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Unknown Prion Disease
Killing Seattle Woman
"... neurosurgeons used some of the same instruments to operate on 12 other patients."
By Tracy Vedder
KOMO-TV Seattle
8-19-4
 
SEATTLE - Doctors at Harborview Medical Center are some of the best in the country, but now they are stumped. They've got a patient with a mystery disease.
 
The illness is not mad cow disease, even though it looks like it. And doctors are turning to scientists at the Centers for Disease Control for help.
 
Doctors know the woman who arrived at Harborview in July has some sort of fatal, brain-wasting disease. But they don't know exactly what it is. And that's critically important for other brain surgery patients who might have been exposed.
 
Harborview neurosurgeons took a brain biopsy -- a small piece of brain tissue -- from the woman in July. They hoped she had some type of treatable vascular disease and needed the biopsy for a diagnosis.
 
"Is this a bacterial disease? Is this vasculitis?" says Chief of Neurosurgery Dr. Rich Ellenbogen. "That's what we were looking for."
 
The initial test results were horrific. "The patient that we're talking about has an invariably fatal disease," says the head of Harborview's Neuro Pathology, Dr. Tom Montine.
 
Doctors don't know the exact disease, but they know it's in a family of always fatal diseases similar to mad cow and Creutzfeldt-Jakob.
 
Dr. Montine describes what happens, "the brain just starts to disappear, it literally just degenerates away."
 
The head of the Creutzfeldt-Jakob Foundation, Florence Kranitz, has seen people die from this disease. "It is dehumanizing, it is probably the most awful disease on the face of the earth."
 
What's even worse? Before Harborview got the results from the biopsy, neurosurgeons used some of the same instruments to operate on 12 other patients. And prions, believed to cause these diseases, are not killed by common sterilization.
 
"If you've ever seen anyone struggling with this disease or die of this disease," says Kranitz, "then you want to make sure that not even one person is exposed to it if it's preventable."
 
Harborview says it does twice the standard sterilization, using heat for twice as long and automatic washers. The standard method of sterilization if a hospital knows there is a risk of prions, is to soak the instruments in caustic solution of lye in addition to the other techniques.
 
That's what Harborview did once it confirmed the possibility that prions were present, but after surgery on 12 other patients.
 
Top experts in the country agree the risk of transmission at Harborview from one patient to another is very, very low. Harborview's Chief of Neurosurgery agrees, "I think the answer to that is miniscule, it is approaching zero."
 
Harborview has no plans at this time to notify those 12 brain surgery patients, because they're waiting for definitive word from the CDC as to the exact disease. That could be months away.
 
http://www.komotv.com/news/printstory.asp?id=32675




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