Sickle Cell Disease

Abstract

Sickle cell disease affects between 70,000 and 90,000 individuals in the United States, the majority of whom are of African-American descent. The genetic basis of the disease is an abnormality in the β-globin gene, which causes the red blood cells to change to a “sickle” shape due to low oxygenation. The life span of patients with this disease has improved over the past few decades, although morbidity remains high. This review covers the pathophysiology of sickle cell disease and the stabilization and assessment, diagnosis and treatment, maintenance and preventive therapies, and cure of patients with sickle cell disease. Figures show hemoglobin electrophoresis; age at death for individuals with sickle cell disease in the years 1979, 1989, 1999, and 2006; sickled cells blocking blood flow; acute chest syndrome; dactylitis; and avascular necrosis. Tables list important trials, topics in need of further research, common complications, most common intravenous pain medications, and indications for transfusion. This review contains 6 highly rendered figures, 5 tables, 97 references, and a list of educational resources.

An Outpatient Pain Plan and Emergency Department Pain Pathway for Adults With Sickle Cell Disease

“…1,6,11,12,14,23 Patients commonly present with pain in their bones, joints, abdomen, lower back, or upper thighs. 15,19 Physical examination of the painful area may be normal. Most patients are experienced at managing chronic and acute SCD pain at home and present to the ED if their home regimen cannot control their pain or their pain is different than usual.…”

“…6,15,19,20 Patients with altered mental status, atypical headache, focal neurologic findings, or new seizures should be evaluated for stroke, because patients with SCD are at higher risk. 6,15,19,20 Patients with hypotension, tachycardia, and fever raise concern for sepsis, aplastic crisis, or splenic sequestration. Rapidly evaluate patients reporting unusual symptoms because they are at high risk for SCD-related complications that can cause them to decompensate quickly.…”

“…6,15,19,20 However, there is no reason to delay treatment for an extensive investigation in a patient with an acute pain crisis. 6,14,15,20…”

“…11,14,15 Despite these practical pathways for managing vaso-occlusive pain in SCD, studies show that health care disparities continue. 3,5,8,11,[15][16][17] The disparities may be attributed to a lack of clinician confidence, knowledge, or experience with acute pain crises or the unique clinical needs of patients with SCD. 3,9,11,12,18 Because of their recurrent pain crises, patients with SCD may be labeled as drugseeking and may not receive necessary care when presenting to the ED.…”

“…Although major health care organizations have created guidelines, they do little to overcome barriers to pain management for patients with SCD because they are not widely implemented by EDs or used by clinicians. 8,11,15,17,18 To improve quality of life for patients with SCD, continued efforts are needed to better educate clinicians about SCD and its associated pain crises, instill confidence in them about treatment, and help them to gain experience using SCD-specific standardized procedures and evidencebased pain management guidelines. 3,5,6,[9][10][11][12]16,18…”