EDS Hypermobility

[SparkleRainbow]

I thought I would start a thread to see how many of you are out there, fighting the battles I am fighting. I have EDS myself, had genetic counselling and even though mine is quite severe I was told no problems with having children. I have three dc, the eldest my ds is the only one believed to have inherited it. He was diagnosed by Prof Grahame at 19 months, and has always had pain, but it wasn't until September last year that he dislocated vertebrae in his neck C2 and C3 by turning his head to talk to me. He has been in recovery ever since. In the last 11 months he has dislocated or subluxated his neck twice, his knee cap three times, his left shoulder three time and the fingers of his left hand once. He has missed so much school, he is in pain almost constantly, he has a health plan and risk assessment to be in school, he is depressed and frightened and he doesn't know at all how serious his neck problem is. I am constantly fighting with some of the Consultants, and school, but there are also some wonderful people too. I would love to hear others stories, very selfishly to know I am not alone and neither is my precious ds.

Sparkle - can you explain the bit about not responding to local anaesthetics and epidurals bit. I've just had one of those OMG moments . Although DS1 is my hypermobile DS - I hadn't really given a vast amount of thought about the rest of the family as in comparison they are not in the same league.
Then i saw the not responding bit and both DS2 and 3 have had problems with this and have had to be 'put under' by gas.
You lot do get me thinking about things did the Beighton myself the other week. 6/9 and in mid 40s - I just like to consider myself as 'athletic'

Misdee - Easy bruising is a well known symptom of EDS hypermobile type, and maybe other types I can't remember off the top of my head, as is easy skin damage and slow healing!

Auntevil - read this link www.hypermobility.org/painandhms.php There is a documented nonresponse to certain local anaesthetics like lidocaine, but not exclusively lidocaine. The issue with epidurals is that they use similar local anaesthesia in the spinal canal to deaden the nerves. My dear father awoke after major surgery, opened from sternum to groin, to remove a tumour which was over 9lbs in weight from his abdomen, with no pain relief as they used an epi or spinal tap (not sure which) and it didn't work for him.

at athletic 40 something with 6/9 on the Beighton scale......very athletic!

sSparkel how is ds

orry been absent ave been back in hospital again

Quick one Ds lates report says tat maxium ds could walk is 225 m and thats when he has done nothing else before hand and is caperable of doing nothing else after and this is done at a slow and odd gait and he stumbles .this was intesive 6 week program of pysio

Yet ffs erm dont let him use a wheelchair still from the physio how the feck else are we meant to mange

but am feeling very scared as of to GOSH tomorrow seemed took long time to getther ebut worried she dismiss everything and say well course he has to mange its only hypermoblity

lisa and missdee it is not normal , unless your in my family .

misdee with dh issues around is heart am suprised they not looked further .

oh and lisaasd

just to confuse you ds3 has eds but he holds his upper arms tight into his body and canot and does not extend them straight been noted by physio and ot

yet is hypermobile in his wrist/elbow/shoulder and fingers as well as elsewhere

Hello, I'm afraid I haven't read all 40 pages of this thread, but I'm hoping someone can advise me.

We have just noticed that DS age 4.5 is hypermobile in his ankles and fingers. Maybe in other joints too. He recently had an OT assessment who rated his fine motor skills in the bottom 0.5% and balance in the bottom 2%. She didn't say anything about hypermobility. (Also he doesn't show a handedness preference and tends not to use his hands in tandem.)

I'm now assuming that his fine and gross motor problems are related to the hypermobility. If this is the case then what's the treatment for hypermobility?

A gymnastics instructor told me that bouncing is good for tightening up loose joints and loosening up tight joints. Should we get a tramopline for DS's ankles?

If I go to the GP who should I ask for a referral to? Is there going to be a massive waiting list? Should we just go private?

Many thanks for advice

Ideally you need a physio referal to do some excersices.Did the OT give you any ? Especially on crossing the midline ? (using both hands together and across the body
How does he wall in extreme cases he may need supports in his shoes ?

Thanks catapult (good name). The ot told us to put toys in bags and jars for him to open, put cutlery and pens in front him centred (to encourage a hand preference), and get him to carry trays. We waited a year to get that advice!
He can walk straight, will often go into waddling/ministry of silly walks mode. He's always dragged his left foot a bit, but that seems to be getting worse. Or maybe it's the crocs I got him. Will supportive boots just make the joints weaker?
Overnight I've been thinking that maybe monkey bars, climbing walls, a trapeze might strengthen his hands. What do you think ?

There's two schools of thought on supportive boots some physics hate them and say that artificial prop up of joins do they won't strengthen and others recognise that takes so much effort to stay upright that they end up so tired doing that and fail in other areas .I would try a good pair of boots ( kickers boots are good )

Not sure how old your ds another good one for working on hand preference is posting things in a box but put box on other side to hand they are using so there crossing the body

THC how are you? Was it as a result of your fall in London that lead you to hospital again? I hope you are all right. They are not going to be dismissive tomorrow at GOSH, I am sure. I agree the advice you are gievn about ds' wheelchair is bonkers. I apprecaite that they want him to keep moving as much as possible, as exercising the muscles should help, but really, there has to be a balance. He needs the wheelchair when you are out, he can move around home without it if he is able.....stupid people who do not think about what they are saying and the practicialities of it.

Oodles - Poor gait, poor proprioception, and poor gross and motor skills can very much be a part of this condition.,If you are really noticing instablity in his ankles I would steer away from a trampoline for now. I would get some boots and physio input and build some strength with physio exercises, then move on to controlled movement on a trampoline with an adult to support, otherwise he could dislocate or twist the ankles and cause more damage. Go to your gp, tell him/her what the OT said, bearing in mind they are not qualified to make a dx, and ask the gp for a referral to a rheumatologist, then you will get a dx and referral to phsyio if needed, or advice about what to do with things like building strength. It should not take more than a couple of months to see an rheumie if your gp will refer. Problems tend to arise if the hypermobility/symptoms are more compliacted, or if you have a gp from the dark ages! It is worth a try before you pay for a referral.

Misdee and lisa you seem to have been dealt the same strong genetic card as THC! Misdee does your dh believe it is not normal yet, or do we need to pop round to sort him out

My ds is ok, but not brilliant. He woke up this morning and screamed in pain when he tried to get out of bed, his neck and middle of his back were extremely painful even to the lightest touch. I had to massage him gently, give him painkillers, carry him to the toilet, get him dressed, help him eat, lift him in and out of the car....but don't worry the dla say he doesn't need more care than an average 8 yr old!! [need humphing emoticon, but am too kanckered to humph] I did get him into school, as I think gentle movement is better than him just lying in bed, or sitting at home, but am leaping to the phone everytime it rings just in case. Although saga there, as BT currently burying phone lines to cottage, which invloved phone being cut off for a while, and trench they dug last week and have already filled in collasping, making a foot wide, metre deep hole which has filled with rainwater, and looks like a normal puddle, until you step in it!

Thanks sparkle. Ive made a gp appt. I'm sorry your little chap is in pain. Mine has lots of other problems but physical pain isn't one of them.

Opps on the puddle lol I end up wheeling. Straight into it and all your hear be squark

Sorry Ds is bad hope he manger school and bit easier

Update GOSh mixed bag One of the teams like well your just have to let him rest and not go out so much

But rest and physio were. Very interested especially as he has been doing their program and no real improvement .They want him to come in do their 2 week intesivd program as they need to tailor make a program for him . Be either November or January but means me and Ds staying in accommodation and asking my mum to cone stay with the older 2 Ds and dd.thoug may need to take dd depends .they said they don't allow people to travel in daily as find dc are tired before they get there

Now just got to wait and see :( so good but not good

I have read on their website about the intensive programme, it is supposed to be very good and have good success rate. I wonder if you could take dd with you, your older two ds would be fine with your mum wouldn't they. What a stupid thing to say, let him rest and don't go out so much....reality check point needed for whoever came out with that gem.

Ds back home and much easier and in less pain than this morning, so that is good. PSDD came in today, and discussed using a scribe and a laptop more and writing less, which school appear to have taken on board, they are really good this school and very positive, but it is hard for them to find the right balance, as they have never dealt with the issues ds has before.

Glad psdd team came in and that Ds is feeling netter time to get them on board with Rio am suprised there not already . Local physio want him to do some at school as in the morning to tired then at school

Am going to give it a go but is 2 fingers to local physio and pead who said no he won't need own tailored program from GoSh When I mentioned it to them previously so there going to have knickers in a twist about it

He will see the Drs again as they will be a constant follow up now And they was surprised that he is not been seen before .erm that be because people refused to refer him

Blinking red tape about the whole process. Your ds is affected far more than my ds and he needs a specialised program.....I would be tempted to add the very crude third finger to local physio and paed!

Get onto PDSS they have been fanastic for ds, they have made the biggest difference, and they should be involved, the physio during the day is proving to be a godsend, he is not too tired, he is active before and after with being at school, and being in that learning enviornment really helps him to focus on it rather than the persuasion that has to happen often at home.

Hi GOSH programe is hard work, but worth while. The accodation will be at the patient hotel opposite the hospital and they give you vouchers for the child so that they can eat at the hopsital restaurant. The patient hotel is lovely, clean and well equiped. The intesive physio rehab is tough on the child, and the second week is spent convincing them to go as it hurts and it's boring! We are due to do our second stint at the 2 week rehab also in January, as we did one 18 months ago, and are on 4lb weights, but need to go to 8lb. It's all a nightmare isn't it? We now have extra investigations at Northwick Park with Prof Pope - and VUD at the Evelina, a year of hospital again and uncertanty. We use a wheelchair for the 'pointless' stuff - I'd rather my 7 year old had some time playing at the park on good days, rather than wasting his energy on walking there and back. GOSH seemed happy with this as did Prof Pope and Grahame, some people love to give opinion on a condition they have only looked up on Wikki - they should try living with it!
I rarely get the chance to catch up with you guys these days, but often read the latest, and it's heartbreaking that all our stories and struggles are so alike.
Here's sending love and best wishes to everyone xx

Hi cars

We may well be there in January but not sure how well will work .Ds does not like strangers and has problems understanding speech especially when busy/noisy

Ds ready does the program at home my main concern is genetics feel there is something more going on and are we trying to fix something that can not be fixed as such as down to chromosome .and weights lol not a hope in he'll I'm expecting Ds to point blank refuse some point in the 2 weeks once that happens they have no chance of him doing

Something local team now struggling with after their intense program he cried when Sue said she was a physio :( but he will do them better for me

And seems maybe problem with accomisation as though they have wheelchair friendly rooms the kitchen area etc are not as they set for kids in wheelchair not adults

Cars gosh ate saying just rest or dont go out :(

..yes, us too, we're being assessed for VEDS amongst others. My son was a refuser, and I hated to be near the gym early in the day as I'd hear him protesting and then he'd cry. The team are kind, but VERY firm and as parents we weren't allowed to either sit in or take them out - other than to come off the program altogether - and when there is only this - muscle building together with the pain clinic you have little choice. My son is also undergoing investigations for AS and the team at GOSH are used to children with complex DX's, but it's hard non the less. I found the support of the other parents invaluable as we were all going through it together, and that helped the children as well as us.

If you call the accomodation manager at GOSH they will book you the right room - I'd try to call them as the rooms are booked up with the appointments in a block.

Trouble is cars we have no date yet was told either be some time November or January which is a pita as I need to arrange childcare for my other 3 dc .

Cars is access to kitchen worried about if workflow are high same with sink in kitchen for washing up I can not reach them let alone make coffee

Or do the vouchers for food cover all 3 meals .not been given any more information other than potential month and they want him to come in and do

Ty for advice to call Cars:)

Seems they have 20 accessible rooms but. Only 5 have wheel in shower and said there often booked out quite quick for people where the dc need also they checked with housekeeping microwave is on the wall at most people eye height workflow are normal height same with communal sink .They admitted they never really thought about the fact adult in wheelchair needing to access Kitchen

So oops we won't be doing any cooking

Hate the waiting for a date now can't organise anything .How long was it once you was told a date after they said dc need to attend the course ?.Was you given loads of notice

That is a hard one THC. I would ring the accomodation manager now, before you have a date, and ask the questions about rooms, accessibility to the kitchen etc, they are be accomodating I am sure, and anyway they have to be under the discrimination laws, laid down by Equalities Act 2010. Not that it will come to that I am sure, but if you ring, tell the manager you haven't got a date yet, but how are they going to be able to accomodate you and ds, and either feed you, or provide appropriate facilities so you can prepare food, she/he will be able be helpful I am sure.

I have done it sparkle hence the oops but they said can n

Not do anything till we have a date . Will give physio a week or two and start chasing as I really need as much time as possible. Seems they never had a disabled parent stay or least one without another adult to help .

Lol funny one place you expect is a hospital but guess GOSH more used to kids in wheelchairs Let alone I need both my chairs power for outside manual indoors officially I could get away just with power but always take both because if power breaks I'm stuck

How is Ds

Really hope it helps Ds.