Self‐management interventions for children and young people with sickle cell disease: A systematic review

Abstract

Background: Increasing numbers of interventions are being developed to support self-management for children and young people (CYP) with sickle cell disease (SCD), but no systematic review has systematically synthesized this evidence regarding their characteristics, effectiveness, acceptability and feasibility for all published intervention types. Methods: The Joanna Briggs Institute guidelines for mixed-method reviews were followed. A systematic search of eight databases and key journals was conducted from their inception to November 2021. Primary research of self-management interventions targeting CYP with SCD aged 8-24 years and reporting any health/ social outcome and acceptability data were included. Design-specific standardized critical appraisal instruments were used. Two independent reviewers screened and appraised the articles. A third reviewer resolved disagreements. Results: Of 1654 articles identified, 38 studies were included. Methodological quality was moderate. Most studies evaluated SCD education, psycho-behavioural, psychosocial and skills training and/or social support interventions. They appear to demonstrate short-term improvements in knowledge, social functioning and medical adherence outcomes. Interventions that were multifaceted in content, combined technological platforms and in-person group-based formats and involved peers, family and care providers were more acceptable and effective. The long-term impact of interventions was limited, including CYP's involvement in the intervention development and implementation.Conclusions: There is inconclusive evidence for any self-management programme.Nonetheless, support from family, peers and care providers appears to be important for self-management interventions' effectiveness and acceptability. Future research needs to prioritize CYP involvement in both intervention design and delivery, their wider social context and include CYP with SCD from non-Black backgrounds.

Hydroxyurea Adherence for Personal Best in Sickle Cell Treatment (HABIT) efficacy trial: Community health worker support may increase hydroxyurea adherence of youth with sickle cell disease

“…Durability of impact should be evaluated. 35,36 Limitations included modest enrollment and missing HbF data, which collectively contributed to reduced statistical power for primary outcomes and analyses of potential moderators. For PDC analyses, large standard deviation and low power likely contributed to lack of between-group significances following the intervention phase.…”

“…Better HU adherence ‐ and the health of youth with SCD ‐ may be improved through continuing CHW‐led support for youth–caregiver dyads for developmentally appropriate self‐management. Durability of impact should be evaluated 35,36 …”

Opioid Use Among Children and Adults With Sickle Cell Disease in North Carolina Medicaid Enrollees in the Era of Opioid Harm Reduction

“…5 Although several studies have tested these nonpharmacologic interventions in adolescents to young adults with the aim of improving the transition to adult SCD care, few studies have tested interventions for children under 14 years of age or their caregivers. [45][46][47] Research to support the management of opioids, as well as nonpharmacologic interventions, to manage complex pain and assist children in coping with chronic SCD at an early age is greatly needed. This study had several strengths, including our partnership with CCNC for this study.…”

“…Initiation of opioids for the treatment of chronic pain in both children and adults is not recommended; ASH guidelines recommend the use of a variety of nonpharmacologic strategies 5 . Although several studies have tested these nonpharmacologic interventions in adolescents to young adults with the aim of improving the transition to adult SCD care, few studies have tested interventions for children under 14 years of age or their caregivers 45–47 . Research to support the management of opioids, as well as nonpharmacologic interventions, to manage complex pain and assist children in coping with chronic SCD at an early age is greatly needed.…”

Self-management needs, strategies and support for sickle cell disease in developing countries: a scoping review protocol

“…However, there is a notable dearth of evidence synthesis on SCD SM in developing countries. For instance, evidence synthesis on SM interventions for SCD in the developing world focused on children and young adults 27. Given the potential of SM to enhance the lives of patients with SCD, there is a pressing need for standardised and context-specific SM support interventions in developing countries.…”