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Lung diseases

Idiopathic Pulmonary Fibrosis

04/14/2010

Question:

My question involves my 89 year old mother-in-law. She has had reduced lung capacity for some time and initially it was diagnosed as COPD. Since she never smoked and was primarily a legal secretary, I doubted the diagnoses. In June 2009 she came to live with us and I took her to a pulmonologist to see if that was the problem. The x-rays of her lungs looked like white footballs and she was hospitalized for tests. They diagnosed her with Idiopathic Pulmonary Fibrosis and she is now on home oxygen and the machine is set at level 4. If she walks a short distance, her oxygen plummets to the 70s, although when she sits still, it remains in the 90s. She cannot take a deep breath, tires very easily and falls occasionally. Her doctor keeps saying there is hope, but from what I have read on-line, this appears to be end-stage IPF. Are these the symptoms of end-stage IPF or is there hope of some sort?

Answer:

These symptoms are consistent with advanced (end-stage) idiopathic pulmonary fibrosis. This disease cannot be cured and is eventually fatal. It is important that the physicians have established a confident diagnosis. This can frequently be done based on the history, physical examination, chest CT scan, and blood tests; a lung biopsy is sometimes required (but usually not necessary).

 

Other conditions that can occur in conjunction with IPF that can result in worse shortness of breath and lower oxygen levels include heart failure, blood clots (pulmonary emboli), and infection. If a confident diagnosis has been established and these other conditions have been eliminated, then there are very few options and it may be an appropriate time to discuss the possibility of hospice with the treating physicians.

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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