Clinical manifestations of sickle cell disease in Africa and its association with foetal haemoglobin parameters.

Abstract

Background: Prevalence of sickle cell disease (SCD) across African countries ranges between 1-3% and contributes up to 7-16% of under-five mortality. In order to bridge the gap in management and cognate research, the SickleInAfrica consortium was established in 2017 to facilitate collaboration among African nations in order to establish regionally relevant healthcare standards for SCD patients. This work utilised the SickleInAfrica platform to study the levels of HbF and F cells and their relationship with sickle cell disease clinical manifestations in Ghana, Nigeria and Tanzania.

Methods: This study enrolled 290 individuals with SCD aged five years and above who were confirmed to be at steady state and were hydroxyurea naïve. Clinical history was obtained using an interviewer-administered questionnaire. Haematological parameters were determined by an automated haematology analyser, while quantification of HbF and F cells was implemented by high-performance liquid chromatography and flow cytometry, respectively. Age-adjusted logistic regression was employed to assess the association of HbF with the clinical manifestations.

Results: The most reported complication of SCD, requiring management in a hospital setting is pain crises, ranging from 66-96% with the highest in Tanzania and lowest in Ghana. HbF and F cell parameters show significant association with transfusion rates, frequency of painful crises and episodes of febrile illness.

Conclusion: This work highlights important differences and similarities across SCD populations in the three countries. This is important especially in development of interventions in the light of personalised medicine.