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.
- 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.
Cystic fibrosis - Diagnosis and treatment - Mayo Clinic