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Overview and Mission

Cystic fibrosis (CF) affects multiple organs and can cause gastrointestinal disease, liver disease, pancreatic disease, reproductive disorders and diabetes in addition to lung disease, the most common cause of CF-associated limitation of life expectancy.

Researchers and clinicians specializing in diverse physiological systems have interacted to provide a comprehensive understanding of the disease. Our approach spans the full spectrum, from detailed studies of the underlying genetic defect, its molecular structure and function to patient-based research and excellent clinical care. Other researchers in our group focus on understanding how bacteria infect the lung of CF patients with the goal of preventing the initial stages of infection that can damage a patient’s lung.

This multidisciplinary approach has characterized CF research at SickKids for decades, from first recognizing of the need for high-calorie diets to maintaining CF patient health (Corey and Crozier, 1976) to the discovery of the CF gene (Riordan, Rommens and Tsui, 1989).

Since 1989, SickKids has fostered and maintained a comprehensive website, with details of over 1600 mutations in the CF gene that cause CF and related diseases. With contributors and users from around the world, the CF Mutation Database is a major resource for clinical and basic research and serves as a model for other genetic disease databases.

State-of-the-art laboratory research in CF is linked to outstanding clinical research and clinical care in a rich collaborative environment that has existed within SickKids for the past three decades. The clinical program for CF at SickKids is known as one of the leading care facilities in the world and has a well-established reputation for its excellence in multidisciplinary care. The synergy between laboratory, clinical research and clinical care places SickKids at the forefront for future CF therapy development and implementation in patients.