Sickle pain is the hallmark of sickle cell disease (SCD). It could be acute, persistent/relapsing, chronic, or neuropathic. Although there is a general consensus that pain is a major manifestation of SCD, there is a controversy as to the types of pain and their interrelationship between acute, chronic, relapsing, persistent, etc. This report first reviews the general approach to the management of acute vaso-occlusive crisis (VOC) pain, including education, counseling, pharmacotherapy, non-pharmacotherapy, and fluid therapy. This is followed by the presentation of five patients that represent typical issues that are commonly encountered in the management of patients with SCD.
These issues are: individualized treatment of pain, bilaterality of pain, use of illicit drugs, tolerance to opioids, opioid-induced hyperalgesia, and withdrawal syndrome. The clinical aspects and management of each of these issues are described. Moreover, such complications as tolerance and withdrawal may persist after discharge and may be mistaken as chronic pain rather than resolving, persistent or relapsing pain.
Abstract source
Sickle cell disease (SCD), a genetic disorder affecting up to 100,000 patients in the USA, impacts multiple organ systems. The emergency department (ED) is frequently utilized by patients with SCD who have severe pain from vaso-occlusive crises. The goal of this systematic literature review is to identify predictors for ED use among patients with SCD in the USA, as high ED reliance is not ideal because of the potential for discontinuity of care as well as higher costs. PubMed and Embase were searched for articles containing the keywords “sickle cell disease” AND (“emergency” OR “acute care”) AND (“utilization” OR “health care”) published between 2000 and 26 September 2019. A total of 26 publications were identified meeting the following inclusion criteria: report of ED or acute care clinic use; report of health care utilization for SCD; and report of ED visits independent of hospital admission, ED revisits, inpatient care visits, and SCD care unit visits.
Articles unavailable in English or those focused on populations outside the USA were excluded. Of the 26 articles included, 4 were prospective and the remainder were retrospective. Qualitative analysis of the articles revealed a higher rate of ED utilization among adults than children, patients with public insurance than private insurance, and patients with more comorbidities, complications, or pain. Age and pain levels were both commonly cited as predictors of ED utilization. Additional prospective and interventional studies are needed to further define predictors of ED utilization and to uncover treatments that decrease ED visits.
Abstract source
