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Pulmonary fibrosis is a type of lung disease that causes scarring of lung tissue. When this tissue becomes thick and hardened, the lungs do not expand and retract normally, which makes it difficult to breathe. People with pulmonary fibrosis often have shortness of breath and low oxygen levels, and they frequently experience difficulty exercising.
Pulmonary fibrosis is progressive, which means it worsens over time. Though it may be caused by diseases and environmental substances, there is no identifiable cause for most cases of pulmonary fibrosis—this is referred to as idiopathic pulmonary fibrosis (IPF). An estimated 10-15% of people with IPF have an inherited form of the disease called “familial IPF.” There is currently no cure for pulmonary fibrosis.
Known causes of other kinds of pulmonary fibrosis (non-IPF) include the following:
According to the American Lung Association, pulmonary fibrosis affects about 140,000 Americans, usually between the ages of 50 and 75. The disease may progress slowly or rapidly. Many people with pulmonary fibrosis live only three to five years after diagnosis.
Symptoms of pulmonary fibrosis may be mild at first. The most common symptoms include:
As the disease progresses, its symptoms get worse. Eventually, the lungs can become so damaged that breathing is labored even when sitting still. In rare cases, symptoms of pulmonary fibrosis progress rapidly, and disability occurs within weeks or months of disease onset.
As a patient at Norton Thoracic Institute (NTI), you will receive a comprehensive evaluation that may include the following:
Each type of pulmonary fibrosis behaves differently, depending on its cause. At NTI, specialists will spend extensive time with you, getting your complete medical history and discussing the clinical data from your evaluation and testing. This information will allow the team to determine the cause, characteristics, and prognosis of your condition.
Treatment for pulmonary fibrosis focuses on preventing additional lung scarring, relieving your symptoms, and helping you stay as active as possible.
Advanced pulmonary fibrosis may require a lung transplant. Norton Thoracic Institute offers lung transplant when indicated and now is one of the largest lung transplant programs in the country and the largest in the western United States.
The Norton Lung Transplant Center is known for streamlined processes that speed up treatment and produce exceptional outcomes.
Although there currently is no cure for pulmonary fibrosis, you can improve your quality of life by following these guidelines:
The Pulmonary Fibrosis Foundation has designated Norton Thoracic Institute as a Care Center for patients with pulmonary fibrosis—so patients with this disease will receive care from a multidisciplinary team that specializes in treating this complex condition.
To learn more about our services, call 602.406.4000.