hypermobile Ehlers-Danlos Syndrome (hEDS) is 1 of 13 types of the Ehlers-Danlos Syndromes. hEDS is the most common type of EDS and is the only one that scientists still haven’t found a genetic marker for yet. EDS is a heritable connective tissue disorder; different types of EDS have different characteristics but share some similarities collectively. hEDS causes generalised joint hypermobility, joint instability (leading to joints regularly subluxing and often dislocating) and chronic pain; it can also affect different parts of the body. It is common for people with hEDS to develop related conditions such as conditions that affect varies organs, as well as conditions such Postural Orthostatic Tachycardia Syndrome and Mast Cell Activation Syndrome and other conditions.
hEDS is much more than ‘being bendy’ and not everyone who is hypermobile will have hEDS or Hypermobility Spectrum Disorder - for most people being hypermobile isn’t an issue or it can be advantageous, just look at dancers, gymnasts and athletes. However for the small number of those who have hEDS being hypermobile can be quite problematic and painful too.
'Joint hypermobility' refers to the joints ability to move beyond normal limits. This is tested using the Beighton Score in which each joint that is classified as being hypermobile a point is given. Their are 9 "points" in total. An alternative to this is the 5 point questionnaire - these testing tools are used when diagnosing a person with hEDS/HSD.
hEDS and Me
I wasn’t diagnosed with hEDS until I was in my mid 20’s but looking back I can see clear signs of me having it as a child. My hypermobility was advantageous to me as a ballet dancer but I often complained of pain throughout my childhood. My pain was always passed of by Drs as simple ‘growing pains’ but now I can see that it was joint pain from my EDS. I also has gastrointestinal problems again that was probably a sure sign and part of my childhood EDS. I do have a spinal curvature, a hyper lordosis, an acute inner curvature of my lower spine that I feel is also part of my EDS too; I had a lot of issues with my hyper lordosis as a child.
When I was first assessed for EDS I had a lot of features of vascular EDS so I had genetic testing done to see whether I had vEDS. I didn’t have the genetic marker for vEDS so I was then diagnosed with hEDS. However I still do have a mixture of the features of both vEDS especially issues with my veins and some mild heart problems and then obviously the features of hEDS as well.
Living with EDS is difficult. I have to be so careful so as not to sublux or even dislocate a joint. Even something as simple as yawning can mean that my jaw will dislocate and then I will have to painfully relocate it. Any joint can sublux or dislocate; some more than others, even ribs can dislocate! It’s also a fine balance on whether to brace or not to brace. I used to wear some braces all the time but now I just wear them when that joints needs the support or post-dislocation. EDS leaves me in constant pain and pain leaves me in constant fatigue but sometimes I struggle to sleep because of the pain making my pain and fatigue levels worse.
As well as EDS affecting my joints it also affects some of my organs. Connective tissue is the ‘glue’ of the body and makes up a lot of things and holds a lot of things together. It makes organs more stretchy so they don’t function as well. My bladder has gone into failure so I now have an SPC which is a type of catheter. My gastrointestinal system is also affected with my gut functioning more slowly. I also have a few issues with my heart. I also have poor vein access so on my medical notes it’s says I’m a DIVA - difficult IV access!
Also I bruise so so easily; I’m always covered in them. My skin is fragile too so I’m more prone to things like pressure sores so I have a hybrid airflow mattress to help prevent them and when I sit on a chair I sit on a pressure relief cushion. Because of the connective tissue in my skin it means wounds heal poorly and I easily get stretch marks etc.
I’m an ambulatory wheelchair user (a part time wheelchair user) for a variety of reasons and for a number of my medical conditions but with my EDS using my wheelchair helps me a lot. It reduces the risk of me subluxing or dislocating; it also means I’m not weight bearing on legs so it eases the pain in my legs joints, especially my hips. Using a wheelchair also alleviates to pain I bear leaning on my crutches that I use to help me walk.
I’m thankful that my EDS doesn’t have as many complications as some of the people with EDS I know have. Yes my life with EDS is challenging and complex but it could be a lot worse and I’m ever so grateful for that.
Helpful links about EDS and HSD
- Hypermobility Syndromes Association
- Hypermobility Connect
- Ehlers-Danlos Support UK
- Ehlers-Danlos Society
- PoTS UK