Saturday, January 11, 2014

Update 2014

     Well, I made it through another year.  Had the holidays at our new home for the first time.  Hosted several parties and lots of people coming and going.  It was fun, exhausting and energizing all at the same time.

     On the amyloid front I continue to get progressively worse, but my spirits are good most of the time. I chose not to attend the Chicago familial convention in October figuring I could get any updates I needed from the ASG website--which I did.  I missed not getting to meet other patients, but the trip felt a little overwhelming at the time.  My energy level continues to diminish, and my mobility is noticeably worse.  I'm down to 3 1/2 days a week at work and seem to be managing that ok.  My weight is down to 125 lbs, and I have a lot of fatigue.  I have a ground floor office now which really helps, and I think its time to give up my regular office on the second floor.  Climbing those damn stairs is just getting to be too much.

     The  diflunisal trial is over and looks encouraging.  While perhaps not a two thumbs up (1 3/4?), it looks like there is now an approved and recognized option available in the US that may help stop or slow the progression of this disease.  It is encouraging enough that I plan to talk to my sons about getting tested.  If they have the gene, they could start taking this now without any real known side effects, and it could help hold off the potential onset of any symptoms.  Meanwhile, other trials are ongoing and may prove even more effective down the road.

I contacted Dr. Poterucha about me restarting the diflunisal as well.  I was in the trial five years ago, but had to stop once I had my transplant.  The trial data could be skewed by transplant recipients, so that is an exclusionary criteria for the trial.  In addition, Dr. Poterucha felt it could further compromise my kidneys which were already being taxed by both the disease as well as my immunosuppressants.  However, given that the drug has now shown some proven effectiveness, and given that I'm getting worse anyway, I talked to him about resuming the diflunisal while monitoring my ongoing kidney function.  We are going to give it a try.  It won't be a cure.  Or at lease they don't expect any reversal of the damage that has been done.  But maybe it will help stabilize the progression and let me hold on to what I still have and prolong my life by a few more years.  Statistically I am already in the end of life expectancy range measured from the date of symptom onset (its now been 7 years).  And while those are just averages and numbers, they are attention grabbing.  Why not try something?, is how I feel.  At least to feel like you have a chance to fight back.

     As it says in the St. Francis prayer that I recite so often:  "Where there is doubt,  Where there is despair, ...hope.  Where there is darkness, ...light."  I continue to try to let my light shine in hope for healing, while maintaining faith that whatever happens, all is well.  Blessings to all.

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