Pulmonary nursing is my specialty. See these two web sites for more info:
Pulmonary fibroses causes thickening of the lungs along with stiffness. As stiffness progresses, it becomes harder to breathe---think of the lung as a balloon that becomes hard and leathery over time.
Prednisone has been the main treatment over the years along with Cytoxan. I would suspect that the new steroidal inhalers should be started first these days as act dirrectly on lungs with minimal side effects, then oral steroids. Good pulmonary hygiene and breathing exercises can help. I teach patients to use prescribed inhalers first then do exercises. Always use broncodilator inhaler first then steriod---as lungs open up can get more of steroid further into lung.
Exercises include relaxation, pursed lip/diphramatic breathing; use of paper party horn then advancing to metal horn; use of peak flow meter to measure volume. O2 therapy often required and usually seen in higher levels than pts with COPD 2-4, can progress as high as 6 liters. There are now Oxygen resevoir tubing that helps with high flow patients. Mini neb treatments utilized also.
Some patients remain stable and life long lives--- >10 years. Iv'e seen few that progress quickly with 6mos-year. A lot has to do with mental fortitude and other underlying illnesses, smoking still etc. Most patients can maximize years if follow treatment plan, seek prompt attention for colds/infection. Need flu shot yearly and should have pneumonia vaccine too.
PM me if more specific info needed.