Exact diagnosis is critical to treatment of pulmonary fibrosis

Bin, &Mn Itaidh MLB Ask Dr. Keith Roach M.D

DEAR DR. ROACH: My husband was recently diagnosed with mild to moderate pulmonary fibrosis. His mother died from pulmonary fibrosis. From what we have read there is no cure, only palliative care. The thought of doing nothing to try to stop the progression is totally unacceptable when there have been such successes with immunotherapy and stem cells. Would he receive more aggressive treatment going to a pulmonary fibrosis care center that has lung disease programs? — C.D.

ANSWER: There are several diseases that may cause fibrosis of the lung, so having an exact diagnosis made by an expert is critical for getting the right treatment. The most common type of pulmonary fibrosis is called “idiopathic pulmonary fibrosis” (it also has been called “usual interstitial pneumonia” and “cryptogenic fibrosing alveolitis”), and I am going to discuss that diagnosis.

The cause of IPF is unknown (both “idiopathic” and “cryptogenic” mean “of unknown cause”), but cigarette smoking is a risk factor. It is usually diagnosed in people who are their 50s and 60s, although some familial cases may come on earlier. IPF can run in families.

Although it is a rare disease, it seems to be on the rise. The major symptoms are cough and shortness of breath.

There is no cure for IPF, but there are treatments available. Two drugs, pirfenidone and nintedanib, are approved for use in the U.S. and Canada. Both of them slow progression of lung disease, reduce exacerbations and have been proven to reduce mortality from the condition, if only slightly. I am concerned that you and your husband apparently haven’t heard of these drugs.

I strongly advise you get a referral to a center for lung disease in order to get your husband the best care. I found 35 IPF trials recruiting patients in the U.S. and six in Canada. A clinical trial will help scientists understand this condition as well as determine the best current treatment. You also can find out what centers have expertise on this condition by seeing where the trials are conducted at www.clinicaltrials. gov. The most aggressive treatment isn’t always the best, but you and your husband deserve to hear about all the options.

The Pulmonary Fibrosis Foundation can help you find experienced centers for treating this condition. You can find them online at www.pulmonaryfibrosis. org. *** Dr. Roach regrets that he is unable to answer individual letters, but will incorporate them in the column whenever possible. Readers may email questions to ToYourGoodHealth@med.co rnell.edu or send mail to 628 Virginia Dr., Orlando, FL 32803.