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Decline in Processing Speed Tells Only Half the Story: Developmental Delay in Children Living with Sickle Cell

Elise Jade Walker1, Fenella Jane Kirkham1,2, Anna Marie Hood3

  • 1Developmental Neurosciences Unit, Biomedical Research Centre, UCL Great Ormond Street Institute of Child Health, London WC1N 1EH, UK.

Children (Basel, Switzerland)
|March 28, 2024
PubMed

Insights

Children with sickle cell disease (SCD) show declining processing speed over time, indicating potential developmental delays. Early monitoring of processing speed is crucial for timely intervention in pediatric SCD patients.

Area of Science:

  • Pediatric Neurology
  • Neuroscience
  • Hematology

Background:

  • Sickle cell disease (SCD) is associated with cognitive impairments in children.
  • Processing speed is a cognitive domain frequently affected in pediatric SCD.
  • Longitudinal changes in processing speed in children with SCD are not well understood.

Purpose of the Study:

  • To investigate the longitudinal changes in processing speed among children with sickle cell disease.
  • To examine the relationship between age at diagnosis and processing speed trajectory.
  • To identify potential factors influencing processing speed decline in pediatric SCD.

Main Methods:

  • A cohort of 103 children with SCD (aged 3-16 years) underwent cognitive assessments between 1992-2001, with some completing follow-up assessments.
  • Linear mixed-effects modeling was used to analyze repeated measures of processing speed index (PSI).
  • The study controlled for MRI-detected infarct status (silent infarct or stroke) and examined the interaction between age and assessment timepoint.

Main Results:

  • Children with SCD experienced a decline in processing speed index (PSI) over time, regardless of initial age group (≤8.99 or ≥9.00 years).
  • The processing speed coding subtest showed the most significant decline.
  • A significant interaction between assessment timepoint and age indicated that processing speed declines were more pronounced with age, possibly reflecting developmental delays.

Conclusions:

  • Processing speed in children with sickle cell disease declines longitudinally, suggesting a potential developmental delay linked to disease progression.
  • Despite advancements in SCD treatment, processing speed remains a concern.
  • Clinical monitoring of processing speed is recommended for early identification of delays and intervention in pediatric SCD.

Keywords:
cognitiondeclinedelaylongitudinalprocessing speedsickle cell disease

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