Through a right heart catheterization I had today, it was determined that I have PAH. This past winter, I struggled through SSB1, and got into SSB2 but was unable to continue, as I was losing ground with the progression of this chronic disease. I’m going to see a specialist within about a week, who will come up with a treatment strategy. Anybody here have PAH, or know someone who does, or did? I’d love to hear your experiences.
My Mother-in-law, currently deceased, life long smoker had PAH in addition to COPD. It can be controlled/slowed. My quick perusal of the literature seems to indicate that the progression is very slow. Can’t comment on the effect of exercise.
Sorry to hear about the diagnosis. But I’m guessing now that the diagnosis explains for your struggling through SSB1 and SSB2.
Pulmonary Arterial Hypertension, like most diseases is separated into degrees of severity. Based on how elevated the pressures are (or how they increase with exercise). With regards to causes; they are largely spilt into 5 groups. Causes of left heart elevated pressures or primary lung conditions (like COPD or pulmonary fibrosis) account for the majority. But there are various other disorders that can be related to PAH. Sometimes no direct cause can be found (in this case it’s termed idiopathic PAH). Right heart Cath, is the gold standard for not only diagnosis but also indicating cause of PAH. Treatment strategy will be
Its good to hear you’re seeing a specialist about it. Because it is a very specific area of medicine and in particular cardiology and pulmonology with many specialists sub specialising in PAH. There are a number of medications (viagra being one) directed at reducing pressures and improving symptoms. But this all depends on what your specialist advises based on your results and functional limitation.
One piece of advice I would have is to make sure you are armed with questions regarding exercise and expectations i.e what heart rate zones you can work out at and for how long. And how these align with your expectations. It is important to be in the same page with your physician regarding this. As PAH will alter your cardiovascular haemodynamics, and how you respond to exercise etc. PAH is a significant and chronic medical condition and it is important to seek specialist advice before undertaking any training programme etc.
Hope this helps.
(Just as a disclaimer this shouldn’t constitute or replace any specialist advice, it is is merely generic advice to help you formulate questions for your specialist)
I couldn’t agree more with these comments by @phillycheese.
I’ve been caring for patients with PAH in a community setting for a few years and would offer this bit of additional advice:
Seek care at an academic or very large institution with someone who only sees patients with this condition. Performing and interpreting the right heart cath correctly is not as straightforward as some think. Further the marketing from drug manufacturers in this field is crazy and I see a lot of community docs treating far outside what the scientific evidence and guidelines recommend.
If drug cost is a concern (assuming you live in the US) then know that there are good generic options.
Finally, don’t be surprised if your doctor tells you to take more than one medicine for this. Trials have shown that early intervention with multiple agents is better than adding more medicine along the way if things get worse.
My full support & understanding for you. I was recently diagnosed with some crazy heart abnormalality. It really throws a spanner into your life. Do whatever it takes to make sure you live, then think about the bike.
I hadn’t checked back on this thread for a couple of days, and I was pleasantly surprised to find your thoughtful responses. These last few days have been a bit of an emotional roller coaster, and I still have not been able to get an appointment date with the specialist down in Portland (Maine). I am so eager to get on with treatment, I’m about to blow a gasket. I may seek a bigger institution in Boston, or perhaps Baltimore, as I have family there.
The cardiologist who performed the catheterization and the pulmonologist who referred me to him, both considered this an idiopathic case, but admitted that it may well be related to radiation treatments that I had for Hodgkins disease back in 1993, when I was 20.
So I’m still riding my mountain bike, though I’m a shadow of what I was even just last summer. When I exercise, I am winded even at low levels of exertion, so I take a lot of short breaks, but I seem to recover pretty quickly. I suspect I’m not very far along in the progression of the disease. I just don’t have much of a sense of what I might expect from treatment. Will I regain much of what I’ve lost, or will I do well just to slow the decline?
Thanks everybody for hearing me. At this point, I have no one but the internet to bounce ideas off of, at least not anyone who knows anything about PAH, or even anyone who has heard of it!
I have to strongly agree to seek care in Boston or Baltimore, both of which have world class medical centers. ( Johns Hopkins. Medicine class of 1980)
Soooooo…Today, I went to a specialist, who by looking at the results from previous tests, and seeing that I had a splenectomy in my past, said he thought that I likely had Chronic ThromboembolicPulmonary Hypertension. He sent me across town to have a Ventilation Profusion scan to confirm it, and indeed, that’s what it is. He prescribed some astonishingly expensive meds (Adempas), and is sending me to Mass General to see a more focused specialist , who will likely want to perform a pretty big deal surgery. The good news is that they can likely cure the disease, and I can be just like normal. Bad news is that they have to open my chest up to do it. My head is spinning.
Surgery is definitely the way to go for that condition. UCSD has the most experience with the surgery nationwide. Duke has done it quite a bit as well and has a good safety record. I’m sure a center like Mass General has a great program.
Good luck man. Message me if you have other questions about it.
Scary shit. But…in a couple of years you’ll be back to smashing out PBs on the bike. And it’ll probably drastically change your perspective on life in general.
About the only advice on the matter I can give is move quickly to get your post-op support network set up for both your physical & mental health. As always, the gang on the TR forum are always super positive and supportive (and crazy knowledgeable!).
(As an egocentric side note, I’m still waiting — 3 months now — for the heart specialist to look at my file; at least you know what your situation is. As I like to say about the universal health care in Canada — crappy but free!)
Heal up & see you back here soon!
Thanks a lot. I’m kind of floored to get a response on here from someone who has real knowledge of this situation. Very comforting.
Ha! Well I can help with lungs! Hips and knees on the other hand… not so much! Saying a prayer for you man. Hang in there.
Lalalalalalalala, I can’t hear you! As a very liberal American,I don’t want to hear anything negative about universal healthcare. So far, my health insurance is playing along very nicely, but I’m scared to death that the rug might get pulled out from under me at some point. That must be very frustrating to wait so long. Aside from being off of the bike, how is your quality of life during all of this?
Yup, super duper frustrating to not have a two tier system. Guess that’s why Canadians go to the US…and Americans go to Mexico.
Quality of life is great, everything is 100% normal when I’m chillin’, I just can’t do any kind of intensity…unless I start taking meds, which just mess you up in a different kind of way.
I’ve just started doing the MAF Method training which, for me, is basically ~70% HRmax/Z2 power — All. The. Time. Keeps me busy whilst I wait for Dr. Feelgood to return from his summer vacation.
Best of luck to both of us!