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Survival of children with sickle cell disease

Charles T Quinn1, Zora R Rogers, George R Buchanan

  • 1University of Texas Southwestern Medical Center, 5323 Harry Hines Blvd, Dallas, TX 75390, USA. charles.quinn@utsouthwestern.edu

Blood
|February 7, 2004
PubMed

Insights

Contemporary treatments have improved survival for children with sickle cell disease (SCD). This study tracked newborns with SCD for 18 years, showing decreased mortality and increased stroke-free survival rates.

Area of Science:

  • Pediatrics
  • Hematology
  • Public Health

Background:

  • Contemporary survival data for pediatric sickle cell disease (SCD) are lacking.
  • Previous studies do not reflect modern therapeutic advancements.
  • Newborn screening enables early identification and cohort definition.

Purpose of the Study:

  • To determine contemporary survival and stroke incidence in children with SCD.
  • To assess the impact of modern therapies on pediatric SCD outcomes.
  • To analyze survival rates (overall, SCD-related, stroke-free) up to 18 years of age.

Main Methods:

  • Defined an inception cohort of newborns with SCD (SS, Sβ°, SC, Sβ⁺) identified via newborn screening.
  • Followed 711 subjects for up to 18 years, accumulating 5648 patient-years of observation.
  • Calculated incidence of death and stroke; determined overall, SCD-related, and stroke-free survival.

Main Results:

  • Twenty-five deaths occurred (mean age 5.6 years); five were infection-related.
  • Thirty subjects experienced at least one stroke.
  • For SS and Sβ° subjects (n=448), death and stroke rates were 0.59 and 0.85/100 patient-years, respectively.
  • Predicted 18-year cumulative survival: 85.6% overall, 93.6% SCD-related, 88.5% stroke-free.
  • No SCD-related deaths or strokes in SC or Sβ⁺ subjects (n=263).

Conclusions:

  • Childhood mortality from SCD is decreasing.
  • The mean age of death in children with SCD is increasing.
  • Infection accounts for a smaller proportion of SCD-related deaths.
  • Modern therapies significantly improve survival and reduce stroke incidence in pediatric SCD.

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