Neurocognitive risk in sickle cell disease: Utilizing neuropsychology services to manage cognitive symptoms and functional limitations

Abstract

Sickle cell disease (SCD) is an inherited blood disorder that is associated with developmental delays and neurocognitive deficits. This review details key findings related to neurocognitive outcomes for children and adults with emphasis on the impact of neurological correlates and disease severity. Associations between neurocognition, demographic factors and social determinants of health are also reviewed. Emerging literature has reported on the neurocognitive impact of SCD in children and adolescents in Africa and Europe, including children from immigrant communities. Neurocognitive deficits are linked to poor functional outcomes, including transition from paediatric to adult care, medication adherence and unemployment. Integrating neuropsychology into multidisciplinary care for individuals with SCD can assist with identification and management of neurocognitive concerns, intervention development, individualized care plan development and continued multidisciplinary research.

Health literacy correlates with abbreviated full‐scale IQ in adolescent and young adults with sickle cell disease

“…The American Society of Hematology recommends regular surveillance for cognitive impairment in children and adults with SCD 45 . To meet these needs, neuropsychological assessment services can be provided via cognitive screenings (generally 15–20 minutes), targeted neuropsychological evaluations (2–3 hours), or comprehensive evaluations (3+ hours) 46 . Brief cognitive screenings (15–30 minutes) using early detection tools (e.g., the Montreal Cognitive Assessment [MoCA] or Mini Mental Status Exam) 47,48 can be also used by healthcare professionals to identify individuals who would benefit from referrals to neuropsychology to elucidate neurocognitive deficits and the potential impact on HL and other daily living skills.…”

“…45 To meet these needs, neuropsychological assessment services can be provided via cognitive screenings (generally 15-20 minutes), targeted neuropsychological evaluations (2-3 hours), or comprehensive evaluations (3+ hours). 46 Brief cognitive screenings (15-30 minutes) using early detection tools (e.g., the Montreal Cognitive Assessment [MoCA] or Mini Mental Status Exam) 47,48 can be also used by healthcare professionals to identify individuals who would benefit from referrals to neuropsychology to elucidate neurocognitive deficits and the potential impact on HL and other daily living skills. It is unclear how may SCD treatment centers are implementing the recommendations for neurocognitive surveillance, and lack of funding may be an obstacle to providing regular surveillance services.…”

Working memory and school readiness in preschool children with sickle cell disease compared to demographically matched controls

“…Sickle cell disease is characterised by early and progressive neurocognitive dysfunction, with notable effects on EF and working memory. 43 Due to these neurocognitive difficulties, preschool children with SCD are at risk for poor school readiness when they enter kindergarten. 30 These school readiness skills, including literacy and numeracy, are highly predictive of later academic performance and grade retention.…”

“…Working memory allows individuals to temporarily store and manipulate information while performing tasks. 12,13 In children and adolescents with SCD, working memory is known to be an area of weakness, 43,45 and has been the target of neurocognitive interventions. 46,47 Working memory difficulties may arise from vasculopathies and subsequent tissue injuries to cortical and subcortical regions, particularly frontal watershed regions.…”

“…Sickle cell disease is characterised by early and progressive neurocognitive dysfunction, with notable effects on EF and working memory 43 . Due to these neurocognitive difficulties, preschool children with SCD are at risk for poor school readiness when they enter kindergarten 30 .…”