Cystic Fibrosis

(CF)

Definition

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
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—mostly the mother since men with CF are often 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
  • Prolapsed rectum
  • 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.

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