title: Challenge of managing sickle cell disease in a pediatric population living in kinshasa, democratic republic of congo: a sickle cell center experience.
abstract: In the Democratic Republic of Congo (DRC), sickle cell disease is not yet really regarded as a health care priority. The patterns of sickle cell disease in patients living in Kinshasa, DRC are discussed and the difficulties encountered in their management are highlighted. The cross-sectional survey is of sickle cell patients and their families attending the Centre de Médecine Mixte et d'Anémie SS de Yolo (CMMASS), Kinshasa, DRC, between January and April 2009. Completed questionnaires were received from 168 respondents (111 girls; 57 boys). Seventy-one percent of the subjects were diagnosed before the age of 2 years but none in the neonatal period. Sickle cell disease was diagnosed in 54.8% of the patients after they had suffered pain crises. Of the 168 subjects, 74.0% had previously received blood transfusions. Seventy-five (45.0%) had more than three severe pain crises per year. A minority of 35.0% reported that they regularly took an antibioprophylaxis. Seventy-five (45.0%) subjects were eligible for hydroxyurea (HU) therapy but in all cases this drug was taken irregularly. Eighty-two percent of drugs were purchased by the parents. One hundred and sixty-three children (97.0%) were vaccinated according to the Expanded Programme on Immunization (EPI), 61.0% against Streptococcus pneumoniae and 16.0% against the Hepatitis B virus (HBV). No case of immunization against Hemophilus influenzae and Salmonella sp was reported. Neonatal screening programs, early educational detection programs for families, use of current method treatments and an implementation of a health insurance system for sickle cell disease will improve detection and management for these and future patients in our population.
authors.affiliation: Division of Haemato-Oncology and Nephrology, Department of Paediatrics, University Hospital of Kinshasa, School of Medicine, University of Kinshasa , Kinshasa , Democratic Republic of Congo .
authors.forename: Michel Ntetani
authors.initials: MN
authors.lastname: Aloni
authors.forename: Leonard
authors.initials: L
authors.lastname: Nkee
mesh_terms: D000293:Adolescent; D000328:Adult; D000755:Anemia, Sickle Cell / Q000453:epidemiology / Q000628:therapy*; D000986:Antisickling Agents / Q000008:administration & dosage*; D001803:Blood Transfusion; D017848:Blood-Borne Pathogens; D002648:Child; D002675:Child, Preschool; D015023:Democratic Republic of the Congo / Q000453:epidemiology; D005260:Female; D006801:Humans; D006918:Hydroxyurea / Q000008:administration & dosage*; D007114:Immunization; D008297:Male; D012189:Retrospective Studies
chemical_list: D000986:Antisickling Agents; D006918:Hydroxyurea
keywords: Antibioprophylaxis; Democratic Republic of Congo (DRC); Kinshasa; children; immunization; management; sickle cell disease