Alex sat dumbfounded at the suggestion, as did I. We couldn’t understand why Dr. Sawnani, Alex’s Pulmonologist for the past 15 years, disapproved of the world Alex had created while living with Duchenne Muscular Dystrophy. We couldn’t understand why Dr. Wilmot, Alex’s Cardiologist, sat alongside, silent and equally serious.
Because of muscle pain and spinal compression fractures throughout Alex’s thoracic and lumbar spine, movement for Alex is painful. Heck, resting is often uncomfortable as well. Lifting, turning, or moving Alex requires him to have grit, determination, and tolerance. Hoisting is tedious and delicate. Sitting in his power wheelchair is awkward, uncomfortable, and borderline absurd at times. When you find a position of comfort, you stick with it as long as possible and make do.
So, it’s no wonder Alex (22) has created a comfortable world within his small bedroom. It’s no wonder he chooses to stay in bed. He eats, sleeps, and everything else you can imagine…on his back…in bed…all day, every day, and has for most of the past several years. He watches movies, sit-coms, and Netflix series in bed. He plays video games alone or with anyone who will join him. He follows and chats with YouTube celebrities on his touch-pad computer. He texts or calls friends and family whenever possible. He talks up a storm with whomever visits. Through it all, he smiles, laughs, and loves with anyone at any time, rain or shine, good days or bad. Often, you’ll hear him laughing while watching a comedy show he’s often seen many times before. It’s a beautiful sound, by the way, and fills our hearts with joy.
God love him.
Alex’s lifestyle is not ideal by any means. Given a choice, he would be out and about and talking with everyone and anyone about anything. Seriously! He is an extremely social being, more than anyone I have ever known. He craves connection and, when he doesn’t have it, he makes do with what he has to entertain himself. I have no doubt he’d love to get into his wheelchair and go to movies, a restaurant, or a bookstore. I’m certain he’d love to visit friends or take a road trip to the next Star Wars convention wherever it may be held. But, he can’t. So, he doesn’t. He simply makes the most of a difficult situation while living with Duchenne, a horribly cruel, progressive, and lethal disease with no cure. Amazingly, he never complains.
Put yourself in his shoes…during an isolating pandemic, no less.
Really, just try.
In our windowless Duchenne clinic room, Alex sat uncomfortably in his power wheelchair while Dr. Sawnani straddled a rolling stool and waited for Alex to respond. It seemed as if Dr. Sawnani had tired of our approach to living with Duchenne. It seemed Dr. Sawnani had grown frustrated we had not followed his recommendation from our last clinic visit six months prior. Then, he told Alex to get out of bed and back into the world.
Alex fidgeted in his wheelchair and sought clarification. “I thought I was doing okay!” Hearing no immediate reply, he dared to ask. “Am I not doing okay?”
Typically one of the most compassionate and caring doctors we know, Dr. Sawnani’s clinical side took over in his response. He described how staying in bed severely threatened Alex’s physical health. He described how it compressed Alex’s lungs and diaphragm, making it harder to breathe, eat, and swallow. He detailed how the supine lifestyle Alex enjoyed will inevitably lead to choking as well as infected bed sores. He shared his belief that such lifestyle socially isolates and threatens the spirit, leading to depression and ultimately resignation to live. He then twisted the dagger by telling Alex - the most joyful soul I have ever known - that Alex is missing out on life and the world around him.
We were stunned. Alex did not know what to say. Heck, I did not know what to say! Sitting before us was the doctor we had most respected, the doctor who had generally assumed leadership of Alex’s overall care, telling Alex that he was doing it all wrong.
Thankfully, Alex held strong. “But I enjoy being in my bedroom. I enjoy watching movies and playing games.” Hearing and seeing no reply, Alex rebutted. His words reflected his confusion and worry. “You make me feel that if I stay in bed my life has no value!”
Dr. Sawnani just stared at Alex without expression and rephrased, “Alex, it’s really no way to live.”
I could not believe my ears. How dare they! How dare the doctors cast judgement on how Alex chooses to live his life with Duchenne. They are not the ones living with this damn disease 24/7/365! They can go home at the end of the day. They can escape Duchenne from time to time. I realize they often sees the dark side of Duchenne, the side where young men succumb to the inevitable in sometimes harsh and suffocating ways. I realize they are trying to help us avoid such a difficult future. I also realize perhaps they have Alex’s best interest in mind and think such stark and challenging directives are the only way to get our attention.
We get that.
But, for God’s sake, to attack a young man’s spirit!? To tell a young man doing the best he can his lifestyle choice was wrong!? THAT I don’t get.
Living with Duchenne is no picnic. In fact, it sucks, plain and simple. Life and vitality is slowly, steadily, and painfully squeezed from young men while the world moves on around them – while they watch their friends and neighbors live a life they can only dream. How dare anyone – ANYONE - tell someone with Duchenne how to live (note: if anything, it should be the other way around).
Now, I know some of you may agree with the doctors. Some of you may tell us that Alex’s supine lifestyle IS no way to live and needs to change. Some of you may believe Alex needs pushed else his decline hastens. We don’t see it that way, because life with Duchenne is not that simple. Every situation is different. No life is the same. It’s a constant balance between pain and tolerance. Between social, mental, and physical. Between quality and quantity. From our experience, a positive spirit is essential to life with Duchenne on all fronts. I cannot imagine living with Duchenne without it.
“Does this mean you think…,” Alex hesitated, “…I’m going to die?”
Dr. Sawnani allowed the question to linger before pursing his lips. He sighed and then spoke of his experience with Duchenne. “Alex, your current lifestyle will significantly shorten your life, yes.” Dr. Wilmot sat alongside and nodded slowly.
Alex’s eyes flared and he grew visibly worried for his future. “But, I enjoy my room! I enjoy playing games and watching movies! I cannot do anything else anyway! I can’t go anywhere!” His eyes darted between the doctors and then to me.
I jumped into the conversation on Alex’s behalf. “We hear you. Really, we do. But, Alex is happy! He really is. He’s full of joy! Doesn’t that matter while living with Duchenne? Isn’t that important?” Dr. Sawnani sat unimpressed and focused on Alex, seemingly ignoring my attempt to defend Alex’s lifestyle…our lifestyle.
“Alex, this is how I see your life progressing and I’m very serious about this.” He waited until Alex’s eyes were locked with his. “If you stay in bed, your lungs will continue to weaken, your diaphragm will weaken, you will have difficulty swallowing, and you will have difficulty breathing.” After a pause to consider his words, he proceeded. His words were direct and deliberate.
“It will be choking that takes you. Not an acute choking, but one that develops over time. You will not be able to clear your throat the way it should be cleared. You will aspirate. Your lungs will become infected. You will require intubation.” He paused and then added. “It will not be pleasant.”
We were speechless. Alex glanced to me, his eyes now glossy. His chest heaved repeatedly with each shortening breath. His feet bounced with nervousness as you tell he just wanted to escape. I swallowed hard beneath my mask and then broke the silence with another defense.
“You want Alex out of bed so he can sit up in his wheelchair, right?” Dr. Sawnani did not acknowledge my question and continued to bore his eyes into Alex. I opened my hands in demonstration towards my son. “I think you need to understand THIS is his same position in bed! Because of back pain, he cannot sit to ninety degrees as you hope. So, he will always be at risk as you describe…whether in bed or in his wheelchair!”
Dr. Sawnani sat unimpressed and then turned the screw tighter. “Let me ask you, Alex. Do you want to be intubated? Do you want a tube jammed down your throat to save your life?” The graphic description was shocking to hear, let alone visualize. “Because that’s the path you’re on. That is your future if you do not get out of bed.” He then asked a stark alternative. “Or, do you just want to die?”
I couldn’t believe my ears and immediately looked to Alex to see his reaction. Thankfully, he held strong and bravely replied. “No, I want to live!”
With perhaps his first showing of compassion, Dr. Sawnani nodded once emphatically. “Good. I am glad to hear that. I’m telling you this, Alex, because I want you to understand the harshness of intubation. I want you to realize what will happen if you do not get out of bed.”
Alex responded, “I do understand. But, I’m happy in bed! I’m not comfortable in my wheelchair! Don’t you understand that?”
Dr. Sawnani leaned back and crossed his arms. He seemed to grow tired of our perceived obstinance. With a casual flip of his wrist he said, “Then, so be it. Just understand what will happen. You will choke. You will be intubated. It may not happen this year, or next. But, it will happen if you stay in bed.” He uncrossed his arms and leaned forward with quiet seriousness. “Alex, your life will be shortened if you do not get out of bed. It’s as simple as that.”
Alex turned to me and pleaded, “Dad, I thought I was doing good.”
I reassured Alex he was doing fine and that we’re doing the best we could. Silently, I wondered the contrary. Is THIS where we are with Duchenne? Already? Is choking the inevitable next act? ARE we leading Alex down a dangerous path? ARE we doing it all wrong? It’s so hard to know.
This is an example of the tenuous balance of life with Duchenne. If it’s not one thing it’s another. If you stick your finger in the dike one place, a leak develops elsewhere. In our case, If Alex doesn’t sit up straight, doctors tell us his internal organs will be affected. If he sits straight as his doctors want, Alex will be in pain and discomfort. If we manage his pain by increasing the dosage of pain medications, then Alex cannot have a bowel movement for nearly a week (and when he does, it is a painful rock). When we use laxatives to manage the stools, we have a mess on our hands with a 200-lb body that cannot be moved easily. Now, add Dr. Sawnani’s imperative to get out of bed, else...
Sensing tension in the room, Dr. Wilmot added his two-cents. “This is why we were insistent for you to come into clinic. This is why we withheld your meds. We needed to see you, Alex. We want you to understand how important it is for you to get out of bed! We want you to understand the seriousness of your situation.”
“I do, I do! I know how serious it is. Don’t you understand that?” Alex replied with conviction and some tears. “But, my back hurts all the time! I can’t go anywhere because of the pandemic. My friends don’t visit anymore. Nobody calls me. All I have is my bedroom and my family. It hurts to even go into another room. So, why? Why should I leave my bed? I’m really happy there. I enjoy my life! Why must that change?”
I was so damn proud of Alex at that point. Here he was, in the face of not only physical adversity but social and clinical adversity, telling his doctors how HE wants to live, how HE makes the most of his challenging situation. From our perspective, they just don’t get it. Or, perhaps, we don’t get it. It’s so hard to know.
The discussion had reached a standstill, with neither side yielding much. The doctors soon stood, weakly fist-bumped Alex with less enthusiasm than they usually have, and departed. Just like that. Our room was eerily quiet.
As I feared, the next stage was damage control. Alex turned to me with panic and emerging tears.
“Dad, what am I doing wrong? They make me feel like the way I live is all wrong. They make me feel like my life is not worth living.” That last statement grabbed my attention. I needed to nip that in the bud immediately.
“No, no, Bud. They’re not saying that. They’re simply stressing the importance that you need to keep moving.”
“Don’t they think I know that?”
I nodded agreement they must. “I hear you, I do. They’re just saying let’s try to get out of bed every once in a while. They just don’t want you to miss out doing things you enjoy like going to bookstores, restaurants, movies….”
Alex’s eyes widened with betrayal. “So, you agree with them? I thought you understood! Dad, my back will hurt! I’ll be uncomfortable!”
“I know. I know. I understand. Let’s just do what we can. They’re just trying to help.”
Silently, I cursed Duchenne. Again.
Several hours later after consultations with other medical disciplines, our day ended and we were amongst the last to leave the Duchenne clinic. The clinic was like a ghost town. No Sawnani. No Wilmot. No nurse Courtney or Kenny-Benny. Just a few doctors and fellows furiously typing the day’s notes in their glass-enclosed central station. The elevator ride down from Building C4 was quiet, just the two of us. The hospital hallways were spattered with people wearing white coats and tired looks. The prior population of parked cars in the multi-level garage had waned. The familiar industrial fan we seem to always park by still roared in mockery of our imminent departure. We were exhausted, like it seemed every time we visited the hospital these days.
Alex had been beaten down, emotionally and physically. I lifted him higher in his wheelchair as best possible, careful not to hurt his back or stretch his side. He winced but swallowed his all-to-familiar warnings that he usually shares with any movement. He then slowly rolled into the minivan and waited while I secured his wheelchair with the four tie-down locks. I wrapped his right hand around his cell phone and positioned his arm on his lap so he could listen to his favorite songs on the drive home. I then started our minivan and weaved through two levels of parking to daylight…finally. It was a beautiful evening, thank goodness. Alex then quietly shared his thoughts.
“Dad, it’s like my life means nothing. I have no control over my body. I can’t do the things I want. They want me to live as they think I should. My back hurts…” He paused and then added quietly. “What’s the point?”
I spoke to him via the rearview mirror. “Alex, you are doing nothing wrong. Absolutely nothing! I’m so proud of you for telling the doctors how you feel. I’m so proud of you for being the loving and positive person that you are. I know living with Duchenne is hard, harder than anything! But, your outlook to life is absolutely beautiful, Bud. Don’t let anyone, ANYONE, not even the doctors, take that joy away from you! You are perfect just the way you are!”
Alex did not respond, he just stared out the car window to the world zipping by him. Soon, he looked to his lap and tapped his loosely held cell phone. His music started to play. I glanced to him again via the rearview mirror and smiled when we made eye contact. Just like old times…
On the next bump in the road, Alex’s cell phone fell from his loose grip…again. It landed on the floor, face-up, and adjacent to his wheelchair though beyond my backward reach. I groaned in frustration that he could no longer control the playlist, but soon smiled because his music – the music he selected - continued to play from the floor….loud and clear.
And when you really think about it…that’s all that matters.