CJD is a disease caused by a misfolding protein, called a prion. (PREE-on) Prion diseases are a group of rare and fatal brain diseases which occur in both humans and animals. In humans, it is known as CJD. Cows get BSE, which stands for bovine spongiform encephalopathy. Deer and elk contract CWD, or chronic wasting disease. There is no cure or clinical diagnosis for CJD. There is no cure for any of the diseases in the prion family.

Sunday, April 09, 2006

Very Inflammatory Article!

Copy and paste this link into your browser for an especially inflammatory article on the NPDPSC:

http://www.sciencedaily.com/upi/index.php?feed=Science&article=UPI-1-20051021-21481300-bc-us-cjdcases.xml

I have a link to the NPDPSC to the right, by the way. I always wondered how that Patrick Hicks case was going to work out. Last I remembered hearing, the family was having trouble coordinating the lab work in France. If you read the article, well, it sounds like the trouble never ended!

The article is interesting and just another shining example of how our national surveillance system seems set up to fail most of the time. With genetic cases like my mom's, it's easy. The Center knows what it is getting in the mail when it knows it is getting tissue from a 56-year-old woman of Polish descent who already lost two relatives on her paternal side to CJD.

I'd sure hate to have to deal with a "sporadic" diagnosis in my family. I watch these sporadic families go through a lot of hell trying to get answers about how their loved ones died. I guess I have it easier since there is even actual research out there on E200K, 129M.

posted by Heather Larson at Sunday, April 09, 2006