Cystic fibrosis (CF) is an inherited, life-limiting disease that affects the body’s exocrine glands, which produce mucus, saliva, sweat and tears.
In CF, a genetic mutation disrupts the delicate balance of sodium, chloride and water within cells, causing the exocrine glands to secrete fluids that are poorly hydrated and therefore thicker and stickier than fluids in people without CF. This leads to chronic problems in various systems of the body, particularly the lungs and pancreas, and the digestive and reproductive systems. In the lungs of a CF patient, the thick mucus and the thinning of the airway surface liquid make it nearly impossible for the cilia to clear bacteria from the airway. This severely impairs the natural airway-clearing processes and increases the potential for bacteria to be trapped, leading to respiratory infections that may require hospitalization. Impairments in these vital lung defense mechanisms typically begin in early childhood and often result in chronic secondary infections, leading to progressive lung dysfunction and deterioration. Although the life expectancy of CF sufferers has increased over the past few decades due to better management of the disease, the median life expectancy today for patients with cystic fibrosis is only 31 years of age. According to the Swiss Society for Cystic Fibrosis (CFCH), there are about 1,000 people affected by cystic fibrosis in Switzerland.