Co-Creation of a Novel Childhood Onset Rare Disease Self-Care Support Tool

Children with chronic health problems are disproportionately affected by later life mental health disorders.1Bai G. Herten M.H. Landgraf J.M. Korfage I.J. Raat H. Childhood chronic conditions and health-related quality of life: Findings from a large population-based study.PLoS One. 2017; 12e0178539https://doi.org/10.1371/journal.pone.0178539Google Scholar, 2Brady A.M. Deighton J. Stansfeld S. Chronic illness in childhood and early adolescence: A longitudinal exploration of co-occurring mental illness.Dev Psychopathol. 2021; 33: 885-898https://doi.org/10.1017/S0954579420000206Google Scholar, 3Taylor R.M. Gibson F. Franck L.S. The experience of living with a chronic illness during adolescence: a critical review of the literature.J Clin Nurs. 2008; 17: 3083-3091https://doi.org/10.1111/j.1365-2702.2008.02629.xGoogle Scholar Treatment regimens, medical monitoring, and life under the ‘shadow of threat’ all contribute to poor mental health outcomes.1Bai G. Herten M.H. Landgraf J.M. Korfage I.J. Raat H. Childhood chronic conditions and health-related quality of life: Findings from a large population-based study.PLoS One. 2017; 12e0178539https://doi.org/10.1371/journal.pone.0178539Google Scholar, 2Brady A.M. Deighton J. Stansfeld S. Chronic illness in childhood and early adolescence: A longitudinal exploration of co-occurring mental illness.Dev Psychopathol. 2021; 33: 885-898https://doi.org/10.1017/S0954579420000206Google Scholar The negative impact on well-being is further compounded by the isolation that comes with a diagnosis of a rare disorder.4Wiegand-Grefe S. Liedtke A. Morgenstern L. et al.Health-Related Quality of Life and mental health of families with children and adolescents affected by rare diseases and high disease burden: the perspective of affected children and their siblings.BMC Pediatr. 2022; 22596https://doi.org/10.1186/s12887-022-03663-xGoogle Scholar Key ameliorators of this negative impact are the engagement and empowerment of children in their own care.3Taylor R.M. Gibson F. Franck L.S. The experience of living with a chronic illness during adolescence: a critical review of the literature.J Clin Nurs. 2008; 17: 3083-3091https://doi.org/10.1111/j.1365-2702.2008.02629.xGoogle Scholar Immune mediated inflammatory disorders (IMID) are characterised by regular hospital appointments, the use of immunosuppressive agents, and multi-site and multidisciplinary care. Families and carers of affected children initially handle these complex care processes, but as children approach the age of self-care they are expected to understand and manage their own care needs. In 2019, patient and family focus groups within Great Ormond Street Hospital, a national (United Kingdom) centre of care excellence for childhood IMIDs, identified the need for support around managing adolescence and expectations of self-care.5Compeyrot-Lacassagne S. Twoney C. Petrushkin H. et al.THU0531 ”Bothering To Listen”: Enabling Patient And Family Involvement In Childhood Uveitis Healthcare.Poster Presentations. BMJ Publishing Group Ltd and European League Against Rheumatism, 2019: 557.1-557https://doi.org/10.1136/annrheumdis-2019-eular.7262Google Scholar A co-creation approach was undertaken for the development of a self-care tool. Using multiple existing ‘patient passports’ which provided useful learning, but which did not address the needs of this population,6Bailie H.N. Liu X. Bruynseels A. Denniston A.K. Shah P. Sii F. The Uveitis Patient Passport: A Self-Care Tool.Ocul Immunol Inflamm. 2020; 28: 433-438https://doi.org/10.1080/09273948.2019.1569240Google Scholar, 7Haupt R. Essiaf S. Dellacasa C. et al.The ‘Survivorship Passport’ for childhood cancer survivors.Eur J Cancer. 2018; 102: 69-81https://doi.org/10.1016/j.ejca.2018.07.006Google Scholar, 8Donovan D.J. Macciola D. Paul E.A. et al.Design and implementation of a patient passport in a pediatric cardiology clinic.Prog Pediatr Cardiol. 2020; 59101208https://doi.org/10.1016/j.ppedcard.2020.101208Google Scholar, 9Greenberg J. Prushinskaya O. Harris J.D. et al.Utilization of a patient-centered asthma passport tool in a subspecialty clinic.Journal of Asthma. 2018; 55: 180-187https://doi.org/10.1080/02770903.2017.1323916Google Scholar, 10Grant C. Pan J. A comparison of five transition programmes for youth with chronic illness in Canada.Child Care Health Dev. 2011; 37: 815-820https://doi.org/10.1111/j.1365-2214.2011.01322.xGoogle Scholar content and format were developed through face to face structured interviews with 12 affected children and their families. The patients were aged 7yrs - 15yrs with disease duration ranging from 6 months – 10yrs, and had inflammatory eye, bowel disorders and / or rheumatological disorders. The interviews informed adaptation of existing content, and development of novel content for the passport. The key stakeholder (families, children and young people affected by rare childhood onset inflammatory disorders) requested that the tool (1) could be personalised, (2) allowed logging of timelines of treatments and procedures and self-completed narratives on the experience of these events, (3) provided access to trusted information sources for their rare disorder, and (4) enabled documentation and descriptions of the clinical teams involved in care. The resulting tool was named ‘CORDSS’, the childhood onset rare disease self-care support passport. The descriptor of ‘passport’ was retained to reflect the “journey” described by many, ie the changing nature, over the disease course, of their lived experience as affected families.5Compeyrot-Lacassagne S. Twoney C. Petrushkin H. et al.THU0531 ”Bothering To Listen”: Enabling Patient And Family Involvement In Childhood Uveitis Healthcare.Poster Presentations. BMJ Publishing Group Ltd and European League Against Rheumatism, 2019: 557.1-557https://doi.org/10.1136/annrheumdis-2019-eular.7262Google Scholar A prototype tool was piloted (n=6 stakeholder families, using the passport for 1 – 4 months), and then underwent two cycles of refinement, firstly to add additional links (embedded quick response, QR codes) to trusted information on generic health domains such as diet, health and mental well-being. The second addition allowed documentation of sources of support for families experiencing urgent care needs when in locations remote from their managing care teams. CORDSS (Figure) is an editable electronic master document which is disseminated in paper form due to concerns around digital inequity and expressed preference for a material product. The tool is completed with input from clinical teams as considered necessary or appropriate. Setting specific adaptation is possible (for example, setting specific QR codes for disease or country specific patient groups and digital resources), but CORDSS has been designed for children with disorders characterised by rarity, chronicity, complexity, and multi-disciplinary care. Two versions of the cover page exist: one with a child-drawn image of a rainbow zebra, an icon for rare disorders, and a simpler front cover for older children, as per expressed preference. The tool has been in use since May 2022. We now present data from an initial survey of the family experience of the passport. A convenience sample of n=8 families (ensuring representation of: female and male children, children and young people from the <8yrs, 8-12, and 12+ age groups); and white and non-white, specifically Black and South Asian, families) were given the tool at a clinic consultation. Children were affected by a range of disorders, comprising: sarcoidosis (n=1); inflammatory bowel disease (n=1); Blau syndrome (n=1), tubulointerstitial nephritis (n=1); and ocular inflammatory disease (n=4). A clinical nurse specialist (CT) spent between four and 20 minutes with them introducing the tool, and supporting completion of pages as directed by the families. At the following clinic consultation they were asked whether or not they would continue using the passport (“yes” / “no” / “unsure”), and asked for any comments they had about the tool. All families wished to continue using the passport (8/8, “yes” responses), and reported that completing the tool was a positive experience (sample quotes: “I wish we’d had this at the beginning, it’s been hard keeping track of everything”; “…filling it in themselves, and actually asking us….about {their} diagnosis…like {they} weren’t trying to avoid talking about it for once”). Families had used the tool to communicate with relatives, school and other healthcare professionals. Additional amendments were suggested, including future creation of an adapted tool that was parents and carer centred, and the addition of pages to which siblings could contribute if the directly affected child allowed. There were also requests for a digitised mobile version of the tool. The co-created content and format within the passport has resulted in a tool which appears to support children and young people, and their families, in taking control of rare, chronic, complex conditions. This resource has been designed for use across different disorders, and in different settings, with the tool introduced for use at any stage of the patient journey. The next steps will be more formal assessment of the impact of this tool as a complex intervention using outcome metrics which explore the impact on self-care behaviours and capture the well-being and global health outcomes of the child and their family. The editable version of the tool can be updated in response to changes in care delivery or disease care updates, and can be changed to address needs for site or disease specific adaptations. The Childhood Onset Rare Disease Self-Care Support (CORDSS) Tool is freely available for non-commercial use on application to the team. AL Solebo is supported by an NIHR Clinician Scientist award (CS-2018-18-ST2-005). This work was undertaken at UCL Institute of Child Health / Great Ormond Street Hospital for children which received a proportion of funding from the Department of Health’s NIHR Biomedical Research Centers funding scheme. The sponsors and funding organizations had no role in the design or conduct of this research. The views expressed are those of the authors and not necessarily those of the NHS, the NIHR or the Department of Health and Social Care.

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