Management of women with inherited bleeding disorders in pregnancy

Abstract

Key content Pregnancy in women with inherited bleeding disorders requires specialised and individualised care using a multidisciplinary approach with obstetricians, midwives, haematologists and anaesthetists. Childbirth presents an intrinsic haemostatic challenge in these women and obstetricians may be the first to encounter haemorrhagic complications. An understanding and awareness of these disorders and close collaboration between obstetricians and haematologists are essential in ensuring a successful outcome. Learning objectives To understand the basic principles in the obstetric management of women with inherited bleeding disorders. To know the options available for prenatal diagnosis. To be aware of the increased risk of haemorrhagic complications in these women and their offspring. Ethical issues Advances in prenatal diagnosis and treatment of inherited bleeding disorders create complex dilemmas for families affected by these disorders. Please cite this article as: Chi C, Kadir RA. Management of women with inherited bleeding disorders in pregnancy. The Obstetrician & Gynaecologist 2007;9:27–33.

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

“…Bleeding risk is dependent on factor levels and the lower the factor level below 40 IU/dL the greater the bleeding risk . Together, haemophilia A and B, von Willebrand disease, and factor VII and factor XI deficiency account for around 90% of all women with inherited bleeding disorders …”

Management of Pregnancy in a Patient with Severe Hemophilia Type A

“…Eight percent of hemophilia A carriers had FVIII levels below 50 IU dL À1 and required prophylactic treatment throughout the pregnancy. 11 Unlike women with mild to moderate factor deficiencies, if the hemostatic defect is severe the abnormality will remain during the entirety of the pregnancy. Women with type 3 vWD show very little or no increase in their FVIII and von Willebrand factor plasma levels during pregnancy.…”

Desmopressin acetate (DDAVP) for preventing and treating acute bleeds during pregnancy in women with congenital bleeding disorders

“…During pregnancy these disorders may present a haemostatic challenge resulting in excessive bleeding and as a cause of obstetric haemorrhage, bleeding disorders are o en underestimated. Inherited bleeding disorders, mainly von Willebrand disease (VWD), haemophilia A and B factor XI and factor VII deficiency account for almost 90% of all women with inherited bleeding disorders (Chi 2007). Deficiencies in factors such as fibrinogen, prothrombin, factor V, FX, FXIII are comparatively rare.…”