Skip to comments.Idaho probes sudden deaths from rare brain disease
Posted on 08/15/2005 11:56:38 PM PDT by Pro-Bush
Idaho probes sudden deaths from rare brain disease Mon Aug 15, 2005 06:23 PM ET
By Adam Tanner
TWIN FALLS, Idaho (Reuters) - In late May Marjorie Skinner played golf well enough to place fourth in a Memorial Day weekend golf tournament. Yet within weeks, the previously vibrant retiree suddenly started losing her ability to speak.
By the time her family buried her on Friday, she was the fifth suspected victim in the same sparsely populated area of Idaho of Creutzfeldt-Jakob Disease (CJD), a rare brain-wasting disease that typically afflicts only one in a million people.
As word of this latest death spread on Monday, local and federal health experts sifted through clues about an illness different from variant Creutzfeldt-Jakob Disease, the human form of mad cow disease.
"Five (cases) in one valley is pretty serious," Sue Skinner, Marjorie's daughter in law, said in an interview. "It's a grave concern in our family."
The mystery has deepened in recent weeks. Only at the end of May did local health officials see a second elderly woman die of the incurable disease involving a malformed protein, or prion, that kills brain cells. After that, they learned of three other suspected cases, including a CJD death in February that was reported only last month.
"Is what is happening in Idaho an anomaly, a statistical fluke? That is possible," said Ermias Belay, a top CJD expert with the Centers for Disease Control and Prevention in Atlanta who is helping advise officials in Idaho. "But once it exceeds 1.5 or 2 per million, you start asking questions."
"If they are all confirmed, it could be odd."
In a year, the United States typically sees fewer than 300 CJD cases, which mete out rapid death to the elderly, according to the Centers for Disease Control.
ANY UNUSUAL HOBBIES?
In Twin Falls, Cheryle Becker, epidemiology manager for Idaho's South Central District Health, is going to families with detailed questionnaires aimed at finding the roots of a disease that could date back 30 years.
She asks about past travels, unusual hobbies and dietary habits, including of organ meats, brain and venison.
"We're asking them if they've consumed elk," Becker said, adding that many hunt venison in this region of the country. "We're not having many people say that they have."
Experts say they do not expect to find a link to eating meat, although locals are asking if there is any connection to the human variant of mad cow disease. "It's very frightening to the community." said Cheryl Juntunen, director of the South Central District Health.
Two confirmed U.S. cases of mad cow disease, one in a Washington state dairy animal in 2003 and the other in a Texas beef cow this year have further heightened concern.
To date, health experts have found few parallels between the women, all of European heritage. Four were Idaho natives, all had children, none had experienced neurological disease.
One had spent time in Britain prior to the outbreak of mad cow disease there, officials said. Several husbands were involved in farming, as is commonplace in a rural farmland region where locals still talk about stuntman Evel Knievel's 1974 attempted jump over the Snake River.
"There are things that lead you to believe this is not variant CJD," Becker said, pointing to the advanced age of the victims and faster death than in mad cow-related cases.
The Centers for Disease Control estimate that spontaneous flaws in cell proteins result in 85 percent of CJD cases. Another 5-15 percent comes from genetic inheritance, leaving just a small percentage of other unexplained cases.
Yet experts say studies of a few past clusters of CJD cases are inconclusive; some say better records and ability to recognize the illness could account for the Idaho mystery.
"I think in the end this may be a statistical fluke," said Christine Hahn, chief epidemiologist for the state of Idaho. "But there is so little known and there have been very few published reports on these clusters."
Families for three out of the five Idaho victims have agreed to autopsies, officials say, and results from those tests may provide essential clues in the coming weeks.
Curious indeed. Please let us know how this story develops if you can. Thanks.
Seems to be rampant in the Democratic party. Look at Dr. Dean, for instance.
They didn't get it all with the recall.
First, many cases of prion disease in humans are likely spontaneous generation i.e. not acquired.
Second, there are prion-based biochemistries in the brain that serve some critical function but which are poorly understood.
Third, a significant fraction of humans have some immunity to prion diseases of the type that is found in beef.
All of which makes it a very complicated and nasty type of disease with little hope of a solution.
I like mine to stand up and Moo at me.
As far as mad cow goes, I don't think it matters if you cook your meat until it's crunchy.
It is still there and just as deadly if it is infected.
A gynecologist, for example?
The prions cannot be destroyed by autoclaving, and can be passed on to the next patient.
I don't think so.
It isn't alive. It isn't a bacteria or virus.
It's an abnormal protein, not a living organism that can be killed. It causes a cascade of abnormal proteins to form from normal tissue.
I still can't figure out what they're so damn mad about.
Standard sterilization will not deatroy prions.
You cannot cook them out of beef and have anything left but charcoal.
IIRC, there was a case in Denver where CJT was passed on to subsequent patients using surgical instruments (endoscopy).
Cool, I don't like my beef well-done! What is CJT? - I know about CJD
Yes, they did get it all. I have a close relative who was very involved in the isolation and destruction of that cow (as well as other animal tissue that might have come in contact with it). The material from that cow never entered either human or animal food.
My bad! I meant CJD.
They caught all the material from that cow. But that cow was presumably infected via contaminated feed, and other cows must have eaten feed from the same infected batch of feed and so would also have been exposed to mad cow disease. Doesn't it seem likely that other cows may have contracted the disease but were never diagnosed?
The real fact in the USA is, CJD isn't a reportable disease in most states, and even then, many are misdiagnosed. Rapid onset is a sure sign of mad cow. This is excactly what happened in the UK. The story doesn't mention if some of the victims were younger.
I also notice they are trying to pass it off to wild meat, and to people who may have traveled to europe, just like they did about 5 years ago when there was another string of deaths by CJD in another state.
The real truth is cows get it naturaly, about one cow in a million, just like humans get it naturaly at about the same rate.
we don't find all the cows, and that's a concern.
We don't diagnose all the human cases properly in all states, and that's a concern as well. It should be reportable in every state, and EVERY single sudden onset case in humans should be investigated and confirmed, which can only be done by autopsy. Regular CJD is slow onset, variant CJD is rapid onset, affects young people within 5 years of eating bad beef.
We need to test every single cow, that's the only way to be safe.
What's worse, is it is transferable human to human from blood products, even surgical equipment. Those prions can servive sterilization. We have to be very carefull with this stuff. It is worse than aids if it breaks out.
That's one of the reasons we like to kill cows off at around 30 months. if they did have BSE, It's concentrated in the spinal tissue and brain, which is why they banned mechanical stripping in order to prevent the ganglea from getting in meat.
Just a hunch, from research my oldest daughter has been involved with for some time now (currently pursuing her PhD).......I'd be looking at blue-green algae blooms in the surrounding ranches.
She's in South Carolina now, studying why bald eagles are dying. In short, loons eat the blue-green algae, eagles eat the loons. When autopsied, the eagles are found to have "holes" in their brains.
Anecdotal, yet possible evidence of linkage between blue-green algaes (which have nasty toxins, folks) and diseases in humans such as Alzheimer's and Parkinsons.
Seems to me, if we were talking about a human to human transmission via a health care provider, a dentist would be a likely mode of transmission.
One question: I was under the impression you had to eat organ meat (i.e. brain, liver, etc.) in order to contact "mad cow disease."
"Just FYI, some of the infected meat from the first "mad cow" went to Idaho."
That may be correct, but you don't develop and die from this disease that quickly. The 6 or so people who died in England from this were (if I've got this right) all linked to some butcher shop were they had gotten meat, but it was decades before they got sick and died.
Do you think it will become common to test all humans for CJD, etc.?
Might just as well. Conventional sterilization temperatures won't kill CD pathogens.
Yeah from Alberta...that would be the big chunk of land above the eastern 2/3 of Montana...
Interesting you should say that. There was an article here just three weeks ago about some pack taking their mechanical spine stripper down (just now); it mentioned the other packs THAT STILL USE THEM.
I think they said it was voluntary...I haven't eaten ground beef that didn't come from our farm since.
Are we talking about the same cow? From an article dated 12/03:
"Despite their assurances of food safety, federal officials have taken the precaution of recalling 10,000 pounds of meat from the infected cow and from 19 other cows slaughtered Dec. 9 at Vern's Moses Lake Meat Co., in Moses Lake, Wash.
Officials say they are still recovering meat and won't know how much has been returned until later this week.
Dr. Stephen Sundlof, head of FDA's Center for Veterinary Medicine, said: "We expect by now that many of the customers who may have purchased some of this meat have been notified by the grocery chain. If not, they can contact those stores" related to the recall."