Résumé
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(s): 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(s): 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.
Résumé
Introduction: The purpose of this study was to evaluate the functional status and ongoing respiratory symptoms of patients who have been discharged home, who were admitted to the intensive care unit (ICU) with severe COVID-19 disease. Long term complications post critical illness, such as post intensive care syndrome (PICS), are well-recognised [1], however, little data exists specific to COVID-19 ICU survivors. Method(s): Local ethics committee approval was secured. Participants were recruited using the electronic intensive care chart database. All patients, from March 2020 to November 2021, who survived Covid-19 were included. Researchers conducted phone interviews with participants and completed three questionnaires;Katz Index of Independent Living Questionnaire, Karnofsky Performance Status Scale and the American Thoracic Society (ATS) Respiratory Disease Questionnaire. Data was input and analysed using excel and stored on a password encrypted computer. Result(s): Database search revealed 61 patients available, 33 of 61 patients were included. 20 (61%) were male, 13 (39%) were female. Mean age was 58, with a range of 25-81. Mean length of ICU stay was 15 days, with a range of 1-74 days. 15 (45%) patients were intubated. Most common Katz score (range 1-6) was the highest score of 6 in 23 (70%) patients. Most common Karnofsky score (range 10-100) was 60 in 11 (33%) patients, with 31 (94%) scoring < 100. The most frequent ATS shortness of breath (SOB) grade (range 0-4) was 3 in 10 (9%) patients, with 23 (70%) patients scoring > 0. 14 (42%) patients had an ongoing cough, 11 (33%) patients had a wheeze and 0 (0%) patients were on home oxygen therapy. Conclusion(s): This study highlights in the majority of patients who survived severe Covid-19 disease to discharge home, there were serious ongoing limitations to their functional status and exercise tolerance, however, they remained independent of basic activities of daily living.