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Cystic Fibrosis

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KEY POINTS

  • 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 is important to prevent infections by washing your hands often and getting recommended vaccines, such as flu and pneumococcal shots.
  • Treatment may include diet, exercise, medicines, and learning ways to clear the airways.

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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 tube that connects the pancreas to the intestines. The pancreas makes digestive juices that help digest food. When this tube is blocked, it’s harder for you to digest food.

CF can affect all races and ethnic groups, but it is seen mostly in white people with northern European ancestors.

There is no cure for CF. It is a life-long disease that gets worse over time. Many people with CF live into their mid 30s. Children born after 2000 may live longer because of better ways to manage CF.

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, he or she is a carrier but does not have CF. If both parents have the CF gene, each child has a 25% chance of having CF.

What are the symptoms?

CF affects people in different ways. Often children start having symptoms soon after birth. Other children may have a milder form of the disease that doesn't show up until they are teenagers or young adults.

Your child may have some, but not all of 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 before they are a year old. Newborn screening blood tests include a test for CF. A positive blood test needs to be confirmed with a sweat test or genetic test.

  • Children with CF have more salt in their sweat. To do the sweat test, a small wire is put on the skin. A weak electrical current causes the area to sweat. The sweat is collected and tested in a lab. This test does not hurt.
  • For a genetic test, a blood sample or a swab of cells from the inside of the cheek are 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:

Medicines

  • Antibiotics to help prevent lung infections
  • Anti-inflammatory medicine, like ibuprofen, to help reduce swelling in the lungs (Nonsteroidal anti-inflammatory medicines, or NSAIDs, such as ibuprofen, may cause stomach bleeding and other problems. Read the label and give as directed. Unless recommended by your healthcare provider, your child should not take an NSAID for more than 10 days.)
  • Bronchodilators, which are drugs that open the airways
  • Medicine that helps your child have thinner mucus
  • Pancreatic enzymes, which help the intestines digest food
  • Special vitamins

Diet and Exercise

Your child may need to eat more calories 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.

Encourage your child to exercise according to your healthcare provider’s recommendations. Exercise can help loosen mucus and make the heart and lungs stronger.

Airway clearance

You and your child will learn ways 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. Secondhand smoke from cigarettes, cigars, or pipes is very harmful to children with cystic fibrosis.
  • Make sure your child drinks plenty of fluids. This will help loosen mucus. If your child is sweating a lot during hot weather or activity, drinking a sports drink can provide both fluids and salt.
  • To help prevent infections:
    • Wash your hands and your child’s hands often.
    • Make sure that your child is up to date with the recommended vaccines, such as flu and pneumococcal shots.
  • 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.

Developed by RelayHealth.
Pediatric Advisor 2016.4 published by RelayHealth.
Last modified: 2016-10-26
Last reviewed: 2015-05-26
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.
Copyright © 2016 RelayHealth, a division of McKesson Technologies Inc. All rights reserved.
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