Understanding chronic pain in pediatric sickle cell disease: Insights from a retrospective chart review

Abstract Background: Sickle cell disease (SCD) is an inherited hemoglobinopathy affecting over 100,000 individuals in the United States and an estimated 8 million worldwide [CDC]. The disease is characterized by structurally abnormal, rigid erythrocytes that aggregate within the vasculature, leading to impaired blood flow and complications such as painful vaso-occlusive crises, stroke, and organ damage. Although current standard of care utilizes opioid-based strategies to manage acute pain episodes, the underlying physiologic mechanisms and clinical recognition of chronic pain in SCD remain poorly understood, particularly in pediatric populations. This study aims to understand the prevalence of chronic pain in pediatric patients with SCD at a single institution. Methods: A retrospective chart review was conducted of children with a confirmed diagnosis of SCD who received care at Riley Children's Hospital Sickle Cell Clinic between January 1 and December 31, 2024. Data were extracted from the electronic medical record, including demographics (age, sex, SCD genotype), disease-related characteristics such as a known chronic pain diagnosis, and disease-related events such as frequency of hospitalizations, documented pain events in the medical record, and number of opioid prescriptions. Descriptive statistics (mean, frequency, standard deviations) were conducted. This study was approved by Indiana University's Institutional Review Board. Results: A total of 441 unique patient charts were reviewed. Twenty-nine were excluded due to being less than 1 year old or not being seen during the study period. Most patients had the Hb SS-genotype (58%) and nearly half (48%) were female. Of the 412 unique patients reviewed, only 16 had an established diagnosis of chronic pain, with an average age of 17 years (SD +/- 2.4). Fifteen of the 412 patients had 3 or more hospitalizations in a year, 12 of whom did not have a diagnosis of chronic pain. Five had 6 or more opioid prescriptions in 12 months. Similar to the larger cohort, most had Hb SS and about half were female. However, the average age of this unique subgroup was younger (13 years +/- 4.6 years). Conclusion: These findings highlight a notable gap between clinical indicators of high pain burden and the formal recognition of chronic pain in pediatric patients with SCD. The under-recognition of potential risk factors for chronic pain, particularly among younger patients, may contribute to delays in appropriate treatment and hinder the development of effective long-term pain management strategies, ultimately diminishing quality of life. Ongoing investigation is critical to clarify chronic pain mechanisms and enhance provider recognition, ensuring timely and appropriate care.