- 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 be conclusively diagnosed only with an autopsy, which did
not take place.
- 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.
- 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,
- 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
- Copyright 2005 The Associated Press. All rights reserved.