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Patient story

Julie Lisante

I was 13 years old when I first heard the words pulmonary hypertension. To a 13 year old girl, those words meant the end of the world. Everything I knew and everything I was used to was going to change forever.

Before those two words, I was a fairly normal 13 year old girl. I grew up in a moderate sized house with a loving family, my hard working and dedicated father, my loving mother, and my sometimes annoying but always fun younger sister. For years, the doctors thought I had something called exercise induced asthma, for I only had shortness of breath and palpitations when I would exercise or move around for a long amount of time. That diagnosis seemed to be fine, given as a lot of my friends had asthma and were familiar with it. But everything changed on January 3, 1999.

I was in gym class that morning, running with the rest of the class, around in a circle for the warm up exercise before the game of the day. Things were going fine, until I felt a burning sensation in my chest. I stopped near the gym teacher and tried to gain my breath back. Usually it would come within a matter of minutes, but the running had ended and the game was beginning and I still was not feeling any better. I made a motion to the teacher that I was leaving for the nurse, and promptly went the few feet down the hallway to see if my inhalers would help.

When I got to the nurse, she seemed shocked that I was so out of breath and my color was blue. She had never seen that on me before and I could tell by her reaction that she knew something was wrong. She sat me down, and gave me oxygen, which helped my color and my breathlessness. She then called my mother and my father, both whom promptly came from work and home to be with me. The nurse explained that the reaction I had wasn't normal of asthma in any way, and set up an appointment for me at a local cardiologist’s office that day.

Naturally, being 13 and confronted with this prospect of something being seriously wrong with me, I was scared. I was subject to a battery of new and strange tests, including the echocardiogram (which I thought was disgusting at the time), EKG, and lung functions tests that really made no sense to me. After the results were in, the doctor called my parents and me into his office. I was nervous, but at the same time, tired, and I wanted to go home. I never expected what he would tell me would end my life as I knew it. He explained to my parents that the echo had found an ASD (atrial aeptal defect) as well as elevated pulmonary pressures. The lung function tests showed limited capacity, far from normal. The diagnosis came soon after that, as pulmonary hypertension. At first, I didn't know what to do. I was numb from the realization that I had something so serious, so rare, that I never heard of it before. Then the reality and the fear settled in, and all I remember doing for the rest of the day was crying.

I was sent to Doctor Robyn Barst in New York City, a leading specialist in pediatric cardiology and pulmonary hypertension. Again, I was subject to a battery of tests and the results confirmed what the first doctor had said. Treatments were limited back in 1999, and my parents decided not to go with Flolan, but rather an investigational medication known then as Ut-15. I was started on the medication, in the third phase trial only a month after initial diagnosis.

At first, I saw no difference. I had no pain, I had no side effects, but that was not to last. I soon found out the real meaning of pain, when little nerves were hit by the tiny catheter, when capillaries were hit, it was excruciating. At the time, there was no other cure for the pain besides medication and heat applied to the area. Through the years I was on the medication and adjusting to life with pulmonary hypertension I went through a lot of things that normal 13 year old girls don't have to deal with. Instead of buying a new dress for graduation, I was laying in bed sleeping off pain medication. Instead of hanging out at the mall with friends, I was in the hospital getting poked and prodded for the study.

Getting back to a "normal" life was probably the hardest thing to do. 13 year old boys and girls tend to not understand everything that goes on in the world, and when one of their classmates suddenly disappears for two months and then returns as if nothing happened, rumors are bound to fly. Pregnancy, fatal disease, suicide watch, any combination of outlandish things that could possibly be cooked up, was spread through the school about me. Trying to tell 13 year olds about pulmonary hypertension is an impossible task, so I clarified my problems with them by saying it was bad asthma and left it at that. Only the closest friends who stuck by me through it all got to know what it really was about.

I've spoken of the horrors of this condition, but I also have something good that came out of it. I know what you're thinking, how can something good come out of something so devastating? Doctor Barst noticed one visit that I was a bit melancholy. She inquired as to why, and I told her in all honesty that no one really understood what I was going through. That was when she gave me the address and phone number of someone who would change my life forever. Her name was Jennifer (not her real name, I changed it to ensure no problems arise from this) and she was my age. She lived not far from me at the time, so my father and I arranged a meeting with her. She had PH ever since her birth, and had been through it all. Of course I was apprehensive about it, meeting someone new and all of that, but it turned out to be the best thing that ever happened to me. We had a lot in common right off the bat, and the thing I remember most about our first meeting was going to the ladies room with Jennifer and we exchanged horror stories about the Ut-15 sites and showed off our scars to each other. In that one moment, in the ladies room of that restaurant, I knew I had found a friend for life, someone who could really understand where I was coming from. We could be there for each other with nothing to hold us down.

Of course, time marches on, and things change. I went on to continue with the Ut-15, eventually changing to Tracleer and currently a combination of Tracleer and the study medication Cialis, while Jennifer moved on to the Flolan and eventually was granted a double lung transplant to cure the PH. I was happy for her, as she deserved the best life she could possibly have.

I went through high school different from the other kids. Limited schedule, classes were moved to make them accessible to me, and my absences were always excused. It was in Junior year, when I was 16 years old, that my life would again change, and this time, not for the better as it had by meeting Jennifer, this time it would change for the worse.

November 8, 2001 is a date that I will never forget. My father, the one who had been a rock and constant supporter of me through this whole battle with PH, died suddenly of a misdiagnosis. The doctors thought it had been pneumonia when in reality it was lung cancer, caused by years of smoking. I was angry at being abandoned, as we were making real progress in the battle with PH. The combination of Tracleer and Ut-15 (Remodulin) was working wonders and I was feeling better than I ever had before. I felt betrayed that he would give up like that, when he had always told me to continue fighting no matter what. I felt alone, without my constant rock there. I fell into a fit of despair after that, for months I didn't go to school, I didn't hang out with friends, and I became lazy with changing the Remodulin site (resulting in a lot of painful infections). It was only after talking a lot with Jennifer that I got back on my feet, and made a promise to continue the battle with PH. To live to see a cure, and to do this for the man who started me down the right path in life, and on the path to a new life with PH.

Five years later, I'm now 21 years old and I no longer have Remodulin as a treatment. Tracleer and Cialis (both pills) are working wonders for me, and if it had not been for Jennifer, my father, and the constant support of my family, I would have never made it this far. I suppose the point I'm trying to make by sharing this long story with you all, is that PH might be a bad thing, but it also drives us to do more than we ever thought possible. If we didn't have PH we would have never discovered this amazing pain threshold we have. If not for PH we would have never had the time to refine our talents in art and music. If not for PH we wouldn't have the kind of friends we have today. In short; PH changes us, whether we like it or not. Sometimes it's good, sometimes it's bad, but there is always a lesson to be learned from the changes. Hope is eternal, and it will never die.

 

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The National Organization for Rare Disorders (NORD) awarded PHA the Abbey S. Meyers Leadership Award in 2012 for outstanding service to PHA members in advocacy, education and other key areas.