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(Photo contributor: Binita R. Shah, MD)

*The authors acknowledge the special contributions of Sreedhar P. Rao, MD, and Shahina Qureshi, MD, to prior edition.


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, with this rate being >90% by age 4 years. The prevalence of splenic dysfunction is likely similar in children with sickle β0-thalassemia, and although splenic hypofunction occurs less commonly and at a later age in children with hemoglobin SC (HbSC) and sickle β+-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 age <5 years 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 (temperature > 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. Parents are advised to bring febrile children with SCD for evaluation of sepsis because of their increased risk despite the fact that majority of such febrile episodes may not be bacterial in origin.


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 2 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 an ED provider. After brief examination, blood should be obtained for a CBC with a reticulocyte count, blood culture, and any other indicated tests, and if there is no known allergy, a dose of a ...

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