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Introduction: Cystic Fibrosis (CF) is a complex, progressive, life-limiting disease predominantly affecting children and young adults. Regular participation in physical activity is strongly encouraged in those with CF as aerobic fitness has been shown to be an independent predictor of survival, and associated with improved quality of life (QOL). In children with CF anaerobic power, aerobic capacity, QOL and nutrition have been identified as predictors of physical activity participation. Following puberty, participation in physical activity declines in girls with CF. With the increasing life expectancy of people with CF, coupled with the emergence of unique disease factors within the adult CF population, it is insufficient to assume that treatment-disease relationships that exist in childhood will continue in adulthood. We aimed to identify clinical and demographic features associated with moderate intensity physical activity participation in adults with CF. Method(s): Adults with CF and stable respiratory health completed clinical assessments of grip strength (hand-held dynamometry), muscle power (vertical jump height), exercise capacity (Modified Shuttle Test-25 (MST-25)) and QOL. Participants proceeded to wear an activity monitor (Sensewear Pro3 armband) to assess daily habitual physical activity participation. Relationships between time spent in moderate intensity physical activity (>=4.8 and < 7.2 METs) and markers of strength, power, exercise capacity and QOL were assessed using Spearman rank order correlations. Wilcoxon signed-rank test was used to assess differences in time spent in moderate intensity activity between males and females. Result(s): Fifty-two adults with CF (26 male), mean age 28 years (range 19-51 years) withmeanFEV1 67% predicted (range 32-110% predicted) completed clinical assessments. Fifty participants provided activity monitor data from a mean 5.4 (SD 0.1) assessment days. Two participants had insufficient activity monitoring data for inc

Dysfunction of CFTR in cystic fibrosis (CF) airway epithelium perturbs the normal regulation of ion transport, leading to a reduced volume of airway surface liquid (ASL), mucus dehydration, decreased mucus transport, and mucus plugging of the airways. CFTR is normally expressed in ciliated epithelial cells of the surface and submucosal gland ductal epithelium and submucosal gland acinar cells. Critical questions for the development of gene transfer strategies for CF airway disease are what airway regions require CFTR function and how many epithelial cells require CFTR expression to restore normal ASL volume regulation and mucus transport to CF airway epithelium? An in vitro model of human CF ciliated surface airway epithelium (CF HAE) was used to test whether a human parainfluenza virus (PIV) vector engineered to express CFTR (PIVCFTR) could deliver sufficient CFTR to CF HAE to restore mucus transport, thus correcting the CF phenotype. PIVCFTR delivered CFTR to .60% of airway surface epithelial cells and expressed CFTR protein in CF HAE approximately 100-fold over endogenous levels in non-CF HAE. This efficiency of CFTR delivery fully corrected the basic bioelectric defects of Cl2 and Na+ epithelial ion transport and restored ASL volume regulation and mucus transport to levels approaching those of non-CF HAE. To determine the numbers of CF HAE surface epithelial cells required to express CFTR for restoration of mucus transport to normal levels, different amounts of PIVCFTR were used to express CFTR in 3%–65% of the surface epithelial cells of CF HAE and correlated to increasing ASL volumes and mucus transport rates. These data demonstrate for the first time, to our knowledge, that restoration of normal mucus transport rates in CF HAE was achieved after CFTR delivery to 25% of surface epithelial cells. In vivo experimentation in appropriate models will be required to determine what level of mucus transport will afford clinical benefit to CF patients, but we predict t

The optimal treatment approach to musculoskeletal pain in cystic fibrosis remains unclear. This study aimed to examine the effect of a combination of musculoskeletal physiotherapy techniques and massage therapy on musculoskeletal pain and ease of breathing. A single treatment session was associated with reduction in pain and improvement in ease of breathing in adults with cystic fibrosis.