So far, we have not experienced too many of these. The losses are less tangible. For example, my heart broke a little this January, when we took a boat out to experience the phosphorescent bay in Puerto Rico. It was an amazing experience and Griffin loved it. The reason my heart broke was because there were other boys about his age on one of the boats, and they jumped huge elated jumps into the water, over and over, leaping back up onto the deck in one smooth movement to jump back in again, shrieking with joy as the water lit up around them. I sat watching them, thinking THIS is what being a ten year old boy is. THIS is what my son should be doing. Ten year old boys swing on rope swings and jump into lakes, they ride bikes all over the neighborhood in the summer, they run around in the woods, they play sports. They play hard. It's not fair that my son has to miss out on this part of childhood. A friend of mine with two boys with Duchenne around Griffin's age recently shared how hard spring is for her family. All of her sons' friends are out gearing up for spring sports, breaking out the bicycles that have been trapped in the garage all winter, and just generally running around outside all day, as all kids do in the spring. Childhood as it is meant to be, the kind we are all nostalgic for as adults. Our boys don't get to have that. It seems insignificant in the face of all of the difficulties ahead, but in some ways, this is the hardest part, the missing out on life.
Griffin's childhood has contained a lot of normalcy so far, and I am so grateful for that. But he is ten, and at an age when our uninvited genetic guest begins to start taking a bit more from him as the months pass. It's still easy to forget some days that this is a serious condition. Our usual focus is on how it impacts his daily life now, and so far it is mostly just something that keeps him in the dugout keeping score instead of batting and running the bases during baseball season (note: I wrote this sentence at the start of baseball season. For the past 3 years, he has played T-ball and "machine pitch," which is our local league's option for those who need to graduate from T-ball but are not quite ready for "real" baseball. This year, he moved on to the minors team. At the time I wrote this, we were preparing him to learn to keep score so that he could practice with the team, but sit out the games. He went along with this plan, but as the season started he decided he wanted to play, and so he did. He never hit the ball, but he was one of the team just like everyone else and he was accepted and treated as such. He went up to bat, he hustled to the base when he was walked, and fielded the ball when it came to him, and despite my fears, I don't remember a time when it felt like anyone was making fun of him or treating him like he wasn't an equal member of the team). However, almost daily my Facebook feed contains at least one announcement of the passing of a child with Duchenne. Today (literally, I mean today, this happened today to a family) it is a fourteen year old. Sometimes it is a 23 year old, a 31 year old, a seventeen year old, a twelve year old.
A ten year old.
All of these boys and men were people with lives, hopes and dreams, with loving parents, siblings, grandparents, and friends whose hearts are forever broken. There is no way to predict when it will be our turn, or when it will be one of his buddies, one of the families we have come to know and love. And I can't begin to imagine how I will cope. It sucks. So I try not to think about it. It's not denial, it's just how I get through the day.
Recently, he had an echocardiogram that showed that Duchenne is starting to take its toll on his most important muscle, his heart. His heart is still functioning in the low normal range, but it has decreased a fair amount since his last scan. This isn't unexpected, but still hard to process as a parent when it actually happens. As parents, we always hope our child will be the exception. He started a new medication for his heart as a result of this information, and all we can do now is hope that it has remained stable when we check it again in six months.
The day after Griffin had his echo, I had a cardiac MRI. This is becoming standard of care for carrier mothers, since often the signs of cardiomyopathy don't show up on the echocardiograms of carriers until long after they start. Outcomes are better when an MRI is done to check for scarring before there are symptoms. "Outcomes are better." Code for "it takes longer to kill you." My MRI did in fact show a small area of scarring. My cardiologist reviewed the notes from the MRI and did an echocardiogram. He could see that the part of my heart where the scarring was detected is not pumping the way the rest of my heart is. He showed it to me. I could see it. I watched on the screen as most of my heart pumped merrily along, but there was one spot that didn't move. Kinda creepy. I'll be starting a heart med soon too.
Lest you start to think we are just moping about over here, feeling sorry for ourselves and our broken hearts, I can assure you this is not the case. Griffin wouldn't have it. We literally drove out of the hospital parking lot the day of these less than awesome cardiac appointments and went straight to an adaptive ski weekend. Sometimes at bedtime, he is struck with the need to ask questions or air concerns he has about his condition. A few weeks ago, during a whispered conversation in the dark of his bedroom with Brian about how he wishes he could do some of the physical activities he sees his friends doing, because it looks so fun, Brian said something to the effect of "it must be hard not to feel sad that you can't." Apparently taken aback by this suggestion, Griffin responded loudly "No! I never feel sorry for myself. I don't feel sorry for myself AT ALL!" And if he doesn't, how can we?
Today, Griffin is completing the screening process for a new clinical trial. This time, it is phase 3, which means the drug has already been shown to be effective, and the company is now doing a larger trial to prove this efficacy and fine tune the dosing before attempting to have it approved by the FDA and made commercially available. It isn't a cure, but it will help, and possibly eventually be an alternative to the side-effect-laden steroids that are our only course of treatment currently.
After Griffin's last clinic visit in February, we decided as a family to see what we could do to help slow or stop the steroid-induced weight gain Griffin has experienced. Getting a handle on this side effect now is important, since as his body slows down and he burns fewer calories, it is going to get harder and harder for us to keep him at a healthy weight. It's also a vicious compounding cycle...the heavier he gets, the harder it is for his muscles to move his body, and the more he slows down. We found out that sugar can cause insulin spikes in people on long term steroids, which causes weight gain. We decided to try cutting out processed and added sugars from our diet, which would be good for all of us. I was a bit skeptical it would make a difference. After a few weeks it looked like he might be a bit slimmer. We noticed his shirts seemed to fit looser. I still thought maybe just wishful thinking on our part. He tried on some snow pants we had stored away because he couldn't zip them anymore. They zipped. He also seems to have more energy, and he is moving faster and more smoothly. A few weeks ago, at his first visit for the clinical trial he is finishing screening for today, I tried to look nonchalant as I peeked over the nurse's shoulder while he stood on the scale. I whipped out my phone and quickly converted the kilograms that popped up on the screen into pounds. He has lost almost 8 pounds! This may not seem like a big deal, but I am elated that we have found a way to keep this most challenging side effect at bay. And as it turns out, avoiding sugar isn't as much of a sacrifice as I had expected, and not so hard to do with a little research, label reading and planning.
Now it is summer, which for Griffin means "swimming season." For children with Duchenne, the water can be a great equalizer. While it slows the rest of us down, the support and buoyancy the water gives him makes it easier for him to move. He spends days at a time in the water, flipping, somersaulting, doing handstands and all of the other things he can't do on dry land. The superpower he covets most is the ability to breathe underwater, because then, he says, he could just live in it. Instead of focusing on what he can't do, he just commits himself entirely to the things that he CAN do and continues to surprise us regularly. We have told him that we will help him find a way to do anything he wants to do, even if it is in an adapted way or a different way. And he is keeping us to our word. Below is a memorable moment from last "swimming season" when we found some friends willing to help us help him "do anything he wants." Who said kids with Duchenne can't wake surf?
