Ehlers-Danos-Syndrome (EDS)
EDS Primer
Hi, my name is Luise and I was told I’m hypermobile as a side note in an unrelated physiotherapy session almost ten years ago. I didn’t think much about it then, but started to look into it when I tackled investigating the source of my growing chronic fatigue and pain several years later. One of the first things I did was join Leith Pilates on another hypermobile friend’s recommendation, and it has taught me a ton! It also helped me make the decision to get a more in depth diagnosis, while also looking at personal management, and Andy asked me to write a blog post about my experiences.
A note at the start: EDS, hEDS and HSD
EDS stands for Ehlers-Danlos-Syndrome, a group of disorders that affect connective tissue and organs in different ways, some affecting your heart, your spine, or your skin especially. The most common one is hypermobile Ehlers-Danlos-Syndrome (hEDS), which is very variable in severity. The other kinds also often come with similar joint and skin issues, so I’ll be talking about them all together here.
EDS is different from Hypermobility Spectrum Disorder (HSD), a recently introduced classification, because of its specific genetic markers and symptoms in addition to hypermobility itself. But the line can be pretty blurry and they can be equally severe. Since HSD is a new term, not all doctors might be familiar with it though! I’ll use EDS here as that’s what I have experience with, and it’s where the majority of research is conducted (a shame imo), but a lot of the points will be just as applicable for HSD.
Side note: EDS is classed as a rare disorder, but in my personal experience that may be more because it’s underdiagnosed. As we’ll see below, it can come with so many diverse symptoms that it’s often not recognised (I complained about many of these to my GP way before I connected them to EDS), especially in people who aren’t strongly hypermobile, or only in a few joints.
Why pursue diagnosis?
An official diagnosis recorded in your medical file is definitely not necessary for everyone. They can take a lot of time, energy, and sometimes money, and rely on your healthcare providers being supportive (which isn’t always the case, especially if you’re already marginalised in some way). I eventually chose to do it in order to get access to more support. Being able to say to an employer that I have a specific condition that requires accommodations is both easier and more likely to yield results than flagging up general fatigue and pain. It also let me access a few more NHS services.
What surprised me was the positive emotional impact it had for me. It can be hard to take your own chronic illness seriously when it’s “not that bad”, and having someone external, an expert at that, acknowledge how it impacts your life, can be really validating. It also helped me get to terms with being long term disabled, which has led to me being both more honest with and more kind to myself.
Beyond bendy joints: Co-morbidities and connections
Since EDS and HSD are primarily connective tissue disorders, and connective tissue is everywhere in our body, they can affect a staggering amount of systems, and some really curious and fascinating connections have been drawn. These are vaguely sorted in order of commonality, and whether they’re used for diagnosis.
A lot of these can have other causes and are worth investigating on their own! But when they appear together, especially with hypermobility, it’s pretty suspicious.
Gut issues: Gut problems are very common in people with EDS, from IBS and constipation to acid reflux and heartburn. See also MCAS further down
Tissue fragility: One of the telltale signs of EDS is stretchy skin, but other effects on the skin are easy bruising/breaking, skin that takes a long time to heal, or abnormal scarring. Some EDS types also have extra soft, velvety skin.
Joint Pain: In particular back pain is very common, as well as postural problems like swaybacks, hunched shoulders, and pigeon toes
Anxiety, depression and other mental health issues:
Fatigue: Both physical, a kind of bone tiredness that rest doesn’t help with, and mental fatigue, also called brain fog, eg inability to focus as well as bad memory
Headaches or migraines: Very common. In some rare cases, the upper vertebrae can be abnormally formed (Chiari 1 malformation) or there can be craniocervical instability. They can also be caused by…
POTS/dysautonomia: The autonomic nervous system is in charge of involuntary functions like breathing and your heart beating, and dysautonomia means your body is failing at that. POTS is a particularly common subcategory, describing inability to deal with postural changes. If you get dizziness or a racing heart whenever you get up from sitting or lying down, it’s worth looking into.
Heavy or painful periods: very common
Bladder issues: Also quite common; e.g. small bladder or incontinence
Dental issues: A lot of hypermobiles have problems with their teeth or gums
Anaesthesia resistance: There’s some research that some frequently used anaesthetics wear off faster, or don’t touch hypermobile people at all
Osteoarthritis: Can be a result of hypermobile joints earlier in life
Mast Cell Activation Syndrome (MCAS): Mast cells play a key role in regulating inflammation in the body. In MCAS, they activate more than necessary and can cause system-wide inflammation, often in response to food (or an environment) rich in histamines. The results can affect your skin like an allergic reaction, your gut, your brain and more. It’s too big a topic for this blog, but I’ve put some further reading about it at the end.
RSI: Because we’re not using our muscles properly, it’s really easy to overstrain
Progesterone: Progesterone softens connective tissue, and is most present in the body around the week of one’s period. Progesterone-based birth control can exacerbate the extra pain that may result.
https://ojrd.biomedcentral.com/articles/10.1186/s13023-016-0511-2
Neurodiversity: No, really! Nobody knows why, but there seems to be a strong overlap.
https://www.ehlers-danlos.org/information/webinar-hypermobility-and-neurodiversity/
Edinburgh services
In order of how long it took me to get referrals through my GP. That was years ago, some of them during the pandemic, so might well be different now!
GP: Is likely to do the Beighton Scale test and ask about the most common side effects before agreeing to refer you
https://www.ehlers-danlos.com/assessing-joint-hypermobility/
Anesthesiologist: Can prescribe painkillers, other help like TENS machine
Physiotherapy: You can refer yourself to the basic service, but in my experience it can be very variable with how much individual therapists know and are willing to accommodate. There’s one EDS specialist that can be accessed through rheumatology
Rheumatologist: Will check for relevant possible heart problems, can prescribe painkillers, order more tests (e.g. X-rays of painful joints), refer for comorbidities like POTS
Headache/Migraine Specialist: Can prescribe specific medication, order tests
Chronic pain service: Group and 1-to-1 options, focus on self management practices like mindfulness, pacing, and sleep hygiene
Things that have helped me
Pilates with someone informed about EDS, like at Leith Pilates
Physiotherapy: Specificity is key. Knowing what muscles exercises tackle so you can make sure you actually use those. Using resistance has also been great for me
Avoiding graded exercise and pushing through pain or fatigue
Joint support: I wear a wrist brace at night, and a knee one for strenuous tasks
Being more aware of my pain and discomfort levels: It gets easy to downgrade pain when you have it all the time, but especially when a doctor asks, it’s worth thinking about how it really impacts you.
Other sources:
Huge amount of EDS resources by healthcare professionals and patients:
https://www.ehlers-danlos.org/
Toolkit designed for diagnosing EDS for GPs, but also useful for talking to GPs about it:
https://hedstogether.com/projects/eds-rcgp-toolkit/
Blog mostly on MCAS and POTS, science and management: https://liminalnest.wordpress.com/2023/01/07/start-here-pinned-post-archives/