14-year-old

I have a 14-year-old who is 15 months past menarche with von Willebrand disease. She has no history of severe bleeding episodes prior to menarche, but since menarche, has bled almost daily. She did stop briefly with Premarin but has failed multiple doses and types of OCPs, progestins, and multiple combination and doses of estradiol and progestins. She has also failed Amicar and Lysteda. She has a normal pelvic ultrasound with endometrial measurements between 4 and 6 mm. She had a Mirena inserted 3 months ago (with a normal endometrial cavity at that time) and has continued to bleed. We are beginning to add oral estradiol at this time. The family is understandably frustrated since she has been bleeding almost daily for 15 months. She has normal thyroid studies, cbc, and ferritin is in the low normal range. We would appreciate any thoughts to help this young woman and her family.

It sounds like she has tried quite a few hormones (doses unspecified). With a Mirena, irregular bleeding is expected with gradual reduction in bleeding over time. Another option is Depo Lupron to shut her down centrally; however, she may continue to have at least one flare bleed until things down regulate. If she does not respond to these approaches, she may need von Willebrand Factor (VWF) concentrate infusion, particularly if her von Willebrand Disease (VWD) is more severe.

Jennifer E. Dietrich, MD, MSc, FACOG
Associate Professor
Department of Obstetrics and Gynecology and Department of Pediatrics
Baylor College of Medicine
Chief of Pediatric and Adolescent Gynecology Texas Children's Hospital
Division Head Pediatric and Adolescent Gynecology
CME Director Department of Obstetrics and Gynecology

Barbara A. Konkle, MD
Director, Clinical and Translational Research, Puget Sound Blood Center
Medical Director, Hemostasis Reference Laboratory, Puget Sound Blood Center
Professor of Medicine/Hematology, University of Washington