Introduction: Children with sickle cell disease (SCD) remain at higher risk for invasive infection with Streptococcus pneumoniae compared to the general pediatric population. Penicillin prophylaxis, pneumococcal conjugate (PCV), and polysaccharide vaccines (PPSV) have reduced the incidence of pneumococcal disease. Methods: A single institution cohort of children with SCD aged <19 years was reviewed over the 14-year period after 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: The cohort included 4,356 children with SCD (24,076 person-years). Thirty-eight pneumococcal bacteremia cases were identified (32 HbSS, 5 HbSC, 1 HbSβ +-thalassemia), 21 (55%) in children age ≥5 years. The median time to culture positivity was 10.6 hours (range 3.4–20.2) from collection. Meningitis occurred in 4 (11%) and acute chest syndrome in 13 (34%). Serotype information was available for 36 (95%) isolates, which included 16 (44%) PPSV23 serotypes and 1 (2.6%) PCV13 serotype (serotype 3). Penicillin nonsusceptibility occurred in 12/31 (39%) at meningitis and 1/31 (3%) at non-meningitis breakpoints. Three (8%) deaths occurred (serotypes 12F, 23B, and 15B), all in children age ≥5 years, who had discontinued prophylactic penicillin. Long-term sequelae occurred in 5 (14%) surviving children, including hearing loss, limb amputation, motor and neurocognitive defects. Conclusion: Pneumococcal bacteremia continues to occur in children with SCD, with a risk of rapid progression to severe disease. Pneumococcal prevention strategies and urgent empiric treatment for fever remain important for children and adolescents of all ages with SCD.

Background: Patients with sickle cell disease (SCD) experience painful vaso-occlusive episodes that increase with age; a subset develop chronic pain (CP). CP is usually managed with acute pain management guidelines despite evidence of ineffectiveness. Buprenorphine, a partial opioid agonist, a potent analgesic with less liking and has a respiratory ‘ceiling effect’. Buprenorphine therefore provides an alternative ‘harms reduction’ approach for CP management in pediatric SCD patients. Methods: This single urban center retrospective study assessed the feasibility of inpatient transition to buprenorphine-containing analgesics in adolescents with SCD and CP. Patients aged 12-20 years transitioned from FOA to buprenorphine between December 2020 and September 2022 were included. Acute care utilization, hospital length of stay, and FOA use in both inpatient and outpatient settings were compared pre- and post-buprenorphine induction for at least 6 months. Results: Fourteen adolescents with SCD underwent inpatient buprenorphine induction and maintenance therapy. Inpatient transition using a micro-induction approach was feasible and well tolerated in this population. There were low rates of adverse events such as opioid withdrawal signs. Maintenance on buprenorphine products was sustainable over the one-year post-induction period. Three patients (21.4%) discontinued buprenorphine during maintenance therapy. There was a significant reduction (p<0.05) in acute care utilization, length of stay, and FOA use (both inpatient and outpatient). Conclusion: Inpatient micro-induction to buprenorphine from FOA in adolescent SCD patients with CP is feasible with minimal signs of opioid withdrawals. This study suggests decreased acute care utilization with buprenorphine.