Utilization of Pharmacologic and Non-Pharmacologic Therapies for Pain in Sickle Cell Disease: A Cross-Sectional Study
Authors: Parisio Katie , Andrews Elyse , Lewis Amanda M. , Schultz Corinna L. 
Published: December 2023

Background: The sickling deformity of red blood cells in sickle cell disease (SCD) results in multisystem sequelae, including severe, acute pain from vaso-occlusive crises (VOCs) and chronic pain from central sensitization. Pain is the main cause of poor quality of life and hospital admissions among patients with SCD. While opioid therapy is an important component of treatment for acute SCD pain, it can be complicated by side effects and toxicities. In contrast, chronic SCD pain is best managed with non-pharmacologic therapies. A multimodal, multidisciplinary approach to pain management that promotes non-pharmacologic therapies can improve the quality of care for patients with SCD by addressing biopsychosocial components of the pain experience and is recommended by the American Society of Hematology (ASH). This study examines current usage of pharmacologic and non-pharmacologic therapies among Adolescents and Young Adults (AYAs) with SCD receiving care at our institution.

Methods: The electronic medical record and Sickle Cell Knowledgebase, a customized data visualization platform unique to our institution, were utilized to conduct a retrospective chart review of AYAs, ages 13-21 years of age, with SCD from 2016-2021. Data collected included demographic information, diagnosis of chronic pain, emergency department (ED) visits and inpatient admissions for VOCs, as well as pharmacologic and non-pharmacologic therapies utilized in the ED, inpatient setting, and, for patients with chronic pain, in the outpatient setting.

Results: The cohort included 90 AYAs with SCD (73% were 13-17 years old). The most common SCD genotypes were HbSS (n=47, 52%) and HbSC (n=32, 36%). The majority were female (n=53, 49%), Black (n=85, 94%), and non-Hispanic (n=86, 96%). There were 95 ED visits for VOC, where the patient was subsequently discharged home, and 246 admissions for VOC during the study period. There was a high frequency of opioid interventions for VOCs during the 95 extracted ED visits (81.8% in 2016, 91.7% in 2021). During VOC admissions, there was consistent use of opioids in 100% of admissions from 2016-2021 with increasing utilization of patient-controlled analgesia (7.7% in 2016, 45.1% in 2021) and subanesthetic ketamine (0% in 2016, 18.3% in 2021) each year during the study period. Compared to pharmacologic measures, non-pharmacologic therapies (i.e., healing touch, massage, art therapy, child life, music therapy, occupational therapy, physical therapy, psychology) were less utilized during VOC admissions. While healing touch and child life therapy were ordered during approximately 85% of VOC admissions, they occurred &lt;50% of the time. All other integrative therapies were ordered between 5-55% of admissions each year and occurred &lt;50% of the time. There was no correlation between length of hospital stay and use of integrative therapies. Seven patients with a diagnosis of chronic pain were identified. All 7 received non-pharmacologic therapies in the outpatient setting, including cognitive behavioral therapy, guided imagery, and psychology consultation. Six received either a gabapentinoid, serotonin and norepinephrine reuptake inhibitor (SNRI), or tricyclic antidepressant (TCA).

Conclusion: Per the ASH SCD guidelines, non-pharmacologic therapies are recommended for SCD-related pain. In our cohort, we found that non-pharmacologic therapies occurred in &lt;50% of VOC admissions and were underutilized for SCD-related pain in AYAs when compared to pharmacologic therapies at our institution. Though we speculate that healing touch and child life were frequently ordered as a default within our admission order set, utilization rates were still low. To better understand barriers to non-pharmacologic therapies in SCD, we are conducting qualitative interviews of patients, caregivers, and healthcare providers using the Consolidated Framework for Implementation Research (CFIR) as a theoretical model. Understanding barriers to the use of non-pharmacologic therapies will be essential to maximizing their implementation and hence maximizing pain control, improving quality of life, and increasing self-efficacy.