Six face remote risk of rare brain disease

Patients treated with same instruments as woman who died

Bill Scanlon, Rocky Mountain News

Published May 19, 2006 at midnight

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LITTLETON - A patient at Littleton Adventist Hospital died of Creutzfeldt-Jakob disease in March, raising a very remote risk that six other patients who had surgeries using the same instruments could contract the rare and fatal neurological disease.

"It's an extraordinarily low risk," hospital CEO David Crane said Thursday, equating the chances of developing the disease from contact with the same surgical instruments as akin to "getting hit by lightning five times in the same day."

In fact, Crane said, there has been no documented case of anyone getting CJD from a surgical instrument since the 1970s, when sterilization procedures were improved.

The case is similar to one at St. Joseph Hospital in Denver five years ago that became a warning bell nationwide about reusing surgical instruments in cases of unknown neurological diseases.

In the St. Joseph case, six patients were operated on with instruments used on a patient that doctors later learned had died of CJD. None of those six have shown symptoms of CJD, St. Joseph officials say. Symptoms usually occur in three years.

In the wake of the incident at St. Joseph and elsewhere, the Centers for Disease Control and Prevention came out with new protocols for sterilizing surgical equipment.

Littleton Adventist Hospital follows and exceeds those standards, but it didn't take an extra step that St. Joseph takes on its own.

At St. Joseph, when surgery is done on a patient whose neurological disease is unknown, the instruments are set aside until a biopsy confirms that the patient does not have CJD.

The hospital also prescreens patients who may have risk factors for CJD, such as rapidly progressive dementia, and follows the protocols for handling CJD cases.

Creutzfeldt-Jakob is in the class of prion-related diseases that include mad cow disease and chronic wasting disease. It is the human form of the syndrome in which the prion proteins go haywire.

The sporadic version of CJD, which the woman at LAH had, cannot be contracted or spread by the eating of contaminated beef or deer meat, the hospital's chief medical officer, Dr. Lawrence Wood, said.

About 15 percent of people with CJD have the variant form, which can be contracted by eating beef or venison from a diseased cow or deer. Those people tend to be younger than those who get the classical or sporadic CJD, many of whom also have Alzheimer's disease.

The woman, who had dementia, went to the hospital in February for treatment of a head injury, Wood said. She underwent surgery to relieve pressure. At the time, CJD wasn't suspected.

But her condition worsened, and on March 14 doctors started suspecting CJD. After she died on March 23, an autopsy was performed and a brain biopsy was sent to Case Western Reserve University in Ohio, the only place in the nation that tests for the disease.

The results came back May 9, confirming CJD, which has been found at the rate of one person in a million nationwide. The hospital would not say how old the woman was or where she lived.

Between Feb. 13 and March 14, six LAH patients had neurosurgery involving the transparent membrane that covers the brain or spinal cord.

LAH uses state-of-the-art sterilization, Wood said, but sometimes that's not enough to kill abnormal prions, which on very rare occasions change from the normal spiral shape to a washboard shape in the brain.

That washboard shape serves as a template for other prions to switch shapes, rapidly pushing forward the disease. The washboard shape is also more resistant to being broken down by enzymes or sterilization.

The early symptoms of CJD can include insomnia, depression, confusion, personality and behavioral changes, strange physical sensations, and problems with coordination and sight. Many of those aren't much different than typical signs of dementia, so it's difficult to diagnose CJD, especially since it's so rare, Wood said.

Nothing but a brain biopsy can confirm CJD, and there is no treatment for the disease.

After the CJD confirmation came back from Case, the hospital notified the Colorado Department of Public Health and Environment.

The hospital has now instituted a more comprehensive fail-safe system, including matching individual patients with individual instruments.

But instruments won't routinely be thrown away after one use, nor will they be given the super sterilization process recommended after CJD has been confirmed, Wood said. To do that would harm the tensile strength of the expensive instruments and hasten their retirement.

The instruments used on the deceased woman and the six others, though, have been disposed of, the hospital said.

Surgeons have contacted all six of the patients and explained the low risk, Crane said.

"This is very surprising and shocking news to patients," he said. "We're going to give them both emotional and medical support. They're still processing this very important news."

After the incident at St. Joseph Hospital, officials there decided to quarantine surgical instruments used on any brain surgery patient whose diagnosis was unknown.

Nonetheless, at least one of the patients sued St. Joseph, saying that he has anxiety every time he has a memory lapse, and that enough was known of CJD at the time that the hospital should have quarantined the instruments until it had gotten a diagnosis from the biopsy. The lawsuit was dismissed last year, the hospital said.

John Pape, epidemiologist for the state health department, said that LAH reported both its suspicions and the confirmation promptly.

The hospital already has more stringent sterilization procedures than those recommended in scholarly papers, he said.

"The tool tracking, that was something that we did recommend as one way of being sure of which equipment is being used on which patient."

Creutzfeldt-Jakob disease

A rare, deadly neurological disease caused by a change in the shape of prions, proteins that normally occur in the brains of humans and many other mammals.

• Types of CJD: Some 160 people worldwide have been diagnosed with variant CJD, which can be contracted if humans eat beef from cows infected with mad cow disease, also a prion disease. The average age of a person with variant CJD is 28. Slightly more common is sporadic CJD, or classical CJD, which typically hits older people. Its cause is unknown, but like variant CJD, it is fatal, and there is no known treatment. The average age of someone with sporadic CJD is 68. About one person in a million in the U.S. has been diagnosed with sporadic CJD. About two cases of classical CJD are reported in Colorado every year. In 2000, four cases were reported, a figure that's in keeping with the 1 in a million average.

• Symptoms: Insomnia, depression, confusion, personality and behavioral changes, strange physical senses, and problems with coordination and sight, followed later by rapid deterioration in brain function.

• Time before death: About five months for sporadic CJD. About 14 months for variant CJD.

• Not chronic wasting disease: Chronic wasting disease is a prion disease affecting deer and elk. Unlike mad cow disease, there is no known case of a human contracting variant CJD from consuming the meat of a CWD-infected animal.Sources: Centers For Disease Control And Prevention; Rocky Mountain News

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