Abstract
Background: Women with sickle cell disorders (SCD) have increased morbidity and mortality in pregnancy due to increased sickle and pregnancy-related complications and require multidisciplinary team management, including obstetrician and midwife with experience of high-risk antenatal care and haematologist with links to a Specialized Hemoglobinopathy Team1. About 15,000 individuals live with SCD in the UK, with 110-200 pregnancies per annum1. Aims: To determine whether King’s College Hospital followed recently published British Society of Haematology (BSH) guidelines for the management of pregnancy in SCD. Pilot a proposed BSH audit proforma and assess mode of delivery, neonatal birth weight and special care baby unit (SCBU) requirements for this cohort. Methods: Pregnancy management and outcomes were audited using a proforma based on recent BSH guidelines (Appendix 1). Review of electronic patient records (EPR) for all women attending joint obstetric hemoglobinopathy clinics with SCD who completed a pregnancy between 01/10/20 and 01/10/21, including miscarriage and termination of pregnancy (TOP). Results: 23 pregnancies resulted in 19 live births (one twin pregnancy), 3 miscarriages (10/40 + 1, 10/40 + 1, 14/40 + 6); and 2 TOPs (10/40, 10/40). Maternal haemoglobin genotype was 74% HbSS, 23% HbSC, and 4% HbS other. The average maternal age at delivery (excluding miscarriage and TOP) was 33.6 years (24.8-40.8 years). Average gestational age at delivery (excluding miscarriage and TOP) was 37.9 weeks (32/40 + 1 to 40/40 + 1). 13 pregnancies were delivered by Caesarean section (9 emergency) and 5 by vaginal delivery (4 forceps). 14 women who had a live birth underwent transfusion during pregnancy, 1 was transfused postnatally and 3 were not transfused. Maternal complications were recorded in 94% pregnancies (excluding miscarriage and TOP). 47% of women had sickle vaso-occlusive crises, 56% had an infection, 17% had acute kidney injury and 6% required Intensive Therapy Unit care. Neonatal complications were recorded in four neonates. Three had intrauterine growth restriction, and were delivered at 32 + 1, 33 + 6 and 38 + 1 gestation respectively, with one requiring care in SCBU. One neonate had jaundice requiring five days of phototherapy. The average neonatal birth weight was 2770g (960-3715g) with a birthweight centile ranging from 0-0.4 to 75-91. Discussion: Audit barriers included an initial lack of access to information due to separate EPR system (BadgerNet) for maternal notes, rectified by collaboration with the obstetric team and access to BadgerNet. Summary/Conclusion: This pilot audit enabled assessment of collecting required data for a proposed BSH audit proforma; monitoring compliance with newly published BSH guidelines for management of pregnancy in SCD1; and provided insight into pregnancy outcomes in women with SCD who delivered within a 12-month period at a single large centre. The higher-than-expected transfusion rate during pregnancy was likely due to ongoing recruitment to the TAPS2 trial, with eligible women offered randomisation to usual practice or pre-emptive red cell exchange transfusion 6-10 weekly from 6-20 weeks gestation. A high proportion of live births were delivered by Caesarean section, reflecting a high proportion of multiparous women with previous Caesarean sections in this cohort. Although complication rates were high, most complications were relatively minor and non-life threatening. Keywords: Pregnancy, Sickle cell disease Reference 1. Oteng‐Ntim E et al. Br J Haematol 2021;194(6):980-995.
Published Version
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