Home HealthTopics Health Centers Reference Library Research
Join us on Facebook Join us on Facebook Share on Facebook

Pulmonary Fibrosis

I was just diagnosed with pulmonary fibrosis

03/30/2009

Question:

I was just diagnosed last week i have an appt. with a doctor this week. I had a biopsy done of my lung can you be straight ith me about an outcome

Answer:

When you meet with your doctor, be prepared to ask several questions:

First, what type of pulmonary fibrosis is it? One of the most common types is "idiopathic pulmonary fibrosis", sometimes called "usual interstitial pneumonitis" or UIP. This is a serious condition that has an average survival of 3 to 5 years, depending on how advanced it is at the time of diagnosis. Other forms of pulmonary fibrosis, particularly those caused by rheumatologic disorders or that develop as a side effect of medications, have a better prognosis.

Second, is transplant a possibility? In patients who do not have other serious medical diseases, are not smokers, and are not obese, lung transplant can be a possibility. In the past, the age limit for transplant was generally considered to be age 65 but some transplant centers will transplant otherwise healthy persons who are older.

Third, what medications can be considered? There is no medical cure for idiopathic pulmonary fibrosis but conventional treatment (prednisone and/or either Imuran or Cytoxan) can be considered. These drugs may slow down the disease in a small percentage of patients but frequently have side effects. N-acetylcystiene is an over-the-counter antioxidant that appears to have some effect in idiopathic pulmonary fibrosis. Pirfenidone is a new, experimental medication that has some promise but is currently not commercially available (an application to the FDA is underway and if approved, the drug may be available later this year).

Fourth, are there experimental studies that you may be eligible for? The only way that we will develop new and effective treatments for idiopathic pulmonary fibrosis is to study new treatments in clinical trials.

Lastly, what can be done to improve your quality of life? Specifically, do you qualify for home oxygen, pulmonary rehabilitation, or a disability parking placard?

For more information:

Go to the Pulmonary Fibrosis health topic, where you can:

Response by:

James N Allen, Jr, MD James N Allen, Jr, MD
Clinical Professor of Pulmonary, Allergy, Critical Care & Sleep Medicine
College of Medicine
The Ohio State University