Pulmonary Fibrosis

What is pulmonary fibrosis?

Pulmonary fibrosis is an illness in which the air sacs of the lungs become inflamed and are gradually replaced by scar tissue. (The air sacs are called the alveoli.) As the disease worsens, the scar tissue makes it harder to breathe. It becomes harder for the lungs to get oxygen into the bloodstream.

Pulmonary fibrosis is also called interstitial lung disease. It is most common in adults between the ages of 40 and 70.

How does it occur?

There are over 100 causes of pulmonary fibrosis. It may be caused by chronic infection, cancer, or long-term use of drugs. Examples of drugs that might cause it are anticancer drugs, hydralazine, nitrofurantoin, amiodarone, and drugs for rheumatoid arthritis. Other causes include long-term exposure to asbestos, beryllium, silica, and coal dust, and sensitivity to proteins made by animals such as birds and rats.

Pulmonary fibrosis may be hereditary. There are 2 ways in which this may occur. One is that the condition is inherited and symptoms show up later in life, usually in the forties or fifties. Or children may inherit a tendency to be extra sensitive to the known causes of pulmonary fibrosis, such as asbestos. Therefore they may be more likely to develop the condition when they are exposed to these causes.

When the cause of the fibrosis is not known, it is called idiopathic pulmonary fibrosis.

What are the symptoms?

Symptoms include:

• shortness of breath
• a dry cough
• trouble doing day-to-day activities such as climbing stairs, walking short distances, dressing, talking on the phone, and eating
• enlarged (clubbed) fingertips
• a tendency to get infections, especially lung infections.

How is it diagnosed?

Your healthcare provider will ask about your symptoms and examine you. Tests that may be used to identify pulmonary fibrosis include:

• blood tests
• pulmonary function test or spirometry to measure how well you are breathing.
• chest X-ray
• CT scan
• bronchoscopy (A lighted tube is placed in your airway to look at the lining of the bronchial tubes, get a sample of bronchial tissue, or get a sample of lung secretions.)
• bronchoalveolar lavage (Your healthcare provider rinses the bronchial tubes with a tiny amount of fluid and examines the cells obtained from the rinsings. Your provider can check the cells for inflammation and rule out infections and tumors as a cause of your symptoms.)
• lung biopsy (your healthcare provider removes a sample of lung tissue to examine).

How is it treated?

The aim of treatment is to reduce the inflammation of the alveoli and stop further scarring. Once scar tissue has formed in the lungs, it cannot return to normal.

Your healthcare provider will prescribe drugs, such as prednisone (steroids), cyclophosphamide, azathioprine, and colchicine. These drugs help to fight the inflammation that can lead to scarring. After taking the drugs for at least 3 to 6 months, your healthcare provider can determine if they are helping. If they are, you may continue to take them, possibly for the rest of your life. Your treatment may be stopped or changed if the treatment is not helping you or is not keeping the disease from getting worse.

Your provider may prescribe oxygen therapy. This helps give your body the oxygen it needs. Supplemental oxygen can help you feel less short of breath and more energetic.

Lung transplants may offer hope for people with severe pulmonary fibrosis and other lung diseases. Talk with your provider to see if you might be eligible for a lung transplant.

How long do the effects last?

Pulmonary fibrosis usually develops slowly, sometimes over years. The best chance of slowing the progress of this disease is to seek treatment as soon as possible. You will need treatment for the rest of your life, usually from a lung specialist.

How can I take care of myself?

• Take all medicines as prescribed by your healthcare provider.
• If you smoke, stop.
• Eat a healthy diet to maintain a normal weight.
• Consider eating smaller, more frequent meals. Many people find it easier to breathe when their stomach isn't completely full.
• Try some moderate exercise, such as walking or riding a stationary bicycle. This helps you maintain strength and lung function. Talk to your healthcare provider before starting a new exercise program.
• Practice breathing exercises as recommended by your provider or your respiratory therapist.
• Get a flu shot every year to help prevent infection.
• Get a pneumococcal pneumonia shot.
• Join a support group in your community.

Call your healthcare provider if you have any questions about your condition or its treatment. If you notice anything unusual about how you are feeling or how your medicines are working, call your provider right away.