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Cystic Fibrosis



  • Cystic fibrosis is a disease that causes the body to make thick, sticky mucus. The thick mucus blocks airways, damages the lungs, and makes it hard to breathe. It also damages the pancreas, which makes digestive juices (enzymes).
  • Wash your hands and your child’s hands often to help prevent infections. Stay up to date with the recommended immunizations (shots) for your child including flu and COVID-19. Ask your healthcare provider about any other vaccines your child or your family may need.
  • Treatment may include diet, physical activity, medicines, and learning ways to clear the airways.


What is cystic fibrosis?

Cystic fibrosis (CF) is a disease that causes the body to make thick, sticky mucus. The thick mucus blocks airways, damages the lungs, and makes it hard to breathe. It can also lead to lung and sinus infections.

The thick mucus can also block the tubes that connect the pancreas to the intestines. The pancreas makes digestive juices (enzymes) that help digest food. When this tube is blocked, it’s harder to digest food.

CF can affect all races and ethnic groups.

There is no cure for CF. It is a life-long disease that gets worse over time, but new treatments, including cystic fibrosis transmembrane conductance regulator (CFTR) modulators that target the protein flaw in CF, usually improve the quality and add years of life to people living with CF (PWCF).

What is the cause?

CF is inherited, which means that it is passed from parents to children through their genes. Genes are inside each cell of your body. They contain the information that tells your body how to develop and work. When you have CF, the genes you inherited cause the body to make thick mucus.

To have CF, a child must inherit 1 CF gene from each parent. If a child gets a CF gene from just one parent, the child is a carrier but does not have CF. If both parents have the CF gene, each child has a 25% chance of having CF. Most children with CF are born to parents who do not know that they have the gene for CF. The parents who are carriers usually do not have symptoms of CF.

What are the symptoms?

CF affects people in different ways. Often a child starts having symptoms soon after birth. Other children may have a milder form of the disease and begin to have symptoms as an older child or teenager.

Your child may have some, but not all these symptoms:

  • Frequent coughing that brings up thick mucus
  • A stuffy nose, wheezing, or shortness of breath more often than other children
  • Frequent chest and sinus infections
  • Foul-smelling, greasy bowel movements
  • Skin that may taste salty, which you may notice when you kiss your child
  • Good appetite but poor weight gain and slowed growth
  • Trouble tolerating warm temperatures

How is it diagnosed?

Most children with CF are diagnosed shortly after birth using newborn screening, a sweat chloride test, and genetic testing:

  • A child with CF has more chloride (salt) in their sweat than those who do not have CF. A positive sweat chloride test means that it was high and it is likely that your child has CF.
  • For a genetic test, a blood sample or a swab of cells from the inside of the cheek is sent to the lab to look for the gene changes that cause CF.

How is it treated?

The goal of treatment is to slow down the progress of the disease and help your child lead as normal a life as possible. Treatment for CF includes:


  • Pancreatic enzymes, which help digest food
  • Special CF vitamins and salt
  • Inhaled medicine to thin and help clear mucus from the airway
  • Bronchodilator medicines to help open the airways
  • Antibiotic medicine to treat lung infections
  • CFTR modulators that target the protein flaw in CF

Meal planning and physical activity

Your child may need to eat more calories along with the pancreatic enzyme medicine to grow normally, keep a good weight, have energy, and fight infections. This may mean giving a baby high-calorie breast milk or formula. For a toddler or older child it means increasing the amount of fat and protein in the diet. Milk products are an easy way to add calories. For example, you can give your child whole milk, use extra butter on foods, or add extra cheese to pizza or casseroles. A dietitian who works with PWCF can help you plan your child’s meals

Encourage your child to stay physically active as advised by your child’s provider. Physical activity can help loosen mucus and make the heart and lungs stronger.

Airway clearance

You and your child will learn to clear your child’s airways. You can do some things yourself, and some require special equipment or a trained person to help you. They include:

  • Chest physical therapy (CPT). This involves firm pounding on different parts of your child's chest and back to help move the mucus out of the lungs so that your child can cough up the mucus. Your child’s healthcare provider will show you how to do this and tell you how often you should do it each day.
  • Deep coughing. This is a deep, controlled cough. A deep cough is less tiring and more effective in clearing mucus out of the lungs than a “regular” cough.
  • Self drainage or autogenic drainage (AD). This technique involves breathing 3 different ways to move mucus out of the lungs. Learning to do it right takes some training and practice. It works best for people over 8 years old.

How can I take care of my child?

Follow the full course of treatment prescribed by your child's healthcare provider. In addition:

  • Keep your child away from smoke. Avoid long-term exposure to wildfire smoke, polluted air, dust, fumes, and chemicals. Secondhand smoke from cigarettes, cigars, or pipes is very harmful to children with cystic fibrosis.
  • Make sure your child drinks plenty of liquids. This will help loosen mucus. If your child is sweating a lot during hot weather or activity, drinking a sports drink with extra salt can help your child stay hydrated.
  • To help prevent infections:
    • Wash your hands and your child’s hands often.
    • Stay up to date with the recommended immunizations (shots) for your child including flu and COVID-19. Ask your healthcare provider about any other vaccines your child or your family may need.
  • Have your child tested as often as your child’s provider recommends to see how well your child’s lungs and liver are working and to check for infections or diabetes. Children with CF have a higher risk of diabetes.
  • Ask your child’s healthcare provider:
    • How and when you will get your child’s test results
    • If there are activities your child should avoid and when your child can return to normal activities
    • How to take care of your child at home
    • What symptoms or problems you should watch for and what to do if your child has them

Make sure you know when your child should come back for a checkup. Keep all appointments for provider visits or tests.

For more information, contact:

Developed by Change Healthcare.
Pediatric Advisor 2022.1 published by Change Healthcare.
Last modified: 2021-07-27
Last reviewed: 2021-06-17
This content is reviewed periodically and is subject to change as new health information becomes available. The information is intended to inform and educate and is not a replacement for medical evaluation, advice, diagnosis or treatment by a healthcare professional.
© 2022 Change Healthcare LLC and/or one of its subsidiaries
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