Hematology (Dec 2022)

Overview of current progress and challenges in diagnosis, and management of pediatric sickle cell disease in Democratic Republic of the Congo

  • Emmanuel Tebandite Kasai,
  • Jean Pierre Alworong’a Opara,
  • Justin Ntokamunda Kadima,
  • Masendu Kalenga,
  • Salomon Batina Agasa,
  • Roland Marini Djang’eing‘a,
  • François Boemer

DOI
https://doi.org/10.1080/16078454.2021.2023399
Journal volume & issue
Vol. 27, no. 1
pp. 132 – 140

Abstract

Read online

Objectives Sickle cell disease (SCD) encompasses health complications, primarily affecting the hematologic system and leading to high death rates in childhood. As a rule, the World Health Organisation (WHO) stepwise gold-standard about the strategies for prevention, diagnosis, and treatment of SCD must be multidimensional. This overview aimed to highlight current advances and challenges linked to strategic issues, diagnosis, the prevalence, and treatment of pediatric cases in Sub-Saharan Africa, particularly the Democratic Republic of the Congo. Methods We searched data on Google Scholar, Medline, PubMed, Science Direct, Scopus, and ResearchGate. Results The laboratory diagnosis of SCD has progressed from conventional electrophoresis to rapid point-of-care tests that allows early neonate screening. HemoTypeSCTM is an affordable test for neonatal screening in DRC. The pediatric SCD prevalence in Sub-Saharan Africa lay within 1–7.7% of homozygous(SS) and 15–40% of the heterozygous(AS) forms of SCD, depending on the method used and the ethnic population tested. Various supportive management protocols for comorbidities and complications exist, but they are not standardized in the Region. Conclusion Notwithstanding some progress accomplished, the disease is still challenging in Sub-Saharan Africa due to limited early diagnostic testing and a lack of specific medications. There is a need for harmonizing therapeutic protocols and conducting controlled valid clinical trials.

Keywords