Pharmacy Clinical Pearl of the Day: Cystic Fibrosis

Cystic fibrosis affects the cells that produce mucus, sweat, and digestive juices.

Clinical Pearl of the Day: Cystic Fibrosis

Cystic fibrosis (CF) is an inherited disorder that causes severe damage to the lungs, digestive system and other organs in the body.

Insight:

  • CF affects the cells that produce mucus, sweat and digestive juices.
  • These secreted fluids are normally thin and slippery. But in people with CF, a defective gene causes the secretions to become sticky and thick.
  • Symptoms may include a persistent cough that produces thick mucus (sputum), wheezing, exercise intolerance, repeated lung infections, inflamed nasal passages or a stuffy nose, and recurrent sinusitis.
  • Risk factors include family history and most common in white people of Northern European ancestry.
  • Diagnosis includes newborn screening and older children, if not screened before.
  • Treatment includes combination products such as Trikafta, Symdeko, Orkambi, and Kalydeco. Pulmonary rehabilitation may also be an option
  • Surgery options include nasal and sinus surgery, oxygen therapy, non-invasive ventilation, feeding tube, bowel surgery, and lung transplant.

Sources:

Cystic fibrosis - Diagnosis and treatment - Mayo Clinic