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Pulmonary Fibrosis


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:

  • Environmental toxins, such as asbestos, silica, certain gases, and ionizing radiation
  • Certain medications
  • Autoimmune diseases, including lupus, rheumatoid arthritis, polymyositis, and systemic sclerosis
  • Inflammatory diseases, such as sarcoidosis or Wegener’s granulomatosis
  • Hypersensitivity pneumonitis, an allergic inflammation of the lungs caused by breathing in dust, bacteria, fungus, molds, chemicals, or other substances
  • Infections.

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

Symptoms of pulmonary fibrosis may be mild at first. The most common symptoms include:

  • A dry, unexplained cough
  • Shortness of breath and difficulty breathing
  • Decreased tolerance for exercise
  • Aching joints and muscles, and a general sense of fatigue
  • Gradual unexplained weight loss
  • Rounding and widening of the tips of the fingers or toes, a condition called clubbing
  • Gastroesophageal reflux symptoms.

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.

Diagnosis

As a patient at Norton Thoracic Institute (NTI), you will receive a comprehensive evaluation that may include the following:

  • A complete medical history that includes your history of medication, environmental exposure, symptoms, and family history of pulmonary fibrosis or related diseases
  • Physical examination looking for symptoms such as crackling sounds in the lungs, which can be a sign of scarring, and clubbing of the fingers or toes
  • High-resolution CT scan to examine your lungs for signs of disease
  • Lung function tests to measure how well air moves into and out of your lungs, and how efficiently the lungs deliver oxygen to your body
  • Blood tests to measure the levels of oxygen and carbon dioxide in your blood, and to identify any immune system disorder
  • Exercise testing to assess lung function during exertion
  • Bronchoscopy to collect samples of cells and small biopsies from your lungs
  • Surgical lung biopsy, such as video-assisted thoracoscopic biopsy, to obtain a sample of lung tissue to confirm diagnosis
  • Evaluation to assess the presence of gastroesophageal reflux disease
  • Lung transplantation evaluation, when indicated.

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

Treatment for pulmonary fibrosis focuses on preventing additional lung scarring, relieving your symptoms, and helping you stay as active as possible.

  • There are currently no medications that stop the progression of pulmonary fibrosis, although there are recently approved agents that decrease the rate of progression of fibrosis in IPF.
  • Corticosteroids such as prednisone and immunosuppressants may be prescribed, depending upon the cause of fibrosis.
  • Oxygen therapy may help supplement your blood oxygen levels if they become too low. This can make it easier to breathe and stay active.
  • Pulmonary rehabilitation lessons teach you how to manage your disease and remain 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.

Living with Pulmonary Fibrosis

Although there currently is no cure for pulmonary fibrosis, you can improve your quality of life by following these guidelines:

  • See your doctor regularly and follow your plan of care.
  • Talk to your doctor about flu shots and pneumonia vaccines.
  • Take medications as directed.
  • Get plenty of rest.
  • Adhere to a balanced diet of small meals, and maintain a healthy weight.
  • If you smoke, quit now. If you don’t smoke, don’t start, and take care to avoid second-hand smoke.
  • Talk to your doctor about how you can exercise with your condition.
  • Find ways to minimize your exertion during activities of daily living. For example, put often-used items in a spot that is easy to reach, get help for physically demanding chores, and find simpler ways to do things.
  • Talk to others about your condition—both in person and online. Tell family and friends how you feel, and connect with others with the disease to learn how they cope.

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.

Learn About Pulmonary Fibrosis Services at Norton Thoracic Institute

To learn more about our services, call 602.406.4000.