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Clinical Summary

Fever in a child with sickle cell disease (SCD) is a medical emergency, because of the possibility of overwhelming Streptococcus pneumoniae sepsis. By age 1 year, 30% to 50% of infants with homozygous sickle cell anemia (HbSS) have diminished or absent spleen function, more than 90% by age 4 years. Though splenic hypofunction occurs less commonly and at a later age in children with hemoglobin SC (HbSC) and sickle β0-thalassemia, they should also be considered at risk. Although availability of conjugated pneumococcal vaccines (most recently Prevnar 13) has reduced the prevalence of invasive pneumococcal disease, prompt empiric therapy with an appropriate antibiotic remains critically important. To some extent, use of Prevnar 7 resulted in emergence of nonvaccine, penicillin-sensitive serotypes as causes of invasive disease, and children less than age 5 should be receiving prophylaxis with penicillin. Patients with pneumococcal bacteremia are often well-looking for a period of several hours prior to sudden circulatory collapse and death; the presence of otitis media or other localized infection does not exclude the possibility of bacteremia. Children with high fever (T >40°C) and/or headache may require lumbar puncture. Although children with acute chest syndrome (ACS) may present with obvious respiratory distress, presentation may be subtle, and careful clinical and radiographic assessment is required. Moreover, although parents are advised to bring children to the hospital because of risk of fulminant sepsis, the fever is often due to viral illness.

Figure 11.1 ▪ Sickle Cell Anemia.

Fever, pain and swelling with erythema can be seen in both osteomyelitis and vaso-occlusive crisis (VOC) usually due to bone marrow infarction; clinical differentiation between the two may be difficult. (A) Osteomyelitis of the humerus with erythema, swelling, and fever were seen in this child with SCD. (B) Swelling of the elbow and forearm with pain, fever and similar signs of inflammation were seen in a different child with VOC. (Photo contributor: Binita R. Shah, MD.)

Emergency Department Treatment and Disposition

Children presenting with fever should be urgently triaged and immediately seen by a physician. After brief examination, blood should be obtained for a complete blood count (CBC) with a reticulocyte count, blood culture, and any other indicated tests and, if there is no allergy, a dose of a long-acting cephalosporin (eg, ceftriaxone) should be administered intravenously (IV). If penicillin resistance is common in the community, and especially if meningitis is present, treatment with vancomycin is recommended. Patients should then be observed carefully for at least 2 hours as laboratory results are retrieved and any additional evaluation completed. All patients should have a chest x-ray as part of their assessment. Even immunized children taking penicillin should be managed urgently and receive empiric therapy. Children with high fever, pain, and/or ACS should be ...

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