Granted, this year there had been changes that were more marked than in previous summers. At night, Mattie now had to be on a BiPAP machine, short for bi-level positive airway pressure. It involves wearing a mask over your nose or mouth or, in Mattie's case, both, that helps you breathe easier when you're short of breath. Mattie also had to wear it during the day if he felt exhausted, such as right after pool time. This was in addition to the pulse oximetry and cardiorespiratory monitors to which he was connected anytime he was sitting or lying at rest since the day he was born, which would let us know if his heart or lungs weren't doing what they were supposed to. The dysautonomic in dysautonomic mitochondrial myopathy means things in the body that should happen automatically don't always. For example, when someone switches from physical activity to sitting, the heart self-regulates by beating more slowly. But Mattie's heart could overshoot the mark and start to "forget" to keep beating while he was at rest rather than simply slow a bit; the fine-tuning just wasn't there. If his heart rate fell too low, the machine triggered an alarm that would signal someone to jiggle him or provide other tactile stimulation or remind him to breathe more deeply for a minute until his heart could receive the "signal" and get its pumping back in sync with his body's needs.
Understanding this condition and how to deal with it came slowly, across the lifespans of all four of my children. The medical community didn???t even have a name for it until my two eldest had died. It was simply called dysautonomia of unknown cause, and early on I was told that subsequent children would not be affected. Not until Mattie was 2 years old and my third child was months from death did doctors understand that it was a condition of faulty mitochondria—an essential component of every cell in a person's body.
I was actually diagnosed first—with the adult-onset form—then the children. Two years later, after my third child died and Mattie was only 4, I was in a wheelchair. Now we were all too familiar with the condition's devastating effects.
We were used to adding supports and medical machinery to compensate for the detrimental effects of this progressive condition, and then we would keep going. But that summer, the changes weren't just in the BiPAP machinery or even in the fact that Mattie didn't have the energy to really swim. Hope, who was two years his junior, was now several inches taller than he was. Mattie's shoe size, in fact, was the same as in kindergarten—a child's 11. Growth taxed Mattie's autonomic system, and somehow his body knew that. In addition, he could not walk across the beach to the ocean. Mattie didn't need his wheelchair because he was incapable of walking; he needed it in large part because he would tire so easily.
In previous years, he had the energy to walk across the sand to the waves so he could bodysurf (always attached to his oxygen). He had to. We didn't always have a special beach wheelchair that could get traction over the sand. But this summer, after the first day of walking out to the water, he said he couldn't go back; it took too much out of him.
We did have a rented beach wheelbarrow that got me down to the waves that first day, and I told him he could hop on while someone pushed. But he said no. He was aware that he lacked the energy to handle the waves. And he knew he wasn't up to getting overheated on the hot sand; his condition also compromised his temperature stability, so that once he became too hot or too cold, his body had a hard time readjusting to normal.