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.

IMG-20160305-WA0000_1
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.

IMAG9488
Happy Woodings xx

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!

11406823_10100178470553235_6491650173096977539_n
Day 5 – I have a HERO and his name is DADDY – my boys, my world, back together

Third time really was lucky for us.

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!

News, a feeding tube and a Very Bad Day

Hugo had his transplant assessment last week.

It was intense. Over two days, we met our (lovely) transplant coordinator, Justine, who gently took us through everything. We saw an anesthetist, surgeon, psychologist, dietician, social support worker, consultant hepatologist and clinical nurse specialist. Hugo had an ultrasound and an ECG, bloods taken and an albumin infusion given. All in all, it went pretty smoothly – in terms of the condition of his liver there were no surprises. Things are getting progressively worse, he needs a transplant… all things we’ve been told before and were expecting to hear.

My one fear in the lead up to his assessment was that he was going to be put on a feeding (NG) tube. We knew that Hugo’s feeding wasn’t up to scratch, but we thought we were just about managing it for him. Unfortunately, we were wrong on this one. Even though his weight has gone up, his arm measurement has started to drop –an indication that he’s not getting enough calories (his weight is skewed by the ascites, so increases aren’t necessarily a good thing for him). It was pretty hard to accept that we were going to have to go down the NG route – two of the things we’ve consistently felt pretty good about were that he was a good feeder, and that he didn’t look poorly on the surface. Suddenly, these were no longer true. He’s going to look like a poorly baby. Everyone is going to be able to see this tube coming out of his nose, and the thought of people looking at him in pity really upsets me. And the fact I can’t get him to drink makes me feel like I’ve failed a little. I know, really, that isn’t the case, but giving your baby nourishment is one of the most basic of parts of being a parent. No matter what we try, we just can’t get him to take what he needs. So we know that if they are insisting on it, it has to be for good reason.

We got home on Wednesday evening, and had a really enjoyable week. Despite having been a bit more of a grouchbag over the last couple of weeks (he’s probably, understandably, feeling totally fed up, as well as lethargic from the dwindling energy supply) Hugo was his usual cheeky self, doing lots of bouncing in the jumparoo, and learning lots of new tricks (reaching out and grabbing is his current favourite). I had my first ever Mother’s Day – not a day we normally celebrate much in my family (barring the famous ‘frog on skis’ I made for my mum once, aged 8 (ish) and again at age 22), but a treat nonetheless, mostly because Ross cooked me bacon for the first time EVER, and I got to stay in bed beyond 8am (with lots of Hugo cuddles during the day too, of course).

A yellow Mother's Day pressie from my boy. How apt!
A yellow Mother’s Day pressie from my boy. How apt!

On Friday, we spoke to Justine and went through the next steps following Hugo’s assessment. Hugo was to be put forward for listing at the transplant team meeting the following Tuesday. I also spoke to the staff at Rainbow ward and penciled in a date to take Hugo in for his NG tube and our training. Also Tuesday. All of a sudden, we had a massive, really important day looming ahead of us.

Monday came, and was what can only be called a Very Bad Day. I woke with a terrible headache, Hugo woke in a grump, and it didn’t get much better. He wouldn’t feed, he refused to nap, he cried – a lot. In spite of my mum coming over to lend a hand, I was totally stressed and suddenly feeling very exhausted and unable to cope. The reality of what was going to happen the next day – him (hopefully) being listed, and the NG tube going in – got to me. We’re actually quite lucky that we don’t have many bad days – we’ve proved ourselves to be pretty good at coping with everything, accepting the path Hugo’s disease is taking as it unfolds and facing the challenges head on. But we do have them. I think the fact that we know we are good at coping helps to us get through of the down days. Having such an amazing support network helps, of course. When Ross got home from work he sent me to bed, and did Hugo’s night feed so I could get a decent rest to build up some energy for the week ahead.

Happily, Tuesday has been and gone, and we’ve got these two hurdles out of the way. Not surprisingly, having the tube inserted was pretty traumatic, both for us and Hugo (although nothing a lot of cuddles and repetitive singing of ‘Incey Wincey Spider’ couldn’t fix). It’s actually much easier to use than we thought, and it’s taken the weight off our shoulders too; we no longer have to fight with him to try and get the volume he needs in. It’s slow going – we’re still working out how much he can tolerate, and we’re yet to meet the daily target from King’s – but we are getting more in him every day. And it’s helping get him back to his normal, chirpy self. We’re back to getting giant smiles when he’s awake, and it’s bloody lovely.

Beaming smiles and full of beans!
Full of tube, beans and big beautiful smiles!

The transplant team also confirmed that Hugo would be listed once he’d had his echocardiogram. We took him to London on Thursday for that, and he got the all clear, so we’re expecting it to happen any time now. We’re still at the very early stages of this part of his journey, but we’re happy that things are moving forwards.

After two nights at hospital (with yet another day of albumin – that’s nine so far!), we’re now back home again and settling back into yet another new routine. Having so much disruption is frustrating, to say the least; it seems each time we get back into our groove, the rug is whipped out from under our feet again and we’re off for a couple of days in hospital. But Hugo is incredibly adaptable and resilient, and if he can take it all in his stride (minus a couple of off days, which he’s more than entitled to!) then so can we. Personally, I think he quite enjoys all of the attention from the nurses when he’s in there – he’s already a massive flirt, charming the pants off them. We’re going to have to watch that when he’s older!

Hugo update – the next step

Last week we had Hugo’s liver review at King’s. We’ve been building up to the appointment for a couple of weeks, with both Ross and I convinced we’d be told the news that Hugo needed a transplant. Of course, I’d have much rather if we’d been able to be positive about his prognosis, but we couldn’t ignore what was staring us plainly in the face.

And – as seems to regularly be the case – our instinct was right. Hugo’s liver is badly damaged and is failing. He needs a new one.

As the appointment progressed, we were told we would be kept in with a view to give Hugo several albumin infusions over the weekend (totally unexpectedly – and for the first time in our spate of hospital trips since November, we were totally unprepared. We hadn’t taken anything. No clean underwear, no pjs, no phone charger. Nothing. Thank goodness for my wonderful and supportive family who, as ever, pulled together and hot-footed it up after work to deliver a bag of essentials). However, his blood results were actually better than expected, and we were home again by the Saturday night.

Snoozing with Mickey in his hospital bed.
Snoozing with Mickey in his hospital bed, during his albumin infusion.

In spite of the unexpectedly good albumin results, things have become progressively worse for our little man. In addition to the signs we were aware of (the increasing jaundice and the ascites), the blood flows into his liver, in particular, are pretty bad. He has portal hypertension, and his hepatic artery is also pretty hard. We’ve been able to see the veins on his belly for a couple of weeks now (with no alarm bells from the QEQM doctors), and we now know that this is because his blood is having to find an alternative route back to his heart.

Although we were fully expecting to be told Hugo needs a transplant, we weren’t expecting the process to be kick-started quite so quickly. Hugo has a rare blood group (B+), and it can take a long time – up to a year – for a deceased donor liver to become available for this group. The doctors told us that if Hugo had to wait that long, he would become a very, very poorly boy.

Not only that, but we believe from our research and conversations with the experts that the sooner the transplant happens, the better the chance is of the liver ‘taking’ (not being rejected by his body).

So the sooner, the better, really.

Proud daddy. Like father, like son.
Proud daddy. Like father, like son.

Thankfully, King’s aren’t hanging around. We’ve already got the appointment through for Hugo’s assessment, following which he can be listed. Ross and I are also both waiting to be assessed to be a living donor. If one of our livers is compatible with Hugo, they will take half of it and give it to him – we’d much prefer to go down this route if we can. Our liver would grow back to normal size and function in about 6 to 8 weeks. It’s pretty amazing what the body (and modern medicine) can do, and incredibly humbling to know that we can do something to help our son in this way.

We have already had time to get our heads around the fact that this was the likely route for Hugo, and to think in depth about what it all means, the logistics and so on. It goes without saying that we are scared. To know that your baby boy needs another major operation within the first six months of his life is heartbreaking – words just can’t do the feeling justice. But we’ve realised we need to think beyond that – to focus on how much better he will be because of it, rather than let ourselves be consumed by the fear and worry that comes hand in hand with all of this. If it goes well, he won’t need the nine different doses of medication he is on now; yes, he will be on meds for life, but much reduced. So even in that respect alone, his quality of life will be improved. Then on top of that, the side-effects of the disease – the ascites, the jaundice – should all fade away. He will finally be able to roll (he’s been trying to for a couple of weeks now, but his belly just gets in the way). We can start to lead a ‘normal’ life, and really look to the future without this big question mark hanging over us.

Keep rollin'...
Keep rollin’…

It seems alien to say that we were hoping for this to happen; of course, in an ideal world you would never wish for your child to need a transplant. However, when you are watching your baby slowly getting poorlier as the days go on, and you know that a transplant is the only option to make an impact on his health, then you do wish for it. And we’re now ready to face it head on and at full speed. One of the frustrating things about the Biliary Atresia, and the Kasai, is that it is very difficult to ever really know what the future holds – at least we can hope that, once he gets his new liver, the road will be smoother for Hugo.

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.

A brief history…

A little bit about me and my family. My name is Polly. I’m a little bit weird (in a good way), I’m obsessed with food, I love dancing, and I’m known to be a bit of a control freak. I’m married to a wonderful, football-crazy man, Ross, and we have a little boy, Hugo, and a slightly chubby cat, Ronnie. We live in a lovely seaside town in Kent and enjoy quite an easy-going, simple life. On the whole, we count ourselves pretty lucky and we’re very happy.

In general, the first few weeks of Hugo’s life were pretty run-of-the-mill. He drank milk. He cried. He slept. He pooed (a LOT). We were tired, overwhelmed and incredibly in love. We felt very grateful that not only had we created the most gorgeous baby boy we’d ever seen, but also that he was so easy.

Mum and bub time
Enjoying some mumma and bubba time, back when everything was so much easier

When Hugo was about seven weeks old, I noticed a change in his routine. In particular, his nappies changed. His poo went from the bright English mustard yellow we learnt about in antenatal class, to a very pale cream. I did the usual checking – Google, Baby Centre and Mumsnet – but didn’t come across anything that worried me in particular. But as most new mums do, I decided to check with the doctor. I fully expected to be laughed out of the surgery as a neurotic first-time mum; I even made plans to go to a local playgroup with a mummy friend later that morning.

How I wish that is what had happened.

Instead, we were referred from one hospital to another, eventually ending up at (what is now our local) Margate. Bloods were taken, scans were done. Doctors spoke around us, but nothing meant anything to us. We didn’t have the slightest idea about what bombshell was about to hit us.

When the consultant finally came along at 8pm that night, he… well, I can’t really remember what he said. I recall he mentioned the words “specialist liver unit”, “London”, “bile flow”, “liver disease”, “surgery”. Other than that, I only remember a whooshing sound, and the distinct sensation that I was falling. We were told that Hugo would be referred to King’s College Hospital in London, and that it was vital that we had an appointment that week. We didn’t know what would happen beyond that. All we knew is our world, our little bubble with our new, perfect little boy, had suddenly changed beyond all recognition.

What followed was nothing short of a whirlwind – something I can now see as a bit of a blessing, simply because we didn’t have the time to stand still and really think about what was happening and how our lives were changing. We went to London for an assessment and diagnosis; more bloods, more ultrasounds, and a biopsy later, it was confirmed he had Biliary Atresia. We were told to expect a call within a week to book him in for urgent surgery; we didn’t expect it the very next day. We rushed around to get prepared – it was like packing for the worst holiday we could imagine.

One brave boy (and one exhausted hubby) at King's
One brave little boy (and one exhausted hubby) at King’s

Just ten days after I first took Hugo to my GP with pooey nappy in tow, he was taken down to theatre at King’s for major surgery.

The shock really hit me for the first time when we saw Hugo in recovery. Our ten-week-old baby boy was lying in a bed surrounded by wires, machines beeping all around him. We both suddenly felt like we’d run a marathon – it physically hit us like a ton of bricks. But, almost immediately, we both also felt a massive rush of relief – the anticipation of how awful it would be was gone; we’d jumped the first hurdle, and we were on the road to making him better.

Happily, we had Hugo back home for his first Christmas. It wasn’t really what we had pictured, as he was on intensive medication for the first few weeks (nine separate medicines in total, to start with), and because of this he had a suppressed immune system. We had to avoid big crowds and illnesses – not an easy feat during the festive season. However, we maneuvered around the family as best we could while keeping him as wrapped up in cotton wool as we could.

Home for our first Christmas
Home for our first Christmas

For three weeks, Hugo steadily made excellent progress. His poo was always very dark, he was gaining weight well, and his jaundice faded away until even the whites of his eyes had lost their creamy-yellow hue. We had regular contact with the Clinical Nurse Specialist and Dietician at King’s, and they were all happy with our accounts of his progress.

We had been told about several side effects of both his illness and the operation that we needed to look out for. So when Hugo woke up one morning in mid January with a fever and looking yellow-ish again, my stomach sank through the floor. Cholangitis. I just knew it. We had just got to the stage where his immune system was back up and running, and we’d been getting excited about being able to take him out and about, to baby groups and just even to Tesco. Have a bit of normality. But we instead packed up and headed to our local hospital, where he was given seven days of IV antibiotics to try and kick the infection.

During his stay in hospital, his liver symptoms got worse again. His jaundice came back, and his poos lost all colour again. Then, shortly after he was discharged from QEQM, we had his first check up at King’s. It didn’t go well. It was heartbreaking. One of the really frustrating parts of this illness is that there is never a prognosis (we’ve tried REALLY hard to get the doctors to give us one. They simply can’t). The worsening of his symptoms could have been caused by the infection he had picked up, or it could be the first indication that, in spite of the early good signs, his liver was failing and that he could well need a transplant (statistically, only about 25-30% of Kasai operations are successful; the rest result in a transplant). We were basically told that the best news we could hope for, in the immediate future at least, was no bad news – all the while we weren’t being told he would need a transplant, we could be positive. Or, at least, hopeful.

Not one to do things by half (he must take after me…), Hugo has since had a bout of gastroenteritis, a couple of colds, and has increased ascites. We’ve been in and out of hospital every week since he first took a turn for the worse. He’s had several albumin infusions and countless blood tests – to the point where he’s nearly run out of accessible veins. His bilirubin level (the jaundice indicator) is currently higher than it’s ever been. But on the flip side, his poo is regaining its colour. So we are seeing steps forward in some respects, while feeling like we are doing nothing but move backwards in others.

For me and Ross, we’re still learning how to cope with everything that has happened, and might happen. Having to come to turns with the fact that we can’t protect him from this, that we can’t influence what is going to happen, has been really hard. It goes against every basic instinct you have as a parent. So having to totally relinquish control of our lives to his illness is difficult, to say the least. We’re developing coping mechanisms and learning not to read too much into his changing symptoms. I’ve realised that being too hopeful is detrimental to my own ability to cope; being perpetually disappointed by the fact that his jaundice hasn’t disappeared when I wake him every morning will just slowly send me mad. So we’re just trying to be the upbeat and normal parents that Hugo needs us to be – the same ones we were before the bottom fell out of our world. And we’re counting ourselves incredibly lucky that he isn’t suffering directly as a result of the liver disease; he’s not in any pain, and he’s still the smiling, happy, cheeky chappy he’s always been. We know it could be so much worse.

Our playful little man
Our playful little man

That’s enough to keep us going for the moment.