In addition to medication, there are several therapies that are available for the treatment of pulmonary fibrosis. These therapies are meant to minimize and manage potential complications of ILD as well as improve or prevent deterioration in quality of life. Treatments may be used alone or in conjunction with each other based on the personalized needs of the patient.
Oxygen therapy is required for some patients with pulmonary fibrosis. Scar tissue that develops in the lungs (pulmonary fibrosis) can interfere with the transfer of oxygen from the air we breathe into the blood stream. This decrease in oxygen in the blood stream causes patients to feel breathless. Depending on the amount of scar tissue in the lungs some patients may need oxygen therapy all of the time while others may need it only during sleep and exercise. There are many systems used to deliver oxygen. Utilizing supplemental oxygen allows patients to remain active, travel and maintain activities of daily living.
A pulmonary rehabilitation program is often recommended for some FPF patients to help them achieve their highest possible level of functioning.
This program includes:
If the disease cannot be managed, lung transplantation may be an option for some advanced cases. Lung transplantation is surgery to replace one or both lungs with healthy lung tissue from a donor. With improved surgical techniques and post-transplant care, lung transplant increases survival and quality of life to selected patients.
Therapies Under Investigation
Antifibrotic and other agents are currently being tested to determine their efficacy in treating IPF. If found to be effective in IPF, these agents may also be a helpful threapy for FPF.
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This information has been approved by David Schwartz, MD (February 2010).