jmfa1957

My wife's mother died of sporadic Creuzfeldt-Jakob disease about 12 years ago. This is a REALLY nasty way to go. We do not know for sure how she contracted it but the fact that she had four back surgeries was a likely cause. She was 60 years old when she died.

Spongiform encephalopothies, or brain wasting diseases, can be attributable to a malformed protein (the term is prion.) Mere contact between a healthy protein and a prion converts the healthy protein into a prion. This prion cannot be destroyed by traditional sterilization procedures such as autoclaving or bleach; we think that she got it from a surgical instrument.

Since the transmission mechanism in people is not well understood, my wife and kids cannot even donate blood. We do know for sure that one can contract CJD from contaminated surgical instruments as well as from eating meat from animals infected with spongiform encephalopothiess, such as Bovine Spongiform encephalopothy (aka Mad Cow,) Scrapie (in sheep,) or Chronic Wasting Disease (deer and elk.) We do NOT know for sure whether or not there are other means of transmission, such as heredity.

Hunters in areas where CWD is known to exist are well advised to have the carcass tested, and to avoid contact with brain and spinal tissue/fluids. Once the prion makes contact with healthy proteins in the nervous system, it will work its way up the neural path and into the brain. There ain't no stopping it and the final weeks of an infected person's life are indeed horrible.