The Influence of Postpartum Hemorrhage on the Quality of Life Among Pregnant Hemophilia a and B Carriers: The Pregnancy and Inherited Bleeding Disorders Study (PRIDES)

Background: In the Netherlands, pregnant hemophilia carriers (HCs) receive prophylactic clotting factor suppletion when third trimester Factor VIII or IX activity levels are < 80 IU/dL to decrease the risk of postpartum hemorrhage (PPH). PPH (≥ 500 mL) and severe PPH (≥ 1000 mL) can lead to adverse outcomes like anemia, prolonged hospitalization, and slow recovery. Furthermore, prophylactic clotting factor suppletion necessitated childbirth in a hemophilia treatment center, increased intravenous infusions, and prolonged hospital stay which possibly affects quality of life (QoL) and childbirth satisfaction. Aim: to assess patient reported outcomes (PROMs) at week 1 and week 6 postpartum and compare results between HCs with and without (severe) PPH. Method: Pregnant HCs (age ≥ 18 years) with an ongoing pregnancy who visited a Dutch hemophilia treatment center were eligible. PROMs data were combined with PPH and clotting factor prophylaxis data from the PRegnancy and Inherited bleeding DisordErS (PRIDES) study. Participants completed the Short Form 36 (SF-36) for QoL assessment, Mackeys Childbirth Satisfaction Rating Scale (MCSRC) for childbirth satisfaction and the Labor And DeliverY indeX (LADY-X) for birth experience. The SF-36 and MCSRS were completed at week 1, and the SF-36 and LADY-X at week 6 postpartum. We used descriptive statistics for baseline parameters. Linear regression compared SF-36 scores at week 1, week 6 and the delta between them for women with and without (severe) PPH. LADY-X and MCSRS scores were analysed by using the Mann-Whitney U test and regression analysis to control for clotting factor suppletion. Outcomes were compared to a general Dutch cohort study (2004-2007) by one sample t-tests and Odds Ratios. Results: between September 2018 and March 2024,87 HCs, mostly hemophilia A (83.9%, n=73/87), completed at least one questionnaire. Vaginal deliveries occurred in 81.2% (n=69/87). The incidences of PPH and severe PPH in this cohort were 37.6% (n=32/87) and 17.6% (n=15/87), respectively. In total, 23.0% (n=20/87) completed the SF-36 at both week 1 and week 6 postpartum. Of these women, 50% (n=10/20) had PPH ≥ 500 mL, and 20% (n=4/20) severe PPH. Week 1 was completed by 80.0% (n=68/87), 38.2% (n=26/68) with PPH and 19.1% (n=13/68) with severe PPH. Week 6 was completed by 51.2% (n=43/87), with 39.5% (n=17/43) experiencing PPH and 20.9% (n=9/43)severe PPH. QoL scores did not differ between women with and without a (severe) PPH at week 1 and week 6 postpartum. At week 6, HCs with either PPH or severe PPH had lower scores on emotional wellbeing as compared to week 1 postpartum (p-values < 0.05). Compared to the general population, HCs had lower scores on ‘Role limitation due to emotional problems’ (mean difference -41.8, p-value <0.001) at week 1. At week 6, HCs had lower QoL scores on 7/8 domains (p-values < 0.05). The MCSRS was completed by 71 HCs, 37% (n=26/71) with PPH There were no differences in MCSRS scores between HCs with and without PPH. HCs with severe PPH (18.8%, n=13/71) reported lower MCSRS scores on the domains ‘Baby’ (beta -1.55, 95% CI -2.82 - -0.27), and ‘General’ (beta -2.70, 95% CI -4.85 - -0.55). Compared to the general population, HCs reported more frequently ‘satisfied’ and ‘very satisfied’ on all MCSRS domains (all p-values < 0.001). The LADY-X was completed by 59 HCs. 41.4% (n=24/59) had PPH and 24.1% (n=14/59) severe PPH. No significant differences in LADY-X scores were found between HCs with and without (severe) PPH. HCs reported more concerns about their newborn as compared to the general population (OR 0.27, 95% CI 0.15-0.48). Conclusions: HCs with PPH reported similar QoL scores at week 1 and week 6 postpartum compared to those without PPH. However, HCs with (severe) PPH had lower emotional wellbeing scores at week 6. HCs with severe PPH have lower satisfaction with general wellbeing and interaction with their newborn during labor. Childbirth experience was similar between HCs with and without (severe) PPH. Compared to the general population, HCs reported lower QoL at 6 weeks postpartum and more concerns about their newborn. However, HCs experienced higher childbirth satisfaction on the MCSRS. The results should be interpreted cautiously due to comparisons with a historical cohort.