title: The burden of sickle cell disease in Cape Town.
abstract: BACKGROUND
  South Africa has a low incidence of sickle cell disease (SCD). However, its demographics are changing because of immigration from sub-Saharan African countries where SCD is prevalent.
  
  
  OBJECTIVE
  We aimed to determine the frequency of SCD presenting to the Haematology/Oncology Service at Red Cross War Memorial Children's Hospital in Cape Town and to measure the associated disease burden.
  
  
  METHODS
  This was a retrospective cross-sectional study of patients first attending the Haematology Service between January 2001 and June 2010.
  
  
  RESULTS
  A total of 58 SCD patients were identified, with an annual frequency that increased over the study period by 300 - 400%. Up to 93.1% (n=54) were originally from other African countries, mainly the Democratic Republic of Congo (62.1%, n=36). One patient had sickle D-Punjab genotype, and all the other patients had the homozygous sickle cell anaemia genotype (Hb SS). Their haematological parameters demonstrated a normocytic anaemia with high white cell counts. The mean number of clinic visits per patient per year was 22.2 (range 0 - 64), and the mean number of hospital admissions per patient per year was 1.2 (range 0 - 5). All the patients were on antibiotic prophylaxis. The majority had at least one blood transfusion (65.5%, n=38), and a significant proportion required intravenous analgesia on admission (29.3%, n=17) and hydroxyurea treatment (36.2%, n=21).
  
  
  CONCLUSIONS
  Over the past 10 years the frequency of SCD has increased considerably, imposing a significant burden and new challenges to the health services in Cape Town.
authors.affiliation: Department of Clinical Laboratory Sciences, University of Cape Town, Cape Town, South Africa. ambroise.wonkam@uct.ac.za
authors.forename: Ambroise
authors.initials: A
authors.lastname: Wonkam
authors.forename: Chido
authors.initials: C
authors.lastname: Ponde
authors.forename: Nan
authors.initials: N
authors.lastname: Nicholson
authors.forename: Karen
authors.initials: K
authors.lastname: Fieggen
authors.forename: Raj
authors.initials: R
authors.lastname: Ramessar
authors.forename: Alan
authors.initials: A
authors.lastname: Davidson
mesh_terms: D000293:Adolescent; D000328:Adult; D000755:Anemia, Sickle Cell / Q000175:diagnosis / Q000453:epidemiology* / Q000628:therapy; D002648:Child; D002675:Child, Preschool; D017281:Cost of Illness; D003430:Cross-Sectional Studies; D005260:Female; D006801:Humans; D007223:Infant; D008297:Male; D008875:Middle Aged; D015995:Prevalence; D012189:Retrospective Studies; D012959:Socioeconomic Factors; D013019:South Africa / Q000453:epidemiology; D055815:Young Adult