Welcome to Alyssa's blog ...

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Central Scotland, United Kingdom
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"

* 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

* Had tongue repaired, 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. Took us at least a couple of days to realise, because we aren't entirely sure how she broke it. And it didn't give way until after she'd already been telling me - in quite a bemused voice - that "her leg was moving, all by itself!" I now know that is Alyssa-speak for 'something is broken! Get X-rays immediately!'

* As a result of the biting injuries (and much deliberating!) the decision was made to remove all of Alyssa's baby teeth, as and when they grew in. This occurred up until her very last molars, when it was decided that we should try "crowning" her molars. Thankfully, it did the trick. And she got to keep her baby molars. We managed to save her upper adult incisor, but she now has a new lower incisor, which is causing a lot of problems. She has had all the dental assistance that she can really have, at this point, so now we wait.

* 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 (usually from tiny wounds she doesn't know she has, which bloom into cellulitis quickly), 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!

Sunday, 28 February 2016

Things are not going very well .... :(

So I did remember about the blog this time.  In November.  Felt that was a bit too soon for another post, particularly as nothing major had really happened, and we were just plodding along fairly happily.  I remembered in December too, and felt similar.  Things appeared to be going well, and I didn't want to jinx us.  Got to the middle of December and things just went crazy, and spiralled out of control!  Obviously not in a good way.  We're still not out of the worst of it, but I'm at least dealing well enough with it, that I can post before the next lot of crappiness hits us (expecting that next week, in all honesty!).

Alyssa had a recheck appointment with her spinal specialist Mr Tsirikos, at Edinburgh Royal Infirmary.  She had repeat x-rays done and we were given the excellent news that her previous spinal fusion was still looking great, and appeared to be holding her spine in the position it was supposed to be in.  Then we were told that she is now developing scoliosis (sideways curving of the spine), and it would need some form of intervention, to prevent it from worsening.  Her congenital insensivitity to pain would obviously mask any discomfort she should feel if it was progressing, but that aside, there had been no evidence of it at her previous x-rays 7 months before that.  Mr T felt that it would most likely progress quickly.  Our options were to attempt a spinal brace - without knowing how she would tolerate it as far as her overheating, it just affecting her positioning (because she is so used to being unable to hold herself up properly due to her weakened muscles and CIP), and it affecting her walking; or further spinal surgery.  As the previous surgical site took so long to heal properly, along with the 'extra' work she was post-operatively, we decided to try the spinal bracing first.  It would not correct the scoliosis, but hopefully stop it from worsening, or at least slowed it down.  So an appointment was made to have her cast for a brace, and another for her to be fitted with it.  With previous experience of her reactions to the casting procedure, I was able to forewarn the OT's and we managed somewhat less stressfully with it .... that and a small fortune spent on magazines, chocolate and Irn Bru!  :-I  We were given an appointment to come back for it to be fitted in January.

The week before Christmas, Alyssa slipped from crawling across the floor and broke her thumb.  Obviously the first time I have ever presumed it couldn't possibly be broken and didn't take her to get it checked, it actually was.  Typical!  She got a splint put on as it wasn't a big fracture, and we carried on as is normal for us.  It was her L hand, so she couldn't get out of handwriting at school, like she'd hoped!  ;-)  A few days later, I suddenly realised that her L elbow was also looking swollen - 2 days before christmas! - and also noted that she had a very tiny wound on her R elbow, which looked ok though.  I decided to keep an eye on both; and watched rather frustratingly as the elbow/arm continued to swell throughout Christmas Eve, giving me no option but to take her to the ward for examination.  After x-rays and much debate, it was still unclear whether she had also fractured something in her L elbow (obviously no major fractures, which would have been evident, but little ones are more difficult to spot, particularly in children), but at least it meant that we could get home again, in time for Christmas.  Just!  She had a fab Christmas day, and loved her presents and family dinner, and we weren't due to have her arm checked again until New Year's eve so she put up with the splint as best she could, and hoped that she'd be told she could have it off then.  As we were sitting for the third (?) orthopaedic doctor to examine her x-rays, I realised that the wound on her other elbow now looked infected, and that a little 'head' had formed on top of the puncture site.  I managed to burst it and remove a lot of fluid, and did so later on that evening - hoping that we could avoid yet another admission for cellulitis - but it must have been building up inside, and her arm started heating up and swelling throughout New Year's Day.  End result being that we started 2016 off in the children's ward at Forth Valley Royal Hospital!  Not quite how I'd hoped we would.  The L elbow was still in the 'unsure if it is fractured' classification, but the orthopaedic consultant said he'd forward them to the orthopaedic consultants at the paediatric hospital we frequent, and see if they wanted to follow it up.  We stayed in the children's ward for almost a week, with Alyssa on IV antibiotics which changed a couple of times, and increased severely in dosage because the cellulitis was just not reducing like it usually did.  It did eventually start to clear, and she was discharged with oral antibiotics with follow-up appointments to monitor it.  Thankfully, after another couple of weeks, it appeared to resolve.

In the midst of this, she also managed to scratch her cornea somehow - requiring a visit to the urgent eye care team!  For the first time, she said that it was "stinging" - which is now appearing to be her term for feeling any form of pain.  Something is changing.  Either she is starting to feel certain types of pain, albeit inconsistently, or she is becoming more aware of the sensations her brain is trying to process.  A very good sign, especially as far as her eyes are concerned, because the earlier it's found, the easier and more successful the treatment is.  That resolved pretty easily with some ointment, and her splints on extra to stop her rubbing at it while it healed.

I also noticed that one of Alyssa's feet in particular seemed to be collapsing inwards, although both weren't looking great.  She has one leg slightly shorter than the other, and the one which she broke is the longer of the 2 and bows inwards already,  This affects her gait, and is presumably also contributing to the scoliosis.  I spoke to her orthotist, and it was felt that her legs and feet had changed quite a bit in a short period of time so she had new orthotic insole impressions made and it was agreed that it was time to contact orthopaedics again.  Her orthopaedic consultant may feel that the new orthotics are enough to control things for now as he recommended them for her, in the first place.  But it was in an "I don't think we need to anything just now" capacity, as he hoped the boots would be enough.  And they have been doing just that, for nearly 3 years now (obviously having been replaced with bigger ones, as she grew), but it's definitely time for a review.  And the hope that we'll get somewhat ok news.  I rarely expect good news, because it's generally disappointing or devastating news that's given.  As positive as I try to be about managing her condition overall, orthopaedics is just one of those areas of her body that is just never going to be nice to Alyssa.

The spinal brace fitting (and the week which followed) was probably one of the worst experiences we've been through, and we've been through a LOT!  It doesn't sound like much.  It's basically a very close-fitting plastic 'corset' which is strapped at the back, and limits her upper body and back movement.  Obviously not the most comfortable of things, but not something which you'd expect to be life-changing, right?  Wrong.  So completely and utterly wrong.  Alyssa absolutely freaked out when the OT was just trying it on her.  Full on hysterics, tears, and screaming!  I didn't expect her to like it, and I knew it was going to take a lot of getting used to, but I didn't expect her to absolutely lose it like she did.  She's endured so much, and just carried on with a fairly minor adjustment period, that I clearly assumed she would just 'dislike' it, then gradually get used to it.  There was a lot of bribery involved that day too.  Especially once the OT announced that she had to START with it on for 6 hours a day!  :-I  This was then to progress to 10 hours a day by 6 days later, and up to 20 hours a day by 10 days!  We were utterly shocked, never mind Alyssa!  I had been prepared for it to build up to a lot of hours per day, but I'd had no idea that it would be so fast a changeover.  The poor kid was just overwhelmed by it, and was the most miserable in the week which followed, that I have ever seen her :-(  It affected how she sat, how she lay down and leaned on anything, her posture, and - worst of all - her ability to walk.  She could barely walk that first day, and I started panicking that she would be back to square one with walking (she was almost 2 before she could take her first steps).  Especially given the fact that Mr T had deliberately not used metal rods in her spinal fusion because he was trying to avoid changing her posture and gait, for that very reason!  He knew that she would find it almost impossible to walk, having her body positioning changed so drastically, so I did not expect the brace to do just that.  The OT's had tried their best to not correct her lordosis (lower back curve) but it clearly did anyway.
Fortunately, as she has adjusted to wearing it, her ability to walk has improved some.  There is still a long way to go, and she is still not walking very far, but much better than she was initially (we're now 3 weeks in) so hopefully it will continue to get easier for her.  She is back in another 3 weeks for further spinal x-rays - both in and out of the brace - so we can see what is happening when she has it on.  There will be a big discussion at that time about how we will proceed for the future because I have several issues about it all.  We have not made it to the warmer time of year yet, so I have no idea how she will cope with the extra layer around her middle - her cooling vest is unlikely to be as effective, when it's worn over the brace, although it will still make some skin contact so it may be ok.  We are supposed to be going to Florida in October (Alyssa still doesn't know it's booked, just that we'll "maybe go, if we can save up enough pennies!") and it will add a whole host of issues to what will face us then, particularly with the overheating aspect.  It would limit what she's actually able to do when we get there, and I'm really not up for finally getting her to Florida and to Disney World - the only place she's really ever wanted to go - and have her unable to enjoy it as much as she can!  The surgical option isn't brilliant either, but I'm thinking that there would be a period of spinal bracing required post-op, but there would hopefully be a long period without it afterwards.  Not in time for Florida in October, but something to perhaps start thinking about in the not-too-distance future.  I've known for some time that Alyssa will be exceptionally lucky to avoid future spinal surgeries (and she does not seem to be lucky with much, poor girl) so it's something which is probably inevitable anyway.  If we continue with the 'bracing only' option, then she will need to wear it until she stops growing - approximately 14-15 years of age.  That also does not sound like something I'd like to do, especially with everything else she has to endure.  So the spinal consultation in a few weeks could be a long one, with much to think about afterwards.

And, lastly, that L elbow which was "maybe fractured" and seemed to be settling down?  It hasn't.  Thought it still wasn't quite right, over the last couple of weeks, but only fully realised last night that her arm is almost as swollen again now as it was when she was first admitted for investigation.  The area around her elbow still feels much more 'bony' than it should, and is still much bigger, but the surrounding tissue is now swollen again.  Ugh.  The poor kid just doesn't get a break.  I had planned to ask the orthopaedic specialist for his opinion of it on Wednesday when I turned up for her feet/legs assessment anyway, but now I'm going to have to make it just as much of a priority.  She fell (her first 'proper' fall since wearing the brace) last week, and landed flat on her side.  Her L side.  So it's very possible that she has re-injured it.  It's also possible that whatever the injury at Christmas was, has just not resolved, and her constant use of it has just caused it to continue worsening again.  Hopefully I'll get answers to that on Wed too .... and they'll be 'ok' answers ... and I can update fairly soon (I'm not going to even attempt to promise that.  It will depend on what the actual answers are, and how quickly I get to process them.)

In finalising - and attempting to end on a good note - we are having a really crappy time just now.  And it's not even March yet.  I'm really annoyed with the Universe right now, and it's seemingly constant attack on my poor girl.  But.  At exactly this point, 3 years ago, she was in hospital with septicaemia and had just collapsed, giving the nurses and doctors a huge shock - because she "looked so well."  We had the 'crash team' firing into the room, trying to make her take oxygen, whilst trying to get extra IV lines into her to counteract the shock she was going into.  And she responded very quickly, despite us then having to live in the hospital for the next 2 weeks.  So it could most definitely be worse.  And we do have the most awesome nurses in our local children's ward, so there is also that!  :-)

She has also had her 9th birthday!!!  How on earth did we make it to 9?!  She was very spoiled, needless to say, with everything else she had to put up with around it.  She got lots of presents, went to the cinema and for dinner with her friends, and got a Wii U - which made her time 'sitting about, adjusting to the brace' much better.  It did become an 'early birthday present' because of how miserable she was, but it was worth bringing it forward a week, to see her little face light up.  It's the little things :-)

                                                                   My special girl!


Anonymous said...

Considering what both Alyssa and her mum have been thorough and go through on a daily basis they deserve the biggest, brightest of medals!.
Anyone who thinks that they have problems should read this blog and think again.
Moira is an inspiration and Alyssa is so bright/cheerful/ cute etc etc that it's hard to believe that she has to endure so much.
I really hope that they make the Disney Trip-- it's an awesome place and Alyssa will have a ball-- I'm sure the Disney company will do all they can to make their trip memorable.

Alyssa's Mum said...

Aww, thank you :) Fingers (and everything else!) crossed xx