Transition to adult care in cystic fibrosis: The challenges and the structure.

In developed countries, it is projected that there will be a 70% increase in the number of adults living with Cystic Fibrosis (CF) between 2010 and 2025. This shift in demographics highlights the importance of high-quality transition programmes with developmentally appropriate integrated health care services as the individual moves through adolescence to adulthood. Adolescents living with CF face additional and unique challenges that may have long-term impacts on their health, quality of life and life-expectancy. CF specific issues around socially challenging symptoms, body image, reproductive health and treatment burden differentiate people with CF from their peers and require clinicians to identify and address these issues during the transition process. This review provides an overview of the health, developmental and psychosocial challenges faced by individuals with CF, their guardians and health care teams considering the fundamental components and tools that are required to build a transition programme that can be tailored to suit individual CF clinics.

Strategies for improving early nutritional outcomes in children with oesophageal atresia and congenital diaphragmatic hernia.

Post-natal growth in surgical lung conditions, such as congenital diaphragmatic hernia and oesophageal atresia with tracheo-oesophageal fistula, is often sub-optimal in the early years of life when lung growth is occurring. Whilst constitutional, behavioural and mechanical factors may contribute to poor feeding and weight gain, there is a common path of management with greater caloric supplementation that may change growth trajectories and potentially lead to better respiratory, anthropometric and cognitive outcomes. We provide simple, single page, feeding supplementation sheets in three age groups: 0-6months, 6-12months and 12-24months that have proven useful for enhancing weight gain in our patients.

Factors That Influence Use of a Patient Portal by Health Professionals.

Patient portals are websites or apps that provide patients with tools to manage healthcare appointments, access their health records, and communicate with clinicians. Patient portals have been demonstrated to be beneficial for improving communication between patients/carers and their healthcare team in a range of health settings. However, there is limited research on the barriers and enablers for implementing patient portals from the perspective of health professionals and healthcare teams, particularly in a paediatric setting. This study aimed to understand healthcare teams' experiences of using a patient portal and, using the Unified Theory of Acceptance and Use of Technology (UTAUT) framework, explore the barriers and enablers to ongoing use. Participants were 11 health professionals participating in the pilot of a patient portal for patients/carers in paediatric care. Data were collected using semi-structured interviews. Analysis of the interview data identified nine themes about implementing a patient portal in paediatric care, all of which aligned with the four constructs of the UTAUT. This study identified that barriers and enablers of the uptake of a patient portal by health professionals in a paediatric context aligned with the UTAUT framework. Value for the patient, improved workflow, and adequate technical and implementation support were highlighted by participants.

Highlights from the nutrition guidelines for cystic fibrosis in Australia and New Zealand.

Optimal nutrition care is important in the management of cystic fibrosis (CF). This paper summarises the '2017 Nutrition Guidelines for Cystic Fibrosis in Australia and New Zealand (NZ)'. CF dietitians formulated 68 practice questions which were used to guide a systematic literature search and review of the evidence for nutrition in CF. Identified papers underwent quality and evidence assessment using the American Dietetic Association quality criteria checklist and the National Health and Medical Research Council of Australia (NHMRC) rankings. Evidence statements, graded recommendations and practice points were developed covering core nutrition topics (assessment and nutrition interventions including oral, enteral and micronutrient supplementation); nutrition-related co-morbidities (including pancreatic insufficiency, CF-related diabetes, bone health and distal intestinal obstruction syndrome); and key new topic areas (genetic modulator therapies, overweight/obesity and complementary therapies). This paper showcases highlights from the guidelines, focussing on new topic areas and geographic and climate considerations for vitamin D, salt and hydration.

Dietary intake in cystic fibrosis and its role in glucose metabolism.

BACKGROUND: Dietary intervention in cystic fibrosis (CF) has historically focused on high-energy diets to address malnutrition, with little attention on diet quality. With increased survival, CF complications such as impaired glucose tolerance (IGT) and cystic fibrosis related diabetes (CFRD) have increased in prevalence. In the absence of consensus on the management of IGT, the role of dietary intake, specifically carbohydrate quality, requires consideration. AIMS: The aims of this study were to: 1) determine nutritional quality of dietary intake at an adult CF clinic and compare this to the Australian Dietary Guidelines 2) explore relationships between dietary intake, including glycaemic index (GI) and glycaemic load (GL), and glucose response variables using continuous glucose monitoring (CGM). METHODS: Adults attending a Sydney hospital were recruited to undergo CGM for five-seven days and record dietary intake using a food record over the CGM period. The relationship between variables of dietary intake, including GI and GL and variables of glycaemic response, including mean amplitude of glycaemic excursions (MAGE), percentage of time in hyperglycaemic and euglycaemic range, were determined. RESULTS: Eighteen participants completed the study with 87 full days of dietary and CGM data. Dietary intake was higher than recommendations in the Australian Dietary Guidelines in relation to grains and protein foods and only slightly higher in saturated fat. Bivariate correlations showed dietary GI was significantly positively associated with percentage of time in hyperglycaemic range. Dietary GL was significantly associated with SD, MAGE and percentage of time in euglycaemic range on CGM. Results remained significant when controlled for energy intake in partial correlation analyses. CONCLUSIONS: This study suggests GI and GL may be important dietary factors influencing glucose metabolism in CF. Further studies exploring low GI or GL diets as a dietary intervention in CF are the next step.