EDS Hypermobility No. 2

[SparkleRainbow]

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.

nev know how you feel. Felt a bit glassy eyed when I read your post. It would be lovely to sit back and let life come at you rather than having to pre-empt everything and look for the problems and solutions first.
Theres some really interesting statistics about dyspraxia and dyslexia links and co-morbidity. The last paed tried to suggest dyslexia as well, but I want to check his tracking first as I think that is more the issue. (((hugs))) from me too.

Cars

what seatingdoes ds have as that can help with problems also not sit up but other core stuff may help

Thanks everyone, it is hard going at times especially when they are struggling on - my son rarely complains. We have been told to 'intercept' his pain - but that's so hard - do you remind them, or encourage them to push through - everyone has a different take on it.
We can see on his face when he is finding life tough - right now he looks ill - a friend said he'd 'gone beyond pale and was now grey'.

Our dypsraxia and dyslexia dx's were from a huge 8 hour assessment at GOSH and are additional to the autonomic dysfunction, and the EDS. I asked regarding links and again some say yes, and others just say that a percentage of the population have these things, and basicaly he has just had a car-crash of difficulties all together. Trouble is they all make each other worse in a tragic circle of physical and mental difficulty. I wonder how life will be for him when he is older.
We are still Home Ed-ing - but have found a school - and have a formal offer of a place.............. but its independant and so off to Tribunal we will have to go as our LEA are less than helpfull, no shock there is there?!

THC - we have a Teezie Breezie and a full sized Breezie, but we have looked at the Posturite chairs. GOSH said at one time he should be hip height to the table, now they suggest that he should be at chin height. Poor boy either looks down from on high or has his lunch right under his chin!

You either laugh or cry, most days a fairly consistant mix of both - but I know I'm not alone - thank you for all your support, it means a lot xx

Hi everyone, sorry I have been awol again.

Big hugs Nev, I am glad you are still with us, even if you don't want to post.

I have read up to date, welcome sonatenatz, nice to have you with us, iyswim I would go back to the gp and ask for a different gp and second opinion, and either get back to see the dx paed, or even better ask to see a rheumatologist (EDS usually comes under their remit, although others get support via paed)

Shall we do a little personal review for newbies? ......... I have eds type III, dx nearly 20 years ago by Prof Grahame. I have 3 dc, only the oldest my ds (now 9) being affected with eds. A positive dx, orginally made by Prof Grahame, when he was 20 months, but confirmed by many over the subsquent years. He is profoundly affected by pain, with extremely hypermobile cervical spine, shoulders, hips, knees, ankles and toe and finger joints. He suffers from urinary and sometimes bowel incontinence, regular subluxations and dislocations, including in his spine which is quite dangerous for him. He has the physical symptoms which are similar to dyspraxia (this is an agreed symptom of eds III, so poor gross motor skills, poor kinesthetic awareness, poor handwriting) he also has neurally mediated hypotension (probably). The current studys do not support a link between true dyspraxia and eds, but many mums on here would say different....I know who I agree with His feet are not truly "flat" but the foot joints collapse under his weight, and he has a pronate as a result. He has a physio routine, and takes daily pain medication (paracetamol and ibuprofen), neck brace and foot wedges and arch supports. He has a paed, two rheumatologists, an orthopaedic surgeon, a physio, a podiatrist, an orthotist, a clinical psychologist and a full time TA for medical support. Academically he is not affected at all. I have probably missed out lots.....sorry.

Ds is having a bad time at the moment, hence by absence. Lots of visits and stays in hospital. We have a family reunion this weekend, so taking everyone off there, so I can get three days of critism and abuse from my MIL in both English and more covertly in German , hopefully I can keep him relaxed enough to be able to enjoy himself. I won't be able to be back on before Monday.

Enjoy break if you can I may suggest ear defenders nit for Ds but for you .. Maybe make most if can and go fir a walk on your own to the nearest cafe

Cars yes we get the different heights maybe ask for a new chair that goes hi~lo so more adaptable that's what holding Ds gets for school on September oh and on wheels to

May s best trying what feels more comfortable for Ds

Thanks for the advice Bizzey and Auntevil,

Lots of the things being said on this thread are similar to my DS although I would say he doesn't sound as bad. He did walk at a normal time although he was much later than his siblings who were all early walkers. The pead advised to have him fitted in ankle boots which do seem to help. The school seem to have been the best help putting things in place.

I think my DH and I are going to go back to see a different GP together with him, I'm also wondering whether we can go directly back to the paed who originally diagnosed him although she also brushed off any link between the hypermobility and his other symtoms, has anyone else done this?
Would it help in getting a second referal if we mentioned EDS to the GP or would they just be likely to be more obstructive to giving us a referal because we would be seen as telling them how to do their job?

OOh sorry that's a bit long. I'm just really pleased to have found a place where people seem to know what I'm talking about

With regard to the boot issue, we were using Piedro from when he first started to walk [with a kay-walker] but as he has grown the ankle needed greater support. Piedro apparently don't go above ankle height until a large size.
We now have 'Nimco' supplied with custom gel orthotics. The orthotics ensure his feet sit properly as he pronates, has flat feet and his ankles sublux fairly regularly and this then dosn't help the knee-cap subluxes. The higher sitting boot has really helped.
What I just fail to grasp is why some children get boots and others don't [obviously its cost, but why should this ever be a valid reason if something is needed that will help].
It takes years of battle to get what our children need, it's crazy.

Sparkle - what do you do to help with the spinal issues - I'm at a loss here, and it really worries me. Has it become worse with time, or have you just learnt how to deal with it as time has gone on? xx

((((((((((((((((HUGS))))))))))))))))) everyone !! Struggling to follow everyones posts at the moment as I have been on a SATS thread I know ..what a turn coat

We had OT yesterday and confirmed what i already knew his hand writting problems are down to h/m and low muscle tone in hands/arms/shoulders/body(posture).

So i now have more exercises to do with him which involve squishing theraputic putty(thats what it says on the tin!!)

They want him to have a slant board but not the special seat as they hope is own strength will improve without it..

He was in agony in the session and I so wanted to tell her to stop but I knew she had to test to see how far he could carry on

Now for the big question because there are too many posts for me to check back on.....shall I try for DLA ??? I wont bore you with what i have to do but he does not seem to be as bad as some others and do you get it ???

They want me to swimming and horse riding and i cant afford it ???

Do you think I would get even the lower rate ??

I have somthing else to ask but I have gone on long enough

I would apply for it , ds3 gets it but has other issues .

Yeah there's some theory of thought that piers don't do a lot that kickers can't do especially the smaller styles

We have splints but that's a pita in the end trying to get castings 18 week wait from saying his ate marking him toll were seen

I'll do the newbie update thing later as that will take ages and I need to sort out tea etc.

DS2 gets middle rate care dla and I'm waiting to hear about mobility. DS1 and DS3 don't get anything but they aren't nearly as bad as DS2.

We were awarded middle care, but are on appeal for mobility and higher care due to 'difference of opinion' at DLA over the inability to walk distance due to crippling pain and dislocations, vasovagual fainting without warning, and therefore needing a wheelchair [and as others have said GOSH are anti this, but agreed that it is needed for him to cope]. Oh and DLA didn't think 3 hourly catherterisation accounted to a 'problem with toileting'!
CAB doing our appeal now. Its all a bag of poo to be honest, but if you don't ask...........

newbie update:

we're slightly different to most on this thread in that DD2 (14, recently diagnosed EDS type 3 by Prof Graeme at the Adoloescent Rheum clinic at UCLH) doesn't have the joint issues that most seem to have - she has Postural Orthostatic Tachycardia Syndrome (POTS) which is basically a lot of fainting. Her ankles are quite weak though, so Prof G has recommended Dr Marten style boots - still waiting for that in writing though for school .

DD1 (18) does have joint pain though, and she's waiting for her appointment date at the National Rheumatic Hospital in Bath (there's some issue as to whether she can go to the teen clinic - 30 days waiting list - or the adult clinic - 3 month waiting list) to see if she also has EDS.

DD3 is very hypermobile, but no issues with that so far (touch wood). And I'm also quite hypermobile (can touch the ground with my hands, and also realised recently that my knees hyperextend).

And this thread is great, I've used it for ranting and moaning, and everyone has been very supportive, so don't worry about jumping straight in with issues .

Thanks everyone...well Isuppose the worst they can say is no ,but i really would like to be able to do more with him than "just" our exercises..

Has any one had any expirience of the horse riding ting ??

NinjaTheGoose was telling me that in out PCT, they are trying to stop piedros - probably due to £££ - but telling them that they believe that research is showing that younger feet develop more strength with other orthotics (inserts etc) than being fully supported iykwim. I know that she has been told to try new inserts for a while before they will consider her DD for more piedros.
THC Ninja had a 6mth wait for her inserts rather than piedros that had been agreed as her order had been stuck on some accountants desk! What are they meant to do in they meantime?

There meant to just get on with it

Having learnt a lot and read a lot I'm leaning toward the fact eds is not at the root of ds3 problems as he is not like most your dc ,

Very hypermobile and low muscle tone from birth . So all gross delayed and still delayed but no dislocations also never really complains of pain and is not lacking in hid physical confidence ( think climbing stuff as he go up on knees bum etc what ever

Think be talk with GOSH kn June feeling were missing something else

And 3 years long wait for the DDD study

Hi all, have been absent for about ten pages, DS has had a chest infection and has been on home IV antibiotics for the last three weeks. Exhausted is not even the word. They also now believe he has diabetes which, for a needle phobic seven yar old, just adds the the trauma of the past few weeks. DS has had several blood tests over the last few weeks and has been sick to the back teeth with it all. They suspect he has diabetes due to low muscle tone, and no weight gain, despite his 3000 calorie intake. I have asked them to consider his BJH as a cause of low weight, but this has been dismissed.

Bizzey, can you ak your physio for any swimming courses they do? DS got referred to hydrotherapy which happens in our local pool. Very useful and ran by our hospital physios so there is continuity of care.

You can apply for DLA but I know that they are rejecting a hell of a lot of applications at the moment. Can you get your GP to write a letter of support? We were told to avoid anything that would over exert the joints. DS does trampolining and we were told not to do this competitively. But horse riding might strengthen areas without extending them? We were also told to buy an xbox kinect to help him do daily exercises, I got a second hand one for about £60.

I get higher rate care, but this is mainly down to his CF, I haven't even included his BJH/dyspraxia yet!

I have not gotten any further with our dx or care. We have missed several hydro sessions as DS can't swim when on IV antibiotics. Add to that the school are on our back for his high level of absence! (they knew the score when they took him on, fuckers).

I would recommend anyone with DC who have sluggish bowels ask for movicol. DS has always had pretty poor bowel movements (and had a large impaction at birth requiring surgery - but this was CF related) but since he was prescribed movicol he seems to be more regular, and less impacted. It's a regular medication but a very mild one.

I feel a bit of an outsider on this thread, as DS has other issues, and his hypermobility isn't even that bad - well he can't write, but that's quite good in comparison.

Reindeer your not an outsider

Ds3 is not as affected as a lot of the dc on here . And knows EDS is not to Blame for a lot of his other issues and beginning to suspect we are missing something else at fault for his issues and no one looking beyond at the bigger picture

The calorie thing I know with CF has problems breaking it down so could be malabsorbation issues Ds2 has weight issues and is on 5000 calories a day but is a tiny 48 kg not bad till you realise he is 6.2 .

But I can recommend Addenbrooks Gastro team who are fab

THC...you were up early !!
Thanks for all the replies !

REINDEER you are not an outsider. I felt like that for a while as my ds had not dislocated or broken any thing ..but I think it just goes to show there is no "norm" in H/M..we are all expieriencing it differently..

The other question I wanted to ask you all but I thiught my post had gone too long so I stopped is to do with EDS(I think)

SENARIO.....

At school some mornings I have noticed the squishy bit of the hand under the thumb(Iknow it has a propper name but can't remember it heel..ball) looks blue and i have always thought it was pen and not washed off properly ....I try and wipe it away with my spit (what would us mums do without our spit!!!)

anyhow the first morning letting him use his therraputty in for drive to school they were blue again and I know he had used his pens that morning i.e not ink .....could this be bruising down to EDS as I am still awaiting pead app for June so have a long time to wait or part of his anti-coagulation problem ????

Any thoughts on the subject thank-you

reindeer you are not an outsider at all. All our DC's are different and DS2's hypotonia is worse than his hypermobility.

THC Your DS seems very similar to my DS2. He has always had very low muscle tone and has never dislocated anything although the physio says that's because he can't do a lot of the things that causes dislocation. He isn't physically confident though. Does your DS have raised muscle enzymes? DS2 does but not high enough to be muscular dystrophy.

bizzey I never noticed it before but my squishy thumb thing looks blue too, weird. Not sure about the DS's as they are asleep.

My newbie update post:

I have dyspraxia and hypermobility, DH has Aspergers syndrome and hypermobility. I have bendy ankles and wrists, hyperextending knees and elbows. I have about a million stretchmarks, I've had 2 miscarriages and I've had PROM with 2 of my children. DH sprains his ankles regularly and his very clumsy.

DS1 is 5.10. He has mild hypermobility. He was a late walker (21 months) and is generally bendy. He has some continance issues and still has some accidents. He wants to be a transvestite when he grows up. People describe him as "the normal one". I can't think why .

DS2 is 4.0. He has severe hypermobility, hypotonia and gross motor delay. He walked at 2.10 and still uses the buggy a lot. He is going to start school in september and he is very excited. We have appointments all over the place at the moment with occupational therapists, orthotics etc. He wears piedro boots and we are waiting for an appointment with the orthopaedic surgeon to sort out something for him to wear for PE at school. He is newly potty trained and is at the stage where he likes to use as many toilets as possible every time we go out.

DS3 is 1.4. He has hypermobility and hypotonia. He can stand with support while leaning on something with his arms and tries to cruise. He has been referred to orthotics but his feet are too small for piedro boots at the moment. The health visitor thinks he is failure to thrive as he is on the 0.4 centile for weight but he is off the bottom of the chart for length so he looks quite chubby. The paediatrician thought some high calorie milk might encourage him to grow in length but he has just got fatter and not any taller so we have an appointment with her and the dietician at the end of the month.

Newbie update

I have 4 dc with EDs

Ds1 no main problems but dyspraxic

Ds2 Gastro issues can't maintain weight

Dd only really handwriting a issue that applys to ds1 and Ds 2 as well

They have dx of EDs type 3 as do I

Then we cone to ds3 he has query type 6b , now looking like cardiac issues to

Did not walk till 3 has seating etc Very low tone wears splints etc

Ok ladies I got fed up of lack of support from GOsh and were going see Proffesor Grahme privately so he can point us in what or who next

Ellie ds3 stays in his boots /splints infact he does not even get changed for PE as it takes to long lesson be over before changed

Sparkle did you survive the weekend with mil and how many bottles of wind were needed

Yes MD was considered for Ds3 . Think next stage is he need tissue biopsy and muscle

I'm back, I survived....just. Drank a lot (ok a huge amount) of white wine, let a lot of nasty comments float over me.....although I did quip back when MIL critised my lack of fluency in german, that I would learn more german when she had learnt some manners!!!

Where to begin...

THC I am glad you have got a postive decision made re GOSH. I think you are right, they are just not even coming close to meeting ds' needs. I hope the private referral is not too expensive.

Reindeer - don't you even think for a moment that you are an outsider. The thing about eds is that it is a syndrome, rather than a disease as such, which by definition means everyone is different......and if it wasn't for all the differences and experiences on here, we wouldn't know half as much as we do, and twice as much as most doctors .

Bizzey -I applied last year for DLA and was refused, even on reconsideration. I had real problems with it....they said his incontinece issues were entirely normal for a 8 year old, as was the amount of help he needed with washing and dressing. They finally recognised that he could not walk far, and frequently fell, and that the falls were potentially dangerous, but then said no anyway. I lost the will to fight any further, which is what they wanted anyway. Ironically we might be more successful if I tried again now, as we have so much more support in place with LA and PCT....but still not sure if I can face those forms again. There may be some support with swimming via your local Physical Disability Support Service and/or your closest PD school. As for horse riding, dd1 does that, it costs me a small fortune, so no other clubs, comics treats etc. Ds could not do it, never in a month of sundays, and his core strength is much better nearly 3 years after starting physio to improve it.

Sonatensatz - there is no harm in trying to connact the paed you orginally saw, directly, but if that doesn't work I would go to a different GP at your practice and ask for a referral either to a paed or a rheumatologist.

mycars - ds' spinal issues are a right nightmare. We get very little help with it. rof Grahame said when ds was 20 months that he had a very hypermobile cervical spine, but we didn't really understand the significance of it. He was 6 when he collapsed having dislocated c2 and c3....it has been a steep learning curve ever since....physio, neck braces, pain relief, learning the neurology symptoms, learning when the panic and when not to....a complete and utter nightmare!