5613393 pregnancy outcomes in women with sickle cell disease in ireland: a retrospective review

Background: Sickle cell disease (SCD) is one of the most common single gene disorders worldwide and is characterised by significant morbidity and early mortality.[1] Pregnancy in SCD is associated with an increased risk of maternal and foetal complications.[2,3] The 2011 RCOG and the 2021 BSH guidelines[5,6] on the management of pregnancy in SCD have provided the basis for best practice care in the UK over the past decade and is the guidance which we follow in Ireland. To date, there is no published data on outcomes for pregnant women with SCD in Ireland. The number of Irish patients with SCD has risen over the past 20 years. Without a national database, the exact prevalence is not known but currently there are at least 600 adults and children with SCD in Ireland, whose population is just over 5 million.[4] Aims: Our study assesses outcomes of pregnant patients with SCD from 2015 to 2022. Our aims were to: Assess adherence to current guidelines Assess pregnancy outcomes and maternal complications Assess transfusion rates amongst our patient cohort. Method: This is a retrospective cohort study. We do not have a directly matched cohort, but have compared our findings to published data on Irish pregnancy outcomes from the Irish Maternity Indicator System National Report and have correlated our findings with studies of women with SCD who were managed in UK centres.[8,9,10] Results: We reviewed outcomes of 29 pregnancies in 19 women over a 7-year period. The median age was 29 (range 20-41) and the predominant maternal sickle genotype was HbSS (65.5%). Before conception, 55.2% of cases had pre-existing complications of SCD, including acute chest syndrome (ACS), pulmonary hypertension (PHTN) and prior stroke. In accordance with current guidelines, 100% of women (n=29) were prescribed folic acid, penicillin, and aspirin prophylaxis. 51.7% (n=15) of women had documented maternal complications during pregnancy, including ACS (34%), vaso-occlusive crisis (34%), gestational diabetes (10%), VTE (3%) and UTI (3%). Two women (7%) developed Covid-19 pneumonitis despite vaccination. There was one case of maternal bacteraemia (3%). 65.5% of cases (n=19) required blood transfusion during pregnancy. One woman was already on a blood transfusion programme for disease modification prior to pregnancy. In 6 cases (20.6%), a transfusion programme was commenced during pregnancy due to prior pregnancy complications or intrauterine growth restriction. During pregnancy, 27.6% (n=8) of women required emergency red cell exchange for ACS. Prior studies have suggested that between 30% and 70% of pregnant women with SCD require at least one blood transfusion during pregnancy.[8,9,10] By comparison, only 2.6% of the Irish general obstetric population required transfusion during pregnancy.[7] 20.6% (n=6) of births were preterm at <37 weeks’ gestation. There was one live preterm birth (3%) at <34 weeks and one intrauterine death (3%) at 23 weeks’ gestation. Similar to UK data[9], 31% of women required critical care stay (n=9) during pregnancy, in comparison with 1.44% nationwide in 2020.[7] Conclusion: It is well established that pregnancy in SCD is high risk, and despite adherence to current guidelines, we have shown very high rates of critical care admission, significant transfusion requirement and hospital admissions. Our findings are comparable to published UK outcomes and they further support the need for a comprehensive specialist care setting for this patient cohort. References 1. Weatherall DJ. ABC of clinical haematology. The hereditary anaemias. BMJ. 1997; 314(7079):492-6. 2. Oteng-Ntim E, Meeks D, Seed PT, Webster L, Howard J, et al, Adverse maternal and perinatal outcomes in pregnant women with sickle cell disease: systematic review and meta-analysis. Blood. 2015;125:3316–25 3. Boafor TK, Olayemi E, Galadanci N et al, Pregnancy outcomes in women with sickle-cell disease in low and high income countries: a systematic review and meta-analysis. BJOG. 2016;123:691–8 4. Central Statistics Office. Census of Population 2022. Available at https://www.cso.ie/en/csolatestnews/presspages/2022/censusofpopulation2022-preliminaryresults/ Accessed 9th September 2022 5. Oteng-Ntim, E., Pavord, S., Howard, J et al, Management of sickle cell disease in pregnancy. A British Society for Haematology Guideline. Br J Haematol 2021; 194: 980-995. 6. Royal College of Obstetricians and Gynaecologists. Management of Sickle Cell Disease in Pregnancy. 2011. Available at https://www.rcog.org.uk/media/nyinaztx/gtg_61.pdf 7. National Women and Infants Health Programme. Irish Maternity Indicator System National Report 2020. June 2021. Available online: https://www.hse.ie/eng/about/who/acute-hospitals-division/woman-infants/national-reports-on-womens-health/irish-maternity-indicator-system-national-report-2020.pdf. Accessed 24.8.22 8. Oakley LL, Mitchell S, von Rege I et al, Perinatal outcomes in women with sickle cell disease: a matched cohort study from London, UK. Br J Haematol. 2022;196(4):1069-1075. 9. Oteng-Ntim E et al, Pregnancy outcome in patients with sickle cell disease in the UK – a national cohort study comparing sickle cell anaemia (HbSS) with HbSC disease. Br J Haematol. 2015;169:129–37. 10. Sharif J et al, Transfusion for sickle cell disease in pregnancy: a single-centre survey. Transfus Med. 2018;28:231–5.