Deer Disease and CJD in Humans 1/18/99
Headline: Brain disease a slow goodbye

Subhead: His illness at center of global debate

Author: By Elaine JarvikDeseret News special writer, Deseret News (Utah)

Doug McEwen's decline began in the summer of 1998 as something so benign you
would have sworn it was just a touch of forgetfulness. Now, six months later,
McEwen and his wife, Tracie, find themselves in the center of a national
debate about "mad cow" disease and the government's role in preventing it.

Tracie McEwen also finds herself facing tough decisions, as dueling scientists
lobby to be the recipient of her husband's brain once he dies.

In early January, Tracie McEwen joined several activist groups --
including the Center for Food Safety and the Center for Media and Democracy -- as a
petitioner in two legal actions demanding changes in the way the government monitors and
prevents mad cow disease and related disorders, including a human version
known as CJD, short for Creutzfeldt-Jakob disease.

"Terminally ill Utah hunter, age 30, could be the first victim of U.S. 'mad
deer' disease," read the headline of a press release issued last week from the
Center for Food Safety in Washington, D.C.

Like everything surrounding mad cow disease and its medical cousins, the
case of Doug McEwen reveals how confusing, controversial and provocative the
subject is. Can CJD be caused by the food we eat? Does that food include deer meat? Are
cases like this the beginning of a world epidemic, or are food activists using
scare tactics?

The issues are further confounded by a string of initials and disease names:
CJD, CWD, BSE, TSE, mad cow, mad deer. What they have in common is a set of
similar and terrifying neurological symptoms, brain tissue that looks like
sponge (hence the term "spongiform encephalopathy," the medical term for the
diseases), and a disease course that ends, always in death.

The disease can incubate for decades before symptoms appear. Diagnosis is
difficult, and reporting of the disease has been lax, so it's not even
clear how
many cases of CJD there are in the United States. Perhaps most
frightening, the
disease agent -- a deviant form of protein called a prion -- can't be
killed by
radiation, freezing or sterilization.

In England, a "new variant" form of CJD has been linked to bovine spongiform
encephalopathy, also known as mad cow disease. So far 40 people have been
diagnosed with BSE, which many scientists believe came from eating British
But this is a point of contention, too. Stanley Prusiner, who received the
Nobel Prize in medicine for his work on prions, is among those who isn't

The link between human disease and "mad deer" disease -- that is, between CJD
and CWD (chronic wasting disease) -- is even more unclear.

Six percent of deer in northeastern Colorado and southeastern Wyoming have
been found to have chronic wasting disease. If it turns out that Doug McEwen has a
form of CJD similar to this "mad deer" disease, his would be the first
confirmed case. "If there's going to be a canary in the coal mine," says one researcher,
"it will be McEwen."

But it may take years to find out.

The beginning

Doug McEwen was 29 when he developed his first vague symptoms of CJD. Tracie
would call Doug at work and tell him to bring home some milk and he'd
forget to do it and then say he didn't even remember her call. That was in early
July last year. Then, in late July, Doug went to Idaho on a business trip.

As usual, Doug would call home every night to tell Tracie and their two
daughters good night. One night, though, Tracie didn't get a call till 11.

"I couldn't remember our phone number," Doug told her.

"Why didn't you call directory assistance?" she asked.

"I can't remember how to spell my last name," he said. By August, when he
decided to quit his job, he couldn't write the letter of resignation
because he couldn't remember how to turn on the computer.

At first his doctor suspected depression or stress, and Doug was sent to a
psychiatrist. In mid-September, on the eve of Doug's first appointment with a
neurologist, the McEwens happened to see Kearns resident Mel Steiger being
interviewed on TV about his wife, Ellie, who died of CJD last March. Ellie's
symptoms -- the memory loss that proceeded eventually to Ellie's inability to
recognize her own family -- sounded distressingly familiar.

"When we got to the neurologist the next day, I still remember him saying 'I
know what you are worrying about, but he is too young and this disease is too
rare,' " Tracie recalls in a letter posted on CJD Voice, a Web site for family
members of people with the disease. CJD generally shows up in people older

When testing of McEwen pointed to the possibility that it was CJD, doctors
decided to send tissue of McEwen's brain to researchers in Ohio. The biopsy
confirmed the CJD diagnosis.

Currently, scientists at the National Institutes of Health are testing Doug's
DNA to determine if he has a genetic flaw that caused the disease. Ten percent
of CJD cases are believed to be inherited. If Doug falls into this
category, his disease will be an open-and-shut case.

But if he didn't inherit the mutated gene, the case gets murkier. Like other
CJD cases, Doug's makes clear how much we still don't know about the disease.

About 1 percent of CJD cases are believed to be "iatrogenic," that is
resulting from medical procedures, including corneal transplants, contaminated brain
electrodes and growth hormones. Doug has had none of these. (A couple of CJD
cases have raised questions about dental procedures, but there has been no
evidence to date that can prove a link.)

CJD cases that aren't genetic and aren't iatrogenic are labeled
"sporadic." But, although the name implies that the disease appears randomly -- the estimate is
that one in a million people for some reason develop mutations in their brain
cells -- it may be that we just don't yet understand what causes CJD, says
Thomas Pringle, a microbiologist and staff member of the Sperling Biomedical
Foundation in Eugene, Ore.

Eating infected meat could be one of those causes. In Doug's case, all that's
known for sure right now is that he is not suffering from "new variant
CJD," the English version that may be connected to English beef. But what about deer

Hunting connection?

According to Tracie McEwen, Doug has been eating deer, elk and antelope
since he was a little boy; as a couple, they ate game two or three times a month in the
fall and winter. As a hunter, he also handled deer carcasses.

Tracie's gut feeling is that Doug did not get CJD from game, especially since
she doesn't think he or his brother ever hunted in northeastern Colorado or
southeastern Wyoming. But other people think Doug could end up being the first
link between CWD and CJD.

What we do know, says Pringle, is that epidemiological studies have identified
several "risk factors" for CJD -- that is, some things that many of the cases
had in common -- and one of those was a diet of "wild game." It "doesn't
prove causation," explains Pringle, but it is a "correlation."

The Utah Department of Health is investigating Doug McEwen's illness, as
well as the seven other cases of CJD in Utah in 1998. Meanwhile, the official position
is that "there is nothing right now to link his illness to eating wild
meat" or handling wild meat, says epidemiologist Gerrie Dowdle, manager of the
department's communicable disease control program.

To suggest that Doug's diet may have caused his disease is "purely
assumption," says Dowdle. "We haven't heard of any cases of humans getting it from elk or
deer. That's not to say it's not possible," she adds.

Can CWD be spread to humans? Scientists at the Rocky Mountain Labs in
Hamilton, Mont., are conducting tests that may provide some part of the answer,
according to researcher Byron Caughey.

Scientists don't even know how deer get CWD. Did it first appear as a sporadic
mutation and then get spread to other deer? Did deer get it from sheep,
who can have a similar disease known as scrapie?

At the Center for Food Safety, Ronnie Cummings's theory is that the
disease was spread to deer at central deer-feeding stations, through deer feed that
includes rendered animal protein -- that is, ground up meat that might have come from
other diseased animals.

(Feeding animals to animals is believed by many to be the cause of the
spread of other forms of the disease, including the bovine version. The Center for Food
Safety's petition filed Jan. 7 called on the FDA to "close serious
loopholes in U.S. animal-feed regulations which currently allow types of cannibalistic
feeding practices known to cause and spread 'mad cow' type disease." These
include "feeding pigs to pigs, pigs to cattle and cattle blood products to

If a deer has CWD, would a hunter be able to tell? "We don't know if
animals can be infectious without showing symptoms," says Beth Williams, professor of
veterinary science at the University of Wyoming and a lead researcher in CWD.
"But we can detect the abnormal protein in the brain of animals before
they get sick." Are hunters shooting deer whose initial symptoms might only include a
clumsiness that makes shooting them easier?

According to Utah state veterinarian Michael Marshall, animal health officials
in Utah are "monitoring carefully" for chronic wasting disease at privately
owned elk farms, "and have found no occurrence whatsoever." In addition, some
150 Utah deer brains have been collected and sent to the state veterinary
diagnostic laboratory at Utah State University. So far all 80 tested have
proved negative, he says.

More questions than answers

Doug McEwen's brain may provide some answers about CJD and perhaps even its
connection to "mad deer" disease. And that means that several different
laboratories will be vying to get their gloved hands on his brain once he

"We want to make sure reliable people get it," says Cummings of the Center for
Food Safety. "We don't trust Prusiner and (Paul) Brown (of the National
Institutes of Health). We think they're capable of repressing results."

But it could take a couple of years for the brain tissue to yield answers.
First researchers have to determine what the strain of CWD would look like once
it was transmitted to humans.

At this point, Tracie McEwen isn't sure she wants to give her husband's brain
away. An autopsy would not only mean no open casket, it would also mean Doug
might not be embalmed.

This past weekend, 220 members of the Utah Funeral Directors Association
met in St. George; high on their list of agenda items was what to do about CJD. The
association has issued no guidelines, says UFDA president Kurt Soffe, but is
trying to educate its members about the dangers of the disease.

One study that strikes Soffe as significant: Prions placed on an inanimate
object were found, three years later, to still be infectious. The inanimate
objects mortuary workers would be concerned with, of course, include embalming
tables and instruments. Cleaning and sterilizing equipment can reduce the
number of prions but don't seem to totally eliminate them. It is not known how much
infectious material is needed to cause disease.

In addition, it turns out, the formaldehyde used by embalmers makes prions
even more durable, hardening the encasement around the protein.

What Soffe and other morticians also worry about are the cases of undiagnosed
CJD. The disease is difficult to diagnose without a brain biopsy or autopsy --
and CJD has symptoms nearly identical to Alzheimer's. There is evidence that
some cases of Alzheimer's have been misdiagnosed CJD cases. Often cited is a
Yale study that found that six of 46 people who reportedly died from
Alzheimer's actually had CJD.

A new study of CJD just reported last week in the British journal "Lancet"
raises even more troubling questions. Researchers in Great Britain
reported that CJD prions could be found in tonsil and appendix tissue.

"Although the concentration of infectious prions is expected to be lower in
these tissues than in the brain, the opportunity for iatrogenic transmission
from appendicectomies or tonsillectomies should not be overlooked. . . .
Perhaps the adoption of universal precautions, based on the assumption
that the material is infectious until proven otherwise, would be the most prudent

There are still more questions than answers. In the meantime, Tracie
McEwen gets calls from well-meaning strangers who insist that they know just the right
herb/magnet/tonic to make Doug well. One woman even wanted to send breast

Doug McEwen continues to get worse, almost daily. He has to work hard to
remember how to use the stairs. His speech has become garbled; he has begun
having seizures and has become incontinent.

Most disturbing, he will often withdraw from his family, refusing to even look
at 8-year-old Sharon when she climbs on his lap.

"This is the worst thing I have ever seen," says Tracie McEwen. "I
wouldn't wish it on my worst enemy."
Copyright 1999, Deseret News Publishing Co.