Eighteen patients who underwent neurosurgery at Forsyth Medical Center in Winston-Salem, N.C., may have been exposed to Creutzfeldt-Jakob disease, a rare and always fatal brain disorder. The hospital says the incident should not have happened, and officials are looking into why there was a lapse in the recommended protocol.
A patient showed symptoms often associated with the disorder, and the diagnosis was later confirmed by tests. In the meantime, the surgical instruments used on that patient did not undergo the enhanced sterilization recommended to eliminate the possibility that they could infect other patients in subsequent procedures.
As a result, the Forsyth Medical has notified 18 patients who subsequently underwent procedures using the instruments that they may have been exposed to the disease, which is characterized by rapidly progressive dementia.
“On behalf of Forsyth Medical Center, I want to offer my sincerest apology to the neurosurgical patients who may have been exposed to CJD while undergoing surgery at our hospital,” Jeff Lindsay, the hospital's president and CEO, said in a press release. “We recognize that the risk to these patients is very small. However, we take any potential exposure seriously.” The hospital says it is taking every necessary precaution to prevent a similar occurrence in the future.
In 2013, there were reports
of at least 15 people exposed to CJD at Catholic Medical Center in Manchester, N.H., and at least five at Cape Cod Hospital in Hyannis, Ma.
In the New England case and in this recent exposure in North Carolina, the patients suffered from sporadic CJD, which is caused by spontaneous transformation of normal proteins into abnormal prions rather than by infection from another source.
Although the condition is rare—and transmission of it via surgical instruments is extremely rare—some advocates say hospitals should be more vigilant given the recent upsurge of potential exposures.
“This should not have happened,” said Lori Nusbaum, associate director of the Creutzfeldt-Jakob Disease Foundation. “If surgical equipment comes into contact with the brain or nervous system of a symptomatic person suspected of having CJD, then the equipment should be quarantined until results come back.”
CJD is “unusually resistant” to disinfection and sterilization by most standard physical and chemical methods, according to a World Health Organization report offering guidance on decontamination methods
for the disease. According to the report, the most stringent recommendations should be applied to instruments that have come in contact with tissue of a person known to be contaminated. “The safest and most unambiguous method for ensuring that there is no risk of residual infectivity on contaminated instruments and other materials is to discard and destroy them by incineration,” the WHO says.
That did not happen in the case of the North Carolina hospital and now patients may have to live with the worry. The incubation period could last years, but progression is rapid, experts say.
“It could take weeks or months from the time symptoms begin to the time of death,” Nusbaum said. According to the CJD Foundation, there is no treatment or cure for the disease. Patients often present with symptoms such as anxiety, depression, memory loss or insomnia—which can be misdiagnosed with other neurological conditions.
Each year, CJD affects about one in every 1 million people globally, and there are about 200 cases a year in the U.S., according to the National Institute of Neurological Disorders and Stroke
. Only about 1% of cases are acquired in the hospital following a medical procedure, but 85% of patients contract the disease sporadically.
Novant Health said in a statement that the system has changed its sterilization policy. All instruments used in brain surgery cases in its hospitals now go through an enhanced sterilization process. (This story has been updated to correct the spelling of Lori Nusbaum's name and to clarify her comments.)Follow Sabriya Rice on Twitter: @MHSRice