Health Library

Cystic Fibrosis in Children

What Are The Symptoms of Cystic Fibrosis?

People with Cystic Fibrosis can have a variety of symptoms, including:

• Salty-tasting skin
• Daily cough, at times with mucus
• Lung infections
• Shortness of breath
• Poor growth or slow weight gain even with a good appetite
• Frequent greasy, bulky, foul-smelling stools or trouble having a bowel movement

What Are The Causes of Cystic Fibrosis?

Cystic fibrosis is a genetic disease. This means that people inherit it from their parents through genes (or DNA).

To have cystic fibrosis, a person must inherit two copies of the defective CF gene, getting one copy from each parent. Carriers are not affected.

How Common is Cystic Fibrosis?

About 35,000 children and adults in the United States have CF.

How Is Cystic Fibrosis Diagnosed?

Most people are diagnosed with CF at birth with newborn screening, or before 2 years of age. A doctor who sees the symptoms of CF will order a sweat test or a genetic test to confirm the diagnosis.

A sweat test is the most common test used to diagnose CF. It is a painless test. A small electrode (disk) is placed on the skin (usually on the arm) to get the sweat glands to make sweat. The sweat is collected, and the amount of chloride (a part of salt) is measured. An elevated level of chloride means that the person has cystic fibros 

Notes:

• Mmol/L is a measure of concentration. The new range for all ages is less than 30 is normal and 30-59 is borderline, 60 and above is high.
• Borderline chloride levels are reviewed on a case-by-case basis.

The best place to get a sweat test done is at a Cystic Fibrosis Foundation-accredited care center.

How is Cystic Fibrosis Treated?

Each day most people with Cystic Fibrosis:

• Take pancreatic enzyme supplement capsules with every meal and most snacks to help digest fat and protein. Even babies who are breastfeeding may need to take enzymes.
• Take multi-vitamins, especially the fat-soluble vitamins A, D, E and K.
• Make sure they get plenty of salt by adding salt to food or eating salty snacks.
• Eat a high-calorie, high-protein diet.
• Clear the lungs at least twice and sometimes up to four or more times a day. This is done using techniques called “Airway Clearance.” In infants, it is done by clapping on the chest in a certain way. In toddlers, children, and adults, it is done using a variety of approaches, such as a therapy vest that shakes the chest wall. This helps move the thick mucus out of the lungs and lessens the chance of lung infection.
• Take liquid medicines that are made into a mist or aerosol and then inhaled through a nebulizer (called aerosolized medicines). There are several types of aerosolized medicines with different actions including thinning the mucus, opening the airways, and fighting infection.

Because the severity of CF differs from person to person, and CF lung infections flare up from time to time, there is no "typical" day. Each child's doctor will decide which, if any, of these medicines and treatments are needed.