J Rush Pierce
Lakewood, CO, United States
Chasm Lake, Rocky Mountain National Park, Colorado, USA. Photo taken by the author 27 August 2016.
It must have started some time before, but I was unaware of it on that pleasant September day, hiking in the rocky foothills of northern New Mexico with my daughter. Arriving back at the trailhead by late afternoon, we shucked our packs into my SUV and paused for a drink of water. I sat to remove my dusty boots when she offered what later proved to be an ominous observation: “Daddy, your left calf is smaller than the other one.”
Some months later, I was unable to mount a steep rocky ledge when leading with my left leg. I reversed my step and then easily climbed up. I recalled my daughter’s comment but ascribed this temporary difficulty to vicissitudes of aging.
A week later I fell during my morning run. All day long I nursed an uneasy, portentous feeling. That evening, I used a measuring tape to determine my calf circumferences. Palpating my tibial tuberosities, I moved inferiorly ten centimeters, drew a line, then circled my calves with the tape. Indeed, there was a three-centimeter difference. I recalled a colleague saying they no longer teach medical students this technique because calf swelling has low likelihood ratios for diagnosing deep vein thrombosis. Oh well, I thought, I am not trying to diagnose DVT. I looked up causes of painless calf atrophy in a sixty-three-year-old man and found a short list of mostly bad diseases.
I saw my primary care physician. She measured my calf circumferences just as I had. “There’s a three-centimeter difference. I want you to see a neurologist.” This took a few months. By then I was observing occasional twitching movements in my leg. In forty years of practice, I had observed fasciculations in just a handful of patients. Is that what I was seeing? I watched YouTube videos of ALS patients who had filmed their fasciculations and grew more apprehensive.
The neurologist detected diffuse hyperreflexia and weakness in my left quadriceps and gastrocnemius. My EMG was markedly abnormal in three motor groups, classic for ALS, an untreatable disease with a median life expectancy of three to five years. I had suspected this diagnosis but was still stunned. Surely I could not be that sick. My children urged me to get a second opinion. I saw a specialist at one of the national ALS Centers who confirmed my diagnosis.
For me, it was the worst imaginable destination. Contemplating life without use of my legs was sad enough. But much larger horrors loomed ahead. Eventually I would need someone to put me to bed, to feed me, to toilet me, an electronic device would talk for me. This loss of independence and dignity, being a constant burden to my loved ones, would bring intense suffering. Who would I be without my self-reliance? I investigated physician-assisted suicide and learned that the most common reasons to seek lethal drugs under the Oregon Death Act were loss of dignity and being a burden to others.
I initially avoided discussing my diagnosis with friends and colleagues. Maybe I was afraid to admit to them (and to myself) that I was going to be different, no longer the energetic eager-to-help person I had always tried to be. I wanted to pretend I was still normal, even though I was already walking slower and taking the elevator instead of stairs. I did not want anyone to hold open a door for me, to help get my suitcase off the baggage carousel, to feel sorry for me.
For two years, my daughter had been urging me to join her in walking the Camino de Santiago. “We’ll do it sometime later, I can’t afford that much time off right now,” had been my previous response. But now my heretofore hurried life had become decidedly different. So we met in northern Spain for this long-delayed father-daughter trip.
During our daily hike and in the cozy albergues where we slept communally each night, we swapped conversation with our fellow peregrinos, pilgrims of the Way. Many had a special reason for their journey: a recent divorce, death of a loved one, survival of a cancer diagnosis, celebration of a special success. Like one arriving empty-handed at a potluck supper, I feasted on these intimate disclosures, without offering my own. “Caminar con mi hija”—to walk with my daughter—the tasteless crumb I offered in clumsy Spanish.
At the end of each day, we celebrated Pilgrim’s Mass in small village churches. I understood little of the spoken Spanish, but the solemn stillness, metrical incantations, and intimate joining of hands for the concluding Blessing of the Pilgrims created a peaceful timelessness. Becoming lost in the present moment of holy celebration softened my anxiety and fear, as soothing as a cool drink at the end of each long day’s walk.
After returning home I enrolled in a clinical trial. I was not looking for a miracle cure. I thought I might eventually help some future doctors’ evidence-based practices. But my real motivation was not so altruistic. I secretly hoped that I might again feel eager-to-help and find a temporary antidote for the anticipated helplessness that lay ahead.
My care team suggested that I attend the local ALS support group. I did not want to go. I thought it would be depressing to see firsthand my future path. You’re being silly, I thought, you always recommended support groups to your patients, why are you any different? So, one cloudy Monday night, I showed up. “Welcome to the club nobody wanted to join,” greeted a friendly man with immovable legs in a motorized wheelchair. To my surprise I came to cherish my ALS support group. They helped me understand that there is special comfort in confession, and that strangers can become good friends when they share a common destination, be it the cathedral of Santiago de Compostela at the end of the Camino or the shrine of silence at the terminus of the ALS trail.
Inconsistent with my previous priorities, I connected with some long-neglected high school classmates, old friends I had not spoken with for decades. Each of these conversations brought me unanticipated, special happiness. It became easier for me to talk about my diagnosis. I developed an interest in mindfulness meditation and though never a regular practitioner, I discovered joy in celebrating the present and what was right in front of me.
Together, my wife and I made a number of decisions: retire early, move into a handicap-accessible house, locate closer to our kids. We felt this was the best way to address a difficult future. Though it took over a year, we built an accessible home, I retired, and we moved to another state. Amazingly, through all of this, my strength prevailed. My legs did not grow weaker. I carried heavy boxes without falling.
It was now over two years since my diagnosis. My neurologist was surprised by the lack of disease progression and repeated many tests. My exam and EMG were now nearly normal. Somehow my disease had gotten better, something ALS never does. Somehow, inexplicably, I no longer had ALS!
My brother told me I had been misdiagnosed. I recalled many of my own incorrect diagnoses, often only self-confessed. ALS is an incompletely understood disease with inexact diagnostic criteria, a situation where it is easier than usual to misstep. I read many books and papers. I repeatedly reviewed my own records, noting my declining and then improved functional rating scores. I became convinced that I had not been misdiagnosed.
So how DO I understand this? Some of my church friends say it was the power of prayer, but I do not think God works that way, answering some prayers while ignoring others. My daughter thinks the Camino cured me. After all, thousands of peregrinos attest to the healing power of that spiritual walk. Maybe it was the investigational drug I might have taken during the clinical trial. Ultimately, I felt that it did not really matter. This is just one of life’s astonishing, stochastic events, onto which I will hang my own emotional and moral attachments.
Triumphantly, I ascend the final 100 meters of a steep rocky ledge to arrive at Chasm Lake. My labored breathing is suddenly arrested when I glimpse the stunning beauty of this alpine lake. On a flat rock, I stretch out my still asymmetric legs, to reflect on my journey and the wonder of this sacred space. Like a huge communion cup, a massive stone cirque surrounds the water of the azure lake. The image of immovable gray granite reflected on a shiny shimmering surface creates a marvelous contrast, inspiring awe, reverence, and disbelief.
I am astonished that I could make this hike, an accomplishment I recently thought would be unachievable. I am overjoyed that I can anticipate a new life with a different ending. I rejoice every day at this incredible gift. But I also have unexpected feelings of sadness and loss. Friends in my ALS support group are still burdened with a horrible disease, and for no understandable reason, I escaped their inexorable fate. Many of them have already reached trail’s end. When I had ALS, I found new intimacy with family and friends, and with myself. This now seems less palpable. When the path was clear and the end seemed near, I celebrated the present with more intensity and joy, my focus was sharper. As I arise to leave, I ponder the route ahead. Where do I go from here? How will I find my way?
Acknowledgement: I read an earlier version of this essay entitled “Now You See It Now You Don’t” at the 2019 Annual Meeting of the Colorado Chapter, American College of Physicians. The Colorado Chapter graciously granted permission to submit a revision for publication.
J. RUSH PIERCE JR., MD, MPH, practiced in West Texas as a primary care internist and geriatrician for two decades. He then served on the faculties at the Texas Tech University School of Medicine and the University of New Mexico School of Medicine (UNM). He retired from UNM in 2018 as Professor Emeritus and moved to Colorado. He currently volunteers as a clinician at Denver Public Health and Doctors Care in Littleton. He cherishes spending time with his family, which includes his wife of 45 years, four children and their spouses, and eight precious grandchildren. He enjoys hiking in the Rocky Mountains.