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Interstitial lung disease and Scleroderma


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#1 Ron

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Posted 02 June 2015 - 07:08 AM

I have been researching pulmonary fibrosis in scleroderma.  I have found out some encouraging information regarding pulmonary fibrosis in people with Scleroderma, I am talking about Interstitial lung disease and scleroderma.

If you research it, do not read about “idiopathic pulmonary fibrosis.”   This is completely different lung disease and has a very poor survival rate.

People with scleroderma and lung disease can have lung involvement and live for many years, unlike idiopathic pulmonary fibrosis (IPF).  
                        
So if you have lung disease and scleroderma, only research it in patients with Interstitial lung disease with scleroderma.

Lung disease is still the leading cause of death in scleroderma.  But remember its not as bad in many cases like idiopathic pulmonary fibrosis.
                    
I only touched on Interstitial lung disease, and not pulmonary arterial hypertension.  I don’t know if I have that, will know more when I get my echocardiogram.

 

 I have Interstitial lung disease, and its test outcome was moderately restricted defect, according to a PFT back in Jan 2013.  I am not so worried now as I was before.  Educate yourself, but don't self diagnose.



#2 Joelf

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Posted 02 June 2015 - 07:13 PM

Hi Ronald,

Thanks so much for your helpful post.

You're absolutely right about different types of Pulmonary Fibrosis. I have Interstitial Lung Disease and the patterns of the inflammation (ground glass) on my lungs was indicative of Scleroderma induced non specific interstitial pneumonia (NSIP), which thankfully is not the same as Idiopathic Pulmonary Fibrosis and it did respond very well to treatment.

I'm intending to survive for many years despite this (unless I get run over by a bus etc.! :wink: )


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#3 Ron

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Posted 03 June 2015 - 05:36 AM

Thanks Jo, when I first was told I had lung involvement I researched idiopathic pulmonary fibrosis. That scared me a lot. It was not until I came here that I found out that lung disease in scleroderma is not exactly the same. It can develop, then stop progressing for years.

I can't wait until I get my echocardiogram and PFT, I want to see how much I deteriorated since January 2013. I do get short of breath now, that just started about two months ago. I think I may have heart involvement as I get erratic heart beats. A ECG done 2 weeks ago came back abnormal, it was faxed to the heart doctor.

I get palpitations and they are really getting bad, I have had them for 20 years, the heart doctor said they were nothing to worry about, but now they are really getting bad and I get dizzy with them now, but never before. So this is the month for most of my tests and doctor visits.

I just want to know what's going on, and its frustrating being in the dark. I am more worried about my heart than my lungs at the present.

Ron

#4 judyt

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Posted 03 June 2015 - 01:59 PM

Hi Ron,

 

As you say you have some odd things going on with your heart rate I thought I would give you the benefit of my knowledge :wink:  Which is just about nothing but I do have some experiences.

 

When I was first diagnosed in 2003, nearly 40 years after my first symptoms started, I had the usual Echocardiogram.   The question then arose "did you know you have had Rheumatic Fever"?   Of course I didn't but what had become obvious was damage to my Mitral Valve which I was assured was Rheumatic Fever related.

 

Over the years prior to my diagnosis I had been experiencing weird happenings with my heart which were always put down by the Doc as palpitations not worth worrying about.

 

What eventuated over the following few years was the discovery that I had a grade one bundle branch block which was causing the erratic heart beat.   That was diagnosed as extra beats, once again not worth worrying about but when it started to happen for nearly a whole day I was given medication which helped a lot.

 

Now I am 6 weeks post op. having had a Mitral Valve Replacement and am on a different drug to regulate my heart rate.   I don't think I will be on that one long term but am due for another echo next week followed by a consultation with the Cardiologist, have to wait and see what that showsl

 

Just as you have described I had episodes of thinking I was having a stroke, nearly fainting and times when I just had to sit down and wait until my heart settled down.

 

I am grateful that there is so much knowledge now about heart problems, and because with all the other problems I do have, it was decided to offer me the Valve Replacement surgery to try to improve my PFT results.   I do know that I don't seem as short of breath as I was so, like you, am keen to find out what is really happening.

 

Best wishes for your next tests.

Judyt



#5 Ron

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Posted 04 June 2015 - 04:41 AM

Hi Judy,

I never had Rheumatic Fever.  I do have Mitral Valve Prolapse, this was ruled out as not serious many years ago, I would say at least 25 years, as well as the palpitations about 20 years ago.  But now there is something funky going on with my heart beats, I get really erratic beats, my family doctor says it's anxiety, I do have really bad anxiety problems, but this new thing with my heart is not from being anxious. I get skipped beats, every 2 - 4 times my heart beats, it's off and on throughout the day and dizzy spells.  No pattern to what makes it start or stop.

There is something going wrong.  I see a heart specialist June 23, and my PFT on the 25th, and I am not sure what the heart specialist has in store for me.

I just want to get in there and figure out what's going on with my heart.  I know that my PFT will be worse than my last one, but at least I will know where I stand.



#6 Shelley Ensz

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Posted 06 June 2015 - 10:45 AM

Hi Ron,

 

As it happens, it's not only the very serious heart and lung problems that can cause symptoms like this. I have had orthostatic intolerance for decades.  It can be part of neurological involvement in systemic sclerosis.  See: Neurological Involvement. 

 

It can get very confusing when there is more than one thing going on, as there usually is in scleroderma. Unfortunately, it usually takes many medical tests to pinpoint the cause.  I felt like a guinea pig extraordinaire before they pieced together the basics for certain symptoms in my particular case.

 

I think you'll just find it good to know, during the waiting-for-answers stage, that there can be symptom causes other than worst-case-scenario-organ-involvement.  It's certainly no walk in the park to live with orthostatic intolerance, or orthostatic intolerance along with other organ involvement and symptoms of scleroderma.

 

But I think most of us can adjust to anything we know we have; it is most often the uncertainty that has us climbing the walls, isn't it?  I hope you get some solid answers soon.

 

:hug-group:


Warm Hugs,

Shelley Ensz
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#7 Ron

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Posted 07 June 2015 - 04:13 AM

Shelley,

 

Oh no, more stuff to worry about.  :dont-know:

 

Ron



#8 Shelley Ensz

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Posted 10 June 2015 - 05:23 PM

Oh dear. That made me laugh! Then I started to worry. Like maybe you were serious! Which I'm sure you were. But you meant it to be funny, right? Oh I sure hope so or you're just going to hate me, because now I'm coming down with the giggles. Often the guffaws aren't far behind!

We can laugh at ourselves and our predicaments, right? Oh I hope so. Yes, I just reread all the guidelines. It appears we are welcome to laugh, but at our own risk.

Thank you for the laugh, Ron, and please accept my sincere apologies if you didn't type that with a little twinkle in your eye.


Warm Hugs,

Shelley Ensz
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Hotline and Donations: 1-800-564-7099

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

#9 Ron

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Posted 11 June 2015 - 03:30 AM

No worries Shelley. :happy:

#10 Amanda Thorpe

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Posted 13 June 2015 - 02:32 AM

Hello Ron

 

I just read your post, I would hope that an ECG or ECHO (as appropriate) would be carried out if someone said they're having palpitations/arrhythmias especially if said person has scleroderma whether they also have anxiety or not. It seems anxiety is the easy out for doctors. Exactly how did the doctor determine that your irregular heart rhythms were anxiety and not the scleroderma affecting your heart I wonder?

 

To cut a long story short, I went from being told a *BBB can be found in the general population to myocardial fibrosis with serious heart failure with (then) 2 year life expectancy within a 14 month period. I am of the opinion that the initial heart involvement should not have been dismissed as it was.

 

I am relieved to know that you have an upcoming appointment with a cardiologist, the heart symptoms may be anxiety but then again... At least you'll know for sure!

 

Take care.

 

*Bundle Branch Block (left ventricular to be precise)


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#11 Ron

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Posted 13 June 2015 - 02:40 PM

Amanda, I do have left anterior fascicular block (LAFB).  The doctors and I dismissed it.  Even the specialist to whom I faxed my ECG.  This ECG was taken in 2012.  I had one last month, no LAFB.  My heart has settled down, I don't know why. 

 

See: Routine EKG finding could signal serious heart problem.

 

I have lots of questions for the heart specialist.



#12 Shelley Ensz

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Posted 18 June 2015 - 06:41 AM

Ron, that's rather curious. Did your doctor explain how it could have gone away, or if it's usual for that finding to appear and disappear? 

 

I have an obvious T-wave abnormality but sometimes they just don't report it out, although normally they do. So if I were you, I'd want to make sure that someone has verified the reading by looking at the graph, and not just the report.

 

ECG's are not entirely foolproof. I had one at a major clinic a few decades ago, and they said the results showed I had had a massive heart attack, with 1/4 of my heart severely damaged. I just laughed, as I doubted I'd still be coherent and upright if that had just happened. And it had not, of course. The problem was in the placement of the leads, and re-testing showed that it was the same as usual.

 

But the thing is, they did re-test right away, so we knew what was wrong, and thankfully it was only technical problems and not actual heart problems.

 

:hug-group:


Warm Hugs,

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

#13 Maz White

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Posted 18 June 2015 - 04:31 PM

Good luck to all you people out there with interstitial lung disease.  My husband lived for three years after diagnosis of interstitial lung disease with his Scleroderma.  He had Scleroderma for 13 years until his rapid decline within 6 months.  He has been gone 10 days now and I miss him but he couldn't live like that



#14 Ron

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Posted 19 June 2015 - 05:41 AM

Maz White, I am sorry for your loss.  It's hard to predict the course of the lung disease.  Just have to keep hope that it will be a long time before the lungs fail.



#15 Ron

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Posted 06 July 2015 - 02:50 PM

Got my results back from the PFT. Didn't say much. Whole bunch of numbers that I cannot figure out.

Lung volumes suggest restriction (both TLC and RV are decreased). The diffusion capacity is reduced. Restriction with low diffusion capacity. O2 saturation is normal.

I will have to wait until I see a lung specialist to get an idea where I am at with this test.

#16 Shelley Ensz

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Posted 06 July 2015 - 05:20 PM

Hi Ron,

Those tests definitely need interpretation, don't they? It sounds to me, with my dire lack of medical training, like pretty standard findings with scleroderma lung involvement.

By normal sats, do you mean entirely normal normal (at rest), or just 88 or above? (The cut off for oxygen supplementation.)
Warm Hugs,

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

#17 Joelf

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Posted 06 July 2015 - 08:01 PM

Hi Ron,

Thanks for the update on your PFT's.

I hope that it's not too long before you're able to see your lung specialist and that he will be able to explain your lung function tests and advise you on the best treatment.

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#18 Ron

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Posted 07 July 2015 - 03:45 AM

Thanks for the reply Shelley and Jo,

My O2 was 92, they could not get a good reading as my finger circulation was poor. I have always had problems with those devices on my fingers, never can get an accurate number.

#19 Shelley Ensz

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Posted 07 July 2015 - 04:54 AM

Hi Ron,

I've had pulse oximeters (and even wrist blood pressure devices) not work during an attack of Raynauds. It can definitely be a problem getting accurate readings with circulation issues.

It's important to make sure you, and your hands, are warm and not attempt any readings during a Raynauds attack. You can also clip the oximeter on to an ear.

That said, I also get Raynauds in my ear lobes, so ear readings might also not be entirely reliable for everyone all of the time.

I had Raynauds after a day surgery a few years ago, which is nearly unavoidable with the combination of medications and the walk-in freezer settings in many recovery rooms. They were reluctant to release me with zero sats, but I pointed out I obviously had fine sats and that Raynauds was already documented, and thus normal for me.

So beware, readings of zero can create some awkward situations with you needing to explain that you are still really alive.

92 is still acceptable but it's not bouncing off the walls terrific, like higher readings, including the elusive but still idealized 100%.

It will be good to see what your evaluation shows.

Meanwhile, it's time to follow the classic advice for single people with scleroderma: Stay warm, and keep circulating!
Warm Hugs,

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

#20 Ron

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Posted 07 July 2015 - 06:41 AM

Thanks Shelley, the ideal method for O2 is a needle in the artery in the wrist and draw blood.   Then they measure the O2 in the lab.  I have had this done 2 times in the hospital, it really hurts, but is very accurate.