Women and girls with inherited bleeding disorders (IBD) face distinct gynaecologic and obstetric challenges, largely due to increased bleeding risk during key reproductive milestones. Conditions such as heavy menstrual bleeding (HMB), which affects a significant proportion of women with IBD, require collaborative management utilizing hormonal therapies and antifibrinolytics. Pregnancy, labour and delivery, and the postpartum period are high-risk phases. While IBDs like von Willebrand disease and haemophilia carriers may not inherently impair fertility or increase miscarriage risk, other severe factor deficiencies (e.g., factor X deficiency, factor XIII deficiency, and fibrinogen disorders) are associated with higher rates of miscarriage and antenatal haemorrhage, often requiring prophylactic factor replacement. Advances in preconception genetic counselling and prenatal diagnosis, including non-invasive prenatal testing (NIPT) and preimplantation genetic diagnosis (PGD), are crucial for informed reproductive choices and delivery planning. Careful assessment of coagulation status is mandatory for procedures like neuraxial anaesthesia, and mode of delivery requires shared decision-making to minimize cranial bleeding risk in an affected foetus. All IBDs, notably von Willebrand disease and haemophilia carriers, elevate the risk of primary and secondary postpartum haemorrhage (PPH), necessitating a multidisciplinary team approach and individualized haemostatic support. Furthermore, overcoming the historical under-recognition of symptomatic female carriers requires systematic screening and education to ensure optimal, lifelong care and reduced maternal morbidity.
Connell et al. (Thu,) studied this question.