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Audiology - an unexpected update

Joint hypermobility & achondroplasia


One of the most common traits of achondroplasia, apart from the distinctive shorter length of the long limbs and consequently shorter height, is joint hypermobility. Joint hypermobility (also referred to as benign joint hypermobility) means that your joints are looser and more flexible than the average person's. Many may know the term "double jointed" which refers to the same thing. 


Joint hypermobility is not exclusive to achondroplastics, I for example have it - most kids have it to a certain degree but outgrow it. Kids with achondroplasia tend to be many times more supple than an average height child is though. The photo above is from last summer so our son is 18 months old or so, he can still sit like that with ease (he often draws on the soles of his feet by pulling his leg up like this) and he will continue to be able to do that for a long time to come. He can do the splits with ease and he uses his extreme flexibility to his advantage a lot of the time (like in the video below where his ability to do the splits helps him get on to the sofa. The video is from when he was 15 months old but he still does this).



While excessive flexibility can allow for some cool party tricks as you can contort your body in ways that are out with the norm it's not an ideal condition to have. This is what my fingers do when I flex them, my thumbs can also bend flat against my arm without any strain. And it takes no effort whatsoever to bend over and put my entire hand under my feet. When I was younger it was a lot more extreme than this (although I have met others with far more flexibility in their joints, one school friend could rotate her shoulders and arms 360 degrees, she frequently dislocated her shoulders). As I've got older my joints have stiffened a bit but I'm still more supple than the average 30 year old - especially as I don't do anything to encourage the flexibility.


I have no doubt that flexibility that you work for by proper exercise, like yoga or gymnastics, is far more beneficial and safe - unfortunately joint hypermobility can cause joint fatigue and pain. It's much easier for someone with hypermobility to injure themselves doing something quite innocuous. I have a lot of clicking joints due to my hypermobility - for example, my jaw clicks every time I chew or yawn and it has recently started getting stuck in a dislocated position. Yes is hurts my jaw when this happens. Getting into positions like I did with my hands or below, however, don't hurt while doing them but they can certainly contribute to my fibromyalgia pains - the doctor who diagnosed me felt the two conditions were strongly inter linked. 



P.S. that's me still managing that despite a 7 month bump.


There is no cure for joint hypermobility but you can work to strengthen the muscles around the joints to try to protect them better and prevent injuries. Unfortunately even then you can still get hurt.
Now as I said it's very prevalent in people with achondroplasia and is the main cause of discomfort for many with the condition. Even if you work to maintain good muscle mass it's still very difficult to not experience pain at the end of strenuous days. And as they are incredibly hypermobile (I've got NOTHING on someone with achon!) it's very easy for them to feel fatigue after moving about and injuries come even easier. They are already prone to curvature of the spine and bowed leg, and as you can guess their supple bodies aid that. It makes posture and good physio very important for anyone with achondroplasia. The appropriate support when they are infants is so important and you can't pull them up by their arms in the same way you would an AH child as you can quite literally feel their joints start to come apart when you pull and they can't use their own strength to help.

It can take the smallest thing to cause injury and great pain sadly. I've suffered with problems with my ankles since I was a child, I now know it's due to my hypermobility and fibromyalgia but I must have seemed like an over sensitive child at times. In any case I have some understanding of what our son is going to have to learn to live with. I wish he didn't because I have no doubt his pain will be greater than mine, and no parent wants their child to suffer. The best we can do is encourage him to continue to be active and strengthen all his muscles, but we also can't protect him entirely. 

Elis will start to have days when he's sore all over and he will hurt his joints. Sadly this happened for the first time the other day. This boy has had a million falls and bumps, he is boisterous with his sisters and fearless with his tricks and climbing. He's had so many bad cracks to his head I've lost count. But the other day was the first time he really injured himself out with a cut or bruise and it took nothing for him to get incredibly hurt. He walked into another kid and fell lightly to the ground  (as I say he's had a lot of my worse falls) and his left ankle has been in agony since. They were standing in a puddle at the time and the uneven ground must have caused him to roll his ankle.

This happened Wednesday morning and up until now he's refused to put any weight on it and cried in pain any time he put pressure on it. His ankle isn't, and hasn't been, bruised or swollen in the slightest (mine have rarely been when I've been in pain either) so we felt secure that it must just have been a very light sprain. We had no cause to believe it would be anything more serious despite his clear pain - even paracetamol seemed to do nothing the first day. We've been putting a few extra socks on to try to help stabilise the ankle when needed, although he's hated putting them on and taking them off. No way he'd have let me squeeze a shoe on!


The first two days I could barely leave his side as he was so reliant on my help. By Friday afternoon, so yesterday, he was finally becoming brave enough to try to move himself. He's since started to use crawling, hobbling while holding on to something, scooting on his bum and moving on his knees to get about. And finally, only a short while ago, he got distracted and stood for a good while with weight on both legs. His sisters both started cheering him on like we'd never seen him stand before. He is clearly still bothered as he hasn't agreed to try again since. But it must mean his ankle is feeling much better and that we'll hopefully see further improvement the coming days.



We're so glad it wasn't more serious but it's a stark reminder of how easy it will be for him to injure himself because of his hypermobile joints despite thriving the way he does. I'm sure this is just the first of many sore joints for him and wish I could protect him from it but being as active he is we just have to promote the building of his muscles and support him when he is too sore to get about.



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