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!

Friday, 26 May 2017

Time for another update

Hey folks,

Despite trying to update again just before Alyssa's hip operation (which was scheduled for the 27th April), I'm only getting round to it now.  Surprise, surprise, it's been a roller-coaster couple of months again....

After being all organised for the surgery; with a care package in place, medical equipment in the house, and a prepared 10 year old, it had to be cancelled at the last minute due to an emergency being admitted and no available HDU (High Dependency Unit) bed for Alyssa.  She was understandably upset by this.  Not because she was looking forward to it as such but because she had gotten her little head around what was about to happen, and was mentally ready for it.  It was difficult for us too because so many things had been organised in advance, and we were also prepared for it.  The cancellation couldn't be helped, but it was disappointing.  I had arranged for 'our last day' of her walking to be fun-filled, and had planned to take her bowling and to the mini-golf which she loves.  She'd been very excited about that so we still went.  It would have been cruel not to take her after telling her about it for weeks!  Returning to school the day of her operation was a bit confusing for the kids in her class (and the school staff) because they had expected her to be off for weeks - and had all made her "Good Luck" or "Get Well Soon" cards.  But we explained what had happened, and that Alyssa would still be having the surgery, just on another date.  (That date is now rescheduled for the 8th June).

                                                    The cards from her classmates ☺️

She beat me at bowling on our day out!

I managed to save face by beating her at mini-golf!  (Ok, so she got tired before we finished the games and kept sitting down, leaving me to take her shots and my shots.  So I essentially beat myself aat mini-golf!) 😄

Since that time, her R leg has been giving way a lot and she's been experiencing the shin pains again - to the point where I had to increase the medication which the neurologist put her on, to the maximum dosage she can have.  I really hadn't wanted to get to that, but hoped that it would be short-term, up 
until the op.  Then it was postponed.  The ortho believes it's referred pain from her hip (though he seems to be under the impression that it's her L leg which she's experiencing the "shocks."  It did 
occur a few times in her L leg, but it's mainly her R leg which is the problem.  The neurologist thinks  that it's probably a progression of her condition.  It may be that it IS referred pain from her L hip, due to her R leg having overcompensated for the L dislocation.  But as far as I'm concerned, the only way to know for sure is to have the hip surgery then see if the shin pains disappear.  Unfortunately, as they have now reappeared even on the max dosage, it looks like we will need to withdraw her from that drug, and trial something else.  The neurologist got back to me last night, so we now have a new plan.  We have to withdraw the current drug slowly, so we've started that already, and we can start to introduce the new one - then hope that it starts to help her.  Neuropathic pain for anyone is awful, especially if it can't be controlled.  We obviously have no idea what she's actually experiencing (apart from her calling them "shocks"), but her lack of 'normal' pain perception means it's a bit of a shock to her system.  We don't really know if it is a severe pain; or if it just feels severe to her because she has nothing to compare it with.  Fractures, cellulitis (and most infections) and most injuries cause her no sensation - to the point where if she doesn't voice them immediately, she'll forget all about them.  Then I'll find the swelling/red area later.  Often days later.  These mysterious nerve pains are clearly distressing to her, and unfortunately leave us yet again with no answers in how to help her.  With any luck, the new medication will help, at least until she has her operation, and then we'll try to figure out if they are indeed related to her hip(s), or a progression of her nervous system issues.

We had some good news yesterday though.  We received confirmation that Alyssa has been accepted into the care and support which Rachel House are very well-known for.  Rachel House is a children's hospice not too far from us which gives those with life-shortening conditions the chance to meet and have fun with others like them.  A place where families can relax, take time out from their medical issues, and just get a break from their lives.  There are support workers, nurses, and doctors on hand to make sure anything needed is available, and they do their best to provide support for the whole family.  The provide respite care when needed, and we can stay there together, but Alyssa can also go and stay for "a sleepover" if and when she feels she wants to.  She's very excited about going to see it and meet the staff there.  She reached a point some time ago now where she was physically unable to keep up with kids her age, and they are all off doing activities which she can't take part in (gymnastics, dancing, horse-riding, trampolining, etc).  At Rachel House, she'll have the opportunity to get involved in activities which aren't limited by her mobility or risk of injury, and she'll not have to feel left out.

Alyssa is surrounded by people who can walk, run, dance, and play football etc.  Things which most of us don't even think about.  But not only does her condition restrict her from these things, her inability to cope with environmental temperatures limits even her attendance of them at times.  While everyone else is out playing in the sunshine (which we don't get very often, thankfully), she's indoors with the air conditioning on, watching them, because she overheats easily and becomes unwell in the heat.  Conversely; while everyone else is out playing in the snow (which we also thankfully don't get too often), she's indoors with the heating on, watching them, because she has a heightened risk of frostbite.   She would LOVE to play in the snow, but being outside in it for even 30 minutes is damaging to her skin and she can be turning blue before she's even aware she's cold.  It's hard for her to fit in in most places, so it will be amazing for her to go somewhere where they've already thought of all the issues children with complex conditions face.  Obviously there will be nobody 'like her' there (and they are fully aware that she is unique, and that she will probably give them a challenge), but they are so welcoming and eager to help that I think she'll have a great time with whatever they come up with.  We are going there next Friday to see round it, and meet some of the staff there, on our way back from her pre-operative assessment at Edinburgh Sick Kids.  The lady from Rachel House thought it would be a perfect way to end yet another day at hospital, by going and seeing all the fun she'll be able to have when she's there properly.  And we'll find out more about how things work. 

I'm aiming to update again soon, but it will most likely be after her operation now - assuming it goes ahead as scheduled this time.  The op itself may be bigger than initially planned now, as the ortho thinks he may have to shorten her R femur (thigh bone) as well.  He believes that her L leg may be shorter once her hip is surgically placed back in the joint, and that this will lead to an imbalance.  However, her R leg has been approximately 0.5 - 1cm longer than her L for some time, so he may not need to do that.  We won't know until she is actually in surgery, and he can assess her leg length once her hip dislocation is reduced correctly.  He is obviously very keen to do as little future surgery as is possible (as am I) so doing it all at once is the best plan.  There is little doubt that she will need further orthopaedic surgery as she ages, but the longer we can put them off, the better.  I'm hoping everything will go as smoothly as it can, and I'll be updating you all with an 'adjusting to the cast but otherwise fairly happy child' post.  

Cheers for reading.  It may well be one of the shortest posts I've written - which is pretty impressive, considering the life we have here - so you hopefully didn't need too much coffee this round!  ;-)  

Wednesday, 15 February 2017

Update 3 of 3 - the 'fully medical' one ....

When we returned from Florida, we saw Alyssa's 'general' orthopaedic surgeon (ie not her spinal surgeon).  We had an in-depth discussion about whether to actually fix her Left hip or not.  Her ortho explained that it would be a very big operation and we'd probably be looking at approximately a year of recovery time.  Needless to say, he was not keen to proceed with it unless it was deemed to be vital to her health.  I was concerned about the implications of not having her hip relocated, especially as far as how it might affect her present spinal issues.  She already curves to that side with her scoliosis (which we are already trying to correct/reduce with her spinal brace) and her L leg is longer than her R leg, due to it being out of joint.  She therefore walks with a very exaggerated pendulum gait; ie swinging from side to side as she lifts each leg.  She can control the swinging to some extent if she is reminded to "not walk like a penguin" but some of it is unavoidable.  I told him that although I did not want her to have such a big operation, we had already discussed with her spinal surgeon that the spinal brace was required in order to avoid major corrective spinal surgery.  The spinal operation would be much more invasive and serious than any hip surgery, with expected complications and repeat surgeries, before we even got to the healing and recover process.  It is a possibility that - even with the scoliosis brace - she will eventually need to have this operation anyway, but her spinal surgeon and I are keen to avoid it for as long as possible.  Having her hip out of joint not only weakens her pelvis (and therefore affects her ability to walk, because her core strength is reduced), it affects her posture, which in turn aggravates the scoliosis.  He decided that he would prefer to speak to another specialist and get his opinion on Alyssa's overall issues, and how doing the op / not doing the op would affect her in the longer term, and said that the new specialist would probably see Alyssa for himself, so he could assess her properly.

We didn't get as far as meeting the new orthopaedic specialist before she developed pneumonia in mid-December and had to be hospitalised for IV antibiotics and fever-reducers.  She had gotten increasingly breathless as the weeks passed after our return from Florida but none of the docs had heard anything to concern them when they listened to her chest, so it wasn't obvious until she was pretty poorly.  I'd even had her tested for anaemia, in case that was what was affecting her breathing, and causing her to fatigue so quickly.  On the days where she actually made it to school, they were having to send her home by about 11am because she was just so tired and pale.  She fell asleep in class on more than one occasion, which tends to happen frequently if she's ill.  They usually let her nap for 15-30 minutes to see if that is enough to 'recharge' her, but any more than that and they know she's unwell, and needs to be in her bed.  She was having to sleep for at least a couple of hours at home, before being able to get up and engage in any form of activity, then going back to sleep at bedtime and sleeping all night.  So the pneumonia was good, from the aspect that at least we knew what was wrong with her, and could begin treating her.  She recovered fairly well from that and was discharged a few days before Christmas.  

I was hoping this meant that we'd dodged an illness over Christmas bullet, but I then had to take her to A+E on Christmas Day because she scratched a huge area of her eye surface (corneal abrasion)!  Something must have irritated her eye and she'd then made it much worse by continuously rubbing at it because it was "itchy" until she couldn't see out of it any longer (and even then, she was still attacking it!).  The A+E doctor was fab, though said he wasn't sure how to proceed, because he'd never dealt with a corneal abrasion so severe in someone so young before.  The abrasion was covering the entire pupillary area of her cornea.  He asked the on-call opthalmologist to see Alyssa and she was prescribed some antibiotic ointment.  We were asked to return to her normal priority eye clinic a couple of days later for a re-examination, which we did.  I was very worried about her sight because we've always been very lucky with regards to her vision (numerous children go blind, or partially blind, from not feeling pain, because they stick their fingers in their eyes as babies, or get particles of dirt etc in their eyes and don't feel it.  They then continue to rub at them, until the eye surface is so damaged that the damage can be irriversible.  Alyssa had managed to have perfect vision and fairly healthy eyes, with only a couple of minor abrasions in the past few years, and I was hoping this would continue.  She's now been for multiple eye checks with various eye doctors and then with her own, and the abrasion has mostly healed, thought there is some scarring to the surface of her eye.  The opthalmologist is unsure if it will resolve completely, but she is hopeful.  I mentioned that my new concern that Alyssa had developed a "squint" (lazy eye) since the eye injury in December.  She tested Alyssa's eyes again and said she agreed, and that we should attempt to correct it as soon as possible.  It is most likely due to her having been unable to see for a period of time, and losing focus in it, because she was using the other eye to compensate.  The optometrist was unavailable that day so we are back on Friday morning to have a full eye test done, and to find out if Alyssa will get glasses with / without a patch, to make her concentrate her focus in that eye, and strengthen it again.  

Alyssa's itching had also increased severely during November and into December, so her dermatologist (who is brilliant!) prescribed a new drug for her to trial.  She was constantly coming home from school with her shirts more soaked in blood than normal, and she literally tore her neck to shreds at one point.  She is somewhat of a guinea pig when it comes to medicines because she is an almost unique little entity so her doctors are learning about the condition from her!  Medicines do not always work the way they should, because her nervous system does not work properly, so a lot of the time it is 'educated guesswork' as to what might help ease or at least reduce her symptoms.  She has been on many drug trials in her life and it seems likely that it will be the case for some time.  The new drug had to be put on hold when I realised she had pneumonia however, as I didn't want her starting a new drug when her immune system was already under stress.  Once it seemed that she had the pneumonia under control, she developed cellulitis again and had to be admitted to hospital for further IV antibiotics.  We only managed to get approximately 48 hrs worth into her - over a period of about 3 1/2 days - because her veins were so difficult to find.  Her insensitivity to pain means that she is not careful with IV cannulas even when they're placed in her arms or hands easily.  You or I feel a certain amount of discomfort if we lean on a cannula, or use that arm/hand once one is in our vein but she feels nothing from that.  So she will lie on them, twist her arms into random positions, and sleep on them.  It doesn't take long for them to bend inside her veins, and become useless.  She was discharged before the infection had resolved due to this issue as there was no point in keeping her in hospital if she was taking antibiotics by mouth, and we had really run out of options for IV's when she wasn't sick enough to warrant much more invasive alternatives.  So although her arm was still hot when I left (and I was uncomfortable with leaving with it still hot), we really had no choice.  She continued the antibiotics for a week, then her dermatologist prescribed a longer term course of prophlyactic (preventative) ones.  She was then unable to start those when she should have either because I realised that the pneumonia had not quite gone away and had flared up again, so I had to abandon the prophylactic antibiotics in favour of ones more targeted to chest infections.  It seems to have resolved finally, so she has now started the longer term ones, which will hopefully prevent her getting cellulitis again.  She has also started the newer drug trial for the excessive itching, though it is too early to say if it is working or not.

The hip:

I saw the new orthopaedic specialist last week about Alyssa's dislocated hip.  He had both hips x-rayed again in very specific views and compared them to her October ones, then physically examined her.  He was visibly stunned that he could put her L hip in and out of joint without her even caring (she did cry that he was holding her knee too tightly, and she was quite upset about that!)  As soon as he loosened his grip on her knee, she didn't care at all.  That blew his mind somewhat.  It's one thing to know about Congenital Insensitivity to Pain .... it's another thing completely to witness it for yourself.  He'd obviously been told about her and had read her notes, but it hadn't completely prepared him for the real thing.  He just kept saying "That's crazy!!  That should be extremely painful!"  I accompanied him to the consultation room to discuss his findings.  I explained that her regular ortho had been very reluctant to go ahead and fix her hip for the reasons mentioned above, but that I was concerned about the problems of not doing it.  He said that sadly her spine didn't even really come into it because the damage already done to her hip is very clear.  The x-rays she had done in October and the ones she had last week show significant erosion and soft tissue damage from her leg bone grinding against her pelvis.  Her abnormal gait (worsened by the dislocation) as well as her abnormal sitting/lying positions mean that her hips are forced into positions they would not naturally be in.  There is really no doubt that her hip needs to be surgically fixed back into her joint in order to prevent further damage.  Unfortunately, the x-rays also show that her R hip joint is not great either, and the ortho doesn't feel that it would take too much for her to dislocate that one as well.  :-(  He asked whether I thought we should fix the dislocated hip first; let her recover, then go back in and stabilise the other one, or just operate on both hips at the same time.  I immediately said that we do both at the same time.  It will be a huge procedure, but the dislocated hip procedure is already huge.  By doing both at once, it's all over in one big operation, there's only one recovery period that she'll have to go through, and she'll only have to deal with one cast post-operatively.  The ortho said that he also felt it was best to just do both at the same time, for the same reasons.  He said that he would also request a CT scan of her hips, so he can determine how much soft tissue damage there is to her L one, and also assess her R one at the same time.   

I do not have a set date for her operation but it is to be at the end of April.  The procedure will involve cutting a small section of bone out of her L thigh bone to aid the curving of her leg towards her pelvis.  The socket of her L hip is not very curved so this will be reshaped, then she will have her hip put back into place and held together with metal plates and screws.  Her R hip will also be stabilised surgically to prevent it from dislocating at a later date.  She will then be put into a Hip Spica cast made of plaster for 6-8 weeks, to keep her hips in the correct position while they heal.  She will be unable to stand, walk or move about by herself, so that's obviously going to be really hard for her - physically and emotionally.  The cast itself will be a nightmare for her (and me) because she is so prone to pressure sores (which she won't feel) and infections, not to mention that it will fatigue her upper body, just sitting in it.  It takes 4-5 HOURS to apply the cast, so it will be put on while she is still under the General Anaesthetic, but normally in a child with CIP, it would be changed frequently to check for wounds from rubbing.  She had pressure sores very quickly from her leg cast, and she wasn't able to walk on it at all - she just wiggled her foot inside it, because it didn't hurt.  This cast will cover her entre pelvic area, and she is bound to wriggle about inside it.  We won't be able to check the state of her skin without removing the cast, which would require a GA each time she needs one put on.  I believe that a GA is not normally given when removing the hip spicas but she reacts very badly to the vibration of the saw, and then immediately starts trying to destroy the skin which has been covered by it.  The air getting at her skin after it being inside a cast for so long is absolutely unbearable for her, and she will tear herself to shreds if we cannot stop her.  I will discuss with her ortho what the best plan for cast checking and removing, as we get nearer to the operation.

We do not expect her to be able to walk for a very long time after her cast is removed but hope that she will eventually get there.  It can take a child of normal strength and ability up to a year to walk properly again, but I expect Alyssa to take longer than that.  It took her until she was almost 2 years old to take her first steps, and I believe it is going to take a lot of work to get her walking again.  Her physio is concerned about her rebuilding her strength afterwards because she didn't lose any muscle when she was fitted with the scoliosis brace, and she couldn't walk as soon as it was put on her - because it changed the angle of her pelvis.  This will obviously be much more severe a process.  I am having to consider things like a special bed, hoists, a special car seat, whether her current wheelchair can be adapted to accommodate the hip spica, and a whole other host of things that are a bit overwhelming at the moment.  And I have no doubt that there are many aspects I haven't even thought of yet, nor that she will prove to have her own factors to add to the mix.  The year ahead is going to be challenging, to say the least.  

I have been asked what she thinks about the operation.  In all honesty, she doesn't really comprehend what's about to happen to her.  She knows she's having an operation.  She knows she's going to be put in a cast for 6-8 weeks.  And she knows that she's not going to be able to walk, or stand, and that it will take a long time for her to manage those things again.  What she understands is a completely different thing.  As adults, you or I might know that we're going to be put in a cast, and comprehend the implications of it.  But we still wouldn't really come to grips with all of the things which will be affected by it until it happens.  She is 9 (10 next week!) and doesn't have the capacity to foresee half of what I do, and she'll wake up in a 'sitting position' in a solid cast.  That in itself is bound to be overwhelming, before coming to grips with an entirely new lifestyle.  I'm not even sure yet how to help her cope with that, but we'll just have to get through each day the best way we can!  And I know we have a load of people who will spur us on, and help us get through those days.  She is having the CT scan on Friday so I will hopefully know more in the coming weeks, and be able to plan out the basics.

And finally: she is ten next week!  TEN!!!  Her life has been very unfair (and cruel!) a lot of the time, but she is a pretty amazing little being.  Despite everything she has to go through, she has the most brilliant personality and sense of humour, and still manages to live in her little 'pink and princess' Disney bubble.  She is very excited about going shopping for her birthday next weekend "on the train!!!!" and we will no doubt come home with yet more toys that she doesn't need, as well as clothes that she does!  But there are definitely worse things that she could be excited about.  Whatever she finds that I think will make the coming months easier, we'll be buying!  

As always, thanks for reading.  I always pass on messages and comments to her when they're left (on here, or on Facebook) after people have read it.  They make her smile :-) 

Tuesday, 7 February 2017

Update 2 of 3 .... The "Florida post" (LOTS of pictures!) :-D

I thought it would be nice to only post some nice things, for a change.  I'm still not entirely sure how we actually made it (and I'm now glad we did - but that's for update 3 ... or now possibly 4!), but we did.  And although it was very stressful for me, Alyssa had an amazing time at Walt Disney World.  She was just in heaven, pretty much all the time!  

We met the loveliest family on the plane on the way home.  Those of you who know Alyssa well will know how much she absolutely adores babies!  And that she can talk for Scotland!  We had a very cute baby in the seats behind us on the flight so she pretty much chattered the ears off the poor little girl ...... and her mum, all the way there!  Wee cutie's name was "Orla," and Alyssa was just in love with her.  The poor family were also in the same exact seats on the return flight too!  I believe I ended up enforcing a nap on that flight - so that Alyssa would sleep AND so that Orla and her family would get a break from her constant nattering!  Her mum and I have become Facebook friends since then, however, so she couldn't have been too bad ...... ;-D

We stayed at Disney's All-Star Movies Resort, and Alyssa's eyes were like saucers when she realised that the buildings, decor and rooms were all made "like the films!!"  It was really cute to see her in total wonderment at it all, and we hadn't even made it to the Magic Kingdom yet!  She was absolutely hyper, but I can't say I blamed her.  We waited a long time to get there.  

This is the first night, with her PINK Magic Band on, stuffing her face with pizza ... and drinking out of her Mickey straw!  :-) 

And just outside our hotel room

We headed to Magic Kingdom the first day (and most days!) and she was just amazed by the whole place.  We've been to Disneyland Paris, but it was a long time ago now, so she didn't really remember it very well.  I was at least prepared for the layout, as Main Street is pretty much the same there.  Her face when she saw Cinderella's castle was just a picture (I feel I'm going to repeat myself a lot in this post, as she was amazed and delighted by pretty much everything we saw, and I'm going to run out of descriptions of her!), and I think she thought that was as cool as it could get ..... until the first parade started!  She was just in her element!  She was still recovering from the dislocated hip / new medicine combination so she was in her wheelchair and couldn't join in with the dancing etc, but she was clapping, cheering and singing along with Jessie, Minnie, Mickey and the Toy Story characters.  It was the happiest I'd seen her in a very long time - and she's usually pretty cheerful, considering everything she has to deal with.  Then we went for a wander, to see the whole park, and work out where things were, and what we actually wanted to see or do.  

Pics of us in front of the castle (VERY rare photo ops of me!  I prefer to be behind the camera!) 


We did visit all of the parks (except the water ones, because she can't go into water unless it's a very specific temperature) but Magic Kingdom was by far our favourite!  I couldn't possibly post all the pictures we have from there, but I'll post some shots of all the characters she met while there ....

Her absolute favourite part of the entire holiday - and pretty much our reason for going!  "Elsaaaaaaaaa!"  :-D

Her 'other' favourites 

All of the other characters who she had to fill her "autograph book" with, and was excited to see ... just not as excited as she was to see the princesses above!



As you can see, meeting characters was high on our list of things to do!  And she had an absolute blast meeting them, even if she did become suddenly 'shy' when she met some of them.  We also went on The Dwarves Mine Train, which she initially looked absolutely terrified on, but soon decided that it was her absolute favourite ride.  Queued for it once without a fast pass - never again!  It took about an hour and 15 mins to get to the front of ride, which is over in about 90 seconds!  

We were also on It's A Small World After All, the Carousel, Winnie the Pooh's ride, the Magic Carpet Ride, Dumbo the Flying Elephant, Peter Pan, and The Little Mermaid rides many times.  And we went on the Teacups ride a few times .....  I even took a selfie (!) of us, being silly on it!  

We had lunch at what became MY favourite place - Belle and the Beast's castle!  I was dying to see inside it - even if she wasn't, though she didn't fully understand what it was until we were inside!  I took so many pictures of it because it was truly amazing.  So much of the film was present, including 'The Rose.'  There was also a really cool picture of the Prince who turned into the Beast when lightning flashed inside that section of the castle!  Only for a minute or so - to the point where it was really difficult to get a picture of it, but well worth the wait for it.  And a lovely cast member took a picture of us outside the castle :-)


The parades and shows were phenomenal!  No other way to say it!  I immediately decided I was ditching my life, and moving there to become a singing, dancing cast member!  :-D  The parades were all amazing to see, and she would have watched the same ones every single day, if I'd let her.  There's so much to do and see that it's just not possible, not to mention the one we couldn't really watch (Elena's welcoming) was always at 3pm, which was just far too hot for Alyssa to be outside at.  There really wasn't enough shade at a close enough distance for her to see Elena, which she was a bit disappointed about, but understood that she would overheat if she sat out in the sun at that time, even with her cooling vest on.  

Pics of her during some of the parades 

 She was also chosen on one of the days to be "Chef of the Day" at the Main Street Bakery!  The loveliest lady came across and asked her if she liked to bake because they were looking for someone to help make some cookies and sweets.  She was instantly excited and practically jumping up and down in her chair!  (She bakes cakes and cookies with her gran.  I can not and do not bake!)  She had so much fun ... and made so much mess!  There was chocolate everywhere, though the ladies who were helping her didn't mind at all, and told her to make as much mess as she liked.  The end result was fab.  2 lovely Mickey Mouse shaped chocolate rice krispie treats - one for her and one for me.  I believe she finished their M&M's stash on one of the cookies!  ;-D

We went to Mickey's Not so Scary Halloween party.  I was initially surprised by the cost of it (one night cost 1/3 of what I paid for the 2 weeks' park tickets!), but as we neared the holiday and almost didn't get to go (again), I decided that I didn't care what it cost.  We were doing it, and we were going to have a ball, and worry about the cost when we came home!  Who knows when - or if - we'll ever make it back again, considering Alyssa's never-ending health issues.  I was absolutely blown away a couple of weeks before we actually went, however, as some of my very good friends (aka my "gym family) had clubbed together secretly, and gathered money to cover the cost of our MNSHP tickets, with some leftover for Alyssa to buy a costume to wear!  It was very unexpected, and I'm very grateful to them for being so thoughtful and kind!  

Mickey's Not so Scary Halloween Party was amazing.  I've never experienced anything quite like it.  I'm not a fan of Halloween usually, as most of my friends know, but there's just nothing like Halloween in the States.  They really go all out for it, which we don't do here.  The costumes being worn were brilliant, the kids got to go trick-or-treating all over the park, and it was just so much fun.  Alyssa is very fussy about sweets and if it's not something she recognises, she won't eat them.  I knew before we went that she wouldn't eat most of them (and I was right!) but she had a total blast just finding all the treat stations, and gathering "candy."  She filled up her bucket, and half-filled one for me too, and chattered the ears off the cast members who were handing the treats out.  She chose a Wonder Woman costume 

Some of the other parks (I appear to have forgotten to take 'entrance' pics of Animal Kingdom!) 

We visited the Kennedy Space Center, a place I've wanted to visit all my life.  It was very cool, though Alyssa was only interested once in "Space stuff" when she could get inside the rocket simulator upstairs.  She did enjoy playing in the play park, and was very pleased to see a friendly squirrel ......!  It is definitely somewhere to visit if you happen to be in Orlando and are taking a break from all things Disney.

And you can't do a Walt Disney World story without mentioning the fireworks at the castle in Magic Kingdom!  You just can't!  I have literally never seen a firework display anything like that.  It actually ruins every firework display I shall ever see again in this country.  They tell a story from start to finish, and the music and narration which goes with it is genius.  Typically, I got so caught up in them that I didn't actually take any pictures (well, not any decent ones), though I did got a lot of fabulous videos.  And there you have it: our Florida / Walt Disney World holiday!  An incredible place for a 9 year old girl, and also for her adult mum.  It's amazing how much it brings your childhood memories back to life, and the minute detail in everything they do and have, just has to be seen to be believed.  I don't know if we'll ever make it back again, but I hope so.  It was well worth the money.

*I have glossed over the few medical issues we had, as I wanted to keep this post about the fun we both had there.  There was a bit of spontaneous adaptation needed a lot of the time due to the heat, the lack of shade, and just general issues that Alyssa had.  But nothing too major, and only stressful for me, not her.  She earned her Florida holiday, so I'm cool with that :-)  

Monday, 30 January 2017

Part 1 of 3: (you know that's not good .... right?!)

I figured it would be a good idea to break up this update, due to it being so in-depth, and because I thought it would be nice to actually have a separate update about the holiday we actually made it to!  This is how life went in the run-up to the Florida holiday:  

So we survived the remaining couple of weeks of the summer holiday without any further injuries, though Alyssa burst her spinal brace somehow (presumably in a fall, as she has many), and had to be cast for another one.  This process is always hard on Alyssa, because there are various temperatures on her skin during the casting procedure, and it is "painful" to her - because heat and cold touching her skin affects her nerves in a way that regular pain does not.  Nerve damage and neuropathic pain can vary from tingling, burning, shocking and numbness sensations.  Alyssa is getting better at describing things she feels, but it is still not clear a lot of the time why she is feeling them, or what is causing them.

She was only back at school for a couple of weeks before suddenly experiencing "shocks" in her R shin - to the point where she was buckling to the ground on it.  Sometimes it was just the sudden jolt which knocked her down, but at others, she was losing feeling in her leg completely!  She had x-rays to rule out another tibial fracture, but it looked perfectly healthy upon x-ray.  This issue unfortunately progressed to Alyssa being unable to stand at all on a couple of occasions, and having to hold on to furniture etc to even wander round the house.  Both of her legs were giving way from under her by the time she was due her spinal x-rays and, needless to say, I was starting to panic about her spine.  
I saw her spinal surgeon and advised what was happening.  He said immediately that it was not her spine which was causing the problems, but that he would have her x-rayed anyway.  Sure enough, her spine looked great, and it was clear that the spinal brace was helping keep her spine lovely and straight.  The spinal nurse contacted the neurology secretary and let her know that the spinal surgeon wanted Alyssa assessed urgently because there was something worrying going on.  That unfortunately didn't take place until the following week, despite lots of phone calls from myself and the spinal nurse, and I was getting rather annoyed.  If any child who "felt pain" was collapsing to the ground, they would have been seen immediately.  Alyssa, who doesn't feel pain like she should (and therefore should be seen instantly if she's feeling something severe) was just being passed back and forth between secretaries of the specialists she sees - but nobody was telling ME about this!  EVERYONE who is suddenly losing sensation in their legs, or collapsing to the floor, should be seen immediately!

She saw her neurologist, who felt that sadly, her condition was just progressing.  That her nerve damage was worsening, and that there probably wasn't any way to stop it.  He had a chat with a geneticist who studies a gene with mutations similar to the one Alyssa has, while I was also present, and asked for advice as to how to treat this new shocking sensation.  Alyssa is so rare that nobody really knows what to do for her, or how to help her.  We can only use educated guesses, and our best judgement .... and to pretty much hope for the best.  We agreed on a form of anticonvulsant which she's not trialled before (she's trialled many of them, for a variety of issues), and the neuro gave me instructions on how to increase the dosage, as the days and weeks passed.  He knew that we were supposed to be going to Florida a few weeks later (our 3rd attempt, due to it being cancelled twice previously due to other injuries/illnesses) and he was keen for us to get there.  
In the week which followed, the shocks seemed to lessen, but her walking ability got worse.  She had randomly mentioned "cracks" which she'd felt, but they weren't consistent, and in various parts of both legs, at different times.  I was still not happy that her entire pelvis had not been x-rayed when she was having her R tibia x-rayed, because we know from past experience that even if she feels 'pain,' it is not always where the issue actually is.  I called the neuro for advice on pain medication because I was having to go into school daily to administer paracetamol and ibuprofen for her - practically unheard of in our lives, as we normally only use those for fevers.  I was advised to take her to our local A+E for pelvic x-rays and to call the neuro secretary back if the x-rays showed nothing obvious, so that we could discuss pain medicine options for her.  I took her to A+E, explained what was going on, and we got her x-rays done.  The doctor was initially quite dismissive about the whole thing, despite me going into details about Alyssa's CIP and the previous hand, leg and foot fractures ......   Then the x-rays came back and we discovered that Alyssa had a dislocated hip!  But it was also the opposite side from the leg which she was collapsing on!  Just typical Alyssa really.

I had to reflect for a while as to what she could have done, to injure her hip.  I finally realised later that night that it was from a fall she'd had from her customised trike ..... 4 months previously!  When I thought about how she landed during it, it made perfect sense that she'd dislocated her hip, and actually amazing that she didn't do more damage than she actually did.  It was a horrific fall, and one which I couldn't believe that she walked away from injury-free.  Turns out that she didn't.  She just didn't feel it when her hip was knocked out of the socket.  We literally had 2 weeks to go, before our much-awaited trip to Florida, and I'd only told her a week prior (after keeping it secret for more than a year, so she didn't get disappointed yet again at it being cancelled), so we were all gutted that it looked like she might not get to go after all.  However, as soon as we realised that it was not an acute dislocation (ie it hadn't just happened, with a traumatic injury), it was agreed upon by all that there was no rush to see Alyssa anyway.  I couldn't see why she couldn't still go to Florida, if it had been present all that time, and nobody was upset about it being dislocated.  So, with less than 2 weeks to go, we finally got the go-ahead for Florida!  To say I was relieved is an understatement.  All our friends and family members had been watching and waiting nervously with us, because everyone was so keen for us to finally make it there.  They were all so helpful.  Some of them even put money in an envelope for Alyssa to go to "Mickey's Not So Scary" Halloween party, and enough leftover to buy a costume for herself.  We just knew that she'd have to use her wheelchair a bit more than normal, but I 'd already planned for that. In the Florida temperatures, even in October, I knew she'd fatigue more quickly, be sweatier and itchier, and need to hide from the sun quite a lot of the time.  So, all I needed was for the meds to stop the shin "shocks" to start working, so she could actually enjoy some of the time there.  Thankfully, it started working as the holiday neared, and she adjusted to it really well.  

Next update will be all about Florida!  It'll be a nice one.  For a change!  ;-)  

Saturday, 6 August 2016

It's never a good sign if it takes me this long to update ...

Hey folks,

A VERY long overdue post.  I'm not going to even pretend that "no news" has been good news.  I can honestly say that this year has been the worst year so far, in Alyssa's life.  I was ready for 2017 by the end of February, and it's just not gotten any better  :( 

When I last posted, Alyssa hadn't long been fitted with her spinal brace and she was still adjusting to it.  As well as life overall, in it.  As always, she was a little trooper and adapted as well as could be expected.  It's still giving her issues when she walks, because of her changed postural positioning (as well as her ever-present reluctance to actually walk in the first place!), but she's getting there - 6 months on.  I'd been concerned about the difference in her gait etc, and wanted to weigh this up against the surgical option.  After discussing that at length with Mr T, I discovered that the surgery would be FAR more complex and major than I initially thought.  She would essentially have to have her entire thoracic (ribs) spine fused with metalwork, to straighten her spine.  With her inability to feel pain added to the normal 'potential complications' there is a huge risk that the fusion will not hold, that the metal implants might need to be removed again in another surgery, only to have a further attempt a couple of months later.  Mr T has only done this operation in a child with Congenital Insensitivity to Pain once, and HE described it as horrendous.  All of the above complications occurred, and the poor child was in hospital for months at a time.  Obviously not the way to go, at this stage.  There is always likelihood that we will face that surgery eventually anyway, even with the bracing at this stage, but the longer we can put that off, the better all-round.  The last spinal surgery was difficult enough, with the wound healing taking 4 months - and that operation was fairly minor!  So I happily agreed to continue with the bracing, and Alyssa made a "pinky promise" that she would make an effort to walk more in it.  Mr T told her that now she'd made a pinky promise, she had to keep it!  He's so good with her, as he is with us, and everyone I've ever spoken to about him.  Just one of the loveliest people I've come across, and very knowledgeable about kids similar to Alyssa, even though they are so rare.

The more concerning news at this same appointment (there's always some, right!) is that Mr T is now concerned that the brace may be affecting Alyssa's previous spinal fusion now, because it has changed her posture so much.  Her spine was fused in the position she used to be in, and now that she is so much straighter, it is putting more pressure on the fusion at her lower back.  He is not sure that this area can take the added pressure, and worried that it will not be strong enough.  So we may need to go back in again, for further fusion.  If that happens, then he will go in 'from the front' instead of via her back - so she will essentially have a caesarian wound - and he will go right through to her spine, to fuse it from the front as well.  That just makes me sad :(  Hopefully it won't come to that, but she doesn't have the best of luck - and if HE is concerned about it, then it's obviously a strong possibility.  I asked how long it would be before we re-xrayed her, to check on it's progress, and he said 6 months ... which would take us to October, when we're supposed to be going to Florida (in our third attempt!), so we brought it forward to September.  I'm absolutely dreading that appointment, but I need to know before October if the holiday can go ahead or not.  The thought of her not getting to go - again - is unbearable, but if the risk of damage to her spine is too high, then it's not even a choice.  Her health has to come first.  She still doesn't know about Florida, for that very reason.  I can't tell her once again that we can't go because something else has happenend.  If we get good news in September then I'll start to make plans for it, but I'm still not planning to tell her until it's almost time.  So many things happen to her, it would be just tempting fate if I tell her too soon!  Everyone around us know about it, and that she doesn't know it's booked etc, so they're all waiting to hear if we can go or not too.  I'm trying not to think about it too much at this point though, because there is absolutely nothing I can do to affect the outcome of those x-rays.  They'll show either that a: her spinal fusion is fine, b: the spinal fusion appears to be weakening, but she can still go and we can worry about it when we come back, or c: that something needs to be done about it so soon that we have to cancel the holiday.  I shall be a nervous wreck that day, but we need to know.

So - that's the spinal stuff out the way .... there's still SO much to cover (did I mention that this has been awful?!). We saw the general orthopaedic specialist in March about Alyssa's feet and L elbow, where we actually got good news (hurrah!).  He felt that her feet weren't too much worse than last time he saw her.  Her R ankle is still flexing far too much, but he didn't think there was anything surgical required at this point, and she should continue to wear her orthotic boots as much as possible, so that her ankles have support.  The L one is doing ok, just a bit lax.  He then examined her L elbow for me, and checked her x-rays.  He said it was very clear that it was new damage, compared to the injury last June - which I'd told the ortho at Stirling - and that she has basically overstretched the ligaments in her elbow.  This is making her radius (one of the forearm bones) to sit 'raised' above the joint  because it is not going fully back into the joint like it should, and it is this which is causing the 'swelling' there.  He then said that surgery to correct this was not advisable, due to it having a very poor success rate, and that it would probably just make it worse.  Her radius DOES go back into the joint if she bends it right up, and it's not actually causing her problems, so he felt it was better to just leave it alone.  She will always have a "dodgy" elbow, but let's face it, she's not exactly prone to doing things normally!  He said that it may start to cause problems when she is older and has stopped growing, because the joint may start to "stick."  If that happens, he will then go in and 'shave' the top of the part of her radius which is sticking up, just to allow her elbow to bend properly again.  This is apparently a much more simple operation, and should sort any issues out.  So that wasn't too awful a month!

In April, she had another bout of cellulitis, which thankfully resolved with IV antibiotics much more quickly than the one in January!  I was really pleased about that, because I was starting to get worried about her immune function, and that it wasn't working properly again.  We only had to stay for 3 days on that occasion, and although I was wary that it had gone (because it was so quick, and because of the time that it caused septicaemia!) but it did actually go away fully that time.  

In May, she went to soft play, fell, and broke one of the bones in her hand (technically her index finger, but in the middle of her palm).  Had to take her to A+E, where I was told that the bones weren't displaced, and she didn't need to have anything put on it.  She was just not to "lift anything heavy" or "put too much weight on it."  :-/  I tried to explain that she would USE IT NORMALLY unless it was at least splinted, because she didn't feel it, and she would MAKE the bones displaced.  That fell on deaf ears unfortunately, and we ended up in the ward a few days later, with a very swollen hand - which she then required further x-rays for.  I was very grateful that - after going through several doctors (and arguing with the on-call orthopaedic consultant, who gave us just the most ridiculous suggestions to try) - a very sensible doctor came on-duty, watched her playing, and immediately said she needed it immobilised!  (Thank you!).  She came home that night with a huge bandage on, and we returned the following day, where we saw the same orthopaedic registrar that we'd seen in December for the thumb and elbow, and he supplied me with a wraparound splint.  Seriously, that's all I was looking for at A+E, or with the on-call orthopaedic doctor!  It should not have been so difficult.

In June, she had yet another admission for cellulitis, right before the summer holidays started.  ie - we didn't actually even make it to the day before the holidays this year, but the day before that!  She's getting earlier by a day every year!  Again, fortunately only admitted for a few days, but she missed the fun of the last days at school, seeing her friends before they broke up for holidays (and as she can't go out unsupervised, she doesn't see anyone for the entire holiday, unlike all the other kids), and it just started the whole holidays off like we started the year off!  

Last month, she re-broke her R hand.  Again, in the palm area of her hand, but her middle finger this time.  It was a bit too coincidental, and we were not aware of any falls/bangs etc, so I quizzed the A+E doc, who agreed that she most likely had a hairline fracture there from the initial incident, and had now managed to do something which had caused that bone to separate too.  Back on with the splint!  At least I was a pro at looking after the hand by that point.  And she was a pro at working out what she could get out of doing!!  Little smarty pants.  
We then discovered that her spinal brace was burst at one side.  Not the best news.  I contacted the orthotics department and was told that without seeing it, they couldn't tell if it could be repaired or not, so I took a trip up (by myself) to see the technician.  He did weld it, but said straightaway that it was just going to burst again, and she'd need to have another one made.  Awesome.  :-/  We got an appointment for the following week, and I couldn't even bring myself to tell her - because I knew how upset she'd get.  I literally waited until we were in with the technician in the 'casting room' before I mentioned that she was going to have to get a new one made.  She instantly started freaking out, screaming that she was scared, and was just completely hysterical!  :(  For those of you who are new to the blog, and Alyssa; although she doesn't feel "pain" like we do, anything hot or cold (to her) touching her skin is absolutely excruciating to her.  She has to bath at a certain temperature because of this, and cannot touch most surfaces with her bare legs.  Particularly metal surfaces.  
The casting table is essentially just a metal frame with a wooden plinth in the middle, which the kids sit on.  They are then suspended on tapes fastened to it, so that the plaster can be wrapped around them easily, but they also have their chin strapped to the top of the table, so that their spines are long.  It does look a bit like a medieval torture device, but her temperature sensitivity just makes it a horrific process for her.  And this time round, she knew what was about to happen!  First time, she didn't really get it.  It was absolutely awful, for her, myself, and my mum.  The orthotists this time round didn't understand what I was saying about her condition so they weren't as careful as the first orthotist and kept acting surprised when she was screaming when they touched her.  It seemed to go on forever, and it was just emotionally draining for all of us.  This time round, it cost me a magazine, a wispa, Irn Bru, and a trip to Smyths Toy Store .... where we got a £50 lego set!  But she definitely earned it that day.  And I earned my weight in chocolate!

That same morning, I became aware that she also had an ear infection because she'd asked me to go through in the middle night to "dry her ear."  The only time she's ever needed her ear "dried" was because it was full of pus!  And her ear practically closed over last time, requriring IV antibiotics again.  I also knew that I couldn't take her to the GP because they would have seen her, but not before we had to leave for the hospital for the casting.  So I was stuck between a rock and hard place, and just had to hope it wouldn't get too severe before I could see the GP the following day.  It progressed really quickly and - it sounds awful, I know it does - but I just couldn't face another trip to the hospital!  Even though I fully expected her to need IV's, I could have cried at the thought of being admitted again.  So I went to the GP, who gave her oral antibiotics and an ear spray (also "cold" so that was a nightmare, spraying it into her ear 4x daily!) and waited it out.  I still expected to have to take her up the following day but I was fairly sure that there was a tiny improvement by the end of the following day, so I decided to just monitor it at home.  Thankfully, there was a much more noticeable improvement by the next morning, so we got away with an oral course, for probably the first time ever!  

I think we got through a full week without a trip to A+E or the ward, and I took her to a little play park for the first time (and our first trip anywhere, 5 weeks into the summer holidays, because we'd had so many medical issues to deal with),  It was very Alyssa-friendly, and she had a great time (and even walked about more than usual, without too much prompting, because there were so many interesting things to see outside of the play park area.  We went back into the play park just before leaving and she tripped over a little border.  Nothing major, just a little trip - and I caught her - then thought nothing more of it.  Got home, and she started complaining that her ankle hurt, "like when the hand did."  I couldn't believe it.  I'm really pleased that she's starting to get some sensation, even if it's fleeting and random, because we at least know that she's done 'something' to a body part.  But she really had only tripped over.  In her orthotic boots, which support her ankles.  I figured she must have gone over on it, and maybe sprained it, so told her she needed to try and rest it (easier said than done with a child who doesn't feel pain - and she didn't feel it that often!), but the following night, she suddenly started screaming that it hurt.  Again, not often, but an actual scream.  So off we went to A+E again.  When your child who doesn't feel pain starts complaining of pain or something which "feels funny," you pay attention.  Fortunately, the A+E doctor we saw that morning was very knowledgeable about nerve damage and neuropathic pain, and immediately said that if she was feeling it, then there must be something wrong.  We had her ankle x-rayed, and sure enough, she had a (very minor) fracture, but on the opposite side of her ankle from where she felt it.  The doctor said that given her nerve issues, that wasn't surprising at all, and recommended a aircast (a wraparound cast, or "boot").  I already had one at home, so he arranged for her to have tubular bandage put on her ankle, and we were allowed to go home.  He also said that she should use it as soon as she was able to - within reason, given her lack of pain sensation generally.  I used the boot for the first day, but I was wary of it because I'm fairly sure that it caused the major break in her other leg, or at least added to the stress on that leg.  She was able to function pretty well with the tubigrip bandage, so I just left her with that.  

And that finally brings us to today!  Today was her first day without the suupport, and she's been using it fine, with no complaints (and more importantly, no swelling), so I think it's healed ok now.  We just have to get through another 2 1/2 weeks of 'holidays' without injury ..... I'm not holding my breath.  I will be VERY glad when school starts back!  

As always, thanks for reading.  If you've made it this far, you deserve a little medal of your own - or at least a couple of coffees!  I shall settle for chocolate and Coke!  ;-) 

She can always manage to smile, at least.  Well, if there's a camera about anyway!  This is her with one of her many camera poses!