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.
Feel free to leave comments here! Discuss who you saw and what you learned while there and network with others who went while informing those of us who couldn't make it.
posted by Heather Larson at Monday, July 10, 2006
I am a media veteran with 12 years experience in radio broadcasting, print journalism and online content writing. You can e-mail me at: hotwords AT mac dot com.
2 Comments:
Heather: I'll write up my notes again like last year. The research day (Saturday) was the best I have attended so far and there were a number of points made that were new or striking for us families. Highlights include: (1) combining therapies to inhibit prions at the NIH lab in Montana; (2) extensive studies of early symptoms of CJD at UCSF in terms of personality/consitution/cognition that Dr. Geschwind reported on; (3) retrospective study of sCJD in the UK which appears to show geographical clusters in terms of where people lived several decades ago that we heard about from Dr. Will at our roundtable; (4) a new human prion disease uncovered at Case Western that is negative on the Western Blot that Dr. Gambetti reported on (5) a well-funded broad prion program in Canada that Dr. Cashman reported on. The panel Q&A was frank and good questions were aired -- I took extensive notes. The roundtables again were the best I have experienced. I got the opportunity to hear Dr. Ghetti, Dr. Will, and Dr. Cashman answer family questions. There was a lot more that struck me as noteworthy on Saturday and as soon as I recover from the intensity of the three days (having lobbied today), I'll get my notes to you all.
The second day (Sunday) the highlights include: (1) a separate session for those with the genetic variety that Deana Simpson (www.cjdinsight.org) arranged that provided more genetic Q&A and a forum for other concerns and questions. Deana says she hopes to expand this next year to the Friday before the full conference; (2) the video of the Medical Education Program which was very professional, engaging, informative, and absolutely "right on" in every way -- the second video on care and end-of-life issues is being developed now and will be completed by the fall; (3) Jonny's Journey -- which Don Simms made with others to tell in simple words and music and videos what occured to Jonny Simms -- left everyone weeping and asking why on earth Jonny was denied compassionate treatment with PPS; (4) the Japanese delegation included ~ 6 people and they showed a video of a number of iatrogenic CJD victims before and during their illness and talked about how the Japanese iatrogenic CJD situation unfolded -- I exchanged cards with them and they gave me material on the lawsuit for dura mater grafts which is what caused these 122 cases of iCJD in Japan -- as caused Danny Blejer's death at 52 so for me this was personally significant to meet them -- the speaker was the young physician son of one of the victims which was tragic and moving. (5) the preparation session for today's lobby was led by Rhonda Rutledge, her daughter Heather Harris; and the Chief of Staff of Senator Sessions of Alabama and it was first-rate, the best I have attended as well. The Chief of Staff was direct, down to earth, succinct, and very helpful. I'd vote for him in a second if he were running for office regardless of his party affiliation. I used a number of the techniques he mentioned.
For me again personally it was significant that there was a table of young people who had lost their parents at the dinner Saturday, which included my son, and some of them got to speak about their feelings and loss. I had wanted to see this type of interaction since several young people approached me in 2004 and wanted to talk with my kids and since Heather suggested a separate meeting like this for last year's conference. Then, after Sunday's closing session, a number of us women -- especially us widows in our late 50's and 60's got together for drinks and again from a personal standpoint it was special to share feelings and concerns and stories about how we were coping or not among ourselves. I made many new friends.
I lobbied alone and what struck me this year was the breadth of what we asked for from additional funding for human prion surveillance to immediate mandatory identification and tracking of cattle to a pilot for voluntary testing by cattle processors like Creekstone. This was my third year and the offices I visited remembered me and were more educated and concerned about prion diseases than ever this year. I plan to personally follow up in district offices in August when session is out. I hurt my right foot wandering around Capitol Hill and hope I don't end up on crutches! But it was again worth it to lobby and I was disappointed there were not more states covered this year. I urge you all to participate in this next year or to get to your congresspeople on these issues before appropriations -- several times if possible.
7/10/2006 6:14 PM
I'd love to hear more about what the UCSF folks had to say about personality/constitution/cognition and how it relates to the stress factor we've all discussed before.
I'd also like to hear about what Dr. Gambetti said about a new human prion disease uncovered at Case that is negative on the Western Blot. That certainly sounds wild! It never ceases to amaze me, but I still do believe with all my heart that we are missing something HUGE when it comes to prion diseases. Slowly, we're chipping away at the mystery though.
I wish I could have been at Congress today lobbying with you. How many families went to lobby Congress?
I will have to contact Deanna. A day devoted to genetics would be wonderful. I'm all about it!
And I especially want to hear about Johnny Simms and what abilities have been restored to his body. Is he eating? Swallowing? Walking? Standing? Sitting up? Comprehending? Communicating? I'm anxious to hear specifics on how far he has come on PPS. It makes me so grateful his family fought so hard for his PPS therapy. Don Simms should be commended for pushing for it. He's helped us ALL move forward by leaps and bounds.
7/10/2006 9:22 PM
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