We have just finished celebrating Griffin's 7th birthday. Every birthday since his diagnosis will be bittersweet; with each year, he is growing a little more mature, becoming more his own person. Like any parent, I feel pride as I watch him grow up. But in the back of my mind, I can't help but also think that each birthday marks the passing of another year, and we have one less year to spend with him. I think we are among a generation of Duchenne parents who can feel less certain about when the final birthday will come. Already, many with Duchenne are living a decade or more longer than their prognosis, as medical advances make it possible to breathe longer and keep hearts beating longer. Just since Griffin's diagnosis two years ago, the landscape has changed. Boys being diagnosed right now are immediately enrolled in clinical trials by their doctors, and told that there is hope and many promising treatments on the horizon. While as parents it can feel like trials are moving at a snail's pace and each day spent unable to do anything to stop the progression of the disease is agonizing, things are actually moving very quickly. But even with all this hope, there are still those who lose their lives as young as the tender age of ten, often due to early cardiomyopathy (for unknown reasons, some develop a fast-moving cardiomyopathy much earlier than typical). We live life expecting that Griffin will not be one of these boys because we have to in order to keep moving forward each day, but there is no way of knowing if he will be or not. That is the hard truth.While we, his parents, grapple with an uncertain future, Griffin continues to be his happy, active self. So far, he has known nothing but improvement. He repeated Kindergarten this year, which has proved to be a very good decision. It has allowed him to catch up with fine motor skills, and move at his own, slow but steady pace. He is having a good year. This is partly because he is really ready to start tackling academics now, and partly because he has had more time for his social skills to mature. But it is also because he is physically stronger now than he was last year. Around the time he was diagnosed two years ago, he began the "honeymoon" stage of increasing strength that can happen with Duchenne. His body's natural growth and development has temporarily overtaken the progression of the disease. He also started corticosteroids last year, and we saw another bump in strength that has so far continued. But the honeymoon can't last forever, and the benefits of the steroids cannot keep pace with the course of the disease for more than a few years. Now, at age 7, he has reached the age where natural history data shows that all children with Duchenne begin to decline, if they haven't already. His 7th birthday is more bittersweet for me than the others have been. It marks the end of an era in which he learned to ride a bike, learned to swim, started running, alternating his feet on stairs, jumping and getting pretty close to being just a regular kid. Last year, I had to go in and talk to his class out of necessity; he was starting to get picked on. The other students didn't understand why he moved slower, why he couldn't keep up with them. This year, I went in around the same time in the fall to talk to the new Kindergarten class, and they hadn't even noticed yet that anything was different about him. I'm not sure how we will explain it to him when he notices the first signs that his muscles are beginning to get weaker instead of stronger.
A few days ago, a photographer from a local newspaper come to our house to take photos for a story the paper is doing on Brian's recent Everest to End Duchenne adventure. While snapping pictures of us outside sledding, he commented that we seemed like a very active family. "We are pretty active," I agreed. After a pause he asked if we felt like we have had to stop being as active as we would like because of Griffin's physical limitations. I told him that I actually think it is the opposite. I suppose it is true that we have had to re-imagine our ideas of what active means, substituting snow tubing for snowboarding, spending a day at the beach instead of hiking up a mountain, or going on a canoe trip on the river instead of surfing in the ocean. But we tend to embrace life more than we did before, and if we have an opportunity for an adventure, we tend to take it. Rarely to we say "nah, I don't feel like it today," because we don't know for how long these experiences will be possible. It can get exhausting, but it is also pretty fun. If Griffin says he wants to have some kind of active outdoor experience, we find a way to do it. I recently sorted through our entire iphoto collection looking for photos and videos to document Griffin's life as background footage for the Everest to End Duchenne documentary that is being created, and it struck me that we had years and years of photos and videos of us doing fun things, going cool places, and having interesting experiences. There are many days when I slip into feeling sorry for myself, but as I was looking through all those photos and videos, I could feel nothing but gratitude for all that is good in our lives.
We are in the process right now of screening for a clinical trial. He just "passed" the blood test to ensure that he meets some of the criteria for participating in the trial. We head off on Griffin's Make a Wish trip to Disney World in a couple weeks (yay, more fun!), and should find out soon after our return if we will be going ahead with "dosing" him. If this trial falls through for any number of complicated reasons, there are many others he qualifies for currently recruiting. Knowing that they are all trials, not treatments, and nothing is guaranteed, it can be somewhat agonizing to decide which to pursue. What if we choose the "wrong" one, the one that turns out not to work at all? What if we choose the one we think is best, and he ends up on the placebo? What if he has some kind of severe side effect? All of the trials involve putting him through a fair amount of poking and prodding, including bloodwork, muscle biopsies, MRIs, ongoing strength assessments, injections, infusions, the list goes on. There are many potential side effects to consider. In all of this though, I have to say that what rises to the surface is the hope that even if the trial drug he takes works just a little bit, maybe it will be enough to stabilize him or slow the course of the disease down long enough to keep him where he is until the next thing comes along, or something is finally approved to treat the disease. Most clinical trials for Duchenne require that participants be 7 years old so that the researchers can be certain that it is the drug, and not the honeymoon phase, causing any increase in strength they observe. I feel fortunate that just as he turns 7, we have the option to get him right into a trial. Maybe, just maybe, 7 will not be the year that marks the beginning of decline for him.
I don't expect a miracle, but in my wildest dreams I imagine him walking up to get his diploma at his high school graduation. Right now, that is where I set my sights. It seems reasonable that within a few years we will have a drug that will work well enough slowing progression that this generation of children will walk longer, be able to brush their own teeth and feed themselves into adulthood, breathe on their own into adulthood. Soon after he was diagnosed, my mom made a comment to the effect of, "he may not have a very long adult life, but we can make sure he has the best childhood a kid can have." I'm not sure what Griffin wished for when he blew out the candles at his 7th birthday party, but my wish for him now, at the end of his 7th year of life, is for many more years of awesome childhood and amazing adventures.
