What is VEDS?

If you’re sitting there thinking, “What in the world is Vascular Ehlers Danlos?” then you are in very good company. It is a very rare genetic disorder and it is actually the rarest and most severe of the twelve different types of Ehlers Danlos Syndrome (EDS). When I say that it’s rare, I mean that I could walk into almost every hospital and say, “Hey! I have Vascular Ehlers Danlos” and the doctors would respond something to the effect of, “how do you spell that?” Why do they want to know how to spell that, you ask? So they can google it. Just like the rest of us. So, in theory, if you continue reading this or follow any of the links I have put at the bottom, you will be more of a pro than the pros are! So let’s get down to business and I will attempt to explain what exactly this crazy thing called VEDS is and what the symptoms and limitations are. However, in my limited ability and knowledge, please do not take all of this as law. This is just the way that I see it and have come to understand it. For more information and a better understanding, the links below have a lot more information.

What is VEDS? Basically, it is a dominant genetic disorder (meaning that it only needs one copy of the gene to manifest itself in an individual; if someone has it, they have a 50% chance of passing it to their kids) that is characterized by a lack of collagen in the vein/artery walls as well as in the walls of hallow organs, such as: the lungs, bladder, uterus (in women), stomach, intestines, etc. That is a really fancy way of saying that the walls of our veins, arteries, and hallow organs are a lot weaker than normal people’s. Do you like metaphors? I do, so here’s one for understanding the whole collagen thing:

Honeycomb Metaphor:  Think of the walls of the arteries, veins, and organs like a wall of honeycomb that fit together perfectly (this is a normal person’s wall). Now, imagine that some of those pieces are missing and then, on top of that, there are some that are misaligned (this is a person with VEDS). Because the walls are not structurally sound, it makes us more likely to rupture. That is why the average lifespan of someone with VEDS, as of the research done a few years ago, is around 48 (don’t worry, our geneticist says that he thinks that number is way too low). Don’t start freaking out on me just yet. A rupture is pretty bad, don’t get me wrong, it is exactly what it sounds like: the walls of the vein/artery or organ bust open and there’s major pain (in most cases, but not all), but that’s never happened to me! God has thoroughly blessed me with 21 full years of zero ruptures and that was before I knew anything about it. Now that I know about it, there are a few things that I can do in order to reduce the risk of rupture, but we will get into that a little later.

What are the symptoms? They range across so many different things that it usually gets misdiagnosed many times before the correct diagnosis is made. Some of them include: soft skin, hypermobility (your elbows and knees go farther than normal people’s do and it can also be in your hands or really anywhere else), translucent skin (you can see your veins, especially on your chest and abdomen), easy bruising, bad eye sight, early onset varicose veins, club foot (when babies are born), old-looking hands and feet, slow wound healing, and some other things that are not as common across the board. Now, please don’t go diagnosing yourself. Most of these things can also run in families and that is exactly what I thought all of my craziness was before this diagnosis. Well, technically it is still true now because my mother and brother also have VEDS, so it runs in the family! But anyway, looking at those symptoms, you can see why it is so hard to diagnose. I mean, come on. How could you look at someone and say, “You have really soft skin and your elbows bend weirdly. Yep. You have Vascular Ehlers Danlos Syndrome.” Yeah… That’s why so many people are misdiagnosed at first.

What are the limitations? Each person’s doctor will probably give each person a different set of limitations. This is what our doctor told us. Don’t lift more than 20 pounds. Don’t run or jog and really don’t sprint (this increases your blood pressure and heart beat which forces more blood through your veins and adds more stress to them, which may lead to a rupture). Don’t go backpacking, which, if you know me, is a really big deal. Don’t go to Six Flags to ride the rollercoasters (increased blood pressure again). Pregnancy is really risky. And don’t do any repetitive motion,. Did that last one throw you for a loop? It did me. So I’m gonna use another metaphor to help explain why we aren’t supposed to do repetitive motions:

Metal Metaphor:  Think of the pop-tab on your coke can or just think of a piece of metal. Now, what happens when you bend that piece of metal back and forth over and over again? It gets weaker and eventually breaks, right? Right. So that piece of metal is just like our arteries. If we bend them back and forth the same way over and over again, they get weaker and eventually will rupture (or this other cool thing called a “dissection”, which is when the many layers of your artery/vein wall decide to get a hole in the inner layer, which allows blood to flow between the layers (which isn’t good at all) which could eventually cause the inner layer to fold in on itself so completely that it fully blocks the blood flow. How neat is that? **please notice sarcastic attitude**). So that is why repetitive motion (including, but not limited to: pushups, squats, weight lifting, or really any other exercise) is no good. But on the plus side, I have an excuse to never run or lift weights again!

 

Here are the links previously mentioned:

Ehlers Danlos Network

Ehlers Danlos National Foundation

Science Direct

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