morbidity and early mortality with a reduced life expectancy of about 54 years. 1-3 SCD affects primarily Black patients and, to a lesser degree, Hispanic patients, affecting more than 100,000 patients in the United States. 2,4,5 Many of these patients are from lower socioeconomic backgrounds, have limited access to care, and are exposed to racial bias. [5][6][7][8][9][10] Because of their recurrent pain crises, patients with SCD may be labeled as drug-seeking and may not receive necessary care when presenting to the ED. 11,12 In addition, patients with SCD have less access to comprehensive team-based healthcare than patients with other genetic disorders such as hemophilia or cystic fi brosis. 10 To highlight SCD and improve the lives of patients with chronic and acute pain, in 2014, the National Heart, Lung, and Blood Institute (NHLBI) developed the seminal guidelines for managing chronic and acute SCD pain. 13 In 2020, the American Society of Hematology (ASH) updated the SCD pain management guidelines in an increased effort to enhance clinician education. 14 Additionally, in 2021, the American College of Emergency Physicians (ACEP) created an effective pain management plan to provide ED clinicians with evidence-based acute pain management guidelines. 11,14,15 Despite these practical pathways for managing vaso-occlusive pain in SCD, studies show that healthcare disparities continue. 3,5,8,11,[15][16][17] The disparities may be attributed to a lack of clinician confi dence, knowledge, or experience with acute pain crises or the unique clinical needs of patients with SCD. 3,9,11,12,18 Although major healthcare organizations have created © VICTOR JOSAN/SHUTTERSTOCK.COM S ickle cell disease (SCD) is a common inherited monogenic disease with many genotypes. The lifealtering genotypes causing the most complications include hemoglobin (Hb) SS, HbSC, and HbS betathalassemia. [1][2][3] These genotypes lead to signifi cant lifetime ABSTRACT Sickle cell disease (SCD), one of the most common inherited diseases, is associated with lifetime morbidity and reduced life expectancy. In the United States, SCD primarily affects Black patients and, to a lesser degree, those of Hispanic descent. These populations are known to have healthcare disparities related to lower socioeconomic status, limited access to healthcare, and racial bias. The quality-adjusted life expectancy of patients with SCD is less than 35 years, because of progressive complications of the disease. The most common complication is severe episodic pain related to vaso-occlusive ischemic events. Despite guidelines, pain management often is delayed as patients struggle with resistance from clinicians based on concerns over opioid use or abuse, overdose, or drug-seeking behavior. Effective pain management can be accomplished with collaboration between clinicians and patients, a documented outpatient pain management plan, and when necessary, an ED clinical pain pathway for acute SCD pain management.
Sickle cell disease (SCD), one of the most common inherited diseases, is associated with lifetime morbidity and reduced life expectancy. In the United States, SCD primarily affects Black patients and, to a lesser degree, those of Hispanic descent These populations are known to have health care disparities related to lower socioeconomic status, limited access to health care, and racial bias. The quality-adjusted life expectancy of patients with SCD is less than 35 years, because of progressive complications of the disease. The most common complication is severe episodic pain related to vaso-occlusive ischemic events. Despite guidelines, pain management often is delayed as patients struggle with resistance from clinicians based on concerns over opioid use or abuse, overdose, or drug-seeking behavior. Effective pain management can be accomplished with collaboration between clinicians and patients, a documented outpatient pain management plan, and when necessary, an emergency department clinical pain pathway for acute SCD pain management
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