Reproductive counseling

Background
The CDC and its partners released a set of recommendations and goals for preconception health. They recommend that women and couples think about their goals for having or not having children and how to achieve these goals, known as a “reproductive life plan.” These recommendations apply to all women and couples, but, given the increased risk of adverse pregnancy outcomes in SCD and the risk of maternal morbidity and mortality, the expert panel determined that several recommendations were particularly relevant for women with SCD and their partners. The “Recommendations” section delineates these.

Heritability In Men and Women With SCD
People with SCD are at risk for having a child affected with SCD if their partners have SCD, §-thalassemia trait, or are carriers of other abnormal hemoglobin’s such as HbC. Women whose partners carry one of these traits can avoid an affected pregnancy by undergoing preimplantation genetic diagnosis (PGD). PGD is testing performed on an embryo during an in-vitro fertilization cycle (see http://www.acoR.ore /mcdimFor*/ 20Patients/faU 179.pdf?dmc= 1&ts=20l307l8T 1252201251). Alternatively, after spontaneous conception, prenatal diagnosis of SCD is possible by chorionic villus sampling in the first trimester or by amniocentesis in the second trimester of gestation.

Fetal Anemia Due to Alloimmunization
Women with SCD are frequently exposed to blood products. The fetuses of women who are alloimmunized are at risk of significant hemolytic anemia or mortality.

Summary of the Evidence

Adverse Fetal Outcomes
Multiple case series and two population studies have documented increased risk of growth restriction, preterm delivery, and stillbirth among women with SCD. Fetal surveillance, which includes growth ultrasounds and antepartum testing (nonstress tests, biophysical profiles, and contraction stress tests), may lead to planned early delivery and can reduce but not eliminate risks.

Risks to the Mother
Compared to women without SCD, women with SCD are more likely to experience preeclampsia,
venous thromboembolism, infections, and maternal mortality during pregnancy. During pregnancy, 40-50 percent of women with SCD require at least one hospital admission.
Although there are no data specifically for women with SCD, the presence of pulmonary hypertension increases the cardiopulmonary demands of gestation. Non-SCD maternal mortality has been reported to be as high as 3W50 percent in women with pulmonary hypertension. Even with current multidisciplinary care, maternal mortality in women with pulmonary hypertension is still reported to be 10 percent.

Recommendations

Evidence reviews on this topic were not performed by the methodology team. The expert panel based its recommendations on a review of the literature and consensus opinion.
Specific Recommendations for Women or Men With SCD
1. Encourage each woman, man, and couple affected by SCD to have a reproductive life plan.
(Consensus—Panel Expertise)
2. As a part of primary care visits, provide risk assessment and educational and health promotion counseling (or refer to individuals with expertise in these disciplines) to all women and men of childbearing age to reduce reproductive risk and improve pregnancy outcomes. Provide contraceptive counseling, if desired, to prevent unintended pregnancy, and if pregnancy is desired, provide preconception counseling.
(Consensus—Panel Expertise)
3. If the partner of a man or woman with SCD has unknown SCD or thalassemia status, refer the partner for hemoglobinopathy screening.
(Consensus—Panel Expertise)
4. After testing, refer couples who are at risk for having a potentially affected fetus and neonate for genetic counseling.
(Consensus—Panel Expertise)
Specific Recommendations for Women With SCD
1. Test women with SCD who have been transfused and are anticipating pregnancy for red cell alloantibodies. (Consensus—Panel Experfise}
2. If a woman has red cell alloantibodies, test her partner for the corresponding red cell antigen(s).
(Consensus—Panel Expertise}
3. If the partner tests positive for the corresponding red cell antigen(s), counsel the woman and her partner about the risks of hemolytic disease in the fetus and neonate, how it is monitored, and how it is treated, or refer them to a maternal-fetal specialist who can provide this education.
(Consensus—Panel Expertise)
4. Counsel women with SCD and their partners or refer for counseling about the following:
(Consensus—PanelExperfise}
a. Pregnancy in women with SCD is considered high risk, and there is an increased risk of adverse pregnancy outcomes including fetal (intrauterine) growth restriction, preterm delivery, and stillbirth.
b. Additional fetal surveillance is required during a pregnancy.
c. There are increased risks to a woman’s health during pregnancy. These risks include an increased frequency of pain crises and an increased risk of thrombosis, infections, preeclampsia, and death relative to women who do not have SCD.
For women who require chronic opioid therapy during pregnancy, there is an increased risk of neonatal withdrawal in their newborns.