(BJOG. 2016;123:691–698) Pregnant women with sickle-cell disease (SCD) are generally considered to have a high risk of developing maternal and fetal complications. However, data from well-designed studies are limited regarding this topic. Therefore, this meta-analysis aimed to determine whether women with and without SCD have similar risks of pregnancy complications, and whether these outcomes differ based on residence in low-income or high-income countries.

Sickle cell disease (SCD) is associated with adverse pregnancy outcome. In women with SCD living in low-resource settings, pregnancy is associated with significantly increased maternal and perinatal mortality rates. We tested the hypothesis that implementing a multidisciplinary obstetric and hematology care team in a low-resource setting would significantly reduce maternal and perinatal mortality rates. We conducted a before-and-after study, at the Korle-Bu Teaching Hospital in Accra, Ghana, to evaluate the effect of a multidisciplinary obstetric-hematology care team for women with SCD in a combined SCD-Obstetric Clinic. The pre-intervention period was assessed through a retrospective chart review to identify every death and the post-intervention period was assessed prospectively. Interventions consisted of joint obstetrician and hematologist outpatient and acute inpatient reviews, close maternal and fetal surveillance, and simple protocols for management of acute chest syndrome and acute pain episodes. Primary outcomes included maternal and perinatal mortality rates before and after the study period. A total of 158 and 90 pregnant women with SCD were evaluated in the pre-and post-intervention periods, respectively. The maternal mortality rate decreased from 10 791 per 100 000 live births at pre-intervention to 1176 per 100 000 at postintervention, representing a risk reduction of 89.1% (P 5 0.007). Perinatal mortality decreased from 60.8 per 1000 total births at pre-intervention to 23.0 per 1000 at post-intervention, representing a risk reduction of 62.2% (P 5 0.20). A multidisciplinary obstetric and hematology team approach can dramatically reduce maternal and perinatal mortality in a low-resource setting.

In Africa, the maternal mortality rate in sickle cell disease (SCD) is~10%. Our team previously demonstrated an 89% decrease in mortality rate in a before-and-after feasibility study among women with SCD living in low-resource setting in Ghana. In the same cohort including additional participants with and without SCD, we used a prospective cohort design to test the hypothesis that implementing a multidisciplinary care team for pregnant women with SCD in low-resource setting will result in similar maternal and perinatal mortality rates compared to women without SCD. We prospectively enrolled pregnant women with and without SCD or trait and followed them up for 6-week postpartum. We tested the newborns of mothers with SCD for SCD. We recruited age and parity matched pregnant women without SCD or trait as the comparison group. Maternal and perinatal mortality rates were the primary outcomes.A total of 149 pregnant women with SCD (HbSS, 54; HbSC, 95) and 117 pregnant women without SCD or trait were included in the analysis. Post-intervention, maternal mortality rates were 1.3% and 0.9% in women with and without SCD, respectively (P = 1.00); the perinatal mortality rates were 7.4% and 3.4% for women with and without SCD, respectively (P = 0.164). Among the mothers with SCD,~15% of newborns had SCD. Multidisciplinary care of pregnant women with SCD may reduce maternal and perinatal mortality rates to similar levels in pregnant women without SCD in low-resource settings. Newborns of mothers with SCD have a high rate of SCD.

Pregnancy is a life-threatening occurrence in women with sickle cell disease (SCD), with increased odds of maternal and perinatal mortality compared to pregnant women without SCD. 1 During pregnancy, women with SCD can also experience SCD related maternal morbidities, including acute vaso-occlusive pain episode, acute chest syndrome (ACS), and venous thromboembolism with expected increased incidence rates when compared to not being pregnant. 2 We previously demonstrated in a case series that 87% of all maternal deaths were due to ACS, with almost 80% of episodes preceded by an acute pain event. 3 In another prospective study, we demonstrated increased incidence rates of acute pain and ACS in pregnant women with SCD 4 compared to historical non-pregnant women with SCD. 5 As a planned follow-up to our prospective cohort study, we extended the outcome in our pre-existing cohort of pregnant women with SCD 4 to determine the impact of pregnancy on acute pain events pre-and postpartum. We tested the hypothesis that pregnant women with SCD have higher incidence rates of acute pain requiring hospitalization and ACS during pregnancy compared to one-year postpartum.