I’ve rambled on a bit in this post, so you might need a brew and half an hour to spare 😉
As I’ve mentioned before on my blog it’s disappointing (I’m being polite) that there is virtually no information on the effect of aging on the hEDS population, despite the fact we’re all going to get old. Quite why the over 50s are ignored when it comes to the management and progression of the disease is baffling because if we knew why some people’s illness progressed well while other’s progressed badly we’d know how effective management in younger adults actually was, but it still seems to be something none-one wants to explore.
According to the 2017 guidelines (known as nosology – why can’t plain english be used instead of medical jargon, the EDS charities need to improve on that) there are 3 phases to hEDS:
- the hypermobility stage
- the pain stage
- the stiffness stage
though as far as I could determine this information is based on a pilot study of only 21 people, so why it made it into the new guidelines and is now relied upon by clinicians as fact is a bit of a mystery.
Although I’m in agreement that, for most people, there are stages to having hEDS the 3 stages listed above don’t really tally with my illness experience. In particular there needs to be a ‘menopause’ stage because there is overwhelming anecdotal evidence that many women don’t even know they have hEDS until they hit their 40s and their hormones begin to wane (I appreciate that men get hEDS too and their illness experience will be totally different).
For me, I had few symptoms of hEDS until I reached my late thirties. Note I said “few” and not “none”. I’ve had gut symptoms my entire life and I started having back pain at the age of 11 which resulted in surgery when I was 16, though it sadly didn’t cure the pain which has been ongoing ever since. But on the whole, I was in the hypermobile stage until I was around 36.
For me the pain stage didn’t follow the hypermobile stage. Nope, I skipped that and went straight to the stiffness stage. Around my late thirties I noticed that my ankles were stiff in the mornings, to the point where I could barely walk to the loo. Thankfully after an hour or so they loosened up but this was the first indicator that there was something wrong (I had no clue I had EDS at the time). My back also stiffened up and I went from someone who could do backflips despite the ever present pain to someone who couldn’t bend to put her socks on virtually overnight.
At around the same time I was emerging from my 10 bedridden years with M.E. and starting to be more active. It was then I noticed chronic, widespread pain. It wasn’t joint pain, more an overall muscle pain which felt like my ligaments were tearing at even the smallest of daily tasks. My fingers, wrists and elbows in particular burned constantly.
By the time I was 41 my feet and knees had joined in the pain party. My neck was so stiff I couldn’t turn it to reverse the car and the range of movement in my hips had reduced down to 30% on the right side and 10% on the left. When I suddenly starting sub-luxing my shoulder and jaw in my sleep I knew something other than M.E. was going on and fought for my EDS diagnosis.
Despite all the stiffness and pain, however, I remained hypermobile in my peripheral joints. I can still touch my nose with my tongue, do the reverse Nemaska, bend my fingers to 90 degrees and do all the other party tricks I always have. My hips, neck and back are a different kettle of fish, though, and the range of movement in my spine diminished rapidly the second I hit 40.
For me, the stiffness and pain stages happened simultaneously and I’m as certain as I can be that they arrived because I was entering peri-menopause. There is an urgent need to study women with hEDS in their 40s because there are hormone drugs already on the market which might help stave off the deterioration which seems to happen when women reach peri-menopause. The physio given to younger women simply doesn’t work when you’re middle aged and I find it infuriating that all the management advice is aimed at children and younger adults. My muscles and ligaments have changed beyond all recognition as I’ve aged and there is a dire need to look at why and how we can best manage EDS in the middle aged and elderly.
I am now 50 and in the late stages of peri-menopause. When I’m resting my pain levels are fine, apart from my back which hurts no matter what I do, but the second I start to do anything the burning and stinging in my muscles starts, consequently I can’t continue activity anywhere near as long as I could a decade ago as the pain simply becomes too much. My stiffness has also ramped up a notch or ten. Far from being confined to certain joints it’s now widespread and there are days when I can barely move first thing in the morning. My body doesn’t loosen up anywhere near as quickly either and the stiffness lingers throughout the day. I have to sit to put on socks and shoes, can barely move my head left or right, can’t chop vegetables, struggle to put on jumpers and walking the dog is like climbing the north face of the Eiger in terms of strain and effort. I “uuuumpf” when I get up out of the chair and can crouch down to pick something up off the floor but can’t get back up again. I literally have the body of an 80 year old. Scratch that – my Dad is more supple than me and he is 80!
The only other information I could find about the phases of hEDS came from the UK’s Royal College of Physicians and states ” The final phase is the emergence of psychosocial sequelae such as anxiety or depression, obesity (often associated with comfort eating), work incapacity, isolation and despair. At this stage, there is often a downward spiral of loss of mobility, self-efficacy and self-esteem as the quality of life diminishes” though there is no citation or link to the research on which this statement is based. Where is the hard data which shows that as we Zebras get older we all become depressed, obese and live in despair, because if that information isn’t available the Royal College should be ashamed of themselves for putting this crap out there. For a start menopausal women tend to gain weight even among the healthy population (as do men over 50), so unless they can demonstrate via well-controlled clinical studies that weight gain is solely down to the progression of hEDS it is irrelevant, and for a second thing women tend to gain self-esteem as they age not lose it, it’s one of the few perks of getting old! My body may have fallen apart during my 40s but I’m happier than I have ever been and have more zest for life, not less. I’m so appalled at the statements made in this piece of mis-information I can barely bring myself to include it, but if this is what our health professionals are reading and basing their views on we need to know about it. It’s a shame really because the article is otherwise sound, if now a little dated.
Going slightly off topic, the UK hypermobility charities are currently fundraising to research the effect of diet on the symptoms of hEDS and hypermobility spectrum disorders and I admit I am not happy. We don’t even know the basics of these conditions, so to look at food intake as a means of treatment feels like we’re putting the cart before the horse. There is no epidemiological information on connective tissue disorders (CTDs), ie how common they truly are in the general population, the ratio of the sexes, at what age diagnosis is obtained or anything about outcome. We have no clue about progression, or how far the family link extends. We don’t even test adequately – all people with suspected connective tissue disease should be gene tested, not least to rule out other types of CTD or Marfans’s Syndrome, and this data put in a bank for study – how else are we ever going to find the gene responsible for hEDS? It’s this basic kind of information which forms the building blocks for research into any disease and data researchers often need when studying illness. No food is ever going to cure my spinal stenosis or my dental overcrowding, my hernia or my genetic predisposition to Osteoporosis. It also doesn’t help us understand why many people with hEDS also have mast cell disease, POTS or M.E., or why puberty and the menopause affects sufferers so profoundly, information much more vital and fundamental to our knowledge of EDS in my very humble, but hugely invested, opinion. For the estimated 10% of us Zebras with MCAS, and who are usually on a restricted diet, altering the foods we eat may be impossible in any event. Yes we need to look at treatment but only once we have a proper grasp of the disease, its incidence and progression. Which isn’t as hard to do as you’d think.
I remember back in the late 1990s when very little was known about M.E. and severely affected patients were completely ignored, Action for M.E. (Britain’s 2nd largest M.E. charity) sent out a survey with its regular magazine to all of its members and received approx 2,300 replies. The results led to a report called ‘Severely Neglected: M.E. in the UK’ which was the largest ever survey done on the disease. It turned much of the information ‘out there’ on its head, made a massive impact on our understanding of the prevalence and impact of severe M.E. and became hugely influential when NHS Clinics to treat patients were eventually established. Over the last two decades, the ME Association have also regularly sent out questionnaires with their quarterly magazine on a wide range of topics, and run a monthly quick survey on their website for anyone with the illness to fill in not just members, all of which have helped our understanding of M.E. Surveys aren’t difficult to do, although collating the data is time consuming, and I really don’t see why the EDS charities don’t do something similar – at least then we’d know some basic information, such as the percentage of men v women, how many people are receiving no treatment, what treatments people had found effective, progression and if ethnicity is a factor (which might help in the quest for the hEDS gene).
It appears to me, and obviously this is only a personal opinion and observation, that the EDS world shies away from asking the hard questions and I’m not quite sure why that is. It’s great that children are being diagnosed with hEDS sooner, but appalling that older people are still being completely ignored despite the fact we are often at the more severe end of the spectrum as our bodies age and naturally deteriorate.
I’m fed up of being ignored. I was ignored as a severely affected M.E. patients for years, as the emphasis was put on those at the mild end of the spectrum because they were well enough to campaign and be more vocal. I’m totally ignored as an MCAS patient, receiving no monitoring or treatment whatsoever. As an older person with hEDS I’m also ignored, for reasons I can’t quite work out. And the fact I live in the north of England (by which I don’t mean Manchester, there is life beyond!) with any of these diseases means I might as well not exist. I can’t name one single EDS event which has taken place north of Birmingham and anyone living in northern Ireland or Scotland must feel completely invisible, a situation which is simply unacceptable.