"This is the worst thing I have ever seen," says Tracie McEwen. "I wouldn't wish it on my worst enemy."
http://www.youtube.com/watch?v=daU1Lx7g6hA
Prion diseases are so awful and the proteins so unpredictable that scientists take extraordinary precautions against infection when studying them in the lab. Patrick Bosque, a neurologist at the University of Colorado in Denver, studied prions in hamsters and mice, which do not appear to be transmissible to people. Yet he routinely wore disposable gloves, shoe covers and a gown, and avoided carrying his lab notebook or other potentially contaminated material out of the lab. Whenever he conducted a procedure that might spray or splash prions, he worked in a special hood to shield his face and upper arms. "Then you're going to tell me I'm going to eat deer?" Bosque asked. "I definitely would not eat deer I thought had been infected."
Is Beef at Risk of Mad Cow Disease Again?
http://www.theepochtimes.com/n2/content/view/45098/
Infected buck found 40 miles from Michigan's U.P.
http://www.freep.com/article/20101113/SPORTS10/101113053/Infected-buck-found-40-miles-from-Michigan-s-U.P.#ixzz15Clw2t5t
Deer Disease and CJD in Humans 1/18/99
Headline: Brain disease a slow goodbye
http://www.purefood.org/Meat/utahcjd.cfm
Mad Deer Disease Hits Wisconsin - Hunters Warned
Health Officials Studying Links
Between Disease And People
http://www.rense.com/general20/maddeerhunterswarned.htm
If Mad Cow Jumps To Humans
Why Not Mad Deer CWD Disease?
By Lou Kilzer
Scripps Howard News Service
6-5-2
http://www.rense.com/general25/deer.htm
Mad Deer Disease: No Joke for Meat-eaters
http://www.northernexpress.com/editorial/random.asp?id=675
Prions in CWD is the same agent that causes Creutzfeldt-Jakob is a rare, fatal illness often described as "fast Alzheimer's"
At least seven people age 66 or younger -- all hunters or venison eaters -- are known to have died of Creutzfeldt-Jakob in the U.S. in the last nine years. The total number will never be known because there's no federal requirement that all cases be reported. Preliminary studies suggest, and some neurologists suspect, that CJD is more common than generally believed -- it's simply misdiagnosed as Alzheimer's. That raises the obvious question: How many people would die of chronic wasting disease before a doctor called it?
In a highly publicized case, three Wisconsin hunters who attended wild-game feasts died of neurological diseases. Two had Creutzfeldt-Jakob, one turned out to have another rare neurological ailment, Pick's disease. Tests are ongoing.
Another victim from Oklahoma died with a freezer full of venison. A 50-year-old Montana elk hunter died last summer; his brain tissue is now being analyzed at one of the world's foremost prion labs, at the University of California in San Francisco. Test results are pending.
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Can humans catch 'Mad Deer Disease'?
Wednesday, May 21, 2003
By Michael Woods, Post-Gazette National Bureau
"Our own nightmare here in the United States is chronic wasting disease of deer," Dr. Corrie Brown said yesterday at the 103rd national meeting of the American Society for Microbiology. Brown, of the University of Georgia in Athens, is an expert on infectious diseases in animals which are used as food.
Chronic wasting disease, also termed "Mad Deer Disease," is caused by a strange infectious protein -- termed a prion (pree-on). CWD also occurs in elk.
It's a cousin of the prions responsible for fatal brain diseases in other animals and humans. Among them are Mad Cow Disease, or bovine spongiform encephalitis, which decimated cattle herds in the United Kingdom and Europe in the 1990s and spread to people who ate infected beef.
About 130 people in Britain have developed the human version of Mad Cow Disease, which is known as variant Creutzfeldt-Jakob disease. The infection is fatal, usually within 2 years after symptoms appear. Estimates of the human toll during the next 80 years range from 540 to 50,000.
http://www.post-gazette.com/healthscience/20030521deer0521p5.asp
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Human incubation is 20 to 40 years, but no one survives more than two years after Creutzfeldt-Jakob disease (CJD) goes active. Unlike AIDS, CJD can kill almost all known species of vertebrates. Unlike bacteria or virus, Prions are not killed by several minutes of boiling, baking, chlorine, alcohol or by any known antibiotic or antiviral agent.
What Soffe and other morticians also worry about are the cases of undiagnosed CJD. The disease is difficult to diagnose without a brain biopsy or autopsy -- and CJD has symptoms nearly identical to Alzheimer's. There is evidence that some cases of Alzheimer's have been misdiagnosed CJD cases. Often cited is a Yale study that found that six of 46 people who reportedly died from Alzheimer's actually had CJD.
More Serious than AIDS: Creutzfeldt-Jakob
Sterilization does not kill prions. They can take extreamly high temperatures even surviving cremation and become released into the air after such burning. Prions are virtually indestructible. Hundreds of scientific reports describe how prions cannot be frozen to death, do not respond to any antibiotics or chemical treatments and withstand temperatures of approximately 1000 degrees F. However, only one study ever done (or released to the public...) on invasive, reusable medical (tonsillectomy) instruments in the UK, over 50% of the instruments were found to be contaminated with deadly prions AFTER REPEATED STERILIZATIONS.
Mad cow disease, chronic wasting disease and similar ailments are all thought to be caused by misshapen forms of special proteins known as prions. In the case of chronic wasting, research shows infection can occur even by proximity to sick deer.
In areas infected with CWD, up to 10 percent or more of deer are found to carry the disease.
Both are transmissible spongiform encephalopathies, or TSEs. They can both be passed from animal to animal, though by different means, and are thought to be caused by misshapen forms of proteins known as prions. These deformed prions eat holes in a creature's brain, inevitably leading todeath.
At the same time, the CDC acknowledged several puzzling cases of patients who died of neurological diseases after eating wild game. In 2003, doctors at the VA Hospital in Seattle reported Creutzfeldt-Jakob disease in three hunters; the CDC would not investigate, saying there was no evidence the men ate tainted meat. A 2002 study documented three Wisconsin men who regularly ate venison and took part in large "game feasts"; two were diagnosed with CJD and one was diagnosed with Pick's disease, a form of dementia.
CWD is spreading
The disease was long thought to be limited in the wild to a relatively small endemic area in northeastern Colorado, southeastern Wyoming and southwestern Nebraska, but it has recently been found in new areas of these states, as well as in wild deer and elk in western South Dakota, and wild deer in northern Illinois, south-central New Mexico, northeastern and central Utah, south-central and south-eastern Wisconsin, central New York, north-east West Virginia, Kansas and west and south-central Saskatchewan. Also, CWD positive moose has recently been discovered in the endemic area of Colorado
They (prions) are also impervious to radiation.”
We're dealing with something that science at the moment simply doesn't fully understand. Total honesty with the public is the only responsible way to proceed."
There is an illness in the wild, leaving a trail of questions as it spreads from state to state.
Are these animals healthy, or stricken with a deadly illness known as chronic wasting disease?
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Hunters turn up CWD in W.Va. deer herd
CHARLESTON, W.Va. — Preliminary test results indicate the chronic wasting disease (CWD) agent was present in five hunter-harvested deer collected in Hampshire County, W.Va., during the 2008 deer firearms hunting season.
“As part of our agency’s ongoing and intensive CWD monitoring effort, samples were collected from 1,355 hunter-harvested deer brought to game checking stations in Hampshire County and one station near the southern Hampshire County line in Hardy County,” noted Frank Jezioro, director for the West Virginia Division of Natural Resources.
The five CWD positive deer included one 4.5 year-old doe, two 2.5 year-old bucks, one 4.5 year-old buck and one 1.5 year-old buck.
Harvest area
All five of the latest positive deer were harvested within the Hampshire County CWD Containment Area, that portion of Hampshire County located north of U.S. Route 50.
However, the CWD agent previously has been detected outside the containment area but still within Hampshire County.
The area in Hampshire County appears to continue to expand as one of the most recent infected deer was approximately 5 miles northeast of any previous known infected deer location.
Detection
The disease has now been detected in a total of 37 deer in Hampshire County — two road-killed deer, one in 2005 and one in 2008; four deer collected by the DNR in 2005; five deer collected by the DNR in 2006; one hunter-harvest deer taken during the 2006 deer season; three deer collected by the DNR in 2007; six hunter-harvested deer taken during the 2007 deer season; 11 deer collected by the DNR in 2008; and five hunter-harvested deer taken during the 2008 deer season.
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So how do you safely dispose of stricken animals?
It isn't as simple as burying their carcasses, or even incinerating them.
Prions are simply proteins, not living organisms, and they can survive almost anything, even hundreds of degrees of heat. Placing infected tissue in a landfill simply removes it, but scientists worry that the prions can leach through soil and groundwater, and spread.
Incineration is possible, but it isn't as easy as burning the carcass in a fire. Temperatures of more than 1,100 degrees Fahrenheit — sometimes up to 1,800 degrees — are required to effectively neutralize prions. Unlike most bacteria, regular cooking won't help at all.
Even many of sterilization techniques used in hospitals, such as autoclaving, are not necessarily effective — though some may be when infected material is dipped in sodium hydroxide, or lye and heated well above the boiling point of water.
A combination of heat — about 275 degrees Fahrenheit — and bursts of unimaginably high pressure — over 100,000 psi — showed promise in reducing prion infectivity, at least in processed meats like hot dogs, in research published last year.
And a similar method has become the default process for getting rid of infected animals. Large vat-like machines known as alkaline hydrolysis tissue digesters, one of which Powers' lab operates, can essentially dissolve entire carcasses.
***
Infected material is placed in a solution of potassium hydroxide — also known as caustic potash — for at least six hours, at 300 degrees Fahrenheit and 60 psi, about four times ambient air pressure.
All remains at the end is a sterile brown, syrupy liquid that can be hauled away to compost.
"It's like a big steam cooker," Powers says. "That'll take care of the prions."
"Disposal issues are tough," says Barbara Powers, director of Colorado State University's Veterinary Diagnostic Laboratory.
