We have three different types of nerves in our bodies: motor, sensory, and autonomic. Motor nerves control movement. Sensory nerves control sensation. Autonomic nerves control the things we don’t think about—digestion, circulation, and body temperature, for example. Most of the people with congenital (from birth) sensory nerve issues also have autonomic nerve issues. Gracie is no exception.
We had a scare a few nights ago. We went out to eat as a family at a restaurant we haven’t visited for a while. It was very busy, and also very hot. Gracie doesn’t do well in hot environments; her internal thermometer cannot recalibrate quickly, so when the external temperature is warm, her internal temperature keeps rising and she cannot cool herself down. The rest of us use our autonomic nerves to trigger our built-in cooling mechanisms, so when we are in warm environments, our bodies start to cool ourselves down with our own internal processes. Since Gracie’s nerves are dysfunctional, this doesn’t work for her. It takes her much longer to adjust to a room’s temperature than it does the rest of us.
While we were at the very hot restaurant, Gracie started melting down immediately. She held it together pretty well, considering, but the longer we were there, the worse she got. Unfortunately, because the restaurant was busy, it took a long time for our server to get our order, bring our drinks, and get the food. By the time he delivered the food, it was over for Gracie. She was complaining that her tummy hurt, she didn’t feel well, and she didn’t want her food. You know something’s wrong when Gracie won’t eat french fries dunked in ranch! Eventually, she lay down under the table while the rest of us continued eating.
Finally, our server came back, and we got the check. We hustled Gracie out of there, taking her outside with no coat so she would cool down faster (it was pretty chilly outside, in the 40s or so). As soon as we got her into her car seat, Gracie fell asleep. Or should I say, she passed out.
At home, Chad carried Gracie into her bed, and I tried to cool her down. I put ice packs on her neck and undressed her. She didn’t wake up. I changed her into pajamas. She still didn’t wake up. I tapped on her shoulder and face, calling her name. She still didn’t wake. By this point, I was getting worried. Should I take her to the ER? Is there anything the hospital could do for her? Would she wake up as soon as we got there? Her vital signs were normal; I didn’t think the hospital would be able to help us. I decided to wait it out.
Since there was school the next day, I had to do our wound care ritual. I removed the bandages from all her fingers, not at all trying not to wake her. Nothing. I washed her hands thoroughly with a wet cloth, and rubbed her face with it too. No response. I re-bandaged all those little fingers, carefully and methodically. She slept through it. Throughout it all, I would periodically tap her shoulders and call her name. I tried pretending she had to get up for school, or that dinner was ready, or her dolls needed her. She did not wake up. She would occasionally turn her head away from me but did not wake up. Nothing I could do would wake her.
I reached out to the other parents of painless kids, and found out that several of the children are unresponsive when they overheat. Indeed, many of them have to sleep for several hours before they can awaken. Although it’s reassuring that this reaction was not unusual, it is scary and nerve-wracking to have a kid who won’t respond.
Gracie woke up the next morning at 6 a.m., eager to do a little playing before school. Other than being more clumsy than usual, she seemed totally fine. (Clumsiness is related to overheating—her muscles get weak so her ataxia is worse than usual when she overheats.) She had a great day at school that day, and was generally completely back to normal.
You would never know she had had a medical emergency the night before. But then, that’s pretty typical of Gracie. She is amazing, both personally and medically.
Interesting… I have the same issue with MS. Heat just shuts me down. Thankfully, I’ve never checked out as thoroughly as Gracie!
I’ve heard that her issues are similar to MS. She also has a hypomyelinated brain, which happens with MS too. Interesting…