Cystic fibrosis (CF) is an inherited (genetic) disease in which excess mucus clogs the lungs, prevents food from being digested, and damages the reproductive system. It is the most common life-threatening genetically inherited disease affecting children and young adults. The incidence of CF occurs in about 1 in 3,600 live births in Canada.
In CF, an abnormal protein called CFTR is produced. This protein changes the way chloride (a component of salt, which is also called sodium chloride) moves in and out of cells. This affects the balance between salt and water in the body, making the mucus that lines the lungs, pancreas, and other organs thicker and stickier.
CF affects all the body's exocrine glands. These glands create and secrete chemicals necessary for proper functioning of the body. The pancreas, for example, is an exocrine gland that provides digestive enzymes for the stomach. The sweat glands provide liquid to cool the skin. In CF, some glands produce abnormal substances. The sweat glands, for example, release high levels of salt. Other glands, like the pancreas, become plugged with mucus. Because of the many organs affected and thanks to newborn genetic screening, CF is usually diagnosed early in childhood, with over half of children being diagnosed before the age of 1.Thanks to good research, more and better medications, and early diagnosis, people with CF are living longer, fuller lives.