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Developmental function in toddlers with sickle cell anemia

F Daniel Armstrong1, T David Elkin, R Clark Brown

  • 1Department of Pediatrics (D-820), PO Box 016820, Miami, FL 33101, USA. darmstrong@miami.edu

Pediatrics
|January 9, 2013
PubMed

Insights

In infants with sickle cell disease, neurodevelopment is generally average, but behavioral and adaptive functions decline with age. Further research is needed to understand these developmental changes.

Area of Science:

  • Pediatric Neurology
  • Hematology
  • Developmental Psychology

Background:

  • Neurocognitive impairment is recognized in children and adults with sickle cell anemia.
  • Limited data exists on neurodevelopmental outcomes in very young children with sickle cell disease.

Purpose of the Study:

  • To assess the neurodevelopmental status of infants in the Pediatric Hydroxyurea Phase III Clinical Trial (Baby Hug).
  • To investigate the relationships between neurodevelopment, age, cerebral blood flow velocity, and hemoglobin concentration in infants with sickle cell disease.

Main Methods:

  • 193 infants (hemoglobin SS or S-β(0) thalassemia) aged 7–18 months underwent standardized neurodevelopmental assessments.
  • Evaluated associations between neurodevelopmental scores and age, income, education, hemoglobin levels, and transcranial Doppler velocity.

Main Results:

  • Mean neurodevelopmental functioning was within the average range.
  • No infants had impaired mental development scores (<70); however, 22 had clinically significant scores (11 psychomotor, 11 behavioral).
  • Poorer performance correlated with older age at baseline; behavior ratings decreased by 2.82 percentile points per month of age. Parent-reported functional abilities and hemoglobin levels showed a negative association with higher transcranial Doppler velocities.

Conclusions:

  • Overall neurodevelopmental functioning in infants with sickle cell disease is within the normal range.
  • Behavioral and adaptive functioning showed a decline with increasing age, even in this young cohort.
  • Further research is required to elucidate the mechanisms underlying the association between poorer developmental function and older age in this population.
Abstract

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