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Idiopathic Pulmonary Fibrosis – Management

April 4, 2021


"How is IPF managed? Similar to COPD management, preventative measures are also important for IPF management. I ask all patients to stay updated on their vaccinations, quit smoking if they have not already done so, and maintain general conditioning with at least 30 minutes of activity daily. For patients with more advanced disease, we consider adding pulmonary rehabilitation, where respiratory and physical therapists will work directly with the patients to improve their exercise tolerance. Chronic aspiration is another risk factor for progression of pulmonary fibrosis. To minimize this risk, I recommend all my patients going to sleep no earlier than three hours after finishing dinner. In patients with more advanced IPF, the use of supplemental oxygen becomes necessary at some point to help relieve the sensation of general dyspnea or shortness of breath. Fortunately, two new medications have been approved for IPF within the last five years. These medications called pirfenidone and nintedanib are anti-fibrotic medications used to slow the progression of disease. It's important to note that these medications will not reverse areas of lungs that are already fibrotic, but can delay further disease progression. These medications have the potential to significantly improve quality of life for patients. It's better to start these medications earlier in the disease course, as there are still significant areas of lung that can be protected. When the disease is already too far advanced, these medications have little benefit, as the lung is already entirely fibrotic. A detailed discussion with your pulmonologist is necessary before these medications can be prescribed. Unfortunately, at this time, there's no agents that can actually reverse areas of fibrotic changes that are already present in the lungs. In patients who are usually younger, usually under the age of 65, and who have no other major medical problems, referral to a lung transplant center is recommended to see if the patient is a lung transplant candidate. Lung transplant will require extensive testing to be done beforehand and after transplant, and is unpredictable when an organ will be available. However, if a patient successfully gets a transplant, they'll often have a significant improvement in their quality of life."

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