An understanding of cystic fibrosis is important when learning about Pseudomonas aeruginosa (Pa) infections.

What is cystic fibrosis?
Cystic fibrosis (CF) is a genetic     disease. It affects the way in which salt and water move in and out of the body’s cells. In cystic fibrosis patients, thick mucus causes blockages of small tubes and ducts in the body. This mostly affects the lungs and the digestive system. When someone has cystic fibrosis, chloride (part of what makes up salt) cannot pass through the cells normally. And more sodium (the other part of salt) may be pumped through the cells than usual—at least in the lungs. Both of these problems may cause lung mucus and fluid to be thicker and stickier than normal and sweat to be saltier than normal.

What causes cystic fibrosis?
Cystic fibrosis is present from birth, yet signs may not show up for weeks, months, or years afterward. Everyone has 2 genes when they are born that determine whether or not they have cystic fibrosis. Both of these genes need to be abnormal for someone to have cystic fibrosis. The only way to get cystic fibrosis is to inherit 1 cystic fibrosis gene from each parent. Typically, each parent of a cystic fibrosis patient has only 1 cystic fibrosis gene and does not have cystic fibrosis.

What does cystic fibrosis do to the lungs?
People with cystic fibrosis have airway secretions that are drier, stickier, and harder to clear than normal secretions. This thick mucus can block the bronchial tubes in the lungs, and can lead to gradual blockage, infection, and lung damage.

For more information about cystic fibrosis, please visit the Cystic Fibrosis Foundation at cff.org .