Abstract
Introduction: Sickle cell disease (SCD) is one of the most
common cause of mortality and morbidity in Africa. There is paucity of
data on SCD from Burundi. Methods: A cross-sectional study was
conducted using a predetermined questionnaires that included
socio-demographic characteristics, basis of diagnosis, common clinical
scenarios, transfusion requirements, usage of hydroxyurea, usage of
folic acid, and knowledge about bone marrow transplantation.
Results: Participants were 174 patients with SCD with male:
female ratio of 1:1. The median age was10 years (1.3 - 42 years). The
diagnosis of SCD was made by Emmel test in 139/168 (82.74 %) and
electrophoresis in 29/168 (17.26%) patients. Nearly 150/164 (91.4%)
patients ≥ 1 episode of Veno-occlusive crisis in the preceding year. Out
of 165 patients, 77(45.8%) required ≤3 admissions, 55 (39.4%)
>3 times and 24 (14.5 %) patients reported no admission in
past 1 year. Out of 166 patients, 131 (79%) reported no usage of
hydroxyurea, while 35 patients (21%) reported taking hydroxyurea. Among
35, only 17 were taking hydroxyurea on regular basis and 18 were taking
irregularly. Majority of patients (96.5%) were unaware of bone marrow
transplantation as a curative treatment option Conclusion: Our
data from Burundi points towards an urgent need to make hydroxyurea
available and affordable. Health systems strengthening with focus on
education and training of the healthcare professionals is a priority.