Pediatric patients with sickle cell disease (SCD) with sleep-disordered breathing (SDB) symptoms showed no difference in performance-based cognitive performance tasks compared to those patients with SCD without SDB symptoms, according to a study published in Developmental Neuropsychology.

The authors found no significant relationship between the Pediatric Sleep Questionnaire (PSQ), Psychomotor Vigilance Test (PVT; P =.86), and Delis Kaplan Executive Function System Trail Making Test (D-KEFS TMT; P =.08) values. During regression modeling, PSQ values did not explain a significant amount of variance in PVT when controlling for child age and a comorbid, non-SDB sleep disorder.

The PSQ also did not explain significant variance in D-KEFS TMT when controlling for caregiver education, comorbid sleep disorder, and history of silent stroke. Patients in the study were found to have lower scores on the D-KEFS TMT, PVT, and Behavior Rating Inventory Of Executive Function Second Edition (BRIEF-2) parent report compared to normative samples.

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Children with a history of silent cerebral infarct (SCI) also had lower D-KEFS TMT scores than those without a history of infarction (P =.01). However, BRIEF-2 parent report scores were found to be correlated with PSQ values during the study. The PSQ value predicted the BRIEF-2 parent report (F(1, 58)=44.64, P <.001, R2=0.44, f2=0.77) during regression modeling. The authors explained that this may be due to an overlap in questions between the 2 questionnaires and biases.

“The PSQ and BRIEF-2 Parent Reports are likely subject to the same biases due to their subjective nature, including social desirability, recall bias, frame-of-reference bias, and perspective bias,” the authors said. “Furthermore, the PSQ includes items that assess the behavioral outcomes of SDB, which may represent overlap in constructs assessed via the BRIEF-2 Parent Report and explain their association.”

The study did show an association between non-SDB sleep disorders and both PVT and D-KEFS TMT scores suggesting that they may play a role in the decreased scores in attention and executive function observed in the study.

A total of 60 caregiver-youth dyads were included in the study. The youth were aged between 8 and 18 years old with SCD and were predominantly Black/African American (96.7%) and male (58.3%). Roughly one-third (33%) of the patients met the PSQ criteria for SDB and 7 had a history of SCI (all male).


Turner EM, Koskela-Staples MSN, Evans BSC, Black LV, Heaton SC, Fedele DA. The role of sleep-disordered breathing symptoms in neurocognitive function among youth with sickle cell Disease1. Dev Neuropsychol. Published online February 14, 2022. doi:10.1080/87565641.2022.2038601