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Updated: Apr 14, 2026
A Precision Medicine Tool for Measurement and Monitoring of Hemoglobin S in Sickle Cell Disease Patients Receiving Transfusion Therapy
Recommendations for the management of sickle cell disease in South Africa
Insights
Sickle cell disease (SCD) presents a spectrum of disorders, from severe sickle cell anaemia (HbSS) to largely asymptomatic sickle cell trait (HbAS). Effective management requires multidisciplinary care and standardized protocols, especially in resource-limited settings like South Africa.
Area of Science:
- Hematology
- Genetics
- Public Health
Background:
- Sickle cell disease (SCD) is a heterogeneous group of disorders including homozygous SCD (HbSS), heterozygous SCD (HbAS), and compound heterozygous states.
- While HbSS patients experience varying severity and vaso-occlusive episodes leading to organ damage, HbAS individuals are generally asymptomatic.
- The chronic and increasing prevalence of SCD poses significant financial burdens on healthcare systems.
Purpose of the Study:
- To provide standardized, locally relevant management protocols for sickle cell disease in South Africa.
- To ensure consistency and facilitate resource sharing among healthcare centers.
- To address the growing demands and challenges of managing SCD.
Main Methods:
- Development of recommendations based on the South African context.
- Emphasis on regular follow-up and multidisciplinary healthcare team support.
- Intention for regular updates to meet evolving demands.
Main Results:
- Established a framework for consistent and standardized SCD management.
- Highlighted the necessity of local protocols for resource optimization.
- Acknowledged the impact of SCD on provincial and national health budgets.
Conclusions:
- Standardized, localized management protocols are crucial for effective sickle cell disease care.
- Multidisciplinary support and regular follow-up are essential for patients with SCD.
- The development of adaptable protocols is vital for addressing the long-term challenges of SCD in resource-limited settings.
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 subject with sickle cell trait are largely asymptomatic. SCD is characterized 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.
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