Idaho Fears a Cluster of Brain Disease

From the moment Joan Kingsford first saw her husband stagger in his welding shop three months ago, she wanted two things: his recovery and to know what had made him sick.

She didn’t get either. Six doctors and several weeks later, Alvin Kingsford, 72, died. Doctors suspect sporadic Creutzfeldt-Jakob disease.

The fatal brain-wasting illness can only be conclusively diagnosed with an autopsy. Though Joan Kingsford wanted an autopsy, no mortician in the area would agree to handle Alvin’s body if his brain cavity had been opened.

They feared they would catch the rare disease, Kingsford said, who become part of a suspected Idaho cluster of CJD victims. But as she grieved over her husband of more than 52 years, the news was nearly more than she could take. As a member of the Mormon church, which like many religions discourages cremation, 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. They just put some embalming in him and told me I had to have a funeral in three days,” Kingsford said. “Everything he laid on they told us to burn. I felt like they didn’t [care].”

State and federal health officials are trying to get to the bottom of Idaho’s nine reported cases of suspected sporadic CJD this year.

Sporadic, or naturally occurring, cases of CJD have no known cause or cure, but they are not due to eating tainted beef. Beef-related cases are classified as variant CJD, known as “mad cow” disease, which has killed at least 180 people in the United Kingdom and continental Europe since the 1990s.

Investigators’ best tool to pin down the diagnosis -- the autopsy -- is sometimes hard to get, said Tom Shanahan with the Idaho Department of Health and Welfare.

Normally sporadic CJD strikes only about one 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. In the last two decades, the most cases ever reported in Idaho in a single year was three, Shanahan said.

Until 2005. Of the nine suspected cases reported so far, two victims tested negative. Three tested positive for a prion disease, though more tests are pending to determine whether the fatal illness was sporadic CJD. Four were buried without autopsies.

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

“I think we’ll have a better and clearer picture once all of the investigations have been completed,” said Dr. Ermias Belay, a CJD expert with the federal Centers for Disease Control and Prevention. “One thing is very clear in Idaho: The number seems to be higher than the number reported in previous years. But so far, the investigations have not found any evidence of any exposure that might be common among the cases.”

The CDC strongly encourages autopsies for CJD victims, but runs into some common barriers, Belay said. Pathologists are often reluctant to perform the procedures, the cost of an autopsy can be high and some families are reluctant to consent.

And sometimes, like Kingsford, families simply can’t find an undertaker willing to handle a potentially infectious body.

CJD is transmitted through a malformed protein, known as a prion, that is 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 CJD victims as long as extra precautions are taken, according to the organization’s guidelines, but the WHO does not recommend embalming patients who have had autopsies.

Many Idaho morticians get their information on safe handling of CJD-infected bodies from Larry Whitaker, a Beaverton, Ore.-based regional salesman for Dodge Co., an embalming chemical and equipment manufacturer. Whitaker doesn’t claim to be an expert, but he does give presentations on CJD risks to groups such as the Idaho Funeral Service Assn.

“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.”

Joan Kingsford was forced to make such a decision about her husband’s remains much sooner than she had expected.

“It was 2 1/2 months before we knew what was wrong with him, and by that time he was in the hospital,” Kingsford said. “He just lost one faculty after another. First he couldn’t walk, and then he couldn’t eat, couldn’t talk.”

When the doctors said they believed he had sporadic CJD, Alvin Kingsford was so far gone that his wife wasn’t sure whether he understood the diagnosis.

A week before his death, she began trying to arrange an autopsy to confirm the diagnosis. Officials with the Idaho Department of Health and Welfare learned about his illness a few days later, Shanahan said, giving them little time to help find a mortician or a pathologist willing to help accommodate both an autopsy and an embalming.

“I wish we could have done the autopsy, because I think people need to know about this,” Kingsford said. “We definitely have a problem in Idaho.”