What are the ASH guidelines for the screening and management of cognitive impairment in patients with sickle cell disorder (SCD)?

Updated: May 12, 2021
  • Author: Joseph E Maakaron, MD; Chief Editor: Emmanuel C Besa, MD  more...
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Answer

Screening for developmental delay or cognitive impairment in children and adults with SCD:

  • Conduct surveillance, using simplified signaling questions, for concerns about developmental delays in preschool-age children and concerns about neurodevelopmental disorders in school-age children, such as academic or behavioral problems or symptoms of inattention, hyperactivity, or impulsivity.
  • For children who have abnormal surveillance results suggesting increased risk for developmental delay or cognitive impairment, screen or referl for formal screening by a psychologist or a pediatrician able to perform screening with the available validated tools

  • For adults, conduct surveillance for cognitive impairment using simplified signaling questions; in those with abnormal results suggesting cognitive impairment, provide formal referral to a psychologist or a primary care physician able to perform more in-depth cognitive evaluation.

Rehabilitation for children and adults with cognitive impairments

The following are recommended for children with SCD and abnormal developmental or cognitive status screens:

  • Developmental, cognitive, and medical assessment to diagnose any related disorders and to identify any modifiable risk factors for developmental delays or cognitive impairments
  • Delivery of appropriate interventions by following the cognitive domain–specific, evidence-based guidelines for these disorders

The following are recommended for adults with SCD and abnormal cognitive status screens:

  • Cognitive and medical assessment to diagnose any related disorders and to identify any modifiable risk factors for cognitive impairments
  • Delivery of appropriate interventions by following the cognitive domain–specific, evidence-based guidelines for these disorders

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