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.

at comments about voetus not getting sufficient nutrients...some people are so ignorant!

Hi, this is so familiar it's scary.
Aunt - we are at GOSH Monday - so will miss you, and will miss missing you - would have been great to meet up.

Sparkle - the genetics are going to take up to a year, so we're on a waiting game, but we know we have internal smooth muscle issues, it's just what else is going on, they're looking at all the connective tissue disorders they say.

The Catheters are going ok - we talked about a permenent site, but he has huge EDS skin and healing problems, and they are concerned about the internal wounds and the external - he scars and the scars stretch and can open back up. The normal healing would be about 6 weeks plus. At the moment we're doing CIC and will take a look at the more permenant options when he is older - he's only 7 and it's like all of this, one step at a time I suppose.

As for the skinny thing, it sounds horrible but he's so skinny he looks like Golum without his clothes on!
The genetics team explained that many EDS children feel 'full up' very quickly, due to the uncomfortable feeling as the stomach stretches. We have to give him regular snacks and energy drinks. He has a dodgy diet as he can't take high fibre as he has fast transit, and has to have high salt and sugar.

Reindeer - this is the one place we can all moan and say the things we can't always say to other people. It's only people who are living this every day who really 'get it', it's hard - hence why I can't always face coming on board, but to know that there are people who understand, and who I don't have to explain everything to, for them to understand means everything.

This is a full time job, and it's for the most important people in our lives, who cannot speak up for themselves, that's why its so hard xxx

bizzey thank you, I would love someone to get through the diagnosis with (along with everyone else on this thread), perhaps we can compare notes? :). Where are you up to now? We are just waiting to get an appointment with the OT having seen the physio (which we got through our CF physio). My DS is in year 3, which I think is like your DS, so we will have similar ideas of where they should be and where they actually are.

My son is classed as clinically malnourished. He is on the 2nd centile and funnily enough we have also made comparisons to gollum! We only know he is so light due to the constant monitoring, as it has a direct effect on his chest and when he's lighter he gets more chest infections. We had a PEG fitted two years ago and DS eats normally during the day too. He currently takes in 3000 calories a day but is still only 18kgs which is frustrating.

mycar thank you for your kind words, I saw that your DS has regular catheters, having they considered a portacath? DS has one and it's been fantastic, especially since he developed needle phobia. His skin tends to take ages to heal, so much so that during a previous operation they tested him for a coagulant condition.

sparkle I'm just off to read your link, to learn some more hopefully.

bizzey and reindeer I have dyspraxia so if you need any advice about that just shout. I don't know much about the education side though as I was diagnosed at 19.

DS2 and DS3 were FTT as babies but not anymore. DS3 has reflux so before that was under control he puked a lot. They are now concerned about his weight and length because he is just under the 0.4 centile for both. I think that's just the size he is meant to be as he is in proportion and I'm 5ft 1in so he just takes after me. DS2 is normal sized and stopped being FTT at 18 months when I became more relaxed about what he was eating and let him eat lots of cake.

I've been looking at wheelchairs for when DS2 starts school. We went to the mobility shop in town to have a look and take one for a spin. DS2 wanted this massive mobility scooter . I liked the manual wheelchair with the big self propelling wheels at the back and handles as well. It pushed like a dream and felt so much lighter than the buggy. It had a strap for a walking stick which I think I could attach our tiny stroller to so I could get to school with ds2 in wheelchair and ds3 in the sling. Then when I get to school I can take ds3 out and put him in the stroller.

Problem is last time we went to wheelchair services a year ago they wanted DS2 to have this massive thing which I wasn't keen on as the last time we had a buggy from them it was huge, awkward and kept breaking down. They said it had to be that one as they said he needs supportive seating. Also now he is older I want him to have a wheelchair ather than a buggy because he is being made fun of for being in a buggy still when children much younger than him are walking.

I'm not sure what to do. He doesn't have supportive seating at home anymore. The wheelchair would cost us £295 which I think is very reasonable although DH isn't keen on spending money on a wheelchair when wheelchair services will give us a SN buggy. I think there's no point in having a buggy that is likely to break down on a regular basis. I'm also a bit worried as I've always had a spare stroller and sling just in case the buggy breaks down or gets left in the car so I can still get the children to appointments and school. Now DS2 doesn't fit in the stroller I've got no back up plan and DS1 has already had 1 day off school this year because the buggy broke down and it took all day to ring round and find someone to help me get it repaired. The school weren't happy with me and I'm worried about it happening again. I'm wondering whether to buy the wheelchair and then have the sn buggy from wheelchair services "just in case". Sorry for rambling on. I find that you understand what it's like better than DH who can drive and will never have to get the children to school without the car.

I know you shouldn't have to fork out for the wheelchair, but from what you have described I can't help feeling it is the best option. Your dh, rather like mine, is not so good at seeing the reality of the daily situation for us, as opposed to for them.

Also absolutely furious just a tiny angry about school not being supportive when the the buggy broke down! How about someone from school volunteering to come and help and collect ds for you...now that would have been the decent thing to do!

reindeer .I find when ther s other issues seems Eds taks backsat in proffsionals eyes

hi sorry im dipping in and out at the moment .Reindeer if they are willing to give sn buggy .Go back to them and say no it will make him be viewed as a baby he needs a wheelchair for indeendence

Be very care ful buying wheelchairs £295 is very very cheap and i would wonder on th fitting on it and how supportive it is .Even the nhs cheap chairs are £600.( action 3 etc)

Home wise you can request an home asseemnt for supportive seating its provided and done y ss OT .We have star chair at home and considering buying a p-pod

.Ds is very small to , is on the 2% line height and weight so is a tiny thing looks more 3/4 than almost 7.

Have been asked by school to get DD refered to Ot as her handwriting and shouder pain is bad and school concerned

Mad few weeks in April .We have week in Scotland .Then back and week of rehab in gosh home for weekend wheree were taking part in undrasing car show then back to Gosh for rehab

hello just had a (very quick) catch up ! ds is really thin and lots of people comment about it even the gp has said she shouldnt be able to see all his ribs protruding but he is constantly hungry and always eating (has allergies though so diet is not very fattening which doesnt help).

ds and dd starting weekly physio at hosp again next week as both not doing brilliantly at moment and dd1 especially tired.

hope everybody is having a good weekend.

Just caught up - a bit hectic at the moment as I'm making bridesmaid dresses (why do i volunteer - note to self - learn to say no
Cars - keep a seat warm for me on Monday - I will use it on Tuesday
Reindeer - not everyone in life has someone to talk to and bounce ideas off - that is why this board is so good as someone always seems to come up with an idea to help ime. Please stay.
This skinny thing is weird. I had to rub my eyes to check that I read '3000 calories a day' . But then thinking about it, all 3 DS eat similar amounts to adults in calories and are all underweight for their height. DS3 is failure to thrive, but they can't justify putting the eldest 2 on that because they are so tall. DS1 is 99.6 centile or above for height - and just over 50 for weight. DS2 is slightly smaller as he stays at the top of the height chart but has never gone off it, and is also 50 for weight. So as they are a good weight for their age, they are tall and skinny. The school nurse said that there was nothing wrong with their weight, they were just too tall
DS1 has dx dyspraxia and I have to remind so many people of how hard he really does work to stay at the level he is at. It is all too easy for his teachers to have this at the back of their mind when he is actually working harder than many of his classmates to do the same amount of work. My one consoling feeling is that effort and determination is something that DS1 has in bucket loads. He's had to. Because of this, I know that he will succeed at what he wants to do because he knows how to pull out the stops to achieve.

Yes, I'm feeling in a very rosy and glowy mood today - and am going to go and give the boys big hugs and remind them just how brilliant they are, because sometimes I forget how hard they try too

Auntevil you brought a tear to my eye, and I had to go and hug all of my dc and tell them how wonderful they are.

Actually I am very pleased with ds at the moment, he has been working so hard on his core strength since June last year, when we finally got him into a decent school and he started working with his TA. They have done so muich physio, groww motor skills work, building self confidence and hydrotherapy, ds finally managed to swim on his back an entire length without any aids, and without putting his feet down once. I nearly cried when I saw him. This has been such an achievement, even at xmas he was still sinking in the middle, lead swimming shorts, but if the ta ot I get him flat on his back and tell him to tighten his stomach and back muscles consciously, then he was able to finally swim and keep it going....a major major achievement, I am so proud of him.

When ds was FTT last year he was 91st percentile height and 25th percentile for weight.... he then rose to 50% weight and the paed said he was still within the category FTT...greater than 2 percentile bands different. I don't think that nurse of yours auntevil understands how it works

THC can the school not refer to the OT for the handwriting and shoulder pain? Seems like something they could pick up.

I'm a regular poster on this board but not this thread.

DS is 9 and has HMS and Asperger's as well as some other difficulties. He went to GOSH for their rehab course but he has been in school 2 weeks full-time now (he started a new school part-time in Jan) and he has just hit the wall physically.

Did anyone get any help/advice from GOSH about managing this in school? We got a basic report which had a throw away line about pacing the school day but what does this mean in real terms.

I am sick of him lying on the floor because he can't keep himself upright all day. School are very good but he got really upset today because he was told he couldn't have breaks but equally he can't be out of the classroom all the time.

We have so many different people involved in our lives but no one gives any guidance.

Seems Ot can't take referrals from schools

Ds3 is really really tiny weight/height

Hi appropriatelytrained....can I ask why your ds was told he can't have breaks? I have not been to GOSH, so I can't answer that bit of your question, however others on here do go to GOSH. It has been acknowledged for my ds that he finds full days difficult, especially when pain levels are high, but no medical professionals have offered any advice. Therefore I have agreed with the school what we will do when this happens and have to say the school have been very proactive about it. The Headteacher suggested shorter days, coming in late and going early at the end of last academic year when ds was tired. At the moment ds gets breaks, he doesn't have to go out to play if he is too tired, he stays in the classroom with a friend. Every day my ds has a eds related activity, either physio, gross motor skills, self confidence activities, or hydrotherapy, they are timetable in every afternoon and are matched to his needs on that day, so if he is very tired he has some downtime. He also has help with writing and moving around school which is helpful too. It wasn't always like this though, in his previous school the Head was an nightmare, to do the best that we could by him under those circumstances his classteacher but some inobtrusive things in place, like she gave up her assembly release times and lunchtimes, so he could have a lie down, and even sleep.

Pacing the day for me could mean starting later, to allow more sleep and or physio before school, doing the more important lessons, adapting playtimes and lunchtimes, to allow rest periods, using other adults or children to scribe, use of IT, use of assembly times for physio or down time..... I have to admit in the previous school I just when in and demanded that this is what his school day was going to look like, at the new school they are more than meeting me halfway because they care.

THC - I will check that with my OT friend, I know it might be different in different PCT's but it is worth asking.

Sparkle so nice to hear of the difference school are making

Hi approaite

Were about to attend the GOSh rehab next month . But I am not impressed with then so far at all

Has Ds had assesmdng from OT about looking to see if needs supportive seating in school ? Which may then help .

That is a good point that THC has made, supportive seating goes a long way to helping with the fatigue, a good rest writing slant desk thingy and a special cushion made such a difference for my ds.

I know I am so lucky with the school, what a shame it is taking so long to undo the damage inflicted on him by the previous school/headteacher/seniormanagement team attitudes.

Thanks. This is the first time DS has said his TA said no to a break. I think he was asking to go out during classroom time and he has been allowed to do this up until now.

But I think there is always this 'drive' to say the child is in the classroom but I don't think this helps him.

I'm not sure what will really as he is so bendy, he just ends up twisted in a little knot on the floor.

Apart from anything esle your ds needs have to consistent message, is he allowed breaks or not. I think a visit to the classteacher, SENCo or head, whoever you think you will get the most support from is in order, then you can stress the importance of breaks to your ds' education and wellbeing...bigger picture and all that.

Ds3 has a breezi chair in class but with a pommel and pelvic supports , were also looking at adding a vest harness to keep him more upright

We have star chair at home . May be worth requesting seating assement

Were more fortunate as ds3 in class of 10 in speech unit so his teacher good and he is known to nap in class in book corner

Does your Ds have a statement as you can get it written in or an IEP ?

That's what I'm wondering about Gosh program as it seemed more aimed at hypermobility nit taking into account his other needs . Or in fact compared to your dc he does mange school better but struggles walking physically using his arms

The go program seems to bd centred around walking jot taking everything else into account . Am worried how ds3 will cope as though he I'd almost 7 he's more 4 add in speech issues and they expect me to leave him with them !!

THC I hadn't thought of that. I'm seeing the HV tomorrow and she is good at getting other proffessionals to do what she wants so I will get her on the case. It will also distract her from trying to get all 3 of my boys to the 50th centile for height and weight at least . DS2 did have a supportive chair at home but it looked like a highchair so of course it only lasted until someone told him it was a baby chair . After that he refused to sit in it and the OT said it was the only chair he could have. The p-pod seat looks really good.

DS2 has his school entry plan meeting on monday next week. What questions should I ask? What should I say he needs? I think tiredness is going to be a huge problem as he normally has a nap in the afternoon. One of the reception teachers (jobshare) currently teaches DS1 and whenever I mention it she just says he'll be fine in a really patronising way.

On a positive note DS2 and DS3's genetic appointment has come through for next month. We've been waiting for that since July.

Ellie - good news the genetic appt is finally through I will put my mind to possible questions to ask or things to raise tomorrow. Am dead tired now, and finally have all dc in bed, they have been too frisky this evening and have worn me down. Off the top of my head though issue could be tiredness, sitting support - carpet, seating, assembly times, lunch times, writing support writing slant, pencil grips, PE curriculum adaptation, emergency issues if dislocation, care plan (sometimes called health plan in school - could email you copy of ds' if helpful) regular meetings to assess success of strategies in place and make more changes or adaptations. Will think more about it tomorrow, I promise.

THC - you can't be expected to just leave him there, until you know if he is going to be comfortable with that. I think you will just have to swt your stall out about that when you get there, and tell them, you will leave when and only when you are confortable that he is comfortable and that they know and understand his specific needs. GOSH's ideas be damned.

yeah im going to be lol protective mum a tad .But yes no way will he mange with room full of starangers he does not fit their normal dc that come in

oo good luck on the gentic appontments

They are VERY strict about just leaving the children and make it very clear that they do not welcome your intervention, participation etc.

You will be dismissed as a fussy, overprotective mother.

But I stuck to my guns. DS has Asperger's and other needs and we needed to plan and visit the physio, school and activity centre. We did that and it worked well for him as he was much mroe settled when I had to go.

Their response - well maybe that will prove to you that you don't have to fuss so much next time

I was startled that they knew so little about other issues such as ASD. I think the whole experience was a bit of a waste of time. I am none the wiser about DS's difficulties, his level of need or how to manage it.

They clearly don't want to get involved in the whole school thing and their focus on the medical model of disability (it's all a matter of willpower) really neglects the significant social barriers are children face in accessing support.