(86) Menstrual management in patients with hypermobility disorders

Background: Individuals with hypermobile Ehlers-Danlos Syndrome (hEDS) and Generalized Joint Hypermobility (GJH) have similar menstrual bleeding experiences as individuals with mild inherited bleeding disorders, likely due to abnormalities of collagen which participates in several steps of primary hemostasis. Managing patients with hEDS and GJH is complex, and many providers may not feel comfortable addressing heavy menstrual bleeding (HMB) symptoms in this population. We therefore sought to describe the clinical and management patterns of adolescent and young adult (AYA) patients with hEDS and GJH presenting to a multidisciplinary gynecology/hematology clinic.

Methods: This is an IRB-approved, retrospective chart review of patients presenting to a multidisciplinary gynecology/hematology clinic at a single, tertiary children’s hospital from 2019—2023. Included were patients with a diagnosis of hEDS (by a geneticist) or GJH (as defined by Beighton criteria) under age 25. Extracted data included baseline demographics, bleeding assessment scores, treatment modalities, baseline ferritin, and presence of depression or anxiety. Descriptive data was summarized using mean and standard deviation, median and range, or proportions as appropriate.

Results: Thirty-five patients met inclusion criteria. Table 1 summarizes baseline demographic and medical characteristics. Many (25%) had a concurrent diagnosis of von Willebrand disease. The median baseline ferritin was 16, and 71.4% also had a diagnosis of anxiety or depression. Table 2 summarizes the management patterns of heavy menstrual bleeding in this group. On average, patients required three lines of therapy before bleeding control was achieved, with 57.1% ultimately utilizing a levonorgestrel intrauterine device (LNG-IUD) with or without another method and 54.3% ultimately utilizing an antifibrinolytic with or without another method. While 91.4% of patients required iron replacement, oral iron was sufficient in only 17.1% of patients.

Conclusions: GJH and EDS are commonly encountered in individuals presenting to combined gynecology/hematology clinics given the high frequency of HMB in this group. Patients often require several lines of therapy to control HMB, have a high frequency of oral iron intolerance, and often have concurrent depression or anxiety, similar to that which has been reported in patients with mild inherited bleeding disorders. Anti-fibrinolytic therapy and the LNG-IUD (either alone or in combination with other methods) are frequently utilized in this population to manage HMB.