Clinical manifestations of sickle cell disease in Africa and its association with foetal haemoglobin parameters
Evans Xorse Amuzu, Florence Urio, Elliot Eli Dogbe, Ponsian Peter, Suraj Yawnumah Abubakar, Chinedu Okeke, Olasinbo Olumuyiwa Balogun, Petronilla J. Ozumba, Alex Osei‐Akoto, Vivian Paintsil, Obiageli Nnodu, Emmanuel Balandya, Julie Makani, Madu Anazoeze, Daniel Ansong, Siana Nkya
Abstract
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. 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. 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. 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. People with sickle cell disease (SCD) have red blood cells (RBC) that do not function correctly. SCD affects 1–3% of newborns in African countries. Foetal haemoglobin (HbF) is a protein mostly found in RBC of newborns that enables the cells to carry more oxygen around the body and can help reduce the seriousness of SCD. We studied the levels of HbF in people with SCD and their relationship with clinical complications of SCD in people from Ghana, Nigeria and Tanzania. We found that low HbF level increases blood transfusions, severe pain and fever. Our work highlights important differences and similarities across SCD populations in the three countries and could be used to better manage treatment of SCD. Amuzu et al. assess the levels of foetal haemoglobin and F cells and their relationship with sickle cell disease clinical manifestations in Ghana, Nigeria and Tanzania. HbF and F cell parameters show significant association with transfusion rates, frequency of painful crises and episodes of febrile illness.