rick_reno

BOISE, Idaho - From the moment Joan Kingsford first saw her husband stagger in his welding shop, she wanted two things: His recovery and to know what made him sick.

She got neither. Alvin Kingsford, 72, died recently of suspected sporadic Creutzfeldt-Jakob disease, the fatal brain-wasting illness. The disease can only be conclusively diagnosed with an autopsy, which Kingsford declined to allow.

State and federal health officials are trying to get to the bottom of nine reported cases of suspected sporadic CJD in Idaho this year. Sporadic, or naturally occurring, CJD differs from the permutation dubbed variant CJD, which is caused by eating mad-cow-tainted beef and has killed at least 180 people in the United Kingdom and continental Europe since the 1990s.

“One thing is very clear in Idaho — the number seems to be higher than the number reported in previous years,” said Dr. Ermias Belay, a CJD expert with the federal Centers for Disease Control and Prevention. “So far, the investigations have not found any evidence of any exposure that might be common among the cases.”

Normally, sporadic CJD only strikes about one person in a million each year, with an average of just 300 cases per year in the United States, or just over one case a year in Idaho. Over the past two decades, the most cases reported in Idaho in a single year has been three.

Until this year.

Of the nine suspected cases reported so far in 2005, three tested positive for an infectious disease of the nervous system, though more tests are pending to determine if the fatal illness was in fact sporadic CJD. Four apparent victims were buried without autopsies. Two suspected cases tested negative.

Still, federal and state health officials are stopping just short of calling the Idaho cases a “cluster,” waiting for final test results from the victims who got autopsies.

Fear of exposure
The best tool of investigators to pin down the diagnosis — the autopsy — is sometimes hard to get, said Tom Shanahan with the Idaho Department of Health and Welfare.

Pathologists are often reluctant to perform the procedures, the cost of an autopsy can be high and some families are reluctant to give their consent, officials say.

Joan Kingsford wanted an autopsy done on her husband, but no mortician in the area would agree to handle Alvin’s body after his brain cavity had been opened. They feared they would catch the rare disease, Kingsford said.

Ultimately, she opted to skip the autopsy and have a traditional funeral service.

“A week before he passed away, the funeral homes said they wouldn’t take the blood out” if an autopsy was done on him, she said. “They just put some embalming in him and told me I had to have a funeral in three days.”

CJD is transmitted through a malformed prion found primarily in the brain and spinal fluid of those infected, Belay said. Standard sterilization procedures don’t eliminate the risk of infection; instead equipment must be soaked in a chemical solution for more than an hour and then heated, according to the World Health Organization.

Mortuary procedures — including embalming — can be done safely on intact bodies of CJD victims as long as extra precautions are taken, but the World Health Organization does not recommend embalming patients who have had autopsies.

Larry Whitaker, a Beaverton, Ore.-based regional salesman for the embalming chemical and equipment manufacturer Dodge Company, offers workshops to his clients on safe handling of CJD-infected bodies.

“When the brain has been removed, it is an extraordinary risk,” Whitaker said. “This is one time I think that cremation has to be more than mildly considered.”

A member of the Mormon Church, Joan Kingsford’s church discourages cremation. She was thrown into making a decision about her husband’s remains much sooner than she expected.

“It was two and a half months before we knew what was wrong with him, and by that time he was in the hospital,” she said. “I wish we could have done the autopsy, because I think people need to know about this.”

“We definitely have a problem in Idaho,” she added.

bnhcomputing

Interesting.....

I was planning on going to ID for a muzzle loader elk hunt in about a week. Given I haven't purchased tags, permits, or airline tickets yet, maybe I'll just skip this until the ID folks get this sorted out.[&o]

BrutalAttack

I've checked with my contacts in Idaho Fish and Game and they claim they have heard nothing as of yet regarding this. They also remain adamant that CJD in humans does not equal CWD in elk. They are obviously incorrect from what I have read. My advice would be to hold off on eating Idaho elk until they get this figured out.

tangozulu

Personally i think the good old beef cow would be a far more likely suspect. I'lll bet an elk is a thousand times safer to eat as I have never witnessed an elk consuming another elk.
Lets face it the USDA has done a terrible job testing for mad cow and the most suspect of all animals are buried on the back 40 of the ranch or feeding the coyotes, and are never tested. State Game Agencies have done a much better job testing for CWD.

BrutalAttack

Elk consuming each other has nothing to do with it. Read up on CJD and CWD. I'm not eating my elk either and I wouldn't just say that for the hell of it.

slee

As long as you don't come in contact with the brain and spinal cord, even in a CWD positive animal the risk is low. There has NEVER been a case reported in the US of a person acquiring variant CJD from wild game. And the issue is not elk eating elk. Once the prion is ingested in another species, it has then crossed the species barrier. That's why infected cattle are killed immediately. It is transmissible to other cows once one has been infected. And they aren't sure exactly how it is transmitted within a species. I'm not sure why Wisconsin irradicated itsdeer population in certain areas onlyto transport new deer in. You can't kill the prion with burying orburning the animals. It can still potentiallysurvive in the soil in those areas. The prion is not known to live in muscle tissue though. The cases reported in the US as of 2004 were from people who visited or lived inEurope in the 1980s and 90s,and consumed food made withinfectedanimal brains. And if my memory serves me correctly, there were less than 10 of thosecases. You need to pick your sources of information very carefully. Iwrote a research paper in Physician Assistant school lastfall on this topic, and there is actually very little in the medicalliterature about variant CJD in the US. The other thing to consider is many cases of sporadic CJD, which is a naturally occuring disease in the human population,go undiagnosed for many reasons. The symptoms mimic several other neurologic diseases. The increase in incidence of sporadic CJD diagnoses could easily be the increased awareness and better diagnostic testingin recent years. Theprimarydiagnostic testsare a head CT, post-mortem examof the brain, and patient symptoms before death.You are at no more risk now than you were 6 months or a year ago as long as you don't handle or consume the brain and spinal cord.

BrutalAttack

Thanks for the information, that's what I have read also. My understanding is that a) it may not be known to be transmitted via muscle tissue but that doesn't necessarily mean it couldn't and b) people would be very likely to handle, andmaybe even eat brain tissue if they have strange habits. I also read that the incubation period could be extrememly long, up to six decades or so. Uncurable and untreatable = not worth the risk. Also, considering the fact that they could have gotten an elk in any part of the state or even out of state (if elk is the source which is highly unlikely) I don't think I'm out of bounds in warning people to be cautious.

tangozulu

Hey lighten up brutal.
Elk eaten elk was just a comment, I'm no biologist. (though I am proud to be married to a very good one) I still would think any grass fed elk is superior to a chicken litter (CRAP)fed beef cow. Exposure to a cut as popular as a t-bone steak is enough to increase your risk of exposure.
I still contend the risk from beef would be shown to be much higher if USDA had a testing program half as intence as those for CWD.

jmfa1957

My wife's mother died of sporadic Creuzfeldt-Jakob disease about 12 years ago. This is a REALLY nasty way to go. We do not know for sure how she contracted it but the fact that she had four back surgeries was a likely cause. She was 60 years old when she died.

Spongiform encephalopothies, or brain wasting diseases, can be attributable to a malformed protein (the term is prion.) Mere contact between a healthy protein and a prion converts the healthy protein into a prion. This prion cannot be destroyed by traditional sterilization procedures such as autoclaving or bleach; we think that she got it from a surgical instrument.

Since the transmission mechanism in people is not well understood, my wife and kids cannot even donate blood. We do know for sure that one can contract CJD from contaminated surgical instruments as well as from eating meat from animals infected with spongiform encephalopothiess, such as Bovine Spongiform encephalopothy (aka Mad Cow,) Scrapie (in sheep,) or Chronic Wasting Disease (deer and elk.) We do NOT know for sure whether or not there are other means of transmission, such as heredity.

Hunters in areas where CWD is known to exist are well advised to have the carcass tested, and to avoid contact with brain and spinal tissue/fluids. Once the prion makes contact with healthy proteins in the nervous system, it will work its way up the neural path and into the brain. There ain't no stopping it and the final weeks of an infected person's life are indeed horrible.