Main Complications during Pregnancy and Recommendations for Adequate Antenatal Care in Sickle Cell Disease: A Literature Review

Abstract

Sickle cell disease (SCD) is the most common monogenic disease worldwide, with a variable prevalence in each continent. A single nucleotide substitution leads to an amino-acid change in the β-globin chain, altering the normal structure ofhemoglobin, which is then called hemoglobin S inherited in homozygosity (HbSS) or double heterozygosity (HbSC, HbSβ), and leads to chronic hemolysis, vaso-occlusion, inflammation, and endothelium activation. Pregnant women with SCD are at a higher risk of developing maternal and perinatal complications. We performed a narrative review of the literature considering SCD and pregnancy, the main clinical and obstetrical complications, the specific antenatal care, and the follow-up for maternal and fetal surveillance. Pregnant women with SCD are at a higher risk of developing clinical and obstetric complications such as pain episodes, pulmonary complications, infections, thromboembolic events, preeclampsia, and maternal death. Their newborns are also at an increased risk of developing neonatal complications: fetal growth restriction, preterm birth, stillbirth. Severe complications can occur in patients of any genotype. We concluded that SCD is a high-risk condition that increases maternal and perinatal morbidity and mortality. A multidisciplinary approach during pregnancy and the postpartum period is key to adequately diagnose and treat complications.

Hydroxyurea treatment for sickle cell anemia during pregnancy and lactation: Current evidence and knowledge gaps

“…Regarding more recently approved SCA therapies, there are no available data on antioxidant L‐glutamine use in pregnant women or animal studies to inform a drug‐associated risk of major birth defects and miscarriage 24 . However, as L‐glutamine is a common amino acid found in the body, it is generally considered to be safe 48 . For the oxygen‐affinity modulator voxelotor, no adverse developmental effects were found in animal reproduction studies, but experiments only tested at exposures up to 2.8‐times (rats) and 0.3‐times (rabbits) the maximum recommended human dose 26 .…”

“…24 However, as L-glutamine is a common amino acid found in the body, it is generally considered to be safe. 48 For the oxygen-affinity modulator voxelotor, no adverse developmental effects were found in animal reproduction studies, but experiments only tested at exposures up to 2.8-times (rats) and 0.3-times (rabbits) the maximum recommended human dose. 26 So far no teratogenesis data are available for the anti-adhesive monoclonal antibody crizanlizumab, although IgG2 is recognized to be the least efficiently transported IgG subclass across the human placenta.…”

Case Report: Obstetric and COVID-19-Related Morbidity and Mortality in Three Patients with Sickle Hemoglobinopathy

“…1 In the Jamaican pregnant population, 2.8% of women have SCD. 2 This group of disorders is highly associated with increased maternal morbidity/mortality, 3 with the most common cause of maternal death being acute chest syndrome (ACS). 2 …”

“…1 In the Jamaican pregnant population, 2.8% of women have SCD. 2 This group of disorders is highly associated with increased maternal morbidity/mortality, 3 with the most common cause of maternal death being acute chest syndrome (ACS). 2 COVID-19, caused by severe acute respiratory syndrome coronavirus 2 (SARS-CoV-2), was officially declared a pandemic in March 2020.…”

Maternal malnutrition during pregnancy among women with sickle cell disease

“…Of the other SCD variants, the most common is SC hemoglobinopathy (HbSC). Several studies have reported maternal and fetal complications in pregnant women with SCD: perinatal mortality, preterm labor, fetal growth restriction, preeclampsia, acute painful crises, and urinary and pulmonary infections [1][2][3][4] .…”