Living With PH and Connective Tissue Disease

[Judie]

Hi Coleen,
I have a co existing diagnosis of Ehlers Danlos Classical Type. I do believe this is the root cause of my PH. I’ve had many prolapsed organs, hiatal hernia etc. I originally sought diagnosis when my daughter (now 33) was 19-20 and having odd joints sublax or dislocation. Ribs, sternum etc.
When you live in your body as you see normal you don’t always realize it’s different. In retrospect there were many clues and symptoms they were just never connected. It is a genetic connective tissue disorder.
I was required to get echocardiogram yearly to assess my heart and major blood vessels around it. It was this yearly screening that picked up my PH last year after many years of normal echocardiogram.
Judie

[Brittany Foster]

Judie,
I know some friends who also have EDS. I’m sure this didn’t help with the PH and the symptoms you were getting. That period of time where you are just being tested and running through test after test is so frustrating. Especially when things aren’t giving an explanation for what may be going on. But I am glad that they were finally able to figure out what was happening and help you get explanation for your symptoms. In a way, that must have been a relief for you especially because it helped you get some treatment for it. Does your daughter also have EDS-hypermobility? Were you genetically tested? I also have genetic conditions so went through a lot of genetic testing myself for other conditions that came back positive.

[Colleen]

@jady1958 you have been through a lot! I’m sorry that PH was added to your already complex medical conditions. It’s a blessing thought that it was diagnosed so that you can be treated and hopefully feel some improvement in symptoms. If I may ask, how has your doctor proceeded in treating your EDS and PH together?

I can relate to what you said about what you see as normal and don’t realize it’s different. For example, it’s been mentioned previously in our forums how a PH patient might not realize how much they need supplemental oxygen because they are so use to living with limited breathing ability. I can see this being similar to your experience with EDS and PH.

[jen-cueva]

I wanted to share this educational opportunity with those with connective tissue disease(CTD) and PH. PHA offers several videos as part of their PHA Classroom. I added the first one in the series so you can view it at your own pace.

Feel free to watch the videos and let us know your takeaways.

Did you learn anything new? Will you ask your healthcare team about what you’ve learned?

[jen-cueva]

Hi @Sofia, I wanted to tag you because you mentioned PH and MCTD. Several other members also manage PH with MCTD and other autoimmune disorders.

However, I’m sorry you are having more bad than good days. Have you discussed this with your PH team and Rheumatologist? Maybe they can offer some tweaking of your medications. In the meantime, try to pace yourself, taking small breaks between tasks and activities, if possible.💜🤗

[sofia]

Thank you. It’s been a difficult year for sure. I work closely with rheum, pulm, & cardiology. By the time I got diagnosed I was already in chf w a pericardial effusion. 10 meds in and trying my best to do everything I can to be around for my family.

[jen-cueva]

Hi @Sofia, bless your sweet heart. I, too, was in CHF when diagnosed. But, thankfully, almost two decades later, with a ton of meds, as you mentioned, my CHF is pretty stable.

I hope you, working closely with your healthcare team, are also feeling your best. Have they discussed trying any of the two newest PH treatments for you? As you mentioned, I admire your resilience and hope of being here for your family. Keep that motivation, my PHriend.

[carol-volckmann]

My PAH was caused by Sclaraderma CTD. While my PAH has remained stable, Sclaraderma continues to cause many health issues: enlarged spleen, enlarged liver, cirrhosis, skin hardened, total bowel inconvenience, small bleeds internally… and more. Nothing seems to help. I thought when I was diagnosed with PAH nearly 19 years ago, that was eventually going to take me out – Sclaraderma will probably be it, but we will just keep up the good fight and try and stay on top of it. I have a good team and they are resisting adding more meds (poisons as they call them) till absolutely necessary. I am 80 and plan to keep on going and see all the joys and laughter I can enjoy and add to!

[jen-cueva]

Hi, Carol Volckmann. Thank you for sharing your experience with PH and scleroderma with Sofia. I’m sure she will also appreciate hearing from you and seeing how well you manage both with an excellent care team! 💜