Cystic fibrosis (CF) is an inherited disease. It causes a defect in certain cells of the lung and digestive system. The defect makes the cells produce a thick, sticky mucus. This mucus can cause:
- Blockages in the lungs and airways
- Problems digesting and absorbing nutrients
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CF is a serious life-long condition but the severity of illness can vary greatly. The average life expectancy for someone with CF is about 35 years. Although, some with mild forms of CF can live to age 60 or beyond.
CF is a genetic disorder. The child will need to inherit the defective genes from each parent in order to develop CF. Parents that have the gene but do not have CF are called carriers.
Factors that increase your chance of CF include:
- Parents who are known carriers of the CF gene
- Siblings with CF
- Parents with CF (mostly mother since men with CF are often sterile)
The abnormally thick mucus of CF blocks certain organs. This causes many of the symptoms of CF.
Symptoms in infants may include:
- Difficulty passing the first stool (meconium)
- Meconium ileus (intestinal obstruction), sometimes requires surgery
- Salty sweat
Mucus that causes blockages in the lungs may lead to:
- Coughing and wheezing
- Shortness of breath
- Difficulty with exercise
- Repeated pneumonia
- Severe, chronic sinusitis
- Abnormally shaped clubbed finger tips
- Barrel chest
- Nasal polyps
Mucus can also block the pancreas. This can block enzymes used to help you digest food. This can lead to:
- Trouble gaining weight
- Poor growth
- Failure to thrive
- Bulky, bad-smelling, floating stools, due to poor digestion of fats
- Insulin-dependent diabetes
- Liver cirrhosis
Other symptoms may include:
- Salty sweat
- Mildly decreased fertility in females
- Prolapsed rectum
- Prevention of sperm production in males
Overall, girls tend to be affected more severely than boys.
The doctor will ask about symptoms and medical history. A physical exam will be done. CF is suspected in a child with classic symptoms, especially if a sibling has CF.
CF is often diagnosed by symptoms, family history of CF or a positive screening test in newborns. The diagnosis may be confirmed with genetic testing. Other lab tests that may be used to confirm CF include:
- Sweat chloride testing (still the standard for making a diagnosis of CF)
- Transepithelial difference measurement
Your doctor may need to check your lungs. This may be done to look for symptoms or determine treatment. Tests may include:
Tests may also be needed to check the pancreas. These tests may be done to assess symptoms or determine treatment.
There is no cure for CF. Treatment is aimed at:
- Improving the amount of nutrition your body receives
- Preventing and treating lung infections
- Keeping the airways and lungs as clear as possible
Treatment for CF includes:
Better nutrition will help improve overall health. It will also improve growth and development in children. Child