Title: Understanding the lived experience and psychosocial needs of children and young people with Juvenile Dermatomyositis: a mixed methods study
Funder: Health Education England

Abstract: Background to the research Juvenile Dermatomyositis (JDM) is a rare, potentially life threatening condition. Beginning with mild fatigue and weakness, it can rapidly progress to children and young people (CYP) becoming bed bound and in extreme cases can lead to CYP needing help feeding and breathing. Whilst JDM occurs at any age, currently there is no known cure, and some CYP can experience fluctuations between remission and relapse throughout childhood into adolescence and beyond. There is very little evidence about the psychological effect JDM has on a CYP, however, through experience from clinical practice and from conversations with CYP and their parents; we know the effects can be profound. In a letter of support for the project, one parent commentsThis research sounds great and something which I also have felt has been sorely needed as emotional and psychological support for children and families with JDM is greatly lacking at present Aims of the work To describe the lived experience ofchildren and young people with Juvenile Dermatomyositis to identify theirunmet psychosocial needs. To explore psychosocial needs further ina large cross sectional study of children and young people with JuvenileDermatomyositis across the United Kingdom. Identify current support for psychosocialneeds across the United Kingdom provided by health care professionals. To share findings with children and youngpeople, health professionals and key stake holders to discuss thedevelopment of interventions to address unmet needs and target servicegaps in the future. Questions to be addressed What are the psychosocial needs of CYPwith JDM? What support is available to meet thepsychosocial needs of CYP with JDM in the UK? Plan of investigation The project will have four phases: Phase I Exploratory - CYP 1.1 In-depth phenomenological interviews with CYP to find out their experiences of living with this chronic illness (n=20). 1.2 Consultation with CYP with JDM advisory groups to explore themes emerging and review in relation to existing validated questionnaires. 1.3 Data analysis and define data tools' to be used in phase 2. Phase 2 Cross sectional - CYP Quantitative self-report tools' to capture cross sectional data from across the UK via JDCBS, based on key factors identified in phase 1 (n = 200 CYP). Phase 3 Scoping of current practice - HCP Scoping current practice for CYP with JDM across the UK being provided by Paediatric and Adolescent Rheumatology services. Phase 4 Dissemination and workshops leading to recommendations CYP & HCP's Sharing of data through a dissemination conference, and workshops for CYP and HCP's to present the findings and inform development of interventions to target psychosocial needs and service gaps for the future. Summary of potential benefits to patients and NHS This study is novel in design, focus and inclusivity for the UK. Every child or adolescent with JDM will be offered the opportunity to contribute their story and every lead clinician will be offered the opportunity to discuss the care they currently provide. This in itself raises the profile and will encourage health care professionals to better assess psychosocial needs tomorrow. Then in a further step, by maximising the opportunity of inviting all the interested participants to a dissemination conference with invited speakers to further discuss what interventions may offer the potential to improve practice and what is being done in other chronic conditions. Followed by workshops with the CYP, CYP advisory group and HCP's involved to consider a much wider intervention-based international project to advance care for CYP with JDM in the future.

Lay abstract: BackgroundJuvenile Dermatomyositis (JDM) is a very rare condition starting in children and young people at any age up to 18 years. The disease often starts as a rash on the face and then progresses rapidly to severe weakness of the muscles. Extreme tiredness can quickly be followed by major difficulties in moving and swallowing and some can become bed bound unable to move. At its worst, this condition can have children and young people in and out of hospital, missing school, requiring daily physiotherapy and being fed through a tube. This is a potentially life threatening condition and currently there is no known cure. In London there is a database kept about this population (over 500 participants in the Juvenile Dermatomyositis Cohort Biomarker Study, JDCBS) and includes patients from all over the UK. Here, at Great Ormond Street Hospital for Children NHS Foundation Trust, more patients with JDM are seen and cared for than anywhere else in the UK. Nurses on our ward have raised concerns about their mood, accompanying anxiety and their ability to cope. A survey of paediatric rheumatology nurses across the UK also highlighted concerns about the psychosocial (social and emotional) health of this group. There is very little research on this topic and current pathways of care do not routinely assess or address these particular needs. AimsThe aim of this research is to identify the psychosocial needs of children and young people with JDM that are currently not properly assessed or treated, and understand what psychosocial support is currently given to this population of patients. In this way, we can raise awareness about the effects of JDM on psychological health by highlighting the problems children and young people face and improve pathways of care to address these as part of standard clinical care in the future. Participants Phase 1 and 2, will include children and young people with JDM aged 8-21 years of age from across the UK. Phase 3 will involve healthcare professionals across the UK with experience of caring for this population of patients. Design and methods This study is in 4 phases; We will interview 20 children and young people with JDM, to find out what it is like to live with their condition. We will use this data to decide what questions are important to them and relevant to ask a larger group of children and young people in the next part of the study. With the help of our young person's JDM advisory group we will identify whether any existing validated (tried and tested) questionnaires are suitable for our use.We will send questionnaire packs to all those with JDM aged 8 years and over across the UK. These children and young people are already part of a bigger research study (JDCBS) and so can be contacted through their local teams.Using an electronic questionnaire we will ask key health professionals in each of the 17 Centres providing rheumatology services for children and young people with JDM, what psychological support services they currently provide.We will share what we have learnt with children and young people with JDM, members of the advisory group and healthcare professionals through a conference and workshops. The key aim is to raise awareness of the issues and work out how we can improve care in the future. Patient and Public Involvement Patient and Public Involvement has been embedded in this project from the very beginning. Before any pen was put to paper, children, young people and their families on the ward were asked where they felt more research was needed. Ten families repeatedly said that looking at mental health was important. Young people expressed concern about how rubbish they felt and their parents commented that they were worried about their child's low mood and felt this needed more acknowledgment by healthcare professionals. This proposal was discussed with the young person's JDM advisory group who have agreed it is a worthy research proposal, given ideas of appropriate outcome measures and agreed to support this project and ensure that the patient voice is heard throughout. Dissemination The results from this study will raise awareness through publications, presentations and websites. More importantly they will be used to discuss the development of interventions in the future, one for children and young people to accurately capture their emotional state (maybe in an app) and one for healthcare professionals to better identify and assess emotional needs (maybe in production of a toolkit to assess CYP needs').

Research activity codes: 4.4 POPULATION SCREENING;6.6 PSYCHOLOGICAL AND BEHAVIOURAL
Health categories: MENTAL HEALTH;INFLAMMATORY AND IMMUNE SYSTEM