Management of Sickle Cell Pain

Abstract While sickle cell disease (SCD) is uncommon in Europe and North America, SCD patients presenting for pain account for an outsized and prominent proportion of all acute care utilization in these countries. Managing SCD pain requires one treatment paradigm for the hallmark, disruptive, infrequent vaso-occlusive crisis (VOC). But it also requires a different treatment paradigm for the under-recognized but sometimes daily chronic pain of SCD. Both paradigms require a biopsychosocial framework and an infrastructure that is lacking in most hospitals. Because providers don’t understand the context of the disease, SCD pain treatment is often poor, viewed from patients’ perspectives. And viewed from providers’ perspectives, frustration emanates from either lack of confidence, or worse, misplaced confidence in managing SCD pain. Disagreement, mutual mistrust, and poor treatment outcomes are common as a result. This book therefore gathers and summarizes the sparse evidence and published guidelines for best practice in SCD pain treatment, most of it surrounding treating acute pain. It combines that evidence with a consensus of senior pain experts from several comprehensive sickle cell centers. It uses this combination to instruct readers, regardless of their background, on how to develop and revise various components of individualized, biopsychosocial pain treatment plans for their SCD patients, as recommended by the literature, as well as how to develop institutional policies that promote safety, efficacy, fairness, equity, compassion, and clear communication of SCD pain management. Like SCD pain, its focus begins in childhood, but rapidly transitions to adulthood. This volume honestly exposes the controversies and knowledge gaps that remain related to treating SCD pain, and fills gaps when possible, based on expert opinion and emerging evidence.