Today, May 15, 2020 is #REDS4VEDS Day; a global campaign dedicated to raising awareness of vascular Ehlers-Danlos syndrome (vEDS).
vEDS is a dominantly inherited, genetic connective tissue disorder. vEDS is particularly serious because of the risk for spontaneous arterial or organ rupture.
vEDS is estimated to occur between 1 in 50,000 individuals to 1 in 200,000 and results from pathogenic variants in COL3A1, which is responsible for producing chains of type III procollagen, a major protein in the walls of blood vessels and hollow organs. The changes in COL3A1 known to cause vEDS, alter the framework used to build connective tissue, which decreases its capacity to withstand stress.
Potentially fatal effects include arterial aneurysm, dissection, and rupture; bowel rupture; and rupture of the uterus when pregnant. Life span is often affected.
The importance of advocacy and awareness
The Ehlers-Danlos Society is proud to be a member of the vEDS Collaborative, a group of patients, family members, researchers, and clinicians, dedicated to developing a patient-centered plan for scientific research, to improve the management of vEDS, and to increase the quality of life for all those impacted by the condition.
The vEDS community has lost some great advocates this year. John DeMasi of Fight vEDS, was a leading light in the vEDS community, fighting for vEDS awareness, and understanding. Chrystal Nichols, another champion in this community, was an incredible advocate for vEDS. We will continue to fight on to improve diagnosis and treatment for all of those impacted by vEDS, keeping John and Chrystal so close to our hearts, and all those who have lost their lives.
Raising awareness of vEDS is vital, and our new awareness video, launched on #REDS4VEDS Day 2020, hopes to highlight some of the experiences of the community living with vEDS, the impact on their families, the importance of education and research, and hopes for the future. There is no better way of hearing what it is like to live with vEDS, than from individuals who have vEDS themselves.
vEDS ECHO: An EDS ECHO program
A vEDS diagnosis is most often sought because of family history; someone’s medical history; or pregnancy complications at a young age. Confirmation helps rule out other possible causes for symptoms as well as establishing a clear need for medical surveillance, treatment, and study of family members.
After diagnosis, the first priority is assembling an integrated care team to provide a clinical and social support network for the individual and their family. Management is complex and requires multiple specialists who can respond to and manage major complications.
In 2019, The Ehlers-Danlos Society launched vEDS ECHO: a program led by Dr. Sherene Shalhub dedicated to increasing awareness and education in all aspects of diagnosis and management of vEDS.
VEDS ECHO is a revolutionary program that seeks to support health professionals around the world in caring for patients with vEDS, facilitated by Dr. Sherene Shalhub, vascular surgeon, a translational researcher at the University of Washington, and Director of the Multidisciplinary Vascular Genetics Clinic at the University of Washington Medical Center. “It is our goal to bridge the physical distances virtually to create a shared multidisciplinary space to care for patients with vascular Ehlers-Danlos syndrome,” said Dr. Shalhub.
Clinical experts run a program that covers the main aspects of vEDS. Participants are able to share their cases and questions in the sessions and are guided to further educational materials and support. Continued Medical Education (CME) credits are available at no charge to participants.
Support for the vEDS community
The Ehlers-Danlos Society offers monthly virtual support groups for those living with vEDS and their families, and also a group dedicated to parents; parents who have vEDS, and those who have children with vEDS.
July 30-August 1, 2021 will see our first vascular Ehlers-Danlos syndrome family weekend, partnering with Camp Joy in Cincinnati, Ohio, USA. We hope to see the biggest gathering of the vEDS community in the world, with educational presentations from world-leading vEDS specialists. The Ehlers-Danlos Society has secured a grant to fund those in the community with a “pay what you can afford” program.
Much more awareness is needed of this type of Ehlers-Danlos syndrome, it is important that health professionals are educated in the presentation of vEDS, genetics and familial impact, possible implications and surgical interventions, and treatment guidance. The Ehlers-Danlos Society is committed to improving the lives of those impacted by vEDS, and facilitating global awareness and education.
Our strength begins with hope.