Introduction: Pain is the primary complication of sickle cell disease (SCD) and uniquely involves both acute pain experiences (i.e., vaso-occlusive crises) as well as chronic pain. SCD-related pain is associated with significant functional impairment and treatment is often limited to costly pharmacological approaches that can have unwanted side effects. While behavioral pain interventions exist for non-SCD pain populations and are recommended by the 2020 ASH Guidelines, behavioral treatments specifically designed to address pain-related functional impairment in pediatric SCD are lacking. We developed and tested the feasibility, satisfaction, and preliminary efficacy of a novel, telemedicine-based pain management intervention developed for youth with SCD-related pain.

Methods: Twenty youth (9-17 years) with SCD completed a 4-week telemedicine SCD-specific pain management intervention (The Balance Program). Participants and caregivers completed baseline and post-intervention Patient Reported Outcomes Measurement Information System (PROMIS) Pain Interference and Pain Behavior measures, Treatment Evaluation Inventories (TEI), and qualitative interviews. Descriptive analyses were used to assess feasibility, acceptability, and satisfaction with the intervention. The Reliable Change Index (RCI) was used to determine the percentage of youth that experienced a clinically significant change in pain interference and pain behaviors following the intervention. Opioid prescription data were obtained from the Prescription Database Monitoring Program. Using paired t-tests, baseline opioid prescription fills (an average of monthly fills) were compared to prescription fills in the month following the intervention. Descriptive analyses were completed on a subset of caregivers (n=5) who reported the number of pain medication doses their child needed in the previous week.

Results: Twenty youth with SCD (age M = 13.15, SD = 2.08; 70% female; 55% HbSS, 40% HbSC) received at least one session of the intervention. Eighteen (90%) of youth completed all 4 sessions. Youth (100%) and caregivers (94%) rated the intervention as moderately or highly acceptable on the TEI. Youth and caregivers described high satisfaction with the intervention during qualitative interviews, with 100% stating they would recommend this program to others with SCD. Compared to baseline, 47% of caregivers and 44% of youth reported reliably significant improvements in pain interference at one-week post-intervention (Figure 1). For those with significant improvements in pain interference, caregivers and youth reported a median T-score difference of 24.8 and 23.5, respectively, resulting in improvements of >2 SDs. Regarding changes in pain behaviors, 65% of caregivers and 31% of youth reported reliably significant improvements following the intervention, with a median T-score improvement difference of 19.3 for caregivers (approximately 2 SDs) and 32 for youth (>3 SDs). There was no significant difference in the monthly opioid prescription fills pre-intervention and during the 1-month period following the intervention, p > .05. For the subset of caregivers that reported on their child's use of pain medication, youth received fewer doses of pain medication in the week following the intervention (Median = 4.00, SD = 2.68) than the week preceding the intervention (M = 6.00 SD = 6.39).

Conclusions: The Balance Program is feasible and highly acceptable for both youth and caregivers. Approximately half of caregivers and youth reported statistically and clinically significant declines in pain interference following the intervention. Though no changes were observed in opioid prescription refills following the intervention, a small subset of caregivers reported using less pain medication for their child following in the intervention. Behavioral pain management interventions can specifically target the unique pain presentations in SCD and be successfully delivered remotely to reduce barriers to care.

Darbari:Global Blood Therapeutics: Membership on an entity's Board of Directors or advisory committees; Novartis: Membership on an entity's Board of Directors or advisory committees.

Author notes


Asterisk with author names denotes non-ASH members.

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