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Cystic Fibrosis

Cystic Fibrosis, also called “CF”, is a disease that affects the glands that produce mucus in the lungs and digestive system. CF may also affect the glands that produce sweat and saliva.

What Causes Cystic Fibrosis?

Your child was born with CF. CF is inherited or passed on to children through genes from both parents. All children are born with certain genes that determine hair and eye color. Your child was born with genes for CF. Parents cannot control what type of genes they pass on to their children.

Early Signs of Cystic Fibrosis

  • Parents may notice a salty taste when they kiss their baby's skin. (Children with CF have too much salt in their sweat and saliva.)
  • A cough that will not go away.
  • Wheezing.
  • Many respiratory infections, such as pneumonia or bronchitis.
  • Stomach pain.
  • Large, loose stools (bowel movements). When solid foods are added to the diet, the stools may be frothy and foul-smelling. Also, oil may appear in the stools and give them a bright yellow or orange color.
  • Failure to grow or gain weight.  

The organs affected by cystic fibrosis:

  • Lungs
  • Liver
  • Pancreas
  • Intestines

Testing for Cystic Fibrosis

If your child has signs of CF, his/her doctor will want to do certain tests.

  • Sweat Chloride Test
    A small sample of sweat is collected and the amount of salt in the sweat is measured. The test is painless. It takes about one (1) hour to collect the sample.
  • Stool Tests A single stool sample will be collected.
  • Chest X-ray This tells the doctor if any lung changes are present.
  • Blood Work To monitor liver function, follow blood sugar levels, and to make sure certain vitamin levels are normal. Once the diagnosis of CF is confirmed, your child may have:
  • PFT's - Pulmonary Function Tests Breathing tests that measure how well the lungs are working.
  • Sputum Culture Checks for bacteria and fungus.
  • Chest X-rays

Chest Physiotherapy

It is very important to keep your child's lungs free of thick mucus. If mucus collects in the lungs, bacteria or fungus can grow and cause lung infections.

Chest physiotherapy (Chest PT) and postural drainage are treatments used to help move the mucus out of the airways. This is one of the best ways to help keep the airways open and prevent wheezing or infection in the lungs. A nurse or respiratory therapist will teach you how to do this. They will also instruct you on aerosol treatment as ordered by your child’s doctor.

Nutrition:

Your child’s doctor and nutritionist will help you plan your child's diet. Below are guidelines for children with CF.
  • Children with CF need extra calories because they use more energy to breathe, and they lose some calories, protein, and vitamins through frequent bowel movements.
  • A diet high in fat and protein, which includes a variety of foods, is needed to promote normal growth. Your child should eat three well-balanced meals a day plus high-calorie snacks and supplements.
  • Most children with CF do not produce enough digestive enzymes. Without them, food cannot be broken down and absorbed into the body to be used for energy and growth. Enzyme capsules must be taken with every meal and snack to digest food, promote good nutrition, and to decrease frequent bowel movements.
  • You may use salt liberally in your child’s diet to replace salt lost in the sweat, especially during hot weather, exercise, or fever.
  • Vitamin and mineral supplements are often needed.

Activity:

Normal play is good for your child. Regular exercise is helpful and encouraged. Exercise strengthens muscles and improves respiratory function. Your child will learn to pace him/herself and not become too tired from play. Bedtime and naptime routines are important too.

School:

School-age children should attend school as often as possible. The teachers, coaches, and school nurse should be aware that your child has CF.

Explain to the teacher and other students that they cannot catch CF. Pamphlets for teachers can be obtained from the CF Foundations/Center.

If medicines are to be taken during school hours, the school nurse and teacher should be aware. For example, the pancreatic enzymes must be taken with meals and snacks.

Prevention of Infections:

If possible, the child should not be in close contact with persons who have colds or other infections.

Keep a record of all routine immunizations and make sure all booster shots are given. A flu shot is strongly suggested each year.

Follow-Up:

Make sure you see your doctor for all scheduled check-ups. If for any reason you cannot, be sure to call.

Research Advances:

The gene that causes cystic fibrosis has been identified. Advances in treatment are hopeful for improving quality of life and longevity.

Disclaimer: This information is not intended to substitute or replace the professional medical advice you receive from your child's physician. The content provided on this page is for informational purposes only, and was not designed to diagnose or treat a health problem or disease. Please consult your child's physician with any questions or concerns you may have regarding a medical condition.

Reviewed: 05/2008