Understanding the health-care experiences of people with sickle cell disorder transitioning from paediatric to adult services: This Sickle Cell Life, a longitudinal qualitative study

Abstract

Background Transitions from paediatric to adult health-care services cause problems worldwide, particularly for young people with long-term conditions. Sickle cell disorder brings particular challenges needing urgent action. Objectives Understand health-care transitions of young people with sickle cell disorder and how these interact with broader transitions to adulthood to improve services and support. Methods We used a longitudinal design in two English cities. Data collection included 80 qualitative interviews with young people (aged 13–21 years) with sickle cell disorder. We conducted 27 one-off interviews and 53 repeat interviews (i.e. interviews conducted two or three times over 18 months) with 48 participants (30 females and 18 males). We additionally interviewed 10 sickle cell disease specialist health-care providers. We used an inductive approach to analysis and co-produced the study with patients and carers. Results Key challenges relate to young people’s voices being ignored. Participants reported that their knowledge of sickle cell disorder and their own needs are disregarded in hospital settings, in school and by peers. Outside specialist services, health-care staff refuse to recognise patient expertise, reducing patients’ say in decisions about their own care, particularly during unplanned care in accident and emergency departments and on general hospital wards. Participants told us that in transitioning to adult care they came to realise that sickle cell disorder is poorly understood by non-specialist health-care providers. As a result, participants said that they lack trust in staff’s ability to treat them correctly and that they try to avoid hospital. Participants reported that they try to manage painful episodes at home, knowing that this is risky. Participants described engaging in social silencing (i.e. reluctance to talk about and disclose their condition for fear that others will not listen or will not understand) outside hospital; for instance, they would avoid mentioning cell sickle disorder to explain fatigue. Their self-management tactics include internalising their illness experiences, for instance by concealing pain to protect others from worrying. Participants find that working to stay healthy is difficult to reconcile with developing identities to meet adult life goals. Participants have to engage in relentless self-disciplining when trying to achieve educational goals, yet working hard is incompatible with being a ‘good adult patient’ because it can be risky for health. Participants reported that they struggle to reconcile these conflicting demands. Limitations Our findings are derived from interviews with a group of young people in England and reflect what they told us (influenced by how they perceived us). We do not claim to represent all young people with sickle cell disorder. Conclusions Our findings reveal poor care for young people with sickle cell disorder outside specialist services. To improve this, it is vital to engage with young people as experts in their own condition, recognise the legitimacy of their voices and train non-specialist hospital staff in sickle cell disorder care. Young people must be supported both in and outside health-care settings to develop identities that can help them to achieve life goals. Future work Future work should include research into the understanding and perceptions of sickle cell disease among non-specialist health-care staff to inform future training. Whole-school interventions should be developed and evaluated to increase sickle cell disorder awareness. Funding This project was funded by the National Institute for Health Research (NIHR) Health Services and Delivery Research programme and will be published in full in Health Services and Delivery Research; Vol. 8, No. 44. See the NIHR Journals Library website for further project information.

Transition Preparation and Satisfaction of Care Among Adolescents and Young Adults With Sickle Cell Disease at the Ghana Institute of Clinical Genetics

“…Participant perspectives may have been influenced by clinic-based education, such as the information sessions on SCD self-management conducted by staff at adult clinic sessions. Similar to our previous study in Ghana with pediatric SCD participants and other reports from elsewhere, issues of peer stigmatization and nondisclosure of health status to friends were frequently revealed 22,27…”

“…Similar to our previous study in Ghana with pediatric SCD participants and other reports from elsewhere, issues of peer stigmatization and nondisclosure of health status to friends were frequently revealed. 22,27 Limitations of our study include the single site study, convenience sampling method used, modest sample size precluding adjustment for multiple comparisons, and the unequal number of participants in the 2 groups, possibly affecting the ability to generate statistically robust findings. Interviewing participants at the adult clinic may have missed patients who did not successfully transition to adult care, thereby precluding assessment of an influence of transition preparation on improved likelihood for continuity of SCD care from pediatric to adult clinics.…”

Reimagining authorship guidelines to promote equity in co-produced academic collaborations

“…They also had an experience of advocacy for sickle cell healthcare improvement. We have detailed elsewhere the different ways in which we worked together throughout the project, and our flexible approach to collaboration and co-producing knowledge with them (Miles et al, 2020;Miles et al, 2018;Renedo et al, 2020). To summarise: the project was co-produced throughout, with the patient and carer experts involved in the initial funding application process, and participating (with payment) at different stages from planning the research to analysis, dissemination, and translation of the findings into policy and practice.…”

How young adults with sickle cell disease define “being a good patient” in the adult healthcare system

“…Finally, the differences in good patient role fulfillment in different care contexts raise questions whether transition programs prepare young adults to navigate multiple, sometimes unpredictable, adult healthcare systems, and whether pediatric care systems provide preparation and opportunities to practice responding when, unfortunately and often unfairly, care challenges arise. 33,34 for in a relatively unique care setting, patients were average by many standardized measures, including SCD impact, stigma, and self-efficacy scores, 15 SCSES scores, 13 and mean MoSCS scores. 14 Finally, young adults with SCD's intersectional identities are incompletely addressed here.…”