A very common but complicated question....

So a question I get asked a lot is "what is actually wrong with you?" and this is a hard one, not just because its a complicated answer but its sometimes hard to differentiate between the people asking that really care and those that are only asking to be nosey and want to know so they can have something to gossip about. Now I know a lot off people are genuinely asking because they care but sometimes it can come across that they're really only being nosey.

Now I suppose if your reading this then I guess you are interested and to be honest its something that I do want to talk about as it will hopefully help give some understanding to people in the future but also if everyone just keeps quiet about their rare condtions then of cause no awareness is every going to be made and the lack of understanding is just going to stay at that, no understanding!

So its complicated the answer to this question so bare with me.

So I have a rare connective tissue disorder called Ehlers Danlos Syndromes or in short EDS. some people ask how EDS comes about and well its inherited through genetics. Now connective tissue is a material made up of fibers forming a framework and support structure for body tissues and organs, sorry its complicated I'm trying to keep things as simple as I can. Now EDS becomes present because of the lack of collagen in the connective tissue, now collagen is just like the glue of the body so imagine all the connective tissue not having enough glue, you can see where the issues come when all of your body is been affected.

I'm going to start with the simple things to explain, because unfortunately its not just as easy as having EDS.

One of the common issues with EDS sufferers is joint pain and joint dislocations. EDS sufferers joints dislocate very easily as there isn't sufficient collagen (glue) helping to hold the joints together. As some people will know I've had various joints dislocate but my main issue was my jaw. My jaw would sometimes maybe dislocate 3 times a week and unfortunately these would be very serious meaning I had to be put under anaesthetic each time. I also had to have it wired multiple times so I couldn't move my jaw joint at all meaning it couldn't dislocate. Joint pain and dislocations are just one of the symptoms EDS sufferers may deal with. There are also lots of others symptoms to EDS such as; highly elastic velvety skin, fragile skin, skin that bruises easily, muscle pain, muscle fatigue, heart valve issues, circulation issue (known as Raynaud's) plus lots of other issues and also conditions that are common with patients suffering from EDS. EDS is a life long condition with no cure at present.

So another condition that I suffer with is Gastroparesis. Gastroparesis literally means paralysed stomach and as a condition has literally changed my life. My stomach doesn't work now which is the reason I cant eat and rely on tubes and medications daily. Its not because I don't wanna eat its because its not physically possible. Unfortunately as a result of my stomach not working my intestines don't work brilliant but still have some function which I am very thankful for as I rely on a tube into my bowel for my nutrientian. Now I plan on doing a separate post on how gastroparesis has affected my life this past year and still does, as there's a lot more too it than I can fit in this post.

I also have a condition known as SMA syndrome (Superior mesenteric artery syndrome) it is a complex digestive condition that occurs when the duodenum (the first part of the small intestine) is compressed between two arteries (the aorta and the superior mesenteric artery). This compression causes partial or complete blockage of the duodenum.

Another issue of mine is my adrenal failure/insufficiency. The adrenal glands are part of the endocrine system, the adrenal glands sit at the top of each kidney and produce hormones which help the body burn protein and fat, control blood sugar, and reacting to stress like a major illness or injury. So my adrenal glands not working means I have to take steroid replacements and I also have to carry an emergency injection with me and has to be taken very seriously, to the point I'm on the ambulance register so if someone was to make a call about me they will be there within 8 minutes. This is because when you are unwell your body reacts by making more of a variety of hormones that aid in helping your body deal with infections and trauma etc, which my body cant do as my adrenal glands dont work. So if I was involved in trauma or have an infection for example I have to give my body more of the specific steroids to help my body over come this which sometimes needs dealing with urgently (told you this was a complicated answer).

Another condition I have is POTS (postural orthostatic tachycardia syndrome) which affects the central nervous system and mainly the heart. The main issue is movement for example from sitting to standing in which a dramatic increase in heart rate and drop in blood pressure comes about leading to light headedness dizziness and quite often fainting. There also is a lot of other issue that come about from pots which I also am going to explain in another post as it is very complicate. At present I take medications which control my heart enough so that I can function.

I also have another very rare condition which is a complex auto immune condition know as Severe Mast Cell Activation Syndrome which in the easiest way possible means my body is allergic to itself, and a very long list off other things too. This condition comes along in a few EDS patients and currently only has a couple of doctors which manage people with it here in the UK. I say manage as there's no doctors that can treat it in the UK these doctors are mainly based in America.

I told you the answer to the question wasn't an easy one! Sorry this post is sooo long but I have only mentioned the easy bits! The other things that are going on are too complicated and I'm sure that's more than enough for one post and also as much as I want to raise awareness I don't feel ready to tell all about my life.

Evie x