Alex arched his eyebrows though his eyes remained closed. His mouth subconsciously opened and slow-chewed imaginary food and his tongue announced its dryness. Clinging to sleep, Alex lay caught between a foggy world of comfort and reality. You could almost see the unwelcome tug-of-war between awakening and sleeping, the latter gradually winning this battle. I smiled and watched this beautiful soul stir and then return to dreamland. His eyebrows relaxed and his chewing ceased. I then waited a moment longer before adding a cold dish of parental sarcasm certain to jump-start his day. My tone reflected the playful smile spread across my face.
“Time to wake up, Dude! Today’s the big daaaay!” I let the words sink in and then, seconds later, watched his eyes fly open in panic.
If Alex had his way he’d sleep well past noon, wake at his leisure, and then live a life he enjoys in his small bedroom. He would check for messages on his cell phone, watch his favorite shows on television, and play his favorite video games on his PlayStation 4. He would call or text family or friends and check to see how they are doing. He would direct message and Snapchat with complete strangers, hopeful they would become his friend. Through it all, he would smile, laugh out loud, and seize every moment to stay connected while he lay in bed with lower back pain. He would continue such behavior well into the night/morning and then do it all again the next day and every day.
Amazingly, he never complains.
Say what you will about his lifestyle, but Alex has built a comfortable world around himself under difficult and trying circumstances. He’s built such a world while living with Duchenne Muscular Dystrophy, a horribly cruel disease that progressively robs him of mobility and independence. It’s a world within which he has learned to thrive despite his chronic pain and complete dependence on others for everything. Seriously, everything. It’s a life he’s built despite the loneliness of seeing and hearing a world move on without him. It’s a life he’s built despite having to ask to simply be moved or adjusted or scratched. It’s also a life he has accepted, thankfully. Despite the frustrations of living with this disease, in an isolating pandemic no less, Alex has built a beautiful world within and around himself that he enjoys. In this world, you’ll find happiness, laughter, and love…for anyone.
We should all take note.
While I stood bedside, Alex’s eyes shuttered hard, his jaw tightened, and his brow furrowed, all in hopes my words were just a dream or that I would just go away. Predictably, Alex peaked one eye open only to find me still standing alongside and smiling as only a teasing Dad can. Between the rhythmic pulses of his nasal Bi-PAP machine, he muttered. “Noooooooo…Do we have to?...I don’t want to go!” The curious eye then closed again, tightly, and his head shook rapidly to escape his reality. He then frowned and offered an alternative. “Can’t we just do a Zoom call?”
So began our day, a few weeks back, of doctor consultations in the Duchenne Muscular Dystrophy Clinic at Cincinnati Children’s Hospital Medical Center. We have known these doctors for years (decades actually) and many have become like family. Each visit, they welcome Alex with smiles and hugs. They encourage and support him as he lives with the beast. They poke, prod, and scan Alex to assess Duchenne’s unforgiving and certain advance, and offer guidance for extended health and tolerance. Some visits, the news is good. Others, not so much.
Today, at age 22, things are becoming real for Alex in his battle with Duchenne. His mobility is generally limited to only his right hand and barely his left arm. He lives with constant lower back discomfort. He cannot sit upright beyond a 45-degree angle. He spends his days and nights in bed. He eats in bed. He “showers” in bed. He toilets in bed. He uses a Bi-PAP ventilator when sleeping and sip ventilation when awake. When he does venture into his wheelchair, his hand slips uncontrollably from the joystick, often leaving him stranded until help arrives.
Through it all, he smiles.
Neither of us is ready for the hard conversations, the hard truths of Duchenne. None of us want to face the reality of life with Duchenne in all its splendid glory. As far as we’re concerned, this disease can suck it. I’m serious! Duchenne is cruel, heartless, and relentless. It’s unforgiving and could care less what you think of it. Through the years, we have learned the only way to cope with the beast is to live it one day at a time (often one moment at a time) and simply do the best we can. We don’t want to glimpse too far into the future if we can help it. We know what’s coming and don’t need reminders. Likewise, we don’t want to stare too long into the past, seeing mobility and independence long since vanished. Most importantly, we don’t want to lose Alex’s joy for life…ever. THAT mentality sustains him! THAT blessing shines powerful and therapeutic for him in its own right. It keeps a smile on Alex’s face and laughter in his belly. It makes each day bearable as they grow harder and darker with time. As we see it, nothing is more valuable in our battle with Duchenne. Nothing!
After a sausage breakfast sandwich, vitamin water, and an assortment of pain medications, we readied Alex for the big day. We toileted, dressed, and groomed him for his second time out of bed in nearly eight months. We lightly applied cologne to impress his nurses and then gently hoisted Alex from his bed. The lift was successful as Alex reported no significant pain. He nodded his readiness to roll across the floor and then lower into his wheelchair that waited in the next room.
With renewed confidence for the day, Alex rolled outside and into our accessible minivan. He chatted continuously, as he is prone to do, while I secured his wheelchair with tie-downs. He thanked me for positioning his cell phone in his right hand so he could listen to his favorite songs on Spotify. I buckled my seatbelt and looked at him via the rearview mirror. I then smiled and told him how proud I was he was out and about. It had been a while. Just like old times – Alex and Dad on the road again. Times like that make you forget about Duchenne. Times like that allow you to feel freedom from the Duchenne.
We should have known better.
With Duchenne, the odds are perpetually against you. You learn that if things seem too good to be true, they probably are. As a result, you learn to focus on the small sphere around you just to get through the day, the moment. Back pain-free? Just wait. Pooping regularly? Just wait. Friends calling? Just wait. Online friend seems nice? Just wait. Generally healthy? Just wait. With realities like these, it’s a wonder and simply amazing that Alex stays so positive.
I often think about others living with Duchenne, those both younger and older. I’ll read about when mobility failed them at a young age, how they fell or stopped walking altogether, and reminisce the sorrow and resolve that follows. I’ll read of lives changed with the first power wheelchair, Hoyer lift, and shower chair, and smile with shared remembrance of maintaining at least some semblance of normality. I’ll read of awkward bed transfers, toilet transfers, and annoying and unfair public inaccessibility, and snort with frustrations that things should not be so difficult. I’ll read of shrinking social circles, the loss of friends, and the hard life questions and then nod with an understanding of the hidden pains doctors somehow fail to fully grasp. In my mind, I’ll whisper support to those families that I know it’s not easy, take it one day at a time, or know you’re not alone. I’ll also sigh and think to myself, you ain’t seen nothing yet.
Then, I’ll consider those more experienced with Duchenne, those living with the beast well into their adult life and currently much farther down the road than us. I’ll think of them making the most of their life despite their increasingly hard circumstances, some I cannot even fathom. I’ll silently toast them for their determination and strength while living with Duchenne. I’ll idolize their parents for their strength to keep pushing and support their sons. I’ll also send prayers when the going gets tough and their hospital stays extend beyond weeks. I’ll then think of them saying to me what are YOU complaining about? YOU haven’t seen anything yet. You think it’s tough now? Just wait until your third or fourth hospital stay with pneumonia. Just wait until they intubate, trach, or install a g-tube into your son. Just wait until his heart or lungs struggle to function. Just wait until….
Sigh. Our future.
We parked in P3 and rode an elevator to the Duchenne Clinic in Building C4. We looked forward to seeing our long-time Cardiology check-in friend, Kenny-Benny. We knew his welcoming face would kick off the day for Alex and help him forget about his back pain and the long day of consultations. But, as if a sign of things to come, Kenny-Benny’s glass office was vacant. His rolling chair sat empty. Alex grimaced in disappointment as if he knew the day wouldn’t be the same.
In our windowless Duchenne Clinic room, we waited as we have done twice per year for much of the past TWENTY years. We chatted about the people we’d see and what they would say. I performed my familiar examination routine as if I was each of Alex’s doctors rolled into one. We did our best to pass the time.
Before long, familiar faces filled our room. Courtney, the ever-smiling and enthusiastic Neurology nurse started our afternoon with it’s-so-good-to-see-you-again’s, high-fives, and hugs. She inhaled deeply alongside Alex, pulled back, and then smiled sideways.
“Whoa, don’t you smell good!” she complimented his scented aroma. Alex beamed with pride and shot me a thankful glance.
Beth, the always-friendly and loving nurse from Cardiology, followed and explained which doctors would visit and when. Before we were ready, the race had begun as our door revolved in rapid succession with visits from Endocrinology, Respiratory Therapy, Neurology, Palliative Care, Rehabilitation, Physical Therapy, Cardiology, Pulmonary, Nutrition, Social, and Psychology - an all-too-familiar cast to an all-too-familiar and exhausting day. Cincinnati Children’s runs a wonderful and thorough clinic where they battle Duchenne with as many resources as possible because this relentless disease affects everything.
Looking back, the first signs of our changing reality surfaced when Palliative Care entered the room. Friendly as ever, the Palliative doctor seemed clinical with Alex’s pain management and abrupt with her continued plan for treatment. “Okay,” she said. “It seems like you are managing your pain well. Let’s stay on the same path with our current prescriptions and we’ll see you in six months.”
This was all well-and-good and well-received, but….wasn’t there an elephant in the room? Didn’t Palliative Care decide to end Alex’s in-home care this past winter (see WITH ALL THE HEADWINDS - david click (davidlclick.com))? Didn’t that topic need at least some attention or discussion?
“Palliative manages Starshine, right?” I pressed the obvious. (Note: Starshine is a home-based service that helps patients who are facing debilitating, life-limiting and terminal illnesses - and their families. Alex had been in this wonderful program from November 2019 - January 2021).
She nodded, though a bit confused as to why I asked.
“Can we talk about why Palliative decided to terminate Starshine’s visits with Alex?”
“Umm, okay,” she replied with confusion. You could tell Starshine was not on her agenda, though should have been.
I looked to Alex to make sure he was okay with the topic. He nodded.
“First off, I would like to say Starshine has been absolutely wonderful for Alex. They visited every week for over a year. They…”
I rattled off all Starshine had provided including nursing care, music therapy, child life therapy, holistic therapy, social work, spiritual guidance, and physical therapy. I specifically named incredible individuals who we will never forget. They came each week with smiling faces, laughter, and friendship. They treated Alex with kindness and humanity. They talked about anything and everything with a social young man who lay immobile in bed. They administered weekly testosterone injections. They became the highlight of Alex’s week within an isolating pandemic. And then, last January, the relationship abruptly ended.
“So, we’d just like to understand. Why did Palliative Care terminate Starshine for Alex?”
The doctor hesitated, and then reiterated reasons that had been addressed over the phone last January. She said Alex is doing well and they have patients with more pressing needs than his. She smiled at Alex and patted his knee as if to indicate how wonderful he is doing. Alex didn’t return the smile as he sat uncomfortably in his power wheelchair. He loved Starshine! He wanted them to resume their in-home visits. He tilted his seat back to relieve pressure and waited for her response. She then explained again that because Alex chose to stay in bed and not get in his wheelchair, Starshine’s services were no longer needed.
To this day, that reasoning confounds us.
“If I may offer a perspective from the front lines of life with Duchenne. Starshine visits were more than just physical therapy. They were invaluable to Alex and our family. They brought him joy, laughter, and social connection when he needed it most. They helped to sustain a young man who craves social connection. They provided a valuable outlet for his outgoing personality. They helped to maintain the smile on his face!”
The doctor nodded her appreciation.
“You must understand life with Duchenne,” I continued. “It’s painful, not only physically but emotionally. It’s socially isolating, especially for a young man whose friends have moved on into college, jobs, or wherever. It’s frustrating because he cannot do anything for himself other than share the joy in his heart. This past year, Alex’s social connection to the world has been severed. Starshine helped to fill that void. That, in itself, is priceless! Can we not restart Starshine to help Alex feel connected, to help him feel a part of the world? As I’m sure you are aware, feeling connected goes a very long way towards overall health.”
The doctor seemed perplexed as to why we would ask such questions and make such statements. “I’ll pass along your concerns to the team, but I don’t think the program will be restarted for Alex at this time. There is really nothing more I can do.”
“Why not?” I challenged.
“Well, like I said, because Alex prefers to stay in bed. That is his choice and we respect that. As a result, our role to support physical therapies so he can get back into his wheelchair are no longer needed. For now, Palliative’s role moving forward will be pain management as we just discussed.”
They don’t get it. It seems as if Alex has been abandoned and services to help him live with Duchenne are only contingent on him getting back into his wheelchair. It’s as if his overall health and humanity are defined solely by mobility.
“If you don’t mind me saying, just because Alex prefers to stay in bed does not mean the services Starshine provides were not needed! He has nobody besides family! Talking with the nurses and the various therapists were the highlights of his week! He NEEDS that social connection! It goes a very long way!”
“I hear you. But, the team has made its decision. We are not in the business to provide social connections to patients. We provide supportive services to get the patient back into the world. Because Alex chooses not to get into his wheelchair, our services are no longer needed.” You could see her becoming frustrated with us.
We sat dumbfounded and equally frustrated.
Next, Dr. Wilmot from Cardiology walked into the room, this time accompanied by Dr. Sawnani from Pulmonary. Dr. Wilmot kicked off the discussion saying that Alex’s heart was doing well, all things considered. We mentioned a few episodes of palpitations that had startled us, but Dr. Wilmot was not overly concerned. Overall, Alex’s heart is strong, he said. He listened to Alex’s heartbeat and backed away pleased. “We need you to continue your current prescriptions and report back to us when you feel your heart palpitating again. Practice deep breathing to help calm any future episodes.”
We thanked him.
The tide then turned with Dr. Sawnani, Alex’s lead Pulmonologist, who had been sitting quietly in the room listening to Dr. Wilmot’s assessment and watching Alex breathe and speak. Before even listening to Alex’s lungs, as he typically starts his physical exam, Dr. Sawnani turned squarely to Alex.
“Alex,” he sighed without emotion. “How are you feeling?”
Alex looked confused with the question and replied. “Good, I guess.” He didn’t understand why one of his favorite all-time doctors was suddenly so serious. Where was the laughter? Where was the smile? Don’t you want to tease me about my search for a girlfriend?
“That is good to hear.” Dr. Sawnani sighed again and then clenched his lips and furrowed his brow, further confusing Alex. He then cut to the chase. “Let me be blunt. You cannot go on living as you do.”
The comment stunned Alex. His smile immediately vanished. His feet bounced with nervousness. His eyes glanced to me for support. His trademark positive demeanor all but disappeared as the doctor he most respects disapproved of the comfortable lifestyle he had built. You could almost see the blood drain from Alex’s face as he dared to ask.
“What do you mean?”
To be continued...