With Duchenne, you know the hard questions about life and death are coming, you just don’t know when. You know they’re inevitable, you just don’t know to what degree. You envision the moment of their emergence. You imagine the time, place, and general setting of their asking. You imagine yourself rising to the occasion, standing tall, tear-free, and delivering with love, patience, and understanding. You imagine your son listening, nodding, and smiling with acceptance.
With Duchenne, however, we have learned that life rarely happens as you imagine.
The bridge I had feared for years first appeared last October after a fun day watching the Cincinnati Bengals beat the Kansas City Chiefs, when Alex asked me if he would die before the age of twenty (http://www.davidlclick.com/blog/the-bridge). The question caught me off guard and I did my best to answer him honestly. It was a tender moment between father and son (in a dusty parking garage, of all places), as Alex shared his innermost thoughts and concerns. While we drove home that day, I told myself that although the bridge had been crossed, more questions about Duchenne, life, and death were certain to follow.
They didn’t, and life with Duchenne continued onward along its familiar, unwelcome, and unwanted path.
Then, nearly eight months later and after a fun-filled weekend of relatives, a Dave Matthews Band concert, and an Angry Birds movie, the questions returned with a vengeance. It was as if Duchenne had been starved for attention and wanted to renew its havoc in our lives.
Hello, Clicks! Did you miss me?
My cell phone rang at 4:30 a.m. early Sunday morning, waking me from a deep sleep.
“Dad,…I need you! Something…weird…is happening to me!”
I immediately tossed the covers aside and raced into his room. Alex explained that as he played a video game, he could breathe in, but he could not push air back out. With a panicked expression, he described how air would only seep out of his mouth when he tried to exhale! I immediately recalled how Alex’s Pulmonologist, Dr. Sawnani, warned us that Alex’s lung strength continues to slide as does his ability to push and pull air in his lungs. Mixed feelings of concern, worry, fear, and guilt filled my brain as I calmed Alex and then positioned his BiPAP over his face and turned on the air. Once his breathing stabilized, the onslaught of questions began after their long hiatus.
At first, Alex asked me to explain why he had difficulty breathing. But after the technical questions were addressed, Alex quickly zeroed in on his primary and most vital concern.
“Dad, does this mean I am going to die soon?”
Immediately, I was filled with anger though did not show it. Damn this disease! No kid should have to think about dying! No kid should have to worry! Do kids with Duchenne not suffer enough!
“No, Alex, this doesn’t mean you are going to die soon.” I reassured, hopeful the sincere recipe I applied in October would suffice again today.
“Then what does it mean?” he demanded. “Why can’t I breathe normally?”
“Well, Duchenne, unfortunately, affects all muscles….” I began before he interrupted.
“Even my lungs?”
Deep breath. Choose your words wisely. Although Alex has heard Dr. Sawnani speak of breathing concerns before, Alex has been convinced for years that Duchenne only affects his legs and maybe his arms.
“Yes, even your lungs.” I answered honestly.
Say something quick to lessen the impact! NOW!
“But, we can help that by using your BiPAP more frequently.”
Alex expressed immediate concern, his voice sounding nasally as he spoke over the air being pushed into his nose.
“Does that mean I have to wear this stupid thing all the time?”
Hear him, but guide him. “No, but we need to wear it as often as possible when you are in bed…when you watch movies or play games.” Do I explain his likely future with ventilation? Do I share with him the strong possibility of a tracheotomy? .....No, not now. Those discussions will come with time. Focus on what he will understand. Focus on the positive. Focus on the….
“But, if I can’t breathe…will I die?” he cut to the chase. The hard questions were getting harder.
“No, if you need help breathing, doctors have other ways to help you.”
Alex grew silent as he searched my eyes for the slightest expression.
Am I saying the wrong thing? Am I saying too much? Am I causing worry?
“I don’t want that, Dad. I don’t want to be hooked up to a machine and tubes the rest of my life!”
You can do this. Acknowledge. Foster hope, yet prepare him for what will likely be his future, because it is clear he is seeking answers. Be gentle.
“It doesn’t necessarily mean that, Alex.”
“But, boys with Duchenne will need it, right?”
“Yes, some do.”
“Will I?” he pressed.
Although the likelihood is high, I deferred. “Maybe. Maybe not.”
Alex looked towards a window, clearly thinking. I could tell he wanted answers. He looked back to me, directly, and changed his line of questioning.
“Dad, does Duchenne kill?”
“Well, everybody dies of something, Alex. You just never…” You could immediately see his dissatisfaction as he rephrased.
“Will Duchenne kill me?” he asked point blank.
Still searching for a soft landing, I said, “Alex, nobody knows how they will die. We could be killed in an accident, we could be…”
“Dad, CAN Duchenne kill me?” he wanted the truth and would settle for nothing less.
Looking him directly in the eye, I then uttered the hardest, yet simplest, most honest word I ever have had to say in my lifetime.
I immediately wondered if I went too far. Late hours and fatigue tend to expose your core thoughts, no matter how you try to control them. But, I also could see that Alex would not accept dancing. He wanted truth. Still, I needed to qualify my response…and quick, so as not to lose him.
“But, Alex, with Duchenne you never know. Remember my friend, Ian, who I’ve told you about? He has Duchenne and is 34 years old! He’s doing great despite his challenges. He is an author, a digital artist, and has a website! He’s an uncle! In fact, when Ian was your age, he worried about his lungs, too! But that was 17 years ago!”
Alex calmed slightly, but I could tell he was still leery of everything and needed more.
“I also have friends named Kitsune, Ricky, and Paul. They all have Duchenne. You should see all they do! Kitsune is 34. Ricky just turned 35. And Paul is 49! So, you see, Alex, Duchenne affects everyone differently. You’re only 17. You have your whole life ahead of you. Don’t worry about your breathing. We can help that! You can do the things you want to do and live a long time!”
You could see Alex relax a little. You could see him understand and reluctantly release his fear because he considers people in their 30s and 40s to be ancient. But, you can also see his concerns were still there, brewing and processing. The questions continued.
“Does everyone with Duchenne live that long?” He probed.
Again, I resorted to honesty. He deserved as much for living with the monster.
“Well, unfortunately, no. As I said, Duchenne affects everyone differently. Some die young. Some live a long time. You just never know, Bud. You just never know.”
Alex reasoned thankfully. “So, if I wear my BiPAP and take my pills, I’ll live a long time, too, right?”
“Yes, Bud. We must do everything our doctors tell us so we can live a long time!”
Alex then relaxed enough to not outwardly worry any further that night, seemingly satisfied with our discussion. Over the past several days, however, he has asked many follow-up questions about life and death. Each question seems to come with a new level of understanding and awareness. Each question also leads me to believe there will be more.
A difficult aspect of Duchenne is that as Duchenne’s physical destruction advances, your son matures emotionally. As his body weakens, his awareness strengthens. It’s an awkward passing. He no longer will accept measured and tailored explanations typically given to a child. He will no longer accept redirection as a means to change or avoid the subject. As he matures, he wants answers and he wants the truth. He wants you to speak to him as a young adult and no longer as a kid.
You’d want the same at seventeen, wouldn’t you?
As tough as it is to cross the bridge of facing our own mortality, I know his questions will only get deeper as we move forward. This current bridge is long and, from this vantage point, I see many more on the horizon. Strangely, the bridges seem to appear after happy and so-called normal times of life, if there are such things.
As you can imagine, these are difficult conversations to have with your teenaged son.
They are emotional.
They are surreal.
They are life with Duchenne…and they’ve only just begun.
So, about the positive side of this nightmare, because there is one…always…if you open your eyes to it. The breathing scare last weekend has reinforced to Alex that he must do everything he can to combat this unforgiving disease. Alex now wears his BiPAP every night, and often while playing video games or watching a movie. He takes all his prescribed medication without taking his usual “days off” he has been prone to demand. He realizes his sleep schedule may need adjusting, and asks to go to bed “earlier.” Hopefully, these positive behaviors will continue forward as his new routine.
The bridge revisited has also been bonding. We look to each other for support and cherish the many moments we live together, all things we should never take for granted though sometimes do. But, perhaps the most powerful outcome of this latest Duchenne episode has been both emotional and physical, as Alex now frequently asks for a hug before I leave the room. It’s almost like he is worried we may never see each other again.
It goes like this.
“Dad, can I have a hug?”
The question usually comes as I finish whatever parental task and then prepare to leave his room. The question accompanies his hands open and resting by his sides because he cannot lift his arms high off the bed.
I smile and reply, “You bet.”
I then bend down, put my head next to his, our chests together, and position my arms around him and near his hands so his wrists can then curl enough to complete the embrace.
I’m telling you there is no better feeling!
After several long seconds, we part. Alex then looks me directly in the eye and tells me he loves me…
…I smile, place my hand on his chest, and tell him the same.