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The Contemporary Adolescent and Young Adult Sickle Cell Population in Malawi
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  • Luke Eastburg,
  • Kambe Banda-Zgamba,
  • Timothy Mutafya,
  • Apatsa Matatiyo,
  • Rizine Mzikamanda,
  • Katherine Westmoreland,
  • Jane Little,
  • Nmazuo Ozuah
Luke Eastburg
The University of North Carolina at Chapel Hill Division of Hematology

Corresponding Author:luke_eastburg@urmc.rochester.edu

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Kambe Banda-Zgamba
UNC Project-Malawi
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Timothy Mutafya
Baylor College of Medicine Department of Pediatrics
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Apatsa Matatiyo
UNC Project-Malawi
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Rizine Mzikamanda
Baylor College of Medicine Department of Pediatrics
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Katherine Westmoreland
The University of North Carolina at Chapel Hill Division of Hematology
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Jane Little
The University of North Carolina at Chapel Hill Division of Hematology
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Nmazuo Ozuah
Baylor College of Medicine Department of Pediatrics
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Abstract

Background: Sickle cell disease (SCD) is one of the most common inherited red blood cell disorders in the world, and the vast majority of infants born with SCD are from Sub-Saharan Africa (SSA). Recent studies have definitively established that hydroxyurea is safe and highly effective for children with SCD in SSA. However, there is a lack of data on the long-term outcomes for children with SCD in SSA beyond early childhood. Procedure: We conducted a retrospective analysis of AYA SCD patients ≥16 yo who received care in a pediatric SCD clinic in Malawi between January 2015 and December 31, 2022 Results: We identified 207 patients ≥16 yo in the SCD clinic electronic database. The vast majority (195, 94%) were alive and receiving care in the pediatric SCD clinic. Three patients had died, and 9 were lost to follow-up. The mean age of patients alive and receiving care was 18±1.9 yo. The mean age at diagnosis was 5.7±1.1 yo. Nearly all AYA patients (N=187, 96%) were taking hydroxyurea for a mean duration of 6±2.7 years. Mean baseline laboratory values were: hemoglobin 8.0±1.3 g/dL, mean corpuscular volume 92±8.4 fl, white blood count 14.2±7.0 × 10 3 /µL, absolute neutrophil count (ANC) 6.2±4.8 × 10 3 /µL, and platelets 343±167 × 10 3 /µL. Among all patients, 21% had at least one transfusion, and 23% of patients had been hospitalized at least once for sickle cell-related complications. Conclusion: We provide the first description of an AYA cohort with SCD receiving routine care with hydroxyurea in a dedicated pediatric clinic under real-world conditions. This study highlights hydroxyurea’s long-term feasibility and underscores the need to enhance the capacity for comprehensive adult care, especially in SSA’s rapidly expanding AYA population.