Sickle cell disease maintenance

In part 1 of this 2 part series on sickle cell disease, we’re going to discuss the general pediatric management of a patient with sickle cell disease including what special precautions and additional routine health maintenance they need. 

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This episode was written by pediatricians Tammy Yau and Lidia Park with content support from Anjali Pawar (UC Davis pediatric hematologist). Pediatricians Tammy and Lidia take full responsibility for any errors or misinformation. 

Key Points:

• Patients with sickle cell disease should receive penicillin prophylaxis from 2 months old til 5 years old or until pneumococcal vaccine series is completed 
• For patients with HbSS or sickle beta zero thalassemia, offer hydroxyurea at 9 months of age, even if they don’t have clinical symptoms. They should also receive stroke risk screening with an annual transcranial doppler
• Patients with sickle cell disease should receive annual screening for retinopathy and nephropathy around age 10
• Patients with sickle cell disease should receive an additional pneumococcal (20 or 23) vaccine and the meningococcal ACWY vaccine at age 10 and men B after age 10 if they have functional asplenia or a splenectomy

Sources:

Pediatrics 2024, A. Yates. https://doi.org/10.1542/peds.2024-066842