Jago: My first real memory of having Alport Syndrome was when I was about 11, when I got my first hearing aid. Because I think I was diagnosed far earlier, but I just don’t really remember it, and my parents kind of kept me out of the knowledge of it for quite a while until they couldn’t, when I had my first hearing aid. Which was a little bit of a shock, but it’s – it’s fine. And didn’t get the second one until a few months later.

Wilf: I was given hearing aids at the age of 7. And they didn’t really suspect anything further. So I had hearing aids for quite a few years. And then my brother had hearing aids as well. And by the time I was 10 or 11, they realised that, you know, something wasn’t right. It wasn’t just my hearing. And they kind of linked that to Alport Syndrome. And that’s when I started taking tablets and things, and going to the sessions, and stuff like that.

It was a big change. Like having hearing aids for the first time, it’s really weird. Because like everyone sound like robots. But after like one, one year, or like two years, just don’t realise it any more. But it was, it was a big shock at first, like just suddenly having hearing aids, having these tablets, going to sessions and stuff like that. Yeah.

Jago: Yeah. It was a massive shock, because it suddenly got you a lot of attention. And also it was a, a big change. But as Wilf said, you kind of sort of forget about it after a while because it just becomes second nature, putting it in. You no longer forget about it. And it does – when it’s in, you don’t notice it, you just realise that you can actually hear stuff.

Wilf: Mmm.

Jago: But the hearing loss itself, it wasn’t that much of a shock, because it comes on so slowly.

Wilf: Yeah.

Jago: It doesn’t really affect you that much. Like if you think back, then you remember that you can hear quite a lot without your hearing aids, but it – over a course of a few months, you can’t tell any difference at all.

I have a slight recollection of not hearing things properly when I was about 12 or 13. There are just a couple of things at school that I think if somebody would say something to me or a teacher was saying something and I was completely unaware that I’d been spoken to. And you know, you’d just think, if you’re getting, you get a telling off for not paying attention and all the rest of that. I don’t think I wasn’t but anyway. And so the deafness was diagnosed, the fact that I was deaf was diagnosed around that time, early teens, 14, 13/14. Something around there as I recollect.

Hearing aids in those days were definitely not as well developed as the current digital aids are. And I had I would say absolutely that the deafness during education was a problem for me. No question in my mind that if you cannot hear properly when you are being educated and you’re thinking you can learn things it’s difficult and I’ve no, no question in my mind that something would be less of an issue these days because of the quality of hearing aids. In those days being deaf and seeking to be educated or being in a sociable professional occasions when you can’t hear properly was a problem, absolute problem. And I sadly certain in my own mind that my education suffered because of that.

I do get worried, yeah. But I mean, at the minute the kidney side of it isn’t affecting him, it’s the ears. The hearing. That’s the major problem at the moment. It’s like getting behind at school. And I don’t know, it just seems like people aren’t aware, deaf-aware more than anything. And battling with school to get equipment and things like that. That’s, that’s the biggest problem at the moment. You sort of put the kidney thing to one side, and that’s what I’ve had to concentrate on more. Yeah.

What sort of things did it say then, on the internet?

Well, it said things like that they know males get the worst symptoms. And that they get hearing loss in early teens. Well because mine had got the hearing loss at infant school, I was thinking ‘oh, mine have got a worse strain than everybody else’s, because it said early teens and mine have got it really early, the hearing loss. And then it said by the age of 25 they’d have kidney failure. So I were thinking ‘oh, well because mine got the hearing loss early then they’re bound to get the kidney failure earlier. And it was just things like that that frightened me, because it could have said, “Sometimes you get it early.” Because when I did say that to my consultant, he says, “Oh, it doesn’t necessarily mean that the kidneys are going to get worse, just because the ears have got worse.” So it was things like that. Yeah.

It was honestly, initially it was very, very difficult. Just before I had the hearing aids I found. I was very disruptive in class I think for the two/three/four year lead up. I think this is a, a general pattern with deaf kids where they, they’re not engaged with the class cos they can’t really hear to the level they should be. They’re disruptive. You know I wasn’t a poor student but I think I was definitely compromised. When I found out I had hearing aids it was, it was I think a seamless a transition as it could be. So, we had someone come in and explain them to my eight year old self, you know to have a talk to them, me about how they would impact my lessons, how to use them. It was hard initially but it slowly became apparent that these were just amazing things like that I was suddenly you know my education took on a new light; it was incredibly liberating for me.

Well I don’t have any recollection of it affecting me professionally. I think by the time I got to 18/19 I was working. I certainly had hearing aids. They certainly weren’t perfect but, but they enabled me to function. I’m going to say effectively. And the reason I would say that is once, once you know you’ve got a problem you don’t know when you start to become deaf. If you are becoming deaf. You don’t know. You think this is the norm. Once you know you have deafness then you can actually to some extent manage your situations, where you sit, how you sit, where you face people to be able to at least, I think mitigate some of the effects of deafness. I’m told I’m an extremely good lip reader but nobody has ever taught me to do that but it’s just, it’s certainly. You do find yourself, put yourself in a situation where you face people rather than their back to you or in a meeting make sure you’re sitting in the right place in the meeting to be able to hear most things or if you’re at a conference or a talk to be sitting near the front. All those things you learn to do just to mitigate and it, and it works. By the time I was 18 I was kind of learning how to do that, but I don’t, in a school or education it’s not always possible to do that. And so I definitely think that was more difficult. I don’t have an issue. I don’t have a recollection of it professionally being an issue.

One of the things that is worrying about Alports is the hearing loss. My cousins had some hearing loss, and has had, has to wear, wear a hearing aid now. Because I like music, and like going to gigs and stuff. Like I went to my first gig after I was diagnosed, just a couple of months ago, and I was very conscious about how loud it was, and how I probably shouldn’t be standing right next to the speaker. And kind of like two days after, my ears were just ringing [laugh], so. That’s probably the thing I’m most worried about, I think, hearing loss.

Have you had your hearing tested at all, or?

No, I’ve not had it tested, but I’ve not noticed any differences in hearing loss. When I was talking to [doctor], my partner made a joke about just sort of hearing what I want to hear. And I think he took that on as something to keep track of, so. In my little medical letter that I received, it was like keep an eye out on like hearing loss and stuff like that, but. Well I’ve not been tested, and I’m just assuming that nothing’s changed since I was young, but.

Yeah. I think so. Just because I think that’s in some ways more disabling than what will eventually be kidney disease, because it’s just a sort of loss of communication. I know they can balance it out with hearing aids, but it’s because I consume a lot of media and things like that, it’s – it is what I’m most worried about, yeah.

And you mentioned there that you got a lot of attention. Can you explain a little bit more about that?

Jago: Just peer members in class, just suddenly getting interested in it. Because you’ve never had it before. It’s quite a cool little gadget, so. Some of them might have thought – maybe a little bit jealous, and thinking that you have supersonic hearing.

Wilf: Little recorder things.

Jago: I didn’t

Wilf: Oh. They had like sort of – the teachers put on those neck things. And then like a microphone. That’s a pretty cool gadget.

Is that like a hearing loop?

Wilf: Yeah, a hearing loop. Yeah. But at first when people start asking about it, you feel very

Jago: Slightly self-conscious, yeah.

Wilf: Yeah, self-conscious. But now we’ve got like these new hearing aids, which you can barely see. They help a lot. But after like a couple of years with hearing aids, you – self-conscious isn’t something you take notice to any more. So, yeah.

Jago: They have – well, mine have four different settings. So they have just a general setting for like a younger generation, that picks up a lot of everything around you. And then my second setting is for a slightly older generation, where it’s picking up everything in a closer proximity. My third one is for picking up Bluetooth devices, like headphones, or my phone when I’m making a call, so I don’t have to have it on loudspeaker. Just so much more helpful, and a lot more private.

Wilf: Really cool gadget.

Jago: And then fourth one is a loop system, so if there’s a loop system on a speaker at like a public talk, a festival, or someone has one of those neck loops, then it comes straight into the ear.

I’ve got a hearing dog. Todd. I had him nine years ago. It’s a hearing dog, registered Hearing Dog For Deaf People. And he’s really changed my life. You know? He, he – well, works to sound in the house, basically. The doorbell, the phone, smoke alarm, alarm clock, cooker timer. He comes and alerts me. He’s a little Cavalier. And he’s, he’s sitting on my feet here. He’s probably snoring away actually, but I can’t hear him.

And he comes to find me when he hears the doorbell go, for example. And he nudges me with his little stub nose. And I say “What is it, Todd?” And he takes me to the sound. And we also fund-raise with him. Which is lovely. You know, people talk to me, which I like a lot. You know? And also people – you know – ask me questions about him.

Which is lovely. And he’s such fun, you know? So I recommend anybody that’s deaf, get a hearing dog.

Yeah. Because being deaf is very isolating. Very isolating. I’ve never really been able to cope with it. I join groups and I tend to drop out after a while because I can’t cope with feeling isolated, because people don’t talk to you. You know? Very sensitive maybe, I don’t know. But I often go to the loo and I cry. That’s pathetic really, but that’s how it makes you feel. But with Todd, I can talk to him instead, can’t I [laughing].

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.