I’ve been promising to do an update on my Ehlers-Danlos Syndrome for ages now, but to be honest I’m finding it difficult to know where to start! I was diagnosed with hEDS at the age of 42 by a rheumatologist who specializes in hypermobility disorders after having my pain and other issues ignored and fobbed off for decades. Eventually, Dr Google and I had a strong suspicion that faulty collagen was behind my lifelong symptoms and this was confirmed in 2010, much to my relief. You can read more about my hEDS journey in this post.
As a child I had the following red flags:
- Widespread joint hypermobility.
- Easy bruising.
- Thin skin which split easily and left wide scars.
- Fatigue on walking or standing for long periods.
- “Knock knees” (which are caused by collapsing high foot arches on standing).
- Inability to run long distances, which affected my lungs.
- Hand pain and fatigue when writing.
As a teenager, these were joined by:
- Spinal surgery for rare congenital spinal stenosis (ie narrowing of the spinal canal).
- Lordosis (ie an exaggerated curve in my mid back).
- Mild scoliosis (ie my whole spine curves to one side).
- Dental overcrowding (I needed 4 molars extracted to make room for the rest of my teeth).
- Daily headaches.
- Chronic nausea.
- Poor proprioception.
In my late thirties, the pain in my back became severe and my mast cell disease, which had lurked in the background all my life, began to escalate.
Then in my forties all hell seemed to break loose:
- Mast cell disease and a newly developed Histamine Intolerance threatened to kill me.
- Subluxing joints (ie joints which partially dislocated but eventually pop back in on their own or with self-manipulation) which particularly affected my ribs, fingers and jaw.
- Loss of hypermobility, particularly in my back. I also had severe ankle stiffness in the mornings, which made walking difficult. I am now very stiff all over.
- Headaches went, to be replaced by horrendously painful migraines both with and without aura.
- Severe, widespread, unrelenting pain.
- Severe eye floaters and very dry, sore eyes.
- Thick patches of skin over bony joints such as my knees and elbows, which can be discoloured.
- Severe, drug resistant GERD (ie acid reflux).
- Muscle weakness.
- The only way I could sleep was on my side propped up front and back with pillows, otherwise pain kept me awake.
It was the fact that my pain levels increased substantially and I started subluxing my ribs in my early forties as I entered peri-menopause which led to me seeking a diagnosis. It is still clinically “anecdotal” that hormones play a significant factor in hEDS for women, but you only have to go on any hEDS forum to know that there is nothing “anecdotal” about the fact that both puberty and menopause play havoc with collagen and many women don’t even know they have hEDS until they hit their forties and the pain and dislocations begin.
I have to be honest and say the entire decade of my forties was a hugely difficult time health-wise. My mast cell disease made itself known in no uncertain terms and my hEDS hugely impacted my daily life. Add to that all the other symptoms of peri-menopause and it was not fun. Not fun at all.
In my late forties, I volunteered to take part in the Hedge research study but apparently didn’t fit the new 2017 EDS diagnostic criteria. Instead I was given the diagnosis of historic Hypermobility Spectrum Disorder. Ignored totally was my rare congenitial spinal stenosis, heart murmur, blue sclera, pizogenic foot papules (I didn’t have enough apparently), atrophic back scar (you need 2 scars for diagnosis – WTF?!), mast cell disease, my brother’s Marfans stature and the fact that his professional football career was cut short due to collagen issues with his knees, and my Mum’s history of spontaneous pneumothorax aged 42 (ie collapsed lung for no reason), pectus carinatum (ie pigeon chest which is strongly associated with genetic connective tissue disorders), mast cell disease and obvious hypermobility. If it looks like a duck and quacks like a duck chances are it’s a god-damn duck and I strongly think that the new criteria for hEDS is flawed and excludes many EDS patients.
I am now 53 and approaching menopause, and my symptoms are changing once more:
- Stiffness is now very limiting. I struggle to put on my shoes, cut my toe nails, raise my arms above my head to wash my hair or look over my shoulder to reverse the car.
- I stopped being able to lie on my front when I was about 43, but I now can’t lie on my back either as it’s just too painful.
- If I sit in certain positions, particularly if my head is leaning backwards, I start to feel…….weird. Hard to explain, but like I’m going to pass out or have a seizure. I’m assuming the weak collagen in my neck is causing some kind of spinal collapse and/or compression.
- For a year now I’ve had intractable pelvic pain and have to sleep wearing an SI belt every night. Despite that, many nights I’m woken by pain. It’s come to something when you can’t sleep on your front, back or either side and at times I’ve had to resort to sleeping sitting up.
- Fatigue is ridiculous.
However, there have also been some positives to my hormones finally vamoosing, including:
- Subluxations seem to have settled down. However this may be due to the fact that I simply no longer do anything, because I can’t cope with the resulting pain from activity.
- My migraines seems to be much less frequent, although when they do appear they are fairly brutal and last for several days (I’m currently on day 4 of a stonker!).
- Treating my mast cell disease and Histamine Intolerance through a low histamine diet has seen an end to my lifelong constipation 😮. Ditto my chronic nausea and it’s now very rare I ever feel sick, after suffering almost daily for forty years.
I think it’s a combination of declining hormones, having the aids, supports and adaptations I need for daily living, and tailoring my activity to my symptoms which have helped my hEDS to stabilize albeit I’m at a fairly low level of functioning. It’s now rare I need to wear my Oval 8 finger splits or my back brace, but that is clearly helped by the fact that I simply don’t do much physical activity any more because if I do I can’t stand the resulting pain. I also use my braces judiciously, for example when I’m editing my photos I always wear my wrist brace as I know if I don’t my right wrist will kill me from using my pen tablet, and I also use a neck brace because sitting still for long periods gazing at my monitor sends my neck into really painful spasms. Getting the right orthotic insoles for my shoes has helped my knee pain, although it did take 2 years and several types before I found ones which suited me (I even had some custom made and they were the worst by far!). Using a push button kettle helped my wrist pain and of course using my mobility scooter has helped with both fatigue and pain and enables me to get out and about without too many negative consequences. Ditto my automatic car.
I was tortured for a whole year in 2020 by dreadful back and neck pain, which I thought was menopause related but which turned out to be more down to my twenty year old mattress and soft pillow. Changing the mattress and pillow on my bed to more supportive memory foam helped enormously, so it’s always worth looking at potential other reasons for new or increased symptoms!
So, that’s where I’m at. hEDS now has a big impact on my every day life and I definitely feel old before my time. It’s bizarre to go from being hypermobile your whole life to suddenly being painfully stiff and inflexible and it’s seriously taking some getting used to. However, so far I’ve found ways of working around my limitations although I mourn being able to sleep in my usual position, or sitting crossed legged which has always been super comfortable even at the dinner table! Only time will tell what the future holds.