We investigated the impact of the Maternal Medicine Network Hub, established at our centre in 2019, on post-partum haemorrhage (PPH) management in women with inherited bleeding disorders (IBDs).
Methods
Data from 63 patients with IBDs between 2020 and 2023 were compared to a general obstetric cohort. Specific data collected included haemoglobin levels, estimated blood loss, and PPH severity.
Results
Post-partum haemorrhage rates above 500 mL and 1000 mL were significantly higher in the IBD group (p = .02 and p = .002, respectively). However very severe PPH (>1500 mL) also known as massive obstetric haemorrhage (MOH) was not significantly different from the general obstetric population (p = .7) in our tertiary centre (The Royal London Hospital).
Conclusion
Multidisciplinary care at the Maternal Medicine Hub may mitigate the risk of extreme blood loss (MOH) in women with IBDs. This highlights the necessity of specialised care in improving outcomes for women with IBDs during childbirth.
VanderMeulenHPetrucciJFlorosG, et al.The experience of postpartum bleeding in women with inherited bleeding disorders. Res Pract Thromb Haemost[Internet]2019,3:733–740. https://pmc.ncbi.nlm.nih.gov/articles/PMC6782019/.
Hews-GirardJCGalicaJGoldieC, et al.Identifying the effect of inherited bleeding disorders on the development of postpartum hemorrhage: a population-based, retrospective cohort study. Res Pract Thromb Haemost[Internet]2023; 7: 100104. https://linkinghub.elsevier.com/retrieve/pii/S2475037923000730.
6.
AlamAUWuCKaulP, et al. (linda). Impact of inherited bleeding disorders on maternal bleeding and other pregnancy outcomes: a population-based cohort study. Haemophilia[Internet]2024; 30: 478–489.
7.
MaasDPMSMSaesJLBlijlevensNMA, et al.High prevalence of postpartum hemorrhage in women with rare bleeding disorders in the Netherlands: retrospective data from the RBiN study. J Thromb Haemost[Internet]2023; 21: 499–512. https://linkinghub.elsevier.com/retrieve/pii/S1538783622076231.
8.
National Bleeding Disorders Foundation. The national hemophilia foundation state of the science research summit initiative: executive summary [Internet]. National Bleeding Disorders Foundation. https:// pubmed.ncbi.nlm.nih.gov/36920857/.
9.
JamesAHJamisonMG. Bleeding events and other complications during pregnancy and childbirth in women with von Willebrand disease. J Thromb Haemost2007; 5: 1165–1169. https://pubmed.ncbi.nlm.nih.gov/17403089/.
10.
HawkeLGrabellJSimW, et al.Obstetric bleeding among women with inherited bleeding disorders: a retrospective study. Haemophilia2016; 22: 906–911. https://pubmed.ncbi.nlm.nih.gov/27704714/.
KujovichJL. Von Willebrand disease and pregnancy. J Thromb Haemostasis2005; 3: 246–253.
13.
JamesAHKonkleBAKouidesP, et al.Postpartum von Willebrand factor levels in women with and without von Willebrand disease and implications for prophylaxis. Haemophilia2014; 21: 81–87. https://pubmed.ncbi.nlm.nih.gov/25333737/.
14.
PuntMvan LeusdenFBloemenkampK, et al.Primary postpartum hemorrhage in women with von Willebrand disease and carriers of hemophilia: a retrospective analysis. Res Pract Thromb Haemost[Internet]2024; 8: 102508. https://linkinghub.elsevier.com/retrieve/pii/S2475037924002036.
JamesAHKouidesPAAbdul-KadirR, et al. Von Willebrand disease and other bleeding disorders in women: consensus on diagnosis and management from an international expert panel. Am J Obstet Gynecol2009; 201: 12.e1–12.e8. https://pubmed.ncbi.nlm.nih.gov/19481722/.
17.
SrivastavaASantagostinoEDougallA, , et al.WFH guidelines for the management of hemophilia, 3rd edition. Haemophilia2020; 26: 1–158. https://pubmed.ncbi.nlm.nih.gov/32744769/
18.
WolfSSardo InfirriSBattyP, et al.Postpartum bleeding in women with inherited bleeding disorders: a matched cohort study. Blood Coagul Fibrinolysis2020; 31: 452–458. https://pubmed.ncbi.nlm.nih.gov/32833805/.
19.
KouidesPAPhatakPDBurkartP, et al.Gynaecological and obstetrical morbidity in women with type I von Willebrand disease: results of a patient survey. Haemophilia2000; 6: 643–648. https://pubmed.ncbi.nlm.nih.gov/11122390/.
20.
SoucieJMMillerCHDupervilB, et al. Occurrence rates of haemophilia among males in the United States based on surveillance conducted in specialized haemophilia treatment centres. Haemophilia2020; 26: 487–493. https://pubmed.ncbi.nlm.nih.gov/32329553/.
21.
MachinNRagniMV. Recombinant vs plasma-derived von Willebrand factor to prevent postpartum hemorrhage in von Willebrand disease. Blood Advances2020; 4: 3234–3238.
22.
ChiCKulkarniALeeCA,et al.The obstetric experience of women with factor XI deficiency. Acta Obstet Gynecol Scand2009; 88: 1095–1100.
23.
BüllerHRBethuneCBhanotS, et al.Factor XI antisense oligonucleotide for prevention of venous thrombosis. N Engl J Med2015; 372: 232–240.
SalomonOSteinbergDMTamarinI, et al.Plasma replacement therapy during labor is not mandatory for women with severe factor XI deficiency. Blood Coagul Fibrinolysis2005; 16: 37–41. https://pubmed.ncbi.nlm.nih.gov/15650544/.
26.
KadirRAEconomidesDLBraithwaiteJ, et al.The obstetric experience of carriers of haemophilia. Br J Obstet Gynaecol1997; 104: 803–810. https://pubmed.ncbi.nlm.nih.gov/9236645/.
27.
BattyPHonkeABowlesL, et al.Ongoing risk of thrombosis with factor XI concentrate: 5 years experience in two centres. Haemophilia[Internet]2015; 21: 490–495.
28.
AhmadziaHKLubanNLCLiS, et al.Optimal use of intravenous tranexamic acid for hemorrhage prevention in pregnant women. Am J Obstet Gynecol2020; 225: 85.e1–85.e11. https://pubmed.ncbi.nlm.nih.gov/33248975/.
KadirRAEconomidesDLLeeCA. The obstetric and gynaecological management of women with inherited bleeding disorders—review with guidelines produced by a taskforce of UK haemophilia centre doctors’ organization. Haemophilia2009; 15: 1–10. https://pubmed.ncbi.nlm.nih.gov/19141132/.
31.
PreskyKOKadirRA. Women with inherited bleeding disorders - challenges and strategies for improved care. Thromb Res2020; 196: 569–578. https://pubmed.ncbi.nlm.nih.gov/31303333/.
32.
McDonaldJH.Handbook of biological statistics. 3rd ed. Baltimore, MD: Sparky House Publishing; 2014. pp. 77–85.
