Given the low prevalence of Vascular Ehlers-Danlos Syndrome, many general physicians have never seen a patient with the condition. Certain specialties may be more likely to have experience with vEDS (such as geneticists, vascular surgeons, and cardiologists); however, in most cases finding a capable group of doctors for a condition like vEDS may require more effort.
Finding Physicians: United States versus Europe
The general recommendation at the time of diagnosis is to establish a Care Team, or a set of doctors to coordinate the patient's care (1). In countries such as France, the United Kingdom, and many other European nations there is an established referral network. After diagnosis, a patient in these countries is referred to a medical center which has both experience and expertise in vascular EDS (1).
Within the United States, there is no centralized referral center. There are, however, many medical centers with vEDS experience and the goal of establishing a Care Team remains the same. University Medical Centers or large free-standing clinical centers with large referral populations are most likely to have experience and knowledge with vEDS (1). A list of medical centers and physicians within the United States who have experience caring for vEDS patients can be found here.
Care Team Composition
A patient’s care team should at a minimum consist of a primary care physician to help manage their general medical care, a vascular surgeon and a general surgeon (1). Often a geneticist is included in the Care Team. Geneticists can be helpful for further information about the disease and evaluation of other family members who may need to be tested (1).
Care Team Objectives
The goal of the Care Team is to help manage the disease, and treat the patient in the event of any major complication (arterial rupture, bowel rupture, etc.)(1). This team will know you and your disease, which will help them make better decisions about your care and improve outcomes.
What is a vEDS care team and how do I get started?
What should I do with my Care Team?
Work with your Care Team to establish a clear protocol of what to do in the event of an emergency (1). Be able to answer questions about: What emergency room should I go to? Who should I contact if I am going to the emergency room? What should I tell the Emergency Room physician about my condition(1)? It can be helpful to obtain an emergency care card or 'passport' which can be given to providers taking care of you in an emergency setting, so they know of your vEDS diagnosis (1).
(1) Byers PH, Belmont J, Black J, De Backer J, Frank M, Jeunemaitre X, Johnson D, Pepin MG, Robert L, Sanders L, Wheeldon N. 2017. Diagnosis, natural history and management in vascular Ehlers-Danlos syndrome. American Journal of Medical Genetics Part C (Seminars in Medical Genetics). 175C:40-47.