Brigette Thompson: This Is My PAH Story

“It all started one morning in 2011 when I went to work. I parked in the parking structure on the first level. I got to the elevator with my work cart. I pushed the button, but nothing happened. I waited and waited and waited. Still nothing. As I stood there trying to figure out what I was going to do (I knew I could not go up the stairs), a man came from nowhere. He picked up my cart, ran up the stairs and was far away by the time I got up the stairs. I yelled gasping for breath, ‘Thank you!’ He said he had to run, that he was on ‘C’ shift and had to clock in.

“I knew there was something wrong with me. Never in my life could I not walk or even run up a flight of stairs, even with the cart.

“My symptoms presented like asthma. I had shortness of breath and tired very easily. I would get on the treadmill and couldn’t do what I used to do. I told one of my doctors, and he said, ‘You’re probably just out of shape.’ I said ‘No, this is different.’

“So, I went to my pulmonologist­—I already had one because I have lots of allergies—and he tested me for asthma. I did not have asthma. He tested me for several things. I did not have any of them. He said, ‘I’m running out of things to test you for. There’s only one thing left; you probably don’t have it because it is so very rare.’ I was fortunate this process with my pulmonologist only took a few months.

“It turned out that my pulmonologist was one of the team of pulmonologists who first diagnosed pulmonary arterial hypertension (PAH). One right heart catherization later, it was discovered that I have idiopathic pulmonary arterial hypertension (IPAH) with a pressure of 103. At first, I was relieved to know that they found the problem. Then Dr. Shapiro began to explain to me what PAH is: high blood pressure in the arteries of the lungs and there currently is no cure. The doctors do not know how to stop the pressure. A terrible feeling came over me. I knew my life would never be the same.

“For starters, on the day that I met with Dr. Shapiro, I was drinking a 32-ounce bottle of water. She informed me that was all the water I could have in a day. I said, ‘Are you kidding? I can finish this in a couple of hours.’

“Limiting my fluid intake was just the beginning of a modified lifestyle that I am still adjusting to.

“As a professor, I could no longer teach five, three-hour classes, so I modified my schedule. Fortunately, I was already teaching some of my classes online.

I opted to participate in a two-year study. That study was a very important study, because from that study, doctors now know that it is better to begin with two medications at the very start of a PAH diagnosis, rather than introducing one at a time. It was a double-blind study. It was revealed that I had been on Letaris. I was then put on Adcirca, and I was feeling better every day. I am proud to have been a participant in that study. A few years later I had another right heart catherization, and my pressure had dropped to about 55!

“Living with PAH is not easy. Fortunately, I am still able to work. Life with this illness means constant adjustments and accommodations. I still find it difficult to explain to people. They see my oxygen tank and they don’t realize that the main problem is my heart.

“I find one of the most frustrating things about living with PAH is that we don’t look ‘sick..’ Even Dr. Shapiro told me once, ‘You look great. If I didn’t know how sick you are, I couldn’t tell by looking at you.’ I recently read that PAH is known as ‘an invisible killer.’

“The best thing that I did for myself was to join support groups. There I have met some incredible people, who like me, are just trying to get through life with this life-threatening, incurable and terminal illness. Already, I lost two friends to PAH. Also, the singer Natalie Cole died of PAH.

“Every day that I wake up is a good day because I am still here. I am thankful for Dr. Shapiro and all of my doctors who help me stay alive. I am Brigette Michelle Thompson and this is my PAH story.”