Pregnancy outcome in homozygous sickle cell disease: observations from the Jamaican Birth Cohort

Abstract

Objective To document pregnancy outcome in homozygous sickle cell (SS) disease and in age‐matched controls with a normal haemoglobin genotype followed from birth for up to 45 years. Methods A total of 100 000 consecutive non‐operative deliveries screened for sickle cell disease at the main Government maternity hospital in Kingston, Jamaica between 1973 and 1981 detected 311 (149 female) babies with SS disease who were matched by age and gender with 250 (129 female) controls with an AA haemoglobin phenotype. These individuals have been followed from birth with prospective assessment of menarche and detailed documentation of all pregnancies. Results There were 177 pregnancies in 71 SS patients and 226 pregnancies in 74 AA controls. Mothers with SS disease had more spontaneous abortions (adjusted relative risk [aRR] 3.2, 95% CI 1.6–6.1), fewer live births (aRR 0.7, 95% CI 0.6–0.9) and their offspring were more likely to have a gestational age <37 weeks (aRR 2.1, 95% CI 1.1–3.7) and low birthweight <2.5 kg (aRR 3.0, 95% CI 1.6–5.3). They were more prone to acute chest syndrome (aRR 13.7, 95% CI 4.1–45.5), urinary tract infection (aRR 12.8, 95% CI 1.3–125.9), pre‐eclampsia/eclampsia (aRR 3.1, 95% CI 1.1–8.8), retained placenta (aRR 10.1, 95% CI 1.1–90.3), sepsis (Fisher's Exact test 0.04) and pregnancy‐related deaths (Fisher's Exact test 0.02). Four of five deaths were attributable to acute chest syndrome. There was no genotypic difference in pregnancy‐induced hypertension or postpartum haemorrhage. Conclusion Pregnancy in SS disease carries risks for both mother and child. The variable characteristics of pregnancy‐related deaths complicate their prevention. Tweetable abstract Pregnancy in SS disease compared with controls showed increased abortions and stillbirths, fewer live births and maternal deaths in 7% patients.

Perinatal outcomes in women with sickle cell disease: a matched cohort study from London, UK

“…SCD is increasingly recognised as a risk factor for preeclampsia and eclampsia. 4,5,14,15 Standardised management for the SCD cohort reported here will have included lowdose aspirin antenatally. 6 Despite this, we observed that pregnancies complicated by SCD had a 3Á5-times risk of preeclampsia/eclampsia.…”

“…SCD is increasingly recognised as a risk factor for pre‐eclampsia and eclampsia 4,5,14,15 . Standardised management for the SCD cohort reported here will have included low‐dose aspirin antenatally 6 .…”

Maternal mortality among women with sickle cell disease in Jamaica over two decades (1998–2017)

“…Lewis et al. report that of 163 non-terminated pregnancies, 57% ( n = 93) resulted in live births, 13 which is comparable to the 55% of live births in the latest series. Some variation in global maternal and perinatal mortality rates may be due to differences in SCD genotypes, other co-morbidities, maternal age and parity however in both developed and developing countries risk differences are similar between SCD and non-SCD women.…”

Sickle Cell Disease

“…In one study of 177 pregnancies in people with SCD compared with 226 pregnancies in people without SCD, rates were higher for preeclampsia (15.7 vs 6.2%), urinary tract infection (7.9% vs 0.9%), and low birth weight (46% vs 19%) in those with SCD. ACS occurred in 22% of patients with SCD who were pregnant . In addition, acute pain episodes are more common during pregnancy .…”

“…ACS occurred in 22% of patients with SCD who were pregnant. 98 In addition, acute pain episodes are more common during pregnancy. 21 A meta-analysis of 11 cohort studies (of which 10 were retrospective and single center and 1 RCT involving 1291 people) reported that prophylactic RBC transfusions during pregnancy (targets including Hb 10-12 g/dL, hematocrit Յ35%, HbA 40%-50%, or HbS<25%-50%) were associated with lower rates of maternal and perinatal mortality.…”