
==== Front
BMJ Open
BMJ Open
bmjopen
bmjopen
BMJ Open
2044-6055
BMJ Publishing Group BMA House, Tavistock Square, London, WC1H 9JR

39260843
10.1136/bmjopen-2024-087723
bmjopen-2024-087723
Original Research
Haematology (Incl Blood Transfusion)
1700
1506
Self-management needs, strategies and support for individuals with sickle cell disease in developing countries: a scoping review
Druye Andrews Adjei 1andrews.druye@ucc.edu.gh

http://orcid.org/0000-0002-9582-8884
Amoadu Mustapha 2*mustapha.amoadu@ucc.edu.gh

http://orcid.org/0000-0002-4875-718X
Boso Christian Makafui 1christian.boso@ucc.edu.gh

Nabe Bernard 1bnabe@stu.ucc.edu.gh

Kagbo Justice Enock 1justicekagbo@gmail.com

http://orcid.org/0000-0002-4102-4995
Alhassan Amidu 1amidualhassan24@gmail.com

Odonkor Frank Offei 1fodonkor36@gmail.com

Lanyo Godswill Sedinam 1godswilllanyo@gmail.com

Davies Anita Efua 1adavies@ucc.edu.gh

Doe Patience Fakornam 3patience.doe@ucc.edu.gh

Okantey Christiana 1christaina.okantey@ucc.edu.gh

Ofori Godson Obeng 1gofori002@stu.ucc.edu.gh

https://twitter.com/Dorcasagyare3
http://orcid.org/0000-0003-0984-718X
Agyare Dorcas Frempomaa 1dorcas.agyare@ucc.edu.gh

http://orcid.org/0000-0003-3100-770X
Abraham Susanna Aba 3sabraham@ucc.edu.gh

1 Adult Health Department, School of Nursing and Midwifery, University of Cape Coast, Cape Coast, Ghana
2 Biomedical and Clinical Research Centre, University of Cape Coast, Cape Coast, Ghana
3 Department of Public Health, School of Nursing and Midwifery, University of Cape Coast, Cape Coast, Ghana
Supplemental material: Supplemental material This content has been supplied by the author(s). It has not been vetted by BMJ Publishing Group Limited (BMJ) and may not have been peer-reviewed. Any opinions or recommendations discussed are solely those of the author(s) and are not endorsed by BMJ. BMJ disclaims all liability and responsibility arising from any reliance placed on the content. Where the content includes any translated material, BMJ does not warrant the accuracy and reliability of the translations (including but not limited to local regulations, clinical guidelines, terminology, drug names and drug dosages), and is not responsible for any error and/or omissions arising from translation and adaptation or otherwise.

None declared.

Dorcas FrempomaaAgyare; dorcas.agyare@ucc.edu.gh
MustaphaAmoadu; mustapha.amoadu@ucc.edu.gh
2024
6 9 2024
14 9 e08772325 4 2024
20 8 2024
Copyright © Author(s) (or their employer(s)) 2024. Re-use permitted under CC BY-NC. No commercial re-use. See rights and permissions. Published by BMJ.
2024
https://creativecommons.org/licenses/by-nc/4.0/ This is an open access article distributed in accordance with the Creative Commons Attribution Non Commercial (CC BY-NC 4.0) license, which permits others to distribute, remix, adapt, build upon this work non-commercially, and license their derivative works on different terms, provided the original work is properly cited, appropriate credit is given, any changes made indicated, and the use is non-commercial. See: http://creativecommons.org/licenses/by-nc/4.0/.

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. The aim of the scoping review was to identify and map self-management needs of individuals living with SCD, the strategies they employed to meet those needs, and the support interventions available to them.

Methods and analysis

The review was conducted following the Askey and O’Malley’s framework to examine the landscape of SCD self-management research. Searches were conducted in PubMed, Scopus, Embase and Dimensions AI, with additional searches in other databases from inception to June 2024 included. Evidence from 14 studies was synthesised to identify self-management needs, strategies and interventions for individuals with SCD.

Results

The review identified diverse self-management needs among individuals with SCD, including knowledge deficits, emotional challenges, physical limitations and barriers to healthcare access. Various self-management strategies were reported, such as nutritional management, psychological coping techniques and proactive healthcare management. Self-management interventions, predominantly delivered by healthcare professionals, focused on providing education, skills training and support to individuals with SCD. The outcomes of self-management interventions consistently demonstrated significant improvements across various dimensions, including self-efficacy, knowledge enhancement, self-care practices and psychological well-being among individuals with SCD.

Conclusion

This scoping review underscores the importance of addressing the diverse self-management needs of individuals with SCD through tailored interventions and support systems to enhance overall well-being and disease management. Healthcare professionals should prioritise the implementation of multidisciplinary self-management interventions that encompass medical, emotional and social aspects of care to effectively support individuals with SCD in managing their condition. Future research should focus on longitudinal studies to assess the long-term effectiveness of self-management interventions in improving patient outcomes.

HAEMATOLOGY
Quality of Life
Review
Chronic Disease
==== Body
pmcSTRENGTHS AND LIMITATIONS OF THIS STUDY

The review was guided by the widely used Arksey and O’Malley framework and reported through the Preferred Reporting Items for Systematic Reviews and Meta-Analyses Extension for scoping reviews ensuring transparency and rigour.

The design and conduct of the search strategy were supported by a chartered librarian, the search strategy encompasses a wide array of databases and academic resources, and studies included not confined to a particular research methodology enhancing credibility and offering a comprehensive view of the subject matter.

The screening and extraction followed a robust process involving independent researchers and reviewers.

The included studies in this review were not appraised.

The review’s reliance on English-language publications may affect the volume of included papers and generalisation of the findings to non-English-speaking contexts.

Introduction

Sickle cell disease (SCD) stands as 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 all-cause 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.47

SCD is associated with a broad spectrum of acute complications, including painful crises, splenic sequestration and acute chest syndrome, alongside chronic complications like 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 significant 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 (Sickle Cell Society, 2018).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 managing 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.1820 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

Specific SM practices and interventions have been studied or implemented in developing countries to address the complex needs of individuals living with SCD.23 These include interventions focused on medication adherence, pain management strategies, nutritional education, coping mechanisms for emotional challenges and the establishment of peer support networks.24 25 For example, SM interventions have involved structured educational sessions on medication adherence, counselling for pain management techniques, nutritional guidance to maintain a healthy diet, psychological coping techniques for emotional challenges and the formation of peer support groups to provide ongoing support and shared experiences.24 25 Incorporating these specific examples underscores the critical role that SM plays in enhancing the quality of life and health outcomes for individuals with SCD in resource-limited settings.

While research on SM in the context of SCD is emerging, it has predominantly focused on primary studies, addressing specific components such as chronic pain, transition, leg ulcers and psychosocial effects.2629 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.24 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 was to comprehensively examine the existing literature on SCD SM, summarising 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

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.30 Scoping review was ideal for this study as it allows for a comprehensive and systematic examination of the entire landscape and diversity of research about SM for SCD. The six-stage framework of Arksey and O’Malley31 was adopted for this scoping review. The steps of the framework are (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 utilised the framework to conduct similar studies.32 33 The protocol for this study has been published.34

Patient and public involvement

No patients are involved.

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 and (3) What are the existing SM support services and interventions and associated outcomes for SCD care in developing countries.

Search for relevant studies

To address the research questions, four major electronic databases (PubMed, Scopus, Embase and Dimensions AI) were 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 illness32 33 35 (online supplemental table S1). The search strategy used in PubMed was modified for search in other databases (Scopus, Embase and Dimensions AI).

Additional handsearch was 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). Examples of searches conducted in all databases are presented in online supplemental table S2. Furthermore, the reference lists of included full-text articles were manually searched to identify other eligible articles that were not captured in the database and internet searches. The last date for the search was conducted on 23 June 2024.

Inclusion and exclusion criteria

The eligibility criteria outlined in online supplemental table S2 for screening search results and full-text inclusion/exclusion are designed to ensure a comprehensive yet focused approach to identifying relevant literature on SM of SCD in developing countries. The inclusion criteria encompass a diverse population, including individuals living with SCD, parents/caregivers and all types of SCD across age groups. The concept criteria target SM strategies, needs, support/interventions and outcomes related to medical, emotional and role management aspects of SCD. The context criteria specify studies conducted in developing countries, published in English, peer-reviewed and from 2000 onwards while the types of studies criteria include quantitative, qualitative, mixed-method and multimethod studies. Conversely, the exclusion criteria eliminate studies focusing solely on healthcare providers, those not related to SM for individuals with SCD, studies not meeting the specified context criteria (language, location and publication date) and non-peer-reviewed materials such as preprints, conference papers, commentaries and letters to editors. These criteria collectively ensure a thorough yet targeted selection of literature relevant to the scope of the scoping review.

Study selection

Relevant articles that were retrieved from the database search were uploaded into the Mendeley software to remove duplicates. Using a predetermined inclusion and exclusion informed by the population, concept and context criteria (online supplemental table S3) titles and abstracts were screened for full-text records independently by 25 trained graduate students and supervised by CMB, MA and AAD. Furthermore, full-text screening was conducted and each eligible article was independently screened by two groups of authors (BN, EK, AA and FOO, GSL and AED) using the eligibility criteria. The screening of full-text records was reviewed by two independent researchers. Disagreements in terms of inconsistencies in the screening process, particularly during the screening of full-text records, were resolved by a third reviewer through consensus discussions and referencing the predefined eligibility criteria.

Charting the data

A data extraction form developed in Microsoft Excel was used to extract data from included studies. Key information that was chartered included 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 study32 two different data extraction sheets were used. One for studies on SM needs and strategies and another for SM support/interventions (online supplemental tables S4 and S5).

Data extraction forms were piloted using five randomly selected articles. This ensured that reliable and accurate data were charted. Thereafter, the extraction was performed independently by six authors in two groups (BN, EK, AA, FOO, GSL and AED). Two authors (CMB and SAA) reviewed the extractions to check for accuracy and completeness.

Collating, summarising and reporting the results

Descriptive statistics (frequency counts) were 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 were 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-reported/practitioners-reported outcomes of SM interventions.

Presentation of results

The search process and selection of records are summarised in a flow chart following the guidelines outlined in the Preferred Reporting Items for Systematic Reviews and Meta-Analyses extension for Scoping Reviews. The results for the study characteristics are presented in tables. Evidence from the thematic summaries of the research questions is summarised in a table (see tables1 2) and narratively described under headings that correspond to scoping review questions.

Table 1 Thematic analysis of specific SM needs/challenges of sickle cell patients

Main theme	Specific SM needs/challenges	Authors	
Knowledge and education needs	Poor knowledge about SCD	36 40	
Knowledge deficit	36	
Emotional well-being needs	Anger, sadness, fear due to chronic illness	38	
Decreased self-esteem	37 39	
Fear of sexual impotence	37	
Feelings of insecurity	37	
Bullying and stigmatisation	38	
Social isolation and loneliness	37	
Need for long-lasting romantic relationships	37	
Difficulty in sexual activity	37	
Social interaction and support needs	Difficulty in interacting socially	28 37	
Medication management needs	Doubts related to medication	38	
Need for effective medication use	
Need to be reminded to administer medication	41	
Physical well-being needs	Need for effective ambulation	28	
Ineffective or insufficient food intake	
Impaired ambulation	
Chronic pain	
Need for rest	
Infection	
Healthcare access and behaviour needs	Delay in care-seeking	40	
Need for self-monitoring	42	
SCDsickle cell diseaseSMself-management

Table 2 Thematic analysis of self-management (SM) strategies used by sickle cell patients

Main theme	Specific SM strategies	Authors	
Physical strategies	Nutritional management	28 42	
Ensuring a safe and hygienic environment	43	
Making needed adjustments in cases where mobility is restricted	43	
Tepid sponging	42	
Hot showers	37 44	
Cold baths	37	
Massage	37 46	
Reduction in activity level	37 42	
Setting new priorities to stay healthy, alter old habits	43	
Avoiding alcohol	42	
Avoidance of extreme temperatures	42	
Preventing malaria	42	
Psychological and emotional strategies	Mental imagery	44 46	
Self-efficacy and living with family	47	
Repetition of positive sentences to adopt to pain	44 46	
Distraction techniques	46	
Deep breathing	44 46	
Relaxation (rest)	4244 46	
Health management strategies	Health assessment	42	
Regular assessment of the effectiveness of safety actions	43	
Frequent visits to a healthcare facility	39 42	

Consultation exercise

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

Results

The results of this review are organised into three subsections: search findings, study characteristics and findings based on the review questions.

Search findings

A total of 1545 studies were identified from the initial search in the main databases and an additional 72 were retrieved from other internet-based sources. After the removal of 301 duplicates with Mendeley reference manager, 1316 titles and abstracts were further screened for full-text records. 34 eligible full-text articles were retrieved out of which 20 were removed because they either did not contain the variables of interest, were preprints, reviews or were outside the scope of the study. Ultimately, 14 peer-reviewed articles were included in the review (figure 1).

Figure 1 PRISMA flow charts. PRISMA, Preferred Reporting Items for Systematic Reviews and Meta-Analyses.

Characteristics of included studies

Most of the studies (n=4) included in this review were conducted in Brazil (n=4), followed by Iran (n=3), then Ghana, Nigeria, Egypt (n=2 studies each) and Saudi Arabia (n=1) (see figure 2 for details). Five studies were quasi-experimental, four were cross-sectional surveys, four were qualitative descriptive designs and one was a mixed-method study (see figure 3 for details). The reviewed studies were conducted between 2014 and 2023. All the studies recruited the sample from the clinical area (mostly from SCD clinic) and targeted adults 18 years and older (n=9), children 1–18 years (n=2) and a combination of adults and children (n=3) (refer to online supplemental tables S4 and S5).

Figure 2 Maps of the countries of publication.

Figure 3 Chart of the number of designs used in the included studies.

SM needs of sickle cell patients

The reviewed studies reported a range of SM needs among individuals living with SCD. SM needs were not always explicitly stated as such but expressed as challenges, concerns, deficits, lack or demands reported by patients in relation to knowledge and education, emotional and social interaction, physical SM and healthcare access.

Several studies reported a knowledge deficit about SCD among patients.3640 This knowledge deficit translated into consequences such poor adherence to medication,38 delay in seeking care40 and poor disease management. For example, in the study by Cecilio et al,38 many patients fail to adhere to prescribed medications due to a misunderstanding of their benefits or the importance of medication adherence in effectively controlling SCD.

The reviewed studies further reported that patients with SCD expressed a range of emotional issues, including feelings of anger, sadness and fear related to living with chronic illness.38 Moreover, studies indicated decreased self-esteem among patients with SCD due to disease complications,37 39 along with fears of sexual dysfunction, feelings of insecurity and experiences of bullying and stigmatisation.37 Social isolation, loneliness and a desire for long-lasting romantic relationships were also indicated as emotional needs of patients with SCD.37 Furthermore, difficulties in socialising and forming connections with others were reported.28 37

Physical SM needs encompassed concerns such as inadequate food intake, impaired ambulation, chronic pain and the need for adequate rest. Patients expressed the need for effective medication use. For example, in two studies, the patients requested reminders to administer medications.38 41

The review highlighted delays in care-seeking behaviour among patients with SCD and the need for timely access to healthcare services.40 Additionally, the need for self-monitoring of health parameters was identified as essential for effective SM.42

SM strategies used by patients with SCD

Studies included in the review reported a diverse SM strategy employed by individuals living with SCD to manage the various dimensions physical, psychological and healthcare management needs.

In terms of strategies employed to maintain physical health, nutritional management emerged as a prominent strategy, with studies emphasising the importance of dietary interventions in managing SCD-related complications.28 42 Additionally, maintaining a safe and hygienic environment and making necessary adjustments to accommodate restricted mobility due to SCD complications were described as important SM strategies.43 Furthermore, temperature regulation techniques such as tepid sponging, hot showers and cold baths were identified as strategies to alleviate symptoms and manage pain associated with SCD.37 42 44 Massage therapy was also recognised for its potential to alleviate discomfort and enhance overall well-being.37 44 Moreover, individuals with SCD employed strategies such as reducing activity levels and avoiding alcohol and extreme temperatures to prevent disease exacerbations and complications.37 42

Psychological and emotional SM strategies encompassed a range of coping mechanisms aimed at enhancing resilience and mitigating the emotional burden associated with SCD. Techniques such as mental imagery, deep breathing, relaxation and distraction were identified as effective tools for managing pain and promoting psychological well-being.4446 Additionally, fostering self-efficacy and maintaining supportive familial relationships were recognised as protective factors against emotional distress and isolation.47 Repeating positive affirmations and establishing new priorities for healthy habits enhanced psychological resilience and coping strategies.45 46

In terms of healthcare management, individuals with SCD engaged in proactive measures such as frequent self-monitoring and self-evaluation of the effectiveness of therapeutic interventions to prevent complications.42 43 Furthermore, adherence to healthcare appointments and the utilisation of healthcare facilities for timely intervention were emphasised as essential components of disease management and preventive care.39 42

SM strategies used by sickle cell patients

SM support/interventions and outcomes

Five studies reported SM interventions that were designed by health professionals to support patient’s SM.364446 48 All five studies employed quasi-experimental designs that involved group sessions. These group sessions varied in size, ranging from large groups to smaller groups. Three studies conducted by Ahmadi et al4446 integrated a combination of large-group sessions, small-group sessions and individual sessions within their intervention frameworks.

The delivery of these interventions involved collaborative efforts between healthcare professionals and patients. Healthcare professionals commonly used didactic methodologies, including face-to-face lectures and practical skills sessions. Patients were actively engaged in SM activities, such as documenting pain triggers in diaries, as observed in Ahmadi et al.4446 Additionally, patients were instructed to undertake SM actions and record these in diaries, including techniques like deep breathing exercises.

Moreover, in studies such as those conducted by Mohammed et al36 and Fouda et al,48 patients were provided with self-directed learning materials that contained SM information. The duration of the intervention programmes varied across studies, spanning from 6 months to 12 months, with each programme incorporating a follow-up period. The follow-up intervals ranged from 3 weeks, as observed in the study by36 to 36 weeks, as documented in the investigations by Ahmadi et al.44 46

The SM outcomes assessed across the studies were self-efficacy, quality of life, psychological symptoms, knowledge enhancement and self-care practice. Two studies evaluated the effect of SM intervention on patient self-efficacy.36 45 Additionally, studies including Fouda et al40 and Ahmadi et al46 evaluated the impact of SM interventions on enhancing the quality of life among participants. One study44 evaluated the effects of interventions on psychological symptoms including depression, anxiety and stress levels among sickle cell patients.36 A study investigated SM practices knowledge enhancement, self-care practices and self-efficacy.

The findings across interventions consistently demonstrated significant improvement across the various dimensions of patient outcomes evaluated. For example, in the Ahmadi et al’s45 study, a majority of participants (50.7%) exhibited moderate self-efficacy, whereas postintervention, the majority (81.2%) demonstrated high self-efficacy levels. Similarly, in the study conducted by Mohammed et al,36 significant improvements were observed in patient knowledge, self-care practices and self-efficacy. Additionally, a significant reduction in the frequency and severity of pain crises, as well as a decrease in hospitalisations, were reported.

Discussion

While there is a substantial body of literature on SCD SM in the USA and Europe as emphasised by Rodigari et al,25 this scoping review focused on developing countries, synthesised evidence from 14 studies to explore SM needs, strategies and interventions among individuals living with SCD, indicating a limited number of eligible studies identified for this specific review. With only 14 studies meeting the inclusion criteria, it is evident that further research efforts are needed to comprehensively understand and address the diverse self-manage needs of individuals living with SCD. The studies included in the review covered a range of participants, including adults, children and a combination of both. Additionally, the geographical distribution of the studies indicates a predominance of research conducted in Brazil, followed by Iran, Ghana, Nigeria, Egypt and Saudi Arabia. There remains a paucity of research from many regions, suggesting potential areas for future investigation. Furthermore, the studies included in the review were mainly cross-sectional in nature. Longitudinal studies assessing the long-term effectiveness of SM interventions are warranted to ascertain their sustained impact on patient outcomes.

Frameworks that define SM for chronic disease from patients’ perspectives conceptualise them as medical management or physical health management, social role management and emotional management dimensions.214951 For example, Lorig and Holman’s study50 conceptualises SM as comprising three main tasks: medical management, role management and emotional management. Generally, the scoping review findings closely align with the medical management aspect of the framework. For example, several SM needs identified, such as medication adherence, nutritional needs and monitoring of health parameters, directly correspond to the medical management task. Additionally, SM strategies identified, such as temperature regulation and healthcare utilisation, demonstrate efforts to address these medical management needs.

The findings of the scoping review also significantly align with the emotional management dimensions, although to a lesser extent. Emotional management involves coping with the emotional consequences of the chronic condition, such as anxiety, depression and stress.52 53 The review identified a range of emotional challenges experienced by individuals with SCD, including decreased self-esteem, fears of sexual dysfunction, feelings of insecurity and experiences of bullying and stigmatisation. These findings have been reported in a review conducted by Rodigari et al25 among people living with SCD in the USA. Specifically, it indicates that issues such as decreased self-esteem, fears of sexual dysfunction, feelings of insecurity and experiences of bullying and stigmatisation are not unique to individuals with SCD in developing countries but are also experienced by those in more developed regions like the USA. This consistency across different contexts underscores the pervasive nature of these emotional challenges for people living with SCD globally. SM strategies such as psychological coping techniques and positive affirmations directly address emotional management needs. There are areas within emotional management, such as addressing specific psychological symptoms like depression and anxiety, where more targeted research is needed in developing. However, in the USA, this has been thoroughly explored.25 There may be a lack of specialised healthcare infrastructure and professionals trained to address mental health issues in the context of chronic diseases like SCD in developing countries. Also, cultural factors may influence the recognition and reporting of psychological symptoms, affecting the research focus and findings.

In terms of social role management, the findings of the review demonstrate limited alignment with this aspect. Role management involves maintaining roles and responsibilities in various life domains despite the challenges posed by the chronic condition.54 The review identified social challenges faced by individuals with SCD, such as difficulties in social interactions. These social challenges have been reported in a recent review by Khan et al55 that explored studies from global context. However, there is limited evidence globally, directly addressing social role management tasks such as maintaining employment, family roles or social participation. This finding highlights the need for more research and intervention efforts to comprehensively address the social role management tasks inherent in SCD SM in developing countries.

Regarding SM interventions, comparison of the findings to what has been recommended for SM support intervention for people living with chronic diseases1851 5658 demonstrated partial alignment. For example, the Department of Health framework (2006) emphasises the importance of providing patients with skills education and training, information, tools and devices and support networks to enhance their ability to manage chronic conditions effectively. It is obvious that the findings of the SM interventions are largely consistent with the aspect of skills education and training. Several studies included in the review implemented interventions aimed at equipping individuals with SCD with the necessary knowledge, skills and techniques to manage their condition effectively. Patients were provided with information about SCD, its management and strategies to enhance self-care practices. Moreover, interventions often incorporated skill training components such as problem-solving, decision-making, action planning and self-monitoring, which are essential for effective SM.

Additionally, many interventions included in the review provided patients with resources such as self-directed learning materials, diaries or other resources containing information about SCD management and self-care practices. These resources served as valuable tools for patients to enhance their understanding of the condition, track their symptoms and implement SM strategies effectively. Additionally, interventions that used reminders for medication adherence or encouraged patients to document pain triggers in diaries exemplify the provision of tools to support SM.

Improving SM of SCD in developing countries involves several key aspects beyond skills education and training. Enhancing access to healthcare services and regular medical follow-ups is crucial. Strengthening community support systems can provide shared experiences and resources while tailoring interventions to be culturally sensitive can improve acceptance and effectiveness. Providing economic support can alleviate the financial burden of managing SCD and offering nutritional education can help patients maintain a healthy diet. Integrating mental health services addresses psychological challenges such as depression and anxiety and educating patients on medication adherence is essential for better health outcomes. Additionally, encouraging safe physical activity with proper guidelines can contribute to overall health and well-being.

However, there is limited evidence directly addressing the aspect of support networks within the findings of the scoping review. Support networks encompass the involvement of healthcare professionals, family members, peers and community resources in providing ongoing support to individuals with chronic conditions.59 Therefore, further research exploring the role of support networks and peer support interventions in facilitating SM among individuals with SCD is required in developing countries. A study by Desine et al60 revealed the benefits of support networks and peer support in SM among people living with SCD. Understanding these support networks can help healthcare providers identify both supportive social ties and less supportive relationships, which can be targeted for intervention. Strong support networks and peer support are crucial for effective SM, as they provide essential resources, emotional support and practical assistance for individuals living with SCD.60

SM interventions are designed to be delivered through a multidisciplinary approach involving health professionals, non-health professionals and peer support workers with lived experience in chronic disease.6163 However, the majority of SM interventions included in the review were delivered by healthcare professionals, such as physicians, nurses and psychologists. These professionals provided structured educational sessions, counselling and guidance on adopting SM strategies. The lack of non-professional delivery of SM interventions for people with SCD in developing countries may stem from limited training and resources for non-healthcare providers. Additionally, there might be a greater reliance on healthcare professionals due to a lack of established peer networks and community support structures.

While the involvement of non-health professionals, such as educators, social workers or community organisers, in delivering SM interventions was mentioned in some studies, it was less common compared with the participation of health professionals. Furthermore, peer support workers (individuals with lived experience of SCD or similar chronic conditions) were not explicitly identified as facilitators of SM interventions in the reviewed studies. In some established SM intervention programmes, such as the Chronic Disease Self-Management Programmes (CDSMP)62 and the Expert Patient Programmes (EPP),57 trained peers often serve as facilitators. Research has demonstrated that their performance is comparable to or even better than that of health professionals.64 65 Therefore, exploring interventions with peer supporters and non-health professionals as facilitators within the context of SM interventions for SCD could be beneficial. Developed countries may have SM interventions delivered by various stakeholders including peer networks, community networks, caregivers support networks and conversational chatbots.66 In developing countries, delivering SM interventions through non-healthcare providers, such as peer networks, community groups and caregiver support networks, offers several benefits. These include increased accessibility and reach, cultural relevance, enhanced trust and rapport with patients, cost-effectiveness and the empowerment of individuals and communities. Non-healthcare providers can provide ongoing support, practical advice from lived experiences and a sense of community, all of which are crucial for effective SM of SCD.

The findings of the review showed that the outcomes of SM interventions for individuals with SCD significantly improve various dimensions of patient well-being. The included studies reported increases in self-efficacy levels, enhanced knowledge about the condition, improved self-care practices and positive impacts on overall health outcomes. Additionally, reductions in the frequency and severity of pain crises, as well as decreased hospitalizations, demonstrate the interventions’ positive impact on health outcomes. The findings align with a large body of research that has investigated the effectiveness of SM interventions across various chronic diseases. For example, studies about the CDSMP and the EPP have demonstrated significant improvements in patient outcomes, including enhanced self-efficacy, improved disease knowledge and better self-care practices among individuals with conditions such as diabetes, arthritis and chronic obstructive pulmonary disease.67 68 Therefore, the findings of SM interventions in SCD align with the broader global evidence supporting the efficacy of such interventions.24 69

Despite its contributions, this scoping review is not without limitations. First, the study did not distinguish between different types of SCD or the varied clinical courses of the disease, which may have different SM needs and challenges. Moreover, there are different complications associated with SCD, such as acute and chronic complications, as well as specific complications like leg ulcers and priapism, which were not differentiated in the analysis. Additionally, the study did not differentiate SM needs and strategies among various demographic characteristics, such as age and gender, which could influence the effectiveness of interventions. However, the review extends our knowledge of SM needs, useful strategies and relevant interventions that can meet the needs of patients with SCD. Additionally, the studies included in the review were not appraised for quality, which could potentially introduce bias into the synthesised evidence. These limitations underscore the need for future research to address these gaps and provide a more comprehensive understanding of SM in SCD.

Implication for policy and practice

Policy-makers should prioritise funding and resources to support the training of non-healthcare providers, such as peer supporters, community groups and caregivers, to deliver SM interventions. Implementing culturally sensitive educational programmes that address medical, emotional and social role management aspects is crucial. Healthcare systems should integrate mental health services and offer regular medical follow-ups to address psychological challenges and ensure comprehensive care. Developing community-based support networks can provide shared experiences, practical advice and ongoing support, which are essential for effective SM. Additionally, leveraging cost-effective tools such as self-directed learning materials, mobile health technologies and conversational chatbots can enhance accessibility and engagement. Encouraging collaborations between healthcare professionals and non-health professionals can improve the reach and effectiveness of SM interventions. These recommendations aim to build a robust support system that empowers individuals with SCD to manage their condition effectively and improve their quality of life.

Suggestions for future studies

Future reviews and studies should focus on conducting longitudinal research to assess the long-term effectiveness of SM interventions for SCD in developing countries. It is essential to explore the role of non-healthcare providers, such as peer supporters and community groups, in delivering these interventions. Additionally, research should investigate culturally tailored interventions that address medical, emotional and social role management aspects of SM. Expanding the geographical scope to include underrepresented regions and incorporating diverse participant demographics can provide a more comprehensive understanding of SM needs and strategies. Evaluating the integration of mental health services and the use of digital health tools in SM interventions can offer valuable insights for improving patient outcomes in developing countries. Future studies could explore the nuanced role of SM during the transition from adolescence to young adulthood in individuals with SCD, particularly focusing on differences in SM strategies and needs between paediatric and adult populations.

Conclusion

This review found that the journey of individuals living with SCD is marked by a myriad of needs ranging from knowledge deficits, emotional distress, physical limitations and healthcare access barriers. However, patients with SCD employ diverse strategies to cope with and navigate their condition. SM interventions, primarily delivered by healthcare professionals, demonstrate promising outcomes in improving patient knowledge, self-care practices and overall well-being. However, there is a need to further explore the role of non-health professionals and peer support workers in delivering tailored SM support. Collaborative efforts between stakeholders, including patients, healthcare providers, researchers and community organisations, are required for developing relevant interventions that address the unique needs of individuals with SCD.

supplementary material

10.1136/bmjopen-2024-087723 online supplemental file 1

Acknowledgements

We express our gratitude to Kwame Kodua-Ntim who serves as a Librarian at the Sam Jonah Library of the University of Cape Coast for his valuable advice and support in developing the search strategy for the primary review.

Data availability statement

No data are available. All data relevant to the study are included in the article or uploaded as online supplemental information.

Review Process File
6 9 2024

Funding: The authors have not declared a specific grant for this research from any funding agency in the public, commercial or not-for-profit sectors.

Prepublication history and additional supplemental material for this paper are available online. To view these files, please visit the journal online (https://doi.org/10.1136/bmjopen-2024-087723).

Provenance and peer review: Not commissioned; externally peer reviewed.

Patient consent for publication: Not applicable.

Map disclaimer: The inclusion of any map (including the depiction of any boundaries therein), or of any geographic or locational reference, does not imply the expression of any opinion whatsoever on the part of BMJ concerning the legal status of any country, territory, jurisdiction or area or of its authorities. Any such expression remains solely that of the relevant source and is not endorsed by BMJ. Maps are provided without any warranty of any kind, either express or implied.

Patient and public involvement: Patients and/or the public were not involved in the design, or conduct, or reporting, or dissemination plans of this research.

Ethics approval: Ethical approval was not required for this review as it relied on the collection and analysis of data from existing literature available in academic databases.
==== Refs
References

1 Kato GJ Piel FB Reid CD et al Sickle cell disease Nat Rev Dis Primers 2018 4 18010 10.1038/nrdp.2018.10 29542687
2 Piel FB Patil AP Howes RE et al Global epidemiology of sickle haemoglobin in neonates: a contemporary geostatistical model-based map and population estimates Lancet 2013 381 142 51 10.1016/S0140-6736(12)61229-X 23103089
3 Thomson AM McHugh TA Oron AP et al Global, regional, and national prevalence and mortality burden of sickle cell disease, 2000–2021: a systematic analysis from the Global Burden of Disease Study 2021 Lancet Haematol 2023 10 e585 99 10.1016/S2352-3026(23)00118-7 37331373
4 Adigwe OP Onoja SO Onavbavba G A Critical Review of Sickle Cell Disease Burden and Challenges in Sub-Saharan Africa J Blood Med 2023 14 367 76 10.2147/JBM.S406196 37284610
5 Akinsete A Treatment of Sickle Cell Disease in Sub-Saharan Africa: We have come a long way, but still have far to go J Global Med 2022 2 10.51496/jogm.v2.79
6 Colombatti R Birkegård C Medici M PB2215: GLOBAL EPIDEMIOLOGY OF SICKLE CELL DISEASE: A SYSTEMATIC LITERATURE REVIEW Hemasphere 2022 6 2085 6 10.1097/01.HS9.0000851688.00394.f4
7 Kavanagh PL Fasipe TA Wun T Sickle Cell Disease: A Review JAMA 2022 328 57 68 10.1001/jama.2022.10233 35788790
8 Mercado SH An Outpatient Pain Plan and Emergency Department Pain Pathway for Adults With Sickle Cell Disease Top Pain Manag 2023 38 1 5 10.1097/01.TPM.0000937036.58946.07
9 National Heart, Lung and BI Evidence-based management of sickle cell disease: expert panel report, 2014 NHLBI, NIH 2014
10 Sickle Cell Society Standards for clinical care of adults with sickle cell disease in the UK 2018
11 Kaur K Faisal A Kennedy K et al Causes of Mortality in Sickle Cell Disease Patients: A Longitudinal Review of Deceased Sickle Cell Patients Blood 2022 140 5440 1 10.1182/blood-2022-159339
12 Houwing ME Buddenbaum M Verheul TCJ et al Improving access to healthcare for paediatric sickle cell disease patients: a qualitative study on healthcare professionals’ views BMC Health Serv Res 2021 21 1 13 10.1186/s12913-021-06245-2 33388053
13 Karvonen J POS0201-pare “mood is happy and downright wild” - health promotion and well-being through peer-led group activity Ann Rheum Dis 2023 3 326 10.1136/annrheumdis-2023-eular.898 36357155
14 Clark NM Becker MH Janz NK et al Self-Management of Chronic Disease by Older Adults J Aging Health 1991 3 3 27 10.1177/089826439100300101
15 Ryan P Sawin KJ The Individual and Family Self-Management Theory: background and perspectives on context, process, and outcomes Nurs Outlook 2009 57 217 25 10.1016/j.outlook.2008.10.004 19631064
16 Johnston SE Liddy CE Ives SM Self-management support: a new approach still anchored in an old model of health care Can J Public Health 2011 102 68 72 10.1007/BF03404881 21485969
17 Nagpal J Rawat S Gupta L et al A stepped wedge cluster randomized trial to evaluate the effectiveness of a community leader-driven kit-based diabetes self-management education approach in improving diabetes control and care: study protocol for the DElhi Diabetes INTervention Trial (DEDINTT) Trials 2023 24 673 10.1186/s13063-023-07712-3 37845694
18 Coleman MT Newton KS Supporting self-management in patients with chronic illness Am Fam Physician 2005 72 1503 10 16273817
19 Lorig KR Sobel DS Stewart AL et al Evidence Suggesting That a Chronic Disease Self-Management Program Can Improve Health Status While Reducing Hospitalization Med Care 1999 37 5 14 10.1097/00005650-199901000-00003 10413387
20 Warsi A Wang PS LaValley MP et al Self-management education programs in chronic disease: a systematic review and methodological critique of the literature Arch Intern Med 2004 164 1641 9 10.1001/archinte.164.15.1641 15302634
21 Grady PA Gough LL Self-Management: A Comprehensive Approach to Management of Chronic Conditions Am J Public Health 2014 104 e25 31 10.2105/AJPH.2014.302041 24922170
22 Jovicic A Holroyd-Leduc JM Straus SE Effects of self-management intervention on health outcomes of patients with heart failure: a systematic review of randomized controlled trials BMC Cardiovasc Disord 2006 6 1 8 10.1186/1471-2261-6-43 16401349
23 Obeagu EI Obeagu GU Peer-to-Peer Learning Networks: Sickle Cell Disease Education Among Adolescents Elite J Public Health 2023 1 34 41
24 Poku BA Atkin KM Kirk S Self-management interventions for children and young people with sickle cell disease: A systematic review Health Expect 2023 26 579 612 10.1111/hex.13692 36597596
25 Rodigari F Brugnera G Colombatti R Health-related quality of life in hemoglobinopathies: A systematic review from a global perspective Front Pediatr 2022 10 10.3389/fped.2022.886674
26 Issom DZ Hartvigsen G Bonacina S et al User-Centric eHealth Tool to Address the Psychosocial Effects of Sickle Cell Disease Stud Health Technol Inform 2016 225 627 8 10.3233/978-1-61499-658-3-627 27332283
27 Labore N Mawn B Dixon J et al Exploring Transition to Self-Management Within the Culture of Sickle Cell Disease J Transcult Nurs 2017 28 70 8 10.1177/1043659615609404 26446139
28 Lacerda FKL Ferreira SL Nascimento E do et al Self-care deficits in women with leg ulcers and sickle cell disease Rev Bras Enferm 2019 72 72 8 10.1590/0034-7167-2018-0005 31851237
29 Matthie N Ross D Sinha C et al A Qualitative Study of Chronic Pain and Self-Management in Adults with Sickle Cell Disease J Natl Med Assoc 2019 111 158 68 10.1016/j.jnma.2018.08.001 30266214
30 Munn Z Peters MDJ Stern C et al Systematic review or scoping review? Guidance for authors when choosing between a systematic or scoping review approach BMC Med Res Methodol 2018 147 60 10.1186/s12874-018-0611-x 30463519
31 Arksey H O’Malley L Scoping studies: towards a methodological framework Int J Soc Res Methodol 2005 8 19 32 10.1080/1364557032000119616
32 Budhwani S Wodchis WP Zimmermann C et al Self-management, self-management support needs and interventions in advanced cancer: a scoping review BMJ Support Palliat Care 2019 9 12 25 10.1136/bmjspcare-2018-001529
33 Riegel B Westland H Iovino P et al Characteristics of self-care interventions for patients with a chronic condition: A scoping review Int J Nurs Stud 2021 116 103713 10.1016/j.ijnurstu.2020.103713 32768137
34 Druye AA Boso CM Amoadu M et al Self-management needs, strategies and support for sickle cell disease in developing countries: a scoping review protocol BMJ Open 2024 14 10.1136/bmjopen-2023-083688
35 Gyamfi J Ojo T Epou S et al Evidence-based interventions implemented in low-and middle-income countries for sickle cell disease management: A systematic review of randomized controlled trials PLoS One 2021 16 e0246700 10.1371/journal.pone.0246700 33596221
36 Mohammed E Hegazy M Ali H et al Effect of an Educational Program on Self-Care Management for Patients with Sickle Cell Disease J Nurs Sci Benha Univ 2021 2 124 46 10.21608/jnsbu.2021.159649
37 Costa DO Araújo FA Xavier ASG et al Self-care of men with priapism and sickle cell disease Rev Bras Enferm 2018 71 2418 24 10.1590/0034-7167-2017-0464 30304171
38 Cecilio SG Pereira S Pinto VDS et al Barriers experienced in self-care practice by young people with sickle cell disease Hematol Transfus Cell Ther 2018 40 207 12 10.1016/j.htct.2017.11.009 30128428
39 Bastos Ferreira Tavares N Monique Araújo do Nascimento N Tavares de Luna Neto R et al Self-care practice in people with sickle cell anemia RBPS 2017 30 1 7 10.5020/18061230.2017.6212
40 Fouda NM Adly RM Mahmoud FS et al Effect of Self-Learning Guidelines on Quality of Life and Self –Care Reported Practice of Adolescents with Sickle Cell Anemia J Nurs Sci 2021 2020 473 84
41 Fowora MA Adherence to self-care management of sickle cell disease among caregivers, 184 2016
42 Druye AA Nelson K Robinson B Self-management for sickle cell disease among patients and parents: A qualitative study Chronic Illn 2024 20 233 45 10.1177/17423953231172797 37151019
43 Al NE Abdulmutalib IAM Self-care Management and Self-efficacy among Adult Patients with Sickle Cell Disease Am J Nurs Res 2018 7 51 7 10.12691/ajnr-7
44 Ahmadi M Shariati A Poormansouri S et al The Effectiveness of Self Management Program on Pain, Fatigue, Depression, Anxiety, and Stress in Sickle Cell Patients: A Quasi-Experimental Study Jundishapur J Chronic Dis Care 2015 4 10.17795/jjcdc-29521
45 Ahmadi M Shariati A Jahani S et al The Effectiveness of Self-Management Programs on Self-Efficacy in Patients With Sickle Cell Disease Jundishapur J Chronic Dis Care 2014 3 10.17795/jjcdc-21702
46 Ahmadi M Jahani S Poormansouri S et al The Effectiveness of self management program on quality of life in patients with sickle cell disease Iran J Ped Hematol Oncol 2015 5 18 26 25914799
47 Amertil NP Ayitey EK Kpongboe DG et al Exploring self-management in adult sickle cell disease patients’ at a Teaching Hospital in Ghana Nurs Open 2021 8 1336 44 10.1002/nop2.750 33369204
48 Fouda NM Adly RM Mahmoud FS et al Effect of Self-Learning Guidelines on Quality of Life and Self –Care Reported Practice of Adolescents with Sickle Cell Anemia J Nurs Sci 2014 400 15
49 Novak M Costantini L Schneider S et al Approaches to self-management in chronic illness Semin Dial 2013 26 188 94 10.1111/sdi.12080 23520989
50 Lorig KR Holman HR Self-management education: history, definition, outcomes, and mechanisms Ann Behav Med 2003 26 1 7 10.1207/S15324796ABM2601_01 12867348
51 McGowan PT Self-Management Education and Support in Chronic Disease Management Prim Care Clin Off Pract 2012 39 307 25 10.1016/j.pop.2012.03.005
52 Franklin M Willis K Lewis S et al Chronic condition self-management is a social practice J Sociol (Melb) 2023 59 215 31 10.1177/14407833211038059
53 Thompson DM Booth L Moore D et al Peer support for people with chronic conditions: a systematic review of reviews BMC Health Serv Res 2022 1 16 10.1186/s12913-022-07816-7 34974828
54 Webel AR Higgins PA The Relationship Between Social Roles and Self-Management Behavior in Women Living with HIV/AIDS Womens Health Issues 2012 22 e27 33 10.1016/j.whi.2011.05.010 21798762
55 Khan H Krull M Hankins JS et al Sickle cell disease and social determinants of health: A scoping review Pediatr Blood Cancer 2023 70 10.1002/pbc.30089
56 O’Connell S Mc Carthy VJC Savage E Frameworks for self-management support for chronic disease: a cross-country comparative document analysis BMC Health Serv Res 2018 18 1 10 10.1186/s12913-018-3387-0 29291745
57 Great Britain, Department of Health Supporting people with long term conditions to self care 2006
58 van Hooft SM Been-Dahmen JMJ Ista E et al A realist review: what do nurse-led self-management interventions achieve for outpatients with a chronic condition? J Adv Nurs 2017 73 1255 71 10.1111/jan.13189 27754557
59 Sunkersing D Martin FC Sullivan P et al Care and support networks of community-dwelling frail individuals in North West London: a comparison of patient and healthcare workers’ perceptions BMC Geriatr 2022 22 1 11 10.1186/s12877-022-03561-y 34979941
60 Desine S Eskin L Bonham VL et al Social support networks of adults with sickle cell disease J Genet Couns 2021 30 1418 27 10.1002/jgc4.1410 33847032
61 Hobbs M Management Program Chronic Disease 2001
62 Lorig KR Ritter P Stewart AL et al Chronic disease self-management program: 2-year health status and health care utilization outcomes Med Care 2001 39 1217 23 10.1097/00005650-200111000-00008 11606875
63 Vadiee M The UK “Expert Patient Program” and self-care in chronic disease management: An analysis Eur Geriatr Med 2012 3 201 5 10.1016/j.eurger.2012.02.003
64 Baksi AK Al-Mrayat M Hogan D et al Peer advisers compared with specialist health professionals in delivering a training programme on self-management to people with diabetes: a randomized controlled trial Diabet Med 2008 25 1076 82 10.1111/j.1464-5491.2008.02542.x 18937675
65 Khan F Saleh F Pathan MF Effectiveness of Diabetes Education by Health Professionals Versus Peers on Improving Diabetes Care: A Quasi-Experimental Study Jundishapur J Health Sci 2018 In Press 10.5812/jjhs.82058
66 Issom D-Z Hardy-Dessources M-D Romana M et al Toward a Conversational Agent to Support the Self-Management of Adults and Young Adults With Sickle Cell Disease: Usability and Usefulness Study Front Digit Health 2021 3 10.3389/fdgth.2021.600333
67 Lorig KR Sobel DS Stewart AL et al Evidence suggesting that a chronic disease self-management program can improve health status while reducing hospitalization: a randomized trial Med Care 1999 37 5 14 10.1097/00005650-199901000-00003 10413387
68 Kennedy A Reeves D Bower P et al The effectiveness and cost effectiveness of a national lay-led self care support programme for patients with long-term conditions: a pragmatic randomised controlled trial J Epidemiol Community Health 2007 61 254 61 10.1136/jech.2006.053538 17325405
69 Hoyt CR Hurwitz S Varughese TE et al Individual-level behavioral interventions to support optimal development of children with sickle cell disease: A systematic review Pediatr Blood Cancer 2023 70 e30178 10.1002/pbc.30178 36583467
