Cystic Fibrosis and the Respiratory System How does cystic
fibrosis (CF) affect the respiratory system? Cystic fibrosis
(CF) is an inherited disease characterized by an abnormality in
the body's mucus glands. It is chronic, progressive, and is
usually fatal. Due to improved treatments, people with CF, on
average, live into their mid to late 30s. Children with CF have
an abnormality in the function of a cell protein called the
cystic fibrosis transmembrane regulator (CFTR). CFTR controls
the flow of water and certain salts in and out of the body's
cells. As the movement of salt and water in and out of cells is
altered, mucus becomes thickened. In the respiratory system,
mucus is normally thin and can easily be cleared by the airways.
With CF, mucus becomes thickened and sticky and results in
blocked airways. Eventually, larger airways can become plugged
and cysts may develop. Lung infections are very common in
children with CF, because bacteria that are normally cleared,
remain in the thickened mucus. Many of