What is the next best step for a 10-day-old patient with a newborn hemoglobin screen positive for sickle cell disease?

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In the case of a 10-day-old patient with a newborn hemoglobin screen positive for sickle cell disease, initiating antibiotics is the most appropriate next step. Infants who are diagnosed with sickle cell disease are at a significantly increased risk of developing severe infections, particularly from encapsulated organisms like Streptococcus pneumoniae.

The standard preventive measure for these infants is the initiation of prophylactic penicillin therapy. This is typically started at around 2 months of age; however, in newborns diagnosed with sickle cell disease, beginning early intervention to mitigate infection risk is critical, even before they reach the two-month mark.

This step is vital because the mortality risk from infections in infants with sickle cell disease, if not managed properly, can be substantial. Initiating antibiotics helps provide a protective barrier against potentially life-threatening infections, thereby improving the overall health outcomes for the infant.

While blood transfusions may be used in the management of sickle cell disease, they are typically not warranted at this early stage for an asymptomatic infant who has only received a positive newborn screening. Bone marrow biopsy is not indicated in this context, and observation alone is insufficient considering the acute risks associated with sickle cell disease in newborns.

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