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!
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