Pulmonary Function and Cystic Fibrosis

Impacts to pulmonary function, along with other symptoms, caused by cystic fibrosis can vary according to the severity of the disease. Generally, cystic fibrosis causes unusually thick mucus to accumulate in the lungs, causing problems with breathing.

The unusually thick mucus caused by cystic fibrosis affects tiny hairs that line the surface of the lung's breathing tubes, which are called cilia. The cilia in ordinary lung function sweep mucus and any breathed in foreign particles, such as dust or germs, directing them up to the throat where they are coughed out or swallowed.

For a person suffering from cystic fibrosis, the mucus is so thick that the cilia are unable to expel the germs and other foreign particles out of the lungs very easily. This in turn leads to frequent lung infections, such as bronchitis and pneumonia, which finally leads to a gradual damaging of the lungs.

During lung infections, the body produces white blood cells that release chemicals that attack the bacteria and surrounding normal cells. When the white blood cells die, they add to the thickness of the mucus and contribute to further damaging of the lungs. The mucus can also cause repeated infections of the upper respiratory system, causing polyps and other growths to appear in the nose and sinus areas. Eventually, as the lungs deteriorate, the heart is forced to pump harder due to decreased lung capacity, which in turn increases pressure on the lungs as blood flow increases. The heart can also become enlarged, leading to heart failure and death.

Cystic fibrosis also adversely affects the digestive system, especially the pancreas.

There is no cure for cystic fibrosis as of yet. The average age of survival for a person suffering from cystic fibrosis is the mid to late 30s. However, modern therapies have allowed some people with cystic fibrosis to live into their forties and beyond.