Dietary intakes in adult patients with cystic fibrosis–do they achieve guidelines?

AbstractBackground: Most patients with cystic fibrosis (CF) require a higher energy and protein intake than their healthy peer group. There are few data on dietary intakes of adult patients. The aim of this study was to determine nutritional intakes in an adult population with CF. The impact of nutritional intervention and disease on macronutrient intake was examined. Methods: Retrospective cross-sectional analysis of 94 unweighed food diaries at annual review (1995–2000). Energy and protein intakes were compared to the estimated average requirement (EAR) for energy and reference nutrient intake (RNI) for protein. The effect of diet alone, oral supplements, enteral tube feeding, and cystic fibrosis related diabetes (CFRD), on macronutrient intake was examined and impact of pancreatic sufficency (PS) and lung transplantation. Results: Mean energy and protein intakes approached recommended CF guidelines, but in 72% of assessments these values were not achieved. Mean energy and protein intakes for patients on diet alone and protein intake for those with CFRD failed to meet recommendations. Oral supplementation and enteral tube feeding regimens increased energy and protein intake above recommended levels. No group achieved 40% total energy from fat. Patients receiving enteral tube feeds had the highest mean energy and protein intakes but lowest body mass index (BMI) and lung function. Conclusion: Adequate mean energy and protein intakes in adult patients with CF mask subgroups of patients who fail to meet recommendations ie. diet alone, diabetic. Oral supplementation and enteral tube feeding increase energy and protein intake but fail to achieve an adequate BMI level in subjects with a decreased clinical status. Individual nutritional assessment remains essential

A pilot study of zafirlukast as an anti-inflammatory agent in the treatment of adults with cystic fibrosis

AbstractBackground: Persistent endobronchial inflammation is in part responsible for the attrition of lung function seen in cystic fibrosis. Leukotrienes act as pro-inflammatory mediators. The aim of this study was to assess the efficacy of the leukotriene receptor antagonist zafirlukast as a potential anti-inflammatory agent in the treatment of adult patients with cystic fibrosis. Methods: Clinically stable patients were enrolled in the study if they had no history or clinical evidence of asthma, bronchial hyper-reactivity, or aspergillosis. They were randomised to receive zafirlukast 20 mg twice daily with all routine treatment for four months or routine treatment alone in an open cross-over design. Primary endpoints were changes in respiratory function tests and a modified NIH clinical score. Results: Thirty patients were enrolled and 25 completed. There was a significant improvement in the modified NIH clinical score but no significant increase in respiratory function with zafirlukast. Conclusions: Patients receiving a leukotriene receptor antagonist in addition to routine treatments showed significant improvement in a clinical score which is a composite of clinical wellbeing, chest radiograph appearance, and physical examination. Respiratory function showed a non-significant trend towards improvement with treatment. Zafirlukast may benefit patients with CF. An adequately powered study is justified on the basis of these results

313 Can patient-reported health-related quality of life predict survival in cystic fibrosis?

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Cystic fibrosis co-existing with trisomy 21

AbstractPrevious reports of children with co-existence of cystic fibrosis and full trisomy 21 suggest a very poor prognosis, with the majority of cases dying in infancy and the oldest reported survivor being 6years of age. We report the case of a young man with genetically confirmed trisomy 21 and homozygous for the F508del cystic fibrosis mutation. Despite the diagnosis of cystic fibrosis being delayed until the age of 2years he has transitioned to adult services and is now 25years of age. Currently he has poor lung function and a continuous ambulatory oxygen requirement