PHighter Friday: Carson

My journey with Pulmonary Hypertension began before I even realized it.  I thought I was out of shape and needed to train harder. Looking back I now know what I didn’t know then; it was PH and not my lack of training that was keeping me from being able to run as far and fast and long as I wanted.  In middle school and the early part of high school I identified myself as an athlete.  I played soccer and softball, cycled, and ran cross country and track. My stress relief was running. I also danced tap, jazz, ballet, and pointe. 

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.

PHighter Friday: Clair

My diagnosis came on much like it does for many other young women.  During high school, I began to notice an increasing amount of chest pain, a racing heartbeat, and shortness of breath.  Characteristic of a developing adolescent, I denied that these symptoms were abnormal.  I attributed my inability to breathe and instances of passing out as being out of shape.  However, after a year of tests, hospital visits, and appointments with doctors and specialists, I was diagnosed with Primary Pulmonary Hypertension. 

As a senior in high school preparing to leave for college, the diagnosis was devastating.  Even though my doctor gave me the green light to go away to college and a positive long-term prognosis, I began to find that the emotional toll of having a progressive disease was more difficult than its physical manifestations.  Even though I attempted to cope with the anxiety that stemmed from my diagnosis with PH, it began to extend to other aspects of my life.  I began to doubt what I could make of and do in my future, and this doubt precipitated new insecurities.

Although there wasn’t a dramatic turning point, the woman with PH I am now is a stark contrast from that newly diagnosed teenager.  I changed the way I took care of myself.  I began to eat healthier by eliminating junk and adding fresh produce to my daily diet.  I also started practicing yoga and working out.  Of course, my workouts are modified.  I’m not running miles upon miles or doing intense cardio, and probably never will. But I keep a consistent schedule of yoga and lead a rock-climbing and yoga club for my students at the school I teach.  I do what I can, and I’m certainly doing much more than I ever thought would be possible after receiving my initial diagnosis

This past summer I spent 6 weeks in Europe.  I climbed Mount Vesuvius, a large volcano in Italy, climbed over 600 stairs to the top of a church in Florence, hiked in the Alps, and visited 6 countries.  The people with me understood that although I said yes to every adventure, I was going to do it at my own pace.  When I climbed Mount Vesuvius, I stopped a little at first, but as the altitude increased, I had to stop every few steps.  In our group, I was the very last person to make it to the crater and I didn’t care. I was only focused on completing my journey to the top no matter how long it took me.

Now a decade into my journey with Pulmonary Hypertension, I’m currently in the process of planning a trip this winter to Zambia, Africa.  Disease or not, everyone has one life.  Dwelling on what will or will not happen will only cause unnecessary worries.  Had I never broken free from my initial doubts or my perceived, grim outlook on my future, I know that I never would have taken the chance to start yoga, or to attempt an ambitious climb to the top of a volcano.  However, I was allowed to do both of these things, and I will continue to experience life, because I am committed to doing the absolute most that I can with my disability.  I will not allow Pulmonary Hypertension – or the psychological toll that comes along with it - to take away my potential and the opportunities that come my way.

PHighter Friday: Anastasia

Slowly but Surely

I wanted to travel the world. I wanted to be a powerful business woman (like Samantha Jones in “Sex and the City”) and have a family. Also, I love sport such as swimming, bicycling, playing basketball, playing badminton and playing tennis.  Most of my childhood was filled with fun activities, travelling, hanging out with friends and breaking the rules. But those dreams were crushed when my doctor told me that I will not be able to do sports that I love, I will not be able to travel, and having children is out of the question….

I was diagnosed with Primary Pulmonary Hypertension when I fifteen years old when I moved to the U.S. Back in Indonesia, I was always chubby and sluggish back in middle school and part of high school. My parents thought it was because lack of exercise and my general doctors in Indonesia did not detect any health issues. When I was about to start high school in U.S., through regular check-up, the family doctor detected irregular heartbeat and I started to notice apparent health issues such as difficulty breathing, chest pain and blue lips. So, I was urged to see a cardiologist and pulmonologist. Sure enough, I had VSD (Ventricular Septal Defect) and PPH (Primary Pulmonary Hypertension).

I was devastated…

For a while, I could not understand why this defect happened to me. Although, overtime I realized that wallowing is not getting me anywhere.

After the diagnosis, I had my open heart surgery on my 16^th Birthday to close the hole in my heart. I am taking medications to control my PPH. It is not easy to integrate this disorder into my life at first.

I realize that I am rare. So, as long as that is the case, why not go all the way.  So the key is to accept that I do have this unique condition and I am going to have to live with it for the rest of my life.

So I learn to get to know myself.

I am thirty years old now. I am working full time as a supervisor, I volunteered at convalescent homes, I go to the gym at least three times a week, I went hiking (5 miles up the hills recently) and I am finishing my bachelors in Management and International Business. I do what I can now and everything else will fall into place. I may not go with the phase of normal people, but I will finish what I start slowly but surely.

I changed pulmonologist who is better and gave me support. She advises that I should exercise and keep doing what I do at my own phase.

I believe that our journey gives us strength; the destination is just the icing in the cake. And getting to know myself is the fundamental part of starting the journey and setting up goals. Also, it is important to surround myself with people who are supportive of me and my goals.

Life may not always go as planned, but it does not mean that I cannot make the best out of the one I have now. Like Mother Teresa said ““Yesterday is gone. Tomorrow has not yet come. We have only today. Let us begin.”