A-Z

Isabella

Age at interview: 68
Age at diagnosis: 66
Brief Outline:

Isabella has eosinophilic granulomatosis with polyangiitis (EGPA), a type of ANCA associated vasculitis previously known as Churg-Strauss syndrome. Isabella would welcome the opportunity to see a vasculitis specialist as she has some unanswered questions.        

Background:

Isabella is a retired local government administrator. She lives on a remote island and is married with two grown-up daughters. Ethnic background: White Scottish

More about me...

Two years ago, Isabella was diagnosed with Churg-Strauss syndrome, a type of ANCA associated vasculitis known now as eosinophilic granulomatosis with polyangiitis (EGPA). Although she has regained a lot of strength, she feels she would benefit from seeing a vasculitis specialist who could answer some of her lingering questions.

Isabella had experienced periods of chest infections, sinusitis, stomach problems and “fleeting” joint pain for 25 years and now suspects vasculitis was “biding its time” in her system. She persevered with work, even when she felt very unwell, but stopped participating in activities such as Scottish country dancing because her health was so unpredictable. She wonders if vasculitis would have had less of an impact on her life if a potential connection between these various symptoms had been considered earlier.

Two years ago, after a winter with more chest infections than usual, Isabella felt increasingly unwell throughout the summer. As symptoms grew to include a floppy foot, peculiar tingling in her arms and legs, and swelling in her hands, Isabella kept returning to her GP surgery and had various blood tests. Although she didn’t realise how ill she was, she was relieved when her GP said she would need to go to hospital.

Living on a remote island with no specialist facilities meant Isabella was transferred by air then road ambulance before being admitted to hospital and then intensive care. She remembers “wonderful” and kind staff who asked lots of questions and did numerous blood tests. She also recalls being very determined to do things for herself in spite of the effort involved in simple things like putting on socks.

With the vasculitis diagnosis came a plan for increased steroids along with cyclophosphamide, a kind of chemotherapy. Isabella was flown home after 14 days but each chemotherapy session and other services also involved a trip in a small plane, which was often cancelled or unpleasant depending on the weather. High dose steroids led to poor sleep and puffiness but in time Isabella’s vasculitis was stabilised with azathioprine, an immunosuppressant.

Nerve damage is a persistent problem for Isabella. When she got home from hospital, she couldn’t believe how much strength she had lost. This affected her ability to walk, chop vegetables, do buttons and shoes, and lift a teapot. She couldn’t feel heat and still gets very cold legs and hands. Exercises from occupational therapists and physiotherapists proved helpful, as did some gadgets. Isabella has always enjoyed the outdoors and feels it made a big difference that she kept doing things like gardening and walking.

Although Isabella can appear all right to anyone who sees her walking up a hill, she still has occasional days of exhaustion that feel like she is covered in “a big blanket.” She remains uncertain about how far to push herself and how much to rest and wonders how much improvement she can expect. As it is such a rare disease, she describes the opportunity to meet and chat with other people who have vasculitis as a “lifeline.”

 

Before Isabella was diagnosed with vasculitis, she’d had “bouts” of chest infections, stomach problems and painful joints for many years.

Before Isabella was diagnosed with vasculitis, she’d had “bouts” of chest infections, stomach problems and painful joints for many years.

SHOW TEXT VERSION
EMBED CODE
PRINT TRANSCRIPT

It’s probably about 25 years ago that I started just all of a sudden, I started getting very, very wheezy and I had never had any of these symptoms in the past, you know, before that. I got very, very wheezy. And it kind of passed and then I went to the doctor’s eventually and I was given inhalers. So, I started using inhalers. And if I took a chest infection it was quite bad sometimes, but in the meantime, if I go back further than that, I actually had, off and on, I had stomach problems, you know, from the age of 20, I suppose. And it was put down as a stomach ulcer. But there were stomach ulcers, that kind of problems in the family, so I don’t know whether that’s got any connection or not. But…

I was getting these chest infections, you know, every so often. Not horrendously bad at that point, but anyway, then I got these awful bouts of very, very, very painful joints, and that’s going back about 20, 21 years ago, probably, or slightly more. But anyway, it was I’d have a pain in a joint, it might just be the joint of my thumb, or it could be my knee, my elbow, my wrist, and it would last for a few days, it would get bright red and be extremely painful and then it would just disappear, but it would be somewhere else. It was fleeting all over my body. And I would have it in my jaw here, or, and I was working at this time, and there would be times where I actually had great difficulty changing gears or, you know, I’d be trying to do it with my other hand.

 

Isabella was “surprised” by how much nerve damage vasculitis caused in her arms and legs.

Isabella was “surprised” by how much nerve damage vasculitis caused in her arms and legs.

SHOW TEXT VERSION
EMBED CODE
PRINT TRANSCRIPT

I will always have nerve damage to my arms and feet, I think. They did say to me that, when I got the nerve conduction tests, that it might heal completely, but I was surprised at how much damage there was in my arms and legs. At that, when I got these tests done, I think I had been on the steroids probably maybe a week or something, and I actually thought that my left leg was perfectly all right then, but they said, “Oh, there’s damage to your left, you know, your left side,” and maybe it was masked a bit by the steroid, I’m not sure.

But it was quite a bit of damage, and they said it might, you know, with time, that it would completely heal, but that really, that not to expect it. I think what he was really saying is, “Don’t expect it.” [laughs] I think that’s really what, and within, if it’s going to completely disappear, the damage, you know, this feeling, I think it would disappear, you know, within two years, so I think I’m probably going to have this forevermore.

I don’t know, it, I find that I can do most things anyway now. I can do nearly everything now, and I’ve got the feeling in my arms, and you know, the pain and the, the feeling and - but it doesn’t actually stop me doing things. So, it’s there, and I think I’ve got so used to it, it’s something I live with. And I think I’ll probably always have to live with it. It’s just yeah.

 

Isabella is determined to have as good a life as possible with vasculitis (“Churg Strauss”).

Isabella is determined to have as good a life as possible with vasculitis (“Churg Strauss”).

SHOW TEXT VERSION
EMBED CODE
PRINT TRANSCRIPT

I think for me, the thing that kept me going was I, it would have been very, very easy to sit back, I think, and, you know, have the vasculitis and the Churg-Strauss get the better of me, but I think from the start I was determined that I would still have a, as good a life as I could possibly have, and get as much strength back as I possibly could. Because all the things I enjoyed doing were outdoor things and yoga and things like that, and I didn’t want to give up that life that I had before.

 

As her vasculitis recovery continues, Isabella is enjoying outdoor exercise and using a fitness monitor.

As her vasculitis recovery continues, Isabella is enjoying outdoor exercise and using a fitness monitor.

SHOW TEXT VERSION
EMBED CODE
PRINT TRANSCRIPT

So yes, my other, my daughter was saying to me, while that, although I’m doing a lot of walking and certain exercise – you know, I move around a lot – I don’t get the heart pumping, so maybe I should do a little more exercise where the heart is pumping. So, I’m going to try that a bit more, because I haven’t, you know.

What do you fancy trying?

[Laughs] I might do a little burst of running and then walking slowly [laughs]. I did that yesterday, and going uphill, I was saying to my husband, “Oh, the fastest my heart is going here is one-o-seven, that’s no use,” because the daughter, who’s super fit, is saying, “You have to be up to a hundred and twenty.” So, the last wee bit getting back to the car, I ran, and it went up to a hundred and forty-seven, so [laughs] maybe I’ll do a bit more of that.

Do you measure – I mean, do you measure things to do with your fitness? I mean, I was just thinking, when you were describing all these exercise sheets and the gardening and the out for an hour every day, I thought, gosh, that must take a huge amount of discipline.

[Sighs] I’m not very disciplined in one way. I’m not the kind of person who does the same thing at the same time every day, you know? Every day, and the same thing. I’ve got a Fitbit so that’s how I was checking my, my heart. But I quite enjoy doing these things. I think that’s the main thing, is that I enjoy doing them. So, I don’t feel that it’s difficult, you know? I like being outside.

 

Isabella likes to help her grown-up daughters, but getting ill with vasculitis meant they were helping her.

Isabella likes to help her grown-up daughters, but getting ill with vasculitis meant they were helping her.

SHOW TEXT VERSION
EMBED CODE
PRINT TRANSCRIPT

But it’s just so peculiar, out of the blue. But actually, getting a name on it was, was good, you know? Because I realised by then, by the time I’d got to hospital, that I must have something pretty serious, right enough. And my daughter kept saying to me afterwards, she said, “You were really ill, you really were ill. You didn’t realise you were, but you were really ill.” [Laughs]. She had her Kindle with her, and she was sitting beside the bed all night long the first night [laughs]. [Inaudible] hobbling about and the next day or two, she was coming and going all the time. You know, she was brilliant actually. And also, I think she was also asking lots of questions, you know. She was asking lots of questions. My other daughter had just had a baby two weeks, three, oh no, three weeks before that. That’s why we were away in July, because she had just had a baby and I went away to help her, and I ended up in [laughs] A&E overnight [laughs]. I remember hoovering the house for her, you know, she had a c-section, and I was hoovering and I was sweating. And, I don’t know, I felt so weak [laughs]. And I was, because she’d just had a baby and I thought, “Oh, I’m here to help her.” You know.

 

Isabella has questions about her vasculitis (“Churg-Strauss”) but finds appointments are short and focused on other things.

Isabella has questions about her vasculitis (“Churg-Strauss”) but finds appointments are short and focused on other things.

SHOW TEXT VERSION
EMBED CODE
PRINT TRANSCRIPT

Aye, it’s funny, and I think it’s maybe more because I do see a respiratory specialist, you know, and I do feel that the vasculitis is my big, the big problem for me. And I do see a rheumatologist, but the appointments are usually fairly short, and you know, we don’t discuss, we don’t discuss my symptoms. It would be good to talk to somebody about my nerve problem. Should I be carrying on even though I have these, find it difficult? Should I be pushing myself or should I be resting myself more? These kinds of things, you know, I would like somebody to answer questions, but you don’t really get that.

Or is there other things that might help? I suppose that’s where maybe the OT did help, back before with that, you know, [inaudible] exercise. But the problem is still there with, with my arms. And occasionally, when I’m in bed, I have got a, you know, when my leg is still a bit tingly, I have got a – and I’ve had to, I’ve stretched my legs and kind of, you know, stretching, do a few stretches. Because my legs are a bit uncomfortable. And also, they are so cold, as well, this is the other problem I have. Especially in the winter, my legs are really cold. And my hands. If I go into the freezer, I really, I have to sometimes put some warm water in the sink and put my arms into the sink to warm them up.

I think the occupational therapists will, will – they do say, “Oh, if there’s anything,” you know, “If you want to talk to us again, get in touch,” but I think that what they will, you know, obviously say is, “This is the exercises you can do to strengthen,” but I’m, I’m thinking from the Churg-Strauss side of it, really, from the other side of it. And yes, and also [sighs] how does, how can it affect long-term, you know? You know, mentally, or, I mean, I think it can affect your brain or, and it can affect your heart or your kidneys and various parts of your body, and just a talk about these things, you know.

 

After reading “horror stories” about vasculitis on the internet, Isabella found a support group “heartening and uplifting.”

After reading “horror stories” about vasculitis on the internet, Isabella found a support group “heartening and uplifting.”

SHOW TEXT VERSION
EMBED CODE
PRINT TRANSCRIPT

Yes. I joined that not terribly long after I was diagnosed because I was looking at lots of different sites that had information about Churg-Strauss and vasculitis. And I read loads and loads of stories from people who had vasculitis and some of them were horror stories. I think after I came home from hospital and reading some of them, it was quite depressing. But then I joined the group, and although I don’t contribute to the group really, on Facebook, I’ve found it really useful because, you know, you read about other people’s experiences and advice they get there. And it was really wonderful to have a bit of a lifeline, I think.

And it was local, you know, more local as well. And also, I went to an event that was on last year, and it was a two-day event, and met a lot of other people who suffered from vasculitis. And that was very, very heartening and uplifting, and some people, you know, that have been through an awful lot in it. A whole variety of different people and talked to a lot of people and it was lovely, actually. You realise – I realised that, you know, some people, you know, have a near normal life and, you know, and you can live with it quite, you know, quite happily. But also, yes. It was gosh, I’m not very good at explaining things [laughs].

It’s great.

[Laughs] Yes, we did lots of different things. We did tai, did we do tai chi? We did tai chi and different talks about sleep problems and, yeah, there was different talks and exercises, and the food was lovely, and it was a beautiful area. And it was all very nice.

 

Isabella would prefer to talk about her symptoms and medication with a specialist in vasculitis.

Isabella would prefer to talk about her symptoms and medication with a specialist in vasculitis.

SHOW TEXT VERSION
EMBED CODE
PRINT TRANSCRIPT

I see a rheumatologist here, locally, it’s about every four months or so. I have been to the respiratory specialist in [large mainland city] last year, and I had an appointment for last month, I think. So, I’ve got a phone appointment with them next week, but when I was seeing them last time, it’s a, it’s the respiratory clinic of a certain doctor, but it’s not necessarily that doctor I see, it’s another doctor and I don’t know if they’re, you know. It might be different ones I see.

But when I mentioned that all about the Churg-Strauss, you know, and that’s maybe why I had the chest infections, you know, maybe it’s a symptom, and he said, “Oh, there’s no connection.” He said, “There’s no connection between them.” I had, think I had said, “There’s a connection with the, you know.” So anyway, he, they usually have a word with the specialist, so I think he had gone off to check on a couple of things and they were taking blood and stuff anyway, and he came back and he did say, “Oh, yeah, there does seem to be a connection between the two,” so [laughs] I think it’s not commonly, you know, known, obviously, that, how connected they are, how very connected they are.

So, I’m, yeah. I would quite like to see a vasculitis specialist, you know, this is something that I don’t know if there’s many of them around or. I see a rheumatologist who sees a lot – most people she sees, obviously, or he/she sees, are going to be, have rheumatoid arthritis or so, and they might have, you know, specialised a bit in the vasculitis, I’m not sure, but it would be, I think, very, very useful for people like me who have a rare disease. And, you know, that you could talk to and they, you know, know all about your symptoms and your, your side-effects of medications and, you know, just to talk more, more, more information from them about the disease. Or even what’s happening in research and that kind of thing as well. That would be very useful.

Previous Page
Next Page