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 size 5 nappy on the school run!

bit better today thanks just really tired. dd1 was really unwell this morning, exhausted and legs hurting a lot.She seems a bit better now.

Im hoping this baby isnt too big, dd1 was 9lb 10 ds was only 7 10 and dd2 was 2 and a half weeks early and 8lb so i dont think itll be a tiny baby but hopefully not bigger than my first !

cant do any swimming at the moment the reflection off the water from the lights triggers a migraine every time as im prone to them lately but warm baths seem to help my ankles.

hope everybody else ok today

I am glad you have tried warm baths. They are notoriously bad at predicting the sizes of babies in my own experience, so fingers crossed. Hope dd1 is ok when she gets up tomorrow.

dd1 woke up today with a really sore right hand and right foot i thinjk its her tendons as she went back to school thurs and has been doing a lot of writing and more walking around than usual, hopefully itll all settle down once shes used to school again, i know it was only a couple of weeks off but she was in bed quite a lot and didnt really write so i think she just needs to build herself up again.

hope everybody ok

Ds had a really bad neck yesterday, I think that was being back at school too. He did physio at school yesterday, and was a little better after that. He is still a bit tearful though, and a bit clingy to me....which is a puzzle. Everything else ok, except my chest joint thing has flared up, so I am in pain and didn't sleep much last night, but ds is ok today.

Hope everyone else is ok, fingers crossed for dd1 ariane.

hi

sirry have been awol a fair bit yes i moved 5 days before christma then chaos of christmas then we went away .

so everything been chaos .Dc back at school this week so am trying to get things straight

still no news from GOSH , am not impressed with them have chased and apprent;y they still not orted out inpatient appointments .

local physio have siad well as good as it gets now and taht they accpeted there be no more improvemnt

sparkle

how things on job front and sorry ds neck giving pain again though yes like you i thin being back in school and not being able go at their own pace does not help

Araine

hope your keeping ok and my dc , ds8,3 lot of water ,ds2 10,6 bit of water but 45 mins in total

dd well 5lb and crash section no water and ds3 6,3 csection but yes i remmber the tiredness especially when you have other dc

THC - Gosh have truned out to be a a real let down. Not good news from the local physio then.

DH has been offered a job internally, which is a demotion, but at least is a job. He has accepted it. Not sure how i feel about it really, but at least money will be coming in every month.

if GOSH aren't being proactive, is there anywhere else which is good? DD2 has an appointment at the Royal Brompton on Monday, and although the consultant we should be seeing is supposed to be fab, it would be handy to have some recommendations of where we should be looking ot be referred to (London/South East, am happy to travel a bit for the right person, after all the mucking around and ignoring DD has had from the local hospital)

A more general query, I see that lots of you/your DC have frequent dislocations. DD (because she faints so much) is bashing and spraining bits of herself which seem to cause more pain than you'd expect, or ot get better as quickly as expected. Currently she has a mildish sprained ankle (did it on Monday, still hobbling a bit) and a sprained thumb (did it Sunday, strapped up by hospital on MOnday, took strapping off Wedesday as instructed, but still couldn't move it yesterday, so got it restrapped up, ad will see a doctor on Tuesday). The nurse seemed very surprised that there wasn't an obvious break to match to the lack of movement and pain levels. Can soft tissue damage be worse with EDS?

Interesting re pain levels - DD seems to be the other way with rating pain very high on the 1-10 scale when perhaps she should toughen up a bit more. Is that worth mentioning, do you think? Or is it likely to muddy the waters? I also need a management plan for when paramedics are getting stroppy because they don't see why they've been called (eg lots of back and neck pain - school tends to err on the side of caution and call for help) - their recent attitude has been that I should somehow know if it's serious or not, despite having no medical training. So now we're actively trying to avoid A&E, but I'm scared I'm going to miss something, and cause more damage by not getting checked out. Difficult call really.

(sorry, may have rambled a bit there ). Hope everything going OK for everyone?

Midnight

GOSH are seen as the gold standard on eds hypermobility etc but think because ds3 does not fit a recognisable model .Brompton are fab [ grin] dd is under them for a separate issue and nothing but praise for them

Feeling we may be in same area or are you a namechanger

They dismiss him We had a fight enough to get him seen by them As there is probably something else lurking in his Gentic make up

Not sure if I can be fussed to push it anymore I chased this up 5 times now think it's June when he has another clinic appointment so will just leave things till then

Locally they feel that it is as good as it going to get now 275m is his limits and that's with odd gait and is very slow 6-7 seems to be the cut off line And because he does not fit a nice little tick box

Pain wise intresting big things he hardly ever complains about But small things like a graze can make him cry a lot

Sparkle sorry on the job front pay cut but glad he has something for now , is Dh looking around ?

Humancatapult - I'm in Surrey (local hospital is East Surrey in Redhill), was ShrinkingViolet for a while, so some of my rants are under that name. The Brompton have been (mainly) fantastic for DD (if you ignore the losing onward referral letters for a few weeks), and the nurse specialist is incredibly helpful and reassuring. I'll see who they recommend for us.

Midnight - Prof Grahame discussed the evidence related to increased sensitivity in pain receptors in EDS, so dd2 is not making a fuss, it really hurts that much. I can link you to some related articles if you like.

Soft tissue damage can seem worse in eds, in terms of what the nurse was referring to for your dd2. In a "normal child" to have the level of pain your dd experienced it would probably have meant a break in the bones, but the narure of eds makes breaks in joints etc less likey and soft tissue damage more likely as the soft tissue does not hold the joint as securely as it should, and it is too elasticated anyway, so stretches too far and therefore gets damaged.

I too have been through the discussion with school and a&e and consultants about the balance of when to call and ambulance. The new school my ds is at are much better at making judgement calls, and will ring me if in any doubt, but it has been a long process of learning for them. Schools are right not err on the side of caution, as they are not medical professionals. Imyself have now seen so much with ds that I am probably too blaise and deal with things when I should hospitalise him...in short, I think I would always rather be on the side of caution, and un-necessary visits to hospital than run the risk of any compliactions and permenant damage.

GOSH have been a bit pants for THC and I can't get to see them, and so I might try going direct to Prof Grahames clinic in UCHL. He did see my ds there years ago and dx him with eds. Having said that Prof G has not been exactly helpful with THC dc either so.....[peed off emoticon needed]

THC - dh is looking, but at least from the position of having a job. Got a bad feeling about today, with the icy paths, very unsettled, so roll on hometime.

Sparkle, thanks for that, v. interesting. I'll mention it to tomorrow's doctor when they look at her thumb.

The doctor today at the Brompton (saw another registrar rather than the consultant herself ) wasn't especially helpful - she sees DC with much much worse EDS than how DD2 is, so wasn't really interested, but said we should ask our paed if they want to refer. Did get the name of a possible new paed at our hospital though, and I think I may see about getting everything switched to him, as he does know what he's talking about with the fainting, then see what he says about the bendiness.

Ds's consultant has said before now that he has other children whos flexibility is more pronounced than ds' but not anyone who is more proundly affected by it than ds, in terms of pain, dislocations, subluxes, affects on groos motor skills etc. The problem is if you get a registrar or consultant who just loks at the beighton or brighton scales, they miss the point and the bigger pitcure. A paed who is thoughtful and listens and is supportive can be just as good I am sure, and they will "send out" for specialised help in other medical areas. You do know that low blood pressure and fast pulse rate, fainting etc is another symptom of eds don't you?

yes, that's how I ended up looking at EDS - through the link with PoTS. But because she's still a child, we can't go to a specialist autonomic clinic, and are being shuffled off into cardiology ("because it's to do with blood" ). So they are coming from a heart point of view, and focusing on what they can do to stop/minimise the faints, they're not especially interested in what else might be going on that the fainting could be a symptom of, rather than just a thing in itself. Brompton Dr said we could ask GP/Paed to refer to a geneticist - would we want that or a rheumatologist in the first instance? Other than the fainting, she's not especially bothered with any actual symptoms, so not sure if it's worth pushing so we know one way or the other. I ca see it would be useful to have a diagnosis for the future (eg during pregnancy), but unless it's affecting her day to day life, then presumably it's not a problem?

But at least the fainting is being dealt with, and we're not just being told "go home and sit on the sofa" anymore...

Her thumb is a partially torn ligament according to today's doctor, thankfully it's not a break, and will heal in 4-6 weeks which is good.

Both ds and I saw a rheumatologist first, but some on here saw a geneticist first, so it might depend on how your gp or paed feels about it. I have never seen geneticist, all my genetics based questions were answered by the rheumatologist. In all fairness eds hypermobile type can not be dx by genetic test yet anyway, but some of the others types can..... Do you want a link about this different types, it might give you and idea on who to push for first. I would always do for a clear dx, then you are all set if things appear later, and it is important to know for painrelief and anaesthesia requirements too, but that is just me, and I like (anal response) need to know what I am dealing with.

we have had sucha bad week,dd1 tearful, tired and in pain.Getting her up in mornings nearly impossible now. Had physio appt on tues, she is so weak and tired they want her to go in every week for couiple of hours of physio. I have no idea what has caused this 'flare up' but she is in a terrible state

ds ok, getting new orthotics next week so hopefully itll help with his leg pain.

I am exhausted, have got horrible horrible spd but iam looking forward to a lie in tomorrow!!

hope everybody else is ok.

Hi everyone

Sorry for the long post!

I was just wanting to ask some advice, if that's ok? I'm fairly new to hypermobility. My dd aged 13 is under assessment for Aspergers and we think has co morbids of dyspraxia/SPD/odd and seperation anxiety. She had an abc assessment done by the rubbish OT service we have here and it showed significant impairment in manipulation and ball skills. They never checked for hypermobility or low muscle tone and discharged her as offer no follow up services.

I've managed to get her an appointment with the physio for a full assessment at the end of Feb as she is reluctant to write, refuses to walk and regularly complains of pain in her hands, arms and knees. Her CAMHS worker used to be an OT and he believes she has low muscle tone. I'm wondering if she could be hypermobile. She doesn't look obviously over bendy but she does have severe flat feet (no arch even when sitting) and over pronated ankles. Does this sound possible?

Because of everything I've learned over the past 18 months we've been going through the diagnosis process I suspected that my 4 year old DS may be hypermobile. I managed to get him referred to the CDC with him being under 5 and because of my daughters difficulties and my concerns about his fine motor skills along with his phonological speech disorder. He went for his observation visits and they have this week confirmed that he is extremely hypermobile. The OT said that in 16 years of being an OT she has only seen 2 other children with thumbs as mobile as his. She says that when his pen grip is corrected to tripod grip that his thumb is popping in and out so mustn't write that way and to let him hold it his way. She didn't know wether to try thumb splints with him so has said she would like somebody with more knowledge than her to see him. She has referred to orthopaedics. Is that the right dept? From what I've read I thought it would be rheumatology. She wasn't sure if they'd see him but wanted to try.

It turns out my sister is also hypermobile! After reading up on it I knew she was. She had an appointment with her GP the day after they told me about my DS because she has unexplained pain in her toes, back, knees and hips and I told her to mention it. He did the beighton test and she scored full marks! He told her that nothing can be done apart from exercise and pain killers and that she'll be good at dancing and gymnastics! That's useful at 29 years old! Is that right that there's nothing else?

I suspect that both me and my other sister also have some hypermobile joints and also my Mum! It's rather mind blowing!

Sorry for the waffling and I hope it makes sense!

hi

we was someone that went to Gentics first to look at other condtions and tehn got referal to pro pope for the EDs who confirmed yes and then referal to gosh

YAy taht was lucky I called and inally got through ti Sue Mallard , after several attemptes and answer phone messages .Pit her on the spot and she admitted that he is getting near the top of lit but shes not started sorting out ( but he has been near the top for ages)

It may be around April time she cant tell me yet as not sorted , like I can drop everything for 2 weeks and sort out childcare for my other dc and i was told accomidatiod need couple months warnoing to makke sure have wheelchair access for me and i can access the kitchen etc

, >She had cheeK to be snotty as i warned her were away first week of April

Im really disillunsioned with GOSH but nowhere else to go just maybe he be seen before we go back in June ( was first seen in August menat to be seen 6 months after .His local physio changed now and the other one not likely to call so we been left out to dry again

araine thin its time of year plus school is wearing them all out and hopeing your getting plenty of rest

I would reccomend proffeor Pope for Dc he was lovely on dx and gave me and dd eds3 and Ds3 eds type 6 which at least for me gives me clearer idea

Sparkle dc still enjoying the nw school?

Hi Everyone

Sazale - ankles that are over extending in the way that you describe sound hypermobile to me. It does sound like you have quite a strong family history. Sorry your sis's gp hasn't been more supportive, but it is the way of things unfortuntely, and to be honest she seems to have been lucky so far, to have not had repeated dislocations etc. I don't mean that to sound unsympathetic towards your sis at all, not in the slightest, eds/hypermobility can be depressing condition, especially as there is so little energy on behalf of the medical profession to be proactive about it. As far as your dc are concerned my ds' orthopaedic surgeon is only involved because he has spinal hypermobility, I have never had an ortho for me, rheumies do everything for us. Having said that each hospital and pct is different, you may have an ortho who is interseted in connective tissue disorders....go with the flow to begin with and then ask for a referral to a rheumie if needed.

Ariane - this is a dreadful time of year for joints isn't it...damp cool if not cold. It always makes my flare up. I do hope your dd is feeling better after the weekend, and that the orthotics have come through for ds.

THC - I am sooooo disappointed with GOSH. People rave about this hospital, but it seems to be just like the rest of the NHS, filled with medical professionals who have forgotten that patients are people who are wading through the system like it is treacle, and somtimes life has to be taken into account...you know like child care, wheel chairs..... gah!

Ds is loving school, but something weird is going on again with his feet, and I wanted your thoughts THC. On sat he was screaming in pain because his feet were so painful. I got his shoes checked oout on sat, and they were still fine. On sunday his feet were so painful he couldn't even bear socks on, he wore nothing on his feet all day. His little toes went cerise pink with blue/purple patches on them. I tried to get him to see the gp on mon, could only see the senior nurse who was concerned and discolouration still being visible. She desctibed it to the gp, who wants to see him, but two days later I am still waiting to hear from her. What do you think, more evidence of possible Reynaulds?

Thanks Sparkle

You didn't sound unsympathetic at all chuck. My sis had numerous daily dislocations as a child but not as an adult. She has all the symptoms of narcolepsy but the sleep study confused the experts (she has sleep paralysis and hallucinations amongst other things)l it would be interesting to research to see if there could be any way the hypermobility could be connected to her difficulties. When I get a min I will do more research. Any particular sites the best?

Right...may be we are at the bottom of this. The gp thinks that eds is making the bones in ds' feet more hypermobile, and causing him pain, as his arches are not supported enough, and have caused him to damage his little toe joints on both sides, which are now inflamed and bleeding internally (aka a deep bruise). So he needs time to recover from the acute injury, then orthotics to support his arches. I am feeling dreadful, he has always only ever worn the most expensive fitted shoes, and they have still damaged his feet, despite me hacving taken him on Saturday and been told his school shoes fitted him perfectly. Rather than waiting for NHS orthotics, the gp suggested and I have already done, I have ordered some paed arch insoles, then is they fit, off the thr shoe shop to be refitted with shoes which fit him and the insoles....! Poor kid, no wonder he was crying in pain on saturday.

Sazale - here are some links, some old faves, some new ones which look useful on first skim.

www.ednf.org/

www.ehlers-danlos.org/

www.bbc.co.uk/health/physical_health/conditions/ehlers1.shtml

www.medicinenet.com/ehlers-danlos_syndrome/article.htm

www.hypermobility.org/

www.benefitsnow.co.uk/handbook/hypermobility.asp

www.medicinenet.com/hypermobility_syndrome/article.htm

Ooo I am a bit cross now, just stumbled on a website, written by a physio, who says a child who is hypermobile and has pain, has the pain because they are "cautious in nature" which makes them less likely to exercise properly, and therefore have weak muscles....and she can cure it all by giving a structured strength building programme, so your child may never have pain again.

Oh clearly all drs have been wrong about my very active couch potato child, who clearly just needs to get out more, and that will make everything get better..... with her programme we wouldn't need to do the hours of physio we do a week, just to attempt to build a bit more stability.....

Anyone want to help me hold her down and force her to walk in our kids shoes for a day.????

Sorry, as you were...

Sparkle, I would have been cross too seeing that. Maybe she can help children who are a bit bendy but not children like ours. I'll help you hold her down.

Does anyone know why some people are just a bit hypermobile and some people have full on elhers danlos and are they genetically linked. Just wondering as my sister, auntie and uncle can hyperextend certain joints, dh and I have a lot of elhers danlos symptoms but don't quite score high enough on the beighton scale. DS1 is slightly bendy, DS2 and DS3 have either got elhers danlos with a couple of other things or they have some rare syndrome that combines everything. I've also miscarried 2 babies although not sure if what they had was linked to EDS or not. The paed told me yesterday that we might never get an answer and I'm finding it so frustrating.