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Story on CJD (Mad Cow-like Disease)

USA Today
January 19, 1999

Rare Illness taking a swift toll on young family

By Anita Manning
Special for USA TODAY

A young man in Kaysville, Utah, is dying of a disease so rare but with
such international notoriety that it has turned what would normally be a
private tragedy into a rallying point for public health activists.

Creutzfeldt-Jakob Disease or CJD, is thought to strike about one in a
million people around the world, most of them over the age of 50.

But Doug McEwen is only 30.

At his age and younger, only five cases of CJD per billion people are
reported each year worldwide, says Lawrence Schonberger of the Centers for
Disease Control and Prevention. "It's rare but it's not unheard of,'' he
says.

But with the unnerving news that surfaced in the past two years about
a variant of CJD, "you can understand the concern,' Schonberger says.'

"New variant'' CJD is the fatal illness that has struck at least 33
young people in the United Kingdom and has been linked to "mad cow''
disease, the latter thought to have emerged because of certain animal
feeding practices. People were infected by eating meats, leading Great
Britain to slaughter thousands of cattle.

There has never been a connection made between feeding practices and
classic CJD, which even looks different under a microscope than its new
variant, Schonberger says.

In McEwen's case, "there was concern initially about it being new
variant because of his age.'' A brain biopsy performed Nov. 24 confirmed
that McEwen has CJD - and ruled out the new variant.

But some people are unconvinced, and many still-unanswered questions
are being investigated by state and federal health officials: How did
McEwen get this disease? Does the fact that he was a frequent plasma
donor suggest a risk to the blood supply?

Could McEwen, a hunter, have contracted this illness by contact with
wild deer and elk, which sometimes carry a disease similar to mad cow
disease? Could he have come in contact with infected beef or some other
food during a year in rural parts of Canada more than a decade ago, when
he was serving as a missionary with the Mormon Church?

Earlier this month, a group of consumer activists and families of CJD
victims, including the McEwens, petitioned the CDC and the Food and Drug
Administration to enact stricter measures to protect the public from
"transmissible spongiform encephalopathies'' (TSEs), the family of
diseases that includes CJD and similar diseases of animals.

The petitioners think there's a much bigger threat to U.S. consumers
posed by animal feeding practices and imcomplete reporting than government
agencies acknowledge.

So the activists asked the FDA to restrict the feeding of animals to
other animals -- a practice associated with the emergence of mad cow
disease in England -- and to ban any TSE-infected material from animal
feed, cosmetics, fertilizer or other products. They also want the FDA to
require meat producers and renderers to keep records for ten years. The
coalition called on the CDC to initiate a national program to test, study
and require reporting of all human cases of TSE.

Michael Hansen, a scientist with Consumer's Union who has criticized
U.S. policies permitting animal-to-animal feeding, says "There's some
suggestive evidence that some of the sporadic CJD might have a connection
with animal products.''

He notes that McEwen hunted and ate game from areas where animals have
been known to be infected with a TSE called chronic wasting disease.

He suspects that in some cases, at least, "there a genetic
predisposition triggered by something in the environment.''

McEwen's wife, Tracie, 28, who is caring for him at home with the help
of friends and hospice, can only speculate. She doubts he acquired the
disease from eating deer or elk, because the whole family ate that food.

But she wonders about that trip to Canada. "LDS (Latter-Day Saints,
or Mormon) missionaries go out and they eat what's served to them. I
wondered if he did ingest something, animal brain or something he didn't
know,'' she says.

But aside from the immediate crisis, what concerns her most is the
idea that her husband might have passed the infection unwittingly to
someone else through his donations of plasma. "He donated well after his
symptoms started,'' she says. "He was at the point where he was
forgetting phone numbers and names, but if I'd thought he had a fatal
disease, he wouldn't
have donated.''

Health officials have said there is no evidence that CJD can be
acquired through blood transfusion, but the FDA has quarantined all the
blood products containing McEwen's plasma donation since last January.
Plasma donated before January is thought to have been already used.
"They're tried to assure me it can't be transmitted that way,'' says
Tracie McEwen, but she's skeptical: "I don't think they know.''

So much is unknown about CJD, first described in the 1920s by two
German neurologists. Symptoms include personality changes, problems with
memory and thinking, and loss of muscular coordination. Death usually
comes within a year of the start of symptoms. A growing number of people,
including family members of CJD victims, believe the disease is
underreported, often going misdiagnosed as Alzheimer's disease or
dementia.

"Dementia is a symptom, not a disease,'' says Mel Steiger of Salt Lake
City, whose wife Ellie died of CJD on March, 19, 1998, at the age of 57.
"I'm of the opinion that a fairly high percentage of Alzheimer's victims
are really sick with CJD.''

At least one small study supports that. In 1989, Yale researchers
reported that of 46 cases diagnosed as Alzheimer's disease, six were
proved at autopsy to be CJD.

In Utah last year, seven people in a population of 2 million were
diagnosed with CJD, including McEwen. State epidemiologist Craig Nichols
says that's an anomaly. "It's not unusual to have clusters, just
statistical abnormalities,'' he says. "The rate over time is consistent
with what you see in the U.S. and Britain.''

Nichols cautions against drawing alarming conclusions based on
incomplete data. "What people have to understand is this is the infancy
of investigation. You need to go back to the early 1980s when we were
doing HIV investigation and look at the controversy and hypotheses at that
time,'' he says. "We will ask about consumption of British beef and wild
game without having any evidence that those are linked to the patient or
any other patient.''

"But people who don't understand how epidemiology operates jump to
conclusions and think that the questions have significance. We do not
know how (McEwen) became infected. We have recommended genetic testing,
because familial links are known to exist. That has not been ruled out.
As to any other source, it's completely unknown.''

Six months ago, Doug McEwen was a big, strapping guy, vital and
healthy. He was advancing as a territory manager for a grocery brokerage
and looking forward to moving into a new home with his wife, who teaches
9th grade geography, and their two little girls, Sharon, 8, and Rilee, 3.

"This is when life is supposed to get good,'' says Tracie.

It was not to be. His illness started in early summer, when he forgot
how to spell his wife's name. In an account of the chronology of events,
which she allowed to be posted on an Internet Web site last week
(www.mad-cow.org), Tracie McEwen writes that he quickly grew more
disoriented.

"By the end of July, he was really having trouble. He was in Idaho on
business and was very late calling home one night.'' When he did call, he
said he'd been unable to remember their phone number - and unable to call
directory assistance because he could not remember how to spell his last
name.

Because his work performance was suffering, Doug quit his job on Aug.
10.

"He thought it was the stress of travel,'' she says in an interview.
"He didn't want to not do a good job.'' But when he went to look for new
employment, he could not fill out applications. That's when he sought
medical help and began a staggering round of tests.

"I was told that he had six to eight weeks left to live, at best,''
she says. "He's still doing pretty well,'' despite bad days.

"He told me he didn't want to continue to live like this,'' she says.
"This is what's troubling - he knows he's dying. He knows when he wants
to say something and can't. He's aware and it's an extremely horrible
situation for him. My husband is trapped and dying in his body and
there's nothing he can do about it. He knows.''

Side Box:

Creutzfeldt-Jacob disease
A distant cousin of the illness know as "mad cow" disease in England,
Creutzfeldt-Jacob disease (CJD) is one of a family of killers that infect
the brain, causing characteristic holes that resemble sponges under a
microscope. They're called "transmissible spongiform encephalopathies,"
or TSEs. The National Institute of Neurological Disorders and Stroke says
there are three major categories:
* Sporadic. The most common type, accounting for at least 85% of
cases, it affects people who have no known risk factor.
* Hereditary. About 5% to 10% of CJD cases in the USA occur in people
who have a family history of the disease and/or test positive for a
genetic mutation associated with the disease.
* Acquired. Fewer than 1% of cases are caused by exposure to brain or
nervous system tissue, usually with the disease.
The appearance of a new variant (nvCJD) in younger-than-average people
in Europe has been linked to consumption of beef contaminated with bovine
spongiform encephalopathy, or BSE, commonly called mad cow disease. It is
one of several TSEs that affect animals.

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