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Recommendations for the management of sickle cell disease in South Africa

Nazeer Ahmed Alli, Moosa Patel, Hassan Dawood Alli, Fatima Bassa, Marius J Coetzee, Alan Davidson, Mohammed Rafique Essop, Atul Lakha, Vernon J Louw, Nicolas Novitzky, Vinitha Philip, Janet Elizabeth Poole, Rosalind Wainwright

Abstract


The spectrum of sickle cell disease (SCD) encompasses a heterogeneous group of disorders that include: (i) homozygous SCD (HbSS), also referred to as sickle cell anaemia; (ii) heterozygous SCD (HbAS), also referred to as sickle cell trait; and (iii) compound heterozygous states such as HbSC disease, HbSβ thalassaemia, etc. Homozygous or compound heterozygous SCD patients manifest with clinical disease of varying severity that is influenced by biological and environmental factors, whereas subjects with sickle cell trait are largely asymptomatic. SCD is characterised by vaso-occlusive episodes that result in tissue ischaemia and pain in the affected region. Repeated infarctive episodes cause organ damage and may eventually lead to organ failure. For effective management, regular follow-up with support from a multidisciplinary healthcare team is necessary. The chronic nature of the disease, the steady increase in patient numbers, and relapsing acute episodes have cost implications that are likely to impact on provincial and national health budgets. Limited resources mandate local management protocols for the purposes of consistency and standardisation, which could also facilitate sharing of resources between centres for maximal utility. These recommendations have been developed for the South African setting, and it is intended to update them regularly to meet new demands and challenges.


Authors' affiliations

Nazeer Ahmed Alli, Department of Molecular Medicine and Haematology, National Health Laboratory Service and School of Pathology, University of the Witwatersrand, Johannesburg, South Africa

Moosa Patel, Clinical Haematology Unit, Chris Hani Baragwanath Hospital, Johannesburg, South Africa; Department of Medicine, Faculty of Health Sciences, University of the Witwatersrand, Johannesburg, South Africa

Hassan Dawood Alli, Department of Opthalmology, St John’s Eye Hospital, Johannesburg, South Africa

Fatima Bassa, Division of Clinical Haematology, Department of Medicine, Tygerberg Academic Hospital, Cape Town, South Africa

Marius J Coetzee, Department of Haematology and Cell Biology, National Health Laboratory Service Universitas Tertiary Laboratories, University of the Free State, Bloemfontein, South Africa

Alan Davidson, Department of Paediatric Haematology-Oncology, Red Cross War Memorial Children’s Hospital, Cape Town, and University of Cape Town, South Africa

Mohammed Rafique Essop, Department of Cardiology, Chris Hani Baragwanath Hospital, Johannesburg, South Africa

Atul Lakha, Department of Haematology, Chris Hani Baragwanath Hospital, Johannesburg, South Africa

Vernon J Louw, Department of Internal Medicine, University of the Free State, Bloemfontein, South Africa

Nicolas Novitzky, Division of Haematology, Department of Medicine and Pathology, Faculty of Health Sciences, University of Cape Town, South Africa

Vinitha Philip, Department of Haematology, Chris Hani Baragwanath Hospital, Johannesburg, South Africa

Janet Elizabeth Poole, Department of Paediatric Haematology-Oncology, Charlotte Maxeke Johannesburg Academic Hospital, Johannesburg, South Africa

Rosalind Wainwright, Division of Paediatric Haematology-Oncology, Department of Paediatrics, Chris Hani Baragwanath Hospital, Johannesburg, South Africa

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Keywords

Sickle cell; Management; Prevention

Cite this article

South African Medical Journal 2014;104(11):743-751. DOI:10.7196/SAMJ.8470

Article History

Date submitted: 2014-05-19
Date published: 2014-06-20

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