Abstract
ABSTRACT Background The Democratic Republic of the Congo (DRC) is the third most affected country worldwide by sickle cell disease (SCD). However, this disease is still orphaned in the country; large-scale control actions are rare, and little is known about its management. Objective To assess current practices in the management of SCD in Kisangani, DRC. Methods This cross-sectional study was conducted in six health facilities in Kisangani. It involved 198 presumed sickle cell patients attending the above health facilities. The study focused on the sociodemographic and clinical data of the participants, obtained through a clinical examination and their medical records. Diagnostic confirmation of SCD was made by high-performance liquid chromatography coupled to mass spectrometry. Data were analyzed using SPSS 20.0. Results The diagnosis of SCD was confirmed in 194 (98.0%; 95% CI: 94.9–99.2) participants, while it was not confirmed in 4 (2.0%; 95% CI: 0.8–5.1) participants. The diagnosis was mainly made by the Emmel test (42.9%). 45.8% of participants had previously been transfused with the blood of their parents. Folic acid was taken by 48.5% of participants and the previous intake of hydroxyurea was reported in 5.1% of participants. The participants vaccinated against Pneumococcus were 13.6% and against Haemophilus influenzae type b 28.3%. Penicillin prophylaxis was received by only 1.5% and malaria prophylaxis by 11.6% of participants. Conclusion Standard-care practices for SCD patients in Kisangani are insufficient. The Congolese government should regard this disease as a health priority and consider actions to improve its management.
Highlights
Sickle cell disease (SCD), the most common inherited genetic disease worldwide, is currently a major global health concern, especially in sub-Saharan Africa (SSA)
When investigating the diagnostic method used in the past for these participants to be considered sickle cell disease (SCD) patients, in general, the Emmel test was predominantly used with 85 (42.9%) participants, while hemoglobin electrophoresis was used in only 12.1% of participants
We report on current practices regarding the diagnosis of SCD and the features of its management in Kisangani, Democratic Republic of the Congo (DRC)
Summary
Sickle cell disease (SCD), the most common inherited genetic disease worldwide, is currently a major global health concern, especially in sub-Saharan Africa (SSA). Some of the most common associated morbidities include chronic pain and intermittent painful episodes, anemia, musculoskeletal problems, stroke, acute chest syndrome and recurrent infections These complications often co-exist, affecting the quality of life for patients, and if not adequately managed, may lead to death. The Democratic Republic of the Congo (DRC) is the third most affected country worldwide by sickle cell disease (SCD). This disease is still orphaned in the country; large-scale control actions are rare, and little is known about its management. Methods: This cross-sectional study was conducted in six health facilities in Kisangani It involved 198 presumed sickle cell patients attending the above health facilities. The Congolese government should regard this disease as a health priority and consider actions to improve its management
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