The increase in life expectancy of people with cystic fibrosis (pwCF) has come with new comorbidities, particularly diabetes. Cystic fibrosis related diabetes is a major challenge in the care of (pwCF) because it is a morbidity and mortality factor at all stages of the disease. Early glucose tolerance abnormalities observed from childhood are also associated with a poor pulmonary and nutritional outcome. This justifies systematic screening with an annual oral glucose tolerance test (OGTT) from the age of 10 years.

communication, collaboration and responsiveness as strengths.Over 50% felt the new model provided holistic care and 'more tools in the tool box'.Other themes were the value of HPCT as an external perspective; improvements in advance care planning and staff support/education.There were perceived weaknesses in co-ordination between the 2 teams.Although the CF Team identified insufficient dedicated palliative care time, the HPCT were concerned about reduced opportunities for developing their skills. Conclusion:The proactive model of working has resulted in tripling of CF referrals to the HPCT.Feedback from CF and HPCT Teams is positive with ideas for improvement.We are re-designing methods for patient feedback and continuing the QI cycle.

important patient reported outcome of fatigue reduction.The ROCK study supports sustained clinical effectiveness at 12 months.

BackgroundSpecific alterations in skeletal muscle related to genetic defects may be present in adults with cystic fibrosis (CF). Limb muscle dysfunction may contribute to physical impairment in CF.Aims and objectivesWe hypothesized that adults with CF would have altered calf muscle metabolism during exercise.MethodsFifteen adults with CF and fifteen healthy controls matched for age, gender and physical activity performed a maximal cycling test and an evaluation of calf muscle energetics by 31P magnetic resonance spectroscopy before, during and after plantar flexions to exhaustion.ResultsMaximal cycling test revealed lower exercise capacities in CF (VO2peak 2.44 ± 0.11 vs. 3.44 ± 0.23 L·Min−1, P = 0.03). At rest, calf muscle phosphorus metabolites and pHi were similar in CF and controls (P > 0.05). Maximal power output during plantar flexions was significantly lower in CF compared to controls (7.8 ± 1.2 vs. 6.6 ± 2.4 W; P = 0.013). At exhaustion, PCr concentration was similarly reduced in both groups (CF −33 ± 7%, controls −34 ± 6%, P = 0.44), while PCr degradation at identical absolute workload was greater in CF patients (P = 0.04). These differences disappeared when power output was normalized for differences in calf size (maximal power output: 0.10 ± 0.02 vs. 0.10 ± 0.03 W/cm2; P = 0.87). Pi/PCr ratio and pHi during exercise as well as PCr recovery after exercise were similar between groups.ConclusionSimilar metabolic calf muscle responses during exercise and recovery were found in CF adults and controls. Overall, muscle anabolism rather than specific metabolic dysfunction may be critical regarding muscle function in CF.

Aims. - The present review examines the efficacy of different strategies to enhance exercise tolerance in cystic fibrosis patients. This review focuses first on the efficacy of indirect strategies to ameliorate physical performance. The second interest of this paper is to analyse the effects of different exercise rehabilitation protocols (i.e. direct strategies) which have been developed in the last few years.Actualities. - Exercise tolerance, which is related to prognosis and quality of life, is reduced in cystic fibrosis patients. The improvement of physical fitness is thus crucial in this population. Indirect strategies such as vitamin supplementation, which first aim to counteract the negative effects of the disease, seem to have only a limited influence on exercise tolerance. However, evidences are now available to justify the incorporation of exercise into the routine care of cystic fibrosis patients.Perspectives and prospects. - Although few data are yet available, the combination of strength and endurance training is potentially one of the best strategy to improve fitness in cystic fibrosis patients. Further studies are needed to investigate the feasibility and the efficacy of such program. (C) 2012 Elsevier Masson SAS. All rights reserved.

L'objectif de cette revue est d'analyser les différentes stratégies susceptibles d'engendrer une amélioration significative de la tolérance à l'effort des patients atteints de mucoviscidose. Dans un premier temps, cette revue analyse l'efficacité des stratégies indirectes (i.e. autres que l'exercice) pour lutter contre l'intolérance à l'effort. Ensuite, elle s'intéresse à l'efficacité des différents protocoles de réhabilitation à l'effort proposés au cours de ces dernières années. L'intolérance à l'effort est importante dans la mucoviscidose et peut avoir des conséquences directes sur le pronostic vital et la qualité de vie du patient. L'amélioration de la performance physique représente donc un enjeu majeur dans cette pathologie. Les stratégies « indirectes » (e.g. supplémentation vitaminique), qui ont pour objectif premier de lutter contre certains effets de la maladie, semblent avoir un effet limité sur l'intolérance à l'effort. Les bénéfices liés à l'exercice physique semblent en revanche suffisamment importants pour justifier l'intégration de programme de réhabilitation à l'effort dans une routine clinique. Bien qu'encore très peu documenté, un programme associant entraînement aérobie et renforcement musculaire semble offrir potentiellement le plus de perspectives dans la réhabilitation à l'effort du patient mucoviscidosique. Des études complémentaires devront confirmer cette hypothèse. The present review examines the efficacy of different strategies to enhance exercise tolerance in cystic fibrosis patients. This review focuses first on the efficacy of indirect strategies to ameliorate physical performance. The second interest of this paper is to analyse the effects of different exercise rehabilitation protocols (i.e. direct strategies) which have been developed in the last few years. Exercise tolerance, which is related to prognosis and quality of life, is reduced in cystic fibrosis patients. The improvement of physical fitness is thus crucial in this population. Indirect strategies such as vitamin supplementation, which first aim to counteract the negative effects of the disease, seem to have only a limited influence on exercise tolerance. However, evidences are now available to justify the incorporation of exercise into the routine care of cystic fibrosis patients. Although few data are yet available, the combination of strength and endurance training is potentially one of the best strategy to improve fitness in cystic fibrosis patients. Further studies are needed to investigate the feasibility and the efficacy of such program.

Pandoraea are considered emerging multidrug resistant pathogens in the context of cystic fibrosis. We report herein for the first time the case of a 30-year-old woman with cystic fibrosis, living in France, who was chronically infected with Pandoraea pulmonicola and who died of Pseudomonas aeruginosa sepsis 3 weeks after bilateral lung transplantation.

We report the case of a 5‐year‐old girl with persistent chest X‐ray abnormalities following an episode of pneumonia who has a complex congenital pulmonary malformation comprising of a congenital pulmonary airway malformation, an intralobar sequestration and two bronchogenic cysts, all present within the same lobe. The observation suggests a common embryological origin of these malformations. Pediatr. Pulmonol. 2010; 45:832–835. © 2010 Wiley‐Liss, Inc.

BackgroundThe present study investigated the validity and the reliability of the oxygen uptake efficiency slope (OUES) as a determinant of exercise tolerance in adults with cystic fibrosis (CF).Methods31 CF patients and 34 healthy controls performed a maximal incremental cycle test with respiratory gas-exchange measurements. OUES was calculated from data taken from different percentages of the entire exercise duration, including 80% (OUES80) and 100% (OUES100). Peak oxygen uptake (VO2peak) and gas exchange threshold (GET) were also determined. The agreement between submaximal parameters and VO2peak was assessed using Bland Altman plots. Test retest reliability was evaluated in CF patients using absolute (SEM) and relative indices (ICC).ResultsOn the contrary to the GET, which was undetectable in 16% of the CF patients, the OUES was easily determined in all patients. Among all the submaximal variables, OUES80 had the best reliability (ICC=0.94, SEM=7.3%) and agreement with VO2peak (r2=0.83, P<0.01; limits of agreement: ±365 mL min−1) and did not differ from OUES100.ConclusionsOUES80 is a reliable and more useful submaximal parameter than the GET and may find use in the interpretation of exercise studies in CF patients who are unable to perform maximal exercise.