Pain in Youth With Sickle Cell Disease A Report From the Sickle Cell Clinical Research and Intervention Program

Objectives: Pain is prevalent among youth with sickle cell disease (SCD). However, previous research has been limited by small sample sizes and lacked examinations of developmental differences in pain, which are critical to minimizing the development of chronic pain as youth transition into adulthood. The primary aim of the current study was to compare pain and pain interference across 4 developmental groups in a large sample of youth with SCD. The secondary aim was to identify risk factors for greater pain and pain interference. Materials and Methods: Utilizing a cross-sectional study design, the expression and predictors of pain and pain interference were compared across 4 developmental stages: toddlers/preschoolers (2 to 4 y), school-aged children (5 to 7 y), preadolescents (8 to 12 y), and adolescents (13 to 18 y). Participants included 386 youth with SCD and their caregivers. Results: Caregiver-reported pain and pain interference and youth-reported pain interference increased across developmental groups and plateaued approaching adolescence (multivariate analyses of variance P=0.002 for pain and P<0.001 for pain interference). Elevated fatigue, anxiety, and perceived difficulties with pain management were the most robust predictors of higher youth- and caregiver-reported pain (beta s ranging from 0.15 to 0.68; P<0.001) and pain interference (beta s ranging from 0.18 to 0.64; P<0.001). Discussion: Disease and treatment-related variables were not associated with pain. Self-reported pain was elevated in older versus younger developmental groups and was largely linked to anxiety, fatigue, and perceptions of pain management, thus highlighting the modifiable nature of factors influencing pain among youth with SCD.