Developmental delay in infants and toddlers with sickle cell disease: a systematic review

Hoyt, Catherine R.; Varughese, Taniya; Erickson, Jeni; Haffner, Natalie; Luo, Lingzi; L’Hotta, Allison J.; Yeager, Lauren H.; King, Allison A.

doi:10.1111/dmcn.15048

Cited by 9 publications

(8 citation statements)

References 55 publications

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“…Various socioeconomic factors (e.g., income-to-needs and maternal education) appear to be predictors of a child’s performance on EF tasks over time ( 21 ). Research has also shown that compromised health, such as is experienced in an inherited disease like SCD, might play an important role in the development of cognitive difficulties ( 22 ). Critically, children with SCD show early signs of reduced performance on measures of EF as well as general IQ ( 23 , 24 ).…”

Section: Introductionmentioning

confidence: 99%

Mind the gap: trajectory of cognitive development in young individuals with sickle cell disease: a cross-sectional study

et al. 2023

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Study objectivesCompared to typically developing children and young adults (CYA-TD), those living with Sickle Cell Disease (CYA-SCD) experience more cognitive difficulties, particularly with executive function. Few studies have examined the relative importance of silent cerebral infarction (SCI), haemoglobin and arterial oxygen content on age-related cognitive changes using cross-sectional or longitudinal (developmental trajectory) data. This study presents cohort data from a single timepoint to inform studies with multiple timepoints.MethodsWe compared cross-sectional raw and scaled scores as age-related changes in cognition (trajectories) in CYA-SCD and age-and ethnicity-matched CYA-TD. We also compared cross-sectional age-related changes in cognition (trajectories) in CYA-SCD with and without SCI to CYA-TD. General cognitive abilities were assessed using Wechsler Intelligence Scales, including the Verbal Comprehension Index (VCI) and Perceptual Reasoning Index (PRI) underpinning IQ. Executive function was evaluated using the Delis-Kaplan Executive Function System (D-KEFS) Tower subtest and the Behaviour Rating Inventory of Executive Function (BRIEF) questionnaire. SCI were identified from contemporaneous 3 T MRI; participants with overt stroke were excluded. Recent haemoglobin was available and oxygen saturation (SpO2) was measured on the day of the MRI.ResultsData were available for 120 CYA-SCD [62 male; age = 16.78 ± 4.79 years; 42 (35%) with SCI] and 53 CYA-TD (23 male; age = 17.36 ± 5.16). Compared with CYA-TD, CYA-SCD experienced a delayed onset in VCI and slower rate of development for BRIEF Global Executive Composite, Metacognition Index (MI), and Behaviour Regulation Index. The rate of executive function development for the BRIEF MI differed significantly between CYA-TD and CYA-SCD, with those with SCI showing a 26% delay compared with CYA-TD. For CYA-SCD with SCI, arterial oxygen content explained 22% of the variance in VCI and 37% in PRI, while haemoglobin explained 29% of the variance in PRI.ConclusionAge-related cognitive trajectories of CYA-SCD may not be impaired but may progress more slowly. Longitudinal studies are required, using tests unaffected by practice. In addition to initiation of medical treatment, including measures to improve arterial oxygen content, early cognitive intervention, educational support, and delivery of extracurricular activities could support cognitive development for CYA-SCD.

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Section: Introductionmentioning

confidence: 99%

Mind the gap: trajectory of cognitive development in young individuals with sickle cell disease: a cross-sectional study

et al. 2023

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“…Slowed processing speed, attention difficulties, and executive dysfunction are often identified as salient neurocognitive symptoms beginning in the preschool period, often continuing into adulthood (7). Neurodevelopmental syndromes have also been linked to disease severity in SCD with as many as half of all preschool-age children showing evidence of developmental delay with elevated rates of neurodevelopmental issues persisting into adolescence (8)(9)(10)(11).…”

Section: Introductionmentioning

confidence: 99%

Sluggish Cognitive Tempo in Pediatric Sickle Cell Disease

et al. 2022

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BackgroundSickle cell disease (SCD) imparts risk for a range of neurodevelopmental and neurocognitive disorders. Sluggish cognitive tempo (SCT) is a distinct syndrome that often co-occurs with attention-deficit/hyperactivity disorder (ADHD) but has not been described in SCD. We investigated the reliability and validity of a SCT measure in SCD and examined associations with biopsychosocial risk factors and functional outcomes.Materials and MethodsCaregivers (n = 85) of children with SCD ages 7-16 reported on socio-demographics and the Kiddie-Sluggish Cognitive Tempo (K-SCT) measure, Behavior Rating Inventory of Executive Function, and Conners 3. Disease-related characteristics were extracted from health records.ResultsThe K-SCT demonstrated excellent internal consistency (α = 0.92) and test-retest reliability (r = 0.82, p < 0.001). K-SCT scores were correlated with ADHD-Inattention (r = 0.64, p < 0.001) and ADHD-Hyperactive/Impulsive (r = 0.46, p < 0.001) scores, as well as functional outcomes, including learning problems (r = 0.69, p < 0.001). In multivariate analyses controlling for ADHD symptoms, SCT accounted for unique variance in learning (b = 9.67, p < 0.01) and executive functioning (b = 5.93, p < 0.01). Nearly all participants (93%) with elevated levels of co-occurring SCT and ADHD-Inattention symptoms had significant learning problems.ConclusionThe K-SCT is a reliable and valid measure of SCT in SCD. SCT symptoms are associated with learning difficulties even after controlling for ADHD symptoms. Further research is needed to understand the biopsychosocial factors that lead to SCT symptoms in SCD and examine long-term implications of SCT.

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“…Among the myriad complications that may arise, children with SCD are at high risk for neurodevelopmental and behavioral disorders. Development delays for children with SCD often emerge early in life (as early as the first year of life) and worsen with increased age (Hoyt et al, 2021;Knight et al, 2021). Recent meta-analyses suggest that cerebrovascular complications, including elevated transcranial Doppler (TCD) velocities and overt and silent ischemic stroke, and chronic anemia are disease-specific risk factors most consistently associated with poor outcomes (Prussien et al, 2019(Prussien et al, , 2020.…”

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confidence: 99%

Practice patterns for addressing developmental-behavioral concerns in sickle cell specialty care.

Schlenz¹,

Phillips²,

Mueller³

et al. 2023

Clinical Practice in Pediatric Psychology

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Objective: Children with sickle cell disease (SCD) are at elevated risk for neurodevelopmental and behavioral disorders. This article describes developmental-behavioral practice patterns among sites who were part of the Dissemination and Implementation of Stroke Prevention Looking at the Care Environment (DISPLACE) consortium in the context of current guidelines for addressing these concerns. Methods: An internal survey was developed for the principal investigators of the DISPLACE study to identify developmental-behavioral clinical practices across the 28-site consortium, including methods for identification, referral practices, access to psychologists, and barriers to services. Descriptive data were pulled from the survey to describe practice patterns. Results: Most sites used informal methods to detect developmental-behavioral concerns, though over one third of sites were using a structured protocol. The most common referral indications for further developmental and neuropsychological evaluation were parent, provider, or school concerns or stroke. Evaluations were predominantly completed by pediatric neuropsychologists and pediatric psychologists. Despite most sites reporting access to a psychologist within the SCD clinic, sites also reported long waitlists and difficulty accessing providers for evaluation and treatment services. Insurance difficulties were also a common barrier. A range of additional barriers were reported at the patient, provider, organizational, and policy/socioenvironmental levels. Conclusions: Many sites in the DISPLACE consortium were adhering to existing care guidelines for pediatric SCD; however, there was also wide variation in practices for which guidelines are absent or unclear. Additional work is needed to inform guidelines, to specify the role of psychology within specialty SCD care, and to overcome barriers to care.

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Developmental delay in infants and toddlers with sickle cell disease: a systematic review

Cited by 9 publications

References 55 publications

Mind the gap: trajectory of cognitive development in young individuals with sickle cell disease: a cross-sectional study

Mind the gap: trajectory of cognitive development in young individuals with sickle cell disease: a cross-sectional study

Sluggish Cognitive Tempo in Pediatric Sickle Cell Disease

Practice patterns for addressing developmental-behavioral concerns in sickle cell specialty care.

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