Sickle Cell Disease in Africa

Abstract

Sickle cell disease (SCD) is common throughout much of sub-Saharan Africa, affecting up to 3% of births in some parts of the continent. Nevertheless, it remains a low priority for many health ministries. The most common form of SCD is caused by homozygosity for the β-globin S gene mutation (SS disease). It is widely believed that this condition is associated with very high child mortality, but reliable contemporary data are lacking. We have reviewed available African data on mortality associated with SS disease from published and unpublished sources, with an emphasis on two types of studies: cross-sectional population surveys and cohort studies. We have concluded that, although current data are inadequate to support definitive statements, they are consistent with an early-life mortality of 50%–90% among children born in Africa with SS disease. Inclusion of SCD interventions in child survival policies and programs in Africa could benefit from more precise estimates of numbers of deaths among children with SCD. A simple, representative, and affordable approach to estimate SCD child mortality is to test blood specimens already collected through large population surveys targeting conditions such as HIV, malaria, and malnutrition, and covering children of varying ages. Thus, although there is enough evidence to justify investments in screening, prophylaxis, and treatment for African children with SCD, better data are needed to estimate the numbers of child deaths preventable by such interventions and their cost effectiveness.

Organ damage in sickle cell disease study (ORDISS): protocol for a longitudinal cohort study based in Ghana

“…Over 305 000 babies are born worldwide annually with SCD mostly in low and middle income countries, and about 75% or more of these births occur in sub-Saharan Africa, posing an increasing health burden 1 and contributing to early childhood mortality. 2 SCD affects approximately 2% of newborns in Ghana. 3 Clinical syndromes of SCD include anaemia, infection, and the consequences of blood vessel blockage (vaso-occlusion).…”

Hydroxyurea Therapy for Children With Sickle Cell Anemia in Sub‐Saharan Africa: Rationale and Design of the REACH Trial

“…The availability of disease‐modifying treatments (transfusions, hydroxyurea, and stem cell transplantation) in developed countries has also improved the medical outcomes of patients with SCA 5, 6, 7, 8, 9. In contrast, without NBS for early identification, and little access to preventive interventions or disease‐modifying therapies, most babies in Africa with SCA die of acute anemia or infection within the first years of life, often without a diagnosis 10, 11, 12, 13, 14. Due to limited availability and use of hydroxyurea or other proven disease‐modifying therapies, a number of herbal, traditional, and perhaps unsafe treatments and procedures are commonly used to treat SCA across Africa.…”

Epidemiology: A moving target

“…In high-income countries, improved management of the disease and medication, including strong painkillers, has meant that patients with sickle-cell disease can expect a better quality of life. In low-income nations, however, those with sickle cell are not as fortunate: in Africa, at least 50% of children born with the condition die before their fifth birthday 3 .…”