Discharge Instructions for Cystic Fibrosis

Your child has been hospitalized with cystic fibrosis. This is an inherited, long-term disease that affects the lungs. It affects the lungs and digestive system most often. It is caused by mutations in a gene called the CFTR (cystic fibrosis transmembrane conductance regulator). These mutations cause the CFTR protein not to work correctly in the body. The defective CFTR protein causes mucus to be thick and sticky. The mucus clogs the lungs and blocks the release of enzymes from the pancreas. This may cause serious lung infections and problems with digesting and absorbing food.

There is no cure for cystic fibrosis. But there are treatments that can help your child have fewer lung infections and digestive problems and improve their life overall.

Preventing infection

• Help keep your child’s lungs clear of extra mucus. Learn how to do chest physical therapy on your child to help with this. This includes postural drainage and percussion. Ask your child's healthcare provider for instructions.

• Medicines called CFTR modulators may be prescribed. They can help the defective CFTR protein work correctly. Help your child take medicines as prescribed by their healthcare provider.

• Remind your child to wash their hands often, and correctly.

  • They should use soap and water and do a lot of rubbing. Scrub for at least 20 seconds. To help meet the time limit, sing the "Happy Birthday" song twice. Teach them to scrub between their fingers and under their nails.

  • Make sure you have 60% alcohol-based hand cleaner when soap and water aren't available.

  • Teach your child to keep their hands away from their face. Germs often get into the nose and mouth and then into the lungs this way.

• Ask your child's healthcare provider about a yearly flu shot and other vaccines.

• Stay away from people who are sick. Also stay away from crowds, especially in the winter, when more people have colds and the flu.

Aiding digestion

• Learn about the special dietary needs of your child. Your child may need pancreatic enzymes to help with digestion.

• If prescribed, make sure your child takes pancreatic enzymes exactly as instructed.

• Nutritional supplements may be advised when healthy eating is not enough. These may include calcium, multivitamins, oral pancreatic enzymes, sodium, or vitamin A, D, E, and K. Talk with your child's healthcare team about supplements your child may need.

• A nutritionist or dietitian can help you and your child. Ask your child's healthcare provider for a referral.

• Some children have problems growing and gaining weight. Talk with your child's provider or nutritionist about which types and amounts of foods or supplements to include in your child's diet.

Other home care

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

• Encourage your child to exercise regularly and drink lots of fluids, as directed by their provider.

• Your child should see their healthcare provider at least every 3 months, or as directed.

• Don't let people smoke in your home, car, or anywhere near your child. Stay away from public places where smoking is allowed.

• Talk with your child about the dangers of smoking. They should not smoke and should stay away from others who do. Teach them about the dangers of e-cigarettes and secondhand smoke.

• Be sensitive to your child’s mental health needs. Living with cystic fibrosis can be overwhelming. Connect with a support group or get professional counseling for your child and other family members as needed.

Follow-up

Make all follow-up appointments as soon as possible after leaving the hospital. Contact your child's healthcare provider sooner if you have any questions or concerns.

Ask about the closest Cystic Fibrosis Center. These centers specialize in caring for children and adults with cystic fibrosis. You can check the Cystic Fibrosis Foundation website at www.cff.org. Or call 800-FIGHT-CF (800-344-4823).

When to call your child's healthcare provider

Call the healthcare provider right away if any of the following occur:

• Sudden shortness of breath or wheezing

• Sudden chest pain or tightness

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

• Symptoms that get worse or new symptoms

• Symptoms of depression

• Severe constipation

• Severe diarrhea

• Belly pain

• Vomiting

• Decreased appetite or weight loss

• More mucus than normal, or mucus that is bloody or dark in color

• Change in color of sputum

• Trouble taking part in daily activities

• More tired than normal

Each child is different. So be sure you understand when to call the healthcare provider about your child's specific symptoms.