The global burden of sickle cell disease in children under five years of age: a systematic review and meta-analysis

Abstract

BackgroundSickle cell disease (SCD) is a common haematological disorder, affecting millions of people worldwide. It is most prevalent in malarial endemic areas in the tropics where outcomes are often poor due to resource constraints, resulting in most children dying before reaching adulthood. As increasing progress is made towards reducing under 5 mortality from infectious causes, non-communicable diseases (NCDs) including SCD have risen to the forefront of the global health agenda. Despite this, the global mortality burden of SCD remains poorly understood. This study aimed to estimate the incidence and mortality of SCD in children under 5 years of age in order to inform policy and develop sustainable strategies to improve outcomes.MethodologyWe performed a systematic literature search of Medline, EMBASE, Journals@Ovid, and Web of Science for studies on the incidence and mortality of SCD in children under 5, with search dates set from January 1980 and July 2017. We conducted random effects meta-analysis to obtain pooled meta-estimates of birth prevalence and mortality rates globally, and for each World Health Organization (WHO) region.Results67 papers were found with relevant data. 52 contained data on incidence and prevalence and 15 contained data on mortality. The overall pooled estimate of mortality from the limited data available was 0.64 per 100 years of child observation (95% CI = 0.28-1.00) with the highest rate seen in Africa 7.3 (95% CI = 4.03-10.57). The global meta-estimate for the birth prevalence of homozygous sickle cell disease was 112 per 100 000 live births (95% CI = 101-123) with a birth prevalence in Africa of 1125 per 100 000 (95% CI = 680.43-1570.54) compared with 43.12 per 100 000 (95% CI = 30.31-55.92) in Europe.ConclusionThere were a number of limitations in the depth and breadth of available data however it is clear that both the highest prevalence and highest mortality of SCD is in Africa. In order to address this burden, there is a need for national comprehensive newborn screening to identify patients, and the development of holistic SCD care programmes to provide therapeutics and education for families and children with SCD. This targeted funding should form part of a broader increased global focus on NCDs in childhood.

Sickle cell disease and malaria: decreased exposure and asplenia can modulate the risk from Plasmodium falciparum

“…Sickle cell disease (SCD) is an autosomal recessive disorder characterized by chronic haemolytic anaemia and painful vaso-occlusive crises. SCD is common in sub-Saharan Africa, with a birth prevalence that in some areas reaches 2% [1], and where it is therefore a true issue of public health importance. Without early diagnosis and appropriate care many children born with SCD do not survive beyond their fifth birthday [1,2].…”

“…SCD is common in sub-Saharan Africa, with a birth prevalence that in some areas reaches 2% [1], and where it is therefore a true issue of public health importance. Without early diagnosis and appropriate care many children born with SCD do not survive beyond their fifth birthday [1,2]. SCD patients also experience increased susceptibility to severe infections, including malaria [3,4].…”

Catastrophic sickling crisis in patient undergoing cardiac transplantation with sickle cell trait

“…There are approximately 11 000 patients with sickle cell anemia in the United Kingdom (SCD) and an estimated 200 000 carriers of the HbAS sickle cell trait (SCT) . Worldwide it is estimated that there are 300 000 SCT births annually . While patients with SCD invariably have mild‐to‐severe anemia with hemoglobin between 50 and 110 g/L, a normal mean cell volume (MCV), and distinctive blood film features, patients who are heterozygous have SCT with typically <30‐45% HbS concentration, and have a normal hemoglobin, blood film, and MCV.…”

Sickle Cell Disease in East African Countries: Prevalence, Complications and Management

“…SCD affects millions of people globally and particularly prevalent among the people in sub-Saharan Africa [1,15]. Over 4.4 million people have sickle cell disease, while over 43 million have SCT [16].…”