Cystic fibrosis (CF) is the most common fatal genetic disease affecting Canadian children and young adults. There is no cure. CF is a multi-system disease, primarily affecting the lungs and digestive system. In the lungs, where the effects of the disease are most devastating, a build-up of thick mucus causes increasingly severe respiratory problems. It may be difficult to clear bacteria from the lungs, leading to cycles of infection and inflammation, which damage delicate lung tissue.
Mucus and protein also build up in the digestive tract making it difficult to digest and absorb nutrients from food. Large quantities of digestive enzymes must be consumed with every meal and snack. As improved therapies have helped to address the malnutrition issues, virtually all cystic fibrosis-related deaths are due to lung disease.
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