Overview
Sickle cell disease (SCD) is a life-threatening genetic disorder affecting nearly 100,000 individuals in the United States, associated with numerous acute and chronic complications requiring prompt medical attention 2.Diagnosis
Annual transcranial Doppler (TCD) examinations are recommended from ages 2 to 16 years in individuals with sickle cell anemia 2.Management
Disease-modifying therapies include hydroxyurea and long-term blood transfusions 2.
Rapid initiation of opioids is recommended for the treatment of severe pain 2.Special Populations
Pediatrics
Daily oral prophylactic penicillin is recommended up to age 5 years 2.
Annual transcranial Doppler examinations are recommended from ages 2 to 16 years in those with sickle cell anemia 2.
Long-term transfusion therapy is recommended to prevent stroke in children with abnormal TCD velocity (≥200 cm/s) 2.Key Recommendations
Daily oral prophylactic penicillin up to the age of 5 years is recommended 2. (Evidence: Strong)
Annual transcranial Doppler examinations from the ages of 2 to 16 years are recommended in those with sickle cell anemia 2. (Evidence: Strong)
Long-term transfusion therapy is recommended to prevent stroke in children with abnormal transcranial Doppler velocity (≥200 cm/s) 2. (Evidence: Strong)References
1 Cantor AG, Jungbauer RM, Skelly AC, Hart EL, Jorda K, Davis-O'Reilly C et al.. Respectful Maternity Care : A Systematic Review. Annals of internal medicine 2024. link
2 Yawn BP, Buchanan GR, Afenyi-Annan AN, Ballas SK, Hassell KL, James AH et al.. Management of sickle cell disease: summary of the 2014 evidence-based report by expert panel members. JAMA 2014. link