Title: An exploratory study to advance self-care support by dietitians in the routine care of children with Cystic Fibrosis
Funder: Health Education England

Abstract: Background Cystic Fibrosis (CF) is the most common genetic condition in the UK, affecting more than 10,400 people (CF Trust, 2017). With early diagnosis and advances in treatments to predominantly prevent its progression, life expectancy is increasing. However, daily treatment burden is considerable and often leads to non-adherence, particularly in adolescence (Bishay and Sawicki 2016). This impacts on nutritional status and lung function and as a consequence, can contribute to premature death. Supporting children with CF throughout childhood to gradually build their knowledge, skills and confidence to self-care, may result in children being better prepared as they transition into adolescence. Whilst there is policy impetus for provision of self-care support (SCS) for children with long-term conditions such as CF (DH/DFES, 2004), there is a lack of evidence about what this should consist of and how it can be implemented in routine clinical practice. The majority of people with CF must take pancreatic enzyme replacement therapy (PERT) with all fat-containing foods, to be able to digest and absorb fat. Dietitians support children with CF at regular hospital clinic visits, to have a diet and accompanying PERT that meets their individual needs. This is to achieve good control of malabsorption and optimise weight gain and growth (Stallings et al, 2008). Dietitians could also be providing individualised SCS as part of routine dietetic care. This research will explore what SCS by dietitians throughout childhood could consist of and how it could be embedded in routine care. Aim and objectives The aim of this study is to develop a logic model (conceptual framework) of a SCS pathway to be delivered by dietitians in routine clinic consultations with children with CF. The objectives align with the key activities in the development stage of the MRC guidance for developing complex interventions (MRC, 2006): 1. To develop a theoretical framework for SCS in the dietary care of children with CF by identifying, describing and critically evaluating existing evidence and theory for SCS of diet in children with LTCs, principally Type 1 diabetes and CF 2. To examine the perspectives of key stakeholders in routine dietetic clinic consultations with children with CF to further develop the theoretical framework for SCS 3. To model the SCS pathway in the dietary care of children with CF, including the relationship to outcomes identified as most important by key stakeholders. Methods The study is exploratory, using a qualitative approach and consists of three phases. Phase 1 is evidence synthesis. An integrative review will be conducted to develop a theoretical framework. Phase 2 is semi-structured interviews with CF dietitians, children with CF and their parents/carers, totalling approximately 50-60 interviews and analysed using Framework (Ritchie and Spencer, 1994). Phase 3 is the development of a logic model and online evaluation by key stakeholders. Anticipated impact This research has the potential to improve the quality of routine CF dietetic care, increase understanding of which outcomes are most valued and inform development of a multi-component intervention to support implementation of the SCS pathway.

Lay abstract: Aim of the research This research will explore how dietitians could support children with Cystic Fibrosis (CF) throughout childhood to build their knowledge, skills and confidence in looking after their diet and gut (digestive system). They may then be better prepared to self-care and stay in control of this part of their CF during adolescence. This is important as it may contribute to living better, and for longer, as adults. Background to the research CF is the most common genetic condition in the UK. It affects over 10,000 people. There is currently no cure but with early diagnosis and lots of daily treatment people with CF are living longer. The average age of survival in the UK is 47 years. CF affects many parts of the body but particularly the lungs and gut. Good care of diet and the gut is linked with better quality of life, better lung health and living longer. Most people with CF need to take tablets (enzymes) with all food to help digest and absorb fat. They may also need a diet high in calories. Dietitians support children with CF to have a diet, and enzymes, that match their individual needs. They do this at regular hospital clinic visits at specialist CF centres, right throughout childhood. Dietitians could also be providing self-care support (SCS) as part of this routine care. This research needs to be done now because: Children with CF and their parents have asked for SCS with diet and enzymes Adults with CF report that diet is a part of CF care over which children can take control There are calls for research in CF to focus on: The gut Ways to help motivate and support people with CF to keep taking their treatment. Design and methods used Little is known about how dietitians could support self-care in CF so this research will look at this in a lot of detail. The research will be in three phases: 1. Review research on SCS of diet in the routine care of children with long-term conditions, particularly Type 1 Diabetes and CF. This is because: There is limited research in CF, but we can learn lots from the research already done in Diabetes Support of the diet in diabetes has similarities to CF.2. Interview key people - children with CF, their parents/carers and children's CF dietitians, to be able to understand: What currently happens in routine dietetic care Their needs, goals and preferences for SCS What might help or get in the way.3. Make a model of what SCS could look like and check this with key people to see how it can be improved and used in routine care. Patient and public involvement Children with CF and their parents have been directly involved in developing the research question, design and methods. They have shared what they think would be important to include and what would make it fun to take part. The Youth Advisory Group (YAG) at the CF Trust (the main UK charity for CF) will be involved throughout to ensure the research stays relevant for people with CF. A parent of a child with CF will also be on the project advisory group which will oversee the research and monitor progress. Dissemination Findings will be shared with the: CF community with the help of YAG, via the CF Trust website and a workshop at the UK CF Conference Wider research community through presenting at relevant conferences and publishing at least two papers in peer-reviewed scientific journals

Research activity codes: 7.1 INDIVIDUAL CARE NEEDS
Health categories: CONGENITAL DISORDERS