Kevin

Age at interview: 42

Age at diagnosis: 6

Brief Outline:

Kevin was diagnosed with X-linked Alport Syndrome as a child. He developed hearing loss and some eye-sight problems, but neither posed him many challenges in his daily life. He had his kidney transplant after 4 years of being on home dialysis.

Background:

Kevin is married and works as a full time college teacher. Ethnicity: White and Caribbean.

More about me...

Both Kevin and his older brother have known that they had X-linked Alport Syndrome since they were children. As Kevin’s brother was first to develop Alport-related issues, Kevin had first-hand knowledge of how his own Alport Syndrome might develop. Kevin developed hearing loss when he was 7, but he didn’t experience any difficulties at school after he received his hearing aids. He also had Alport-related eye issues, but he hasn’t had any difficulties since he had surgery to replace his lenses.
The topic of Alport Syndrome wasn’t “a big deal” in Kevin’s family though, and dealing with the condition felt like a normal part of daily life. They never discussed it much until later when the condition started to have a more significant impact on their lives. Even then, Kevin feels that their conversations never revolved around Alport Syndrome, as “it was quite boring to talk about it a lot”.

Kevin’s kidneys started to decline when he was 22. He remembers feeling constantly nauseous, which he put down to his lifestyle initially. He developed kidney failure a few years later, and started CAPD dialysis when he was 26 years old. His dialysis schedule soon became part of his normal life, but he was too sick to keep his full-time job. What followed was a period of financial difficulties: because of a 6 monthdelay in receiving benefits, he had no income for half a year. 

Kevin found the dialysis most difficult. He describes it as “a big pause in your life” and “you’re just kind of waiting for the next chapter”. However, he made an effort to do things he enjoyed, like going rock climbing with his friends because “you can’t just switch your life off”. He received a transplant after four years of being on the waiting list, but it took him a few years until he fully recovered. He continues taking medication for his kidney, 15 tablets a day in total. Admittedly, he is not particularly worried about finding out what each individual tablet does because he has full trust in his doctors. Also, since he works as an IT teacher, he is also wary of looking up Alport Syndrome on the internet.

Only recently Kevin contacted Alport UK, a national charity and support group, because he recognised that he could help newly diagnosed people by talking to them. However, in the past he never felt that being part of a support group would benefit him personally.

Kevin’s message to other people with Alport Syndrome is that “it’s not as bad as you think”. He would advise them to trust their doctors.

Kevin talks about his brother going through treatment first and that this was part of his ‘normality’.

We didn't talk about it much, like I say, because there wasn't much to talk about. But as we've got older that had a real big impact on me, in a positive way, because - unfortunately for him, but because he was older, he'd go through everything first. So he was on dialysis about four years before I was. And so every procedure that he had, I was very likely to have. But I'd already seen him go through it. So I was quite clear about what I was going to be having, and what it was going to be like, and what the options were going to be. So I think that really helped me, because I didn't have to give it that much thought, because it was kind of presented to me with somebody else doing it first. So we talked about it at that stage. So I remember when my brother went on dialysis in the first place, he went on the CAPD as well. He went on a slightly different version, he was on the overnight one. You know, whereas I went onto - doing it four times a day. Yeah. But yeah, that was - that was when we started to talk about it much more, because it was then having a real significant impact on his and then my life. So, so yeah, that was quite different for us. And it was the same thing - he had a transplant about three years before me. So then I saw what happened when you have a transplant, and what the procedure is, and how that all works. So, so yeah, so it was - I was kind of - I feel at least kind of fortunate that it was still part of my normality, because I'd just seen it all happen a few years in advance before it happened to me. So there was none of it that came as a big shock, or was like particularly surprising. The day to day aspects, we did talk about it a bit, about how it makes you feel and so on. But  there's only so much - it was quite boring to talk about it a lot [laughing]. But it was handy to have to someone that understood exactly what, what it was like to go through it. But , but again, as I say, it's not something that we've spent a great deal of time talking about. It would come up occasionally in conversation, but it was just part of normal life, so we talked about other things, you know, what was on the TV or anything else really.

After missing some appointments, Kevin started going to his clinic again for regular check-ups, which led to conversations about dialysis.

I missed. I just kind of went off the radar medically a little bit, I think. Wasn't something that was planned, but I think it's like most of teenagers, early twenties - you think you're indestructible and it won't happen to you, it always happens to somebody else. And so I just kind of - I think it was when I as about 18, 19. In fact I remember, I'd started university when I was 19, and I think I'd got one appointment that I was either late for or couldn't make. And after that I don't remember having an appointment for about four years. I'm sure I was getting them through the post, but as a student I was moving quite frequently as well, so the, the unit wouldn't have had my most up to date address. And I'm pretty sure that what would have happened is that my brother then started to deteriorate. And being three and half years older than me, I probably at that point thought 'yeah, I probably need to get back in touch with the unit and start getting checked again'. And so I did. And I had a couple of periods of ill health. Think the first one was when I was around 22. I got really chronically exhausted. I was working a lot at the time, but I ended up taking about four months off work. Because I just couldn't recover as quickly as I would have normally done. And that's when I really went back to the unit. And it was at that point that somebody at the unit mentioned that, you know, at some point on the horizon I would be looking at dialysis. And as I recall it, then I was quite just annoyed by that. Because it wasn't something that I'd given a lot of thought to. And then I got better, and I was okay for another few years. But I was being checked fairly regularly at that point. And I do remember, as I mentioned before, that I was sort of being nauseous and vomiting a lot in the mornings. But I was single at the time, so I didn't have anybody to tell me that that's not normal. Because, you know, I was having quite a hectic lifestyle at the time. I just put it down to that. And then a few, about a year later , I was with my girlfriend - now my wife - and she was, you know, taking me into work and I asked her to just pull the car over while I was sick. And she was quite perturbed by that, and said, "That's not normal, you need to tell somebody about that." So I mentioned it to my dialysis unit, got - had some more checks. And then they said, "No it's, it's heading that way now, so we need to start thinking about dialysis."

Kevin feels more in control of his health through looking up his test results on Renal Patient View.

Renal Patient View. And it's, basically it's linked in to the hospital's blood results. So as soon as my results are available, they're posted on the site. And it's linked to the hospital's website. So usually I've seen my results before my doctors [laughing]. But what it's done over the years has allowed me - for example, if I've had some checks because I'm feeling unwell, I can see my results. And several times I've seen my results and been able to ring my specialist and say "I don't feel well, I've had my bloods done, this is what's there." So rather than having to wait for an appointment, I can talk to somebody over the phone. And he might say, "Well okay, increase this medication or decrease this. And then we'll see you in a week's time and we'll see what's happened." So they can take action much more quickly. And it does make me feel much more in control of what's going on. So in the old days I'd have a blood test and I'd have to wait a fortnight to see a specialist, to tell me what those results were. Now, I can have my - I can have my bloods done on Tuesday, and get my results on Wednesday night. And that's really helpful actually. It makes me feel that I'm in charge of my results, not just somebody else.

Kevin says that Alport Syndrome didn’t affect his decision not to have children.

It didn't affect my decision to have children or not. I, in the end I decided not to have children. I've got a stepson, which is more than enough. But no, I don't think it had any impact on my decision either way. I think - I might be wrong about this, but I think because it's X-linked, it would be very unlikely that if I had a child that the child would have Alports anyway*. But yeah, it wasn't really a concern, there were bigger factors. I think possibly the fact that I was on dialysis for most of the early part of my marriage might have had some impact. When I think about it actually, it wasn't the fact that it was dialysis, it was the fact that knackered [laugh] with the idea of introducing another child into that would be just too much. But it's not something I feel that I've missed out on. And it wasn't something where I thought 'I want to do it, but I can't'. Just wasn't something that came up really, so. No, it wasn't - wasn't an issue.

*A father with X-linked Alport Syndrome can pass AS to any daughters.

Kevin explains his medication after transplant and how this is constantly adjusted.

I mean you're always on a lot of meds once you've had a transplant. So a lot of what was going on was trying to adjust the medication to make the kidney work effectively without making me feel too sick at the same time. So yeah, so there was a constant adjustment with the meds. I was on quite a high steroid dose, dose for quite a long time as well, which gave me some of the minor side effects, but nothing too concerning. I started sprouting hairs where nobody would normally sprout hairs. Not on my head, obviously, that didn't work. But yeah, other than that it was really just a case of constantly adjusting the medication. And that still goes on, I mean, we're still trying to adapt my meds now. The latest one is that my calcium is too high. Which, it gives them cause for concern. But I have to take calcium tablets, to - as a phosphate binder. So it's trying to balance the binding phosphate, but not overdoing it on the calcium. So that's - you know - that's what we're working on at the moment. But as far as I'm concerned it's, these are small potatoes really. This is, this is minor adjustments to something that's working quite well. 

So, yeah. But we did have a bit of an issue couple of months ago, because they stopped my calcium altogether, to see what would happen with that. And what happened with that wasn't great, because I suddenly couldn't feel my hands and feet particularly well, and I was really ill. So we sorted that out, and I'm alright now, but yeah. I mean, you get little blips from time to time. But yeah, there's a lot of meds. But again, I don't really think about that. That's my new routine. So whereas I used to have to dialysis four times a day, now I just take some tablets twice a day. It's not that big a deal. It's just some tablets.

And how many do you take? So you say twice a day?

I take, in total - think there's - at the moment, I take about fifteen tablets in a morning. And five at night. But I just take them all at once, so it - it looks more dramatic than it is. It's just a big handful and a glass of water, and that's it.

Kevin was sick most mornings which was a sign his kidneys were failing.

Essentially, the - because the kidneys were, had very little function at that point, my body was just basically [laugh] having a really sort of bad reaction. So yeah, I was, I was sick most mornings. And yeah, I couldn't really get going in a morning until I'd sort of vomited the contents of my stomach out. Which, yeah looking back was pretty unpleasant. But it's strange what you get used to. So yeah, you just kind of - you think it's normal until somebody really points it out and says that's not normal [laughing]. So yeah, so that was the first thing I remember being - and the reason I was doing that is because I wasn't sleeping particularly well. And, and so the toxicity was, was quite high at that point. And my kidneys just weren't processing what they needed to do. So it had to react some way, and unfortunately that was the way I was reacting.

Kevin used to go rock climbing with friends during dialysis and was glad they weren’t ‘overly sympathetic’.

I tried to do different things. I mean, I would - I would - When I look back, I was still trying to do some activities. So I used to go - I was still rock-climbing [laughing]. You know - mental, when I think about it, but. I'd taken up rock-climbing with some friends of mine in my early twenties. And I was determined not to stop. So I couldn't, I couldn't forward-plan, so I had a couple of friends who would go rock-climbing. I'd aim to go with them. Sometimes I could, sometimes I couldn't, depended how I felt. But when I look back, I was really grateful, because my friends weren't overly sympathetic. My friends would just take the mickey. Which was far better than having lots of hand-wringing and people, you know, overly sympathetic. So yeah, so my friends would just rib me about it. I remember [laugh], I remember once I'd go halfway up a cliff face, I felt nauseous. My friends were just laughing. It was like, "Oh, here he goes again." Which was exactly what I needed. Because I just needed people to be normal. And yeah, you don't want - well, I didn't want people being overly concerned or, you know, worrying about me. I just wanted my normal life. 

And so what I'd do would be, I would dialyse before we set off. I'd tape all my catheter up, to make sure it was pretty stable. And then - we'd go rock-climbing quite locally. We wouldn't go too far. And if we were gonna go out for the day, I'd just take a, like a dialysis fluid with me, and then stick it on the roof of the car. I've never told my nurse this, because she would have freaked. But just stick the dialysis bag on the top of the car. Dialyse cold. Usually you warm dialysis fluid up, so it's like body temperature before you put it in. But it's cold when you put it in and you haven't warmed it up, you do know it. But it was that, or stay at home. So I wasn't gonna stay at home. So yeah, just dialyse in the car, and then go and do some more climbing, and then go home.

Kevin is aware his transplant won’t last forever but feels his work will fit around his future.

Yeah I mean, I suppose - right now, I feel okay about the future. But I am aware that transplants don't last forever. I was only in my early thirties when I had the transplant, so it's not gonna last me the rest of my life. So at some point, I'm either gonna end up back on dialysis, or I'm gonna have another transplant, or both. I have no idea when that will be. It might be next year, it might be ten years, twenty - I don't know. So I can't try working around that. So I'm just getting on with my life. And one of the reasons, if I'm honest - well, not one of the reasons, one of the minor reasons for me doing the teacher training is that I was aware that it was probably a job I could do if I was dialysing. I might only be able to do it part-time, but I was conscious that when I went into teaching that this would be something that I might be able to continue with if my health does deteriorate. And that's important, because like I say, I didn't have anything that I could do last time, and I don't want to be in that situation again. I've had some ups and downs with my health when I first went into teaching - I was only teaching two hours a week when I started, just kind of the interim. But because of that, I also know that if I need to take a step back a little bit, then I can at least carry on teaching. So yeah, so that's one of the things I've been thinking about for my, my own future. But the rest of it? I'll just deal with it when it arrives. Can't give it too much thought because it's not here yet. And we'll just deal with it when we have to.

Kevin was told he had early onset cataracts but is unclear if cataracts occur as the result of having Alport Syndrome.

More recently I've had some issues with my eyesight, which is linked to the Alports as well. So that's been - again, so I think of the eyes separately, as separate, and the kidneys as something else as well. I had the lens replacements done on my eyes, so I've effectively - I've had cataracts starting. I think that's partly because of the medication we have to take, after the transplant. You know, which can make that accelerate. So last year I had both my lenses replaced. And that's - again, I didn't realise it at the time, but that's had a huge impact on making things so much easier. I mean, previously I think I was a minus ten and a minus eleven, my eyes. And I had sort of really heavy glasses [laughing]. Whereas now I'm - I think minus one, and one and a half, so. You know, I can see without my glasses, which was quite strange, having gone all those years not being able to see much at all. But yeah, so that was linked to the Alports as well, so.

Ah, okay. So yeah, with your eyesight issues then, did that then start - what age were you, and when did that start? Could you tell me a little bit about that?

I started with some form of short-sightedness when I was around sort of 14, 15. Whether that was due to Alports, or just getting a bit short-sighted, I've no idea. But the problems really started - I say problems, the issues started after I'd had my transplant. I think maybe two or three years in. Every time I got new glasses, within three or four months they weren't working effectively because the back of my eyes were changing quite frequently. So I was having to - because obviously you have to do it privately, so I was paying quite a lot of money out every year for, you know, a new prescription probably every six months. And every time the eyes were more and more and more short-sighted. So yeah, it because a bit of a, a bit difficult because I couldn't always do - I couldn't always see what I wanted to see [laughing], for a start. And it started - when it really became problematic was when I was driving at night, I was getting quite a lot of dazzling from oncoming headlights. So I mentioned that to my optician, and he referred me then to the specialist at the hospital. I went there, and he said "Yeah, you've got sort of an early onset cataracts, so we'll get those taken off and put new shiny lenses in." And that's what they did, and it's been great [laughing]. It's really made a huge difference.

And how did you know that that was linked to the Alports? Was that then - did the specialist say that, or?

Yeah, the specialist mentioned - you have to be - I can't remember if he said it was linked to the Alports, or the fact that I'd had the transplant. I'm not quite sure which of the two it was. But I do know that every time I went to see the specialist, he called all his team in to have a look. Because I know that the lenses are cone-shaped, in patients with Alports. Rather than just the normal shape. So, so that's quite unusual. And I do remember him saying that the operation would be a little trickier because of that. So normally the cataract operation takes sort of ten, twenty minutes - you know - for one eye. Mine took about an hour and a half. Because they have to do it much more slowly, and the technique is slightly different, I think. But I know my surgeon just found it quite interesting, it was a different procedure for a change. It was no more difficult, it just took a bit longer. But yeah, it's worked fantastically well.

Kevin describes his cataracts operation and how positive he feels about the operation.

I mean, before the operation my - I mean, obviously - there's obviously a small risk with the op, same as there is for having a cataract, I think it's about two percent chance that you might have a significant problem afterwards. So I was, I wasn't particularly concerned about that. I mean, people play, play the lottery with much worse odds than that. So, so yeah. So I wasn't particularly concerned about it, but I've always been quite squeamish about my eyes. Anything else, you can cut, slice and do what you like. But I've always been really squeamish about it, couldn't put contact lenses in. I tried those when I was I think about 20, couldn't bear it, I had to - you know - I couldn't even let the optician put the second one in, I had to have that one taken out and that was it. Couldn't do that anymore. So I wasn't looking forward to the operation at all. And I did feel slightly ridiculous, because when I went to the waiting room for the operation - because there's lots of people having the operation on the same day, but they're mostly sort of nice old ladies in their sort of seventies. And they'd already had one operation, they were waiting for the second. And they said, "Oh, it's no big deal." So I did feel a bit silly, that I'm a sort of bloke in my early forties, sort of worried about this operation, and all these lovely old ladies are telling me they've just had it and its fine. But the operation itself was incredibly straight forward. Sort of few drops in the eye, you can't feel anything. My, I suppose my big fear - I thought I'd be able to see somebody sort of coming towards me with scalpels and things. You don't see anything like that at all. They shine a bright light in your eye, you're gowned - obviously you can't see anything out of your other eye anyway. They shine a big white light in your eye, so all you can see is white. And you can hear them talking, and they talk you through it and it's really nice, and its fine, it's calm. And then sooner than you realise, they say, "Okay, that's it, it's done." And then you just wear a patch on your eye. Take it off the next day, and you can see. It's - what you can see is a bit wonky for a few days, but once that calms down, it's amazing the difference. Because I can get away with not wearing glasses at all now. The only reason I wear them is because once you've had the cataract operation, you have fixed vision, you can't focus, you can't change focus, so your eyesight at fixed at distance for focus. So I need for close reading. So I wear varifocals now, just to make it more convenient. But I could just wear reading glasses when I need them. But because I'm teaching, I'd be forever taking glasses on and off, which I was doing for a few weeks. I thought 'this is ridiculous', so I got varifocals to make it easier. But yeah, I mean. I go canoeing a couple of times a year, up in Scotland. So I'm particularly looking forward to going this year, because I won't need to wear glasses, which always get covered in water and which you then can't see anyway, so. So yeah, things like that will make a big difference.

So it wasn't as scary as you thought, the operation?

No, not all. I felt ridiculous after the first operation. I just thought 'I can't believe I was dreading that, it was so easy'. So when I went for the second one, a couple of months later, that was just no big deal at all. I didn't give it a thought, because I knew it was really straight forward. So yes, it was surprising, because I was - I was more concerned about having the first cataract done than I was about my transplant. Because obviously when I had the transplant, I knew all about that, I knew I wouldn't be awake while they were doing it, so it didn't really bother me. But yeah, I wasn't looking forward to the first eye operation at all. But it turned out it was really easy.

Yeah, it was fine. And you said you had a second operation? So, what - they do one eye first and then?

Yeah, they do one eye at a time. Just because for about three or four days afterwards, your eyesight's really off. So if they did both, you'd be blind [laughing]. So, so they tend to do one at a time. And I guess also if you have some bad reaction, then you've at least got one eye that's still working.

Kevin felt his life was ‘paused’ and he got quite depressed when he was out of work.

And so I then realised actually all the things that I'd been feeling are because of the deteriorating kidneys. And so I'd already stopped working. I was half-heartedly trying to find another job, but I knew really that I wasn't gonna be able to manage it. But I didn't - I hadn't settled myself with the idea that I wasn't gonna work. I think part of me knew that I wasn't really up to it, but another part of me thought 'well that's not an option, you've got to work, everybody works'. But once I started on dialysis, I think I just kinda settled with the idea that I'm just gonna have to manage this now as it is. So it was the financial issue that was much bigger than the health issue, for me. Because I'd gone from - you know - I'd always been independent, always had my own house. And always, you know, had the lifestyle that I wanted. And suddenly yeah, I had a lot less money to manage on. And that was a much bigger deal than - at the time, anyway - than the primary health issue. So that took some getting used to, more than anything else, the adjusting your lifestyle to a more meagre income was - yeah, that was a much bigger deal.

I mean, I felt that - you feel almost like arrested development. You just feel you're just kind of paused. And that, you know, you're not really functioning on any significant level, until - you're just kind of waiting for the next chapter, if you will, this is just a big pause in your life. But yeah, I mean, I did find it really stressful, just not being able to, to use my brain to do anything useful. And also not feeling a bit - even if the opportunity had been there, not feeling that I could do it. Because I was just so tired all the time. Yeah. So, you know, most of the time it was okay but yeah, there were some - there some times when it was quite, you know, quite depressing.

Kevin explains how he managed financially the first six months of doing dialysis.

So how did you manage, financially, then? So there was a point when you just said - you had to sort of say I can't work anymore, and -

Yeah. It didn't go very well for the first six months. I initially tried to claim - I can't even remember what benefits they were at the time, but... I effectively tried to claim sickness benefits and so on at the time, and was unsuccessful because there was a two week gap in my National Insurance contributions from leaving one job to starting another. And this kind of went round in circles, and I just had no, no income at all for six months. And then I mentioned it to my Mum, who still lives in London, who went apoplectic. Went down to the Houses Of Parliament, went to see my MP in his office and about six days later, a cheque arrived for six months back pay for my benefits. And it was all fine from thereon in. But without having somebody else to do that for me - usually, previously I'd have done that myself. You know, I'd have been banging on doors. But I just wasn't up to it at that point. So yeah, so I'm always eternally grateful that my Mum doesn't take things lying down. So after that, I then had some income at least. And I stayed living, I was living with a friend. And then I got a place of my own, for about six months. But I [laugh], my wife will now say that I moved in by stealth - she was living a couple of miles away, and we realised about, think it was - some point in our relationship, that I hadn't been home for about three months. We said well it seems I live here, then. So I got rid of my other place, and we started living together. Which made it much easier financially, because there was two incomes paying for one rent, rather than one each. So yeah, so from then on it was a little easier. But yeah, I do remember those first six months being really stressful. Because I was ill anyway, and then I think the worry about just having no income. So I was living on what savings I had from when I had been working, but they very quickly got depleted.