Caring for Africa’s sickle cell children: will we rise to the challenge?

Abstract

Background Most of the world’s sickle cell disease (SCD) burden is in Africa, where it is a major contributor to child morbidity and mortality. Despite the low cost of many preventive SCD interventions, insufficient resources have been allocated, and progress in alleviating the SCD burden has lagged behind other public-health efforts in Africa. The recent announcement of massive new funding for research into curative SCD therapies is encouraging in the long term, but over the next few decades, it is unlikely to help Africa’s SCD children substantially. Main discussion A major barrier to progress has been the absence of large-scale early-life screening. Most SCD deaths in Africa probably occur before cases are even diagnosed. In the last few years, novel inexpensive SCD point-of-care test kits have become widely available and have been deployed successfully in African field settings. These kits could potentially enable universal early SCD screening. Other recent developments are the expansion of the pneumococcal conjugate vaccine towards near-universal coverage, and the demonstrated safety, efficacy, and increasing availability and affordability of hydroxyurea across the continent. Most elements of standard healthcare for SCD children that are already proven to work in the West, could and should now be implemented at scale in Africa. National and continental SCD research and care networks in Africa have also made substantial progress, assembling care guidelines and enabling the deployment and scale-up of SCD public-health systems. Substantial logistical, cultural, and awareness barriers remain, but with sufficient financial and political will, similar barriers have already been overcome in efforts to control other diseases in Africa. Conclusion and recommendations Despite remaining challenges, several high-SCD-burden African countries have the political will and infrastructure for the rapid implementation and scale-up of comprehensive SCD childcare programs. A globally funded effort starting with these countries and expanding elsewhere in Africa and to other high-burden countries, including India, could transform the lives of SCD children worldwide and help countries to attain their Sustainable Development Goals. This endeavor would also require ongoing research focused on the unique needs and challenges of SCD patients, and children in particular, in regions of high prevalence.

Association of sickle cell disease with nutritional status among under-five children and mediating role of hemoglobin level: secondary analysis of data from 2018 Nigeria Demographic and Health Survey

“…Furthermore, low Hb is a consistent predictor of stroke and mortality among children with SCD [12,40]. This mediation analysis, therefore, highlights the critical need for interventions to optimize Hb level among under-ve children with SCD; including universal point-of-care screening in early infancy, hydroxyurea therapy, and judicious use of blood transfusion [9]. The pitfalls of blood transfusion in African settings concern unavailability of transfusable blood, transmission of infections, and alloimmunization.…”

“…Tackling undernutrition among under-ve children with SCD will also contribute to overall progress toward Sustainable Development Goal Target 2.2 [8] in Nigeria-where high prevalences of growth faltering and SCD coincide. Characterization of SCD-nutritional status relationship at the national level is necessary to integrate nutritional approach to SCD management into a tailored public health package for children with SCD [9].…”

“…status poses considerable threat to well-being of children with SCD and render them prone to adverse prognosis. Integration of a nutrition-oriented approach into a de nitive SCD care package [9] and its implementation at scale is needed to mitigate nutritional vulnerability of these children.…”

Improvement of Sickle Cell Disease Morbi-Mortality in Children: Experience Gained in a Remote Area of an African Country

“…This model has not integrated the primary health care system into the intar of malaria, tuberculosis or HIV that are properly managed at health centre levels even in remote areas of Sub-Saharan Africa. A major barrier to progress has been the absence of large-scale early-life screening [43], the high cost of screening with conventional methods (hemoglobin electrophoresis) as well as the lack of standardized and systematic medical follow-up to all patients screened [16]. In the last few years, novel inexpensive SCD point-of-care test kits have become widely available and have been deployed successfully in African field settings.…”

“…Other recent developments are the expansion of the pneumococcal conjugate vaccine towards near-universal coverage, and the demonstrated safety, efficacy, and increasing availability and affordability of hydroxyurea across African continent. Most elements of standard healthcare for SCD children that are already proven to work in the West, could and should now be implemented at scale in Africa countries [43].…”