Ron

Crackling sound from the lungs

34 posts in this topic

Thanks for the reply. I was diagnosed with Scleroderma back in 1987. Had a brief 30 day stay in the hospital when my kidneys went haywire, they thought it was related to Scleroderma. After a biopsy it was not related. They gave me another diagnosis of Scleroderma. I remember some nurse that came into my room for counseling and thought I was upset at the findings. I wasn’t. Even in 1992 when I was diagnosed with Diffuse Scleroderma, it didn't bother me.

 

It was not until I saw the 3rd x-ray results I saw yesterday seeing the progression over 3 years that has me worried and depressed. I think I am finally coming to terms with having Scleroderma. I recall seeing something that in most cases the cause of death is from the lung involvement. Many years ago it was kidneys.

 

Ron

Share this post


Link to post
Share on other sites

Hello Ronald,

 

Over the years the main cause of death in scleroderma has changed, it was from kidney involvement and is now, I believe, lung involvement. However, this is not because lung involvement has become worse or ever was worse but because survival of a renal crisis has improved. Doctors are now aware of renal crises in scleroderma, I have a friend who had a renal crisis some 20+ years ago and nearly died before they tied it to the scleroderma, today they would be more on the ball and as we know, prompt diagnosis also enables prompt treatment.

 

Now lung involvement, including pulmonary fibrosis and pulmonary hypertension, is the most critical symptom. Well that's what it says "on paper" but of course the most critical symptom is the one that affects the individual the worst. I have a friend with complete gastric failure, the doctors pronounced her terminal but she's still alive and has even gained weight albeit through TPN (total parenteral nutrition). She was advised that gastrointestinal involvement was the biggest killer and I have also read that somewhere myself. I myself have myocardial fibrosis and had a 2 year life expectancy back in 2010 but thanks to a biventricular ICD I'm still here!

 

I am sure that statistically something or another is more likely to kill in systemic scleroderma but then how accurate are the stats and what about individual circumstances? Firstly stats, there must be as many undiagnosed and misdiagnosed people with scleroderma as there are those diagnosed with it and I wonder what they die of and whether that would shift the focus from lung back to kidney or to the heart or remain on the lung? Of course personal circumstances are also a big player, someone with systemic scleroderma living in the US who has say moderate to severe pulmonary hypertension may well live longer than someone living in India with say complete gastric failure. Someone with pulmonary fibrosis living in the UK, diagnosed and treated immediately may well live longer that someone with kidney involvement, misdiagnosed and then given high dose steroids. It's swings and roundabouts for sure!

 

For you the priorities are diagnosis of the systemic involvement, which you now have, then treatment which you will soon have hopefully and support which will be ongoing if you stick around...I means as in if you choose to keep posting!

 

I am still amazed at how people buck the trend and continue to live for years when they should in fact have kicked the bucket or passed over or whatever euphemism is preferred. Remember that you have lived for nearly 30 years with this disease so you have had a good long run and it's perfectly possible that you will continue to do so! Clearly you're doing many things right!

 

Take care. 


Amanda Thorpe

ISN Sclero Forums Senior Support Specialist

ISN Video Presentations Manager

ISN Blogger

(Retired) ISN Sclero Forums Assistant Manager

(Retired) ISN Email Support Specialist

International Scleroderma Network (ISN)

Share this post


Link to post
Share on other sites

Hi Amanda,

 

I have not been doing things right.  I went for many years, about 15 years, where I didn't do anything about scleroderma.  I just wanted to forget about it and not worry.  My course of the disease has been very slow, but the last 3 years its been getting worse faster than all the other years.  I am in for a round of tests and specialists. We will see what the future has in store for me once I get all my tests done.

Share this post


Link to post
Share on other sites

Hi Ronald,

 

I'm very sorry you've found out that your lung problems are due to scleroderma. Unfortunately it's normal to encounter more challenges at the ten or twenty year mark, with or without treatment of any sort. 

 

It sounds like at least you are not guilty of taking too many medications. Therefore, you probably haven't worn out your liver or kidneys yet, which would mean you'll be in much better shape to undertake any necessary treatment now.

 

Please keep us posted on how your tests are going. I know its a slog and a waiting game.  I'm thinking of you and sending good thoughts your way.

 

: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

Well I had my first short of breath incident with Scleroderma on Wednesday, May 13, 2015.  I went to the grocery store on a short errand run and while doing my weekly walk through the store, by the end I was out of breath, like I walked 10 miles. Never happened before.  I am also just getting over the flu, had it since May 3rd.  Still coughing up phlegm from the lungs.        

I went to the doctor the next day and she gave me a rescue inhaler.  She said use this next time I experience shortness of breath, or go to the emergency department at the hospital.

Does this thing work?  Anyone else here use them?  I am set up for a bunch of tests, I am interested in finding my pulmonary function test and compare it with the one I had done 2.5 years ago.

Share this post


Link to post
Share on other sites

Hi Ronald,

 

I'm sorry you had an episode of shortness of breath. I hope you get some more answers soon.

 

Please keep in mind that I'm not a doctor, and have no medical training at all. It's my understanding that rescue inhalers can work near-miracles for the most common causes of shortness of breath (dyspnea), such as most lung problems including asthma, allergies, COPD, and inflammation.

 

However, they won't resolve an episode of shortness of breath if it is caused by other things, such as a pneumothorax (collapsed lung), or issues outside of the lungs such as pulmonary hypertension, heart failure or heart attack,

 

Since so far you've had an isolated event, and it was perhaps mostly inspired by the infection or a clogging of the airways due to phlegm, you might only need the inhaler temporarily.

 

You might want to consider some self-monitoring while things are getting sorted out. They recommend that all people with systemic scleroderma take their own blood pressure daily, to detect and alert for the initial signs of sudden kidney failure or other complications. Simply taking and charting your blood pressure and pulse once a day would establish your baselines (if you aren't already doing this.)

 

Then, since you have lung concerns now, you might really like to have a little device for measuring your oxygen saturation. An oximeter is just a tiny (and rather inexpensive) device you clip onto your finger whenever you want. Normal sats for entirely healthy people are probably about 96 to 100%.

 

Your lung doctor can advise you as to what they consider a reasonable cut-off point for you, but the general guideline for oxygen supplementation to be covered by insurance companies is that sats regularly fall below 88%, on testing in the doctor's office, either at rest or with a medically supervised six-minute walk.

 

Those technical details aside, you can buy a pulse oximeter online, or at a drug store. They are often used by athletes. The peace of mind they can deliver for someone with lung problems is amazing!  You can know whether or not you are over-exerting, or if your shortness of breath is caused by an actual lack of oxygen -- or something else.

 

Tracking readings once a day for years can establish a pattern, and you can know very quickly whether you are improving or worsening with medication or exercise. It's something to consider, at least, if you don't already have and use one.

 

: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

"It's normal to encounter more challenges at the ten or twenty year mark, with or without treatment of any sort."

 

Shelley, I had no idea and am two years short of my own ten year anniversary as it happens.

 

Interestingly, when I first saw my scleroderma expert and asked what my life expectancy was, at least 10 years was the reply I was given. At that point I had no internal organ involvement and found the reply interesting. Normally you hear the reply to life expectancy in terms of no more than, not at least!

 

This would make sense as the doctor would have known that things can change at the 10 year mark.

 

Well, I shall have to wait and see what happens two years from now. It's been am interesting journey thus far and at least I know that will continue!

 

Of course nothing could change and I could plod along very much as before, awaiting the 20 year mark etc. Scleroderma is nothing if not unpredictable and we all have our own experience of it so let's see what happens next!

 

Take care.


Amanda Thorpe

ISN Sclero Forums Senior Support Specialist

ISN Video Presentations Manager

ISN Blogger

(Retired) ISN Sclero Forums Assistant Manager

(Retired) ISN Email Support Specialist

International Scleroderma Network (ISN)

Share this post


Link to post
Share on other sites

Hi Amanda,

 

As you well know I passed the 10 year mark in the 1970's and am now approaching 50 years at least.   OK so now the wheels are falling off and I am only 12 years past diagnosis but I don't count that as valid because I remember the first Gastro attack in 1950's so I think I have done pretty well.   It seems that the later one gets the first indcations then the gentler the onset might be.   However, I guess there are others here who can go back further than me.

 

Judyt

Share this post


Link to post
Share on other sites

I had another episode of shortness of breath after a trip for groceries.  I used my rescue inhaler and it didn't do any thing but gave me heart palpations about every 4 beats.  Went to the doctor, they sent me for an ECG immediately and waiting for results.  Had tightness in the chest, so they gave me a prescription for nitroglycerin spray.
            
I am reluctant taking it, because they don't know the whole picture about Scleroderma.

Share this post


Link to post
Share on other sites

Hi Ronald,

 

I would agree with you about being very cautious taking new drugs when the original cause has not been found.   The sad fact is that very few people you come across will actually know anything much about Sclero and how to treat us.

 

I am often breathless, and I have had Pulmonary Hypertension but that has been put down to the Mitral Valve damage which I have had repaired.   At this point I don't notice any improvement but I am still recovering from the Pneumonia I contracted at the time.

 

Different doctors have different opinions about why I am breathless and in the meantime I just jog along puffing as I go.

 

Judyt

Share this post


Link to post
Share on other sites

I have to ask, are you a smoker? I had crackling in my lungs and got short of breath easily, but since I quit smoking 5 months ago the crackling is gone and I can go a little farther without getting short of breath.

 

My sclero doctor says my lungs sound much better and my next pulmonary function test will probably be better.

Share this post


Link to post
Share on other sites

Quiltfairy! Had it been 5 months since you gave up smoking? May I say that's amazing and well done and keep on keeping on! It CAN be done!

 

Take care.


Amanda Thorpe

ISN Sclero Forums Senior Support Specialist

ISN Video Presentations Manager

ISN Blogger

(Retired) ISN Sclero Forums Assistant Manager

(Retired) ISN Email Support Specialist

International Scleroderma Network (ISN)

Share this post


Link to post
Share on other sites

How fantastic and good for you, Quiltfairy! :congratulations:

 

5 months without smoking........you must feel so much better for it and that's not counting the effect it will have had on your lungs!

 

I'm sending you some balloons and doing a Sclero Happy Dance for you to celebrate!!

 

:balloons::happy-dance-line::balloons:


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

Does anyone else with pulmonary fibrosis notice they breathe more than the normal rate per minute?

I am about 32 breaths per minute.    
 

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