What is Cystic Fibrosis?

Cystic Fibrosis (CF) is an inherited disease that causes problems with a group of glands, called the exocrine glands. Exocrine glands normally produce thin, slippery secretions. In CF, the secretions are thick and sticky. CF mainly causes problems in the respiratory and digestive systems, but other systems are affected as well.

Normally, glands in the airway walls of your lungs produce thin, slippery mucous for a specific purpose – to help trap dust and bacteria that we inhale on a daily basis. This thin mucous is then moved up towards the trachea (windpipe) by tiny beating hairs called cilia where it can be coughed up or swallowed. In CF lungs this mucous is thick and sticky, making it harder for the cilia to do their job of sweeping the mucous away from your lungs. This thick mucous then builds up in the airways and plugs them up. This can cause air trapping, airway closure and infection.

Thick mucous may also block the ducts of the pancreas. The pancreas is a gland located below and behind the stomach. It is one of the important organs of the digestive tract. It supplies enzymes needed to digest food. When openings from the pancreas are blocked, enzymes cannot reach the small intestine. As a result, food is only partially digested and the nutritional value reduced. In fact, 85% of people with CF don’t secrete enough enzymes to completely digest food. This leads to weight loss and poor growth.

Thick sticky mucous can also block the ducts of the liver called bile ducts. Bile ducts are small tubes that carry bile form the liver. Bile is used for fat digestion.