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Table 1 Recommendation for penicillin prophylaxis

From: Organizing national responses for rare blood disorders: the Italian experience with sickle cell disease in childhood

Recommendation Evidence
Penicillin prophylaxis is strongly recommended for all children with SCD (homozygous SS, SC disease, Sβ °thalassemia) up to 6 years of age. A
Penicillin prophylaxis is recommended in subjects with genotype SC, even if no clear study demonstrates its benefit in this form of SCD. C
Prophylaxis with oral penicillin should be done, but because it is not available in Italy, the alternative drugs described in Table 3 or the parenteral formulation can be used. C
Antibiotic prophylaxis should begin between the second and third months of life in children who received a neonatal diagnosis of SCD. A
In Italy, newborn screening is not provided; therefore, the pediatrician who cares for an infant at high risk of SCD (geographic origin, family history of hemoglobinopathy) should test the infant for the presence of the disease, even in the absence of symptoms, and prescribe prophylactic penicillin by the third month. B
It is controversial whether it is necessary to continue penicillin prophylaxis beyond 5 years of age, although it is certainly more prudent to recommend the continuation of prophylaxis throughout life. C
Long-term prophylactic therapy causes problems of adherence, which may be limited by clear information about the benefits of prophylaxis, the risks of a low adherence, and the involvement of parents in the management of disease and care of the child. C