Assessment and prevention of SCD complications during pregnancy

	Before conception	During pregnancy	Delivery and postpartum
Medication management	Discuss whether to continue or discontinue hydroxyurea Stop ACE inhibitors and ARBs Stop iron chelator if taking Start folic acid 4 mg daily	Review use of hydroxyurea Start aspirin for preeclampsia prevention in the second trimester or after 12 weeks Continue folic acid 4 mg daily	Discuss timing to restart hydroxyurea if discontinued Restart other discontinued medications when safe Discuss contraception and preferred methods*
Fetal diagnosis	Confirm diagnosis in mother Test father Review likelihood of SCD in fetus Review alternative reproductive options (IVF with PGT, donor egg or sperm, adoption)	CVS, amniocentesis, or cell-free fetal DNA in maternal blood for single gene NIPT	Cord blood for newborn screen (including hemoglobinopathy testing)
Infectious risk^¶	Update vaccinations Ensure vaccination for encapsulated organisms, especially pneumococcus	Update vaccinations and ensure vaccination for encapsulated organisms (especially pneumococcus) if not done prior to pregnancy; avoid live vaccines during pregnancy Treat any fever as a medical emergency Increase surveillance for asymptomatic bacteriuria (eg, monthly urine culture) Perform infectious disease surveillance as in all pregnancies	Treat any fever as a medical emergency
Pregnancy complications (eg, preeclampsia and fetal growth restriction)	Pregnancy is considered high-risk Baseline testing (blood pressure and urine protein) and blood pressure control if needed	Increased maternal surveillance (blood pressure and urine protein) Use of aspirin is discussed above under medication management Increased fetal surveillance (serial ultrasounds for growth and antepartum testing)	Vaginal delivery is appropriate in most cases; cesarean birth is reserved for obstetric indications Induction between 37 and 39 weeks depending on comorbidities and SCD genotype
Vaso-occlusive pain	Obtain baseline pulse oximetry Review pain plan Start hydroxyurea or regular transfusions if pain episodes are frequent	Maintain hydration Use pain plan to rapidly treat pain episodes Opioids can be used to treat pain during pregnancy Consider hydroxyurea or regular transfusions if needed	Maintain hydration Use pain plan to rapidly treat pain episodes Recognize that epidural anesthesia may reduce pain and stress Prescribe hydroxyurea or regular transfusions if needed Monitor the neonate for opioid withdrawal if chronic opioids have been used
Hypertension and sickle nephropathy	Baseline testing (chemistry panel, kidney function, urine albumin or protein)	Obtain baseline testing if not completed prior to pregnancy Perform frequent surveillance Avoid NSAIDs (or use sparingly between 20 and 30 weeks, and avoid use after 30 weeks)
Iron overload^Δ	Iron studies If severe iron overload is present, delay pregnancy until adequately treated Use prenatal vitamins without iron if iron overload is present Avoid iron chelators during pregnancy	Use prenatal vitamins without iron if iron overload is present	Repeat iron studies Hold chelation therapy until iron studies are done and degree of iron overload is assessed
Alloimmunization^Δ	Testing for alloantibodies, with partner testing for the antigens if implicated in HDFN	Extended RBC phenotypic matching to facilitate transfusion if needed Evaluate for HDFN if clinically relevant alloantibodies are present
VTE prophylaxis	Education about increased VTE risk, signs and symptoms of VTE, and whom to call if symptoms occur	VTE prophylaxis during any hospitalization (other than for labor and birth) unless contraindicated	VTE prophylaxis following vaginal or cesarean birth unless contraindicated; continue for 6 weeks postpartum
Other complications	Obtain baseline FEV₁ Refer to ophthalmology for dilated retinal examination if not done in the past year Individualize approach to baseline echocardiography	Monthly CBC Individualized approach to baseline echocardiography	Incentive spirometry during hospitalization

Counseling should be available prior to conception and throughout the pregnancy and postpartum. It should include discussions of potential maternal and fetal risks and appropriate surveillance and interventions to reduce these risks. Advice listed here is in addition to other routine obstetric care.

SCD: sickle cell disease; ACE: angiotensin converting enzyme; ARB: angiotensin receptor blocker; RBC: red blood cell; NIPT: noninvasive prenatal testing; CBC: complete blood count; STD: sexually transmitted disease; NSAIDs: nonsteroidal antiinflammatory drugs; FEV₁: forced expiratory volume in 1 second; HDFN: hemolytic disease of the fetus and newborn; CVS: chorionic villus sampling; DPMA: depot medroxyprogesterone acetate; IUD: intrauterine device.

* Contraceptive choices include progesterone-only (except DPMA) and the levonorgestrel IUD. Estrogen-containing contraceptives increase VTE risk and the copper IUD increases bleeding risk.

¶ Individuals with SCD are immunosuppressed due to functional asplenia and are at risk of potentially life-threatening sepsis. Any fever in an individual with SCD should be treated as a medical emergency.

Δ Many individuals with SCD develop complications of frequent transfusions including transfusional iron overload and alloimmunization against RBC antigens.

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Assessment and prevention of SCD complications during pregnancy