Friday, May 19, 2006

 

What's Bird Flu--Prions May Be the True Deadly Menace

I have posted about prions before, but they returned with a vengeance recently in a Denver operating room. Here's the lead:

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.

Creutzfeldt-Jakob disease is the same thing as mad cow (bovine spongiform encephalopathy), scrapie, chronic wasting disease and kuru--all caused, I believe, by molecules called prions. I couldn't tell from the story at first whether the hospital gave the disease to the person who died mere months later or if the person who had the operation already had the disease. The first impression was the worse.

I had my ACL reconstructed at that hospital (and Diomedes was nice enough to drive me home from the hospital and bring me take away dinner) a decade or so ago so the news about 'my' hospital had an extra locus of impact for me.

Here's the scary part:

[The hospital] 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.

There is no cure, no treatment, no vaccination (nor is any possible with our medical knowledge) and you can't see them. The definitive diagnosis is a brain biopsy after you're dead. We don't know why some prions go rogue or how they break the brain/blood barrier. They are not alive as we usually use that term ( and are just protein with no RNA or DNA), and they create the problem merely by touching molecules and inducing them to change shape with a catastrophic effect on the brain--that is, you go crazy and die. The only good thing is that they appear to be somewhat rare. At least we hope so.

The Avian flu is a lightweight next to these things and we don't know if we're spreading them around more or not. They certainly are increasing in the deer and elk population of my state. And I still eat deer and elk.

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