Sociodemographic Profile and Clinic of Polytransfused Sickle Cell Patients in Kinshasa: Case of the Center for Mixed Medicine and SS Anemia

Background: Sickle cell disease is an autosomal recessive hereditary hemoglobinopathy. Patients with sickle cell disease (HbSS, Sβ, SC phenotypes) with permanent hemolytic anemia very often require transfusions. Objective: To describe the socio-demographic and clinical characteristics of polytransfused sickle cell patients in Kinshasa. Methods: This is a descriptive cross-sectional study of polytransfused sickle cell patients followed at the SS Anemia Mixed Medicine Center (CMMASS). Socio-demographic and clinical data were recorded on survey sheets. The parameters of interest were the district, age, gender, linguistic area, level of education, marital status, religion and place of screening for sickle cell disease, according to age of screening, weight, number of transfusion and the clinical signs of major sickle cell disease. Results: the median age of polytransfused sickle cell patients is 29 years. The female gender is in the majority at 51.1%. The majority of polytransfused sickle cell patients (32.2%) come from the Mont Amba district and are from the Greater Kasai area (34.4%). All polytransfused sickle cell patients are single. The majority have high school level (56.6%), are from revivalist churches (75.6%), and were first diagnosed with sickle cell disease at CMMASS (63.75%). The median age of screening was 13 years, the median number of transfusions is 24. Vaso-occlusive crisis is the main reason for consultation (77.7%). Conclusion: This study reveals that the median age of polytransfused sickle cell patients is 29 years. The majority of patients are female, single, from secondary school, praying in revival churches, from the district of Mont-Amba and from the Grand Kasaï area. Screening for sickle cell disease is done late, the median number of transfusions is 24. Vaso-occlusive crisis is the main reason for consultation for polytransfused sickle cell patients.