Cystic Fibrosis Complications

Cystic fibrosis is an inherited disorder caused by a mutation in the CFTR chloride channel. Meconium ileus may be seen in newborns, and can be the first clue in establishing a CF diagnosis.

Patients can also present with recurrent sinopulmonary infections, such as pneumonia and sinusitis. Viscous, sticky mucus plugs can make clearance of pathogenic bacteria difficult, increasing the risk of infection by bugs like Pseudomonas and Staph Aureus. The chronic inflammation may also lead to nasal polyps, a finding of which should prompt a sweat chloride testing in children. Nail clubbing is also commonly seen in association with all the pulmonary issues.

Infertility commonly occurs due to blockage or absence of the vas deferens in males, and thickened cervical mucus in females.

Pancreatic insufficiency can be caused by blockage of the pancreatic ductules. Digestive enzymes become clogged up in the pancreas, which may result in the cysts and fibrosis for which the disease is named. The lack of pancreatic digestive enzymes also leads to steatorrhea. This pancreatic insufficiency also results in malabsorption of fat soluble vitamins--notably vitamin K--which can cause coagulopathy. To help aid digestion, CF patients can take enzyme supplements before eating.

Treatment options for cystic fibrosis focus on preventing these complications from developing in the first place. Azithromycin can be used to minimize inflammation, thereby reducing the severity of the complications caused by cystic fibrosis.