The American Society of Hematology (ASH), the International Society of Thrombosis and Haemostasis (ISTH), the National Hemophilia Foundation (NHF), and the World Federation of Hemophilia (WFH) have developed complementary clinical practice guidelines on detection and diagnosis. manages von Willebrand Disease (VWD), the most common hereditary inflammatory disorder in the world. The guidance was published today in Blood clots.
VWD affects about 1% of the world’s population, and is the most common inflammatory disorder. Although VWD occurs equally among men and women, women are more likely to notice the symptoms due to heavy or excessive swelling during their menstrual periods and after childbirth.
This inherited condition leads to a reduced production, absence, or abnormal function of the von Willebrand clotting protein factor.
VWD can cause abnormal bleeding from minor or minor injuries, frequent runny noses, bruises, swelling in joints, and heavy menstrual periods and post-partum bleeding in women. Symptoms can vary from patient to patient or in a single patient during his or her lifetime.
Primary care providers, pediatricians, obstetricians, and gynecologists who monitor for abnormal bleeding often refer their patients to a hematologist for further testing and management. Many people with mild symptoms do not get an immediate diagnosis and live for several years with untreated bleeding or do not realize they have VWD until they can get potentially hard blood. banned.
“While VWD is a common inflammatory disorder, it is also complex, presenting challenges in timely diagnosis and appropriate management of bleeding for patients,” said 20H ASH President Martin S. Tallman, MD, of Sloan Kettering Memorial Cancer Center.
“Because diagnosis is not straightforward and symptoms go deeper, reliable guidance is needed to help improve the quality of care for patients. There are no better partners for ASH in this endeavor than ISTH, NHF , and WFH, which has a common knowledge in VWD from the scientific and patient perspectives around the world, has contributed to this effort. “
In particular, the guidelines make key statements on laboratory test thresholds to classify VWD to be more inclusive of patients suffering from bleeding, but whose blood function does not meet. to the thresholds currently accepted for diagnosis.
In addition, the guidelines for the first time recommend that VWD patients who suffer from frequent bleeding and reduce the quality of life of normal VWD prophylaxis – injectable concentration of clotting protein – several times a week. per week. Together, the guidance on judgment and control brings together 19 recommendations.
The guide was developed by two expert panels made up of 32 people, including US-based and international hematologists, individuals living with VWD, and scientists with experience in it. the evidence synthesis and evaluation and management development method.
Clinical issues were developed and prioritized by the panels, and an international study was completed to identify the most important clinical issues. A systematic review of the available evidence was conducted by the University of Kansas Medical Center and the panel cited this evidence to make recommendations.
This process sheds light on the lack of robust evidence on which to base recommendations, so the report calls for further research.
“These guidelines are a good example of multi-agency collaboration ensuring that the development guidance represents the most up-to-date and relevant advice on detecting and treating the disorder. This is a common, but often misunderstood, blow, “said ISTH Vice President Claire McLintock, MD.
“It was a great pleasure to work as part of the amazing team that developed the leadership in judging VWD. “
People with VWD made up about a quarter of the writing of each control panel. As full voting members, they applied their knowledge to all aspects of the rigorous management development process, from prioritizing clinical issues, through assessing the available evidence, to considering factors such as stakeholder values and preferences, equality. , and feasibility. .
Their involvement, with the active support of panel chairs and collaborative bodies, was crucial in ensuring that proposals were developed with the greatest appropriateness and strong community confidence. Collaboration with WFH and NHF was critical to engaging the wider VWD patient community.
With their extensive established networks of action with healthcare professionals in centers for holistic care of inflammation problems and patient groups across the U.S. and worldwide, the four collaborative organizations, ASH, ISTH, will play an important role. NHF, and WFH in the creation and distribution of managed resources, and their implementation in the individual care efforts and wider advocacy.
“This guidance is a vital step in our drive to address the difficulties people go through in obtaining proper, timely diagnosis and appropriate treatment. The challenge ahead is to educate both those living with VWD and recommendations-led healthcare professionals, ”said Leonard Valentino, MD, President and CEO of NHF.
We were excited to work with ASH, ISTH, and WFH to create these and bring them into the community. In 2017 we gathered members of the global VWD community for the first WFH International Symposium on VWD. Representatives from countries around the world told us that clinical practice guidance was the only tool they needed to provide holistic care for people with VWD. “
Cesar Garrido, President, Hemophilia Universe Alliance
“The WFH is very proud to deliver these guidelines today, with our partners from ASH, ISTH, and NHF, to the global community. As well as offering guidance to clinicians and individuals. with VWD while involved in personal care decisions, they form the basis for important advocacy campaigns to access the recommended diagnostic methods and treatment options in all develop a country. “
The publication of the guide will be accompanied by educational tools and resources to help patients, hematologists and other healthcare providers understand and implement the recommendations.
Source:
American Society of Hematology