Abstract
Sickle cell disease (SCD) affects the red blood cells, which become sickle-shaped, leading to their adhesion to vascular walls, impeding blood flow and causing the unpredictable, abrupt onset of intense pain episodes in the form of vaso-occlusive crises (VOC) as well as affecting multiple organ systems. The primary aim of this review is to assess the effectiveness of opioid analgesic intervention for pain management in sickle cell disease during an acute painful crisis. A literature search was done electronically on PubMed Central (PMC), PubMed, and Google Scholar databases. The reports included in the study were from 2010 to 2021, and the bibliographies of retrieved studies are included in the references. This systematic review was undertaken as per the Preferred Reporting Items for Systematic Review and Meta-Analysis. This study included reports discussing opioid analgesics in SCD patients during VOC in different settings. After extensive research, there were no clear current opioid treatment patterns described, and our conclusion suggested conducting more evidence-based research to improve the quality of VOC management and outcome.
Highlights
BackgroundSickle cell disease (SCD) is the most common inherited blood disorder that prevails in a specific population worldwide, primarily African Americans
The Cooperative Study of Sickle Cell Disease defines an acute pain crisis as an episode of pain in the extremities, back, abdomen, or head lasting more than two hours that leads to a clinic visit that cannot be explained by any etiology other than SCD
This excludes some pain episodes that can be manageable at home, chronic pain, as well as other multiple etiologies that can lead to acute painful complications of SCD such as acute chest syndrome, dactylitis, osteomyelitis, priapism, and right upper quadrant syndrome [5]
Summary
BackgroundSickle cell disease (SCD) is the most common inherited blood disorder that prevails in a specific population worldwide, primarily African Americans. Acute painful crisis (APC) is a consequence of microcirculation occlusion by sickle hemoglobin-containing red blood cells (RBCs), leading to ischemia, inflammation, and subsequent tissue damage [3]. These pain crises are often recurrent and unpredictable forms of acute pain [4]. The Cooperative Study of Sickle Cell Disease defines an acute pain crisis as an episode of pain in the extremities, back, abdomen, or head lasting more than two hours that leads to a clinic visit that cannot be explained by any etiology other than SCD. Multiple epidemiologic data indicated that up to five percent of SCD patients can have three to ten episodes of severe pain every year [8]
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