I first met Andy (all names changed for privacy) after he was admitted to hospice care with Duchenne Muscular Dystrophy, a genetic disease carried recessively on the X chromosome. One in 5,000 males are born affected, the most common and severe type of muscular dystrophy. It has no cure. Andy was first diagnosed when he was in kindergarten. He would fall down more than the other children. Innocent at first, until his weight started to change. He could only walk on the tips of his feet and he fatigued more easily. His body developed scoliosis as compensation for the deterioration of his legs and hips. By fourth grade, he was completely dependent on a wheelchair. He was no longer able to attend junior high school after he became completely bedbound. At age 16, his pediatrician recommended palliative care as comfort became a daily struggle for him.
My team typically assigned patients by geographical location, so each Nurse had a designated area. Andy’s home was in the area of a Nurse who happened to be on vacation the day he was due for his first nurse visit. I was short one patient, so I made him the last stop on my southern route. My job was to assess his comfort and make sure he and his parents understood the principles of his pain medication. I was to review and reiterate the principles explained on admission and then pass on his care when his primary Nurse returned on Monday.
I was not prepared for what I faced when I pulled into the driveway. I was greeted at the door by his mom, Diane. She was cheerful, but you could see fatigue and worry on her face. One turn to the left into the darkened master bedroom where a compacted form lay facing away from me on a queen-sized mattress on the floor. I could only see a tangle of unruly black hair peeking from a blanket. I introduced myself as I walked around so we could see each other face to face.
I had already worked in hospice care for four years and had never seen such an expression of sheer anguish as in this teenager’s sunken eyes. He responded only with a moan. Immediately I asked Diane about his medications. Our team had ordered liquid morphine which was on a bookcase, still in its box. The Admission Nurse had tried to explain its purpose to Andy, but he refused any type of narcotic. He would burst into tears if it was even mentioned.
His sole form of relief came from repositioning. Eagerly I offered to assist Diane to turn Andy to ease pressure from his back and hips. Again Andy moaned and called out “No” as he only trusted his mother for this task.
Not wanting to disrupt their already well-established routine, I stepped aside so I could observe. Diane pulled his Superman cover back, exposing a frail, ghastly, white body. He had dark patches of dried skin on his back and lower legs. His spine obtruded unnaturally against his skin. Diane covered him with a blue washcloth before pulling him forward and upright. Again his gaunt face winced. His entire torso was contorted to the right, his rib cage jutted towards his hip with just a few inches separating them. Diane reached for a second washcloth which she rolled and tucked in for padding.
Andy relaxed at last with the final addition of a regular pillow for support. He was able to talk to me briefly, never smiling. I asked Diane how often he needed repositioning to keep him alleviated. “Every ten minutes,” was her response. Now “comfortable” he would then use his right hand, his only still functioning limb, to check the weather report on his laptop. He was terrified of storms.
This was his normal daily routine. Scattered intervals of sleep and a constant juggle of worry and suffering when awake. Constant paranoia about things beyond his control. It made me ill to see that level of agony, both physical and emotional. After nine years in hospice, I have yet to see it again. I was angered that the healthcare system had failed this individual. His pediatrician was reluctant to prescribe him any type of narcotic due to his young age and fragile health. Andy’s high anxiety made him petrified to try anything new.
Thankfully I was able to convince Andy to trust our team. I told him he didn’t deserve to be in constant torture. I assured him he always had the right to make his own decisions. He agreed to try liquid medication that could be given hourly. His fears were reduced with an anti-anxiety pill. He let the Care Navigator console him. Despite being very private, he allowed the Personal Care Assistant to bathe him in his hospital bed. Our Hospice Physician grew close to his family.
One of my happiest days as a Nurse was the first time he was able to sit upright for 20 pain-free minutes. Gradually he was able to tolerate longer intervals into his new normal. He was able to watch entire episodes of his favorite cartoon, Naruto. He could play Crash Bandicoot on his PlayStation. He no longer obsessed about the next storm, whether it be in the sky or in his own contracted frame.
The greatest metamorphosis was his demeanor. He smiled more and was able to explore his passions. He loved the cooking channel and debating about the existence of aliens with his dad. He anticipated the future with excitement. We saw Andy celebrate two birthdays that no one expected him to see.
Andy’s first major decline was after the death of his beloved dog, Stinky. She had been part of their family since he was three. As an only child, the pair practically grew up together. The overweight Chihuahua Terrier mix, near-blind and missing several teeth, seldom left Andy’s side in her happy 15-year life.
Relentlessly his muscles became weaker, putting more strain on his already exhausted heart. His pulse was always above the normal range. He could only tolerate small meals of soft foods. Difficulty swallowing is another cruel symptom of muscular dystrophy. His weight dropped to a skeletal 50 pounds. His illness had long ago surpassed any possibility that could reverse the devastation of chronic malnutrition. He slept more. He could no longer tolerate sitting up at all. His pain doses were increased so that he could rest comfortably.
I remember touching his forehead while he slept before I turned to leave what would be my last visit. His parents declined to have the On-call Nurse check on them later that night. I assured them I would be back to see them first thing “tomorrow.” Andy died early that morning. I knew he was declining, but planned on seeing him that next morning. He was my fighter after all.
Andy’s life was marred by disease, but his pain didn’t define it. His world was small and quiet, but I was fortunate that it included me. Just like all my hospice patients, he deeply affected me and I’m thankful that I met him. His death was devastating, but was also one of the most rewarding of my life experiences. Forever he will remind me of my commitment to affirming life and serving those who have been touched by a terminal illness.