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Emergency Medicine Management of Sickle Cell Disease Complications: An Evidence-Based Update
Erica Simon1, Brit Long1, Alex Koyfman2
1Department of Emergency Medicine, San Antonio Military Medical Center, Fort Sam Houston, Texas.
Insights
Sickle cell disease (SCD) complications require prompt recognition and management by emergency physicians. Understanding these acute issues in SCD patients can reduce severe illness and death.
Area of Science:
- Hematology
- Emergency Medicine
- Genetics
Background:
- Sickle cell disease (SCD) impacts ~100,000 people in the US.
- Altered hemoglobin structure in SCD leads to serious complications, often needing emergency care.
Purpose of the Study:
- To update emergency physicians on diagnosing and managing SCD complications.
- To provide evidence-based guidance for acute SCD care.
Main Methods:
- Literature review on SCD complications.
- Focus on emergency department management strategies.
Main Results:
- SCD causes significant morbidity and mortality from various acute crises.
- Key complications include stroke, acute chest syndrome, pain crises, organ failure, and hepatic/renal issues.
Conclusions:
- Emergency physicians must identify acute SCD manifestations for timely intervention.
- Effective emergency management decreases morbidity and mortality in SCD patients.
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.
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