Prion disease
Mad Cow Home ... Best Links ... Search this site

Family grief at CJD victim's death
Jennifer Cooke: kuru deaths continue in 1999
Family of CJD victim awarded damages
Mountains of rotting cattle discovered
Search for CJD blood test
Development of Proprietary Reagents for Diagnosis of Mad Cow Disease
Call for government action as sheep prices plummet
EU scientists: no guarantee US is BSE-Free
How a scientist obtained thousands for a BSE test he could not prove
Head blow clue to Alzheimer's
France won't import British beef just yet

Family grief at CJD victim's death

Fri, Sep 3, 1999 By Melanie Harvey, PA News
The family of a teenager believed to be Britain's longest sufferer of CJD spoke today of their grief at her death. Donna Marie McGivern, 17, died at home in Coatbridge, Lanarkshire, last night in the arms of her devoted parents, James and Marie. They had both given up their jobs to care for their daughter after they were told a day after her 15th birthday she was almost certainly suffering from the human form of mad cow disease.

Today the couple were being comforted by their close-knit family at the house where they tried in vain to help their daughter battle against her debilitating condition. William Cowie, Donna Marie's uncle, described his late niece as "a diamond" and paid tribute to her for the way she fought the illness. "Donna Marie put up a very brave battle all the way through," said Mr Cowie, who travelled from his home in London last night to be with Mr and Mrs McGivern.

"All the family are gathering to be with Marie and James and they are obviously still in shock and trying to come to terms with what has happened." Mr and Mrs McGivern compiled a dossier about their daughter's illness for the House of Commons committee, outlining how over 15 months their daughter deteriorated from a lively, active girl to an invalid.

Mr Cowie said Donna Marie's parents had wanted to try to ensure their family's experience would help other sufferers. "They wouldn't wish it on any other family and if it did happen to anyone else they would hope they would get more help and support from the authorities," he said. "At the moment they are still in shock and being comforted by their family. We are all coming together to support them."

A post mortem examination was expected to be carried out in Edinburgh today on the body of the teenager. Donna Marie had won medals for athletics and dancing at school but started suffering pains in her legs and became prone to bouts of moodiness at the beginning of 1997. Her concentration during classes at St Ambrose High School, Coatbridge, began to drift and teachers became concerned about her.

Then her eyesight started to fail. Donna Marie went to her GP, who referred her to the Southern General Hospital in Glasgow and tests were carried out to see if she was suffering from a brain tumour. These were negative. Eventually her condition worsened, she was unable to walk and doctors told the McGiverns their daughter was suffering from a degenerative brain disorder, probably CJD.

Doctors then delivered the heartbreaking news that she could not be cured. The teenager was left unable to speak and as the illness worsened could only lie on her bed, with her parents always nearby. Donna Marie's case was examined by MPs as part of their inquiry into BSE.

nv CJD victim loses battle for life

BBCNews UK: Scotland 5 Sep 99
A Lanarkshire teenager has died after battling for two years with the human form of mad cow disease. Donnamarie McGivern from Coatbridge is believed to have survived longer than anyone else stricken by the incurable illness. Donnamarie, 17, was diagnosed with new variant CJD in June 1997.

At first doctors thought she was suffering from a brain tumour after she complained of headaches, leg pain and double vision. Just days after her 15th birthday, surgeons at the Southern General Hospital in Glasgow revealed to her family that Donnamarie could be a victim of the disease.

The teenager was unable to speak for the last two years of her life, and in the final stages of her illness she could only open or close her eyes. Both her parents gave up their jobs to look after their only daughter, caring for her at home in Coatbridge. She died there on Thursday night.

A post mortem examination was due to be carried out at the CJD surveillance unit at Edinburgh's Royal Infirmary. Her parents Marie and James were too grief stricken to talk of the loss of their only daughter. They and son Thomas, 13, are being comforted by family.

Marie and James made the decision to care for her themselves, always having someone with her in her little upstairs bedroom, rather than put her in hospital. For the past two years they have devoted themselves for caring for her and praying for a miracle. They even took her on a pilgrimage to Lourdes in France.

A close family member said: "She was just beautiful and she was a fighter. She fought this all the way. It was only her bravery and the care of her parents that kept her alive so long. "We want the world to know that this is what this terrible illness does to a young life. It has taken a precious life away and wrecked a family."

Prime Minister Tony Blair who is in Lanarkshire on Friday, was sent a personal copy of a video showing Donnamarie being looked after at home. He wrote a letter to her parents expressing his deepest sympathy.

The family's parish priest, Father Hugh Kelly, said he had the utmost admiration for the way they had coped with Donnamarie's illness and death. He said: "I first went to that house when I heard that Donnamarie had this illness, when it was diagnosed. "I went there to offer them sympathy, comfort, a prayer and the support of the parish community. "But when I visited that house, roughly monthly since that time, I always came away from the house having gained - I always came out strengthened."

Jennifer Cooke: kuru deaths continue in 1999

Sydney Morning Herald, Saturday, August 28, 1999 Jennifer Cooke, author of Cannibals, Cows and the CJD Catastrophe, Random House, rrp $24.95.
A form of mad cow disease that devastated a cannibal tribe in the highlands of Papua New Guinea may hold the key to estimating the potential toll from Britain's BSE scandal, as JENNIFER COOKE reports.

IN A grass hut in the village of Ai, 1,300 metres above sea level, a subsistence farmer dies, like many of his forebears, of shivering sickness. At the end, Yani contracted pneumonia, typical of victims of the disease that the Fore tribespeople call kuru.

His wife and children are witnesses to his historic passing, in the settlement in Papua New Guinea's Eastern Highlands where time seems to have stood still for the past 50 years. For the 49-year-old tribesman's death is a milestone in the annals of disease research - the end of an epidemic that once threatened to wipe out his people.

Yani (not his real name) died last September from a rare and highly virulent brain disease that superstitious locals once believed was wrought by vengeful sorcerers from hostile tribes. It was once the scourge of the Fore people, a culturally and linguistically distinct group among the 700-odd tribes comprising the 4.2 million that inhabit the rugged ranges of the world's largest island.

Kuru is as old as the collective memories of the oldest Fore villagers, for whom Western dates have little meaning. Remembered events, like the first sighting of a white man in the 1930s, the first encounter with a plane, downed during World War II, and the first road in the area in 1956 helped place kuru among the group since at least 1915. The epidemic took a huge toll on women of child-bearing age and skewed gender ratios. It reached its peak in 1957 with about 200 deaths annually of the 12,000-odd Fore, and a few from other groups with whom they intermarried.

The mysterious disease was the chief cause of mortality in women, with the female-to-male breakdown of adult victims running at 30:1 during the worst years. The youngest victim was a four-year-old boy, although girls were twice as likely to contract the disease as boys. Several members of one family were struck down - boys, girls, mothers, aunts, grandmothers and the odd male relative - sometimes with several years lapsing between cases, suggesting a hereditary link.

But inherited disorders do not threaten whole populations. Too many people were dying for that to be the only cause. A more logical source, infection, seemed to be ruled out by the absence of noticeable fever or antibodies. The symptoms, including a distinctive staggering walk and trembling head and limbs, usually became obvious about 18 months before a victim died.

Kuru became known erroneously as "the laughing death", because gales of uncontrollable and inappropriate laughter (or tears) would sometimes seize sufferers. The condition caught the attention of a visiting journalist and best-selling Australian author, Jon Cleary, who included it in his 1960 novel, North from Thursday.

WHEN Australian patrol officers entered the highlands in the mid-1950s, the kuru area was in wild country where entry to outsiders was restricted until 1958. But that did not stop an enthusiastic American scientist, Carleton Gajdusek, from trying almost single-handedly to solve the riddle of kuru, a disease previously hidden from Western science.

Repeated requests to leave, strongly worded letters from Australian administrators and letters from his former boss, Australia's second Nobel prizewinner for medicine, Sir Frank Macfarlane Burnet, who was in the process of sending Australian scientists into the field to study kuru, failed to budge him. The Fore lands were too remote to enable the scientist to be physically evicted.

The American remained for most of 1957, studying kuru symptoms, helping build a bush hospital, learning the local language and interviewing the relatives of victims. He took countless blood and urine samples, performed autopsies under primitive conditions, and alerted the world to the "exciting problem". Later, when he helped prove that shivering sickness and similar diseases like the rare Creutzfeldt-Jakob disease (CJD) were infectious, through transmission experiments with chimpanzees, Gajdusek would also win a Nobel prize for medicine.

In May 1960, the hypothesis from some Adelaide researchers that kuru was entirely a genetic disease led to the bizarre notion, aired in Federal Parliament, of quarantining the 2,300 square kilometres of often impenetrable, jungle-clad mountains and valleys of the kuru area, south-east of Goroka, into "a vast concentration camp". This would theoretically prevent Fore men, who vastly outnumbered their women, from moving away and spreading the disease.

Gajdusek's initial research was expanded by Michael Alpers, a young Australian whose appointment in 1961 as a kuru researcher was the only concrete result of the doomed quarantine proposal. By 1967, Alpers's methodical charts and figures of kuru deaths revealed the surprising finding that no child under 10 had died of the disease in the previous six years. What had changed in that time?

Alpers became the first person to announce officially that cannibalism was responsible for the spread of kuru. The ritual endocannibalism - "transumption" as Alpers today prefers to call it - practised by the Fore people involved the cooking and eating of relatives, who died naturally or in combat, as a sign of love and respect. The men usually ate the flesh, an important source of protein, apart from precious pigs, in subsistence farming hamlets.

But the women - and young children - ate offal and brain, the latter being the most infectious part of a shivering fever victim. Bones were baked over fires for several weeks and sprinkled over vegetables. In the north Fore region, the body was buried and dug up again so maggots could be cooked and eaten as a bonus.

Whereas Westerners wear black and attend a wake after someone dies, the Fore women would scoop the brains of their dead relatives out of their skulls by hand before cooking. They then wiped the residual liquid and cadaver tissue over their paint-daubed bodies, leaving it caked in their hair and on their bodies for weeks after a mortuary feast.

As the women mourned, the highly infectious agent was entering their bodies to start a long incubation that would end in the fatal shredding of their brains, riven through with microscopic holes like sponges.

Resistant to all cooking, the disease agent - since dubbed a "prion" or corrupted protein - was consumed orally and maybe even transmitted conjunctivally via children's eyes wiped by a caring mother. Broken skin or the scratching of common tropical sores may also have been entry points for the kuru organism.

Yani was infected like this at mortuary feasts he attended as a youngster. Everyone except leprosy victims was eaten, but those who died of kuru were considered a "delicacy", Alpers says. Because they were inactive before death, they were considered "more tender than warriors killed in battle". No-one born since 1960, about the time cannibalism ceased, has died of shivering fever.

Yani was about 49 when he died. His age, placing him about 10 when cannibalism stopped, is important for researchers grappling with forecasts of the likely incubation periods of human victims of Britain's mad-cow disease scare.

"For the first time, no-one is dying of kuru," says Alpers who travelled to Sydney this week to announce at the Frontiers of Neuroscience Research symposium that the epidemic is all but finished, four decades after the end of cannibalism in the Eastern Highlands.

Alpers believes odd cases may crop up until well into the new millennium, but there are no longer any groups of Fore people in terminal stages of the disease.

Alpers, 65, the director since 1977 of the Papua New Guinea Institute of Medical Research, is the keeper of kuru knowledge. He alone has continued to track the disease, although no scientific papers on the subject have been published since the early 1980s. He believes it evolved from an early case of CJD that changed slightly in its many passages through cannibalistic feast subjects.

"Kuru, long dismissed by mainstream scientists as an exotic disease of a small Stone-Age population far removed from the plagues of the Western world, has turned out to be exactly the opposite," Alpers says.

Since it was first transmitted to chimpanzees in 1966 and found to be infectious, kuru has become the model for measuring incubation periods from the accidental spread of CJD through the "high-tech" cannibalism of body parts. The longest incubation period for accidental medical transmission of CJD via grafts of human brain lining in neurosurgical operations is more than 17 years. In extracts of human growth hormone from cadaver-derived pituitary glands, the incubation period is as high as 30 years.

Yani's death shows that transmissible spongiform encephalopathy can kill more than 40 years after infection.

This is vital information for those estimating the death toll among millions of Britons exposed to mad cow disease-infected hamburgers, pies and sausages since the bovine spongiform encephalopathy (BSE) epidemic among cattle erupted in the early 1980s.

Genetic factors may explain why some elderly Fore women have never succumbed to the disease, despite eating many relatives - the incubation period for kuru may be longer than the normal human lifespan.

Alpers, due to retire and return to Australia next year, expects to be based at the Centre for International Health at Curtin University in Perth. He says his kuru scouts will remain vigilant for any more cases as the "general results of the kuru epidemic have a lot of implications" for the outbreak of new-variant CJD (vCJD), which has claimed 45 lives in Britain, France and Ireland in just four years.

"If the minimum incubation period is about 10 years, which is what you might expect from bovine-to-human transmission compared with human-to-human, then we have the likelihood that this is just the slow beginning of a major epidemic with an incubation period that might peak in 25 years from infection," Alpers says.

"That's a real possibility. Let's hope we are wrong and that the agent of vCJD behaves differently from kuru."

Family of CJD victim awarded damages

Fri, 10 Sep 1999  Guardian and Listserve
Comment (Lynette Dumble):
"Although it's my personal viewpoint that no amount is ever sufficient to compensate for CJD, but it's somewhat incomprehensible to us downunder that no American family, to date, has sued the NIH for damages over the CJD legacy of the US human pituitary hormone program - I met the family of a woman doctor from Long Island, New York, who developed CJD when she was 32 years old - 15 years after her last human pituitary growth hormone injection, and that family never took up the option to seek damages - the whole situation was muddied by the family's hope that the NIH would provide a cure [an impossible dream, which I nonetheless understood].

"It seems strange that in the "land of litigation" there is zero action, whereas there have already been damages settlements to the families of four Australian women who contracted CJD as a result of human pituitary gonadotrophin, and a number like the below in the UK."

"Is it possible, that the NIH has some intimidatory process which inhibits litigation? It just doesn't gel that from 2400 "official" human pituitary hormone recipients in Australia, that the government has paid damages [confidentiality agreements prevent disclosure of the amount] to four families, in the UK, £4.5m has been paid to 22 of 3,000-odd "official" human pituitary hormone recipients [not 2,000 as reported below], but in the US, not a dime has been awarded [nor or sought] to a single one of 6,000-odd human pituitary hormone recipient!!! "

Family of CJD victim awarded £1.4m

Thursday September 9, 1999 Sarah Hall The Guardian The family of an orthopaedic surgeon who died from CJD accepted £1.4m damages in the high court yesterday - the largest amount awarded in connection with the disease.

Neil Kreibich contracted Creutzfeldt-Jakob disease after being treated as a youngster with human growth hormone taken from the pituitary glands of contaminated corpses. He died in July 1997 at the age of 37.

Mr Justice Morland said the department of health should pay the record figure to his widow Elizabeth, 37, from Newcastle upon Tyne, and the couple's three children, all under seven.

The sum dwarfs all previous settlements for families and victims of CJD, including £142,500 awarded yesterday to the family of Ian Herman, a shipyard fitter from Middlesbrough who died at 36 in 1997, and the previous highest amount - £175,000 in January to the widow of motorcycle courier Donald Spear.

A total of £4.5m has been awarded, but individual payouts vary widely. In the 22 cases of people who died from the dis ease the lowest sum was £70,000.

In the 36 cases brought by those who have developed psychiatric problems through fear of having contracted CJD after being treated with the growth hormone, figures range from £340,000 to £5,000.

Yesterday David Body, solicitor for the families in all the cases, said: "This sum is significantly larger than any other award because Mr Kreibich, as an outstanding orthopaedic surgeon, had a tremendous career ahead of him. He also had a wife who was wholly dependent on him financially and three very young children."

Mr Body, of the firm Irwin Mitchell, said the award was also significant since it heralded the end of the first successful group action against the makers of a medical product in this country, as well as being the first CJD litigation to be brought internationally.

"We have established a mechanism whereby further CJD claims will be dealt with swiftly and fairly."

A further eight cases in which victims died have yet to be dealt with and up to 50 psy chiatric claims are being assessed. Between 1977 and 1985 nearly 2,000 children in the UK suffering from stunted growth were given the hormone treatment, but it was withdrawn after several children in the US died of CJD.

A Department Of Health spokeswoman said: "We accept the judgment and we shall continue to work with legal representatives of those eligible for compensation to reach settlements as soon as possible."

Mountains of rotting cattle discovered

Sunday August 22, 1999 London Observer Antony Barnett 
Revealed: the secret BSE peril: The discovery of mountains of rotting cattle remains in storage has aroused safety fears. A few miles to the north-east of Margaret Thatcher's birthplace in Grantham is a monument to one of the greatest national disasters that occurred during her years in power. There are no plaques to mark the event. Nor is there anything to commemorate the victims of the tragedy that has so far claimed 43 human lives.

Certainly the troop of Scouts who regularly pitch their tents at the campsite 300 yards from Barkston Heath airfield are blissfully unaware of what lies so close to their sleeping bags and guy ropes.

Why the secrecy? It is simply that government officials would rather no one knew this place existed, let alone ask awkward questions about it. The men from the Ministry seem to have done their job well. Anyone passing the handful of former aircraft hangars that lie half a mile or so from the picturesque Lincolnshire village of Belton would not guess at the macabre scenes they contain. They would have no inkling that these hangars are a potent symbol of the costly legacy of the BSE crisis.

But then anyone getting too close to these hangars would soon realise something rotten was afoot. The putrid stink would make anyone retch, even if they had the protective mask the workers are made to wear.

The hangars contain 100 ft-plus mountains of the ground-up remains of slaughtered cows. The animals have been boiled and mashed into a meaty dust in the Government's desperate efforts to halt the spread of mad cow disease and get the export ban on British beef lifted. The cattle remains look at first glance like tall dunes of gritty, dark brown volcanic sand. But on closer inspection the bits of bones and teeth sticking out of the mounds offer a reminder of their grisly origin.

Since 1996 the culling of 3.5 million cattle over 30 months old has created similar meat and bonemeal stockpiles all around the country. The plan was to incinerate this bovine waste at 1,000ÉC to kill off any infectious BSE proteins that might enter the food chain and give humans CJD. But four years on, there is only one incinerator up and running in the entire country. Based in Southampton, it is only capable of handling 15,000 tonnes a year.

With 80,000 tonnes of these potentially contaminated animals being culled and ground up every year, more than 65,000 tonnes need to be stored somewhere new every 365 days. Stacked to the rafters in warehouses around Britain, this meat and bonemeal is in danger of becoming a BSE time bomb the Government can't get rid of.

The whereabouts of these dumps are shrouded in secrecy. The private contractors that are making millions from running these dumps do not need special planning permission or licences. But for the first time The Observer can publish a map of sites where these potentially deadly stores are located.

Officials continue to play down the health risk posed by the dumps. They argue that most of these cows did not have BSE, but were killed simply because cattle over 30 months old were regarded as a greater BSE risk than younger animals.

But The Observer has seen correspondence from the Ministry of Agriculture which admits that as many as one in a hundred of these animals might have been incubating mad cow disease while not showing clinical signs. This might appear only a small number, but the fact that 407,000 tonnes of mashed-up cow is now held in storage means that up to 4,000 tonnes of potentially BSE-infected material could be sitting in warehouses around the country.

Most worrying for residents living close to these stores is that the protein which is thought to transmit the disease to humans is virtually indestructible - even incineration is not proven to be completely effective. Environmental regulation governing the sites states the buildings should be airtight to prevent contaminated dust from escaping and being ingested or absorbed by humans. But as our investigation reveals, the regulations are not always kept to.

Earlier this month a group of councillors and environmental health officers from north Lincolnshire were invited by the Ministry of Agriculture to visit the BSE stores near Grantham. They are fighting government plans to dump 60,000 tonnes of meat and bonemeal near homes in a village called Blyton. But far from being reassured, the group were horrified at what they saw.

District councillor David Lomas explains: 'We went concerned and came away scared witless. We saw dead vermin and a dead pigeon in the stuff, showing quite clearly animals can get in and out. We saw gaps in the wall and ventilators spewing dust into the atmosphere. One hangar even has a sign warning that the roof is fragile.'

He added: 'The so-called sealed containers that carry the stuff on the trucks are simply open-top tips with canvas over the top. And the trucks that deliver the stuff, which are supposed to be cleaned before they leave, drive off still stinking of the meat and bonemeal. 'What is really appalling is that the Ministry appears to be playing with words to reassure people, telling them everything is sealed and safe when quite obviously the opposite is the case.'

The Environment Agency, which had representatives at the inspection, is now writing to Snowie, the Scottish contractor which runs the Barkston Heath site and a further three BSE dumps, including one in Edinburgh. The agency is demanding that the company complies with the regulations.

Malcolm Snowie, who runs Snowie, strongly denies that the firm has been lax: 'The odd animal might get in when the truck arrives, but the important thing is they don't get out. All our walls are reinforced and the chance of dust escaping is minimal.'

But locals are not so sure. Peter Dunlop, the camp warden who runs a Scout camp down the road from the dump, was shocked to find out what was being kept in the aircraft hangars. He said: 'Scouts use the site throughout the year and I am worried that if it rains the dust could seep out and get into the water supply. And what happens if there was a fire? It doesn't bear thinking about.'

This fear is not just hypothetical - stockpiles of ground-up cattle are known to combust spontaneously. Last Christmas, firemen were called to a warehouse near Preston's marina after smoke was seen billowing from the building storing the rendered carcasses. Firemen were called to a similar event at a BSE dump near Exeter. Such tales have sent a chill through the residents of Blyton, who have formed an action group to stop the Ministry of Agriculture's plans to use disused aircraft hangars in their village.

Mandy Thompson, whose family lives 300 yards from the intended dump, said: 'Nobody can give us cast-iron guarantees that this material is risk-free and we could be breathing this stuff in for years, risking contracting a hideous disease. Why won't they spend more money and store it in proper buildings that are safe, away from communities?'

Last week the House of Commons' Public Accounts Committee warned that the culling of 30-month-old cattle will have to continue for at least another 15 years. Two more incinerators are in the pipeline. Meanwhile, as officials wonder what to do, the BSE mountains get bigger.

Danger alert over secret dump of dead BSE cows

Sunday August 22, 1999 London Observer Antony Barnett, Public Affairs Editor
Potentially lethal waste infected with BSE from secret dumps of the remains of slaughtered cattle is escaping into the environment. The Observer has learnt that the Environment Agency is conducting an inquiry into the storage of 50,000 tonnes of rendered cattle carcasses in former aircraft hangars in Lincolnshire. The agency was forced to act after dead vermin were found inside one of the stores and gaps were seen in the walls of the buildings.

These dumps should be airtight to prevent contaminated dust escaping and being ingested by humans who might then risk contracting CJD, the human form of the disease. The revelations highlight the public health hazard caused by the continuing slaughter of hundreds of thousands of older cattle a year, designed to halt the spread of mad cow disease. There are now more than 400,000 tonnes of BSE waste stored in the dumps around the country.

Before the 1997 general election John Prescott lambasted the Tory government for 'incompetence' in dealing with the stockpile of cattle carcasses. Yet two and half years later there is still only one incinerator capable of burning the dead animals. The carcasses have to be burnt at a temperature of 1,000 degrees Centigrade to kill the protein which is thought to transmit BSE to humans. The furnace, in Southampton, can only cope with a fraction of the 80,000 tonnes of cattle remains that are created each year, and the giant stockpiles are growing by 65,000 tonnes a year. In many cases local residents have no idea what is being unloadeand stored near their homes because the private contractors who run thesites do not need special planning permission.

On page 10 [offline] The Observer publishes a map revealing the location of these 13 stores. The agency's inquiry into the Lincolnshire dump, near Barkston Heath airfield, follows a problem that is only the latest affecting these stores. Councillor David Lomas who visited it, said he went in `concerned and came away scared witless.' The Ministry of Agriculture, which runs the slaughter scheme, denied there was a major public health risk.

Britain Probes Mad Cow Storage Risks

Reuters Online Service Sun, Aug 22, 1999
Potentially lethal waste infected with mad cow disease could be leaking into Britain's environment because the storage dumps in use are not airtight, a Sunday newspaper said. The Environment Agency uncovered the potential threat during an inquiry into the storage of 50,000 tons of cattle carcasses in former aircraft hangars in Lincolnshire in east England, the Observer newspaper said.

It said the agency was forced to act after dead vermin were found inside one of the stores and gaps were seen in the walls of the building. The dumps where the carcasses were stored should have been airtight to prevent contaminated dust escaping and being ingested by humans, it said. This could lead to people contracting Creutzfeldt-Jakob Disease (CJD), the human form of bovine spongiform encephalopathy (BSE) known as mad cow disease, the report said.

Environment Agency officials were not available for comment. The Ministry of Agriculture said the issue did not threaten public health. "As far as we are aware there is no public health risk involved," a spokesman told Reuters. He said the cattle were slaughtered because they were over two and a half years old -- as part of a program to cull cattle which could be potentially contaminated -- not because they had been infected with BSE.

"Some of them will come from herds where BSE has been identified. But there is no question that all of them are infected with BSE," he said.

British beef was banned globally in 1996 over fears of mad cow disease. The 15-nation European Union authorized exports to resume to the bloc from August 1. Cattle carcasses have been put into storage because there is only one furnace in Britain capable of burning them at the required temperature of 1,000 degrees Celsius to destroy the disease.

The furnace can only cope with 15,000 of the 80,000 tons of cattle remains that are generated each year, which means the piles of carcasses grow by 65,000 a year, the paper said.

Search for CJD blood test

BBC News 20 Aug 99
BBC Science Correspondent James Wilkinson reports work is to begin at a laboratory in Leeds to develop a blood test for the new kind of CJD linked to beef infected with BSE (mad cow disease). If successful, the test could be used to screen blood donations to ensure they do not carry the agent that causes CJD - the human form of BSE.

The new test will be based on an existing technique developed by the biotech firm Proteus International. It is currently being used in the Republic of Ireland to make sure meat products removed from cow carcasses are not contaminated with BSE.

The government wants to know if this technique can be developed into a screening test for CJD in human blood.

Blood supplies

Although there is no evidence that CJD - Creutzfeldt-Jakob Disease - can be passed on in blood, the decision has already been taken to treat donated supplies to remove any risk of transmission. But leucodepletion - the removal of white blood cells - is time consuming and expensive. Some reports suggest it will cost the health service millions of pounds. It is also not 100% effective.

For these reasons, a test for the agent that causes CJD would be preferable. If it can be made to work, it may also be possible to use the test to diagnose CJD in patients before any symptoms have developed.

Research facility

This might allow doctors to treat patients earlier, should an effective treatment also emerge one day. The government has pledged £500,000 to set up a research facility within the Public Health Laboratory Service in Leeds. It has asked microbiologist Stephen Dealler, an outspoken critic of government policy in the past, to help develop the test.

Dr Arthur Rushton, development director at Proteus, said the company's technology was offered to the Ministry of Agriculture several years ago but was rejected. "I think there has been a sea change in the attitude of the government, and quite rightly."

About 2.5 million blood donations are made each year in the UK.

Development of Proprietary Reagents for Diagnosis of Mad Cow Disease

Aug. 30, 1999 corporate press release
WESTBROOK, Maine--(BW HealthWire)----IDEXX Laboratories, Inc. and Caprion Pharmaceuticals, Inc. announced today that they will collaborate on the development of Caprion's proprietary reagents for the diagnosis of Mad Cow Disease and other Transmissible Spongiform Encephalopathies (TSEs).

IDEXX will receive exclusive global rights for veterinary applications in diagnostics and therapeutics, while Caprion retains all rights to human diagnostic and therapeutic applications. IDEXX has made an equity investment in Caprion, and will support research at Caprion's Montreal-based laboratories. IDEXX will also conduct development work in its laboratory in Westbrook, Maine, contributing expertise in diagnostic test development. IDEXX will market veterinary products resulting from the collaboration and pay Caprion royalties on product sales.

The goal of the collaboration is development of a rapid diagnostic for detecting Bovine Spongiform Encephalopathy (BSE), commonly known as Mad Cow Disease, in live cattle. Current detection methods require brain tissue samples from slaughtered animals. A live animal diagnostic would allow governments and producers to implement cost-effective surveillance programs to protect and validate disease-free status.

Abnormal prion proteins, thought to trigger BSE, are also associated with the human condition, Creutzfeldt-Jakob Disease (CJD). The recent emergence of ``new variant CJD'' in the United Kingdom and France is believed to be linked to BSE-diseased cattle.

``BSE and other TSEs are important health concerns,'' stated Dr. Erwin Workman, executive vice president and chief scientific officer of IDEXX. ``The veterinary market currently has no proven diagnostic for BSE in live cattle. Caprion's reagents and skills in the prion field position us well to take on this important challenge in veterinary health care.''

``IDEXX's depth in product development and in marketing and sales of advanced veterinary diagnostic technology makes them an ideal partner for Caprion,'' stated Lloyd M. Segal, president and CEO of Caprion. ``Their ability to transform sound technologies into marketable products is well proven.''

     IDEXX Laboratories, Inc.
     Denise Sposato
     (207) 856-0322
     
           or
     Caprion Pharmaceuticals, Inc.
     Lloyd M. Segal
     (514) 940-3604
     

Call for government action as sheep prices plummet

Thu, Aug 19, 1999 By Mark Woods, PA News 
The crisis-hit sheep farming industry has today put forward a four-point plan, including a 1.7 million advertising campaign, to try and ease the burden on the country's lamb farmers. Don Curry, chairman of the Meat and Livestock Commission (MLC) said he would be putting pressure on the agriculture minister Nick Brown to act as prices continue to fall.

Mr Curry was speaking at the first major sale of the year in the north of England at Bellingham, Northumberland. During a day's trading prices for breeding ewes fell to almost half of last year's mark. With the average price dropping to under 32 per head around 700 sheep were left unsold. Mr Curry, who himself farms in Northumberland, said: "The Sheep Statutory Council arm of the MLC has endorsed a four-point plan today aimed at taking pressure off the market and tackling the crisis in the industry. "

A 1.7m promotion campaign is to be launched, primarily on television but also with point of sales promotions aimed at increasing demand. "Governmental support is also to be requested for a disposal scheme for the older cull ewes that will take ewes off the depressed market and ease some of the pressure.

"It is unlikely that the government would endorse a scheme that envisages direct compensation to farmers, but as a short term measure we would urge the government to implement a proper disposal scheme. "We will also be requesting that a private storage scheme be set up for lambs with the market being so depressed at this time. "This would enable the industry to take quantities of lamb off the market and reintroduce it when conditions were more favourable."

Mr Curry also said that the MLC would seek government assistance to alleviate extra-production costs placed on farmers through measures imposed in the wake of the BSE crisis. As farmers packed up and left the sale, where the top price paid for an animal was 50 compared to 80 at the same sale last year, Mr Curry added: "We must apply as much pressure as possible on the minister.

"The gravity of the crisis in the sheep industry must be recognised and action must be taken. "There are many factors why we are where we are including the continuing strength of sterling and its affect on exports. "The consequences of the BSE crisis and the fall in the price of sheep skins from around 12 per skin a year ago to 1.50 now have also taken their toll.

"All these factors together with a general lack of confidence in the industry lead to the reaction we saw here today. "As well as the effect on farmers there are also welfare issues with many farmers being left with animals that are effectively worthless."

Auctioneer Trevor Simpson said: "These kind of sales are hill farmers bread and butter and they had no butter and almost no bread here today." "I don't know how much longer some of them can survive. "Many of the older cull ewes have no value after costs have been deducted so some are worthless in many cases."

[Scrapie is still widespread in Britain, preclinical animals are sold for human consumption, and BSE may have passed back into sheep and developed new properties. -- webmaster]

EU Scientists: No Guarantee US is BSE-Free

Thu, Sep 18, 1997 REUTER Campaign for Food Safety 
BRUSSELS - European Union scientists said Thursday the United States had probably been free of madcow disease in the past but there wa no guarantee it would escape it in the future. The independent but influential Scientific Veterinary Committee issued a statement saying a U.S. BSE monitoring program "has probably been effective in detecting BSE should it have occurred...it is not an absolute guarantee for the present situation."

"At present the committee cannot guarantee that cattle from the United States have not been exposed to and thus do not carry BSE infectivity, though there is no positive evidence that they do so," it added. The committee met for a two-day session ending Wednesday. The ruling is significant because it comes at a time when the United States is seeking an exemption from planned Brussels legislation banning certain imported beef products, including tallow -- a substance widely used in the cosmetics industry.

The U.S. has said it is very concerned by the ban, due to take effect in January, which it says has no scientific basis and could affect billions of dollars' worth of exports to Europe. Winning "BSE-free" status from the committee would have been a powerful weapon in their argument.

The EU has imposed the restriction over fears that the products could carry bovine spongiform encephalopathy (BSE) and could transmit a similar fatal disease, Creutzfeld-Jakob Disease (CJD), to humans. The veterinary committee also considered the situation in Australia, where it said there was no evidence for the occurrence of BSE and the risk of future occurrence was low.

How a scientist obtained thousands for a BSE test he could not prove

The Independent 22 Aug 99
HARASH NARANG has claimed for many years to be able to detect BSE before the appearance of any symptoms. Now we know, finally, that this is not true. Dr Narang has had his research project scrapped because he was unable to meet the strict criteria needed for validating a medical test.

He has been a thorn in the side of the scientific establishment with his outspoken views on BSE and has been told by the Government's Medical Research Council (MRC) that independent scientists have been unable to approve his alleged test because he could not respond to "practical difficulties" with its use.

The MRC said yesterday that the problems arose last year and it had given Dr Narang an extra nine months to find a way to overcome the difficulties of validating the first phase of the two-phase project to get the test to work in a clinical setting using the urine of people suspected of suffering from new variant CJD - the human form of BSE.

Dr Narang had access to a specially built electron microscopy laboratory and several researchers at the University of Leeds to prove that his test really worked. Experts from the National CJD Surveillance Centre in Edinburgh co-ordinated the supply of urine samples from patients.

But the project did not get beyond the first phase, costing pounds 125,000 and expected to last only six months, because of problems with contamination. Dr Narang's test used distilled water that had not been filtered, so enabling bacterial strands to contaminate samples and so be confused with the microscopic fibres he believed indicated a positive result for BSE or CJD.

The MRC said that Dr Narang has been given every opportunity to respond to the criticisms of his scientific protocol but he has declined, bringing the work to an end before the second phase of the 18-month project could begin. George Radda, the secretary of the MRC, said that without proper validation that could be open to scrutiny by independent scientists, the technique of Dr Narang could not be considered a viable test. "The present evaluation has not succeeded. In the judgement of the scientists at Leeds university, everything possible has been done that could have been done by the MRC," Dr Radda said.

A test for BSE would provide enormous benefits both for farmers and doctors concerned about the spread of new variant CJD in the human population. Dr Narang has repeatedly claimed that he has developed an accurate diagnostic but had been prevented from validating it by government officials.

"I already had a diagnostic test for BSE which I had developed for [sheep] scrapie in 1985 and 1986 and published in 1987," Dr Narang told the inquiry into BSE. If the test has been developed early in the BSE epidemic, diseased animals could have been eliminated from the human food chain, Dr Narang said.

Dr Narang has already received tens of thousand of pounds of public money to develop another version of his BSE test, with the help of scientists at the London Hospital - but this ended in failure, as well. Dr Narang was suspended from this post as a clinical microbiologist at the Public Health Service Laboratory in Newcastle upon Tyne because of allegations of misconduct. He was later made redundant.

Narang's Claims

1 That he discovered an unusual form of CJD at the end of the 1980s which he believed was caused by BSE. In fact, the new variant form of CJD was only discovered at the end of 1995 and confirmed as totally new in 1996. Its links with BSE were only established after experiments on mice were published in 1998.

2 He claimed in 1997 he could prove that chickens can be infected with BSE, although he insisted on keeping the carcasses of the hens to himself until he could finish his research. No published research has ever established that chickens can be infected with BSE.

3 In 1996 Dr Narang cast doubt on tests that were supposed to give milk a clean bill of health for being BSE-free. However, there is no direct evidence that milk can pass on the BSE agent.

4 Dr Narang claims that BSE is caused by a new type of virus containing genetic material known as nucleic acid. He claims to have identified the nucleic acid but most other scientists believe that a virus is not the cause of BSE. As yet, his results have not been replicated.

5 Blood transfusions can transmit CJD and the human form of BSE. No evidence that they can has emerged but government advisers have taken precautions against it being passed on in white blood cells.

Head blow clue to Alzheimer's

 Wed Sep  1 1999 press release
Head injuries sustained in road or other accidents trigger physical changes in the brain that may lead to Alzheimer's disease, scientists have found. Researchers who studied pigs that were subjected to violent jerks, such as might occur in a car crash, while anaesthetized found changes in their brains similar to those in the early stages of dementia.

It has long been known that people who have suffered a sever blow to the head are at greater risk of developing Alzheimer's disease. Now, scientists at Pennsylvania University school of medicine believe they have discovered the mechanism. An estimated 15 million people worldwide suffer from the condition, which is characterized by development of abnormal plaques and tangled bundles of fibers in the brain.

Without suffering any external damage to the skull, the pigs were forced through a very rapid acceleration or deceleration. Nerve fibers within the pig's brains were found to have snapped apart. This set off ! ! the same biochemical process that can lead to development of amyloid plaques in the brain's tissue.

Dr. Douglas Smith, who headed the research, said: "Our study suggests that even moderate brain injury resulting from a tremendous change in velocity can cause axonal damage sufficient to launch an insidiously progressive degenerative process."

Comment (webmaster): This issue has come up many times in CJD as well. Numerous victims were in accidents involving head trauma prior to a CJD diagnosis.

France won't import British beef just yet

Wed, Sep 1, 1999 The Associated Press
France will hold off importing British beef despite the European Union's decision a month ago to lift the three-year-old embargo, a newspaper reported Wednesday. France's decision follows a similar one by Germany, which also is still banning British beef that was a prime source in the transmission of the so-called "mad cow" disease.

France will continue to ban the imports while a committee of experts reviews evidence and decides whether the product is safe, the Le Monde newspaper reported. The experts' conclusions are not expected before the end of September.

The committee was set up at the demand of the government of Prime Minister Lionel Jospin, the paper reported, citing government sources. It said the committee wants to obtain reports of inspections made in Britain under the auspices of the European Commission that were not passed onto the Commission's scientific committee. Without the reports, the real risks of eating British beef cannot be evaluated, according to Le Monde.

Days after the European Commission lifted the ban Aug. 1, Germany said it would not comply before the end of September, pending a study into the legality of the Commission decision and checks to ensure British beef was meeting safety standards. The Commission imposed a global ban on British beef exports in March 1996 after tens of thousands of British cattle were struck down by bovine spongiform encephalopathy, commonly known as "mad cow" disease, apparently after being fed the ground remains of sick sheep. Medical researchers linked the cattle disease to a new strain of a fatal human brain ailment called Creutzfeldt-Jacob disease.

Mad Cow Home ... Best Links ... Search this site