- We hear that CJD is such a rare disease, yet, here again,
we have a cluster of (at least) 4 deaths in one New York State County.
- We periodically hear about patients exposed to CJD via
surgical or dental procedures as was the case at Emory University Hospital
when the hospital had to alert 500 patients to the fact that they were
exposed to CJD at the hospital after a patient died testing positive for
- Much of Ulster County, New York is situated in the Catskill
mountains and is predominately rural.
- There is a lot of deer hunting in the area and many residents
will tell you that they do eat deer meat.
- Ulster Deaths Probed
- By Jesse J. Smith
- Freeman Staff
- KINGSTON - At least four area residents have died from
an extremely rare brain disease in recent months, and state health officials
are investigating whether there is any link between the cases.
- On Tuesday, Dr. Joel Mandelbaum of Hurley Avenue Family
Medicine said he had been directly involved in the treatment of three patients
with Creutzfeldt-Jakob disease (CJD) in the past three months. All three
cases were confirmed through analysis of spinal-cord fluid or brain tissue,
said Mandelbaum who declined to name or give details about any of the victims.
- All of the patients died within the last 45 days, Mandelbaum
said. A fourth death, that of Kingston resident Richard Tobey occurred
at Benedictine Hospital on Saturday night. Family members confirmed that
Tobey, 59, was positively diagnosed with CJD following a brain biopsy at
Mount Sinai Medical Center in Manhattan last week. Benedictine Hospital
confirmed Tuesday that two people had died of the disease at their hospice
facility after being diagnosed and treated elsewhere.
- CJD is an invariably fatal disorder caused by abnormal
proteins called prions which infect and destroy healthy brain tissue. According
to the U.S. Centers for Disease Control, CJD occurs in about one in every
1 million people each year. The most common form of the disorder is known
as sporadic CJD and occurs spontaneously. Other variants may be inherited
from family members or acquired through tissue implants, the use of contaminated
surgical instruments or the administration of hormones extracted from contaminated
human organs. The most rare, but best known form of the disease, commonly
called Mad Cow Disease, is contracted by eating the brain or spinal tissue
of infected cattle. While 143 people have died of Mad Cow CJD in Europe,
the only case confirmed in the U.S. was in a Florida woman who previously
lived in England. So far, none of the Ulster County CJD cases have been
identified as the Mad Cow variant.
- "This is a very unusual disease, and a very unusual
cluster," said Mandelbaum. "We have to find out if there is some
common cause and, if so, eliminate it."
- State Health Department officials, citing patient privacy
laws, had declined to discuss the existence of an unusual number of CJD
cases in Ulster County since August when they were questioned regarding
unconfirmed reports of a CJD cluster. On Tuesday, however, a department
spokesman confirmed that they were investigating the situation.
- "It's a bit of a puzzle and we are trying to solve
it now," said Health Department spokesman William Van Slyke, who declined
to give an exact number of CJD cases locally. "But at this point we
don't see anything which would cause concern or a threat to the public
- Family members of two known CJD victims, Tobey and Lake
Katrine resident Colleen Staccio, 48, who died at the Benedictine hospice
on Aug. 28, said that their loved ones had no family history of CJD and
had never traveled to places where Mad Cow outbreaks had occurred. Staccio
and Tobey were young compared to most CJD victims, whose median age is
68. Both victims had back surgery at Kingston Hospital in the mid-1990s,
however there is no evidence of a link between those treatments and their
deaths from CJD. The medical and travel histories of the two unnamed victims
- Asked to comment on the report that two of the victims
had had back surgery at the hospital, Kingston Hospital spokeswoman Deb
Muise said the hospital is not investigating any active cases of CJD at
- Relatives of Staccio and Tobey said that they had heard
informally from nurses and other medical personnel that there had been
an unusual number of CJD cases locally, but said they were thwarted in
their attempts to gather more information by patient confidentiality laws.
- "Most of them, as soon as you mention (other CJD
cases) just close up tight as a drum," said Richard Tobey's daughter
Stacy Tobey. "We just want answers. We don't want anybody else to
go through what we have gone through these last six weeks."
- Dr. Marc Tack, a Kingston-based infectious disease specialist,
said he was familiar with some local CJD cases, but saw no evidence of
any common cause. According to Tack, considering the lengthy incubation
period of the disease, it is unlikely that a victims infected from a common
source would die at such close intervals.
- "I'm concerned in that I would like more information
and more answers," said Tack. "But the fact is, you are more
likely to die crossing the Kingston-Rhinecliff bridge tonight than from
- Patricia A. Doyle, PhD
- Please visit my "Emerging Diseases" message
board at: http://www.clickitnews.com/ubbthreads/postlist.php?
- Zhan le Devlesa tai sastimasa
- Go with God and in Good Health