Once again I have been perseverating on how Sarah died, and to a certain extent on how my mother died, which fills up the extensive mental real estate not preoccupied by the subject of where Sarah is now. I know that Sarah most likely died during a seizure. Her death certificate states “Asphyxia” as the cause of death with “Complications of cerebral palsy” as the secondary cause of death.
My mind will work over and over what must have happened. We know several other special needs kids who went to sleep one night and did not wake up in the morning. I have heard from a mom friend who has a kid with a different rare genetic disorder of sitting next to their child on the couch one minute interacting, and the next, the child is completely gone. I can’t imagine the pain of that scenario.
I have lately been reading a book by Dr. Sherwin Nuland called How We Die. Dr. Nuland says that there are ten thousand pathways to death’s door but every death is caused ultimately by lack of oxygen, which the body must have to continue its vital functions. We are obligate aerobes – we must have oxygen to live. He describes some of those pathways, particularly in older people. It was helpful to my understanding of how my mother, who had liver, kidney, brain, and blood pressure issues, ultimately succumbed.
The CDC says that SUDEP (Sudden Unexpected Death In Epilepsy) can be caused by a few different mechanisms: pauses in breathing during the seizure that reduce the oxygen in the blood to a fatal level; cardiac arrythmias from the seizure itself, or obstructed breathing during the seizure. Or a combination of these things. As Dr. Nuland writes “Oxygen is the focal point of the lens through which the sustaining processes of all living things must be studied.”
I don’t like to think about Sarah gasping for air or unable to breathe. But Dr. Nuland believes that once the sustaining oxygen falls below critical levels, it is accompanied by the body’s production of endorphins (endogenous morphine) and the injured or dying person is usually in a tranquil state. I’m glad to hear this, because he essentially says that there is no such thing as “dying in your sleep.”
I contacted the SPG-4 Foundation last week and asked them if they would consider adding an “In Memoriam” link or page to their very nice website. I do not know if there are any other children with Early Onset Complex SPG4 (or whatever the term du jour they are using for Sarah’s type of SPG4) who have died during the longitudinal study, but I think it would be nice to memorialize Sarah and all such kids. I think it would also be good for the parents and families of such kids to support each other. And, I think it’s important to recognize that this condition can potentially be fatal. I don’t want to scare the families but we are all obligate aerobes, right?
The First Year Without Sarah 