We have a young woman diagnosed with VWD who has very heavy menstrual cycles and is not able to tolerate any hormonal support, including levonorgestrel IUD. She also has endometriosis. She has had five periods in the last eight weeks and will use desmopressin beginning tomorrow, one spray to each nostril for two days. She is so ill from her periods she has had to quit her job and suspend schooling. She will be having further lab testing including FXIII, dense platelet granules and plasminogen. We would like to start her on tranexamic acid if her insurance will cover it. Any other suggestions?

Carolyn Solomon, RN, Nurse Coordinator and Pediatric Thrombosis Coordinator and Hemophilia Nurse
Michigan State University Center for Bleeding and Clotting Disorders

In our evaluation, we would want to know how old this young woman is, how severely affected she is with VWD and how far from menarche she is. We would be interested to know the hormonal treatments that have been attempted and the extent to which a gynecologist has been involved. We do know she has failed hormonal treatments, including the levonorgestrel IUD, and you are trying desmopressin.

We would be interested to know her thyroid function, her current hemoglobin, and a current assessment of her iron stores. With eight periods in two months, she is having irregular bleeding, not just heavy bleeding, and we would not expect that her bleeding is going to respond to hemostatic therapy alone; it will likely require the joint efforts of the hematologist and the gynecologist. She will need cycle control and hemostasis. It is not clear that all potentially tolerable hormonal manipulations have been tried, such as monthly short courses of progestin to induce withdrawal bleeding. This may give her cycle control, especially when used in conjunction with desmopressin or antifibrinolytic. 

Concerning tranexamic acid, there are no clinical trials in adolescents.  As far as the dosing, if she is adult weight/size, then we would expect the same dose as that for an adult woman. In a young woman with VWD, when cycle control is achieved, and antifibrinolytics and desmopressin have failed, we have no reservations about using VWF concentrates to achieve hemostasis.

Andra James, MD, MPH
Founder of the Women's Hemostasis and Thrombosis Clinic
Duke University Medical Center
Associate Professor of Obstetrics & Gynecology
Duke University School of Medicine
Durham, NC