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

Days 94–109: milestones

Hugo turns one in a week!

It’s got my head in a bit of a spin. A year ago, I’d just started maternity leave. I was happily nesting, eating cake and fantasising about what life would be like when my little boy came to greet us, totally oblivious to what the year had in store. Now, looking back, it all feels very surreal; blurry, yet very vivid at the same time. Somehow, time managed to slow down while the year flew by, and now we’re here. We’re nearly 16 weeks post transplant, we’ve been at home and hospital free for nearly three months (the longest EVER – touch wood, of course), and we’re fast approaching his first birthday. I feel like I don’t quite know how we got here.

Cheeky monkeys
Cheeky monkeys

Even though I’m obviously really super mega excited about it, I also feel a tinge of sadness when I look back over his first year. When I think about everything he has had to go through and overcome to get to this point, I can’t help but feel that way; no parent should ever have to question whether their baby will make their first birthday, but as his liver disease progressed, we did. I also feel a bit sad that we can’t throw Hugo a huge party to celebrate. Yes, I know he’s not going to know anything about it, and that throwing a party isn’t something I’d necessarily have done if things had turned out differently for us, but it’s one of the ‘normal’ things that I’m actually a bit miffed that we can’t do this year.

I know I shouldn’t really focus on it. I should just feel happy. But it’s hard not to reflect a bit; it’s just who I am. And honestly, I am INCREDIBLY happy and VERY excited. I also know that actually, we’re very lucky that we’re at home for his birthday (TOUCH WOOD!), as that has never been a given. We’ve made some tentative, weather-dependent plans – a picnic in the park this weekend with friends and family, and a trip to the zoo on his birthday. There will be a monkey cake and balloons, of course. And I know that wherever we spend the day, even if it buckets down with rain (as it has so far this week, grr) and we have to cancel all of our plans, we’re going to have a lovely day, because Hugo will be in it. We can’t fail to have smiles on our faces with him around.

Other than my tendency over the last few weeks to think a bit too much about all of that, things have been really bloody good! We’ve had one full set of blood results back and are awaiting the latest EBV results, but everything – as far as we know – looks good. His LFT’s this week are still all normal, which is fab considering one of his immunosuppression meds was reduced to allow him to fight the EBV. And as for the EBV, it has come down from 2million copies/ml (which was up from 70,000 copies/ml) to 200,000 copies/ml, which they seemed to be happy with (although I don’t actually know what the level should be). They’re continuing with the fortnightly bloods for now, and keeping a close eye on it, as ever.

We’re hoping that he will be going down to monthly bloods very soon, because we’ve had a bit of news from King’s this week. We’ve been given a date for his Hickman line to come out! Obviously, we know from experience not to focus on the date itself, but the fact that it’s booked in is a real positive. It’s not for a couple of months, so we’ve got time to get his bloods stable and to the point that they’re definitely happy for it to go. We’ve found the Hickman line to be such a double-edged sword. On the one hand it has been such a massive relief to have it there, so we’ve not had to hold Hugo down for the doctors to prick him to take bloods or insert cannulas. But on the other hand it’s such a restriction – we can’t give Hugo a proper bath or let him roll around without a vest on, in case he pulls at it (which he tries to do) or gets it wet or dirty; it’s a direct line to his heart and a massive infection risk. So now we can look forward to relaxing around him a bit more, and letting him splash around in water again, but we’ve also got to start taking him to hospital to be pricked for bloods again. It also means another stay at King’s, another surgery and general anaesthetic for Hugo (he’s notched up more operations in his first year than me and Ross put together). Ultimately, though, it’s a really good thing; it’s the last piece of plastic leftover from Hugo being critically ill, the last telltale sign (other than his scar, of course), and it’s going!

Proud of my boy and how far he's come
Proud of my boy and how far he’s come

In other news, Hugo started his physio sessions this week. Aside from us being absolutely soaked from head to toe by a passing white van speeding through a puddle on our way in to the hospital (about which I’m still fuming), and Hugo refusing to roll, prop himself up, or generally move at all while we were there after being a bit freaked out by one of the toys they wanted him to play with, it was pretty good. They went through some exercises for us to incorporate into his playtime to help him start to bear his weight on his legs and develop his core strength, and we’re already seeing a real difference! He can now plant both his feet on the floor with straight legs (before they were always a bit weak and ‘froggy’), and he can hold himself up on in the crawling position all fours on his own. All the while smiling and chuckling away at how exciting it is. I know this is going to sound really stupid, but even though I know that the reason he’s not done any of this yet is because of how long he’s spent in hospital, when I see all the younger babies achieving these milestones months before Hugo it makes me feel a bit of a failure. But hearing from the pros that actually, it’s just a lack of opportunity for Hugo that’s prevented him from doing it so far, and seeing such immediate progress, has really helped to snap me out of that particular mindset. He’s getting more and more motivated to move, and now I actually need him to slow down a little bit as I’ve STILL not baby proofed the flat…

So life, in general, is pretty good right now. We’re carrying on towards our own version of normal. Ross is fully back in the swing of work life, and I’m starting to think about going back to work now too, which fills me with all sorts of excitement and dread. We’ve had days out, nights out, baby playdates and visits from friends. I’ve somehow managed to stick to the exercise, although the weekly pizza has somehow wormed its way back into my diet (little sneak). We’ve got a mini break to the New Forest with the fam to look forward to in a few weeks, thanks to my mum. And Hugo is being a little gem to be with; he’s so happy, so cute, so funny, so lively and so loving, it melts my heart. He’s just so much fun to be around (even when his play starts to get a bit vicious and I lose chunks of my hair in his fists), and he seems to be changing and doing something new every day.

Although he STILL won’t say mumma. He just laughs and says dadda when I try and coax it out of him. He’s a little monkey, but I wouldn’t change him for the world.

Days 59 – 93: three months on and National Transplant Week

It’s hard to believe that Hugo’s transplant was three whole months ago. Where on earth has that time gone?! I realise that I’ve not written a blog, or given a Hugo update, for a while; we’ve had a pretty busy month, and thankfully it’s been without a single trip to hospital. August was officially full of home, fun and making memories. In fact, I think this is actually turning into our longest stretch at home with Hugo ever. Since he’s been born. TOUCHING WOOD!!

Before I get onto the Hugo update, I just wanted to do a bit more canvassing on behalf of the organ donation community. Tomorrow (Monday 7th September) is the start of National Transplant Week. The demand for organs is on the rise, due to a mixture of an aging population, alcohol and obesity. But there are fewer transplants happening; there are three people reportedly dying while waiting on the list every day. According to the British Liver Trust, 96% of us would take an organ if we needed one, but only 29% of the population is on the Organ Donor Register. It just doesn’t feel right; it DOESN’T ADD UP. Ross and I recently watched the BBC documentary, Transplant Tales, and we were shocked to discover that 40% of potential donor organs are refused by the family. This is one of the highest refusal rates in Europe. It’s not enough just to sign yourself up to the register; the NHS relies on your family’s consent to allow them to fulfil your wish. So this week, there is a call for everyone in the UK to discuss organ donation. Sign up to the register, but more than that, tell your loved ones what you would like to happen after you die. Talk about it. Actually, scrap that. Shout about it. Make it clear what your wishes are; one conversation could be the difference between you giving a desperately needed second chance to another family, or that opportunity being missed.

Of course, we didn’t end up needing a donor organ for Hugo. We were lucky enough that we could go down the living donor route. But this doesn’t detract from how we both feel about organ donation; we still lived on the torturous tenterhooks of the waiting list with Hugo for three months. He probably wouldn’t be here with us now, heading with great strides towards his first birthday, if he hadn’t had a transplant. His new liver has absolutely transformed him. We are living now, not just existing, not just waiting and watching.

This is a feeling that is only strengthened by the amazing and incredibly inspirational kids (and their families) we’ve met since we were thrown unexpectedly and unwillingly into the liver world nine months ago. Babies and children who suffer from an array of health problems, whose only chance of survival is to receive a life saving gift from a total stranger. Some of these kids have had their transplant; some are still waiting. We are lucky to be able to count these families as our friends, and watch with absolute joy and awe as they fight, thrive and survive against the odds. We go through their journeys with them, as they share in ours. If you ever needed another reason to sign up, then these kids are it.

We met all of these kids at some point during our various stays at King’s. All of them have had a new liver for different reasons. Eli had an liver disease that was so rare, they had to come up with a name for it – Notch 2 gene related liver disease; Tilly, Sam and Mali all had Biliary Atresia; Jess had pancreaticblastoma, a very rare form of cancer; and Harry had acute liver failure at 11 years old. They are all so strong, and such examples to all of us – all of the above pictures are post-transplant. They, along with anyone who has ever received a new organ, are the faces of National Transplant Week. They are why we need to turn the stats around. 29% of the population on the organ donor register?! It’s not enough.

So please, if there is one thing you do this week, it’s have that chat. And then tell the world. Get on Facebook, Twitter or Instagram, and use the hashtag #sayidonate and get people talking. Do it… Please.

As well as it being the start of Transplant Week, Monday also marks another milestone for us. After three months, Ross is declared ‘officially’ recovered and returns to work. He’s done incredibly well – for a man who can’t stand mess, and who happens to be married to a somewhat lazy woman, he must have been chomping at the bit to speed up his recovery at times. And aside from the few instances he’s been told off (both by me, and by King’s), he’s been very patient and let his recovery take priority. We’re both looking forward to him going back to work – it’s another step towards that thing we call ‘normal’, even though we don’t really know what that is anymore. It goes without saying that I’m incredibly proud of him.

As for Hugo… In general, he’s been really well. It’s not been totally smooth (when is it with Hugo?!), but nothing has happened that hasn’t been manageable. When his last bloods came in, the initial results were encouraging – liver function was normal, Tac levels were stable – so King’s told us his bloods would be going to monthly, a really positive step forward. But a few days later, we found out that his EBV DNA was raised. EBV is the virus that causes glandular fever. It is very common, and most people’s natural immune system is enough to keep the virus in check. But in immunosuppressed people, not only can it develop into glandular fever and result in that person being very poorly (and probably in hospital), but it can react with one of the meds and develop into PTLD – post-transplant lymphoproliferative disorder: cancer. King’s have reduced Hugo’s immunosuppressants to allow his body to fight the virus, and told us to watch out even more carefully (although I’m not sure that’s even possible) for signs of illness. This leaves him with a slightly increased risk of rejection, but this is something we’re going to have to get used to – the balance of risks being managed and decided by someone else. We have faith that the team at King’s know their stuff, and are always acting in his best interest. He’s had some repeat bloods now, and we’re just sitting tight for the EBV results. So fingers crossed, everyone!

As much as we feel like we’re settling into our groove now, when it comes to doing stuff with Hugo outside of my immediate comfort zone (that is, being at home, or out with Ross), I’m still struggling a bit. Yes, we’re having loads of fun, living life to the full and we’re making loads of lovely memories, but we’re still doing this within relatively tight boundaries at the moment. I want nothing more than to be able to completely relax and enjoy life as it is, to not worry about whether or not our plans might pose a threat to Hugo. I would LOVE to be a mum who could just take their baby to a social event or play group without a second thought, one who could happily hand her baby round groups of people without having to worry; not only is it good for him in developing his social skills, but he’s becoming such a heavy and wriggly lump now that it’s exhausting keeping hold of him! But I just can’t be that person… not yet, anyway.

Now that we’re starting to get back into (our own version of) normal life, we’re faced with the reality of what Hugo’s disease and treatment means for him, and for us. Although he’s no longer battling with end-stage liver disease, and he’s essentially healthy, he’s still not a ‘normal’ child. His liver disease has not been ‘cured’, it has been (and is still being) treated. On the face of it, he looks totally well, like any other child of his age, so I can understand that people think he’s totally ‘better’. But he’s at much more risk than he seems, and it can be hard to balance that knowledge with functioning in day-to-day life. I’m still too nervous to take Hugo anywhere crowded, so I either don’t take him, or I don’t go. We took him to a family wedding last week, and although the day was beautiful and it was amazing to be out with the family, I could also feel myself becoming increasingly anxious as the day went on; once he was at home being babysat for the evening, I finally felt able to relax. It still felt too soon. I’m slowly getting better at rationalising the risks and my need to be in control. I don’t feel myself physically tensing up when I sense a risk anymore – not as much, anyway – but I’m still a complete slave to antibac. The thing is, I know that as time goes on, he’s going to go off to nursery, to school, to work, and I’m not going to be able to protect him in the way that I can now. I’m just going to have to figure out a way to deal with that as and when the time comes, but for now I’ll keep doing what I can to protect him.

Anyway, Hugo’s really thriving in his own environment now. He’s a bit behind where he might have in his development been at this age, but I think that’s totally expected given how long he has spent in hospital beds during his short life so far. But he’s catching up incredibly well; at their recent visit, the local Occupational Therapist was surprised at how well he was doing, as his referral from King’s had suggested that he would need a lot of attention from them. He needs some physiotherapy; although he is rolling now, he needs some help in developing his leg strength to start crawling and standing. He is always trying to move about, so I’m sure once he’s shown how he’ll be off (that reminds me, I really need to baby proof the flat…) He’s taken to teething on my chin – something that’s becoming increasingly painful as his teeth get closer to coming through (any day now, we hope – he’s still 100% gummy bear at the moment!), but I love it all the same. He’s turned into a total chatterbox (dada, nana, bla bla, no and blowing raspberries are his favourites. Not mumma, of course), and he’s developed some incredibly cheeky characteristics. He knows when he’s being cheeky – he has a telling glint in his eye. It’s beautiful.

As the days go by Hugo is becoming more and more our baby, rather than our baby with a liver transplant. For so much of his short life, he has been defined by what he has been going through, and it feels as though we’ve finally turned a corner with this. Yes, it’s still a major part of him – it always will be, and we have absolutely no problem with that. But it’s just a part of him now, rather than being his all. He’s just gone from strength to strength over the last few weeks, even with a virus lurking in the background. I’m just so proud.

I love my famalam
I love my famalam

Days 37–47: new blood

I reckon some people won’t believe the U-turn we took after my last post. I almost feel a bit reluctant to write about the good side of life at the moment, in case I’m jinxing it. But then, I guess, if I were to follow that line of superstition I’d have driven myself so mad I’d have been sectioned by now.

We lasted about four hours after the last blog was posted before we were on our way back to QEQM with Hugo. Yes, I know. You can’t make this shit up. Thankfully, this time we had only short, if a bit too frequent, stays in hospital. And even more thankfully, something totally random allowed us to catch a brewing blip extra early.

On the Saturday, Hugo wasn’t quite himself – not quite so happy, a bit more lethargic, a bit whinier than usual. But it was a scorching hot day, so you could forgive him. And anyway, we’re all allowed an off day every so often. But we started to worry as every time we picked him up, he screamed in pain. We had Ross’s sister, Amy, over to spend some proper quality time with Hugo (and to have some bubbly with me), and as we were giving him his wash (normally his happiest and most excitable time of day), he was inconsolable. Crying to the point of exhaustion. We examined his underarms, and noticed that he was slightly red and swollen on his right side – the side where his Hickman line is. A quick phone call to Rays later, and we were on our way over to Rainbow.

in hospital
Back in hospital. Sigh.

Without wanting to sound all self-pitying about it (that’s not my style), I really did feel a bit fed up at that moment that every time I’ve tried to plan something for me, it doesn’t happen because we end up in hospital. Ross tells me that it will get better, that I will get my life back, but it can feel pretty bleak at times. I guess it’s all part of this journey, and I know that Ross is right, really (don’t tell him, though!) My mum told me to try and think longer term, essentially backing up what Ross had said; things WILL get easier, we WILL get our lives back. It’s just a case of WHEN. We’re still only just out of the eye of the storm, so we can’t expect it to happen just yet. And even though I know that, it can still be pretty deflating to keep losing my grip on having a life and to go back to hospital life.

We ended up only being at hospital for about 3 hours that night. Hugo perked up loads on the way over, and the redness and swelling had faded by the time we got there. Typical of Hugo, really. They gave him a thorough check over, x-rayed to check the line was in the right place, checked it was flushing and working properly, and took some routine bloods and line cultures (the immediate worry with anything unusual at the moment is a line infection). Everything seemed fine – his liver function tests had all come back ok, there was just the full blood count to come back, so we went back off home. The doctors said they would ring if anything was amiss.

And of course, at 8am the following morning, the phone rang. Hugo’s CRP (infection markers) was high. It should be below 10, but his was 116. They wanted him back ASAP for further bloods, observations and probably to start him on IV antibiotics, even though he had no signs of an infection – usually he has a temperature, but this time there was nothing. King’s wanted him started on antibiotics on Sunday after the CRP was even higher at 166, so we settled in to the room expecting to be there for a week. On the Monday, his CRP had come back down to 116, and the nurse told us that the antibiotics were just once a day, so we could actually take him home and have him treated there! He had a total of five days of antibiotics, and by the Thursday his CRP was down to 30 so King’s were happy that they had done their job. We had managed to nip it in the bud again, even though we didn’t suspect an infection when we took him to hospital; all the while these markers were raised, he still didn’t show any signs of infection – no temperatures, he wasn’t off his food (he even enjoyed his first hospital meal on the Monday, and we all know how rank they are), no cough, no cold. It was weird – where was the infection?!

Unfortunately, it turned out that the infection (we never discovered where it was, by the way) wasn’t the only thing we had to contend with last week. About two hours after we got Hugo home on the Monday, QEQM rang to say that his haemoglobin (HB) was really low, and that he needed an urgent overnight blood transfusion. Obviously, this was a really scary thing to be told. I took Hugo back over, and he had repeat bloods in the early evening. The sample eventually came back and it wasn’t as low as they had thought (it was 68 instead of 55; they want it about 70); the earlier bloods must have been contaminated, or diluted. They thought he would probably need a transfusion, but it wouldn’t happen overnight. So, again, I packed him off back home.

We eventually heard quite late on the Tuesday that King’s did want Hugo to have a transfusion the following day, as his HB had dropped to 66. By the time we’d been told this, the community nurse had been out to take more bloods, and shortly after speaking to QEQM they were back to take YET MORE bloods for the cross match. Now, I don’t know if it’s just me, but this seems totally stupid. Hugo had bloods taken on Saturday, Sunday, twice on Monday and twice on Tuesday. I mean, REALLY? No wonder his HB was dropping – they weren’t leaving him with anything!

Hugo had his blood transfusion on the Wednesday. It took about 3 hours, so we were there and back within a day. And he responded amazingly. His bilirubin had come back as normal on the Tuesday, which is excellent, but the community nurse thought he still looked a bit sallow, a bit pale. As soon as he’d been pumped full of fresh B+ blood he turned the most perfect, gorgeous colour. Pale pink, with rosy cheeks. We finally, for the first time in his life, know what colour our baby should be. We now have a benchmark for him. And not only did he look amazing, he became so much happier and full of energy. I know, I keep saying this is improving. But it really, really is.

Happily, the transfusion has boosted him so much that we haven’t needed to take him back in. So even though we’ve had a blip, since his discharge from King’s on July 1st, the time we’ve spent at home is currently outweighing the time spent in hospital. Hugo’s cheeky character is really starting to shine through now, and he’s doing new things all the time. He pulls funny faces at us. He is almost rolling, and is figuring out how to move to get what he wants. He dives upside down and chuckles with so much glee that I cry with laughter. He comes out on trips to town, for lunch or just a mooch, and is (almost) always as good as gold. He even let us both go out for Ross’s cousins 30th birthday; Ross’s dad and step-mum came to babysit, and instead of us ending up in hospital, we actually made it out. Ross had to take it very easy, of course, whereas I allowed myself to get a bit glammed up and let my hair down for the first time in months. It felt good… Ok I didn’t feel quite so good the next day! But I was buzzing from just getting out and seeing all the family, from having some normality, so it was worth the hangover.

After his transfusion, we saw a blood transfusion practitioner, and she spoke to us all about blood. The whole blood donation process is incredible – another amazing part of our NHS and what it does. There are so many things that you don’t even consider when you’re being treated by our medical system, that you take for granted. She told us that blood bags are measured into two sizes: neonatal, and adult. Nothing for paediatrics, nothing for those between three months and 18 years – not in our area, anyway. So although Hugo only needed 100ml of blood, his bag was the standard adult size of 300ml. The other 200ml has to go to waste. I very naively thought that the blood was stored in some kind of barrel, and they siphoned off what they needed. Donated blood also only lasts for 35 days. That feels like a really, really short period of time. It feels like there was potentially so much being wasted, which just makes the whole donation system even more valuable.

As soon as we got home from Hugo’s transfusion, we signed up to donate blood. I’ve never done it before as I’m rather squeamish – I hate even having blood tests. But I’ve kind of had to put all of that to one side while being in hospital with Hugo. And anyway, even if I didn’t feel a bit better about the actual process, the fact that Hugo has had so many blood products, and a few transfusions, it’s a no brainer to both Ross and me now. We then found out that there was a blood drive in Deal the following Monday, which happened to be our second wedding anniversary. We thought it was the ideal way to spend the day; to give a bit back to a system that has saved our boy countless times. Unfortunately, we weren’t able to get down there, but we’ve got the next date in our diaries and we’re both really excited about starting our donations.

If my blog can achieve anything, it’s to encourage people to buy into the amazing NHS that we have. To help raise awareness, to get people to sign up to the organ donor register, and to give blood. So many people being treated in our hospitals rely on the generosity of others, and sometimes it takes it being so close to home to really highlight the importance of it. Of course, I now wish I’d donated blood for the last twelve years. But I haven’t. And I’m not going to waste my energy regretting that. Rather, I’m going to do something – I’m going to give blood. I know Hugo would give a huge high five to anyone else doing the same.

Days 15–36: little things make all the difference

When your life is whipped out from beneath you, when you take your child to hospital for something relatively ‘simple’, expecting to take them home within a week only to be told that actually, you can’t, everything remains in a spin for quite some time. Living in hospital is a totally surreal existence, one that can only really be understood by those who have done it. I certainly became totally institutionalised up at King’s; barring two days at home and two days at QEQM we were there for 73 days, so I guess it isn’t really surprising.

Happily, we’re now back at home again. And Hugo has lasted an amazing ten days so far! Ok, we’ve spent two days at King’s in this time, but this was planned prior to his discharge. The team at King’s never figured out exactly what was behind his blip, despite all of the tests and scans. He seems to be over it, whatever it was. But because they didn’t diagnose, they couldn’t say for sure that it was dealt with, so they were nervous packing us back off home. Totally understandable. However, I had become rather desperate to get him out of there by then – he wasn’t receiving any treatment at that stage, only four-hourly obs by the nurses – so at ward round on Wednesday we pushed for discharge on the agreement that I would drive him up to London for day clinics whenever they wanted. And they agreed.

What a difference a month makes...
What a difference a month makes…

To start with, being back home was almost as surreal as living on the hospital ward. I had to find my feet again. It’s been a bit scary being away from the security blanket that the ward had become – and I’ll admit that I miss being there a little bit. Not in the sense that I want to go back; obviously, I don’t! But it became very familiar and I felt like I formed some real friendships up there (I’ve caught myself talking like some of the nurses, so I must have been around them for too long), so it’s strange not being around those faces and surroundings anymore. I’ve also been a bit nervous of getting too comfortable, just in case something else was to happen. But thankfully, things, so far, have been good. Really good.

There are so many things that I didn’t realise how much I had missed, how much I would appreciate when I got home. Little things that make all the difference. Such as…

  • Eating off a plate. Ok, that’s really boring. But it’s something I didn’t get in hospital – the canteen food came in polystyrene tubs and the ward only provided paper plates and plastic cutlery. Sure, I’d eat away from the hospital as much as possible, but even then you weren’t guaranteed a plate; burger on a board, anyone?! Chips in a mug?! NO. JUST NO. Put it on a plate, please.
  • Fresh, home cooked food. Mostly, anyway; I was pleased to see that the local pizza takeaway still had my number stored on their system. Having a fridge full of fresh food, and not having to label it ‘Hugo Wooding Bed 6’, is blooming lovely. I’m not even bothered with the washing up, something I NEVER used to do – it’s a symbol of normality to me, and I’m actually enjoying it… a little bit, anyway.
  • Being near my friends again. I’ve missed so much of their lives – growing babies, moving away, changing jobs; just life carrying on in general. It’s nice to finally feel like we’re back in the fold a bit. Everyone has been so excited for us to be back, but also really understanding of our restrictions at the moment, so we feel incredibly supported and very lucky. Which makes the whole transition a lot easier.
  • Living with my husband. Even though we spent as much time as we could together while we were at King’s, it felt a bit like we were back to dating – we’d spend a few hours together, maybe go for dinner, then we’d go and sleep in different places. It was a weird place to be in after seven years together, and it only really hit me how bloody awful it had been when I got back. Ross is still recovering, and is doing really well; his secret abs are healing (during his surgery, they discovered that his core muscles were actually really tight. Abs. They were a bit surprised, though, as no-one could see them…) and he’s getting stronger every day. Having some quality family time, on our own, and seeing my boys play together after everything they’ve been through is the best feeling in the world.
  • Having freedom. Having variety. Having my own transport. Not having to ask permission to take Hugo out for an hour. Being in charge again. Something that I definitely took for granted before, and something that I now appreciate more than anything. And we’ve certainly been making the most of it with slow walks and lazy days going where we want, when we want. Bliss.

Then, of course, there’s Hugo. Where do I start?! Well, first off, Hugo’s two day appointments at King’s were just that – DAY appointments. We’ve spent every night since being home in our own beds. His bloods are going in the right direction – his bilirubin is now nearly back to normal, his Gamma GT is now below 200, which is the best yet since transplant, although there’s still a way to go. His other key liver function tests are all within normal range, and his scan was fine. His colour is really good, his eyes are becoming beautifully clear, his poos are still nice and dark, and his pee clear. So far, so good.

Aside from Hugo’s clinical wellness, he has made one other major step forward since coming home. He drinks! Actually, he doesn’t just drink – he guzzles. We decided to try adding Nesquik to his milk last Wednesday, just to see whether it really was, as we suspected, the flavour that he didn’t like. And hey presto – just like that he started feeding, and he hasn’t looked back. He’s not even that bothered by taking his meds orally now. In fact, he’s doing so well that on Saturday, 116 days since we first started the NG feeding and 36 days post transplant, we took the tube out! This was a massive milestone for us – we didn’t think it would be out so soon. His feeding has been one of the biggest struggles we’ve had through all of this, and we’d have been happy if he was feeding and we could take the tube out by his first birthday in September, so for him to achieve it so soon is just incredible. And it’s made a huge difference to him, too.

Hugo has always been a happy boy. But his happiness has reached new levels, and losing the tube is a huge part of that. When he sees his bottle, instead of screaming and getting freaked out he goes crazy with excitement. Mealtimes have become so much more fun, as we don’t have to worry about him getting puree all over his tube tape; we can relax with him. He’s trying new flavours every day, and he’s even starting to eat some of the food I’ve made him. If he sees a Dairylea triangle, a yoghurt pot or a banana, he goes totally beserk. He HAS to have it fed to him NOW. He CAN’T wait, it HAS to be NOW. He’s a right little chunky bum, and it’s amazing. It feels like we really have our baby boy back now; he was always such a gannet in the first couple of months of his life, and we lost that part of him for a while. He has done us so, so proud.

Hugo is, in fact, so happy now that he won’t take naps. Even when he is clearly shattered, as soon as we put him into his bed he wakes up, ready to play. I genuinely think it’s because he now feels so well, he doesn’t want to lose any hours in the day to sleep. He has a jam-packed schedule of bouncing in his jumparoo, learning to roll (he’s really, really nearly there) on the play mat, sitting up, watching cartoons (normally Peppa Pig, which I absolutely love), flying, chatting, and laughing his head off to do. There’s just no more time for sleep.

I keep having to pinch myself to believe that this is our life at the moment; it seemed so far away at times, and every time it was nearly within my grasp it slipped away again. I know it may not last indefinitely; he’s going to face challenges as the days, weeks and months go on. But right now, this is what I imagined being a mum would be. He’s home, he’s happy and he’s thriving, and it makes the last few months so, so worth it.

My happy family :)
My happy family 🙂

Updates (days 1–13) and ground rules

Sorry for the delay between blog posts again. I know everyone has wanted an update. But I’m sure you‘ll all understand that my boys have been keeping me pretty busy… We’re now at day 13 post-transplant, and things (SO FAR!) seem to be ticking along in the right direction. Touch wood, fingers crossed, and all that.

Overall, Hugo is doing pretty blooming well. His bloods are generally within normal range now, with a few exceptions – and nothing the doctors are currently concerned about; they expect them to fluctuate a bit as things settle post-op. His ultrasounds haven’t highlighted any major issues. He’s now rid of all of the surgical plastic that adorned him for the first few days – arterial and neck lines, ventilators and breathing support, catheter and drain ALL GONE. And his jaundice is all but cleared (and his bilirubin is currently normal!!!) – his skin and eyes look amazing, even if I am scrutinising them somewhat for tinges of creaminess out of paranoia (and yes, my mum, Justine and Gill are all telling me off for doing this!), his wee is clear and his poo is dark! He’s still not drinking his bottle yet, but he’s tolerating the NG feeds and he’s doing incredibly well with his solids, and he’s gaining good weight – 100g in 4 days is pretty swift!

The important thing in amongst all of the ups and downs happening every day is that his liver is still working. His daddy’s liver is doing its job. His body is still dealing with what was, essentially, a major trauma, so it is natural for things to peak and trough as the days go on. They’re monitoring his bloods daily, keeping a close eye on liver and kidney function, immunity and immunosuppression. So far, it seems to be going quite smoothly. But this is Hugo, and he doesn’t like to do things the easy way. We’re all expecting a bit of turbulence. But one major comfort, to me, anyway, is that turbulence is expected, and isn’t by any means catastrophic. And because they’re keeping such a close eye, they’ll spot any potential problems at their very early stages. They’re on top of it.

The other thing I must mention is his mood. I’ve always been super proud of Hugo for maintaining such a sunny disposition throughout his life so far. And even though I was told to expect a difference in him post-transplant, I didn’t really think he could get much happier. Well, I was wrong. It turns out, he can be MUCH happier! On Wednesday evening (day 5), something suddenly clicked in him, and he just started kicking around, gurgling and smiling. It was almost as if he realised how bloody amazing he felt! And then on Saturday (day 8), he started laughing for the first time. Ever. A proper little belly-laugh – without a doubt the best sound I’ve heard in my entire life. It’s like he’s seeing everything again through new eyes – new, mesmerising white eyes – he never knew he was ill, so I guess he just didn’t know how well he could feel. It’s made me pretty giddy with happiness. The only thing missing from the picture right now is Ross, and home.

As far as Ross goes, he’s been back at home now getting some serious R&R in before Hugo and I eventually make it back. He’s got a long old road ahead of him – 3 months off work – but he’s doing amazingly well so far. He’s had his first check up since being discharged and the Prof is happy with everything. Luckily, he’s had his mum with him the majority of the time while he’s been at home and I’ve been up here, with other family members all pitching in with a food shop, taking dinner round or just giving him some company and a helping hand. It’s taken a massive weight off my mind, and even though I miss him to bits, it’s much better for him to be home than for me to keep trying to look after both of them up here so soon after they’ve both had major surgery.

As for home… well, I’ve deliberately not been asking the doctors about home yet. And although they’re starting to mention the H-word, I don’t want to get fixated on a possible date, only for it to move and then to feel deflated. So even though I’m dreaming of it – my sofa, my bed, my bathroom, my family and my friends – I’m making myself remain focused on getting Hugo home WELL. If that takes more time, then so be it.

We have, however, started to thing about home in terms of the reality of Hugo now being a post-transplant kid. He no longer has Biliary Atresia. However – and this is a BIG however – his transplant is not a cure, and even though it has made him better, in that he is no longer a chronically and critically ill baby, it is not the end of his liver journey. His challenges have not disappeared; rather, they have changed. So I wanted to explain a bit about what this means in real terms for Hugo, and lay out some ground rules for when we do get him home. I’m sorry if this dampens the mood of Hugo being well a bit. I don’t mean to put a downer on the party. But it’s necessary. Ross and I don’t want to take the fun out of us being home, or out of Hugo’s life, but the simple fact is we have fought long and hard to get him back to being well. And we want to blooming well keep it that way!

Everyone following Hugo’s journey will know that he needed this transplant to give him life. But, even though it’s given him life, it’s not the same life as most other children will lead. In order for Hugo to do well with his new liver, he will be on immunosuppression (tacrolimus and prednisolone) for life. This means that he will be much more vulnerable than most when it comes to colds, viruses and infections. Yes, over the years, Hugo will need to develop his own immune system. And to do that, he will need to be exposed to various germs. But right now, at the start of this new – and hopefully improved – chapter of his life, he does not need to be exposed to anything unnecessary. For Hugo, certain viruses can actually be quite severe, and infections can be very high risk. Something that may just be mild to you or me could hospitalise him – he won’t be able to fight things in the same way. He will also be at a greater risk to some cancers because of his medication; glandular fever (EBV), in particular, is a known risk to him developing post transplant lymphoma.

So while we want to have the most “normal” (hate that word) life possible for Hugo, I’m sure you can all understand we’re not willing to take any unnecessary risks when it comes to his health. Particularly with Ross in his line of work! So, until we specify otherwise, these are the rules:

  • If you have any symptoms of a cold or virus at all, however mild you may deem them to be – STAY AWAY!
  • If you have chicken pox – STAY AWAY! Chicken pox can be very harmful to Hugo, so we must be informed if you think he may have been exposed to it at any time.
  • If you have a cold sore – STAY AWAY! One of the major risks he is exposed to is the cold sore (herpes simplex) virus. It is very contagious and can attack the liver directly, particularly in the first year.
  • No kissing Hugo on the face. See all of the above.
  • Always, always, always wash your hands – thoroughly. It’s the best defence we have against anything, particularly infections. Whenever you are interacting with Hugo, you must must must wash your hands. Before any contact, before feeding, after nappy changes etc. So don’t be surprised if you want a cuddle and we ask you to do this first. Hopefully, in time, this will become a matter of course – we know it already is for our close family. And I apologise in advance for the horrid dry hands you’ll get if you are around Hugo a lot – these are now part of the territory.
  • Speaking of cuddles – please don’t be surprised if we don’t hand him round for cuddles, particularly when we’re in groups. If we don’t offer Hugo cuddles, there’s probably a reason – so please don’t ask.
  • Please do not expose Hugo to smoke. Actually, this has always been one of our rules. We will not take him anywhere smoke-affected, and ask for you not to bring it to him.

The coming months and years are going to be tricky, but as things go on they will get easier. As Hugo slowly gets stronger, and his bloods start to stabilise, we will be able to slowly relax the rules. After all, he does need to go to school and work eventually – we can’t keep him wrapped up in cotton wool forever! But this process will take time. We are asking our friends and family to be patient and back us in this process.

One thing we don’t want is for people to feel like we have to be avoided. We would much rather you make plans and have to cancel them than avoid making plans to see us altogether.

The other thing we’ve discussed is the immediate few days after coming home. Although I’ve desperately missed everyone back home, we haven’t lived together as a family for nine weeks now. When we do get back home, we will want – and need – some time to adjust to Hugo’s new routine, his new meds and his new challenge, as well as just simply enjoying having him home and watching him flourish and start to catch up with his milestones (he’s already starting to sit unaided now). Just spend some much-needed time together as a family. Husband, wife and son. And jumparoo, of course! So for the first few days, we will not be seeing anyone – that means ANYONE. We won’t even answer the door. Once we’re bored of each other’s company, we’ll start to arrange to see people.

I hope everyone can understand and support us while we are in the transition period of being back home. It’s going to be hard enough, when the time comes, to be away from the safety net of the ward. Ultimately, the single goal we have – and we feel you share – is for Hugo to have a long, healthy and happy life. The last thing we want is to be under pressure to do things with Hugo that we’re not yet comfortable with. Please, give us time. We will get there. And, to be honest – if you don’t get it, tough. This is one area in which we are not prepared to compromise. But I know that those who are close to us will really get it and support us. So thank you, in advance, for that.

Third time lucky!

On Friday 5th June 2015, exactly 14 weeks after we were told that he needed a transplant and after two failed attempts, it finally happened.

HUGO GOT A NEW LIVER!!!

Yayyyyyy!!

A quick warning before I start – there are pictures of the livers further down. Read on at your own risk!

Hugo’s been stable over the last fortnight – which is why I haven’t blogged in a while; there wasn’t a huge amount to say. He’s been a very active and playful boy, and needed very little clinical intervention from the doctors; we were pretty much just lodging on the ward for the last week. However, as we’d had this date in mind for transplant since the first cancellation in May, and given the difficulties we’d had getting him into surgery, the team has remained incredibly focussed on getting him ready for his op. Everyone was determined that this would be the date – Hugo needed it to be done – and so they really did do everything possible to make sure it happened, including keeping him in isolation when he had cleared the adeno (although I think this was as much to do with my nagging about it as anything else – anything to shut up the anxious mother!) Despite him being in the best possible condition as the week progressed, Ross and I were still very nervous that we would get stuck at the intubation stage again, and had several chats with the team about whether they would take Hugo down first; we were scared that we would have the same situation when they were halfway through Ross’s surgery, and that we might lose Ross’s liver as a result. But on Thursday we were told – firmly – that this was not the way they did things, and they weren’t going to change their protocol. They were totally confident – and, we realised that if they weren’t totally confident, they probably shouldn’t be taking him down in the first place. We had to trust them.

Ross and I took time out to spend a nice evening together on the Thursday – we went to a local pub for dinner, and then just chilled out together until Ross was admitted onto his ward for the night. I wasn’t expecting either of us to get any sleep, but somehow we both managed to get some. I got up with Hugo at about 5am, and Ross came up at 7am in his surgical gown to have some cuddles before he went down. Mainly with Hugo, of course, but I managed to steal some. My sister had come up the night before and stayed at Ron Macs, so when Fiona (Ross’s lovely coordinator) and the Blues (as she called them) came to get Ross just after 8am, I left her with Hugo and went down with him, and I stayed with him until they kicked me out so they could get him under. It was scary leaving him, knowing that the surgery was happening – they had the first one of my boys.

Hugo, despite being hungry from being nil by mouth, was in a pretty playful mood when I got back up to him, so the time from then passed quite quickly. Fiona turned up at our room at 10am – I thought it was his turn to go down. But she’d come to tell us there was a delay because of another emergency that had happened. As frustrating as it was, there wasn’t anything I could do. Emily (my sister) later said how surprised she was that I hadn’t flown off the handle. But I knew there wasn’t any point, and I didn’t want to make myself any more stressed than was necessary. And as it turned out, Fiona was back up half an hour later, this time with Justine (Hugo’s lovely coordinator) and the Blues in tow, coming to collect my boy.

Even though I’ve done the walk down to the theatre before, this time it felt different. I knew it was really happening. I was so scared, but I was also relieved, as I knew that meant that Ross’s surgery must have been going to plan. After I handed Hugo over, I couldn’t feel my legs. Shock kicked in; all this time, we’ve been trying to get to this point, and now it was happening. I still didn’t believe it until I heard (quite quickly) that Hugo had been safely intubated! But it was real. Now I just had the wait.

The time actually passed quicker than I thought it would as the day progressed. Emily arranged for her boyfriend to bring my niece Violet up for the day, and she provided the perfect distraction. She didn’t know what was going on, and although I was incredibly grateful to the support I had (as well as my sister and Chris, Ross’s mum came up with a friend, and we had a huge amount of support from our friends and family at home and at the hospital for which I am incredibly grateful – although unfortunately my mum had a cold so I couldn’t let her come, which was really tough), Violet just lightened the mood, running round the garden at Ron Macs, waving at planes and playing on the swings. It’s very likely I would have been sat in silence just watching my phone, worrying and obsessing over what was or wasn’t happening if it wasn’t for her.

At about midday, I heard that Ross’s liver was out, and they were going to start closing him up. The news was that his liver was PERFECT. Hugo’s extraction, however, was taking much longer than anticipated, as he had lots of adhesions to his bowel. In the end, they had to remove a sizeable chunk (about 7cm) of his bowel and reconstruct it – it must have been pretty severe. But they got the liver out, and Ross’s went in. The plumbing-in of the arteries, veins and roux loop (the new bile flow system formed in the Kasai back in December) then went smoother than expected. By the time I was able to see Ross was in intensive care, late afternoon, I was given the news that Hugo was about to be sewn up!!

When I got to Ross in intensive care, I found out that the epidural that was meant to be providing his pain relief after surgery hadn’t worked, so he woke up from the operation (bearing in mind this is major abdominal surgery) with absolutely no pain relief. It took them a while (far too long, in my view) to get him sorted and comfortable; he ended up on a morphine pump, which he definitely made the most of (FYI, morphine-drunk Ross is HILARIOUS). But given the trauma that he went through, and the fact he had no pain relief at all to start with, he did AMAZINGLY. When I told him that Hugo had his liver, and that he was well and he just smiled. You could see the relief on his face. I think he’d have taken any amount of pain to hear that news.

Back on the ward - the morphine-drunk hubby. No. 1 dad!
Back on the ward – the morphine-drunk hubby. No. 1 dad!

When I saw the surgeon, Hector, he talked me through the operation. After I’d given him a huge hug, of course. All in all, they were very happy with how it had gone. He said, again, how good Ross’s liver was – if they could have designed the perfect liver for Hugo, that would have been it. It just goes to show how things are meant to be – if he’d had the cadaveric liver a fortnight previous, they wouldn’t have been able to close him up. But they managed to close him with Ross’s – muscle and skin. He was also happy with how the ‘plumbing’ had gone – the blood seemed to be flowing well, and it had showed early signs of functioning. The liver was good.

Hugo’s liver, on the other hand, had been in a terrible state. It was, essentially, a dead organ. Black, green, bilious and rock hard. Like a piece of evil that was squatting inside my son. Hector told Ross that it’s up there with the worst he’s seen in a baby that age. How Hugo lasted as long as he did, and how he found the strength to be as alert, as active, and as happy as he was, just astounds me. I knew he was doing well, but to see the real, stark truth of what was happening inside him was a real punch to the stomach. I think it’s safe to say that he couldn’t have lasted much longer without his transplant – and this was really why we were kept in hospital. So they didn’t let him die. It was incredibly upsetting, yet at the same time it just made me even prouder of him (if that was even possible) that he just carried on regardless. I knew he was very, very poorly, but as strange as it sounds, I never really saw it in him, as he never acted poorly. He had a resolute will to live. I am so in awe of my baby boy.

Because of the adhesions, and the severity of the diseased liver, Hugo is at a higher risk of complications post-transplant. His bowel walls are thin and damaged, so he may suffer a perforation. And his portal vein is at risk of blockage as it is very narrow, caused by the portal hypertension (his body had formed shunts to deliver blood to the liver, after which the veins can become narrow and collapse). If he does suffer a perforation or blockage, he will require more surgery. Suffice to say, they are keeping a very, very close eye on these things. And even though I hate the thought of him going in again, I don’t feel as scared, as I know he doesn’t have that thing inside him any more. I feel like we can conquer his obstacles now.

I finally got a call from Fiona at 7.30pm to tell me that Hugo was closed and up in PICU. When I got that phone call, the relief finally flooded out of me. Whereas that morning, I’d felt full of lead when both of my boys were in, I suddenly felt the weight lift. It was done. They were both safely out of surgery, and in intensive care. After 98 days of waiting, it was finally done. Hugo had a new liver; he no longer has Biliary Atresia. Even though we know the next stage won’t be easy, we are finally on the other side of that hurdle; one where we can believe our little boy will get better and start to have the life he so truly deserves. We can finally start to look forward.

We’re now at the end of day 5 post-transplant, and they’re both doing well. I’m trying my best to split my time between the two of them; it’s not easy, as I want to look after both of them as much as I can. But the exhaustion is so worth it as I see them both get better. Ross is still fragile, and he’s getting tired very quickly. But his scar is looking great and he’s getting stronger by the day. He’s now getting out of bed and has made it up to see Hugo a couple of times, and they’re now talking about his date for discharge. Unfortunately, he’s getting totally crap care on his ward. It’s surprising, as King’s is such a good hospital, but his nurses genuinely don’t seem to care that he’s had major surgery; on day 2 they removed his cannulas and told him to take paracetamol tablets instead of having IV. I mean, really?! Thankfully, Fiona has been kicking butt for us, and they’ve been bending their strict visiting hours for me so I can get in and help him out a bit, but it’s added to the stress of it all unnecessarily.

And Hugo… well, at the moment (touch wood and everything), Hugo looks just incredible. He’s had some ups and downs already – all par for the course – which made the weekend quite difficult. But he’s overcoming the challenges, hour by hour and day by day, and taking the small steps that we need him to take. His colour is already so improved – his bilirubin (the part of the blood that turns him yellow) is now at about 60, so he looks much less jaundiced. It should be under 10, but it went up to about 450 at it’s highest. His belly looks amazing – his ascites has totally gone (you’d hope so after they took over a litre of fluid away from his belly on Friday!) But that’s not the important thing. The important thing is that the liver is currently working! The liver function tests are all returning to normal ranges, so far. The graft seems to be working. It’s VERY early days, of course. We’ve been told to expect a bout of rejection – it’s quite normal. And although he’s off the breathing support, out of PICU and back on Rays, he’s still at risk of a range of complications. He’s not yet feeding orally, but he’s tolerating milk well and everyone seems confident it won’t be long until that picks up. We’re not out of the woods yet. We’re taking each day as it comes. And so far, so good.

I still can’t find the words to describe how I feel about what Ross did on Friday. I choke up when I think about it. Lots of people have called him a hero – including me – but it doesn’t really do it justice. Neither does proud, even though I am – VERY proud. I’m actually totally in awe of him. Ross is my best friend, he is a wonderful husband and an amazing father. But he’s done something in the last week that has touched my heart in a new way. He saved my baby’s life. He kept saying that it’s what anyone would do in this situation – that may well be true, but he actually did it. He gave Hugo another chance. What do you say to the man who does that? Thank you, I guess!

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Day 5 – I have a HERO and his name is DADDY – my boys, my world, back together

Third time really was lucky for us.

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.

The thing about Hugo

It’s a strange thing, having a poorly baby.

In the time immediately after Hugo’s diagnosis it was near impossible to see beyond his illness; it will, after all, be with him (and us) for life. Our conversations about him centered around his liver, his colour, his treatment and whether we think he needs a transplant, rather than ‘normal’ baby talk. It took a while for me to really start to see him as my baby boy again – that feels really strange to say, but it’s true. Because of course he’s still our baby. Yes, he has a chronic liver disease, but that’s only one part of him. And if it wasn’t for the (currently few) physical signs – the scar, the swollen belly, the jaundice, the medicine – you wouldn’t know he was poorly.

He doesn’t act poorly. He acts in exactly the same way he did before he started being poked and prodded by the doctors on a weekly basis. It’s amazing. If it were me going through what he is going through, I would probably (definitely) be feeling more than a bit sorry for myself, and would probably be a massive pain in the arse because of it.

Three in a bed. One of his recent stays in hospital.
Three in a bed. One of his recent stays in hospital.

But not Hugo. He’s got such an awesome little character coming through now, there are times where I genuinely forget that he’s so poorly, which is a blessing in itself. I’m sure every parent reading this will understand that feeling of bursting with love for your baby; you think you couldn’t possibly love them any more, but every day you do.

So I thought, before the blog progresses into more difficult, sometimes serious topics, I’d share with you what makes him into the funny, brave little boy he is.

He’s strong. He bounces back from the physical pain he goes through at an incredible rate. The day after his operation, he did a big sneeze. Ross and I froze, worried that he would have really hurt himself (I was in so much pain after my c-section I couldn’t even laugh. Sneezing would have been agony. But we don’t need to go into what a wimp I am…) But Hugo didn’t even flinch. He just carried on like nothing had happened. And even though he’s had so many blood tests we’ve lost count, he just takes them in his stride – at his last one, he didn’t make a sound. He let the doctor get what he needed, and he carried on playing. I admire him so much for that.

Despite all of the fluid on his belly, he is still loves a bit of tummy time. As insignificant as this may seem, it makes me very proud.

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Poor, abused Mickey…

He’s awesome at playing. He really loves monkeys – give him a monkey toy to play with and he’ll happily smoosh it into his face for hours at a time. But more than anything, he just likes to have toys on his head. We regularly wake up to find him sleeping soundly with his Mickey Mouse teddy lying somewhat inappropriately on his head; goodness knows what poor Mickey goes through each night to get there.

He loves The Simpsons. We think he probably feels an affinity for them and their yellow skin…

He sleeps. Seems simple, but it’s something that eludes many of my mummy friends. Sure, some nights we need to help him settle every hour, but those nights are becoming the exception rather than the rule. Most nights, he’ll stir, do a cough and a fart, and go back to sleep. He’s such a boy. It genuinely makes my day.

He is a little chatterbox. He used to talk to us by poking his tongue out, but he’s now found his voice, and boy does he use it! It is the most perfect sound I’ve ever heard – it makes me melt every day.

My smiley boy.
My smiley boy.

And last, but most definitely not least, he always greets us in the morning with a whopping big smile. On the days where I wake up feeling like I can’t do it, I can’t cope with what lies ahead, he gets me through it, and manages to put a smile on my face and a spring in my step.

That’s the thing about Hugo. Despite everything he has to go through, he remains a thoroughly happy little boy. For every tear I’ve shed, he gives me a thousand more smiles; for every time I’ve felt helpless, upset or angry about his illness, how it will affect all of our lives and just how bloody unfair it is, he makes me feel ten times happier that I get to be the one to share all of this with him. Yes, being the mum of a baby with a liver disease is difficult, and completely heartbreaking at times, but the thing is that even through that, he makes me feel so lucky. I’m just so unbelievably proud of him.

BA-Aware

Seeing as one of my main motivations for this blog is to help raise awareness of liver disease, it seems appropriate to kick off with a quick lesson in jaundice and Biliary Atresia.

Quick disclaimer – all of this comes from our education at King’s and own understanding of the disease. Every case, like every child, is different, so how Hugo presented and what has been through may not be true of others.

Biliary Atresia is a rare liver disease (affecting approximately 1 in 15,000 babies) where there is a lack of bile flow from the liver to the intestines, caused by an inflammation or blockage of the bile ducts. The reduction in bile flow causes damage to the liver, where the acid is stored up, and can also affect the digestion of vital nutrients (for which bile is essential), potentially causing failure to thrive. Despite research into the disease, there isn’t a known cause yet.

The three main signs to look out for in BA are prolonged jaundice, yellow wee (should be pretty much clear in young babies) and pale poo (I’m talking chalky grey/white).

It is relatively common for babies to be born with jaundice (yellowing of the skin and eyes); what isn’t so well known is that there are two types of jaundice. Newborn jaundice (physiological) will usually clear within a couple of weeks, and isn’t an indication of liver problems. However, if the jaundice doesn’t clear in this time, it can be the first sign that there is something more sinister going on.

Once the condition is suspected, there are several tests that are carried out, including blood tests, ultrasound and a biopsy. Once the diagnosis is established, the baby must have an operation called the Kasai, with the aim of removing the affected bile ducts and gall bladder, rebuilding the digestive system and re-establishing the flow of bile from the liver to the gut. The success rate of the procedure is pretty low; about 25-30% of babies will retain their own liver afterwards, with the rest needing a transplant at some stage of childhood.

There are also various other problems that can occur with this liver disease, including cholangitis (an infection of the bile ducts in the liver), ascites (collection of excess fluid in the abdomen), portal hypertension (increased pressure in the blood vessels), pruritus (itching) and failure to thrive.

A yellow-tinted Hugo in hospital awaiting his Kasai. December 2014.
A yellow-tinted Hugo in hospital awaiting his Kasai. December 2014.

Hugo was born with jaundice that, following our discharge from hospital when he was born, was never followed up by any medical or health care professional he saw (apart from the standard “just put him in the sunlight”). It was (and still is) clinically pretty mild, however it was there nonetheless. He also had bright yellow wee from a very early stage; something which I asked about but was told it was totally normal. The first time it was suspected that there was something serious going on was when his poo went pale at seven weeks. Suffice to say, I feel devastated that, now I know the signs, I know that the BA could have been picked up sooner.

Once the alarm bells rang with the GP, his diagnosis and resulting treatment happened very quickly. He had his Kasai at ten weeks, and the assessment from the surgeon after was that, although the damage sustained by the liver was consistent with the bile building up from day one, he was relatively positive about Hugo’s chance of keeping his liver. Unfortunately, Hugo has suffered some of the complications mentioned above (to date, cholangitis and ascites), and his regular liver function tests are showing a progressive decline, rather than improvement. Everything seems to be pointing towards Hugo needing a transplant.

The specialists can never say for sure that Hugo’s prognosis would have been any different had he been diagnosed and had his Kasai earlier; I’m convinced that the early signs being missed has made a difference. I would urge all parents to learn and remember these signs, as the effective treatment of Biliary Atresia is time critical. Early diagnosis and treatment is so, so important. So please, spread the word. We are proof that you can’t always rely on the professionals to know it themselves. But that is a topic for another time.