Chapter 10: Management of Pregnant Women and Newborns With Sickle Cell Disease

Overview

Pregnant women with sickle cell disease experience significant morbidity and mortality compared to the general population in both low-middle and high-income settings. Women with sickle cell disease are more likely to experience hypertensive emergencies (preeclampsia and eclampsia), intrauterine growth restriction (IUGR), low birth weight, fetal demise, venous thromboembolism, peripartum cardiomyopathy, and maternal death when compared to age-matched healthy pregnant women in both low-middle– and high-income settings.^1-5

The highest rate of maternal mortality among pregnant women with sickle cell disease occurs in sub-Saharan Africa, where the risk of maternal mortality can be as high as 29 times that of pregnant women without sickle cell disease.⁶ The maternal mortality rate of pregnant women with sickle cell disease living in sub-Saharan Africa, without any intervention, is approximately 6000 to 10,000 per 100,000 live births,^2,6-8 as compared to 542 per 100,000 live births in women living in the same environment without sickle cell disease.

In high-income settings, pregnant women with sickle cell disease have an increased risk of pregnancy-related complications compared to those without sickle cell disease, but no evidence of increased mortality.³ Specifically, in a pooled analysis of published studies, women with sickle cell disease, compared to women without sickle cell disease, had a higher rate of preeclampsia (pooled odds ratio [OR], 1.86; 95% confidence interval [CI], 1.22-2.82), eclampsia (pooled OR, 2.07; 95% CI, 1.43-2.99), cesarean delivery (pooled OR, 1.62; 95% CI, 1.26-2.08), and bacterial infection during pregnancy (pooled OR, 3.03; 95% CI, 1.86-4.92)³.

Furthermore, in the same analysis, pregnant women with sickle cell disease living in low- and middle-income settings have a statistically significant increased odds ratio of eclampsia, cesarean delivery, and bacterial infection when compared to women without sickle cell disease.³ Similarly, the analysis showed that pregnancy in women with sickle cell disease had an increased odds of IUGR (OR, 2.79; 95% CI, 1.85-4.21), prematurity (OR, 2.14; 95% CI, 1.56-2.94), and perinatal death (OR, 3.76; 95% CI, 2.34-6.06).³ The ORs of morbidities in pregnant women with sickle cell disease are summarized in Table 10-1.

Collectively, data from the pooled analyses strongly suggest that mortality in pregnant women is likely related to the gap in medical care between low-middle and high-income settings.³ Regardless of the setting, there is an increased risk of maternal morbidities.^...