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

Abstract

Background: Sickle cell disease (SCD) is a public health problem in the Democratic Republic of Congo. If reference sickle cell centers have been implemented in capital-cities of African countries and have proven to be beneficial for SCD patients, it has never been set up ina rural area for families with very low sources of income. Method: A cohort of 143 children with SCD aged 10 years old (IQR [inter quartile range]: 6–15 years) (sex ratio male: female = 1.3) were clinically followed for 12 months without any specific intervention, outside management of acute events ,and then 12 months with a monthly medical visit , a biological follow-up and regular prophylaxis. Results: The median age of patients at the diagnosis of SCD was 2 years (IQR: 1–5). The implementation of standardized and regular follow-up in a new sickle cell referral center in a remote city showed an increase in the annual mean hemoglobin level from 50 to 70 g/L (p = 0.001), and a decrease of the lymphocytes count and spleen size (p < 0.001). A significant decrease (p < 0.001) in the average annual number of hospitalizations and episodes of vaso-occlusive crises, blood transfusions, infections, and acute chest syndromes were also observed. Conclusions: Creation of a sickle cell referral center and a regular follow-up of children with SCD are possible and applicable in the context of a remote city of an African country. Those simple and accessible measures can reduce the morbimortality of the sickle cell children.