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janey

PH (Pulmonary Hypertension) and Scleroderma

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PH (Pulmonary Hypertension) and Scleroderma.

 

In scleroderma, 10–15% of patients have evidence of pulmonary arterial hypertension and another 10–15% of patients have pulmonary hypertension as a complication of interstitial lung disease and/or left ventricular dysfunction. Pulmonary Hypertension Society. 2010. (Also see:Pulmonary Hypertension)

 

This item was posted in the ISN Newsroom. Please check the newsroom daily for updates on scleroderma and other related articles.


Janey Willis

ISN Support Specialist

(Retired) ISN Assistant Webmaster

(Retired) ISN News Director

(Retired) ISN Technical Writer for Training Manuals

International Scleroderma Network (ISN)

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This article was very interesting to me as a right heart catherization in January 2009 showed mild PH (39 with a mean of 25). Both my cardiologist and pulmonologist said no treatment at this time but my scleroderma specialist wanted to put me on Letairis (she said with scleroderma you should treat it at 20). I applied for the LEAP program, which you have to do to go on the drug, and then the transplant came along and my nephrologist said no new drugs. I was very worried about the side effects and cost so that was fine with me.

 

I just had an echo and it showed no signs of PH. I showed it to my pulmonologist who said if I was a "normal" person (without scleroderma or a new kidney) he would treat the PH. I have to make an appointment with my cardiologist and see what he says. I may need another right heart cath. My breathing has greatly improved, but I do breathe heavily when I lay down in bed at night which is a symptom.

 

Thanks for posting this.

 

miocean


ISN Artist

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Count me in, I have postcapilliary pulmonary hypertension as a result of left ventricular global dysfunction. No treatment has been mentioned yet.

 

Take care.


Amanda Thorpe

ISN Sclero Forums Senior Support Specialist

ISN Video Presentations Manager

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(Retired) ISN Sclero Forums Assistant Manager

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International Scleroderma Network (ISN)

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I'm one of that low percentage. my right heart cath showed PHT with exertion and after being involved in a study group for Ambrisentan (letairis), it appeared that my PHT was improved by use of the drug. Amazingly enough, after the study was finished, my insurance company actually approved continued use of the drug, notwithstanding the fact that usually it is only given if PHT is present at rest (mine is only present with exertion). It is an extremely expensive drug, but it seems to help my shortness of breath. I also have pulmonary fibrosis, secondary to scleroderma. We just recently took a trip to Africa, and I did just fine. Not sure if that's thanks to the letairis, but I'm certainly not discounting the possibility. I have done better on my 6-minute walks since starting the drug, although my breathing tests have not shown any improvement (still about 60% of normal lung function). But, hey - I haven't had to go on oxygen, so I'm not complaining!


Sharon T.

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Sharon T,

I'm glad to hear that this drug is helping you with your breathing issues. Any thing that helps us to breath easier with less effort is a good thing. I sure hope this drug continues to work for you.


Strength and Warmth,

Sheryl

 

Sheryl Doom

ISN Support Specialist

(Retired) ISN Chat Moderator

International Scleroderma Network (ISN)

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I'm on Letairis and have been for several years now. (They tried me on calcium channel blockers first and I immediately developed horrible lower extremity edema - I couldn't get any shoes on at all.) I'm having some progression now, but it certainly did improve things. I could almost pinpoint the day it kicked in! My insurance company didn't fight too hard about it (probably because I failed the CCB's), but I do have to get it via their own specialist pharmacy. My co-pay is very reasonable ($25), but if I were to pay for it out of pocket, it would be $6000. :sick2: My rheumatologist told me the other day that I am "living proof that early intervention is very important."

 

Never mind world peace, wouldn't it be wonderful if our doctors could just agree?!


Jeannie McClelland

(Retired) ISN Director of Support Services

(Retired) ISN Sclero Forums Manager

(Retired) ISN Blog Manager

(Retired) ISN Assistant News Guide

(Retired) ISN Artist

International Scleroderma Network

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