Title: Understanding Fatigue in Children and Young People with Sickle Cell Disease to Guide the Co-Development of a Fatigue Self-Management Intervention
Funder: Department of Health and Social Care

Abstract: BACKGROUND Sickle cell disease (SCD) is the most common inherited blood condition in the UK. It is a life-long, life-threatening condition that mainly affects people of African or African-Caribbean origin. One common symptom of the condition is persistent fatigue. For children/young people (CYP) with SCD, fatigue is one of the most disabling symptoms they experience. Research shows that fatigue significantly affects their physical, social and emotional wellbeing. Despite this it is a symptom overlooked by clinicians and no interventions have been developed to help CYP with SCD manage their fatigue. We aim to address this neglect by co-developing a fatigue self-management intervention tailored to CYP with SCD and which will aim to help reduce the impact of fatigue on their daily lives and improve their quality of life. Due to the lack of evidence to inform intervention development, we will conduct an evidence synthesis and qualitative study to underpin future co-development. RESEARCH AIMS To synthesise current evidence on the characteristics of self-management interventions for CYP with SCD. To understand how CYP and parents define, experience and manage SCD fatigue. To identify the key components and delivery methods of an acceptable and feasible fatigue self-management intervention. METHODS In stage one we will conduct a systematic mixed-method review of self-management interventions for CYP with SCD, focusing on evaluating their characteristics and associated acceptability, feasibility, effectiveness and implementation barriers/enablers in order to assess their relevance for a fatigue-specific self-management intervention. There are no fatigue self-management interventions to review for CYP with SCD or other long-term conditions. In stage two we will conduct an exploratory qualitative study to investigate SCD-related fatigue and its management from the perspectives of CYP with SCD, parents and healthcare providers. Their preferences in relation to the content, format and delivery of a fatigue self-management intervention will also be examined. Fifteen CYP (aged 12-24), five parents and ten healthcare professionals will be purposively sampled from one NHS Trust. Data will be generated using a creative qualitative interviewing approach with CYP, and through in-depth interviewing with parents and healthcare professionals. Data will be analysed using a grounded theory approach with our PPI advisors being involved in the analytical process. IMPACT AND DISSEMINATION The results from the study will inform the development of a fatigue self-management intervention to improve the health and wellbeing of CYP with SCD. This study will enable us to develop a theory of how CYP with SCD self-manage fatigue to underpin intervention development. We will identify the preferences of CYP, parents and healthcare professionals, regarding the content, format and delivery of a fatigue self-management intervention taking account specific issues around the cultural and developmental appropriateness. We will also understand the contextual factors influencing acceptability, feasibility and sustainability of the intervention from the perspectives of different stakeholders. We will work with the Sickle Cell Society and our PPI and NHS stakeholders to ensure wide dissemination of the study findings to lay, professional and academic audiences using different media to target different audiences.

Lay abstract: BACKGROUND Sickle cell disease (SCD) is the commonest inherited blood condition in the UK. It is a life-long, life-threatening condition that mainly affects people of African or African-Caribbean origin. People with SCD have anaemia which makes them feel tired all the time. For children/young people (CYP) with SCD, being continually tired has a negative impact on them physically, socially and emotionally. However, health services overlook the tiredness they experience and there is nothing available to help CYP live with and manage their tiredness (fatigue). AIMS We aim to tackle this neglected area by working together with CYP with SCD, parents and healthcare professionals to develop a way of helping CYP manage their fatigue. We hope this will reduce the impact of fatigue on their daily lives and improve their quality of life. Firstly, we need to understand more about how CYP live with and manage fatigue and find out what CYP, parents and healthcare professionals think should be included in a self-management programme for fatigue. Once we have this information, we will use it to develop a programme with CYP, parents and healthcare professionals. RESEARCH DESIGN/METHODS In the first stage of the project we are going to find out what we already know about self-management programmes that have already been developed for CYP for SCD by reading and summarising studies that have already been published. This information will help us understand what types of programmes work best in improving health and wellbeing and what CYP think about them. In the second stage of the project we will talk to 15 CYP with SCD, five parents and 10 healthcare professionals to help us understand SCD fatigue better. We will also find out from them what they think would be best to include in a fatigue self-management programme and how this should be provided. PATIENT AND PUBLIC INVOLVEMENT We have worked with a group of young people with SCD in developing this application. They have helped us develop the research idea, aims and how to carry the study out. They will form the PPI advisory group for the project and will be involved in all stages of the study including our dissemination activities. DISSEMINATION We are going to work with the Sickle Cell Society (SCS), a national charity, in disseminating our findings through their social media channels, website and monthly e-newsletter. We will also develop our own social media presence to raise awareness of the study and its findings. We plan to co-produce a short lay summary with our PPI advisors; give talks at conferences and meetings; write papers for healthcare professionals and other researchers to read; and hold a public exhibition of the artwork produced by CYP participating in the project.

Research activity codes: 7.1 INDIVIDUAL CARE NEEDS
Health categories: BLOOD