Guest guest Posted February 16, 1999 Report Share Posted February 16, 1999 By Sue Reinert The Patriot Ledger The government isn't doing enough to protect the nation's population against Creutzfeldt-Jakob disease and related animal varieties, such as mad cow disease, says a coalition of activists that includes families of CJD victims. Ten families and three farm and food safety advocacy groups submitted filings with the U.S. Food and Drug Administration last month. They want the FDA to tighten animal feed rules intended to prevent the spread of mad cow, and make CJD a reportable infectious disease, just chicken pox or measles. The groups and relatives acted amid increasing worry about the implications of Britain's mad cow disease. Researchers believe the fatal brain disorder spread to thousands of cows through feed that contained brain tissue and other nervous system material from infected animals. At first, British officials insisted the disease could never spread to humans by eating beef from infected cattle, but scientists now strongly suspect that mad cow is linked to a new form of CJD that appeared in the mid-1990s. So far, the British have reported 35 cases of what doctors call new-variant CJD: 34 in Britain and one in France. U.S. officials emphasize that mad cow has never turned up in cattle here, nor has anyone reported the new form of CJD. And while the timing of the new CJD cases implies a tie to mad cow, reseachers haven't definitively proved a link, scientists point out. Some scientists also fear that the lobbying by families and activists will spark unjustified panic. ``We're very sympathetic to people who have had problems with this disease in their life, but that's not a justification for creating undue concern in the rest of the population or to talk about this being some sort of major epidemic,'' said Lawrence Schonberger, an epidiemologist at the Centers for Disease Control. But the unanswered questions about CJD and its relatives -- known as transmissible spongiform encephalopathies, or TSEs -- leave plenty of room for worry. Scientists believe an abnormal cell protein called a prion causes CJD and the other disorders, but they don't know how it attacks the brain, and some researchers disagree with the theory. There's no conclusive diagnostic test except a brain autopsy -- after the victim dies. Researchers know TSEs can spread from person to person; the first evidence was a disease called kuru that devastated New Guinea tribespeople who practiced cannibalism. Scientists have demonstrated that the diseases can travel from animal to animal, and from person to animal, but they don't know exactly how. Despite the unknowns, scientists take comfort from the steady -- and tiny -- rate of CJD infection. It's remained at one to two cases per year per million people worldwide, year after year. Families of CJD victims and other activists question the statistics. Doctors can miss the diagnosis and without a brain autopsy some cases won't be reported, said Sheryl Trainor, whose mother, Jean, died of the disease Dec. 11 in Scituate. ``A lot of these death certificates don't read CJD,'' even when doctors know the patient had the disease, she said. That's why families want CJD to be reportable, she said. Studies show that doctors often mistake Alzheimer's disease for CJD, according to the FDA petition. In one study, autopsies showed that three of 54 patients who supposedly died of Alzheimer's had CJD, a 5.6 percent error rate. Another report found a 13 percent error rate among 46 Alzheimer's patients, the petition said. ``That's a high percentage,'' Trainor said. If mistakes are that common, the government may be overlooking thousands of undiagnosed CJD cases, she said. Scientists dispute the argument. The research cited by activists hasn't been published where it would be scrutinized by other scientists, said Schonberger of the Centers for Disease Control. Some doctors may miss a case or fail to identify CJD as the cause of death, but not many, Schonberger said. The CDC questioned neurologists in 1996 and found they didn't identify about 13 percent of their CJD cases, said Ermias Blay, another CDC epidiemologist. That wouldn't add much to the total, he said. Dr. Clarence J. Gibbs, one of the foremost CJD researchers in the world, said: ``I doubt very much that a significant number (of cases) go unreported. Of course, there are always going to be people who slip through the hole.'' Gibbs has developed a new spinal fluid test that can diagnose CJD most of the time. Patients with CJD and those with Alzheimer's may show similar dementia symptoms at first, but CJD victims usually deteriorate much more rapidly. CJD patients also typically show abnormal electroencephalogram patterns, but not always, Gibbs said. Sometimes doctors must test patients more than once. The first spinal fluid sample taken from Jean Trainor, the Scituate homemaker who died of CJD, was negative. A subsequent sample tested positive. Sometimes it takes time for the suspect proteins to appear in spinal fluid, Gibbs said. Even brain biopsies -- removing a sample from a patient's brain before death -- don't always detect the disease if neurologists take the sample from an unaffected area, Gibbs said. Still, he said, ``We at NIH and CDC believe we have a pretty good handle on the disease.'' And he warned that making the disease reportable could backfire on relatives of victims. ``It may put the family in jeopardy as far as insurance is concerned, as far as jobs are concerned.'' Massachusetts officials also believe the state's figures are sufficiently accurate. Dr. Alfred De, director of communicable disease control for the Department of Public Health, began looking at the number of CJD cases after Britain's mad cow outbreak. ``I was concerned it might be an issue somebody might be interested in,'' De said. Working from death certificates, he found the CJD rate stayed at about 1.17 cases per million population for seven years. He doesn't remember when he stopped keeping figures, but it was several years ago, he said. De has confidence in the figures. ``About half our cases have either a brain biopsy or an autopsy,'' he said. ``My impression is (doctors) were doing much more than I expected.'' Scientists and health officials do agree with families on one point: more autopsies would help. Less than one-third of the U.S. cases of CJD were confirmed by an autopsy, the CDC's Schonberger said. Health officials want more autopsies not because they think there are many unreported cases of classic CJD, but because without the examination they could miss cases of the new strain that has been linked to mad cow. An autopsy is the only sure proof of new-variant CJD. The new disease worries health officials more because ``it has more public health problems,'' Schonberger said. For one, prions associated with new- variant CJD seem more likely to be carried by white blood cells than in cases of classic CJD, according to the National Institutes of Health. That means people might get infected from blood transfusions or blood products, although no illness has ever been traced to contaminated blood. Health officials are still worried. Britain has banned plasma products, such as anti-clotting medicines, for hemophiliacs that was made from British plasma donors. In December, an FDA advisory committee recommended that some donors who spent time in the United Kingdom after the mad cow outbreak be barred from giving blood. The committee intends to recommend specific standards after it finds out how a ban would affect the supply of blood. Meanwhile, some transmission routes are still emerging. In the past 10 years, more than 80 people got the disease from contaminated dura mater: a tough brain lining that has been used as a graft in surgeries and was apparently taken from people who had died of CJD. More than 100 people have been infected because they took human growth hormone that had been harvested from corpses. Hormone makers stopped using human sources for the product as early as 1970, but the first cases began appearing in 1985 because of the long incubation period for CJD. Some family members see parallels between the official reaction to CJD and the initial dismissal of AIDS in the early 1980s. ``How many people died of AIDS because people thought it was a rare disease of homosexuals?'' Sheryl Trainor wondered. ``I think there's a lot of unanswered questions out there and I want answers.'' Copyright 1999 Patriot Ledger2/15/99 11:05 AM Quote Link to comment Share on other sites More sharing options...
Guest guest Posted February 18, 1999 Report Share Posted February 18, 1999 Reading these doctors' comments makes me all the more convinced that we should all try our hardest to make it to the June conference and let our voices be heard!!! Maybe when some of these doctors can put faces to names from phone calls or email, they will get a little more sympathetic to our plight. We are here as a group fighting CJD for two main reasons...in memory of the loved one we lost and to try to make sure that this disease is researched and stopped so that more families will be saved of the heartache we all went through! in California === It's never too late to be what you might have been - Elliott ---BevAlso@... wrote: > > From: BevAlso@... > > By Sue Reinert > The Patriot Ledger > The government isn't doing enough to protect the nation's population > against Creutzfeldt-Jakob disease and related animal varieties, such as mad > cow disease, says a coalition of activists that includes families of CJD > victims. > Ten families and three farm and food safety advocacy groups submitted > filings with the U.S. Food and Drug Administration last month. They want the > FDA to tighten animal feed rules intended to prevent the spread of mad cow, > and make CJD a reportable infectious disease, just chicken pox or measles. > The groups and relatives acted amid increasing worry about the > implications of Britain's mad cow disease. Researchers believe the fatal brain > disorder spread to thousands of cows through feed that contained brain tissue > and other nervous system material from infected animals. > At first, British officials insisted the disease could never spread to > humans by eating beef from infected cattle, but scientists now strongly > suspect that mad cow is linked to a new form of CJD that appeared in the > mid-1990s. So far, the British have reported 35 cases of what doctors call > new-variant CJD: 34 in Britain and one in France. > U.S. officials emphasize that mad cow has never turned up in cattle here, > nor has anyone reported the new form of CJD. And while the timing of the new > CJD cases implies a tie to mad cow, reseachers haven't definitively proved a > link, scientists point out. > Some scientists also fear that the lobbying by families and activists will > spark unjustified panic. > ``We're very sympathetic to people who have had problems with this disease > in their life, but that's not a justification for creating undue concern in > the rest of the population or to talk about this being some sort of major > epidemic,'' said Lawrence Schonberger, an epidiemologist at the Centers for > Disease Control. > But the unanswered questions about CJD and its relatives -- known as > transmissible spongiform encephalopathies, or TSEs -- leave plenty of room for > worry. > Scientists believe an abnormal cell protein called a prion causes CJD and > the other disorders, but they don't know how it attacks the brain, and some > researchers disagree with the theory. There's no conclusive diagnostic test > except a brain autopsy -- after the victim dies. > Researchers know TSEs can spread from person to person; the first evidence > was a disease called kuru that devastated New Guinea tribespeople who > practiced cannibalism. Scientists have demonstrated that the diseases can > travel from animal to animal, and from person to animal, but they don't know > exactly how. > Despite the unknowns, scientists take comfort from the steady -- and tiny > -- rate of CJD infection. It's remained at one to two cases per year per > million people worldwide, year after year. > Families of CJD victims and other activists question the statistics. > Doctors can miss the diagnosis and without a brain autopsy some cases won't be > reported, said Sheryl Trainor, whose mother, Jean, died of the disease Dec. 11 > in Scituate. > ``A lot of these death certificates don't read CJD,'' even when doctors > know the patient had the disease, she said. That's why families want CJD to be > reportable, she said. > Studies show that doctors often mistake Alzheimer's disease for CJD, > according to the FDA petition. In one study, autopsies showed that three of 54 > patients who supposedly died of Alzheimer's had CJD, a 5.6 percent error rate. > Another report found a 13 percent error rate among 46 Alzheimer's patients, > the petition said. > ``That's a high percentage,'' Trainor said. If mistakes are that common, > the government may be overlooking thousands of undiagnosed CJD cases, she > said. > Scientists dispute the argument. The research cited by activists hasn't > been published where it would be scrutinized by other scientists, said > Schonberger of the Centers for Disease Control. > Some doctors may miss a case or fail to identify CJD as the cause of > death, but not many, Schonberger said. The CDC questioned neurologists in 1996 > and found they didn't identify about 13 percent of their CJD cases, said > Ermias Blay, another CDC epidiemologist. That wouldn't add much to the total, > he said. > Dr. Clarence J. Gibbs, one of the foremost CJD researchers in the world, > said: ``I doubt very much that a significant number (of cases) go unreported. > Of course, there are always going to be people who slip through the hole.'' > Gibbs has developed a new spinal fluid test that can diagnose CJD most of > the time. > Patients with CJD and those with Alzheimer's may show similar dementia > symptoms at first, but CJD victims usually deteriorate much more rapidly. CJD > patients also typically show abnormal electroencephalogram patterns, but not > always, Gibbs said. > Sometimes doctors must test patients more than once. The first spinal > fluid sample taken from Jean Trainor, the Scituate homemaker who died of CJD, > was negative. A subsequent sample tested positive. > Sometimes it takes time for the suspect proteins to appear in spinal > fluid, Gibbs said. > Even brain biopsies -- removing a sample from a patient's brain before > death -- don't always detect the disease if neurologists take the sample from > an unaffected area, Gibbs said. > Still, he said, ``We at NIH and CDC believe we have a pretty good handle > on the disease.'' And he warned that making the disease reportable could > backfire on relatives of victims. > ``It may put the family in jeopardy as far as insurance is concerned, as > far as jobs are concerned.'' > Massachusetts officials also believe the state's figures are sufficiently > accurate. Dr. Alfred De, director of communicable disease control for the > Department of Public Health, began looking at the number of CJD cases after > Britain's mad cow outbreak. > ``I was concerned it might be an issue somebody might be interested in,'' > De said. Working from death certificates, he found the CJD rate stayed > at about 1.17 cases per million population for seven years. He doesn't > remember when he stopped keeping figures, but it was several years ago, he > said. > De has confidence in the figures. > ``About half our cases have either a brain biopsy or an autopsy,'' he > said. ``My impression is (doctors) were doing much more than I expected.'' > Scientists and health officials do agree with families on one point: more > autopsies would help. Less than one-third of the U.S. cases of CJD were > confirmed by an autopsy, the CDC's Schonberger said. > Health officials want more autopsies not because they think there are many > unreported cases of classic CJD, but because without the examination they > could miss cases of the new strain that has been linked to mad cow. An autopsy > is the only sure proof of new-variant CJD. > The new disease worries health officials more because ``it has more public > health problems,'' Schonberger said. For one, prions associated with new- > variant CJD seem more likely to be carried by white blood cells than in cases > of classic CJD, according to the National Institutes of Health. > That means people might get infected from blood transfusions or blood > products, although no illness has ever been traced to contaminated blood. > Health officials are still worried. Britain has banned plasma products, > such as anti-clotting medicines, for hemophiliacs that was made from British > plasma donors. In December, an FDA advisory committee recommended that some > donors who spent time in the United Kingdom after the mad cow outbreak be > barred from giving blood. The committee intends to recommend specific > standards after it finds out how a ban would affect the supply of blood. > Meanwhile, some transmission routes are still emerging. In the past 10 > years, more than 80 people got the disease from contaminated dura mater: a > tough brain lining that has been used as a graft in surgeries and was > apparently taken from people who had died of CJD. More than 100 people have > been infected because they took human growth hormone that had been harvested > from corpses. > Hormone makers stopped using human sources for the product as early as > 1970, but the first cases began appearing in 1985 because of the long > incubation period for CJD. > Some family members see parallels between the official reaction to CJD and > the initial dismissal of AIDS in the early 1980s. > ``How many people died of AIDS because people thought it was a rare > disease of homosexuals?'' Sheryl Trainor wondered. ``I think there's a lot of > unanswered questions out there and I want answers.'' > Copyright 1999 Patriot Ledger2/15/99 11:05 AM > > ------------------------------------------------------------------------ > Quote Link to comment Share on other sites More sharing options...
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