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BACKGROUND Cystic fibrosis (CF) is a life-limiting congenital disease, with most patients dying at a young age of progressive lung disease. Lung transplantation offers hope for many but may not… Continue Reading
UNLABELLED Palliative care is not well understood in CF. Unmet needs of patients with CF, their families and staff were explored. METHOD Focus groups and interviews with forty-two participants (12… Continue Reading
BACKGROUND Cystic fibrosis (CF) can result in distal intestinal obstruction syndrome (DIOS) due to inspissated mucus. This paper describes the clinicopathological characteristics of adult CF patients… Continue Reading
Development of evidence-based practice requires investigation of the attitudes and needs of patients, families and healthcare professionals, particularly for sensitive subject areas. Cystic fibrosis… Continue Reading
Introduction The improvements in lung function, symptom burden and quality of life with the use of the gene potentiator ivacaftor in patients with G551D CF mutations have been established. Previous… Continue Reading
The effects of the potentiating agent ivacaftor on well-being and cognitive function are little known in adults with CF and G551D mutation. Objectives To evaluate the effects of ivacaftor on… Continue Reading