I believe that I may be a little unique in that I was older than most PH patients when I was diagnosed. I was nearly 78. But that doesn’t change the fact this disease hits as hard as in a younger person. I am a retired RN who never really knew much about this disease, and it wasn’t even a diagnosis when I was in school, but determined to learn all I can. I have been diagnosed nearly three years at this writing.
The first indication was almost eleven years ago. I went to my primary care doctor with shortness of breath and severe ache across the back between my shoulder blades. He sent me to a cardiologist for a full workup. He did the usual tests but apparently did not find anything. So I was sent to a reputable pulmonologist who diagnosed me with asthma after a series of PFTs. He never did hear any wheezes, but I have, very infrequently, had to use a rapid action inhaler when pollens are bad or around some cats. After several months of medications, which did not work, they were discontinued as the PFT’s were said to be normal. On my last scheduled visit he told me, “Why are you here? You don’t need me.” I left in tears! I knew something was wrong!
I had pneumonia the next spring. In the meantime the SOB would be off and on, depending on my activity. But I had taken up a new hobby a few years before. My ancestors were Scottish, and after a trip to the British Isles and Scotland, I had a chance to learn to play the bagpipes. It takes a lot of air, but I could manage and was really doing well, and was really enjoying them. We would play for special events, march in parades, etc. Then in the fall of 1999 I found I just couldn’t play them. I could manage for a few minutes and then just had to stop. After resting for about ten minutes I could play again, and then I would have to stop again! I was so very frustrated, and it broke my heart to have to give them up. And I had SOB much more frequently.
I had to take “breaks” just trying to put a meal together. What seemed to bother the most was using my upper body—putting dishes in the cupboard. Folding laundry. Weeding flowers. Washing my hair. Singing in the church choir. I knew something was wrong. Really wrong. And I had this intense ache across my shoulders; my chest felt like it had an Ace bandage wrapped around it all the time. So back to the doctor. And back to the cardiologist with the same result, except he thought I should be checked for Myasthenia Gravis. So off to a nationally known clinic, where it was determined I was ok neurologically. I also saw a pulmonologist there, who at the bottom of his report mentioned the slight possibility of PH due to the scleroderma. But no suggested follow up.
So, back again to my PCP. My first doctor had retired, so his replacement sent me to another pulmonologist. I won’t even talk about him. He shouldn’t even be practicing! I had an RN friend who had the same symptoms, and she introduced me to the possibility of pulmonary hypertension. Luckily she didn’t have it, but now I had an idea of what to look for. Finally I told my PCP she had to find me a good pulmonologist, and a new one had just started practicing locally. So off I go again. And this one LISTENED and TALKED TO ME! Of course he had to do his own tests, but eventually he did a RHC and lo and behold I had PH. By that time you could have told me anything—JUST TO KNOW WHAT WAS WRONG! It is mild but I have been on Tracleer since the diagnosis, which was in 2005. So it took at least six years for my diagnosis. I wouldn’t wish this on anyone!
I was diagnosed with Scleroderma in 1988—20 years ago. It was called ‘morphea’ at that time. I was also told that as long as it just on my extremities, it would not bother me (internally) but if it went on my trunk, it might. Well, I had it mild enough that it really didn’t bother me much, and yes, it started on my trunk about the time I began having internal problems. No one warned me about PH; in fact no one let me expect much of anything with it. Now, of course, more is known, and also more is happening to my body, with fibrosis developing in my lungs. And no doctor ever seemed to connect scleroderma to my breathing problems.
Doctors—some seem to really care; to some if you didn’t fit the “mold,” too bad. One rheumatologist I saw checked my fingers, bent them back and forth and when I said they didn’t hurt, told me he couldn’t do anything for me! I understand he is very good for rheumatoid arthritis! Once, when I was telling a doctor how hard it was to do housework, he said ”I think you are allergic to housework!.” Several have asked me about depression- and I tell them-“No, I am not depressed, but I soon will be if I don’t get some answers!”
It has been hard adjusting to my “new normal” I gradually had to give up many of my activities, including the choir. Giving up my bagpipes and the choir were two of the hardest things I had to do. But I do what I can. I read, watch what TV is any good, and knit teddy bears for our church. And help my husband with jigsaw puzzles. I had given up knitting as the shawls and blankets were too heavy, but can handle small items. But I have the love and support of my whole family, and my husband has been great. He goes with me to appointments, meetings, etc and knows almost as much about PH as I do. He is my “househusband.” And my girls have become very knowledgeable about PH.
The one thing I can do is help advocate for PH. I do what I can when admin sends out pleas to contact your congressman. I helped set up a program at church for November’s PH month. I try to educate anyone who is interested and maybe even if they are not! I attend both scleroderma and PH support groups. If I am hospitalized I tend to educate the nurses, etc. I may have even been obnoxious, but hope not. And when new patients come along, I help and encourage as much as I can.
I guess the biggest thing I have learned is that you have to advocate for yourself. No one else is going to do it for you. If you have questions, write them down and then ask them! If you are uncertain about a treatment or med, ask! And I hope that someday doctors will receive more training in the diagnosis of this disease and know who to refer you to. And make sure whomever you go to, make sure he listens and talks to you as a person! And then accept your “new normal” life.