Cognitive and Emotional Factors and Pain-Related Outcomes in Youth with Sickle Cell Disease
Authors: Williams Zaria , Hardy Steven J. , Berger Carly , Griffin Danielle , Weisman Hannah , Liang Hua , Darbari Deepika S. 
Published: November 2020

Children with sickle cell disease (SCD) experience acute episodes of pain that may require visits to emergency department (ED) or hospitalizations. SCD is associated with cognitive deficits that are particularly evident in domains of executive functioning (e.g., attention, working memory). Many patients/parents also report emotional concerns related to SCD. It is not known the degree to which these concerns impact disease self-management and healthcare utilization. This study investigated cognitive and emotional factors as contributors to pain-related healthcare utilization (visits to ED and hospitalizations) in children with SCD.
The cohort included 112 youth with SCD (all genotypes) between ages 7-16 years who received care at Children's National Hospital in Washington, DC. Measures of socioeconomic status (e.g., parent education, family income, 1- vs. 2-parent households), cognitive abilities, and emotional functioning were extracted from an existing database of participants who had previously enrolled in the IRB approved study of computerized working memory training. Cognitive measures included the Wechsler Intelligence Scale for Children (WISC-V) Full Scale IQ and attention, working memory, and executive functioning tests from Cogstate, a computerized cognitive assessment. Emotional functioning was measured using the Worry 1 (i.e., anxiety about SCD) and Emotions (i.e., frustration and anger about SCD) domains of both the child- and parent-rated Pediatric Quality of Life Inventory Sickle Cell Disease Module. Healthcare utilization was measured through chart review, separated into ED visits for pain and hospitalizations for pain 1 year and 3 years after enrollment.
The mean age of participants was 10.61 (SD=2.91) year with majority being females (n=65; 58%). Eighty-three (74%) had sickle cell anemia (HbSS or HbSβ0 thalassemia). The median number of ED visits for pain was 1 visit (IQR=2) across 1 year after enrollment and 3 visits (IQR=6) across 3 years after enrollment. The median number of hospital admissions for pain was 0 admissions (IQR=1) across 1 year after enrollment and 1 admission (IQR=4) across 3 years after enrollment (pain hospitalizations = 1; IQR=4).
Linear regression models were applied to explore whether cognitive and emotional variables were predictive of healthcare utilization for pain. Variables included in the final model were selected by using the Akaike information criterion. Results indicated that attentional abilities were significantly associated with ED visits and hospitalizations for pain, both at 1 year and 3 years after enrollment (p's&lt;.009), such that better performance on the Cogstate attention task was associated with fewer ED visits and hospitalizations. Child-rated emotional quality of life significantly predicted ED visits for pain over 1 year (b=-0.004, p=.049) and hospitalizations for pain over 3 years (b=-0.006, p=.013), such that higher emotional quality of life was predictive of fewer ED visits and hospitalizations. Parent-rated child emotional quality of life was also predictive of hospitalizations for pain over 3 years (b=-0.007, p=.020). Neither SCD genotype nor socioeconomic status significantly impacted healthcare utilization.
Results demonstrate that children's cognitive and emotional functioning play an important role in pain management and should be an integral part of comprehensive pain management plans for children with SCD. Attentional abilities emerged as a reliable predictor of pain-related healthcare utilization and may signal that poor attention makes it difficult to implement strategies to distract from pain. It could also suggest that children with poor attention have greater difficulty with behavioral aspects of disease self-management (e.g., medication adherence, avoiding pain triggers). These are important considerations, given that youth with SCD are at risk for disease-related neurological sequelae including reduced attentional abilities. Emotional functioning (specifically, frustration and anger about SCD) also predicted healthcare utilization for pain, highlighting the potential impact of stress on pain onset and management. Clinicians and researchers should consider cognitive and emotional factors when evaluating risk for pain in SCD and incorporate these factors when developing strategies to reduce healthcare utilization and costs.

Disclosures
Darbari: Hilton Publishing: Consultancy, Other: Expert advisory board for developing gaming app for SCD; Global blood therapeutics: Consultancy, Honoraria, Other: participated in advisory board meeting ; Novartis: Consultancy, Honoraria, Other: steering committee for SPARTAN study .