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Updated: Oct 4, 2025
A Precision Medicine Tool for Measurement and Monitoring of Hemoglobin S in Sickle Cell Disease Patients Receiving Transfusion Therapy
A predictive algorithm for identifying children with sickle cell anemia among children admitted to hospital with
Peter Olupot-Olupot1,2, Roisin Connon3, Sarah Kiguli4
1Busitema University Faculty of Health Sciences, Mbale Regional Referral Hospital, Mbale, Uganda.
Insights
Sickle cell anemia (SCA) is a significant cause of severe anemia in African children. A new algorithm efficiently identifies children with unknown SCA, improving diagnosis and care in regions with limited screening.
Area of Science:
- Hematology
- Pediatrics
- Public Health
Background:
- Sickle cell anemia (SCA) is prevalent in sub-Saharan Africa, affecting about 1% of births.
- Severe anemia is a common reason for pediatric hospital admissions, but SCA's contribution is understudied.
- The Transfusion and Treatment of severe anemia in African Children Trial (ISRCTN84086586) enrolled 3983 children with severe anemia across four African hospitals.
Purpose of the Study:
- To determine the prevalence of SCA in children admitted with severe anemia in Uganda.
- To identify clinical characteristics associated with SCA in this population.
- To develop and validate an efficient algorithm for identifying children with unknown SCA.
Main Methods:
- Genotyping for SCA was performed on all trial participants upon completion.
- Baseline characteristics were analyzed based on SCA status (known, unknown, or non-SCA).
- A diagnostic algorithm using seven admission criteria was developed and tested for identifying unknown SCA cases.
Main Results:
- SCA was identified in 12% (known) and 17% (unknown) of Ugandan participants (n=3483).
- Children with SCA were less likely to have malaria but more likely to present with splenomegaly or a history of blood transfusion.
- The proposed algorithm identified 73% of unknown SCA cases with a low number needed to test (two).
Conclusions:
- SCA is a significant contributor to severe anemia in Ugandan children.
- The developed algorithm provides an efficient and cost-effective method for identifying children with unknown SCA in resource-limited settings.
- Improved identification of SCA can lead to better management and outcomes for affected children.
Abstract:
Sickle cell anemia (SCA) is common in sub-Saharan Africa where approximately 1% of births are affected. Severe anemia is a common cause for hospital admission within the region yet few studies have investigated the contribution made by SCA. The Transfusion and Treatment of severe anemia in African Children Trial (ISRCTN84086586) investigated various treatment strategies in 3983 children admitted with severe anemia (hemoglobin < 6.0 g/dl) based on two severity strata to four hospitals in Africa (three Uganda and one Malawi). Children with known-SCA were excluded from the uncomplicated stratum and capped at 25% in the complicated stratum. All participants were genotyped for SCA at trial completion. SCA was rare in Malawi (six patients overall), so here we focus on the participants recruited in Uganda. We present baseline characteristics by SCA status and propose an algorithm for identifying children with unknown-SCA. Overall, 430 (12%) and 608 (17%) of the 3483 Ugandan participants had known- or unknown-SCA, respectively. Children with SCA were less likely to be malaria-positive and more likely to have an affected sibling, have gross splenomegaly, or to have received a previous blood transfusion. Most outcomes, including mortality and readmission, were better in children with either known or unknown-SCA than non-SCA children. A simple algorithm based on seven admission criteria detected 73% of all children with unknown-SCA with a number needed to test to identify one new SCA case of only two. Our proposed algorithm offers an efficient and cost-effective approach to identifying children with unknown-SCA among all children admitted with severe anemia to African hospitals where screening is not widely available.
