Chapter 26: Emergent Clinical Complications of Sickle Cell Disease

Overview

Sickle cell disease (SCD) comprises a group of recessively inherited hemoglobinopathies that impact approximately 100,000 adults and children of various ethnicities and backgrounds in the United States and millions more worldwide.^1,2 Although the clinical features of SCD vary from patient to patient, common clinical complaints in the emergency department (ED) result from a persistent hemolytic anemia and vasculopathy within the microcirculation, which leads to repeated episodes of vaso-occlusion and ischemia-reperfusion that can result in acute and chronic end-organ damage of every organ system.^3,4 Patients with SCD have a shortened life expectancy compared to the general population due to these complications; however, most children with SCD in the United States can now expect to live to adulthood thanks to modern medical advances, including mandatory neonatal hemoglobinopathy screening, disease-specific immunization practices, early initiation of penicillin prophylaxis, hydroxyurea therapy, transfusion protocols, and parent education.⁵

Patients with SCD spend a great deal of time in an ED, yet care in the ED is a neglected area of sickle cell research. The negative attitudes toward SCD patients with pain have been compounded by racial stereotypes, the effects of the disease in limiting educational and employment opportunities, suboptimal medical coverage, and the large doses of opioids often required to obtain pain relief.⁶ Ethnic disparities in ED care have been reported,^7-9 and adults with SCD experience longer delays in the initiation of analgesics compared to other patients with pain.¹⁰ Barriers to rapid care in the ED are common across the country, including overcrowding, low nurse-to-patient ratios, insufficient staff coverage, inadequate funding, and slow flow of patients to the wards, in addition to high patient acuity of other patients in a busy ED.

The term sickler is often used by medical practitioners to refer to children and adults with SCD. Although using a label is common practice in the ED to rapidly refer to a specific patient (CF’er is used for a patient with cystic fibrosis and asthmatic for a patient with asthma) and often not meant to be derogatory, many patients with SCD find the term offensive.¹¹ In fact, the term sickler is pervasively used across the United States, and studies have found that emergency medicine physician use of the term sickler is associated with negative attitudes toward people with SCD.^11,12 Ultimately, this is a term that should be avoided in practice.

The total financial costs of SCD admissions from the ED are nearly $2 billion per year in the United States.¹³ The transition from pediatric to adult care is an especially vulnerable time for these patients, during which ED utilization dramatically increases, likely due to both biologic and social causes.^14-16 Some literature suggests that admissions may be avoided by reducing stigmatization and improving care delivered in the ED, which is cited as a top priority area in great need of improvement ...