Karen

Age at interview: 58

Age at diagnosis: 53

Brief Outline:

Karen has eosinophilic granulomatosis with polyangiitis (EGPA), a type of ANCA associated vasculitis previously known as Churg-Strauss syndrome. She has found exercise and a calorie-controlled diet an important part of her recovery.

Background:

Karen is a retired insurance company chief executive who now does freelance work for an online estate agent. Ethnic background: White English.

More about me...

Given the mix of symptoms she experienced, Karen is not surprised that vasculitis is difficult to diagnose. In 2013, she was taking time out to travel the world after 30 years in the insurance industry. However, in spite of repeated treatment, she was becoming increasingly unwell with asthma, colds, lumps on her arms, and a chest infection. She developed polyps up her nose which affected her breathing and made her embarrassed to go out. Even after the polyps were removed, Karen started getting rashes, and her pain and fatigue grew worse.

In 2015, Karen became so unwell that her parents came to look after her. Following tests in accident and emergency and treatment from paramedics, she was admitted to intensive care with a heart problem and breathing difficulties. During a month in hospital she had numerous tests and biopsies. Karen remembers that it was a junior doctor who - having spent time talking with her and reading her notes - first thought she might have eosinophilic granulomatosis with polyangiitis (EGPA), a type of ANCA associated vasculitis then known as Churg-Strauss syndrome. After the diagnosis was confirmed, Karen started aggressive treatment with steroids and chemotherapy to bring it under control.

Karen recalls coming home as lovely, but that she spent a lot of the first six months sleeping. High-dose steroids compelled her to eat, and two biscuits easily became a whole packet. In 2016, when her bloods were normal and she was signed off from the heart clinic, something “twigged” and Karen decided to embrace life again and get fitter. A personal trainer supported her to monitor calories in and out, with a small deficit each week. Over the following year, Karen lost the extra weight and felt better in herself.

Karen considers herself to have advantages in living with vasculitis. She has the support of her parents, brother and friends. She has not had to worry financially and has a freelance job where she can choose how much she takes on. Karen is happy to keep taking the daily tablets (low doses of azathioprine and steroids) that suppress her immune system and allow her to enjoy an active, fun and normal life.

Karen says she completely trusts the vasculitis team who made her well again. She particularly appreciates the continuity of the relationship with the consultant and specialist nurses, in person and over the phone. She knows that, when she has an infection, abnormal blood results or concerns, these are investigated quickly and thoroughly. She does however wish that it was easier to explain to other people what vasculitis is. This would help them understand why she takes extra care over infections and sometimes has to rest up, even though she looks well.

Given how “strange” vasculitis is, Karen is not surprised that it’s difficult to recognise and understand.

Well, it’s strange to begin with. I mean which illness do you, the symptoms are, you know, late onset asthma, lumps on your arms, polyps up your nose, you know, it’s just, you would never join those dots. So, it’s no wonder that people didn’t recognise it.

They are weird quirky things the immune suppressant illnesses are. I don’t think we fully understand, you know, about how our cells do this, that and the other. You know, it’s just like coronavirus, you know, we’re learning daily different things about that. And there’s still some huge question marks that people can’t answer, why it attacks certain groups, you know, and you start to ponder on that, why it attacks old people but not kids.

This world has some very strange ways of manifesting things and the way I look at it, there’s coronavirus and vasculitis and anything else would, very, very difficult to understand it, you know, even with the amount of knowledge that some people have and the amount of research that’s being done. These are things that are changing all the time.

A junior doctor spent time with Karen and “put two and two together.” Specialists were there “within the hour” and diagnosed her vasculitis (“Churg-Strauss”).

I was kept in the ICU for about four days and four nights, monitored all the time, they, they’d basically given me drugs to slow my heart down and make me rest, until they could find out what was wrong.

After the four days when it wasn’t so critical, I was moved to a private ward, again there was more scans, more tests, and the, the key presentations I had, was this breathing difficulty with the asthma, it was pains in my back and across my neck, I had lumps on my arms and a rash on my legs, and I’d had these polyps up my nose.

Anyway, this went on for about two or three weeks with all the various different tests and, where I’d been away travelling, they had to check that I hadn’t picked up anything from abroad, I’d travelled to a number of unusual countries. They then started to do biopsies on the small lumps on my arms and give me more scans.

And I suppose I was very fortunate, at one point I had to go to a scan…

…I had a junior doctor come with me, and she was in the ambulance with me, we got stuck in traffic, when we got to [teaching hospital] there was a hold up with one of the machines wasn’t working and we had to work, wait for an hour or so, and then she had to take me back, so she spent the morning with me. And consequently, she really looked into my file. And I think where she was fairly, newly out of college or university, she put two and two together at last and said, “I think this lady’s got Churg-Strauss.” Immediately I said to her, “How do I spell that?” because I knew that my mum would want to know, and she said, “I don’t know,” she said, “It was something we did but I think that’s what the issue is.”

And so then, within the hour, they then got consultants from the relevant department, the lupus [unit at my other large teaching hospital], came over, had a look at all the symptoms and said, “Right, we need more bloods,” et cetera, et cetera, and they said, “Yes, it’s Churg-Strauss,” and then from then on, because they’d identified it, because the biopsy confirmed what it was, they could then start treatment.

Once Karen was home from hospital, she continued with chemotherapy for her vasculitis (“Churg-Strauss”). Her parents used to take her out for lunch before it.

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.

Karen views herself as “in remission” from vasculitis (“Churg-Strauss”) and is happy to keep taking immunosuppressants.

Yes, I suppose, so for me I suppose being in remission came when my bloods had been normal for, for sort of three or four months, also when they reduced my heart tablets and I was signed off by the cardiac area of [the large teaching hospital]. So that was about a year and a half, a year after everything happened. And it might’ve been around that time that it triggered the fact that I wanted to get fit and healthy and lose weight.

But yes, it’s almost like looking at a graph and you’re bobbling about and then suddenly the line comes down and it starts to go straight, and that’s I suppose the way I would visualise it. And yes, on a couple of occasions there’s been a little peak where I might’ve had an infection or, you know, there’s something of concern, there’s always a concern that the drugs I’m on can affect my kidneys, so they watch that closely but that’s not a problem, it’s never been a problem.

I think as well, I’d rather view myself as being in remission, I am very happy to continue the drugs I’m on. I know a lot of people really want to try and get off the drugs they’re on. I recognise the fact that long-term steroid use, we don’t really know how it could affect anyone, but I take the view that I’d rather have 20 years of active, fun life on steroids than try to get off them and feel ill or even worse, put myself in the position where the Churg-Strauss could come back. So, I’m not worried about taking the drugs and I’ll continue to take them.

So what drugs are you on at the moment?

The key drug I’m on is called azathioprine, and that was one of the drugs that was highlighted, that caused me to shield when we had lockdown, and the other drug I’m still on, five milligrams of steroids a day. Then I take one heart tablet, and then various other tablets to, you know, strengthen my bones and look after my stomach and all the rest of it, and I’ll take painkillers from time to time if I need to.

So yeah, it’s a handful of tablets every morning but I don’t, that doesn’t concern me. I’d rather take them and feel like I do.

Karen is relieved that she hasn’t had to worry about her finances while living with vasculitis.

I was very fortunate because I haven’t had to worry financially about anything. So, in the year I recovered, you know, I didn’t have to worry about earning a living or holding down a job, and consequently as well going back to things like going to the gym and having a personal trainer, I’ve had the finances to do that. But I should imagine that, for a lot of people, especially if they’ve got families that they’ve got this type of illness, it’s much more difficult because they’ve got to earn a living and they’ve got to look after a family, I’m very fortunate that it’s sort of at a time in my life where I don’t have to worry financially about anything.

With her vasculitis in remission, Karen enjoys life, but has thought about what she could do if she becomes very ill in the future and wishes “to end it all.”

So, there’s one thing you may wish to include or not to include. But I was having a conversation, as you often do with friends. Especially my age they’ve got parents that, you know, are on their last legs or whatever, and you end up having that conversation about, “Oh, well, you know, if I was that ill, I’d rather go to Switzerland.”

And I was having that conversation a few weeks ago with a friend of mine who’s got a very ill father and we were chatting, and I said, “Well I haven’t really got to worry about that because if I was very ill, and I wanted to end it all I’d just have to stop taking my drugs.” And they looked at me with complete and utter shock and I said, “Yes, I’ve thought about it.” But it would be something I could do. Now I wouldn’t do it lightly, obviously. But if very later on in life I did want to do that I could. And I see that as an advantage. But I don’t know that it’s something that automatically crosses a lot of people’s minds [laughs].

It’s interesting that you say that because it, it was one of the things that I was going to ask you about but I just, I got the feeling you didn’t really want to go there.

No, no, it’s something I had thought about. And especially, I mean I haven’t got any dependents, I’ve got no kids, so that’s something very often later on in life, I’m not saying that you ask your kids to do something, but it’s a conversation you might have with them. And, you know it is a quite a recurrent theme I think in families, you know, when you have parents that are very ill or very bad Alzheimer’s or something like that, how you manage that. And I think I’m quite fortunate that I can, it’s something I can manage myself. If I wish to.

Karen’s vasculitis team feels like a “friendship,” but she doesn’t need so many appointments now.

Yes, so what they do, at [the other large teaching hospital in major city] is in your department you have an overall consultant, and for me that’s the person that saw me in hospital, and he continues to see me or now maybe a telephone conversation every six months. We’re just looking at changing that to every year.

So, I would see him going forward once a year, or have a conversation with him once a year, and then in the six months in between I’d have a consultation with one of the specialist nurses. So, they have about three specialist nurses, and one of them’s called nurse [surname], and again, she was the first nurse I saw when I came out of hospital, and I continue to mostly speak with her.

And it’s, because [laughs] both the consultant and the nurse, we spoke at length [laughs] when I saw them about me losing weight, and they both started to do what I was doing about Fitbit, measuring your calories, de-de, and they both lost weight, and you could visibly keep going and seeing them and they’d, “Oh, yeah, yeah” and we would chat about the fact, that we’d all been losing weight the same way. And so, you know, the friendship I had with them, the fact that if I’ve, they know that if I’ve asked for an appointment there’s something wrong or I need to know something, they know I’m not, I haven’t just got a cold, or...

Karen wishes she had learnt earlier in life – before vasculitis – how much exercise can improve wellbeing.

And I really am fully convinced that by trying to motivate myself to do forms of exercise that you can improve your wellbeing. Now whether that’s just individually or whether that’s across a certain disease, I wouldn’t know, and certainly I think that’s been repeated through lockdown? As I was shielding, you know, I would know by my watch that if I was out gardening all day and walking about or decorating that I was still burning off the calories. But then there were days obviously in lockdown where it’s a bit dull and damp and you stay indoors, and you don’t walk about so much.

So, at the end of lockdown, I’d put on three kilos. I’m now working to get that off and doing some cycling, and how much better that makes you feel when you lose that bit of weight or when you’re doing that exercise.

So, I think that’s one of the major things I’ve learnt, which I think really in many ways I wish I’d learnt earlier in life that, you know, to do a lot of exercise and to motivate yourself not only makes you feel good, but it also helps you to combat all sorts of illness [nods]. So, I think that sums up sort of where I’ve come from.