A Local Experience of Pregnancy Outcomes in Sickle Cell Disease and Red Cell Exchange
Authors: Turner Rose , Dowsing Claire , Crighton Gemma , Mason Kylie D. , Szer Jeffrey 
Published: October 2019

Abstract
Aim
Pregnancy outcomes in women with Sickle Cell Disease (SCD) have traditionally been poor with rates of both maternal and fetal complications greater than that seen in non-SCD populations (Oteng-Ntim et al, 2015). This association has been demonstrated across both low- and high-income countries with no significant correlation to discrepancies in health care (Boafor et al, 2015). Complications associated with pregnancy in SCD include preterm delivery, intrauterine growth restriction (IUGR), low birth weight, fetal distress in labour, Cesarean section, eclampsia, sickle cell vaso-occlusive crisis, infection, postpartum hemorrhage, and perinatal and maternal death. The introduction of prophylactic Red Cell Exchange (RCE) for high-risk SCD patients has seen a significant reduction in SCD-associated morbidity and mortality (Josephson et al, 2007). Limited studies have examined whether prophylactic RCE also translates to improved pregnancy outcomes in the SCD population with mixed conclusions (Asma et al, 2015). Whilst the association between SCD and adverse pregnancy outcomes has been well characterised in large multi-centre retrospective studies internationally, local data of pregnancy outcomes in SCD, particularly in patients receiving regular RCE, is lacking.
Method
We conducted a retrospective review of pregnancies within the SCD population managed at the Royal Melbourne Hospital between 2001 (date of commencement of regular RCE program) and 2018 to identify pregnancy complications and outcomes. Medical records across two sites (the Royal Melbourne Hospital and the Royal Women's Hospital) were reviewed to identify patient demographics, RCE received, mode and date of delivery, birth weight, fetal and maternal complications, and outcome of pregnancy.
Results
We identified 10 pregnancies amongst 5 patients with SCD managed during this time. Three patients had HbSS and two HbSC disease. Mean maternal age at time of pregnancy was 27 years. Two patients were receiving regular RCE prior to pregnancy and continued throughout at 3 to 4 week intervals, with a further patient commenced on RCE at 27/40 as per local hospital practice. Of the 3 women receiving regular RCE during pregnancy, all had live births (n=7); 1 via normal vaginal delivery (NVD) and 6 elective Cesarean-section due to cephalopelvic disproportion. Six of these pregnancies were at term, with one induced at 32/40 due to line sepsis as a complication of RCE. Two pregnancies were also complicated by gestational diabetes.
Two patients were not managed with regular RCE. The first declined treatment throughout both pregnancies, with pregnancy 1 complicated by intrauterine growth restriction (IUGR) with delivery via NVD at 37/40, and pregnancy 2 complicated by placental abruption and fetal death in utero (FDIU) at 22/40. Both pregnancies were also complicated by gestational thrombocytopenia. The second patient was on hydroxyurea (HU) at time of conception, initially continued during pregnancy in the setting of normal morphology scans and a rare blood phenotype prohibitive of RCE. HU was subsequently ceased at 27/40 due to the development of IUGR. She was commenced on RCE at 30/40 but suffered placental abruption and FDIU at 34/40. No offspring had a haemoglobinopathy of clinical significance.
Conclusion
RCE was well-tolerated and associated with good and possibly improved maternal and fetal outcomes in this small cohort of sickle cell patients. Larger studies are required to further characterise the benefit of prophylactic RCE during pregnancy in the SCD population.

Disclosures
Szer: Alexion Pharmaceuticals, Inc.: Consultancy, Honoraria, Other: Travel Support , Research Funding.