How is idiopathic pulmonary fibrosis treated?

There is no cure for IPF. The damage caused by the lung scarring cannot be reversed. In addition, the progression of the disease cannot be predicted or stopped.

There are currently 2 medications approved for use in IPF. These medications have been shown to slow the scarring and help preserve lung function.

Lung transplant surgery may be considered in some patients, most often patients who are under 70 years old who have advanced disease.

Other treatments for IPF are supportive. Patients may need additional oxygen, which supplied in a small portable oxygen tank, to help them breathe. Patients are also encouraged to enroll in an exercise program known as pulmonary rehabilitation to help improve their breathing. Patients should also receive immunizations for pneumonia and yearly influenza to avoid illness, which can be more severe in people with IPF. It is important to stay active, follow a healthy diet, get plenty of rest, and quit smoking.

Some exciting new treatments are currently under investigation. The Cleveland Clinic is 1 of 26 medical centers across the United States that excel in the treatment of IPF. These medical centers make up the IPFnet, a network of research centers dedicated to the study of IPF. The network’s goal is to find effective therapies for patients with both the early and advanced stages of this devastating disease. Please consult your doctor if you are interested in taking part in a clinical trial.

What are the complications of idiopathic pulmonary fibrosis?

When lung tissue becomes scarred, it is less able to deliver needed oxygen to the body. This puts a strain on the right side of the heart, which may lead to high blood pressure in the lungs, a condition known as pulmonary hypertension.

Some patients may have an exacerbation of IPF, which is a sudden increase in lung scarring. During an exacerbation, the patient may cough more, and have more shortness of breath episodes. The causes of an IPF exacerbation are not known, but you should contact your doctor if you suddenly feel worse.

It is also very important to take measures to avoid becoming sick, as it may be more difficult to recover from lung infections. Patients should:

  • Pay close attention to hand washing.
  • Keep up-to-date with immunizations.
  • Avoid direct contact with others who are sick.

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