Our most recent struggle with Duchenne began innocently enough. Alex’s doctors had called early on a Monday afternoon to express their critical and impatient need to see him and assess his health. They knew Alex’s back had been causing him serious problems for years. They knew Alex’s pain and anxiety had confined him to bed for the last five months. Add in vital importance to assess Alex’s heart and lungs, and his doctors’ reasoning was solid.
They said we could not delay. They had a bed available in the Transitional Care Center (TCC) at Cincinnati Children’s Hospital and we needed to act before it would be filled. Flu season would start any day now, they said, and a hospital stay then would be dangerous for Alex because of his weakened pulmonary condition. We considered our options (though knew the answer) while they awaited a response. After some discussion, we agreed.
“Okay,” they said with relief and confidence. “Here’s how it’s going to happen…”
Upon hanging up the phone, I wondered if we had done the right thing. Although Alex lay in bed, he was comfortable. Although he has had terrible back pain all summer and fall, the pain had been manageable for the most part. Reclined, Alex was happy…very happy! He smiled often and laughed frequently. Like always, he enjoyed movies, video games, and visits from friends. We enjoyed our time together as a family in the cozy comforts of his second floor bedroom. What more could we ask? Why rock the boat? Was it really necessary to disrupt such comfort?
However, we also knew the importance of getting him out of bed and back into the real world. Alex is a social kid, and needs connections beyond Mom and Dad. He loves movies, restaurants, and bookstores. He relishes communication with anyone, anywhere, anytime. He befriends complete strangers and compliments everyone he meets whether they notice him roll alongside or not. He pets every dog he meets, without exception. He forms a fist to bump for everyone who takes the time. If you engage, you’ll be friends for life. Seriously.
We also knew that for Alex’s doctors to manage his chronic pain and anxiety, they he needed to see him in person. They could not safely prescribe medications without a direct evaluation of Alex’s overall health. Likewise, we knew the importance of monitoring Duchenne’s progression and adjustments to health care as needed, especially for his heart and lungs. Thus, we knew a visit to Cincinnati Children’s was vital for many reasons.
But that smile! Why pull it from his face?
We knew a trip to 3333 Burnett Avenue would certainly introduce logistical complications. Because of Alex’s severe back pain, he could not sit in his wheelchair and, hence, could not travel as we normally do in our accessible van. Thus, he needed ambulance transport on a gurney to protect his back. Because Alex’s bedroom was on the second floor of our home, he would need to be carried down the stairs on a stretcher (note: because he could not sit in his wheelchair, we could not use our elevator). This, we knew, would be awkward even if performed by seasoned EMTs, because Alex is a big kid.
After hanging up with Alex’s doctors, I imagined how the day would unfold. I visualized calling 911 as they instructed, and explaining our situation. I imagined hearing the approaching sirens and seeing the ambulance’s strange arrival to our home. I pictured EMTs lifting Alex onto a stretcher, carrying him carefully down the stairs, and then placing him onto a gurney. I imagined Alex smiling, comfortable, and chatting with each and every EMT as they rolled him into a waiting ambulance. I like your mustache! I could hear Alex say. I’m a big Star Wars fan! Do you like Star Wars? I’m sure he would ask in hopes of engaging in intergalactic conversation. I saw myself needlessly reassuring him of his care as they closed the ambulance doors. I envisioned following the ambulance on the 30-minute drive to Cincinnati Children’s. I imagined an overall successful trip where we could address his pain, get him back to using his wheelchair, and get an important update on his health.
But first, I had to break the news.
“Hey Bud, today’s the day,” I whispered to Alex about an hour after speaking with his doctors. He was in a deep sleep and didn’t immediately respond, so I gently shook his shoulder and then repeated my unwelcome announcement over the rhythmic sound of his pulsing BiPAP machine. Alex’s eyes opened with disorientation that transitioned to acute awareness.
“Nuh-uh,” he voiced in a quiet yet nasally tone as his eyes widened.
I reluctantly nodded the reality and grimaced with understanding. Alex’s resistance was immediate.
“Nooooooo,” he pleaded. “I’m not ready!” His brow furrowed and his head shook in quick bursts of denial, each one hopeful to stop the plan that would begin right after lunch and after taking his prescribed pain meds for the trip. Tears soon welled within his eyes, crested, and then pushed down his cheeks as he mounted his resistance. Today was the day he feared, though he knew it would come.
He knew his doctors needed to see him. He knew Mark from Social Work called every week to help with Alex’s anxiety. He knew Beth from Cardiology phoned to voice her support for a visit. He knew Dr. Sawnani needed to see him to assess his lungs and breathing. He knew Michelle in Palliative care called often to check on his pain.
But, it seemed, we always found some reason to postpone – Alex just wasn’t ready or we had schedule conflicts. The Friday before, we postponed a Children’s visit because we had a house-full of family visiting from out of town. The week prior we postponed because Alex was just not ready. Before then, the same reason. Although, it seemed there was never a good time, a pattern was obvious to all.
Now, finally, a plan had been set in motion.
Alex was to go through emergency admittance and then move to the TCC for an inpatient stay and evaluation lasting up to a week. There, he could rest comfortably while doctors from neurology, pulmonary, cardiology, endocrinology, rehabilitation, physical therapy, social work, psychology, psychiatry, and palliative care could visit, evaluate him, and provide a care plan moving forward. There, Alex could get his x-rays, blood tests, CO2 monitoring, zoledronic acid infusion, EKG, Holter monitoring, and cardiac MRI. There, doctors could evaluate Alex’s current state of health and develop a plan for his comfort and well-being with an ultimate goal of getting him out of bed.
Still, Alex’s worry compounded. Although he was in pain, he hoped the doctors would, instead, come to him. Why can’t they come here? He cried. Don’t they know I can’t get out of bed? He pleaded. Don’t they know how much my back hurts? He begged. But now, with a plan in motion and squarely beyond his control, his eyes expressed fear as if he knew the outcome.
As we would soon learn, his fears were not unjustified.
His first call for help came when we heard the sirens echoing in the distance. It had only been a few minutes since I hung up the phone when emergency sirens first penetrated our walls and then grew louder with each passing second. Alex looked at me in desperation. Noooooo, he expressed with watery eyes. Not today! Not now! I’m not ready! I tried to lighten the mood and tell him not to worry and that we needed to do this.
“Dude, we can’t wait any longer,” I shrugged as if everything was out of my control. The sirens grew louder as they entered our neighborhood. I could see Alex tense up.
“Dad, pleeeeease!” He begged as fear gripped him hard and tears fell.
I told him it was for the best and then held his hands. We maintained eye contact a while longer before I told him I needed to go outside and meet the ambulance. “We got this,” I told him. “You can do it!” I encouraged. I squeezed his hands and then walked out of his bedroom and down the stairs. I unsuccessfully commanded our dogs to stop barking as I stepped outside to meet the first-responders. As I briefed the EMTs, I imagined Alex waiting upstairs alone. I could feel his anxiety. I could sense fear.
Moments later, two EMTs stood with me at Alex’s bedside and evaluated the challenge laying before them. As Alex chatted nervously, they considered how best to move an immobile 200+-lb body out of a second floor bedroom, down turning stairs, and then outside to a waiting gurney and ambulance. They realized their limitation and radioed for assistance, leading to the arrival of a hook-and-ladder fire truck and another fire vehicle along our now crowded curbside. Concerned neighbors stood outside their homes. The entire scene seemed overkill, but there was really no other way to get him to the hospital.
Alex nodded to me and then held his breath as five EMTs rolled him onto a stretcher. The roll was rough and elicited Alex’s first cries of pain. “Ow, ow, ow, ow!” He warned and then pleaded for them to stop. He asked to be rolled back as his back pain intensified. They continued as if they had a job to do. Within seconds they had him strapped onto the stretcher and then lifted on the count of three.
Alex looked to me in panic. Everything was moving way too fast for him. I reassured him he will be fine.
Alex begged the EMTs to take him back to his bedroom. He feared what was to come. He turned his head again to me, but our visual connection fell apart amongst the crowd of five EMTs carrying him on a stretcher. As they twisted around corners and carried him down the stairs feet-first, he screamed in pain that his back was hurting and that his already-turned-inward ankles were breaking against the stretcher due to gravity and steepness of the stairs. Alex pleaded for them to readjust his ankles, but they continued their awkward, step-by-step, march down the stairs. The descent seemed an eternity, as did Alex’s screams of pain and cries for help with each step. I stood helpless and watching from the base of the stairs. Our now silent dogs stood by watching, too, as their concern for Alex grew.
Once outside, they placed Alex on the concrete sidewalk to readjust their grip. He promptly screamed in pain that his back was breaking against the hard concrete. The EMTs promptly lifted the stretcher and placed Alex on the gurney. Alex looked to me with tears and pleaded. “Can we go back, Dad? Pleeeassse! It hurts! It hurts! What if my back completely breaks on the ride?” I assured him he was in good hands with the ambulance crew and they would drive safely. I set up Alex’s sip ventilation for him to use along the way, if he needed. I gave him a full hug, kissed his forehead, and then told him how proud I was that he was doing this. I told him I know it is hard, but he is doing it! Alex sighed deeply, gave a quick nod, and then flashed a proud half-smile between his tears before I stepped out of the ambulance.
While the ambulance pulled away, my mind raced for what had transpired and for the pain our son had endured. Should we have just stayed home? Should we have let Alex’s back heal in bed? Should we have pushed back against the doctor’s insistence to see him? What more did they need to know than they already knew? Duchenne sucks. Period! Why put Alex through such pain to confirm that understanding? What would we gain?
My emotions were a mess watching the ambulance drive away, and I could barely speak with concerned neighbors who stepped forward and expressed their support. I dutifully answered their questions to what was happening, though stood eager to disengage and jump in our van before my tears crested. Once in the van and driving to catch up with the ambulance, they did.
What had we done? Hours before our son slept peacefully. Hours before he had comfort. Our little family had normality. Now, we had pain, hard pain…and the week had only just begun.
God, how I hate this disease.
TO BE CONTINUED