What a year it's been ...

 So, as is pretty standard for us - despite how often I want to update more - it's been a full year since the last update.  I'm sure everyone was hit very hard with the pandemic, and although it wasn't as hard on us as it was for most people (we'd already been living like hermits due to all of Alyssa's surgeries and complications), there were still several factors which affected us.  Particularly with regards to her recovery and rehabilitation from her R hip removal.  

When Covid-19 really took off here in March, everything shut down, as with the majority of the world.  This unfortunately meant that only emergency clinic appointments could go ahead, and swimming pools were completely closed.  Frustrating for us with the timing of Alyssa's hip removal and her much-needed hydrotherapy but completely understandable, given the circumstances.  It just seemed so unfair that - after an 8 month battle to save her hip, with body casts, long periods of immobilisation and isolation, and multiple hospital admissions - she didn't even get a chance to rehabilitate once her surgical scar healed.  She literally got that one hydrotherapy session where she walked without having a hip and then the world fell apart.  For everyone this time though; not just for us, which is generally the norm.  She was able to manage some crawling in the house, though it was difficult for her after such a long time being immobilised, and she was one-sided because of the swelling on the R side, but she gave it a good try.  Unfortunately, she'd end up with good periods and bad periods, with the bad periods meaning she spent a great deal of time back in her bed.  The effort of even trying to crawl caused fatigue, and without having the pool to take her weight, it really knocked her energy levels.  She was still having issue with her leg swelling and being 'painful' (we believe the swelling causes pressure, which gives her whatever her version of pain is) so that would cause her to stay in bed, but being in bed just made it more difficult for her to crawl once she felt better again.  In short, a neverending vicious cycle of events.

Around July - just as everyone here who had been shielding from Covid was told they could start going out again, we discovered that Alyssa's immune system was not functioning properly, and looking back at previous blood results, had been affected since March.  Her white blood cell count (wbc) was abnormally low and we realised that she would be unable to fight infections even as well as she had previously, which was always slower than everyone else.  Lots of testing began, whilst keeping her as shielded as possible, as nobody wanted patients at hospital unless it was absolutely vital.  I also did not want her to be near any hospitals unless absolutely vital, especially once it became clear that she could not fight infections like she should.  Regardless of Covid, she has always been prone to cellulitis and required days of IV antibiotics to get rid of it (despite all of 2017-2020 admissions for the multiple leg issues, her main reason for admissions has been the recurring cellulitis episodes), so I didn't want her exposed to any more than she needed to be.  But obviously we also needed to investigate why her wbc's were so low, in order to try to correct it and hopefully prevent the need for shielding.  It turned out to be a long process (and we still don't actually have answers yet, the investigations are still ongoing due to aspects I'll cover here further on) but she was referred to haematology at Glasgow Sick Kids so that they could do more specialised testing.  In August, she had an ingrown toenail on her R foot, which didn't hurt her (obviously) but annoyed her ... so she basically just attacked it.  The joys of not feeling pain.  With having very little white blood cells, it became infected almost immediately and I had to have her admitted to the ward for IV antibiotics.  They were shocked by how quickly it had turned to cellulitis, even knowing her so well, and her history of it.  We stayed for around 5 days while the IV antibiotics did their job - with multiple cannula attempts due to the scarring in her poor veins, and her inability too look after cannulas once in her arms etc because she doesn't feel them when they're in.  We got 2 weeks of oral antibiotics and we were once again home.  For a little while.  In October, she had a thumbnail which was annoying her, and .... you guessed it, she just pulled it off.  Very quickly, it too was badly infected and the infection was spreading so I had to have her admitted again.  Pretty much the same process as with the toe ensued.  Both the doctors and I (and our families) had tried to explain to her that it was even more vital than ever that she not pick open wounds or cause new wounds because her body couldn't fight infections any longer, but if something doesn't hurt you then it's difficult to understand.  She'd simply get a sensation she didn't like and do whatever was required in order to stop it, then be happy once whatever was annoying her was no longer there.  Very frustrating for us, but undoubtedly for her too.

By November, her wbc's were still very low and we saw the haematologist for the first time.  He said that there were basically only two reasons for them to be so low: either her bone marrow wasn't producing them any longer, or it was, and something was "eating them up" (ie her body was attacking itself with an autoimmune disease or one/some of her medications were destroying them).  He told me that if she got a fever at all, or required a hospitalisation for any reason, he was to be contacted immediately as his protocol for antibiotics would be different to the standard one for something without low wbc's.  He booked her in to have bloods taken for a whole host of tests, including autoimmune antibody ones.  He said that the autoimmunity blood results would take a while but needed to arrive at the lab on a specific day so that they could be tested and the sample not be ruined.  She was booked in a couple of weeks later to have those bloods taken and he said he'd call with the regular ones once they were ready, and he expected the autoimmunity results around January.  He then noticed that she was a bit anaemic so he decided to correct that even though it was minimal, to give her body the best function possible, while we tried to figure out the cause of her low wbc's.  Eventually we discovered that the blood sample did not reach the lab in time for the deadline and the sample could not be tested (it was taken again last month and we are once again waiting for results of it).  We mostly plodded along in January with trying to have her at school safely, and not overtire her, so a plan was made that she would only attend one specific area of school and she would do all lessons from there.  Minimal staff and students would be allowed in, and only those absolutely necessary would be allowed in her personal space.  She was upset that she wasn't able to see her friends even once she went back to school but we explained that it was to keep her as safe as possible, and we couldn't risk her catching Covid, especially with her immune issues.  I in particular didn't think she would fight it very well, even though some of her doctors were still saying that they really weren't seeing kids with many issues from it.  I've done this for 14 years now however, and I know how her body responds to things better than anyone - including Alyssa herself.  She was already showing signs that she was potentially fighting a chest infection, so I was monitoring for that already.  I didn't need her near any bugs, particularly one which might affect her lungs.

Just as we were about to leave January behind, I got a phone call from Test & Trace to say that one of Alyssa's carers had tested positive for Covid.  There was obviously nothing I could do about it at that point - it had already happened - so I just prepared to watch for symptoms.  By the next day, I knew Alyssa was ill, and the headaches I'd been having since the night before were becoming more and more severe so I was pretty sure that I/we had it.  I really didn't want to have to take her to hospital without knowing one way or another, and I could tell very quickly that an admission was coming.  Her oxygen levels were starting to dip but not worryingly at that point so I arranged for home tests and we both did them on the 1st Feb but before we could even get results, she spiked a fever and I had to have her admitted to the children's ward, as per the haematologist's instructions.  I warned them immediately that there was a good chance it was Covid; that only the minimum number of staff should come near us, and we were admitted to an isolation unit within the ward where Alyssa was tested again.  They could see that she was having difficulty breathing so she was put on oxygen and then x-rayed in the unit via a mobile x-ray machine, where they could see a consolidation in one of her lungs.  It was presumed at that time to be a chest infection but once the Covid results came back as positive, the doctors decided that it was more likely to be a result of that and not actually a separate bacterial infection.  She was put on IV antibiotics anyway as per the haematologist's recommendations, and had several blood tests done to make sure nothing else was going on at the same time.  She was given steroids to help her lungs and she made a dramatic improvement within a couple of hours of the steroids being administered.  Her paediatrician was delighted at how quickly she responded and, after making sure her oxygen levels stayed up for 24 hours after she was taken off the oxygen, she was allowed to go home.  Sadly, although the steroids had caused her to improve, they had actually just masked the fact that her lungs weren't able to cope and within 24 hours, her oxygen levels were seriously low.  I had to take her back up again, where she was put on a higher level of oxygen, started back on IV antibiotics and steroids, and x-rayed again.  This time, the x-rays showed that both lungs were badly affected (it had only been a matter of days since the first x-rays!) and she was struggling to breathe.  

This escalated very quickly the following day and by teatime, the paediatricians were seriously concerned and contacted both ICU there, and also the Sick Kids hospitals, because they didn't know what else to do for her.  She had to be placed on a high-flow oxygen machine from ICU because her lungs weren't getting enough oxygen even with the amount of oxygen she was getting from the nasal cannula, and it didn't go up any higher.  The high-flow O2 machine, as it sounds, pumps oxygen into the body at a really high rate ... but it also then pumps any infection back out into the room, so the staff couldn't even turn it on without having to go back out and come back in with full personal protective equipment (PPE) on:  special filtration masks, visors, full-length gowns, and gloves covering the ends of the sleeves so no Covid particles could get inside their clothing.  It was very surreal seeing everyone in the level of protection gear you only really see in virus disaster movies, but it was also very understandable in the circumstances.  They switched on the machine and Alyssa was immediately upset because it was really overwhelming having so much oxygen fired at her, but she gradually adjusted to it, and the paediatricians began to discuss what the options were for her.  After going away for perhaps half an hour, they returned with members of the ICU team and a more detailed discussion took place.  The paediatricians said that they were worried that they could not provide Alyssa with the care she needed (they usually stabilise children with any serious condition and then those children are taken by ambulance to one of the Sick Kids' hospitals for more specialised care).  They had been in touch with both Sick Kids' hospitals and both were also wary of taking her because they felt they did not have much experience with Covid either, because they'd seen so little of it in children referred to them.  They felt that the adult ICU team where we were would have more experience, because they'd dealt with so much of it since the start of the pandemic.  The adult ICU team were concerned that Alyssa would not cope in ICU, especially as it would require her to stay by herself (she started getting distressed just at the thought of that) and that although she was technically over the weight limit of someone in their care, the equipment they had would most likely be too big for her.  A long discussion took place because in order to even send her to the Sick Kids' hospitals, they would have to sedate and intubate her as she could not be taken off the high-flow O2 machine, but it was too dangerous for everyone around her - including paramedics in an ambulance - to be near her whilst she was breathing the air out (and even that equipment was too big for her, and kept popping out of her nose).  Eventually it was decided that if she stabilised over the next 2 hours on the highest level of oxygen output, she would stay where she was, the ICU team were right downstairs and would be in charge of the machine and oxygen levels, the Sick Kids' hospital would be in charge of her treatment, and the paediatricians would ensure that she got whatever they needed her to get.  They were also worried that she had blood clots in her lungs and that she wasn't able to breath because of those, but they couldn't send her to CT because they couldn't take her off the machine - and she couldn't go whilst attached to it.  Fortunately, she began to stabilise, and she was calmer with the 'staying where she was' plan as well because she knows everyone there.  It's a bit of a second home to her, the staff are amazing, and it was definitely better for everyone if she was calm, so that she would breathe as normally as she could.  The decision was also made to just start her on blood thinners, just in case there were blood clots forming, which is a common complication with Covid-19.  As usual, access to her veins became an issue, and they couldn't get the IV drugs into her.  She ended up requiring a Central Line fitted, and she just lay there and tolerated it being put in, with her only comments being "He's pulling on my neck" once the anaesthetis was stitching it in place.  She didn't care about having it inserted, nor the fact that he was literally forcing the needle through her skin repeatedly ... just that he was pulling on her (that was him pulling the thread through her skin!).  

I was suffering severe headaches throughout this because I had Covid too, but I was nowhere near as ill as she was.  The sight of your child attached to a machine; literally the only thing keeping her breathing, is just something else entirely.  I don't think you can actually put into words what it feels like at the time; you just go with it, and hope that you get a good outcome.  I'm not sure I can put it into words even now.  I received a flood of messages; asking how she was, asking me to tell her they were thinking of her, and it was lovely to see so many people sending her love and well wishes but it was also really difficult to reply to them - whilst in the middle of it all.  I generally use Facebook to update people because it's the source I have most people on, and one post can pretty much let everyone know all at once.  Close family members were obviously being updated as soon as I could possibly manage, but there was so much going on at any time, the plan was changing constantly, and having such severe headaches meant I struggled to even look at my phone/ipad at times.  I know that it was very frustrating for everyone to not have as many updates as I usually post but I physically couldn't manage any more than I did; I was exhausted and trying to process what was happening.  I posted some pictures of her whilst she was attached to the machine because a lot of people didn't really understand what was going on, nor how ill someone could actually be with Covid.  People are so used to her being in hospital and always looking happy, regardless of the reason, so it was a real shock for them to see her so poorly.  She's so used to hospital life that it doesn't normally affect her much now (she actually quite likes it in hospital because she's spoiled by the nurses and play staff!) but this admission was definitely abnormal, and a major adjustment for her.  But, as she does, she accepted it, and adapted to the treatment, even though she hated it ... and kept removing the oxygen at any given time!  I asked her if she understood what it was doing for her and she said "Giving me air." ..... so I can't say she just didn't appreciate the severity.  It was a reminder that despite being 13 at the time, she was still very much a child.  'I don't like it so I shall remove it, even though I know I can't breathe properly without it' pretty much sums that up.  She kept saying that she hoped that she'd be home for her birthday (which was towards the end of Feb) and I had to just keep saying that it wouldn't be the first birthday she'd spent in hospital, and we weren't going home too early again.  I wanted to make sure she didn't suddenly deteriorate like she did the first time we went home!  She was attached to the machine for around 10 days before they started gradually reducing the amount of oxygen she was receiving, and eventually transferred back to the regular nasal cannula that she'd started off on, two weeks before.  She was monitored for another few days, and then taken off the oxygen completely, and we came home again after she had gone 24 hours without oxygen.  We were pleased to be home, and the cats and dog were pleased to see us!

We were home for about 3 days when she decided that a cannula site at her ankle was annoying her (the cannula had blown mid-infusion when she was receiving antibiotics, and a small lump was still there).  I reminded her that it would go away, just as the previous ones had.  She could not accept this, or couldn't wait for it to go away on it's on, or both; so she decided to gouge the lump out.  She literally dug a hole into her own ankle with her fingers overnight one night, and then announced to me that it was "wet."  She had been on the blood thinners right up until discharge so by the time I saw it, there was just a black hole 3cm x 4cm in her ankle, approximately 1cm deep!  I had to get oral antibiotics immediately - in an attempt to keep her out of hospital for her birthday and because I knew the paediatricians wouldn't be able to get a cannula into her at that point anyway.  They'd already had to have a Central Line put in, in order to give her the treatment for the Covid!  The GP gave me orals for her, we managed to ride out her birthday with the orals keeping the infection contained, and then I had to have her admitted once it began spreading again.  We spent another 5 days in hospital, after only being home for almost a week, and she was put on IV antibiotics again.  Fast forward to now, 3 months and many dressing changes later (3x a week), a biopsy to make sure there was nothing nasty there as well, and it is almost fully healed!  The level of destruction that girl can cause is just unreal.  

With that in mind, and because that issue is now almost resolved, she threw another eye injury at me this week!  There is always something going on with that child!  She told me she had something in her eye ... after 10 mins of rubbing at it, and I removed something tiny from her eye, which was already very red and inflamed. I put more of her eye ointment in it, and told her to close it and leave it alone so that no further damage could be done.  When I checked it the next morning, it looked okay, so I took her to school and advised them that she had potentially scratched her eye again, and what to watch for.  They called me mid-morning to say that she was rubbing at it and complaining that it was "burning."  A sure sign of a corneal abrasion in Alyssa. I managed to get an emergency eye care appointment for her that afternoon and just had to put more eye ointment in until I could get there, because I could see she was going to attack it (and we know how much damage she did to it last time!).  Fortunately, this time, I caught it early and kept her away from it and there is minor damage.  A "small pinhole" is on her cornea, which the consultant was pleased about, but decided that he was going "all out" with treatment, because it was Alyssa!  He was the same consultant on the night she practically blinded herself, and we weren't sure if her vision would return in that eye initially, so it was good luck that he was on.  We've come away with two different types of antibiotic drops/ointments and he'll see her again next week just to make sure it's healing well.  Obviously, if she starts to go at it again, she'll be seen sooner but so far, the ointment is helping and she's left alone.  

From an immune system perspective at this time:  the Covid ironically helped her once she got over it, thanks to the many teams of doctors/medics involved, because the steroids helped her wbc's to increase very suddenly, and significantly.  So she came away with a bit more protection than she'd had for the previous year.  Having had Covid, she also had a bit of armour against that for a time too, so her ankle has healed slowly but well, and she hasn't had any more infections since February.  Her levels are dropping again now, and her Covid armous is most likely slipping now too, with no vaccine available for under 16's here yet, so she's still shielding.  But she is very lucky to be here!  And she wouldn't be, without the amazing care of the paediatricians, nurses and ICU team at FVRH, and the haematology and infectious disease specialists at Glasgow Sick Kids' hospital.  The paediatricians at FVRH deserve a special mention because they fought for her at every stage, with very little knowledge or experience of Covid at that point (and never having seen any child become sick like she did), but they went out of their way to make sure they found out anything and everything which could possibly help her.  They were shocked and horrified to see how ill she was, and they had to learn about Covid and it's many complications at high speed, and without any warning.  They truly are a special bunch of people, as are the nurses and play leaders.  

If you've managed to read to this point, you're probably one of the people who sent us love and well-wishes during the Covid period (and most likely at every other admission too!) so I'd also like to say a long overdue huge thank you to you as well.  The level of love and support Alyssa receives from so many people in so many walks of life is just mindblowing, and very much appreciated.  Here's to hoping the rest of 2021 is uneventful for us.  It's highly unlikely, but we can hope!  

Phew! Made it by the deadline ... (3 of 3)

Brief summary for those skipping posts 1 and 2:  Alyssa had a couple of surgeries after the initial  planned one, was immobilised for many months (much longer than ever anticipated, and more than the once expected), and had a knee which started dislocating due to the extended period of immobilisation, and her thigh muscles weakening.  She'd broken her arm and had a cast on so wasn't able to get into hydrotherapy for a period, but we eventually got her progressing once again, and back on to her feet.  

Year 3:  May 2019 - May 2020 (this will be the longest one ...)

Around the beginning of May last year, just as we were preparing for Alyssa to start walking a little bit at school (closely supervised, of course), she started feeling the "shocks" again, which she's had in the past.  They are believed to be a type of 'breakthrough' nerve pain, although obviously nobody really knows why she feels them, or how to prevent them.  However, she always gets them when she has a fever, and they have increased in frequency before when there has been an orthopaedic injury, so they are - whilst very uncomfortable for her - somewhat of a warning system for me, that something is wrong with her.  Somewhere.

The shocks kept increasing in frequency throughout May, mostly at night, until she was often screaming and just 'twitching' constantly, so it was very obvious that something was wrong, but unfortunately not what that something was.  It went on for about 3 weeks but I was stuck in a "What do I do?" scenario as, without knowing what was wrong with her, meant I didn't know who to contact, to try and help her.  As you all know, she has multiple specialists, all of whom are excellent, but I need to have an idea of where to start before contacting them.  If I don't know what is going on with her, even the best doctor will never be able to work it out.  Something I'm sure which is common in those with rare and complex conditions.  

On the 29th May, I turned up to collect Alyssa from school and her aide informed me that Alyssa had had a little fall (she actually hadn't fallen but literally gone down onto her knees, leaning on the zimmer, whilst transferring to the toilet).  I stopped her and asked Alyssa if there were any injuries I needed to be aware of, which surprised her aide because she clearly hadn't thought of it as a fall.  Rightly so, but the school were still very new to Alyssa and her not-always-obvious injuries.  Alyssa said her leg felt "thick," which immediately made me concerned, as "heavy" or "thick" are Alyssa's words for the feeling she has when her legs or arms are swelling.  I took her home and examined her leg, and her R thigh seemed a little swollen, but it was very difficult to tell for sure, due to the previous surgeries and metalwork in both hips, and her gaining weight from being immobilised for so long.   As the evening progressed, however, it became very clear that her thigh was swelling, and she started getting more and more shocks until she was literally just screaming.  I had no choice but to take her to A+E, and have her x-rayed.  There, we discovered that she had fractured her R femur, in the middle of where the existing femoral plate was, and the plate had essentially been holding her leg together.  I knew (finally) what had been wrong with her for weeks, now that her leg was swollen and we could see where the problem was.  She was the most uncomfortable I'd ever seen her up until that point, and actually crying with pain.  Her leg was so swollen that she was in agony, and the A+E doctor had to give her oral morphine, to try and ease the pain she was feeling.  And then he asked what I wanted to do, regarding getting her leg fixed.  He contacted the on-call orthopaedic doctor there for advice, and I told him directly that nobody there was touching her, and we were going to Edinburgh.  After all the complications she had with the L leg, with a doctor who actually listened to me, I wasn't about to even contemplate how badly things would go wrong locally, with doctors I've already met, and battled with.

We were sent via ambulance in the middle of the night to Edinburgh, and saw the ortho team there.  Her surgeon came to see us the next morning and - after asking what had happened and me explaining what had happened at school - said he'd operate that afternoon, and repair it.  But because the plate had already been fixed to her femur for 2 years, it was going to be difficult to remove, before a new plate could be attached.  He still seemed a bit suspicious that the school weren't being completely honest about the 'fall' but I assured him that I'd known for at least 3 weeks that something was wrong with Alyssa, I just didn't know what, or where to look.  And that I'd also asked Alyssa what happened, and she said herself that she actually tripped getting out of her chair because her leg felt thick, which meant that her leg was already swelling when she got her leg stuck in her chair and went down onto her knees, and the injury was there first.  He returned from theatre saying that the operation had gone well, and that I was right about the length of time Alyssa's leg had been broken, because there was new bone formation at the fracture site - which takes at least 3-4 weeks to occur.  It explained why she'd been getting all the shocks, and I was happy we finally knew what was wrong with her.  She was, once again, barred from weight-bearing, although we were pretty much professionals at the immobilisation/housebound/post-op swelling period by this point.  She was unhappy for a good few weeks (due to the swelling, not the fact that, once again, she'd just had major orthopaedic surgery!), but things started to improve as the swelling reduced.  

Alyssa was seen and x-rayed 2 weeks later - ortho was taking no chances by this point - and things looked good.  There wasn't much healing progress, but we hadn't expected much, so soon after the operation.  We went back again around 3 weeks later, and her ortho was probably the happiest I've ever seen him when it came to Alyssa's x-rays (he'd previously told us that he can't eat on the days he knows she's coming in, because he feels nauseous until he sees the x-rays and knows everything is ok).  He was grinning about how well it was healing, and thought we could go a bit longer before the next set, which I was pleased about, but also worried about.  Things don't generally tend to go to plan for us.  However, I let everyone know that her leg was healing well, and that the ortho was very pleased, and we carried on.  Around 2 weeks later, at the beginning of August, she started experiencing the shocks again.  As before, I didn't know what was causing them to suddenly start up and then begin increasing in intensity and frequency again.  She had no fever, was otherwise well, and we'd just had the x-rays done, which showed how well her leg was healing, so the thought that something could be wrong with her leg didn't even occur to me at this point.  I checked for cellulitis in her various places and found nothing, she had no chest infection symptoms, so I was genuinely out of ideas.  I contacted her neuro, and we discussed it all, and he wondered if her condition was just progressing, and she needed to have her nerve pain medications adjusted.  We started a regimen to increase one of her medications slowly, and I hoped that the increase would help as quickly as it had the previous time she'd had it increased.  Unfortunately, they got worse, and - just as I was feeling like it was exactly like when I discovered Alyssa's leg was broken - her R thigh started swelling again, and the screaming became unbearable.  This time, I headed straight for Edinburgh, instead of going to our local hospital first.  She was admitted immediately (all of the ortho team knew her very well by this point!) and she had x-rays done ... which showed that the metalwork in her leg was all coming away.  They didn't know why, so she had bloods taken, and was sent for an ultrasound, which showed a huge amount of fluid at the fracture site.  They went ahead and drained 40ml of fluid from the site, which didn't look infected, but was sent for testing anyway.  Then we got her blood results back, and it was clear she had an infection.  She still had no fever, redness, or any other signs of infection, despite the fluid build-up and her blood results.

Upon admission to the ward, we were told that her ortho was away that week, although he always wanted advised whenever Alyssa was seen/admitted/treated in any way, so they would contact him, but they would just follow his plan.  Nobody wanted to interfere with her care, after everything he'd already done, and him knowing her best.  A plan was made to immobilise her leg completely by putting her back in another hip spica (full body cast), but because they couldn't see her leg, and needed to be able to assess it, until her blood results started looking better, she was put into traction instead.  Something which proved to be the hardest thing she's ever experienced, but one we had no choice about.  Her ortho actually came in to see us even though he was supposed to be away, and was clearly devastated about the situation.  He explained that he couldn't go in to operate because the bone was so soft due to the infection that her entire hip would collapse, and the metalwork would just pull away completely.  He needed her into the body cast as soon as possible, in an attempt to keep her leg so still that he could get the bone to still heal.  He said that they'd had good results in getting fractures to heal even where infected, as long as the patient was on constant targeted antibiotics, throughout the healing process, and he really hoped that it would prove to be the case for Alyssa.  It was at this point that he dropped the bombshell that if it didn't, she might lose her hip completely.  

I have to be honest and say I was stunned by that for a moment.  I think I deal with everything we have thrown at us pretty well, but that was one of those moments, which just blow your mind.  With everything we'd already been through in the 2 years prior to this, it never occurred to me for a second that we might end up with her losing a hip.  And not even the hip which started all of this!  It was genuinely gutting.  However, we had to get on with whatever we could, to try and save her hip.  She was in traction for almost a week - with me being very concerned about her getting pressure sores and not even feeling them - and then she suddenly started screaming again.  I knew she couldn't be in the traction for another weekend without it causing problems and, although we never thought we'd ever actually want her to be in a body cast again, found ourselves asking for it to be put on.  Alyssa got to the stage she was practically begging for it to be put on, just to get out of the traction.  It really was a horrific experience for her, and unfortunately, did indeed cause her to have big welts underneath her, which nobody could see until the traction was removed.  Fortunately, they healed quickly and, as her blood results had improved faster than the doctors had expected, it was agreed that she would get the spica on, and we could go home once she was comfortable in it again.  

Alyssa was x-rayed practically every 2 weeks from that period on (we were already in August when she was admitted), so the ortho could see if there was any bone growth at her hip.  She was actually fairly settled by this point, already.  She was frustrated about having to be in yet another cast and unable to do most things again, but she wasn't getting any shocks, although she was getting muscle spasms from the cast etc.  This continued until early October, by which point, her ortho was more and more sure that the hip wasn't healing.  Something he was devastated about, as he was optimistic at every single stage, and absolutely determined that if he could save it, he would.  He ordered a CT scan of her hip and femur on the day where he'd resigned himself that it wasn't healing, and she was booked in to have the cast off, and the metalwork - and hip - removed for the following week.  We prepared Alyssa for the fact that she was losing her hip, and what that would mean for her (she'd be unlikely to walk again, but she also wouldn't keep getting these shocks, and she'd be healthy again).  She went through periods of being upset that she wouldn't be able to walk again, then happy that she'd have the cast off.  I warned her on the day of the cast removal that we hadn't had the CT results back yet and that if there was any possible chance of still getting her hip to heal, and it meant another cast, then we would be doing that.  That nobody wanted it for her, but the longer term plan was our goal, and nobody wanted to have to resort to something so drastic.  She didn't really understand the implications of having her hip removed, despite our chats about it, but she accepted that another cast was going to happen if it meant another chance to save her hip.

On the day, her ortho came through to the ward and said he was pleasantly surprised that the CT had actually shown more positive results than he had expected.  And he was hoping to give her hip another chance, but putting another cast on.  Alyssa was immediately upset by this, but I reminded her that we'd already discussed it, and I'd told her that we needed to at least try.  So she accepted that as well.  Poor kid.  Always having to deal with the lesser of two awful decisions.  We came home, and life went back to our normal.  She got everyone to sign her cast, which cheered her up - especially once it was in "rainbow colours," and we waited to see what would happen.  Mid-November, she started getting the shocks again, all of a sudden.  I was instantly concerned that the infection was spreading again, after so many months on the antibiotics, and she was seen as an emergency.  She had more bloods done, and more x-rays, then another CT scan.  Her bloods showed that the infection still seemed to be under control, but the x-rays still showed very little improvement.  We discussed how uncomfortable she now was in her cast and decided it was time to have it removed.  She'd been originally scheduled to have the hip and metalwork removed on the 28th November, when the cast should have come off in October, so we decided to take the cast off her, and see what happened.  If everything went well, the op would just be cancelled.  If not, the date was at least there for the surgery to go ahead.  

 On the 18th November, she got the cast taken off.  Within 3 days, her leg had swelled up again and she was screaming (bearing in mind that she still wasn't weight-bearing, just in her wheelchair to get home, and then in her bed).  I tried to ride it out initially, but she was just following the same pattern as previously, and I knew her leg was just going to swell and swell until it was unbearable for her.  So back we went again, and she was admitted once more for x-rays, bloods and pain medication for the swelling.  She then came down with a tummy bug, which proved to be a highly infections norovirus, and she was really poorly with that during her stay.  Her ortho had been away that week, so only contactable by phone, and came to see her when he returned.  She was a bit dehydrated, and had stayed so still because of the norovirus, that by the time he came to see her, she looked much better.  She was happier, and the swelling was much improved.  I honestly couldn't fault him thinking that it was better to leave things conservatively, despite him being prepared to come back in that day and have to operate on her.  We came home, and her leg started to swell again.  This time, I left it for an extra day or two.  Hoping it was just going to be something which we were just going to have to ride out until it went down again, but we only got to that weekend before I had no choice again.  Back we went once again, and she had further x-rays.  None really showing any improvement in bone healing, but also not showing that her hip had collapsed.  We were in limbo because there was no obvious course of action.  We finally decided the metalwork which had come partly out in August when the infection kicked off, was digging into her thigh muscles now that the cast was off and she was more mobile in her bed.  That it was this which was irritating her thigh and causing the swelling.  Her ortho really didn't want to go in and remove her hip because he could still see healing going on, and I wasn't prepared to leave her like she was, so we compromised.  He went back in on the 28th November, and removed the screws which were sticking out, and replaced them with shorter ones.  The following day, she was bouncing!  So much so that he jokingly asked if he could go back in and put the dodgy metalwork back in, just to keep her still!  I laughed and said "But this is how she should have been during all of this.  I should be at home, trying to keep her from doing things to herself.  It's our normal."  He was clearly nervous, but we had to carry on and see what happened, and her hip got another execution stay.

Things went really well for about 10 days, and then she developed a completely new symptom - muscle jerking / tremoring.  It started with her saying her leg was twitching, but nobody able to see anything, and progressed into something which was very visible.  Her whole leg would jump repeatedly, and it started to swell again.  I called the ortho team and they advised to take her locally and have her x-rayed - to save us the longer journey back there again - and they would look at the x-rays from there once they were done.  Nothing obvious could be found, and it was queried if it was actually neurological.  I was then stuck between taking her back to Edinburgh, or taking her to Glasgow, where we see her neurologist, because I'd have had to start from the beginning with everything that had been going on with her legs etc.  A bit like I'm doing here, and you can see how long that is!  Eventually it was decided that if things didn't settle down, I was just to take her to Edinburgh, and they would admit her.  If it was felt to be neurological, they could at least try to make her comfortable, and contact neuro themselves.  Things didn't settle down, and we went back into the ward again.  

I should really point out here that the ward staff at Edinburgh Sick Kids, and the entire orthopaedic team, are simply amazing!  Just brilliant, caring individuals who are always delighted to see us, although sad that we're there.  Unlike previous experience in other hospitals, the ortho doctors listen to everything I have to say, and we make decisions together.  I've never felt the need to fight doctors there, in order to get what I know she needs, because it's just a given that I know what I'm talking about.  There is no-one like Alyssa, and they all know it.  And that I am the only expert there is, when it comes to her.  

We were in mid-December by this point, and I was becoming exhausted.  As was Alyssa.  Which wasn't just so bad, because she could sleep during the journeys back and forth to hospital.  I obviously could not.  We ended up in hospital repeatedly the rest of the month, until she was put onto a slow-release form of morphine, and had her diazepam increased.  Bizarrely, the diazepam - which should ease muslce spasms - did nothing for the tremoring, and the morphine - which should ease the pain of the tremors but not stop them - stopped them.  Her nervous system really does not work like everyone else's.  And that made the pain team's job really difficult.  They kept upping medications which would provide pain relief in most people, but didn't touch her version of pain.  And they were really upset that they couldn't make her comfortable.  However, once the increased diazepam and the slow-release morphine were properly in her system, and she was a bit more settled, we went home again.  Due to see her ortho on the 27th December, and hoping we'd make it through until then, without yet another hospital stay over the Christmas period.  We had a very close call because she worsened again just as we got to Christmas Eve, but I managed to get hold of her GP, who gave me enough increased meds to do me until we saw the ortho on the 27th Dec.  And we stayed home, for Christmas.  I've never been so happy to have a 'boring' Christmas, because it at least meant we were at home!  On the 27th, she was doing relatively well on the increased meds, so her ortho prescribed her more and said he still thought the hip was healing, albeit slowly.  I reiterated my previous concerns in November about us needing to draw a line because she was now in limbo.  We were so busy trying to save her hip that she wasn't able to do anything, and the irony would be that if she lost the hip, she could go back to doing whatever she ended up capable of doing.  I hadn't even let her sit on the floor until Christmas Day - from the previous May - because I was so scared about what she would try to do, and he said that we were getting to a point where she'd need to start testing it out.  We agreed on another x-ray 4 weeks later, and a reassessment at that point.  So towards the end of January, she was x-rayed again.  No huge progress showing, but we agreed that it was time for her to start doing things.  "Carefully."  As if she ever does anything carefully!  

Alyssa was very excited about this and immediately wanted to start crawling.  I was terrified that she'd try to just take off, as soon as she reached the floor, but her thigh was so swollen that she actually couldn't.  She ended up R-side heavy, and was almost falling over whilst trying to crawl, and I then realised that her poor hip wasn't even going to get a fair trial, because it was also going to be road-tested at an angle.  She had to stop 3 times just to make it along the hallway, because it was so tiring for her, which at least meant she couldn't overdo it.  In the end, she only managed to crawl perhaps 3 times that whole week, with recovery days in-between, in her bed.  Her leg would swell and she'd start screaming, then it would go down a bit and she'd want to do some stuff, then the whole process would repeat itself again.  I warned her that this might be something which we were just going to have to ride out, because her hip hadn't done anything for a full 8 months now, and it would most likely be irritated and swell when she used it.  However, that nagging feeling of 'not knowing for sure without an xray' was at the back of my mind, and I could only stand it for a day or two before having to take her back, and find out for sure.  The x-rays that day showed the metalwork had just pulled straight out of her leg, and that was why her leg was swelling again.  Disappointing, but at least the decision was finally made for us.  Everyone was so upset for us, especially "after all those months and procedures," but to be honest, I was glad we'd done what we had.  We'll always be sure that we tried absolutely everything that we possibly could, in order to give that hip a chance of recovery, and we'll never have to look back and regret not trying.  Alyssa was initially a bit disappointed at the news because she "wanted to walk again," but I reminded her that she is unique.  That she walked about on a fully dislocated hip for 4 months before we even realised, and then for a year in total, before the first hip operation.  And that she's also walked about on fractures, which nobody else would have been able to do.  So there were no guarantees that she would never walk again, once her muscles had time to strengthen.  She accepted that too.  

On the 6th February, she had her R femoral head and part of her R femur removed, and the whole inside of her leg cleaned out.  Her ortho came back from theatre, very surprised, and said that despite the x-rays consistently looking good, and her blood results looking excellent, her hip was just mush.  The bone was so soft and infected, and it never would have healed.  So we could have still been going through all of this now.  As it is, she's home, and making progress that none of the ortho team could have predicted.  She managed to walk in the hydrotherapy pool on her very first attempt back - just blowing the minds of myself and my mum, and Alyssa's physio - and she's been managing to stand at home, for up to a minute.  She would obviously like to start trying to walk already, but we're not letting her until she strengthens her L leg much more.  There's now approximately a 3cm leg length difference, which affects her ability to stand, and her balance, but there are options out there once she's stronger.  And when we're not in lockdown during the current pandemic!  However, the lockdown has proved to be the longest period that Alyssa has ever been out of / away from hospital in her whole life.  I'm so thankful that it didn't kick off during the months of November to February, because that would have added even more stress to what was already an absolute hell to live through.  She's currently having phone and video consultations, which have proved interesting, and she's managed to finally 'meet' other kids around the world who have similar pain insensitivity issues to herself, via Zoom!  

I'd love to say that everything is fabulous, and of course we've had a great run, for which I'm very grateful.  However, something is going on again.  And I have no idea what, at this point.  I have the familiar nagging feeling once that it's going to end in a hospital admission, because we're already experiencing the increasing shocks each night, but I don't even know who that will involve, nor which hospital, because I don't know the reason for them yet.  Here's hoping it's something a bit easier than the last 3 years.  I think we deserve it!

Thanks to everyone who made it to the end of this. I know it must feel like you've aged since starting reading the update.  I know I feel like that just from putting it together, and reliving some of it.  But it's worth it, to know that so many people care about Alyssa, and want to keep up with how life is going for her.  Here's a picture of her standing and - absolutely deservedly - being very proud of herself!  Not bad for a kid who only has one hip, and a shortened leg, whom nobody thought would ever walk again.  Never say never!  😊

*Something I forgot to add is the lack of symptoms she displayed with what was clearly a very severe infection.  At no time did she spike a fever, or seem unwell, and the infection markers in her blood were very high.  The typical thing every doctor looks for when determining if someone is ill or not.  If she hadn't started getting the increased shocks, which built and built, I never would have known there was anything wrong with her, until she was septic once again.  That is probably the scariest and most dangerous part of this condition.  To never really know when there's something wrong with her until she's severely ill, and at the point of collapse.  There are usually signs once she's past a certain point (blue lips, ghostly pale skin, and shocks in her legs) but by that time, she should already be in hospital.  Even then, knowing that there's infection somewhere but not where is very frustrating.  As cruel as it sounds, I'd rather have the increasing shocks as a warning system than her not having any symptoms at all.  They'll keep her alive longer!

Phew! Made it by the deadline .... (2 of 3)

For those of you who've skipped to post 2; she had to have a couple of surgeries after the initial hip one - another one to her L hip/leg, and one to repair her L ulna/elbow.  And was immobilised for the majority of the year following the first surgery.  I'm making this post cover from Oct 2018 - May 2019, because so much of the last year's hell started in May.  (And I'm just getting it in before June this year!)

Year 2:  October 2018 -  May 2019

After Alyssa's L arm fracture was finally resolved - and I'd had her surgeon from Edinburgh check it for me, due to the conflicting information I was given each time I went to our local hospital ortho team - she was able to start getting about again.  Her physio and I were worried that she would lost muscle and strength again after having to be immobilised yet again, for the 3 months it took to repair her arm properly (For anyone who didn't read post 1:  she was using all her weight on her arm, on the zimmer frame, therefore we had to ban her from walking again - as she couldn't walk without it).  What I didn't add to post 1 was that, right in the middle of the arm issues, we realised that her L leg was starting to give her new issues again.  Her leg was "clunking" and we couldn't just feel it when we were holding her leg, but see it happening, as things progressed.  We were very concerned that something new was going on with the L hip/femoral plate, after all the complications she'd already had with it, and I had to arrange to have her seen again by the orthopaedic team.  Eventually, after many more x-rays and examinations, it was determined to be most likely her knee, and due to her thigh muscles wasting because of the extended immobilisation period.  Her bones were also thinning, which was obvious on x-ray, and the only thing we could do was wait for the arm nightmare to be over.  She wasn't strong enough to weight-bear on land ... but we couldn't take her into hydrotherapy because of the cast on her arm!  It eventually came off in September, though another one had to go on for a couple of weeks, because her arm kept swelling (she'd already had a couple of changes but this was supposed to be it off for good.  She was distraught with each and every cast change because the saw vibrations are excruciating to her, and the cast material "burns" her as it heats up, to become solid.  Each lovely member of staff is trained to tell children not to worry, that the saw won't hurt, and it's just noisy, "like a big hoover," but that just taught Alyssa to be even more fearful.  She knew it was going to hurt her; she's had to many casts, so she just felt they were lying to her.  I had to keep asking them not to tell her that, because she knew it wasn't true, and she was starting to panic about anything else they told her.

We got her back to hydrotherapy as soon as the cast was off, and we were relieved to discover that her muscles weren't quite as weak as we'd feared.  She was obviously not safe to allow full weight-bearing, but we did a block of hydro, and then had her start crawling and doing kneeling exercises again.  By the beginning of December, she was able to stand independently again, and began shuffling around the house again.  She wanted to try walking outside but a: it was too cold for her to be out, and b: we couldn't risk her falling outside.  Indoors, we could hoist her if she went down too quickly, or fell.  Outdoors was much riskier, we couldn't hoist her, and she was much too heavy to lift.  So she made the most of being up and about again, and gradually got stronger and stronger.  Unfortunately, as soon as she was crawling about and spending more time on the floor, it wasn't long before the cellulitis kicked in again.  But before she needed admitted for that, she developed another bout of pneumonia.  Despite my best efforts to get her oral antibiotics to keep her out of hospital until at least Boxing Day, she had to be admitted on Christmas Eve ... and we didn't come home until the 5th of January.  Well, we had a brief stopover home on New Year's Eve into New Year's Day, when they ran out of veins and 'hoped' they could get away with sending her home on oral antibiotics, but predictably, she had to be re-admitted for another few days of IV antibiotics before we could come home and stay home.  She had another couple of weeks of oral antibiotics at home - because of her previous history of septicaemia from infections that everyone thinks has gone away - and she seemed to recover fairly well from it all.  

We plodded along for a couple of months with relatively little issues (about time, we thought!) and she even started transferring from her wheelchair to her school chair for little periods of time, once everyone around her was trained, and confident enough to assist her.  Needless to say, the school staff were understandably terrified of anything happening to her, especially as she literally started school there with the broken arm!  They'd been told about her issues and her not feeling pain, but it's one thing to know, and another altogether to witness.  But they were troopers and gradually got more confident about her moving about.  Her physio and I decided in April that it was time to perhaps let her start trying to walk around at school - not in the mainstream area - but in a nice-sized room which she spends a lot of her school time in anyway.  And not when it was full of other children, whom she might bump into, or just fall over.  We made sure we had everything in place at the beginning of May, and decided we'd go for it after the May holidays.  Alyssa's physio was planning to go in on the first day and just give the staff any help or encouragement they needed, but Alyssa ended up with what appeared to be a tummy bug, so in the end it was cancelled.  I think we were probably thinking that finally we were making the progress that we'd expected, many months before.  Sadly, as happens all too often in our lives, things were just about to get much much worse than they had before...  

More to follow in post 3... 

Phew! Made it by the deadline.... (1 of 3)

Ok, so the deadline was pretty much set by me, and ended up being 3 years.  
3... years.  
That seems insane.  And yet, here we are:

To those of you who've followed our story for a long time, or who see posts on Facebook, you'll know that "no news is good news" has never applied to our lives.  No news generally means things are not going well, and I either don't physically have time to update, or mentally just can't face it.  Both of those scenarios have occurred repeatedly over the last 3 years, in what has felt like an eternity at times.

So, as it would be a ridiculously long post to try and update 3 years worth, even in summary, I shall attempt to break it down into 'years.'  That way, you can get as much or as little detail as you like, and can skim to the last year, for a quicker catch-up, if you wish

Year 1 - June 2017 - October 2018:

Alyssa's bilateral hip reconstruction took place in June of 2017 and the operation went well; her surgeon was very pleased by the outcome of it.  Alyssa went into a full body cast, as we had previously discussed, for almost 6 weeks.  I won't even pretend that it was fun, or easy, in any way.  Alyssa adapted to it really quickly, far more quickly than the rest of us.  But it was awful.  There is just no way to keep a cast clean which is from ankles to ribs, and despite our best efforts, it was a state when it finally came off.  Fortunately, her skin wasn't as badly damaged as we thought it would be, and she coped relatively well.  When it came off, however, she was as itchy as we expected, but she also had new sensation issues which meant anything bumping into the bed, or the safety bars banging down when lowered would send her screaming.  Definitely not something we were used to.  And something which hung about for a few weeks, although it felt like longer.  It did gradually improve.  

Her physio was involved throughout, as always, and started Alyssa with strengthening exercises as soon as Alyssa was able to tolerate having her legs touched (sensation issues, not pain, as such).  Alyssa progressed well, with us almost immediately having to restrict her, because she we knew she would do too much, having no post-operative pain, only new sensory ones to deal with.  Things seemed to go well until the middle of August, when Alyssa thought it would be a good idea to try and pull herself up into a chair.  She managed.  The first time.  Then she decided to do it again, and got stuck.  With all of her weight on top of her L leg, which was trapped underneath her.  I managed to get her out, and she still seemed fine ... until a few hours later, when I was notified by her carers that her thigh was swelling.  And then she started to scream!  

Alyssa screaming means something is very wrong.  And that episode became the first of what was to become many times where we realised she was now feeling 'pain' - or her equivalent - when things were swollen.  (Whether it was from the pressure, or whether it's something to do with an orthopaedically-related swelling is still unclear, as she has since had cellulitis again, and been unconcerned about the size of her arm).  I rode out the evening with her screaming intermittently, because her condition is so complicated that taking her to hospital every time would mean being there almost every week.  I need to have a rough idea of what's going on, before I even attempt to have her examined, because doctors have very little to go on.  They're usually looking to assess a pain response, which she doesn't usually have.  And even when she does, it's not a typical pain response, and can't be relied upon.  As she continued to worsen, and the "shocks" she feels when something is wrong increased dramatically, I knew she had done something to herself, so we headed to A+E to figure out what was going on.  

The titanium plate in her L leg had snapped, and the sharp ends were digging into her thigh.  One of the screws had already come loose and was tracking up her leg, and another looked unstable.  It seemed unlikely that she could have snapped the plate from just getting stuck in a chair, but it was the only thing she had done, as she was still not able to weight-bear, and wasn't left unsupervised for more than a couple of minutes at a time.  The doctors contacted her surgeon at Edinburgh, who called me to ask if Alyssa had fallen, and I explained that all she had done was try to pull herself into a chair, gotten stuck, and then her thigh had started swelling.  He didn't seem convinced, but agreed that she needed to be seen as an emergency, and we were seen by him a few days later.  He appeared to have convinced himself that her leg might still heal as it was, because it had been almost 12 weeks since her surgery, and that would normally be long enough for a decent amount of bone tissue to have formed.  I wasn't convinced this time.  He still didn't really understand the difficulties caused by her pain insensitivity at this stage, though sadly, that was about to be the start of a nightmare for us all.  By the time he x-rayed her, just a few days after she'd been x-rayed locally - and despite not moving out of her bed the entire time - another screw had come loose, and tracked up her leg, and the plate edges were separating further.  He was very shocked, but still seemed to think that the situation might still correct itself.  And so began almost 2 weeks of debating about whether to go in and remove the broken plate.  I was getting frustrated because I knew that wouldn't happen (too many years of experience) and because she could literally do nothing.  She had less quality of life than when she'd been in the body cast!  He agreed to operate, and initially mentioned just removing the plate and leaving her leg as it was, which really freaked me out.  He still didn't understand that - in a child like Alyssa - you have to go overboard to protect her - because she won't protect herself.  I knew that she would just try to get up on that leg again, and do more damage, before we inevitably had to go back in and again, and have a worse state to repair.  I reminded him that, whilst the x-rays may have looked good - the soft tissue damage wouldn't be obvious on x-ray, and it was simply impossible that a snapped piece of metal wasn't causing constant damage to the muscle in her thigh.  The sharp ends were having to rotate into her thigh every time we rolled her in bed, which we had to do.  So he ordered a special new plate (he didn't see the point in putting a similar one in again) and we prepared for emergency surgery.

To say that the poor man was traumatised when he returned from theatre would be an understatement.  I've never seen a doctor so shocked, and I've seen many taken completely by surprise at what Alyssa has managed to do to herself, and carry on.  He looked absolutely horrified and told us that when he opened her leg, it "looked like an explosion had gone off inside."  I didn't have the heart to even add anything to it, because it was clear that he had finally gotten the message that Alyssa is like no-one else.  And the pain insensivitity affects not just the injury itself, but the healing process and recovery process.  The rep from the company who makes the plates had also been present during the operation and simply said he couldn't understand how the plate could have snapped, or how the screws could have come loose, because they 'lock' into the plate, as they are screwed in.  What baffled them more was that she'd managed to snap a titanium plate, but her femur was still intact.  So she literally snapped titanium and not her bones!  Wonder Woman, eat your heart out!  She was given a strict immobilisation plan (no weight-bearing at all for a minimum of 12 weeks) and he saw her at least monthly up until we reached the 12 week mark, but she still didn't manage to weight-bear for almost 8 months from the date of her initial surgery; the swelling in her leg was just too much for her to lift her leg.  For whatever reason, her body now swells beyond what would be considered 'normal' with any injury, and I believe it is her body's attempt to protect itself from her.  The rest of us would naturally avoid using a limb which was injured because pain would limit our ability to do so.  With her not having that seemingly annoying sensation, her body is left with little defence, and swells to the point where she can no longer use that limb.  Because she had begun to feel pain from swelling meant that, for the first time ever, we had to deal with 'pain' on a regular basis.  This by itself overwhelmed her because it was so unnatural to her, and her brain couldn't cope, and neither could she.  She was very good at putting on an appearance when people visited her, or her brain was distracted a lot of the time because of it, but she became very emotional a lot of the time.  

We made it to March of 2018 with slow progression from being allowed to crawl, to then managing to stand (with the aid of a standing frame, initially) and by the beginning of May, she was managing to shuffle around the house with a zimmer frame.  Which was actually really cute, because none of us had ever seen one so tiny!  She became more and more independent as the weeks went on, and we had to begin restricting her again, as her confidence grew.  Unfortunately, despite telling her to wait for me to help her, she tried to get out of bed by herself, slipped, and fell out of bed - taking my laptop table and laptop with her.  I was literally in the same room as her and she couldn't wait the 30 seconds or so it would have taken me to get out from the table and help her.  She immediately shouted that she "was fine!" ... and appeared to be so, until 4 days later, when her L elbow began to swell.  Turns out that she had smacked the underside of her L arm on the table when she fell out of bed, and snapped her ulna!  She had a half plaster/half bandage cast put on at A+E that Saturday and I was told that it was to be on until the Monday, to allow the swelling to go down, and then a solid one would be put on then.  Alyssa initially had been 'sore' when her arm swelled up, but I knew that as soon as the swelling went down, she would go back to trying to use it normally.  And that is exactly what happened.  Even by the Saturday afternoon, she was leaning on her elbow, smacking it around, and she literally spent the entire weekend trying to rip the cast off!  She kept complaining that it was "annoying," and that she "couldn't use that arm."  I explained - multiple times - that that was literally the point of the cast.  To stop her from using it!  She said she wished that it was a splint instead, because then she could just take it off.  I said that was exactly why it wasn't a splint!  I'd explained to the local ortho that not feeling it would affect it's ability to heal but, as is common for orthopaedics, in our experience, this was taken no notice off.  Despite the ends of her ulna still being displaced (separated) in the cast, everyone we saw seemed to think it would heal, without the need for surgery.  We argued for 4 weeks, and on the very night before she was due to have the last consulation before agreeing that surgery may be necessary, Alyssa was literally up until 2am, smacking her arm off of her bed ... because it was itchy!  Unsurprisingly, when she had x-rays the following day, the ends of her ulna were separated even further, and her elbow was dislocating.  Surgery was booked immediately, and she had another metal plate added to her collection.  In total, from start to finish - and including the operation period - she was in a cast for 13 weeks.  Her elbow is still not normal, and will never be normal again.  But she can use it, and it's not dislocating constantly, as they feared it might do.  

All of the arm stuff also put paid to any walking she was able to do, however, as she was putting all of her weight on her arms over the zimmer frame if allowed to walk.  So she ended up immobilised again, for another 3 months.  And taking me over the "year" that I was planning to break these posts down 
into.  How very typical of her!  

More to follow ... 

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!  ;-)  

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 :-)