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- Laboratory tests may never prove what killed 68-year-old
Francis Will of Evansville, but his widow and daughters are convinced
they know the cause.
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- They believe it was a form of the rare Creutzfeldt-Jakob
disease, or CJD, an invariably fatal brain disorder related to what became
known as mad cow disease in Britain.
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- Local forensic pathologist John Heidingsfelder also suspects
it may be some form of the disease.
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- Heidingsfelder said within the last year he has seen
three suspected cases, one confirmed by autopsy, in the Tri-State.
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- He said that s far more than he would anticipate seeing
in his lifetime, based on international surveillance reports that the disease
strikes only one in 1 million people each year about 200 a year in the
United States.
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- But Will s family and the relatives of other people who
ve died of CJD in this country believe it s occurring more than anyone
realizes. They also believe there have been cases in the United States
of a new variant of CJD that is believed to pass from infected cows to
humans who ate food made from the animals, particularly the brains.
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- Francis Will was fond of brain sandwiches and ate a lot
of them, his widow and daughters said. They believe the beef-brain sandwiches
he ate caused the illness that led to his death on July 10.
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- His widow, Eloise Will, said she now tells everybody
not to eat brain sandwiches.
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- It s such a terrible disease that I can t stand to think
of someone else having it, she said. It has been described as the disease
from hell. It is terrible. You have to see it to believe it.
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- Most researchers discount a link between the disease
and diet.
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- There s absolutely no evidence of a link with the food
supply in this country, said Dr. Joyce Snider, a neurologist and researcher
in the department of neurology at Washington University School of Medicine
in St. Louis.
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- There s a lot of surveillance going on of our cows and
nobody s found that. And it s not because we haven t looked, she said.
We all want to know if that s happening in this country, and it doesn
t seem to be.
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- The disease basically destroys the brain, causing it
to become pitted or spongelike. There is no known treatment. It acts rapidly,
causing dementia and loss of body control. Most people stricken with it
die within three to 12 months.
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- My father lived 11 months after the first noticeable
symptoms, said Vicki Chandler of St. Louis, one of Will s three daughters.
He had always been so healthy. In the 37 years he worked at Bristol (Bristol-Myers
Squibb), he missed one day.
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- Memory loss
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- His first symptoms were memory loss, she said.
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- When he retired, he and Mom would go out to eat every
day. They had a weekly routine, based on which restaurant had the best
special of that particular day. And at one point, Dad stopped the routine
and went just to one place. It was the only one he could remember how
to get to.
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- After he died, Chandler said the family found notes he
had written to himself as reminders, before it became obvious to us.
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- The family tells a horror story of trying to find out
what was wrong when it became obvious something was. Every test, including
MRIs of the brain, came back normal. Doctors diagnosed it as various things,
including anxiety and depression, and medicated him for those illnesses.
One said it was a movement disorder that Will would just have to live
with, Chandler said.
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- Another daughter, Kathy Bredahal, a nurse at Barnes-Jewish
Hospitals in St. Louis, suspected it was Creutzfeldt-Jakob disease because
Will s symptoms were similar to that of a CJD patient she had once helped
take care of at the hospital.
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- Bredahal arranged for her father to be seen by neurologists
at Barnes Memory Diagnostic Center. After several hospitalizations and
numerous tests, they concurred that he probably had CJD, which can only
be confirmed by brain biopsy or autopsy.
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- Autopsy challenge
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- Bredahal said the family decided not to have a biopsy
done before his death but to wait and have an autopsy. She made arrangements
for her father s brain tissue to be tested at the National Prion Disease
Pathology Surveillance Center in Cleveland and at the National Institutes
of Health s prion study center in Maryland.
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- Then came the challenge of finding someone to perform
the autopsy.
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- Bredahal and Chandler said both Deaconess Hospital and
the Vanderburgh County coroner s office declined to allow the autopsy
to be performed in their facilities because of fear of infection if their
father did have CJD.
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- Deaconess Hospital officials declined to comment on the
case when contacted by the Evansville Courier & Press, citing patient
confidentiality. Heidingsfelder, who performs many autopsies at the coroner
s office, said he has not been allowed to do autopsies there in cases
where CJD is suspected.
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- He said Coroner Dennis Buickel and many others across
the country don t want to accept the risk of infection for their staffs
or facilities.
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- Normal disenfectants are known not to work (against CJD),
so the table may be contaminated for an unspecified period of time, Heidingsfelder
explained.
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- One of the world s leading experts on CJD, Paul Brown
of the National Institutes of Health, has described it as probably the
single most resistant organism on the face of the Earth. You can boil
it, you can put it in formaldehyde, you can autoclave it for a little
while, you can treat it with the usual disinfectants . . . and it s like
you didn t do anything.
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- It s actually incompatible with biological life as we
know it.
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- Heidingsfelder said special precautions are needed in
the cases.
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- Bredahal said a local funeral home agreed to allow the
autopsy of her father at its facility. But she and other family members
have been disappointed in the results. She said the brain tissue wasn
t fresh-frozen and there was a delay in getting it to the neurology laboratory
at Indiana University School of Medicine.
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- Not seen in U.S.
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- Without the fresh or frozen tissue, she said, the tests
still under way may not be able to determine if her father had the new
variant CJD as the family suspects.
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- But if the family is right, it would be the first case
of variant CJD diagnosed in the United States.
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- We ve not seen that in this country, said Dr. Bernardino
Ghetti, a neuropathologist at IU where the tissue from Will s brain is
being diagnosed.
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- Ghetti is co-director of the national prion disease surveillance
center.
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- The center is located at Case Western Reserve University
School of Medicine in Cleveland, where co-director Dr. Bernadino Gambetti
has been one of the leading researchers into the group of diseases that
includes CJD. The center was established when Ghetti was president of
the of the American Association of Neuropathologists in 1996-97 and is
attempting to develop a national database.
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- Ghetti said the neuropathology lab at IU is the only
lab in this state capable of doing a CJD diagnosis. It is performing the
diagnosis on Will, but Ghetti said it would be unethical for him to discuss
that specific case.
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- He said the national center has seen only a small number
of CJD cases about 100 a year but that doesn t represent the entire
story, because many cases aren t autopsied and some that are aren t reported
to the center.
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- He and other researchers would like for states to be
required to autopsy and report suspected CJD cases.
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- One problem, he acknowledged, is that some facilities
won t do the autopsies for fear of infection, and others aren t equipped
for it.
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- Ghetti said about half the suspected cases seen by the
national center turn out to be actual prion diseases.
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- What the families believe is important, he said, but
the proof is coming from the autopsy.
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- Mystery diseases
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- CJD is one of a handful of rare and mysterious brain
diseases that affect humans and a small group of animals. Scientists efforts
to understand them has been called one of the great biomedical quests
of the last 20 years.
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- The four known diseases in the group that occur in people
are Cruetzfeldt-Jakob disease, Gerstmann-Straussler-Scheinker disease (GSS),
fatal familial insomnia, and kuru an ailment once associated with ritualistic
cannibalism among Fore highlanders of Paupau New Guinea.
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- Related animal disease include sheep scrapie and bovine
spongiform encephalopathy (BSE), which became known as mad cow disease.
Other similar disorders are also found in domestic cats, mink, antelope
and mule deer. The leading theory among researchers is that the diseases
are caused by a prion - a mutated protein in the brain. The prion folds
incorrectly and causes the pitting or spongiform shape.
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- According to information from the National Institutes
of Health, the diseases can be contracted in one of three ways: It is
acquired through transmission by exposure to brain or nervous system tissue
in about 1 percent of the cases; is inherited in about 5 to 10 percent
of the cases, and occurs sporadically for no known reason in about 85
percent of the cases.
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- A new form of CJD, called the new variant, emerged in
Britain in 1995.
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- Heidingsfelder said the three known or suspected cases
he has seen in the Tri-State in the last year were totally unrelated. Two
were in Illinois. But their occurrence has caused him concern that the
incidence may be increasing and that the disease may be underreported.
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- The only other known case of CJD to be documented in
Evansville was Nathan Lieberman s death in 1990.
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- No one could say what caused it, said his son, Phil
Lieberman. He said his father was ill for two years.
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- After his death, brain tissue was sent to the national
surveillance center in Cleveland and tests confirmed the disease.
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- Lieberman said his parents had visited England in the
early 1980s, which is when the mad cow disease outbreak there began but
wasn t widely known.
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