Summary of recommendations for SCD in pregnancy
| . | Possible problem . | Management . |
|---|---|---|
| Before conception | Iron overload | Check ferritin level before prescribing any iron. If ferritin is elevated, prescribe prenatal vitamins without iron (prenatal gummy vitamins) instead. Prescribe folic acid. ACOG recommends 4 mg per day. |
| Hypertension | Screen for hypertension and treat to lower systolic blood pressure to ≤140 and diastolic blood pressure to ≤90. | |
| Sickle cell nephropathy | Perform a first morning void urine albumin-to-creatinine ratio and, if abnormal, consult with or refer to a nephrologist. | |
| Proliferative sickle retinopathy | Refer to an ophthalmologist for a dilated eye examination if none performed within the last year. | |
| Pulmonary hypertension | Consider screening Doppler echocardiography every 1 to 3 years. Refer any woman with disordered breathing or symptoms of pulmonary embolism for Doppler echocardiography. | |
| Functional asplenia | Vaccinate against pneumococcus, H influenzae type b, and meningococcus. Update other immunizations. | |
| Alloimmunization | Test for red cell antibodies. If positive for antibodies known to cause hemolytic disease, test for corresponding antigen(s) in the father. If he is positive, counsel about risks in a pregnancy. | |
| ACE inhibitors | Discontinue before conception | |
| Fetal hemoglobinopathy | If father’s status is unknown, refer for testing. If father has SCD, HbS trait, β-thalassemia trait, or is a carrier of another abnormal Hb such as HbC, refer for genetic counseling. Preimplantation genetic testing is possible with in vitro fertilization. | |
| During pregnancy | Issues not addressed earlier | Address as soon as possible. |
| HU | Discontinue if not already discontinued. Whether and when to restart HU requires careful consideration and discussion with the patient. See the text. | |
| Chelation agents | Discontinue | |
| High risk for preeclampsia | Start low-dose aspirin starting at 12 wk gestation. | |
| Severe anemia | Monthly CBC | |
| Infection | Urine culture every 1 to 3 mo | |
| Adverse pregnancy outcome | Anticipate serial ultrasonography for fetal growth every 3-4 wk starting at 28 wk gestation; antepartum fetal heart rate testing starting at 32 wk gestation; and delivery at 37 wk gestation for SS genotype or severe phenotype. By 39 wk for others. | |
| Cesarean delivery | Consider preoperative transfusion to increase Hb levels to 10 g/dL. | |
| History of stroke | Low-dose anticoagulation (enoxaparin, 40 mg per day) in addition to low-dose aspirin. | |
| History of VTE | Full-dose anticoagulation with low–molecular weight heparin (enoxaparin, 1 mg per kg every 12 h). | |
| VTE prophylaxis | Low-dose anticoagulation with low–molecular weight heparin (enoxaparin, 40 mg daily) (1) during hospitalizations for vaso-occlusive crises and continuing throughout the remainder of the pregnancy and (2) starting 12 h after a vaginal delivery and 24 h after a cesarean delivery and continuing for 6 wk postpartum. | |
| Vaso-occlusive crises | Evaluate for other complications. Prescribe prompt and aggressive opioid analgesia. Avoid NSAIDs. Transfuse only if there are other indications. Administer oxygen if the oxygen saturation is <95% by pulse oximetry. Initiate incentive spirometry for patients who are hospitalized. |
| . | Possible problem . | Management . |
|---|---|---|
| Before conception | Iron overload | Check ferritin level before prescribing any iron. If ferritin is elevated, prescribe prenatal vitamins without iron (prenatal gummy vitamins) instead. Prescribe folic acid. ACOG recommends 4 mg per day. |
| Hypertension | Screen for hypertension and treat to lower systolic blood pressure to ≤140 and diastolic blood pressure to ≤90. | |
| Sickle cell nephropathy | Perform a first morning void urine albumin-to-creatinine ratio and, if abnormal, consult with or refer to a nephrologist. | |
| Proliferative sickle retinopathy | Refer to an ophthalmologist for a dilated eye examination if none performed within the last year. | |
| Pulmonary hypertension | Consider screening Doppler echocardiography every 1 to 3 years. Refer any woman with disordered breathing or symptoms of pulmonary embolism for Doppler echocardiography. | |
| Functional asplenia | Vaccinate against pneumococcus, H influenzae type b, and meningococcus. Update other immunizations. | |
| Alloimmunization | Test for red cell antibodies. If positive for antibodies known to cause hemolytic disease, test for corresponding antigen(s) in the father. If he is positive, counsel about risks in a pregnancy. | |
| ACE inhibitors | Discontinue before conception | |
| Fetal hemoglobinopathy | If father’s status is unknown, refer for testing. If father has SCD, HbS trait, β-thalassemia trait, or is a carrier of another abnormal Hb such as HbC, refer for genetic counseling. Preimplantation genetic testing is possible with in vitro fertilization. | |
| During pregnancy | Issues not addressed earlier | Address as soon as possible. |
| HU | Discontinue if not already discontinued. Whether and when to restart HU requires careful consideration and discussion with the patient. See the text. | |
| Chelation agents | Discontinue | |
| High risk for preeclampsia | Start low-dose aspirin starting at 12 wk gestation. | |
| Severe anemia | Monthly CBC | |
| Infection | Urine culture every 1 to 3 mo | |
| Adverse pregnancy outcome | Anticipate serial ultrasonography for fetal growth every 3-4 wk starting at 28 wk gestation; antepartum fetal heart rate testing starting at 32 wk gestation; and delivery at 37 wk gestation for SS genotype or severe phenotype. By 39 wk for others. | |
| Cesarean delivery | Consider preoperative transfusion to increase Hb levels to 10 g/dL. | |
| History of stroke | Low-dose anticoagulation (enoxaparin, 40 mg per day) in addition to low-dose aspirin. | |
| History of VTE | Full-dose anticoagulation with low–molecular weight heparin (enoxaparin, 1 mg per kg every 12 h). | |
| VTE prophylaxis | Low-dose anticoagulation with low–molecular weight heparin (enoxaparin, 40 mg daily) (1) during hospitalizations for vaso-occlusive crises and continuing throughout the remainder of the pregnancy and (2) starting 12 h after a vaginal delivery and 24 h after a cesarean delivery and continuing for 6 wk postpartum. | |
| Vaso-occlusive crises | Evaluate for other complications. Prescribe prompt and aggressive opioid analgesia. Avoid NSAIDs. Transfuse only if there are other indications. Administer oxygen if the oxygen saturation is <95% by pulse oximetry. Initiate incentive spirometry for patients who are hospitalized. |
ACOG, American College of Obstetricians and Gynecologists; NSAIDs, nonsteroidal anti-inflammatory drugs.