Interstitial Fibrosis

[anacyde]

Hi and thank you for taking my questions.

This past Sept, my husband started with a cough we thought was a cold...only no one else caught it.  The cough held on for four weeks before I finally convinced him to see our doctor.  His chest and heart sounded fine and a chest x-ray was ordered (Nov. 05).  We didn't hear back so we assumed everything was ok.

On Friday, our doctor told us they found Interstitial Fibrosis on the x-ray.  We repeated the chest x-ray yesterday and do not have results yet, but they look very much the same.

He was exposed to asbestos as a child (pipe insulation at home) as well as various dusts.  He was working in our garage cleaning a lot of nasty dust off the floor when these symptoms started in Sept.

He is 38, non-smoker, healthy otherwise.  He sometimes gets winded a bit easily with activity - but other times he is fine.  The cough he was seen for is now gone.  It was semi-productive and Mucinex helped, as did occasional cough syrup.  He suspected the mild occasional SOB was due to 4+ years of no exercise and 40 lb weight gain.  He does have other symptoms: joint pain, extreme fatigue (for 12+ years, not recent), malaise, bloodshot eyes.

What I've read about fibrosis is bleak.  Is it possible to have lung fibrosis and live a normal life span?  What factors determine progression?  Are there any conditions that would present this way on films besides deadly ones?

I have included a link to his x-ray and would like your opinion - free of liability - just your opinion.

http://img.photobucket.com/albums/v260/octoberbaby/mikelungs.jpg

[National Jewish]

Idiopathic pulmonary fibrosis (IPF) is one form of interstitial lung disease (ILD).  This causes inflammation or swelling that leads to scarring or fibrosis in the interstitium of the lungs.  The interstitium is the area between the air sacs and the blood vessels.  Usually this scarring is spread throughout both lungs.  The most common symptoms of ILD are shortness of breath with exercise and a dry cough.  Some people also may have a fever, weight loss, fatigue, muscle and joint pain, and abnormal chest sounds, depending upon the cause.  IPF does not have a good prognosis.  It is often not compatible with a normal life span.  But you must not assume that the diagnosis of pulmonary fibrosis is the correct diagnosis, with this scanty evidence.

Your husband's asbestos and other dust exposure could be the cause.  The chest x-ray findings could even be compatible with hypersensitivity pneumonitis (HP).  This is inflammation of the air sacs of the lungs.  It is caused by an allergic reaction to inhaled biologic dusts that are small, 5 microns or less in diameter.  Biologic dusts include animal or plant proteins, microorganisms, and rarely low-weight chemicals.  The symptoms of HP include coughing, wheezing, and/or shortness of breath, especially with activity.  Once exposure stops, the symptoms usually clear within a day.  Continued exposure can eventually cause permanent scarring of the lungs.  Masks are usually not helpful, but respirators can significantly decrease the exposure.  However staying away from the cause will help the most.  Inhaled steroids are used to decrease the inflammation before this becomes scarring.

Pulmonary function tests will measure the effect of the scarring on your husband

[anacyde]

Hi and thank you for the info.  I'm really sorry to hear of your husband's diagnosis of IPF, I understand the disease is extremely frustrating.  Have you ever seen a forum called Pulmonary Fibrosis Freinds on Yahoo?  It's a great place for people affected by pulmonary fibrosis directly and indirectly.  You can find the group here:

http://health.groups.yahoo.com/group/PFF/

Yesterday we did the CT scan.  Last Friday we saw a pulmonologist.  He said the second chest x-ray was clear, and he doesn't know if he would have called "interstitial fibrosis" on the first scan anyhow.  He did say there is some mild scarring and "calcification", consistent with an old infection (he has had some severe ones in the past) or possibly an inhaled particulate (which precipitated a nasty cough and our November visit to the doctor for the initial chest films).  He also mentioned the possibility of asthma or acid reflux and is doing a PFT soon to evaluate his lung function.

Have you been following with a pulmonologist?  I know, probably a stupid question...but if your husband's IPF has remained stable for five years, he may not have IPF, and that would be awesome.  I'd be getting more opinions.  I already have additional doctor appointments made for my husband to be sure of what is going on because of our mixed results.

I wish you both the best, I sincerely hope that your husband's fibrosis remains stable and does not progress and that you have many happy, healthy years together.  I hope the PFF group can offer some support to the both of you as well :)

[Lori_B]

I'm sorry your husband is going through this.  There are a lot of different types of pulmonary fibrosis - some treatable, some not.  If I were you I would get him to a pulmonologist as soon as possible for a CT Scan which is much better than an x-ray in determining what is going on. The pulmonologist may also want to do a bronchoscopy in order to hopefully determine what type of fibrosis (if any) your husband actually has.  If he does have a treatable form of fibrosis, it is VERY important to get him started on the appropriate treatment as early as possible.  

I am no expert by any stretch.  My husband was diagnosed with a form of fibrosis - IPF - around 5 years ago and I am still trying to figure out what is going on.  The one piece of advice I would give you is not to worry yourself needlessly.  Get him to a good pulmonologist, find out what is going on, then take it a day at a time.  I spent the first year convinced I would lose my husband at any time.  5 years later, nothing has changed.  He's still here and his fibrosis hasn't gotten any worse. Life goes on...

Good luck to you!
Lori

[anacyde]

Nevermind the link, I removed it.