Jane X

Age at interview: 55

Age at diagnosis: 51

Brief Outline:

Jane has two rare autoimmune diseases. Primary biliary cholangitis, affecting her liver, developed in 2005. Then, after several years of additional symptoms, MPO positive AAV was diagnosed in 2017. Jane has learnt to enjoy life at a slower pace.

Background:

Ill health meant Jane retired four years ago from her career as a pharmacist then spent two years as a shop assistant before giving up work completely. Jane is married with three grown-up children. Ethnic background: White British.

More about me...

Jane says that, by the time vasculitis was diagnosed, she had made so many adjustments to her life she had forgotten how it felt to be well. Before giving up her career as a pharmacist, for example, she had hired a cleaner and a gardener and was buying ready meals. Her symptoms also came and went in unpredictable ways and had increased gradually. As a result, she suspects that at medical appointments she underplayed how bad things were.

As Jane grew up seeing the effect of rheumatoid arthritis on her mother, she half expected she might develop that condition herself. Instead, in 2005, a rheumatologist identified that fatigue, bone pains and achiness were a result of primary biliary cholangitis, a rare autoimmune disease of the liver. Once diagnosed, this was managed well with standard treatment that didn’t involve suppressing the immune system.

In 2012, Jane’s liver doctor suspected that sores on her fingers were a sign of vasculitis, so sent her back to rheumatology. Unfortunately, Jane recalls the sores being described as a trivial matter that didn’t require additional treatment. She says this was followed by several years of fatigue, joint pain, sores on her fingers, and red eye, during which her liver condition became less well controlled. In 2013 Jane had the first of what she calls “an attack,” where the pain and stiffness escalated over a few hours to the extent she couldn’t move without help.

Throughout, Jane’s GP listened, knew that something was wrong, and arranged investigations. But the way the symptoms came and went, usually in the middle of the night, made it a challenge. Jane “turned into a bit of detective” by writing down what was happening and taking photographs of symptoms that could be seen. The vasculitis diagnosis was pursued following a rheumatology appointment where she had a very visible sore; she therefore wonders if videos might have been better at helping doctors understand earlier what was going on.

After being diagnosed with MPO positive ANCA associated vasculitis, Jane was prescribed immunosuppressive treatment - steroid injections and azathioprine - which she found “transformative.” Although she has to pace herself, she has more energy and can get much more done. Before treatment, she says she would start a family roast dinner and they would end up with meat sandwiches; now she enjoys cooking, gardening, knitting and walking, and is learning Welsh.

In addition, as Jane had been ill for a long time, she worked with a qualified rehabilitation exercise instructor to get her strength back. She progressed from an older person’s chair exercise class, to over-50s circuits, to aerobics, which is continuing online during the COVID-19 pandemic.

Although Jane had empathy for people who are chronically ill through seeing the impact on her mother, she says she hadn’t realised the loneliness and isolation that it can bring, even when you have family around. Both before and after diagnosis she has found patient groups a source of hope, and appreciates the support and time they offer.

Jane X found it peculiar when her symptoms would flare up very suddenly then disappear. She now knows this was vasculitis.

I had my first of what I would call an attack. So, my symptoms would flare up very suddenly from a baseline of being somewhat in pain and uncomfortable, over a matter of hours, always in the evening, to go from like I’m all right, sat here like this, to not being able to move, to my husband and my son having to carry me upstairs because I just couldn’t move and being in absolute agony.

And then it would go off over the next 48 hours or so, and then I’d be back to where I was again. And initially that happened fairly wide-spaced apart, so there was one attack in October, there was one I remember very particularly in the December of that year because it was the day before my birthday [laughs]. And I sat there, I had a friend for tea, and I thought, “I’m not going to be able to get up and let her out the door,” because over the evening, while I’d been sat with her, it had just kicked off again.

And then I thought, “Right, I’m going to go back to rheumatology.” [Laughs]. I had a further attack in the Easter that was very bad, and it was starting to be more frequent. So, I got, went in touch with my GP who was very good. She knew me very well and she always listened. Because she’d known me for years, she knew that I didn’t go in just for nothing and she could see by then that there was something wrong. So, she sent me back.

So, I didn’t get to see them for six months or so? It wasn’t obviously – because they’d seen me twice already, I think, [laughs] and it wasn’t considered an urgent issue. And then I saw a very nice rheumatologist and she took all the details and what I was saying about how I felt. And, by this stage, I’d turned into a bit of a detective, and I was writing down all my symptoms and taking photographs of things, so I would get rashes, whole fingers would just swell up out of nowhere, one swollen finger. Or a bright red eye. And I must admit I was quite glad of those things because it was something I could see.

Because when something comes and goes like that and you’re sort of completely incapacitated and then the next day you’re just in pain but you’re all right, it makes you feel like you’re going a bit mad.

After doctors realised how ill Jane X was, they “really did work hard” to get to the answer - vasculitis.

But I can’t fault them at the end, when it was bad, the efforts they made to find out what it was, in the last sort of few months before I got diagnosed, once this person had seen the sores on my hand, they referred me to dermatology to look at the rashes, they referred me to ear, nose and throat to biopsy my nose, they referred me to ophthalmology to look at my red eye, I had a CT scan. And then I had to go to the lymphoma doctors because I had enlarged lymph nodes, so they had to rule out lymphoma. So, you can see by the time I actually got diagnosed, I was quite ill. Fortunately, I didn’t have lymphoma, they thought it was all connected to the problem. So, they really did, once they got going, they really did work hard to get an answer.

Jane X says a bad day with vasculitis means being prepared to cancel plans. This can be hard on family.

It’s no good making cast-iron plans, really, you’ve got to be able to, prepared to cancel at the last minute [laughs] if, you know, if you’ve planned to go, I don’t know, out for a meal, if I’ve planned to go out for a meal and it’s a bad day and I don’t think I’m going to enjoy it, then I’ll cancel it and go another day.

And I think that’s where it’s hard on your family. Because they probably feel fine, [laughs] they were probably looking forward to going. And sometimes they’ll go on their own to a thing if I can’t go, which I don’t mind. It’s better that than struggling to it.

You know, during the time I was pretty unwell before I was diagnosed, one of my daughters got married and I was so worried about what I would be like if I’d had a bad day, [laughs] but I was lucky, I had a good day. But that can be frustrating because there must be people who have a bad day when it was a really important day to them, and that’s never happened to me, but it must be hard when it does.

Jane X doesn’t think the benefits system is set up to understand illnesses like vasculitis.

And I think there’s more important things in life than money, I suppose, provided you’ve got enough, and you don’t need more than that. But I was quite lucky, I suppose, when it happened. It must be quite a blow if you – because a lot of patients with vasculitis are younger, much younger than me, and it must, if you get that sort of felling you down in the middle or the beginning of your career and you lose all your career, that must be, that must be terrible.

Because they, you know, are probably not in a position where they can say, “Right, enough’s enough, I’ve had enough of this.” And it’s going to condemn them to probably living in poverty, most likely. And I think, I think that’s a shame. Because I don’t think that – it’s political, really, but I don’t think that the benefits system understands these sorts of illnesses. I’ve never tried to apply for benefits because I read the questionnaires and think, “Well, I can make my dinner now, and I can do this and I can do that,” but. Am I ill? Has it affected my life? Most definitely. Fortunately, I made enough pension contributions and I’ve got enough years National Insurance that I will get a pension, but if you’re 20-odd then it’s, it must be terrible. And I suppose it’s that very big decision at that sort of age, just what do you do?

So, I think they should go by: how serious is the illness? What does the doctor say about the illness? Not, “Can you cook your dinner?” Because in fact, you might think you can. I look back on the couple of years when I was really ill and realise that actually, probably, I was ill enough at that point to have, but I didn’t, I didn’t do it. I didn’t. Because you don’t really, it’s only when I look back, I think, “Well, no, I couldn’t cook a dinner, really, because I’d start a dinner and then I’d not finish it.” Or and sometimes I couldn’t have walked 200 metres, now, or I could only have done it in pain. But because it’s a fluctuating condition and you’re not always the same, it’s not quite the same. It’s not, you can’t definitively say, “I can’t do this, I can’t do that,” because some days you might be able to. Now I’m lucky that that doesn’t, doesn’t matter to me, but I think it’s very unfair for younger people.

Jane X was a pharmacist. Although she kept working full-time for a while with vasculitis, “the cost to me was too high.” She misses “the intellectual side” but gets round it by reading.

Oh, yeah. It was hard, it was. It was [sighs]. At the time, I was feeling [sighs] pretty ill and I wasn’t enjoying the job, so it was easy to walk away from it because I wasn’t enjoying it either. I don’t know how much of that was because I wasn’t really that well or how much of it was just because I just didn’t enjoy that job, and I think a lot of people around that sort of age do make a change because the job that was ideal for you at 25 is not necessarily the job that’s ideal for you at 50.

And I just wanted to do something that was easier as well, but whether that was borne of everything else being such a struggle? Maybe it was. I was still able to do the job but the cost to me was too high. I wasn’t able to do anything else; it was the job and that was it. By that stage, you know, to do the job, to keep doing it full-time, I had a gardener to do my garden, a cleaner to do my cleaning, I was buying ready meals all the time. And not enjoying it and not enjoying my time off because I was so exhausted from having been there. I just thought this is, it’s like being on a treadmill, I don’t want to do this anymore.

And so, I went to work in just a food shop, because I thought, “Well I’ll view that as being paid to exercise.” I get the exercise working in the food shop, I won’t get any of the stress. And I quite enjoyed that. I do think, though, if I hadn’t ever been ill, I’d probably still be working. As a pharmacist. In fact, I’m pretty sure I would be. Because not many people retire at my age from that profession.

So, but it doesn’t upset me really, because I just don’t think there was any other, there was no, it was the best decision for me. I made it, nobody, nobody said to me, “Perhaps you’d be better,” you know, because I was still able to do it perfectly well. I knew that I was going to be better - and I’d always said that I wasn’t going to work until I was very old, I was going to retire and I was going to enjoy it – this was before I was even ill – because my father died at 48 and my mother at 68, so I didn’t see a great deal of point in [laughs] working until I was 67 to have no retirement at all. And having had that experience, I was always very determined that I wouldn’t, wouldn’t work. And I never would probably would have worked beyond 60 anyway.

And I wouldn’t say I miss it. I miss the intellectual side of it more, but I get around that by just reading.

Jane X would rather not have vasculitis but is “really pleased with how much better I am.”

You never know how you’re going to be. This morning I got up and my ankle was swollen up for no reason at all, just was. Now it’s gone down, but this morning I had my walking stick around the kitchen. And now I don’t need it. But that’s frustrating still.

I don’t expect, I don’t expect a miracle. There’s a limit to what doctors can do and I’m really pleased with how much better I am. Really pleased. But of course, I’d rather be well, I’d rather not have it. Anybody would rather not have to live with it. But I think, well, I’ll do the best I can with it. And I’m happy now, I’m happy with the sort of level of, you know, I wouldn’t, I wouldn’t say, “Oh, I’m not well enough, I need more treatment,” I’m happy with how I am,” you know? When I go and see the doctor now, I tell him, you know, I’m a hundred per cent better than what I was and I think this is good, you know, it’s a good pitch to be at because it’s, it’s not really possible to turn your immunity off so much as you don’t have any symptoms, I don’t think.

I’ve never heard of anybody say, “Oh, right, it’s gone away, I just don’t have it anymore,” you will still have days when it’s not so good? And there is no pattern to it or rhyme or reason to it, or – well, not that we know what it is. It just is. Some days are worse than others. You’ll have spells, I might have a week or so when it’s worse than normal, but it never goes back. And I don’t think it ever will, really. I hope not, anyway. I don’t think I’ll ever be like I was before I was on the treatment. And if I got like that again, I suppose they’d try something else.

Yeah, because there are other things.

Yes. Yeah, there are, yeah. I’ve been lucky, I’ve not really needed the really big guns things that they need. As I say, it’s not gone for my kidneys or anything like that, so, you know, people who have that sort of complication often have to have much stronger drugs, at least at the beginning. So, I’ve been lucky that way. I was lucky that they’d found it before it really, I imagine had it been left, it would have done something. So, I was lucky it didn’t.

Jane X moved house but travels back to her rheumatologist for vasculitis care as, “once you’ve found one you trust, it just makes so much difference to your life.”

And it’s been good for me, I’m glad we moved.

I was worried about my doctors, [laughs] but I was able to keep the rheumatologist, so that’s been, I wouldn’t have wanted to, I couldn’t, I said to my husband when we moved, “If I can’t keep this rheumatologist, I’ll have to find one, even if I have to go privately. I can’t go back to square one, I can’t start again with one that doesn’t listen.” [Laughs]. I wasn’t bothered about the liver doctor, I thought “Well that’s fairly clear-cut, they know what that is, that’s straightforward.” But rheumatologists, I think it’s so much more complicated. And you get differences of opinions between them. So.

But I was able to keep the one I have. So now I travel, and I stay overnight in a hotel. Takes me four hours to get there, to stay overnight in a hotel, four hours to come back. And I’d travel further to keep this particular rheumatologist [laughs] because once you’ve found one you trust, it just makes so much difference to your life. Once you’ve found the right one.

Although vasculitis needs strong drugs, Jane X says don’t be frightened, as they can be “transformative.”

And don’t be frightened of the drugs, the drugs that they give you are strong drugs and obviously not everybody will be suited by them, but if they were, they’re transformative.

I had, like, feeling sick at first with mine, which was solved by having more for breakfast, and it settled down, and it’s made an enormous difference to what I can do. I could [sighs], I could do less than 10 per cent of what I can do now. I mean, I’m limited now because of this coronavirus lockdown, of course, I have to shield. But if it wasn’t for that, I could go out, I could do a couple of things in the day. Before, I would only do one thing, one day, and I might not do anything then for the next three.

You know, I would, I would have one – and that might be a very small job as well, I mean, the job might be: dust your living room. And that would be it. I would start to make meals and get too tired halfway through to finish them? So, I’d say “Oh I’m going to do roast dinner” and then we’d end up with a meat sandwich. Because I’d have to change part way through. But now I could, you know, I can do what I want. It’s so much better. It’s well worth getting it sorted if you can.

Rather than exaggerating vasculitis symptoms, Jane X says people may “underplay” them because they’ve “forgotten what living normally is.”

And I think for doctors, on the whole, patients maybe, doctors think patients overplay their symptoms? But patients who have got a chronic thing that’s come on slowly actually underplay their symptoms because they’ve learnt to live with them?

So, you adjust your life, you know, over the period when I was ill, first of all I got somebody to clean the house. I gave up doing things, I cut my hours at work twice and then I gave up the highly pressured job to do one where I didn’t have to think, because I just didn’t want the pressure of it anymore. You let things go, you just don’t do things, you never go out at night, or do anything like that because you just haven’t got the energy. But then when the doctor says, “How are you?” you sit there and go, “Well, I’m all right,” because maybe that day you don’t feel too bad? Or just because you’ve just got used to living that way and you think, you’ve forgotten what living normally is.

I think that that would be the thing I would say to doctors. Don’t think your patient is making it up or exaggerating it; they’re most likely not telling you the whole of it. They mean to, but they’ve learnt to live with it and therefore they don’t. They’re not able to explain it.

And I mean, obviously, that’s a problem for doctors because if you say to them, “Oh, well, it comes and goes and I’m all right between,” you know? During the periods they were looking to see what I’d got, they thought maybe I’d got gout and then they thought a periodic fever, there are these conditions called Mediterranean fevers? Obviously, it was neither of those. The worst misdiagnosis I had was once when I had a very bad attack, it was right at the beginning when I didn’t really know that it was something that was repeating itself and being unable to walk and having to be carried to the car, I got taken to the out-of-hours doctor who diagnosed me with a chest infection [laughs]. He said he could hear a rub on the lungs, which was probably the vasculitis. But. You don’t get many people with a chest infection who go from okay to not being able to walk over the space of a couple of hours.