Cystic fibrosis (CF) is the most common fatal genetic disease affecting approximately 4,000 Canadians. 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 (average of 20 pills a day) 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.
At the present time the median predicted age of survival in Canada is approximately 48 years, but half of CF patients who died in 2011 were under the age of 34.