Question: What do you recommend for idiopathic pulmonary fibrosis?

Dr. Hibberd's Answer:

We have recently become very aggressive in treating idiopathic pulmonary fibrosis. At present, many centers advocate immunosuppressive therapy. In some cases, even lung transplantation is considered if the fibrosis (a lung-scarring condition) becomes progressive or cannot be controlled, especially if it is associated with respiratory failure and the need for respiratory support.

Treatment is supportive with optimizing function for the remaining unaffected lung tissue, and using oxygen supplementation when needed.

There is no immediate treatment for the more severe cases that fail immunosuppressant therapy except for a lung transplant. They have been quite successful for some, but not all patients will qualify for this extraordinary means of managing failing lung function.

Idiopathic means "cause unknown." Pulmonary refers to "lung tissue." Fibrosis refers to scarring and replacement of normal tissue by scar-type tissue with no functionality or elasticity and "fibrotic change."

Fibrosis of lungs, which produces a "paper bag" effect versus the normal healthy balloon effect of a healthy lung, interferes with the normal oxygenation of blood in the lungs. This sets the stage for respiratory failure, followed by multi-organ failure and death. Be sure you are under the care of a pulmonologist, who will work with your primary care physician in managing the treatment options that will serve you best.