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Care During Chemotherapy and Beyond
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Side Effects - Symptoms & Solutions

Pulmonary Fibrosis

What is pulmonary fibrosis?

  • Pulmonary fibrosis is an inflammation of the lung, not usually caused by an infection, which evolves into damage and scarring of lung tissue. You may develop pulmonary fibrosis many months after you have completed chemotherapy or radiation therapy. You may have been previously diagnosed with pneumonitis after you have completed therapy.
  • In order to breathe, your lungs need to be stretchy (compliant), in order to fill up with air - like a balloon. In pulmonary fibrosis, the scarred and damaged lungs are hard, and non-compliant, when you breathe. This may cause problems, such as shortness of breath, cough, and other heart and lung problems, such as pulmonary hypertension.
  • Pulmonary hypertension is when pressure in the lung becomes too high. Serious side effects may occur. If it pulmonary hypertension occurs suddenly, you can stop breathing.
  • Pulmonary hypertension may put pressure on the right ventricle of the heart, causing it to enlarge over time. This may lead to a form of heart failure, called right-sided heart failure.

You can develop pulmonary fibrosis from:

  • Certain chemotherapy drugs. These include bleomycin, mitomycin, BCNU and busulfan.
  • Lung injury - Your lung may be damaged from radiation therapy (usually associated with breast or lung cancer, or Hodgkin's disease), or due to trauma
  • Chemicals and occupational exposure- inhaling chemicals, pollution, and environmental irritants, over a long period of time, as a part of your profession may cause pulmonary fibrosis
  • Autoimmune disease - your body's immune system is attacking your lungs, as a part of the disease, which may cause fibrosis. This may happen in systemic lupus erythematosus (SLE), or scleroderma.
  • Your fibrosis may be idiopathic, meaning, your healthcare provider may be unsure why you developed this disease.
  • The shortness of breath (dyspnea) you experience from pulmonary fibrosis may be mild or severe.  You may go through periods when you feel well, and then go through periods when you feel ill.
  • To diagnose this disorder, your healthcare provider may order a chest x-ray, pulmonary (lung) function tests, and perhaps, a bronchoscopy.

What are some symptoms to look for?

  • You may experience no symptoms at first. However, as the disease progresses, you may experience increased coughing, and shortness of breath. 
  • You may be overly tired, or very weak (fatigued). It may be hard for you to do your normal activities as the pulmonary fibrosis worsens.  This may include walking to the door, or climbing stairs.
  • With severe forms of pulmonary fibrosis, you may develop worsening lung function. The lining of your lungs will become less stretchy, making it harder to breathe.
  • You may have trouble lying flat in bed, and you may have to sleep on 2 or more pillows. Your shortness of breath may cause you to wake up in the middle of the night.
  • If you develop severe pulmonary fibrosis, and your heart may not be working as well, your legs may be swollen, especially in your feet and ankles. You may gain "water" weight easily, or feel bloated.

Things you can do:

  • Make sure you tell your doctor, as well as all healthcare providers, about any other medications you are taking (including over-the-counter, vitamins, or herbal remedies). 
  • Remind your doctor or healthcare provider if you have a history of diabetes, liver, kidney, or heart disease. If you have a family history of heart disease, stroke, high blood cholesterol, or high blood pressure, in a first or second-degree relative, you may be at risk for certain problems. Notify your healthcare provider if you have any of these diseases in your family.
  • If you are smoking, you should quit. If you do not smoke, avoid smoke-filled rooms. Smoking first or second-hand can further damage lung tissue. Discuss with your healthcare provider techniques that can help you quit.
  • Avoid people who are sick. Wash your hands often, with soap and water, for at least 15 seconds at a time. Use tissues when you sneeze or cough.
  • Do not share eating or drinking utensils with anyone.
  • If you are over the age of 65 years, or have an altered immune system due to chemotherapy, chronic disease or steroid use, the Centers for Disease Control (CDC) recommends that you receive a flu vaccine every year, and a pneumonia vaccine every 5 years. Discuss this with your healthcare provider if this is right for you.
  • Pulmonary (lung) rehabilitation is the mainstay of therapy for those with pulmonary fibrosis. This includes exercise, air circulation, and oxygen therapy.
  • People with lung problems need to circulate air from the bottom of their lungs and out of your lungs (oxygenation), to prevent infection and pneumonia. Using an incentive spirometer for 15 minutes a day, twice a day, can help promote oxygenation.
  • Some people may have no secretions or sputum. But if you do, controlling secretions through coughing and deep breathing will help you to breathe easier. Remember, if you are dehydrated, your secretions will be thicker, and harder to "bring up". Make sure to drink 2 to 3 liters of fluids (non-alcoholic, non-caffeinated) per day, to remain well hydrated. Taking warm showers or baths, and using a vaporizer, may help to thin out your secretions, if they are present.
  • Try to exercise, as tolerated, to promote air exchange (oxygenation), and to maintain your optimal level of functioning. Walking, swimming, or light aerobic activity may also help you to lose weight, and feel better. Make sure to exercise, under the supervision of your healthcare provider, and discuss with your healthcare provider how you can create a specific exercise program to suit your needs.
  • Oxygen therapy may decrease the amount of work that you have to do to breathe. Discuss this with your healthcare provider.
  • If you are experiencing heart failure, which may be caused by severe pulmonary fibrosis, you may be told to reduce the amount of salt you are eating in a day. Many times, it may be restricted to about 2 grams of sodium per day. A diet lower in salt may decrease the amount of work that is placed on your heart. You should discuss this with your healthcare provider how you can specifically use your diet to control your symptoms.
  • Try to avoid "environmental allergens" (such as smoke, pollution, seasonal allergies), as well as things that may cause allergies in your home (hair sprays, mold, dust mites, and pets). These may trigger an episode of shortness of breath, and make your symptoms worse.
  • Keep a diary of your any abnormal symptoms, such as excessive fatigue, or shortness of breath, if these are occurring regularly. Write down the foods that you have eaten, the exercise or activity you were undergoing when the symptoms occurred, and how you felt before they occurred. This diary may be valuable in identifying certain "triggers" of your symptoms.
  • Questions to ask yourself, may include:
    • Did my symptoms occur gradually, or did this episode come on all of a sudden? Was I feeling anxious? Did I perform any kind of activity, or was I resting?
    • Did I eat any different kinds of foods? Was I around any pets? Did I travel recently? What did I do differently?
  • With severe breathing problems, sleeping at night with your head of the bed elevated may make it easier to breathe. You may do this by sleeping on 2 or 3 extra pillows. This will help lung expansion (spreading out), as well as promote the drainage of secretions.
  • Use relaxation techniques to decrease the amount of anxiety you have. If you feel anxious, place yourself in a quiet environment, and close your eyes. Take slow, steady, deep breaths, and try to concentrate on things that have relaxed you in the past.
  • You should restrict the amount of alcohol you take in, or avoid it all together. Alcohol may adversely interact with many medications.
  • Participating in support groups may be helpful to discuss with others what you are going through. Ask your healthcare provider if he or she is aware of any support groups that would benefit you.
  • If you are ordered a medication to treat this disorder, do not stop taking any medication unless your healthcare provider tells you to. Take the medication exactly as directed. Do not share your pills with anyone.
  • If you miss a dose of your medication, discuss with your healthcare provider what you should do.
  • If you experience symptoms or side effects, especially if severe, be sure to discuss them with your health care team.  They can prescribe medications and/or offer other suggestions that are effective in managing such problems.
  • Keep all your appointments for your treatments.

Drugs that may be prescribed by your doctor:

  • Depending on your lung function, and your overall health status, your doctor may recommend that certain drugs be used to help your lungs function more effectively, and decrease symptoms.
  • Because pulmonary fibrosis is caused by a thickening of your lungs, and restricts your breathing, some of the inhalers and pills that work for other lung diseases may not be as effective. However, if you have pulmonary fibrosis in addition to bronchitis, or another type of lung disease, your healthcare provider may prescribe these to decrease your symptoms.
  • Treatment of pulmonary fibrosis is aimed at preventing the disease for those who are at high risk, and identifying symptoms of lung damage early.
  • There is no good treatment for pulmonary fibrosis. However, supportive care may include corticosteroids, immune system-suppressing agents, pulmonary (lung) rehabilitation, and oxygen therapy.
  • Some of the common drugs that are used to treat pulmonary fibrosis and related pulmonary hypertension may include:
    • Antibiotics - Antibiotics are usually not helpful for a true diagnosis of pulmonary fibrosis, because there is usually not an infection present. However, your doctor or healthcare provider may order antibiotic pills or intravenous (IV), depending on how severe your illness is, if he or she suspects a bacteria infection, and your overall health status. Commonly prescribed antibiotics for bronchitis, pneumonia and respiratory (breathing) problems include azithromycin (Zithromax®), and levofloxacin (Levaquin®). If you are prescribed antibiotics, take the full prescription. Do not stop taking pills once you feel better.
    • Calcium Channel Blockers - These medications may be given to treat high blood pressure in your lungs (pulmonary hypertension), which may be a result of your fibrosis. A few common drugs include Verapamil HCL (Calan®), and Diltiazem (Dilacor XR®).
    • Corticosteroids: Steroids work by decreasing inflammation and swelling, which may be present with certain lung disorders, such as pulmonary toxicity, pneumonitis, and pulmonary fibrosis. People may benefit from steroids, either inhaled, by pill form, or in the vein (IV).
    • People who have an outbreak of severe shortness of breath and airway inflammation may be ordered a steroid pill, such as prednisone, for a short period of time. This may help with your shortness of breath, and chest discomfort, if any is present.
    • Cough medications/Decongestants - may help you to be more comfortable if you are coughing a lot. Guaifenesin is an active ingredient in many cough medications, may be given alone, but is often combined with other drugs, such as codeine, to help your cough.  Guaifenesin may also be combined with pseudoephedrine (Sudafed®

      ) as a decongestant, or any one of many medications, depending on your symptoms.  Another common medication you may receive is Hydrocodone Bitartrate-Homatropine Methylbromide (Hycodan®

      ). This is a narcotic antitussive (anti-cough medication), which will help relieve your cough.
    • Diuretics - may be known as "water pills", as they work to prevent or treat lung congestion by making you urinate out extra fluid, if your fibrosis has progressed into heart failure. . Some examples of this medication may include furosemide (Lasix®), and Hydrochlorthiazide. You may receive this medication alone or in combination with other medications.
    • Immune suppressing agents - such as cyclophosphamide (Cytoxan®) and azathioprine (Imuran®), are used alone or in combination with steroids if the cause of your pulmonary fibrosis is unknown, or idiopathic.  Sometimes, your doctor or healthcare provider
    • Narcotics - Such as Morphine Sulfate may be useful, in certain cases of shortness of breath, to relieve your body's need to breathe. Morphine is called an Opiate. Opiates tell your breathing center in your main brain stem not to breathe so hard. This might make you more comfortable. If you are taking morphine, though, make sure you discuss with your healthcare provider common side effects, such as constipation, drowsiness, nausea and vomiting, and how to control these side effects.
    • Oxygen therapy - If you are experiencing shortness of breath at rest, or on exertion, your healthcare provider may see if oxygen therapy is right for you. You may take oxygen when your symptoms are at their worst. For example, some people are only on oxygen at nighttime, and not during the day. Some take oxygen when they are performing activities, but not all the time.
    • Vasodilators -are drugs that work by opening up or "dilating" the vessels. These drugs may be given to you if you have developed pulmonary hypertension as a result of your fibrosis. These may include isosorbide dinitrate (Isordil®).
  • Your healthcare provider will discuss with you which treatments are helpful to you.
  • Do not stop any medications abruptly, as serious side effects may occur

When to call your doctor or health care provider:

  • Fever of 100.5º F (38º C), chills, sore throat (possible signs of infection if you are receiving chemotherapy).
  • If you cough up blood
  • Shortness of breath, chest pain or discomfort; swelling of your lips or throat should be evaluated immediately
  • Feeling your heart beat rapidly (palpitations)
  • Any new rashes on your skin
  • Any unusual swelling in your feet and legs
  • Weight gain of greater than 3 to 5 pounds in 1 week.

Note:  We strongly encourage you to talk with your health care professional about your specific medical condition and treatments. The information contained in this website is meant to be helpful and educational, but is not a substitute for medical advice.