Keywords

hereditary spherocytosis, hemolytic anemia, splenectomy, pediatrics

 

Authors

  1. Schuettge, Anna Gould MSN, CPNP-PC

Abstract

Abstract: This literature review seeks to provide an overview of the symptoms, surgical management, infection risk, and long-term care of children with hereditary spherocytosis (HS). HS is a hemolytic anemia, ranging in severity from carriers who are asymptomatic to anemic and hyperbilirubinemic patients requiring splenectomy. The gold standard of surgical management for HS is total splenectomy, which potentiates infection risk, particularly that of overwhelming post-splenectomy infection. Another surgical option is partial splenectomy, which may conserve some immune function but threatens return of disease symptoms. Infection risk is managed with a vaccine regimen and prophylactic antibiotics for total or partial splenectomy. Long-term care and education remain essential for the HS patient's well-being post-splenectomy.