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)

• Published in: Blood 2024-11, Vol.144 (Supplement 1), p.1208-1208
• Main Authors: de Vaan, Anne, Kruip, Marieke JHA, Eikenboom, Jeroen, Doeff, Elke A, Punt, Marieke C, Coppens, Michiel, Nieuwenhuizen, Laurens, Schols, Saskia EM, Mäkelburg, Anja BU, Moenen, Floor CJI, Duvekot, Hans JJ, Middeldorp, Annemieke JM, Bloemenkamp, Kitty WM, Schutgens, Roger EG, van Galen, Karin PM
• Format: Article
• Language: English
• ISSN: 0006-4971; 1528-0020
• DOI: 10.1182/blood-2024-207237

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 a