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Pregnancy management of sickle cell disease Preconception counseling

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 Referral to maternal‐fetal medicine to discuss maternal/fetal risks during pregnancy.

 Paternal screening (hemoglobin electrophoresis and/or alpha‐thalassemia genetic screening) for risk assessment of fetal genetic inheritance.

 Discontinuation of hydroxyurea and ACE inhibitors prior to conception.

 Evaluate for chronic opioid use; counsel accordingly regarding the risk of neonatal abstinence syndrome.

 Maternal screening for end‐organ damage and baseline lab assessment including CBC, reticulocyte counts, liver function tests, renal function tests, type and screen, rubella and varicella immunity screening, EKG, and echocardiogram (if numerous crises/history of acute chest syndrome).

 Discuss maternal immunization as SCD causes patients to be functionally asplenic. Patients with SCD should receive the following (in conjunction with SCD provider).Haemophilus influenzae type B (Hib) vaccine: one dose during their lifetime.Meningococcal vaccine: two‐dose series at least eight weeks apart initially and revaccination every five years.Pneumococcal vaccine: one dose of PCV13 followed by one dose of PPSV23 at least eight weeks later. Repeat PPSV23 five years after initial PPSV23.Yearly influenza vaccine.

 Ensure patient has established care with a SCD provider/hematologist for chronic disease care and health maintenance.

 Recommend folic acid supplementation (4 mg/day).

Protocols for High-Risk Pregnancies

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