Document Directory

08 Jun 01 - CJD - Czechs Confirm First Mad Mad Cow Outside W. Europe
08 Jun 01 - CJD - First Czech Mad Cow case confirmed
08 Jun 01 - CJD - Officials try to quell fears, BSE test looms
08 Jun 01 - CJD - Oxford's Aptamers May Help Detect And Treat BSE Prions
08 Jun 01 - CJD - So far, Mad Cow disease not a problem here
08 Jun 01 - CJD - Germany to sue over bse costs
07 Jun 01 - CJD - Czechs Find First Suspect Case of BSE, Await Tests
07 Jun 01 - CJD - Czech Republic Confirms Suspicion of Mad Cow Case
07 Jun 01 - CJD - Scientists search for way to detect Mad Cow
07 Jun 01 - CJD - BSE meat risk from abattoir culls
07 Jun 01 - CJD - Weak BSE rules 'leave food chain still at risk'
07 Jun 01 - CJD - BSE infection loophole
06 Jun 01 - CJD - 'Meat for humans could still have Mad Cow infection'
06 Jun 01 - CJD - Report raises new fears over BSE
06 Jun 01 - CJD - BSE 'cross-infection' risk
06 Jun 01 - CJD - New Risk Of Mad Cow Disease
06 Jun 01 - CJD - CJD gaining attention in United States
06 Jun 01 - CJD - On the USDA's Front Line Against Mad Cow Disease
06 Jun 01 - CJD - Mad Cow fears put renderers under microscope
06 Jun 01 - CJD - Agencies work to keep Mad Cow disease out of U.S. pastures
06 Jun 01 - CJD - Biologists warn of disease among deer



08 Jun 01 - CJD - Czechs Confirm First Mad Mad Cow Outside W. Europe

Reuters

YAHOO--Friday 8 June 2001


PRAGUE (Reuters) - The second test on a Czech cow suspected of having Mad Cow disease was positive, Agriculture Minister Jan Fencl said on Friday, confirming the first case of BSE outside western Europe.

``(A new test) confirmed the original finding,'' Fencl told reporters. He said that another test to verify the results would be carried out in Germany. ``I expect the result... in a laboratory in Germany in the middle of the next week,'' he said.

Bovine Spongiform Encephalopathy (BSE), a fatal, brain-wasting disease, first appeared in cattle in Britain in the 1980s but a second wave swept last year into France, Germany, Italy and Spain. The Czech case was the first in eastern Europe.

Fencl did not say what action the government would take, but veterinary officials had said earlier that suspected animals from the affected herd would be slaughtered. All slaughtered animals older than 30 months would be examined.

Hungary instantly said it would withdraw all licences for import of Czech beef, and allow only tested meat to enter the country.

Poland's chief veterinarian said the country was banning beef and beef product imports from the Czech Republic, and neighbouring Slovakia also banned all beef imports as from June 9.

Before the suspected case, Czech officials had tested more than 10,000 animals this year and found no signs of the disease.

Scientists believe BSE is transmitted through infected cattle feeds and may cause the fatal variant Creutzfeldt-Jakob disease (vCJD) in humans. More than 80 people in Britain have died of the incurable vCJD.

The suspected Czech cow was bred at a cooperative farm in Dusejov, some 120 km (70 miles) southeast of Prague. It came from a herd with 389 cows including 18 that are pregnant.

The European Commission had listed the Czech Republic as a country likely to present a BSE risk, because it had imported meat-and-bone meal that had been identified as the source of BSE.

The Czechs had protested against such classification, saying the imported meal was not fed to Czech cattle because it was too expensive.

Retailers said the news would almost certainly lead to a drop in beef sales. ``There's going to be a panic. I know Czech customers,'' said Radim Patek, marketing director at retail chain Julius Meinl.

The Agriculture Ministry has been investigating if the cow could contract the disease through milk feed, in which milk fat was substituted by rendering-plant fat.

Agriculture in the Czech Republic accounts for only about 4.4% of GDP. There were 1.57 million cattle in the country at the last count in 2000.


08 Jun 01 - CJD - First Czech Mad Cow case confirmed

Staff Reporter

BBC--Friday 8 June 2001


The Czech Republic has discovered what is believed to be the first case of BSE, or Mad Cow disease, outside western Europe.

The Czech Agriculture Ministry made the announcement after two tests confirmed the disease in a six-year-old cow from a farm in Dusejov, about 150 kilometres (90 miles) south-east of Prague.

In April, the European Union placed the Czech Republic on a list of countries believed to be at risk of the disease.

EU imports

The country has imported cattle and meat on the bone from EU countries where the disease was known to be present.

The infected cow was from a herd of about 400.

The results of the German test are expected next week, and an agriculture ministry spokesman described the finding as "not final yet," pending those results.

The Czech Republic began testing for BSE at the beginning of the year.

More than 10,000 head of cattle have been tested for the disease, Czech authorities said.

Mad Cow disease has been linked to variant CJD, a fatal human disease that affects the brain.


08 Jun 01 - CJD - Officials try to quell fears, BSE test looms

Reuters

Prague Business Journal---Friday 8 June 2001


June 7 - The Czech Republic quickly sought to reassure the public on Thursday that local beef is safe as the country braced for the possibility it is the first in the region to record a case of Mad Cow disease.

Positive results from an initial test on a six-year-old cow were announced on Wednesday and showed the animal may have developed the disease, raising fears that Bovine Spongiform Encephalopathy (BSE) may have spread into eastern Europe.

Results of a second test will be released on Friday at around 1200 GMT, and if positive, they will be passed on to a German laboratory for further confirmation.

Officials and grocers have already begun trying to calm public fears that local beef is tainted.

"I want to assure the public that meat from Czech cattle that is on the market is still safe," Agriculture Minister Jan Fencl said.

BSE has spread in herds in Britain, France and other west European countries but, until now, it has not been suspected in the Czech Republic or the east European region as a whole.

In April the European Commission listed the Czech Republic as a Category III country - likely to present a BSE risk because it had imported significant amounts of live cattle and meat-and-bone meal from EU countries where BSE has been confirmed.

Scientists believe BSE is transmitted through infected meat-and-bone meal fed to cattle and may cause the fatal, brain-wasting variant Creutzfeldt-Jakob disease (vCJD) in humans. More than 80 people in Britain have died of vCJD.

Before the suspected case was announced, Czech officials had tested more than 10,000 animals and found no signs of the disease, prompting them to ask to be taken off the EU list.

SUPERMARKET SIGNS ASSURE THAT BEEF IS SAFE

At the bustling British-owned Tesco supermarket in the centre of Prague, customers searching for a steak or some ground beef are now greeted with a sign assuring them that all of the products on the shelf have been tested and are safe.

But a shopper called Jana, with two young children in tow, looked at the sign, put down some beef she had intended to use to make goulash, and instead picked up some chicken.

"It (the beef) is probably safe, but I would rather be careful right now. I still don't know if I believe what the politicians say," she said.

Agriculture in the Czech Republic accounts for only about 4.4 percent of gross domestic product and cattle farming a small fraction of that.

But the Czechs have been quick to fill the void left by many EU producers after the BSE outbreak all but halted their sales abroad. In the first quarter Czech beef exporters shipped 5,245 tonnes (live weight) of beef, the highest January-March total in three years.

Some of the other countries listed as category III - Poland, Hungary, Estonia, Lithuania, Slovakia, Cyprus, Switzerland and Albania - said they were waiting for confirmation about the disease from the Czech Republic before deciding whether or not to take any action.

"We will withhold action until we have official confirmation of BSE from the Czech ministry. We cannot base our actions on suspicions," said Agnieszka Prawdzic, spokeswoman for Poland's chief veterinarian.

All of these countries except Albania and Switzerland - which is the only country outside the EU to have confirmed the presence of native cases of Mad Cow disease - are candidates for EU membership.


08 Jun 01 - CJD - Oxford's Aptamers May Help Detect And Treat BSE Prions

Staff Reporter

Our World---Friday 8 June 2001


New technology that could eliminate the risks of "Mad Cow" disease (Bovine Spongiform Encephalopathy, or BSE) transmission to humans via infected food or blood transfusions may now be on the horizon.

Oxford University and V.I Technologies Inc. (VITEX) have teamed up in agreements which will license RNA aptamers -- molecules designed to stick to the prions which cause Creutzfeldt-Jakob disease (CJD)-- developed at Oxford's Dunn School of Pathology, to VITEX for development into a range of tools which can both detect and remove prions, for use in the food, medical and agricultural industries.

Prions are recognized as the infectious agents that cause CJD by attacking the brain, killing cells and creating gaps in tissue, giving the brain a characteristic sponge-like appearance.

Currently there are no diagnostic tests available to confirm if CJD is present other than a brain biopsy or autopsy, and there are no treatments available -- CJD is invariably fatal, with 90 per cent of patients dying within one year.

It is hoped that the new aptamers will form the basis of a reliable diagnostic tool for CJD and also point the way toward potential treatments which could utilize the aptamer to remove the infectious prions from the brain and bloodstream.

VITEX will also be funding further research at the Dunn School into the potential applications of the new aptamers to the blood transfusion industry.

Current testing agents for prions in blood can only detect them once they have risen above a certain level, meaning that transfusions could potentially transmit a risk of CJD. The aptamers could serve as a filter through which blood could pass and remove any undetected prions, ensuring that new standards of blood safety are reached.

The aptamers are manufactured from a resistant RNA-like chemical and are specially designed genetic strips which recognize and bind to the protein sequences in infectious prions.

Dr. William James, head of the research group which designed the aptamers, said, "We are very excited about this breakthrough in prion research and look forward to the collaboration with VITEX, which will enable us to make sure that our discovery is turned into something that could make a real difference in CJD diagnosis and prevention."


08 Jun 01 - CJD - So far, Mad Cow disease not a problem here

By Bob Guinn, Clemson Extension Agent

Low Country Now---Friday 8 June 2001


Mad Cow Disease, also known as BSE or Bovine Spongiform Encephalopathy, was first diagnosed in 1986 in the United Kingdom. It has since affected approximately 170,000 cattle in the U.K. and other European countries. A pre-emptive slaughter and destruction of nearly 4.5 million cattle has crippled the British livestock industry and also affected the tallow, gelatin, and pharmaceutical industries.

Recent scientific studies suggest that BSE may be linked through consumption of BSE-infected beef products to new variant Creutzfeldt-Jakob disease, a fatal neurodegenerative disease in humans.

It is thought that the problem started when rendered carcasses of livestock (including infected sheep, where BSE was thought to originate) were fed to cows and other animals as a protein-rich nutritional supplement. During rendering, carcasses from which all consumable parts had been removed were milled and then decomposed in large vats by boiling at atmospheric or higher pressures, producing a liquid mixture of protein under a layer of fat (tallow). After the fat was removed, the slurry was dried into a meat and bone meal product that was packaged by the animal food industry and distributed to owners of livestock and other captive animals, such as laboratory animals, breeding species, and pets.

It appears likely that changes in the rendering process that had taken place around 1980 allowed the disease in infected carcasses to survive, contaminate the protein supplement, and infect cattle. Cattle carcasses and carcass wastes were then recycled through the rendering plants, increasing the levels of the now cattle-adapted pathogen in the protein supplement and eventually causing a full-scale BSE epidemic.

The U.S. Department of Agriculture has taken aggressive measures to prevent the entry of BSE into the United States. These measures include banning cattle and cattle products from countries where BSE has been reported or where adequate surveillance programs are not in place; monitoring and testing for BSE in U.S. cattle; and educating producers and the public about the disease. Other countries with confirmed cases of BSE in native cattle include Belgium, France, Ireland, Luxembourg, the Netherlands, northern Ireland, Portugal, and Switzerland. BSE has not been detected in the United States.

Clemson Extension Agent Bob Guinn can be reached at 470-3655 or by e-mail at rguinn@Clemson.edu


08 Jun 01 - CJD - Germany to sue over bse costs

By Claus Hulverscheidt and Hugh Williamson in Berlin and Rainer Koch in Brussels

Financial Times--Friday 8 June 2001


The German government is to file a case in the European Court of Justice against the European Commission over the financing of the commission's programme to eradicate BSE, or Mad Cow disease.

Government sources confirmed on Wednesday that the Finance Ministry would prepare the legal challenge. Berlin is unhappy that 30 per cent of the 430m ($364m) programme agreed in March is to be paid by European Union member states.

Mr Fischler in March pushed through the programme under which the EU would buy up to 1.2m head of beef cattle older than 30 months either to be destroyed or to be slaughtered and stored. The EU said it could not finance the estimated costs alone and EU member states were asked to contribute 30 per cent of the cost of the programme.

Facing strong opposition from agriculture ministers and having only four states in favour, Mr Fischler pushed the plan through the EU's administrative committeey under qualified majority voting.

Hans Eichel, German finance minister, fears Mr Fischler's actions could constitute a precedent, which it was important to oppose now.

People close to the government told Financial Times Deutschland: "If we allow the Commission to exceed its competencies in such a fashion, it will in future try more and more often to tap member states' coffers whenever there is a case of disputed financing."

Germany's foreign ministry does not share the view of the finance ministry and is concerned that a court case could damage Germany's standing within the EU. Renate Kunast, German agriculture minister - who in March criticised Mr Fischler's plans - is also concerned over Mr Eichel's initiative.

The finance ministry feels that since the agriculture commissioner's plan was a market support initiative, it should have been financed from the EU budget, according to EU treaties. Germany was in principle prepared to see some of the EU's spending on agriculture to come out of national budgets, but there should be no "co-financing" by the "back door", said people close to the government.

On Tuesday the EU council of ministers' legal service on Tuesday said it had doubts as to the legality of the "Commission's solo effort".

The finance ministry was stressing that Mr Eichel was not concerned over the cost but the principle of the case. This was proved by the fact that Germany would have to pay less under the Fischler plan than if the programme were funded from the EU's budget.

As Germany was the EU's biggest net contributor of funds it would also shoulder the largest share of common agriculture expenditures.


07 Jun 01 - CJD - Czechs Find First Suspect Case of BSE, Await Tests

By Alan Crosby

Pittsburgh Post-Gazette---Thursday 7 June 2001


PRAGUE (Reuters) - The Czech Republic said on Wednesday it suspected that a six-year-old cow on its territory had developed Mad Cow disease, arousing fears that BSE may have spread beyond western Europe.

``At this point, we can confirm the first suspicion of BSE in the Czech Republic,'' Czech Agriculture Ministry press official Hugo Roldan told Reuters.

Roldan said the animal was from a herd in Dusejov village in the Jihlava region, 70 miles southeast of Prague.

``This is the first test, it is necessary to confirm the first result, so we are sending the sample to a laboratory in Germany to confirm or refute our results,'' he added.

BSE -- Bovine Spongiform Encephalopathy -- has spread in herds in Britain, France and other west European countries but until now has not been suspected in the Czech Republic or the east European region as a whole.

In April the European Commission listed the Czech Republic as a country likely to present a BSE risk because it had imported significant amounts of live cattle and meat-and-bone meal from EU countries where BSE has been confirmed.

Scientists believe BSE is transmitted through infected meat-and-bone meal fed to cattle and may cause the fatal, brain-wasting variant Creutzfeldt-Jakob disease (vCJD) in humans. More than 80 people in Britain have died of vCJD.

The ministry said the cow came from a cooperative farm that has 371 cows. Some 18 pregnant cows were in the affected stall. Pavel Kovar, of the ministry, told Reuters milk feed substitutes were being investigated as a possible cause of the case and added that bone meal had not been fed to the herd.

``We are investigating if the contamination could have happened through feeding of milk feed substitutes, in which milk fat was substituted by rendering-plant fat,'' Kovar said, adding that the mixtures were imported.

``We know they (the affected farm) used the substitutes but must confirm that it was at this time. It is after all six years ago,'' he said.

Roldan said officials would begin testing all cows older than 30 months if the tests confirm the case. So far Czech officials have conducted over 10,000 tests this year.

``HIGH RISK'' COUNTRY

The European Commission declined to comment on the Czech discovery until the results of further tests were known.

``We would need to wait for any confirmation before commenting,'' Beate Gminder, spokeswoman for EU Health and Food Safety Commissioner David Byrne, told Reuters.

On April 3 the European Commission listed the Czech Republic as a Category III country, which means that it was ''likely to present a BSE...risk, even if not confirmed, or presenting a low level of confirmed BSE risk.''

It based its assessment on the amount of live cattle and meat-and-bone meal imported into countries in question.

Other countries listed in category III are Poland, Hungary, Estonia, Lithuania, Slovakia, Cyprus, Switzerland and Albania. Switzerland is the only country outside the EU to have confirmed the presence of native cases of Mad Cow disease.

All the countries except Switzerland and Albania are candidates for EU membership.

Czech officials had urged the EU to revise the list, dropping their country from it.

The Czechs banned feeding all meat-and-bone meal to cattle in 1991 and Czech officials have said the country had not imported British bone meal, and did not feed meal from other countries to cattle because it was too expensive.

The European Commission's category II country list, where a BSE risk is deemed to be unlikely but not excluded, contained the United States, Canada, India, Pakistan and Colombia.

Category I countries -- deemed highly unlikely to contract the disease -- include Argentina, Australia, Brazil, Norway and New Zealand.


07 Jun 01 - CJD - Czech Republic Confirms Suspicion of Mad Cow Case

Reuters

SouthDakota.com---Thursday 7 June 2001


PRAGUE, Czech Republic (Reuters) - The Czech Agriculture Ministry on Wednesday said it had found the country's first suspected case of BSE, or Mad Cow disease, and was awaiting further test results to confirm the findings.

Ministry press official Hugo Roldan said the animal was from a herd in the village of Dusejov in the Jihlava region, 70 miles southeast of Prague.

``This is the first test, it is necessary to confirm the first result, so we are sending the sample to a laboratory in Germany to confirm or refute our results,'' Roldan told Reuters.

He said officials would begin testing all cattle over 30 months if the tests confirmed the case.

So far Czech officials have conducted over 10,000 tests this year for BSE, or Bovine Spongiform Encephalopathy.


07 Jun 01 - CJD - Scientists search for way to detect Mad Cow

By Tracy Wheeler, Beacon Journal medical writer

The Beacon Journal--Thursday 7 June 2001


They hope to develop blood test for disease before damage is done

CLEVELAND: On the ninth floor of Case Western Reserve University's biomedical research building, Man-Sun Sy's lab looks like any other.

With diligent precision, a young researcher squirts a bluish gel from a syringelike device into a small water-filled container. A piece of paper, about the size of a Post-It note, swishes back and forth in a petri dish next to him.

If Sy and his team of researchers find what they're looking for, a dark band will appear on the paper. And with each dark band they discover, they inch closer to providing some answers to dealing with Bovine Spongiform Encephalopathy -- Mad Cow disease.

The hope is to develop a blood test that could detect Mad Cow or its human cousin, Creutzfeldt-Jakob, before the diseases bore irreversible, microscopic holes in the brain.

Their task is not an easy one, though.

For starters, the elements that cause the diseases -- renegade proteins known as prions -- have never been found in blood. Scientists don't even know if prions can exist there.

Still, Sy, a Case Western pathology professor, takes an optimistic view.

``Just because we can't find it, doesn't mean it isn't there,'' he said. ``If it is there, we'll find it.''

A lot of people are hoping so.

Mad Cow and Creutzfeldt-Jakob now can only be diagnosed after death, through microscopic inspection of brain tissue.

With a blood test, a farmer could learn if a cow is infected before the meat goes to market.

With a blood test, a blood supply could be checked for infection before a transfusion.

With a blood test, the disease could be detected early, while it is still in its long incubation period, before the brain destruction begins.

And that, ultimately, could lead to a treatment or cure.

``If there was a test like that,'' said Case Western neuropathology professor Robert B. Petersen, ``all of a sudden there would be an incentive for the big pharmaceutical companies to develop a therapeutic because they now have a market.''

It's all a matter of that ``if'' -- if prions exist in the blood; if they can be detected.

Found in tissue

The fact that prions have been found in the appendix, the lymph nodes and tonsils leads researchers to believe prions can be found in the blood, too.

But as Petersen pointed out, prions could be more like the bacteria that cause tuberculosis -- present in tissue, but not in blood.

``A person can have it and maybe it just sits there,'' he said. ``You could never find it in the blood. You could find evidence of it in the body; that's what the skin test is all about with TB.''

In addition to the work at Case Western, other types of tests are in development around the world to detect prion diseases before the destructive proteins bore their telltale holes in brain tissue.

Some researchers are looking to radiographic images of the brain to detect prions. But this type of test would be a matter of poor timing, Sy said. Once the prions are present in the brain, the damage has begun. The same would be true with a test to detect prions in spinal fluid.

Investigating lymphatic tissue -- another diagnostic test in development -- makes more sense, Sy said, since prions have been found there. But a lymphatic tissue test would require minor surgery, and if follow-up tests are needed, this tissue is in limited supply.

So a blood test offers the most promise to the most people. Unlike a radiographic brain scan, it could detect the disease early. Unlike a lymphatic tissue test, it could be repeated regularly without incisions or risk of infection.

The body's immune system is what makes it difficult for researchers to develop a blood test for prions.

Normally, when foreign invaders, such as HIV or streptococcus bacteria, attack the body, the immune system springs into action, setting loose an army of antibodies to fight the intruder.

Those antibodies in the blood are like footprints in the sand -- clear proof of what was there before.

Unlike a virus or bacterium, prions don't trip the immune system's alarms. No antibodies are deployed; no footprints are left behind.

Prions go unnoticed, undetected and unabated.

The reason? Prions have a nearly identical chemical makeup to good protein in the body. The only difference is in the shape, the outward appearance.

``Just like you and I have the same exact genetic material but we have on different clothes,'' Sy explained.

Abnormal proteins

It's the abnormal shape that makes prions harmful. The shape allows the prion to latch onto healthy proteins one at a time, turning good proteins bad and slowly creating a colony of destructive prions.

Since researchers can't use the immune system to detect intruding prions, they must come up with another way to find them.

Sy and his Case Western colleagues have managed to do that in mice by creating a strain of rodents in which a specific protein -- known as a prion protein -- is deleted from the genetic code. When these mice are infected with prions, Sy said, ``they recognize it as foreign and create antibodies like crazy.''

The hope is that these mouse antibodies can be used to detect prions in the blood of humans and other mammals that carry prion diseases.

So far, by adding the mouse antibodies to brain tissue, the Case Western researchers have been able to locate specific proteins that most closely resemble prions.

That's not the same as finding the infectious prions, though. It will take more research -- at least a year's worth -- to move from finding proteins in brain tissue to the ultimate goal of finding prions in blood.

Researchers don't know whether any blood test will be able to detect classic Creutzfeldt-Jakob, or whether it will only be able to detect variant Creutzfeldt-Jakob, the version of the disease contracted by eating Mad Cow-tainted beef.

Dr. Pierluigi Gambetti, director of the National Prion Disease Pathology Surveillance Center housed at Case Western, expects that it will be easier to test for Mad Cow and variant Creutzfeldt-Jakob because in these diseases, ``the bad prion goes from the gut to the brain,'' meaning it has to enter the bloodstream to get there. Classic forms of the disease start in the brain.

It's also unclear how accurate a blood test could be. Prion diseases can incubate in the body for decades.

``The question that comes up, then, is whether there are prions circulating on a daily basis,'' Petersen said. ``The answer is, no one really knows.''

In other words, a negative test result might mean a clean bill of health. But it also might mean the prions are simply dormant, waiting to strike a decade or two from now.

Keeping hope alive

All this adds up to make a blood test for prion diseases seem like an impossible task. But that hasn't squelched hope.

Just ask Melissa McMillian, spokeswoman for America's Blood Centers, which won't accept blood donations from anyone who has spent at least six months in Great Britain since 1980.

``It would enable us to more accurately screen donors, so we're not (turning away) healthy donors,'' she said. ``We do not want to defer healthy donors, people who are very good donors, who enjoy donating blood, who are very upset when you tell them they can't donate because they're quite proud of it.''

Or ask Sandy Allen, of Midland, Texas, who watched her mother die of Creutzfeldt-Jakob a decade ago.

``A blood test would be fantastic,'' Allen said. ``At least you could know. At least you could get your life in order.''

If the local hospital could offer such a test, she'd be there tomorrow.

``I'd be down there first thing in the morning, for my sake and for my husband's sake,'' Allen said. ``I wouldn't want him to go through what my dad went through when Mom died.''

Tracy Wheeler can be reached at 330-996-3721 or tawheeler@thebeaconjournal.com


07 Jun 01 - CJD - BSE meat risk from abattoir culls

James Meikle, health correspondent

Guardian--Thursday 7 June 2001


Consumers might still be eating BSE infected meat because of accidental cross-contamination in some abattoirs, scientists warned last night.

They said urgent consideration must be given to the possibility that potentially dangerous material from slaughtered older cattle not destined for the food chain had been transferred to meat bound for human consumption.

The EU rules, which banned animals destined for either of the two groups of meat being killed on the same day, were probably not enough to eliminate risk, since rogue prion proteins, thought to be a key factor in both BSE and its deadly human form, were not eradicated by the conventional sterilisation procedures.

A Royal Society and Academy of Medical Sciences team queried in a report published last night the theoretical risk in eight slaughterhouses from 394 licensed for slaughtering old and young cattle.

Many of the government's independent advisers on BSE-like diseases contributed to the report. The food standards agency said it would examine the threat, which it was taking seriously.

Cattle older than 30 months, which are those considered most likely to harbour infective BSE material, have not been allowed as human food since 1996, but some abattoirs contracted to cull the animals have also produced meat for butchers and supermarkets Spot checks for the Ministry of Agriculture suggest that one in every 250 older animals slaughtered is in the later stages of BSE without having displayed outward signs of the disease. Nearly 180,000 cases of BSE have been formally identified in the UK in nearly 15 years.

Brian Heap, master of St Edmund's College, Cambridge, the chairman of the group, said last night: "According to one report, some prions may still be active even after heating to 600C, although this observation needs to be confirmed by further research... we need to establish that work surfaces and equipment in abattoirs are not contaminated."

Operations at the "dual use" abattoirs were suspended in February because of foot and mouth restrictions. Sir John Krebs, chairman of the food standards agency, said last night they would not be allowed to restart work until further checks. "There are strict rules to prevent cross contamination but these need to be re-examined [after] this report."

Concerns about shortcomings in sterilisation already have been raised with regard to contamination of patients during surgery via instruments passing on variant CJD, the human form of BSE. This has already led to huge delays in tonsil operations, mainly on children, while disposable instruments are supplied. Other reviews are being conducted into the potential risks of eye and brain operations and blood transfusions.

The working party said that the costs of further controls needed to be carefully weighed against the benefits.

Figures from the Department of Health suggest 101 Britons have now contracted variant CJD, seven of whom are still alive.

The report endorses the need for tests for detecting early signs of variant CJD in humans, BSE and similar diseases in livestock. It calls for measures to remove the backlog of remains of nearly 5m older culled cattle that have been held in storage pending incineration.

Northern Ireland has become the only UK region to gain permission to resume exports - worth 200m - of meat, dairy products, and live pigs and sheep, following Europe's lifting of restrictions linked to foot and mouth disease. The decision will be formally adopted by the EU probably no later than Thursday. The export of live sheep will start again on July 1.

The lifting of restrictions has been made possible because there have been no foot and mouth cases reported in Northern Ireland for six weeks. But exports of live cattle remain banned, and controls of livestock and produce going to Northern Ireland from the rest of Britain will stay for the time being. Controls on the movement of farm animals in the province will also stay in place.


07 Jun 01 - CJD - Weak BSE rules 'leave food chain still at risk'

By Roger Highfield, Science Editor

Telegraph--Thursday 7 June 2001


Meat sold for human consumption could become infected with Mad Cow disease because the persistence of BSE makes accidental cross-contamination at slaughterhouses possible, said a report published yesterday.

Cattle aged over 30 months, a few of which were infected with BSE, were being killed at abattoirs where meat was being prepared for human consumption, said the study by the Royal Society and the Academy of Medical Sciences.

European Union rules forbid these two activities on the same day. But this rule might not be strict enough because abnormal prions, the proteins that cause BSE, might survive sterilisation methods.

One recent American study, which has yet to be verified, suggested that some prions might remain active even after heating to 159F, said Brian Heap, the Vice-President of the Royal Society and chairman of the working group that prepared the report.

He told the Foundation for Science and Technology in London last night: "Since cattle aged over 30 months are culled because they might be infected with BSE, we need to establish conclusively that work surfaces and equipment in abattoirs are not contaminated after the usual cleaning and sterilisation procedures."

The report said more than 430,000 tons of meat and bonemeal, possibly contaminated with BSE, and a further 200,000 tons of tallow were being stored in Britain until they could be safely destroyed. Prof Heap said: "We believe that the authorities should consider eliminating this waste with meat-eating bacteria, or through incineration without air at 290F, to provide gasses that could be burnt to generate electricity."

The report highlighted how, 15 years after BSE was first identified, quick, effective and reliable tests for the disease were long overdue. It said sensitive blood and urine tests should also be developed for detecting vCJD infection early in people. Such tests would reduce the risk of transmitting infection on medical equipment during clinical procedures.

Sir John Krebs, chairman of the Food Standards Agency, said last night it would examine the risk of BSE cross-contamination because of the report. But there were only eight dual use abattoirs out of 394 in Britain, he said.


07 Jun 01 - CJD - BSE infection loophole

Interview

Channel 4 News---Thursday 7 June 2001


The Food Standards Agency has tightened its rules on abattoirs following a new report on the dangers of contamination by Mad Cow disease.

Today a group of scientists warned that we could be at risk if abattoirs used for destroying cattle over 30 years are not thoroughly disinfected before they slaughter meat intended for sale in the high street.

Their evidence could also affect doctors operating on people infected with CJD because surgical instruments might harbour the disease.

We're joined by our Science Correspondent, Andrew Veitch:

Jon Snow:

Andrew this is a bit late in the day isn't it? Why have they only found out about this now?

Andrew Veitch:

Quite. It turns out the Ministry of Agriculture has known about the abattoirs for four years.

There are eight of them which process food and also get rid of the cattle most at risk of BSE - those over thirty months old.

Obviously they're supposed to be thoroughly disinfected after chopping up the older cattle. And four years ago, Maff said that on the basis of a risk analysis from the government's science advisers, SEAC, this was OK - the risk of food being contaminated by the BSE agent was too small to worry about.

But times have changed. The Food Standards Agency now controls abattoirs - and tonight they're saying there will have to be an urgent rethink.

It happens that the abattoirs are closed at the moment because of the foot and mouth disaster - and the agency says they'll stay closed until people from SEAC and the Royal Society which drew up tonight's report can come up with a new assessment of the risk.

Jon Snow:

And the new report is warning of a possible risk of getting the human form of BSE from surgical instruments - I seem to remember the Department of Health saying it was going to put hundreds of millions into new decontamination centres, and it was going to pay for disposable instruments?

Andrew Veitch:

Disposable knives for taking out tonsils, which are among the tissues most likely to be infectious, and 200 million to decontaminate instruments.

Sadly the disposable knives have yet to arrive - and tonight's report says there needs to be an urgent study of the "theoretical risk" that hospital decontamination procedures might not be enough because one study has shown the infective agent can survive the amazingly high temperature of six hundred degrees centigrade.

Now that finding needs to be checked out, but the warning will be taken seriously because it comes from some of country's leading experts on BSE.

Now the hospitals do point out that if knives are properly decontaminated, the potentially infectious bits will be washed off before they're heated and sterilised.

But Professor Heap's report does show how little we still know about this disease - After all a hundred and one people have developed it, and we still don't know how many are going to get it.


06 Jun 01 - CJD - 'Meat for humans could still have Mad Cow infection'

Ananova

PA News--Wednesday 6 June 2001


Meat sold for human consumption could be infected with Mad Cow disease because of accidental cross-contamination at slaughterhouses, experts have warned.

Scientists say urgent action is needed to investigate the theoretical risk of abattoir premises and equipment being contaminated with BSE .

The experts, stressed that normal cleaning and sterilisation procedures may not be enough to prevent cross-infection at abattoirs where at-risk cattle are culled.

Cattle over the age of 30 months, which may be infected with BSE, are routinely slaughtered and prevented from entering the human food chain, claim the scientists from the Royal Society and the Academy of Medical Sciences.

A number of slaughterhouses contracted to carry out the culling also produce meat destined for butchers and supermarkets.

European Union rules forbid both activities taking place on the same day.

But in their report, Transmissible Spongiform Encephalopathies, the scientists voice concern that cross-contamination might still occur.

Professor Brian Heap, vice-president of the Royal Society, who chaired the working group, said: "Urgent consideration needs to be given to the possibility of cross-infection in the few abattoirs in the UK that handle both the slaughter of animals for food and the culling of cattle aged over 30 months that may be incubating BSE."

He said there was some evidence that prions - the protein agents thought to transmit BSE and related diseases - may still be active even after heating to 600C.

"Since cattle aged over 30 months are culled because they might be infected with BSE, we need to establish conclusively that work surfaces and equipment in abattoirs are not contaminated after the usual cleaning and sterilisation procedures," he said.


06 Jun 01 - CJD - Report raises new fears over BSE

Staff Reporter

ITN--Wednesday 6 June 2001


"We need to establish conclusively that work surfaces and equipment in abattoirs are not contaminated after the usual cleaning and sterilisation procedures" - Professor Brian Heap

Meat sold in shops, supermarkets and restaurants could be infected with Mad Cow disease at abattoirs, a group of scientists has warned.

The experts, from the Royal Society and the Academy of Medical Sciences, fear that normal cleaning and sterilisation procedures may not be enough to prevent cross-infection at abattoirs where both at-risk and uninfected cattle are killed.

In their newly published report, they call for an urgent investigation into whether premises and equipment at slaughterhouses which handle both types of cattle could be contaminated with Bovine Spongiform Encephalopathy.

The Consumers' Association said the Food Standards Agency should urgently investigate the concerns raised by the report and ensure that any risk of cross-contamination in slaughterhouses is prevented.

"Given the spread of BSE across Europe, it's also essential that the European Commission acts on the concerns that have been highlighted," the Association added.

"This report reiterates the importance of constantly keeping potential routes of infectivity and BSE control measures under review."

Cattle over the age of 30 months, which may be infected with BSE, are routinely slaughtered and prevented from entering the human food chain.

A number of slaughterhouses contracted to carry out the culling also produce meat destined for butchers and supermarkets. European Union rules forbid both activities taking place on the same day.

Professor Brian Heap, vice-president of the Royal Society, who chaired the work, said: "Urgent consideration needs to be given to the possibility of cross-infection in the few abattoirs in the UK that handle both the slaughter of animals for food and the culling of cattle aged over 30 months that may be incubating BSE."

He said there was some evidence that prions - the protein agents thought to transmit BSE and related diseases - may still be active even after heating to 600C.

"Since cattle aged over 30 months are culled because they might be infected with BSE, we need to establish conclusively that work surfaces and equipment in abattoirs are not contaminated after the usual cleaning and sterilisation procedures," he said.

The report, Transmissible Spongiform Encephalopathies, also highlights the risk posed by the backlog of potentially infected material building up in storage hangers.

More than 430,000 tonnes of meat and bone meal and a further 200,000 tonnes of tallow are currently awaiting safe disposal in the Britain .

"There is always a danger of leaks into the environment through, for example, infestation of rodents or invertebrates," said the report.

The experts said consideration should be given to eliminating the waste using meat-digesting bacteria or high temperature incineration without air at 850C.

The cost of the latter solution could be cut by using the gases to generate electricity.


06 Jun 01 - CJD - BSE 'cross-infection' risk

Staff Reporter

BBC--Wednesday 6 June 2001


Meat sold for human consumption could be infected with BSE because of accidental cross-contamination at slaughterhouses, warns a group of leading scientists.

Experts are calling for urgent action to investigate the theoretical risk of UK abattoirs being contaminated with Mad Cow disease.

Routine cleaning and sterilisation procedures might not be enough to safeguard premises which cull cattle at risk of BSE and supply meat to butchers, says a new report.

Scientists from the Royal Society and the Academy of Medical Sciences, who published the Transmissible Spongiform Encephalopathies report, are calling for new measures to prevent any risk of cross-infection.

Cattle over the age of 30 months, which might be infected with BSE, are routinely slaughtered and prevented from entering the human food chain.

Contamination fears

Some slaughterhouses contracted to carry out the culling also produce meat destined for butchers and supermarkets.

Abattoirs are forbidden from carrying out both activities on the same day, under European Union rules.

But this does not rule out the possibility of cross-contamination, according to the report's authors.

BSE fears have led to a decline in beef sales

Brian Heap, vice-president of the Royal Society, said: "Urgent consideration needs to be given to the possibility of cross-infection in the few abattoirs in the UK that handle both the slaughter of animals for food and the culling of cattle aged over 30 months that may be incubating BSE."

He said there was some evidence that prions - agents thought to transmit BSE and related diseases - might still be active even after heating to 600 C.

"Since cattle aged over 30 months are culled because they might be infected with BSE, we need to establish conclusively that work surfaces and equipment in abattoirs are not contaminated after the usual cleaning and sterilisation procedures," said Professor Heap, who chaired the working group.

Disposal backlog

The report also highlights the risk posed by the backlog of potentially-infected material building up in storage hangers.

More than 430,000 tonnes of meat and bone meal, and a further 200,000 tonnes of tallow, are currently awaiting safe disposal in the UK.

"There is always a danger of leaks into the environment through, for example, infestation of rodents or invertebrates," says the report.

The experts say consideration should be given to eliminating the waste using meat-digesting bacteria or high temperature incineration without air at 850 C.

The Consumers' Association says the Food Standards Agency should urgently investigate the concerns raised by the report and ensure that any risk of cross-contamination in slaughterhouses is prevented.

"Given the spread of BSE across Europe, it's also essential that the European Commission acts on the concerns that have been highlighted," the Consumers' Association adds.

"This report reiterates the importance of constantly keeping potential routes of infectivity and BSE control measures under review."


06 Jun 01 - CJD - New Risk Of Mad Cow Disease

Reuters

YAHOO--Wednesday 6 June 2001


Cross-contamination

Normal cleaning and sterilisation may not be enough to prevent cross-infection at abattoirs where at-risk cattle are culled, experts from the Royal Society and the Academy of Medical Sciences stressed.

Cattle over the age of 30 months, which may be infected with BSE, are routinely slaughtered and prevented from entering the human food chain.

But a number of slaughterhouses contracted to carry out the culling also produce meat destined for butchers and supermarkets. European Union rules forbid both activities taking place on the same day.

Urgent action

Professor Brian Heap, vice-president of the Royal Society, who chaired the working group, said: "Urgent consideration needs to be given to the possibility of cross-infection in the few abattoirs in the UK that handle both the slaughter of animals for food and the culling of cattle aged over 30 months that may be incubating BSE.

"Since cattle aged over 30 months are culled because they might be infected with BSE, we need to establish conclusively that work surfaces and equipment in abattoirs are not contaminated after the usual cleaning and sterilisation procedures," he said.


06 Jun 01 - CJD - CJD gaining attention in United States

By Tracy Wheeler, Beacon Journal medical writer

The Beacon Journal--Wednesday 6 June 2001


Experts keep watch as deadly variant spreads in Europe

In the middle of August, June Burdick simply stopped feeling well.

By Labor Day, her walk had taken on a noticeable wobble. And she was talking nonstop -- something out of character for this reserved 73-year-old resident of rural Nebraska.

Within days, her speech became slurred, her walk grew clumsier and her left arm turned uncontrollable, flinging itself into the air as if shooing away flies.

The CAT scans, bone scans, MRIs, vital signs and spinal taps showed nothing unusual.

By mid-September, doctors diagnosed Burdick as having a stroke, ``simply because they couldn't find anything else wrong with her,'' said her daughter, Marianne Fisher. ``They did a test for everything they could think of, then redid them again. The doctors were as frustrated as we were. They were running out of tests to do. They even tested her for Lyme disease.''

Burdick's behavior grew increasingly bizarre over the next two weeks. She laughed at everything, as if drunk. She sat in her bed, pretending to conduct an orchestra. She feared the dark, along with the colors blue and green.

By Oct. 8 -- less than six weeks after the symptoms began to show -- Burdick was dead. It wasn't until four days before her death, after a fOur Worldhour trip to see a doctor in Fort Collins, Colo., that her family finally began to learn what was wrong.

``The doctor walked in... and within five minutes, we had a probable diagnosis of CJD,'' Fisher recalled. ``Of course, we'd never heard of it.''

Mad Cow gets attention

Many people are unfamiliar with Creutzfeldt-Jakob disease -- CJD for short. But it's gaining attention in the United States as a new version -- variant Creutzfeldt-Jakob (vCJD) -- spreads through Europe, leaving a rising death toll in its wake.

This variant was discovered in Great Britain in 1996. Scientists concluded it was caused by the consumption of beef tainted by Mad Cow disease.

Both Mad Cow and Creutzfeldt-Jakob are part of a family of neurological diseases called transmissible spongiform encephalopathy. Scrapie in sheep and chronic wasting disease in deer and elk also are part of this disease family.

Scrapie has never passed CJD to humans, but three CJD deaths -- two hunters and a person who ate venison -- have been linked to infected deer and elk in Colorado, Wyoming, Nebraska, Montana, South Dakota and Oklahoma.

In all these diseases, abnormally shaped proteins called prions ravage the central nervous system. Prions are virtually indestructible and invisible to the body's immune system.

In some cases, prions are believed to enter the body through tainted meat or surgical equipment, slowly latching onto and altering healthy proteins. In other cases, prions form on their own.

``Through the course of your lifetime, you make bad proteins and your cells know how to degrade them,'' said Robert B. Petersen, an associate professor of neuropathology at Case Western Reserve University. ``They identify them, shunt them off, degrade them, and start over without (allowing) any type of pathological process. When you age, apparently these mechanisms that do the surveillance -- the cellular cops -- break down. They're just not as efficient.''

When that system fails and prions thrive, disaster follows. Though the prions can be dormant for decades, they will eventually gnaw away at the nervous system, drilling microscopic holes throughout the brain.

The first sign of both CJD and vCJD is blurred vision or a loud ringing in the ears, followed by a loss of balance, arm and hand tremors and dementia. Death is a certainty.

The classic form of Creutzfeldt-Jakob typically strikes between the ages of 50 and 75, after decades of lying dormant in the body. In 85 percent of the cases, the cause is unknown. About 10 percent to 15 percent of the cases have a genetic origin and about 1 percent are contracted through medical procedures. Death occurs quickly once symptoms appear, often within three to six months.

The victims of vCJD are much younger, with 28 being the average age of onset. And the symptoms, while similar, last longer, up to 13 months until death.

``It's just such a horrendous thing to see somebody's mind go so fast,'' Fisher said of her mother. ``Thank God she didn't linger.''

On the lookout

If variant Creutzfeldt-Jakob ever reaches the United States, Dr. Pierluigi Gambetti will be one of the first to know.

Gambetti is director of the National Prion Disease Pathology Surveillance Center, housed at Case Western Reserve University. The center tracks and investigates suspected cases of CJD and vCJD in the United States.

Since the center opened in 1997, 480 cases have been investigated, with 294 diagnosed as CJD. None was identified as vCJD.

However, Gambetti said not every suspected case of CJD or vCJD makes its way to the center. In some cases, the doctors simply don't recognize the symptoms and misdiagnose the death as a stroke or Alzheimer's. In others, doctors suspect CJD, but the family refuses to permit an autopsy for personal, religious or financial reasons. And in still other cases, pathologists are too scared to perform autopsies, fearing exposure to the deadly prions.

It's estimated that CJD is the cause of one to two deaths out of a million. Based on a U.S. population of 284 million, Gambetti expects to find a little more than 400 cases of CJD each year. Instead, he saw only 111 in 2000, about a fourth of what was expected.

``The chance of losing an important case is high,'' he said. ``I won't be completely happy until we reach 80 percent, at least.''

Two studies have found that doctors often don't recognize CJD. One by Yale University found that 13 percent of suspected Alzheimer's patients had actually died of CJD. Another at the University of Pittsburgh showed that 5 percent of Alzheimer's patients were CJD victims.

Devastating illness

Like Burdick's family, Kate Foster, of Killeen, Texas, had to wait weeks for doctors to recognize CJD when her 62-year-old husband became ill. Marvin Foster's symptoms surfaced last May, shortly after shoulder surgery. A diagnosis didn't come for two months.

A former paratrooper, Marvin Foster was a strong, vibrant man. But for five months before he died on Jan. 24, he couldn't walk, talk or swallow.

``Those were the hardest months,'' Kay Foster said. ``Myself and my children were in denial. We couldn't accept the fact that he had this and there was no cure. One doctor along the way said he had encephalitis and give it a year and he would get better. We hung on to that.... ''

``We had never heard of this before he was diagnosed.''

And those who have heard of it are often confused by a diagnosis of CJD, thinking that it means a loved one has Mad Cow disease. For a death to be linked to Mad Cow, however, an autopsy would have to find vCJD.

Epidemiologists can quickly tell the difference between the two.

Under a microscope, classic CJD would show up in brain tissue as tiny holes. In vCJD, the holes would be surrounded by blotchy white patches of protein referred to as plaques.

If such a sample were to arrive at Case Western Reserve University, ``it would jump out at me,'' Gambetti said.

Believing in cover-up

Sandy Allen, who lives on a small farm in Midland, Texas, isn't reassured that scientists have not found a case of vCJD in the United States.

She believes that both vCJD and Mad Cow are already here -- and that the government is covering it up.

``I don't know if the government will ever fess up to it,'' she said. ``I like to say, `They'll do it when the Mad Cows come home.' ''

Her concern about Mad Cow disease is understandable. Her mother died of CJD 11 years ago, and every time she hears a ringing in her ear or stumbles over her feet, she worries that she has been stricken, too.

``Oh, I think about it all the time,'' she said. ``I'm not worried about cancer. I'm not worried about heart disease. I'm deathly worried about CJD. I'll do myself in. I'm not going to go through that.

``How do I know I didn't eat what my mother ate? You don't know what's in sausage anymore. You don't know what parts of the animal are used for what.''

On the other hand, Fisher doesn't think it really matters whether vCJD is in the United States. CJD is here, killing people.

``When you say there's no (Mad Cow) in the United States,'' Fisher said, ``I don't think people understand that's not the same as saying there's no CJD in the United States.''

Tracy Wheeler can be reached at 330-996-3721 or tawheeler@thebeaconjournal.com


06 Jun 01 - CJD - On the USDA's Front Line Against Mad Cow Disease

Marc Kaufman

Washington Post--Wednesday 6 June 2001


On the USDA's Front Line Against Mad-Cow Disease; Detwiler Key to Keeping Illness Out of Country

For almost five years now, U.S. Agriculture Department veterinarian Linda Detwiler has been on a daily lookout for mad-cow disease and all its related afflictions of sheep, elk and people.

She has traveled to meet European Union officials in Brussels so many times she knows exactly which flights will let her make the round trip in a day. She has spent time in countless slaughterhouses, rendering plants and feedlots in every state except Hawaii and Alaska.

So far, the work of Detwiler and those alongside her in other federal agencies has succeeded: The disease, which has caused almost 100 deaths in Europe and devastated the British beef industry, has never been found in American cattle or people.

Detwiler, who leads the mad-cow working group at USDA's Animal and Plant Health Inspection Service (APHIS), has played a key advisory role in the development of national policy on mad-cow and related diseases, and in 1997 recommended a controversial ban on importing all European cows, sheep and goats -- years before they were found to be infected outside the United Kingdom.

More recently, she was at the center of a bitter dispute over the March confiscation of two flocks of European-born sheep being raised in Vermont. Detwiler was convinced the sheep had to be destroyed because there was a possibility, however slight, that they were carrying the mad-cow agent.

But the flock owners and their supporters believed the government was overreacting. During the final court-ordered seizure, protesters carried signs denouncing "Dr. Deathwiler."

For the no-nonsense Detwiler, raised on a New Jersey hog farm and enamored of farm animals all her life, the criticism hurt and led to some restless nights. It also had a certain irony to it -- Detwiler has spent much of her government career working to protect sheep, and is considered a top expert in that small and specialized field. In fact, she is leading the mad-cow effort today largely because she knows so much about sheep.

"After I graduated veterinary school, I went to work with the government on the disease scrapie in sheep," she said, referring to another brain-wasting condition. "It was quite a backwater and I remember one of my teachers telling me I could do better. I swore to myself I would do it only a year."

But little-known scrapie turned out to be central in the spread of mad-cow disease. Many believe Mad Cow was created and spread by feeding parts of scrapie-infected sheep brains and nervous tissue to cows, which were later slaughtered and consumed by people. So 15 years after she learned the ins and outs of scrapie as a USDA vet in central Ohio, Detwiler's somewhat arcane speciality has enormous international relevance.

Mad-cow disease, or Bovine Spongiform Encephalopathy, was first detected in cows in the United Kingdom in the late 1980s, and elsewhere in Europe last year. Scientists initially believed it could not cross the "species barrier," but it was found in humans in 1996. "Since then," Detwiler said, "my life has never been the same."

Although the number of people harmed by mad-cow disease worldwide has been small, its initially mysterious origins, the fact that it can spread and its deadly consequences for humans have kept it in the public eye.

The disease has decimated the European beef industry -- millions of cattle have been destroyed, and sales were off 35 percent last year -- and there has been enormous pressure to keep it from doing the same here. The fact that British, and later European, health officials misunderstood the disease for years, and didn't stop its early spread, has made the job of keeping it out of the United States even more pressing.

"The lesson here is to be aggressive about limiting all risks," Detwiler said. "That means known risks, and theoretical ones, too."

Referring to the Vermont sheep, she said it is always difficult to "depopulate" a herd. But living on a farm as a child, she quickly learned that sometimes individual animals had to be sacrificed for the general health of the group. "I grew up with hog cholera, so I know how it feels to lose some animals to protect the others," she said.

In dealing with a danger such as mad-cow disease, perception of risk can be as important as the risk itself. So part of the federal effort -- and Detwiler's job -- has been to explain time and again that mad-cow disease is not present in the United States, and that if it was ever discovered, it would be quickly contained.

The effort has been generally praised, although a recent editorial in the New England Journal of Medicine called for greater federal scrutiny, saying the USDA has not done enough testing of U.S. cattle. Detwiler, recently back from a week in Europe observing mad-cow surveillance there, said she would like to see more testing, too.

Caroline Smith DeWaal of the consumer group Center for Science in the Public Interest has often criticized federal policies on mad-cow disease and other Food Onlineborne illnesses, but says she has been impressed by Detwiler's work at APHIS.

"In retrospect, their actions were critical in preventing the disease from coming to the United States," DeWaal said.

Working at such a visible public job is nothing Detwiler ever intended to do. That's hardly surprising, given the image of government work for veterinarians when she was a student.

"I travel a lot and talk at conferences and with students," she said. "I tell them something I certainly never heard when I was coming up -- that working for the government can be very challenging and really quite rewarding."


06 Jun 01 - CJD - Mad Cow fears put renderers under microscope

Mark Kennedy

Montreal Gazette---Wednesday 6 June 2001


First, there was the smell, a blend of garbThe Agebin and barnyard.

It hit us the moment we pulled into the parking lot of Lomex, an industrial plant in Montreal's north end.

Then, there was a thunderous bang every few minutes. The kind you hear in duck-hunting season. KABOOM!

"Sorry," Lomex president Martin Couture would later say, after providing a 20-minute, gut-wrenching tour inside the plant. "We have a problem with seagulls."

The solution: a cutout figure of a hunter in the back of a truck, whirling from side to side, with the occasional blast adding to the effect.

Cosmetics to Plastics

Lomex runs a rendering plant in the city's Riviere des Prairies district, where leftover parts of slaughtered animals are sent. Twenty-four hours a day, trucks arrive from across Quebec, the Ottawa Valley, and northern Vermont.

Everything deemed unfit for human consumption - the stomach, the offal, the organs, feathers - is ground, boiled and turned into products.

Some remains are rendered into tallow, used in the manufacture of cosmetics, soaps, shampoos, paints, candles, tires, perfumes, textiles, plastics, inks, polishes, cleaners and solvents.

Much of the rest becomes a reddish-brown powder called meat and bone meal (MBM). Rich in protein, it is sold as a supplement for animal feed. It can be legally fed in Canada and the U.S. to pigs and poultry.

For decades, cow-based MBM was also fed to cows. But that was prohibited in Canada and the U.S. in 1997, once it became apparent that the practice spread mad-cow disease in Britain.

Now, with new concern that mad-cow disease could spread world-wide, the spotlight is on rendering companies - a business so little-known it was once called the "invisible industry."

There are calls for Canada and the U.S. to match the precautions taken in Britain and Europe. There, MBM is banned in feeds given to all animals - not only ruminants like cows and sheep, but also pigs and poultry.

Critics say the ban should be extended here for at least two reasons:

- What if mad-cow disease, known as Bovine Spongiform Encephalopathy (BSE), can be spread to pigs and poultry? Scientists know that some humans who eat BSE-infected meat contract the fatal neurological disorder known as Creutzfeldt-Jakob disease (vCJD).

In one experiment, a pig got BSE after its brain was injected with the contaminant. But there have been no reported cases of non-ruminants showing mad-cow symptoms just from eating MBM (a fact critics dismiss, because the animals are likely slaughtered before BSE's incubation period - four to seven years in cows - runs its course).

- Even if pigs and poultry can't get BSE, what if MBM produced for pigs and poultry accidentally gets into cow stalls? It's a potentially disastrous problem called "cross-contamination," one that experts say contributed to the spread of BSE in Britain and Europe.

Author Wants Complete Ban

Among those urging the federal government to impose a full ban is William Leiss, president of the Royal Society of Canada, and co-author of Mad Cows and Mother's Milk, a book on Britain's response to the threat in the 1980s.

"Stop recycling animal protein," he says. "All of it. Period. That's the answer, because of what we know. The extraordinary toughness of this agent. You can't kill it. It survives autoclaves. It survives chemicals and disinfectants."

But Humphry Koch, Vancouver-based vice-chairman of the National Renderers Association, says such a move is unnecessary and could cause new problems. Koch says Canada's 26 plants that produce MBM, owned mostly by three companies, receive about 3 billion pounds of animal waste a year.

"If we weren't there, everybody would know it. From an environmental point of view, we provide a tremendous service. And at the end of it, we produce useful products. And we believe they're safe."

If you couldn't feed MBM to animals, Koch adds, you'd still have to dispose of it. "What do you do with it? Do you put it in garbage dumps, where you'd have rats and all the other stuff? Do you burn it? If you do that, there are environmental consequences."

This fall, Koch will become chairman of the National Renderers Association, the trade group representing the industry in Canada and the U.S.

He says the industry - which calls itself "the original recyclers" - has been around since "biblical times."

"I guess everybody wonders, could it happen here?" he says of the BSE crisis that has enveloped Europe.

"My response to that is that we have very strong safeguards in place. And if there are better ways of doing it, we're open to looking at them. But we should do it in a scientific manner, not in an emotional manner."

Dr. Brian Evans, chief veterinarian at the Canadian Food Inspection Agency, says the government regulator is considering whether to extend the ban on MBM to all animals.

But he agrees that society should consider the consequences. If the meat and bone meal is incinerated, for instance, there could be increased levels of air-borne dioxins to worry about, as well as fallout on grazing lands. Still, he said, it's a valid issue for public debate.

U.S. Found Problems

Critics like Mike McBane, of the Canadian Health Coalition, say the risk of cross-contamination poses a major threat. He notes, for instance, how in January of this year the U.S. Food and Drug Administration issued a report that many of that country's feed mills were not following rules to prevent cross-contamination.

Shortly afterward, U.S. regulators determined that 1,222 Texas cattle ate a small amount of meat and bone meal after a mill accidentally shipped the wrong feed. There's no evidence it was BSE-contaminated. But that's not the point, say critics. It could have been.


06 Jun 01 - CJD - Agencies work to keep Mad Cow disease out of U.S. pastures

By Erin Heath, National Journal

GovExec.com---Wednesday 6 June 2001


"Mad-cow disease" is a phrase that often evokes wisecracks. Europe has had to shoulder huge financial losses from the disease's devastating effects, but the image of a frenzied cow shaking and mooing uncontrollably still elicits chuckles, even there. In fact, crazy-cow miniature toys were all the rage in France last Christmas.

Mad Cows, however, are no laughing matter for the U.S. officials who are charged with maintaining animal health and food safety. Their job is to keep out the disease that has been responsible for the deaths of hundreds of thousands of European cows and, scientists believe, almost a hundred people.

Indeed, U.S. officials have their work cut out for them. In January, about 1,000 Texas cows were quarantined after officials discovered they might have eaten animal feed banned because of its links to mad-cow. And in March, federal agents seized a flock of sheep in Vermont that had been quarantined for two and a half years because some of the sheep showed signs of having a brain disease related to mad-cow.

Here's some background: The technical name for mad-cow disease is Bovine Spongiform Encephalopathy, or BSE. It's part of a little-understood group of diseases called transmissible spongiform encephalopathies, or TSEs, which are characterized by the way they create spongelike holes in the brain. TSEs are rare, but they are found in a number of species.

Scientists discovered mad-cow 15 years ago in Britain. The number of affected cattle grew, peaking in 1993 at 1,000 new diagnoses a week. The outbreak, of course, wreaked havoc on the beef industry. But the real problems for beef producers didn't start until 1996, when the British government released a report linking mad-cow to 10 human deaths.

Scientists compared the symptoms of these 10 people with those caused by Creutzfeldt-Jakob disease, a rare human TSE that occurs in one in a million people a year worldwide. But unlike Creutzfeldt-Jakob victims, who usually are age 55 or older, these 10 were much younger, some even in their teens.

Scientists decided their illness was a new, separate TSE they dubbed "new variant Creutzfeldt-Jakob" disease, or vCJD for short. Like the classic Creutzfeldt-Jakob, vCJD destroys the brain and is invariably fatal. It has an exceptionally long incubation period, lasting from five to 20 years.

Researchers soon found evidence that those who died of vCJD had gotten it by eating beef tainted by mad-cow disease. The European Union banned British beef exports for three years. The market for beef plummeted.

The majority of cattle that have succumbed to mad-cow have been from Britain, but cases have also cropped up in about a dozen other European countries. As of early February, 98 people have been diagnosed with vCJD-94 from Britain, three from France, and one from Ireland.

How does mad-cow spread, and how did it get into the meat sold in grocery stores and restaurants? The most likely explanation isn't for the faint of stomach. After cows are sent to slaughter, the meat is removed and the leftover scraps, such as bones, hooves, and organs, are cooked and melted in a process called rendering. This stew is then added to animal feed. Issues of cow cannibalism aside, proponents of rendering call it an efficient way to give cattle protein and to use all of the leftover bits from a slaughtered cow.

The mad-cow agent (it's not exactly a virus or bacterium) is thought to reside mainly in the bovine brain or spinal cord. Researchers believe that the brains and spinal cords of infected cows were mixed into feed given to other cows, thereby spreading the disease. Parts of the infected cows could also have made their way into the human food chain, causing vCJD.

Mad-cow disease and vCJD have never been found in the United States. The Agriculture Department and the Food and Drug Administration--as well as officials from U.S. Customs, the Centers for Disease Control and Prevention, and other agencies--have teamed up to try to keep the disease out.

The USDA has been relatively ahead of the game on imports. In 1989, the United States became the first country to ban the importations of live ruminant animals--those that chew a cud, such as cows, goats, and sheep--and ruminant products from countries where mad-cow had been discovered. The department expanded the ban in 1997 to include all European countries, regardless of whether they had the disease.

The FDA also did away with cow cannibalism in 1997, banning farmers from feeding to ruminants animal protein that is derived from ruminants and most mammals. In December, the USDA prohibited the import of all rendered protein from all European countries. And the FDA has forbidden blood donations from anyone who spent six months in Britain between 1980 and 1996.

Three studies--one done by the USDA in the early 1990s, one done last year by the European Union, and one to be released in June by Harvard University--predict that the risk of mad-cow showing up in the United States is slight. But because so little is known about the disease, it can't be written off, said Linda Detwiler, a senior staff veterinarian at USDA's Animal and Plant Health Inspection Service.

The USDA performed 11,954 cattle inspections from 1990-2000. (Officials must euthanize the cows to check their brains for BSE.) But critics say this number is not enough. "We do think that the government could do more," said Peter Lurie, deputy director of Public Citizen's Health Research Group. The USDA's goal is to increase the number to 5,000 cattle inspections annually, and to do them at more locations.

Lurie estimates that less than 1 percent of products subject to FDA rules are physically inspected at U.S. borders. Stephen Sundlof, the director of the FDA's Center for Veterinary Medicine, acknowledged that only a small percentage of imported products are physically inspected, but he said that records of all products that can contain material related to mad-cow, such as live animals, animal feed, and food products, are checked. Problems can arise, however, "if a substance is misdeclared, either accidentally or intentionally. Then the chance of it being picked up in a routine inspection is relatively rare," he said.

The FDA plans to increase the number of inspectors at the ports and at feed mills, Sundlof said. In January, the FDA revealed that in a two-year inspection of more than 9,100 feed firms, close to 1,700 firms were not even aware of the 1997 feed ban. Of the firms that handled prohibited material, 28 percent did not include a label on their products cautioning that the feed should not be given to cows or other ruminants. Since then, more firms have been inspected and more have complied with the rules.

Lurie and others have also questioned certain meat-processing methods and practices that could cause brain and spinal cord tissue to enter the meat supply. One practice that has already been reformed is the use of pneumatic stun guns.

Researchers found that the guns, which beef producers use to put down cattle before slaughter, sometimes caused bovine brain matter to go into other parts of the cow's body. This happened less than 2 percent of the time, according to a study funded by the National Cattlemen's Beef Association and the American Meat Institute. But the risk was enough to cause beef producers to reconsider using the stun guns, said Gary Weber, the beef association's executive director of regulatory affairs. "In a very short amount of time, the [beef] companies stopped using them, and the manufacturers stopped making them and offered alternative products," he said.

Cow parts aren't used only in animal feed. They're found in more places than people realize, from soap to sweets to sporting goods. A small number of dietary supplements use cow brain and glandular material, Lurie said. But the FDA hasn't been able to regulate them since the Dietary Supplement Health and Education Act became law in 1994. "Dietary supplements are the Wild West out there at this point," he said.

Some vaccines--such as polio and diphtheria, tetanus, and pertussis (DTP), which are required for children to enter many public schools--also use bovine material such as cow blood. Since 1993, the FDA has issued a couple of guidance letters asking vaccine companies not to use material from cattle raised in countries at risk of mad-cow disease. But the FDA found out last year that at least five companies had not complied.

Still, government officials decided not to pull the vaccines off the market because the chances of anyone getting a mad-cow-related disease are "remote and theoretical." Lurie concurred, but said the Bush Administration should take a stricter, better-safe-than-sorry approach.

Europe's mad-cow crisis hasn't lowered demand for beef in this country. But the impact would be devastating if mad-cow were to be discovered in American cattle, Weber said. "It only takes one case," he said. "Beef purchases would probably go down between 30 and 50 percent." Think of it this way, he said: Every 1 percent of consumer demand equates to about $350 million a year just in farm and ranch income. A 30 percent decline in demand would mean a loss of $10.5 billion to the beef industry.

Because of the level of uncertainty surrounding mad-cow, and because of the 100 percent fatality rate of its human counterpart, vCJD, mad-cow disease will continue to be a hot-button issue. Federal officials, meanwhile, will continue to judge their success on the number of Mad Cows found in the United States: zero.


06 Jun 01 - CJD - Biologists warn of disease among deer

Bill Graham

Kansas City Star--Wednesday 6 June 2001


Biologists in Missouri and Kansas are watching deer herds for signs of chronic wasting disease - an illness similar to Mad Cow disease.

The Missouri Department of Conservation on Thursday asked for the public's help in watching for listless or sickly looking deer, so those animals can be tested for chronic wasting disease.

"This is precautionary," said deer biologist Jeff Beringer. "Hopefully we will never see it."

But the disease seems to be slowly spreading among deer in Colorado, Wyoming and western Nebraska.

Chronic wasting disease, unlike Mad Cow, does not harm humans. [The little data we have actually show quite the reverse--that chronic wasting disease prions from deer and elk can indeed affect human brain tissue--BSE coordinator] And the disease is not known to affect livestock.

But it is fatal to deer. It's known as a transmissible spongiform encephalopathy disease, which results from mutated proteins that cause a degeneration of the brain. Biologists are unsure how the disease spreads.

If the disease does arrive in Missouri or Kansas, the best hope of containing it will be to quickly identify it and reduce deer numbers in that area. That's why the public is being asked to keep watch for sickly deer.

"The best thing is to try and keep it out of your state," Beringer said. "If it does start, the next best thing is to try and contain it."

The department also will be conducting random tests on animals killed during next fall's deer hunting season to watch for the disease.

Kansas is also monitoring for chronic wasting disease, said Lloyd Fox, big game biologist for the Kansas Department of Wildlife and Parks. About 300 deer were tested during the past two hunting seasons.

The disease has been most prominent in mule deer, which are found in western Kansas. But whitetail deer, found in Missouri and eastern Kansas, can also get the disease, Fox said.

Deer with the disease will be thin, appear weak, salivate excessively, have drooping ears and appear to be unafraid of humans. How to help

Anyone spotting a deer with these symptoms in Missouri should contact a local conservation agent or call Jeff Beringer at 1-(573) 882-9880, Ext. 3211. In Kansas, contact a local conservation agent or call Lloyd Fox at 1-(620) 342-0658