Chronic Wasting Identified in an Ohio Deer: Be Cautious Before You Prepare Your Food

Columbus Dispatch

Chronic Wastings, Scrappies, Mad Cow, and a disease I don't know the name of in Squirrels are all prion diseases caused by a misfolding of an Amyloid protein from an alpha helix state to a beta pleated sheet state which is nonfunctional to the cells. The beta pleated sheet state is less stable in a natural environment (the proteins are most likely to be in a helix state when dissolved in water); however, it is self-catalyzing so that over time if a single misfolded protein exists, it will eventually induce misfolding in all the other proteins until mainly the beta form is present.

In humans this is known as CJD (Creutzfeldt–Jakob disease), and can either be contracted naturally through genetic illness, can be acquired spontaneously over time through the accidental misfolding of an amyloid protein or can be acquired through eating foods, receiving tissue or organ donations with tainted tissues, or receiving surgeries with instruments that were previously exposed to a patient with prion diseases. Even autoclaving, UV lights, and chemical baths have proven ineffective in sterilizing equipment following contamination because the denatured protein, once misfolded, goes back to the misfolded state eventhough a denatured protein that has never been misfolded will go back to the alpha state. As far as I am aware, the reason behind this mechanism is still unknown.

TL/DR Pick up from beyond here

If you see a deer, sheep or squirrel in nature that has large patches of fur missing, or is clearly ill, do not eat it, and if you see the same in your sheep or cows and decide to butcher it anyway, do your best not to cut into the spinal column or get any of the brain tissue into the meat. I have heard it is possible to butcher these animals safely, but I wouldn't personally want to risk it, and I believe the FDA's stance is, once they are ill, they are not to be consumed.

Anyway, this disease isn't the slow progression like Alzheimer's in most people, and it can be very rapid and aggressive, leading you to perish unnecessarily young by slowly losing your cognitive functions and your ability to control your own body. If you have brain surgery ever, consult with your surgeon and ask that they use new or disposable instruments whenever possible. Often, these brain surgeries cost insurance companies 10s of thousands of dollars, so I think they can afford new instruments in most instances.

One thing I haven't heard of is using incineration to sterilize steel instruments, and as far as I can tell, that should work. If you break the carbon bonds on the protein, it is destroyed, so why they aren't doing this is beyond me.

I have shied away from cutting into central nervous system tissue on deer ever since CWD has been documented. I don't cut antlers out of the skull, and I don't cut into the spine any more.

a reply to: butcherguy

Good catch. I was unaware that the prion was in antler tissue, but apparently it is.

Pub Med