Cluster of Mad Cow-Like (CJD) Diseases in NY State Has People Worried

The Contagion of Fear

October 25, 2004
By MARC SANTORA

NY Times

The disease's fatal grip on the brain seems to come out of
nowhere, afflicting an otherwise healthy person and turning
him into an incoherent muddle. In Ulster County in upstate
New York, that was how death came to at least two people in
the last year. The rare brain malady called
Creutzfeldt-Jakob disease caused the death of one resident
in November 2003 and of a second this fall.

About one in a million people worldwide die from the
mysterious disease each year, and there is no medical
explanation for why the disease afflicts humans in its most
common form.

There has been a recent buzz in Ulster County about the
strange illness, but only within the small circles of the
victims' families.

Then the community discovered that a 60-year-old woman had
died in August from an illness that seemed similar to
Creutzfeldt-Jakob disease. The woman's family did not allow
an autopsy, so it cannot be determined if she had the
disease. But after comparing notes, people found out that
another person in nearby Dutchess County died of the
disease this year.

There have been other unusual deaths, including that of
another woman in Ulster County, Colleen Staccio, 46, who
died in August from a condition her doctors initially said
was Creutzfeldt-Jakob disease but whose autopsy showed
otherwise, and that of Richard Tobey, 59, who died of the
disease two weeks ago.

The community's response has gone from curious to downright
scared.

State health inspectors investigating the deaths said they
could not discuss specifics of the cases, citing privacy
concerns. They did say that they saw no risk to public
health.

That has done little to ease public concern.

A state health investigator who looked into the deaths
agreed to speak with The New York Times to ease public
concern but insisted on anonymity. He said that, at most,
the deaths represented a "statistical anomaly."

The investigator said that the woman whose family refused
an autopsy had been sick when she moved to Ulster County,
that Creutzfeldt-Jakob disease had been ruled out in Ms.
Staccio's case and, most important, that none of the deaths
were the result of a form of Creutzfeldt-Jakob disease that
can be caused by outside factors like infected meat.

That form of the disease, called variant Creutzfeldt-Jakob
but more commonly referred to as mad cow disease, is what
brought the malady to public attention in the late 1990's.

Many people ate cattle in England infected with bovine
spongiform encephalopathy, and as a result came down with
the variant form of the disease. The cases caused a panic
around the world.

In the United States, there has only been one documented
case of someone dying from variant Creutzfeldt-Jakob, a
woman in Florida who had lived in England.

Despite the fact that there is little evidence of that form
of the disease here, the fear in Ulster County is that some
outside factor is causing the deaths. That is not the case,
state health officials said. They confirmed the deaths in
Ulster and Dutchess Counties and another case in nearby
Orange County.

But, they noted, every year in New York State, around 20
people die from what is called sporadic Creutzfeldt-Jakob
disease. The deaths in upstate New York are not considered
out of the ordinary, except that people are talking about
them.

Because so little is known about the disease, the fact that
answers were hard to come by only added to the confusion.

"They're saying it's not a big deal, but I don't buy that
at all," said Jane Scordalakis, who was window-shopping
last week in Kingston, N.Y., where several of the victims
lived and were initially treated.

A staid, small city, Kingston is a community where
individuals often cross paths. "They said there were two
people in the hospital at the same time with this disease,
but that there is no correlation," Ms. Scordalakis said.
"How do they know that there is no correlation?"

Creutzfeldt-Jakob disease is caused by a misformed protein
- called a prion - in the brain. According to the World
Health Organization, roughly 85 percent of the cases of the
disease around the globe are sporadic, meaning people who
get the illness, typically those over 65, are exposed to no
known risk factors.

The disease can lie dormant for years, even decades, and
doctors do not know what sets it off. There are only a
handful of known prion diseases that afflict humans, and
all are fatal. Creutzfeldt-Jakob disease has been the
subject of serious medical study for the past 10 to 20
years, but the cases in Ulster County are typical of the
way the disease works.

Mr. Tobey was in relatively good health when he got sick
this past summer. At first, his daughter, Stacey Tobey,
noticed only little changes - the world seemed to be
getting fuzzier for her father.

"Basically, he progressed to where he couldn't eat,
couldn't swallow, couldn't speak," Ms. Tobey said. Doctors
were puzzled by his rapid deterioration. At first they
thought it might have been related to his sleep apnea, then
they thought he must have suffered a stroke.

In fact, the state health inspector said, reports of
symptoms that seem to suggest Creutzfeldt-Jakob disease
often turn out to be caused by strokes or the rapid onset
of Alzheimer's disease.

A biopsy confirmed that Mr. Tobey was indeed suffering from
Creutzfeldt-Jakob disease. He died less than three months
after he first started showing symptoms.

Richard Joseph Da Silva, 58, from Highland Mills in Orange
County, suffered in a similar manner before he died in May
of what his wife, Ann Marie Da Silva, said was listed on
his death certificate as Creutzfeldt-Jakob disease. Mrs. Da
Silva, contacted The New York Times after reading about the
cases in Ulster County. She was upset that the state health
officials had not gotten in touch with her to find out more
about Mr. Da Silva's death.

One senior state health official, who said he did not want
to be named because of patient privacy concerns, said
investigators rarely contact families in deaths involving
dementia, an effect of the disease. A New York State law
requires health inspectors to examine every death caused by
dementia - which means they examine hundreds of cases a
year. In the Health Department's division of chronic
disease, several people are devoted solely to this task.

When the department investigates deaths believed to have
been caused by Creutzfeldt-Jakob disease, it first
determines the actual cause of death and then decides if
there is a cluster of related deaths or just a geographic
coincidence. More often than not, officials said, it is a
coincidence.

For instance, in New Jersey, a series of deaths from
Creutzfeldt-Jakob disease in the Cherry Hill area in recent
years caused a stir after people started looking at the
death certificates and saw all sorts of perceived patterns.

Dr. Clifton R. Lacy, the New Jersey health commissioner,
said that a lengthy investigation found nothing out of the
ordinary, but that did not stop the public from worrying.

The New York health investigator who looked into the deaths
in Ulster County said, "Often the geographic patterns that
you see have no relevance."

But Mrs. Da Silva said, "I am just feeling very
frustrated."

She said she had read extensively about the disease and was
looking for any possible clues. For example, she said that
her husband had served in the Vietnam War. "He saw the
worst of the worst," Mrs. Da Silva said. "He did deal with
open brains, he was holding people's body parts."

Until the weeks before his death, Mr. Da Silva had been in
good health and had worked for years as a dean at Rutgers
University, Mrs. Da Silva said. Then, last spring, he
suddenly changed.

"He started shaking," Mrs. Da Silva said. "He just became
very frightened and he kept saying, 'There is like a glaze
over everything I am seeing.' ''

Mr. Da Silva died a few weeks later, on May 13, 2004.

Florence Kranitz, the president of the Creutzfeldt-Jakob
Disease Foundation in Ohio, said the frustration of
relatives of those who die from the disease was
understandable. "Sporadic C.J.D. is a very enigmatic form
of this disease and it seems to happen for no apparent
reason," she said. "This is a scary disease. It's one of
the most horrible on the face of the earth."

Mr. Tobey's widow, Barbara, said she got a crash course on
the disease. "I am very puzzled," she said. "I want more
answers as to what killed my husband."

But sometimes, health officials say, there are no answers.

Samme Chittum contributed reporting for this article.

http://www.nytimes.com/2004/10/25/nyregion/25brain.html?ex=1099730753&ei=1&e
n=099d06402fb07cb9

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