Lynn

Age at interview: 54

Age at diagnosis: 50

Brief Outline:

Lynn has atypical Behçet’s disease (a systemic vasculitis), which has damaged the small blood vessels in her heart. She also has ankylosing spondylitis. Lynn wants people to understand just how hard it is to go through a lengthy “diagnostic odyssey.”

Background:

Lynn is medically retired from her role as an intensive care nurse and now uses her time and experience for Patient and Public Involvement in research. She is married with two grown-up children. Ethnic background: White British.

More about me...

Before vasculitis, Lynn worked as an intensive care nurse. When she needed the NHS herself, she feels it let her down, and this still hurts. Lynn has lost her career and her driving licence and has had to build a new life. Her experience has made her passionate about promoting patient involvement in research and service improvement.

Lynn describes the “emotional angst” of a four year “diagnostic odyssey” and “multidisciplinary merry-go-round” as she sought answers for an unknown problem that meant every day felt “like I was being tortured.” She had always looked after herself and, apart from a history of anxiety and depression which she felt she had managed well, was fit and healthy.

When Lynn was 46, there was a gradual worsening of a stiff pain in her hip, shoulder and lower back, accompanied by weight loss, sweats and fatigue. This affected her ability to move, work, sleep and enjoy life. Her GP identified and treated nutritional deficiencies but there was no improvement. She began to have headaches, visual disturbance and jaw pain, and feared going blind. Lynn also had “bizarre” symptoms such as dizziness, losing the thread of what she was saying, and being unable to open her eyelids in the morning.

As she tried to get help for these multiplying but seemingly unconnected symptoms, Lynn grew increasingly frustrated that no-one seemed “curious” beyond their own specialty or knowledge and that some were suggesting it was “all in my head.” At a time when she had never been so ill, Lynn felt she was having to spend 24 hours a day in a search for answers. With the support of her husband, she travelled across the UK to see specialists, both privately and via the NHS, for second opinions.

Ultimately Lynn was diagnosed with an atypical variant of Behçet’s disease, a type of systemic vasculitis, which tied in with a longer history of mouth ulcers. This was accompanied by another inflammatory condition, ankylosing spondylitis. The vasculitis had also caused microvascular angina (a problem with her heart), and blepharospasm (the problem with her eyelids).

Lynn now receives a biologic drug called infliximab by infusion every six weeks at a day hospital. Although she lives with symptoms on a daily basis, this has given her a quality of life she values. Through her heart condition she also accessed cardiac rehabilitation and support with exercise which helped her “reclaim Lynn.”

Lynn makes a number of suggestions for practical service improvements that will see a “systemic” disease treated as such. These include having care coordinators, better awareness of “red flags” for vasculitis, and the option for patients to see and add things to their medical notes. She also feels that access to psychological support and allied health professionals (such as physio and occupational therapists) would help people find the tools they need to live the best life they can.

Lynn doesn’t always talk about the more “bizarre” symptoms of her vasculitis.

I think you used the word ‘bizarre’, a whole load of bizarre symptoms that you wouldn’t, you wouldn’t even mention. What kind of things have you felt unable to mention at your different appointments?

[Sighs] So I think things like – so I have ongoing sweats and whatever, just weird feelings that come over me, that I just, you know feel a bit dizzy or just don’t, or just don’t feel great. I think it, you know, I can, I can be out, I realise that I come across – well, so my perception of myself is that people think I’m quite articulate, but I can be out in a shop and ordering something and I can see [points to head] the next word that I want to see – that I want to say - but I can’t, [gestures speaking] but I can’t get it out. And I’m there like, looking like a fish out of water, you know? Just, and losing the thread of, I mean, you know, my husband will tell you. You know, I start a conversation with him and then I just kind of, my mind just wanders into this kind of sort of trance-like state and I just don’t, and I just don’t finish it, and whatever.

All of these kind of things that make it, that make it quite, and before I, before I developed the blepharospasm, the really acute onset of it, I had this thing which is, since found out is called apraxia of the eyelids, where in the morning I couldn’t get my eyelids open. And I told a couple of people about that, and their reaction to it was just such that I just didn’t mention it anymore, because, like, they were just looking at me as if to say, you know, what’s, and it’s actually it’s all caught up with the, it was a very early warning symptom that, that something wasn’t – that something wasn’t right, you know? And you know, and it does sound really bizarre, “What do you mean you can’t open your eyelids?” You know, I mean, I literally couldn’t get my, couldn’t get my eyelids open in the, in the mornings, I had no control. No control over them.

Before getting a vasculitis diagnosis, Lynn was devastated to find out that some healthcare professionals didn’t believe her symptoms were real. She went to discuss this with her GP.

[Tearful] And when I reflect on that now, I think what it was and what was so important to me, was that people believed me. That belief in me and my symptoms, because it was really hard for me to believe in them. Because what degree of arrogance did I need to think that I knew better than these medical professions that I’d seen? So, to keep that when the little voice in the back of my head was telling me that, that, you know, “Just say none of this is real, Lynn, just say you have manufactured this.” So that wee voice at the back of my head was there anyway, and to hear that from people, was, was absolutely devastating. And where do you go with that?

So, after that, after the thing with the psychiatrist and my rheumatologist saying that, I went to see my GP and I took my husband with me. And. So my GP gave me 40 minutes that day, and what he said to me was, “Lynn,” he said, “I can think of less than five occasions in my whole career” – and he’d been a GP for about 30 to 40 years, he said, “Where I have said to a patient that their symptoms are psychosomatic and in their head,” and he said, “And I’ve made sure when I’ve said it, that I’ve been right.” He said the problem is very, very difficult to disprove. Because we talk about evidence-based medicine and whatever, don’t we? You don’t, you can’t get evidence, it’s like, people feel that they can say that to you when they, when they run the tests that they have at that moment in time, and think about things and whatever, and they can’t find an explanation of what happens to you, so therefore the default is - and the default may be more in females of a certain age because I was, you know, when all this started I was perimenopausal and then, you know, I was menopausal at that time, you know? So, females of a certain age or just females, that it’s okay to say yes, this is - “Essentially, this is in your head,” without evidence to back that up, or evidence, just, “Our tests are negative, and we can’t explain this,” and that becomes the evidence. And how do you dispute that?

And you know, and I said to my GP at the time, “If there is any way that I could live with my symptoms, I would,” because I’d need to be a masochist to put myself through this, to keep on searching for answers, to go and see people, to go and have these consultations.

As Lynn feels that her vasculitis diagnosis is “hard-won” and “fragile,” she doesn’t take it for granted.

I look at my diagnosis as, it was hard-won. It took a lot of time and, and effort. So that was a, that was other people saying, “Yeah, you know what? This is not all in Lynn’s head, there is something wrong with her,” that meant a lot as well. And, and I think that when your diagnosis is hard-won, when people are saying, “Yes, I think this is, yep, this is what we think you have and we’re prepared to treat you for this, but you know what? You don’t quite meet the diagnostic criteria for it,” and whatever, it feels very, the fear at the back of my mind is that - and I think especially what happened with the locums, when my consultant went on mat-leave, is that my diagnosis was hard-won, and it would be really easy to lose it. That I could go to an appointment and, say my consultant moves on to another hospital or something happens or whatever, that I could go and someone that doesn’t know me, that doesn’t know my history, that doesn’t have the same understanding of this rare disease or whatever, could turn round and say - because this, because I know that this has happened, I know that this has happened to people with vasculitis - and say, “No, that’s not your diagnosis. No, we’re not going to treat you for this anymore.”

And what could I do about that? Apart from kick up the biggest stink and have to go through the whole complaints process again and whatever? And that’s always at the back, at the back of my mind, is that, how, how fragile my diagnosis is, and how it’s in someone else’s gift to bestow it on me. And how that bestowal of the diagnosis affects the treatment and the care that I can access, and how little control or say I have over all of this. And I think when you’ve fought really hard for a diagnosis and whatever, you never take it for granted. Never take it for granted.

Before her vasculitis diagnosis, Lynn – an intensive care nurse - “led myself up the garden path” trying to find out what was wrong because “no one else was doing it.”

This was one of the hardest things I had ever done in my life, to continue the search. And at times it got overwhelming, and I had to draw back from it and I had to not - because it felt that all I was, that the illness and my symptoms had almost become a bit of an obsession, that all I was doing was spending my time researching, looking up things. And I was also aware that I was – that although I was a nurse and so could read research and could understand a lot of the terminology and whatever and had access to resources that I could travel all over the UK and that I could see - I saw quite a few people privately.

It was, so I had lots of, there was lots of positive things enabling me to do that, but I led myself up the garden path so many times because I was doing this with symptoms and thinking, “Is it this or is it that or is it the next thing?” because I shouldn’t have been the person that was doing it, because I didn’t have the type of knowledge to try - it was like, going to see people and various test results that came in and whatever, it was like collecting pieces of a jigsaw that were eventually put together, and then my diagnosis was revealed. And it would have been quicker, it would have been cheaper for both myself and the NHS, I mean, every single part of me was scanned numerous times.

And it was just, it was so wasteful, the fact that I was a highly-trained – you know, in intensive care, where there’s real shortages in appropriately-trained, you know, I was at home on sick leave when I could have been in ITU, caring for people. It was just such a waste, I wasn’t, I wasn’t the right person to do this, but the responsibility had fallen on me because no one else was, was doing it, and I was the person that was asking the questions, that was saying, “Could it be this? Could it be that?” that was trying to think of the next person to see.

A biologic drug gives Lynn a better quality of life with vasculitis but “wasn’t the miracle cure that I was hoping for.”

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.

Lynn tries to keep thoughts about what vasculitis will do next at the back of her head – but new symptoms make her wonder if it is “running amok.”

So, I think it makes it, so when you develop other symptoms, you’re thinking, “Is this something else? Is this another way in which this you know, this disease is just kind of running amok?” And part of me – I think I live every day with this kind of thought at the back of my head: “What’s going to be next? What’s it going to attack next? And if it does it subtly and whatever, how far is that damage going to go before I get symptoms. Or before I realise what’s, what’s happening.” Given that I’ve had ongoing damage and whatever, when on paper, my disease is well-controlled. And that’s, I don’t, and although it’s at the back of my head, I don’t think about it too much because, because you would drive yourself crazy.

With medical retirement for vasculitis, Lynn could contribute to her family’s financial stability.

And also, the impact on my family of these things, because when I was, when my work let me go, they wouldn’t support me for ill-health retirement? They said because I was still undergoing treatment and whatever, that they couldn’t say what was going to happen. So then, luckily, within a year, I kind of looked at this again and I thought, you know, “This is going to be me, I’m not going to be able to get back to work,” so I got in touch with the pensions people and found out that because my work hadn’t supported me at the time, that the only ill-health retirement I could get was tier two, which was, which was the higher tier. So, I got in contact with occupational health and they said because I’d been away from work for six months, that they couldn’t, they couldn’t help me.

So again, I went to my GP, and I said, you know, “I need to, applying for ill-health retirement, could you fill out the forms for me?” And I also said to him, “Would it, would it be helpful if I did you a couple of pages on the practical impact of my disease? The symptoms that I have on a day-to-day basis, what it impacts on what I can and can’t do,” and whatever. And he said, “Yeah, Lynn, you know what, that would be really helpful.” So then, and I said, and obviously I said, you know, “I am more than prepared to pay your fee to do this,” because I knew it would be a lot of work for him. So, I put the forms in, he got them back to me quickly, and you know what? He had photocopied about a hundred pages of clinical letters, had read and understood what I said and had, about what I could and couldn’t do, and used that to, in the points that he was, in the way he’d answered the question, and he’d also numbered the clinical letters to say, “Well, yep, you know, this letter is evidence for the point I’m making here,” and whatever.

So, within two weeks of me submitting that, I had got tier two ill-health retirement, and I just couldn’t have - and again, the fact that he was willing to do that and had done that, just meant – so I bought him a very nice bottle of red wine to say, to say thank you, which I think he was quite touched at as well. But it just, I think for me to go from someone who had, who had earned a wage, who had felt that she was contributing, to nothing, because I knew there was no point in applying for Personal Independence Payment because I wouldn’t, because, because I didn’t meet their criteria. So, to have felt that I was contributing to some form of financial stability for the family going forward, was just so, was so important to me, and for him to do that, to, you know?

Lynn developed a heart problem with vasculitis. Contacting a specialist and sharing the reply with her consultant was “teamwork.”

I talked yesterday about doing the things that I do to manage my, to manage my disease, and everything and it reminded me as well that when I developed microvascular angina and had the specialist angio as part of the clinical trial and then, you know, when they gave me the results there and then and I said, “Well,” you know, “How can you relate this to my underlying autoimmune disease” and they were like, “Well we don’t know” kind of thing, so I [laughs] so I went, I went home and back to good old Doctor Google, you know, kind of Googled, looked at the research that there had been and saw that, it was a vascular rheumatologist that I knew in London had done some research in this so I emailed him. And I got a really good practical reply from him that helped me make sense of what had happened to me and what I needed to do, and whatever, it was absolutely great. So, I printed it off and at the next appointment with my consultant I took it and I gave it to my consultant. And she said, “That’s great Lynn,” she said, “That’s actually really, really helpful” and she acted on like a couple of his, a couple of his recommendations.

So, you know, not everyone knows a vascular rheumatologist down in London, not everyone would be able to kind of Google that and not everyone has a rheumatologist that would be okay with them doing that. But to me that’s teamwork. You know, that’s collaboration, that’s me being part of the team and that’s putting me at the top of the pile. What my needs are, and what I need to, because I needed to understand what was happening to me to come to terms with what had happened to me and to know what I could do to kind of help myself and what to look out for going forward. And I managed to get to the source of information to allow me, to allow me to do that.

For Lynn, when healthcare professionals acknowledge poor care, it’s “a human connection that has got nothing to do with complaints or blaming.”

So, my, the consultant that I had, that I have just now, said to me at one point, he said, “You know, Lynn,” he said “I realise that this couldn’t have been easy and I realise the amount of effort that you had to put into all of this,” and, and whatever. And that was, and when I lost my job, the last meeting I had with the kind of, sort of, the head of surgical services was there, and so the top HR person and whatever, the guy that was sort of head of surgical services said to me, “You know,” he said, “This hospital hasn’t,” he said, “Your care in this hospital hasn’t been great, has it not?” and actually, just to have that acknowledgement meant so much, because I don’t think we acknowledge it. Because I think we’re afraid to acknowledge it, and I think that healthcare professionals don’t like to be seen to criticise other healthcare professionals, but actually just to be in that space, with someone, to, for them to kind of put themselves in your shoes and kind of say, “That must have been really difficult,” [emotional] just that human connection that has got nothing to do with complaints or blaming or finger-wagging or whatever. Just to realise all the baggage that, that you have as a result of that, and what hard work it was and whatever, and we just, we don’t acknowledge that, we just don’t.

And I think, sometimes with the really, really good healthcare professionals and doctors and, and whatever, I think if you’re really good at your job, it’s sometimes difficult to understand just how bad some people are, are at theirs. And how the language that we use and how we make people feel can just, yeah, can just be, can just be awful. And it, and it’s not, it’s not about kind of, “There, there,” or, it’s, like I say, it’s just that very human, just acknowledging that. And it can even just be for a couple of minutes, and then moving on just feels almost like a form of reparation. In a way. And they don’t do it. And I don’t know if that’s a protective mechanism or because their brain just doesn’t work that way, or, because they don’t...

Patient and public involvement in research is very important to Lynn. Being a nurse now “seems like another life.”

So, I don’t tend to tell people anymore that, that I was a nurse and whatever, because it, that seems like another life. It, I think sometimes with illness what you, and I, maybe it depends on the degree to which your illness has affected you and whatever, and maybe this is because I lost my job and had to medically retire, but in a way my life was pre illness and then it’s been post illness and the post illness life has been very different and in many ways I’ve had to build a new life. For myself, which I’ve done.

Probably some of it, so I, you know, I would have said when I was a nurse that I was patient-centred and that I understand, I understood what it was like to be a patient because I looked after patients every day. And it took becoming a patient to make me understand that I didn’t, that I didn’t have a clue. And to know that patient involvement in either research or services or whatever, are, is so very, very important, just to understanding what people go through on that journey.