Pulmonary Fibrosis Progression and Exacerbation

Pulmonary fibrosis is a progressive disease that naturally gets worse over time. This worsening is related to the amount of fibrosis (scarring) in the lungs. As this occurs, a person's breathing becomes more difficult, eventually resulting in shortness of breath, even at rest.

Patients with pulmonary fibrosis experience disease progression at different rates. Some patients progress slowly and live with PF for many years, while others decline more quickly.

There is no cure for pulmonary fibrosis, but treatments can slow the progression of the disease in some people. Maintaining a healthy lifestyle and working closely with your care team can help you best manage your PF.

PF Exacerbation

Some patients will experience a sudden worsening of their pulmonary fibrosis, which is referred to as an acute exacerbation. This occurs when there is a triggering event that results in a sudden increase in the processes that lead to scarring. As the lung scarring gets worse, patients have a harder time breathing. The damage to the lungs during an acute exacerbation is permanent.

To determine if you are having an acute exacerbation, doctors take into consideration your symptoms, oxygen levels, CT scan results and often suggest other tests such as a bronchoscopy to assist with diagnosis.

Much is unknown about how to best treat and prevent exacerbations. If your doctor knows what is causing the exacerbation, the trigger will be treated. For example, if you have an infection, you will be prescribed antibiotics. Otherwise, doctors will provide supportive care—for example, extra oxygen.

During an acute exacerbation, many patients require hospitalization. If your exacerbation is severe, you may require ventilator support. This means that you are placed in a machine that acts as your lungs. Patients who are placed on a ventilator during an acute exacerbation are at an increased risk of dying. This is why it is important to talk with your doctor and family about your wishes and fill out an advance directive as soon as you are diagnosed with PF. When a serious health event occurs, the advance directive will help your family and care team honor your wishes if you are not able to speak for yourself.

Page last updated: March 22, 2020

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