Research

The burden of sickle cell disease in Cape Town

Ambroise Wonkam, Chido Ponde, Nan Nicholson, Karen Fieggen, Raj Ramessar, Alan Davidson

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.

Objectives. 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 indentified, 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' affiliations

Ambroise Wonkam, Division of Human Genetics, Department of Clinical Laboratory Sciences, Faculty of Health Sciences, University of Cape Town

Chido Ponde, Faculty of Health Sciences, University of Cape Town

Nan Nicholson, Haematology/Oncology Service, Red Cross War Memorial Children’s Hospital, and Department of Paediatrics and Child Health, University of Cape Town

Karen Fieggen, Division of Human Genetics, Department of Clinical Laboratory Sciences, Faculty of Health Sciences, University of Cape Town

Raj Ramessar, Division of Human Genetics, Department of Clinical Laboratory Sciences, Faculty of Health Sciences, University of Cape Town

Alan Davidson, Haematology/Oncology Service, Red Cross War Memorial Children’s Hospital, and Department of Paediatrics and Child Health, University of Cape Town

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Keywords

Sickle cell disease, Migration, Cape Town, sub-Saharan Africa, South Africa

Cite this article

South African Medical Journal 2012;102(9):752-754.

Article History

Date submitted: 2012-03-27
Date published: 2012-06-28

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