Evidence-based interventions for reducing sickle cell disease-associated morbidity and mortality in sub-Saharan Africa: A scoping review
Objective: Sickle cell disease is a lifelong illness affecting millions of people globally, but predominantly burdensome in sub-Saharan Africa, where most affected children do not live to adulthood, despite available evidence-based interventions that reduce the disease burden in high-income countrie...
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| Format: | Article |
| Language: | English |
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SAGE Publishing
2023-09-01
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| Series: | SAGE Open Medicine |
| Online Access: | https://doi.org/10.1177/20503121231197866 |
| _version_ | 1797682912205733888 |
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| author | Emmanuel Emenike Arji Ujunwa Justina Eze Gloria Oluchukwu Ezenwaka Neil Kennedy |
| author_facet | Emmanuel Emenike Arji Ujunwa Justina Eze Gloria Oluchukwu Ezenwaka Neil Kennedy |
| author_sort | Emmanuel Emenike Arji |
| collection | DOAJ |
| description | Objective: Sickle cell disease is a lifelong illness affecting millions of people globally, but predominantly burdensome in sub-Saharan Africa, where most affected children do not live to adulthood, despite available evidence-based interventions that reduce the disease burden in high-income countries. Method: We reviewed studies evaluating evidence-based interventions that decrease sickle cell disease-related morbidity and mortality among children living in sub-Saharan Africa. We used the Joanna Briggs scoping review methodological framework and grouped identified evidence-based interventions into preventative pharmacotherapeutic agents, newborn screening and comprehensive healthcare, disease-modifying agents, nutritional supplementation, systemic treatment, supportive agents and patient/carer/population education. Results: We included 36 studies: 18 randomized controlled trials, 11 observational studies, 5 before-and-after studies and 2 economic evaluation studies, with most of the studies performed in West African countries. Included studies suggest evidence-based interventions effectively to reduce the common morbidities associated with sickle cell disease such as stroke, vaso-occlusive crisis, acute chest syndrome, severe anaemia and malaria infection. Evidence-based interventions also improve survival among study participants. Specifically, our review shows hydroxyurea increases haemoglobin and foetal haemoglobin levels, a finding with practical implications given the challenges with blood transfusion in this setting. The feasibility of implementing individual interventions is hampered by challenges such as affordability, accessibility and the availability of financial and human resources. Conclusion: Our review suggests that regular use of low-dose hydroxyurea therapy, sulphadoxine–pyrimethamine chemoprophylaxis, L-arginine and Omega-3 fatty acid supplementation and establishment of specialist stand-alone sickle cell clinics could reduce the sickle cell disease-associated morbidity and mortality in sub-Saharan Africa countries. |
| first_indexed | 2024-03-12T00:06:47Z |
| format | Article |
| id | doaj.art-066448ed692e47ef8984c1106d6aa00e |
| institution | Directory Open Access Journal |
| issn | 2050-3121 |
| language | English |
| last_indexed | 2024-03-12T00:06:47Z |
| publishDate | 2023-09-01 |
| publisher | SAGE Publishing |
| record_format | Article |
| series | SAGE Open Medicine |
| spelling | doaj.art-066448ed692e47ef8984c1106d6aa00e2023-09-16T10:04:02ZengSAGE PublishingSAGE Open Medicine2050-31212023-09-011110.1177/20503121231197866Evidence-based interventions for reducing sickle cell disease-associated morbidity and mortality in sub-Saharan Africa: A scoping reviewEmmanuel Emenike Arji0Ujunwa Justina Eze1Gloria Oluchukwu Ezenwaka2Neil Kennedy3School of Medicine, Dentistry and Biomedical Science, Queen’s University Belfast, Belfast, UKDepartment of Family Medicine, WellSpan Good Samaritan Hospital, Lebanon, PA, USADepartment of Paediatrics, Enugu State University Teaching Hospital, Parklane, Enugu, Enugu State, NigeriaSchool of Medicine, Dentistry and Biomedical Science, Queen’s University Belfast, Belfast, UKObjective: Sickle cell disease is a lifelong illness affecting millions of people globally, but predominantly burdensome in sub-Saharan Africa, where most affected children do not live to adulthood, despite available evidence-based interventions that reduce the disease burden in high-income countries. Method: We reviewed studies evaluating evidence-based interventions that decrease sickle cell disease-related morbidity and mortality among children living in sub-Saharan Africa. We used the Joanna Briggs scoping review methodological framework and grouped identified evidence-based interventions into preventative pharmacotherapeutic agents, newborn screening and comprehensive healthcare, disease-modifying agents, nutritional supplementation, systemic treatment, supportive agents and patient/carer/population education. Results: We included 36 studies: 18 randomized controlled trials, 11 observational studies, 5 before-and-after studies and 2 economic evaluation studies, with most of the studies performed in West African countries. Included studies suggest evidence-based interventions effectively to reduce the common morbidities associated with sickle cell disease such as stroke, vaso-occlusive crisis, acute chest syndrome, severe anaemia and malaria infection. Evidence-based interventions also improve survival among study participants. Specifically, our review shows hydroxyurea increases haemoglobin and foetal haemoglobin levels, a finding with practical implications given the challenges with blood transfusion in this setting. The feasibility of implementing individual interventions is hampered by challenges such as affordability, accessibility and the availability of financial and human resources. Conclusion: Our review suggests that regular use of low-dose hydroxyurea therapy, sulphadoxine–pyrimethamine chemoprophylaxis, L-arginine and Omega-3 fatty acid supplementation and establishment of specialist stand-alone sickle cell clinics could reduce the sickle cell disease-associated morbidity and mortality in sub-Saharan Africa countries.https://doi.org/10.1177/20503121231197866 |
| spellingShingle | Emmanuel Emenike Arji Ujunwa Justina Eze Gloria Oluchukwu Ezenwaka Neil Kennedy Evidence-based interventions for reducing sickle cell disease-associated morbidity and mortality in sub-Saharan Africa: A scoping review SAGE Open Medicine |
| title | Evidence-based interventions for reducing sickle cell disease-associated morbidity and mortality in sub-Saharan Africa: A scoping review |
| title_full | Evidence-based interventions for reducing sickle cell disease-associated morbidity and mortality in sub-Saharan Africa: A scoping review |
| title_fullStr | Evidence-based interventions for reducing sickle cell disease-associated morbidity and mortality in sub-Saharan Africa: A scoping review |
| title_full_unstemmed | Evidence-based interventions for reducing sickle cell disease-associated morbidity and mortality in sub-Saharan Africa: A scoping review |
| title_short | Evidence-based interventions for reducing sickle cell disease-associated morbidity and mortality in sub-Saharan Africa: A scoping review |
| title_sort | evidence based interventions for reducing sickle cell disease associated morbidity and mortality in sub saharan africa a scoping review |
| url | https://doi.org/10.1177/20503121231197866 |
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