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Pulmonary Fibrosis

IPF

12/03/2008

Question:

After many tests most recently a lung biopsy my mother(age 69)was diagnosed and advised in teh "end stage" of IPF. We were expecting that result however what does that really mean as far as life expectancy. She is on 24/7 oxygen and only taking prednisone. We know that there is no cure but her question basically is does this mean she has a few years or is it more a matter of a few months. Obviusly nobody can predict the future but what does end stage with IPF typically mean? Many Thanks!

Answer:

Many patients with idiopathic pulmonary fibrosis (IPF) do not have a steadily declining course and instead can have sudden periods of worsening followed by periods of the disease being stable. Each patient is different and it is not possible to predict which course any given patient will have.

Once patients with IPF require continuous oxygen, they are generally in an advanced stage. The pulmonary function tests can also be a useful guide and as a general rule, if the forced vital capacity (FVC) is less than 50% of normal or if the diffusing capacity (DLCO) is less than 35% of normal, then a patient is likely to be in an advanced stage.

As patients enter an advanced stage, they have more and more of the lung tissue replaced by scar. The average life expectancy for IPF is 3-5 years from the time of diagnosis but this can be considerably less if patients are already in an advanced stage when they are diagnosed.

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