NJ Creutzfeldt-Jakob cluster turns suburban mom into crusader

NJ Creutzfeldt-Jakob cluster turns suburban mom into crusader

By LINDA A. JOHNSON
Associated Press Writer

October 2, 2005, 10:53 AM EDT


CINNAMINSON, N.J. -- Janet Skarbek's life was forever altered when she read the obituary of an acquaintance in June 2003.

A 56-year-old woman who had worked with Skarbek's mother at the Garden State Park racetrack near Philadelphia had died of Creutzfeldt-Jakob brain disease, the human version of mad cow disease. Barely three years earlier, a 29-year-old accountant at the Cherry Hill track had died of the same rare, always fatal disease.

Skarbek wondered: How could two of just 100 administrative employees at the track be felled by a neurological disease health officials say kills just one in a million people each year, usually after age 60?

"That's the day it started," she recalled.

Almost overnight, Skarbek changed from suburban mother of two, tax manager and Sunday school teacher into an Erin Brockovich-like crusader fighting to keep mad cow disease from spreading through the U.S. food supply.

Skarbek, 37, began combing obituaries and over time identified 18 people she believes died of CJD from 1993 to 2004 and had eaten regularly at the same restaurant at the now-closed racetrack. She also spotted possible clusters elsewhere or learned of them from loved ones of people whose deaths were classified as sporadic CJD.


snip


http://www.newsday.com/news/local/wire/newjersey/ny-bc-nj--madcowcrusader1002oct02,0,2088131.story?coll=ny-region-apnewjersey

I wonder where that "resturant" got its meat. This really gives me cold chills.

Tests show two women died of brain-wasting disease

By CHRISTOPHER SMITH
Associated Press writer



BOISE, Idaho -- Preliminary tests on the remains of two Idaho women show they died of the brain-wasting illness Creutzfeldt-Jakob disease, but additional tests are needed to determine whether it was the naturally occurring form or the variant related to mad cow disease.

Idaho Department of Health and Welfare officials announced the findings Wednesday after notifying the families of the women, one of whom was in her 60s and lived in Twin Falls County and the other who was previously identified by her family as 53-year-old Kathy Isenberg of St. Maries. Because of privacy restrictions, state health officials do not release names of individuals suspected of dying from the disease, which can only be conclusively diagnosed post-mortem.

The results bring to three the number of confirmed deaths this year in Idaho due to diseases related to "prions," or malformed proteins. Earlier this year, tests by the National Prion Disease Pathology Surveillance Center at Case Western University in Ohio determined that another Twin Falls County woman had died from a prion-related disease believed to be Creutzfeldt-Jakob disease.

Additional tests are under way at the lab to determine what form of CJD was responsible in the three confirmed cases.

"Generally, 85 percent of the tests come back as the sporadic, or naturally occurring form, 14 percent come back as the familial form that is passed down through generations and less than 1 percent come back as the variant form," said Tom Shanahan, spokesman for the Idaho agency. "There's never been a variant case acquired in the United States."


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http://www.casperstartribune.net/articles/2005/10/01/news/regional/9c121aece6ea98198725708a007f8e6c.txt

5 people from the small town of Twin Falls, and a woman from Cour d Alene area have died of CJD.
http://www.casperstartribune.net/articles/2005/09/16/news/regional/753b649d25f36d418725707e00590ddf.txt

My interest was peaked.
I found this site useful. http://science.bio.org/bse.news.html

Southeast Asia. Isn't that where the bird flu is coming from?

......If the prion disease is here.
For the prion to be maintained in livestock, does not require that animals be fed animal sourced feeds. It can spread in a herd in other wayts than via feed.
In sheep, TSE (tranmissable spongiform encephalopathy) is tramnsmitted within a herd, through birthing fluids from mother to daughter. Soil where an infected flock has been is a source of infection.

Mad cow is not an infection with a virus or a bacterium. It is caused feeding animal protein cows.

http://whyfiles.org/012mad_cow/

Mad with concern
The first known TSE appeared in sheep, but now people, cows, elk, deer, mink, rats, mice, hamsters and possibly monkeys all get various types of the disease.

Although neither mad cow nor vCJD has been seen in the United States, vigilance and concern are both mounting.

Drugs and vaccines, including those given to millions of American children, have been made with products that could carry mad cow disease, in defiance of repeated requests from the Food and Drug Administration, the New York Times reported:

"The nine vaccines include some regularly given to millions of American children, including common vaccines to prevent polio, diphtheria and tetanus," the paper wrote. "They also include the anthrax vaccine, which the government requires for soldiers serving in the Persian Gulf."

Federal officials said the practice was still safe, and that nobody is known to have caught CJD from vaccine.

>snip<

Messed up in Texas
the state of texas, with cattle skulls overlaying the state's shapeThe alarm bells rang in Texas after 1,200 cows were fed protein-rich byproducts of other cows. Until a few years ago, this was standard practice in the United States, and was considered a smart way to recycle slaughterhouse offal -- brains, spinal cords, spleens that wouldn't do too well in the butcher shop -- unless you love head cheese.

>snip<

But exactly this "like to like" feeding technique lay at the heart of the United Kingdom epidemic. Long after cows began going mad and dying of a brain-destroying disease, the government allowed their byproducts to be fed to other cows.

"It is unfortunate that there has been a failure to comply with FDA regulations," says Judd Aiken, a prion researcher at the University of Wisconsin-Madison. "The key to the whole thing is that we stop the feeding of sheep and cattle to cattle."

Four or five years after the British stopped feeding cattle to cattle, there was, he says, a "precipitous drop" in the mad cow rate. Every mistake, he warns, sets back the date for ensuring that the slow-acting disease is removed from the food chain.

Prion-positive ewes give birth to lambs that will become positive and symptomatic for TSE.
In sheep, its called scrapie. Its studied as a model for mad cow disease.

Soil where Scrapie -positive sheep have lived will remain infective (a source of prion infection) for years.

In Iceland, a sheep farmer had prion (scrapie)in his flock. His flock was destroyed. He removed the top few inches of soil. He got new sheep.
They got scrapie.

Its tougfh. The prion is pretty nearly indestructable.

" Although neither mad cow nor vCJD has been seen in the United States, ....."

There was that "mad" holstein from Mabton, Washington, and a more recent BSE-positive beef cow out of Texas.

Human remains in cattle feed may have caused mad cow epidemic
By Nic Fleming, Science Correspondent
(Filed: 02/09/2005)


The original cause of the 1986 BSE crisis has remained unknown

The mad cow disease epidemic could have been caused by the feeding of material containing human remains to cattle, a scientist claimed yesterday.

Alan Colchester, a professor of neurology at the University of Kent, said the most likely origin of BSE and the subsequent deaths from variant Creutzfeldt-Jakob disease was the import from the Indian subcontinent of bone meal containing infected human remains.


The original cause of the 1986 BSE crisis has remained unknown
Since the first case of BSE was reported in Britain in 1986, the original cause has remained unknown.

The most widely favoured candidate has been the transmission of sheep scrapie, a fatal degenerative disease that affects the nerve system, to cattle through feed.


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http://www.telegraph.co.uk/news/main.jhtml?xml=/news/2005/09/02/nbse02.xml&sSheet=/portal/2005/09/02/ixportal.html

we just called 'mad cow' something different so the beef people wouldn't scare us.

i don't touch fast food beef, only chicken, if i eat meat at all from fast food. or supermarket meat.

and the government knew about it and kept quiet, I don't even want to think about what middle America would do about it. To think that our government would sit by and let millions of American children poison themselves is just beyond comprehension.

BSE is being kept hushed in the US. Many of the cases come from animal food concentrates.

first things the Democrats will push through Congress once they regain power. And if it's found that Bush and his buddies have been protecting the meat industry all this time, the Republican party is finished.

but remember Oprah's comments a few years ago?

entertaining.

They've just come up with a way to test the blood.
It used to be that the brain tissues and brain stem were tested.Animal had to be dead.
Now with a blood test, live animals may be tested. and THE TEST may SHOW PRIONS BEFORE THAE ANIMAL BECOMES SYMPTONATIC. The prions accumulate in the brain and nerve tissue at the last stage of the disease.
When a prion-positive animal is still incubating the disease, and is asymptomatic , prions accumulate in lymph tissues. A healthy appearing animal MAY have lymph tissue full of prions(sausage, anyone?)

fence to obtain samples?

And Erin would have to have access to some very rare tests.
Even if you've got a prion-carrying animal in hand.
There arent very many labs worldwide that do the prion test even on the conventional animal tissues.