Friday, February 10, 2006
Mad cow
disease death in Utah: Eureka widow looks for answers
HEIDI TOTH - Daily Herald
A Eureka man had the only confirmed case of Creutzfeldt-Jakob Disease,
commonly known as the human form of mad cow disease, in Utah last year.
Max Sorensen died on Dec. 30 at age 62, just five weeks after doctors at the
University of Utah diagnosed him with the degenerative brain disorder. He
was autopsied and quickly buried in a sealed metal casket, without being
embalmed because of the risk it would present.
The worst part of the devastating ordeal for his wife, Julie Sorensen, is
the uncertainty. She's not sure when he got the disease or how he got it,
and she's not getting much help from the doctors, because they don't know
much more.
"I don't think they have any idea," she said. "They know so very little
about it they really can't give us any definite answers."
CJD is most commonly known as the human form of mad cow disease, but most of
the cases are unrelated to the bovine disease. The disease is rare; about
one in a million people worldwide have it. Most have the sporadic type; they
have no known risk factors but got it anyway. It's not contagious except
through contact with an infected brain, spinal fluid or nervous tissue.
Sorensen's death was the first case in Utah in the past few years. The state
average is about two deaths a year, said Dr. Susan Mottice, an
epidemiologist for the Utah Department of Health. Some years there are
several deaths, while other years there aren't any. CJD is a neurological
disease caused by infectious prions, a type of a normally harmless protein
in the body. When a prion mutates to become infectious, the person has CJD,
even though it can remain dormant for up to 40 years, according to the
National Institutes of Health.
Early symptoms of the disease include problems with muscle coordination,
personality changes and impaired vision. As it gets worse an infected person
can have involuntary muscle jerks, blindness, weakness in arms and legs and
eventually a coma.
"You would see a rapid deterioration in things like motor skills, speech and
the ability to care for oneself," Mottice said. "To some degree it might
resemble Alzheimer's, except it's more severe and it would progress much
more rapidly."
About 85 percent of CJD cases are sporadic; another 5 to 10 percent are
genetic, according to the National Prion Center at Case Western Reserve
University. The rest are acquired through the transplant of infected tissue,
contact with infected tissue or with contaminated instruments or the
ingestion of contaminated meats. Trying to protect people from this form of
the disease is why United Blood Services and the American Red Cross stopped
accepting blood donations from people who have spent a significant
consecutive amount of time in countries with a higher incidence of
degenerative brain diseases.
Sorensen is suspected to have had a sporadic form of the disease, although
Julie Sorensen and her family think it might be related to his hunting
habit. She believes handling animals' horns could have been what got her
husband sick.
"We all feel that this has come from deer, we really do," she said.
Chronic wasting disease, another prion-caused degenerative disorder, has
been found in Utah deer as recently as October, according to the Utah
Division of Wildlife Resources. Doctors don't know enough to verify her
guess; Mottice said there is no evidence linking chronic wasting disease to
CJD, and scientists at the National Prion Center believe there's a
possibility chronic wasting disease can be transmitted to humans.
Doctors do believe people who eat infected cows can become infected and will
eventually develop variant or acquired CJD after an incubation of about 12
years, according to the center. Mottice said she doesn't know of any of
these cases that were acquired in the United States; most were picked up in
Europe. Infected beef products usually include or were in contact with the
spinal column or nervous tissue also, not just muscle, she said.
There's no cure and no way to treat CJD yet because doctors aren't sure what
they're dealing with. The disease hasn't been linked to a virus or a
bacteria, and the prion has a number of unique characteristics that make it
difficult, as the Sorensens found out.
"It's not a live organism, so there's no way they can kill it," Julie
Sorensen said.
It also doesn't have any genetic material, according to the National
Institutes of Health, and a long incubation period. The harmless and
infectious prion are almost identical; both are made of amino acids but the
infectious prion is folded in a different way. In some sporadic occurrences,
harmless prions simultaneously change into the infectious form and then
alter prions in other cells in a chain reaction.
But while she wants to know the answers, Julie Sorensen is trying to put her
husband's disease behind her and focus on their 42 years of marriage.
"His kids and grandkids were just his whole life, and everything that he
done, he done with them," she said.
Heidi Toth can be reached at 344-2543 or htoth@heraldextra.com. This email
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CJD belongs to a family of human and animal diseases known as transmissible
spongiform encephalopathies.
Spongiform refers to the characteristic of an affected brain, which becomes
filled with holes.
Human types
Kuru -- identified in people of an isolated tribe in Papua New Guinea and
has almost entirely disappeared
Fatal Familial Insomnia and Gerstmann-Straussler-Scheinker disease -- very
rare hereditary diseases found in a few families throughout the world
Animal types
Bovine spongiform encephalitis -- found in cattle; more commonly known as
mad cow
Scrapie -- found in sheep and goats
Feline and mink encephalopathy
Source: National Institutes of Health
This story appeared in The Daily Herald on page A1.
http://www.heraldextra.com/content/view/165116/3/