Sickle Cell Disease

Women with sickle cell disease are at increased risk for miscarriage, preterm labor, and other complications due to impaired oxygen supply and sickling infarcts in the placental circulation. Pregnant women are at increased risk for vascular occlusive events, particularly during the third trimester and the postpartum period. Painful crises present similarly in the pregnant and the nonpregnant state. Treatment of painful crises in pregnancy is similar to that for nonpregnant patients. Cornerstones of management include aggressive hydration and analgesic therapy. Both oral and intravenous narcotics can be used. Caution should be exercised when using nonsteroidal antiinflammatory agents, particularly after 32 weeks of gestation, because of oligohydramnios and a significant risk of premature closure of the fetal ductus arteriosus. Hydroxyurea should not be used in pregnancy because of its teratogenic effects. Sickle cell crisis that does not respond to conservative management is commonly managed using partial exchange transfusion via automated erythrocytapheresis. Simple transfusion alone can be helpful when the hemoglobin level is <6g/dL.9 The incidence of fetal death in utero can be minimized during sickle cell crisis by using information from continuous electronic fetal monitoring in a woman with a potentially viable fetus. While fetal heart rate patterns are frequently nonreassuring during the acute episode of vasoocclusive crisis, fetal heart rate patterns tend to normalize as the crises improve. In utero resuscitation (oxygen, intravenous hydration, left lateral uterine displacement, fetal scalp manipulation) should be initiated prior to consideration of emergency delivery.

While the majority of sickle cell crises in pregnancy are vasoocclusive crises, aplastic crises present a unique problem as they are usually caused by parvovirus B19 infection, which has been associated with hydrops fetalis. This disease thus presents a risk to both the pregnant woman and her fetus. Careful sonographic fetal assessment should be performed to evaluate the fetus for evidence of parvovirus infection. Once patients with sickle cell disease has been exposed to parvovirus B19, they are unlikely to acquire the infection a second time due to acquired immunity.10 Then the pregnant patient with a past history of aplastic crisis is unlikely to experience it again.

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