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Paul, Christine and Emma

Brief Outline: Paul has X-linked Alport Syndrome and has had three transplants. For Paul and his family, the stability of work and looking forward to holidays have been very important in maintaining normal family life.
Background: Paul works as full time stock manager. He and Christine are married with two children. Emma, their daughter also works full time. Ethnicity: White English.

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Paul and his brother were the first to be diagnosed with Alport Syndrome in their family. Paul was diagnosed at birth. Sadly his brother passed away at the age of 22 from an aneurysm.

Paul has had three kidney transplants to date. He developed kidney failure at the age of 10 and was on hospital based dialysis for approximately 5 years before he received his first transplant, which lasted 5 years. Subsequently, Paul decided to go back on home CAPD, then haemodialysis over a total of 5 years. His second transplant lasted for 14 years, which was followed by another 11 years of haemodialysis. Paul got his third kidney transplant recently, which he nicknamed Dave, following a common practice among transplant patients. Paul has also developed eye problems and hearing loss. The former was solved by a surgery, but hearing loss, Paul says, is very difficult. His first pair of hearing aids was provided by the NHS, but he hardly wore them. He has private ones at the moment, which are of better quality and feel more discrete. However, he is aware that not everyone can afford to have private hearing aids. Therefore he hopes that the NHS would find a way to collaborate with private companies to provide people with more financially feasible solutions.

Being on dialysis for nearly 20 years in total was both mentally and physically taxing for Paul and his family. Yet, Paul is aware that transplantation is not a cure. He follows strict drug regimes, and he has been struggling with various health problems, such as stomach pains that he hopes will cease once his post-transplant medication is adjusted. Paul feels he has witnessed major improvements in drug treatment over the past few decades. Still, he maintains that taking medication is like keeping a fine balance between protecting the transplanted kidney and ‘poisoning’ the body. Paul has full trust in his consultants when it comes to treatment. He also prefers to get his Alport Syndrome-related information from his doctors rather than look on the Internet.

Paul’s wife, Christine believes, “life is still out there… to get on with”. Paul and Christine got married after Paul’s second transplant. They have two children: their son is not affected, but their daughter, Emma, carries the Alport gene. Paul and Christine have always tried to maintain a normal family life. On the other hand, they think their children “should grow up understanding what’s going on”. Emma is fully aware of how Alport Syndrome will affect her in the future, and she intends to consult with a geneticist before she decides about having children. 

Even when he was on dialysis, Paul enjoyed holidays abroad with his family. Such holidays required a lot of planning with regards to arranging health insurance and dialysis sessions at foreign hospitals, all of which were financially demanding. Yet, for Paul it has been crucial to have something to look forward to, as these were the things that “keep you going”. Work has been a very important aspect of dealing with Alport Syndrome for Paul. He considers himself lucky; his employer and colleagues have been fully supportive throughout these years, which made it possible for him to continue working full-time. Paul himself also wanted to keep working, as it provided him with a day-to-day routine as well as a purpose, so he could “pull through” the difficult times.

Paul feels reluctant about peer support. Personally, he prefers to do as little as possible with “hospitals or kidneys”, which, he reflects, is a “defence mechanism… against the whole disease”. He notes: “I don’t want to talk to someone else about kidneys, I want to talk about football or politics, …or other stuff”. Christine agrees: “if you’ve got to fill up sixty per cent of your life with dialysis and hospital appointments, …you want the other forty per cent to be something completely different”. Still, given his experience, Paul feels that he could help others with similar health issues. He makes sure to post positive messages on online peer support forums from time to time. He is also active in fundraising and awareness-raising campaigns.
 

Paul and Christine said they had genetic counselling before starting a family.

Paul and Christine said they had genetic counselling before starting a family.

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Paul: Well we didn't know about the genetic side of it, did we, until after we were married. 

Christine: No.

Paul: Or at least the passing on off the Alport.

Christine: So we went along to see someone in the genetics department up at [name] hospital. And they explained, you know, how it would pass on in our family. Because obviously it's different, depending on whether it's a male or female passing it on. And so once we had the information, we still decided that we still wanted a family. I think in your words, you would have said - and you did say, didn't you - that if Paul could have had his life all over again, would he still have done it? Because you've got to think if you bring children into the world, would you - how do you feel about them perhaps going through a similar situation. And you always said that you would always still want to live your life. It wasn't something - there were good and bad, highs and lows, but all in all he would have wanted to live his life, so. And also, there was a lot of research going on. And they were saying how they were researching into our family genetics. And then, thinking maybe if we had children in the next few years, and by the time they needed to grow up and think about having their families, a lot of research was going on. So hopefully research would have moved everything on, to be more positive, maybe. So we didn't let it stop us having a family.

Paul: No.

Christine: But it's important to go into it, understanding the pros and the cons, and what to expect.
 

Paul, Christine and Emma talk about Emma getting symptoms in the future.

Paul, Christine and Emma talk about Emma getting symptoms in the future.

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Christine: We were always told that probably the first real - on top of having a, you know, a bit of urine and protein in her urine, every year showing. The first real probably problem that's out of Alports would be for Emma that if she fell pregnant, she would probably have a bit of a problem with high blood pressure in the pregnancy.

Emma: Fainting while pregnant.

Christine: So, and obviously the whole bigger issue of having a family, but - while she was pregnant, the high blood pressure.

Emma: And –

Christine: And then the next thing really for Emma would be that probably later on in life, there could be that sort of ten, fifteen percent chance that later on in life she might develop kidney problems. Which has happened in the family, with your Mum and your Mum's sister. That certainly was the case for them. 

How do you feel about that?

Christine: But then, you know, that's - that's part of –

Emma: Well I just kind of accept it. It'll happen or it won't happen. Hopefully I'll have nice children that will look after me [laughing].

Paul: What, like mine who want to look after me and put me in a home, yeah.
 

Paul, Christine and Emma discuss how Emma has ‘positive choices’ when considering family planning in the future.

Paul, Christine and Emma discuss how Emma has ‘positive choices’ when considering family planning in the future.

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Christine: Our daughter has inherited the gene. And so our daughter, Emma, will have that in front of her as far as when she decides to have her family, that either girl or boy for her will have a fifty-fifty chance of inheriting the Alports gene. But we've spoken to genetics about that. Emma's –

Paul: Well, and Emma has, was with us.

Christine: Emma's been, we took Emma when she got into her teens, to get the latest information from them about it, so that she was well informed and well equipped as she was going into her adult sort of life. And now Emma's thinking of getting the latest information again, from the same lady up at genetics, now that she's got a partner. So - but obviously Emma's - Emma's got her head in gear about what the situation is. And she knows that there are certain things that she can do, to try and make the next stage as best as it can be. So, you know –

Paul: Or eradicate Alports from our family.

Christine: - it could be, you know, that –

Paul: And die out with me.

Christine: As, well we spoke about this last time we, we took Emma up there. And hopefully there'll be some more information when she goes soon. But hopefully Emma will get the opportunity to have a type of IVF treatment that will actually be able to pinpoint whether that –

Emma: Egg.

Christine: - embryo, whatever, is carrying the gene. So ideally, will give her the choice to, you know, eradicate Alports down her line. Which is marvellous, because if you –

Emma: - come back.

Christine: - if you take it back to when Paul and I were thinking about having our family, that was the one thing we hoped for, in us deciding we wanted a family, because Paul based it on the fact that he would not - he would have lived his life again, if he had chance, you know? It's had its bad things, but it had good. So we decided to have a family. So but that - we're at the stage now where we're hoping our hopes are gonna be realised. That, you know, Emma will decide to have a family, have some good choices, and be able to go down the positive route. That's really it, isn't it.

Paul: Summed it up perfectly.
 

Paul, Christine and Emma discuss Emma being able to have IVF in the future.

Paul, Christine and Emma discuss Emma being able to have IVF in the future.

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Christine: Emma's been, we took Emma when she got into her teens, to get the latest information from them about it, so that she was well informed and well equipped as she was going into her adult sort of life. And now Emma's thinking of getting the latest information again, from the same lady up at genetics, now that she's got a partner. So - but obviously Emma's - Emma's got her head in gear about what the situation is. And she knows that there are certain things that she can do, to try and make the next stage as best as it can be. So, you know –

Paul: Or eradicate Alports from our family.

Christine: - it could be, you know, that –

Paul: And die out with me.

Christine: As, well we spoke about this last time we, we took Emma up there. And hopefully there'll be some more information when she goes soon. But hopefully Emma will get the opportunity to have a type of IVF treatment that will actually be able to pinpoint whether that –

Emma: Egg.

Christine: - embryo, whatever, is carrying the gene. So ideally, will give her the choice to, you know, eradicate Alports down her line. Which is marvellous, because if you –

Emma:- come back.

Christine: - if you take it back to when Paul and I were thinking about having our family, that was the one thing we hoped for, in us deciding we wanted a family, because Paul based it on the fact that he would not - he would have lived his life again, if he had chance, you know? It's had its bad things, but it had good. So we decided to have a family. So but that - we're at the stage now where we're hoping our hopes are gonna be realised. That, you know, Emma will decide to have a family, have some good choices, and be able to go down the positive route. That's really it, isn't it.

Paul: Summed it up perfectly.

Christine: Did I? Good. Because that's the most we really want, you know?

Paul: [laughing]

Christine: Because when you've got something like a genetic illness disease, that's the biggest guilt trip, isn't it, to - you know. I know probably Paul's mum had, has had that guilt trip and had to live with it, because she's seen one of her sons die, and she's had all the things that Paul's had to endure. So hopefully, you know.

Paul: Well - her sister and her nephew all died from it.

Christine: Yeah. Yeah. But that - it's amazing how in that fairly short amount of time really, how things have moved on.
 

Paul talks about his medication changing over the years depending on what stage he was at.

Paul talks about his medication changing over the years depending on what stage he was at.

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And can you tell me about like medication throughout?

Paul: Yeah. Lots.

What sort of medication you've been on, and?

Christine: [laughing]

Any side effects, and?

Paul: Mmm. Side effects?

Christine: And there is always a side effect. 

Paul: Oh, crikey. I don't know. There's just so many different medicines, depending on what stage I was at. I've been on things to make - fill me up with potassium, because I hadn't enough potassium. When you're on dialysis, you've got to keep off potassium, and you can't have any potassium. So you've got to take that. And phosphate as well, build up your phosphate. When you're on dialysis you've got to keep off phosphate so you have to take phosphate binders to bind the phosphate when you get rid of it.

So it's contradicting the whole time, all the way through. It's .

Christine: Mmm. And that was a - the drugs and that are a constant battle, along with the nutrition. Nutrition, isn't it, and the diet.

Paul: Yeah. What you can and can't eat and drink. And what you, what your drugs are, it's just a constant balancing act. I've had drugs like Minoxidil, which makes your hair grow, which obviously didn't work. [laughing] All sorts of things, haven't we. I'm currently on lots of immunosuppressants for the transplant. Which, the last time I was at clinic, about six weeks ago, they doubled my - one of them. And I think that's what's causing my stomach problems [laughing] at the moment. [dog barks in the background] Which I've got to speak to them the next time I go up about, and hopefully they'll reduce it a little bit. Or at least I think that's what it is, I just - I'm confused now, because there's so many people around with bugs at this time of year as well. But it just seems like it's happened since then.

But I don't know whether that's just a coincidence. 
So it's a case of telling them how I'm feeling, and hopefully they'll look into it and, and do something about it. But yeah.

Christine: Do you think they've changed a lot, since when you were perhaps on your first transplant?

Paul: I'm on completely different drugs that I was ever on, on my first transplant. And even my second transplant.

Christine: A whole new set of drugs, so things have moved on, and.

Paul: Completely different. The first transplant, second one, third one. Completely different. The only one that's stable is the prednisolone steroid, which makes your fat go - makes your fat go - which makes your face go rounded. The kidney face, they call it.

Christine: That's his excuse [laughing].

Paul: It makes you eat a lot. And it gives you a bloated face look.

Which I think some earlier photographs of me and my brother were pretty horrendous in early days, because they - it was, it was a lot of steroids in those days. They don't give you quite so much now. But there was a lot of steroids in those days.
 

Paul and Christine explain how Paul had dialysis in between his three transplants.

Paul and Christine explain how Paul had dialysis in between his three transplants.

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Paul: From that point, I had approximately five years of dialysis at [name] hospital. And then I had a transplant on Christmas Eve, 1980. Christmas Eve 1980? Yes [laughing]. Which lasted five years. It was - it basically just rejected, at the end. The last year or so was all about rejection. Backwards and forwards to the hospital, with lots of steroids trying to keep it going, but it wasn't having it. So, that just went, and I went back onto dialysis. That was in 1985. And I went onto I think I went onto CAPD at that point. Did a couple of years CAPD, but kept on getting peritonitis. At that point I was living in a flat with my brother. Probably wasn't the cleanest of flats for two teenagers to have CAPD in.

Christine: [laughing]

Paul: And so I got peritonitis a few times. In the end they was just getting too bad and too painful, so I went back onto haemodialysis. And then - ah, it's gone. And then my Mum actually then dialysed me at home for a few years, had the machine. The room was already set up, because my brother had previously dialysed at home, for a few years. He'd had a transplant. But then I needed it [laughing]. So the room was turned back into a dialysis room, and I dialysed there for a few years. And then in January 1990?

I had my second transplant. And that lasted fourteen years. Again, it wasn't kind of rejection, it just ran out of steam. Basically what I was told, it just - you know - given up. It wasn't rejecting or anything, it'd just given up. So, back on dialysis again. And I was on dialysis for - I went on, back on dialysis in 2004, July. Just at the start of the school holidays. I remember [laughing]. And I was on dialysis for eleven years, until September 2015. And then I had my third transplant. And fifteen months later, touch wood, it's - it's been up and down, but it's working.

Again, yeah. I was in my - I was 20, to - what was it - 20, well 19 to 24. So I - yeah, I was feeling quite strong I think, before that, I was on dialysis. Again, I was still working. I was still doing things, wasn't I. I was quite active still then. I don't think I was - yeah, I don't think I was - I started going downhill at all dramatically on dialysis then. So I was still quite good.

How did you –

Paul: That's how, that's how I felt anyway.

I mean, again - I feel I do alright on dialysis. But when you actually kind of look back, or people look at you, you've got a completely different perspective on how you actually were on dialysis. I feel I was doing things, and getting on with things.

Christine: Yeah.

Paul: But I probably wasn't.

Christine: No, I think you did very well on dialysis. 

Paul: I think the fact I was at home, I was dialysing at home at that point. And I - we were living in our - we'd bought a house and we were living together at that point. But I was going back to my Mum's to dialyse, three times a week, in the same town. So I'd go back there, and home dialysis is a lot easier to manage than going backwards and forwards to the hospital. Because you can pick and choose your times, and.

Yeah, can you tell me a bit more about that? Yeah.

Paul: Maybe just - you can work all day, go home, set the machine up, and then you do a bit more into the night. Or you can sort of like - if you want a weekend away, a long weekend away, you can take the Friday off from work, dialyse Friday morning. And then come home late Monday, and dialyse late Monday, so –

Christine: It's more flexible, I suppose, more –

Paul: It's just more flexible, you can change things around a little bit more.

Christine: Yeah.

Paul: So, yeah. Yeah, it was - again, it was a case of adapting, just getting on with it.

Christine: And home comforts. Yeah, all the home comforts are around you, aren't they. 

Paul: Yeah. Yeah, the house. I had a nice buzzer that I could pull any time, and it would go in the kitchen, and my Mum would come running up [laughing]. "Just beans on toast, please" [laughing]. Which you could only a do a few times [laughing] before you didn't get anything. So yeah, home dialysis is definitely the better option.
 

Paul and Christine said it was nerve-wracking when Paul did dialysis abroad on their family holidays.

Paul and Christine said it was nerve-wracking when Paul did dialysis abroad on their family holidays.

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Christine: There is more now, but I'm saying at the beginning - you know - to be in that position is one thing. But then to try and do things like holidays abroad, family holidays abroad, etc., are then another humungous task to get up, and. And very, nerve-wracking.

Paul: Very nerve-wracking.

Christine: You know? For all of us, to think about flying somewhere abroad and we've got to dialysis units where there's perhaps not been a lot of English spoken, and things. So, you know, when you think of something like a dialysis session. But we have got –

Paul: It's been fun. It's been good fun, some of it.

Christine: We've, we've done it. We've, we've meet some fabulous people, haven't we really, in the dialysis units abroad. 

Paul: The doctors and nurses, brilliant.

Christine: But it's been, you know, a bit scary at times. Not that anything has gone wrong. Nothing has ever gone wrong. But the whole experience, you sort of have to override it, and enjoy the holiday, you know?

Paul: I think it's just –

Emma: And you have to come off the transplant list.

Christine: Yeah, and you have to come off the transplant list while you're abroad.

Paul: Yeah.

Christine: Because obviously you can't get back.

Paul: But I mean, it's just the build-up to that first session, more than anything. Arriving, and wondering what the unit actually looks like, although you've seen some photographs of the inside, and things like that.

Christine: Mmm.

Paul: And what machines they've got, yeah. But to arrive, and who you're gonna be talking to, what they're gonna be like, and what's the unit gonna be like? Are they gonna be friendly? Can Christine come and sit with me for the first few sessions? And that sort of thing, you know? Just to have someone there, a bit of support. It is very nervous, very nerve-wracking. And particularly when we arrived in Italy, and we were walking towards the dialysis unit, and it's just this old run-down building with this old lady, Italian lady, leaning out the window, wasn't it, with her washing hanging out. And we're like 'oh no, what have we come to?' But we actually went round a corner, and it was quite a modern building, and it was –

Christine: [laughing] Had we got enough, had we got enough time to get back on a plane to England, for another dialysis, for the next dialysis session?

Paul: [laughing] But it was –

Christine: But it's always worked out.

Paul: Yeah. And once we got in there, they were - they were absolutely brilliant. And I mean, nothing against our dialysis units, but the Turkey ones were so much more efficient. Yeah. Yeah.
 

When Paul and Christine got the call which then didn’t go ahead they actually felt very positive leaving the hospital.

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When Paul and Christine got the call which then didn’t go ahead they actually felt very positive leaving the hospital.

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Christine: We had a call, and we went.

Paul: No, we actually had a meeting with the transplant –

Christine: No, I'm talking about the -

Paul: Yeah, I know. But before that, we had a meeting with them. First one said "No chance." Second one said "We'll look at it, we'll have a word with [the doctor]. We'll look at it, and we'll see what we can do." And soon as he took over, I got a -

Christine: It was more positive, wasn't it.

Paul: Yeah. He, he kind of, "No, we'll get something sorted. Don't worry, there will be an opportunity."

So you must have felt quite hopeful that you - 

Paul: Yeah. Well, we just - yeah. Okay, we've told other, you'll probably just - we only met him first time, second time possibly. And we didn't know, didn't know him, really. So we didn't know what to take him from the next person, you know?

So we just got back on with it, and the dialysis. And then probably a year, year and a half later, we got one call. Middle of the night. Rushed up to [the hospital] as quick as we could. Sat there for a long time.

Christine: Hours [laughing].

Paul: It was over a day, wasn't it. Oh, and I had dialysis as well, went on dialysis for a few hours as well, to get ready, get my system all right and everything. And then while I was on dialysis, he came and said the kidney wasn't viable, it wasn't going to go ahead. So although we were disappointed, it had actually given us hope.

We went away from there quite hopeful, didn't we.

Christine: It was - yeah, it was a strange feeling. Because we didn't expect to get that call. And then we did. And you would think that then it not going ahead, the outcome would be - well, we got something we were told we were never gonna get. That was it. That was that chance come, and gone. It's, you know. The chances were thin. So it's just never gonna - that's it, it's gone. That moment has come and gone. It wasn't viable. Let's go back. And we thought that actually would knock you. In my mind, when got told that, I thought 'he's not gonna survive that'. But actually, on the way home -

Paul: Mmm. I was buzzing. Mmm.

Christine: - we, it sort of went in the complete other direction. Because actually, getting the call made us realise that there was always the hope. And the chance.

Paul: You actually are on the list. Do you know what I mean? You don't know that you're on the list. You're told you're on the list, but you've got no real way of knowing that they're actually looking at your name when a kidney becomes available. You've got no way of knowing that. And you can be on it for ten years, like I was, and not even know that - you know - are they looking at my name? Have they just forgotten it? Or have I dropped off the bottom somewhere? Have they forgotten about me? You don't know.
 

Paul and Christine talk about how Alport Syndrome has affected Paul and how he has dealt with it.

Paul and Christine talk about how Alport Syndrome has affected Paul and how he has dealt with it.

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Paul: It is a case of, you know –

Christine: - for you. Yeah.

Paul: - that defence mechanism of - I've got anger against the whole disease. I don't like it, I don't want it, I've never wanted it. I hate it. But I direct that anger by, you know, keeping it away from our life as much as possible. Obviously it has an impact, and it's going to, and sometimes you can't keep it out. But when you can, try and - try and keep it away as much as possible.

That's what I do. That's just my way of dealing with it.

Christine: Well life at home went on pretty much normal. Because it wasn't - to a degree, wasn't under our noses, was it.

Paul: But then the effects of dialysis. I would come home, I know I would be tired. I'd be really hungry. So I'd want something to eat straight away. That's the only time I ever ate, was straight after dialysis. I didn't have an appetite the rest of the time. I'd only eat straight after that. I'd have a big meal, and then I'd go to bed. Because I'd dialyse - I'd leave work about half-two, three o'clock. I'd get to dialysis about four. Get on the machine, do four hours. Probably back home by eight. Have something to eat. Straight to bed. Sleep. Get up in the morning, feel a bit more refreshed. And then go off to work. It kind of - yeah, that was just the way of dealing with it, wasn't it.

Christine: Yeah, I suppose everybody deals with it very differently, and what suits one family might not suit another.

Christine: I mean, there's times when it has got you down, because - you know - it's the nature of the, how it is, but. 

Paul: Yeah –

Christine: Does depend on what sort of person you are, and how you deal with it really, and what - how you deal with it as a family.

Paul: I think the younger you are. Coming from my point of view, I've always lived with it. So I can remember being in hospital at like 5, 6 years old. And I can remember my Mum and Dad going off, and the lifts closing, and - you know - I was there on my own, or with my brother, or whatever. And I had to get on with it, and had to deal with it. And I can remember that many a time, being left in the children's ward at all sorts of different ages. And just seeing your parents walk over the bridge, out towards London Bridge.

Christine: To go home, yeah. Yeah.

Paul: Yeah. And you couldn't. And you had to just turn around and get through the night. Or couple of days, or weeks, or whatever it was you were in. So I think I built up a-. A very hard person, haven't I.

Christine: Well it's made you the person you are, hasn't it. It's made you the person you are, I wouldn't say hard. But you have to deal with things don't you.

Paul: Cold-hearted beep, you would say [Laughing].

Christine: No, no. You have to build up your own defences, don't you, against a horrible type of illness, any type of illness. So, you know, you have to build up barriers to stop yourself getting overcome by it all, or hurt by it all. And that's just - it's just made you who you are, really. In some respects, that can be a really good thing. You know? Out of bad does come good. You know, so it's made you deal with things differently, that's all.

Paul: Yeah. I don't know whether I'd have been stronger if I hadn't have had all the problems, or what. I don't know. But I am - but I do put up a big fight about it. 

Christine: And it was different when you were younger. So if you're a person, you know - if you're young in years, you have got more energy and perhaps you shrug off some things, don't you. But as you get older, it's not so easy, I suppose, if you're going into.

Paul: It's certainly got harder the older I've got, to deal with things. And I think that's a case of you know what's coming. You know what you've got to go through. Like this third transplant was - don't want to put people off having a transplant because I'd do it every time, but it was hell and back. It was, it wasn't good. But you get through it, and you come out the other side. But because I knew what was coming, I was dreading the call. As much as I was hoping for one, I was dreading it at the same time. I knew what I had to go through, what was coming up.
 

Paul and Christine talk about their feelings about going privately and being on the NHS.

Paul and Christine talk about their feelings about going privately and being on the NHS.

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Paul: But that was the frustrating thing as well, because when I actually kind of admitted it to myself, and when I started to do something about it - and I don't like having a pop at the NHS at all because they've been fantastic, and I wouldn't be here without them. But just some things like you go for the hearing, and they say "Yeah right, you need a hearing test." Right, yeah. "Yeah, you need hearing aids." Right, there you go. Boom, boom. "Right, measure up." Boom, boom, boom. There you go. And it was the over the ear ones. And big, pretty big. And I wasn't comfortable with them at all. I hadn't had, been able to talk to anybody about what I'm, what I'm gonna get. What I can get. What options were there, or anything like that. I wasn't able to talk to anyone about that. And it was – 

Christine: All the better options weren't, weren't available, were they.

Paul: It was a case of well, now I'm working full-time still, I'm in meetings a lot at work, I've got to be confident in what I'm saying, I've got to be confident in myself. Now, there's plenty of people that can handle that, and fair play to them. But I can't. I wasn't comfortable with them. And I didn't - I wore them once. And I didn't even wear them outside the house. I wore them once in the house, and they just went to the back of the cupboard. And we then went private, and went to Boots. And we had to pay a couple of grand for them. But I've now got these small ones.

Tiny, aren't they.

Paul: Which just fit in the ear. And I felt better with them. Whether people can see them or not still, I don't know. But I just felt instantly better, putting them in. So from that moment on, I said "Right, I've got to do it." So like I put them in, and I've worn them ever since. But I just wasn't comfortable with these ones at all. But they didn't give you any choice. They didn't even say, "Well you don't have to have these, but we can give you a voucher for £400." That would save the NHS wasting these hearing aids by giving them to you, and you putting them in the back of your cupboard. You have a £400 voucher, you can go and buy private ones if you want. Yeah? That just seems so simple to me.

Paul: You know? The NHS aren't then paying out for all these things that get thrown in the cupboard and never used.

Christine: Yeah.

Paul: I'm actually getting the cost of those, to go ahead and buy something that actually I'm gonna use. And it's gonna make a difference.

Christine: Well it didn't seem as if it was covered. Regarding under sort of your - what can I say? People obviously know, consultants and that know that hearing is part of what can happen. 

Paul: Yeah, they don't. Yeah.

Christine: But it's not dealt with, so - so it's not -

Paul: Not by the consultants either that you see for your transplant. It's not dealt with.

Christine: Yeah. It's - yeah.

Paul: They don't - yeah. They're separate. "That's your hearing, go and see a hearing specialist about that."

Christine: Yeah, it's sort of -

Paul: It's not part of the disease, as far as they're concerned.

Christine:  They don't - it hasn't encompassed as part of the bigger picture of the, of Alports. That's what we felt.

Paul: It is just purely treating the kidney failure.

Christine:  We did speak to some people about - and they said you could apply for some help. Because obviously not everyone can - well not even we could, to be honest - pay out £2,000. And that's not the end of it, obviously –

Paul: I've got to get a new pair soon.

Christine:  - technology has got to move on for hearing –

Paul: And there's no way we can afford another £2,000. I'm in a good job, I earn reasonable money.

Christine: But it's a lot of money.

Paul: But I've still got to pay for my family. I'm not - if I have to pay, I will pay it in the end, because I've got to. But it's kind of like well, hang on - I'm putting so much back into the system, you know, I should be able to get some help towards the cost of these. Which is, you know, down to this disease which I've had to put up with.

Christine: Or just a - yeah, or just that there'd be - it be recognised and, patients be helped with that side of things. And.

Paul: But as soon as you start filling out any forms - are you in full-time employment? Yes. Right, that's the end of the form for you, basically. I'm in full-time employment, I'm not gonna get any support. No matter how much I've struggled to keep in full-time employment over the 32 years, I could quite easily have given up and be claiming benefits, whatever. But I haven't.

Christine: Yeah. It's a difficult one.

Paul: It's - as soon as you tick that full-time employment, you don't get any support at all, or help. And that is just - I'll do it, I'm not gonna start protesting about it all, I'll get on with it and I will pay it, if we can somehow. But, you know, it just makes things even harder to deal with, when you're dealing with everything else.
 

Paul and Christine discuss Paul’s eyesight and whether his cataracts were caused by medication or Alport Syndrome.

Paul and Christine discuss Paul’s eyesight and whether his cataracts were caused by medication or Alport Syndrome.

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Paul: Yeah. Well, I don't - I don't know how, to what extent it affects, Alports affects the eyesight. But we, we didn't look into that any further did we. We was supposed to go up and have some tests at [the hospital], but –

Emma: I didn't have tests on my eyes.

Paul: But it was one of those things we said yeah, we'd be interested in possibly helping with, but we actually never got into it after that. But I had my - I had cataracts removed when I was what? Early twenties? Mid twenties? I can't remember now.

Christine: It was when –

Paul: It was early twenties.

Christine: Yeah, it was.

Paul: It was early twenties. So I had cataracts growing all the time, because of all the steroids I was taking, etc. And Alports, I'm assuming. But I, we don't know, I don't know exact reasons for that. I know why you get the hearing problem, because of the -

Collagen, isn't it.

Christine: Yeah, collagen.

Paul: Yeah, the collagen missing in the kidney and the ear, it's the same one, from the DNA. But I don't know about the eyes. But I had cataracts growing, and I had those taken out. I had lens, artificial lenses put in. And I've actually just had some laser treatment on the left one, because if you have lenses put in at an early age, fibres start growing across them.

Okay.

Paul: So they've had to blast a hole through that, so that it's clear. It was starting to get all blurry, so I went and got that sorted out. And that's, that's another thing. It's just, it's just a hundred and one things you have to deal with, and - you know - as I say, that's why a dentist appointment is just another pain in the backside. More so than for most people. So that's sorted. So my, yeah - I wear glasses. It's like anybody else so, you know, I don't - you know, that's not a problem. I haven't had to, because I had the lenses put in. But that was just another part of it, so. I don't regard that as - I don't know. I never thought of that as being Alports. I thought of that as being the steroids, which was connected to it, I suppose. Which was a kidney problem.

Christine: Some things are connected in a roundabout way, isn't it.

Paul: Yeah. Yeah. Yeah.

Christine: Because it's to do with the treatment maybe, that causes something.

Paul: Yeah. Yeah.

Christine: Or a side effect.
 

Paul says he feels it’s important to have something to fight for and a constant like work.

Paul says he feels it’s important to have something to fight for and a constant like work.

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Paul: Yeah, you've got to have it. You couldn't do it on your own. There's now way. You'd crack up. You'd crack up big time. You have to summat to fight for. You have to have something in your future, ie the kids, and - and the missus [laughing]. And holidays to look forward to.

And you have to have something constant in your background all the time, ie work, to make you get up every day and fight, to keep fighting for. Yeah, you've got to have something to look forward to.

And you have to have something constant in your background all the time, ie work, to make you get up every day and fight, to keep fighting for.

Christine: And you've always had something. You're a big sort of diary person, aren't you. Always have something like a, you know, a month ahead that there's a something to look forward to, and do something. Meal out with a friend, day at the beach, or whatever it is, isn't there.

Paul: Yeah, you've got to have something to look forward to.

Christine: Forward-thinking of nice things.

Paul: If you don't make them, if you feel rubbish or whatever, or you're ill, you find you can't do them. But if you've got them in the diary, you've got something to look forward to, so.
 

Paul says his work have backed him ‘all the way’ through 3 transplants and 17 years of kidney dialysis.

Paul says his work have backed him ‘all the way’ through 3 transplants and 17 years of kidney dialysis.

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Christine: Yeah, you've never - never gone through a period where you've wanted to sit at home and feel sorry for yourself, and be ill. No. You've always worked.

Paul: But that's also because of the support I've had from my employer. I've been with the same person, same company, for 32 years, since school. And they've backed me all the way. And I've been there through two failed transplants, two new transplants. Eighteen, seventeen years of dialysis. And that was leaving work early to go and dialyse at the hospital. When I was on CAPD they provided me with a room with a key, to put my storage in and actually go and dialyse in that. In fact, they actually commented that it's harder to keep me out of the office, [laughing] than get me into it, so.

Christine: So you've been very lucky there, haven't you. Because when we say you've worked full-time, that's probably not easy for a lot of people out there that have gone into renal failure, and on dialysis and things, because if you haven't got that backing and support–

Paul: I totally understand how people can't do it. I totally - my brain set was just get up and go in, whether I felt rubbish or not. It was a case of getting up and doing it, and you'd feel better or you'd be keeping your brain going. Even if I felt rubbish at work, I'd still be there doing it. It's - that, it was kind of a backbone for me. 

Christine: Yeah. Yeah.

Paul: For those years, through everything. It was - it's been there - as much as kidney failure has been my life the whole time, well [supermarket] has been in there, in the background the whole time. They've backed me left, right and centre. There was one instance straight after my third transplant - and it happened with the other two, as well. But two weeks after my first, my third transplant, we got a call from my manager saying "Don't worry about pay, you're on full pay no matter how long it takes to come back." Now, that just - pfft - takes the weight off your mind. 
 

Paul and Christine talk about how they used the company Freedom to arrange dialysis abroad.

Paul and Christine talk about how they used the company Freedom to arrange dialysis abroad.

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And you mentioned the company, Freedom, was it? 

Paul: Yeah.

And so they organise, or help organise -

Christine: They actually do trips for dialysis patients. They go to - I think they go to Greece, and Spain, and places like that, for groups of dialysis patients. It's not really me, or us. But I can understand lots of people taking advantage of that. But then they also help you, I think it's something like £60, or something like that, and they'll sort out the dialysis for you, and book it for you, and all that side of it. So there's –

Christine: They do, yeah. We booked our own flights and hotel, and things, around where the dialysis unit was where we wanted to stay. But they would deal with all the liaising between the two dialysis centres, and get all the right paperwork in place, and make sure that - you know - Paul was fit and healthy, and had all the right –

Paul: Was on the right dialyser, and the right amount of hours, and the right drugs.

Christine: So the right equipment is over there when you get there, etc.

Okay.

Christine: And it's still a stressful thing, isn't it, as you said - going into that first –

Paul: And insurance costs a lot as well [laughing].

Christine: - into that first, yeah.

Paul: Well, that's why I've always - that's why we've always kind of gone for two weeks, knowing that I'll get a one week holiday [laughing]. You go for two weeks, you know, fourteen days - two days travelling, six days on dialysis. You know, you're only left with a week. Less than a week to actually have as a holiday as such.

Christine: Mmm. Yeah.

Paul: Because by the time you've finished dialysis, you know - okay, I could - I sleep on the, by the pool or whatever. But you still don't feel great, and it's still not enjoyable as such.

Christine: Yeah, so you have to sort of be careful what you plan. Well [dog barks in the background] you know your limitations, don't you, and what you can and can't do. Got to take into account the heat and everything, haven't you.

Paul: Well that's a benefit. Because it means you can drink a lot more [laughing].

Christine: Yeah [laugh]. Yeah, it has always been - yeah. Yeah. Forty degrees heat, and you can drink a lot more.

Paul: Yeah I was - yeah. I'm - I was restricted to 500ml a day, fluid, on dialysis. But when you're on holiday, I was drinking whatever I want. Because I was just sweating it off during the day. So I was literally coming off the machine at a certain weight, and I was going back on it, less than that weight.

Christine: Yeah. Yeah.

Paul: Because I was sweating off fluid the whole time. If I didn't drink, I wouldn't keep up with that, so. It was good [laughing].

Christine: You've got to be able to manage that, haven't you. That could be a bit tricky, that.

Paul: And we did feel with the Turkey ones, as well - we went to Turkey one year for two weeks, and then a couple of years later we wanted to go back, but we wanted to go for three weeks.

Christine: Mmm.

Paul: And I phoned them up and we had a discussion [dog barks in the background] and we got –

Christine: Yeah, you can even haggle with dialysis, can't you [laughing].

Paul: We got seven dialysis - we got nine dialysis sessions for the price of seven.

Really?

[All laughing]

Christine: So yeah, you can always haggle. In Turkey you can, yeah.

Paul: We got them down. Cheap as chips. They were going - cheap enough –

[All talking at the same time & laughing]

Emma: But America, no. No. Flat rate.

Paul: Maybe it was just Turkey, I don't know, but.

Christine: But, yeah. 

Paul: They like to haggle.

Christine: Yeah. Got a free handbag thrown in at the end as well.
 

Christine met Paul at work when he was on dialysis so she knew and accepted his illness from the start.

Christine met Paul at work when he was on dialysis so she knew and accepted his illness from the start.

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Christine: So, we both met at work. So we worked at the same place. And as I say, Paul was on CAPD when we met. You used to use that system, didn't you, for dialysis. So, yes. And you - the funny thing is, Paul said if ever you got the transplant [laughing], you would ask me to marry you. And then a little while later –

Paul: Thinking I'd have a few years. Six months later –

Christine: [Laughing] a little while later.

Paul: [Laughing] or it wasn't even that.

Christine: [Laughing] no. Along came the second transplant. So yeah, we got married in '91. In '91. So I've always known Paul with, you know, all the problems that Alports brings. And dialysis, etc. But then you had your transplant. And we had quite a number of years. So, how many years?

Paul: Fourteen. 

Christine: Fourteen years. And that's when we had our family then. So, Emma and [my son] came along in those years, didn't they, so. But we've always had to deal with the Alports, haven't we. And it just becomes - in your family, it just becomes the norm, sort of thing. You know, it's there, and you just - just deal with it, really. It's hard to explain, you know. So many people will - if they say "What is Alports?" And you explain, and dialysis, and things. And they're sort of a bit blown away with all what goes on around it. But when you actually live with it, you just - just get on with it, really. Yeah.
 

Paul says not to give up and to keep fighting.

Paul says not to give up and to keep fighting.

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Paul: Oh. Don't give up. Three things. This is, this is gonna sound really, really corny, I know. But [laughing] three things I've been told. One was by my consultant when I first went onto dialysis, and he said "Don't dialyse to live" - ah, say it the other way around. "Don't live to dialyse, dialyse to live." That was what he always said to me.

Christine: Mmm.

Paul: And, you know, I think a lot of people have taken that on, on that BKPA site. I said it a long time ago, and I hadn't - I hadn't seen it before then, and I see it a lot now. Another one was my Mum, who said, "You know, you've got to work harder than the next person, just to keep up with them." And my Dad said, "Don't let the bastards grind you down" [laughing]. That's my Dad, being blunt as usual. 

Christine: [laughing]

Paul: I kind of live by those. You know, it's all about attitude, I think. Yeah, you can - sometimes you can't control your attitude and you can be depressed, and. Through the bad times on dialysis I was really depressed, in bad places, but. Don't know, it's about fight.

Christine: Mmm.

Paul: You know? It's about digging in and getting on with it. You've got to have something to fight for, though. There's no point, you know - you've got to have something. And I totally understand there's people out there that haven't got any they haven't got a family, or friends, or - like I've got. So I totally feel for them, and I know it's hard to do it on your own.
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