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Pregnancy complicated by sickle cell disease is high-risk for both mother and fetus. Surveillance helps manage problems such as vaso-occlusive crises and alloimmunization. Maternal problems can arise from chronic underlying organ dysfunction such as renal disease or pulmonary hypertension, from acute complications of sickle cell disease such as vaso-occlusive crises and acute chest syndrome, and/or from pregnancy-related complications. Fetal problems include alloimmunization, opioid exposure, growth restriction, preterm delivery, and stillbirth. Couples should be counseled that a pregnancy with sickle cell disease is high risk for both fetus and mother and be made aware of the increased risks of adverse pregnancy outcome. Risks of adverse fetal outcomes are reduced but not eliminated with fetal surveillance. This review provides recommendations, screening and clinical management during prenatal and puerperium of patients with sickle cell disease. Genetic screening can identify couples at risk for offspring with sickle cell disease and other hemoglobinopathies and allow them to make informed decisions regarding reproduction and prenatal diagnosis.
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