Quality of Reproductive Care in Women with Von Willebrand Disease

 描述(由申请人提供)：在美国，大约有200万女性(占所有女性的1%)可能患有未确诊的出血性疾病，但其中许多疾病多年来一直没有被发现或从未被诊断出来。患有出血性疾病的妇女月经大出血、缺铁性贫血、产前出血、产后出血的风险增加，并可能接受不必要的子宫切除术和其他可能导致额外并发症的子宫手术。女性常见的出血性疾病是von Willebrand病(VWD)，定义为von Willebrand因子的数量或质量缺陷。为了减少未确诊的VWD并发症的发生率和发病率，专家指南建议对月经过多的青少年进行VWD筛查，并在对月经过多的青少年进行子宫切除术之前进行筛查。对于诊断为VWD的孕妇，建议对VWF进行晚期监测，以最好地计划安全的分娩和减少产后出血的风险。尽管VWD是一种常见疾病，据估计，美国女性的患病率为160万，但过去在这一领域的研究主要限于单一机构环境或血友病治疗中心的患者登记。因此，病情较轻或前往治疗中心的机会有限的妇女比例偏低。我们的长期目标是进行研究，帮助降低患有潜在出血疾病的女性生殖出血的频率和发病率。利用管理索赔数据来研究VWD女性的出血并发症将为我们的团队提供独特的途径，接触到大量和多样化的被诊断为VWD的女性，包括那些患有轻度疾病的女性。在拟议的研究中，我们将利用ClinformaticsDataMart，一个代表4,500万覆盖生命(包括7,000名患有VWD的育龄妇女)的纵向卫生保健数据库，调查2000-2013年期间VWD妇女的生殖卫生保健质量。我们建议的具体目的是：1)调查距离血友病治疗中心的距离以及其他患者和机构的特点对月经过多和因出血过多而接受子宫切除术的妇女进行VWD筛查的频率的影响；2)确定产后出血的人群频率和时间，并比较VWD或DO与未接受晚期妊娠VWF监测的妇女产后出血的频率。查明诊断为VWD的妇女和VWD高危妇女在生殖保健方面的差距，将有助于开展有针对性的教育和宣传工作，并改进干预措施的实施，以减轻VWD对育龄妇女的影响，消除获得高质量护理的障碍。我们的研究旨在找出实施NHLBI和其他有关VWD评估和管理的专家指南的障碍，并支持2020年健康人目标，以增加VWD女性患者得到及时和准确诊断的比例。

 DESCRIPTION (provided by applicant): Approximately 2 million women (-1% of all women) in the United States may have an undiagnosed bleeding disorder, yet many of these disorders remain undetected for years or are never diagnosed. Women with bleeding disorders are at increased risk for heavy menstrual bleeding, iron deficiency anemia, antepartum bleeding, postpartum hemorrhage, and may undergo unnecessary hysterectomy and other uterine surgeries that can lead to additional complications. A common bleeding disorder in women is von Willebrand disease (VWD), defined as a quantitative or qualitative deficiency of von Willebrand factor. To decrease the frequency and morbidity of complications from undiagnosed VWD, expert guidelines recommend screening for VWD in adolescents with heavy menses and prior to any hysterectomy for heavy menses. For pregnant women with diagnosed VWD, third trimester monitoring of VWF is recommended to best plan for a safe labor and delivery and reduce the risk of postpartum hemorrhage. Although VWD is a common disease, with an estimated prevalence of 1.6 million American women, past research in this area has been primarily limited to single institution settings or patient registries derived from hemophilia treatment centers. Women with milder disease or limited access to treatment centers are therefore underrepresented. Our long-term goal is to perform research that aids in decreasing the frequency and morbidity of reproductive bleeding in women with underlying bleeding disorders. Utilizing administrative claims data to study bleeding complications in women with VWD will provide our team unique access to a large and diverse population of women diagnosed with VWD, including those with mild disease. In the proposed study we will utilize the ClinformaticsDataMart, a longitudinal health care database representing >45 million covered lives (including >7,000 reproductive-aged women with VWD), to investigate the quality of reproductive health care in women with VWD during the time period of 2000-2013. The specific aims of our proposal are to: 1) investigate the impact of distance to hemophilia treatment centers and other patient and facility characteristics on the frequency of VWD screening in adolescents with heavy menses and women undergoing hysterectomy for excessive bleeding, and 2) identify the population-based frequency and timing of postpartum hemorrhage, and compare the frequency of postpartum hemorrhage in women with VWD or do and do not undergo third trimester VWF monitoring. Identifying gaps in reproductive care in women with diagnosed VWD and those at risk for VWD will allow for targeted educational and advocacy efforts, and improved implementation of interventions to mitigate the consequences of VWD in reproductive-age women and eliminate barriers to high­ quality care. Our research seeks to identify barriers to implementation of the NHLBI's and other expert guidelines regarding the evaluation and management of VWD, and supports the Healthy People 2020 goal to increase the proportion of women with VWD who receive a timely and accurate diagnosis.