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Cystic fibrosis

Patient Education
Last reviewed

Cystic fibrosis (CF) is a life-shortening, inherited condition that affects about 30,000 Americans and about 70,000 people worldwide. CF causes bodily secretions to become thick and sticky, interfering with the function of many organs and systems in the human body.

Effects of cystic fibrosis

Illustration showing areas where CF affects the human body

  1. Airways: Thick, sticky mucus clogs air passages, impeding cilia—tiny, hair-like protrusions from cells that line the airways—from removing mucus and inhaled contaminants from the airway. Repeated lung infections can lead to lung damage, causing breathing problems and difficulty getting enough oxygen into the body.
     
  2. Liver: Bile in the liver and gallbladder is thick and has difficulty moving into the intestine, potentially causing inflammation and scarring of the liver. Pancreas: Because pancreatic secretions are abnormally acidic, digestive enzymes are unable to reach the intestine, leading to incomplete digestion and poor absorption of nutrients. Lack of nutrition prevents normal growth in babies and children.
     
  3. Pancreas: Because pancreatic secretions are abnormally acidic, digestive enzymes are unable to reach the intestine, leading to incomplete digestion and poor absorption of nutrients. Lack of nutrition prevents normal growth in babies and children.
     
  4. Intestine: The earliest clinical manifestation of CF, occurring in 13 to 17 percent of infants with CF, is meconium ileus. This bowel obstruction happens when the meconium (the first stool after birth) in a child’s intestine is even thicker and stickier than normal meconium, creating a blockage in the ileum, a part of the small intestine.
     
  5. Fertility: 97 percent of male CF patients and 20 percent of females are infertile. Thick secretions in the sperm canal can block sperm from getting through. Women are often able to conceive but may have decreased fertility because of thick cervical mucus blocking the entry of sperm.
     
  6. Sweat glands: The sweat of people with CF has a high concentration of chloride and sodium. This effect is not harmful but was an indicator in the early days of diagnosing CF.