Background & Aims

Sickle cell disease (SCD) is the world’s most prevalent heritable blood disorder, which is characterized by vaso-occlusive pain crises that pose significant risks to quality of life. Pain phenotypes are variable in SCD and patients can exhibit wide-ranging outcomes even when possessing similar biological disease features (e.g., SCD genotype). There is burgeoning evidence that cognitive and emotional factors may play a role in the onset and experience of pain in pediatric SCD but it is unclear how these factors may interact to affect pain outcomes. We sought to examine the influence of processing speed—the cognitive domain most impacted by disease processes—and emotional control on pain frequency and pain-related impairment in children with SCD.

Methods

Patients with SCD between ages 7-16 were recruited from a large children’s hospital in the United States. Patients and primary caregivers completed the PedsQL SCD Module and parent-proxy PedsQL SCD Module, respectively. PedsQL subscales of interest (pain frequency and pain-related functional impairment) were utilized for the current analyses. Cognitive processing speed was measured using the Wechsler Intelligence Scale for Children, Fifth Edition (WISC-V), which produced a standard score where lower scores reflect lower processing speed. Emotional control was assessed using the caregiver-reported Behavior Rating Inventory of Executive Function (BRIEF), where higher scores indicate greater difficulties. To examine the cumulative influence of low processing speed and poor emotional control on pain outcomes, processing speed and emotional control scores were dichotomized (<85 and >55, respectively) to reflect low- and high-risk groups.

Results

89 participants enrolled and completed study procedures. The mean age of participants was 10.42 years (SD=2.92), most were female (n=52; 58%), and the majority were diagnosed with a severe SCD genotype (n=67; 75%). Thirty-seven percent of participants fell in the high-risk category for processing speed, 31% fell in the high-risk category for emotional control, and 13% met both high-risk criteria. Lower processing speed was correlated with child- and caregiver-reported pain frequency (r=.41, p<.001 and r=.25, p=.02). The high-risk processing speed group (i.e., low processing speed) exhibited significantly greater pain frequency as reported by children (t(87)=2.93, p=.003) and caregivers (t(85)=2.25, p=.014). Poorer emotional control was associated with increased caregiver-reported pain frequency (r=-.23, p=.03) but no pain outcomes varied by emotional control risk-grouping. Participants meeting both high-risk criteria reported the greatest pain frequency (F(2,84)=4.40, p=.015).

Conclusions

Processing speed and emotional control contribute to pain frequency in youth with SCD. Clinicians should consider cognitive and emotional functioning when conceptualizing pain presentations and formulating treatment plans.

References

Hardy SJ, Williams Z, Berger C, et al. Neurocognitive and emotional factors predict pain-related healthcare utilization in children with sickle cell disease. Pediatr Blood Cancer. 2023;70(6):e30346. doi:10.1002/pbc.30346

Presenting Author

Steven Hardy

Poster Authors

Steven Hardy

PhD

Children's National Hospital

Lead Author

Megan Connolly

PhD

Children's National Hospital

Lead Author

Sydney Forman

BS

Children's National Hospital

Lead Author

Deepika Darbari

Children's National Hospital

Lead Author

Topics

  • Pain in Special Populations: Infants/Children