Current perspectives of sickle cell disease in Nigeria: changing the narratives

Expert Rev Hematol. 2019 Aug;12(8):609-620. doi: 10.1080/17474086.2019.1631155. Epub 2019 Jun 25.

Abstract

Introduction: Sickle cell disease (SCD) is an inherited blood disorder characterized by clinical heterogeneity that may be influenced by environmental factors, ethnicity, race, social and economic factors as well as genetic and epigenetic factors. Areas covered: The present review was carried out to provide a comprehensive assessment of the current burden of SCD and treatments available for persons with SCD in Nigeria with the aim of identifying surveillance and treatment gaps, informing to guide the planning and implementation of better crisis prevention measures for SCD patients and set an agenda for new areas of SCD research in the country. This review assessed medical, biomedical and genetic studies on SCD patients in Nigeria and other endemic countries of the world. Expert opinion: Integration of hydroxyurea therapy into the management of SCD and surveillance via new-born screening (NBS) for early detection and management will improve the survival of persons with SCD in Nigeria. However, it will be important to carry out pilot studies, initiate strategic advocacy initiatives to educate the people about NBS benefits, develop collaborations between potential stakeholders and design sustainable financing scheme.

Keywords: Fetal hemoglobin; Nigeria; genetic counseling; genetic modifiers; hydroxyurea; neonatal screening; quality of life.

Publication types

  • Review

MeSH terms

  • Anemia, Sickle Cell / complications
  • Anemia, Sickle Cell / diagnosis*
  • Anemia, Sickle Cell / epidemiology
  • Anemia, Sickle Cell / genetics
  • Genetic Counseling
  • Hematopoietic Stem Cell Transplantation
  • Hemoglobins / genetics
  • Humans
  • Hydroxyurea / therapeutic use
  • Nigeria / epidemiology
  • Prenatal Diagnosis
  • Quality of Life
  • Stroke / complications

Substances

  • Hemoglobins
  • Hydroxyurea