Updated Australian consensus statement on management of inherited bleeding disorders in pregnancy

Scott Dunkley, Julie A Curtin, Anthony J Marren, Robert P Heavener, Simon McRae and Jennifer L Curnow
Med J Aust 2019; 210 (7): . || doi: 10.5694/mja2.50123
Published online: 15 April 2019


Introduction: There have been significant advances in the understanding of the management of inherited bleeding disorders in pregnancy since the last Australian Haemophilia Centre Directors’ Organisation (AHCDO) consensus statement was published in 2009. This updated consensus statement provides practical information for clinicians managing pregnant women who have, or carry a gene for, inherited bleeding disorders, and their potentially affected infants. It represents the consensus opinion of all AHCDO members; where evidence was lacking, recommendations have been based on clinical experience and consensus opinion.

Main recommendations: During pregnancy and delivery, women with inherited bleeding disorders may be exposed to haemostatic challenges. Women with inherited bleeding disorders, and their potentially affected infants, need specialised care during pregnancy, delivery, and postpartum, and should be managed by a multidisciplinary team that includes at a minimum an obstetrician, anaesthetist, paediatrician or neonatologist, and haematologist. Recommendations on management of pregnancy, labour, delivery, obstetric anaesthesia and postpartum care, including reducing and treating postpartum haemorrhage, are included. The management of infants known to have or be at risk of an inherited bleeding disorder is also covered.

Changes in management as a result of this statement: Key changes in this update include the addition of a summary of the expected physiological changes in coagulation factors and phenotypic severity of bleeding disorders in pregnancy; a flow chart for the recommended clinical management during pregnancy and delivery; guidance for the use of regional anaesthetic; and prophylactic treatment recommendations including concomitant tranexamic acid.

  • 1 Institute of Haematology, Royal Prince Alfred Hospital, Sydney, NSW
  • 2 The Children's Hospital at Westmead, Sydney, NSW
  • 3 Australian Haemophilia Centres Directors’ Organisation, Melbourne, VIC
  • 4 Royal Prince Alfred Hospital, Sydney, NSW
  • 5 Royal Adelaide Hospital, Adelaide, SA
  • 6 Haemophilia Treatment Centre, Westmead Hospital, Sydney, NSW



We are grateful to Steph P'ng, John Rowell, Tim Brighton, Huyen Tran and Ian Douglas for their helpful feedback and comments. We acknowledge Ruth Hadfield for medical writing and editing assistance.

Competing interests:

No relevant disclosures.

  • 1. Nichols WL, Rick ME, Ortel TL, et al. Clinical and laboratory diagnosis of von Willebrand disease: a synopsis of the 2008 NHLBI/NIH guidelines. Am J Hematol 2009; 84: 366–370.
  • 2. Nichols WL, Hultin MB, James AH, et al. von Willebrand disease (VWD): evidence‐based diagnosis and management guidelines, the National Heart, Lung, and Blood Institute (NHLBI) Expert Panel report (USA). Haemophilia 2008; 14: 171–232.
  • 3. Gouw SC, van der Bom JG, Marijke van den Berg H. Treatment‐related risk factors of inhibitor development in previously untreated patients with hemophilia A: the CANAL cohort study. Blood 2007; 109: 4648–4654.
  • 4. White GC 2nd, Rosendaal F, Aledort LM, et al. Definitions in haemophilia: recommendation of the scientific subcommittee on factor VIII and factor IX of the scientific and standardization committee of the International Society on Thrombosis and Haemostasis. Thromb Haemost 2001; 85: 560.
  • 5. Plug I, Mauser‐Bunschoten EP, Brocker‐Vriends AH, et al. Bleeding in carriers of hemophilia. Blood 2006; 108: 52–56.
  • 6. Pavord SR, Rayment R, Madan B, et al. Management of inherited bleeding disorders in pregnancy. Green‐top Guideline No. 71 (joint with UKHCDO). BJOG 2017; 124: e193–e263.
  • 7. Dunkley SM, Russell SJ, Rowell JA, et al. A consensus statement on the management of pregnancy and delivery in women who are carriers of or have bleeding disorders. Med J Aust 2009; 191: 460–463.
  • 8. Lee CA, Chi C, Pavord SR, et al. The obstetric and gynaecological management of women with inherited bleeding disorders – review with guidelines produced by a taskforce of UK Haemophilia Centre Doctors’ Organization. Haemophilia 2006; 12: 301–336.
  • 9. Canadian Hemophilia Society. Inherited bleeding disorders affecting women. (viewed Aug 2018).
  • 10. Street AM, Ljung R, Lavery SA. Management of carriers and babies with haemophilia. Haemophilia 2008; 14 Suppl 3: 181–187.
  • 11. Gouw SC, van der Bom JG, Auerswald G, et al. Recombinant versus plasma‐derived factor VIII products and the development of inhibitors in previously untreated patients with severe hemophilia A: the CANAL cohort study. Blood 2007; 109: 4693–4697.
  • 12. Chi C, Kadir RA. Management of women with inherited bleeding disorders in pregnancy. Obstet Gynaecol 2007; 9: 27–33.
  • 13. Stirling Y, Woolf L, North WR, et al. Haemostasis in normal pregnancy. Thromb Haemost 1984; 52: 176–182.
  • 14. Kadir RA, Lee CA. Obstetrics and gynecology: hemophilia. In: Lee CA, Berntorp EE, Hoots WK, editors. Textbook of haemophilia. 3rd ed. Oxford: Wiley, p. 337.
  • 15. Chi C, Lee CA, Shiltagh N, et al. Pregnancy in carriers of haemophilia. Haemophilia 2008; 14: 56–64.
  • 16. James AH, Konkle BA, Kouides P, et al. Postpartum von Willebrand factor levels in women with and without von Willebrand disease and implications for prophylaxis. Haemophilia 2015; 21: 81–87.
  • 17. Kadir RA, Lee CA, Sabin CA, et al. Pregnancy in women with von Willebrand's disease or factor XI deficiency. BJOG 1998; 105: 314–321.
  • 18. Bolton‐Maggs PH. The management of factor XI deficiency. Haemophilia 1998; 4: 683–688.
  • 19. Lavee O, Kidson‐Gerber G. Update on inherited disorders of haemostasis and pregnancy. Obstet Med 2016; 9: 64–72.
  • 20. Kadir RA, Economides DL, Braithwaite J, et al. The obstetric experience of carriers of haemophilia. BJOG 1997; 104: 803–810.
  • 21. Domschke C, Strowitzki T, Huth‐Kuehne A, et al. Successful in vitro fertilization and pregnancy in Glanzmann thrombasthenia. Haemophilia 2012; 18: e380–e381.
  • 22. Peavey M, Steward R, Paulyson‐Nunez K, James A. Successful prophylactic regimens for transvaginal oocyte retrieval in women with bleeding diatheses. Haemophilia 2013; 19: e189–e191.
  • 23. Kadir RA, Davies J, Winikoff R, et al. Pregnancy complications and obstetric care in women with inherited bleeding disorders. Haemophilia 2013; 19 Suppl 4: 1–10.
  • 24. Sanchez‐Luceros A, Meschengieser SS, Marchese C, et al. Factor VIII and von Willebrand factor changes during normal pregnancy and puerperium. Blood Coagul Fibrinolysis 2003; 14: 647–651.
  • 25. Srivastava A, Brewer AK, Mauser‐Bunschoten EP, et al. Guidelines for the management of hemophilia. Haemophilia 2013; 19: e1–e47.
  • 26. Kouides PA. Obstetric and gynaecological aspects of von Willebrand disease: best practice and research. Clin Haematol 2001; 14: 381–399.
  • 27. Federici AB, Mazurier C, Berntorp E, et al. Biologic response to desmopressin in patients with severe type 1 and type 2 von Willebrand disease: results of a multicenter European study. Blood 2004; 103: 2032–2038.
  • 28. Mannucci PM. Use of desmopressin (DDAVP) during early pregnancy in factor VIII‐deficient women. Blood 2005; 105: 3382.
  • 29. Australian Government Therapeutic Goods Administration. Prescribing medicines in pregnancy database. (viewed Nov 2018).
  • 30. Karanth L, Barua A, Kanagasabai S, Nair S. Desmopressin acetate (DDAVP) for preventing and treating acute bleeds during pregnancy in women with congenital bleeding disorders. Cochrane Database Syst Rev 2015; (9): CD009824.
  • 31. Huq FY, Kadir RA. Management of pregnancy, labour and delivery in women with inherited bleeding disorders. Haemophilia 2011; 17 Suppl 1: 20–30.
  • 32. Nazir HF, Al Lawati T, Beshlawi I, et al. Mode of delivery and risk of intracranial haemorrhage in newborns with severe haemophilia A: a multicentre study in Gulf region. Haemophilia 2016; 22: e134–e138.
  • 33. Ljung R, Lindgren AC, Petrini P, Tengborn L. Normal vaginal delivery is to be recommended for haemophilia carrier gravidae. Acta Paediatr 1994; 83: 609–611.
  • 34. Van Der Linde R, Favaloro EJ. Tranexamic acid to prevent post‐partum haemorrhage. Blood Transfus 2018; 16: 321–323.
  • 35. WOMAN Trial Collaborators. Effect of early tranexamic acid administration on mortality, hysterectomy, and other morbidities in women with post‐partum haemorrhage (WOMAN): an international, randomised, double‐blind, placebo‐controlled trial. Lancet 2017; 389: 2105–2116.
  • 36. Bolton‐Maggs PH, Chalmers EA, Collins PW, et al. A review of inherited platelet disorders with guidelines for their management on behalf of the UKHCDO. Br J Haematol 2006; 135: 603–633.
  • 37. Haljamäe H. Thromboprophylaxis, coagulation disorders, and regional anaesthesia. Acta Anaesthesiol Scand 1996; 40(8 Pt 2): 1024–1040.
  • 38. Horlocker TT, Wedel DJ, Benzon H, et al. Regional anesthesia in the anticoagulated patient: defining the risks (the second ASRA Consensus Conference on Neuraxial Anesthesia and Anticoagulation). Reg Anesth Pain Med 2003; 28: 172–197.
  • 39. Stedeford JC, Pittman JA. Von Willebrand's disease and neuroaxial anaesthesia. Anaesthesia 2000; 55: 1228–1229.
  • 40. Curnow J, Pasalic L, Favaloro EJ. Treatment of von Willebrand Disease. Sem Thromb Hemost 2016; 42: 133–146.
  • 41. Rodeghiero F. Von Willebrand disease: pathogenesis and management. Thromb Res 2013; 131 Suppl 1: S47–S50.
  • 42. American Academy of Family Physicians. Advanced Life Support in Obstetrics (ALSO). (viewed Mar 2019).
  • 43. Babarinsa IA, Hayman RG, Draycott TJ. Secondary post‐partum haemorrhage: challenges in evidence‐based causes and management. Eur J Obstet Gynecol Reprod Biol 2011; 159: 255–260.
  • 44. Hoveyda F, MacKenzie IZ. Secondary postpartum haemorrhage: incidence, morbidity and current management. BJOG 2001; 108: 927–930.
  • 45. Begley CM, Gyte GM, Devane D, et al. Active versus expectant management for women in the third stage of labour. Cochrane Database Syst Rev 2015; (3): CD007412.
  • 46. James DK, Steer P, Weiner C, Gonik B. High risk pregnancy: management options. 4th ed. St Louis, MO: Elsevier Saunders, 2011: p. 1504.
  • 47. Royal Australian and New Zealand College of Obstetricians and Gynaecologists. Management of postpartum haemorrhage (PPH). (viewed Nov 2018).
  • 48. Demers C, Derzko C, David M, Douglas J. Gynaecological and obstetric management of women with inherited bleeding disorders. Int J Gynaecol Obstet 2006; 95: 75–87.
  • 49. Andrew M, Paes B, Milner R, et al. Development of the human coagulation system in the healthy premature infant. Blood 1988; 72: 1651–1657.
  • 50. Kulkarni R, Lusher J. Perinatal management of newborns with haemophilia. Br J Haematol 2001; 112: 264–274.
  • 51. Kulkarni R, Lusher JM. Intracranial and extracranial hemorrhages in newborns with hemophilia: a review of the literature. J Pediatr Hematol Oncol 1999; 21: 289–295.
  • 52. Veldman A, Josef J, Fischer D, Volk WR. A prospective pilot study of prophylactic treatment of preterm neonates with recombinant activated factor VII during the first 72 hours of life. Pediatr Crit Care Med 2006; 7: 34–39.
  • 53. Australian Haemophilia Centre Directors’ Organisation. Guidelines for the treatment of inhibitors in haemophilia A and haemophilia B. Melbourne: AHCDO, 2010. (viewed Nov 2018).
  • 54. Australian Haemophilia Centre Directors’ Organisation and National Blood Authority, Australia. Guidelines for the management of haemophilia in Australia. Melbourne: AHCDO, 2016. (viewed Nov 2018).


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