Celebrating today’s victories to honor my younger self
8 months after a provider's discovery, I can hardly believe the changes I feel
When I was diagnosed with pulmonary hypertension (PH) in 1999, doctors prepared my parents for the reality I was facing. Just two weeks before my fourth birthday, they were told such words as “progressive” and “terminal.” The doctors set the trajectory of my PH journey before us, and it was a line that could only decline, eventually ending in tragedy.
My fight with PH ended up being much longer than anyone had anticipated. Instead of 10 months, the median pediatric survival for idiopathic PH around 1995, I miraculously survived past my college graduation at 22. One year later, at age 23, my life succumbed to its limit with the disease. I received the life-extending heart and lung transplant, which we were prepared for all those years ago. It’s still the only option for end-stage PH.
The experience of those 19 years with PH had a heavy influence on my mindset surrounding life, death, and my health expectations. That declining line remained in my head, even after a transplant completely transformed my circumstances.
While my transplant recovery was prolonged and complex, I was surprised to see improvement in those first two years. Transplant stopped my PH, and the decline I was accustomed to was no longer a sealed fate. For the first time in my life, I was getting stronger and seeing numbers that were getting better rather than worse.
Everything changed, however, in 2021. Infections I’d battled on and off started to overpower my lungs. With time, my X-rays grew cloudy and my stamina dwindled. The following spring, a bronchoscopy led to a new rare diagnosis: pulmonary alveolar proteinosis, which is almost nonexistent in the world of lung transplants. In an even more confusing twist, I tested negative for the autoimmune markers that cause this illness.
Simply put, there was no apparent reason for this condition to be happening to me.
My team developed a plan to treat the illness, but without a known cause, it wasn’t easy to adequately address. Over the next two years, the decline was extreme. I lost so much of the function that I’d worked hard to build in the years since my transplant. Physically and mentally, I surrendered to this new reality. It was a comfortable mindset for me after all those years living with PH. My CT scans grew worse, displaying dismally cloudy lungs clogging up with more and more protein from month to month.
A cause is determined
In October of 2023, another twist took place. A case of pneumonia sent me to the emergency room, where my records passed the eyes of a new provider on the transplant team. He possessed niche knowledge of this illness and its connection to lung transplant. He understood the cause to be a very rare complication of a transplant medication I started taking in 2019. With my team’s blessing, he recommended I stop the medication immediately. It was a simple solution for an issue that had caused so much destruction.
The improvement since then has been indescribable. My scans have improved for the first time since this condition began, the infections have dissipated, and my energy and disposition are drastically better. Just yesterday, I was able to golf with my family for the first time in two years. I’m walking regularly and getting back to some of my favorite daily practices, including cooking and creating art.
With this gift comes a seed of doubt. I’ve been paranoid, monitoring my oxygen levels and daily steps, and any slight change can quickly cause me to panic. I’m susceptible to believing that tragedy is always around the corner, as the doctors promised all of those years ago. I’ve never had such a significant redemption in my health, and it’s been challenging for me to accept the progress as permanent.
With that said, as I continue to have renewed firsts eight months later, I’m grateful for where I am. No matter what happens next, I’m just thankful that I had the opportunity to return to this level of health for a time. I don’t take it for granted, and I’ll forever be amazed that an answer found its way to me before I became compromised beyond repair.
I cherish every additional benefit for this body that can’t function fully. I’ll be spending this summer savoring the ability to get outside, enjoying time with loved ones, and moving meaningfully through my day-to-day. After so many years of many rare occurrences, and even more miracles, I know these actions would mean so much to my younger self, who was told she had so little life waiting for her. I honor her through the struggles.
But I especially celebrate her in the victories she never knew to expect.
Note: Pulmonary Hypertension News is strictly a news and information website about the disease. It does not provide medical advice, diagnosis, or treatment. This content is not intended to be a substitute for professional medical advice, diagnosis, or treatment. Always seek the advice of your physician or other qualified health provider with any questions you may have regarding a medical condition. Never disregard professional medical advice or delay in seeking it because of something you have read on this website. The opinions expressed in this column are not those of Pulmonary Hypertension News or its parent company, Bionews, and are intended to spark discussion about issues pertaining to pulmonary hypertension.
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