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From: TSS (
Date: November 19, 2004 at 6:46 am PST

-------- Original Message --------
Subject: Eye procedure raises CJD concerns
Date: Fri, 19 Nov 2004 08:19:35 -0600
From: "Terry S. Singeltary Sr."
Reply-To: Bovine Spongiform Encephalopathy

Eye procedure raises CJD concerns

By Steve Mitchell
Medical Correspondent

Washington, DC, Nov. 18 (UPI) -- A New York man who died from a rare
brain disorder similar to mad cow disease in May underwent an eye
procedure prior to his death that raises concerns about the possibility
of transmitting the fatal disease to others, United Press International
has learned.

The development comes on the heels of the announcement Thursday by U.S.
Department of Agriculture officials of a possible second case of mad cow
disease in U.S. herds.

Richard Da Silva, 58, of Orange County, N.Y., died from Creutzfeldt
Jakob disease, an incurable brain-wasting illness that strikes about one
person per million.

Richard's wife Ann Marie Da Silva told UPI he underwent a check for the
eye disease glaucoma in 2003, approximately a year before his death. The
procedure involves the use of a tonometer, which contacts the cornea --
an eye tissue that can contain prions, the infectious agent thought to
cause CJD.

Ann Marie's concern is that others who had the tonometer used on them
could have gotten infected.

A 2003 study by British researchers suggests her concerns may be
justified. A team led by J.W. Ironside from the National
Creutzfeldt-Jakob Disease Surveillance Unit at the University of
Edinburgh examined tonometer heads and found they can retain cornea
tissue that could infect other people -- even after cleaning and
decontaminating the instrument.

"Retained corneal epithelial cells, following the standard
decontamination routine of tonometer prisms, may represent potential
prion infectivity," the researchers wrote in the British Journal of
Ophthalmology last year. "Once the infectious agent is on the cornea, it
could theoretically infect the brain."

Prions, misfolded proteins thought to be the cause of mad cow, CJD and
similar diseases, are notoriously difficult to destroy and are capable
of withstanding most sterilization procedures.

Laura Manuelidis, an expert on these diseases and section chief of
surgery in the neuropathology department at Yale University, agreed with
the British researchers that tonometers represent a potential risk of
passing CJD to other people.

Manuelidis told UPI she has been voicing her concern about the risks of
corneas since 1977 when her own study, published in the New England
Journal of Medicine, showed the eye tissue, if infected, could transmit CJD.

At the time the procedure was done on Richard Da Silva, about a year
before he died, she said it was "absolutely" possible he was infectious.

The CJD Incidents Panel, a body of experts set up by the U.K. Department
of Health, noted in a 2001 report that procedures involving the cornea
are considered medium risk for transmitting CJD. The first two patients
who have a contaminated eye instrument used on them have the highest
risk of contracting the disease, the panel said.

In 1999, the U.K. Department of Health banned opticians from reusing
equipment that came in contact with patients' eyes out of concern it
could result in the transmission of variant CJD, the form of the disease
humans can contract from consuming infected beef products.

Richard Da Silva was associated with a cluster of five other cases of
CJD in southern New York that raised concerns about vCJD.

None of the cases have been determined to stem from mad cow disease, but
concerns about the cattle illness in the United States could increase in
light of the USDA announcement Thursday that a cow tested positive on
initial tests for the disease. If confirmed, this would be the second
U.S. case of the illness; the first was detected in a Washington cow
last December. The USDA said the suspect animal disclosed Thursday did
not enter the food chain. The USDA did not release further details about
the cow, but said results from further lab tests to confirm the initial
tests were expected within seven days.

Ann Marie Da Silva said she informed the New York Health Department and
later the eye doctor who performed the procedure about her husband's
illness and her concerns about the risk of transmitting CJD via the

The optometrist -- whom she declined to name because she did not want to
jeopardize his career -- "didn't even know what this disease was," she said.

"He said the health department never called him and I called them (the
health department) back and they didn't seem concerned about it," she
added. "I just kept getting angrier and angrier when I felt I was being

She said the state health department "seems to have an attitude of don't
ask, don't tell" about CJD.

"There's a stigma attached to it," she said. "Is it because they're so
afraid the public will panic? I don't know, but I don't think that the
answer is to push things under the rug."

New York State Department of Health spokeswoman Claire Pospisil told UPI
she would look into whether the agency was concerned about the
possibility of transmitting CJD via tonometers, but she had not called
back prior to story publication.

Disposable tonometers are readily available and could avoid the risk of
transmitting the disease, Ironside and colleagues noted in their study.
Ann Marie Da Silva said she asked the optometrist whether he used
disposable tonometers and "he said 'No, it's a reusable one.'"

Ironside's team also noted other ophthalmic instruments come into
contact with the cornea and could represent a source of infection as
they are either difficult to decontaminate or cannot withstand the harsh
procedures necessary to inactivate prions. These include corneal burrs,
diagnostic and therapeutic contact lenses and other coated lenses.

Terry Singletary, whose mother died from a type of CJD called Heidenhain
Variant, told UPI health officials were not doing enough to prevent
people from being infected by contaminated medical equipment.

"They've got to start taking this disease seriously and they simply
aren't doing it," said Singletary, who is a member of CJD Watch and CJD
Voice -- advocacy groups for CJD patients and their families.

U.S. Centers for Disease Control and Prevention spokeswoman Christine
Pearson did not return a phone call from UPI seeking comment. The
agency's Web site states the eye is one of three tissues, along with the
brain and spinal cord, that are considered to have "high infectivity."

The Web site said more than 250 people worldwide have contracted CJD
through contaminated surgical instruments and tissue transplants. This
includes as many as four who were infected by corneal grafts. The agency
noted no such cases have been reported since 1976, when sterilization
procedures were instituted in healthcare facilities.

Ironside and colleagues noted in their study, however, many disinfection
procedures used on optical instruments, such as tonometers, fail. They
wrote their finding of cornea tissue on tonometers indicates that "no
current cleaning and disinfection strategy is fully effective."

Singletary said CDC's assertion that no CJD cases from infected
equipment or tissues have been detected since 1976 is misleading.

"They have absolutely no idea" whether any cases have occurred in this
manner, he said, because CJD cases often aren't investigated and the
agency has not required physicians nationwide report all cases of CJD.

"There's no national surveillance unit for CJD in the United States;
people are dying who aren't autopsied, the CDC has no way of knowing"
whether people have been infected via infected equipment or tissues, he

Ann Marie Da Silva said she has contacted several members of her state's
congressional delegation about her concerns, including Rep. Sue Kelly,
R-N.Y., and Sen. Charles Schumer, D-N.Y.

"Basically, what I want is to be a positive force in this, but I also
want more of a dialogue going on with the public and the health
department," she said.

Copyright 2004 United Press International


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