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

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. PATIENT AND PUBLIC CONTRIBUTION: Three young people with SCD recruited acted as the review advisors. They were formally trained in the review process and involved in every aspect of the review: the design, conduct and interpretation of the findings. CYP involvement in the interventions' development and implementation was analysed as part of the review. This systematic review was conducted as part of a wider research project titled: Understanding fatigue experiences of CYP with SCD to guide the co-development of a fatigue self-management intervention. Two of the young advisors involved in the review were also involved in the development of the project funding application.

Research knowledge transfer to improve the care and support of adolescents with sickle cell disease in Ghana.

INTRODUCTION: Effective transfer of research findings to key knowledge users, particularly in low- and middle-income countries, is not always achieved, despite being a shared priority among researchers, funders, healthcare and community stakeholders and decision-makers. A constructivist grounded theory study conducted in 2015-2019 in Ghana that explored sickle cell-related fatigue in adolescence resulted in numerous implications for practice and policy. Peer-reviewed funding was obtained to support disseminating these findings to relevant stakeholders. METHODS: Key steps in implementing this study dissemination project included: (1) identifying and attracting target stakeholders from healthcare and community organizations; (2) tailoring tools for communication of research findings for the stakeholder groups and (3) designing interactive workshops to facilitate knowledge sharing and uptake. FINDING: Despite the COVID-19 pandemic, 50 healthcare and community stakeholders participated in the dissemination workshops. The dissemination activities contributed new layers of understanding to the original research findings through discussions. Through the workshops, participants identified culturally valuable and actionable recommendations that they could take forward to improve care and support for young people with sickle cell disease in Ghana. A follow-up 6 months post the workshops indicated some positive knowledge usage and benefits. CONCLUSION: This dissemination project provided a unique opportunity for researchers and stakeholders to share in the interpretation of research findings and to strategically plan recommendations to improve SCD-focused care and support for young people in Ghana. Further research dissemination should continue to be grounded in locally generated knowledge, include systematic, long-term evaluation of dissemination outcomes and be adequately financed. PATIENT AND PUBLIC CONTRIBUTION: Public involvement in this study was critical to the research dissemination project. The Sickle Cell Association of Ghana (Kumasi chapter) actively supported the project's development, organization and facilitation. Parent members of the Association, the Association's executive members and volunteers, and the health professionals involved in sickle cell care at the Komfo Anokye Teaching Hospital participated in the project workshops. They contributed to the knowledge transfer and uptake.

The practice of pilot/feasibility studies in informing the conduct of HIV related clinical trials in sub-Saharan Africa: A scoping review.

Introduction: Pilot/feasibility studies represent a fundamental phase of the research process and play a vital role in the preliminary planning of a full size HIV clinical trial. Published HIV clinical trial protocols were reviewed to establish the extent to which the proposed HIV clinical trials are informed by a prior pilot/feasibility study. Methods: The JBI methodology for scoping reviews was followed. Six databases were systematically searched to identify articles for inclusion. Results: Thirty two (32) published HIV study protocols were included. Articles were in the English language and were published in the past 10 years (2011-2020). The review results showed that the majority of HIV-related clinical trials in sub-Saharan Africa were not informed by pilot/feasibility studies. The results further indicated that the number of HIV clinical trials informed by a pilot/feasibility study have been on the increase in the 8 years' period since 2012, a trend that indicates positive uptake of pilot studies in HIV related studies. A few select countries (South Africa, Uganda, Zimbabwe, Malawi and Kenya) comprised more than 70% of all clinical trials that were informed by a pilot/feasibility study, conducted in sub Saharan Africa. Conclusions: Although there is an increasing interest among researchers to integrate pilot/feasibility studies in HIV related research, limited countries in sub-Saharan Africa appear to have embraced this trend. Strategies that can motivate researchers to engage in a culture of incorporating pilot/feasibility studies in HIV related research should be implemented.

Biographical accounts of the impact of fatigue in young people with sickle cell disease.

Children and young people (CYP) with sickle cell disease (SCD) are a 'missing voice' in the debate on biography and sociology of chronic illness, meaning we know little about the social consequences of the illness for CYP. This paper examines the meaning of fatigue (a common symptom) for adolescents with SCD. Analysing 24 in-depth interviews with adolescents aged 12-17 years in Ghana, we draw on the distinction proposed by Bury (1988) between 'meanings as significance' and 'meanings as consequence' to examine biographical aspects of fatigue. We argue that concepts of 'biographical disruption' and 'normal illness' do not easily accommodate the experience of CYP with congenital chronic illnesses like SCD, as their sense of (un)disruption and normality/continuity is contextualised relative to normative expectations about what it is to be a young person. At biographical transition points, illness/symptoms present from birth may evolve, shift and become experienced as 'new', 'different', or 'non-normal'. They may become restrictive rather than continuous or disruptive. These experiences are influenced primarily by normative biographical expectations and the pursuit of identity affirmations. We propose that biographical restriction, biographical enactment, biographical abandonment and biographical reframing are more relevant concepts for understanding the experiences of CYP living with SCD.

Characterising the outcomes, impacts and implementation challenges of advanced clinical practice roles in the UK: a scoping review.

OBJECTIVES: In response to demographic and health system pressures, the development of non-medical advanced clinical practice (ACP) roles is a key component of National Health Service workforce transformation policy in the UK. This review was undertaken to establish a baseline of evidence on ACP roles and their outcomes, impacts and implementation challenges across the UK. DESIGN: A scoping review was undertaken following JBI methodological guidance. METHODS: 13 online databases (Medline, CINAHL, ASSIA, Embase, HMIC, AMED, Amber, OT seeker, PsycINFO, PEDro, SportDiscus, Osteopathic Research and PenNutrition) and grey literature sources were searched from 2005 to 2020. Data extraction, charting and summary was guided by the PEPPA-Plus framework. The review was undertaken by a multi-professional team that included an expert lay representative. RESULTS: 191 papers met the inclusion criteria (any type of UK evidence, any sector/setting and any profession meeting the Health Education England definition of ACP). Most papers were small-scale descriptive studies, service evaluations or audits. The papers reported mainly on clinical aspects of the ACP role. Most papers related to nursing, pharmacy, physiotherapy and radiography roles and these were referred to by a plethora of different titles. ACP roles were reported to be achieving beneficial impacts across a range of clinical and health system outcomes. They were highly acceptable to patients and staff. No significant adverse events were reported. There was a lack of cost-effectiveness evidence. Implementation challenges included a lack of role clarity and an ambivalent role identity, lack of mentorship, lack of continuing professional development and an unclear career pathway. CONCLUSION: This review suggests a need for educational and role standardisation and a supported career pathway for advanced clinical practitioners (ACPs) in the UK. Future research should: (i) adopt more robust study designs, (ii) investigate the full scope of the ACP role and (iii) include a wider range of professions and sectors.

"Body as a Machine": How Adolescents With Sickle Cell Disease Construct Their Fatigue Experiences.

Research exploring illness experiences of young people with sickle cell disease (SCD) has, to date, ignored fatigue, despite the distinctive anemic nature of SCD. To examine adolescents with SCD fatigue experiences, we conducted narrative and picture-elicitation interviews with 24 adolescents in Ghana. A grounded theory, "body as a machine," was constructed from the narratives. Fatigue represented the most restrictive and disruptive aspect of growing up with SCD. Its meaning and significance laid in what it symbolized. Fatigue represented a socially undesirable feature that was stigmatizing, due to the expectations of high physicality in adolescence. Fatigue was therefore a major threat to "normalcy." The social significance of the physical body and its capacities shaped the adolescents' fatigue experiences. Managing fatigue to construct/maintain socially acceptable identities dominated the adolescents' lives. Consequently, there is a need for a recognition of the significance of fatigue to adequately support young people growing up with SCD.

Characterising the evidence base for advanced clinical practice in the UK: a scoping review protocol.

INTRODUCTION: A global health workforce crisis, coupled with ageing populations, wars and the rise of non-communicable diseases is prompting all countries to consider the optimal skill mix within their health workforce. The development of advanced clinical practice (ACP) roles for existing non-medical cadres is one potential strategy that is being pursued. In the UK, National Health Service (NHS) workforce transformation programmes are actively promoting the development of ACP roles across a wide range of non-medical professions. These efforts are currently hampered by a high level of variation in ACP role development, deployment, nomenclature, definition, governance and educational preparation across the professions and across different settings. This scoping review aims to support a more consistent approach to workforce development in the UK, by identifying and mapping the current evidence base underpinning multiprofessional advanced level practice in the UK from a workforce, clinical, service and patient perspective. METHODS AND ANALYSIS: This scoping review is registered with the Open Science Framework (https://osf.io/tzpe5). The review will follow Joanna Briggs Institute guidance and involves a multidisciplinary and multiprofessional team, including a public representative. A wide range of electronic databases and grey literature sources will be searched from 2005 to the present. The review will include primary data from any relevant research, audit or evaluation studies. All review steps will involve two or more reviewers. Data extraction, charting and summary will be guided by a template derived from an established framework used internationally to evaluate ACP (the Participatory Evidence-Informed Patient-Centred Process-Plus framework). DISSEMINATION: The review will produce important new information on existing activity, outcomes, implementation challenges and key areas for future research around ACP in the UK, which, in the context of global workforce transformations, will be of international, as well as local, significance. The findings will be disseminated through professional and NHS bodies, employer organisations, conferences and research papers.

Reducing repeat paediatric emergency department attendance for non-urgent care: a systematic review of the effectiveness of interventions.

OBJECTIVE: Non-urgent paediatric ED (PED) visits appear to contribute a large portion to the growing use of EDs globally. Several interventions have tried to curb repeated non-urgent attendances, but no systematic review of their effectiveness exists. This review examines the effectiveness of interventions designed to reduce subsequent non-urgent PED visits after a non-urgent attendance. METHOD: A systematic review design. A systematic search of four databases and key journals was conducted from their inception to November 2018. Experimental studies, involving children aged 0-18 years presenting to an ED for non-urgent care, which assessed the effectiveness of interventions on subsequent non-urgent attendance were considered. RESULTS: 2120 studies were identified. Six studies, including four randomised controlled trials (RCTs) and two quasi-experimental, were included. Studies were of moderate quality methodologically. All studies originated from the USA and involved informational and/or follow-up support interventions. Only two RCTs demonstrated the longest duration of intervention effects on reducing subsequent non-urgent PED attendance. These studies identified participants retrospectively after ED evaluation. The RCT with the largest number of participants involved follow-up support by primary physicians. Meta-analysis was impractical due to wide heterogeneity of the interventions. CONCLUSIONS: There is inconclusive evidence to support any intervention aimed at reducing subsequent non-urgent PED visits following a non-urgent attendance. The long-term impact of interventions is limited, although the effect may be maximised if delivered by primary care providers in children identified after their ED attendance. However, further research is required to evaluate the impact of any such strategies in settings outside the USA.

Adolescents' experiences of living with sickle cell disease: An integrative narrative review of the literature.

BACKGROUND: Sickle Cell Disease is the commonest monogenic haemoglobinopathy worldwide. Living with a long-term condition such as sickle cell disease during adolescence constitutes a significant challenge for the key stakeholders due to the combined effects of chronic illness and adolescent development. For adolescents with sickle cell disease to be cared for and supported appropriately and effectively, it is crucial that health professionals have a comprehensive knowledge and understanding of how adolescents experience living with the condition. While there is developing literature about how adolescent's experience sickle cell disease, this body of research has not been critically reviewed and synthesised. OBJECTIVE: To identify, critically appraise and synthesise primary research exploring adolescents' experiences of living with sickle cell disease to make recommendations for practice and research. DESIGN: Integrative narrative review. DATA SOURCES: A systematic search of 10 electronic databases and key journals was conducted to identify studies from the inception of databases to September 2016. REVIEW METHOD: Inclusion criteria: adolescents with sickle cell disease aged 12-19 years, primary data on adolescents' own perspectives, and published in English. Data were extracted on study contexts, methodology/design, theoretical constructs, participants, and key findings. Findings from included studies were synthesised using the integrative narrative approach. Additionally, the methodological quality of studies was assessed using the Hawker et al. (2002) appraisal checklist. RESULTS: 683 studies were identified, of which 40 fulfilled the inclusion criteria. Nine broad themes emerged: knowledge and understanding of the condition, symptom experiences, self-management, attitude to treatment, healthcare experiences, social relationships, difference and striving for normality, school experiences, and emotional well-being and coping. Majority of the studies were of moderate quality methodologically. Quality assessment demonstrated a high risk of bias in three studies. CONCLUSIONS: Sickle cell disease impacts on multiple facets of an adolescent's life. While there are similarities in the experience of living with sickle cell disease and living with other chronic illnesses, there are essential differences in relationship dynamics and healthcare experience. The adolescents expressed less confidence in generic healthcare providers. The review highlights areas relating to symptom management and health service provision that has been under-researched and need further exploration to understand adolescents' experiences and their support needs fully. Nursing care and research should focus more on adolescents' developmental wellbeing, promote peer support network among adolescents with the condition and with adolescents with other chronic illnesses and collaborate with adolescents to ensure service development are developmentally and culturally appropriate.