WASHINGTON, Oct. 29 (UPI) -- Federal officials have investigated 20 cases for possible human mad cow disease in the last 10 years in New York, where state officials currently are looking into a cluster of five cases of a related disease, United Press International has learned.
Five cases of what initially appeared to be a fatal, incurable brain illness known as Creutzfeldt Jakob disease recently have been reported in Ulster county and surrounding areas in southern New York.
The cause of CJD is unknown, but it is such a rare disease -- striking only one person out of a million on average -- that a cluster of cases appearing in a small area would be unusual.
Some family members of the patients have expressed concern that some of the cases in the Ulster county area could be a related disease known as variant CJD, which has been linked to consuming beef products contaminated with the mad-cow pathogen and also to infected-blood transfusions
"I believe there's definitely a problem in this area," said Brent Tobey, who lives in Ulster county and whose father, Richard Tobey, 59, died earlier this month after being diagnosed with CJD.
Brent Tobey told UPI that when his father was being treated before his death, in Benedictine Hospital in Kingston, medical personnel told him there were additional CJD cases in the area.
"One of the medical staff said eight," Tobey said. "Somebody else said they had seen five cases of it."
According to documents obtained by UPI under the Freedom of Information Act last July, the Centers for Disease Control and Prevention in Atlanta investigated 20 cases in New York from 1994 to 2002 for the possibility of vCJD.
The CDC's Freedom of Information Office described the cases as "patients CDC has investigated for possible vCJD." Only one case of vCJD has been detected in the United States -- a Florida woman diagnosed in 2002 who subsequently died last June and is thought to have contracted the disease in England. The only confirmed case of mad cow in U.S. herds occurred last December in Washington state.
UPI also has learned that New York recorded 23 cases of CJD in 2003 and 28 in 2001, which is about four and nine more, respectively, than would be expected based on the state's population size.
Health officials said the cases in the Ulster county area did not pose a risk to the public at large.
"We don't see any threat to the public health here whatsoever," William Van Slyke, deputy commissioner of the New York State Department of Health, told UPI. Van Slyke said the state generally sees about 20 cases of CJD per year.
Van Slyke noted one of the Ulster county cases -- that of Colleen Staccio, 46 -- already has been autopsied and no evidence of CJD or vCJD was found. In another case, the person recently had moved in to Ulster county prior to her death so the case officially will be listed as having occurred in her prior place of residence, which Van Slyke said was in the metropolitan New York City area.
Yet another case occurred in neighboring Dutchess County, so only two of the five cases actually occurred in Ulster county, Van Slyke said.
Asked about Tobey's claims of hearing of additional cases, Van Slyke said the only way to confirm CJD cases is via an autopsy.
"Any physician talking to the public without laboratory-confirmed results is doing a significant disservice to patients and should stop," he added.
Tobey said he has tried to find out more about the cases, but health professionals have been unwilling to talk to him.
"I keep getting doors slammed in my face," he said. "Nobody wants to talk to me about it."
Although New York health officials talked to his stepmother about his father's death, officials never spoke with the other family members and the CDC has never contacted the family, Tobey said.
"The media want to talk to us more than the health department and that doesn't make any sense," he said.
Tobey noted that his dad was a "huge beefeater" and that he can no longer bring himself to eat ground beef.
"Every time I look at ground beef, I want to throw up now," he said.
At present, the CDC's age cut-off for investigating potential vCJD cases is 55. That is because nearly all incidences of the disease worldwide have been in people under that age. The 20 cases investigated by the CDC in New York, however, include patients ranging in age from 30 to 54. Generally, the CDC investigated up to four cases in the state each year from 1994 to 2002, with the highest number being four cases each in 1996 and 1999.
CDC officials said they will conduct a "special review" of the cases in the Ulster county area that are under age 55. A special review consists of obtaining as many details as possible about each case from medical reports and other sources to rule out vCJD and "we will be doing that with the state in this case," agency spokeswoman Christine Pearson told UPI.
Pearson said that, at this point, no CDC officials are in the state investigating, but the agency will offer its expertise at the state's request.
She said the agency has not detected any increase in CJD cases in the United States that would indicate there is a problem with vCJD. "We've had a fairly stable rate of CJD cases in the United States" of about 300 per year, she added.
According to official records provided by the New York Health Department to UPI in April, the state recorded 23 CJD cases in 2003 -- 18 upstate and five in New York City. In 2001, the rate was higher, with a total of 28 cases -- 19 upstate and nine in New York City.
New York state's population is just over 19 million, so it would expect to see about 19 cases per year, given the average rate of the disease.
New York Health Department spokeswoman Claire Pospisil said in an e-mail to UPI at the time the number of cases in the state are within the normal expected range.
"Nationally, we see one case per million and New York's stats are in line with that," Pospisil wrote.
Although UPI had requested to see a breakdown of the cases by county, New York officials refused to release the information.
"We do not release county-level data when cell sizes are small, because of the potential to identify someone diagnosed with CJD and jeopardize patient confidentiality," Pospisil wrote. She also did not respond to an e-mail UPI sent after the reports of the cases in the Ulster county area surfaced asking for updated figures for 2004.
The area where the southern New York cases occurred is just two counties away from a northern New Jersey area that saw five CJD cases within 15 months in the two-county region of Morris and Somerset, as UPI reported in March.
The New York Times reported earlier this week it had learned of another CJD death in Orange County, N.Y., which lies between Ulster County and the cluster in northern New Jersey. Ann Marie Da Silva told the Times her husband Richard Joseph Da Silva, 58, died from the disease in May.
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