Pulmonary Fibrosis

What is pulmonary fibrosis?

• Pulmonary fibrosis is also called interstitial pulmonary fibrosis, interstitial lung disease, or ILD. This includes different problems that affect the lungs. With pulmonary fibrosis, the lung tissues are damaged in some known or unknown ways. The air sacs in the lungs become inflamed (swollen) and scars are formed. The lung tissues eventually become thick and stiff. This leads to decreased transfer of oxygen to the blood and makes breathing hard.
  
  
• The lungs are two hollow organs in your chest. These stretch like a balloon when you breathe in, and collapse as you breathe out. The lungs are made of lobes (sections), blood and lymph vessels, nerves, and alveoli (air sacs). The lungs connect to tubes or airways which bring air into the lungs.

What causes pulmonary fibrosis?

Sometimes, the exact cause of pulmonary fibrosis is not known. The following are some of the risk factors and common causes of pulmonary fibrosis:

• Chemicals or toxins: Being around certain chemicals a lot may cause pulmonary fibrosis. These chemicals may include asbestos, silica, or pesticides. Those who also raise birds, cut or polish stones, or work in hair parlors may increase their risk.
  
  
• Cigarette smoking: Smoking or being around smokers may also cause pulmonary fibrosis.
  
  
• Diseases: Infections and certain diseases, such as sarcoidosis, diabetes and arthritis, may trigger pulmonary fibrosis.
  
  
• Drugs or radiation: Antibiotics and certain treatment for depression and cancer (radiation) may cause pulmonary fibrosis.
  
  
• Genetics: Your chance of having pulmonary fibrosis is higher if you have a family member with pulmonary fibrosis or other lung diseases.

What are the signs and symptoms of pulmonary fibrosis?

You may have one or more of the following signs and symptoms:

• Trouble breathing, especially with exercise.
  
  
• Dry cough (without fluid).
  
  
• Tiredness and weakness.
  
  
• Clubbing of fingertips and nails (large and bulb-like).
  
  
• Weight loss.
  
  
• Heart failure with leg swelling.
  
  
• Chest pain.

How is pulmonary fibrosis diagnosed?

You may have one or more of the following:

• Blood gases: This is also called an arterial blood gas, or ABG. Blood is taken from an artery (blood vessel) in your wrist, arm, or groin. Your blood is tested for the amount of oxygen and carbon dioxide in it. The results can tell caregivers how well your lungs are working.
  
  
• Blood tests: You may need blood taken to give caregivers information about how your body is working. The blood may be taken from your hand, arm, or IV.
  
  
• Bronchoscopy: This is a procedure to look inside your airway and learn the cause of your airway or lung condition. A bronchoscope (thin tube with a light) is inserted into your mouth and moved down your throat to your airway. You may be given medicine to numb your throat and help you relax during the procedure. Tissue and fluid may be collected from your airway or lungs to be tested.
  
  
• Chest x-ray: This is a picture of your lungs and heart. Caregivers use it to see how your lungs and heart are doing. Caregivers may use the x-ray to look for signs of infection like pneumonia, or to look for collapsed lungs. Chest x-rays may show tumors, broken ribs, or fluid around the heart and lungs.
  
  
• Computerized tomography scan: This test is also called a CT or CAT scan. A special x-ray machine uses a computer to take pictures of your lungs. Before taking the pictures, you may be given dye through an IV in your vein. The dye helps the lungs, heart, and blood vessels show up better in the pictures. People who are allergic to iodine or shellfish (lobster, crab, or shrimp) may be allergic to this dye. Tell your caregiver if you are allergic to any of these.
  
  
• Open lung biopsy: An open lung biopsy is done in the operating room. You will be given medicine to make you sleep. The skin on your chest will be cleaned with a special kind of soap. During the biopsy a small amount of tissue will be removed from your lung. The tissue will be sent to the lab for tests. Your wound will have stitches or staples, and may have a bandage.
  
  
• Pulmonary function tests: Pulmonary function tests (PFTs) help caregivers learn how well your body uses oxygen. You breathe into a mouthpiece connected to a machine. The machine measures how much air you breathe in and out over a certain amount of time. PFTs help your caregivers decide the best treatment for you.

How is pulmonary fibrosis treated?

There is no cure for pulmonary fibrosis. Your caregiver may help slow down the process and treat your symptoms if pulmonary fibrosis is found early. You may have one or more of the following:

• Medicines:
  
  
  • Immunosuppressants: These medicines are often given with steroids to weaken the activity of your immune system. The immune system is the part of your body that fights infection.
    
    
  • Steroids: This medicine may be given to decrease inflammation.
  
  
• Oxygen: You may need extra oxygen if your blood oxygen level is lower than it should be. You may get oxygen through a mask placed over your nose and mouth or through small tubes placed in your nostrils. Ask your caregiver before you take off the mask or oxygen tubing.
  
  
• Surgery: You may have surgery to remove a part of your lung. You may also have a lung transplant if your lung has very serious disease. The diseased lung is removed and replaced with a healthy and donated lung.

Where can I find support and more information?

Pulmonary fibrosis is a life-changing disease for you and your family. Accepting that you have pulmonary fibrosis is hard. You and those close to you may feel angry, depressed, or frightened. These are normal feelings. Talk to your caregivers, family, or friends about your feelings. You may also want to join a support group. This is a group of people who also have pulmonary fibrosis. Contact the following support group for more information about pulmonary fibrosis:

• American Lung Association
  1301 Pennsylvania Ave. NW
  Washington , DC 20004
  Phone: 1- 202 - 785-3355
  Phone: 1- 800 - 548-8252
  Web Address: www.lung.org
  

• National Heart, Lung and Blood Institute
  Health Information Center
  P.O. Box 30105
  Bethesda , MD 20824-0105
  Phone: 1- 301 - 592-8573
  Web Address: http://www.nhlbi.nih.gov/health/infoctr/index.htm
  

• Pulmonary Fibrosis Association
  P.O. Box 75004
  Seattle , WA 98125-0004
  Phone: 1- 206 - 417-0949
  Web Address: http://www.pulmonaryfibrosisassn.com
  

Care Agreement

You have the right to help plan your care. Learn about your health condition and how it may be treated. Discuss treatment options with your caregivers to decide what care you want to receive. You always have the right to refuse treatment.

Learn more about Pulmonary Fibrosis