Apparently, they took me into ICU, [sighs] according to him, my, that night, the first night there, my heart stopped twice. It was very, so those days, I can see them through his perspective, through what he remembers. And he even, like, when we go there, he even has people, you know? So once, I remember we were walking down those corridors and he stopped, a short gentleman, he was like, “Look, there she is, you know, she’s alive, she’s walking in front of you.” [looks confused and laughs] And I don’t, I don’t remember that person. Apparently, that was the person that was with me in ICU. In ICU they’re very, it’s like, one nurse, one patient, they’re very organised like that.

[Sighs] What I remember from ICU is, I think after I woke up, I saw my mother. I remember I was thirsty, initially they didn’t want to give me water, they just put some bit of water on a sponge, and they did like this on my lips [gestures]. I remember having an oxygen mask on my face and they were saying, like, “You should sleep,” and I was like, “I can’t sleep because it’s too cold too in this oxygen mask.” A lot of things going on, a lot of movement, but I couldn’t see a lot. I could just hear.

Anyway, after I think two nights I remember sleeping there, I moved to a different, to a different ward and things were different. There was a lot of, so after, after they… Apparently, I also got dialysis when I was in ICU, according to my husband. I don’t remember. I had some in here, [points to neck] I had some wires, some, I don’t know, I think they… Who knows what they did during that time, but here, like, I had three or four things? That was again, really weird when I actually realised. I didn’t know but at some point, I actually realised that there’s [points to neck].

And he discussed the options, which were either cyclophosphamide or rituximab, and explained that the cyclophosphamide was a long-established treatment, known to be very effective but could have a lot of unpleasant side-effects. Whereas rituximab was a much newer drug and that was – so it wasn’t as, it wasn’t as certain to be effective, and, but it would definitely have fewer, possibly no side-effects. So, my response was simply to say, “Well look, if we go with the one that’s more effective, the cyclophosphamide, but I find I can’t tolerate the side-effects, can we stop that and switch to the rituximab?” So, he said “yes,” in which case in my view, it was a no-brainer, one goes for the one that is proven.

So, we went on to the cyclophosphamide and he explained that in parallel with that, one would normally take steroids as well. He’d explained the, you know, the two stages of the treatment first in its initial treatment to hit the vasculitis hard, to try and suppress it, and then to be followed by the maintenance therapy that tries to just keep it under, under control.

He’d obviously also explained that there was no cure for it because, so far, research had not found out what caused the problem. But again, he explained also, and I’m sorry I’m doing this sort of in reverse order, but he explained how it worked. What it did was cause the immune system to attack oneself rather than to attack external infections, so the requirement of all this treatment was basically to suppress the immune system.

Now, in talking about normally going on to steroids, he then spoke about a trial that was underway with an alternative to steroids, because again he explained that steroids have some nasty side-effects. So he was, had some involvement with this new trial of a new drug, and would I be interested in going on that? So, I asked the same question: if we go on that and we find it’s not working, can we switch to the steroids? You know, and again, he said, “Yes, we can. We can stop the trial at any time and go on to steroids.” So, I said, “In that case, we’ll go on to the trial.”

But, overall, my fatigue was improving. I was going, “Look, if I can just get back to work…” Then when I switched from the oral methotrexate to the injections, I couldn’t believe the difference. The difference was like night and day. I, my symptoms, my side effects of taking medication on a Sunday night could have lasted to a Thursday, so I had no time to work. On a Monday, I felt absolutely awful, whereas when I had, took the injections and they started to work, they, I got more… had less side effects, they lasted, you know, on a Monday, sometimes, or being a wee bit dizzy and a wee bit, I would call myself dopey. [laughs] I’m not really, you know, I couldn’t drive, or I couldn’t make a decision, and I could, you could maybe say words back to front or not think of the word that you were wanting to say [laughs]. That’s just how you feel, you don’t feel in control of your own body on the Monday, but I could function to a certain degree, which was brilliant, and then I was able to go to work on a Tuesday, Wednesday and a Thursday, you know, and…

The other good thing about the injection – which I wasn’t aware of at the time – was it isn’t absorbed by the liver; it goes straight into the bloodstream. So, the efficacy of it is actually higher. So, you actually, I needed less, so I was taking 25 milligrams of the tablets and I only needed 20 milligrams of the injection, but the 20 milligrams of the injection was, I think, is it like, the tablet says 50 per cent absorbed, whereas the, the injection is maybe 70 per cent absorbed.

So it was, it was magic. And I don’t know why it took… my team here in Northern Ireland told me I couldn’t go on to it because they were worried about infection at the injection site. And with the warfarin there would be a lot of bruising. Supposedly. But my specialist in England told me that that was a load of nonsense, basically [laughs]. And I couldn’t believe it was like night, night or night and day.

And you’ve said it’s the unpredictability that’s really hard to deal with because, presumably – from what you’ve said – when it flares, there’s nothing that you can say has led to it flaring, it just happens.

Well, a lot of the time, infection can lead to it, okay? And that’s why I have the antibiotics, okay? So, if somebody’s had something in the house, they’re not feeling too well, they’ve been infective, you think “Oh, that might be it.” So, a lot of the time, it’s an infection. And that’s why I try, I try the antibiotics, try the steroids up. If that doesn’t work, well it definitely is a flare, but it’s often – it can be triggered by an infection, this is the problem. And we get [inaudible] immune system. So bad, like last September I was away, I got bitten by horseflies, oh, it was multiple, it was awful. Ended up, we were out in Braemar in the caravan, I had to come back in on Saturday and end up in A&E, massive cellulitis, I mean massive. They said to me “Double your dose of amoxicillin. Ten days. If it doesn’t settle down and it’s not feeling better in about three or four days, you’ll have to come and get IV antibiotics.” It was really, really nasty cellulitis. So again, that was from a bite, an insect bite, and it just [makes phew sound] massive.

So infection is often a trigger and that’s why I have antibiotics handy, because that’s one of my first lines, “Okay, just try an antibiotic,” see if it knocks it on the head. If it doesn’t, steroids, okay, doesn’t, okay, we need more. So, there’s a certain sort of, I don’t know, line of action I would take. So, a lot of the time it, it is infection, and that’s why, it sounds awful, maybe I do stay away from people. Because I don’t want infections [laughs]. You know, subconsciously? You know, people carry bugs, I’m very, and people around me are very wary and the family are very wary, my in-laws, you know? Don’t, mm. I think it’s caused a bit of isolation, [laughs] to tell you the truth. People don’t want to come near me. For fear of giving me something if they’ve got something. A lot of that goes on. Yeah. Mhm. Yeah.

So, at the point that happened, they took me off the immunosuppressants and sort of said that the Behcet’s was less of a threat than the infections at that point, and I still had a lot of the immunosuppressants in my system, they said it would take about six months for me to sort of get back to the level that I was before.

So, I went back to the Behcet’s Centre. We had a lot of in-depth discussions about what this meant for my life, and yeah, I quite quickly decided that I was not happy to be without some level of immunosuppression? I wasn’t going to go back to that place that I was as a teenager, that Behcet’s ruled my entire life, and that I have an appreciation that there was some risk involved, that, that I wouldn’t have a life if I didn’t take that risk, because I’d spend so much of the time having flares, being bed bound, not being able to speak or eat, that I just wasn’t willing to go back to that place. Because of course that in itself makes me at risk for infections, so yeah.

So, they decided to start me on biologic therapy, which was sort of a last resort treatment because it’s very expensive, but so far, I’ve done really quite well on it. I inject myself every other week, it’s sort of become part of routine, for someone that’s undergone a lot of medical procedures it’s not a big deal.

And I’ve had a couple of infections, and I’ve had one very serious infection, but aside from that I’m quite happy and sort of when I, when I commit to something now it sort of, I tell people that, “Look, I’ve got this condition, I’m prone to infections, things happen, I end up in hospital unexpectedly, it’s not an issue of my commitment to something, it’s just sort of the way I am.” And I feel like the effort I put into things otherwise makes up for it a lot?

I have followed that, up to a point, it just, just over a month ago now, I was struggling to make the drop from ten milligrams to nine, so I’ve, I’ve returned to ten and, it’s one of the areas where my previous PMR experience I think makes things easier for me in that I have the, you might call it confidence to, deviate where appropriate from the prescribed path. It’s not being cavalier, it’s not deciding I know best and so on, but it’s just realising that the schedule that’s been mapped out is a suggestion. But I think as I said earlier, the disease will go at its own sweet pace and if that’s not tying in with the reduction in steroids you’ve got to be able to say, “No, just I can’t go down, let’s just stop here a bit.”

It’s a discretion that has to be used carefully, though, because you do read in the online sites about people who have quite a bit of trouble, if they play around with the doses too much you can end up with what they call yo-yoing and you, if you go up too quickly and then try and come down, the, the whole pattern of your inflammation I think can get disrupted, it never truly settles and you can end up with I think just more problems in the long term. I don’t know the details of why it happens but it’s very much the tone that I pick up from what I’m reading.

So, I think I, trying to be careful about it, don’t be too dramatic, give, give the aches and pains a chance to go away when they do appear because, when you do a drop down, say from ten to nine, the body may react just to the reduction in steroid, but they reckon that any impact of steroid withdrawal will go away after two, three, four days. So, I gave it two weeks before I said, “No, this is, this is lasting so let’s just go back up.”

Since coming off the IV and the 60 milligram prednisolone tablets, I’ve tapered down, and I got down to about seven or eight. And then I thought – and then I chatted with this patient in [large city] and asked her how she was getting on, a few, a couple of years later. And she said, “Oh, I’ve got down to five.” I said, “Right, if she can get down to five, I can get down to five.”

I did take it very slowly, but I went down, slowly, half-milligrams. I went down to five. Up until then I’d had no relapses whatsoever, and suddenly within a couple of days I had a relapse. Severe headaches, recognisable, these headaches. Temple headaches.

And so, the doctor – I’d changed practice by then, practice in [town] – she got me straight, she got straight on to the rheumatology and they put me straight back on to 60 milligrams of oral prednisolone. And then I came down, tapering down again, and they said, “Whatever you do, don’t go below, below seven.” And I’d been told this by my rheumatologist, so it was my fault. Trying to be clever. So, I’ve been on seven now for some time and I’ve never had a relapse. Seven is a good place to be, actually.

So basically, from then I’ve just been kind of up and down with the prednisolone, tapering, you know, it seems to be like every time I get down to ten milligrams or lower the, I start getting a bit headachy and the, the CRP goes up again, so I’ve got, I’ve been a bit up and down with that.

They’ve been trying from the, well the consultant’s been trying from the beginning to get me on to tocilizumab, which I think, I think that’s how you pronounce it, which is a, a drug that helps you get off the steroids quicker. But being me, I’m quite phobic about taking tablets and like I read all the, you know – it’s from previous experience – I read all the leaflets and you know, if I think the side effects are going to be bad, I just don’t take them; I’m a GP’s worst nightmare [smiles].

But the tocilizumab, when I read that it said it’s not recommended if you have diverticular disease because it can inflame it, so I’d mentioned that to her so she said, about methotrexate – which is a cancer drug which is scary in itself – and I didn’t like what that could do, plus you’ve got to have blood tests for your liver and you’d be on it for life and I just said, “I really don’t want to go on these other drugs, I feel as if, you know, why, you’re on a horrible drug and then you’ve got to go on to another horrible drug to get you off this horrible drug.”

But I feel as if I’ve been quite pressurised into taking these drugs and I just don’t want to take them. You know, I became quite upset and quite stressed about it because I thought, you know, “They, they’re the experts and, you know, am I doing the wrong thing by not, by refusing to take it?” So, I think they’ve kind of given up and they’re trying to support me just coming off the prednisolone by the tapering. But I’ve said, I’ve said to the, the consultant on Tuesday that, you know, if when I get down to below the ten milligrams again, and I start having problems that I would maybe consider going on these, one of these other horrible things, but….