Clinical Features and Outcomes of Pneumococcal Bacteremia in Children With Sickle Cell Disease in the Pneumococcal Conjugate Vaccine Era

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INTRODUCTION: Children with sickle cell disease (SCD) are at higher risk for invasive infection with Streptococcus pneumoniae compared with the general pediatric population. Penicillin prophylaxis, pneumococcal conjugate (PCV), and polysaccharide vaccines (PPSV) have reduced but not eliminated the risk of pneumococcal bacteremia. METHODS: A single institution cohort of children with SCD aged <19 years was reviewed over the 14-year period since PCV13 licensure (January 2010-December 2023) to identify and characterize the clinical features and outcomes of S. pneumoniae bacteremia, including serotypes and antibiotic susceptibility. RESULTS: -thalassemia), with median age 5.3 years. The median time to culture positivity was 10.6 h (range 3.4-20.2). Meningitis occurred in four (11%) and acute chest syndrome in 13 (34%). Serotype information, available for 36 (95%) isolates, included 16 (44%) PPSV23 serotypes and one (2.6%) PCV13 serotype (serotype 3). Penicillin nonsusceptibility occurred in 12 out of 31 (39%) at meningitis and one out of 31 (3%) at nonmeningitis breakpoints. Three (8%) deaths occurred (serotypes 12F, 23B, and 15B), all in children aged ≥5 years, who had discontinued prophylactic penicillin. Long-term sequelae occurred in five (14%) surviving children, including hearing loss, limb amputation, and motor and neurocognitive defects. CONCLUSION: Pneumococcal bacteremia continues to be a concern in children with SCD, with a risk of rapid progression to severe disease. Pneumococcal prevention strategies, immunizations, and urgent empiric treatment for fever remain important for children and adolescents of all ages with SCD.