As my grandmother used to say when we were looking for something and finally found it: "it's always in the last place you look!" It didn't dawn on me until I was an adult that her statement was in jest: I just thought it was funny that it was true- you always found something in the last place you looked- what a coincidence!
Such was the situation with figuring out the somewhat confusing results of the Level 3 Baird test I had last August. After seeing several different specialists to interpret the results, all of whom said they really couldn't make heads nor tails of it, I finally found someone who told me that it made perfect sense to them. And yes, there is a diagnosis!
After my initial response of relief and and happiness that *finally* there is an answer; a name for this, and treatments, the reality started to sink in and I felt my spirits settling into a funk. Another serious, chronic illness to contend with. It's true that even with CF there are new symptoms from time to time and things I haven't yet dealt with, but for the most part, it's something I'm very familiar with. I have a lot of learning to do in order to bring myself up to speed with this latest issue.
I have a mild case of pulmonary hypertension, also known as exercise induced PH. The reason that none of the other specialists were able to figure this out is because it's rare and a very small specialty. Most physicians, even pulmonary and cardiac specialists, aren't familiar with it. Only a PH specialist would be able to interpret the results and make the diagnosis. So there is is, at least we finally know what it is and it can be treated.
I asked if this is secondary to the CF lung disease or a separate entity altogether and the answer was: "It's difficult to know". Obviously, the lungs and heart as well as the major vessels that oxygenate the blood are all very closely connected and what affects one part of the system will affect the others. However, this doesn't appear to be as a result of CF, but more something that is occurring in conjunction with it. Will it get worse? There isn't an answer for that either. Some literature indicates that exercise induced PH is an early or mild form of PH, but Dr C said his opinion was, after having treated patients with this for 20 years, that it is a milder form and may never get any worse. No guarantees, of course.
The treatments are oxygen and some oral, inhaled and IV drugs. At the moment I'm only requiring O2 with exercise so am doing all I can on that front already. There is a drug, sildenafil, which helps some patients to feel better and lessen the symptoms of fatigue and shortness of breath - more commonly known as Viagra- which they are going to try me on. As the NP said, I don't really want to know how it was discovered that Viagra helps patients with PH, but there is clearly potential for a very funny story there. Drug companies being the considerate entities that they are, the brand name has been changed to Revatia when used for PH. Thank goodness I don't have to walk into CVS to hear them announce: "Your Viagra 'script is ready!". At least there is that.
The PH clinic nurse has pointed me to the PHA, which has a very helpful website with lots of teaching materials and information for the newly diagnosed. With the snowstorm which is coming for this week it looks like I'll have plenty of time indoors to get myself up to speed. I'm fortunate that chronic illness isn't something foreign to me, but as with anything new, it's going to take some adjustment and a bit of time for me to come to terms with this latest bit of information.
