Tuesday, 24 January 2012

Living with a child with HMS

My Son Hadleigh Evans Age 7                                                                                          
Joint Hypermobility / Type 3 Ehlers Danlos Syndrome describes the joint laxity that results from connective tissue problems, which causes joints, muscles, tendons and ligaments to be laxer and more fragile than is the case for most people. With this comes vulnerability to the effects of injury as well as pain, discomfort and exhaustion. Its invisible so is often mistaken for laziness. The increased compliance (laxity) in the joints and the tendency of poor muscle strength and endurance affects a childs movement skills in numerous ways. The disorder affects 1 in 10,000 to 15,000.  More awareness and understanding is desperately needed.

Here are some  of the symptoms, you may have some or all, Hadleigh has all:

A characteristic posture in standing with knees bent backwards and a slightly protuberant abdomen.
Child may not like standing erect and always something or someone to lean on.
Chronic Fatigue.
Poor balance and agility skills - due to weakness and poor development of coordination.
Slumped sitting posture.
Fallen Arches (Flat feet).
Bruises easily.
Back and/or joint pain.
Fidgeting - usually to relieve numbness & pain.
Sit between the legs (W-sitting) or sitting propped up on one knee.
Poor endurance - tire very quickly, find school day very tiring.
Lack of strength and endurance for many childhood activities, such as climbing on the jungle gym, riding scooters, playing ball games, running and keeping up in the playground.
May have pain in the legs when walking or at night - this is usually due to the tightness in the muscles.
If the hypermobility is associated with a very cautious nature, the child will also avoid any activities that require mental or physical effort .
Although the joints are hypermobile, the child may have some quite tight muscles which are often the source of discomfort in sitting and standing.
Child may walk on the toes, and have difficulty walking with a normal heel strike.
High and Narrow Palate resulting in dental overcrowding.
Porcelain skin.

Some interesting facts about Hypermobility Syndrome/Type 3 Ehlers-Danos Syndrome
Actress Cherylee Houston has type 3 EDS and uses a wheelchair; she made history by becoming Coronation Street's first full-time disabled actress.

The condition may have contributed to the virtuoso violinist Niccolò Paganini's skill as he was able to play wider fingerings than the normal violinist

Musician Noah Baerman has been a long-time supporter of EDS research, selling his first CD, Patch Kit, to benefit the Ehlers–Danlos National Foundation.

The condition is mentioned in the song "Dorsal Horn Concerto" by the British comedy band the Amateur Transplants.

Ehlers–Danlos-like syndromes have been shown to be hereditary in Himalayan cats, some domestic shorthair cats, and in certain breeds of cattle. It is seen as a sporadic condition in domestic dogs.

My Story
After a very traumatic birth, my son Hadleigh was born on 7 January 2005 and he was perfect.  I soon settled into Motherhood and I loved it.  Hadleigh was a good baby. As time went on, other babies seemed to be sitting up at his age (around 6months), but he wasn’t. I just put it down to the fact he had a baby rocker seat and it was because I had put him in that. At around 11 months he could sit up, but only for a short amount of time, then he would just flop back.  We always made sure there were cushions behind him because there was never any warning he would just suddenly go. I can remember sitting opening christmas presents with him whilst panicking he would flop back, which of course he did.  By the time he was 15 months he still couldn’t crawl.  He used to roll around the floor to get where he wanted and then pull himself to a sitting position by an item of furniture, I trusted him a little more with holding himself up, but even at this age he occasionally fell backwards.  When he was 18 months he used to scoot along the floor on his bottom!  He got to his 2nd birthday and the Health Visitor decided we should visit the CDC (Child Development Centre).  There, after quite a lot of various appointments and tests etc they diagnosed him with Joint Hypermobility/Ehlers-Danos Syndrome.  We were advised we would need to see a Physiotherapist and an Occupational Therapist, and so over the next few months we did.  He would wake 3-4 times a week during the night screaming in agony with either leg pain, especially in his knees, or wrist pain. After using Calpol for sometime i realised this wasn't helping Hadleigh at all so i tried Nurofen Suspension and massaged the painful area until it went, I would feel him slowly relax, its like his joints were totally stiff and locked. These night aches still occur today only much less frequently.
He was still having 3 sleeps a day and sleeping through the night (other than the night ache waking). At age 2 and half, Hadleigh took his first step, it was a very wobbly one, but it was a step nonetheless!! I cried with joy of course.  From that moment on he started to take a few more, very slowly but surely. By the time he was 3 he could walk. At this point I was constantly scared because at any given moment, Hadleigh would just fall straight over.  For the next year, every time he fell over he would smash either his face or his head, his wrists were not strong enough to support the fall.  The slightest knock or nudge and he would be over.  He still had a buggy and would go onto have one to this day. I put him into swimming lessons at this age as this was very good for Hadleigh’s joints and he excelled at it!  He was down to two sleeps a day at this age.  Once he reached 4 years old, he started in Reception (full time school) I was worried about him.  Up until he started he still had one sleep during the daytime.  His teacher was amazing.  He did drop off to sleep, quite often.  She didn’t make a big issue of it, she did all she could to help him, for example he was allowed an afternoon snack to try to keep him going.  He was given inner soles for his shoes to wear all the time, this was to help him to stop locking his knees backwards.  The Occupational Therapist recommended a larger buggy, which arrived straight away, a sloping writing desk, a pencil grip and a sit and move cushion at school to help him sit up rather than leaning all over the desk, which would then in turn encourage him to not fall asleep and be able to write better, sadly these weren‘t provided.  Unfortunately, when he was 5 he moved up to Year 1 and the teacher wasn’t anywhere near as supportive of Hadleigh’s diagnosis.  She constantly complained about him falling asleep, which by this time he would suddenly just go, no warning, even if he was standing.  The desk and cushion had still not been supplied, even though I had offered to purchase them myself.  She constantly called me in and complained about his behaviour and really did not understand him at all.  Many people just saw Hadleigh as lazy, including some friends and family! This part of Hypermobility is extremely difficult for a parent to deal with, especially one on their own as i am, you end up feeling like your 'making a fuss about nothing' and if your not careful you start questioning yourself, as i did!  During all of this time we were back and forth to Physio and the CDC.  The Physio were really pleased with his progress.  The innersoles seemed to be working but he was given some Heel Blocks to go underneath the original innersoles as well.  Finally after leaving Year 1 with a report saying remarks like ‘Insolent’ and ‘Disrespectful’ Hadleigh moved up to year 2 at age 6.  Wow what a difference a teacher can make!! He is doing fantastic, good reports, excellent reading and writing and really enjoying his school day.  I put this down to the fact he started off this year with his sloping desk (1 year late!) his sit and move cushion (also 1 year late) and a fantastic fully understanding teacher! And to top it all he has only fallen asleep a handful of times, one time during African Drumming! He is also in the very early stages of Piano lessons, something which his teacher also picked up on as he is able to extend his fingers over the keys already!  He still wears his two sets of inner soles.  He gets extremely tired and is always in bed by 7pm and has at least 12 hours sleep per night and the odd night with painful aches but we have just learnt to cope with it. I was lucky enough to have help purchasing a beautiful orthopaedic mattress last year, his has helped no end.  To top it all he finally learnt to ride his bike last summer! It was a proud moment and I had another tear.  He is a wonderful lad and very grown up. Everyone he meets is taken in by his lovely nature and of course his gorgeous looks, he has also learnt that bending his fingers back wows his peers although I don’t really think it’s a good idea to be doing that!

Hypermobility on Holiday
Going on holiday was always difficult for us and in then end it was much easier to have two long weekends, one at the beginning and one at the end of summer, we found a whole week just to tiring for both Hadleigh and myself, although very daringly we are going for a whole week this year!  We enjoy holidaying in the UK, mainly at caravan sites that offer onsite entertainment.  I first took Hadleigh when he was 18months old.  He thoroughly enjoyed it! He has always been a very forward, open child and he could talk perfectly clearly just over 1 years of age.  We would go down to the Clubhouse much earlier than everyone else so we could get a place near the front with somewhere to park his buggy out of the way.  He would scoot himself along the dance floor, which was great until it got busy, at this point I had to remove him because he just got trampled on by all the other excited children.  By 7pm when all the other children of his age were starting to enjoy the entertainment, Hadleigh was ready for his buggy and often missed the lovely characters that come on and dance and sing on stage and then meet all the children.  He would sleep through the entire show! We always made sure we got a caravan near to the clubhouse to save the trek back!

We went every year after that.  Each year he would stay awake a little longer but never past 9pm which is what time he stayed awake until last year.  He adored the characters and met and hugged them all. These Caravan sites are perfect for a youngster with Joint Hypermobility or any other mobility disability because absolutely everything you need is right there.  They have an onsite mini supermarket, Hairdresser, Launderette, Restaurant, Fish and Chip shop, pub, Daytime Entertainment including arcades and games, a lovely park, bouncy castles, trampolines, mini cars and alike, swimming pools and live daytime and evening entertainment. They have different stuff on each day ranging from picnics with the characters to art making and creating. They also have sports activities like football, golf, basketball, swimming lessons and even  rock climbing, we haven’t ventured into a lot of these yet as Hadleigh isn’t ready but when he is we will be! There really is something for all the family and you can even bring your dog! There are staff around all day long so if you have any needs or requirements they go out of their way to help you with whatever it is and are always friendly and smiling and very approachable.   There is 24hr security on the whole site and if you need anything during the evening hours these guys will bring it to you!  The Caravans are like a mini home from home, they have always been very clean and well equipped.  Anything that isn’t in the caravan you just call the number and they bring it to your door.  Each caravan has steps with rails but you can request additional support.  You can also hire just about any item you can think of including a wheelchair. The ease of access around the park is fantastic.  Every building has a slope to the door for ease of access on wheels and all around the park is a 10mph speed limit.  From the moment we arrive its fun and friendly and whenever its time to leave we are always upset, even though we are completely shattered.  The parks are nearly always situated on a beach so there is an access point from the park straight onto the beach.  They are also normally never very far from the main town centre.  The site we went to last year is situated right next to the beach and a little train comes every half hour and drives you into town or there are buses as well.  In the town there are even more activities such as fair rides, huge arcades, horse and kart rides, donkey rides, circus, model village, wax works and much much more as well as lots of great seaside shops and top high street stores too! I love it and I would recommend it to any family especially those with a child with a disability, they would love it and so would you!!

Im hoping by creating this blog I can help raise more awareness of this Syndrome.  Its a little more than growing pains and laziness and it really is about time it was widely recognised. If you would like to add something then please feel free and if i can help in anyway then i will.

     Hadleigh's sit and move Cushion and Sloping writing desk

                                   Hadleighs Inner Soles and Blocks

Below are some really useful links that may well be of interest to you.  Should you contact any of the links please mention where you found them.  Thank you

One ladies story about life with EDS

A guide to Disabled Holidays

The Hypermobility Syndrome Association

Ehlers Danlos National Foundation