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Disease-related factors and family functioning as predictors of executive functioning in preschool children with sickle cell anaemia: a preliminary analysis

Disease-related factors and family functioning as predictors of executive functioning in preschool children with sickle cell anaemia: a preliminary analysis
Disease-related factors and family functioning as predictors of executive functioning in preschool children with sickle cell anaemia: a preliminary analysis
Background

School children with sickle cell anemia (SCA) score significantly lower on executive functioning tasks involving attention, inhibition, working memory, and processing speed. There has been limited research on the impact of SCA on the executive development of preschool-age children, although recent intervention studies have shown that executive functions are trainable at this early age. Emerging research suggests that executive functioning deficits are detectable in the preschool years in the correct assessment setting. We hypothesized that, in addition to an effect of family environment, children with more severe disease factors, i.e. low hemoglobin, transcranial doppler (TCD) velocities, and more hospital admissions, would perform poorly on executive functioning tasks.

Method

Ethical approval was obtained from the local National Health Service (NHS) committee. Children with homozygous SCA (HbSS), aged 36-72 months, without co-morbid disorders or history of stroke, were recruited and underwent the Weschler Preschool and Primary Scale of Intelligence (WPPSI), and the National Institutes of Health (NIH) toolbox tasks of executive functioning, including the flanker inhibition control and attention test and the pattern comparison processing speed test. They also completed behavioral tasks including scrambled boxes for working memory and picture deletion for selective attention/inhibition. Parents completed the Children’s Behavior Questionnaire (CBQ) and the Family Environment Scale (FES). A group of typically developing comparison children was also recruited.

Results

Eight children with SCA (4m: 4f; mean age=4.87 years, range=3.58-5.91) and 23 age-matched comparison children participated. In SCA, no associations were found with hemoglobin and performance on neuropsychological tasks or parent reports of behavior. There were trends for associations between TCD velocities and processing speed on the NIH picture comparison task (r=.769, p=.074, N=6),and number of admissions in the past year and performance IQ (r=-.68, p=.06, N=8) and parent-reported levels of attentional focusing (r=-.68, p=.06, N=8). Parent-reported levels of FES family cohesion were related to TCD velocity (r=.92, p=.01, N=6), and NIH toolbox processing speed (r=.86, p=.03, N=6). Greater emphasis on parent-reported FES active–recreational events correlated with FSIQ (r=.78, p=.039, N=7) and working memory (r=.82, p=.025, N=7). No associations or trends were observed between FES family cohesion and processing speed (N=15) or FES active-recreational and FSIQ (N=23)/working memory (N=14) in a comparison group of typically developing children. Inhibition and attention, as measured by the NIH toolbox test and the picture deletion task, were not related to disease-related or family functioning factors in children with SCA but parent reports of activity level and inhibition were related to performance on the picture deletion task. CBQ activity levels and PDTP commissions were also found to be associated (r=.896, p=.006, N=7) as well as CBQ inhibition and PDTP omissions (r= .896, p=.006, N=7).

Conclusion

This preliminary investigation demonstrates that even at this young age, children with SCA can be investigated for deficits in discrete areas of executive functioning. These findings show the potential benefit of neuropsychological assessment in the identification of children who are at a higher risk of future stroke morbidity. This preliminary evidence, particularly the three-way associations between cerebral blood flow velocity, processing speed, and family cohesion, also suggests that early family interventions in young children with SCA could potentially improve school readiness skills and academic performance in the long-term. Executive functioning skills act as a protective factor in academic attainment and may play an important role in combatting the effects of higher rates of school absences that children with SCA often experience.
0006-4971
4941
Downes, Michelle
668e4207-edc2-4834-b0ca-24d5375ef586
de Haan, Michelle
72c96996-93a0-4629-9c61-4efc7c6e697d
Kirkham, Fenella J.
1dfbc0d5-aebe-4439-9fb2-dac6503bcd58
Kaya, Banu
93b818c4-6ab5-4881-9ba7-b875189b2b24
Telfer, Paul
4f57150d-6011-4689-82ab-d32aa65f32c7
Downes, Michelle
668e4207-edc2-4834-b0ca-24d5375ef586
de Haan, Michelle
72c96996-93a0-4629-9c61-4efc7c6e697d
Kirkham, Fenella J.
1dfbc0d5-aebe-4439-9fb2-dac6503bcd58
Kaya, Banu
93b818c4-6ab5-4881-9ba7-b875189b2b24
Telfer, Paul
4f57150d-6011-4689-82ab-d32aa65f32c7

Downes, Michelle, de Haan, Michelle, Kirkham, Fenella J., Kaya, Banu and Telfer, Paul (2014) Disease-related factors and family functioning as predictors of executive functioning in preschool children with sickle cell anaemia: a preliminary analysis. Blood, 124 (21), 4941.

Record type: Article

Abstract

Background

School children with sickle cell anemia (SCA) score significantly lower on executive functioning tasks involving attention, inhibition, working memory, and processing speed. There has been limited research on the impact of SCA on the executive development of preschool-age children, although recent intervention studies have shown that executive functions are trainable at this early age. Emerging research suggests that executive functioning deficits are detectable in the preschool years in the correct assessment setting. We hypothesized that, in addition to an effect of family environment, children with more severe disease factors, i.e. low hemoglobin, transcranial doppler (TCD) velocities, and more hospital admissions, would perform poorly on executive functioning tasks.

Method

Ethical approval was obtained from the local National Health Service (NHS) committee. Children with homozygous SCA (HbSS), aged 36-72 months, without co-morbid disorders or history of stroke, were recruited and underwent the Weschler Preschool and Primary Scale of Intelligence (WPPSI), and the National Institutes of Health (NIH) toolbox tasks of executive functioning, including the flanker inhibition control and attention test and the pattern comparison processing speed test. They also completed behavioral tasks including scrambled boxes for working memory and picture deletion for selective attention/inhibition. Parents completed the Children’s Behavior Questionnaire (CBQ) and the Family Environment Scale (FES). A group of typically developing comparison children was also recruited.

Results

Eight children with SCA (4m: 4f; mean age=4.87 years, range=3.58-5.91) and 23 age-matched comparison children participated. In SCA, no associations were found with hemoglobin and performance on neuropsychological tasks or parent reports of behavior. There were trends for associations between TCD velocities and processing speed on the NIH picture comparison task (r=.769, p=.074, N=6),and number of admissions in the past year and performance IQ (r=-.68, p=.06, N=8) and parent-reported levels of attentional focusing (r=-.68, p=.06, N=8). Parent-reported levels of FES family cohesion were related to TCD velocity (r=.92, p=.01, N=6), and NIH toolbox processing speed (r=.86, p=.03, N=6). Greater emphasis on parent-reported FES active–recreational events correlated with FSIQ (r=.78, p=.039, N=7) and working memory (r=.82, p=.025, N=7). No associations or trends were observed between FES family cohesion and processing speed (N=15) or FES active-recreational and FSIQ (N=23)/working memory (N=14) in a comparison group of typically developing children. Inhibition and attention, as measured by the NIH toolbox test and the picture deletion task, were not related to disease-related or family functioning factors in children with SCA but parent reports of activity level and inhibition were related to performance on the picture deletion task. CBQ activity levels and PDTP commissions were also found to be associated (r=.896, p=.006, N=7) as well as CBQ inhibition and PDTP omissions (r= .896, p=.006, N=7).

Conclusion

This preliminary investigation demonstrates that even at this young age, children with SCA can be investigated for deficits in discrete areas of executive functioning. These findings show the potential benefit of neuropsychological assessment in the identification of children who are at a higher risk of future stroke morbidity. This preliminary evidence, particularly the three-way associations between cerebral blood flow velocity, processing speed, and family cohesion, also suggests that early family interventions in young children with SCA could potentially improve school readiness skills and academic performance in the long-term. Executive functioning skills act as a protective factor in academic attainment and may play an important role in combatting the effects of higher rates of school absences that children with SCA often experience.

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More information

Published date: 6 December 2014

Identifiers

Local EPrints ID: 424294
URI: http://eprints.soton.ac.uk/id/eprint/424294
ISSN: 0006-4971
PURE UUID: e111e504-dc52-4fde-81d3-91b62e4c08e0
ORCID for Fenella J. Kirkham: ORCID iD orcid.org/0000-0002-2443-7958

Catalogue record

Date deposited: 05 Oct 2018 11:35
Last modified: 12 Dec 2021 03:16

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Contributors

Author: Michelle Downes
Author: Michelle de Haan
Author: Banu Kaya
Author: Paul Telfer

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