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Tuesday, March 11, 2014
Hereditary pulmonary fibrosis
My brother died of pulminary fibrosis at 55 and I was looking to see if it was something that I could inherit through the family gene`s. I am now 51 and have some of the early symtoms that my brother had as I recall. When laughing it develops into a period of coughing, etc. I see by some of what i read that 10-15% are cases within a family. Is this true and should I be concerned based on my brother dying from this sickness?
Several genes have been identified that can be responsible for hereditary pulmonary fibrosis including the TERT and TERC genes. This month, a new mutation of the SFTPA2 gene has also been identified to be associated with hereditary pulmonary fibrosis. Tests for the TERT and TERC genes are commercially available but only account for about 10% of cases of inherited pulmonary fibrosis.
Because we are still unable to test for most cases of inherited pulmonary fibrosis, two of the things that family members of patients with idiopathic pulmonary fibrosis can do is to
- see their primary care physician on an annual basis and
- have the lungs checked for the presence of "crackles" and to report symptoms of persistent cough or persistent shortness of breath to their physician.
In patients with these symptoms, we often obtain pulmonary function tests and a high resolution chest CT scan, tests that can pick up pulmonary fibrosis in an early stage.
James N Allen, Jr, MD
Clinical Professor of Pulmonary, Allergy, Critical Care & Sleep Medicine
College of Medicine
The Ohio State University