Emergency Medicine Management of Sickle Cell Disease Complications: An Evidence-Based Update

J Emerg Med. 2016 Oct;51(4):370-381. doi: 10.1016/j.jemermed.2016.05.042. Epub 2016 Aug 20.

Abstract

Background: Sickle cell disease (SCD) affects approximately 100,000 individuals in the United States. Due to alterations in the structural conformation of hemoglobin molecules under deoxygenated conditions, patients with SCD are predisposed to numerous sequelae, many of which require acute intervention.

Objective: Our aim was to provide emergency physicians with an evidence-based update regarding the diagnosis and management of SCD complications.

Discussion: SCD patients experience significant morbidity and mortality secondary to cerebrovascular accident, acute chest syndrome, acute vaso-occlusive pain crises, SCD-related multi-organ failure, cholecystitis, acute intrahepatic cholestasis, acute sickle hepatic crisis, acute hepatic sequestration, priapism, and renal disease. Emergency physicians must recognize acute manifestations of SCD in order to deliver timely management and determine patient disposition.

Conclusions: A comprehensive review of the emergency department management of acute SCD complications is provided. Comprehensive understanding of these aspects of SCD can assist physicians in expediting patient evaluation and treatment, thus decreasing the morbidity and mortality associated with this hemoglobinopathy.

Keywords: acute chest; acute pain crisis; cerebrovascular accident; sickle cell disease; transfusion.

Publication types

  • Review

MeSH terms

  • Abdominal Pain / diagnostic imaging
  • Abdominal Pain / etiology
  • Acute Chest Syndrome / diagnosis
  • Acute Chest Syndrome / etiology
  • Acute Pain / drug therapy*
  • Acute Pain / etiology
  • Anemia, Sickle Cell / complications*
  • Anemia, Sickle Cell / therapy
  • Brain Ischemia / diagnosis
  • Brain Ischemia / etiology
  • Brain Ischemia / therapy
  • Emergency Medicine / methods*
  • Evidence-Based Medicine
  • Female
  • Humans
  • Infections / diagnosis
  • Infections / microbiology
  • Intracranial Hemorrhages / diagnosis
  • Intracranial Hemorrhages / etiology
  • Intracranial Hemorrhages / therapy
  • Kidney Diseases / diagnosis
  • Kidney Diseases / etiology
  • Male
  • Multiple Organ Failure / etiology
  • Practice Guidelines as Topic
  • Priapism / etiology
  • Priapism / therapy
  • Transfusion Reaction