Susan's Story

My name is Susan. Until I was 48 years old, I was mostly a normal person. I was married, had three nearly grown children, had a teaching job I just loved and was relatively healthy--or so I thought.

I had had trouble breathing when I walked up any incline for about a year and a half. I thought that I must be out of shape and so I started to walk more to get back in shape. Several months before my 49th birthday, I also started having terrible gas pains; not in my abdomen where you would think, but in my back and around my heart. Sometimes I even thought I was having a heart attack. One day I walked across campus and up a long flight of stairs. When I got to the top of the stairs I was completely out of breath. I was desperately gasping for air and my heart was pounding out of my chest. I felt like I had just run a marathon and wasn't going to make it. I had to lean up against the wall at the top of the steps and wait until I got could get control of my breathing before I was able to continue down the hallway. That's when I knew that something was horribly wrong and I was sure it was my heart.

In December 2000, I had a stress test at my local hospital. I did less than two minutes on the treadmill before my heart rate was high enough to get an accurate reading on the EKG. Good news! My heart was in good shape. The cardiologist who read my stress test recommended I get an echocardiogram to rule out pulmonary hypertension, a disease that I had never heard of. The echo confirmed his suspicions but I still had no idea how serious it was. When my PCP got the results of the echo, he immediately made an appointment with a Pulmonologist. The Pulmonologist ordered many tests (I thought at the time that it was every test known to man) and referred me to a PH specialist. When I saw the PH specialist, he had even more tests done to confirm the PH and rule out anything else. He decided that I did have PH and that it was secondary to CREST syndrome. The good news is that it was caught fairly early and that I was a good candidate for Flolan and a transplant. I started Flolan, a continuous IV medication in 2001 and it saved my life. I taught for a semester after that but in early 2002, after a second bout with pneumonia that required 18 days in the hospital, I decided it was time to retire from my beloved profession.

It's been almost 8 years since all this started. When I was diagnosed, Flolan and lung transplant were the only treatments for PH. Fortunately, the Flolan worked so well that there was no need for a transplant. Still, I had a lot of side effects with Flolan and went through a period of depression. Since then there has been an explosion of treatments. In 2005, I was converted from IV Flolan to IV Remodulin in combination with Viagra. This combination reduced many of the side effects that I had been having with Flolan. Although I am still disabled to work, the new therapy mitigated many of the side effects of Flolan and gave me back a large measure of the quality of my life. I am still very functional and lead a full, rewarding life. But I still hope for a cure for PH someday.

Susan