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Hydroxyurea use in Children with Sickle Cell Disease: Do Severely Affected Patients Use It and Does It Impact

Susan E Creary1, Deena J Chisolm1, Terah L Koch1

  • 1Division of Pediatric Hematology and Oncology, Nationwide Children's Hospital, The Ohio State University, Columbus, Ohio.

Pediatric Blood & Cancer
|January 23, 2016
PubMed

Insights

Hydroxyurea (HU) use is high in hospitalized children with sickle cell disease (SCD), but underutilized in those with severe SCD. HU may prevent hospitalizations rather than improve outcomes during them.

Area of Science:

  • Pediatric Hematology
  • Sickle Cell Disease Management
  • Pharmacotherapy Outcomes

Background:

  • Expert guidelines advocate for hydroxyurea (HU) in children with hemoglobin SS and Sβ(0) sickle cell disease (SCD).
  • HU is also recommended for consideration in children with clinically severe hemoglobin SC or Sβ(+) SCD.
  • This study evaluates HU utilization patterns and their impact on hospitalization outcomes in pediatric SCD patients.

Purpose of the Study:

  • To determine the rate of hydroxyurea (HU) use in hospitalized children with sickle cell disease (SCD).
  • To assess if HU is differentially prescribed for children with clinically severe SCD.
  • To compare hospitalization outcomes (length of stay, ICU admissions, transfusions) between HU users and nonusers.

Main Methods:

  • Retrospective analysis of 2,665 children (ages 2-18) with SCD using the Pediatric Health Information System (2011-2014).
  • Clinically severe SCD defined by recent ICU admission or ≥3 prior admissions.
  • Propensity score weighting used to adjust for confounding factors.

Main Results:

  • Approximately 80% of identified children with SCD received HU during hospitalization.
  • Nonusers had significantly higher rates of recent ICU admission (30.1%) and prior admissions (33.9%) compared to HU users (p < 0.001).
  • After propensity score weighting, no significant differences were found in length of stay, ICU admissions, or transfusion rates between HU users and nonusers.

Conclusions:

  • Hydroxyurea (HU) use is prevalent in hospitalized pediatric sickle cell disease (SCD) patients.
  • A notable proportion of children with clinically severe SCD do not receive HU.
  • Findings suggest HU should be prioritized for preventing hospitalizations in children with SCD, not solely for managing acute events.
Abstract

Keywords:
Hematologyhydroxyureanonmalignantsickle cell disease

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