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Updated: Jun 3, 2025
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Sensitivity and Specificity of a Signaling Question for Surveillance of Cognitive Functioning in Pediatric Sickle
Steven J Hardy1,2,3, Megan E Connolly1,2,3, Sydney Forman1
1Division of Hematology, Children's National Hospital, Washington, District of Columbia, USA.
Insights
A simple caregiver question about academic performance in children with sickle cell disease (SCD) often misses cognitive issues. Further screening is needed for accurate identification of neurological risks in youth with SCD.
Area of Science:
- Pediatric Neurology
- Neurodevelopmental Disorders
- Sickle Cell Disease Management
Background:
- Sickle cell disease (SCD) is associated with neurological complications, impacting cognitive and academic development in children.
- Current clinical guidelines recommend using signaling questions to monitor cognition in youth with SCD.
- Evidence is limited on the accuracy of these signaling questions for identifying cognitive deficits.
Purpose of the Study:
- To evaluate the effectiveness of a single caregiver-reported signaling question in identifying cognitive impairments in children with SCD.
- To assess the association between caregiver-perceived academic performance and objectively measured cognitive functioning in youth with SCD.
Main Methods:
- Caregivers of 89 youth (ages 7-16) with SCD answered a question about their child's academic performance relative to peers.
- Children with SCD underwent cognitive assessment using the Wechsler Intelligence Scale of Intelligence, Fifth Edition.
- Statistical analyses examined the relationship between academic performance ratings and cognitive test results.
Main Results:
- Caregiver ratings indicated most children performed on grade level (67%), with 21% below grade level.
- Cognitive testing revealed 36% of participants had low IQ (≤85), indicating significant cognitive impairment.
- The signaling question showed low sensitivity (42%-54%) for detecting cognitive difficulties, despite acceptable specificity.
Conclusions:
- While caregiver reporting on academic performance is a simple monitoring tool for children with SCD, it has limitations.
- Children identified as performing below grade level warrant referral for cognitive screening.
- The low sensitivity suggests a high rate of false negatives, necessitating additional questions for comprehensive cognitive assessment in youth with SCD.
Background:
Sickle cell disease (SCD) confers neurological risks that contribute to cognitive and academic difficulties. Clinical guidelines state that cognition should be monitored using signaling questions. However, evidence is lacking regarding the extent to which signaling questions accurately identify children with cognitive issues.
Procedure:
Caregivers of youth with SCD between ages 7 and 16 years (n = 89) responded to a signaling question about their child's academic performance: "How would you rate your child's academic performance relative to their peers?" Response choices included below grade level, on grade level, or above grade level. Youth with SCD completed the Wechsler Intelligence Scale of Intelligence, Fifth Edition to assess cognitive functioning.
Results:
Most children were described by caregivers as being on grade level (67%), followed by below grade level (21%), and above grade level (11%). Cognitive testing revealed that 36% of participants had a low IQ (≤85; i.e., ≥1 SD below the mean), 60% had an IQ within normal limits (86-114; i.e., <1 SD below or above the mean), and 4% had a high IQ (≥115; i.e., ≥1 SD above the mean). Academic performance grouping was significantly associated with differences across cognitive domains (all p < 0.001). Cognitive functioning varied by academic grouping for younger (ages 7-11; all p ≤ 0.002) but not older children (ages 12-16; all p ≥ 0.05). The signaling question's sensitivity for identifying those with cognitive difficulties was low (42%-54%); specificity, negative predictive value, and positive predictive value estimates were acceptable (87%-93%, 74%-84%, and 53%-79%, respectively).
Conclusions:
Asking caregivers of children with SCD to report on academic performance is a simple method of monitoring cognition. Children rated as below grade level should be referred for cognitive screening or evaluation. However, this question will likely result in a high rate of false negatives, and additional questions may be necessary to determine when to refer patients without obvious academic concerns.
