Cystic Fibrosis: What Is It?

Cystic Fibrosis(CF) is an inherited condition that causes sticky, thick mucus to build up in the lungs and digestive system. CF becomes a disease when it disrupts the normal function of epithelial cells. The CF gene directs the body’s epithelial cells to make an abnormal form of a protein called Cystic Fibrosis Tansmembrane Conductance Regulator (CFTR).

CFTR is found in cells that border the lungs, digestive system, sweat glands, and genitourinary system. When the CFTR is not working properly, epithelial cells cannot control the way chloride travels across the cell membrane. This interrupts the critical balance of salt and water that is needed to keep an ordinary thin coating of mucus and fluid inside the lungs, pancreas, and passageways in other organs.

The mucus becomes thick, sticky and permanent. The mucus in lungs usually catches and traps germs before disposing them. With CF, the mucus cannot dispose these germs because it adheres to the lungs. If germs stay in the lungs for too long, the lungs may become infected and diseases begin to form.

Children with CF have trouble gaining weight even with a normal diet because in the pancreas thick mucus may block the passages that usually carry essential enzymes to the intestines to digest food. The child’s body cannot process and absorb nutrients in the normal way.