Systemic Vasculitis

So eventually, I had the diagnosis. I think the main thing the medical profession and my GP surgery were worried about was that it had taken a long time to diagnose, and because of the issues of giant cell arteritis - because of the issues of possible loss of eyesight, and the other one that was mentioned was possible aneurysm – so they wanted to put me on high levels of steroids very quickly. So, I was put on 50 milligrams of steroids along with other types of medication alongside that, calcium, etcetera. But the main worry was they were urging me to go and get the tablets right away, and I did that.

And they did say to me within about 48 hours I would feel wonderful, [laughs] such a rapid difference, and I have to say they were right. The steroids at that point made me feel like leaping like a gazelle, I have to say. I was, I was joyous, the headaches were gone, the physical symptoms were gone, and I felt like Superwoman. And my first kind of rheumatology appointment, I was kind of knocked on the head a bit about that, they said, “You will feel like Superwoman just now, but you probably won’t be feeling like that because we have to wean you off them.”

And I was still incredibly ill at that point and was reliant on being carried, kind of needing support from others, and not very able, and deaf at that point. No hearing at all. And immediately, I was meeting kind of doctors in rheumatology, and a few days later, was started on high-dose steroids and rituximab.

And it’s amazing with the steroids, that within two days I went from feeling incredibly ill to feeling much, much better. It was, it was amazing, the impact of it. Suddenly within two days, night sweats went down, swelling went down, loads of pain went down, and we started on the rituximab that continued on for a number of weeks. And that was all incredibly helpful, and they were such a supportive team. And the hearing loss and, and the deafness continued for some time, but then eventually at some point, the inflamed parts around my adenoids at the back of the throat kind of shrunk and then was able to clear the fluid and my hearing came back, which was a wonderful experience.

And at that point, things were looking quite good. I’d had kind of solid treatment, I’d been reducing my steroids, my symptoms were improving, I was able to go for long walks and be able to be outside a lot.

And that’s when they then moved me to another hospital for into the renal unit because obviously my kidneys were affected by it, my lungs were affected by it and generally I think they put you on the renal unit, all vasculitis patients go on the renal unit. So, they put me in my own room on the renal unit and that’s when I started treatment for the vasculitis, which initial treatment was chemotherapy, so it was three rounds of cyclophosphamide that I had the first one I had when I was in hospital as an inpatient and also immediately a really high dose of steroids for a significant amount of time, I think it was initially 60mg of steroids that they started me on.

And yeah, so that all happened when I was in hospital as an inpatient and then when I left hospital, I was in there as an inpatient for I believe about a week in the renal unit and then when I left hospital they then continued the treatment with the cyclophosphamide and I think my final round was early January time, so my first one was November, my last one was about early January, late December. I think it, yeah, I think it was actually early January because my birthday was kind of at the end of December and we, we postponed it like a week or so, so that I didn’t have to have it done when it was my birthday. So, I finished the cyclophosphamide and then we get started onto usually like a background immunosuppressant, so I started treatment on a drug called azathioprine which I’ve now been on since I started it, not really any problems with it been on that for four years or so now. And then they start the weaning down of the steroids over time and that’s where I’m up to now is: had it for four and a half years, still continuing to try and reduce the steroids that’s it really I guess. yeah. I’m not sure what else to say [laughs].

It’s such a lot for anybody, never mind somebody who’s 19.

Yeah. Yeah it was a, it was kind of, yeah, I guess it was, it was a lot for at 19 to be like, “Oh you’re having chemotherapy,” it’s like, “that’s strange,” also find out all this world of like, you know, “oh, we’ll treat you with chemotherapy,” it’s like, I thought, I thought that was a drug that they use for cancer, you know? It’s not something that you realise that they use for other really serious things. You know, it was kind of my world sort of got turned upside down.

So, I was in hospital for another week where I had intravenous steroids, again another heart drug, and Churg-Strauss is an illness where it affects four, possibly four different parts of your body, as far as they know now, and that’s your heart, your lungs, your kidney or your bowel, and unfortunately in my case it’s my heart because that obviously is something that’s quite crucial.

But with the steroids, I started to feel a lot better. I was released from hospital, quite strange after being there for a month, and, returned home, which was lovely to do, to actually come back to home, it was the middle of winter, it was beginning of December, but just to be there you know, and, and have sort of familiar things around you and be able to very slowly do things for yourself.

And then in January, I started to go back up to London every two weeks for, chemotherapy, which was part of the treatment. So, the treatment they gave me was quite aggressive. The consultant that I had, [name of consultant], he felt that you know, you should look at it in the whole and try and remedy everything at the same time, and do that, and it was very effective, and after four treatments in London, and we used to combine those going up with having lunch in one of the restaurants, my parents used to take me. So, it wasn’t just going to London for the chemo, it was going up there, having a bit of lunch in a fish restaurant that was nice and, and then go and have the chemo and travel home.

I then had, I was still on a load of drugs, heavy dose of steroid tablets, so even after the chemo, we got round to sort of February/March, still very tired every day, used to spend a lot of time just sleeping or on the settee, and gradually getting back to life and that took about six months before everything sort of started to return to normal, and even then I don’t think I could’ve done a full-time job after that.

And then the next days were [laughs] were just tired, I mean all I do remember is having to get blood transfusions. There was a day, it was the Friday, it was just an onslaught, they put in, so I woke up to the, on the Friday morning, they said they would put an internal line - he didn’t explain that, so out of all the bad news, he kind of threw in, he was like, “I could give you a line that comes out your neck for the plasma,” he said, “But I think you’ll probably need it for dialysis.” So that’s how serious they were about my dialysis, he said, “So I’m going to put in an internal line, and you’ll go to theatre for that and it’s fine, it’s nothing major.”

So I still hadn’t thought anything of this and then the Friday morning, then couldn’t eat, had to, you know, fast, obviously. And then was down in this theatre, the next thing, and they’re like talking about – and that again, when I think back, that was, that was horrible because you’re awake, [laughs] so you’re actually lying and aware that somebody is like cutting you. I mean, they slightly numb it, but, actually, they did ask, “Do you want to be slightly sedated?” I was like, [smiles] “Yeah, would you, would anybody not want?” and they were like, “Well, you know,” and I’m saying, no, I said, “I’m quite happy with the maximum sedation, thank you.” [laughs].

And thank goodness, because it just, it felt like people moving you around, you’re kind of in this theatre environment that’s like, you know, just again, it’s all these stressful-inducing moments [laughs].

And then, it was just everything was happening. Came up and there was a woman wanting to do research with me. I’m still trying to have something to eat by this point, and they were taking me down to do a plasma there and then, like, I mean, I remember, I think I had 20 minutes from back to theatre, still groggy, woman asking me about some research and me still not having a clue what had happened. My neck was stiff but I just didn’t know what had happened to me. So found myself then being wheeled down, it was just everywhere, that was a, it was a manic three days, really.

But the Friday, this day that I’m talking about, was the day of everything, so then, turned up in this unit with the big machine, and all the wires and all these bags and them explaining that, you know, I think I got 68 bags of plasma all together, so, it was going to go on for, I think about five days? So, five days for four hours every day, I was attached to a machine.

I feel like I’m talking about somebody else, do you know? That’s funny. I feel like that it’s someone else’s story, almost, because it’s always like that when you look in on it, isn’t it? You’re in it so you just do it, but when you’re looking in on it, it feels totally different.

Okay. Now, I understand that, so they didn’t say why or, they didn’t say much, they said, “This, we need to do this, we need to…” A lot of people with a lot of forms, you know, this procedure could be, “We need to do this because what you have is life-threatening and we have to move it.” I was like really not aware of what’s happening, and they were coming and explaining things and, you know, wanting me to agree. I was like, “Okay, you can do whatever you know best, I can’t, I don’t have the, I can’t process all of this right now, so whatever you guys think is best, just go ahead and do it.”

Okay. They done that. So, what happened, they started with the plasma transfer. Now, my vascath here [points to right groin] sustained the first plasma transfer, [sighs] but the second one, I remember the nurse complaining that, you know, this, something that, it’s blocked here, it’s not working. It went through but with, you know, “Let’s stop, wait a while,” so it was very hard to actually do it. What happened, meanwhile, a blood clot formed [points to right groin area] here.

And the, the nurses insisted, like, “Look, we can’t carry on using this vascath. So that, it’s not possible.” And they also want me to do more plasma transfer, so we are moving forward, we are pushing to do this. “So, let’s change it, let’s put the vascath in the other leg.” Okay. Now, I understood that the main concern and their main concern was to avoid bleeding, right, in the situation. Nobody thought of clotting. So, I was a bit atypical, and I developed a clot that went to my lungs, and then it went to my heart.

And [sighs] in all that, in, I don’t know. I just remember that they also give me, they were giving me a blood transfusion. I was very upset that they weren’t telling us anything that, you know, we didn’t know what’s happening and we didn’t… And I’ve asked my husband to, I want, I said, “I want apples, bring me apples,” and he went to buy apples. And then I woke up and I saw my mother’s face? So, I missed three? Two, three days, something like that? Apparently, he came back and I wasn’t breathing? So ,what happened, that blood clot went to my lungs and to my heart. It was very terrible because he found me not breathing, so he was there through all of that experience which kind of was a very traumatising and shocking event for him to go through.

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.

I mean that – that was what, 2000, February 2003, and I’m still here, and at one point I really thought I would never see my kids grow up, it was that bad. But I won’t say it’s, it’s been easy; it hasn’t. I get, even now I get regular flare ups. I’m not off steroids, I don’t know if I’ll ever get off steroids. I still take methotrexate once a week and I’ve also started another course of rituximab. I know when it’s flaring, I’m very, I’ve kind of got quite good at when it’s flaring because I know the signs and symptoms. It’s ENT, I call fresh blood, pain, I think the exhaust, the tired – the fatigue is, is one of the biggest problems. There were times I just couldn’t get off the sofa but a lot of it I try and manage it now.

Did the steroids make a difference to the headaches?

No, they didn’t. It took, took a wee while before - I think they eventually did, but it took me quite a few months. I can’t remember now whether it was when I got on to the methotrexate or not. I can’t remember, I’m sorry about that. And the headaches – I’m not bothered with the headaches now. Haven’t been for quite some time. The consultant did tell me that it wouldn’t be a quick cure. It would be, she didn’t say how long, but as I say, that was the end of 2018, so it has taken a little time. But, other than fatigue, I don’t find it restricting me at all.

And what about the polymyalgia side of it? Do you still have symptoms of that?

No, no. I don’t. I haven’t had that for quite a long time. That, no, the steroids seemed to take care of that and I’m still clear of that. I occasionally - but very seldom - I’ll maybe get a slight feeling here [touches both temples] and I do take note of it. But it hasn’t happened for fully a month, and it never lasts any time. Very short. But I don’t really have a lot of trouble at all, I’m really very, very pleased with the way everything’s going.

I tried this, various other drugs, they were mycophenolate, cyclophosphamide, methotrexate, so I had been through a lot of them which none of them were working and by then my disease was still causing destruction and damaging cartilage around my body. I had scleritis in the back of my eyes, posterior scleritis. I had, by then I had 40 per cent hearing, tinnitus, saddle nose deformity, achy bones, joints, I had been having a lot of scans.

During July I became unwell and was admitted into [the tertiary referral hospital] with sepsis, I was allergic to azathioprine, which was the latest drug that I was on, so they took me off of that and put me on the next one.

[The tertiary referral hospital] was eventually given permission to administer rituximab in 2011, so for two years they were fighting for me to go on this drug, and during this time the damage was continuing. I was finally given rituximab in 2011 to 2013, which did bring my disease into remission.

But they don’t know how long I will be in remission. Rituximab is a new, is a new drug. They don’t know. Obviously, it affects people differently; some people have to have further treatments. But yes, it’s now, what 2013, is, was my last infusion, so that’s seven years, and off the drugs for two, so it’s obviously the right, doing well for me.

I mean I used to just think I was just lazy because I was permanently tired. And then it wasn’t until, it took a while to get a treatment that worked, I started off with methotrexate, which did nothing, and then, and I did, I wrote these down because I knew I wouldn’t remember the names, let me just get my bit of paper.

Yes, I started off with methotrexate and then they tried colchicine and then azathioprine, none of which worked, and I was on prednisolone for a while, which worked a treat, but I put on about three million stone, but it, I mean I felt like a different person: I could run up and down the stairs, I was as, you know, I felt 20 years younger, but as fat as a house. And then [laughs] then they started me in infusions of infliximab and suddenly, everything just came together, and the infliximab worked a treat.

So I, I went, I would go to the [tertiary referral centre] every six weeks for this infusion and, and then that changed, the treatment changed, it would've been April 2018, it was after I’d come back, I'd been to New Zealand. And it changed to, I now inject myself with adalimumab once a fortnight. So now, I only have to go to the [tertiary referral centre] once every three months, which is so much better. Because obviously it’s a bit of a hike from here, you know, it’s a good hour each way, it’s not on the doorstep. And the, touch wood, the adalimumab is working well and the symptoms are under control.

I have, I do occasionally get flare ups you know, where the ulcers will come back. I mean I do still get the occasional ulcer, but they're self-inflicted, you know, I’ve, I’ve bitten the inside of my mouth or something. But every now and then I have had a, a proper flare up where it’s come back with a vengeance and I've had several, and they used to give me a prescription of prednisolone which I would take but I’d rather, I now know that the flare up will dissipate, and particularly now I’m doing the injections every two weeks, by the time my second, next injection is due, that will take care of the flare up. So, I’ve knocked the prednisolone on the head – as effective as they are, I don’t like taking them anymore because I know I’ll just, I mean I’m fat enough as it is [laughs].

So, at the moment things are well controlled, and I said to one of the doctors, one of the consultants there, once the infliximab and the adalimumab started working, that I couldn’t believe how ill I had felt and how rotten I had felt for so many years, until all the symptoms had gone away. It was just amazing, and I just felt like a different person, you know, it’s, you don’t realise how debilitated you are until that debilitation is taken away.

I went on infliximab, and when I went on infliximab, the hope thing came in again. I thought, “This is it, I’m going to have infliximab and I’m going to be like, ‘Yayhay,’” you know? “Doing cartwheels, back to normal life” and whatever, and it didn’t. But it gave me a quality of life that I was, that I was missing. Because when I think, when I think back to the pre-infliximab days, I felt, every day I felt like I was being tortured. The pain, I couldn’t sleep from the pain, the fatigue, the other symptoms and whatever. My quality of life was awful. Bearing in mind that nothing helped the pain; I didn’t take anything for the pain because I’d tried all the drugs apart from opioids, because to me, that was a slippery slope. I wasn’t prepared to go on opioids, to start taking opioids, for something that I didn’t have a diagnosis for, because it only ever treats the symptoms, it doesn’t get to the - and I maintained throughout all of this that the key to me, to me getting symptom control was to understand what was causing the symptoms, and to target the underlying disease. Not to treat the, and to treat me. Treat the patient, not the disease.

So, I went on infliximab and it wasn’t the miracle cure that I was hoping for, because what is - which draws me to another maybe tangent around the understanding of things like cure and remission and relapse and whatever, and do we understand what that means to, to a patient?

And should we be talking, you know, can I ever, can I ever be cured if I live with the damage that’s already been, been done? You know, remission? So, my bloods have been, my bloods have been fantastic, for years now, but I still live with symptoms every day, so remission from whose point of view? And whose point of view is important in this, but it certainly brought me back a quality of life that I, that I had been missing. The terrible thing was that, in many ways, it was too late. It was too late for my job because I’d already lost my job, already been dismissed on capability grounds, and also because I’d had, developed this form of dystonia called blepharospasm, I’d lost my driving licence as well. So quite profound changes and whatever, to my life.