When we finally entered the right building on the grounds of the National Institutes of Health in Bethesda, the sight of patients and doctors from every corner of the world had an immediate impact. Seeing very sick children hooked up to medical devices and being wheeled around the hospital lobby by their families, we were reminded of how lucky we were to be where some of the best genetic research and medical care in the world was taking place. Under the guidance of Frances Collins, the NIH’s inspiring director and doctor who presided over the Human Genome Project, everyone around us seemed to understand they belonged to one big human family. My rare genetic disease was just a small hiccup in the universe of the NIH.
After my voluntary participation at the hospital in a clinical study on Cushing’s Syndrome, my surgeons were armed with some 2,500 test results. They were the most experienced doctors in the country at performing bilateral adrenalectomies. My pre-surgery consult was routine and straightforward. But then how much of life goes exactly as we plan? During what turned out to be a long 8-hour surgery, the surgeons discovered their laparoscopic instruments had nicked my intestines in three places. Both of my adrenal glands had been successfully removed. Cushing’s had produced 12 tumors on each. But the careful inspection and repair of the intestines, which had to be done outside the abdomen, took many hours.
My immediate recovery in the ICU was complicated by repeated runs of ventricular tachycardia and a painful bloating. Three days later, after several blood transfusions to address low blood counts and my continuing discomfort, a CT exam was ordered by the ICU and revealed a large hemorrhage in my abdomen. Another tachycardia episode at the end of the exam gave the technicians some extra anxiety. The emergency had my doctors rushing in to the hospital in the middle of the night. Liters of blood had to be drained laparoscopically from my abdomen, but the source of the hemoperitoneum proved hard to find, so a large general incision was made. Unfortunately, this second 8-hour surgery began with one of the scariest experiences of my life. The general anesthesia hadn’t fully kicked in when I was wheeled into the operating room. I found myself conscious on the operating table, unable to move a muscle or communicate. I was listening to the surgeons talking with each other as they began reopening the laparoscopic incisions from my first surgery. I know I was screaming at them but the screams were all in my head. I had no way of knowing how long I would be awake. Finally, the anesthesia took hold and the longest minutes of my life were over. It’s a trauma I wouldn’t wish on anybody. The source of my bleeding turned out to be a small left adrenal artery. In order to get at it and clamp it, the doctors had to remove my spleen.
Back in the ICU, my heart was still erratic. Over the month I stayed there, I don’t know how many Code Blues I experienced. At one point, one of the head cardiologists from the Walter Reed, the military hospital across the road from the NIH, was called over for a consultation. He recommended the one heart medication that finally controlled my arrhythmia.
After decades of living with excess cortisol in my body, nearly four times the regular amount, adjusting to a new biologic dose of hydrocortisone was hard. My fatigue was incredible. A social worker at the NIH had warned me that recovery would feel like coming off a heroin addiction. I could barely keep my eyes open. The room always seemed dark but I couldn’t sleep. Day and night became unrecognizable. Tubes were stuck down my throat and nose at various times with some not very pleasant results. But even when I wanted it all to end, I still had an awareness of the incredible professionalism of the doctors and ICU nursing staff who were taking care of me. I had never experienced anything like this level of medical care. When the young head surgeon made a point of visiting me by himself to apologize for all the complications, his frustration and compassion were palpable. At that moment, all I wanted to tell him was how grateful I was that when things went wrong, he was there and his skill and agility made all the difference. I told him the NIH was one of the most inspiring places in the world because of doctors like him, and I meant it.
Robert Frost has a famous poem, “Acquainted With The Night,” about a figure who is out in the rain and outwalks “the furthest city lights.” Too often, the Cushing’s experience involves a slow disintegration of emotional support. My wife and children endured a lot of changes. They had the strength to give me the time and space to rebuild and reconnect, something no Cushing’s survivor can take for granted. There’s a part of this experience that can’t be shared with anyone, but even the smallest gestures of support can be enough to keep one getting up each morning and feeling grateful. Now the battle against the Covid-19 pandemic is shining an even more dramatic spotlight on our human vulnerability. We’re all being humbled together by this. The sense of being ambushed is overwhelming, whether one’s facing a rare genetic disease or a novel coronavirus. But seeing the dedication and compassion of healthcare workers from all over the globe, I can’t help feeling overwhelmed by gratitude once again.