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Updated: Mar 17, 2026

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[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.
Abstract:
Research consistently indicates that children with sickle cell disease (SCD) face multiple risk factors for neurocognitive impairment. Despite this, no empirical research to date has examined the impact of neurocognitive functioning on quality of life for this pediatric group. Thus, the current study aims to examine the relationship between executive functioning and quality of life in a sample of children with SCD and further explore psychosocial and family/caregiver resources as moderators of this relationship. A total of 45 children with SCD aged 8 to 16 years and their caregivers completed measures of quality of life, behavioral ratings of executive functioning, and psychosocial functioning. Hierarchical linear regression models were utilized to determine the impact of executive functioning on quality of life and further test the interaction effects of proposed moderating variables. Controlling for age, pain, and socioeconomic status (SES), executive functioning was found to significantly predict child- and parent-reported quality of life among youth with SCD. Psychosocial resources of the primary caregiver or family was not found to moderate the relationship between executive functioning and quality of life. These results provide the first empirical evidence that lower executive skills negatively predict quality of life for children with SCD, supporting clinical and research efforts which aim to establish efficacious interventions that target cognitive decrements within this pediatric population.
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