Management of menorrhagia in women with inherited bleeding disorders: general principles and use of desmopressin

Abstract

The haemostatic system has a central role in controlling the amount and the duration of menstrual bleeding, thus abnormally prolonged or profuse bleeding does occur in most women affected by inherited bleeding disorders. Whereas irregular, premenarchal or postmenopausal uterine bleeding is unusual in inherited or acquired heamorrhagic disorders, severe acute bleeding and menorrhagia at menarche and chronic menorrhagia during the entire reproductive life are common manifestations. Prevalence and morbidity of menorrhagia in inherited bleeding disorders have been poorly investigated. It can be estimated that 40% to 60% of currently menstruating women with type 1 or 2 and more than 60% of women with type 3 VWD complain of menorrhagia with a significant impact on their quality of life. Menorrhagia may be particularly distressing in adolescents because of their delicate emotional equilibrium. Similar epidermiology has been described in other inherited disorders like factor XI deficiency, platelet functional defects and in carriers of haemophilia A and B. Women presenting with ''isolated'' menorrhagia, that is without significant additional bleeding symptoms, a situation reported by up to 15% of healthy women, do not demand investigation to exclude an occult bleeding disorder. A multidisciplinary approach is required for diagnosis and treatment. Gynaecological supervision is always required to exclude organic causes unmasked by the bleeding disorder. Treatment options are similar to those for menorrhagia in general with the addition of desmopressin and replacement therapy and the exclusion of non-steroidal anti-inflammatory drugs. The therapeutic plan should take into consideration the patient's preferences, age and severity of bleeding. Iron supplementation is of paramount importance. Remedies used in clinical practice for menorrhagia in general (tranexamic acid, combined oral contraceptives [COC], levonorgestrel intrauterine system [LNG-IUS]) are first tried. In case of failure or contraindication (COC and LNG-IUS are best avoided in adolescents), before considering surgical options, treatment with desmopressin becomes the preferred choice in patients known to be responsive. The availability of desmopressin preparations for self-administration makes home treatment feasible in well selected cases. The treatment is efficacious and safe provided that patients are instructed to self-administer the agent only during the first two or three more heavy days of menstrual period, for a maximum of three to four doses and no more than two consecutive administrations at a 12-h interval.

von Willebrand disease

“…Prolonged or excessive bleeding following surgery or trauma is often reported. Affected females also frequently experience menorrhagia (usually occurring since menarche) 11 and prolonged or excessive bleeding following childbirth. 12 The utility of standard clinical assessment tools to score occurrence of symptoms and their severity as part of VWD diagnosis is becoming recognized.…”

von Willebrand factor to the rescue

“…Menorrhagia also has a negative impact on overall life activities. 83,84 The daily management of symptoms, the unpredictable and potential life-threatening nature of bleeding episodes, and concerns about the future are furthermore associated with anxiety and depression. 80 Overall, it is clear that treatment strategies that are preventative rather than reactive, with a sustained effectiveness, would be particularly valuable for these persons.…”

Hereditary bleeding disorder, factor ix deficiency in females: a case series

“…while, prevalence and morbidity in females with menorrhagia in inherited bleeding disorders have been poorly investigated. Currently 40% to 60% menstruating women complain of menorrhagia with a significant impact on their quality of life, estimated to be of type 1 or 2 and more than 60% of women with type 3 vonWillebrand Disease [4]. In cases, where there is no family history of the disorder the condition is the result of a spontaneous gene mutation.…”