Emergency Department Management of Patients With Sickle Cell Disease

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Delivery Method:	Online
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Publication Date: August 2024 (Volume 26, Number 8)

CME Credits: 4 AMA PRA Category 1 Credits™, 4 ACEP Category I credits, 4 AAFP Prescribed credits, and 4 AOA Category 2-B CME credits. CME expires 08/01/2027.

Authors

Peer Reviewers

Abstract

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. This review 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. Novel treatments that may have future impact on the management of patients with sickle cell disease are also reviewed.

Case Presentations

An 18-year-old woman with sickle cell disease presents with bilateral hip and low back pain...

• As you enter the room, the patient appears to be resting comfortably on a stretcher and is scrolling through her cell phone. Her vital signs are within normal limits and her physical examination is grossly unremarkable.
• The patient reports that her pain started after a recent upper respiratory infection and has been worsening progressively for 3 days. She now rates it a 10 out of 10 in severity. She is on chronic opioid therapy with hydromorphone and has been taking oral acetaminophen, ibuprofen, and oxycodone for breakthrough pain around the clock, with minimal relief. She says this pain feels similar to prior pain crises.
• The nurse has already placed a peripheral IV and sent laboratory tests. The CBC returns with a hemoglobin of 10.2 mg/dL, which is consistent with the patient’s baseline.
• You wonder whether this patient is having a true pain crisis and what pain medication would be best to provide. You also wonder whether “drug seeking” is a concern…

A 42-year-old woman with sickle cell disease presents with fever, chest pain, and shortness of breath...

• On arrival, the patient appears to be in acute respiratory distress. She is coughing, and reports severe pain in her chest.
• The patient is febrile to 39°C, tachypneic to 40 breaths/min, and hypoxic to 86% on room air. Her heart rate is 90 beats/min and her blood pressure is 110/70 mm Hg. Lung examination is notable for crackles and decreased air movement at the bilateral bases.
• A STAT portable chest x-ray shows bilateral lobar infiltrates. She is placed on oxygen via a nonrebreather mask. Her oxygen saturation improves to 94%, although she remains dyspneic.
• You wonder whether this patient needs expanded antibiotic coverage and whether there is a role for a blood transfusion…

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