Women with sickle cell disease (SCD) are more likely to experience pregnancy complications, affecting both the mothers and their babies, according to a new study published in Revista Brasileira de Ginecologia e Obstetrícia.

Investigators from the Department of Obstetrics and Gynecology at the Universidade Estadual de Campinas in Brazil conducted a narrative literature review detailing the various complications that women with SCD experience. Most complications arose in women with the HbSS genotype compared to the heterozygous genotypes (HbSC, HbSβ thalassemia).

Maternal complications included vaso-occlusive crises (VOCs), infections (especially urinary tract infections), thromboembolic complications (such as deep venous thrombosis and stroke), pulmonary complications, preeclampsia, chronic renal failure, cesarian section, and maternal mortality.

VOCs caused the highest rate of morbidity and hospitalization, while pulmonary complications such as acute chest syndrome were more likely to cause maternal death. The most common complication was urinary tract infections followed by VOCs. Fetal complications included growth restrictions, premature birth, low birth weight, distress during labor, placental abnormalities, and perinatal mortality, including stillbirth.

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Recommendations to minimize pregnancy complications for women with SCD included close, frequent monitoring during pregnancy by a multidisciplinary team, encouragement for these women to stay current on their immunizations, and discontinuation of medications. Providers may arrange shorter intervals between follow-ups if they suspect fetal impairment.

Pregnant women with SCD should stop taking iron chelators, angiotensin-converting enzyme inhibitors, and hydroxyurea, the authors said. Other medications like voxelotor are safe during pregnancy, but not during lactation or breastfeeding. Prophylactic blood transfusions may prevent severe chronic anemia during pregnancy which can negatively impact the baby’s growth.

Clinicians commonly use simple transfusions over exchange transfusions, and conflicting recommendations exist regarding the time of initiation of these prophylactic transfusions. Some studies encouraged starting transfusions in the second trimester, while others suggest the third trimester, citing the risk of alloimmunization that coincides with more frequent transfusions.

The literature encourages a baseline urinalysis followed by frequent screens for urinary tract infections and asymptomatic bacteriuria because of such infections, changing body temperature, and dehydration trigger more severe complications like VOCs.

Frequent laboratory assessments should check hemoglobin, reticulocyte, and lactate dehydrogenase levels to inform hematological interventions during pregnancy, as well as liver enzymes and platelet count to diagnose preeclampsia and hemolysis, elevated liver enzymes, and low count syndrome. Following cessation of breastfeeding, providers should assess for osteoporosis and/or osteopenia with a dual-energy X-ray absorptiometry scan.

“The management of pregnancy SCD patients requires a multidisciplinary approach to achieve favorable maternal and fetal outcomes, with accurate and timely diagnosis and treatment of its complications,” the authors concluded.

Reference

Figueira CO, Surita FG, Fertrin K, Nobrega G de M, Costa ML. Main complications during pregnancy and recommendations for adequate antenatal care in sickle cell disease: a literature review. Rev Bras Ginecol Obstet. Published online February 9, 2022. doi:10.1055/s-0042-1742314