Because I was so used to exercising all the time I eventually gave-in to the fact that something was wrong, it wasn’t just being out of shape. I asked my pediatrician for an inhaler and when I said it wasn’t working she sent me to a pulmonologist. That started me down the 2 year long road that many PH’ers experience when they are originally diagnosed. The reasons I was given for my trouble breathing ranged from hyperventilation to a vocal cord disorder to psychological pressure I was putting on myself to be perfect. I was eventually referred to St Louis Children’s Hospital where they did a right heart cath and found that I had PH. My pressures weren’t very elevated and the docs thought for sure I had secondary PH, so I endured months of testing only to end back up on the operating table for another cath. This time though they went in through my neck so I could sit up and ride the exercise bike with the catheter in. My pressure spiked with exercise. I was told I had exercise induced primary pulmonary hypertension (what is now called idiopathic). At this point they started me on Tracleer, which was at the time a newly approved PH medication, and Procardia XL.
My doctor told my parents if I was his daughter he would get a second opinion, so we did. I started being seen at Columbia Presbyterian Hospital in New York City. This was a long way from my home in western KY, but seeing one of the best doctors was worth it. It was at Columbia Presbyterian that they discovered interstitial markings on my chest CT and recommended I get an open lung biopsy to diagnose what they felt sure was Pulmonary Veno Occulsive Disease (PVOD). The best place to have this done, they said, was St Louis Children’s Hospital. So there I was, instead of attending my first day of my junior year of high school I was being wheeled into surgery for a lung biopsy. The results showed PVOD, and a week later I was on the lung transplant list. At that point lungs were still allocated by time on the list instead of severity of illness, and my doctor told my mom his biggest fear was that I wouldn’t make it to transplant. I spent a couple weeks completely grief-stricken. I had just been told I had a disease that 150-200 people worldwide were diagnosed with (most people were diagnosed with the disease in autopsy) and that if I hadn’t gotten a transplant by the time I graduated from high school I likely wouldn’t be alive any more. And as they say, the rest is history…
After those 2 weeks I decided I wasn’t going to let this disease define me. I was going to be the anomaly. I started speaking to civic groups to raise awareness for organ donation and raising money for the Children’s Organ Transplant Association. I wasn’t going to let PVOD and PH stand in my way. I applied for colleges just like I would have without PVOD (although St Louis became a top city on my list of places to be since my doctors were there). I attended Washington University in St Louis and graduated with a BA in anthropology in four years. My first year there was hard, I had to learn how to take care of myself, and figure out how to tell people about PVOD without them thinking I wasn’t able to do anything. After I graduated I stayed in St Louis to work at a research center and started riding my bike, albeit slowly, dancing salsa 3 times a week and doing yoga at least twice a week. These were all things I dabbled in during college but I was able to really pursue them once I was out of school. Another thing that came with being out of school was getting moved from pediatric care to adult care. This meant I was re-evaluated for transplant and since the qualifications for lung allocation had changed from time on the list to illness severity I wasn’t eligible to be on the transplant list.
After that year of working I went back to school for a Masters of Public Administration, but not before I did a cross-country road trip with a college friend of mine. We camped in a tent every night, making our way from Kentucky to California via all the national parks we could (think up to Mt Rushmore and Glacier national park to the redwoods, the Grand Canyon and back to LA. It was amazing. I was able to camp and hike and almost forgot I had PVOD.
When I went back to school I worked 20-30 hours a week at a law firm and took classes at night. I learned a lot in those 2 years, but I think the most important thing I learned wasn’t in the classroom. Right before winter break my first year of the MPA I thought I was getting my usual end-of-semester-cold and I put off going to the doctor. By the time I got home for break I wasn’t eating and couldn’t walk from my bedroom to the living room without being exhausted. Finally on Christmas Day night my sister said to me “If I felt as bad as you look I would go to the ER.” Leave it to a younger sister to be completely blunt. So I agreed to go to the ER. The next morning I had to be intubated and flown to St Louis because my lungs were no longer working. I was sedated on a ventilator for 6 days. The doctors still aren’t quite sure exactly what happened but some combination of mono and another virus allowed me to have 5 liters of fluid in my lungs. I was basically drowning. The lesson I learned with that was to trust my instinct if something feels wrong and that I should always err on the side of caution.
This past year I completed my Master of Public Health at Washington University in St Louis and to my parent’s great relief have been gainfully employed for the past 6 months. I am now working at a large non-profit research company, Battelle Memorial. I am doing public health research and am currently working on a study to see trends in childhood obesity and characteristics of programs in communities that have lower rates of childhood obesity.
My life definitely changed with my PH and PVOD diagnosis. I tell people that it really has shaped me as a person. I am a stronger person because of it. I face adversity with much less fear than I think I would have otherwise. Despite the fact that most PVOD patients don’t respond well to Tracleer, and most have a negative reaction, I am lucky that Tracleer seems to be working for me. I am able to do more now than I was when I was diagnosed, despite the fact that I was a training athlete at the time. Today I’m an avid bike rider, I just started rock climbing, and I volunteer with several organizations; 12 years ago I was told that without a lung transplant I wouldn’t be here today. Management of the disease is still a daily part of my life, but I don’t let it define me or dictate my life. The advice I try to remember when I have bad days or when I am feeling down is to remain positive and to keep fighting. I’m so glad that I refused to let the diagnosis be the death sentence it seemed. Having a good outlook has been the best thing for me.