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Updated: Nov 19, 2025
A Precision Medicine Tool for Measurement and Monitoring of Hemoglobin S in Sickle Cell Disease Patients Receiving Transfusion Therapy
Transcranial Doppler screening in Nigerian children with sickle cell disease: A 10-year longitudinal study on the
IkeOluwa A Lagunju1,2, Adeyemi Labaeka1,2, Joy N Ibeh1,2
1Department of Paediatrics, College of Medicine, University of Ibadan, Ibadan, Nigeria.
Insights
Primary stroke prevention in children with sickle cell disease (SCD) is feasible. Hydroxyurea (HU) therapy, often at higher doses, significantly reduced stroke risk indicators in Nigerian children with SCD.
Area of Science:
- Pediatric Neurology
- Hematology
- Public Health Interventions
Background:
- Stroke prevention programs for children with sickle cell disease (SCD) are effective in resource-limited settings.
- Hydroxyurea (HU) is used to manage SCD severity.
Purpose of the Study:
- To evaluate the long-term outcomes of the Stroke Prevention Programme for children with SCD in Ibadan (SPPIBA), Nigeria.
- To assess the efficacy of hydroxyurea (HU) in reducing stroke risk in children with SCD.
Main Methods:
- A longitudinal study followed 396 children with sickle cell disease (SCD) for at least 5 years.
- Transcranial Doppler (TCD) assessments were conducted regularly.
- Children with elevated TCD velocities (≥170 cm/s) received dose-escalation hydroxyurea (HU) therapy.
Main Results:
- Follow-up ranged from 5 to 10 years (mean 7.2 years).
- Hydroxyurea (HU) dose escalation beyond 20 mg/kg/day was needed in 69.1% of cases to significantly reduce time-averaged mean of maximal velocities (TAMMV).
- Stroke incidence was low at 0.08 per 100 patient-years, with two stroke events observed.
Conclusions:
- Most Nigerian children with SCD and elevated TCD velocities showed significant TAMMV reduction within the first year of HU therapy, often requiring higher doses.
- Individualizing hydroxyurea (HU) dosage may be crucial for optimizing primary stroke prevention outcomes in children with SCD.
Background:
Primary stroke prevention programmes for children with sickle cell disease (SCD) have been shown to be feasible interventions in resource-poor countries. Different hydroxyurea (HU) regimens have been utilised in ameliorating the severity of SCD.
Objective:
To determine the long-term outcomes of the stroke prevention programme for children with SCD in Ibadan (SPPIBA), Nigeria.
Methods:
A longitudinal study of 396 children with haemoglobin SS disease who had been on the stroke prevention programme for a minimum period of 5 years. All enrollees had nonimaging TCD performed at baseline and thereafter 3-monthly or annually. Children with TCD velocities ≥170 cm/s were treated with HU by dose-escalation regimen.
Results:
The mean age at first TCD examination was 102 ± 46.7 months and the period of follow-up ranged from 5 to 10 years (mean = 7.2 ± 1.7). Time to significant decline in TCD velocities ranged from 5 to 35 months, (median = 10.0 months). The minimum dose of HU required to achieve significant decline in TCD velocities ranged from 15 to 31 mg/kg/day, mean 23.7 (±3.9). HU dose escalation beyond 20 mg/kg/day was required to attain significant reductions in the time-averaged mean of maximal velocities (TAMMV) in 69.1% of the cases. Two stroke events occurred giving a stroke incidence of 0.08 per 100 patient-years.
Conclusion:
The majority of Nigerian children with SCD and elevated TCD velocities achieved significant decline in TAMMV within the first year of HU therapy but on higher doses of HU. It might be important to individualise HU doses for optimal outcomes in primary stroke prevention.
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