Factors That Predict Quality of Life in Pediatric Patients With Sickle Cell Disease

By Keightley Amen - Last Updated: December 2, 2021

Sickle cell disease (SCD) impacts health-related quality of life (HRQOL) through many physical and emotional pathways, including acute and chronic pain, sleep disruption, and frequent visits to the emergency room. A recent report in Frontiers in Psychology offered insights into what specific biopsychosocial factors might predict HRQOL in pediatric patients, in an effort to reveal what aspects might be more amenable to intervention.

“Pain is often referred to as the ‘hallmark of SCD,’ given that painful episodes and persistent pain frequently occur throughout the lifespan and are often the first presentation of the disease,” wrote the authors, led by Anna Hood of the Developmental Neurosciences Unit and Biomedical Research Centre at the University College London Great Ormond Street Institute of Child Health in London. “Although this focus is warranted, other pain coping targets also play a role in HRQOL.”

To evaluate the impact that other factors have on HRQOL, the researchers analyzed baseline data of 30 children with SCD aged 8–15 years who were enrolled in the Prevention of Morbidity in Sickle Cell Anemia Phase 2b (POMSb2) study, a randomized, controlled clinical trial of auto-adjusting continuous positive airway pressure. That study involved cognitive testing, assessments of sleepiness, and HRQOL measures. It asked patients and caregivers to report information on demographics, pain burden, and executive function. Chart reviews provided information on disease-modifying SCD treatments, medical issues such as neurologic problems, laboratory values, and number of visits to the emergency room in the previous year.

The current, secondary analysis considered several biopsychosocial variables. It found that certain factors independently predicted HRQOL, with large effect sizes that were clinically significant:

  • cognition, specifically executive function, attention, and processing speed
  • frequency of emergency department visits
  • pain burden
  • sleepiness

Several factors were not associated with HRQOL:

  • demographic factors (including age, gender, and neighborhood deprivation)
  • laboratory values (including hemoglobin, hematocrit, and mean oxygen saturation)
  • treatments

“These findings identify specific, measurable symptom categories that may serve as targets to improve HRQOL that are responsive to change,” the authors wrote. “This knowledge will be useful for multimodal interventions for pediatric patients with SCD that include sleep management, pain coping strategies, and executive function training.”