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Family: Idaho man died of brain-wasting CJD

Published December 24, 2013 5:49 pm

This is an archived article that was published on sltrib.com in 2013, and information in the article may be outdated. It is provided only for personal research purposes and may not be reprinted.

Boise • A 65-year-old central Idaho man died earlier this month after his family said he developed a rare brain-wasting disease that results in blindness, weakness of the extremities and coma before killing its victims.

Kenneth Tedford Wotring of Salmon died on Dec. 17. His family said he developed Creutzfeldt-Jakob disease, according to his obituary.

The case hasn't yet been confirmed by the Idaho Department of Health and Welfare, which tracks instances where Creutzfeldt-Jakob is suspected. Tom Shanahan, a spokesman at the agency, says an autopsy must be performed first before it confirms presence of the disease.

"That's a family decision," Shanahan said Tuesday, adding his agency couldn't comment on specifics of such cases due to medical privacy laws.

Jan Wotring, his wife who according to the family's obituary was at his side when he died, didn't immediately return a phone message on Tuesday.

Tammy Bowerman, the owner of the funeral home in Salmon where Wotring's obituary appeared, said Tuesday the family had an autopsy performed on Wotring.

Bowerman wasn't certain if it had been completed or whether it had confirmed the disease.

According to the National Institutes of Health, Creutzfeldt-Jakob disease is a rare, degenerative, invariably fatal brain disorder. Typically, symptoms start about age 60, and about 90 percent of individuals die within 1 year.

In the early stages of disease, people may have failing memory, behavioral changes, lack of coordination and visual disturbances. Eventually, many victims fall into a coma.

Its cause is often a mystery.

"Some researchers believe an unusual 'slow virus' or another organism causes CJD," according to the NIH's Institute of Neurological Disorders and Stroke, based in Washington, D.C. "However, they have never been able to isolate a virus or other organism in people with the disease."

Nationwide, there are only about 200 cases annually. There have been at least 20 confirmed cases in Idaho since 2005.

So far this year, three cases of the disease have been confirmed in the state, not including Wotring's.

One case involved somebody with a genetic predisposition to the disease.

Two were considered sporadic CJD, meaning their origin is unclear, Shanahan said.

"We just haven't found any common threads."

A third form, acquired Creutzfeldt-Jakob disease, is the rarest and can be transmitted by exposure to brain or nervous system tissue that carries the disorder.

Meanwhile, beef-related cases are classified as "variant CJD," also known as "mad cow" disease, which killed at least 180 people in the United Kingdom and continental Europe since the 1990s, though there's never been a case of this form of the disease reported to have been transmitted within the United States, Shanahan said.

However, concerns about emergence of a variant form linked to beef — and the associated public health risk — is one reason his agency and the federal government is so interested in instances when Creutzfeld-Jakob is suspected.

"They really want to monitor to make sure we're not seeing any variant CJD creep in," Shanahan said.

Attention on Creutzfeldt-Jakob intensified in Idaho in 2005, a year in which there were five confirmed cases but a number of additional suspected cases where no autopsy was performed — in some instances because medical practitioners feared they could contract it.

In Wotring's case, "he fought his battle with this devastating disease from his home overlooking the Continental Divide," according to his obituary, which indicated he was an avid outdoorsman who had previously worked for the U.S. Forest Service, the federal agency that manages much of the public land surrounding Salmon.