Diane

Age at interview: 39

Age at diagnosis: 30

Brief Outline:

When Diane needed emergency heart surgery, it was unclear what had caused the problem. She now knows she has Takayasu arteritis, a type of systemic vasculitis. Diane’s care is provided both in Northern Ireland and by a vasculitis specialist in England.

Background:

Diane works part-time. She lives with her husband and their two dogs. Ethnic background: White Northern Irish.

More about me...

Diane recalls her 30th birthday in 2011 as both the holiday of a lifetime and the first sign of health problems - swollen legs, aches and pains, and fatigue. Two months later she noticed pains in her chest, which got increasingly worse over a few days. Following tests, she was advised it was likely to be costochondritis (inflammation of the chest wall) and that the pain should pass within six weeks.

Instead of getting better, the pain worsened. Diane was on her computer booking tickets for a concert when she suddenly felt the room spinning and had difficulty breathing. She managed to phone her mum who called an ambulance. As Diane explains it, without anyone knowing, her aorta (the main blood vessel of the heart) had torn.

At the hospital, her family were told to expect the worst, as the chances of her surviving transfer to a specialist hospital and then a major operation were small. In an 11-hour operation, the heart surgeon discovered the extent of the damage to Diane’s aorta. He replaced affected parts with synthetic material, which meant she would need to take blood thinners for life.

Pathology tests on the parts removed from Diane’s heart suggested she might have a type of vasculitis called giant cell arteritis (GCA). Diane says a rheumatologist told her that this was unlikely because she was so young. As a result, she focused on recovery from the surgery and on a phased return to full-time work. Unfortunately, Diane struggled over the next six months with fatigue and muscle pain and was disappointed when it was suggested this might be due to anxiety.

Diane describes the “lifeline” of discovering a vasculitis support group. She says this helped her understand that she was living with a chronic illness. It also gave her the confidence and contacts to request referrals to a specialist vasculitis doctor in England and an immunologist in Northern Ireland.

Rather than GCA, the vasculitis specialist suggested that she had another type of systemic vasculitis called Takayasu arteritis. Diane’s current medication is low dose steroids along with weekly self-administered injections of an immunosuppressant (methotrexate). She knows there are other treatment options if necessary and trusts her specialist’s advice.

Vasculitis left Diane “knocked for six” but she says cardiac rehabilitation and a charity programme for taking control of your illness helped rebuild her confidence. She is glad that she sought help for her mental health and feels the benefits of counselling should be available to everyone with a chronic illness.

Diane and her husband have had to scale back the pace of life and rethink plans for work, travel, and family. However, on a daily basis, she is grateful for her two dogs who give a focus for her attention, keep her active, and mean she never feels alone. 

Diane says it’s hard to get her head round the damage vasculitis did to her body without her knowing.

No. No, no. You don’t, you don’t expect it. The scariest thing, the thing that I still maybe haven’t really got my head round was how few symptoms I had. And how, you know, it must have, the damage must have been going on for months, maybe years, but how few symptoms I had in such a short space of time. I just can’t understand that and that’s the scary thing because I don’t understand how much damage could have been caused and going on inside my body, whenever I didn’t feel it was happening.

You know, it was only really, what, a Saturday evening when I started to have a wee bit of chest pain and a few breathing difficulties to the Friday, when [laughs], when it was, the aorta was absolutely bursting, you know?

And I think too, because it’s a disability, it’s not a, you can’t physically see it, it’s an invisible illness, you struggle with every person you go to see. If you’ve a blue badge, you know, you park in a blue badge parking bay, somebody walking past could look at you funny and you think, “Oh flip, they’re looking at me, thinking that I look healthy and normal, and what am I doing parked there?” [laughs]. But actually, you’re breathless walking from the car to the shop and you’re in complete agony walking from the car to the shop, but they can’t see, they can’t see what’s going on inside you.

Vasculitis caused tearing and damage to Diane’s aorta (the main blood vessel coming out of her heart).

So unbeknown to me, my aorta had dissected at this point, and that’s why I was feeling all the pain in my chest. So, they rushed me to the hospital, the local hospital. They ran some tests and then found out that, yes, there was a problem with the aorta. So, they… at this stage I was very, very unwell and my family were basically told to expect the worst.

They said they were going to transfer me – I was sedated - transfer me to the regional hospital for major, for major heart surgery, but they warned them that the chances of me surviving the ambulance journey was, was 50-50. And then when they got me to the regional facility, the cardiothoracic surgeon came and seen them and said, “Look, I’ll try my best but there’s a very sort of small 20 per cent survival rate for this type of surgery.”

He expected to be in and out in about six or seven hours, but it actually lasted 11 hours. So, when they went in they realised that my ascending aorta from, from the aortic valve right up to the arch, was all diseased and there were ulcers, and the ulcers had formed pinholes, so that’s where the dissection and the regurgitation was coming from. So that’s the amount of pain I was feeling. So, they had to replace the aortic valve, so they replaced that with a mechanical valve, so that meant I was on blood thinners for life. And then they replaced the ascending aorta up to the arch, so they took all that away.

So, at this point, we still didn’t know what, you know, what caused it. So, I was sort of… my main focus was thankfully I survived the operation. They were concerned then, obviously, was there any brain damage, because they were worried, I was maybe unconscious, so without oxygen for a while. But again, thankfully, there doesn’t seem to be an awful lot of damage. I [sighs] am a wee bit more forgetful than I used to be but, and there’s certain things that I can’t really remember, you know, my long-term sort of memory? But apart from that, day to day, it hasn’t really, you know, affected brain function too much.

Diane thought she might need a biologic drug, but her vasculitis consultant suggested switching from oral methotrexate to injections.

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.

Side effects of Diane’s vasculitis medication include frizzy hair, nausea and what “people maybe won’t talk about” – low libido.

Medication side effects, my hair would have been lovely and curly and hard to manage, but ever since I went on to - I can’t quite figure out whether it was the prednisolone or the methotrexate or a combination of both - but my hair just is now curly, hard to manage, and frizzy. And [clears throat] I went through a phase before, so before I went on to the medication, just sort of in the last year, year and-a-half, I had just got the hair sorted out from the last time being on the medication. And I had lovely treatments done and it was all looking fabulous, and I would have had blonde colour through my hair, and we toned it down so it wouldn’t be as frizzy.

And then the next thing, slap-bang. I think I had my last hair treatment in December and then I was told in January I would have, had to go back on to the methotrexate again and the steroids. So [sighs], yeah, so now I don’t even bother, I just normally put it up in a ponytail [laughs].

But then, obviously, you’ve got the horrible side effects of the, the methotrexate: the nausea, the irregular bowel movements. And then your, the steroids and the hydroxychloroquine – like we talked about a wee bit – it definitely impacts your mood. The other thing that a whole, a lot of people maybe won’t talk about is that it definitely affects your libido, so that obviously then affects your relationship with your husband or your partner.

Diane’s collapse from vasculitis left her fearful of being alone. Now she has dogs Trixie and Ruby.

And then your mental health, so all of this sort of affects your mental health. You have the fear of it happening again. In the early days, I, because I was misdiagnosed and I went home and I was on my own, like, if my family had of realised how serious, what was going on, they would never have left me alone. But I was on my own and just, just the fear of being left alone was unbelievable. My husband had to take about three months off work because I was afraid to be left [laughs] on my own, and then thankfully my sister-in-law was breeding dogs at the time, and he got me a wee puppy.

And I got that for my, for Christmas, so it was September my illness and my heart surgery, and then I got her on Christmas night, Trixie. And it just focussed my attention away from me on to somebody else. I call her my therapy dog because [laughs] she is my therapy dog. And she, it’s so funny, because she comes up to you and she kind of sniffs, sniffs, sniffs your breath. If you’re not feeling well, or you’ve forgotten to take your tablets, or, you know, she’s really, really sensitive. So, I, honestly, I do call her my therapy dog [laughs].

So, she came along and obviously, because we don’t have children, you know, then I was going back to work and I thought, “I can’t leave Trixie on her own.” So, then we got another miniature schnauzer six months later, Ruby. So, we’ve the two of them and Ruby was there to keep Trixie company, but really Trixie keeps Ruby company. Because Trixie was on her own so she was used to being the only dog, and Ruby now can’t do without her but.

So, we’ve two dogs and they, yes, they focus my attention, you don’t feel alone if you’re in the house on your own, because you’ve got them. They get you out for walks, you have to walk them. You know, you have to look after them.

Vasculitis damaged Diane’s heart. Supported fitness schemes helped as she was “afraid of exercising.”

I did do, was it six-week or eight-week cardiac rehab course? Now, my consultant had said, “Look, this is more for people that are maybe a wee bit older, that have had, you know, bypass surgery. But, you know, if you think it’s going to help you, by all means do it.” Now I did it and I was obviously one of the youngest ones there, [laughs]. The next person was maybe in his… I was sort of in my 30s, he was maybe in his early 40s and then everybody else was sort of 60, 70-plus.

But it was, it was good because I was afraid of exercising. Because I didn’t know my limit. So, they had physios there, they had occupational therapists there to help you, they done like a wee mini fitness circuit and they let you, monitor, monitored your heart rate and took your blood pressure before and after, and they made sure that you didn’t go past your limit. And so, you could almost… it allowed you to gauge how much you could do, and, and know your limit. So that made, that made things an awful lot easier because you weren’t afraid to go out for a walk anymore, you weren’t afraid to, you know, go, go to the gym and maybe do the, the treadmill or the, I love the, I loved the cross-trainer whenever I was at the gym, so there were all of those.

And then after your six-to-eight-week cardiac rehab, then there was a scheme called the Fit and Well Scheme that you could go and do. So, there was about six or seven of us from… because we all formed, you know, a friendship. And then we went, and we done the Fit and Well Scheme and then it kind of ended and then you were kind of left, “Oh dear, what do I do now? I’m on my own.” So, I maybe… then, at that point I think I was going back to work, so I didn’t really probably follow it up and continue, you know, as, as much as I probably should have.

But it sounds like it gave you what you needed at the time.

It gave me confidence, yeah. Yeah, definitely. And it also, you know, you had talks from dietitians and pharmacists and, you know, you picked up a lot of information from the cardiac rehab. And I have known a few people with cardiac – that have had cardiac surgery since and I’ve recommended that they go to it. So yeah.

Working from home during the COVID-19 pandemic is going so well for Diane that her employer is thinking about making it permanent.

But, as normal, everything that happens with me, I had to, I had to fight with them, basically to get working from home. This was before the shielding letters came out and it was made mandatory that you start working from home. So, it was about the second week in, in March and then by sort of two weeks later, even my husband was working from home, you know, every, everybody [laughs] was working from home at that point.

So, I think that’s a positive, and occupational health, I went to see - well, I didn’t go to see them, I had a video conference with them over the summer because they were wanting me to increase my hours from 20 hours a week to 24, but in that consultation, occupational health says that they don’t want me back in the office until COVID is gone, you know. You know, it’s, well, it’s never going to be gone, but it’s under control. And that, in fact, because working from home is working so well with me, for my fatigue, you know, you can manage it better. You know, you can take an hour off in the middle of the day if you need to go down and rest and get back into it. It’s just so much more flexible. He actually suggested that it’s, it’s more permanent for me and that I only go into the office on an occasional basis, so even after COVID, I think this will be good. I think I’ll have to fight to get that because [smiles] nothing happens without you actually fighting for it in my work.

With vasculitis, Diane doesn’t know if she’d have the energy for a baby, even if she did get pregnant.

Then, I suppose, related to that, there’s the fertility, fertility side of things. We were married in 2009, September 2009, then my sister got married the following year, so we were trying not to get pregnant [laughs] in that year. And then after that, we were obviously trying very hard. Now obviously whatever was going on with the vasculitis probably didn’t help things, but then after that, after the heart operation, we had to wait sort of a good 12 months, then I started to flare up, then I went on to methotrexate. You have, you can’t conceive while, while you’re on methotrexate.

Then I had to decide, you know, do we, we need to get some help? We went down the fertility treatment side of things and that was all put on the backburner because then I had to go back on to the methotrexate again. So that causes a lot of, lot of issues with relationships and stuff. And it’s, it’s not just the relationship with your husband; it’s the relationship you have with your sisters that, that are having children; it’s the relationship that you have with your friends. So, the first time one of my friends fell pregnant, instead of feeling happy for them, you were feeling sad about yourself. And I suppose I had to come to terms with that.

And now, I mean, I’ve sort of, I’m approaching 40, I’ll be 40 in July this year. I know that it mightn’t happen, and I know that at the moment, obviously, I can’t because I’m on methotrexate, but I don’t think I would have the energy. I don’t have the energy to look after myself some days never mind, you know, a baby. But [sighs], who knows? People have children in their 40s now, you know, it’s not uncommon. But that, obviously, is a major, major impact.

With vasculitis, Diane had to see several medical specialties when she wanted to have a baby; she found this “wasn’t really joined up.”

So, I had to go to see cardiology to see whether he thought it was wise and he says, “Well, it’s going to be high-risk. It’s your own decision, I can’t tell you whether, whether not to or to get pregnant, but this is what you’d have to do.” So, I had to switch to the, to the Clexane, then I had to go and see the obstetrician, and she went through all the other medication I was on and what I would have to come off if I was to get pregnant or be pregnant.

And what else was there? There was somebody else that I never actually got to see, I can’t remember what type of “ologist” it was, [laughs] but when I went back to see the fertility specialist, he says, “Oh, well I’ve written to such and such and they haven’t come back to me.” It was one of the doctors I’d seen, actually, maybe it was the obstetrician, hadn’t sent her letter back to him. So it was, wasn’t really joined up, and the only reason, the only reason why I went to see all those people was because I had the fertility appointment prior to being so ill. I think everybody was kind of afraid to say yes or no or maybe. And we then, at that point, because I was on methotrexate, we decided to leave it until after I was off methotrexate, about six months or so, and feeling well again. And then I never really got to that point because I was off it for a few years, back on to it again.

So. And then, with fertility, you, the weight thing, so I’ve actually, from - when I went to see him, my BMI was too high - my BMI has probably shot up, actually, instead of going down. So, if I was to go back for fertility treatment, he wouldn’t do it because I would have to probably lose about five or six stone before, before that was even an option, if I wasn’t on methotrexate and if I was feeling healthy enough.

But, again, that was me sort of initiating that process, if you know what I mean? If I hadn’t have pushed for that, that wouldn’t have happened. The specialist in England said there’s no reason why I can’t, if the vasculitis is active, then that probably would have been the reason why I couldn’t before, but he says, “Obviously, we’d have to monitor you very carefully.” And he has had many successful pregnancies for people with Takayasu, so it’s just a matter of managing it.