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Lung Health for a Child with Cystic Fibrosis

If your child has cystic fibrosis, you need to work closely with your child’s doctor and the rest of his or her healthcare team. This helps your child to stay healthier, feel better, and have a better quality of life. It also slows the decline in your child’s lung function. Your child’s doctor may suggest many ways to maintain lung health. These include chest physical therapy (CPT), medicine, nutrition, and exercise.

Chest physical therapy (CPT)

Girl sitting in chair wearing vibrating vest.
A vibrating vest is an airway clearance device that helps loosen mucus.

Chest physical therapy helps loosen and clear thick secretions from the lungs. It greatly improves lung function and reduces the amount of lung damage over time. You will work with a physical therapist to learn how to do CPT. CPT often includes 3 methods:

  • Postural drainage. This method places your child’s body in positions that allow mucus to drain.

  • Percussion. This is a clapping method to loosen secretions. An airway clearance device, such as a vibrating vest, is an alternative to manual clapping.

  • Coughing. This is used to help remove lung secretions.

Medicine

Your child may need medicine to prevent or treat lung problems. Many are taken with a nebulizer. This is a device that turns medicine into a mist that your child can breathe in (inhale). Medicines can include:

  • Antibiotics. These can prevent or treat lung infections. They may be taken by mouth (oral), inhaled, or given by IV (intravenously).

  • Bronchodilators. These help to open airways.

  • Anti-inflammatory medicines. These help to decrease airway inflammation.

  • Medicines to thin secretions. These include dornase alpha or hypertonic saline.

  • CFTR (cystic fibrosis transmembrane conductance regulator) modulators. These can help improve lung function. They include ivacaftor or lumacaftor. These medicines can help the defective CFTR protein to work correctly. With cystic fibrosis, the CFTR protein does not work well because of changes (mutations) in the CFTR gene. Mucus then becomes thick and sticky. And there are blockages in the lungs and digestive system. Ask your provider if these medicines are right for your child. 

  • Oxygen therapy. This may be advised to treat low levels of oxygen in your child's blood when they are participating in physical activities or attending school or work.

  • Nutritional supplements. These may be advised when healthy eating is not enough. They may include calcium, multivitamins, oral pancreatic enzymes, sodium, and vitamins A, D, E, and K.

Nutrition

Man holding toddler girl on lap helping her breathe through nebulizer mask.
A nebulizer turns medication into a fine mist that your child can breathe in.

Improving your child’s nutrition can also improve lung health. It does this by limiting infections. It also helps with other problems related to cystic fibrosis. You will work with your child’s healthcare team to do 1 or more of the following:

  • Increase the number of calories your child eats.

  • If advised by the healthcare provider, give your child more foods high in antioxidants (chemicals that reduce damage from inflammation in the body).

  • Give your child prescribed pancreatic enzymes to help with nutrient absorption.

  • Give your child advised vitamin supplements to replace those not well absorbed from food.

Exercise

Encourage your child to be active. Exercise helps your child stay healthier. It improves your child’s overall condition and helps him or her feel better both physically and emotionally. It also helps to loosen mucus, which makes it easier to breathe.

Follow-up care

Your child should see a doctor who is trained in treating cystic fibrosis every 3 months. If a cold or other breathing problem occurs, your child may need to see the doctor more often. Your child can see his or her regular healthcare provider for minor problems not related to cystic fibrosis. Be sure to keep all follow-up appointments. In an age-appropriate way, explain cystic fibrosis to your child. Then he or she can ask providers questions during clinic visits.

When to call your child's healthcare provider

Call the healthcare provider right away if your child has any of the following:

  • More coughing or more sputum

  • Symptoms that get worse, or new symptoms

  • Wheezing

  • Decreased appetite

  • Fever of 100.4° F ( 38°C ) or higher, or as advised by the provider

  • Chest pain or tightness

  • Belly pain

Call 911

Call 911if your child has any of these:

  • Wheezing that gets worse or doesn't get better after treatment

  • Shortness of breath or trouble breathing

  • Trouble swallowing or talking

  • Chest pain or chest tightness that gets worse or doesn't get better after treatment

  • Blue, purple, or gray skin color

  • Fainting or loss of consciousness

© 2000-2019 The StayWell Company, LLC. 800 Township Line Road, Yardley, PA 19067. All rights reserved. This information is not intended as a substitute for professional medical care. Always follow your healthcare professional's instructions.
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