When medical professionals are trying to determine the cause of medical symptoms, they start by ruling out the most obvious causes. I suspect that many rare diseases aren't rare but underdiagnosed. With medical advances in testing, diagnosis, and treatment many rare diseases are becoming more treatable and longevity statistics are changing. Unfortunately, at this time the majority of those diagnosed with any form of PH are diagnosed later in life after it has done serious, irreversible damage.

I am "blessed" with two rare, incurable medical conditions, PAH and Sjogren's. My PAH is caused by Sjogren's disease. If you look on the internet or most other resources, Sjogren's is considered a nuisance disease that primarily causes dryness. This is untrue. Most medical professionals are not trained to properly diagnose and treat Sjogren's. Like it's sisters in the systemic rheumatic autoimmune disease family (RA, Lupus, and Scleroderma), Sjogren's can impact every organ in the body, but especially the pulmonary system, much in the same way as Lupus does. Few doctors know that Sjogren's is the second most commonly diagnosed rheumatic autoimmune disease after RA. Yet little research or medications have been developed specifically to treat Sjogren's. Most of the medications used to treat Sjogren's are used off label. As awareness increases, so does research and medication development.

PAH awareness and research has grown tremendously in the last several years. There are new advances in how the disease is treated and managed. Thus, life expectancy numbers are slowly increasing. Of course, this depends on how quickly PAH is diagnosed, how much damage has been done, how aggressively PAH is treated, and how well one tolerates the medications. Currently, PAH is treatable to a certain degree, but that treatment comes with a hefty price tag.

Hi Rachel. The literature says 1 in 10 people over the age of 65 have been diagnosed with PH. PAH is more rare with an estimated 50-100 people per million being diagnosed. It seems like doctors are becoming more aware of the disease so people who were being misdiagnosed in the past are getting the correct diagnosis now. I hope that helps.

PAH is more common in women;the symptoms are mostly fatigue,shortness of breath so easily unrecognized as very common symptoms.I,my self did not recognize it as a disease as I was busy caring for 3-4 years for my I’ll husband and who passed away in 2023.Thought then I was depressed.Finally told my PCP how sob,and turned I was a echo confirmed PAH compared to echo from 2019..
Autoimmune diseases like lupus,Sjogren’salso more common in women.
There is no cure but life expectancy and quality of life much improved the last few years with new meds.But Diabetes and heart disease are also chronic illnesses.
let us all hope we benefit from these new treatments and our support group…Flora