From the EM Model
8.0 Hematologic Disorders
8.5 Red Blood Cell Disorders

Editors Note: In last month’s ACEP News, “Focus On… Critical Decisions,” we presented an overview of the pathophysiology of sickle cell disease in children, focusing on common and concerning presentations. In this month’s article, we continue the discussion, looking at emergency department evaluation and management of this condition.

Objectives

On completion of this lesson, you should be able to:

1. Explain the rationale for specific diagnostic studies and treatments.
2. List the indications for blood transfusion in a patient with SCD.
3. Describe the indications for hospital admission.

Sickle cell disease (SCD) is a hereditary hemoglobinopathy that is characterized by anemia and a wide array of pathology secondary to intermittent small blood vessel occlusion. It is estimated that 1 in 600 African Americans in the United States has the disease; SCD can also be seen in people of Indian, Mediterranean, and Middle Eastern descent. Children with SCD presenting to the emergency department represent a unique challenge. Even though vasoocclusive crisis (VOC) is the most common presentation, there is a much higher incidence of potentially life-threatening pulmonary, central nervous system, gastrointestinal, and infectious complications in children because of the unique anatomic and physiologic characteristics of children. This, along with the challenges in obtaining an accurate history and physical examination, makes it important that emergency physicians understand the complexities of SCD in children.

Case Presentation

A 7-year-old boy is brought in by his parents because the child had a seizure at home about 1 hour earlier. The seizure was described as generalized, uncontrolled shaking that lasted about 5 minutes. The patient had been complaining of a headache for several hours that was not relieved by over-the-counter analgesics. There is no fever, history of recent illness, nausea, or vomiting. The patient does not have a history of seizure disorder; his only significant medical history is SCD, although he has not had any problems in the previous year.

His vital signs are blood pressure 95/50, pulse rate 90, respiratory rate 20, temperature 36.7°C (98°F), and pulse oximetry 98% on room air. On physical examination, the child appears tired and is lying quietly on the gurney. He is able to answer questions appropriately. His pupils are equal and reactive to light, and extra-ocular movements are intact. There is no facial asymmetry. There is no nuchal rigidity. His lungs are clear to auscultation; his heart has a regular rhythm and normal rate, and there are no murmurs. His abdomen is nondistended, has normal bowel sounds, and is soft and nontender to palpation. Strength testing reveals mild weakness in the left upper and lower extremities.

CRITICAL DECISION

What diagnostic tests should be ordered?

As with most disease processes, the use of laboratory and radiographic testing is guided by the history and physical examination. However, given the unique nature of the disease in children, emergency physicians should have a low threshold for ordering certain tests.