Dean

Dean has Hughes-Stovin syndrome, a very rare type of systemic vasculitis. It has mainly affected his lungs, and he has to take life at a slower pace than before.

Dean was working in a local supermarket and going regularly to the gym when, in 2013, he had repeated chest infections. He says they made him feel freezing cold, and shiver and shake, even when the weather was hot. Although antibiotics brought temporary relief, the cycle always started again. When scans showed blood clots in Dean’s lungs and heart, he was treated with blood thinning medication.

Unfortunately, Dean then coughed up a large amount of blood. This happened several times over a few months, and each time he was rushed to hospital. On the third admission, Dean had to go to intensive care, where he recalls holding his wife’s hand and saying sorry to her. It was a shock to him and his family to realise that he was so seriously ill and that doctors were consulting with colleagues across the country to try to understand what was going on.

In March 2014, Dean was diagnosed with Hughes-Stovin syndrome, a very rare condition that has been considered as a variant of a type of vasculitis called Behçet’s disease. He was told a specialist vasculitis centre would take over his care but that it was a four-hour drive away. Dean remembers absolutely sobbin and being comforted by the doctor. The next few months were a blur of hospital visits and treatment that included stomach injections to thin his blood and drug infusions to suppress his immune system.

Pain, fatigue and the unpredictability of his condition meant that Dean was unable to return to his job, so the family had to tighten their belts. As part of his recovery, doctors at the specialist hospital recommended he try going to the gym again. He was disappointed that none of the gyms he approached were prepared to take the ris until his GP intervened.

In 2017, Dean coughed up blood again. When his local hospital was about to send him home, he says he insisted they get in touch with the specialist hospital, who re-started treatment. Dean describes himself now as sort of stabl on a number of drugs including a blood thinner (rivaroxaban), immunosuppressants (azathioprine and prednisolone), a maintenance antibiotic, and strong painkillers.

Dean trusts the doctors who are most involved in his care and appreciates that he can call the specialist vasculitis nurse directly for support. However, he is frustrated that health information is not easily shared between doctors, hospitals and patients when, within his specialist hospital, doctors can quickly see results of scans and blood tests carried out there, and patients can access their information online.

Dean says it is difficult to get his head round what has happened, because it is so rare. He finds the best way of dealing with it is to live day by day. He plays snooker, mows the lawn, goes to the gym and walks but – due to breathlessness, pain and fatigue – this has to be at his pace. He says he does what he can, then rests to let his body recover.

Dean keeps all his clinic letters to help coordinate care between local staff and vasculitis specialists.

Age at interview 47

Gender Male

Age at diagnosis 40

So did you say that your lung specialist is also in [hospital in other region] or your lung specialist is in [local acute hospital]?

I’ve got both; I’ve got one in [local acute hospital] and one in [hospital in other region].

[Laughs] right.

Okay? I’ve got a vasculitis, [laughs] this one, vasculitis specialist in [hospital in other region] and because – and I don’t know whether it was due to the steroids or not – but I did get osteoporosis, so obviously I’ve got an osteoporosis doctor here, rheumatology specialist, the rheumatology, lung specialist here; lung, vasculitis at [hospital in other region]; and just one GP. I used to have more GPs that understood me illness at my surgery but I’m down to one, the other one retired, so.

I tend to have five, or six, port of calls because I also use the vasculitis nurse, if I’m not sure on anything I ring her and I’ve got a direct number to her, you know, so if I’m thinking, “Hang on, that doesn’t look right,” or there was a doctor looked at me bloods and he says, “Oh, I think you should stop this and I think you should stop that,” and I’m thinking, “I’m sure my bloods have been a lot lower than this,” so I said to him, “Look, can you ring [hospital in other region,” and [they] then, the vasculitis nurse rang me and said, “No, that figure’s good.”

You know, so if a doctor, another doctor who doesn’t know the illness interferes that’s when you get your problems, you know, or, even I’ve had the, the osteoporosis, not the main specialist that normally sees me, somebody else, she’s tried to interfere and I think then she’s gone back to him and he’s said, “No, just leave it. When I see him next, I’ll sort it.” So, it’s when other doctors interfere with the five or six that I tend to use, that’s when I get me problems.

Do you feel that you have to, I mean it must be difficult dealing with all these different people? Do you, how do you deal with that, how do you remember everything that people have said, and?

Do you know what, believe it or not I take every letter with me? I’ve got every single letter, even going back to, even when I was ill in Spain, that’s in me file, my folder here at home. And I just take every letter with me because you can’t remember everything. If you’re trying to sort something out, here, what a specialist has said, say in [hospital in other region], they’ll maybe say, “Well what date have,” you’ve got to have that letter in front of you, so I keep every, I don’t throw anything away, so I can go back seven years of letters, you know.

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When a gym felt Dean’s vasculitis would be too risky, a doctor went “above and beyond” to get him in.

Age at interview 47

Gender Male

Age at diagnosis 40

They wanted me to go to a gym. I tried to get in at a gym, the gyms, every gym I knew, turned me down. Even though I’ve got specialists saying, “He should be fine, don’t push him, he should be fine, just do light workout.” Me GP, “Light workout.” I went to a gym very close to me and I spoke to one of the instructors and I’ve showed her all the letters from the GP, me specialists and what me doctor thought. Well, I told her what me doctor thought; I didn’t have a letter from me doctor. So, on the same day they rang me back and said, “We don’t want you. We don’t want to take the risk.” Okay, that’s fair enough, you know, but what, what am I supposed to do?

But that afternoon I spoke to me doctor, he rang me up just on the off chance to tell me something about me blood tests. Now my bloods have to be obviously lower than everybody else’s because me immune, me white cell count has to be less, so he, if there’s anything comes up, he rings. So, he rang me, just told me what was going on with me bloods, and I then, I just basically said to him, “This gym doesn’t want me.” And he said, “I’ll ring them.” And you’re thinking, “This is a doctor going sort of above and beyond what he should be.” So, he did and got me in believe it or not, [laughs], it sounds strange, but he got me in.

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Dean’s body “won’t allow” him to do as much as fit people. At snooker or the gym, he is often asked why he needs a rest.

Age at interview 47

Gender Male

Age at diagnosis 40

I do something, I’ll, you know, I could bring me jigsaw by me and, you know, do a bit of a jigsaw or I do reading, I’m not sort of just doing nothing, but actually not doing anything physical. Because you just can’t, and if you do something physical you can’t do it the day after. And that’s the thing, people say, “Well why can’t you come?” – even me dad did, you know, “Can you come to the gym tomorrow?” Well, I’ve just done an hour and a half exercise, I can’t do another hour and a half 24 hours later. Me body just won’t allow it and I’ve only got to do so much, and it is the tricky thing of people trying to understand that.

Or if I go to the, me snooker place, where I go, and I’m struggling with me breathing and I say to somebody, “I’ve had a frame, I want a rest.” And you’ve got some of the, the older fellas they’ll say, “Oh, come on, surely you should be able to play another game.” But I look fit and healthy – and I am, compared to what I was when I was at me illest at under ten stone, and now I’m about 13½ – which sounds a big jump, but really at six foot tall you should be around 12, 12½, 13 – because of it all being internal, and there’s probably scarring on me lungs from all the trauma it’s had to cope with, that nobody can understand it.

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As his vasculitis symptoms are unpredictable, Dean feels working in retail is “too much” for his body.

Age at interview 47

Gender Male

Age at diagnosis 40

I couldn’t imagine going to a work in retail trying to pick up a case of beer, or two. I used to be able to pick say two cases of beer up, or two cases of coke in the local [convenience store], you know, to stock the shelves, and what happens if then you start bringing blood up and there’s customers, oh, I just can’t imagine it.

So financially, luckily, it doesn’t matter. I was only working part time and it was just, we can live on what, you know, the wife earns. But support wise it’s very difficult because they see somebody fully, you know, they see me fully, and I look fully fit. How can you explain to somebody that one day you can do this but the following day you can’t?

And they’d sort of said, “But you’ve got two hands.” Well, yeah, could you really sit on a checkout for five hours, you know, just putting stuff through? I don’t know, but then after that are they wanting you to say do three hours on the floor and then is it too much for your body and would they understand?

And I just think right now it’s just better not to try anything rather than try some form of work.

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Dean can’t work due to vasculitis. He would like to know if he is entitled to more financial support.

Age at interview 47

Gender Male

Age at diagnosis 40

I have tried to go to see, you know, the Citizens Advice, but they just said, “Try this PIP,” and then you just hit a brick wall, you know, and you don’t know where to turn. I’m still at that situation, I’d love to know is there anymore, you know, money I could be claiming, should me wife claim in some ways that she’s me carer, because I don’t really go out without her anywhere, so in some ways she’s me carer. But I think when I, we had a quick look into that, she had to give up part time work, she had to be only in part time work. I think. That’s what we read, that was so many years ago, maybe that’s changed. But there’s no way of finding out easy. I don’t think there’s, you know, I’ve looked on websites to try and, and nothing seems to be a straightforward, “You can do this, and you’re entitled to this.”

I mean I’ve, you know, you’ve got a house, you’ve got critical, you know, illness cover. Yeah, if you’ve got cancers, they’ll, they’ll pay, pay out. If you’re one in 50 people in the world, they won’t pay out. So, you still have to pay your mortgage, you know. You just, you just have to tighten your belts and get on with it as best you can. It’s very tricky.

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Dean coughed up blood again. This was a sign that his vasculitis had relapsed, but a hospital doctor wanted to send him home.

Age at interview 47

Gender Male

Age at diagnosis 40

But only a few years ago, again, I was just, I was doing nothing, and I coughed up blood, so you goes back to the doctors, and the doctor, you know, have to go back to the hospital. Now this one doctor, he said, “I’m going to send you home.” And I thought, “I’m not having this, I’m not being sent home, he’s not even checked where this blood’s come from and what’s going on.” You know, especially when I’ve thought, you know, only a few years earlier it, hang on, the start off was a little bit of blood to nearly six pints of blood coming out of me.

So, I sort of forced his hand and said, “No, you, no, I’m stopping in, I’m not going back as an outpatient, you’re going to contact [hospital in other region], you’re contacting this doctor or this doctor, this nurse and you see what they say. It’s not you telling me what you think.”

Embed Print Transcript

Dean keeps all his clinic letters to help coordinate care between local staff and vasculitis specialists.

Age at interview 47

Gender Male

Age at diagnosis 40

So did you say that your lung specialist is also in [hospital in other region] or your lung specialist is in [local acute hospital]?

I’ve got both; I’ve got one in [local acute hospital] and one in [hospital in other region].

[Laughs] right.

Okay? I’ve got a vasculitis, [laughs] this one, vasculitis specialist in [hospital in other region] and because – and I don’t know whether it was due to the steroids or not – but I did get osteoporosis, so obviously I’ve got an osteoporosis doctor here, rheumatology specialist, the rheumatology, lung specialist here; lung, vasculitis at [hospital in other region]; and just one GP. I used to have more GPs that understood me illness at my surgery but I’m down to one, the other one retired, so.

I tend to have five, five, five or six port of calls because I also use the vasculitis nurse, if I’m not sure on anything I ring her and I’ve got a direct number to her, you know, so if I’m thinking, Hang on, that doesn’t look right or there was a doctor looked at me bloods and he says, Oh, I think you should stop this and I think you should stop that and I’m thinking, I’m sure my bloods have been a lot lower than this so I said to him, Look, can you ring [hospital in other region and [they] then, the vasculitis nurse rang me and said, No, that figure’s good

You know, so if a doctor, another doctor who doesn’t know the illness interferes that’s when you get your problems, you know, or, even I’ve had the, the osteoporosis, not the main specialist that normally sees me, somebody else, she’s tried to interfere and I think then she’s gone back to him and he’s said, No, just leave it. When I see him next, I’ll sort it So, it’s when other doctors interfere with the five or six that I tend to use, that’s when I get me problems.

Do you feel that you have to, I mean it must be difficult dealing with all these different people? Do you, how do you deal with that, how do you remember everything that people have said, and?

Do you know what, believe it or not I take ever letter with me? I’ve got every single letter, even going back to, even when I was ill in Spain, that’s in me file, my folder here at home. And I just take every letter with me because you can’t remember everything. If you’re trying to sort something out, here, what a specialist has said, say in [hospital in other region], they’ll maybe say, Well what date have you’ve got to have that letter in front of you, so I keep every, I don’t throw anything away, so I can go back seven years of letters, you know.

Print Transcript

When a gym felt Dean’s vasculitis would be too risky, a doctor went above and beyond to get him in.

Age at interview 47

Gender Male

Age at diagnosis 40

They wanted me to go to a gym. I tried to get in at a gym, the gyms, every gym I knew, turned me down. Even though I’ve got specialists saying, He should be fine, don’t push him, he should be fine, just do light workout Me GP, Light workout I went to a gym very close to me and I spoke to one of the instructors and I’ve showed her all the letters from the GP, me specialists and what me doctor thought. Well, I told her what me doctor thought; I didn’t have a letter from me doctor. So, on the same day they rang me back and said, We don’t want you. We don’t want to take the risk Okay, that’s fair enough, you know, but what, what am I supposed to do?

But that afternoon I spoke to me doctor, he rang me up just on the off chance to tell me something about me blood tests. Now my bloods have to be obviously lower than everybody else’s because me immune, me white cell count has to be less, so he, if there’s anything comes up, he rings. So, he rang me, just told me what was going on with me bloods, and I then, I just basically said to him, This gym doesn’t want me And he said, I’ll ring them And you’re thinking, This is a doctor going sort of above and beyond what he should be So, he did and got me in believe it or not, [laughs], it sounds strange, but he got me in.

Print Transcript

Dean’s body won’t allow him to do as much as fit people. At snooker or the gym, he is often asked why he needs a rest.

Age at interview 47

Gender Male

Age at diagnosis 40

I do something, I’ll, you know, I could bring me jigsaw by me and, you know, do a bit of a jigsaw or I do reading, I’m not sort of just doing nothing, but actually not doing anything physical. Because you just can’t, and if you do something physical you can’t do it the day after. And that’s the thing, people say, Well why can’t you come – even me dad did, you know, Can you come to the gym tomorrow Well, I’ve just done an hour and a half exercise, I can’t do another hour and a half 24 hours later. Me body just won’t allow it and I’ve only got to do so much, and it is the tricky thing of people trying to understand that.

Or if I go to the, me snooker place, where I go, and I’m struggling with me breathing and I say to somebody, I’ve had a frame, I want a rest And you’ve got some of the, the older fellas they’ll say, Oh, come on, surely you should be able to play another game But I look fit and healthy – and I am, compared to what I was when I was at me illest at under ten stone, and now I’m about 13½ – which sounds a big jump, but really at six foot tall you should be around 12, 12½, 13 – because of it all being internal, and there’s probably scarring on me lungs from all the trauma it’s had to cope with, that nobody can understand it.

Print Transcript

As his vasculitis symptoms are unpredictable, Dean feels working in retail is too much for his body.

Age at interview 47

Gender Male

Age at diagnosis 40

I couldn’t imagine going to a work in retail trying to pick up a case of beer, or two. I used to be able to pick say two cases of beer up, or two cases of coke in the local [convenience store], you know, to stock the shelves, and what happens if then you start bringing blood up and there’s customers, oh, I just can’t imagine it.

So financially, luckily, it doesn’t matter. I was only working part time and it was just, we can live on what, you know, the wife earns. But support wise it’s very difficult because they see somebody fully, you know, they see me fully, and I look fully fit. How can you explain to somebody that one day you can do this but the following day you can’t?

And they’d sort of said, But you’ve got two hands Well, yeah, could you really sit on a checkout for five hours, you know, just putting stuff through? I don’t know, but then after that are they wanting you to say do three hours on the floor and then is it too much for your body and would they understand?

And I just think right now it’s just better not to try anything rather than try some form of work.

Print Transcript

Dean coughed up blood again. This was a sign that his vasculitis had relapsed, but a hospital doctor wanted to send him home.

Age at interview 47

Gender Male

Age at diagnosis 40

But only a few years ago, again, I was just, I was doing nothing, and I coughed up blood, so you goes back to the doctors, and the doctor, you know, have to go back to the hospital. Now this one doctor, he said, I’m going to send you home And I thought, I’m not having this, I’m not being sent home, he’s not even checked where this blood’s come from and what’s going on You know, especially when I’ve thought, you know, only a few years earlier it, hang on, the start off was a little bit of blood to nearly six pints of blood coming out of me.

So, I sort of forced his hand and said, No, you, no, I’m stopping in, I’m not going back as an outpatient, you’re going to contact [hospital in other region], you’re contacting this doctor or this doctor, this nurse and you see what they say. it’s not you telling me what you think

Print Transcript

Dean can’t work due to vasculitis. He would like to know if he is entitled to more financial support.

Age at interview 47

Gender Male

Age at diagnosis 40

I have tried to go to see, you know, the Citizens Advice, but they just said, Try this PIP and then you just hit a brick wall, you know, and you don’t know where to turn. I’m still at that situation, I’d love to know is there anymore, you know, money I could be claiming, should me wife claim in some ways that she’s me carer, because I don’t really go out without her anywhere, so in some ways she’s me carer. But I think when I, we had a quick look into that, she had to give up part time work, she had to be only in part time work. I think. That’s what we read, that was so many years ago, maybe that’s changed. But there’s no way of finding out easy. I don’t think there’s, you know, I’ve looked on websites to try and, and nothing seems to be a straightforward, You can do this, and you’re entitled to this

I mean I’ve, you know, you’ve got a house, you’ve got critical, you know, illness cover. Yeah, if you’ve got cancers, they’ll, they’ll pay, pay out. If you’re one in 50 people in the world, they won’t pay out. So, you still have to pay your mortgage, you know. You just, you just have to tighten your belts and get on with it as best you can. it’s very tricky.

Print Transcript