The CBC and the reticulocyte count are the two most important tests that should be considered when treating an SCD patient with complications.

When a pediatric SCD patient with pulmonary complaints presents, the emergency physician should obtain a chest radiograph. The presence of cough, fever, wheezing, or shortness of breath should prompt a search for pneumonia, pulmonary infarct, and acute chest syndrome. Other studies should be ordered based on the clinical presentation. Given the susceptibility to encapsulated bacteria, emergency physicians should have a low threshold for ordering a urinalysis, blood cultures, and a lumbar puncture. CT scanning should be obtained in the SCD child presenting with a headache or a neurologic deficit.

CRITICAL DECISION

Does the patient require analgesics and/or antibiotics?

Immediate and aggressive analgesia (typically in the form of parenteral narcotics) is the mainstay of treatment for SCD patients presenting to the emergency department and is the required intervention in the patient suspected of experiencing a painful crisis. During the course of the emergency department visit, the patient’s pain level should be regularly assessed to ensure adequacy of analgesia. A number of pain assessment tools for children exist (visual analog scale, numeric rating scale, Wong-Baker face scale). The choice of tool depends on factors such as age, cognitive ability, and emotional state.¹

Acetaminophen and nonsteroidal anti-inflammatory drugs (NSAIDs) can be used for the management of mild to moderate pain. Opioids are considered to be the drugs of choice for more severe episodes of VOC pain. There are no studies that demonstrate superiority of one drug over another. Oral agents such as codeine (0.5-1 mg/kg), oxycodone (0.05-0.15 mg/kg), and hydromorphone (0.03-0.08 mg/kg) may be tried. For severe pain, parenteral narcotics are indicated. Morphine is one of the most frequently used parenteral narcotics in this setting.

The starting dose is 0.1 to 0.15 mg/kg; it may be given intravenously, intramuscularly, subcutaneously, or rectally. The patient should be reassessed frequently; repeated doses can be administered until adequate pain control is achieved or until sedation or respiratory depression approaches an unacceptable level. Additional adverse effects include rash, pruritus, hypotension, and nausea and vomiting. Meperidine had been used commonly as an analgesic for SCD. However, of all the opioids, it likely has the least favorable pharmacologic profile. Given the availability of other, equally effective, narcotics, emergency physicians should avoid meperidine.

As SCD patients are at high risk for infection by organisms such as Pneumococcus, Staphylococcus species, Haemophilus influenzae, and Salmonella, and as children with SCD have a several hundred-fold higher risk for sepsis than healthy children, one must have a low threshold for administering appropriate antibiotics. A careful history and physical examination coupled with appropriate diagnostic studies will guide the choice of antibiotics.

CRITICAL DECISION

Which patients require blood transfusions?

Transfusion in the acute setting is used to treat significant and symptomatic anemia and to reduce or prevent complications.³ Severe anemia usually occurs in the setting of aplastic crisis and hyperhemolytic syndrome. In general, SCD patients exhibiting signs (severe tachycardia, hypotension) or symptoms (dyspnea, syncope, severe pain) along with a significant drop in hemoglobin or an absolute hemoglobin below 6 g/dL will benefit from simple red cell transfusion. Exchange transfusion (in which the patient’s abnormal RBCs are removed and replaced with normal RBCs) is generally accepted for the treatment for acute chest syndrome, stroke, intrahepatic cholestasis, acute splenic sequestration, and multiorgan failure.³ Although not well studied, it is also used for the treatment of priapism that is unresponsive to pharmacologic therapy. All transfusions should be done in consultation with a hematologist.

CRITICAL DECISION

Who requires admission to the hospital?

In general, children who present to the emergency department with complications of SCD should be admitted to the hospital. This is because of the high risk for associated sepsis/infection, anemia, and acute chest syndrome. Additionally, the difficulties inherent in obtaining an accurate history and a reliable physical examination in children make it important that the emergency physician have a low threshold for admitting these patients. The patient with typical, uncomplicated VOC with adequate pain control in a timely fashion in the emergency department (typically an older child with the ability to communicate effectively) may be discharged. Those discharged from the emergency department must have a supportive home environment and close followup care.

Case Resolution

In the case of the boy who had a seizure, an emergent CT of the head showed an area of hypodensity in the right parietal lobe. There was no evidence of hemorrhage. Both a pediatric hematologist and a pediatric neurologist were consulted. Because of the patient’s neurologic symptoms, arrangements were made for an exchange blood transfusion, and he was admitted to the pediatric ICU.