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Pulmonary Fibrosis

If diagnosed when you have 60% lung capacity

03/03/2009

Question:

My brother lives in England and was recently diagnosed with Pulmonary Lung Fibrosis. When diagnosed they found he has 60% lung capacity. Does this mean he is too far advanced to be helped?

Answer:

Many measurements determine how advanced idiopathic pulmonary fibrosis is. There are also many different measurements of lung capacity and each has different meanings.

Probably the most important measurements to look at are the oxygen level when doing a 6-minute walk test and the change in pulmonary function tests over time.

If the oxygen level falls below 90% while walking, then this indicates a more advanced stage. Similarly, if the forced vital capacity or the diffusing capacity falls by more than 10% over time, then this indicates that a patient is more advanced.

Currently, there is no cure for idiopathic pulmonary fibrosis but there are some treatments that might help such as N-acetylcysteine and pirfenidone. Pirfenidone is not yet available commercially in the United States or Europe but it may be later in 2009.

Our current thinking about pulmonary fibrosis is that if the decision is made to treat, the best chance that treatment will work is if the treatment is started as early as possible in the disease progression.

Lung transplantation is also an option for patients with more advanced disease. Although transplantation has historically been limited to patients under age 65, many transplant centers are starting to transplant older patients if they are otherwise in excellent health.

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