Cystic fibrosis (CF) is an autosomal recessive disorder that predominantly affects individuals of Northern European descent, occurring at a rate of 1 in 3500. It is caused by a genetic mutation in a gene on chromosome 7, most commonly the ΔF508 mutation, that codes for the cystic fibrosis transmembrane conductance regulator (CFTR) protein. This results in thicker mucus secretions and obstruction pathologies in multiple organs, including the lungs and sinuses.

Sinus disease and chronic sinusitis are common complications in CF patients, as thick mucus obstructs the sinuses. However, the greatest concern is lung disease due to mucus plugging and obstruction, leading to bronchiectasis and purulent sputum production. Pulmonary function testing, including spirometry, is crucial for evaluating and monitoring the progression of CF.

The treatment strategies for CF primarily focus on optimizing lung function, controlling respiratory infections, clearing airways of mucus, and managing other health complications. Medications, therapies, and lifestyle adjustments are used to achieve these goals.

Antibiotics are often prescribed to control respiratory infections, which are common in CF due to lung mucus build-up. Bronchodilators widen the airways and make breathing easier, while mucus thinners help liquefy the thick mucus, aiding its clearance from the lungs.

A newer class of drugs, CFTR modulator therapies, has shown promise in treating CF. These medications, such as ivacaftor (Kalydeco) and lumacaftor (Orkambi), work by improving the function of the faulty CFTR protein, helping to balance the chloride and sodium in the cells and reducing the thickness of mucus.

Airway clearance techniques (ACTs) are critical to daily CF management. These include special breathing and coughing exercises, devices, and therapy vests that help to loosen the mucus in the lungs, making it easier to cough.

Regular exercise is encouraged for individuals with CF as it can help clear mucus from the lungs and improve overall lung function. A high-calorie diet is also recommended to combat the chronic inflammation and malnutrition associated with CF.

In advanced cases of CF-related lung and liver diseases, lung and liver transplants may be considered. Though these are major surgical procedures with considerable risks, they can potentially extend life and improve the quality of life for those with severe CF.