Physical activity and exercise training in young people with cystic fibrosis: Current recommendations and evidence

Despite the acceptance of physical activity (PA) being integral to a young person's health, children with disability often exhibit low levels of PA. In young people with cystic fibrosis (CF) the importance of exercise and daily PA is acknowledged by clinicians and their support teams, however, there is a lack of knowledge related to its prescription. CF is a recessive genetic disorder affecting the lung, pancreas and sweat glands. CF is the most common life shortening genetic disease in the Caucasian population for which there is no cure. In the UK, CF affects over 9000 people, with 4000 under 16 years of age. Only about half of the CF population can expect to live beyond 40 years of age. Besides drug therapies, rehabilitative exercise programmes form an important component of treatment and long term exercise programmes are considered positive treatment strategies, but all lack any detailed prescriptive information. Several reviews and editorials have highlighted the lack of evidence based research in PA and exercise training in young people with CF; but advocate a greater need for understanding the role of exercise in therapeutic interventions. The purpose of this review is to update the reader on the current recommendations and evidence in PA and exercise training for young people with CF. These developments have extended our understanding of PA and exercise training in children and adolescents with CF, and its implementation in the management of this chronic disease.