Sociodemographic and Biomedical Correlates of Developmental Delay in 2- and 4-Year-Olds with Sickle Cell Disease

Schatz, Jeffrey; Reinman, Laura; Bills, Sarah E.; Johnston, Julia

doi:10.1097/dbp.0000000000001011

Cited by 6 publications

(12 citation statements)

References 24 publications

(38 reference statements)

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“…4 Consistent with pre-vious cross-sectional data, cerebrovascular complications were found to be an important factor influencing developmental delays. 4,8 The present longitudinal data support a more specific temporal association between new cerebrovascular morbidity and the emergence of developmental delay.…”

Section: Discussionsupporting

confidence: 77%

“…The cross-sectional results of this sample are reported elsewhere. 8 Among these children, 43 children completed both screenings. Participation among repeaters versus nonrepeaters was unrelated to gender, sickle cell genotype severity, or screening outcomes at age two years.…”

Section: Methods and Resultsmentioning

confidence: 99%

“…Participation among repeaters versus nonrepeaters was unrelated to gender, sickle cell genotype severity, or screening outcomes at age two years. 8 Caregivers completed the Ages and Stages Questionnaire, 2nd edition, which assesses the development of youth aged 4 to 60 months by examining expected developmental milestones for the child's age in domains of communication, gross motor, fine motor, problem-solving, and personal-social skills (6 milestones/domain). 9 The ASQ has demonstrated strong psychometric properties in large, diverse samples within the United States and in low/middle-income countries internationally.…”

Section: Methods and Resultsmentioning

confidence: 99%

“…4,7 Limitations of this study are that few risk and protective factors were examined to understand the reasons for change, and the "at-risk" outcome on the ASQ-3 was treated as evidence of developmental concerns, which is inconsistent with most prior studies of SCD. [2][3][7][8] In the present study, we examined the temporal stability of developmental screening outcomes in children with SCD screened at ages two and four years and the influence of interim risk/protective factors on status change. We determined the impact of variables associated with increased or decreased risk for developmental delay from cross-sectional studies to examine their longitudinal impact on screening outcomes.…”

mentioning

confidence: 99%

“…A new onset of cerebrovascular morbidity and severe genotypes were hypothesized to influence the onset of developmental delays between ASQ screenings. 4,7,8 Attending preschool and initiation of formal developmental intervention services after an initial positive screening were hypothesized to predict the shift from positive to negative screening results. 3,4,8 Variables that have been posited to impact early childhood development in SCD (e.g., Hb, HbF, hydroxyurea use), but which have not demonstrated associations in prior early childhood studies, were not examined due to limited statistical power.…”

mentioning

confidence: 99%

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Changes in the developmental status of preschoolers with sickle cell disease

Johnston

Schatz

Bills

2022

Pediatric Blood & Cancer

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Developmental monitoring and screening are recommended strategies for identifying children with sickle cell disease at high risk for cerebrovascular complications. Studies examining developmental screenings have provided little data on change over time. We examined screenings longitudinally in 43 children screened as two‐year‐olds and four‐year‐olds using the Ages and Stages Questionnaire, 2nd edition. Only two‐thirds of children had stable screening outcomes. A new onset of cerebrovascular complications predicted the emergence of developmental delay (P = 0.017). Multivariate analysis suggested a benefit from formal developmental interventions. Regular developmental screening during the preschool period is important to identify systematic changes in developmental status.

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Section: Discussionsupporting

confidence: 77%

Section: Methods and Resultsmentioning

confidence: 99%

Section: Methods and Resultsmentioning

confidence: 99%

mentioning

confidence: 99%

mentioning

confidence: 99%

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Changes in the developmental status of preschoolers with sickle cell disease

Johnston

Schatz

Bills

2022

Pediatric Blood & Cancer

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Neurocognitive functioning in preschool children with sickle cell disease

Heitzer

Cohen

Okhomina

et al. 2021

Pediatric Blood & Cancer

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Background Children with sickle cell disease (SCD) experience neurodevelopmental delays; however, there is limited research with preschool‐age children. This study examined neurocognitive risk and protective factors in preschoolers with SCD. Procedure Sixty‐two patients with SCD (60% HbSS/HbSβ0‐thalassemia; 40% HbSC/HbSβ+‐thalassemia) between the ages of 3 and 6 years (mean = 4.77 years) received a neuropsychological evaluation as routine systematic surveillance. Patients were not selected for disease severity, prior central nervous system findings, or existing cognitive concerns. Thirty‐four patients (82% HbSS/HbSβ0‐thalassemia) were prescribed hydroxyurea (HU) at the time of their neuropsychological evaluation. On average, these patients had been prescribed HU at 2.15 (standard deviation = 1.45) years of age. The average dose was 28.8 mg/kg/day. Besides genotype, there were no group differences in medical or demographic factors based on HU treatment status. Results Patients with HbSS/HbSβ0‐thalassemia scored below normative expectations on measures of intelligence, verbal comprehension, and school readiness (false discovery rate‐adjusted p‐value [pFDR] < .05). Age, sickle genotype, and HU treatment exposure were not associated with measured neurocognitive outcomes (pFDR > .05). Greater social vulnerability at the community level was associated with poorer performance on measures of intellectual functioning, verbal comprehension, visuomotor control, and school readiness, as well as parent report of executive dysfunction (pFDR < .05). Greater household socioeconomic status was positively associated with academic readiness. Conclusions Preschoolers with severe SCD (HbSS/HbSβ0‐thalassemia) perform below age expectations on measures of intelligence and academic readiness. Sociodemographic factors were stronger drivers of neurocognitive performance than disease severity or disease‐modifying treatment. Neurodevelopmental interventions targeting the home and broader community environment are needed.

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Implementation of two developmental screening programs in sickle cell disease specialty care.

Schlenz¹,

Schatz²

2023

Clinical Practice in Pediatric Psychology

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Objective: Developmental screening is a critical component of care for children with sickle cell disease (SCD), who are at elevated risk for neurodevelopmental disorders. This report describes the implementation of two related developmental screening programs implemented in different SCD specialty care settings with the purpose of describing screening protocols, outcomes, and lessons learned. Methods: Program 1 reviewed medical records for 201 children with SCD screened at ages 2 and 4 years. Program 2 reviewed program tracking and visit notes for 155 screenings across 67 children screened between 9 and 66 months of age. Key outcomes included characteristics of children screened, screening results, concordance between parent concerns and screening outcomes, and access to evaluation and intervention services. Results: Each program identified a substantial number of children with developmental concerns, including 42% of screenings in Program 1 and 36% of unique children screened in Program 2. Program 1 resulted in 56% of identified children receiving follow-up developmental services and 62% receiving developmental monitoring. Program 2 resulted in 58% of identified children receiving further evaluation following developmental screening, with 67-75% of children with neurodevelopmental diagnoses receiving intervention services following evaluation. While parent concerns were related to screening outcomes, screening instruments detected many children whose parents did not express developmental concerns. Conclusions: Routine developmental screening is a feasible, acceptable, and effective method for identifying concerns in children with SCD in specialty care. Flexible and collaborative care and sustainability are key considerations for effective programming, with pediatric psychologists uniquely positioned to provide optimal integrated care. Implications for Impact StatementChildren with sickle cell disease are at high risk for neurodevelopmental disorders and benefit from early, high-quality identification and support for these concerns. Routine developmental screening delivered by a psychologist in specialty sickle cell disease care is a feasible, acceptable, and sustainable approach for meeting these important needs.

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Sociodemographic and Biomedical Correlates of Developmental Delay in 2- and 4-Year-Olds with Sickle Cell Disease

Cited by 6 publications

References 24 publications

Changes in the developmental status of preschoolers with sickle cell disease

Changes in the developmental status of preschoolers with sickle cell disease

Neurocognitive functioning in preschool children with sickle cell disease

Implementation of two developmental screening programs in sickle cell disease specialty care.

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