Stop SMA Clinical Trial - Univ of Utah. Visit #8

Aug 18, 2011

This was our last trip to Utah and it couldn’t have come at a better time.  Brielle and Brooke had some blood work drawn last week and Brooke had some concerning results.  The ones most concerning were her low white blood cells, high thyroid and very low platelets.  This can occur as a result of a separate disorder, such as leukemia, bone marrow failure, an immune system problem or as a medication side effect.  Dr. Swoboda took Brooke’s lab work very seriously and placed her under a “fall risk” right away.  If Brooke accidentally fell it could be life threatening because her blood will not clot. 

Brooke started the day with EMG testing.  She’s done this so many times that she knows how to do it herself!  :)  This records the electrical activity from the brain and/or spinal cord to a peripheral nerve root (found in the arms and legs) that controls muscles during contraction and at rest.  Dr. Swoboda specifically looks at compound motor action potential (CMAP) and Motor Unit Number Estimation (MUNE) scores. It it known to be a painful test but with sedation Brooke handles it very well. Then after this is finished, she gets some blood drawn to monitor medication levels and see if anything has changed since her last blood draw a week ago.

At this visit, her CMAP and MUNE scores have tripled since November 2010! Yay!!  The bad news is that her medications levels could be too high and causing this and unsafe to continue at these levels.  Brooke will have more blood work taken early next week to see if the medication is responsible for the abnormal bloodwork or if there is something more serious happening called chronic “idiopathic thrombocytopenic purpura (ITP).” Idiopathic means unknown cause. ITP occurs when the immune system mistakenly attacks and destroys platelets.  They would then have to check her bone marrow to see what is going on. 

Right now, we will just take one day at a time and see how her blood work comes out next week.  We are praying so hard that it is just the medication causing the abnormal labs.

On a good note, Brooke’s DEXA bone scan revealed steady incease of bone growth and is very close to “normal” for her age!!

 

More pictures from our visit:  

BruisingPlaying in the cribMessing with the blood pressure machine. :)Me and Brookie in the Children’s play area. Couture to Cure SMA Clippie (Etsy)