holes in the credibility of the government's chief advisers.

This began when evidence from tests carried out by Dr Stephen Whatley seemed to confirm that the trigger for the BSE epidemic might have been MAFF's own insistence that millions of dairy cows should be treated with abnormally high doses of OP chemicals, significantly weakening their immune system.

Then earlier this year came the shocking revelation of how widely in the late 1980s the pharmaceutical companies were "harvesting" brain and lymphoid tissue from dead cattle in slaughterhouses to manufacture human vaccines. These could well have come from cattle infected with BSE. This has now led to a possibly even more disturbing revelation.

Not only were the pharmaceutical companies scavenging material to turn into vaccines. It has emerged in evidence from abattoir owners that they were also regularly collecting hormones from dead cattle to be injected into both other cattle and human beings.

And although there is no definite evidence that vaccines could have spread the infection, there is, alas, plenty of evidence that the injection of infected hormones can cause human Creutzfeld Jakob Disease (CJD). Furthermore the practice of injecting cows with hormones from other cattle was very widespread. if these hormones happened to come from BSE-infected animals, this could provide a much more plausible explanation for the wildfire spread of the epidemic than the use of infected cattle feed.

When this point was recently raised at the inquiry, it created as much of a moment of drama as Sir Donald Acheson's earlier squirming over vaccines. Interviewed over a video link, the distinguished American epidemiologist Professor Wayne Martin put precisely this question: was it possible that the decline in BSE after 1992 came from ending "the usage of bovine materials in pharmaceuticals"?

Stunned silence.

The only sound was that of the embarrassed shuffling of papers by the assembled official experts, led by MAFF, s chief epidemiologist John Wilesmith. Lord Phillips suggested: "I think Mr Wilesemith is the man really to turn to."

The stammered response he eventually got was: "Obviously it is possible to go back to pharmaceutical companies to detemiine their use of bovine tissues." In other words, we don't know because we never looked into it.

When Phillips pursued the question, by asking whether the cause of the epidemic might have been that "all cattle were injected with this particular product", Wilesmith was eventually forced to admit: "If that was the origin we have lost the trail."

In other words, he couldn't rule out that hormones might have played a crucial part in the BSE epidemic; but it was all so long ago we may never know. It was a reply which should raise rather larger questions in the judge's mind than Mr Wilesmith was obviously competent to answer.

TSEs in blood

Cambridge Healthtech seminars; Feb 13-15 2000 in DC
Opinion (webmaster):
This meeting, which focuses on perceptions and reality about TSE and blood, is open to the press and interested public though it is not free by any means and some of the talks may be technical. The Cambridge Healthtech web page will carry the abstracts.

The print brochure is unusual in tone. Each abstract contains a piece of horrific experimental news (transmission by blood transfusion, high persistent infectivity in plasma fractions, etc. ) followed by a totally upbeat assessment of how great this bad news really is.

This pattern is a consistent feature of the TSE/blood field: the glass is half-full (of infectious agent) but at the same time it is half-empty (infectivity levels might have been worse). But who will raise their glass on high to join this toast?

It all has to do with providing a legal umbrella for the funding agency or pharmo. Nobody can use that experiment to say you should have recalled such-and-such a blood product because plainly embedded right in the abstract is this crazy conclusory statement saying such-and-such blood product is the healthiest thing to come along since wholewheat unsliced bread. (And nobody is going to fund your next experiment unless the abstract is done right.)

Not one person with confirmed CJD has been able to come forward and provide scientific proof that they acquired their disease from the use of a blood product.

(Victims are of course incapacitated by the disease; few have scientific training, access to a level 3 containment lab, or funding to conduct the necessary studies; the disease is also extremely complex. The webmaster is not being sarcastic here but simply quoting the abstracts.)

Below is a Medline abstract for a just-published paper that strikes a reasonable balance:

Risk of transmission of CJD by transfusion of blood, plasma, and plasma derivatives.

J Clin Apheresis 1999;14(3):135-43 Vamvakas EC
Studies in experimental animals and case-reports of transmission of Creutzfeldt-Jakob Disease (CJD) by blood transfusion or by albumin products have raised the possibility that CJD may be transmitted by transfusion. The risk of transmission of CJD by transfusion remains theoretical, since no confirmed case of CJD has ever been causally attributed to the receipt of a blood transfusion, no confirmed case of CJD has developed in recipients of clotting factor concentrates, and no cluster of CJD cases has been reported following the administration of a pooled plasma derivative to which a donor who subsequently developed CJD had contributed.

However, based on a review of the hitherto available data, it is impossible to conclude at this time that CJD is not transmitted by blood or plasma transfusion or by the administration of pooled plasma derivatives.

This review discusses the findings of the animal experiments and the human studies that investigated the potential for transmission of CJD among humans by transfusion, and explains the statistical difficulties associated with proving the negative hypothesis that CJD is not transmitted by transfusion.

California Blood Banks Face Crisis

Sun, Nov 21, 1999  Associated Press. By JOHN HOWARD Associated Press Writer
California's blood banks have lost nearly $33 million in three years, and 14 of the state's 18 major blood centers face cuts in service because of dwindling funds, Blood Centers of California says.

The problems stem in part from higher operating costs and from inadequate reimbursements from the government and health management organizations to cover the costs of required screening and testing, the group said. Less money also means fewer donor drives and that could affect the state's blood supply.

"The public must be aware that unless something changes, the blood supply will be in jeopardy," Cathy Bryan, chief executive officer of the Blood Center of the Redwoods in Santa Rosa, said Saturday. Blood Centers of California, a nonprofit, represents facilities that handle more than 90 percent of the state's blood supply.

The centers collected about 897,000 pints of blood this year and received requests for 1.12 million pints, she said. Other sources, such as small hospital blood banks, the Red Cross and the Arizona-based Blood Systems, Inc., distribution network have helped make up the difference.

The blood centers' losses have increased about $5 million a year over the past three years, figures show. The centers, all monitored by the Federal Drug Administration and state health authorities, showed $5.1 million in losses in 1997, $10.8 million in losses in 1998, and $16.9 million losses so far this year.

Further French BSE, but no further UK imports

Agriculture Ministry Paris; November 22, 1999
A new case of mad cow disease has been discovered in southwest France in Savoie "departement", bringing to 26 the number of cows found with the disease this year, the Agriculture Ministry said Monday.

The Agriculture Ministry said new cases of the disease will continue to appear until 2001, five years after stringent prevention measures were taken against the illness, which has an average incubation period that long.

The latest case of bovine spongiform encephalopathy, commonly known as mad cow disease, involved a milk cow born in March 1991 in the Alpine region of Savoie, All 281 cows in the herd were slaughtered last week.

In August, the European Union lifted a ban on the import of beef from Britain, where mad cow disease first appeared. But France has angered Britain by continuing to ban imports. The European Commission, the executive arm of the European Union, has started legal proceedings against France.

France and Britain are in talks to break the impasse. France says it needs more assurance about the safety of the meat, but Britain insists its herds have recovered from the mad cow crisis.

Mad cow disease has no known cure, scientists believe the disease may be linked to an equally fatal human brain ailment, Creutzfeldt-Jakob disease....

French experts in beef ban meeting

Tue, Nov 23, 1999  By Geoff Meade, European Editor, PA News in Brussels
French food health experts who sparked the latest beef crisis were today considering whether it is now safe to allow imports from Britain. The meeting of the French Food Safety Agency follows weeks of wrangling between London and Paris and the launch of formal EU legal proceedings against France.

Technically, French Prime Minister Lionel Jospin could go ahead and lift the ban on British beef without the Agency's approval. But politically he needs the backing of the body he set up earlier this year to boost to consumer confidence over food safety.

The talks in Paris could last days and the outcome will depend on whether the Agency is convinced that a series of Anglo-French discussions have removed doubts about the spread of mad cow disease.

The UK Government insists it has done nothing but "clarify" existing stringent health and safety measures in Britain - measures which led to an EU-wide accord to lift the ban on August 1 this year, three-and-a-half years after it was imposed to calm consumer fears about BSE.

France broke the accord because the Agency said in its first-ever food safety judgment that it was still "premature" to put British beef back on French shelves. Now Prime Minister Jospin has sent the issue back for a second Agency verdict, insisting that his government has won significant concessions from the UK.

On the table is an EU Commission statement from week on meat labelling. It makes clear that France will in future be able to apply a "British" label to beef from the UK produced under the Date-Based Export Scheme, which sets strict health and safety standards.

The statement was seen as clearing a major stumbling block for France, which, like other EU countries, cannot oblige a beef exporter to label the meat by country of origin. The rule is designed to prevent such information being used unfairly to steer consumers away from products which rival the importing country's own beef. [The rule has the effect of allowing price under-cutting by producers using the cheapest and least unwholesome methods of production, forcing legitimate producers to also use hormones, sanitary waste, and rendered meat product. --webmaster]

But with the labelling hurdle cleared and with France insisting it has also gained concessions on the way cattle are tested for BSE, hopes are high that the Food Agency will give the go ahead and get the French government off the hook. Whatever the outcome of the Agency's talks, Mr Jospin has one more week to send written justification to the EU Commission of the continuing ban.

If Brussels is not satisfied with the response, the next legal steps leading to the European Court of Justice in Luxembourg will be triggered.

Tim Yeo, shadow agriculture minister, said: "The European Union's own scientific steering committee has given British beef a clean bill of health. Yet weeks later we are still waiting for the French to lift their illegal ban.

"Now it looks like Nick Brown, our weak agriculture minister, is willing to listen to renewed French demands for concessions. "Nick Brown must realise that when you have the law on your side you should not need to make concessions. British beef is safe, yet he is willing to stigmatise its safety to comply with France's hypocritical demands. "His whole approach to this crisis has been that of compromise."

Pushing livestock too hard

Thu, Nov 18, 1999 COMTEX Newswire By Lester Aldrich, Bridge News  Kansas City
Modern production practices and genetics may be pushing livestock to the brink of good health, all in the name of faster growth or more production, university professors said. The economic pressure for higher yields from the same or slightly higher inputs is too much to resist, but the tradeoff appears to be more frail animals that can't fight disease as well as their less genetically modified ancestors.

"Producers are just responding to economic signals," said Derrell Peel, livestock marketing economist at Oklahoma State University. But he wonders where these signals are taking US agriculture. To solve a perceived production need in some areas, the industry has used the easy, short-run solution of taking one trait or practice and pushing it to the extreme, Peel said.

Doing so can have unintended consequences in other areas of the animal's physiology. For instance, in dairying, the economic benefits of 3 and 4 milkings a day and using the hormone Bovine Somatotrophin (BST) to increase milk production to boot, is "mining" calcium from the cow, he said. They can't take in enough to accommodate the drain on their calcium reserves and their bones, and they end up being done with their productive lives early.

However, Donald O'Connor, staff epidemiologist for the Division of Animal Health in Wisconsin's Department of Agriculture, said the use of BS T hasn't changed the average 2 1/2- to 3-year age of cows in the state's dairy herds. It does increase feed and mineral needs, but the animal is capable of eating and absorbing the higher nutrient needs. The rumen (the first of 4 stomachs in a cow), after all, holds 55 gallons.

O'Connor thinks some dairy owners aren't using BST because they don't want the added hassles of once-a-day injections, the increased feed needs--which can rise by 30% to 50%--or the push toward 3 milkings a day. He sees no reason to think BST shortens the useful life of a cow.

Temple Grandin, animal science professor and animal behavior expert at Colorado State University, said that in an effort to accommodate that increased nutritional need, breeders are opting for increased appetite. This helps the animals that become cows, but dairy animals placed into feedlots are beginning to show other things like increased chewing or licking on fences and other equipment. While she is not against high production techniques or genetics, Grandin said breeding for sharply increased production alone depletes the animal's ability to perform in other areas.

In her experience, the ability to fight off disease appears to be the area that loses out. That wouldn't be a problem if there were no diseases, she said, but this isn't the reality of the world. There seem to be more cases of Johnes Disease, for instance, than there were several years ago, and she wonders if it is related to losing resistance in the name of increased production.

Grandin said swine also pay the price for genetics that gear them toward rapid growth. Fifteen years ago, "nobody knew or bothered about PRRS (Porcine Reproductive and Respiratory Syndrome)," among others, and now it's a big deal.

Hog producers have gone to great lengths to insulate themselves from PRRS and still have fast-growing animals by building units well away from traditional swing-producing areas, Grandin said.

But it was to no avail. Resistance was so low that a truck driving by on the distant highway introduced it to the herd. Some scientists worry that the level of disease resistance is so low, and the genetics of the US swine herd so similar, that the right disease could wipe out entire herds if it ever got started, Grandin said.

Peel said a recessive gene in hogs that produces very lean meat also makes the meat pale, soft and exudative. Breeders are working to get it, but selecting for one trait brought it out in the first place.

Grandin has pushed US hog breeders for years to select for less nervous pigs, even at the expense of rapid growth, and some now are working on it. In extreme cases, modern pigs are so nervous they can die of a heart attack just being moved from their pens into the truck.

The poultry industry isn't to be left out either, Grandin said. She recently returned from Australia where growers had imported US birds with faster-growth genetics. Producers there acknowledged they grew faster than their previous lines, but they were very susceptible to disease if there were ventilation problems, for instance.

She said there was so much muscle on US chickens a few years ago that some didn't have the heart and lungs to support it. The industry is breeding strength back into the birds now, resulting in fewer heart attacks and stronger legs.

Gerald Havenstein, head of North Carolina State University's Poultry Science department, said it was not true that modern chickens have health problems related to faster growth rates. Parent stock is kept for more than a year before they are culled, and there aren't serious problems associated with them.

Most broilers, by contrast, are slaughtered at 42 to 60 days of age, Havenstein said. This is possible because of the modern white broiler, which emerged in 1957. At 6 weeks of age, the modern chicken will be 3.5 times as heavy as the average bird in 1957.

Grandin and Peel were concerned that US livestock industries had gone so far in their use of production-oriented genetics and practices that parent stock would be hard to find if, for some reason, breeders decided they had to start over.

Both were glad for state and county fairs where children competed with exotic and purebred livestock because it may just represent the seeds of future livestock strains. Even Havenstein pointed to the state fairs when asked if parent stock might be difficult to find. The gene pool is available to reintroduce certain traits to the lines in current use.

USDA seeks sheep slaughter over BSE

By Guy Gugliotta Washington Post Staff Writer Monday, November 15, 1999
USDA Seeks to Slaughter 365 Sheep
Officials Fear Vermont Herds Came in Contact With Mad Cow Disease

The Agriculture Department wants to slaughter 365 sheep under quarantine at two Vermont farms because they may have come in contact with mad cow disease.

Controversy over the sheep's fate underscores policy makers' continuing wariness over the family of degenerative brain afflictions believed to be spread by oddly shaped proteins called prions.

There has never been a case of mad cow disease, known as bovine spongiform encephalopathy, or BSE, reported in the United States, but 50 people in England have died from a similar disease, apparently after eating contaminated beef.

And while alarm has subsided over the possibility that cows contract BSE from feed derived from sheep brains, unease lingers over whether sheep can carry BSE for years after ingesting feed derived from contaminated beef.

"This is brand new," said George Beran, a veterinary professor at Iowa State University and author of a handbook on diseases that affect both humans and animals. And rudimentary detection methods make "depopulating the herd" the only sure way to combat a prion disease outbreak.

It was uneasiness over the science of BSE that prompted the Agriculture Department to keep a close watch on 65 East Friesian and Beltex milk-producing sheep imported by Vermont farmers Larry Faillace, of Warren, and Houghton Freeman, of Greensboro, in early 1996.

The importers wanted to build a new cheese-making industry. "The average American sheep does 100 pounds of milk in a year, while ours do 1,000 pounds," Faillace said.

The USDA was initially concerned about whether the sheep were infected with scrapie, the ovine equivalent of BSE, but the animals were quarantined and cleared both in Belgium and the United States.

According to Linda Detwiler of the USDA, however, European research released just after the herd's arrival in Vermont suggested that BSE could be transmitted to sheep. And immediately after that, England reported that deaths from brain disease could be linked to consumption of contaminated beef during an outbreak of BSE in the mid-1980s.

The Agriculture Department began to monitor the Vermont herd, buying animals the farmers wanted to sell and closely examining the brains of animals that died. Faillace and Freeman bred their herds and made their cheese. This month, they had 365 animals between them.

Questions about BSE and its cousins remain unanswered. Beran said scientists are certain that scrapie--a disease with a long history--was transmitted to cattle some time in the "recent past," because the 1980s outbreak was apparently BSE's debut. But scientists have not been able to determine how or when the cross-species transfer was made, nor have they been able to duplicate it in the lab, Beran added.

Research was made even more difficult because the prions for scrapie and BSE are almost identical. Nonetheless, at this point, scientists have reached consensus that BSE is transmitted when cows eat feed made from bovine--not ovine--brain tissue, which is offered to them as a protein supplement, Beran said.

This, however, did nothing to dispel uneasiness over whether sheep could carry BSE and transmit it to cattle. In September 1998, the European Union issued an opinion paper saying that Europe could not be sure that its sheep were uncontaminated.

The next month, the state of Vermont--acting on the advice of the USDA--put the Belgian herds under quarantine, forbidding the sale of breeding stock or meat. USDA and the owners now are at an impasse. The agency wants to buy the sheep and destroy them, but Faillace and Freeman are refusing to sell.

"There's nothing wrong with our sheep, and the risk is theoretical," said Faillace, who says he can sell all the cheese he can make. "We just want USDA to get off our backs."

But Detwiler, senior staff veterinarian for the Animal and Plant Health Inspection Service, said the Agriculture Department would persevere, even though the sheep were ostensibly disease-free: "We're just trying to be ultraconservative."

Sheep Owners in Fight With USDA

Tue, Nov 16, 1999 By ROSS SNEYD Associated Press Writer
EAST WARREN, Vt. (AP) -- Larry and Linda Faillace are excited about their plans to change the American sheep industry. Since importing their first East Friesian sheep from Belgium three years ago, they've established a breeding stock of milk-producing animals and introduced a line of sheep-milk cheese.

But the couple's flocks have been locked up under quarantine amid government fears that a disease related to mad cow is lurking under the wool. The U.S. Department of Agriculture wants to buy the animals for slaughter to check for illness.

Behind all of this is BSE, or bovine spongiform encephalopathy, the scientific name for mad cow disease. The fatal brain ailment has affected some 180,000 head of British cattle and has been linked to a similar illness in humans, known as Creutzfeldt-Jakob disease. At least 50 people have died in Britain after they apparently ate contaminated beef.

Sheep are susceptible to scrapie, a disease thought to be caused by the infectious protein behind mad cow disease. Some scientists believe British cows contracted mad cow disease from feed containing meat from sheep with scrapie. Scientists say the Faillaces' sheep are not infected with scrapie but there's no way of knowing for certain whether the animals are infected with the sheep version of mad cow disease.

With European sheep milk already imported to the U.S. market, the Faillaces have refused the USDA offer. They say it's proof the government has gone mad, not their sheep.

"With these diseases, if you wait ... till you have conclusive proof before you take preventive action, you're too late," said Dr. Linda Detwiler, the agency's senior staff veterinarian. "And sometimes, you're a decade too late."

The Faillaces' enterprise began while they worked in British agriculture. Larry has a doctoral degree in animal physiology, and worked with scientists who would later head investigations into mad cow disease. After settling in Vermont, the couple decided to breed and sell the East Friesians -- then unknown outside Europe -- because they produce 10 times the milk of typical American breeds.

Along the way, they met millionaire philanthropist Houghton Freeman, who was looking for a way to help struggling Vermont farmers diversify. By 1996, with a business plan and USDA approval, they shipped their first sheep. The flocks produced beyond expectations, and the Faillaces and Freeman began breeding them to sell and export, once the USDA agreed. A thriving cheese business also was launched using milk from the sheep.

Then, almost two years after the importing began, the USDA raised concerns that the sheep had come from the region in Belgium where cows were infected with BSE. More than a year later, their flocks -- a total of 365 animals -- remain under quarantine by state agriculture officials on behalf of the USDA. None of the sheep can be sold for breeding or meat, although cheese from their milk is still produced and sold.

"The only way we could make money this last year was from cheese," Linda Faillace said. Federal food officials are now questioning whether the milk is appropriate for humans, while millions of pounds of sheep milk cheese continue to be imported from Europe.

For now, fearing the ruin of their breeding business, the Faillaces and Freeman are refusing to back down and sell their flocks. The USDA has said it would be willing to allow the sheep to be exported back to Belgium, and has offered help in establishing a new stock of milk-producing sheep.

"This isn't easy," Detwiler said. "It's definitely not our intent to put them out of business but to help them to restock with something that would be viable for Vermont agriculture, but not put livestock and human health at danger."

Genome of radiation-resistant bacterium probed

Thu, Nov 18, 1999 Reuters World Report by Maggie Fox, Health and Science Correspondent 
A pink bacterium that shrugs off the worst radiation and which has been taught to thrive on toxic waste is yielding genetic secrets that could lead not only to better waste clean-up but better treatments for cancer, researchers said on Thursday.

Teams at The Institute for Genomic Research (TIGR) in Rockville, Maryland and at the Department of Energy said they had sequenced the genome -- the entire collection of genes -- of Deinococcus radiodurans.

They say it seems to have thousands of unique genes that help it clean up the damage done to its DNA by radiation, helping it survive where any other creature would die.

Found living happily 40 years ago in a can of food that had been irradiated to kill germs, D. radiourans has intrigued scientists ever since. It can survive 1.5 million rads of gamma irradiation -- a dose 3,000 times the amount that would kill a human. It also pops back to life after being dried out and can live through high doses of ultraviolet radiation. Just last year, researchers genetically engineered it to eat up toxic chemicals such as toluene and mercury.

"This is a significant accomplishment," Secretary of Energy Bill Richardson said in a statement. "Besides the insights into the way cells work, this new research may help provide a new safe and inexpensive tool for some of the nation's most difficult cleanup challenges."

The team at TIGR, which has now sequenced the genomes of 10 important micro-organisms, used the "shotgun" method to make multiple copies of every piece of genetic material in the bacterium. Writing in the journal Science, they said they overlapped these to make a full map of its genes. They found about 3,100 genes arranged on two circular chromosomes, TIGR president Claire Fraser said.

The secret to Deinococcus's toughness seems to be a large number of genes that allow it not to prevent damage from radiation, heat and other assaults, but to repair that damage quickly and efficiently enough to allow it to survive. Radiation, heat and chemicals create breaks in the double helix of DNA that makes up the genes. This can kill a cell outright, or cause it to make mistakes as it replicates itself -- mistakes that can kill it, or cause cancer and other disease.

"A unique mechanism may contribute to D. radiourans' resistance to DNA damage," the researchers wrote in their Science report. "This organism transports damaged nucleotides out of the cell, which potentially prevents their reincorporation into the genome." In other words, it throws out the damaged pieces of genetic material.

"Other cells try to recycle building blocks of DNA," Fraser said in a telephone interview. "The last thing you want floating around in cytoplasm of the cell is a large number of nucleotides that have been damaged in some way by the radiation."

In addition, the bacterium has other unique genes that must help it clean up damage before it causes harm. "This absolutely has implications for understanding DNA damage in cancer," Fraser said. Many cancers are known to be caused by mutations in the genes responsible for fixing damaged DNA.

"Somewhere between 500 and 1,000 genes in this organism are going to be unique to Deinococcus," Fraser said. "My guess is that some are absolutely critical to the ability of Deinococcus to withstand radiation."

A year ago Michael Daly and colleagues at the University of Minnesota inserted four genes into Deinococcus that gave it the ability to break down some kinds of toxic chemicals and to convert mercury into a less dangerous form. The Energy Department hopes to make use of genetic information about Deinococcus to figure out even better ways to do this.

Comment (webmaster): This should not be confused with radiation resistance of prions. That is a property of proteins generally. DNA however is adversely affected. In this bacteria, radiation does cause lethal double-stranded breaks. However, the bacterium can repair them provided the cell is still healthy metabolically.

It seems unlikely that irradiation of meat will help much. Bacteria such as this will continue to flourish until radiation levels are such that the meat itself would be severely damaged.

Another British lion dead from BSE

MAFF correspondence 20 Nov 99
MAFF sent the webmaster further details concerning a second British lion that died of TSE (presumbably BSE from feed: split spinal cords and skulls). This lion was born in November 1981 at zoo X [name of zoo not released] and transferred to zoo Y [name of zoo not released] in November 1985, where slight ataxia was first noted in March 1999> The and the animal was uthanased in May 1999 at age 18.

There is no indication that it was related to the previous lion "Lumpy": as:

Lumpy +  Panthera leo lion born 1986 Woburn SP died 1998 Edinburgh zoo UK [since 1994]

Zoo animals affected by BSE are tracked at other places on this web site (1, 2, 3, 4)

French are right in beef war, saY CJD families

Fri, Nov 12, 1999 By Jon Coates, PA News
Families who have had their lives torn apart by the human form of mad cow disease today called for the beef war to end. They claim that for most people, the lifting of the French trade ban on British beef has become an issue for the wrong reasons - and that the French Government is right to protect the health of its population.

Since it was discovered in 1995 the new strain of Creutzfeldt Jakob disease (CJD) has claimed 49 lives in the UK. Roger Tomkins, 53, of Norfolk, watched for two years while his daughter Clare died a "terrible" death from new variant CJD. "The so-called war between France and Britain has caused the point of the issue to be totally lost," he said. "My daughter died from new variant CJD and the cause of this was eating infected meat from an animal. "Everyone in this country who ate meat from a bovine animal was put at risk of catching the disease."

The former company director, who is now semi-retired due to ill health, has attended every day of the public inquiry into BSE, or mad cow disease, in London for the last two and a half years. He said: "I have listened to the inquiry detail the inadequate action that was taken over beef over the years. "I am still not convinced that all the new measures talked about being put into place to prevent cattle becoming diseased are policed properly or are safe. "I am sceptical about saying our beef is 100% safe as I have not been convinced of this."

Mr Tomkins, whose daughter Clare died at the age of 25 in April last year, also lost his wife Dawn to cancer eight weeks later. He believes the stress of Claire's death was a major factor in that of his wife. Clare was bedridden and was unable to communicate or control her bodily functions. She could not swallow so had to rely on an automatic pump by her bed to clear her saliva and prevent her choking.

She needed 24-hour-a-day nursing which her mother provided until she was struck down with ovarian cancer. Now Mr Tomkins has only his elder daughter Lisa, 30, left. She helped him nurse Clare when Dawn became ill. Mr Tomkins says it is up to each individual in Britain to decide whether they choose to eat beef or not, but it is not eaten in their house.

He admires the French leaders for acting to protect their people. He said there were around 3,000 cases of Bovine Spongiform Encephalopathy - BSE - reported in the UK last year and there would be in excess of 2,000 cases this year, so the disease has not been eradicated.

Mr Tomkins, who moved from the former family home in East Peckham, Kent to Norfolk for privacy, said: "If you have ever seen anyone suffer from new variant CJD you would not take any risks with beef.

"I watched my daughter die a terrible, terrible death and if the French leaders are saying they do not want to put even one of their people at risk, who are we to say differently?" He added: "Sadly there will always be an `us and them' mentality between the British and French, but the disease we are talking about is no joking matter. If you catch it, you die, its as simple as that.

"I admire the French leaders for sticking to their guns on this issue. "The political war that is being waged in this country at the moment is very sad, as the politicians are missing the point that people are dying from eating beef."

Mr Tomkins said that until there is more testing and research completed people can never be 100% sure that British beef is safe. "We were responsible for the birth of this disease," he said. "We have gone a long way towards eradicating it but I am not convinced we have completely done this. "There is still too much that is not known about the subject, that is not known about the protein that causes BSE."

Peter Clayton, of Gregswood Road, Tunbridge Wells, Kent, whose wife, Julie, 39, is dying from new variant CJD, agrees with Mr Tomkins. He also appealed for people not to bicker over British beef, claiming the "war" with France risked trivialising the suffering the disease has caused.

"I would like to remind people that the beef war is about more than simple bickering among politicians - it's about people's lives," he said. "Banning or boycotting French beef won't bring my children their mum back." Mr Clayton, who has two sons, Nicholas, 14, and Jack, seven, has watched his wife be reduced to a "cabbage" in less than two months.

"She just lies there frightened and confused," he said. Mrs Clayton was incapable of walking, speaking or even sitting up in bed within just four weeks of being struck down with the disease. She has to be fed through a tube and cannot communicate with her husband or children. The public inquiry into the causes of BSE and how it was spread was opened on March 10 1997 and is still ongoing.

``I eat British beef,'' says U.S. farm chief

Reuters World Report  Fri, Nov 12, 1999
U.S. Agriculture Secretary Dan Glickman on Friday shunned British beef scares of his countrymen and France and said he was happy to eat the red meat. "I've been to Britain and eaten the beef and I am not worried as a person," Glickman said, asked whether he thought British beef was as safe as other European food.

Glickman was trying to strike a conciliatory tone over a separate trade dispute involving hormone-treated U.S. beef which is banned from the European Union. Washington says scientists should decide whether any food is safe for the public to eat.

But Glickman's efforts to take the politics out of food were drowned by fresh in-fighting inside Europe as Paris accused the British of being anti-French in their row over mad cow disease.

"There is in Britain a kind of anti-French, Francophobe fury, with xenophobic overtones," French Farm Minister Glavany said in an interview with French TV news channel LCI. Glavany said Britain overlooked the fact that Germany, the United States and many Commonwealth countries also continued to ban British beef.

France and Britain are deadlocked over France's refusal to import British beef in defiance of an EU ruling that a three-year-old ban imposed on health grounds can now be lifted. Glickman, in Rome for a United Nations food conference, did not say whether the United States would import the beef but the Clinton administration has said it welcomes the European Commission's insistence that science govern food trade.

Blood Donor Ban in New Zealand

21 Nov 99 The Associated Press
WELLINGTON, New Zealand - Any person who spent six months or more in Britain between 1980 and 1996 will be banned from donating blood in an effort to protect supplies from a fatal brain disease linked to mad cow disease, New Zealand officials announced today.

New Zealand's health ministry and blood service also said a filtering process would be put in place to identify any white blood cells associated with an aggressive strain of Creutzfeldt-Jakob Disease before transfusion, a move expected to cost the equivalent of $4 million each year.

The new strain of Creutzfeldt-Jakob Disease has been linked to the British outbreak of bovine spongiform encephalopathy, or mad cow disease. The spongiform diseases eat holes in the brains of victims, and no cure has been discovered.

No New Zealander has ever contracted the disease, chief medical adviser Colin Feek said. There is also no evidence that it can be transmitted by blood transfusions or by any type of person-to-person contact, he said.

The European Union imposed a worldwide ban on British beef in 1996 when mad cow disease was linked to a similar fatal, brain-wasting disease in humans. The EU lifted the ban in August, saying British beef was now safe.

Feek said there was no evidence that people who visited Britain have become infected - ``even those who have eaten beef while traveling there.'' ``This is a case of erring very strongly on the side of caution, but we want to assure recipients of the blood products that the blood supply continues to be as safe as possible,'' Feek said.

The ban will come into effect from Feb. 17. Feek said the policy was not being implemented immediately as the blood service wants to ensure it is ``introduced consistently, effectively and without significant impact on our ability to collect sufficient blood for patients.

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