This is How the Story Begins

My wife, Shannon, suffered from mitochondrial disease – or mito as it is known to the victims, friends, and family of those afflicted. A mito patient’s mitochondria are not effective at “producing the power” that cells need to function and that organs need to thrive. It is relatively rare; something like 1 in 3,000 are affected by it.  Its cause is not well understood, there is no truly effective treatment, and there is no cure.  On the other hand, mito is critical to understand because the cellular function that mito patients lose is though to play a role is an entire constellation of conditions, including ALS, autism, and Parkinson’s.

Shannon was diagnosed in her early twenties, around the time that we were married.  Her primary concern was whether or not she would pass it on to any children.  At the time, she was advised that the greater risk was to her health in carrying a child to term.  She was undeterred; we married and had two very healthy boys.

During her 20s and 30s, mito wasn’t really on our radar. That approach made sense because there wasn’t a treatment that was anything beyond a hope and a prayer – and because the best doctors could tell her was that she either would either (a) experience a slow descent caused by the unavoidable and untreatable cumulative effects of mito or (b) die of some other natural cause before mito had a chance to hurt her. She did try things that were thought to help, such as co-enzyme Q10 and L-carnatine, but all she could tell they were doing was creating unpleasant side-effects.  Mito was out of sight and out of mind.

In her 40s, the signs of mito’s impact began to appear. Her vision – never great – got worse.  Ocular myopathy, droopy eyelids, etc.  She had problems with digestion. She started to have problems chewing and swallowing. She hardly ever complained.  What she was was afraid of what she saw coming – an active mind trapped in a body that couldn’t function.

She was spared that future by her death.  After she and I dropped our youngest at College, she finally underwent arthroscopic shoulder surgery as an outpatient.  The doctor came to tell me about the success of the procedure and sent me to get the car. Unfortunately, all the years that all of her systems were not being properly “fed” with energy caught up to her. Though she had awoken fine from the procedure, recovery was too much for her and she collapsed while I was driving around to pick her up.  She died 11 days later and the blogging started.  It’s been a three year journey that I never wanted to take, but I have tried to do it in a way that honors Shannon.   Even though she isn’t here anymore, I still want to make her proud.