Current challenges and new approaches to implementing optimal management of sickle cell disease in sub-Saharan Africa

Ally, Mwashungi; Balandya, Emmanuel

doi:10.1053/j.seminhematol.2023.08.002

Cited by 6 publications

(6 citation statements)

References 99 publications

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“…Ally and Balandya (2023) [23] Narrative Recommended folic acid supplementation in the management of SCD to prevent folate deficiency which could arise as a result of elevated RBC production.…”

Section: Study Type Resultsmentioning

confidence: 99%

“…In most sub-Saharan African countries, the recommended daily dosage of folic acid among people living with SCD is 1-5 milligrams daily. The 5 milligrams of folic acid per day was reported in some reviewed articles by Ally and Balandya, Dixit et al, and Nnajekwu et al [20,23,30]. Also, folic acid 1 milligram daily was reported in some reviewed articles by Williams et al, Bhange et al, and Hur and Durojaye [21,29,22].…”

Section: Folic Acid Use and Dosagementioning

confidence: 98%

“…In order to prevent folic acid deficiency, which could result from increased RBC production, folic acid administration was advised in a 2023 study conducted by Ally and Balandya in the management of SCD [23,34]. There is conflicting evidence, according to Dixit et al, Williams et al, and Nnajekwu et al, that folic acid supplements are beneficial for SCD patients [20,29,30].…”

Section: Folate Supplementation and Folic Acid Deficiency Preventionmentioning

confidence: 99%

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Folic Acid in the Treatment of Sickle Cell Disease: A Systematic Review

Arrey Agbor,

Panday,

Ejaz

et al. 2024

Cureus

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Sickle cell disease (SCD) is a group of inherited genetic disorders that is caused by a mutation in the gene that codes for hemoglobin subunit β. This systematic review aimed to evaluate the effect of folic acid in the treatment of SCD patients. We retrieved 3730 articles from PubMed, PubMed Central, Google Scholar, and ScienceDirect databases. We employed a search technique that involved framing keywords, such as folic acid, folate, and sickle cell illness, and the Medical Subject Headings (MeSH) strategy in PubMed. We chose research articles that had been published during the last 10 years, as well as case reports, systematic reviews and meta-analyses, literature reviews, randomized controlled trials, and observational studies. Exclusion criteria included paid full-text articles, abstracts, non-English studies, and patients who do not have SCD. The 2020 Preferred Reporting Items for Systematic Reviews and Meta-Analyses (PRISMA) criteria were used in the design of our systematic review. It was found that the majority of SCD patients were receiving regular folic acid supplements and that their plasma folate levels were either increased or within normal range, with no discernible impact on other clinical outcomes such as hemoglobin levels, infections, or pain crises. SCD patients produce more red blood cells than healthy individuals, and nearly all SCD patients receive daily folic acid supplements. On the other hand, not enough information is available on folic acid's potential benefits in the management of SCD; thus, there is a need for more large clinical trials.

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Section: Study Type Resultsmentioning

confidence: 99%

Section: Folic Acid Use and Dosagementioning

confidence: 98%

Section: Folate Supplementation and Folic Acid Deficiency Preventionmentioning

confidence: 99%

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Folic Acid in the Treatment of Sickle Cell Disease: A Systematic Review

Arrey Agbor,

Panday,

Ejaz

et al. 2024

Cureus

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“…Automated RBCX is a specialized service, and unfortunately, due to the scarce resources, its diffusion is limited in Africa and in other low-and middle-income countries. However, efforts in recent years to incorporate at least manual exchange blood transfusion into routine care are ongoing in some countries of sub-Saharan Africa [31]. In Tanzania, for example, manual exchange blood transfusion was first reported in 2017.…”

Section: Discussionmentioning

confidence: 99%

“…In Tanzania, for example, manual exchange blood transfusion was first reported in 2017. Since then, it has been performed in both public and private tertiary health facilities in patients with either acute or chronic complications [31,32], but no cases of pregnancy women with SCD are reported.…”

Section: Discussionmentioning

confidence: 99%

Red Blood Cell Exchange as a Valid Therapeutic Approach for Pregnancy Management in Sickle Cell Disease: Three Explicative Cases and Systematic Review of Literature

Valentini,

Pellegrino,

Ceglie

et al. 2023

JCM

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Pregnancy in women with sickle cell disease (SCD) is a high-risk situation, especially during the third trimester of gestation and in the post-partum period, due to chronic hypoxia and vaso-occlusive phenomena occurring in the maternal–fetal microcirculation: as a result, unfavorable outcomes, such as intra-uterine growth restriction, prematurity or fetal loss are more frequent in SCD pregnancies. Therefore, there is a consensus on the need for a strict and multidisciplinary follow-up within specialized structures. Transfusion support remains the mainstay of treatment of SCD pregnancies, whereas more targeted modalities are still controversial: the benefit of prophylactic management, either by simple transfusions or by automated red blood cell exchange (aRBCX), is not unanimously recognized. We illustrate the cases of three SCD pregnant patients who underwent aRBCX procedures at our institution in different clinical scenarios. Moreover, we carried out a careful literature revision to investigate the management of pregnancy in SCD, with a particular focus on the viability of aRBCX. Our experience and the current literature support the use of aRBCX in pregnancy as a feasible and safe procedure, provided that specialized equipment and an experienced apheresis team is available. However, further research in this high-risk population, with appropriately powered prospective trials, is desirable to refine the indications and timing of aRBCX and to confirm the advantages of this approach on other transfusion modalities.

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Special edition of the Seminars in Hematology series on Global Hematology Care

Schuh

2023

Seminars in Hematology

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Current challenges and new approaches to implementing optimal management of sickle cell disease in sub-Saharan Africa

Cited by 6 publications

References 99 publications

Folic Acid in the Treatment of Sickle Cell Disease: A Systematic Review

Folic Acid in the Treatment of Sickle Cell Disease: A Systematic Review

Red Blood Cell Exchange as a Valid Therapeutic Approach for Pregnancy Management in Sickle Cell Disease: Three Explicative Cases and Systematic Review of Literature

Special edition of the Seminars in Hematology series on Global Hematology Care

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