Integrative Strong Body and Mind Training for Sickle Cell Disease (I-STRONG)—an Interdisciplinary, Multicomponent Treatment Approach for Pediatric Chronic SCD Pain

Introduction: Chronic pain is prevalent among 20% of adolescents living with sickle cell disease (SCD), contributing to impaired quality of life and daily functioning. Clinical guidelines suggest individualized chronic SCD pain management integrating pharmacological and nonpharmacological modalities and mostly leveraged indirect evidence from similar chronic pain conditions (e.g., fibromyalgia) to inform this conditional recommendation. The paucity of evidence for integrative therapies for chronic SCD pain underscores a critical unmet need for an evidence-based interdisciplinary approach for youth with SCD and chronic pain. We aimed to 1) utilize community engagement and patient and caregiver perspectives to adapt and refine an integrative multicomponent treatment program (I-STRONG) designed to meet the unique needs of chronic SCD pain, and 2) optimize feasibility and acceptability of I-STRONG through iterative pilot testing.

Methods: An existing treatment protocol for fibromyalgia that combines cognitive-behavioral therapy (CBT) for pain and neuromuscular exercise training was adapted for chronic SCD pain to develop I-STRONG. Adaptations were guided by the ADAPT-ITT model, a systematic framework to adapt treatments to new populations while maintaining fidelity to evidence-based interventions. Adaptations were informed by semi-structured qualitative interviews with adolescents with chronic SCD pain and caregivers (n=24), community advisory boards, and interdisciplinary experts representing hematology, psychology, physical therapy, rehabilitation medicine, and sports medicine. Patient eligibility included: 12-18 years old, stable (≥ 3 months) course of disease-modifying treatments as applicable, persistent or chronic pain (per Pediatric Pain Screening Tool), no co-occurring chronic pain condition unrelated to SCD, and no contraindication for participation (e.g., weight bearing restrictions). A pilot clinical trial of I-STRONG (n=12) was conducted to iteratively optimize feasibility and acceptability. Primary outcome was typical pain intensity assessed at baseline, post-treatment, and 3-month follow-up (primary endpoint). Patient global impression of change in pain was assessed at post-treatment and follow-up.

Results: Adolescents were on average 16.3 years, 42% female, and 100% Black/African American; caregivers were on average 40.8 years, 100% female, and 89% Black/African American. Patient and caregiver-informed adaptations emphasized group-based, family-centered, virtual intervention representing lived experiences of SCD and chronic pain. Refinements included values-based motivation building, differentiating SCD pain and fatigue from muscle soreness, neuromuscular exercise modifications informed by common SCD comorbidities, individualized modifications in the context of pain, and flexible scheduling.

Adolescents with chronic SCD pain (n=12) engaged in I-STRONG, a virtual 8-week, 16-session group intervention combining CBT and neuromuscular exercise training, co-led by experts in psychology and physical therapy. Strong feasibility was supported by 75% consent rate, 100% study initiation, 94% session adherence, 97% intervention fidelity, and 92% retention at primary endpoint. High levels of acceptability were reported via Treatment Evaluation Inventory at post-treatment by patients (M= 34.4, SD =4.2), caregivers (M=37.4, SD=4.0; range 9-45) and qualitative feedback. There were no serious adverse events related to the intervention. Per patients’ global impression of change in pain since the start of treatment, 82-90% reported pain improved and none reported worsened pain at post-treatment and follow-up. Typical pain intensity decreased from baseline (M = 5.8, SD = 2.6) to follow-up (M = 4.3 SD = 2.4; p < .07).

Discussion: I-STRONG is an integrative, multicomponent program for chronic SCD pain management with strong feasibility, acceptability, and preliminary efficacy to improve pain. Community engagement and partnerships with individuals with lived experience of chronic SCD pain and their caregivers shaped adaptations for access, engagement, relevance, satisfaction, and sense of belonging. Results informed further optimization of I-STRONG toward a planned multi-site, randomized clinical trial to address critical gaps in evidence of integrative therapies for chronic pain management in pediatric SCD.