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

Abstract

Introduction

Sickle cell disease (SCD) poses a significant global health burden, particularly affecting individuals in developing countries with constrained healthcare resources. While research on self-management in the context of SCD is emerging, it has predominantly focused on primary studies, and there is a notable dearth of evidence synthesis on SCD self-management in developing countries. This scoping review aims to identify and map self-management needs of individuals living with SCD, the strategies they employed to meet those needs, and the support systems available to them.

Methods and analysis

The review will be conducted following the Arksey and O’Malley’s (2005) 29 framework to comprehensively examine the landscape of SCD self-management research. Searches will be performed in PubMed, Scopus, Embase and Dimensions AI, with additional searches in other databases and grey literature. Indexed literature published in English from inception to January 2024 will be included. Reference list from included studies will also be searched manually. Two teams will be constituted to independently screen titles, abstracts and full text against the eligible criteria. Data will be extracted from included studies onto a customised data extraction form.

Ethics and dissemination

Ethical approval is not required for this review due to the fact that it synthesises information from available publications. The findings will be disseminated through publication in a peer-reviewed journal. Also, the findings will possibly be presented at relevant international and national conferences. This protocol has already been registered with the Open Science Framework. The study characteristics such as design and setting will be descriptively analysed and presented as graphs, tables and figures. Thematic analysis will also be conducted based on the study objectives and presented as a narrative summary.

STRENGTHS AND LIMITATIONS OF THIS STUDY.

• This novel review adopts a comprehensive approach to include a variety of literature to provide a map of the landscape of knowledge on the emerging topic of self-management for sickle cell disease in developing countries.

• Planning and conducting the review is guided by the widely used Arksey and O’Malley framework and reported through the PRISMA Extension for Scoping Reviews ensuring transparency and rigour.

• The design and conduct of the search strategy was supported by a chartered librarian. The search strategy encompasses a wide array of databases and academic resources. Studies included are not confined to a particular research methodology, which enhances credibility and offering a comprehensive view of the subject matter.

• The search strategy, screening process and data extractions will include a pilot phase, discussion with experts. Modifications will be made where necessary to increase feasibility and reliability.

• The review’s reliance on English-language publications may introduce biases, limiting the inclusivity of perspectives from non-English sources and potentially neglecting valuable insights.

Introduction

Sickle cell disease (SCD) is the most prevalent severe inherited haemoglobin disorder globally, with a substantial disease burden. With an estimated annual birth of 300 000–400 000 affected children worldwide,^{1 2} the number of individuals living with SCD increased from 5.46 million to 7.74 million between 2000 and 2021. The Global Burden of Disease study of 2021 ranked SCD as the 12th leading cause of mortality.³ Despite its pervasive distribution, SCD’s impact is particularly severe in developing countries, notably in sub-Saharan Africa, the Middle East and parts of India, where constrained health resources and weak healthcare systems impede individuals’ access to adequate healthcare.^4–7

SCD is intricately associated with a broad spectrum of acute complications, including painful crises, splenic sequestration and acute chest syndrome, alongside chronic complications such as chronic anaemia, SCD nephropathy, pulmonary hypertension and avascular necrosis of the femoral head.^{8 9} Typically, SCD-related complications manifest from infancy, persisting across the life course and potentially impacting multiple organs.⁸ The occurrence of these complications is unpredictable, introducing uncertainties associated with living with a life-threatening condition and presenting emotional challenges that disrupt the social lives of affected individuals.¹⁰ Without adequate care, SCD results in significant mortality, and survivors grapple with substantial health challenges that affect all facets of their quality of life and well-being.^{10 11}

The primary objective of SCD management is to mitigate acute and chronic complications, ultimately enhancing both survival and the quality of life for affected individuals.¹⁰ Despite significant progress in medical care that has positively impacted SCD outcomes in high-income countries, the scenario is distinctly challenging in resource-limited settings. Managing SCD in developing nations introduces unique challenges, including limited healthcare resources, inadequate access to specialised care and economic disparities.¹² Achieving treatment goals in such contexts necessitates effective self-management (SM) alongside clinical care.

SM emerges as a critical component indispensable for individuals living with chronic conditions like SCD.¹³ It can be conceived as the active role patients play in handling their chronic conditions. This proactive engagement involves daily practices, tasks or behaviours aimed at controlling the impact of one or more chronic conditions on their quality of life.^{14 15} Alternatively, SM is also viewed as the support provided by health systems and community agencies to individuals with chronic conditions and their families, facilitating effective SM.¹² Related concepts include SM support, education, intervention and preparation.^{16 17} Best practices in SM support, coupled with standard SM programmes, empower patients with the knowledge, skills and confidence to manage their diseases, resulting in improved health outcomes and enhanced quality of life.^18–20 Beyond individual benefits, SM contributes to health systems by reducing healthcare costs, optimising resource utilisation and fostering collaboration between patients and practitioners.^{18 21 22}

While research on SM in the context of SCD is emerging, it has predominantly focused on primary studies whereby specific components such as chronic pain, transition, leg ulcers and psychosocial effects are addressed.^23–26 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. One of the ways to achieve this is to map existing evidence on SM interventions for people living with SCD in the developing world. Hence, this scoping review aims to fill this gap by mapping existing literature to identify crucial SM needs, strategies employed by individuals with SCD and the support systems available to them. This exploration is vital for tailoring healthcare interventions, educational programmes and policies to meet the specific needs of patients with SCD in developing countries. Thus, the purpose of this scoping review is to comprehensively examine the existing literature on SCD SM, summarise evidence regarding SM needs, strategies and support services for individuals living with SCD in developing countries. The findings will inform future research and systematic reviews and contribute to the development of context-specific and standardised SM interventions for SCD in these settings.

Methods and analysis

Scoping reviews are types of evidence synthesis approach that enable researchers to explore the breath or extent of the literature, map and summarise the evidence, and inform future research.²⁸ Scoping review was ideal for this study as it allows for comprehensive and systematic examination of the entire landscape and diversity of research about SM for SCD. The six-stage framework of Arksey and O’Malley²⁹,²⁹ will be adopted for this scoping review. The steps of the framework are as follows: (1) identifying the research question, (2) identifying relevant studies, (3) study selection, (4) charting the data, (5) data collating and (6) summary and reporting the results. We also followed the example of two publications that have used the framework to conduct similar studies.^{30 31} This protocol has been registered at the Open Science Framework on with registration number DOI: https://doi.org/10.17605/OSF.IO/V6H79.

Patient and public involvement

Patients and or the public were not involved in the design, conduct and reporting plan for this scoping review.

Identifying the research question

The guiding questions for this review are as follows: (1) What are the self-expressed SM needs among patients with SCD in developing countries, (2) What strategies do patients with SCD and parents of children with SCD use to meet their SM needs, (3) What are the existing SM support services and interventions for SCD care in developing countries and (4) What are the outcomes of SM support and interventions from the perspectives of patients with SCD and caregivers in developing countries.

Search for relevant studies

To address the research questions, four major electronic databases (PubMed, Scopus, Embase and Dimensions AI) will be searched to retrieve relevant literature. In consultation with a chartered librarian, a search strategy that uses a combination of controlled vocabularies such as Medical Subject Headings (MeSH) and keywords was developed. The search terms were informed by terms used in previous systematic and scoping reviews and other publications on SCD and SM in chronic illness^30–32 (online supplemental table S-1). The search strategy will be used to search the electronic databases from inception to January 2024. Additional search will be conducted in other internet-based resources including EBSCO Host (Health Source—Consumer Edition, Health Source: Nursing/Academic Edition, MEDLINE with Full Text, google, HINARI, World Health Organization Library, Google Scholar, ProQuest and university repositories). Furthermore, the reference lists of included full-text articles will be manually searched to identify other eligible articles that were not captured in the database and internet searches.

Study selection

Relevant articles that will be retrieved from the database search will be uploaded into the Mendeley software to remove duplicates. Using a predetermined inclusion and exclusion criteria informed by the population, concept and context criteria (online supplemental table S-2) titles and abstracts will be screened for full-text records independently by 25 trained graduate students and supervised by GOB, CO, GO and AAD. Furthermore, full-text screening will be conducted and each eligible article will be independently screened by two groups of authors (SAA, BN, JEK, PO and AAD, CMB and MA) using the eligibility criteria. Screening of full-text records will be reviewed by two independent researchers. Disagreements that persist will be resolved by a third reviewer.

Charting the data

A data extraction form developed in Microsoft Excel will be used to extract data from included studies. Key information that will be chartered will include the title of the study, author, publication year, publication country, study population, study objectives, study design/intervention, SM needs, patient/parent’s SM strategies, SM support services/interventions and key outcomes of SM support from patients with SCD and caregivers’ perspective. Consistent with a similar previous study,³⁰ two different data extraction sheets will be used. One for studies on SM needs and strategies, and another for SM support/interventions (online supplemental tables S-3 and S-4).

Data extraction forms will be piloted using five randomly selected articles. The extraction form will be modified based on discussions from the pilot to ensure reliable and accurate data are charted. Thereafter, the extraction will be performed independently by eight authors in two groups (GOO, BN, RO-D, AAD and CO, PFD, DFA and FN). Two authors (CMB and SAA) will review the extractions to check for accuracy and completeness.

Collating, summarising and reporting the results

Descriptive statistics (frequency counts) will be used to organise the characteristics of included studies including year and country of publication, populations of included studies, SM support/interventions types delivered to patients and types of studies. The main questions of this scoping review will be addressed by providing a thematic summary of the key findings related to patient reported SM need, SM strategies reported by patients, SM support or interventions delivered by practitioners to patients and the patient/practitioners reported outcomes of SM interventions.

Presentation of results

The search process and selection of records will be summarised in a flow chart following the guidelines outlined in the Preferred Reporting Items for Systematic Reviews and Meta-Analyses Extension for Scoping Reviews (PRISMA-ScR). The results for the study characteristics will be presented in tables. Maps will be used to present the geographical distribution of countries of included studies. Evidence from the thematic summaries of the research questions will be summarised in tables (see online supplemental table S-5) and narratively described under headings that correspond to scoping review questions.

Consultation exercise

A chartered librarian (KK-N) will be consulted for assistance in searching and retrieving relevant literature for this scoping review. Additionally, experts on the subject of SM and SCD will be consulted for inputs regarding the appropriateness of the data, sources of other relevant literature and perspectives that may not be apparent in this review.

Ethics and dissemination

Ethical approval is not required for this review because it relies on the collection and analysis of data from existing literature available in academic databases. Living with SCD poses substantial challenges for patients and their families, necessitating a comprehensive understanding of SM needs and effective strategies. This scoping review aims to explore the existing literature on SM interventions for SCD within the context of developing countries. We believe that our study will be of interest to researchers, healthcare practitioners, patients with SCD and families. Therefore, we aim to publish our research in reputable scientific journals. We also intend to present the findings at relevant international conferences. Furthermore, we hope to share our findings in patients and parents’ groups meetings.

Ethics statements

Patient consent for publication

Not applicable.