‘Good ride, cowboy! Good ride!’ — From the song “Good Ride Cowboy,” written by Jerrod Niemann, Bryan Kennedy, Richie Brown and Bob Doyle and recorded by American country music artist Garth Brooks
Editor-In-Chief, Chester “Trip” Buckenmaier III, MD, COL (ret.), MC, USA
Pam and I have had our share of hard knocks this summer. From the ongoing personal and financial trauma of our sailboat/home lightning strike to our latest bout of COVID-19, the hits just seem to keep on coming. I also (finally) finished a fictional novel, but was utterly unprepared for the hell that is getting published. Publishing science, by comparison, seems positively easy. We have been holding up in Maine at our family cabin, and while it is hard to be melancholy at the lake, I have managed to feel a bit lost lately among the endless book agent queries.
I was snapped out of my personal “pity party” by another family blow, the passing of my nephew, Jack Timperley, who finally succumbed recently to complications associated with Fanconi Anemia (FA). This rare genetic disease impacts enzymes involved in genetic repair. Despite all conventional wisdom concerning Fanconi’s patient lifespan, Jack survived well into his mid-20s. You should not feel abashed if you have not heard of this condition before. I have never encountered another FA patient in my career beyond my nephew.
I sheepishly admit that early in my medical career, I used to refer to patients with rare genetic anomalies as having a “paragraph disease.” This aphorism was reflective of my anxiety toward these patients, since they often appeared late at night while I was on call alone. The textbooks usually had only a paragraph description of the disease. Invariably, the paragraph would end with warnings regarding airway management difficulties and to proceed with caution (thanks, no, thanks). I can trace most of my “high sphincter tone” anesthesia moments to paragraph disease kids late at night.
I became so self-absorbed trying to get through these challenging anesthetics with my career unscathed that I never considered the unique person and family relying on me for help. As I matured as a physician, in no small part due to my relationship with Jack, I corrected this character flaw.
Jack was inspirational to everyone who had the good fortune of crossing paths with the young man. His enthusiasm for life was generally infectious. Jack never allowed himself to be defined by FA. It was hard to bemoan silly personal problems when within his sphere of influence. Despite the rather harsh hand of cards he was dealt at birth, Jack always tended to end with a winning hand. Jack’s interactions with modern medicine due to his condition were too numerous to count. Throughout his life, he developed medical problems that I felt, as a professional, he would not survive. Time and again, Jack would bounce back because the “professionals,” like myself, were not considering his irrepressible spirit. Jack was a fighter and, despite his condition’s reality, he would somehow beat the odds. I have recognized my nephew’s spirit in many horribly wounded servicemembers in my career. These were the casualties I loved because regardless of the extent of their injuries, I knew they would do well.
Jack Timperley (https://www.jacktimperley.com/) did do well and, while I am saddened at his passing, I am energized by his uncompromising life. Jack died, subjecting himself to surgery in a bid to upgrade his condition. He knew the risks and bravely left instructions for his family, should things go wrong, as they did. Jack’s light was all too brief but so intensely bright.
I relate the story of my nephew and his rare genetic anomaly that took his life because tonight, somewhere in federal medicine, one of you will care for a “paragraph” disease patient. Instead of lamenting your hard luck, revel in the opportunity to extend the life of a superstar like Jack.
With pride and humility, I dedicate this editorial to Jack and all the bright lights like him. To my nephew, “Good ride, Jack! Good ride!”
