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Cystic Fibrosis

Cystic Fibrosis

Definition

Cystic fibrosis (CF) is an inherited disease. It causes a defect in certain cells of the lung and digestive systems. 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
Cystic Fibrosis
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CF is a serious life-long condition, but the severity of the 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.

Causes

CF is a genetic disorder. A child with CF inherits defective genes from each parent. Parents who have the gene, but do not have CF, are called carriers.

Risk Factors

Factors that increase your chance of CF include:

  • Parents who are known carriers of the CF gene
  • Siblings with CF
  • Parents with CF—more often the mother since men with CF may be sterile

Symptoms

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
  • Salty sweat
  • Intestinal obstruction, sometimes requiring surgery

Mucus that causes blockages in the lungs may lead to:

  • Coughing and wheezing
  • Shortness of breath
  • Difficulty with exercise
  • Abnormally shaped clubbed finger tips
  • Malformed chest

Mucus can also block the pancreas. This can block enzymes used to help you digest food. This can lead to:

  • Bulky, bad-smelling, floating stools, due to poor digestion of fats
  • Diarrhea
  • Trouble gaining weight
  • Poor growth
  • Failure to thrive
  • Malnutrition
  • Dehydration

Other symptoms may include:

  • Jaundice or other symptoms of liver disease
  • Chronic nasal congestion from chronic sinus infections
  • Rectal prolapse
  • Nasal polyps
  • Excessive thirst or urination that may indicate diabetes mellitus type 2
  • Stomach pain or swelling from intestinal blockage
  • Prevention of sperm production in males
  • Mildly decreased fertility in females

Overall, girls are affected more severely than boys.

Diagnosis

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
  • Transepithelial nasal potential difference measurement

Your doctor may need to check your lungs. This may be done to look for symptoms or to determine treatment. Tests may include:

  • Chest and/or sinus x-rays
  • Lung function tests
  • Sputum cultures

Tests may also be needed to check the pancreas. These tests may be done to assess symptoms or determine treatment.

Treatment

There is no cure for CF. Treatment is aimed at:

  • Improving the amount of nutrition your body receives
  • Preventing and treating lung and sinus infections
  • Keeping the airways and lungs as clear as possible

Treatment for CF includes:

Prevention

If you have the defective genes, there is no way to prevent CF.

Adults can be tested to see if they carry the genes before having children. Prenatal testing can determine if a baby will have CF.

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