- WASHINGTON (UPI) -- Federal
and New Jersey authorities appear to have bowed to pressure from Congress
and are investigating a potential cluster of cases of a fatal brain disorder
called Creutzfeldt-Jakob disease in the southern part of the state, United
Press International has learned.
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- However, the authorities deny congressional pressure
influenced their decision to investigate the suspected cases of the illness,
one form of which has been linked to mad cow disease.
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- The investigation was spurred by the persistence of Janet
Skarbek, a private citizen in Cinnaminson, N.J., who has been collecting
information about local CJD cases for nearly a year. Skarbek has compiled
a list of at least nine -- and as many as 13 -- CJD cases with ties to
the Garden State Race Track in Cherry Hill.
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- Skarbek became concerned about the disease after Carol
Olive, a woman with whom her mother had worked at the Race Track, died
last May of CJD.
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- As Skarbek's research turned up more cases of the rare
disease, she began urging the Centers for Disease Control and Prevention
in Atlanta, and the New Jersey Department of Health and Senior Services,
to investigate. But both agencies dismissed her pleas, claiming there was
no evidence of abnormally high occurrences of CJD in the state.
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- Undeterred, Skarbek recently contacted several members
of congress and senators, some of whom showed particular interest. Rep.
Rob Andrews, R-N.J., and aides to Sen. Richard Durbin, D-Ill. -- who has
co-sponsored a Senate bill that would increase surveillance for CJD --
wrote letters to the health agencies, urging them to investigate.
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- Now, after weeks of denying there is a cluster or rise
in the rate of CJD in southern New Jersey, both the CDC and the state health
department appear at least to be willing to closely examine the cases identified
by Skarbek.
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- Dr. Eddy A. Bresnitz, the New Jersey state epidemiologist,
sent an e-mail on Feb. 25 to a Senate aide, saying his department along
with the CDC was investigating the cases.
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- The agency "is in the process of verifying whether
they are N.J. residents and whether the diagnosis was confirmed in all
individuals, which is often not the case," Bresnitz wrote in the e-mail,
which was obtained by UPI. "We are working with the CDC (Dr. Larry
Schonberger) on this assessment as the CJD unit he heads coordinates surveillance
efforts nationwide," the e-mail continued.
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- CDC spokesman Tom Skinner offered few details about the
investigation, other than to say it was being spearheaded by the New Jersey
health department. "They're kind of taking the lead and we're supporting
them," Skinner told UPI.
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- DHSS spokeswoman Jennifer Sciortino said the agency had
obtained the list of cases from Skarbek only a few weeks ago.
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- "We don't have a definitive timeframe right now
when the whole investigation is going to be wrapped up," Sciortino
told UPI. "Once they reach a definitive conclusion, it will be made
public," she added.
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- Rep. Andrews, who sent a letter on Feb. 11 to both the
CDC and the DHSS, said he was persuaded by the facts Skarbek presented
to him that the cases warranted further investigation.
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- "I also thought she had been dismissed by the CDC
and the New Jersey health Department far too quickly," he added.
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- Andrews said he will continue to monitor the situation
to ensure the CDC and the DHSS take a thoughtful look at the situation.
He called their decision to move ahead with gathering more information
about the cases a step in the right direction, but he said he will not
be satisfied until they deliver a thorough answer explaining these cases.
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- In January, both the DHSS and the CDC emphatically refuted
the possibility there was an increased number of cases of CJD in New Jersey.
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- "There is no cluster of cases of CJD in New Jersey
nor is there an increased incidence of CJD in the state," Bresnitz
told UPI at the time.
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- However, as reported by UPI, the CDC in late January
privately requested information about the cases from Skarbek.
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- Asked about the apparent shift in position, Sciortino
said, "Our position has not changed since you spoke to Dr. Bresnitz
in January. At this point, we are verifying the diagnoses and where the
individual resided at the time of death."
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- CJD clusters, which have occurred in several states in
recent years, received renewed attention following the discovery of a single
case of mad cow disease in Washington state in December. The concern was
prompted by the fact that more than 150 people have contracted variant
CJD -- the human form of the illness linked to mad cow disease -- in the
United Kingdom and Europe, following an outbreak of mad cow in British
herds that began in the 1980s.
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- None of the U.S. clusters has been tied to tainted meat
and all appear to consist of sporadic CJD -- which is not thought to be
connected to mad cow. However, two recent studies raise the possibility
that at least some sporadic cases could be due to mad cow, otherwise known
as bovine spongiform encephalopathy or BSE.
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- Research in mice by John Collinge of the Imperial College
of London indicated the mad cow pathogen can cause both variant CJD and
sporadic CJD. Also, Italian scientists reported last month they had identified
a new strain of mad cow disease that shows many similarities to sporadic
CJD.
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- One racetrack employee, Carrie Mahan, 29, died from a
mysterious condition in 2000 that initially appeared to be vCJD. Subsequent
tests, however, ruled out both vCJD and sporadic CJD, and it remains unclear
exactly what killed Mahan. But at least one expert, Dr. Omar Bagasra, a
biologist at Claflin University in Orangeburg, S.C., suspected it could
be a new strain of CJD related to mad cow-infected meat.
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- The uncertainty that often surrounds CJD cases is one
reason why Sen. Durbin introduced his bill calling for increased surveillance
of the disorder.
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- "Right now we're not tracking instances of BSE outbreaks
or CJD," Durbin's spokesman Joe Shoemaker told UPI. "It's hard
for us epidemiologically to say there's a cause and effect without some
of those baseline comparisons," he said.
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- Not all of the victims Skarbek included on her list lived
in New Jersey, but all had ties to the race track -- either as employees
or attendees of events there. The race track is no longer in operation.
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- Skarbek said she thinks the racetrack cases are related
to the consumption of meat infected with the mad cow pathogen because sporadic
CJD is too rare -- scientists think it occurs in only one per million people
-- to have happened by chance.
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- "Finding all of these cases related to the racetrack
is statistically impossible, if it's one in a million and its supposed
to be a coincidence," she told UPI.
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- Even if some or all of Skarbek's cases turn out to be
coincidental, there still appears to be an unusual number of cases in a
three-county area in the southern part of the state, according to the New
Jersey Department of Health's own records.
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- Those records show seven people in Burlington, Camden
and Gloucester counties have died from sporadic CJD since 2000. An eighth
confirmed case -- a 70-year old man who died in Gloucester county in September
-- does not yet appear to be included in the state's data.
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- Eight people over four years is double the expected number
of cases for an area with a population of 1.2 million. Only about four
people should have developed CJD over that time period.
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- It is not clear how many of the cases identified by Skarbek
overlap with those included in the New Jersey Department of Health's records
because the state data do not provide identifiable information.
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- Skarbek said she recently identified a possible ninth
case in Burlington County: a 72-year-old man who died on March 2 and currently
is being autopsied for CJD.
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- Dr. John Trojanowski, a brain researcher at the University
of Pennsylvania in Philadelphia, said if that rate were sustained over
time, it would indicate a true increase. But the rate may return to normal
in future years, so it could turn out to be just a coincidence, he added.
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- Skarbek said she remains skeptical the CDC and New Jersey
officials will release findings that support her claim of the CJD cases
being tied to tainted meat.
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- "I think they're going to do their best to downplay
it," she said. "I'd love for them to prove me wrong because then
I can stop worrying about my mom, but I know in my heart I'm correct."
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- - Steve Mitchell is UPI's Medical Correspondent. E-mail
sciencemail@upi.com
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- Copyright © 2001-2004 United Press International
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