From the Wisconsin Sate Journal (7/24/09)
By Doug Erickson and Mark Pitsch
UW Hospital has told 53 patients they face an “extremely low” risk of contracting a rare but deadly brain disorder because they may have been operated on with contaminated surgical instruments.
The instruments had been used on a woman who died Tuesday of the brain disorder, Creutzfeldt-Jakob disease. She had surgery at the hospital in June.
Hospital officials say they immediately stopped using the surgical instruments once tests confirmed the woman’s diagnosis. However, the 53 patients all had neurosurgery during a 40-day window when the instruments were still in use, said Dr. Carl Getto, the hospital’s chief medical officer.
The situation poses no risk to the general public and an “infinitesimal” risk to the 53 patients, Getto said. UW Hospital officials said the instruments used went through normal sterilization procedures after each surgery, but CJD experts believe normal sterilization is not enough.
The hospital plans to continue using the surgical instruments once they go through the heightened sterilization process, an approach questioned by a national expert who said his hospital destroys the instruments used during brain surgery in cases where the patient may have been suffering from the disease.
“We don’t even want to have a theoretical risk for the (disease) to be transmitted,” said Dr. Michael Geschwind, a CJD expert at the University of California-San Francisco Medical Center.
CJD is an always-fatal neurological disorder that kills about 390 people per year in the U.S. It is characterized by rapidly progressing dementia, with death often coming within a year from the onset of symptoms.
“It’s a horrible situation, and I’m sure the university is as devastated by this as they possibly can be,” said Florence Kranitz, president of the Creutzfeldt-Jakob Disease Foundation in Akron, Ohio.
‘Not mad cow’
The disease can set off alarm bells because it belongs to a family of human and animal diseases that includes a bovine version often referred to as “mad cow” disease. However, UW Hospital officials said the woman died from a type of the disease distinct from the bovine version. (See Q&A)
“This is not mad cow disease,” said Dr. Nasia Safdar, a specialist in infectious diseases who oversees infection control at the hospital. “(People) need not be concerned about that relationship.”
There are two forms of CJD: classic and variant. UW Hospital officials say the dead woman had classic CJD, the most prevalent form.
Although mad cow disease occurs only in cows, eating infected beef is thought to be the cause of the variant form of CJD. No case of variant CJD has been documented as originating in the U.S. Three cases of it have been reported in the U.S., but the victims were exposed elsewhere, according to the Centers for Disease Control and Prevention.
Test results from Case Western Reserve University in Cleveland confirmed the woman died from classic CJD, Safdar said. More test results from the same lab in a few weeks will be able to conclusively rule out the variant form, she said.
UW Hospital is basing its diagnosis of classic CJD on the patient’s symptoms, age, travel history and family history and the course of the disease, Safdar said. “It doesn’t fit any profile (of variant CJD),” Safdar said.
Due to patient privacy, hospital officials would say only that the woman was in her 50s and had been transferred to UW Hospital on June 8 from a regional hospital in Wisconsin. The woman suffered an upper-respiratory infection in March and April and saw her family doctor in May, Getto said.
Because she showed unsteadiness walking, vision problems and memory loss, doctors at the regional hospital determined she had viral encephalitis, a brain infection. Geschwind, the UCSF expert, said CJD is commonly mistaken for viral encephalitis.
Getto said the woman also had a brain tumor, which doctors thought was causing her symptoms. There was no reason to suspect CJD because of the tumor, he said.
During surgery June 11 to remove the benign brain tumor, surgeons took samples of adjacent tissue, Getto said. The tissue was tested on site during the surgery, and there was no indication of CJD, he said.
However, over the next month, the woman’s condition declined unexpectedly and rapidly, so a sample of tissue from the earlier biopsy was sent to the National Prion Disease Pathology Surveillance Center at Case Western, Getto said. Prions are deformed proteins that can cause brain disease.
Monday, UW Hospital officials received a preliminary report that the sample had tested positive for CJD. Immediately, staff pulled all instruments used in previous neurosurgical procedures and began re-sterilizing them according to federal guidelines for equipment used in confirmed cases of prion diseases, Getto said.
The woman died at the hospital Tuesday.
On Tuesday, immediately after confirmation of the positive result, the hospital staff began identifying patients to be notified. All 53 patients have been notified by FedEx letter and a phone call, Safdar said.
The letter reads in part: “First and foremost, we wish to reassure you that the risk of exposure to CJD as a result of surgical instruments is extremely low. In fact, there have been no cases of transmission from surgical instruments to patients since 1976, when the standardized sterilization processes now used throughout the country were put in place.”
Other hospitals have potentially exposed patients to CJD in recent years through surgical equipment, according to the Centers for Disease Control and Prevention. But the agency has not verified a resulting case of CJD.
The UW Hospital patients range in age from 3 to 83, although the great majority are between 30 and 60, said hospital spokeswoman Lisa Brunette. Most are from the Midwest, primarily Wisconsin and Illinois, she said.
UW Hospital will offer them free consultations with neurosurgeons in addition to visits they already have scheduled with their regular neurosurgeons. They also can receive free psychological counseling, Getto said. Because the disease is so rare and the risk of transmission so small, the letter is meant to be “informative and reassuring,” he said.
The patients will not be able to find out immediately if they contracted the disease because there is no test for pre-symptomatic CJD, Safdar said. Symptoms might not appear for years or decades, she said.
The patients will be under no restrictions imposed by UW Hospital, although individual organizations may decide not to accept blood or organ donations from them based on their exposure, Safdar said.
Kranitz, the president of the CJD Foundation, said if the exposed patients need to have other medical procedures, they are required to say they may have been exposed to CJD. Some medical centers may deny them care, she said.
“Considering how they were exposed, I would think that (UW Hospital) should be more than willing to provide the necessary care,” Kranitz said.
Brunette, the UW Hospital spokeswoman, said the patients should be able to get medical care in the future.
“These people don’t have a disease. They don’t have a diagnosis,” she said. “That wouldn’t be a factor for us. I don’t think it should be a factor for another institution.”
Workers not at risk
Transmission of the classic form of CJD occurs only through direct implantation of tissue into the brain, spinal cord or eye, Safdar said. That’s why health-care workers and others are not at risk. “You’d have to have an instrument with a patient’s tissue on it and then penetrate it into the brain, spinal cord or eye,” she said.
Oral transmission — for instance, from a doctor’s finger into his or her mouth — has not been reported as a route, she said.
If UW Hospital surgeons had suspected CJD prior to the woman’s brain tumor surgery, they would have instituted extra layers of precaution, Safdar said. They would have disposed of the instruments used for the tissue biopsy and sequestered all other instruments used in the surgery until receiving a conclusive diagnosis, she said.
Geschwind said there are no national protocols guiding doctors and hospitals to treat brain diseases that could potentially be CJD. But he said any time a patient has rapid dementia or other symptoms of the disease, such as loss of motor function, CJD should be considered a possibility.
At the UCSF Medical Center, doctors destroy all instruments after operating on patients that possibly could have CJD, he said, which costs the hospital up to $20,000 per operation.
While incinerating the instruments is an option, Safdar said the hospital chose to follow CDC guidelines for heightened sterilization because “they do work.”
The instruments are being sterilized by being soaked in sodium hydroxide for an hour and then heated to 250 degrees Fahrenheit for an hour in accordance with the guidelines, she said.
Brunette said hospital staff has not calculated the value of the instruments and did not consider cost in deciding whether to save or destroy the instruments.
Although no sterilization process is known to completely destroy all prions, the CDC guidelines — and additional cleaning and disinfecting — lower the tissue load “several fold,” which is adequate for preventing transmission, Safdar said. Asked what risk future patients have if operated on with the surgical instruments, Safdar said, “I would say none.”