My disease is rare. Does anyone care? Ask The VEDS Movement

As I write this, I’m about a week away from my yearly (sometimes twice-a-year) checkup at Cleveland Clinic. This year, instead of boarding a flight, my family and I are traveling there in our RV. The days and weeks leading up to these check-ups always leave me a little (or a lot) anxious. The good news is that on this visit, I’m not getting the full battery of tests ( the dreaded CT scan with contrast) that I usually get, because I just “passed” that test back in the spring. This time, the cardiologist just wants to check on my aortic valve, which began leaking somewhere along the way. So far every visit to Cleveland has been mostly good news and I’m happy that my family will be with me this year.

I’ve lost track of how many times I’ve been to Cleveland. But the first time was eventful, when I was shipped there for surgery on an aneurysm that was quickly growing in my abdomen. After that surgery, we knew that we were going to get to know Cleveland well. It’s part of life with a genetic condition.

When I was diagnosed with Vascular Ehlers-Danlos Syndrome (VEDS) four years ago, the information came on a two page printout, with lots of new words and medical journal citations. My wife had to retrieve this document from the records office of one of the hospitals that had saved my life that year.

At first, we were excited. “Hooray! We know why my arteries are exploding! Now what medicine do I need?”

Then, we started Googling. Never a good idea. There’s a lot of very generic information about VEDS out there: “the most serious form of EDS,” “no cure,” “median life span of 48,” “prone to spontaneous ruptures of major arteries.”

The euphoria of identifying my condition wore off pretty fast.

We looked for a silver lining. There was a tiny piece of good news on that page. My specific mutation of VEDS is associated with a “later onset” and “better surgical outcomes.” The fact that I was reading that page was proof of that. But real information was difficult to come by.

Awareness about VEDS is improving. Unfortunately, with limited resources, and VEDS being one of the rarest of 13 types of EDS, there isn’t really a VEDS equivalent to the American Cancer Society or the American Heart Association. Research is limited by lack of funding, and lack of subjects. There just aren’t that many of us to put together trials. And we’re not exactly the easiest kind of patients to experiment on. (Don’t give me a placebo.) With a disease this rare, it can start to seem like it’s not worth curing. We have to hope that gene therapy cures for other diseases will benefit us in the long run.

It can feel pretty isolating and many VEDS people may never meet another person who has it. I’ve met one in person. Weirdly, we could have been brothers. (He was dashing just like me.)

The good news is that a community focused on supporting those affected by VEDS, educating professionals on VEDS, connecting VEDS patients with each other, and supporting research has begun to form. And it’s coming from an unlikely place. The Marfan Foundation.

Marfan Syndrome is a condition different from VEDS that is often associated with extremely tall people (there’s suspicion that Abraham Lincoln had Marfan Syndrome, as well as Andre the Giant – although I read conflicting information on whether he a different growth abnormality.) The thing that VEDS and Marfan’s have in common is the risk of problems with the aorta, such as aortic aneurysm, dilation, and dissection. 

Because of the similar risks we face, The Marfan Foundation has started something called The VEDS Movement. It’s led by Katie Wright, VEDS sufferer herself, whose own experience getting diagnosed in the Seattle area put her in touch with some of the condition’s top experts. Her tireless effort to get answers for herself will end up helping everyone diagnosed from here on out.

I met Katie Wright (virtually) when she started hosting a monthly VEDS support group. She had answers to questions I never asked – and I considered myself knowledgable. She knew all the prominent VEDS specialists on a first name basis. She understands the alphabet-soup of different mutations. She talks about meeting with her local hospitals so that they have a plan for her in place. She hosts a podcast where she interviews people with VEDS. I think it might safe to say that Katie Wright knows more people with VEDS than anyone who isn’t a specialist ever has.

The VEDS Movement put together a detailed toolbox for patients that answered many of the questions that puzzled Monica and I when I was diagnosed. I’m going to rip them right out of an email she sent me and put them here:

The VEDS Movement:

1.       Institutional Directory: A list of institutions around the country who have indicated they are caring for individuals diagnosed with VEDS. https://thevedsmovement.org/resources-and-answers/find-a-doctor/

2.       Emergency Preparedness Kit and VEDS Emergency Card: This kit contains a letter to the Emergency Department, a VEDS Emergency Card, a fact sheet for EMTs and Paramedics, and a checklist to help you prepare documents you may need in an emergency. https://thevedsmovement.org/what-to-expect/emergency-preparedness/

3.       Ask a Question: The registered nurse in the Help and Resource Center is here to help you with any questions you have about you or your family member’s diagnosis. Visit https://thevedsmovement.org/resources-and-answers/ask-a-question/ to reach the Help and Resource Center.

4.       Virtual Support Groups: There are several virtual support groups that meet monthly and are a great way for individuals in the community to connect with others in similar situations. https://thevedsmovement.org/resources-and-answers/virtual-support-groups/

5.       Charging Forward: Virtual Medical Symposium Series: This medical symposium series features the leading experts of the Foundation’s Professional Advisory Board presenting on relevant topics for the VEDS community. https://thevedsmovement.org/resources-and-answers/virtual-symposiums/medical-series/

6.       Events- Events are a great way to connect with others in the community and the experts. There are a variety of events to choose from, including Annual Conference, Regional Symposiums, Walk for Victory, and Camps for Kids and Families. Visit https://thevedsmovement.org/events/ to learn more.

7.       Parent Toolkit– We have numerous resources in our Parent Toolkit for parents of children with VEDS. https://thevedsmovement.org/resources-and-answers/parent-toolkit/

8.       Research: We keep a current list of research studies enrolling individuals with VEDS on our website. https://thevedsmovement.org/research/for-patients/

Reach Katie Wright at kwright@TheVEDSMovement.org. She also has a great blog and podcast.

TheVEDSMovement.org


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