At Least Four Recent Cluster
CJD Deaths In Ulster Co. NY

From Patricia Doyle, PhD
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 CJD.
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 health."
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 remain unknown.
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 this time.
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 CJD."
Patricia A. Doyle, PhD
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Zhan le Devlesa tai sastimasa
Go with God and in Good Health



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