This is the PH Association Patient Support Line: (Phone number can only be seen by the question and answer creators). They should be able to answer your questions and address your concerns. I would suggest looking for a support group in your area too.

Joan, I know some medication side effects can be alarming or scary. However, I believe the benefits can far outweigh the risks. Regarding PAH specifically, data shows that patients that are able to tolerate medications aimed at treating at least 2 of the 3 pathways typically increase their survival rates beyond 5 years. Those that are able to tolerate medications that treat all 3 pathways have the best survival rates. Knowledge is power.

I have an autoimmune disease, Sjogren's, which is in the same family as Scleroderma. Two years ago, after being diagnosed with Sjogren's, my life was turned upside down. I went from healthy to chronically ill overnight and had to accept that medications to manage my condition was now part of my life. A PAH diagnosis was a punch in the gut. I'm 64, retired. I have a good life, a wonderful marriage, great kids, and 4 grandkids I adore. There is still so much life I want to live. That means I have to figure out how to live that life and best manage not only Sjogren's but PAH as well.

Take time to grieve. If you feel stuck, you might want to find a counselor that can help you work through this. Mental health greatly impacts physical health.

A normal pulmonary pressure is 8-20, so yes, 50 is high. There are many people who have pressures much higher. PAH is considered not curable and terminal. However, you must bear in mind that statistics are based on past numbers. Treatment of PAH has advanced and there are quite a few clinical trials that look promising. Treatment is focused on slowing disease progression. There are lots of factors that must be taken into account with regards to prognosis. There are many good articles on this site. I would suggest you also go to www.pahinitiative.com. There are excellent videos there that discuss PAH specifically including diagnosis, the treatment pathways, follow-up care, etc.

I have PAH as well. It felt like a death sentence when I first was given the diagnosis. Now that I know more about the disease and the treatment protocols, I am less frightened and more hopeful for my future.

https://www.myphteam.com/resources/4-tips-for-f...

Good article about finding a support group, Joan.

The longevity for PH and PAH continues to increase! And there are medications to manage this disease much better. Of course, th underlying cause can have some effect! But, I just keep plugging along 😀👍👍💪😀

@A myPHteam Member I am sorry your doctor got annoyed with you for asking about the side effects, but you are very lucky to have a very good doctor who understands the disease and know how to treat it. I would trust the doctor and allow her to treat you and see how it goes on the meds. I had some trust issues with my doctors and meds as well as my doctor prescribed something I had a severe reaction to before. It turns out that I can tolerate that medication now, even though I could not before. My pulmonary pressure was 41 on the first echo I had. My treatment was not typical for PH and also my doctors seems to not all agree on if I have PH or not which is confusing, however my cardiologist put me on amlodipine to treat it and my pressure came down to 27 about 2 months later and down to 17 when I was recently hospitalized for stroke symptoms (was recrudescence from old stroke). Like Christine said, the medication can def increase life expectancy and getting your pressure down is def a priority. Some have pressures as high (or higher) than 100, but as I understand it, the higher the pressure, the harder your heart needs to work and doing every thing you can to get it down will help your body the most.

Mentioned Users

A myPHtea...