21 posts in this topic

Ronald, sorry for the delayed response but I was away for a few weeks.

 

I have pulmonary fibrosis with interstitial lung disease. I also have pulmonary hypertension. My 2013 CT scan showed the beginning of honeycombing so I started the lung transplant process but 2 centers turned me down due to the dismotility of my esophagus. My 2015 CT scan stated there was no longer ground glass which my pulmonologist said means there is no longer inflammation. I declined tremendously on my 6 Minute walk test this year and my pulmonary function tests went down as well. My lung capacity is 38%. My diagnosis is end stage lung disease. I currently use oxygen at night and for exertion. I take Adcirca for the pulmonary hypertension, diagnosed 2 years ago.

 

I was diagnosed in 2004 at the age of 51. One of my earliest lung symptoms was my lungs would get tired when I ran. I have been on and off oxygen over the years but am very frustrated right now because I am shorter of breath than ever before, even with the oxygen. There has been no change in my CT scan since 2013. My Echo was "fabulous" according to my pulmonary hypertension doctor. My right heart cath showed my mean pressures to be 26-30 with a peak at 40 (over 25 is PH) so mine is minor. I showed no improvement with 100% oxygen or nitric oxide during the cath. My PH doctor can't explain the increased SOB and told me to manage my blood pressure. She is not making any medication changes. My cardiologist said my heart is good and my blood pressure was 110 over 70 although it sometimes goes up to 150. He said I am not in heart failure. I take metoprolol for high blood pressure. I see another pulmonologist this coming week so I will let you know her take on things.

 

It is very frustrating when I hear the "I don't know why's" from the doctors regarding so many issues with scleroderma and I go to some of the best specialists in the NY area. I cautiously have high hopes for the pulmonologist I see next week, she is a new specialist for me. I don't expect a cure but I would like an explanation. They keep on telling me things like I have a long time to live, that having a lung transplant would be trading one disease for something worse as life expectancy after transplant is not that long, that in 5 years things can change in the medical world, etc. 

 

In the meantime, I live a very full life, travel as much as I can and do the things I love to do. I have a portable oxygen converter that makes things much easier. I hope I've answered your questions and reassured you that you can live a long while with lung involvement and beat the statistics you will read on the subject. I'll let you know what the pulmonologist tells me this week.

 

miocean

 

 

 

 


ISN Artist

Share this post


Link to post
Share on other sites

Update: The pulmonologist had nothing to add. She said that sometimes changes are occurring at the microscopic level and don't show up right away on tests. I am to make sure I use the oxygen, exercise, and lose 10 pounds. I currently use a portable oxygen concentrator that has a pulse flow; I may need to change to one with continuous flow. I am to repeat PFT's and 6 minute walk testing sometime in the fall.

 

miocean


ISN Artist

Share this post


Link to post
Share on other sites

Hi Miocean,

 

Well, no news is good news with Scleroderma (my rheumatologist said he liked nothing more than to see a "boring" ECHO, with everything normal!! ;) ) and your pulmonologist sounds as if she's on the ball, so hopefully, things will stablise a little for you.

 

Best wishes,


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 Miocean,

 

Thank you for the update on your pulmonary visit. What I couldn't really tell from your last post is whether or not you were entirely happy with the results of that visit?  I know you had high hopes for it, and I often find myself crushed after getting up my hopes, for nearly anything. 

 

Did you feel satisfied with the care, and explanations?  I absolutely agree that many changes (both good and bad) occur that don't show up on tests, either right away, or ever.

 

I've even had times where I've been feeling and functioning a lot better than usual, but my test results look the same or even worse. That makes it really hard to weigh my perceptions against reality, although if I'm feeling better, it's a very hard sale for anyone to convince me I'm actually worse, and perhaps vice versa, too.

 

: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 Shelley,

 

Well, I was disappointed. She is not a magician. I would have liked more "outside the box" thinking but as I've learned from years of dealing with this disease, no one really knows...

 

miocean


ISN Artist

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