Abstract
Life expectancy in patients with sickle cell disease (SCD) has increased with the institution of newborn screening, antibiotics and Hydroxyurea (HU). Prior studies have reported maternal mortality rate to be 2.2 times that of normal pregnancies. With improved high risk obstetrical services and hematological care, more women with SCD are choosing to carry a pregnancy (P). In order to see if outcomes have improved, we performed a review of women with SCD who had recent P at our hospital with a focus on maternal and fetal outcomes.
Patients (pts) were identified by a chart review of all women of childbearing age followed at both the Comprehensive Sickle Cell Center and the high risk obstetrics clinic. Data included: genotypes of SCD, fetal complications, P outcomes, transfusions (T), hospital admissions, previous HU use.
71 pregnancies were identified in 53 women from 2008 to 2013.
See Table 1. Live births rates were equal in both GR I and GR II. The mean gestational age (weeks) in GR I was 35.13 and GR II 38.28 (p=0.0077) with more preterm births in GR I 16/36 (44%) compared to GR II 2/18 (11%). The most common mode of delivery was C-section in both GR I and II. Reasons for C- section were fetal distress 60% (20), repeat C-section 27% (9), other (5% or less) for breech, elective, preeclampsia, placenta previa. There were equal numbers of induced and spontaneous labor in each group. Complications in GR I 8/36 (22%) and GR II 2/18(11%) included PIH, Chorioamniotis, placenta previa, death (cardiac arrest).
Genotypes | Elective Abortion | Miscarriage | Still birth | Live birth | Total (n=71 pts) |
SS/SB 0 thal group(GR) I | 6 (13%) | 5 (11%) | 3 (6%) | 33 (70%) | 47 (66%) |
SC/SB+ thal group(GR) II | 3 (12%) | 3 (12%) | 0 | 18 (76%) | 24 (34%) |
Genotypes | Elective Abortion | Miscarriage | Still birth | Live birth | Total (n=71 pts) |
SS/SB 0 thal group(GR) I | 6 (13%) | 5 (11%) | 3 (6%) | 33 (70%) | 47 (66%) |
SC/SB+ thal group(GR) II | 3 (12%) | 3 (12%) | 0 | 18 (76%) | 24 (34%) |
Low birth weight occurred in 20/33(61%) of GR I and 3/18(17%) of GR II (p= 0.0016) births. Other complications (41 % of births) including IUGR, meconium aspiration, oligohydramnios and Apgar scores <7 @ 1 min occurred equally in both GRs.
Maternal outcomes: During P mean values for events in GR I and GR II respectively were: transfusions 5.56 units (U) and 1.22 U (p=0.0022), admission for vasoocclusive crisis (VOC) 3.33/pt and 1.44/pt (p=0.0173). Other complications were urinary tract infection (7), pneumonia (4), acute chest syndrome (4), cardiomyopathy (1), renal failure (1), hepatic crisis (2), DVT (1). Six patients were on HU pre- pregnancy (PP). In these patients 13 simple blood T were required PP and 56 during P (p=0.0297). VOC events increased from 13 PP to 35 during P (p=0.1011). 2 patient required exchange T during P.
Despite improved antenatal care for SCD women, P remains a high risk event for mother and fetus, with lower birth weights and more preterm deliveries in SS/SB 0 thal pts compared to SC/SB+ thal. Prior use of HU PP was not protective. New insights and studies are warranted to understand the pathophysiology of SCD causing adverse pregnancy outcomes and possible effects of HU withdrawal.
No relevant conflicts of interest to declare.
Author notes
Asterisk with author names denotes non-ASH members.
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