Neonatal screening and clinical care programmes for sickle cell disorders in sub-Saharan Africa: Lessons from pilot studies

Abstract

Despite the widespread use of neonatal screening programmes for sickle cell disease in Western regions, few studies have focused on the special healthcare needs in sub-Saharan African countries. The purpose of this review is to evaluate the need for a neonatal screening programme for sickle cell disease, and if justified, to propose a realistic healthcare programme for sickle cell newborns in those countries based on personal experiences in Kinshasa (Democratic Republic of the Congo) and Ouagadougou (Burkina Faso) as well as from a review of the literature. There are well-established criteria for the development of neonatal screening programmes for sickle cell disease in sub-Saharan African countries. In particular, in regions where incidence of the disease is 0.5 per 1000 or higher, a sickle cell screening programme can be proposed that includes the systematic screening of all newborns, or the targeted screening of those newborns who have a mother with a sickle cell or haemoglobin C trait. Screening should be preferentially organized using cord blood, with a simple, effective and affordable screening method such as isoelectric focusing. If necessary, confirmation of results should be performed using another cost-effective technique such as citrate agar electrophoresis at an acidic pH. There is also a need for a sickle cell disease clinical care programme which should include: infection prophylaxis with penicillin and malarial prophylaxis; family training to identify early severe or persistent symptoms and the gravity of malarial crises; the evaluation of nutritional status and adequate fluid intake; and the importance of regular medical visits. Improved knowledge of the diagnosis was found to reduce the need for unnecessary and unsafe blood transfusions. This paper provides an overview of practices employed in neonatal screening and clinical care programmes for sickle cell disease in sub-Saharan African countries. The development of these programmes is pivotal to improving the health care of those affected by haemoglobin disorders. However, such programmes require major economic and organizational resources, which must taken into account and balanced against other local health priorities.