NASCC Standards Expand Cognitive Screening in Sickle Cell Disease
Key Takeaways
- For people with sickle cell disease across the lifespan, NASCC standards described in Pediatric Blood & Cancer call for earlier and more consistent surveillance, screening, and evaluation for development and cognition.
- Consistent early-childhood surveillance and screening in SCD were reported to identify about 1 in 3 children with an associated neurodevelopmental disorder by age 5.
- Annual signaling-question surveillance should start at 9 months; developmental screening was listed at 9, 18, and 30 months, although a perspective in the same publication also mentioned 24 months, and autism screening at 18 and 24 months or when developmental concerns are raised.
- Social-emotional screening is included at 9, 18, 24, or 30 months and annually at ages 3 and 4 years, along with cognitive screening approximately every 5 years beginning at the start of elementary school; the standards also note that additional cognitive screening may be warranted when surveillance identifies concerns, in addition to at least one neuropsychological evaluation before transition to adult care.
- The standards were reported to apply to all sickle cell disease genotypes even though neurologic risk was described as stronger in HbSS and HbS-beta zero.
In sickle cell disease (SCD), the National Alliance of Sickle Cell Centers (NASCC) outlined a lifespan framework in the Pediatric Blood & Cancer standards from Schlenz and colleagues for surveillance, screening, and evaluation of development and cognition. The workgroup comprised 10 psychologists, 2 physicians, and 1 occupational therapist, with representation from pediatric and lifespan neuropsychology, pediatric psychology, developmental pediatrics and neurodevelopmental disabilities, hematology, and occupational therapy. The standards were described as applying across all forms of SCD, although neurologic risk was noted to be stronger in HbSS and HbS-beta zero, and as building on prior American Society of Hematology (ASH) guidance.
SCD was described as being associated with deficits in processing speed, executive function, and reasoning, with difficulties that can emerge as early as infancy and increase with age. According to the authors, consistent early-childhood surveillance and screening can identify about 1 in 3 children with SCD who have an associated neurodevelopmental disorder by age 5. That estimate was presented in the context of reducing delayed identification of neurodevelopmental or neurocognitive impairment.
Across infancy, preschool, school age, and transition, the reported age-based screening framework for sickle cell disease paired annual developmental surveillance through signaling questions with scheduled early-childhood developmental, autism, and social-emotional screening, recurring school-age cognitive screening, and formal developmental or neuropsychological evaluation when suspicion was high. The process also included interpreting results for families and identifying intervention resources inside and outside the clinic, positioning serial assessment as part of ongoing cognitive care rather than a one-time checkpoint.
Schlenz and colleagues described the NASCC framework as a lifespan approach to developmental and cognitive care in SCD with a strong pediatric starting point. The standards emphasized multidisciplinary implementation across primary care, SCD specialty teams, psychologists, occupational therapists, and other experts as needed. The focus remained coordinated cognitive care from early childhood through transition to adult services.
Clinician Questions
How do the NASCC cognitive-care standards expand on earlier ASH guidance for sickle cell disease?
The NASCC standards for sickle cell disease were described as building on earlier American Society of Hematology guidance that focused on cerebrovascular risk and common comorbidities, while extending the framework to surveillance, screening, and evaluation of development and cognition across the lifespan.
Why were serial assessments emphasized in cognitive care for sickle cell disease?
Serial assessments were emphasized because neurodevelopmental and neurocognitive difficulties in sickle cell disease were described as emerging as early as infancy and increasing with age, so repeated follow-up can capture changing needs and new medical complications that affect thinking, learning, and attention over time rather than offering only a single snapshot.
How were the NASCC standards meant to fit sickle cell centers with different local resources?
Schlenz and colleagues described the framework as flexible because communities differ in access to subspecialty services, with implementation framed around tailoring services to individual needs while coordinating among primary care, SCD specialty teams, psychologists, occupational therapists, and other experts as available.