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

Br J Haematol. 2022 May;197(3):260-270. doi: 10.1111/bjh.18041. Epub 2022 Feb 4.

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.

Keywords: functional; neurocognitive; neuropsychology; sickle cell disease; transition.

Publication types

  • Review

MeSH terms

  • Adolescent
  • Adult
  • Anemia, Sickle Cell* / drug therapy
  • Anemia, Sickle Cell* / therapy
  • Child
  • Cognition
  • Humans
  • Neuropsychology
  • Severity of Illness Index
  • Transition to Adult Care*