Know Who Should Get Penicillin for Sickle Cell Disease

Ensure optimal penicillin prophylaxis in pediatric sickle cell disease (SCD). Learn when to start, how to improve adherence, and when to deprescribe.

2 minute read

Overview

Penicillin prophylaxis for pediatric sickle cell disease (SCD) needs your attention, since adherence is low and some kids no longer need it. About 100,000 US patients have SCD. The sickled red blood cells can lead to splenic infarction, impairing its immune functions and increasing risk for invasive pneumococcal disease.

Penicillin prophylaxis for 15 months prevents pneumococcal infections in 1 in 10 patients with SCD and may improve survival. But fewer than 1 in 3 patients with SCD are adherent to prophylactic antibiotics. And meds are often continued unnecessarily.

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Clinical practice

Recognize when to start prophylaxis

Prescribe penicillin for patients with HbSS and HbS thalassemia beta zero SCD genotypes or for other genotypes if the patient had a splenectomy.
Confirm your patient’s genotype with their hematologist if you’re unsure, and review their surgical history.
Begin penicillin by 2 months of age, and continue antibiotics until at least 5 years old.
Choose oral penicillin V suspension first-line, it has the strongest evidence for prophylaxis. Use 125 mg twice daily for ages less than 3 years old and 250 mg twice daily for older patients.
Consider amoxicillin 10 mg/kg/dose (max 250 mg/dose) twice daily as an alternative, some kids don’t like the taste of penicillin suspension.
Reserve oral erythromycin for cases of a true penicillin allergy and use the same dosing as penicillin V.

Emphasize adherence and its importance

Tell patients to expect frequent refills, reconstituted antibiotics can expire quickly.
Look into using penicillin or amoxicillin tabs as alternatives for convenience or during shortages. Their longer expiration makes refills less frequent and they can be split and crushed if needed.
Assess for deprescribing opportunities. In general, stop penicillin at age 5, since acquired immunity should increase as patients get older.
Also ensure patients are current on their pneumococcal vaccine series before discontinuing their antibiotic.
Watch for exceptions where patients older than 5 should continue prophylaxis if they have high infection risk. This includes patients with prior invasive pneumococcal disease or if they had a splenectomy.
Defuse hesitation to stop prophylaxis for low-risk patients past 5 years old. Evidence suggests continuing prophylaxis for them isn’t associated with improved infection outcomes.

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References

U.S. Department of Health and Human Services. Evidence-Based Management of Sickle Cell Disease. Expert Panel Report, 2014. https://www.nhlbi.nih.gov/sites/default/files/media/docs/sickle-cell-disease-report.pdf.
Eastep TG, Kendsersky RM, Zook J, Moore A. Penicillin Prophylaxis in Patients With Sickle Cell Disease Beyond Age 5 Years. J Pediatr Pharmacol Ther. 2023;28(6):519-523.
Hirst C, Owusu-Ofori S. Prophylactic antibiotics for preventing pneumococcal infection in children with sickle cell disease. Cochrane Database Syst Rev. 2012;(9):CD003427.
McCavit TL, Gilbert M, Buchanan GR. Prophylactic penicillin after 5 years of age in patients with sickle cell disease: a survey of sickle cell disease experts. Pediatr Blood Cancer. 2013;60(6):935-939.

This note has been edited and reviewed by the pharmacy doctors on NPS team.