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.

Time for a new gp grtb4! Alternatively take these articles and shove away!

www.mayoclinic.com/print/ehlers-danlos-syndrome/DS00706/DSECTION=all&METHOD=print

www.ehlersdanlosnetwork.org/causes-symptoms.html

www.ehlersdanlosnetwork.org/typesofehlersdanlos.html

www.ehlers-danlos.org/

www.ehlers-danlos.org/index.php?option=com_content&task=view&id=15&Itemid=10 - some extra reading for her!

www.wrongdiagnosis.com/e/ehlers_danlos_syndrome/symptoms.htm

www.cincinnatichildrens.org/health/heart-encyclopedia/disease/syndrome/eds.htm

emj.bmj.com/content/25/3/175.abstract - article about avoiding too many xrays

inthefringes.wordpress.com/eds/ - a personal account but i think very useful!

I would contact your primary care trust and enquire how you lodge a complaint, and get referred without going via the gp. It is ridiculous. I am about to write letter to Rheumatologist about last Friday's debacle.....I am still incensed!!!!

Ds still has a chair for lunch times. I spoke to the Head about it again today, he told me he rollocked staff over it, saying we may have a lack of common sense in this school, but the one thing we do not lack is enough chairs! - Brought a smile to my face! Have to update his risk assessment again post Friday incident, poor kid is as gumpy as hell not surprisingly.

glad the head sorted it .And sadly changing gp/surgery not an option its the only one that serves my village .Where you cant be ill less give 2 weeks warning

Is a star chair the cardboard one? DS2's breezi chair is fine, it's the buggy that he hates. We have got an assessment booked for january at wheelchair services so fingers crossed they will be able to get us something more suitable.

I didn't realise EDS could involve co-ordination problems. DS1 isn't technically EDS I don't think as he only has 6 hypermobile joints but his co-ordination is awful. The school secretary knows him very well now as he seems to spend most of his time in her office with a wet paper towel on his head. He also seems to spend a lot of time being signed in late because the buses have gone haywire because of the weather. It's 1.5 miles so we could walk but I can't see either DS1 or I making it that far without falling on the ice.

no its more padded than the brezzi one , goes up and down with a lever at the back which we find handy depending if he is sat at the table eating or wants to sit at small table to scribble

I had a phone call from ds's consultant rheumatologist last night, after I sent her my letter describing Friday's dreadful care for ds. She is carrying out an investigation with the Head of paediatric a&e, she is also going to chase the ortho referral.....again. We will see.

Dh and I had to go into school yesterday to complain there too, it turns out a member of the senior management team ridiculed (sp?)ds' disability and special needs to a grandparent of another child. This place is beyond me, I respect the new head, and the staff who directly care for ds at the moment are second to none, but the attitude of other staff is unbelieveable. We have given school a certain amount of time to investigate and decide and implement how they are going to change attitudes in school, after that if we are not satisfied we have told them that we shall remove the children, I will resign as a fairly high profile governor....we will see again.

I am so fed up of having to fight every fight, for just reasonable care, and i am just so tired.

biopsy went ok by the way, painful and bruised but that it nothing new to our dc is it!? Have to wait for the results now.

sparkle thats terrible i hope they take the complaint very seriously,how could anybody be so horrible to ridicule a sick child

They say they have, but actions speak louder than words. I saw the teacher concerned on Friday, it took all my will power not to go and shout at her in front of her class.

Anyway, how are you all doing Ariane?

i really cant believe anybody being so cruel if it was me i prob would have gone and shouted at her what a nasty piece of work she must be. its the last thing you need what with everything you have to deal with at moment.

we are all in bit of a sorry state, have spent the last week all terribly unwell, the baby got croup which then turned to a throat infection and she has got a hernia from coughing so much,ds has been very unwell for last 4 days, massive temp and awful sore throat and cough he is now on antibiotics 4 times a day,iam sitting here with the most terrible earache,swollen neck and horrendous sore throat,my voice has completely gone and i can only manage liquids no real food so am feeling weak and really sorry for myself! dd has been staying at my mums in the hope she avoids whatever we have, my ex came round at weekend to help with ds and has now got it so not sure if he will be ok for his knee op on thurs.

im just hoping and praying this is the last illness we get for a while as we are all completely run down and so tired!

Ariane - I hope you are doing better. All my dc been down with the a horrid virus which sounds exactly like the same one you have all had. I have managed to avoid it myself so far.....fingers crossed.

I'd like to echo squintytiger's post further up the thread. I had fainting fits for years and years and, finally, a fantastic SMO referred me to Prof Matthias (who diagnosed POTS) who then referred me on to Prof Grahame (who diagnised EDS III). I've been on Midodrine since 2006 and it has changed my life as well. I got my driving licence back and I could do things with my children without the fear of fainting and dropping/hurting them.

I had horrible pregnancies and multiple miscarriages but have since managed to carry three children to term.

My middle child has a diagnosis of EDS III and I can only say that the care I have had has been amazing. My GP and local Trust have been brilliant and I have had loads of physio and other therapies that have made a real difference to our lives.

Ariane

Sounds like the flu bug that is doing the rounds touch wood we have advoided it to now.

Sparkle any news on your tests yet

as for here i ahve given up the community paed has decided to sit on the request for the referal she wants to see what else can be acheived aa wait and see game .Think she wants to wait for genetics throw up so that be at least March

Unfortunatley when we saw Genetics he told me yes Edstype 3 but he then put in his letter that in his opions yes Eds is a possiablity that does not answer for everything else.So Paed now wants to wait as seen that as well it might not be

Have given up arguing after the Gp said well i seen a nt child dislocate and there in lot of pain so ds would scream if his joint went .Then she admitted same breath shes never seen anyone with hypermoblity dislocate

Am sick to death of fighting them

You have had to fight so much grtb4, if you had the strength left in you, I would write to the genetist who said to you yes EDS3, and then wrote a wishy washy response to the paed. Ask if he/she changed his/her mind since they saw you, explain their lack of a definitive answer has stalled any support being given for your child and it is about time that somebody other than his mother, took responsibility for leading the care of this child. I know you have been through so much and I completely understand being sick of fighting them, I will help you write a letter if you want. I need something to distract me, I have been called back to the breast clinic early following my biopsy results.....so don't want to think too much about me. If I can help then use me, please!

My ds is being challenging, and would appreciate your thoughts everyone, but have to rush off now, will post on this subject later. Hope you are all surviving the snow.

GRTB4 we have had this. Neuro pead said yes it's EDS3 but then said he prefers to call it benign congenital joint hypermobility so parents don't get confused and think their dc has one of the more serious types of EDS. That star chair sounds really good. I wanted a panda chair for DS2 because it goes up and down but OT said it was too supportive so we got the breezi chair. We've changed OT since then and the new one seems to be a bit more free with the cash (although on the downside she is fairly clueless) so I'll ask her at DS2's next appointment.

May be it is just me, I am very tired and a bit grumpy at the moment, but I find it unbelievably arrogant for any medical professional to say yes they have eds3, but I will make up my own name for it so parents don't get confused with other types of eds. How about he does his job properly and explains about it not being the other types of eds. It limits the information parents can get, it limits other professionals understanding of the patient as we know that information sharing between medical professionals is poor at best, and if the child starts with other less common symptoms of eds3 it makes it harder to get them dx. Ellie, I am glad you have a clear dx, sorry to rant, but there are many parents/patients out there who don't for reasons like this, it makes me so cross. Sorry I said I was a bit grumpy at the moment.

I wasn't happy about that either. DH has Aspergers so he misinterprets things at the best of times so a basic answer would have been useful. Have you had your breast clinic appointment yet?

I have some sympathy for the medical professionals at the outer reaches of EDS3. When I read peoples' experiences on here, I sometimes think - oh, yes, I find something similar to that (eg finding it amazing how people can stand still in queues without feeling faint and needing to squat down to avoid passing out; having totally flat feet; or having very thin, fragile gums; or having a family tendency to osteoporosis and earlier than average onset of osteo-arthritis; or having rather pale skin with the veins apparently very close to the surface; or being able to do interesting party tricks with hypermobile joints). But then I read about the extreme pain and dislocations and that doesn't relate at all to my own experience to date, yet this is supposed to be the most significant and debilitating aspect of the condition (and why I haven't ever pursued a diagnosis! I don't, after all, have any symptoms that I can't easily deal with as being just part of the way I am made up, ie a variation of an acceptable norm).

My ds1 also, despite having significantly more physical issues and a diagnosis of EDS3, also doesn't dislocate so far as I'm aware, despite being alarmingly loose jointed (although I think he may subluxate in some joints and doesn't even attempt some things that probably would result in a dislocation!). I can't help feeling that ds1 would be like me (ie wouldn't need a diagnosis) if it weren't for the fact he also has AS, so is also hypotonic as a result of his neurology, which makes his hypermobility seem worse and provokes a tendency to other symptoms he might not otherwise have (eg bedwetting). Or is the hypotonia a symptom of the EDS3 being worse in his case, rather than a result of the AS???? Where does one condition end and another begin? I'm quite certain there would be different approaches to ds1's bladder problems, depending on whether the main causative factor was believed to be his hypermobility (and possibly over-stretchy bladder) or his unusual neurological profile as a result of his AS...

In other words, I think there is more than a simple case of weak connective tissue going on in many diagnoses of EDS3, which makes the list of potential symptoms suffered as a result of EDS3 rather long and unpredictable, as it depends partly on what is also going on at a neurological level. And since when has the medical profession ever been any good at dealing with conditions that overlap many areas of medicine?

With EDS you can 'cross-over', have one type but with many signs and symptoms of another. My son has severe EDS3 with vascular cross over being investigated. It isn't a simple 'this is what you've got, and everyone who has this is the same' condition, as we who have, or have children with this know all too well. My son had dreadful treatment at his school, with his physical condition and ability being made fun of in front of his peers, and their parents. At one point he was hauled out of a chair, only to have his shoulder slip, resulting in a call to me and a 'don't know how this happened' conversation. They hadn't banked on him telling me! That's why I Home Ed, I couldn't send him there anymore, the way they treated him was appaling.

Can I just ask if anyone is having problems with more frequent subluxations, or dislocations in this freezing weather. We wrap him up in thermal leggings and long sleeves, but for the past 3 weeks he has been in the most dreadful pain during the night, with popping and crunching of knees and ankles, only easing once the sockets have put themselves back in again. He gets restless legs, and is constantly moving them trying to get comfortable. Some nights we get very little sleep, making the following days harder to cope with, and then the cycle is hard to break.
There may already be an answer to this here, if there is - sorry, I try to read through as much as I can, but haven't had the chance of late. Merry Christmas to everyone and their families xx

I get more severe pain and click and grind more in extreme weather conditions, eg. very hot and very cold, for my ds the most significant factor for him is growth spurts which leave him crippled with pain, subluxations and dislocations. I haven't noticed any weather effect for him yet, but to be honest he can't go out in this weather really too much danger of falling with his unstable neck vertebrae. The restless legs is a very familiar thing for us, restless body too constantly adjusting it.
There something called overlap disease where connective tissue disorders cross over, does just have to be with eds, but can include other connective tissue diseases/syndromes. Mycars that is shocking behaviour from the school I am not surprised that you home educate, I have thought about doing that myself.

News on my biopsy is that the growth is benign, I have to have it cut out some time in january, in case it becomes malignant. They think I have one in the other breast too, but they can't find it

Hope you are all having a Merry Christmas and wishing you a very Happy New Year.

Hi hope everyone had a good holiday

I'm sitting on the fence ATM I certainly shall chase it up ,waiting on genetics in March to see if chromsome abnormality

physio/Ot now on side infact physio is coming to next TAC meeting to adress a few comments made by sw etc that don't even know ds.They kept on saying ds does not need wheelchair at all and they want it gone but physios coming and says in her oriffesional opion as someone who works closely with ds he does need it and will continue to need it his physical problems are very real confirmed his hips/joints knees are severley affected , not me making him lazy

also there coming to carry out assement as ds can't get in the school taxi has to be lifted in.That way they can see if way to solve it also ds has Sn trike of another forum and there going to come look at adaptions for it to

been a whole change in their attitudes which id fantastic .Though we do have paed appontment next week what should be his first day back at school then tac meeting next day so be 10 th before I get s break :(

Sparke I'm so glad it's benign .Hope everyone else us coping ok .Ds was fine in snow but ice us a no no for him

painwise cold us ok it's the damp that does it for him

happy new year everybody.

glad its benign sparkle

Happy New Year everyone.

grtb4 - it almost feels like you might get somewhere with the physio and ot on side, but I don't want to jinx it, so I will cross my fingers quietly.

Got a letter yesterday telling me a doctor is coming to examine ds for the dla, today.....nothing like short notice eh! I have tried to speak to the organisation which is sending the dr to find out his experience and to make sure he doesn't say inappropriate things to ds, but so far I have got the assurance that he is a medical doctor (YEP I should hope so too!) I have asked ds's class teacher to come to the house for the appointment and she has agreed, so she can give school's point of view of ds's needs. Ds has had a bad nigh for pain, and has needed two high doses of pain meds and his collar to get him into school, I would lay odds he won't make it to the end of the day, so we shall see what happens.

Hope everyone is well.

Hi sparkle how had dla medical gone?

Hope everybody is doing ok