Human 'mad cow' toll higher than admitted, says scientist
CJD causing US blood shortages
Wendi Altschul Nofi, 34, dies of CJD
British BSE crisis to cost $5.5 billion
Meat industry journal reviews "Mad Cow USA"
Progress in understanding and treating Alzheimer's
13 July 98 By JAMES MEIKLE, The Guardian. Distributed by Scripps Howard News ServiceAt least 16 more Britons may have died of the human form of bovine spongiform encephalopathy -- BSE, or "mad cow" disease -- than the 27 official victims -- and that they began dying 11 years before the first recognized case in 1995, a scientist is expected to say Monday.
Harash Narang, laid off from his British government-funded job four years ago, believes British authorities have either blocked or undermined important tests into both the cattle disease and its transmission to humans.
Narang -- who believes that the human disease, known as new-variant Creutzfeldt-Jakob Disease, should be named after him -- will testify Monday at a BSE inquiry. The 56-year-old.microbiologist alleges in a written statement that he was "victimized" by the Public Health Laboratory Service (PHLS) and that he identified several patients with atypical symptoms from traditional CJD long before the new variant was announced in March 1996, and linked to the eating of infected beef.
The first acknowledged victim was Stephen Churchill, 19, who died in May 1995.Narang claims he identified the condition, which follows a pattern of psychological problems, depression, instability, coma and death, in 1988. He says that the first death probably occurred in 1984 and involved people in their 60s as well as the mainly young people identified by the CJD Surveillance Unit in Edinburgh.
Narang claims he was ordered to stop experiments on possible transmission of BSE to humans in 1990. When he refused, the rodents involved were killed.
He says: "Had these experiments been completed ... and had the preliminary indications been confirmed, we would have been in no doubt about the link between BSE and CJD and many lives could have been saved."
Narang, who believes the infectious agent in BSE is a virus, says: "The government should have kept on open mind and should have encouraged a wide range of approaches rather than shutting down any line of inquiry which did not conform.
"The PHLS has denied any victimization. It said it sought to bring his work to scientific and professional attention despite his "misconduct." He was subject to two sets of disciplinary proceedings. The first was over "unsafe practices," for which he was given a written warning; the second was over unauthorized investigations into CJD victims.
The disciplinary investigation into the second case in 1993 found conduct that "justified instant dismissal," but he was released from other duties to work in London while on PHLS pay.He was laid off in November 1994 and lost a claim for unfair dismissal.
PA News Sun, Jul 12, 1998 By John von Radowitz, Science Correspondent, PA NewsA link between mad cow disease and CJD could have been established six years earlier than it was had crucial research not been prematurely stopped, the BSE inquiry will hear tomorrow.
Microbiologist Dr Harash Narang claims in evidence that if the work he started in 1988 had not been terminated "many lives could have been saved". Dr Narang, one of the Government's most outspoken critics over BSE, maintained in a 42-page statement to the inquiry that his attempts to investigate the problem were consistently blocked by officials.
His most striking claim was that the Government missed an early chance of recognising the link between BSE and the new strain of CJD now known to be triggered by the cattle disease. In March 1996 the then Health Secretary Stephen Dorrell announced the discovery of 10 cases of "new variant" CJD which experts thought were probably caused by eating infected beef. Since then scientists have become convinced that contaminated meat is the cause of the disease.
Dr Narang said he first encountered "atypical" cases of CJD with unusual symptoms and brain lesions in 1988. At the time he was working for the Public Health Laboratory Service (PHLS) in Newcastle. He started that year carrying out experiments aimed at tracing the source of the infective agent. This was done by injecting hamsters and mice with brain tissue from dead CJD victims and observing the effect on the animals.
"By 1990 these experiments were well advanced and were giving clear preliminary indications that they were CJD cases infected with the BSE strain," said Dr Narang. But in the midst of the experiments, it was alleged, Dr Narang was ordered to destroy all the animals. He claimed that when he refused, they were destroyed anyway.
In his statement, Dr Narang said: "Had these experiments been completed in their entirety and had the preliminary indications been confirmed we would have been in no doubt about the link between BSE and CJD and many lives could have been saved.
"Had we known of the link in 1990 then the passing of infected material into the food chain would have been prevented earlier, and sloppy practices in abattoirs and butchers would not have been acceptable and would not have occurred."
In its own statement refuting the claims, the PHLS Board denied any attempt to halt Dr Narang's experiments continuing, and said the animals were moved to a veterinary laboratory while their facilities were refurbished. The up-graded PHLS animal house was opened in 1991 with the return of Dr Narang's surviving animals. It was now closed. According to the PHLS there was no evidence that the tests could have produced dramatic information about the link between BSE and CJD.
Dr Narang also claimed he was thwarted in his attempts to prove the effectiveness of diagnostic tests for BSE that he had developed. Experiments on an electron microscopy technique were halted by the Ministry of Agriculture, Fisheries and Food in 1991. Last year Mr Narang won Medical Research Council funding for a project at Leeds University to verify a urine test, but claimed this had been beset by problems and lack of support.
He said: "Had any of these various tests been funded or supported in any way by Government bodies it would have been possible to set up a slaughter house test for diagnosing subclinical BSE in cattle that had not yet shown the symptoms of the disease, but which were entering the national diet. In this way, the disease could have been eradicated."
But he said his employers and others had "consistently interfered with and hindered" his researches. To a large extent he had been forced to rely on the support of a private businessman, Ken Bell.
Dr Narang was made redundant from the PHLS in 1994 after two sets of disciplinary hearings, one for breaches of safety rules and the other over complaints about his investigations into the family backgrounds of CJD victims.
In the conclusion to his statement he maintained that the handling of the BSE crisis was "influenced by the preferences and prejudices of civil servants whose main concern was to protect the well being of the farming industry rather than to tackle the problem on a scientific basis and to protect their own position".
Ed Gehrman 1 July 1998The article summarizes current thinking of an alternate possible explanation for TSEs.
Dealler website 12 July 98Send a cow charity. The EC decision to consider lifting the EU export ban on cows born after AUg 1996 has been welcomed by the charity called Send a Cow, which send cattle to African nations.
No evidence that nvCJD cases are rising 27 in UK plus 1 in France is a similar rate to previously. All have had the same genetics: met/met at codon 129 of the prion gene. British beef sales up
The warning also affected CVL scientist Martin Jeffrey who told the Inquiry that his paper on the scrapie like disorder in a nyala had its title changed to omit the word scrapie and delayed because of the number of references to scrapie.
Cull is on target. Jeff Rooker said 71,000 animals had been slaughtered so far in a programme which had been extremely difficult and complex to run. He said that most of the animals that were being sought had already been slaughtered and so the number actually killed in this programme had actually been quite low.
Forecast of falling beef consumption. Predictions that it will fall by about 10 percent by the middle of the next decade.
'Real progress' over beef ban. EC unanimously voted in favour of ending the ban. This now has to go to the veterinary committees and the individual countries. (This would initially only really apply to farmers that had never had a case of BSE, never fed infected meal, never imported any cattle from an affected herd...so it only actually applies to 80 herds in the UK at this time.
July 9 1998 BY MICHAEL HORNSBY Times agriculture correspondentA BUTCHER charged with selling beef on the bone has been cautioned after agreeing to plead guilty.
Dejon Terry, owner of Palmers Quality Butchers at Bletchley, Buckinghamshire, was to have appeared at a pretrial hearing before magistrates in September. But Milton Keynes council said yesterday that it had decided not to proceed with the prosecution because Mr Terry had dropped his not-guilty plea and signed an undertaking not to repeat the offence.
He admitted having sold a "pre-packed forerib of beef with the bone in situ" on March 5 in breach of the Beef Bones Regulations 1997. The rules were introduced last December after scientific advice that nervous tissue attached to bones might carry "mad cow" disease.
Mon, Jul 13, 1998 Business Wire American Red Cross, Philadelphia Susan Snyder Sponar, 215/451-4239 Kristine Horner, 215/451-4240 1-800-26 BLOOD[Note: Earlier this year ABC on-line reported that CDC might recommend ending or drastically changing CJD screening at blood banks, saying it was a hypothetically risk, while the blood shortage is real. -- webmaster]
PHILADELPHIA With blood supplies low across the country, the American Red Cross makes a special appeal to type O first time donors to give blood this summer. Veteran donors are asked to "Bring a Buddy" when they donate.
The American Red Cross national inventory system has no excess blood supplies to supplement local collections. Currently, one-third of the 38 Red Cross blood regions are down to a day's supply of type O blood and nine of them are on emergency appeal, meaning that they are below one day's supply of type O blood. Optimally, the Red Cross tries to have a three-day supply of blood at all times. It is anticipated that demand will rise as surgical schedules return to normal after the holiday week.
"Our local blood supply must be replenished every day to help us prevent an emergency situation," said Laurene Cianfrani, who as chief operating officer of the Penn-Jersey Region of the Red Cross oversees the distribution of blood products to nearly 100 hospitals in Southeastern Pennsylvania, South and Central New Jersey. The challenge of maintaining a safe blood supply is typically more difficult during the summer vacation season when schools close and people are distracted. Local students, both high school and college, are key donors for the regional blood supply.
Because schools here close for the summer, the Penn-Jersey Region of the Red Cross will lose nearly 300 donations from first time donors each week during July and August. Concerned about meeting emergencies during and immediately after the Fourth of July, Red Cross issued an appeal for donors. Donor response averted that holiday crisis. Red Cross urges donors to help maintain the momentum and not let donations drop.
Transfusions of red blood cells have increased by 3.3 percent locally over the last two years because many hospitals are on the cutting edge of medical technology, using advanced procedures in transplantation, oncology, neonatal care and cardiology. Daily shipments of blood from other Red Cross centers are needed to supplement local collections to meet this high demand.
Only five percent of the population actually donates blood, despite the fact that an estimated 95 percent will need blood at some time in their lives. To meet the constant and urgent need for blood, the Red Cross asks more people to take that first step toward a lifetime of saving lives this summer. Type O donors are strongly encouraged to donate. A rise in the number of trauma cases may occur during the summer months. Type O-negative blood can be safely transfused to most patients in an emergency situation.
Giving blood is safe, easy, and it saves lives. To donate blood, you must be healthy, at least 17 years old and weigh 110 pounds or more. In New Jersey, 17 year olds must bring a signed Red Cross parental consent form with them when they donate. Federal regulations require that donors wait 56 days between donations. Please bring some form of ID.
First time and veteran donors are urged to "Bring a Buddy." Call the American Red Cross at 1-800-26 BLOOD to find a convenient location and time for your donation.
Long Island Voice 1 July 98 David DavisArticle summary: The death of Wendi Altschul Nofi from CJD and the effect on her husband and three children is described. She was one of 8,000 American children who received contaminated human growth hormone from pituitaries. The author of the article himself was a member of the patient group and has written deeply moving articles about the situation the group finds itself in as time goes on.
Wendi was one of 2,000 people who was never notified by NIH, even though she lived her entire life within 25 miles of the town where she received the 3x a week injections and was easily locatable through social security and motor vehicle registration. Ignorance of her condition led doctors and family to keep her on a feeding tube for 2 1/2 years after her condition became hopeless.
Husband Mike Nofi is angry with NIH for lack of notification and lack of advice. He refused their request for a brain autopsy.
April 22, 1998 This was sent to Oprah Winfrey, reprinted here by permission"I am the madson of a deadmom who died of madcow.(heidenhain variant creutzfeldt-jacob disease.) I sat with her for 10 weeks and watched as this hidious disease ate her brain up. She wrote in her journal that she started to see brown spots on sept. 27, 1997. These were her first symtoms -- apprx.10 days later she was blind, about 2 weeks later she had lost control of her coordination, walking, and speech.
She would get these uncontrolable jerks that at times would take 3 of us to hold her down. Around the 8th week she was totally bedridden. She died in the 10th week on 12-14-97. THANK GOD!
If you ever see this disease, as I did with my mom, you will truly believe that madcow is here. I truely believe that is what my mom died of. They can call it what ever they want to.
Now, I will take this a step further. My neighbor's mother also died of c.j.d. She died on 12-14-96, they had diagnosed it as Alzheimers, until the autopsy he demanded ruled out alzheimers and ruled in c.j.d.
About a month ago my neighbor called me over, he had been going through some old boxes of his mom's and came across some pills he thought I should see. When I read the ingrediants I just about sh*t!
INGREDIENTS: vacuum dried bovine brain, bone meal, bovine eye, veal bone, bovine liver powder, and bovine adrenal. It was a cow in a pill! This woman taking these pills died of c.j.d. Could it be madcow in a pill?
I called the texas dept. of health (T.D.H.) the next day, and the following day they were out here and got the pills. I had located the manufacture and called with a bogus story and a list of doctors that would prescribe them in houston. The T.D.H. called a few days later, asking for the list of doctors, their phone numbers, and told me they would take it from there. I need not persue it any further!
Not to long ago, 4 or 5 weeks, a girl showed up at my door. She had called crying a week earlier and could not talk. She had seen a story on T.V. about my mother. Anyway, when I first saw her I knew she had seen it too (madcow). Her mother had died of c.j.d. on 2-14-97.
This disease is here and you can call it what ever you want,c.j.d., n.v.c.j.d., b.s.e. or madcow, for what it is. But, that young man who died of n.v.c.j.d. in England, Steve Churchhill, had the exact same symptoms as my mother. There is also a girl in Ft. Worth Texas who called me. She had seen an article about my mom in the Dallas Morning News. Her dad had died of c.j.d. so far we have come up with about 18 people who has died of c.j.d. in texas, 15 confirmed. I have heard from other people it is up to 32.
I am tired of hearing this crap about nv-cjd being in just young people. That same old line about how nv-cjd victoms are much younger and their clinical course from first sign of symtoms to death is much longer. Any diseases clinical course is going to be longer in younger people, because their body and organs are much younger and healthier. But, in the end, their brains are full of spongeform holes, just like the older folks. Just because the plagues are more extreme, does not mean its a different disease. Could it not be just a more extreme case of typical c.j.d.????
Greed is what it is all about. They banned feeding cattle to cattle. But, are still allowed to feed those downer cows to pork and poultry. Then they are still allowed to feed the pork and poutry byproducts back to the cows. Now Dr. Gibbs writes that the prion-protien can survive the digestinal track and composting process. So the prion-protien goes right back to the cow. We must ban feeding all animals to animals. Its just an endless cycle of greed thats killing people.
I have requested that further test be done on my moms brain.(frozen tissue, paraffeine sections and serum) be sent to case western reserve university in Cleveland, Ohio. Dr. Pierre Lugi Gambetti. I hope you find some interest in this. I just don't believe we are being told everything. The gov. lied about asbestos for 75 years."
July 8/98 ReutersLONDON -- The U.K. National Audit Office was cited as saying today that the cost of clearing up Britain's mad-cow crisis is set to top $5.5 billion, and that it could take until 2003 to incinerate huge stocks of meat, bone meal and tallow from cattle slaughtered because they were judged at risk from the disease.
Since March 1996, over 1.35 million cattle have been put to death under one cull scheme, affecting those over 30 months old. The report said total spending on tackling the disease, compensating farmers and paying slaughterhouses, renderers and other professionals was almost 1.5 billion pounds ($2.5 billion) in 1996-7, of which 800 million pounds would come from the European Union.
It forecast additional spending between 1997 and 2000 at 1.9 billion pounds, taking the total to 3.4 billion pounds ($5.5 billion).
Friday, May 29, 1998 FROM: THE MEATING PLACE Dan Murphy writes for Meat Marketing and Technology magazine.
"Mad Cow USA: Could the Nightmare Happen Here?" Authors: Sheldon Rampton and John Stauber
Had enough discussion of the "mad cow" scare? If you're like most people in the meat industry, the answer is yes. Nevertheless, a recently released book called "Mad Cow USA: Could the Nightmare Happen Here?" might be worth skimming through.
Why? For the depth and detail of the investigation of key issues surrounding the now nearly 10-year-old tale of bovine spongiform encephalopathy. Authors Sheldon Rampton and John Stauber, the latter a principal in the PR Watch organization and an outspoken critic of governmental efforts to prevent BSE in the United States, cover familiar ground to readers of the highly publicized "Deadly Feasts," released last year. That book, whose author Richard Rhodes benefited from the kind of high-powered marketing campaign reserved for Pulitzer Prize winners, first broached the hypothesis that consumers who ate BSE-infected British beef were being stricken by Creutzfeldt-Jakob disease, the human equivalent of BSE. Creutzfeldt-Jakob disease is a fatal neurological disorder that attacks the human brain in much the same manner as BSE destroys the brains of cattle.
Rampton and Sheldon did not benefit from their publisher's sense of timing, obviously, but their "insider's" approach to several of the key events in the during the past three years offer insights and information previously underreported.
For example, the authors tackle the testimony and the transcripts surrounding the Oprah Winfrey Show on dangerous foods and the resulting lawsuit by a group of Texas cattlemen with the vigor of a couple of cub reporters covering their first murder trial. With detailed, though slanted, summaries of the exchanges between Oprah and her guests on that April 1996 show, the authors provide a thorough documentation of why the controversy arose--and why the resulting trial ended in disappointment for the cattlemen.
Later chapters follow the remarkably persistent, and often complex, research of Richard Marsh, a University of Wisconsin-Madison scientist, who pioneered the links between BSE and scrapie, a relatively common neurological disease of sheep, and a rare transmissible spongiform encephalopathy found in mink. Marsh's work strongly suggested that the cause of the TSE in mink was ranchers who fed them meat from downer cattle. Although no one wanted to hear it at the time, 1985, the suggestion was also made that these downer cattle could be harboring BSE without showing overt symptoms.
The implications of that theory, the authors hammer home, is that BSE exists among U.S. cattle, even though no cases have been documented in more than 10 years of Agriculture Department surveillance. As a respected scientist, Marsh himself was careful not to jump to that conclusion, but Rampton and Stauber harbor no such reservations.
As self-styled public relations "watchdogs," the two authors turn in some of their best reporting in discussing the genesis of the "veggie libel" laws, the Texas version of which was the basis for the failed Oprah lawsuit filed by Cactus Feeders President Paul Engler. Going back to the Alar scare of the late 1980s, Rampton and Stauber present a condensed yet highly analytical summary of the PR battles between agri-business firms and their alleged governmental allies, and consumer groups sniping at weak or ineffective regulations governing not only use of pesticides but the "abuses" in the meat industry, such as the lag time that elapsed before the Food and Drug Administration banned the use of ruminant-derived proteins from animal feeds.
If you enjoy the "he-said, she-said" blow-by-blows that have marked the ongoing investigation of President Clinton's alleged transgressions, you'll love Mad Cow USA's recap of the legal and public relations skirmishes triggered by the veggie libel laws.
The final chapters of the book recount the rather tortuous route taken by FDA in reaching its decision to impose a ban on use of rendered beef proteins in feed. Industry skeptics will seize that account as proof of monumental governmental incompetence--at best--and decry the fact that agri-business is in bed with the regulators--allegedly.
Industry supporters will note that the complexities so well-summarized in this book are proof positive that a "rush to judgment" was inappropriate, and that FDA took a prudent approach to the dilemma.
The bottom line to any reasoned assessment of the information in "Mad Cow USA," and all other investigations purporting to get to the "truth" about the threat of BSE, is that no cases have yet been detected in the United States. Does that mean none could ever arise? Only a fool would believe that.
But does it mean that government and industry should be waging an all-out effort to aggressively eliminate any and all risk factors associated with BSE, no matter what the economic consequences?
Again, only a fool, or perhaps a pair of them, could advocate that course.
Sheldon Rampton & John Stauber From Common Courage Press in Monroe, Maine Hardcover, 246 pages, ISBN 1-56751-111-2 For a review copy or author interview - Call (207) 525-0900 Or email to: firstname.lastname@example.org
PR Newswire Tue, Jun 30, 1998BOSTON -- Despite the enormous research effort directed towards understanding Alzheimer's disease, the cause of brain deterioration that leads to memory loss and cognitive decline is unresolved. A new animal model of Alzheimer's disease reported in the July issue of Nature Medicine provides insight into this question and may serve as a model for developing drug treatments for the disease.
The new study implicates the protein in the senile plaques, called amyloid-beta (A-beta) as a cause of brain deterioration in Alzheimer's disease. These fibrous clumps of protein were first observed by Alois Alzheimer in 1907, but their role in the disease was unresolved. In 1990, Bruce A. Yankner, M.D., Ph.D., associate professor of neurology at Children's Hospital, Boston, discovered that A-beta could kill brain cells grown in the laboratory. However, the role of the protein in the intact brain was unresolved.
The new study by Changiz Geula, Ph.D., assistant professor of medicine at Beth Israel Deaconess Medical Center, Bruce A. Yankner at Children's Hospital, Boston, and co-workers shows that when the A-beta protein is introduced into the brains of aged animals, at levels similar to that which occur in Alzheimer's disease, it causes profound brain cell death. In addition, the study implicates the aging process as an insidious partner in the disease.
"Our results show that the aged brain is selectively vulnerable to the effects of A-beta. In addition, animals that are closest to man appear to be the most susceptible to A-beta," says Geula, helping to explain why previous experiments in mice have been inconclusive.
"Not only does this explain the rodent results, it also provides the community with a good model of AD," writes Nature Medicine's editor Adrian Ivinson, in an editorial appearing in the same issue. "What we're seeing emerge in these studies is a clearer picture of what may be the cascade of events that leads to brain cell dysfunction and death in Alzheimer's disease," said Zaven Khachaturian, Ph.D., director of the Alzheimer's Association Ronald and Nancy Reagan Research Institute.
"The study also provides future directions for drug treatment," says Yankner. "If we can learn why the aging brain, but not the young brain, is susceptible to the toxic effects of A-beta, we may be able to target the susceptibility factor with drugs."
The research was conducted at Beth Israel Deaconess Medical Center and Children's Hospital, Boston, and was supported by a grant from the Swiss pharmaceutical company Novartis Pharma, Ltd. BI-Deaconess and Children's Hospital are major clinical research and teaching affiliates of Harvard Medical School.
UPI US & World : Tue, Jul 14, 1998RICHMOND, Va. -- Japanese researchers have developed a way to make 3-D images of the brain that can quickly distinguish Alzheimers disease from another form of dementia.
In a study published in the current issue of Radiology, a research team at the Hyogo Institute for Aging Brain and Cognitive Disorders in Hajime, Japan, reports they have developed a software program that takes five minutes to convert two dimensional magnetic resolution brain images to three dimensions.
The researchers found that almost the entire surface of the brains of Alzheimer's patients atrophied or decreased in size compared with the brains of those without the disease.
Patients with a disease called frontotemporal dementia had brains that atrophied in the front and on the sides. Frontaotemporal is the most common form of dementia in relatively young patients and is commonly misdiagnosed as schizophrenia.
The team tested 18 Alzheimer's patients, 18 with frontotemporal dementia, and 18 without dementia of any kind. This development is "new and exciting" according to Dr Anthony Proto, of the Medical College of Virginia in Richmond and editor of Radiology.
"This development needs to be out there so other scientists can learn of it and apply it to their patient populations and see what happens," said Proto.
Proto told United Press International that this method would require little in the way of capital outlay for hospitals since many already have MR devices in place. All they would need is the software.
Magnetic resolution is a technique which magnetizes protons in the body which in turn sends images to a device that translates them into two dimensional images. At this point the only way to tell for sure that a patient has Alzheimer's is to perform a brain biopsy after death.
But according to Alzheimer Canada spokesperson, Debbie Krulicki, Alzheimer's diagnoses with present methods in living patients is now 80 to 90 percent accurate. However, Krulicki added, it can sometimes take up to three months to eliminate other possible reasons for short-term memory loss. That, she says, is lost time in properly treating patients, and for families to properly plan for the future.
There is no cure for Alzheimer's or frontotemporal dementia at present, but Proto told UPI, proper and easy diagnoses will enhance and help monitor the treatments when they do become available.