Patterns of von Willebrand Disease Screening in Girls and Adolescents With Heavy Menstrual Bleeding

2018 ◽  
Vol 131 (6) ◽  
pp. 1121-1129 ◽  
Author(s):  
Amanda E. Jacobson ◽  
Sara K. Vesely ◽  
Terah Koch ◽  
Janis Campbell ◽  
Sarah H. OʼBrien
Haemophilia ◽  
2019 ◽  
Vol 25 (3) ◽  
Author(s):  
Amanda E. Jacobson‐Kelly ◽  
Sara K. Vesely ◽  
Terah Koch ◽  
Janis Campbell ◽  
Sarah H. O’Brien

2019 ◽  
Author(s):  
Kimberly Huhmann ◽  
Andrea Zuckerman

Heavy menstrual bleeding is a common presenting problem in the adolescent population. The average age of menarche is between 12 and 13 years. The most common reason for heavy menstrual bleeding soon after menarche is from an immature hypothalamic ovarian access, which spontaneously resolves once cycles become ovulatory. However, the broad differential diagnosis for heavy menses in adolescents includes coagulopathy, thyroid disease, sexually transmitted infections, specifically chlamydia, and chronic medical conditions. Von Willebrand disease is the most common bleeding disorder that can present with heavy menstrual bleeding at menarche or shortly after. A thorough history and physical exam with occasional labs needs to be completed and can assist in narrowing the differential diagnosis. Treatment of heavy menstrual bleeding consists of hormonal and nonhormonal options: combination oral contraceptive pills, patches, or rings taken continuously or cyclically; progesterone-only pills; progesterone implants; progesterone intrauterine devices; cyclic tranexamic acid; cyclic aminocaproic acid; and GnRH agonists with add-back therapy. This review contains 3 tables, and 28 references. Key Words: adolescent menses, anovulation, bleeding disorder, heavy menstrual bleeding, immature hypothalamic ovarian axis, menarche, treatment of heavy menses, Von Willebrand disease


Blood ◽  
2008 ◽  
Vol 112 (11) ◽  
pp. 3399-3399
Author(s):  
Andra H James ◽  
Evan R Myers ◽  
Chad Cook ◽  
Ricardo Pietrobon

Abstract Background: Case reports and small case series suggest that women with von Willebrand disease (VWD) are at a very high risk of bleeding complications with hysterectomy. Because the procedure may be beneficial to women who suffer from heavy menstrual bleeding, an understanding of the true risks involved is essential for appropriate decision making. Objectives: To estimate the incidence of bleeding and other complications in women with VWD who undergo hysterectomy. Methods: The United States Nationwide Inpatient Sample (NIS) from the Healthcare Cost and Utilization Project of the Agency for Healthcare Research and Quality for the years 1988–2004 was queried for all hysterectomies for nonmalignant conditions. Data were analyzed based on the NIS sampling design. Bivariate analyses were used to examine the differences between women with and without VWD. Multivariate analysis was used to adjust for potential confounders among women who underwent hysterectomy for heavy menstrual bleeding. Results: 545 of the 1,358,133 hysterectomies were to women with VWD. Women with VWD were significantly more likely to experience intraoperative and postoperative bleeding (2.75% versus 0.89%, p < 0.001) and require transfusion (7.34% versus 2.13%, p < 0.001) than women without VWD. One woman with VWD died (odds ratio = 28.49). Conclusions: While the risk of bleeding complications from hysterectomy in women with VWD is smaller than previously reported, women with VWD did experience significantly more bleeding complications than women without VWD. Nonetheless, for women who have completed childbearing, the risks of hysterectomy may be acceptable.


Blood ◽  
2017 ◽  
Vol 130 (Suppl_1) ◽  
pp. 674-674
Author(s):  
Amanda E. Jacobson ◽  
Sara K. Vesely ◽  
Terah Koch ◽  
Janis Campbell ◽  
Sarah H. O'Brien

Abstract Background Bleeding disorders in women are under-recognized and under-treated. Women are equally as likely as men to have bleeding disorders other than hemophilia and are disproportionately affected by these diseases due to the bleeding challenges of menstruation and childbirth. The most common bleeding disorder identified in women is von Willebrand disease (VWD). Heavy menstrual bleeding (HMB) is the most common symptom in women with VWD, occurring in up to 93% of patients. Among women with HMB, the reported prevalence of VWD ranges from 5─20%. Women with VWD are also more likely to be diagnosed with hemorrhagic ovarian cysts due to ovulation-associated bleeding and endometriosis due to increased retrograde menstruation. As a result, women with bleeding disorders are more likely to undergo hysterectomy and also undergo hysterectomy at an earlier age than women without bleeding disorders. In 2001, the American College of Obstetrics and Gynecology (ACOG) recommended VWD screening prior to hysterectomy in women with HMB. The actual frequency of VWD screening in clinical practice is unknown. Objectives In this study, we assess patterns of VWD screening in a nationally representative sample of women undergoing hysterectomies for HMB. Methods We used the Truven Health MarketScan® Research Databases which include the medical prescription claims of over 109 million covered lives as well as Medicaid data on 8.6 million patients from 14 states. The MarketScan Databases contain patient demographics, physician and facility claims and pharmacy claims. Procedure codes were used to identify women ages 10-44 years undergoing hysterectomy or hysterectomy alternative (HA) from 2011-2013. Subjects were required to have 12 months of continuous enrollment prior to surgery date. We utilized ICD-9 codes to categorize hysterectomy indications and only included women with a diagnosis of excessive bleeding as the indication for surgery. Women with fibroids, genital tract malignancy, and previously diagnosed bleeding disorders were excluded. We defined VWD screening as a laboratory claim for either VWF:Antigen and/or VWF:Activity within the 12 months preceding hysterectomy. To determine if patient and facility level characteristics impacted access to specialty hematology care and/or screening for VWD, we collected the following information: 1) known bleeding disorder diagnosis and/or endometriosis prior to surgery; 2) age; 3) whether patient was living in metropolitan statistical area (MSA; used as a surrogate marker for urban vs rural inhabitance); 4) number of miles and approximate travel time to nearest Hemophilia Treatment Center (HTC). We used ArcMAP® software to calculate distance between the MSA and nearest HTC. MSA data was only available for commercially-insured patients. (Figure 1) Logistic regression was used to assess factors related to the occurrence of VWD screening. Results We identified 13,790 women who underwent hysterectomy/HA for HMB. We excluded 138 with known bleeding disorders leaving 13,652 women in our final analysis (Table 1). Of these, 74 (0.5%) were screened for VWD within 12 months preceding surgery. There were 2,000 women (15%) who underwent other coagulation tests, most commonly prothrombin time and partial thromboplastin time. We had MSA data on 11,557 commercially-insured women, of whom 72.4% lived within a MSA. Women living in a MSA were screened more often than those outside of a MSA (p=0.013). For those living within a MSA, the odds of being screened for VWD was lower in women with endometriosis (OR=0.54, 95% CI 0.31, 0.97; p=0.038) and women living &gt;100 miles from the nearest HTC (OR=0.29, 95% CI 0.11, 0.81; p=0.017). Discussion This study demonstrated that despite ACOG expert recommendations, the frequency of VWD screening in a nationally-representative population of publically and commercially-insured women undergoing hysterectomy for HMB was very low. Greater distance from a HTC or a prior diagnosis of endometriosis further reduced the likelihood of VWD screening. It is important to increase awareness that a diagnosis of endometriosis does not rule out the presence of a bleeding disorder. This study brings to light the need for the hematology community to improve education and awareness among women's health providers in order to identify women with bleeding disorders and allow for optimal medical management of HMB prior to surgical consideration. Disclosures No relevant conflicts of interest to declare.


Hematology ◽  
2019 ◽  
Vol 2019 (1) ◽  
pp. 148-151
Author(s):  
Andra H. James

Abstract This is the obstetrician’s view on 3 different clinical scenarios involving bleeding and thrombotic disorders. In the first scenario, an 18 year old with a history of heavy menstrual bleeding since menarche presents with abdominal pain and ultrasound findings suggestive of a hemorrhagic ovarian cyst. The association with an underlying bleeding disorder is recognized. The goals of management, which are controlling hemorrhage and preserving fertility, are stated. Ovarian suppression, the most effective method to prevent recurrent hemorrhagic ovarian cysts, is outlined. Long-term management of heavy menstrual bleeding with hormonal contraception is described. In the second scenario, the same patient returns 5 years later for a preconception visit. The potential risks to an unborn baby with von Willebrand disease (VWD) are addressed. The natural rise in von Willebrand factor (VWF) during pregnancy is discussed, but the fact that women with VWD do not achieve the same VWF levels as women without VWD is emphasized and the implications are presented. In anticipation of pregnancy, the need for nonhormonal management of heavy menstrual bleeding and hemorrhagic ovarian cysts is mentioned. In the third and final scenario, the patient’s cousin with factor V Leiden seeks consultation regarding the risks of thrombosis with in vitro fertilization. The steps of assisted reproductive technology are described. The strategies to prevent venous thromboembolism by preventing ovarian hyperstimulation and reducing the likelihood of multiple gestation are detailed.


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