From the Guidelines
Penicillin prophylaxis is strongly recommended for patients with Sickle Cell Disease (SCD) under 5 years of age to prevent invasive pneumococcal disease, a leading cause of morbidity and mortality in this population [ 1 ].
Rationale
The rationale for penicillin prophylaxis in this population is based on the high risk of invasive pneumococcal disease due to splenic dysfunction, low levels of circulating antibodies, and complement deficiency [ 1 ].
Recommended Regimen
The recommended regimen is oral penicillin V, administered at a dose of 125mg twice daily for children under 3 years old, and 250mg twice daily for children between 3 and 5 years old [ 1 ].
Initiation and Duration
Prophylaxis should be initiated as soon as possible after diagnosis and continued until at least 5 years of age, or as directed by a healthcare provider [ 1 ].
Key Points
- Penicillin prophylaxis has been shown to reduce the incidence of pneumococcal bacteremia by 84% compared to placebo [ 1 ].
- Children with SCD still have increased rates of invasive disease despite penicillin prophylaxis, highlighting the importance of adherence to prophylaxis and vaccination recommendations [ 1 ].
- The protective effect of pneumococcal polysaccharide vaccine among SCD patients is not consistent, making penicillin prophylaxis a crucial preventive measure [ 1 ].
From the Research
Rationale for Penicillin Prophylaxis in SCD Patients Under 5 Years
The rationale for penicillin prophylaxis in patients with Sickle Cell Disease (SCD) under 5 years of age is based on the increased risk of life-threatening pneumococcal infection due to absent or non-functional spleens and a decreased immune response 2.
- Children with SCD under the age of five are at a higher risk of pneumococcal infection, with an incidence rate of 10 per 100 patient years of pneumococcal septicaemia in children under the age of three 3, 4, 5.
- The American Academy of Pediatrics recommends the use of penicillin prophylaxis in children with SCD under the age of five and in older children who have had a previous severe pneumococcal infection or have functional/surgical asplenia 2.
- Prophylactic penicillin regimens have been shown to significantly reduce the risk of pneumococcal infection in children with homozygous SCD, with minimal adverse reactions 3, 4, 5.
- The evidence suggests that penicillin prophylaxis is effective in preventing pneumococcal infection in children with SCD under the age of five, but further research is needed to determine the ideal age to safely withdraw penicillin 3, 4, 5.
Key Findings
- The incidence of pneumococcal infection is higher in children with SCD under the age of five 3, 4, 5.
- Penicillin prophylaxis is effective in reducing the risk of pneumococcal infection in children with SCD under the age of five 2, 3, 4, 5.
- The American Academy of Pediatrics recommends penicillin prophylaxis for children with SCD under the age of five 2.
Study Limitations
- The certainty of the evidence for all outcomes was judged to be low due to limitations in the included studies, such as incomplete outcome data and allocation concealment 5.
- Further research is needed to determine the ideal age to safely withdraw penicillin and to assess the impact of discontinuing penicillin prophylaxis at various ages on incidence of infection and mortality 3, 4, 5.