EDS Hypermobility 3

[elliejjtiny]

Not sure how to start this off so I'll just copy and paste sparkle's opening post from the last thread and hope I've spelt hypermobility right in my thread title .

We reached 1001 threads, so here we go with a new thread. This thread was originally started so we could get together and share experiences and expertise in Ehlers Danlos Syndrome, Marfans, Benign Joint Hypermobility, and any other medical conditions including connective tissue disorders which cause joint hypermobility, pain and all the associated symptoms.

[auntevil]

ellie Lets hope that DS2 keeps thinking its cool as it will make the drudge of shopping a little easier.
DS3 (5 in July) still slips easily in and out of the shopping trolley seat - kept in there by edible bribes. Probably the only advantage of being a teeny one - that and other people's lower expectations of his behaviour
Bet he can't wait to start propelling himself in his new wheels

[elliejjtiny]

Photo should be on there now .

I think it's an unwilling thing rather than a size thing auntevil as DS1 can still get in the trolley if he wants and he is considerably bigger than DS2. DS2 is a dead weight at the best of times and although he can move his legs in all sorts of positions I struggle to get his legs to move through the leg holes in the trolley when DS2 doesn't want to.

[auntevil]

Bless him, he definitely doesn't look unwilling to be in the freezer aisle in his "borrowed wheels"

[SparkleRainbow]

Ellie - He looks like a little man with the world at his feet.....well wheels anyway!

Midnight - My ds has had a few episodes of VVS, but does not have an official dx yet. I used to have postural hypotension as a child, then they finally got round to recognising my very fast heart rate and very low blood pressure. That, tests, etc led to dx of Neurally Mediated Hypotension, which is completely linked to/caused by the EDS. I also have a mitral valve prolapse, not it is not too severe.

Busy week this week, Ds has lots on at school, but school are beiong very supportive and understnad he will keel over at some point. He also has hospital appt with nice Paed this week, to discuss fact that ortho surgeon wants to mri whole spine to see if spinal tethering causing incontinence, rheumie thinks this is rubbish, and believes his nerves are affected by position they run in spinal column, being too close to the surface than is "normal"......to MRI or not to MRI.....Will see what she says.

[timeandmoney]

Hello

I started a thread earlier today before knowing there was this one. I wonder if any of you can advise, if you don't mind.

18 month old DS has started taking about 10 or so unaided steps but still lacks confidence. I've presumed hypermobility as I was diagnosed when pregnant with him, and it runs in my family - though none of us have been that challenged by it.

I took him to a physio drop in today at the Children's Centre - thought why not? - and although they were too busy to assess him, they said he had very hypermobile fingers. But they weren't too worried, said he was close to walking unaided, even if his gait is immature.

He's totally weight bearing, climbs, crawls, has good fine motor skills - can pick up a piece of sweetcorn no problem - and has recently held a crayon and scribbled, even if it wasn't with finesse!!

My concern is - when will I know if his hypermobility is a 'problem'? When will I know if he'll experience pain? I got quite upset today about this. And should I take him to a proper physio? The woman at the Children's CEntre said it probably wasn't worth it at his age. She also said they see lots of hypermobile children, with hands like his, which made me feel like it wasn't anything out of the ordinary (even though she said she could bend his fingers back 90 degrees, although I've never seen him do this)

Thanks for reading

[bizzey]

HELLO timeandmoney....and welcome...I saw your thread on the other post and wanted to reply but didn't have time to do it properly or even know if I had the right information to give you to give you peace of mind ...??

On this thread there are alot of us who have very different expiriences of H/M and EDS and we are on our third thread now and all have different expieriences with it (I joined thread 2) and I i really dont want you to be looking on the downside of all our messages when you read them back as your LO is only 18 months old and ours are a bit older !

But ...the only reason I understood and learnt more about this was on MN so I will give you my history....but at 18 months I think it might still be a wait and see... my ds3 is 8 yrs next monthand only just been given formal dx

It was first noticed at his 8/9 month H/V check where h/v noticed he didn't want to weight bear (would not "bounce "on my legs) and was a slow slak/lazy feeder with slow (non-existant)weight gain.
I have an unrelated (we think at the moment ) problem with walking in my family which is why my H/V refered me to peads to get him asessed.

He was diagnosed with HM in his ankles which was causing the lack of stability in his feet ....she said it would be like trying to stand on a wobbely board iykwim..

At that age they are not bothered as they can strengthen up as they grow....I only started to get concerned ?2/3 years ago..

IMO PLEASE DONT WORRY !!! 18 months is very young... things can change...

Stay on here and some one might come along with more help than me ...there is alot of help and surport and and if you need a chat ?off load/moan !!

HTH

[SparkleRainbow]

Hello Timeandmoney - welcome to the thread, nice to have you...iyswim!

As Bizzey has said 18 months is still very young, although I certainly knew my ds had eds before that, and the gp agreed. The most improtant thing for you at the moment has to be just to be aware, and keep a keen eye out on his development. Don't expect a lot of support from the medical profession as most on here would agree that support is not often forthcoming, however there is bags of support and a wealth of experience on here, whether it is a big question, a little issue, or just a chat! I will plump up the sofa cushions and make room for you by moving my substantial backside up a bit

I think it is a good thing to have gone to the drop-in centre yesterday, well done for facing that demon. I am sorry you felt a bit upset afterwards, but I can completely understand why.

As Bizzey says, don't worry too much about what you might read on here, most of our dc are older than your little ds, and some have some more complex problems which are out of the "norm" for eds/bjhs.

I am not sure how much you know, so forgive me if I am saying stuff you already know about....bear with me.

Ehlers Danlos Syndrome is a autosomal dominant genetic connective tissue disorder, there are several different types, most of which include a degree of joint hypermobility as one of their symptoms. Benign Joint Hypermobilty Syndrome is considered by most experts to be the mild end of the scale, but basically to still be EDS Type 3, There are people on here who have, and or their dc have different EDS types, have an BJHS dx or have Marfans. Some people have a clear dx, others are still struggling to get one. Some dc are just affected in their joints, others have more pronounced affects elsewhere in the body. The one thing I have learnt about eds over the last 22 years is that it is different for everyone.

My ds is 9, he was dx by Professor Grahame at UCHL when he was 20 months old. This is very unusual, Prof Grahame even said so himself at the time. I have EDS, and although he is my first ds I knew from birth he had it, he was too flexible. He was late to crawl, roll over and walk, but not excessively so. His fine motor skills were always excellent, picking up small objects etc. I knew that his joints just moved too far. I knew what to expect but still in hind sight I can see I missed pain signals from him even as a small child. He cried too much when he tried to roll over, his shoulders were subluxing.....I didn't realise at the time. At 3 he regularly complained of leg pain, particulary mid calf, and had terrible cramps. It was only when he was school age that I started to notice real differences with his peers. He tired easily, he struggled to be able to dress himself, his handwriting was poor, he would sublux fingers and knee caps very easily. At 6 he really developed the probelms he has now, he dislocated his cervical spine, shoulders, knees, fingers and hips. His handwriting deteriorated rapidly, he was in severe pain nearly every day, his gross motor skills were very poor compared to peers, he also started fainting and having vasovagal episodes, lost weight and had poor weight gain.

Depending how you rate your gp, would determine what I would do next. It is really difficult to spot whether they are in pain or not, but if you think he might be, and your gp is a "friendly" I would pop along and discuss your concerns, whilst acknowledging that it is early days. A rheumatologist is best placed to make a dx, although some gp's refer to Paediatricians, having said that they might not want to make a dx at this stage. Still a chat about what to look out for might be reassuring for you. A physio is not qualified to make a proper dx, although they will have a very good idea. My ds has an excellent physio, but she always says we have to check things out with the rheumie.

Bottom line, don't worry, most people who have eds/bjhs have little or no problems at all, try and remember that.

Sorry that is a bit of a marathon post....did it help.

[TheHumancatapult]

Hi time and money

I ahve 4 dc with eds and only one of them experiances any real problems with it.£ have type 3 and just to be different my youngest probaby as more rarer type

often dx before 5 i unursual unless strong family line or extreme problems .

Ds got offical dx of Edsage 6 though was classed as hypermobile from 18 months .Though yes he is evere and yes he does struggle .He stil has a normalish childhood

sparkle how is Ds and wahey at least look like the Pd team are going to get involved with ds3 and transition though will see

[timeandmoney]

Thanks to all who responded. Really appreciate it. Feel better today!

I've not noticed DS being in any pain; he is a very smiley, uncryy baby...I do notice that he's bendy, but I've never noticed his fingers going back, for instance. So hopefully his 'range' isn't too bad.

I've decided to wait a couple of months. He met all his milestones fine - not early, but not worryingly late. Sitting up - 6 months, crawling 10 months. Walking has been the latest - he's 18.5 months and he's now walking unaided of up to 15 steps but I've noticed, day by day, how much stronger/more balance he's getting. He has a big head (not noticeable, strangely, but at the top of the centiles) and tiny, tiny feet which haven't helped.

My nephew has hypermobility syndrome and he was diagnosed at 11. he's massively hypermobile but is very fit and he's only had a bit of pain in one knee. He's got to do exercises daily. I'm hypermobile but only found out during pregnancy - my joints post DC have worsened, for sure, and my arches in feet collapsed - but I'm also into a lot of exercise (4 times a week) which I think helps.

So - I hope to ward off the worst for DS and will keep an eye. We do tons of exercise with him (stuff he enjoys) and will keep him active and fit. If in 2 months he hasn't progressed as much as I'd like with walking, then I'll get a complete physio assessment.

Thanks again. Hope that wasn't too boring a read!!

And if there's anything I should do - tips/recommendations - to keep him well now, then do advise.

[SparkleRainbow]

THC

I am glad the PD team are getting involved with transition....they were fantastic for me and ds, really got behind us and made it happen. I have my fingers crossed that they are as good for you and ds.

Ds is ok, tough week ahead though, he has got a couple of performances with his class, one being until really late, 9.30 ish on Wednesday night, which will mean I won't get him home until welll gone 10. They are just little school things, playing the violen with his class etc, but a lot of physical stuff in being on his feet all day until late, and so I know come thursday he will suffer. Last month was such a bad month, we got through so much medication, and guess what he grew 1/12 cm in a month! He is eating very little too, tiredness related maybe. Physio is rather stuffering, which I know is a false economy in the long run.....

How is ds doing? Is your appt with Prof G next week? Hope you are ok, will try and message you later.

[elliejjtiny]

Hi timeandmoney. My DS3 is probably the youngest on this thread, he is 17 months old. He has hypermobility and hypotonia (low muscle tone) and probably has EDS type 3 but it's a bit early to tell really. Your DS sounds very similar to my DS1 at that age. He walked at 21 months but hit all his other milestones at average times. He is now nearly 6 and he is a bit bendy and quite often comes home with an "I was given first aid today" sticker on his jumper but other than that he is fine. If your DS doesn't cry a lot then he is probably not in pain. My DS2 has more severe hypermobility and he has always been a very grizzely baby.

[TheHumancatapult]

Sparkleno guarantee that he will meet their critera . Got to wait and see .but getting nervous now as have Proffesor Grayham next week then local oars 2 data after so hoping he can refer us to people that can help .I shall be bringing up his height or lack of it moreto the point .

Orthtics tomorrow as well

POTS has reared it's head as a possible cause if his dramatic collapsing so be intresting see east happens

Hope the after school stuff goes well and dies not hit him to hard . Were enjoying the break from Physio as was advised to stop for a while

[SparkleRainbow]

Big day with school today, has started the day needing pain killers..... not sure he will make it to the end, time will tell.

THC - Who has suggested POTs, seems eminently pausible to me. Especially if hospital was a bit warm, as they often are, that makes me and ds go all wobbly. Who is going to investigate it for him?

[SparkleRainbow]

THC - good luck with orthotics today.

Timeandmoney - I think Ellie's advice about whether your ds is crying or fussing a lot is a golod indication of whether he is experiencing pain. In hindsight I can see that my ds was doing this, and you are ahead of the game compared to me.

[timeandmoney]

Hi Ellie and Sparklerainbow

Thanks for replying to me.

No, he's not a crier or a fusser. He's quite placid in some ways. I work part-time and his child carer and I have talked about this. She hasn't thought he's in pain, and she's very much 'on it' with him. He also has crawled a lot as he only started seriously walking about 4 weeks ago (17 months) and crawled from 10 - and he's very fast without indicating it hurts his hands or wrists.

I have weak wrists, though, so it wouldn't surprise me if it turns out that he does.

Thanks again - hopefully his journey with bendy joints won't be too bad. Bendy fingers crossed!

[elliejjtiny]

On the subject of pain, when should I be giving pain relief? DS2 whinges a lot and sometimes says his legs hurt but not often and I struggle to work out whether he is in pain or just tired. The only way I can tell is to give him calpol or neurofen and if he seems happier then it was pain but if not then it's tiredness. Is there a better way of doing it? He is 4 and has probably been in pain on and off since birth so it's hard for him to explain when it hurts and when it doesn't.

[bizzey]

elliejjtiny...I have been thing the same regarding pain relief ....pain can cause tiredness... and tiredness can make pain worse !!

I dont really want to be doing regular pain killers ..i doubt if it can be good for their little insides and have not been "medically"advised to so not questioned it with any doctor yet.

He is going on a school trip next week which will involve alot of walking and being told to "hurry up"!!!! We live in London so public transport is always used and then he will be walking around the British Museum. The walk to station and school is 15 mins (therefore x2).

Was thinking of giving him calpol and neurofen before he goes to school..?

Do think it might keep the pain at bay ???

[SparkleRainbow]

Giving out pain relief is a difficult one. I probably didn't start giving it out regularly until ds turned 6, and we had the incident with his neck. It is not good, but my ds does have to have it regularly to be able to function. I am careful that school and I only give ibuprofen on a full stomach, but ds says the ibuprofen works better than paracetamol.

I do use the pain killers prophylactically for ds, when I know he has a strenuous day ahead. I don't know if this is the right thing to do, but it is what I do anyway. Before we went to London and did the Natural History Museum in half term I dosed him up before we went, and during the outing too. He still had significant pain, but I think he recovered faster afterwards.....could be coincidence though as we had the use of the wehhel chair too.

[SparkleRainbow]

Ellie - if he says specifically that his legs hurt, I would give out a dose of paracetamol.

[elliejjtiny]

Thanks sparkle and bizzey. I give him calpol when he says his legs hurt but there are a lot of times when he just lies on the sofa grizzling and I'm never sure what to do then.

[auntevil]

Pain relief is a tricky one. I don't like giving any of mine any medication. With DS's IBS he has been given pain relief, which the gastro paed did want him to take continuously. I argued that he only needed it when the pain got over a certain threshold. I thought that the only way we would ever get to the route of what causes his IBS (unless it is the hypermobility - dr is out on this one) is if we can tell what triggers the most pain. Yes there is a logic in there somewhere
Had the paed's report on DS today. Some interesting bumpf on how broad hypermobility is as a dx - and that one paed's opinion of mild is another's opinion of more severe etc. Lots of stressing that hypermobility is normal - and occurring at a normal% in general society - blah blah blah.
One interesting thing that she did not really mention at the meeting was sport - and how gentle sport will improve his general well being/core strength, health etc - which I can go with - but that he needs to be wearing wrist and ankle supports whilst doing anything. Have any of you been advised this, and if so, how practicable was it at school. This is remembering that DS cannot get dressed on his own - Do school help with this?

[SparkleRainbow]

Auntevil -Sport is an interesting one, as is whether school will help him with changing. I adapted ds' sports clothes to make it easier for him, and make him able to wear his aspen collar (the school's top is a t shirt and he can't get that over his head, and can't wear it with the collar) he also goes bare foot inside (whereas everone else wears pumps) as he can't fit his feet inside pumps. At his old school I refused to let him wear formal shirt and tie, he couldn't do the buttons, but much to their dismay I gave him a polo shirt to wear as he could pull that over his head and collar. They really didn't like it, but they weren't going to take me on over it .
Ds's old school would not help at all, some individual teachers adapted pe for him, and supported him but most couldn't give a flying ....... His new school would do anything for him, anything to help him join in, so guess it will depend on the school. Ds's present, fantastically inclusive school adapt all pe activities around ds, he used to always wear his collar, but now only when he is experiencing more severe pain.
I guess if you present it in the way that his Paed has said you must do this, and he needs help with dressing, they will toe the line and help.

[SparkleRainbow]

For newbies this is an Aspen Collar, and what my ds has to wear to protect his extremely hypermobile cervical spine.
www.blatchford.co.uk/clinical_services/960149%2023%20Vista%20Aspen%20Collar%20Pat%20Info.pdf

[auntevil]

I spoke to the sports co-ordinator today. She is kind, but pretty useless. She did get back to me though and tell me that the physio will come and talk to me on Monday as to what the best supports might be.
The sports co-ordinator did say that DS's wrists worry her all the time - she is convinced that one day they will snap as they flick back and forth through every activity. She was also worried about his right ankle as he often goes down on it - umm, why had I not been told? I know he has a problem with that one in particular, but there's me thinking that its all due to trips at home

[TheHumancatapult]

yup ds3 i another that has uniform sort of that works best for him .

Pe at the moment ds3 does not get changed just becuase it takes him to long and lesson be over

Orthtucss hmm tosser is how i descibe new one he made his descion apparently with 10 seconds of meeting him without looking at how he walk.But will tacke him after next week

we have appoinment and have reps out to look at seating for ds3 on 3rd July so my chasing payed of

and the PD team are going to go and assess his needs to see if meets their ful critera frigging budget cuts but it looks very possibile