The Influence of Postpartum Hemorrhage on Patient Reported Outcomes Among Women with Von Willebrand Disease: The Pregnancy and Inherited Bleeding Disorders Study (PRIDES)

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Background: In the Netherlands, pregnant women with von Willebrand Disease (VWD) receive prophylactic clotting factor suppletion when third trimester von Willebrand factor activity or Factor VIII 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: assess patient reported outcomes (PROMs) by the Short Form 36 (SF-36), Mackeys Childbirth Satisfaction Rating Scale (MCSRS) and the Labor And DeliverY indeX (LADY-X) at week 1 and 6 postpartum in women with VWD with and without (severe) PPH. Method: Pregnant women with VWD (age ≥ 18 years) with an ongoing pregnancy who visited a Dutch hemophilia treatment center were eligible. PROMs data were combined with data on PPH and clotting factor prophylaxis 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 childbirth 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, 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, 81 women with VWD, mostly VWD type 1 (76.5%, n=62/81), completed at least one questionnaire. Vaginal deliveries occurred in 78.8% (n=63/81). The incidences of PPH and severe PPH were 36.3% (n=29/81) and 16.3% (n=13/81), respectively. Overall, 23.5% (n=19/81) completed the SF-36 at both week 1 and 6 postpartum. Of these women, 36.8% (n=7/19) had PPH and 15.8% (n=3/19) severe PPH. Week 1 was completed by 79.0% (n=63/81), 36.5% (n=23/63) with PPH and 14.3% (n=9/63) with severe PPH. Week 6 was completed by 58.7% (n=47/81), 34.0% (n=16/47) had PPH and 12.8% (n=6/47) severe PPH. QoL scores did not differ between women with and without a (severe) PPH at week 1 and 6 postpartum. No difference was found in the change of SF-36 scores between week 1 and 6 in women with and without (severe) PPH. Compared to the general population, women with VWD had lower scores on the ‘Role limitations due to emotional problems’ domain at week 1 (mean difference -41.8, p-value < .001). At week 6, women with VWD had a lower QoL on 7/8 domains (p-values <0.05). Overall, 63 women with VWD completed the MCSRS, including 36.5% (n=23/63) with PPH. There were no differences in MCSRS scores for each domain between women with and without PPH. Women with severe PPH (14.3%, n=9/63) had lower satisfaction score on the ‘Baby’ domain (beta -1.93, 95% CI: -3.75 - -0.11). Compared to the general population, women with VWD reported higher childbirth satisfaction on all MSCRS domains (p-values < 0.001). 62 women completed the LADY-X, 32.8% (n=20/62) with PPH and 13.1% (n=8/62) with severe PPH. No significant differences were found between women with and without (severe) PPH. Women with VWD reported more concern about their baby in comparison to the general population (OR 0.21, 95% CI 0.12-0.39). Conclusions: Overall, women with VWD with (severe) PPH report similar QoL at week 1 and week 6 postpartum as compared to those without (severe) PPH. Childbirth satisfaction was lower in women with severe PPH on the ‘Baby’ domain and childbirth experience did not differ between women with and without (severe) PPH. Compared to the general population, women with VWD had a lower QoL at week 6 postpartum and more concerns about their newborn. However, women with VWD report higher childbirth satisfaction on almost all MCSRS domains. These results should be interpreted cautiously due to comparisons with a historical cohort.

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