Nigeria carries the world's highest burden of sickle cell disease (SCD). Approximately 150,000 infants are born with homozygous sickle cell disease (HbSS) in Nigeria every year β representing roughly 45% of all sickle cell births globally. An estimated 25β30% of the Nigerian population (approximately 40β50 million people) carry the sickle cell trait (HbAS).
This creates an enormous public health challenge: any two AS parents have a 1 in 4 chance of producing an SS child with each pregnancy. In Lagos, where the population density is highest and genetic mixing is extensive, sickle cell disease is a daily clinical reality across every age group.
Haemoglobin β the oxygen-carrying protein in red blood cells β is coded by two genes, one inherited from each parent. In sickle cell disease, a point mutation in the beta-globin gene causes haemoglobin to polymerise (solidify) under low-oxygen conditions, distorting red cells into the characteristic sickle shape. Sickled cells are fragile, obstruct blood vessels, and are destroyed rapidly β causing the disease's hallmarks.
| Parent 1 | Parent 2 | Possible child genotypes | Risk of SCD |
|---|---|---|---|
| AA | AA | All AA | Zero |
| AA | AS | 50% AA, 50% AS | Zero |
| AS | AS | 25% AA, 50% AS, 25% SS | 25% per pregnancy |
| AS | SS | 50% AS, 50% SS | 50% per pregnancy |
| SS | SS | All SS | 100% |
| AS | AC | 25% AA, 25% AS, 25% AC, 25% SC | 25% SC per pregnancy |
The most common and most feared manifestation β sudden, severe pain caused by sickled cells obstructing blood flow to bones, joints, and organs. In Lagos, crises are frequently triggered by: malaria infection (the most important trigger in the Nigerian context), dehydration, cold exposure (particularly air conditioning), physical exertion, and infection. Adequate hydration, malaria prophylaxis, and prompt treatment of infections significantly reduce crisis frequency.
The chronic destruction of sickled red cells (haemolytic anaemia) causes the characteristic pallor, fatigue, and jaundice of SCD. Baseline haemoglobin in HbSS patients is typically 6β9 g/dL β below the normal range. Acute anaemic crises (aplastic crisis from parvovirus B19 infection, or splenic sequestration in young children) are life-threatening emergencies.
Chronic sickling damages multiple organ systems over time: the spleen (functional asplenia by age 5 β dramatically increasing infection risk, particularly from encapsulated bacteria); the kidneys (sickle cell nephropathy β a significant cause of chronic kidney disease); the brain (stroke occurs in 11% of HbSS children by age 20); the eyes (proliferative sickle retinopathy); and the heart (cardiomegaly and cardiac failure).
About the author: Dr. Adebayo Okonkwo is a General Practitioner at Mascot Healthcare, Akoka, Lagos. All statistics cited in this article are from WHO, FMOH Nigeria, and peer-reviewed journals β with sources available on request.
Haemoglobin electrophoresis at Mascot Healthcare, Akoka. Result. By appointment.