It affects about 70, 000 people worldwide, primarily caucasian (~90%).
The defective gene codes for a chloride
channel that is present on the inner surface of the lungs, GI tract, pancreas
, liver, and other organs. Lack of function of the channel results in thick, viscid mucus secretions in those organs. Obstruction of the airways results in infection and lung damage
, and can be life shortening.