In early May 2018, having been on Symdeko for a couple of months, I went to my CF clinic. There was significant improvement in my PFTs; I was over 50%, the best they’d been in over a year. Symdeko seemed to be working! Just a month later, my world came crashing down on me when I was listed for a lung transplant after spending nearly three weeks on a ventilator.
It started out like any other CF exacerbation for me — increased cough, shortness of breath, and fevers. However, it quickly became evident that this was no typical exacerbation. Even with supplemental oxygen, my oxygen saturation was below 90%, and I was put on BiPAP. Even on BiPAP, my oxygen levels didn’t stabilize. So I was moved to the ICU, put on a ventilator, and later put into a medically induced coma.
A little over a week into the hospitalization, my doctor called my wife. He said, “I think these lungs have taken her as far as they can. She needs a transplant.” My wife and I never explicitly discussed transplant, thinking it was years in the future. After a meeting with my wife, parents, and sisters, it was decided that even though I was still in a medically induced coma, I would be transferred to the Hospital of the University of Pennsylvania (HUP) for a transplant evaluation.
The doctors at HUP were able to stabilize me, and I was awakened by a nurse practitioner asking me if I wanted a lung transplant. I vigorously shook my head. No, why would I want a lung transplant? And where was I? Once I was brought up to speed up on the current situation, they began an abbreviated transplant evaluation and education for my family and me.
On the evening of June 7, my doctor told me the transplant board had decided I would be listed the following morning. Due to my critical condition, he speculated I would be near the top of the list. He explained that it might be possible for them to get me off a ventilator without a transplant and recover some lung function, but they were concerned what the outcome would be when I inevitably got another infection. I didn’t just cry, I sobbed.
I mourned the loss of the years I thought I still had until I needed a lung transplant.
On June 8, I was listed for a bilateral lung transplant, and early in the morning the next day, I was informed there was a match for me. The lungs were considered “high-risk,” so accepting them was optional. I decided to move forward with the high-risk lungs and was given an estimated time for my transplant. My surgeon explained what would happen, and I was scheduled for the late afternoon. As the hours slowly ticked by, I had my family take the clock off the wall. The surgery that was supposed to occur in the afternoon that day started just after midnight on June 10.
Surgery went well, and after several days in the ICU I was transferred to the transplant floor, where my recovery began in earnest. I received additional transplant education, and A LOT of physical therapy. I’d been immobile in a hospital bed for close to three weeks at that point, and my muscles had atrophied significantly. Not only could I not walk without the help of a special walker, but I couldn’t stand or sit up on my own either. I had undergone a tracheostomy before my transfer to HUP and was still unable to eat or speak, partially due to vocal cord paralysis. I attempted to write, but my hands were too swollen, and I had tremors — a side effect of an anti-rejection drug. It was a slow, frustrating process. I had fallen into a deep depression and lacked motivation. I spent hours staring at the wall, while family and friends tried to engage me in any activity.
I eventually reached the point where I could sit and stand on my own and walk with a walker. There was discussion of discharge. I thought I was finally going home — then I was informed that I would need to spend time at University of Pennsylvania's acute rehab facility. I was crushed. There seemed to be no end to this nightmare. My transfer to the rehab was further delayed by a bowel obstruction — the first of almost a dozen I would have in the first three years post-transplant. Rehab consisted of 90 minutes of physical therapy and 90 minutes of occupational therapy daily. I cried every morning, I was so homesick.
Finally, 67 days after I walked into the ER and four days before my 36th birthday, I was going home!
Once I was home, I began my regimen of PT and pulmonary rehab three times a week. I walked on the treadmill at a monumentally slow pace, remembering that I’d been running 5Ks just over a year ago. My competitive spirit eventually kicked in, and, with the persistent encouragement of my family and friends, I “graduated” from rehab a week early.
But even at home, being post-transplant was overwhelming. I could not drink tap water, so I tediously made ice from a gallon container of spring water. I felt like my 35 years of “experience” as a CFer was worthless in this new life of taking dozens of different pills, recording daily vitals, and managing steroid-induced diabetes. With the help of a therapist, I slowly began to process my feelings of anger and sadness over everything that had happened. It was a slow and challenging recovery.
Four months after transplant, my wife and I took a trip to Busch Gardens. My wife marveled at how I could walk around the whole park without getting out of breath. While on a roller coaster, I teared up — this was my new life. It was going to be full of new challenges, but also new opportunities, and I was going to make the most of it.
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