Q&A | Nicki Roest: I almost died from Wilson’s disease

Nicki Roest was diagnosed with Wilson’s disease, a rare and life-threatening liver condition, in 2016 and went on to have the year from hell. Having first experienced symptoms in 2014 while living in New Zealand, where she’s from, doctors concluded Nicki had “a viral thing” or fatty liver. Things seemed to level out, and she and her husband Tim continued with their plans to move to the UK. Two years later, by which point they were living in London, Nicki’s symptoms started presenting again.

After a number of appointments with doctors who seemed largely unconcerned – despite some test results she now knows should have raised red flags – Nicki finally went to A&E. She was immediately admitted to hospital, and things escalated quickly from there. She shares her story with Sarah.

When you went into A&E the doctor went straight into gear, right?
Yeah. He wasn’t letting on too much, but I found out later that he was really concerned. Basically, they checked my bloods and the results had shot up. I’d had tests just before then as well, and the results were completely different. I was just going downhill really fast. He got me admitted, then over the next two days I had back-to-back tests of my brain and my heart and loads of blood samples taken. I had a biopsy, and that’s what came back proving that I had Wilson’s disease – which they can actually treat with medication, if it’s caught early. They put me on pills to try to prolong how it was going, because I was turning yellow. My eyes and skin were all yellow.

It must have been freaky once that stuff started happening.
It was. Tim stopped work and was in hospital with me all the time. My best friend was so upset she stopped working for a few days, and was in hospital with me as well. I asked one of the professors, at what point should I tell my parents that they need to come over? And he said now. He’d warned of it a few days earlier – they’d started mentioning things like a transplant – but I thought, surely not. Surely things will work out. I was on the medication for a few days and I had another test on the brain. An EEG, they put all these things on your head to see what your brain’s doing. Once the liver’s really toxic it can start affecting your brain as well, and you can get things like hand tremors. Every day they’d get me to put my arms out and see if my hands were starting to tremor. These were all signs that I was getting quite bad.

“They’d started mentioning things like a transplant – but I thought, surely not. Surely things will work out.”

I found out I had Wilson’s on a Friday, and I was transplanted on the following Friday. Within that week, I started the medication and then they decided, this isn’t working enough – your brain’s starting to show signs that it’s getting a bit cloudy. My parents got flights pretty quickly, and I think they arrived maybe a day-and-a-half before I went into surgery. They got there just in time. The doctors decided they had to list me on the emergency transplant list and told me that Wilson’s was one of the conditions that you can go straight to the top of the list, because it’s so rare and it’s not self-inflicted. They said, ‘The first appropriate match that comes up you’ll get’.

Why is that? Because it’s really severe?
At that stage it was that my liver was so far gone, but I think also just because it’s really rare. I think if I had alcohol poisoning or something, other people would be ahead of me. They did say no to one liver because it was too big, and that’s when I started getting a bit nervous about whether they would find one in time. They started saying things like, ‘If we have to put you in a coma we can do that and just keep you going until we get a liver’. There’s this path, and then they just tell you at what stage of the path you’re on, but they tell you how far the path can go.

“They [said] no to one liver because it was too big, and that’s when I started getting a bit nervous about whether they would find one in time.”

Once I started learning all this news, I sort of just went numb. I wasn’t able to cry or really feel much emotion. It was like my body took over, because it just had to get itself through somehow. I remember thinking, why am I not sad that this is such a horrible thing? I was sad, I hated it, but in the latter days I just went really numb and I couldn’t feel anything, which was quite bizarre. Even waiting for a liver – I started to become really removed from the fact that it was actually a donor. Someone else had to die in order for me to get that. Which was horrible, but I was so desperate to get better.

I was actually quite ready to have the surgery, I just wanted to get it done. We got the call that they’d found a match at 10am one morning, and then I didn’t actually go into surgery until 2am because there were a few delays, and they had to do all these extra tests on the liver. Obviously they’re very thorough with the whole process, but we just spent all of those hours waiting to find out if we were going into surgery or not.

I don’t even know if I was scared, maybe a bit, but I just so wanted to get it done. And I was so confident that I was just going to go in and out – of course I’d thought ‘I don’t want to die’ and was scared of dying and surgery, but I had gotten past a certain point. I had no idea what to expect, really, on the other side. That was all quite a shock to start to come to terms with.

What happened on the other side?
It went pretty well, but then they kept me sleeping for ages. Then about a day-and-a-half after, I had some bleeding, so I actually went back in for another surgery. The first time they woke me up I was quite peaceful, and then they had to take out the breathing tube and everything, which was all fine. When they tried to wake me up the second time it was really, really bad. I don’t know why, but they wouldn’t take my breathing tube out. I was awake, but I had this massive tube and I felt like I was constantly choking. It was awful, it felt like torture. I couldn’t talk or make noises, and I was really thirsty, and I just remember tapping my hand on the bar of the bed, to try and get the nurses’ attention.

“I had this massive breathing tube and I felt like I was constantly choking. It was awful, it felt like torture.”

I was trying to symbol to them, ‘How long?’ Pointing to my wrist, like ‘How much time until this comes out of my mouth?’ I couldn’t handle it. I was constantly choking. They just kept hitting me with drugs until I passed out again. It was pretty horrific, but I have really vivid memories of that, and that second time waking up in ICU. Then finally – it felt like hours and hours, but it probably wasn’t that long – finally they said it was safe enough to take out my breathing tube, and then I was ok.

Usually you don’t really remember too much from being woken, or it’s quite peaceful. But that one was pretty horrific. I felt quite scarred from that for a while. Then I was in ICU for six-and-a-half days, hallucinating on these painkillers, and I was convinced that someone had a 10-pound baby and they were wandering around the ward with it. The nurse was like, ‘Do you know where you are? There are no babies here’.

I had really weird hallucinations linked with my friends visiting me, and they were getting stuck in these strange places. They were really crazy dreams, but they felt really real. And I had envisioned that all the doctors and nurses had gone down into this other room for a Halal party. One of the doctors had called out to order the special chicken. The chicken hadn’t arrived or something, and I was like – the doctor, he can’t get his chicken for the Halal party. Tim told me, ‘There’s no party. The doctors and nurses are not off partying’.

How did the donor process actually work?
I think there are a couple of types of lists – there’s a normal donor list that people are on, when they’re not too critical. People can wait years for a donor, but I had acute liver failure. If you don’t get a liver soon you’re going to die kind of thing. It’s quite a huge process, and sometimes you might think one’s right for you and then they tell you, ‘Oh no it’s not actually, sorry’. I remember the surgeon coming in and putting his hand on my abdomen to try and measure the size. I think I was on the list for maybe four days. Pretty fast.

You were released and then had to go back in, right?
Yeah. I’ve been in and out so much. I think I was out for a week or so, and then I had to go back in because I had a virus that came with the donor liver. It’s like the flu, but 10 times worse. I had been doing alright and then I started going down again. I fought that off, but I went back into hospital shortly after, because I started getting all this chest pain, and not being able to breathe properly. Whenever I lay down I’d be alright in a certain position, and then when I got up I’d be struggling to breathe. We went to A&E, and it turns out that my right lung completely – almost to the top of my chest – had fluid on it.

Royal Free Hospital, Nicki’s unofficial second home.

I had to have two chest drains put in – they put them between your ribs to try and drain the fluid. Then one of the junior doctors came in, and they’d discovered that I also had an infection in my liver. She came to talk to me and said, ‘You need to mentally prepare yourself to be here for quite a long time’. Basically, the bile ducts had started leaking into my liver, and it was creating these collections of bile, which then became infected. I was on IV antibiotics three or four times a day for a couple of months. I was trying to gain weight and move about a bit and trying to recover, but if anything I was getting more and more drained. They said ‘We’ll probably have to operate on you in the new year’.

Then, when I was looking forward to going home, my surgeon had one of his associates come and talk to me, and say ‘We’re probably going to do this hepatectomy on you tomorrow’, which is another big surgery, cutting me open again to take out half my liver.

And this was the new liver?
This was the new one, and it’s really rare to have to do that. My surgeon said it’s almost unheard of to have to do a hepatectomy on a transplanted liver. So his associate’s sitting there telling me, ‘We can do this surgery tomorrow’ and I’m like, ‘No thank you, I don’t want it tomorrow’. I said, ‘I think it’s going to be sometime next year, if it even happens. I’m going to get better’. Then my surgeon had to come and talk to me, and he was really serious, and that’s when I started realising how serious the situation was. He said, ’What don’t you understand? This infection is killing you. You’re actually dying’. Then I just broke down, because obviously that’s really hard to hear. I knew it was bad but I didn’t realise how serious the infection was.

“My surgeon said it’s almost unheard of to have to do a hepatectomy on a transplanted liver.”

He told me they had been going to do a surgery in a month, but that I wouldn’t be strong enough in a month. He then convinced me to do the hepatectomy, and then if that failed they would put me on the transplant list again, and I would have a whole ‘nother transplant.

It’s too much!
I don’t know how I even dealt with it all, really. For a few days I was really, really upset and really down. I felt like I was in this black hole and I couldn’t get out. Then the idea of going through the whole surgery and ICU again, and recovering – you come out of surgery and you’ve been in bed for days and you have to learn how to walk again. You have to get rid of this fluid, because your legs and everything just get massive, and really heavy.

I was thinking about having to do all of that again, and then I convinced myself that it was only a few weeks, and you recover quite a lot within a few weeks. So I was like, ok I can do it. We had to have a few meetings, and I had to also discuss the possibility of a family donor, in case they didn’t have the option of getting a different donor and I needed part of someone’s liver. Luckily it didn’t get to that point, but we had all these really intense meetings with surgeons and stuff, which was quite scary.

Would that have involved one of your parents, say, giving you part of their liver?
Yeah, Tim even said, ‘You can test me’. But I was saying, ‘No, I need you to be strong for me – I can’t let you be going through the same thing as well’. Luckily it didn’t get to that point, the hepatectomy worked a treat. My surgeon was apparently delighted with how it went, but again the nurses and doctors only tell you what you really need to know. I found out a lot later that they were really concerned. I guess they have to keep you as strong and positive as possible, so you only know what you really need to know.

I think that second time I was only in ICU for two-and-a-half days, if that. They actually moved me to the ward quite fast, and I didn’t like it because I felt like I still needed so much attention and help, and I couldn’t move, and I had all these drains coming out of my abdomen, and cables everywhere. It’s quite scary then going onto the ward and you’ve just got your little button, to call for help if you need it.

Did you find that the hospital staff were good though? Were they quite responsive?
The hospital staff have been incredible the whole time. So many people complain about the NHS, but I felt like I had so much care and everyone worked so hard. It actually makes me really sad that they have to work so hard for how they get treated. Especially the nursing staff – they’re often short-staffed and they just have to get by. They might have screaming patients, ones that are trying to escape. They also end up having some patients that they’re probably not meant to, but it’s that whole thing where there aren’t enough care homes, so they end up having some elderly people that shouldn’t really be in hospital, but they’re in hospital because they can’t really go anywhere else.

“So many people complain about the NHS, but I felt like I had so much care and everyone worked so hard.”

And the doctors are happy with your progress?
All my blood tests are really good. I’ve had several endoscopies, which is the one where they put the camera down your throat, through your tummy. I’ve got these stents in my bile ducts, to my liver. Because they were getting a bit blocked, and that helps with the flow. But eventually they should take it out and stay out, so then I won’t have to keep having those procedures.

So from now on it’s maintenance? 
Yeah, I just have to go to clinic and stuff for check-ups. I’ve had lots of complications, but I’ve met lots of people who have also had transplants, and everyone’s had them for different reasons, and some people are in and out within a month, and are fine. Others have more complications. You have an 80% chance of being readmitted in your first year. I remember reading that and thinking, ‘Oh, ok that’s quite a high percentage but it might not happen for quite a long time’, and then I was already getting readmitted within my first two weeks. It’s all quite surreal now, to think back on.

Once you recover, you’re meant to just go back to living a normal life as much as possible. You just have your scar. Apart from that you should be able to live pretty normally, and you can get back into stuff like exercising further down the track.

It’s so, so good you’re on the other side of it.
I know. Once I learnt that I was going to be in hospital for a really long time, that was all quite hard to deal with. But then I just reached a point where I was like, ok I’m in hospital for however long, and just had to get over it. Now I’m on the other side it’s so amazing. It fees quite surreal that I was even stuck there for so long. I feel a lot more myself. I’m very happy to be where I am now.

In terms of the donor, I haven’t found out about mine. I want to. I think I want to. It’s just taken me a while to come around to the idea. But you can find out their age, their sex and how they died. We will write to the family as well, because you can do that through the hospital, but it’s taken a while to process everything. So we haven’t done that yet.

I think that’s fair enough, taking things one step at a time –
Yeah definitely. But I think it will be nice, because obviously around that same time they were having a really hard time as well, and it’s really a pretty amazing gift to receive. I’m actually really glad that I was here in London, because I think if I had been in New Zealand the outcome could have been quite different – just because of the population, and the fact that there’s not as many donors there.

How does it feel to be back at work finally after having been unable to work for so long?
I used to dream of going into the office, thoughts of returning to normal life and seeing my friends kept me going. It was about nine months before I wen’t back to work. When I first started going into the office – even just one day a week – it was exhausting and quite overwhelming. I built up slowly and I’ve been working every day for the last two weeks. I’ve noticed a huge improvement over that time. It feels so good to return to some normality, seeing familiar faces and catching up with people – they’ve been awesome. It has taken quite a while to regain confidence in myself and my surroundings but I’m getting there.

Nicki Roest is a London-based graphic designer from Auckland, New Zealand. Find her at www.nicolaroest.com and LinkedIn.

Sarah Illingworth is a freelance journalist and Editor at Impolitikal. She has an MSc in Poverty & Development from the University of Manchester. Read more by Sarah.