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. Parts of the reproduction system, sinuses and sweat glands are also affected by CF. Cystic Fibrosis is a multiple organ disease. For people with CF, life includes a daily routine of therapy and visits to the CF clinic. Otherwise, most CF individuals lead normal lives in terms of education, physical activity and social relationships. At the moment there is no cure for CF, but there is hope. Thanks to advancements in research and clinical care, increasing numbers of children are surviving into adulthood. Nearly 60% of all individuals with CF in Canada are adults.

CF is one of the most common hereditary diseases, meaning you are born with it. One in every 3,600 children born in Canada has cystic fibrosis. The CF gene is recessive which means a person must inherit two defective genes in order to have CF. Therefore, if you have CF you must have received one CF gene from each of your parents. It is estimated that one in every 25 Canadians is a carrier of the CF gene. A person who is a carrier has only one copy of the CF gene and therefore does not have CF. But this person can pass on the gene to his or her children.


Source: cfeducation.ca

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