Steve

Age at interview: 60

Age at diagnosis: 59

Brief Outline:

Steve has microscopic polyangiitis (MPA), an ANCA associated vasculitis. This has caused end stage kidney failure and lung scarring. Symptoms first appeared when he was 39 but were not diagnosed until his health suddenly deteriorated 18 months ago.

Background:

Steve retired from the advertising business at 55 and is undertaking a PhD. Having cared for his wife when she had cancer, he is widowed and lives alone. Ethnic background: White British.

More about me...

Steve was admitted to intensive care following a relapse of vasculitis around the time of the COVID-19 national lockdown in 2020. At his interview, three weeks after returning home, he is “just about managing.”

Steve first developed breathlessness and skin rashes in 1999, which were thought to be due to another condition. Because they went away, he forgot about it. Other than high blood pressure, Steve felt healthy until rashes and exhaustion returned over the course of a few weeks in 2018. He deteriorated quickly and, fighting for breath, was rushed to intensive care by ambulance. Steve was diagnosed with MPO-ANCA associated vasculitis (also known as microscopic polyangiitis (MPA)), causing kidney failure and lung scarring, and he began a period of dialysis along with aggressive treatment to suppress his immune system.

Steve is under the care of kidney (renal) services with support from lung (respiratory) services and has received regular blood tests and reviews. However, his vasculitis flared again recently. Steve is surprised that he didn’t recognise he was relapsing until it became an emergency; he puts this down to not thinking clearly due to a lack of oxygen. He now has kidney dialysis several times a week, may need a lung transplant, and describes a return of side effects of high dose steroids, in particular hand cramping.

Steve is open about his condition but frustrated by the constant need to explain it, and to justify the limits it places on what he can do and eat. He wishes he could direct people to a website with answers to their questions. In healthcare, he would have welcomed better information, such as a ‘Hello newbie’ pack, and dieticians emphasising what he can (rather than can’t) eat and drink. Steve also wishes psychological support was offered routinely. He particularly values the “little human touches” by hospital staff.

Living with vasculitis has made Steve more serious and focused in how he uses his limited energy. His PhD research is his main project and “legacy.” He is looking forward to a post-coronavirus world when he can spend time safely with friends, having dinner and “not talking about the bloody disease.”

Thinking about vasculitis as an invader or unwelcome visitor living inside him helps Steve “to be me.”

I think you referred to… when you’re talking about vasculitis you are saying “It,” and “Thing” …

Yeah.

… and you mentioned at one point like an animal.

Yeah.

It’s almost like it’s not part of you?

Yeah, that’s a good point. I suppose I do think of it as a kind of alien in me, or an animal in me, an invader. Yeah, I do think of it as an, I know it hasn’t come from outside the body because it’s autoimmune. So, by definition, it’s come from inside. But a bit like cancer I suppose, you know, cancer’s in the same category, it comes from inside the body, in a sense you could argue it’s quite a natural part of the way the body behaves you know, just cancerous cells dividing too quickly and out of control, autoimmune disorders are the autoimmune system going into overdrive and in a kind of a way doing its job too well.

So, you know, I know it is from the body itself that it comes, but you still think of it as a kind of unwelcome visitor that’s living inside you. I do feel distanced from it, I do feel it’s something else. I find that’s important because I don’t want to be def-, I know everybody says this, but I don’t want to be defined by it, I don’t want to be, “Oh, the guy with vasculitis, kidney failure and scarring in the lungs,” I want to be me, who happens to be living with this awful chronic illness.

Steve is frustrated that people expect him to know what causes vasculitis when “medical science doesn’t.”

And I have to, I find, it’s worth saying I actually find myself putting quite a lot of time and energy into explaining that to people. You know, I have to explain quite a lot, that dealing with the kidneys is not the same thing as dealing with the vasculitis because so few people have heard of vasculitis, so few people are familiar with what an autoimmune disorder is. They ask all sorts of questions, well-intentioned, but they ask all sorts of questions about what causes it, you know, what have you done to deserve this, blah, blah, blah, and you think I’ve got, you, the questions just numb your brain because you’ve got no idea how to respond to them. What causes it? Well medical science doesn’t know what causes it, you’re asking me? Good grief you know, and you get very frustrated because you just want to kind of shout at people sometimes, “Just stop asking all these questions. “No, it’s not down to an infection.” No, it’s not, “I haven’t been drinking bleach,” no, I haven’t you know, oh, whatever it is, “I haven’t been taking crazy drugs” or anything. “No, it’s just a thing that happens to people.”

You know, when I was in hospital, in the next room to me was a teenager with the same condition, and on the other side was an 80-year-old lady with the same condition, and at that point I was in my late 50s, so there you go, it happens to a range of people and there’s very little you can do about it. You’ve either got it or you haven’t and it’s either going to flare up and attack you or it isn’t. And that’s it.

Steve was rushed to hospital “fighting for breath.” His vasculitis was diagnosed in intensive care.

But eventually I woke up, in the early hours of one morning in December 2018 and I was really fighting for breath. I live on my own, so it was quite panicky, and I called the GP emergency line, they came to see me, and they immediately called an ambulance, and I was blue lighted into the local hospital, and then from there, the acute assessment unit there diagnosed kidney failure.

And the main diagnostic point that they spotted was that they could smell a kind of urine smell on my breath, which is a sure sign of kidney failure caused by the vasculitis as I was about to find out.

I was then transferred by ambulance to the regional hospital, in [large city], which is the specialist renal unit, or kidney unit, for the region. And over the course of that 48 hours, I went straight into intensive care because my lungs were in fact quite waterlogged, they thought they might have to put me on a ventilator. In the event, they didn’t, which I was very thankful for, I just needed high, high flow oxygen.

I received blood transfusions and a treatment called plasmapheresis, which is where the plasma in the blood is replaced, and that’s to get down the level of vasculitis antibodies in the blood, so that it’s not active, and isn’t attacking other organs.

So, over the course of my stay in intensive care and then on the kidney ward, which was a total of about five weeks altogether, I was diagnosed as having ANCA vasculitis with MPO antibodies, which is also known as microscopic polyangiitis as I’ve come to learn, and that had by the sta-, by the time I got to hospital, that had attacked, both kidneys? Pretty much entirely destroyed one of them and left the other with very little function. So, in kidney terms my, my estimated GFR at that point, which is a measure of kidney function, was nine, and it’s never gone above that since. And it had also caused some scarring, or fibrosis in the lungs, particularly the right lung.

Vasculitis damaged Steve’s kidneys. He says dialysis isn’t painful, but he’s “just exhausted” afterwards.

So, I've just, very recently, come out of another three weeks in hospital, and I don’t think you could honestly say I was recovering yet, I think I’m coping, just about managing. I’ve still got a degree of fatigue, although it’s not as bad as it was recently, and as you can probably tell I’ve still got a degree of breathlessness, although that again isn’t as bad as it’s been.

So I think I’m, the way I describe it to people is I’m, I’m making progress but it’s a long slow road, and, I constantly have to reinforce to people - I know they’re all enquiring and asking me with the best of intentions, and I really appreciate that - but, one often has, you know, I often have to stop people and actually say, being home again does not mean being well, it does not mean being recovered, it’s just another step on the journey, and they’re not, they’re not at all the same thing.

So, I’m now on, also on regular dialysis, so I have to go to my local hospital, three times a week for the afternoon session of dialysis, and that’s, that’s four hours a time. Being on dialysis is in itself something to, that one has to learn to live with of course, and, and get used to. It’s not a painful process, there’s no, actually being dialysed is absolutely fine, but afterwards, certainly for the evening afterwards you can feel it, just exhausted, and it’s a real trial getting home. The taxi, the taxi, there’s a taxi service provided by the hospital, which is wonderful, but even so, you know, getting home, walking up the few steps to the front door, and getting into the flat and freshening up and having some dinner and everything is just a huge trial after that.

Although the symptoms were similar, Steve was too ill to recognise his vasculitis was flaring badly.

What I have been told, and I’ve also been told this is quite common, is that the reason I didn’t join the dots, and you know, recognise the symptoms as one, one complete set of serious symptoms, the reason I didn’t do that was probably because I was in fact very short of oxygen and my mental functioning was impaired, and I just wasn’t thinking straight.

And that’s what, what I mean when I say I really let myself go you know, my, my personal hygiene went down the drain, and that is just so unlike me. I’m usually extremely fussy about that. Well, you know, people think I’m really obsessed about it, and it just went, and in fact it took a colleague at work to like sit me down and say to me, “There’s something seriously wrong with you and you need help”, and that was in fact the day before I woke up in the early hours and could barely breathe. So, it just came on very, very quickly.

I think I just wasn’t thinking straight. I look back at work I did in that period and it was, it’s awful, I don’t know what I was thinking. I’ve had to re-do it all. So, you know, I think the balance of my mind was affected by, frankly, just by a lack of oxygen in my blood.

With vasculitis always on his mind, Steve loves time with people “not talking about the bloody disease.”

One of the, you know, one of the most valuable things, and it kind of… I’ve been moved to tears at times by this, one of the most valuable things is just spending time with people and not talking about the bloody disease. Just not talking about it. Not thinking about it. Just a few hours of relief because, when you’re on your own, you know, you, you’re constantly thinking about it, it’s the major fact of your life. If you’re not doing that you’re thinking about your next prescription renewal, and how you’re going to order it and how you’re going to get it collected you know, or you’re thinking about this little extra symptom that’s suddenly popped up, or you’re looking at your results online and thinking, “Oh, that’s gone up and that’s gone down and what does that mean and should I be calling the doctor about that?” and, you know, or, it’s constantly there all the time.

So, it’s, you know, pre-coronavirus, pre-lockdown, it was wonderful just to be able to go round to someone’s house for an evening and just have a nice evening of not talking about it.

As “chronic illness is all around us,” Steve believes everyone benefits if he is open about vasculitis.

I decided right from day one that I was going to be completely open and honest about it. I know that a lot of people don’t like talking about their illnesses and I’ve actually had colleagues at work - you know, and I decided, I asked, as soon as I became ill the first time around, I actually asked a colleague at work to send round an email to everybody telling them exactly what was going on. And, and the reason for that is that I think everybody benefits by it. I think it’s better not to have secrets about your health because I think it leads to greater understanding of what it is to be ill and to have a chronic illness.

And, and the funny thing is that since then, several people in my personal life and in my professional life, several people have actually come forward to me and they’ve said, “Actually Steve, the thing is, I’ve got a chronic illness too, I’ve just never told anybody.” So, and it’s amazing you know, it’s chronic illness is all around us, all the time and I believe we’d all benefit from being more open and honest about it, and I think it would manage our expectations of each other, much, much better than we generally manage to do.

Living with vasculitis has made Steve think about his “legacy in the world.”

And you know, sometimes lie awake at night, getting very anxious about it. And what I mean by that is you get worried about how much life you’ve got left, quantity, and you also get rather worried about quality you know, what quality is that life going to be, or you get you know, you even have sort of nightmare visions of ending up in a wheelchair, you do, you inevitably think these things.

And I’ve found it very helpful to talk to my doctors and the nurses about that, and that’s been, that’s been very reassuring. You know, they don’t, they don’t gloss it over, they’re, they’re honest, which I appreciate you know, but at the same time a lot depends on factors that are specific to you, and it’s important to understand that and keep it, keep a grasp on it.

I would also say that, on the upside [laughs], which sounds a strange thing to say, but on the upside there is a benefit, and the benefit, it’s kind of something that you hear a lot, but I think you hear it a lot because it’s true, and that is, it does focus the mind. It does make you think, “Well you know, if I’ve only got a few years left, well I’d better use them well.” It does make, it does kind of wake you up in a sense, and make you feel that, I know this sounds a bit grand, sorry about that, but… other patients have said the same to me, you kind of feel what’s my legacy in the world, what am I going to leave behind, and you want to leave some kind of, yeah, achievement behind you.

You know some people, for a lot of people that’ll be having children and bringing them up, and that’s a wonderful thing. I haven’t done that, I don’t have kids, so you know, for me it’s, it’s doing my research and making a contribution to that field. So that’s become you know, very, very important to me.

Vasculitis damaged Steve’s kidneys. Rather than “rules” about what he can’t eat or drink, he would have liked this information presented more accurately as a “series of possibilities.”

As I say, what I would, I think what I would really have appreciated was an info-, a printed information pack that basically said, “Hello newbie.” You know, “this is a whole new experience in your life, welcome, welcome to it, here’s how to get going as a kidney, as a vasculitis patient, and as a kidney patient,” you know. “Try, try this kind of diet, try eating these dishes, approach it like this” you know, and so on.

A great example of that is coffee. So, you know, coffee is a high potassium food, if you’ve got kidney problems resulting from vasculitis, one of the first things the dietitians say to you is, “No coffee,” and if you’ve been drinking coffee your whole life that’s a pain you know, your heart sinks at that. Actually, when you research it yourself, what you find out is that roasted ground coffee, like an espresso or something, is actually quite low in potassium, it’s instant coffee that’s very high. So, and if you know, like roasted ground coffee just comes in a small Nespresso size cups, like a large espresso cup. And actually, a cup of that in the morning is not going to do you any harm at all, it’s actually quite, quite low in potassium so you’re perfect, you’re perfectly okay with that.

I kind of wish I’d been given that advice rather than just “No coffee, coffee’s bad, coffee’s potassium, you can’t have it”, you know, so that makes a big difference.

I mentioned oranges you know, I was told “No citrus from now on, absolutely no citrus.” Well, I’ve researched it since and found out that there are certain forms of citrus like Satsumas and mandarins that actually are quite low in potassium. It’s oranges that, that are very, very high. I wish I knew that from the start because it does mean that every few days I can have a Satsuma, or a mandarin, and actually, the difference between a life where you never eat citrus again, and a life where you can eat something citrusy every few days, is incredible. That’s an enormous difference, emotionally that’s an enormous difference.

So that’s what I mean when I say I wish it had been presented as, you know, a series of possibilities rather than a series of rules against, which is what it was.

Vasculitis damaged Steve’s kidneys. “Little human touches” make a big difference to his healthcare.

Yeah. So little things that make a huge difference? One of the consultants, so I was lying in the bed in the hospital, this is on my most recent hospital episode and, she, this was the one who took her mask off and, she just spoke very honestly, smiled, made a connection, and on the way out she just put her hand on my knee, through the bed clothes, and just gave it a little shake. And [sighs] that, [emotional] oh god, just, just that made such a difference, just that. Of course, she washed her hands immediately afterwards and all the rest of it, which was the right thing to do but, you know, just that little bit of human contact made such an enormous difference.

Earlier before coronavirus one of the, another one of the consultants he told me about a research study that he was engaged in, and he sent me a copy of it and asked for my opinion on something and you know, I gave him some ideas and some views and he really appreciated those and fed them back to his research team, and they were about how to conduct the next stage of the project.

And you know, that made a big difference because it showed that he was prepared to, you know, engage with me on a very adult to adult basis and not a kind of, that kind of old-fashioned doctor/patient relationship, so that made a big difference.

I think when it comes to that it’s the little things, again it’s the cliché, you hear it all the time, but you know, it’s the little things that mean a lot. I think you hear that because it’s true, you know. Those are the two I can think of. I’m sure there have been others along the way.

Oh, yeah, I mean now I’m on dialysis you know, one of the nurses who you know, just occasionally, you’re lying there for four hours and the machine is just whirring away and your blood’s going up and down the tubes, blah, blah, and you know, she just like, from the nurses’ station she’d just look over and give me the thumbs up you know, and I’m give her the thumbs up back just meaning, “Yeah, everything’s okay, nothing to worry about.” You know, just that. Just little things like that.

The fact that the lady who brings the tea trolley round you know, will give you a cup of milk rather than tea because you don’t like, I don’t like tea, and then she’ll come back a moment later with some biscuits you know, just that makes a big difference. And the fact she says, “You’re very welcome,” and calls you by your name because she’s bothered to look at your name, you know, that’s all of that makes a tremendous difference. I think it’s the little human touches that really matter in the course of the treatment, and as you, as you relate to your healthcare professionals, to the team.