Welcome to Alyssa's blog ...

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My name is Moira, and I hope to share with you what my daughter's life has been like so far ... so you can all truly understand and appreciate the gift of pain, which we take very much for granted! Alyssa does not feel "peripheral" pain, which means she does not feel pain anywhere other than internally. This has led to many unintentional injuries and self-mutilation. My aim is to not only find others like Alyssa, and help those who may be going through what we are, as well as raising awareness about this condition, and how feeling pain is actually a GOOD thing! I am thankfully now part of a support group run on FB which is an amazing group of people, who all have varying types of experience with pain insensitivity. I can be contacted directly via understandingalyssa@hotmail.co.uk

Self-injuries to date:

The following will give you some idea of what Alyssa has already done to herself ... so far!

* Knocked a few of her own teeth out while "teething" and caused huge ulcerated sores in her mouth, from "rubbing" her teeth on her tongue and inner cheeks

* Bitten straight through her lower lip - didn't even flinch!

* Chewed the end of her tongue off, resulting in emergency repair and incisor removal. After having the tip of her tongue repaired, she then began chewing the side of her tongue as soon as her molars erupted

* Chewed a finger almost down to the bone

* Torn entire patches of skin off, and is scarred fairly extensively as a result! :-(

* Broken both feet - and I had to argue with doctors for almost 10 weeks with one of them, because they didn't believe it was broken! Even a lot of doctors haven't heard of Pain Insensitivity!

* Broken her left leg, just under the knee, and walked about on it quite happily for at least a couple of days. We'll never know how she broke it. Any time she says "my --- is moving, all by itself," we get x-rays done!

* She had to have all of her baby teeth removed, as and when they came in, due to all the biting injuries.
She is still dealing with the after-effects of that, as a teen.

* Required spinal surgery to correct a vertebral slippage issue, which she was completely unaware of. The op itself was pretty straightforward. The post-op period was lengthy, and anything but fun.

* Developed septicaemia from one of her many episodes of cellulitis because nobody realised it hadn't gone away, and was just grumbling away as an abscess in her elbow. When she collapsed, it was scary!

* Managed to dislocate her left hip, falling from her trike .... but it took us 4 months to realise, because she didn't feel it!

* Had corrective surgery performed on both hips. Unfortunate complications ensued, which eventually caused the entire removal of her Right hip, and part of her femur.

Thursday 8 March 2012

Can't believe how much time has passed ... so quickly!

Wow!  I can't believe it's March already, and I haven't updated the blog!  :-/  So much has (obviously) happened since I last posted - some good, some not so good - but overall, Alyssa is doing well.  "The L leg" is still being monitored, though the orthopaedists are fairly happy with it.  It's still a bit bowed, but she's also still a bit weak on it, and it is definitely still improving, so we're hoping it will continue to straighten out as she strengthens.  There were concerns at one point that it was very bowed and that this might lead to shortening of that leg, but the ortho was less concerned about it at our last check-up in January.  


In November, Alyssa had her first ever Caudal Nerve Block procedure performed - to try and relieve the internal nerve pain she does feel, rather excruciatingly - and (on the actual morning she was admitted for this) I discovered that her L foot was hugely swollen and purple!  :-O  I had been aware that she'd tripped whilst at her dad's house that weekend, but was a bit shocked at the bruising, because it had seemed fine after multiple checks of it.  The nurses who had just admitted her were stunned because they'd just witnessed her happily wandering about on it.  And it LOOKED like it might be broken, to us all!  They couldn't believe she wasn't bothered about it at all, and quickly ordered her a fully-enclosed cot to stay in after her GA, which we'd also been "debating" up until they'd witnessed this for themselves!  There was definitely no debate after they'd seen her foot, and realised she really DIDN'T FEEL PAIN!  There were yet more xrays ordered, this time of the foot.  I was told that there were no fractures (but was a bit skeptical this time round, after spending 10 weeks arguing about the other foot, earlier in the year!) and then her ortho consultant phoned me to say that he'd seen something in her foot that he hadn't seen for many years.  That she had a very rare thing called Kohler's disease (sounds worse than it actually is!) and basically had suffered some disruption to one of the bones in her foot, and it had died off.  This usually causes swelling and "pain," though obviously Alyssa was oblivious to that aspect, and that it does eventually grow back.  But the swelling could last months, so to just allow her to rest it and wear her boot-cast for a small period of time, then just let her build her exercise back up again.  

Because of the foot injury, I was a bit distracted, and didn't really pay attention to whether I thought the Caudal Nerve Block had actually worked or not (I was told to be prepared for it to take up to a couple of weeks to make any difference, and - by the time she finally had it done - she was almost screaming 24 hours a day with it).  Gradually, as the week progressed, I started to realise that she was falling asleep more easily, and screaming less through the night ... then also at naptime in the afternoons, and eventually the screaming stopped altogether, unless she was actually needing the loo.  Sadly, it didn't really affect that aspect, but just allowing her (and me) to get some sleep was a HUGE bonus.  In January, we discussed it's success, and agreed that we would just repeat it when it started to wear off, and that I would just call to arrange it when I felt that her symptoms were building again.  That time is literally just happening now, and she is booked in again for another CNB in a couple of weeks.  The anaesthetist had  hoped that it would last 6 months, but feels that, over time, the effects will lengthen.  And 4 months is definitely not bad, considering she was screaming almost round-the-clock by the time we got to November!  


December passed relatively smoothly, and we had a lovely Christmas and New Year, with lots of lovely prezzies from "Santa."  She did unfortunately tear her neck open again, after a sudden increase in itching over the holiday period, but nothing too major, and certainly nothing like we've dealt with before.  She also started obviously sweating more, and this probably contributed to the increased itching.  In January, I had  discussions with several of the specialists regarding the medications she was taking, and if any of them could be increased or altered, as I felt that the main one responsible for helping her itch reduction was no longer working as effectively.  After much research and checking, there was an alternative drug suggested, but pharmacy checking has found this to be ULTRA-expensive, and something which would require a special case to be written up, in order for her to receive it.  She probably would pass this easily, given her condition is so rare, but I don't feel that we're at that point yet.  The medication IS still working, it's just not working as well as it did initially, and - despite the potential damage this medication can also do - she is also MUCH better on it than she has ever been, when we've tried to withdraw her from it.  There may come a time when we do indeed have to apply a special case, and request the other drug, but we are just going to plod along for now, while things are still "ok" with her medicines and skin.


February made it much more obvious that something was not quite right with Alyssa, and she has been tiring more and more quickly lately.  In hindsight, I realised that this was also partially true in January also, it's just difficult to notice that she's "more" fatigued, when she fatigues so easily, most of the time.  But it got to ridiculous levels, were she was getting up from a nap (of 3-4 HOURS) and still complaining about how tired she was.  Or being up for an hour again, and either yawning her little head off, or actually falling asleep again in the car.  This is an ongoing process at the moment in that we still don't know entirely what's going on with her, but she's had a load of tests done, and redone, and it's looking like she's just had one infection after another lately, and her poor little body hasn't had time to recover properly.  As with everything which affects Alyssa, fatigue is the usual response to how she copes, and sleep is a necessity.  So we're not doing huge amounts of anything right now, just trying to let her rest, and recover.  And we're still ruling things out just now, so hopefully we'll either get some answers, or she'll just continue to improve again.  


Alyssa also TURNED 5!! in February!!!  She's getting a big girl!  :-) 




We're always being told to try and strengthen her muscles, but that's a difficult thing to do with a physically delayed child who genuinely fatigues with exercise (and non-exercise).  And - despite being now 5 years old - we've never actually managed to find a trike that she could successfully ride on - which the rheum feels would be the most helpful (next to swimming, and Alyssa obviously has multiple issues with swimming!) to Alyssa, because it would allow her to use her leg muscles, but without putting them in dangerous positions.  When she does any form of exercise, her lack of pain response to her joints being out of the "normal" range of motion, actually puts her in more danger of injuring herself.  She just wouldn't feel it if it happened!  And "overstretching" with Alyssa is a very real concern, due to both her hypermobility AND her pain insensitivity.  In people with hypermobility (previously termed "double jointedness" which is actually a misnomer), their joints can be stretched further than they should actually go, but this causes pain and the person will ache for some time and realise that they should not have allowed their joints so extend so far.  Alyssa never gets this warning, and will happily let her joints go wherever they let them, or worse, FORCE them to go into positions that SHE feels comfortable in.  Which again, is contradictory, because she doesn't really ever feel UNcomfortable.  She just knows that there are certain positions where she feels SOMETHING, so she will go out of her way to put her body in those positions, at the destruction of those very joints.  And she doesn't understand that she is doing any damage.  


Because of this, I have decided to get a custom-made special needs trike built for her, and I am looking into finding a company who will come to see Alyssa, and configure a trike which will do everything Alyssa (and I!) need it do.  I will be looking at various trikes over the next couple of months, as well as getting recommendations from others who have used similar companies.  And I am excited to announce that VALIDUS GYM is having a fundraiser, to help get Alyssa her new trike.  This is just overwhelming of them, and I am very grateful to them, as well as to all of the local businesses who are donating gifts, time, and services, as raffle prizes.  It is amazing how many people come together to get my special girl what she needs! We are very lucky people, who greatly appreciate it!  Thank you to everyone who is contributing, and to Validus Gym!  :-)  




  

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