[Formula: see text]Executive functioning and health-related quality of life in pediatric sickle cell disease

Taryn M Allen1, Lindsay M Anderson1, Jennifer A Rothman2

  • 1a Department of Psychology & Neuroscience , Duke University , Durham , NC , USA.

Insights

Children with sickle cell disease (SCD) experiencing poorer executive functioning report lower quality of life. This study highlights the need for interventions targeting cognitive skills in pediatric SCD patients.

Area of Science:

  • Pediatric Hematology
  • Neuropsychology
  • Child Psychology

Background:

  • Children with sickle cell disease (SCD) are at increased risk for neurocognitive deficits.
  • Previous research has not investigated how neurocognitive functioning impacts quality of life in this population.
  • Executive functioning is crucial for daily life activities and overall well-being.

Purpose of the Study:

  • To examine the relationship between executive functioning and quality of life in children with SCD.
  • To explore if psychosocial and family/caregiver resources moderate this relationship.
  • To provide empirical evidence for targeted interventions in pediatric SCD.

Main Methods:

  • A sample of 45 children (aged 8-16) with SCD and their caregivers participated.
  • Participants completed measures of quality of life, executive functioning (behavioral ratings), and psychosocial functioning.
  • Hierarchical linear regression analyses were used to assess relationships and moderation effects.

Main Results:

  • Executive functioning significantly predicted both child- and parent-reported quality of life in children with SCD, even after controlling for age, pain, and SES.
  • Psychosocial resources of the caregiver or family did not moderate the executive functioning-quality of life link.
  • This study provides the first empirical evidence linking executive skills to quality of life in pediatric SCD.

Conclusions:

  • Lower executive functioning is associated with diminished quality of life in children with SCD.
  • Findings support the development of interventions addressing cognitive impairments to improve quality of life for youth with SCD.
  • Further research should explore specific cognitive deficits and their impact on daily functioning and well-being.

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