Death from S. pneumoniae in HbSC Disease: Was Expanded Pneumococcal Vaccination Too Late?

Despite proven efficacy, only 70% of eligible individuals in the U.S. are vaccinated against Streptococcus pneumoniae. This is especially concerning for patients with sickle cell disease (SCD), as they are susceptible to invasive pneumococcal disease owing to functional asplenia. Early and complete vaccination is crucial in preventing pneumococcal sepsis and its complications, including the rare but often fatal fat embolism syndrome. Here, we report a case of an adolescent male with Hemoglobin SC (HbSC) disease who presented to our pediatric emergency department (ED) with abdominal and back pain. Notably he had received all vaccinations according to published guidelines, however had not yet received the expanded pneumococcal vaccine. He was in his usual state of health the previous day. He arrived to the ED mildly febrile, tachypneic, and tachycardic with altered mental status. His condition rapidly deteriorated into acute respiratory failure, coagulopathy, and multiorgan dysfunction syndrome. Unfortunately, he succumbed following multiple cardiac arrests less than 12 hours into his illness course. This case highlights to pediatric and adolescent providers that invasive pneumococcal disease remains a significant risk for vaccinated, adolescent patients, even with mild SCD genotypes. Moreover, it underscores the critical importance of early, complete pneumococcal vaccination in SCD patients. The newer expanded pneumococcal vaccines could enhance protection for this vulnerable population.