Our family is often seen swimming upstream. We have 8 kids. We home educate. We drive a very non politically correct vehicle. (15 passenger vans are NOT green! Ours is actually silver!) And we spend our Friday nights at the ER while most of America is sleeping.
Will thoroughly enjoyed playing with a friend on Friday. It was a rare opportunity that was enjoyed to its full capacity. Unfortunately, what had been simmering all week became a full blown mito crash that night. He went to bed on time even though he argued with me that he had not slept well all week. At midnight, he was wide awake, came downstairs where I was working (yes, I know. I should have been in bed as well, but that basement is almost finished!!!) and said, "Feel this." He was referring to his heart rate which was a whopping 132 beats per minute. Considering that 100 is tachycardic and is our "protocol number" for an ER visit for fluids, he was well beyond the point of wondering whether we should go. No waffling here. I sent him off to get dressed while I gave instructions to my oldest two boys who also should have been in bed but weren't, emailed Billy who then came home from work in order to go with us, and packed "The Bag." (My gym bag is always ready to go. All I have to do at any given time is throw in either gym clothes or regular clothes and I'm set for an indefinite period of time.)
After deciding to head to MCV instead of Mary Washington since they have a pediatric ER, his records are all there, and his specialists are all there, we headed south. The entire trip down I kept hearing him sigh in the back seat. Not a good sign.
The short story is Will's body does not make enough energy and he has no reserve capacity. While this seems like no big deal, it's a very big deal. His organs all require ATP (energy) in order to function properly. In its absence, damage occurs and it's irreversible. That is why this disease is progressive. With each crash, we potentially damage those organs even more. Not crashing is the goal but we haven't been able to prevent these crashes from occurring regularly. The sighing on the way down was an issue because it represents his body burning excess oxygen trying to create more energy that it just is not able to produce. It is the first time that has happened, which has me concerned... Is this a progression of the disease?
In previous ER visits, one bag of fluids is all he has needed to stabilize. Last night, one really didn't quite cut it. They were looking at admitting him because they really have no clue what to do with mitochondrial disease, when I asked them to hang a second bag and run a second bolus (a lot of fluid in a short period of time). Finally, his heart rate dropped below 100 but it still bounced all over the place which means that his heart rate was inconsistent...another sign that his autonomic nervous system is not coping well.
So, we finally were discharged and arrived home at 645 this morning, just in time to say good morning to my youngest three. Billy and I managed a couple of hours of sleep (thank you older kids for helping!) but we are exhausted.
On Tuesday, I will have to call Dr Teasley and determine if we need to make any further changes in his protocol and/or medications. Until then, we just watch him for further signs of distress which will provide us with another opportunity to visit the MCV ER and hang out with Kati, our nurse last night who promised to keep a look out for William and snag him as her patient when he returns. She actually read through Dr Shoffner's notes (Atlanta dr) which totally earned my respect because they are anything but an easy read. (She just wanted to understand Will's condition so she could help. Amazing!)