Sickle cell disease is a chronic hematologic disease that affects over 100,000 people in the United States. Many of these patients will present to the emergency department seeking treatment for an acute complication. Vaso-occlusive crisis, the most common recurring complication, can be difficult to manage because of the stigma patients face surrounding management of their pain. Patients with sickle cell disease presenting with conditions such as pain, infection, respiratory distress, stroke, or priapism must be given special consideration, as management can differ from that of the general population.
Our recent issue Emergency Department Management of Patients With Sickle Cell Disease
evaluates the current guidelines and literature on acute complications related to sickle cell disease to dispel misconceptions about seemingly harmless interventions and provide clarification on those that are more controversial.
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Here are a few key points:
- Because of worldwide migration trends, persons with sickle cell disease (SCD) can be found in all parts of the world.
- The primary complications of SCD are severe pain, vaso-occlusive crisis (VOC), splenic sequestration, acute chest syndrome (ACS), priapism, stroke, and infection.
- IV opioids are typically preferred for rapid and consistent effect, but intranasal fentanyl was found to be noninferior for ED management of pain,74 and may be preferable if there are challenges in IV access, such as in pediatric patients.
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