- Rose and Jim Henry wanted a home brimming with babies
and togetherness.
-
- In nearly 50 years of marriage, the high-school sweethearts
had 10 children and rarely spent time apart.
-
- So after Jim Henry died, when the grown children stopped
by their mother's Olympia home to find her sitting alone in the dark or
forgetting to bathe and eat, some assumed she was simply adjusting to life
alone in a big house -- the same home once filled with family.
-
- But after watching their mother swiftly consumed by a
mysterious ailment and die, Henry's children opted for an autopsy.
-
- "I have a lot of brothers and sisters and we all
wanted to know for the benefit of the family, was this Alzheimer's?"
said Jeanne Giese, Henry's oldest.
-
- The autopsy was conducted shortly after her death in
1997 at age 74. But because of bureaucratic bunglings yet to be explained,
the family didn't get the results until early this year -- just a few weeks
after the nation's first case of mad cow disease was discovered near Yakima.
-
- The conclusion: Rose Henry suffered from Creutzfeldt-Jakob
disease.
-
- Now, largely because of that mad cow case, more people
are wondering whether their elderly relative might have suffered from CJD
-- a degenerative disease of the nervous system -- or its variant, the
human form of mad cow disease.
-
- And with so little known about the deadly diseases, some
are asking if any of those cases might be traced back to bad beef.
-
- Not likely, say most scientists.
-
- But at the same time, doctors are being urged to more
carefully diagnose dementia and pursue autopsies for suspect cases -- only
an autopsy can determine whether CJD is present and in what form.
-
- Many elderly people with dementia or other symptoms of
Alzheimer's probably are never seen by a neurologist. And although Alzheimer's,
CJD and human mad cow disease look quite different to a specialist, there
are common symptoms that many doctors might not recognize.
-
- "I think it's a rare enough condition that most
neurologists have not seen very many cases, either," said Dr. Eric
Larson, director of the Center for Health Studies at Group Health Cooperative
and head of a research study on Alzheimer's and aging.
-
- Unlike the Henrys, most families with a loved one with
dementia do not opt for an autopsy.
-
- Jeanne Hall went to her grave in January at age 83 without
an autopsy.
-
- Now her son isn't sure that was the right decision.
-
- When Hall started losing her balance about a year ago,
Steve Hall took her to the doctor. She'd been treated for vertigo. She
thought she had wax in her ears.
-
- "She was a tough old lady," said Hall, a Tri-Cities
contractor. "She was trying to fix herself."
-
- The first doctor said she probably had a stroke. The
second thought she might be suffering complications from a brain tumor.
-
- Meanwhile, Jeanne Hall continued to deteriorate. Her
speech slurred. And she had trouble directing her legs, said Hall, who
watched her stand up from the couch, take several steps to one side and
spin around to face him.
-
- "She got to her feet and did a cross step like Charlie
Chaplin," Hall said.
-
- Her movements reminded him of the cows, described in
news reports on mad cow disease, that couldn't walk straight or stand up.
-
- Doctors assured him his mother did not die of human mad
cow disease, but Hall wonders why they never suggested an autopsy.
-
- "Older people tend to be put on a shelf," Hall
said. "Why wouldn't you have an interest in following up with people
like my mom who die if not mysteriously, then certainly undiagnosed?"
-
- Closer watch
-
- The aftermath of Rose Henry's death illustrates why the
system doesn't always work. After her death in August 1997, her family
waited for the final word. The children paid for an autopsy.
-
- More than six years later, her records surfaced.
-
- Prompted by a Seattle Post-Intelligencer reporter's request,
Dr. Tom Montine, head of neuropathology at the University of Washington,
dug up Henry's slides.
-
- He studied the brain samples himself and confirmed the
preliminary diagnosis of traditional CJD, for which there is no known cause.
-
- "Obviously with Rose Henry there was a breakdown
in communication," said Montine, who arrived at the UW in 2002. "We
can't let cases fall through the cracks and have families wondering what
really happened."
-
- A new statewide surveillance system is meant to close
the loopholes that could be letting other cases such as Henry's go undiagnosed
or unrecorded.
-
- Otherwise, "We're never going to know anything about
these diseases," said Jo Hoffman, state epidemiologist. "We're
never going to know what the risk factors are and we're never going to
know how people are exposed."
-
- About one in a million people come down with traditional
CJD each year. Since 1997, 35 people in Washington state have died of traditional
CJD, according to state health department records. Less than half of those
cases were diagnosed with an autopsy.
-
- Scientists believe people contract variant CJD by eating
beef from cows infected with bovine spongiform encephalopathy, or mad cow
disease.
-
- The mutant proteins believed to cause these brain-wasting
diseases are called prions.
-
- There are about 150 known cases of human mad cow disease
in England and Europe. Only one U.S. resident has been diagnosed with mad
cow disease -- a Florida woman who grew up in England.
-
- Closer surveillance is unlikely to unearth a hidden epidemic
but it's possible some CJD cases are being missed, say doctors and researchers.
-
- "Not everybody's going to have the stereotypical
pattern (of symptoms)," said Montine. "Do they have CJD? Are
they basically flying under the radar?"
-
- Next month the state Department of Health will send out
hundreds of letters to neurologists and other doctors urging them to report
all suspected cases of human prion disease to the state and talk to families
about having autopsies performed.
-
- Tissue samples would then be sent to the National Prion
Disease Pathology Surveillance Center in Cleveland. Opened in 1997, the
center is responsible for tracking prion diseases and spotting new strains.
-
- Biochemical tests can easily distinguish between CJD
and other varieties of dementia, but in a doctor's office, it's often difficult
to pinpoint the source of a patient's symptoms.
-
- There are some key differences, however.
-
- CJD hits quickly, for example, with symptoms that advance
rapidly for one to two years before death. Alzheimer's takes years to progress.
-
- CJD and its variant form are also distinct.
-
- The average age of traditional CJD victims is 60. In
England, British scientists and doctors suspected they were dealing with
a new strain when younger people started dying of a brain-wasting disease
that looked like CJD, Montine said.
-
- Human mad cow disease also often starts with psychological,
rather than physical, symptoms.
-
- "If you weren't tuned in, you might miss it, at
least initially," said Dr. Christina Marra, a UW neurologist.
-
- Depression and personality changes are the first signs,
followed by pain, tingling and other sensory problems.
-
- "Almost everyone (diagnosed with human mad cow disease)
with rare exception was first seen by a psychiatrist," Marra said.
-
- Some research suggests the source of infection for the
two forms of CJD could be related.
-
- When injected with misfolded proteins believed to cause
mad cow disease, mice genetically engineered to have human traits came
down with a disease that resembles sporadic CJD, according to a 2002 study
in England.
-
- And, in a study published last month, Italian researchers
said they found a second strain of mad cow disease that looks a lot like
traditional CJD.
-
- Labeling traditional CJD "sporadic" is a way
of getting around the fact that no one knows what causes it, said Dr. Laura
Manuelidis, a neuropathologist at Yale University. Parting with the prevailing
theory, she believes the culprit is more likely some type of infectious
agent, not misfolded proteins. "Spontaneous CJD just means we don't
know where the infection is coming from," she said.
-
- There's also evidence that some cases of CJD could be
mistakenly tagged Alzheimer's or some other type of dementia.
-
- Swiss officials found a CJD rate of three cases per million
after improving their tracking system -- triple the previously accepted
rate.
-
- In a small study at Yale led by Manuelidis in 1989, researchers
discovered that 13 percent of patients diagnosed with Alzheimer's had CJD.
-
- "The conclusion people might draw is that CJD is
underdiagnosed and clinically can't be discriminated that easily from Alzheimer's
disease," Manuelidis said.
-
- Still, only three cases of prion disease have surfaced
among the hundreds of autopsies performed as part of a 20-year research
study on Alzheimer's at the UW, according to Gerard Schellenberg, a genetic
researcher at the Veterans Affairs Puget Sound Health Care System. [Note:
Schellenberg's first name has been corrected since this was originally
published.]
-
- "Those brains are looked at extreme carefully and
you just don't turn up random CJD cases," Schellenberg said.
-
- For the victims and their families, of course, statistics
are not the point.
-
- Losing their mother
-
- "Look at that woman over there. She looks so weird!"
Rose Henry shouted one Sunday morning at Mass. The mother of 10 children,
Henry had strict rules about how to conduct yourself in public. The girls
should act like ladies, the boys like gentleman. No arguing with strangers.
No rude outbursts.
-
- "I grabbed her elbow and I said, 'Mom, be quiet!'
It was like she didn't know she was being inappropriate," Giese said.
-
- Other changes followed.
-
- Henry began reacting to noise like someone burst a balloon
near her ear. A slammed kitchen cabinet door or a car backfiring caused
her to leap up and gasp.
-
- "The boys were in denial," remembers Patty
Anderson, one of the couple's six daughters. "They said she's just
trying to get used to Dad being gone."
-
- Those who lived closer knew better. They just didn't
know why or what was gnawing away their mother's mind.
-
- An internist told the family she had dementia. Giese's
brothers and sisters prepared themselves for their mother's long slide
into oblivion.
-
- A trained nurse, Giese didn't buy it.
-
- "When the others would say she obviously has Alzheimer's,
I would say, 'No, it doesn't fit. She's going too fast.' "
-
- Henry eventually lost her balance, hung Army blankets
on the windows to keep out light and tried to use the TV remote to make
phone calls.
-
- At times, says her family, it seemed Henry knew she could
no longer rely on her mind.
-
- "She would pace around and wring her hands and she'd
say 'Stay with me, stay with me, I'm afraid.' It was so sad," Giese
recalls.
-
- When Henry started having seizures, a neurologist spotted
signs of CJD on a brain scan.
-
- She died four months later, blind, unable to talk or
walk.
-
- Unfamiliar with the two varieties of CJD until recently,
the family has always referred to their mother's disease as "mad cow."
-
- And they still wonder if the beef Jim and Rose ate on
a dream trip to Ireland and England in 1978 had anything to do with their
mother's death.
-
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