Days 177–274: happy days!

I don’t know how it happened this quickly, but today Hugo is nine months post transplant. Nine! We can now say that Hugo has officially been ‘healthy’ for longer than he was poorly. I say ‘healthy’ because things aren’t quite settled down yet, but still – how bloody incredible is that?! Incidentally, it’s also just over a year since we were first told that he would need a transplant, and also just over a year since I first published my blog.

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Happy to be nine months post transplant!

It never really occurred to me that it was only just over three months between us being told Hugo needed a new liver and the transplant happening. It certainly didn’t feel like it at the time, but three months is not that long. Obviously, when you’re in the whirlwind of end stage liver disease, every day can feel like a year in itself, but now looking back at how quickly it really happened, it seems completely unreal. We went from having a poorly but stable baby in March, to living in hospital by the end of April on a continuous feed, regular albumin infusions and ascitic taps, with two failed attempts at the transplant in May before it finally happened at the beginning of June. What really strikes me now, though, when looking back, is how calm everything really is at the moment. Sure, things still aren’t completely settled, but it the insane and hellish rollercoaster of last year finally seems well and truly behind us.

There have been various points in the months since Hugo’s transplant that we’ve felt like this. Life begins to take shape again, we start to relax and then something happens that dumps a great bit load of uncertainty and anxiety straight back into the middle of our lives. And even though I know that the bumps over the past few months are really nothing in comparison to what we were fighting before, they can still hurt. Because essentially, all we want is for Hugo to be healthy, to be happy. And at the moment, touching wood, he is.

In fact, he’s doing so well that we’ve got a plan in place again to get his Hickman line out. Of course, there’s a million and one things that could change and prevent this happening again – not to mention Hugo’s tendency to never, ever play by the rules – so we’re not going to get our hopes up too much (unlike last time) but the fact we’ve got a plan in place again is a massive step forward in itself. The last time this was being discussed was back in September, before his EBV became a real issue. Now, though, his bloods all seem to be heading in the right direction – I’m convinced that the new iron supplement he started a month ago is behind the improvement in his results; I don’t think King’s would ever officially agree with me, but given the consequences of anaemia can include low Hb and becoming more prone to infection, it seems to add up. Three weeks after starting the supplement, his Hb and albumin are back to normal, and the EBV is now the lowest it’s been since it first poked its nasty head up last summer. And the other really happy news is that his histology (biopsy) results from last week are now back and are all good. There are some tiny traces of EBV DNA in some of the cells, and a little bit of inflammation in the oesophagus relating to reflux, but most of the results are EBV-negative. They can effectively all but rule out PTLD, which was one of the major reasons the line was being kept in. It feels a little bit like Groundhog Day – they thought they had ruled it out in November, but this time it feels more believable, because this time he actually looks and feels like he really is well. And even though he’s been off his main immunosuppressant since November, his liver function is still amazing. So IF his bloods next week are good, they will be stretched out to monthly. And IF the next set is ok, and his ultrasound and review look good, then they will book him in for the line to come out. He’ll have one more set of bloods before the surgery date, and IF they are stable, then we’re going ahead. They are three great big fat ‘ifs’, but they’re tangible hurdles now rather than a never-ending stretch of not knowing. It’s a better, rather brilliant, place to be.

When we found out at his routine clinic in January that Hugo was anaemic, we did a bit of research about iron and the supplement he was given. Listed among the signs of anemia is lethargy and lack of energy, but he didn’t suffer from either of those things. The thing with Hugo is that, given that he became progressively poorlier from birth up to his transplant at eight months of age, we’ve never had a real gauge for how much energy he should have. He just kind of got on with whatever reserves he had, so every time we’d get on top of one part of his health and he’d improve, we’d kind of assume that we’d reached his optimum. So when King’s tested for the iron levels and asked if he had been lethargic, we said no. Because we really, truly didn’t think that he was – he’s always been so playful, so active, we had no cause to think that.

Well, it turns out we were wrong. So, so wrong. At every stage of his life so far, he has amazed us with his tenacity, his jollity, his sheer strength. Now though, he is Hugo – but powered by rocket fuel.

His physical, cognitive and behavioural development has taken such strides forward that he’s pretty much hitting most of his milestones now, and following the normal patterns of mental leaps –given the obstacles he’s faced in his first 18 months, that blows my mind a bit! He now has some serious pace on him (he is all but running on his hands and knees), and is getting so close to walking; before the iron, he was only just able to pull himself up – and he didn’t enjoy it much at all. Now, he is cruising around the furniture and is taking steps with our help. Things seem to have just clicked into place mentally, almost overnight. He really knows his own mind now – and he certainly knows how to tell us exactly what he wants; he mostly enjoys being upside down, playing hide and seek in his tent and throwing balls all around the flat. He is a real bookworm, and loves cuddling up reading stories with us. He is a master stacker of all toys. He sings, he kisses, he chats, he claps, he cuddles, he climbs, and he laughs – all the time. He’s just incredibly self contained and incredibly happy. He’s also becoming increasingly cheeky and naughty, deliberately disobeying us when we tell him not to do something. He bites us, he throws tantrums and he grinds his teeth. As much as I don’t want a naughty child, even all of this makes me very happy (apart from the teeth grinding. I can’t STAND it). Partly because he has a real cheeky, knowing glint in his eye when he is doing it – we really need to learn to tell him off as he just makes us laugh too much. But mostly, it makes me happy because it is just completely and utterly normal.

These are the kind of things that I expected to happen as a mum. They’re the kind of things that our friends and family experience day to day. It feels like we finally have something they can relate to with Hugo, and us with their kids. That’s what’s really lovely about life at the moment – it feels like we’ve finally got ours back. Yeah, it’s not the same as it was, and we’re still a bit restricted, but our new version of normal is actually bloody great! And we’re slowly starting to relax in terms of what we feel comfortable doing with Hugo – he’s having more play dates (and loving them – he’s a very sociable and friendly little boy), but we’re still avoiding baby groups and soft plays until at least after this flu season. We’ve got a good balance of routine and variety, and Hugo is thriving on it. He’s no longer spending 24 hours a day with me, and although it’d have been nice for him to have missed me just a little bit more when I went back to work in January, it’s been really good for him. Good for both of us, actually – being his mum is the single most amazing, fulfilling job I could ever have, but I totally lost all sense of myself when I was a hospital mum, and it’s nice to have a bit of ‘me’ back now. He absolutely loves spending a couple of days a week with his grandma and having more time with his daddy again, and what’s even better is he’s always really happy to see me when he’s not been with me for the day.

So yeah, life is pretty good right now. This is, without a doubt, the healthiest and happiest Hugo has ever been – and in turn the happiest Ross and I have been, too, in a long time. It’s impossible not to smile when you’re around that little boy – he is just so full of joy and life. I have to keep reminding myself we can’t take this for granted, that there are no guarantees, that we don’t know what may come around the corner; heaven knows we’ve had the rug pulled from beneath us enough times now. But equally, we’ve been through a hell of a lot to get to the stage we’re at now, and I just want to enjoy it while it lasts.

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Happy Woodings xx
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“I never liked roller coasters anyway…”

This week was turning out to be the best one we’ve had since we’ve been up at King’s. The sun’s been out, Hugo’s not only been a blast to be around but clinically, he’s been in the best condition since the Hickman line mishap, having appeared to have quickly kicked the adenovirus out of his system, and I had a trip home planned for some serious R&R (and a few bubbles, of course).

The week didn’t start brilliantly – it was Ross’s birthday on Monday, his first one as a daddy, and he didn’t get to see Hugo. Ross and I both stayed at Ron Mac’s on Sunday night, so I saw him briefly in the morning, but he had to leave really early to get to college – where, it turned out, there was a surprise mock exam awaiting him. We both felt a bit sad about how his birthday had turned out.

Hugo's first piece of art for his daddy's birthday. He's a clever one!
Hugo’s first piece of art for his daddy’s birthday. He’s a clever one!

Hugo, however, had other ideas. He kick-started our fifth week in hospital with some serious leaps forward in his mood and development. In spite of the discomfort he’s in from teething, his smiles have been bigger and better than ever this week. And they haven’t just been reserved for Ross, my mum or me. He’s charming the pants off of everyone who comes in to see him. He’s stepped his cheekiness up a notch, too, with lots of chatting and bubble blowing, learning to blow raspberries (although he’s still not quite there with the high fives – YET), and cuddling Mickey in the most inappropriate way. It’s so nice to have a lively, chirpy little man back in the room. It really gives me the strength to keep going and, more importantly, keep things as normal as they can be for Hugo up here.

It must help that we’ve been able to take him out almost every day. Obviously, if he requires anything clinical – albumin or antivirals, both of which take a few hours – we’re still confined to his room. But these requirements are becoming less frequent. So on the days where he is well enough, and the sun is shining, we’ve taken him out. Normally, we go for a wander around Ruskin Park to see the squirrels and ducks, and then head up Denmark Hill to get some supplies and to Café Pronto for lunch (I’d recommend it to anyone who comes to Camberwell and needs a decent lunch) before heading back to hospital. He is always so well behaved when we take him out – he must appreciate the change of scenery as much as we do. Having the freedom to do all of this is a real relief for all of us; it really puts a smile on all of our faces.

One of the major steps forward we’ve taken is with his feeding. Hugo’s still on the continuous milk feed, and we’ve been very cautious with increasing it for fear of him being sick, which is both counter-productive and unnecessarily distressing. So I’ve been really focusing on his solids. And on Wednesday we had the breakthrough we’ve been waiting for – we managed to give him three meals! He’s been happily eating breakfast for a couple of weeks now (and we’ve branched out from rice pudding too – he’s also wolfing down blueberry and pear baby brekkies and banana flavoured rice), but come lunch time or dinner time he would scream the place down when approached with anything. But something clicked on Wednesday. When I gave him his lunch of orchard chicken, followed by a chocolate biscuit and custard, his reaction was amazing. He was actually excited to be eating! I decided to try for a third meal, albeit a snackier one – a Laughing Cow soft cheese triangle, some yoghurt and a banana – and the same thing happened. In fact, I’d go as far as to say he LOVED it. Not only that, but as his arm fat dropped this week (his only true measurement at the moment) we decided we needed to be braver with his milk feeds and push them up to his target 30mls an hour. And he tolerated it. We’d finally cracked it and were able to get the calories he needs into him! I was so bloody proud of him, I treated the staff and other residents to a little dance up and down the ward!

This week was also my opportunity to get home for a couple of nights, recharge my batteries and let my hair down. I’d pretty much planned my time, filling Friday up with eating lots of imaginary chocolate with my niece Violet, seeing friends for some drinks and getting a bit of pampering in, too. Perfect, really (other than my boys being up in London and not at home with me, obviously). Ross came up from work on Wednesday, and on Thursday we had a brilliant family day – sun shining, ducks following us round the park (slightly scary!), lunch out. Hugo barely slept but was really happy nonetheless. We took him with us for an early dinner before I hopped in the car to drive home, and he was loving being in the pub playing with his toys. And then it happened.

We got The Call.

Actually, we got The Call seven times – our phones were on silent! Poor Gill (transplant coordinator) and Melissa (our lovely nurse for the day) had been desperately trying to catch us before I left for home. But we eventually picked up – a B+ liver was on its way to King’s, and Hugo might be the one to have it.

Hearing that news was like boarding the biggest and scariest roller coaster we’ve ever been on. Neither of us could finish dinner. We stopped Hugo’s feed and sped back to the ward. When we got back, we were told that the liver was definitely intended for Hugo, and that everyone was happy that Hugo was well enough to go through the operation; all that was left was for Hector, the surgeon, to eyeball the liver when it arrived at King’s and make sure he was happy with it.

Things from then became very surreal, and went by in such a blur that it didn’t really feel like it was happening to us; time went both quickly and in slow motion. We asked for Hugo to be given an examination and X-ray, as it didn’t seem like they were planning on doing one routinely – Hugo had only been checked over by Emer, the consultant, two hours prior, and she was happy with his condition. However, we were conscious that we’d already had one surgery cancelled because he had adenovirus, so we wanted him checked over. Another doctor came and gave him the once over, and he had his X-ray. Everything was fine. We had to pack up our room, as we’d be discharged from Rays when he was taken down. We met the anesthetist, and the surgeon came up to go through the risks and get our consent. We were told that, if it was going ahead, it would happen very quickly – and they weren’t wrong. Hector came up at about 9.40pm and said that the liver was beautiful, albeit a bit bigger than they wanted him to have, but because of his ascites they were completely happy to go ahead. And then about two minutes later, our room was full of nurses, anesthetists, and the on call transplant coordinator Matilda, and we were on our way down with Hugo. Whirlwind doesn’t do it justice. At this point, he’d had about three hours sleep since waking up at 6am that morning and yet, rather than being overtired and grizzly, he was still giving us the biggest smiles and blowing raspberries at the team.

Fitting in a cuddle with Mickey before going down to theatre. And yes, Mickey has a name badge on...
Fitting in a cuddle with Mickey before going down to theatre. And yes, Mickey has a name band on…

We took the call from Gill at 6.30pm, and at 10.10pm Hugo was officially in surgery.

After calling our parents and sisters to tell them, and putting our stuff in the store on the ward, we headed back to our room at Ron Mac’s to get our head around what was happening; the next time we’d see Hugo, he’d have had his liver! We’d told ourselves that until he was taken down, we wouldn’t believe it was happening. But it was happening. In a few hours, we’d be on the other side of the hurdle we’d been heading towards for three months – it was so exciting and nerve wracking at the same time. Matilda told us she would call after the first three hours, and then every couple of hours after that until he was out. The operation was expected to take anything from six to ten hours, so we’d see him in PICU the following morning. We were told to try and get some sleep – hah!

But. And I’ve said this before. There is ALWAYS a ‘but’ with Hugo. My phone rang at midnight, less than two hours in. It was Matilda. She told us not to worry, but asked for us to come to PICU to see Hector. Those of you who know me well, know I don’t run. But I learned how to run at that moment. I ran so fast that Ross couldn’t even keep up. We both knew in that moment that it hadn’t happened, but we didn’t know why, and the fear I felt in those ten minutes are the most gut wrenching I’ve ever felt in my life.

When we finally got there and saw Hector and the anesthetist, they explained to us that when they’d put Hugo under and ventilated him, his saturation (lung function) had dropped significantly. They found the bottom of his lungs were full of secretions, probably from the adeno, which had been compressed and hidden by the ascites restricting his lung capacity. When they had fully inflated his lungs, it came loose, and became a problem. They had decided that they couldn’t continue with the transplant.

As awful as it was to crash that hard, Hector explained that if they had gone ahead, the potential complications that Hugo may have suffered could well have been so severe, they may have killed him. We knew, even through the shock, that they had made the right call. These guys are the best in the world at what they do, and there is a reason for that; they make the right calls when the chips are down.

The next day in PICU
The next day in PICU

Hugo was kept sedated and on the ventilator overnight, and we spent the day in PICU with him on Friday. PICU is a weird place; I don’t think it can really be explained. Hugo coped with it characteristically well. They suctioned a lot of secretions from his lungs over the day, and at 2pm they took him off the ventilator. When we saw him after that, he was pretty much back to normal, kicking around and ravenously hungry. At 6pm, the nurses came from Rays to take us back to the ward – less than 24 hours after we got the call – and I decided I wanted to try and feed him a bottle, rather than just start the NG up. Bearing in mind that he hasn’t taken a bottle in weeks, he went to town on it! After the massive highs and lows of the day leading up to that, he gave me what I needed. He gave me a boost, he let me cuddle and feed him, and he gave us a glimmer of hope that when the time does come, we’ll be able to feed him orally and not need the tube. He’s back on the NG feed now, but still… It felt amazing.

It probably goes without saying that Ross and I have been left reeling a bit from this ride. I mean, how close can you actually get to it happening without it being taken away?! All of the doctors and nurses we saw on Friday were totally gutted for us and shell-shocked – many of them are so invested in his care and well being, and know him (and us) so well after five weeks here, that they really felt the blow. Everyone we’ve spoken to has said this hasn’t happened before – they’ve never had a child get as far as going into the theatre, only to be wheeled straight back out. Thankfully, they hadn’t started to split the liver when they found this problem, and the whole piece went to an adult in need of it. It wasn’t wasted. The family that had lost a loved one that day will still have the precious knowledge that a life has been saved. That has been a massive comfort to me.

The next big question in our minds is how to get Hugo over this before the next date for Ross to be the donor. It doesn’t even bear thinking about what would have happened had this not been discovered during his surgery – we would have lost Ross’s liver as an option. This is where we can believe that, although it was a rough ride, it happened for a reason. It happened so that we can do everything in our power to get Hugo’s lungs clear for the next date. We’ve been very explicit to the team in our concerns about this, and that even though we fully trust the judgment of the consultants and surgeons, we now need to trust that Hugo is ready for the surgery to happen. It’s not going to be easy.

Now, back on the ward, we’re not in a much different place than we would have been had this not happened at all. Obviously, we are totally gutted to have lost a high quality B+ liver. But we still have Ross as an option. Hugo is still feeling the effects of the anesthetic and tube a bit, so even though he’s still given us smiles today he’s also rather grizzly and much sleepier than normal. I didn’t get my break at home, but that can wait. We still have a date for the transplant to happen. And we know more about his condition than we would have done had this not happened.

Back on Rays and waking up smiley again
Back on Rays and waking up smiley again

My sister put it right when she said that if someone could try to devise a way to torture us, they couldn’t get much closer than this. We’ve felt more emotions in 24 hours than we thought possible – shock that a liver had actually come up, and fear about Hugo going in for surgery. We finally allowed ourselves to be excited about the prospect of getting Hugo on the road to recovery, to then have the rug pulled from underneath us again just two hours later. Ross’s head has been in a spin from being geared up to be the donor, to accepting it couldn’t be him, for it then to change again. We’ve had adrenaline, numbness, and total and utter exhaustion. And the whole thing has been so surreal that it could actually not have happened at all.

I think it’s fair to say that this particular roller coaster has been totally horrendous. As Ross said – “I never liked roller coasters anyway”; this is one we desperately want to stop.

A transplant date – or so we thought

This should have been the update we’ve been gearing up to since February, the one where we could tell everyone that Hugo’d had his transplant. But we seem to be living in a world of big fat ‘shoulds’.

It wasn’t long after my last post, where were given the green light for Ross to be the donor, that we were given a date for the transplant to take place. The date was set for Tuesday 12th May; we were given a week’s notice. The week then kind of slipped away from both of us. Even though there wasn’t really anything to prepare, as such – we’re already up at hospital with everything we need – there was still a kind of pressure on the days leading up to the transplant. I was making plans for company and support for the day of the operations, while Ross tied things off at work for a couple of months and started to ready himself for the surgery.

Reading his OT notes on how to be less wonky-headed!

But – I’m starting to feel like I’m a bit of a broken record here – things never seem to be that straightforward with Hugo. Towards the end of the week, I noticed Hugo started to sound a bit congested. I started to worry, despite Ross trying to reassure me. I mean, Hugo’s luck had finally started to turn and things were happening… right?

Well, no. The nurses also noticed, and did some swabs to check for viruses. And, of course, they came back positive. Hugo had picked up the adenovirus, which is just a common cold, but is enough to put a hold on the transplant. We knew that any bug could risk the surgery going ahead; the risks associated with suppressing his immune system while he’s poorly is significant, and there is also a risk that he could need to stay in intensive care for longer, possibly be intubated and on the ventilator longer, and develop other complications such as pneumonia. In short, putting him through a transplant while he’s poorly could kill him.

So even though he had barely any symptoms, on Monday he was deemed poorly enough to postpone the op. To say we were gutted is an understatement, but we both totally agreed with the decision (especially when he spiked a temperature on Monday night). The fact that one of the lead consultants told us that he wouldn’t agree to his child going for the surgery cemented the seriousness of it in my mind; we hear so much clinical speak on a daily basis, to hear the human perspective from a senior medical professional took me by surprise.

Hugo’s old man face (he really was enjoying playing with his toy and balloon!)

Even though we agreed with the decision, we had hoped that it was going to be a matter of days until he could go again. But the new date we’ve been given is a long way off (Ross and I have bickered back and forth about whether to tell people the date at all, and have settled on a ‘not yet’ approach – it’s too early to tempt fate on the new date yet. So for those who were going to ask – don’t). This is where I’ve got really angry – not an emotion I’ve felt much of during our ups and downs so far. But the length of time we have to wait now to get Hugo better made me angry. And it won’t come as a shock to those who know me well that I’ve let people know that!

One of the reasons for the delay is the lead surgeon is on a sponsored bike ride to Barcelona for the next two weeks. Ok, granted, he’s raising money for the liver unit here at King’s. It’s not like he’s off on a jolly somewhere. It’s a good cause. And yes, there are several other factors which all contribute to the next available surgical slot falling into next month; all I keep hearing is ‘logistics’ – I used to love logistics. Now I bloody hate them! But the fact that Hugo’s operation, recovery and – ultimately – bringing him home and getting our lives back is delayed by weeks because of a bike ride and logistics made me mad. I mean, REALLY?! Even though he’s actually quite stable at the moment – he’s not needed a tap now for 12 days, although he’s having more regular albumin to try and keep the ascites under control – he is still deemed in urgent clinical need of a liver transplant. It’s just too bitter a pill to swallow for me; it just doesn’t make sense.

We’ve now found out, though, that the adenovirus is in his blood (we were hoping it was just respiratory), which means that it is unlikely he would be deemed clinically well enough for the transplant for another week, at least. So we’ve now managed to find a way to accept that this is just the way it’s meant to be. Yes, it’s going to be shit being in hospital for another few weeks, but it’s something that we’ll manage because we have no other choice.

Totally unphased by this weeks set-back – Hugo in a playful mood

So, the silver linings. We always manage to find them! Because Hugo has a respiratory virus, we’ve been moved out of the nursery into a room, so we’ve got privacy and some peace and quiet! Which has made a huge difference to my sanity, if nothing else. I’ve been telling pretty much everyone I see that I’m not taking Hugo back into the nursery now before his op – I just can’t risk him catching something else and having another date cancelled. Everyone is very sympathetic to the situation, and they’re all being incredibly supportive, so I do believe this will be kept in mind with the ward logistics (gahh) as the weeks go on.

We’ve had amazing support from our families, as ever. My mum has been here every week with me, and this one was no different. My sister also came to stay for a night, which was really nice as I’ve missed her so much. Ross had to go back to work, which was difficult for both of us, but is here as much as he can be as always. And he’s taking a couple of days off so I can get home for a night or two and not miss seeing him over the weekend – I’ve been feeling increasingly homesick, and need a break from living in hospital to see my friends and be in my flat. But he gives me the strength to keep going up here and I wouldn’t be able to sacrifice my one night with my husband to do that.

Enjoying the sunshine on an outing to the park


As for Hugo, since becoming ‘poorly’ he’s actually been in a better mood than for the last few weeks, and he seems to be making strides in his play and development. We were given a set of velcro supports from the Occupational Therapist which has done wonders for his head shape; the illness has rendered him a little flat-headed on his right side because he’s not able to roll (although he now sleeps on his side – still with Mickey on his face, of course!) and has to spend so much time in his hospital bed. We’ve a little way to go, but he’s definitely less wonky! He’s taken the step from just putting things in his face to putting them in his mouth – spoons, toys, and chocolate biccies. He’s almost sitting up on his own – again, his belly is a hinderance, but he seems totally unphased by it. He’s learning new ways to play and reaching out for toys (or our faces) all the time. He’s almost laughing – his face and his eyes are already there, we’re just waiting for the noise now. We’ve been taking him out every day for a walk around the park (weather and treatment permitting, obvs), and he’s starting to notice and enjoy the new experiences – squirrels, flowers, and the wind in his face. And I’ve nearly taught him to high-five – I’m determined to achieve this before his transplant… I’ve got to have my goals, after all!

Mumma and bubba playtime

I’m just so bloody proud of how much he is learning in this environment. And again, I might be like a broken record here, but he makes it so easy to cope. He makes both of us feel very lucky, and to achieve that in the midst of this situation is quite something!

Progress!

This week, we took a big step forward to getting Hugo well again. Ross finished his assessment to be a living donor on Friday, and after a very nervous two week wait following one of his clotting results coming back as out of range, he’s been given the green light to be Hugo’s donor!

Happy parents – sunshine and progress

Even though it’s finished on a high, it’s been a bit of a rocky week. We’re still at King’s with Hugo. And we were told on Tuesday that he’s not well enough, and is unlikely to become well enough, to take him home now before the transplant. Given the fact that we only brought him up here for a “straightforward” procedure, and only expected to be here for up to a week, to say this came as a blow is a massive understatement. For one, I didn’t bring many clothes; I brought enough to last a week and yes, there is a washing machine on the ward, so I’ve been able to stretch it out pretty well. But four t-shirts and pairs of pants can only last so long!

The thing that really hit me with this news is just HOW poorly Hugo has become. I know it must sound stupid – he’s got end-stage liver disease, after all! But we’ve moved from a progressive decline in his health to a sudden and unexpected drop. He was simply too fragile (even though he was stable) before his admission for the Hickman line to cope with two surgeries and the effects of these. It’s knocked him right off his perch. And the doctors – rightly so – have told us that it’s no longer possible (or fair) for us to try and manage his condition on our own from home. When we had him at home, we had some semblance of a normal life – at the very least, we were close to our friends and family, we had his jumparoo, his bedroom, our own comforts. All of a sudden, we’re two hours from home, living on a ward with three other poorly babies, their families, nurses and bleeping machines (and this is all night. ALL NIGHT. Sleep deprivation has taken on a whole new meaning). It’s a pretty sobering change of circumstances.

On top of that, we’ve also decided that we should re-home our cat, Ronnie. We’ve had her for six years, and I’m totally gutted. But we’re hardly ever there now, and even though we have amazing neighbours who look after her at the drop of a hat (and who she loves), it’s just not the same as her having a family to live with. She’s still at home, and we think we’ve found a loving owner for her. But I hadn’t said goodbye. So I made a quick dash home mid-week, arriving back at my flat at 9.30pm on Thursday night after a stop off at my sisters for dinner, and leaving again at 7.30am the following morning with our transplant case in tow, and having said goodbye to Ronnie.

Final sofa snuggles with my Ronster 😦

So from the practical side of things, we’re now more prepared. Which for us (and me in particular) is a massive boost when it comes to coping with the rollercoaster that is living in hospital. We’ve also moved to a bigger room in the Ronald McDonald house, one with a bath and which can fit a travel cot in, so we have space to take Hugo there (when we can), and also for my sister to bring my niece up. I’ve actually upacked my bag (not something I normally do when away from home, even on holiday), and am now living out of a wardrobe again, rather than a suitcase. It’s a small thing, but it makes a big difference.

And always being ones to try and find the silver linings, we’ve also relinquished the responsibilities for Hugo’s medical care to the nurses. We’ve got a huge amount to think about and take in while we’re here, so not having to keep on top of his medications, for instance, and not having to be the ones giving them to him for a while, is a welcome break.

Despite everything the doctors have thrown at him – more albumin and increased diuretics – we’re no longer able to keep his ascites under control. This week it started to affect his breathing and his heart rate, and his tummy went from about 52cm when we first came up to King’s, to 60.5cm, and was tight as a drum. The only option when it reaches this stage is for them to tap some of the fluid off. It provides some immediate relief, but it’s only a temporary solution, and it comes with risks. We didn’t realise quite how temporary it is. He had his first tap on Wednesday, after an urgent ultrasound following some concern about his respiratory rate, and they removed 500ml of fluid. The difference in his appearance, mood and respiration was instant! But even by the Thursday morning, the effects had dwindled. He needed his second tap on Friday. This time, they took 675ml and he lost 3.5cm. But in less than 24 hours, his belly had already gained back 2.5cm. The problem is that every time they do a tap, they have to put a needle into his abdomen, and with that comes risk of infection. They could leave a drain in, but that carries even more risk. In the lead up to transplant, it is vital for him to be in the best health possible. It’s a very fine balance, but he’s reached the point where he can’t manage comfortably without them.

“I love you this much…”

Amazingly, in spite of how poorly he is, how unstimulating the environment is and how uncomfortable he must be, he is smilier than ever at the moment. And even though he’s really struggling to tolerate his milk – he’s now on a slow continuous feed just to try and get as many calories into him as possible in the time before transplant – he’s doing really well with his solids. His favourite is rice pudding  – he smiles all the way through breakfast when this is on the menu. His resilience is shining through once again. I’m astounded by him, and he’s given me the strength to get through the next few weeks. Even though the situation is shit, he reminds me every day of just how lucky we are to have him.

Cheeky smiley Hugo

So with all of this bearing down on us, the timing of Ross’s assessment couldn’t have been any better. We now have a light at the end of the tunnel.

Our lucky charm – a four leaf clover, given to me on Thursday night by my sister

The biggest hurdle we had was his haematology appointment. This was what we’d been worrying about since we found out his blood results a fortnight earlier. I went down with him for the meeting, only to be turned away – the process has to be so independent, I wasn’t even allowed to sit in. And as it turns out we didn’t need to be so worried. The particular clotting function under query (protein C), Ross was told, is out of range, but is not abnormal. There is zero additional risk to him having surgery.

He also had most of the remaining standard appointments – CT scan, psychologist, anaesthetist. TIck, tick, tick. All that’s left now is seeing the consultant hepatologist, and his independent assessment. Both formalities. It’s frustrating for Ross that he has to come back again to get these bits tied off, but as soon as they’re done we will be moving very quickly towards the thing that has been our focus for the last two months – Hugo getting a new liver.

A very relieved daddy and a happy Hugo just after a tap

Although we’ve pushed for a date, Fiona (the coordinator) can’t give us anything for definite until absolutely all the boxes are ticked. But we’ve been told that the transplant will happen within three weeks. Possibly two. Because Hugo is so young, they don’t necessarily need two theatres to get it done, which is often what can prolong the wait. He will remain on the list for a cadaveric liver, so if one comes up in the meantime, he’ll have that. Either way, by the end of this month, Hugo SHOULD (I can’t yet say will, as I don’t want to tempt fate) have his new liver and be well on the way to recovery.

Now, we’ve got the next phase of this journey to contemplate. For Ross, it’s a major operation, and up to three months off work to recover (the fact that driving is central to his job playing a big part here). I know that he’ll do amazingly. And seeing what this news has done is so uplifting – he’s beyond happy. He really wanted to be the one to help Hugo – to have matching scars and a bond that will tie them for life (well, an even bigger one than they have already). Now, he can. For me, it’s a case of getting my head around what that day will bring – both of my boys having major surgery on one day is a very scary concept. Ross will be in theatre for up to seven hours, Hugo for around ten. They will overlap. I know I won’t be on my own; my mum will be with me every step of the way, as will Ross’s. But it’s not going to be easy.

Cuddles with my boy 🙂

For Hugo, though, it’s a new lease of life. We’ve been told what to expect from all angles – the scary stuff as well as the good. But I can’t focus on the scary side – what it will be like seeing him in intensive care. Or at least, I’ve got to try not to think about it. Instead, we’ve got to focus on just how well he should be after. There’s still a long road ahead – with rejection among one of the many hurdles to encounter along the way – and it won’t be easy, but the success rate of transplants is so good (much better than the Kasai) that we can really start to think about Hugo being on the other side of this. We’ll have a Hugo without the yellow skin and eyes, and with a normal belly. A Hugo without his NG tube, with his old appetite back. Our Hugo. The thought fills us both with so much excitement – and the fact that it is now almost in our grasp is just a dream (almost) come true.

Hugo the Brave

So much has happened in the last fortnight, it’s hard to know where to begin.

Up until last Sunday, we’d had a pretty enjoyable couple of weeks. To start with, we managed to last 15 days without Hugo having any bloods taken!! We still had two trips over to QEQM to have a new NG tube (he’s now learning how to pull it out, the little rascal), but I’m not classing those as actual, real hospital trips. So we’ve been able to make the most of the sunny days with lots of walks along the seafront, and have had the chance to catch up with some friends and family without having our plans cancelled. We even made it out to go and watch my brother-in-law’s band (the Blue Moons – if you’re local to Deal, go and watch them. They’re excellent) playing at a pub round the corner. It’s been the most blissful time since Hugo was diagnosed in November, and a little glimmer of what life will be like when we’re on the other side of this and he’s better. Ross also found out he passed the exam he sat in January – I’m so proud of him, given everything that we’ve been through during his studies! So we’ve had some pretty happy times, which has been lovely.

We’ve had a couple of trips to London with Hugo. The first, which was now ten days ago, was for the next stage of Ross’s living donor assessment, and for Hugo to be reviewed while we were at King’s (making the most of being up there). Having three of us, Hugo’s buggy, feeding equipment (we’re now using a pump, which comes with a LOT of extra crap) and overnight bags for all of us packed into my little Picanto to tear up the motorway was not the preferred way of spending the hottest day of the year so far. But we made it up in time to enjoy the last of the afternoon sun with a walk up to the pub on Denmark Hill for dinner from the Ronald McDonald house where we stay when we’re up here. The RM house is an amazing place – we don’t know where we’d have been without it. The charity is so worthwhile – providing rooms (a “home from home”) for families with children at King’s to stay, without the monstrous costs. There is a £20 deposit for your key, which you can – and we do – donate. That’s it. And it’s such a well-equipped place – the rooms are all so comfortable, it has a great kitchen, lovely garden, and lots of support. We’re hoping to do some fundraising for the house when Hugo’s had his transplant – £5,000 sponsors a room for three years with a plaque on the door. You can also get a leaf, bird or treehouse plaque with Hugo’s name engraved up on the wall in the house. We want a room, and for Hugo to be up on that wall. So watch this space!

Chilling in a room at Ron Macs – the house that love built

Hugo’s review was pretty straightforward – as we had another planned trip shortly after, they decided not to bother with bloods. We reviewed his meds, made some adjustments, had an ultrasound (the best one he’s had in a while), had another NG tube (I mean, really?!) and that was pretty much it for him. Ross had his ultrasound and x-ray, and – most crucially – his blood results and a meeting with the surgeon, Professor Heaton. 99% of everything so far is looking good – he’s B+, which is a blood match, his liver isn’t fatty, most of his bloods came back perfect. One didn’t, however. And unfortunately, it’s a liver function that’s out of normal range – one of his clotting functions. Basically, his blood clots quicker than normal, which would put him at a higher risk of DVT and pulminary embolism during surgery than most. And also, as it’s a liver function, this is something that Hugo would inherit should the transplant come from Ross. We don’t know yet how much of a blocker this is, but Ross has had to be referred to see the haematologist to determine whether it is possible to continue down this road. They’ve not ruled it out, but there is a chance it will be. We’ve been assured the haem team are enablers, not blockers, so it would only be stopped if there was very good reason. Ross is so invested in doing this – he really wants to be the one to help Hugo, to save his life. I hate that it’s so out of his (and our) hands. We’ve got to wait another week for these appointments, and for the final parts of his assessment (CT scans etc). It may not sound like long, but it’s too long for us.

Hugo charming the nurses – as ever

The thing is, Hugo is starting to get worse. His belly has been looking pretty good over the last couple of weeks (although more on this later), but that’s pretty much where the good ends. He’s not tolerating his feeds very well, and is being sick quite a lot. He’s now starting to scratch. He’s getting rattier and rattier – even cuddles aren’t providing any comfort at the moment, which I’m finding incredibly hard. They think this could be down to high ammonia levels in the blood. Nothing that can be fixed now; the doctors can’t really say much other than “he needs a transplant”. It’s reaching the stage where we’re just watching him get yellower and poorlier, and we’re just hoping that we get a liver soon. We’ve been told he has time – but we don’t have an indefinite amount of time. And that is beyond scary.

Fiona (the living donor co-ordinator) seems reluctant to assess me at the moment, as Hugo is still so young my liver will still be quite fatty. So she suggested at the start of the week that we give out her number to anyone who has volunteered to be a donor, and I believe she’s been kept pretty busy with phone calls from friends and family over the last couple of days. Of course, we’re hoping it doesn’t get that far. But the fact that there are so many people who are willing to be considered, and that have actually called her to put themselves down to be looked at, is the most touching thing. So just a quick and very public message to those of you (and you know who you are) who have put yourselves forward – thank you, so so much. You’ll never know how much it means to me and Ross, and we’ll never be able to find the words to really explain it. But however this pans out, we’ll always be incredibly grateful to you all. And we’ll probably owe you a drink or two when this is all over.

Our home for the week (at least)

Right now, we’re staying on the Rays of Sunshine ward at King’s, as Hugo has had a Hickman line put in. This is basically a semi-permanent line into one of his major blood vessels to allow easy access for taking bloods and giving IV treatments (albumin, fluids etc). We asked for it, as it means NO MORE NEEDLES! We’ve reached breaking point with his veins; he needs bloods too often to keep getting at him the way we are. It would also mean his bloods could be taken at home by the community nurses, which will provide a welcome break from the constant hospital trips (allthough I reckon Hugo will miss the attention from the nurses at QEQM!) Even though it’s scary that he’s had to go through another operation, it’s a relatively straightforward procedure (in comparison to the surgery he’s already had, and the one looming ahead of him, anyway) and it will alleviate some of the constant nastiness of his current care. Or so we thought. After having his slot cancelled on Tuesday, he eventually  had the operation on Wednesday. It took a couple of hours longer than we were told it should (he also had his hernia fixed while in theatre – two birds, one stone, and all that), and when we got him back after four hours we were told his heart rate had increased after the line went in, and they’d had to adjust its position. Ok, a slight complication, but it was dealt with. The next morning the doctors on Rays carried out an x-ray, and it turned out that the line had somehow slipped, and was now far too far into his heart to be left untouched. It could be quite dangerous. As frustrating as it was, it happened and had to be dealt with. So yesterday, he had to go back into theatre to repeat the op. Happily, this time he was out in two hours, and they’ve x-rayed several times to confirm the line is in the right place. Unhappily, it meant he needed more cannulas in the interim, which they struggled to get in and kept falling out, so he’s now covered in prick marks and bruises again. It kind of defeated the whole object of us being here – he shouldn’t need to be pricked anymore. But hopefully, that’s it done now and we can finally offer him that relief.

Sneaking in some sleepy cuddles

 

Our boy was certified brave after operation number two of the week

 

However – and there’s always a however with Hugo at the moment, it seems – because Hugo has been nil by mouth for a total of three days (something he’s not actually been that bothered by – I think he’s enjoyed the break!), he missed some of his meds, including his diuretic which helps with the ascites. Because of that, coupled with a lack of nutrition and the standard effects of having surgery and a general anesthetic, his ascites has boomed again. We’d just managed to get his belly under control – as I said earlier, it’s the best it’s been since January over the last few weeks. But it jumped up in size by about 4cm from Wednesday to Thursday, the veins became very prominent again and it went from being very soft to very, very tight. He was also not peeing. Recognising the signs, I told the doctors that I thought he needed albumin. I know my son, and I know that he is not capable of flushing out the fluid himself. Unfortunately, the doctor didn’t listen to me  (or look at Hugo) and instead just looked at his numbers, which suggested that he didn’t need albumin. A battle to get him some relief from his symptoms is the last thing we need to deal with at the moment, but it’s what I was unexpectedly faced with. Thankfully, I had others to turn to – one of the (lovely!) transplant coordinators, Gill, a senior nurse and the ward matron. When they looked at Hugo, they could instantly recognise that he needed something as he looked clinically more unwell, and swiftly did some arse kicking on my behalf. When the consultant came on ward round, he took one look and said “Hugo needs albumin”, among other things (daily weight and girth measurements, and restricted fluid intake while the ascites remained at large). Twelve hours after I knew he needed it, it finally happened. His tummy hasn’t gone down in size yet (something not helped by the repeat surgery), but we’re on their backs now to keep on top of it and not let it get even worse.

Had the operation been as straightforward as hoped, we’d have been home after 24 hours. But as it stands, it looks like we’ll be here for a few more days. With the complications in general and the increased ascites, Hugo is STILL off feeds (all that hard work and tears put into getting his weight up, gone, as we can’t get any milk into him), so we have to wait to get his fluid balance sorted and his feeding back up to scratch here at hospital before we can go home. This morning, we also had a question mark raised over another blood level that is now out of kilter (his haemoglobin). So I’m resigning myself to at least another two or three nights here. Thankfully, Ross is here at the moment. He hasn’t been able to stay with us much on this trip – if he is the donor, he’ll need about three months off work, and he just doesn’t want to test their kindness and support, which is fair enough! He made it up unexpectedly midweek to give me a night off (I was getting beyond coping point with the sleep deprivation), and he’s here now for the weekend. Hugo loves having his daddy here; we both miss him like crazy when he’s at home without us. But it’s our lives at the moment, and we have such wonderful support that we know that we’ll somehow make it through.

Family time with the fishies

 

Mainly shits, and not many giggles

Those of you who have regularly read my blog so far will know that I try and maintain a positive stance on everything happening to Hugo, and our family. I’ve made a conscious decision to try and find the silver linings in each situation because a) I don’t think anyone would want to read a depressing blog and b) moping gets you nowhere. So I try to keep upbeat, laugh at what I can and remind myself that it could be worse.

However, being optimistic isn’t always possible. This goes for whether you lead a relatively ‘normal’ (sorry, I hate that word) life, or whether you have something shit to face every day. Unfortunately, the last couple of weeks have fallen into this bracket. Mainly because Hugo has been poorly. Normal (sorry, again) poorly, not liver poorly. And he’s been thoroughly miserable with it, which is fair enough. But we’ve really been missing our cheeky, happy little man, and not having him around has made it much tougher to deal with the daily grind.

Our days revolve quite heavily around numbers at the moment, with blood levels and feeding targets to try and keep on top of. So it seems fitting to tell you about our last couple of weeks in numbers.

3 – The number of trips to hospital we had last week. Also the number of blood tests he had. This shouldn’t have been the way the week panned out, but his first test on Monday (purely routine) clotted. So I had to take him back in the next day. We managed a couple of days at home after that, but then on Good Friday he was being very poorly (from both ends. I’m sure you don’t need the details) so we had to take him to hospital for IV fluids and observations overnight and through the Saturday.

1.5 – The number of albumin infusions Hugo had. The 0.5 happened on Tuesday. King’s advised that he didn’t need albumin, but I ruled against them as his albumin level, at 29, was low, he has a history of requiring infusions (his running total is now 10.5), his belly was getting larger and more tense, and (most importantly) he had a very precious cannula in that I was determined would be used; I didn’t see the point in taking it out and repeating bloods two days later, by which time he would have definitely needed albumin, when he had a working cannula in. It just seemed like needless pricking of my baby boy. So, anyway, we went ahead and gave him albumin. But the cannula wasn’t as effective as we’d hoped, it stopped working halfway through; Hugo’s hand started to swell and he was screaming in pain – even our nurse commented it was very unusual for him to be so upset. So they took it out and he got half the infusion (and a LOT of cuddles). Still, better than nothing, I guess.

30 – My new age. Yes, I turned 30 on Saturday. Waking up on one of QEQM’s blue plastic pullout chair-beds with Hugo being poorly (he was sick on the stroke of midnight, and then sneezed his tube out at 7am. I kid you not) and without my hubby was not an ideal way to start the day. But it got better as the day went on. Ross arrived early with a McDonald’s breakfast and some wonderful gifts (and my first ever Mummy birthday card). My mum and sister came and cheered the room up with banners, party hats and a lovely yellow cake. Instead of a pub lunch at the Jackdaw in Denton (where we got married a couple of years ago), I had a bizarre hospital canteen lunch picked by my mum (a very questionable shepherd’s pie and chips – she’s still being teased about this). We even managed to coax some smiles out of Hugo; these have been very rare the last couple of weeks, so this was a real treat. The doctors decided mid-afternoon to make use of Hugo’s cannula and gave him albumin before discharging him, so my sister took me home and we managed to go out for one or two (or ten) birthday drinks in the evening.

Also, 30 is the target minimum albumin level. Hugo’s is regularly below this. King’s have now said that there’s not much point in chasing that number, as he’s never going to hold the albumin. It’s now more a case of monitoring the ascites and not letting it get too bad. Hurry up, transplant…

22 – The number of days Hugo has been on the list, so far. He’s now also on the priority list, although we’ve been told the list for a B+ liver at King’s is very short, so we don’t think this will make much difference. We’re still jumping out of our seats whenever the phone goes. Ross is totally determined to be the donor, while I’m wishing for a cadaveric liver to become available before it comes to that. Either way, we’re still waiting.

2 – The number of cakes I got for my birthday. This is only worth a mention because the nurses on the ward very sweetly managed to source and decorate a cake for me when they found out it was my birthday. Becoming part of the furniture there has its perks.

Also, the number of times we had poo on our carpet over Easter. Poo. On our carpet. Once was Hugo, who managed to explode some out of the side of his nappy when bouncing in his jumparoo. The first we knew was when we looked up from our lunches (while hungover, may I add) to see him happily stamping away in a puddle of poo. He managed to totally avoid the jumparoo itself though, which was a stroke of luck as I don’t know what he’d have done without it while we were washing the seat. The second was a friend’s baby, who wriggled off the changing mat mid-change and onto the floor. Thankfully, Ross is a little obsessive over cleaning, and we therefore always have a cupboard full of Vanish and bleach.

Unknown – The amount of prosecco I drank on Saturday night. I had originally planned to go out with Ross and some friends, and Ross’s mum was going to babysit. However, when we had to take him into hospital on the Friday I decided to cancel – I was in a total state after the stressful week we’d had, and I couldn’t think of anything worse than facing people. Even people that I know wouldn’t care if I was a crying wreck all night. I couldn’t tell how Hugo was going to be, and I didn’t want to feel pressured to go out on top of caring for him, so I cancelled. However, as he perked up on the Saturday, and the doctors were happy for him to be discharged after the albumin, I decided that there’d be no harm in having one or two drinks with my sister. We ended up meeting up with one of my school friends who Ross had arranged to come down from Norwich as a surprise, and another couple of friends. And I ended up having a brilliant night and getting totally shitfaced.

Hair officially let down for the night.
Hair officially let down for the night.

6 – Hugo’s age, in months. I won’t go too deeply into how it makes me feel, that he’s six months old (and I’m six months through maternity leave already!), and we’ve spent four of those months living in and out of hospital. Suffice to say, it doesn’t fill me with happiness. Don’t get me wrong. I love the bloody bones of him. He’s totally and utterly completed our lives, and I cherish every day with him more than I could ever put into words. But I so desperately want to be able to really enjoy being his mum, just for a bit, without the constant stress, anxiety and heartache that we’ve suffered with him. To obsess again about sleepless nights, vaccinations and whether he’s hitting his milestones would be such a privilege at the moment. Rather than albumin, bilirubin (which, incidentally, is currently about three times higher than it was before his Kasai in December, and is steadily climbing), hernias and medicines. To my mummy friends reading this – please don’t misconstrue that as me belittling the day to day challenges we all face as parents, or thinking that they are insignificant. I’m not, at all. I know being a parent can be incredibly difficult, whether your baby is well or not. I just wish, from time to time, almost ruefully, that they were my only worries.

5 – The number of NG tubes that poor Hugo has had since last Monday. The first one was a routine replacement, and that should have been it for a month. But for some reason, the tube that we’d been sent as our spare was really short. Not useable, in fact. So just a few minutes after that was put in, it was taken back out again and replaced with a longer one. Then, at 7am on my birthday, Hugo did the most almighty sneeze, and out popped the tube. He managed to pull the third replacement out on Tuesday night, and then sneeze the fourth one out the following morning. His fifth tube of the fortnight, so far (and MASSIVELY TOUCHING WOOD), is still firmly in place. It’s such a shame, as his mood when the tube is out is so different. It’s probably because, when he doesn’t have it in, we’re not forcing a huge volume of milk (800mls a day is the current target from King’s) into him that he doesn’t want (but absolutely needs). I can only guess it’s like when you’re nine months pregnant, and not feeling much like eating, but being forced to eat a giant roast dinner five times a day. It’s going to make you grouchy, uncomfortable, and a bit vomity. On a positive though, we’ve now started to put most of his meds down the tube, too. We know it might mean he has it for slightly longer, but the little guy just needs a bit of a break, and this is one way we can give that to him. He’s even started to let us feed him real, actual food now, without screaming. Which is pretty lovely, and very, very messy.

And finally…

1,989

The number of people who have looked at my blog (and a total of 3,634 views) at the last count. Am a bit amazed by this. Thank you!