Patient, Caregiver, and Healthcare Provider Perspectives on Pharmacologic and Non-Pharmacologic Therapies for Pain in Sickle Cell Disease

Background: Pain is the main cause of poor quality of life among patients with sickle cell disease (SCD). While opioid therapy is the mainstay of treatment for acute SCD pain, it can be complicated by side effects and toxicities. 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. Guided by the Consolidated Framework for Implementation Research (CFIR), this study utilized qualitative interviews with patients, caregivers, and healthcare providers at our institution to assess barriers to the use of non-pharmacologic and pharmacologic therapies for pain in SCD.

Methods: Semi-structured qualitative interviews were conducted with adolescents and young adults (AYAs; ages 13-21) with SCD, caregivers of AYAs with SCD, and healthcare providers at our institution. AYAs had either a documented history of chronic pain and/or ≥1 admission/year for vaso-occlusive crises (VOCs) for at least one year from 2016-2022.

After informed assent and/or consent were obtained, individual semi-structured interviews were conducted. Open-ended interview questions explored existing knowledge, perceptions, and barriers to pharmacologic and non-pharmacologic therapies for pain in SCD. Interviews proceeded until no new themes emerged in each group (i.e., thematic saturation).

Interviews were analyzed using descriptive content analysis facilitated by Dedoose software. The CFIR and prior research informed a preliminary codebook, which was revised as new themes emerged. The coders independently coded transcripts and then met to discuss and resolve any discrepancies in coding.

Results:Demographics: Eighteen AYAs, 17 caregivers, and 22 healthcare providers were interviewed. Of the AYAs, 61% were female, mean age was 17 years, and the most common SCD genotype was HgbSS (56%). Of the caregivers, 88% were mothers, with a mean age of 43 years. There were 15 AYA/caregiver dyads who participated. The following healthcare providers who care for patients with SCD were interviewed: physicians (31%), nurse practitioners (14%), nurses (14%), child life specialists (14%), psychologists (9%), art therapists (4.5%), healing touch providers (4.5%), physical therapists (4.5%), and social work (4.5%).

Themes: Barriers to the use of non-pharmacologic therapies for pain management in SCD can broadly be grouped into two major themes: lack of knowledge about options and lack of access to non-pharmacologic options. Regarding lack of knowledge, most AYAs and caregivers endorsed a lack of knowledge about specific non-pharmacologic therapies (e.g., yoga therapy, healing touch, acupuncture), including their benefits, risks, and their abilities to reduce pain. Like AYAs and caregivers, many healthcare providers had limited knowledge about specific non-pharmacologic therapies and the evidence in support of their usage. Regarding lack of access to specific options, the majority of participants questioned the ability of patients to access non-pharmacologic options for pain management including availability of options and cost of access.

Additional themes included a desire for more education about pain and pain management options. AYAs and caregivers were particularly interested in more information about yoga. Additionally, AYAs and caregivers were interested in education early in life before pain episodes occurred.

Conclusions: Emerging themes among all groups include an interest in non-pharmacologic therapies for pain in SCD, but with limited knowledge on benefits, risks, options, and access to these therapies. As the American Society of Hematology (ASH) guidelines recommend non-pharmacologic therapies for sickle cell pain, it is crucial to implement these therapies into the management of pain in SCD. Education about different options paired with implementation options for ways to incorporate non-pharmacologic therapies is necessary to optimize pain control and improve quality of life. A particular focus on yoga therapy, with availability of a yoga therapist in medical settings, and organized yoga education events specific for SCD may be beneficial to engage individuals in yoga therapy with a trained yoga-therapist. As a next step, evaluation of these potential implementation strategies will occur within our health system.