I've noticed this discussion recently on social media.
The problem is that there is a difference between 'a progressive condition' and 'a condition which can cause progressive levels of disability'. With the former, deterioration is inevitable. It will happen in every case. In the latter, it will happen in some cases.
With hypermobility type Ehlers Danlos Syndromes (which many experts consider as the same as JHS and BJHS), the genetic fault that has caused the faulty collagen remains constant. It doesn't change. It doesn't progress in a medical sense of the word.
But the symptoms can get worse. The level of disability can progress.
Mine got worse. Lots worse. I deteriorated from a 20 year old with a few quirks and vulnerabilities, to a 28 year old wheelchair user, retired on permanent ill health grounds, with unstable joints throughout her body and who struggled to hold a conversation or eat.
With the benefit of hindsight and everything I have learnt since being diagnosed at 24, I can see that the reasons for my deterioration were 3 fold.
1. I was overdoing it. I was doing more than my body could cope with, and ignoring all the signs that my body wasn't coping. Because I didn't think they were real symptoms, and I didn't want to 'give in'.
2. With injuries and pains I often rested more than I should have done, causing muscle wastage - and more pain and instability. (some rest post injury is important, but I now know that keeping moving and slowly increasing use is important.)
3. I lived in positions where my ligaments were being stretched (slouching at the computer, twisting my legs into 'comfortable' positions, poor shoulder posture etc.)
So my condition progressed. I deteriorated.
But since then, I have improved - HUGELY.
No, it was not a magic cure.
But I have improved. What happened? A combination of factors.
1. I started a medication for POTS which meant my body could cope with the basics of eating and talking without throwing a compete hissy-fit.
2. I learned about managing my HMS/EDS, much of which was through the Hypermobility Syndromes Association (HMSA).
3. I crashed out. Big time. 5 weeks in hospital time. There I learned (the hard way) that when my POTS symptoms started building, I should take evasive action (cool down/get my core and leg muscles working/lie down/drink rehydration stuff) instead of waiting for symptoms to reach avalanche status and swamp me - In short I learned to pace. To put into practice what I'd learned from the HMSA.
And I built the confidence to communicate about my management choices rather than just do what I thought I was being told by the next medical professional I saw. Learning to communicate about my choices in a way they understood and could respect, so we could work together to improve my management.
When I went home instead of trying to do as much as I could to prove I wasn't 'giving in', I started listening to my body. I learnt that I couldn't cycle for 10 minutes - it made me unable to function for days. But I could cycle for 2 minutes provided I concentrated on keeping my joints in good positions and my muscles nicely activated. 'cos that only tired me out for an hour. (By slowly building up, I now can cycle for half an hour and more!)
And I worked at living with poise. Not like I have a poker up my...spine. But upright, strong, and ready for movement. Like a gymnast about to do a run-up for the vault.
I admit I tend to go in cycles. I start committed to my exercise regime, careful with joint positions, I pace well etc - and improve. As I improve, other things start taking priority....business, housework, socialising. And I start to deteriorate again. Then I suddenly realise, feel a little foolish, and start being good again. - and as I am good, I improve. Until I get complacent and the cycle starts again.
But the important thing is that I don't go so far over my body's limits any more. I recognise the warning signs so that when I start being good again I haven't caused new damage to my ligaments.
And at 32 I would say I am stable. Not because my condition has fundamentally changed, but because I know how to live well with it.
The problem is that there is a difference between 'a progressive condition' and 'a condition which can cause progressive levels of disability'. With the former, deterioration is inevitable. It will happen in every case. In the latter, it will happen in some cases.
With hypermobility type Ehlers Danlos Syndromes (which many experts consider as the same as JHS and BJHS), the genetic fault that has caused the faulty collagen remains constant. It doesn't change. It doesn't progress in a medical sense of the word.
But the symptoms can get worse. The level of disability can progress.
Mine got worse. Lots worse. I deteriorated from a 20 year old with a few quirks and vulnerabilities, to a 28 year old wheelchair user, retired on permanent ill health grounds, with unstable joints throughout her body and who struggled to hold a conversation or eat.
With the benefit of hindsight and everything I have learnt since being diagnosed at 24, I can see that the reasons for my deterioration were 3 fold.
1. I was overdoing it. I was doing more than my body could cope with, and ignoring all the signs that my body wasn't coping. Because I didn't think they were real symptoms, and I didn't want to 'give in'.
2. With injuries and pains I often rested more than I should have done, causing muscle wastage - and more pain and instability. (some rest post injury is important, but I now know that keeping moving and slowly increasing use is important.)
3. I lived in positions where my ligaments were being stretched (slouching at the computer, twisting my legs into 'comfortable' positions, poor shoulder posture etc.)So my condition progressed. I deteriorated.
But since then, I have improved - HUGELY.
No, it was not a magic cure.
But I have improved. What happened? A combination of factors.
1. I started a medication for POTS which meant my body could cope with the basics of eating and talking without throwing a compete hissy-fit.
2. I learned about managing my HMS/EDS, much of which was through the Hypermobility Syndromes Association (HMSA).
3. I crashed out. Big time. 5 weeks in hospital time. There I learned (the hard way) that when my POTS symptoms started building, I should take evasive action (cool down/get my core and leg muscles working/lie down/drink rehydration stuff) instead of waiting for symptoms to reach avalanche status and swamp me - In short I learned to pace. To put into practice what I'd learned from the HMSA.
And I built the confidence to communicate about my management choices rather than just do what I thought I was being told by the next medical professional I saw. Learning to communicate about my choices in a way they understood and could respect, so we could work together to improve my management.
When I went home instead of trying to do as much as I could to prove I wasn't 'giving in', I started listening to my body. I learnt that I couldn't cycle for 10 minutes - it made me unable to function for days. But I could cycle for 2 minutes provided I concentrated on keeping my joints in good positions and my muscles nicely activated. 'cos that only tired me out for an hour. (By slowly building up, I now can cycle for half an hour and more!)
And I worked at living with poise. Not like I have a poker up my...spine. But upright, strong, and ready for movement. Like a gymnast about to do a run-up for the vault.
I admit I tend to go in cycles. I start committed to my exercise regime, careful with joint positions, I pace well etc - and improve. As I improve, other things start taking priority....business, housework, socialising. And I start to deteriorate again. Then I suddenly realise, feel a little foolish, and start being good again. - and as I am good, I improve. Until I get complacent and the cycle starts again.
But the important thing is that I don't go so far over my body's limits any more. I recognise the warning signs so that when I start being good again I haven't caused new damage to my ligaments.
And at 32 I would say I am stable. Not because my condition has fundamentally changed, but because I know how to live well with it.
If we keep doing more than our bodies can cope with, we will get worse. If we consistently do less than our muscles need to in order to stay strong, we will get worse. But if you think about it, this is actually true for everyone. That is what happens to every human being who does too much or too little. It is why athletes retire young - and frequently with a long list of injuries and why people get injured doing 'unaccustomed exercise' all the time.
During those pre- and early post-diagnoses years, many bits of me have worsened - been more stretched. And I expect I'll stay stretched. But I don't expect to pick up more outside of the general deterioration that happens with life in general unless I have a bad accident or significant illness or develop another condition which makes managing my HMS/EDS even harder - and thinking about it, those types of things can happen to healthy people too and cause a deterioration of their physical condition too.
It is more, as a friend 'Flick' said to me "relapsing/remitting (flare/rehabilitate), with some cumulative tissue damage. Un-managed it mimics progressive."
My body will never be one that can cope with 'average'. It never has been. Finding the right balance and maintaining it will always be harder for me than the average bod, but it is doable.
During those pre- and early post-diagnoses years, many bits of me have worsened - been more stretched. And I expect I'll stay stretched. But I don't expect to pick up more outside of the general deterioration that happens with life in general unless I have a bad accident or significant illness or develop another condition which makes managing my HMS/EDS even harder - and thinking about it, those types of things can happen to healthy people too and cause a deterioration of their physical condition too.
It is more, as a friend 'Flick' said to me "relapsing/remitting (flare/rehabilitate), with some cumulative tissue damage. Un-managed it mimics progressive."
My body will never be one that can cope with 'average'. It never has been. Finding the right balance and maintaining it will always be harder for me than the average bod, but it is doable.
And I am extremely grateful to the HMSA, the hypermobility clinic at UCH London, and my cardiologist for helping me learn to manage my condition, so that I can respect and understand it instead of fear it.
To all you who have not yet been able to find the right balance, don't give up. It is hard work, but also very, very rewarding.
(Images are extracts from the book "You know you have HMS or EDS when..." by Hannah Ensor.)
To all you who have not yet been able to find the right balance, don't give up. It is hard work, but also very, very rewarding.
(Images are extracts from the book "You know you have HMS or EDS when..." by Hannah Ensor.)
While I can see where you are coming from, I've found that for me it has been different. I am as active as possible, which is walking a few miles everyday (with a lot of trouble). I do my physio exercises which seem to help in some areas but hurt in other areas. However, it is also thought I may have something autoimmune going on as well because I am swelling up like a balloon, so I suppose it is very different for me.
ReplyDeleteI so needed to read this Hannah - thank you. Pacing and being 'good' is so worthwhile but so hard.
ReplyDelete... No offence intended, Hannah, but it really doesn't feel particularly pleasant to essentially be accused of causing my own deterioration. So far there is no POTS medication that has been capable of helping me (technically I have postural orthostatic hypotension, but close enough) because of all my comorbidities and meds I have to avoid for those reasons, and I have 80+ joint dislocations every day. That I can't be active enough to "get better" is not my fault, and I'm sorry to say this but the way you've phrased this idea sounds and feels a bit too much like victim-blaming. I won't show it to my sister; it would only make her depression worse.
ReplyDeleteNormally I respect your opinion, but you could consider rephrasing some of this one, unless blaming people who can't pace themselves for whatever reason or can't be more active than they are is what you intended. =/
Hi Trialia, I'm sorry that you felt that my blog came across as victim blaming - that wasn't how it was meant at all. I wasn't aware that anything I had said implied that I was to blame for my own disability - let alone that others are to blame for theirs. The fact that HMS/EDS is so complicated, and often has other conditions (such as POTS or gastro issues) complicating it further, plus it frequently being diagnosed only when it has already become disabling (how can you manage a condition you don't know you have, or which you have been incorrectly told is psychosomatic/all in your head?) - and once diagnosed it can take years to get good advice from a medical professional who really knows their stuff, and even then there is a huge amount of trial and error in that the exact things that help and things that hinder are different for everyone. [Fortunately in my experience Drs are much more aware than they were 20 years ago, so things seem to be improving for future generations of HMS/EDSers.] So it’s not surprising that many of us have significant levels of disability.
DeleteNot finding the way to manage our EDS well, and consequently getting worse is the normal position, because it EDS so complex. There is no blame attached to that.
But what I was trying to say is there are things that we can do to help ourselves – not cure, but help. In some cases people may, with, for example, pacing and the right physio, find that their condition improves and their level of disability becomes almost non-existent. For others (like me) the pacing and physio I am able to do, when combined with meds, joint supports, and many other aids and techniques accumulated over years, are able to stop a downward spiral, and improve a little. In others the techniques, meds, aids and everything else may simply slow the downward spiral. The end point does not make any individuals effort more or less worthy – we are just all different. The point of my blog was that HMS/EDS isn’t automatically an ever-worsening level of progressive disability. There are things that can help, and things that can make us worse, so if we keep trying to make good decisions, trying to find a level our body can cope with, we can, to varying degrees, help ourselves.
If I could do physiotherapy, I would be. I can't even lie still without dislocating my hips after a while. My shoulders and hips go out in my sleep. My generalised joint instability is such that no physiotherapist advises land physio on me - they just can't do it without several somethings going out of place. Hydrotherapy helped a little when I could get it, but it's so difficult to get time in the pool - and to cope in said pool without fainting from POTS, even now that I'm on midodrine (I was already doing everything I could in the lifestyle sense to manage my POTS, but it wasn't working alone). My specialist said that if I ever try hydro in a standard pool, or even a kids' pool, that I'm going to need a wetsuit, because the cold fries my nerves. Unfortunately, there you've got the issue of affording it.
DeleteI have been pacing myself very deliberately for years. For a decade I've done all I could possibly do to balance my health without overdoing it. I've tried as many treatments as I could access, and continued doing everything I could. But I am still a full-time powerchair user outside my own home, and my condition will only continue to deteriorate.
A lot of that has to do with the fact that it took 15 years for me to get a doctor who knew enough about EDS to diagnose me and get me treatment at last. That decade and a half did too much damage to my body; my specialist tells me that I won't improve, but that we *can* focus on slowing the deterioration. And so that is what we do. But physical exercise in the traditional sense isn't really an option for me any more. I'm on a litre of Oramorph a week for the pain; nothing weaker works now, as my body habituates quickly, like many EDS patients. I'm having 18 of my 25 remaining teeth removed under general anaesthesia sometime in the next couple of months, because they've come to the point where they break off and crumble so badly, and leave so many exposed nerves, that even the staff of my local dental hospital say they can't keep up.
It probably doesn't help that I have to manage comorbid bipolar disorder, OCD and PTSD as well as being on the autistic spectrum, but I do all I can.
I found this post in its original form very hurtful to begin with, because I am so tired of being accused of not trying hard enough, or of causing my own illness, or simply of being lazy. I'm not sure why you don't seem to be able to understand that it could be read in such a way - you seem reasonable in most other ways. I suppose 2 years later I can sort of see how it might not have been intended that way... and yet.
Hi Trialia, sorry to hear you and your sister are facing such difficult times and so many health challenges. You're doing well to keep going! Good you have a motorised chair to allow you to get out and about-that's so important. I've commented below and included some thoughts on your situation/situation of those similar to yours. It might have something that perhaps will be of use to you. I'm sorry the NHS isn't giving you the help you need, but I hope this year some positive support will come your way to allow you to improve or stabilise even one area of your condition(s).
DeleteHi as I read this my thoughts were if I'd been diagnosed sooner maybe I could of helped myself. Unfortunately for me and many others we only get diagnosed once the damage is done. And we loose the life's we once had however on a positive note some of what you say is sensible we should pace and listen to our bodies. Good luck hope you never get to 47 and find yourself in the mess I was in. X
DeleteGreat read. Made perfect sense to me and was different from other experiences I have read in a way I could relate to. Many thanks for the insights.
ReplyDeleteGreat read. Made perfect sense to me and was different from other experiences I have read in a way I could relate to. Many thanks for the insights.
ReplyDeleteHi!
ReplyDeleteYour story was very helpful! I don't know if I have EDS or not but my doctor says that I most likely have it. I now know that i should be a little bit more careful. Thank you and I hope that your health will improve!
What an awesome post on your personal rehabilitation journey.
ReplyDeleteI have been putting all the things that helped me with early exercise pacing, diet changes, lifestyle things in lots of detail on my blog to try and help people with EDS-HT with their efforts to get stable.
Love you to check it out www.generationhypermobility.com
Thank you for this great post highlighting the difference between progressive and deteriorating! Good to read about someone else progressing with the aid of multiple therapies and strategies. It requires such trial and error-it can feel like nothing works without the right guidance and a lot of persistence/adaption. The first physio I tried post diagnosis didn't help and I was made to feel I wasn't doing it enough, even when the physio could see me shaking with effort! Luckily, I got access to the ROH rehab programme and was shown similar exercised but adapted to a level I could manage. E.g. the bridge was just me tilting my pelvis a tiny bit and holding without my knees knocking together/shaking. Now I can actually lift my bum off the floor-woo! Yet that first physio had me trying to do the full bridge, ignoring the fact my core wasn't activating, which is apparently obvious if your knees can't remain still and stable. I've learnt that small focused exercises are much better than big movements and a little often is better than a big session. Your example of cycling is great, again it would have been easy and very very understandable to write off cycling as impossible. Instead reducing it to 2 min (or less) and focusing on quality movement has allowed you to build up. I have one of those seated cycle machines so you can sit on the sofa (no balancing required) and do just a bit. My friend has set up a physio area in her living room to make it easier to do a bit now and again. It made me smile to read that you also go in cycles of doing well at your exercises and self care and then it slips until you start spiralling. Keeping up self care and making the most of any improvements achieved is a tricky balance to achieve. I'm a health coach and teach people how to manage their conditions, and I still do it to some extent!
ReplyDeleteI noticed the comments/discussion on those who are very severely impacted, and can imagine they must constantly feel they are hitting a brick wall. It must be very hard to know where to start, especially, as you did mention in your article, when other conditions complicate things. It's a shame the lady who commented hasn't been able to access a hydrotherapy pool regularly if she felt that helped. I find hydro pools trigger PoTs as hot, but if I take midi, extra water and use the the seated/lying down equipment I can use and benefit from them. Normal pools make muscles spasm and aren't so accessible. A friend uses a cool vest to manage the heat intolerance, but sadly they aren't available on NHS :( But in terms of the very unstable joints some NHS OT departments will provide a custom made compression suit to keep joints in place/reduce dislocations. That could be of use for the hips or ribs/shoulders. When my hips were much more unstable I used to always were spanks type compression wear to hold things in a bit and for improving muscle strength I found a set of EMS shorts to strengthen the muscles without any movement of joints. I often wonder why EMS isn't being used in EDS patients with very unstable joints?! I hope maybe she/others can look past the upset and find some hope-to push for access or support to make some progress in their condition/situation.
This is a complex issue because EDS is linked to such a huge variety of health issues, some of which cannot be prevented by exercise, pacing and listening to our bodies. For example, I been headache-prone for many years and this has deteriorated, meaning that I can no longer watch a film without having a severe headache during/after it (it's something to do with screens as I also can't use a computer for more than 3 minutes at a time). I also developed pain when speaking, which is rare but seems to be linked to my EDS. Leg pain has remained stable for several years now, but I got arm pain following a very unpredictable event. So, hmm, since all this (and more) has happened in just about six years I'm not very optimistic about not getting more health issues, no matter what I do.
ReplyDeleteThis comment has been removed by the author.
ReplyDeleteI agree that this article can come off as offensive to some EDSers. Esp. since so many doctors- both before and after our diagnosis - told me & my EDS kids that we should simply walk & we would get better. ( Including from an EDS geneticist "specialist"). My then-teen daughter and I were routinely blamed for causing our own disability by not being active enough. (And, unfortunately, our experience is very common.)
ReplyDeleteAnd this is despite the fact that we both had severe Chiari, tethered cord, and severe cranial & cervical instability. Tethered cord prevented us from being able to walk more than a block on a good day - and not at all on a bad day.
Constant Chiari headaches - 24/7 pain that felt like someone hit me with a baseball bat in the back of my head - prevented me from being able to focus, talk or even be around any noise.
My spine and joints are so unstable that there is no way that I can "hold them together" long enough - or well enough - to exercise at all. PT's who focus on EDS won't touch me. When I'm upright, the weight of my own arms dislocates my shoulders. And I was *physically* unable to sit up straight because my neck was- in the words of my EDS neurosurgeon - "essentially broken".
I averaged two neurosurgeries every year for 5 years. My cervical spine is completely fused (from skull to T1), which was done over multiple surgeries. The ligaments in my neck were repaired. I've had 3 tethered cord surgeries & a fusion in my Lumbar spine. Now the vertebrae above my lumbar fusion is unstable & I need another fusion there.
My Spinal instability & multiple fusions make it very painful - & sometimes impossible - for me to sit up for more than 30 minutes at a time. Riding in the car is pure torture - after more than 15-30 minutes in the car, I am in agony. I feel like I've been run over by a train.
Not to mention that POTS prevents me from moving around or being upright for more than 5-10 minutes without sweating profusely, becoming out of breath and dizzy,even though I'm on a high dose of Midodrine. I even have to lay down when I'm waiting in a doctors office.
So, no, not everybody with EDS is able to exercise, or hold their joints together, or make themselves better. Some of us have permanent joint and nerve damage & we simply can't - physically.
You're a lucky one, and you shouldn't assume that others can have the same experience as you.
a mom here with a 15 yr old, on the cusp of a diagnosis.I see all kinds of unfamiliar terms Chiari and some in the form of alphabet soup here: POTS.??? (is that what's happening with the cold hands?) Her sister also has a chromosome disorder that manifests in similar symptoms of low muscle tone and chronic pain, from fractures, muscle spasms and headaches too. (an extra chromosome on every cell) We have been scolded by many for not doing enough preventative and have yet to have a providor that has another patient with the same syndrome as the older sister. Right now we are still looking for a geneticist for my 15 yr old. As Mom, I am afraid to preclude a future to communicate to her symptom wise, let alone management and preventative care. PT is unfamiliar with EDS and was just as unfamiliar with her sister. I have learned to count on the experience of patients to assist us with understanding etc. So please continue discussing your journeys. We have a young one here that we may be able to help with this early diagnosis!! Her sister had an early diagnosis in accordance to what others have said about their journeys and the knowledge was invaluable in directing her care and planning for whatever independence we can muster. She was nine when a chromosome analysis showed us why it was all happening. We also felt less crazy when others shared their journey of living with these conditions without knowing what was happening til much later in life.
ReplyDeleteHelp me with ideas for bedding, furniture- should I put a ramp in? Should I move her bedroom to the bottom floor-get her classses at school to one floor? How are you handling the skin rashes? does anything help the headaches and stomach pain? Are there certain foods that may be a form of preventative care? anyone have a story of acceptance of the knowledge at the time of diagnosis? Anyone remember being 15-with or without a diagnosis and how you felt? Did you do sports or other physically challenging activities and have to give them up? Who has helped you most? Is it family or what kind of therapists? Are we talking about a pool therapy of some sort? (we have been convinced that she became allergic to the lake about five years ago and the saltwater pool also seemed to result in a rash too)
Please know that this kid is responding as a sibling to a special needs child and does not want to accept that this joint dislocation and other symptoms are serious stuff. She will jokingly, twist her spine and is always wanting to crack her joints. Tell me if this is okay or not? I want to know if this provides some relief to her as to why she insists on cracking her joints? At some point she will need to accept what is happening and plan for preventative and management.
Thank you for your stories.
I have had neck and 'head' pain for over 20 years. I did some study on the vagus nerve and discussed this with my Physio. I have had three sessions with her doing trigger point pressure points and even though it it very painful, it is so wonderful to be free of neck and throat discomfort. I also have noticed dysphagia has ceased and GORD has improved.
ReplyDeleteI was diagnosed with EDSh when I was 67, so had suffered for all my life, wondering what was the matter with me!. My mother died not being diagnosed, but I visited her doctor after I'd been diagnosed and asked if he thought she would have had it, and he immediately said yes.
I have learnt that I really need to pace myself, otherwise I suffer for many days afterwards. I try to see the things I can be thankful for, even though EDS is a pain. We must keep on 'keeping on'!