105 Chapter 5 Heavy menstrual bleeding (HMB) In our cohort 65% of the women who ever menstruated had significant abnormal bleeding during their menstruations. In 40% of all patients who menstruated, abnormal menstruation contributed to an increased total BAT score. This means that menstrual bleeding disorder in OI contributes significantly to an increased BAT score. Menorrhagia is the most frequent health problem for a woman during reproductive life, occurs in about 30% of women, is underdiagnosed and poorly treated 41. It has a major impact on a woman’s quality of life for which the NICE guideline on heavy menstrual bleeding encourages clinicians to focus their interventions on improving quality of life rather than focusing on blood loss 42. Besides this already large problem in the general population, menorrhagia is a prominent feature of most bleeding disorders 43–45. Among patients with moderate to severe Von Willebrand Disease in the Netherlands a prevalence of menorrhagia of nearly 80% was demonstrated 46. No literature is available for the prevalence of menorrhagia in patients with OI, but our study suggests an increased prevalence of menorrhagia in OI compared to the general population. With regard to the major impact on quality of life and the relatively large prevalence of HMB in OI it should be noted that a substantial proportion of women with menorrhagia with abnormal laboratory haemostasis has shown to respond to therapy with desmopressin and/or tranexamic acid with decreased bleeding and improved quality of life 47. Therefore, pro-active treatment for HMB in OI should be considered. Postpartum haemorrhage (PPH) In our cohort 53 female patients stated they had ever been pregnant. Of these, 47 patients reported ever having given birth. 16 Out of 47 women (34%) experienced significant PPH which is much higher than the incidence of PPH in many high-income countries 48. Recent research on pregnancies with OI shows significant higher rates of blood transfusion compared with US normative data, a significant increase in pre-pregnancy bleeding and a non-significant increase in post-partum bleeding compared with non-OI pregnancies 49,50. PPH though is worldwide known as a major contributor to maternal death 51 and even mild haemostatic abnormalities can be a significant risk factor for severe PPH 52. Also previous PPH results in a much higher risk of recurrent PPH during a subsequent delivery 53,54. The high prevalence of PPH in OI as found in this study highlights the need for awareness of possible bleeding tendency in pregnant woman with OI. Veen et al. found that PPH can be the first symptom of an inherited bleeding disorder, but also emphasises assessing the bleeding history as 75% of the women with a bleeding disorder had additional bleeding symptoms other than PPH 55. In our cohort the percentage of women with PPH and an elevated BAT score, meaning at least 1 significant bleeding symptom other than PPH, was 63%. The use of a structured
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