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Wednesday, April 16, 2014
What to Expect with Idiopathic Pulmonary Fibrosis
I am wondering if you can help me. My mother was diagnosed with IPF this week. She has been told that over 50 percent of her lungs have been damaged and the area around her lips is taking on a blueish color. Could you tell me what stage of this disease she is likely to be at and what to expect. She is not keen to go on the steroids as would rather have her quality of life than be pumped full of drugs.
There are a number of ways of determining how far advanced pulmonary fibrosis is. Two common tests that we use are the forced vital capacity (FVC) and the diffusing capacity (DLCO). Although opinions vary, we generally consider patients with an FVC less than 55% of predicted or a DLCO less than 35% of predicted as being advanced stage.
Regardless of the results of those tests, if she is having blueness around her lips, that could be a sign of a low blood oxygen level and home oxygen can help improve shortness of breath in that situation. We generally start home oxygen if the oxygen saturation falls below 89% either at rest or while walking.
Another option for her, if she is in an early stage, is involvement in a clinical trial of experimental treatments for pulmonary fibrosis. There are several studies currently underway. One of the biggest is the CAPACITY study of pirfenidone in early stage pulmonary fibrosis. The internet link for that study is: http://www.capacitytrials.com/wt/page/index
James N Allen, Jr, MD
Clinical Professor of Pulmonary, Allergy, Critical Care & Sleep Medicine
College of Medicine
The Ohio State University