Multidisciplinary care for the pregnant sickle cell disease patient

Sickle cell disease (SCD) in pregnancy raises the risk to mother and baby as well as complications of SCD, but working closely with the patient’s hematologist and a maternal-fetal medicine specialist improves the chances of an uneventful pregnancy and delivery.

Sickle cell disease (SCD) in pregnancy raises the risk to mother and baby as well as complications of SCD, but working closely with the patient’s hematologist and a maternal-fetal medicine specialist improves the chances of an uneventful pregnancy and delivery.

Early prenatal care and regular monitoring throughout the pregnancy are especially important for SCD patients.

“Pregnant patients with SCD can develop more severe symptoms and are at a higher risk of preterm labor, low birth weight, preeclampsia, venous thromboembolism, infections, intrauterine growth restriction, perinatal and maternal mortality,” said Mureena A. Turnquest Wells, M.D., OB/GYN with Norton Children’s Maternal-Fetal Medicine. If there are no contraindications, low dose aspirin should be given from the beginning of the second trimester to reduce the risk for preeclampsia

Pregnant SCD patients are more likely to experience anemia and painful crises than at other times, and nearly half of these patients have at least one hospital admission during pregnancy, according to the National Heart Lung, and Blood Institute 2014 Expert Panel Report.

Exams every two to three weeks during the second trimester and weekly during the third trimester may be indicated.

Hydroxyurea can be effective for preventing recurrent pain crises or acute chest syndrome, but isn’t advised for routine use in pregnant SCD patients as it is suspected to increase the risk of birth defects. Hydroxyurea is contraindicated for breastfeeding. Partial exchange transfusion therapy is an option in some cases, particularly if hemoglobin is less than 6gm/dl.

Hydration, oxygen and pain management for vaso-occlusive crises

The cornerstone of managing painful vaso-occlusive crisis include hydration, oxygen therapy and pain management. The major objective is to end the pain. Some patients may need chronic opioid therapy, which can increase the risk of neonatal withdrawal.

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Due to the risk for fetal growth restriction, serial growth ultrasound studies should be done in the second half of the pregnancy. Fetal well-being should also be monitored in the third trimester with nonstress test or biophysical profile because of the increased risk for stillbirth.

All women with sickle cell anemia should receive folic acid supplementation of 5 mg daily. Iron deficiency anemia is not common in women with SCD and should be confirmed by low ferritin level before supplemental iron is given.

Given that the pregnant patient with SCD has usually had multiple transfusions, there is a high rate of alloimmunization. These antibodies can result in fetal anemia and can make cross matching for maternal blood transfusion more difficult.

Delivery and postpartum considerations

There are no contraindications to vaginal birth in the patient with SCD. Cesarean section should be reserved for the usual obstetrical indications. During labor the patient should be kept well hydrated, well oxygenated (maintain oxygen saturation at or above 95%) and warm in order to prevent a crisis.

Close surveillance in the postpartum period is critical because the risk of thromboembolism is greatest during this period. There are recommendations for pharmacologic thromboprophylaxis, particularly in the patient who has undergone cesarean section.

Breast feeding should be encouraged unless the patient plans to resume or begin treatment with hydroxyurea. Combined estrogen-progestin contraception has not been shown to increase the risk for complications and can be used in women with SCD if there are contraindications for its use.

The newborn should be screened for SCD.

Blood transfusions in pregnant patients with SCD are indicated in the following cases, according to a 2016 paper in Hematology.

  • Acute anemia
  • Acute ischemic stroke
  • Acute pain
  • Acute chest syndrome
  • Acute priapism
  • Multiorgan failure, acute sickle cell hepatopathy, severe sepsis

Transfusions to maintain a hematocrit of more than 36% do not reduce complications of pregnancy, according to the Centers for Disease Control and Prevention.


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