Fire Drills and Wishes

Post date: Oct 6, 2011 7:50:56 PM

Our family practiced a home fire drill recently. It was a task Ian needed to complete for one of his Cub Scout achievements. It was early in the morning before school. Ian had already finished breakfast and even had his teeth brushed and hair combed. Amazingly, we still had about half an hour before we had to leave the house for school. That never happens.

So we practice our fire drill. We talk about staying low to the ground to avoid smoke. To check the door for heat before opening it. The window is an alternate escape route. He can open the window, push the screen out and crawl through it to fresh air and safety. Then we practice it, trying to be really quiet. Connor is still sleeping. Ian thinks it fun and cool to actually crawl through the window. While we reassemble and close the window, he walks back in through the garage door.

We sit again to review and discuss: Wait by the neighbor's mailbox for all of us to meet. Don't try to come back into the house. Suddenly Ian says, "What about Connor? I can't leave him. He wouldn't be safe! I want Connor to be safe." Then he starts to cry.

A week later, we are driving to dinner. We're going to eat Italian at the restaurant down the street. Ian loves the lobster ravioli in pink sauce, that rich cream and vodka sauce that is so yummy to dip their fresh baked bread in. I need to check Connor's CGM again. After all, it has been at least five minutes since we last checked it and for Connor, so much can change in five minutes. His glucose could drop 50 points. It could rise 100 points. Well maybe I exaggerate, but just a little. Despite my fears that the school staff would think me paranoid and neurotic, just the opposite happened. After 3 days of school and watching his glucose levels rise and fall, they brought in an aide who's main job is to watch his numbers and report them to the nurse every 5 minutes. Yes, every five minutes. Turns out we might be paranoid, but apparently with reason.

I can't get to Connor's CGM from where I am in the front seat. Ian, sitting next to Connor, is used to reaching over and checking it for me. He yells out the number, "It's 168 and the arrow is going angled up." The arrow indicates how quickly Connor's glucose is rising or falling. Then, "Banana!" (He sees a yellow car. It's a new game we play. We yell "Banana!" if we see that rare yellow vehicle. Not as rare as you might think once you start looking for it though. Rather similar to mitochondrial illnesses, come to think of it.)

Then Ian asks, "Will Connor always have diabetes?"

"Probably. His pancreas, where insulin is produced, doesn't have enough energy to make it right now. There is a small possibility that his pancreas could one day produce enough insulin so that he doesn't need us to give him more. However, it's not likely." We talk a bit about possibility versus probability and that even though it is possible for Connor's body to suddenly work better, the probability is that it won't. But we should always keep hope.

Ian, contemplatively, " I wish Connor would grow out of being diabetic. It sure would make things easier. And then we wouldn't have to prick his fingers anymore and there'd be no more Pods and Dexcoms. " Then with enthusiasm, "He wouldn't get as much pain. Wouldn't that be good? I think that would be a nice miracle."

We are reminded how hard it is to be the "healthy" sibling. He can have all the lobster ravioli he wants tonight. And dessert.

We've become well acquainted already with hematology, gastroenterology, and endocrinology as the "-ologies" that actively monitor and manage his different symptoms. Hematology and we recently celebrated a divorce, or at least a long-term separation, as Connor has not needed transfusions for a very long time and his blood counts have been good. Nephrology is the latest specialty we are flirting with. The new medications recently added, daily blood pressure checks and labs, labs, labs are mostly being ordered by nephrology. Connor's condition has progressed and he has developed Fanconi syndrome.

Fanconi's is a disorder of the kidneys where the kidneys are unable to reabsorb certain nutrients back into the body and they instead get excreted with the waste. "There's not much we can do," is a sentence we hear often and dread. There's nothing we can do to make his kidneys work better, they either will or they won't. What we can do is give him additional medications to supplement the nutrients his body is losing. It's a balancing act because too much of one can affect another, so we hope our doctors are fine chefs who can find the right recipe.

To recap, Connor has a mitochondrial illness in which a large proportion of his mitochondria are dysfunctional. Mitochondria are sub-cellular organelles that produce energy. When they don't work, the body simply doesn't have enough energy to do its job. It's like a factory where the lights are always flickering and not everything works all the time. Sometimes the power goes out and we're left in the dark - waiting for the next burst of light, hoping that the next set of energy will last a while and nothing breaks. Unspoken is the hope that we even get another run and that this one isn't our last.

I'm finding out now that mitochondrial illnesses are not rare. One statistic states that every 30 minutes a child is born who will develop a mitochondrial disease by age 10. Pearson syndrome, what Connor has, is still considered extremely rare. We've found just a handful of families worldwide who have children with Pearson's via online social groups. It is a small club no one ever wanted to join, but we've been able to share helpful information and commiserate on the lack of medical information and guidance a disease as rare as this has. The wider groups that encompass other mitochondrial diseases have also proven useful (UMDF and MitoAction are two) but the amount of information can be overwhelming and not always helpful. For all mito-related illnesses, the message seems to be constant and consistent: no patient is the same, and we can only try to treat symptoms as they appear.

For now, Connor is cheerful and happy despite his kidneys not cooperating with our goals. We continue to enjoy the cooler weather and made a recent trip to the pumpkin patch. After all, Halloween is just around the corner. Folks are starting to ask what the boys will be for Halloween. We seem to have set a precedent that may be hard to top. Ian has ideas brewing already - something to do with his recent "faux-hawk" hairstyle. As for Connor, no matter what he dresses up as, he is still "the King" around here.