| 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 overloadΔ | - 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
|
AlloimmunizationΔ | - 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
|