Froedtert Warns of Rare Brain Illness Risk; Surgery May Have Spread Creutzfeldt-Jakob Disease

By JOHN FAUBER and MARK JOHNSON

Froedtert Hospital is alerting about 100 people who had surgery this week that a patient who had an operation late Monday might have a rare, fatal brain disease that could have been spread through surgical instruments to other patients, hospital officials said Thursday.

The hospital in Wauwatosa closed its operating rooms and canceled scheduled procedures Thursday after receiving results of a preliminary test that indicated the patient might have Creutzfeldt- Jakob disease, part of the family of so-called prion diseases. These fatal brain disorders include mad cow disease and chronic wasting disease, which has infected Wisconsin deer.

The instruments used in the surgery Monday had been subjected to standard sterilization procedures afterward, officials said, but that might not have been effective and the instruments could not be tracked down.

Froedtert workers used a concentrated bleach solution to sterilize all the instruments that might have been involved. A supply of unused instruments allowed the hospital to continue doing emergency and trauma surgeries. Other surgeries resumed about 6 p.m. Thursday.

A sample of the patient’s brain tissue has been sent to a lab in Ohio to determine whether the 79-year-old woman has Creutzfeldt- Jakob. Preliminary tests, which use spinal fluid, often yield false positives, but confirmation should be completed by the middle of next week, according to G. Richard Olds, an infectious disease expert and professor at the Medical College of Wisconsin.

The spinal fluid test suggests there is an “intermediate” chance that the woman has Creutzfeldt-Jakob disease, he said.

Olds said he suspects that tissue sample test will be negative but added that “we wouldn’t have done what we did if there wasn’t some risk.”

Hospitals seldom find themselves in the situation now confronting Froedtert, partly because Creutzfeldt-Jakob is so rare.

“It is extremely unusual,” said Nasia Safdar, a clinical assistant professor of infectious diseases at the University of Wisconsin School of Medicine in Madison.

Creutzfeldt-Jakob occurs in about one in 1 million people a year in the U.S., roughly 250 to 300 cases. There is no cure.

As for hospital-acquired Creutzfeldt-Jakob disease, Safdar said the medical literature contains no more than a few cases.

Patient had brain biopsy

At a news conference Thursday, Olds said a routine spinal fluid sample was taken from the woman at another hospital before she arrived at Froedtert, where she had a brain biopsy because doctors suspected she has cancer.

Olds said he could not name the other hospital or what precautionary procedures, if any, it has taken.

If the woman has Creutzfeldt-Jakob, the odds are small that it would have been transmitted to any of the roughly 100 patients who subsequently underwent surgery, Olds said. Still, the hospital is notifying patients who had surgeries this week to alert them to the situation.

“Obviously we’ve made a lot of people nervous,” he said. “It’s likely this will turn out to be a false alarm.”

In a statement, the hospital said that even if Creutzfeldt-Jakob is confirmed, the risk to patients and staff “is extremely low.”

The hospital characterized the preliminary spinal fluid test as “ambiguous.” It said the test was more consistent with a bacterial infection and did not have any of the classic pathological findings of Creutzfeldt-Jakob.

Olds said Froedtert did the responsible thing by stopping surgeries and notifying patients. He said other hospitals have had similar scares but did not act until after a final confirmation was obtained.

‘It was just too much’

Still, family members who might already be stressed over their loved ones going through surgery now have one more thing to worry about.

“I just cried. This is it for me. It was just too much,” said Irene Harris of South Milwaukee, who learned of the news Thursday afternoon and worried what it meant for her husband, Michael. He had undergone roughly 10 hours of back surgery on Wednesday.

Harris called her husband’s floor Thursday, trying to learn more about the infectious disease she heard discussed on the news. She wanted to know whether her husband could be given a test to make sure he’s OK. She spoke to a hospital employee who could not answer most of her questions. A staff member later reassured one of Harris’ relatives.

Even if the disease was transmitted to some of the patients, because of its typically long incubation period, symptoms might not appear for years or even decades. The only definitive test for the disease requires brain tissue samples.

In 2002, a British medical journal reported on the case of a man who developed Creutzfeldt-Jakob 38 years after contracting it during a medical procedure.

Jim Kazmierczak, a state epidemiologist, said that there have been 10 documented cases in humans worldwide in which the disease was spread through tainted instruments.

“This poses no risk to the public at large, and assuming the patient has CJD, which is not a given, the risk to the people on whom the instruments were used is very minimal,” he said.

Hard to destroy

The instruments used in the patient’s surgery were entered into the normal sterilization process afterward. But because prions can be very difficult to destroy, normal methods may not be adequate. For instance, an autoclave uses steam and pressure to kill bacteria in minutes, Olds said, but it might take many times longer to destroy prions.

One reason prions are so difficult to destroy is that they are not typical infectious agents.

The so-called prion theory holds that prions are neither viruses nor bacteria and that they contain no genetic material. Rather, prions are misfolded proteins that are only slightly different in structure from normal proteins found in the brain.

“The issue with prions is they are extraordinarily resistant to disinfection and sterilization,” said UW’s Safdar. “It’s very difficult to make sure those instruments are free of prions.”

A RARE DISEASE

1.2 per million: The annual rate of deaths from confirmed Creutzfeldt-Jakob disease in the U.S. from 1999 to 2004.

22: Deaths from confirmed Creutzfeldt-Jakob in Wisconsin in the same period.

Source: U.S. Centers for Disease Control and Prevention.

Kawanza Newson and Ben Poston of the Journal Sentinel staff contributed to this report.

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