Idiopathic Pulmonary Fibrosis?

My sister has this-my understanding is that it is a fatal condition with a varying course of little time to 4 years years. Are there any treatments along the way? What are the signs that end stage is approaching? What is end stage like?
She's a very practical no-nonsense woman, and the doctors are not being very forthcoming. She's getting frustrated. And her insurance carrier says she can't go to different doctor.
Any information would be appreciated

I disagree with Wulfie. The end stage of Pulmanary Fibrosis is very ugly. This particular disease stiffens the lungs and thickens the cell walls which inhibits the transfer of oxygen into the body. While prednisone may help to some extent the side efftects may not be worth it to her. Bronchodilator therapy while somewhat helpful is only a stop gap. Most people with this diagnosis will require supplimental oxygen in increasing levels. I strongly recommend talking to a palliative care specialist. This kind of doctor will discuss everything from oxygen therapy to pain management to how your sister whats her final days to be managed. They will even include spiritual services if she is so inclined. What is so special about this is that they help to make the most of her quality of life while making her transition during the various stages of the disease as comfortable as possible.

The "idiopathic" means the cause is unknown.

Pulmonary fibrosis as you know, destroys the lungs by making them stiff and unable to do their job.

The disease is serious, and almost always results in death in a few years after diagnosis. There are some treatments including steroid Prednazone, and some other chemicals, but none of them work very well.

Sorry I don't have better news. As for the prognosis, usually, the patient ends up on oxygen which makes up for the loss of capacity. Generally, the person dies from pulmonary failure like a heart attack. At least, it isn't really ugly, but it is uncomfortable for the patient since their heart rate soars, and they become really weak, and house bound.

I have IPF. I have been seeing two pulmonologists. Pulmonary Fibrosis is a slow degenerative disease. People such as Robert Goulet and Evel Knievel just died last year, but in their early 60's. I am 46 years old, the doctors said I could live out a normal life span unless it progresses to the point where a single lung transplant is needed. I moved from Ohio to Arizona, the drier air helps, the colder and more humid air in Ohio was worse. After a single lung transplant, lifespan is 2-10 years. Final stages are painful, feels like a small child sitting on your chest. She needs to see a Pulmonologist ASAP. Get info from Cleveland Clinic, Cleveland, OH.