Pulmonary Fibrosis

What is pulmonary fibrosis?

Pulmonary fibrosis is the scarring of your lung tissues over time. It is also called interstitial lung disease. The air sacs and tissues in your lungs swell, scars form, and the tissues become thick and stiff. This affects how much oxygen you get and makes it hard to breathe.

What increases my risk for pulmonary fibrosis?

The cause of pulmonary fibrosis may not be known. The following may increase your risk:

  • Family history of pulmonary fibrosis or other lung disease

  • Chemicals, such as asbestos, silica, or pesticides

  • Tobacco smoke

  • Medical conditions, such as acid reflux, arthritis, or diabetes

  • Medicines, such as antibiotics and antidepressants

  • Treatment for medical conditions, such as radiation

What are the signs and symptoms of pulmonary fibrosis?

  • Trouble breathing that gets worse over time

  • Dry cough

  • Fast heartbeat

  • Clubbing of fingertips and nails (become large, blunt, and rounded)

  • Tiredness and weakness

How is pulmonary fibrosis diagnosed?

Your caregiver will check your oxygen level to see if you need extra oxygen. You may also need any of the following:

  • Blood tests may show damage to your organs from a lack of oxygen.

  • Pulmonary function tests (PFTs) will show how much oxygen your body is getting. 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.

  • A CT scan , or CAT scan, is a type of x-ray that uses a computer to take pictures of your lungs. The pictures may show tissue damage. You may be given a dye before the pictures are taken to help caregivers see your lungs better. Tell the caregiver if you have ever had an allergic reaction to contrast dye.

  • An echocardiogram is a type of ultrasound used to check for high blood pressure in your lungs and heart. It may also show damage to your heart tissue.

  • A lung biopsy may be needed to confirm that you have pulmonary fibrosis. A sample of your lung tissue is sent to a lab for tests.

  • A bronchoscopy is a procedure to look inside your airway. 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.

How is pulmonary fibrosis treated?

There is no cure for pulmonary fibrosis. The goal of treatment is to slow the progress of the disease and treat your symptoms. You may need any of the following:

  • Extra oxygen may be needed 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.

  • Medicines may be given to decrease lung tissue swelling and help prevent scarring.

  • Surgery may be needed if you have severe trouble breathing. You may need part of your lung removed or a lung transplant.

How can I manage my symptoms?

  • Use oxygen at home as directed. It is usually given through a nasal cannula. This is a pair of short, thin tubes that rest just inside your nose. Tell your caregiver if your nose gets dry or if the skin gets red or sore. Never smoke or let anyone else smoke in the same room while your oxygen is on. This can cause a fire.

  • Do breathing exercises at home to help you breathe more easily. Breathe out with pursed or puckered lips. Use your diaphragm to breathe. Put one hand on your abdomen and breathe in, causing your hand to move outward or upward. This helps make more room so your lungs can take in more air.

  • Go to pulmonary rehabilitation (PR). PR is a program run by specialists who will help you safely strengthen your lungs and prevent more tissue damage. The plan includes education about your condition, exercise, breathing strategies, and ways to conserve energy. PR may help decrease your breathing problems and help you function better in your daily activities.

  • Do not smoke. If you smoke, it is never too late to quit. Smoking increases the damage to your lung tissue. Ask your caregiver for information if you need help quitting.

  • Get the flu vaccine every year as soon as it becomes available. You may also need the pneumococcal vaccine to prevent pneumonia.

Where can I find more information?

  • 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
  • Pulmonary Fibrosis Foundation
    230 East Ohio Street
    Chicago , IL 60611-3201
    Phone: 1- 888 - 733-6741
    Web Address: www.pulmonaryfibrosis.org

When should I contact my caregiver?

  • You have a fever.

  • You cough up bloody mucus.

  • You are wheezing.

  • You have swelling in your legs or feet.

  • You need more oxygen than you used to.

  • You have questions or concerns about your condition or care.

When should I seek immediate care or call 911?

  • You have sudden severe trouble breathing.

  • You have a fast heartbeat or chest pain.

  • Your shortness of breath gets worse.

  • You feel so dizzy that you cannot stand up.

  • Your lips or fingernails turn blue.

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. The above information is an educational aid only. It is not intended as medical advice for individual conditions or treatments. Talk to your doctor, nurse or pharmacist before following any medical regimen to see if it is safe and effective for you.

© 2014 Truven Health Analytics Inc. Information is for End User's use only and may not be sold, redistributed or otherwise used for commercial purposes. All illustrations and images included in CareNotes® are the copyrighted property of A.D.A.M., Inc. or Truven Health Analytics.

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