Jump to content
Sclero Forums
jlang

Meds for PH

Recommended Posts

I haven't been officially diagnosed with scleroderma or pulmonary hypertension, but have a positive SCL-70 and mild PH per echos.   The echo indicated 43-48 PH pressure last time I went.  It's been fluctuating from 37-55 for the past 10 years.  I haven't had the right heart cath so I don't know for sure.

 

At what pressure reading would they start treatment? What is considered moderate or severe PH?

Share this post


Link to post
Share on other sites

Hi,

 

It won't hurt to remind you, and possibly others who are researching on our site, that Scleroderma is not the only condition which can cause Pulmonary Hypertension.

 

In the early days of my diagnosis of Scleroderma, and incidentally 37 years at least after the first onset of Raynauds, I had an Echo which showed mild PH.  

 

After a few years, I had a consultation with a Gastroenterologist regarding possible treatment for bowel involvement.   His announcement that I had PH anyway and there was no cure for that so no point in exploring anything else, sent me into a flat spin - to say the least.   After a few weeks, the casual remark from somebody that I needed a Cardiologist set me off on another track which ended up with the PH being identified as caused by Mitral Stenosis as a result of Rheumatic Fever which I was not aware of ever having.

 

The Mitral Stenosis was repaired without too much fuss thankfully and now I have NO PH.

 

I see that you have a positive SCL-70 but you probably know from researching here that not everybody goes on to develop Scleroderma.   So what I am saying is that it would be a good idea to make sure that nothing else is going on which could cause mild PH.

 

Best wishes

Judyt

Share this post


Link to post
Share on other sites

Hi Jlang,

 

Please keep in mind that I have no medical training, and ask all these questions of your pulmonary doctor as well.  The way it usually works is that if they suspect pulmonary hypertension, you are referred for a right heart catheterization. During the cath, they run several tests, to see if you are a "responder" or a "nonresponder".

 

Initial treatments can be as simple (and cheap) as a diuretic, and, if you are a responder, a calcium channel blocker.  It can get more complicated, and tremendously more expensive, as the illness progresses.  The most important thing is to undergo the right heart cath if your doctor recommends it.

 

:emoticons-group-hug:


Warm Hugs,

 

Shelley Ensz

Founder and President

International Scleroderma Network (ISN)

Hotline and Donations: 1-800-564-7099

 

The most important thing in the world to know about scleroderma is sclero.org.

Share this post


Link to post
Share on other sites

Hi Jlang,

 

I would reiterate Shelley's advice about having a right heart catheterisation to determine whether or not you are suffering with Pulmonary Hypertension. I've included a link to PAH for you to give you some more information.

 

Kind regards,


Jo Frowde

ISN Assistant Webmaster

SD World Webmaster

ISN Sclero Forums Manager

ISN News Manager

ISN Hotline Support Specialist

International Scleroderma Network (ISN)

Share this post


Link to post
Share on other sites

Hi Jlang,

 

I was recently diagnosed with PH via right heart catherization where part was done during exercise (I had to lift saline bags over my head.) Based on a wedge pressure of 30 under exercise I was started on medication and a diuretic. The diuretic set all my labs off for my kidney transplant anti-rejection medications so I had to cut back on it. 

 

I am currently undergoing testing for a lung transplant and saw a PH specialist. A right heart catherization will be repeated but this time it will be done with a chemical to induce stress. I also am having multiple tests done on my lungs, heart (including a left heart catherization,) and my GI system. 

 

With your pressures under Echo higher than normal, has your doctor ever suggested a heart catherization? Are you short of breath at all? If so, is it when you are sitting still, moving around, or laying down at night?

 

Judyt, it is interesting to me that what was thought to be PH ended up being something else. I asked the PH specialist if she would be my PH doctor and she said, "that depends on if you have PH or not." She also said she had an idea of where the transplant doctor was heading with all of the testing. Before I read your post I had the thought that perhaps it might be something else. 

 

miocean


ISN Artist

Share this post


Link to post
Share on other sites

Hi mio and Jlang,

 

Well, I think we are verging on being pedantic here!!!   I did have PH for a while, it was real PH but it turned out that it wasn't caused by my lungs.  

 

Instead it was caused by Rheumatic fever damage to my mitral valve.  

 

That type of PH is very treatable, and in my case I no longer have PH (as far as I know because I haven't had an Echo since 2010).

 

Judyt

Share this post


Link to post
Share on other sites

Create an account or sign in to comment

You need to be a member in order to leave a comment

Create an account

Sign up for a new account in our community. It's easy!

Register a new account

Sign in

Already have an account? Sign in here.

Sign In Now

×