Penicillin prophylaxis in children with sickle cell disease

Children who have sickle cell disease and are under the age of five years are at increased risk of life-threatening pneumococcal infection due to absent or non-functional spleens and a decreased immune response. To prevent pneumococcal infection, the American Academy of Pediatrics recommends the use of penicillin prophylaxis in children with sickle cell disease under the age of five and in older children who have had a previous severe pneumococcal infection or have functional/surgical asplenia. These recommendations are based on two landmark studies, the first evaluating the effectiveness of penicillin prophylaxis and the second evaluating the duration of prophylaxis. Although the mortality rate from infection has been reduced following penicillin prophylaxis, altered immunologic response and penicillin-resistant S. pneumoniae remain a concern. This paper will review the literature that supports the use of penicillin prophylaxis, potential problems associated with prolonged therapy and recommendations for prophylaxis.