If you’re sitting there thinking, “What in the world is Ehlers Danlos Syndrome Type IV or Vascular Ehlers Danlos Syndrome?” then you are in good company. It is a very rare genetic disorder (about 1/200,000 or so) that also happens to be 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 Syndrome” and the doctors would respond with 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 most of 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 more information.
What is VEDS?
Basically, it is a chronic and progressive autosomal 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). VEDS 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, stomach, intestines, etc. In other words, 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 have a rupture/hemorrhage or dissection. That’s why the average lifespan of someone with VEDS, as of the research done a few years ago, is around 50 (don’t worry, our geneticist says that he thinks that number is too low, not to mention my family has a history of nice, long lives). 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, which causes internal bleeding accompanied by a sharp, knife-like pain at the site of the rupture/hemorrhage. And if you aren’t sure what a dissection is, it is when the walls of the veins or arteries separate from the inside out. As the inner layer comes loose, the blood flow gets between the layers and separates the layers further, which can also cause a blockage of the vein/artery. Which in turn can cause a rupture due to the increase in pressure and decrease in structural integrity at that particular area. Fun times, right?
Wanna know something cool? That’s never happened to me, which is a big deal considering that 20% of people have experienced a rupture by the age of 20, and that number increases to 80% by the age of 40. Most of the ruptures to major arteries tend to be fatal, however, those to the intestines or less vital organs can be contained in most cases. God has thoroughly blessed me with 21 full years with no ruptures, and that was before I knew anything about it. Now that I am aware, 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?
The symptoms of VEDS 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 (meaning that your joints go past the “straight” or “locked” position), joint pain, translucent skin (you can see your veins through your skin), easy/excessive bruising, subluxations (when your joints go out of socket and right back in so that it isn’t actually considered a dislocation), chronic pain, bad eye sight, early onset varicose veins, 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’s still true because my mother, brother, grandfather and a few others 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, your elbows bend weirdly, and you have a lot of bruises. 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 them a different set of limitations. This is just what our doctor told us: Don’t lift more than 20 pounds. Don’t run or jog and definitely don’t sprint. Why? Because this increases your blood pressure and heart rate, 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 because it is something I do each year. Don’t go to Six Flags to ride the roller coasters (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 any other 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, especially at each joint. No matter how strong the piece of metal is, 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, throwing a ball, or really any other exercise) is no good. Each person is different, so whereas one person with VEDS may be able to go their whole life lifting weights and doing other repetitive motions all the time and have no negative repercussions from it, another person with VEDS might experience a rupture after a short time of that same kind of activity. But on the plus side, I have an excuse to never run or lift weights again!
So the doctor told us something else too. He said that surgery is something they will avoid at every cost. Meaning that unless I am dying or at risk of dying, they will not do any kind of surgery. Why? Because he said that trying to sew us up after a surgery is like trying to sew a wet paper towel… Let’s imagine that for a moment. Actually, go try it. Just go soak a paper towel, then tear it apart, and try to poke holes in either side to sow it back up… I bet it doesn’t work too well… That’s what our insides are like. Well, at least that’s what most VEDS patient’s insides are like. I don’t know what mine are like. I’ve never actually had a real surgery. Hopefully I’ll never have to find out!
Yes, it sucks. Yes, chronic and progressive daily pain is terrible and can be a lot to deal with when your entire body decides to rebel against you just because you slept the wrong way. But in the end, it’s just life. This is how I have been my whole life, so I have never really known anything different. Granted, the aches and pains that I have now are nothing compared to what I had in my childhood because some of the more negative symptoms of VEDS sometimes don’t really set in until your 20s. So, here’s to a life of crazy adventures outlined in pain and colored with joy.
Gentry Bass
Here are the links previously mentioned:
A Path Less Traveled 