“[My hope is that] the public and the doctors [become] educated about this insidious disease.”

By Barbara Thompson

“Here’s my pulmonary hypertension (PH) story but there are many more just like it:

“The main thing that we all have in common is being misdiagnosed for so long and the lack of awareness of the symptoms for pulmonary arterial hypertension (PAH). Had I known them, I might have been able to avoid being bedridden for almost two years. Prior to my diagnosis, Chuck (my husband) took me to 14 different doctors, I had three hospital stays and many trips to the emergency room (ER).

“I was at the doctor’s office a lot because of the bronchitis that just would not go away. I even went once with my feet and ankles being so swollen that I couldn’t put on my shoes. My new primary care physician was with the University of TN Medical Group. We thought that if I were with a group of newly graduating doctors, they would be more up-to-date on ‘newer or rare’ diseases.

“Since I have other illnesses and am on so many medications, I requested to see the same doctor on every visit so that he would be familiar with my individual case. Finally, Dec. 9, 2002, he ordered an echocardiogram to make sure I did not have a fluid buildup around my heart. Well, he called me on the Dec. 12 to let me know I had PAH. It went right over my head. Since I had never heard of it, it couldn’t be too serious, right? I asked him about a lap-band or having a gastro-bypass and he said to get a handle on the PAH then worry about the weight that I had gained being bedridden for so long. The shortness of breath, the heaviness of my chest, the swollen ankles, the extreme fatigue, hoarseness and lightheadedness were all attributed to my weight, asthma and ulcerative colitis. I also have lupus anticoagulant that caused three strokes in 1991, at the ripe old age of 37.

“After the diagnosis, I went to a PH specialist. He said that he did not think that I had PAH, although the cardiologist who did the right heart catheterization said I had mild PAH. My PCP was not convinced that I did not, so he sent me to a local pulmonologist, who sent me to another cardiologist for right and left heart catheterizations. Turns out, my pressure level increased since no treatment was started earlier. PH is progressive and does not get better without treatment. The pressure increased from 26 mmHG in February to 31 by May normal is 7-12. I was in Class IV, meaning that I had the inability to carry out any physical activity without symptoms and the right side of my heart was enlarged. Dyspnea (shortness of breath), hoarseness and extreme fatigue were always present even at rest. Chest discomfort was increased by physical activity. I began treatment with Tracleer, the first oral FDA-approved treatment, in June 2003. Chuck and I started a support group so other PH patients would realize that they were not alone in this PHight!

“One of the sad parts about this disease is that it may be indicated by a non-invasive test called an echocardiogram — if only the physician knew about PH and its symptoms! The problem is, many, (in our case 13 different ones), do not know about PH. I do not blame anyone. If you don’t know about a disease, you just don’t know. But this has got to change. The public and the doctors must be educated about this insidious disease or at the very least be aware of it and its symptoms.

“I’m now 65 and really miss working with my husband, daughter and son in our family-owned wedding business. I have been unable to participate in the weddings since 2000 due to this disease. I was bedridden and it took over two years for me to be diagnosed. Since I was not getting sufficient oxygen, I do not remember most of those two years, but my family remembers every minute of it. I wondered for the longest time why God would take the weddings, my girls, the brides and working with Chuck every day, from me since I loved it all so much. I know now that it is to raise awareness of PH and its symptoms. The Pulmonary Hypertension Association (PHA) launched a campaign in 2012 (I think), using the zebra as a mascot of sorts to raise awareness, and I jumped onto that train wholeheartedly and became known as The Zebra Lady, wearing something with a zebra print every day of the year. I’ve developed several graphics that include my zebra named PHyllis and zebra print. With the assistance from PHA, I had a card made that I give out on a daily basis that explains the zebra analogy on one side and briefly describes what PH is on the other.”