NEW YORK/SAN FRANCISCO -
Family and friends of
American victims of Creutzfeldt-Jakob Disease, the fatal brain
disorder sometimes linked to mad cow disease, on Friday
questioned whether the wasting illness that killed their loved
ones was actually due to eating contaminated U.S. beef.
After federal authorities said on Tuesday that a cow in
Washington state was found to have mad cow disease, public
health experts have been calling for a review of the U.S.
Agriculture Department's screening procedures for cattle.
But some victim's families have gone further, saying that
the human form of the disease may have already hit the United
States and that the government has been lax in its testing
possible links and enforcing safety standards.
"The most frustrating part of this disease is that there
are no answers," said Chris Turnley, whose brother Peter
Putnam, who grew up in Washington state, died of the disease
last October at age 35. "They need to figure out the cause but
also start figuring out treatments."
So far, none of the roughly 300 cases of Creutzfeldt-Jakob
Disease diagnosed in the United States each year has been
linked to U.S.-produced beef, said Pierluigi Gambetti, director
of the National Prion Disease Pathology Surveillance Center at
Case Western University.
But Dr. Michael Greger, a doctor in Scarsdale, New York,
and coordinator for Organic Consumers Association, said it
would be wrong to take comfort from that statistic. The disease
has a long incubation period and few dementia-related deaths in
the United States are investigated.
"There have been no confirmed cases, but just as there
weren't any confirmed cases of mad cow disease, it is a
function of how hard one looks for it," Greger said.
The variant of the illness linked to mad cow disease was
first reported in Britain, where about 150 people have died and
where mad cow disease was first identified in 1986.
The disease is marked by sudden and escalating neurological
and muscular symptoms, including confusion, depression,
behavioral changes and impaired vision and coordination.
Creutzfeldt-Jakob Disease occurs spontaneously at a rate of
about one case per 1 million people. It is incurable and always
fatal. A related illness, known as new variant CJD, has been
linked in Europe to eating meat from cattle infected with mad
Patricia Ewanitz of Port Jefferson Station, New York, says
she wonders about the death of her 58-year-old husband six
years ago, diagnosed as Creutzfeld-Jakob.
"This didn't have to happen," said Ewanitz, co-founder of
the CJD Voice support group. "We've been warning them
(government agencies) that every cow that goes into the food
chain should be tested."
In Kansas, 62-year-old Linda Foulke died of the disease
last Sunday, and a specialist at the Wesley Medical Center in
Wichita confirmed the diagnosis of Creutzfeldt-Jakob, the
Wichita Eagle reported on Friday.
Bill Patton, Foulke's son-in-law, said doctors told the
family the type of Creutzfeldt-Jakob disease Foulke contracted
was different from the type tied to mad cow disease. But Patton
was quoted as saying the family was worried there might be a
Wesley Medical Center spokeswoman Cheryle Olsen said she
would not comment on the case other than to say the family was
likely too grief-stricken to understand the situation clearly.
Meanwhile in Putnam's case, his family says his death is a
mystery and they are awaiting word from a British laboratory on
a brain biopsy test. He first showed symptoms of the disease
while living in Alaska.
Additional reporting by Jim Christie
in San Francisco, Carey Gillam in Kansas City and Toni Clarke
in New York
Copyright 2003 Reuters Ltd