Richard Y
Richard Y was diagnosed with X-linked Alport Syndrome aged 23. He has had no hearing loss. Ten years later after going on dialysis, he received a kidney donation from his cousin. He has had CBT to deal with anxiety post-transplantation. He worked full time throughout.
Richard Y works as a full time engineer. He and his partner have an 18 months old daughter. Ethnicity: White English.
More about me...
Richard Y was doing his undergraduate degree when he passed blood in his urine which then led to a diagnosis of X-linked Alport Syndrome. The diagnosis didn’t have much impact on him in his 20s as he never developed hearing loss or eyesight problems. Because not much was known about the disease at the time, the doctors told Richard that he was only likely to develop renal failure later on in his 50s. “Life took over,” Richard says about the years when he started missing hospital check-ups and slowly forgot about the disease.
Ten years after the diagnosis, Richard Y started feeling “groggy” and “tired” after a trip abroad. His subsequent blood test revealed that his kidney function was down to 8 per cent which was a real shock at the time. He was immediately put on peritoneal dialysis, which he could set up at home. Dialysis soon became a routine for him and his partner. Richard received a live donation from his cousin after 18 months on dialysis. He felt “really humbled” because he felt that, “somebody was gonna give me something that money can’t buy”.
Richard Y noticed the effect of his transplant surgery straight away. He continued to go to regular hospital check-ups, but once the frequency of his appointments became sparser, he suddenly became nervous. He realised that he was given “something quite precious”, and he needed constant reassurance that he was doing okay.
One thing he noticed after his operation was that he became moodier and short-tempered, but there was no support system in place for post-transplant people. Eventually, Richard signed up for Cognitive Behavioural Therapy (CBT), and started working through the fears and anxieties he had accumulated over the years.
Richard Y was working full-time throughout. Looking back, he doesn’t quite know how he managed, but he believes that being able to work gave him the focus to get through the difficult periods. It provided him an “escape” from the illness by making him “feel as normal as possible”. His employers were fully supportive of him all the way through.
Richard Y’s partner was his main support during all these years. She took part in genetic counselling when they decided to have a family, and soon after, his partner gave birth to their daughter after Richard’s transplantation.
Although he is not in touch with other people with Alport Syndrome, he is a major advocate of organ donation groups and is active in awareness-raising campaigns. Still, he is undecided about the opt-int/opt-out system in Wales. He is concerned that many people will choose to opt-out, and those who opt-in would do so because they feelpressured. He also fears that organ donation might become commercialised. An alternative to the Welsh system, Richard proposes, could be investing in educating the general public and particularly healthcare staff about organ donation.
Richard Y’s message to other people with Alport Syndrome is to be their own advocate and to get as much information as they can from reliable sources.
Richard Y explains how he passed urine like ‘Ribena’ when he was in the first year at university. This led to various investigations including a biopsy and his diagnosis.
Richard Y explains how he passed urine like ‘Ribena’ when he was in the first year at university. This led to various investigations including a biopsy and his diagnosis.
Richard explains how he missed appointments but felt fit and healthy. He then became ill and was shocked to learn he was in renal failure.
Richard explains how he missed appointments but felt fit and healthy. He then became ill and was shocked to learn he was in renal failure.
So I was called in to see my GP. And his first words were "I need to take, send you to hospital, refer you to hospital. Now." He said "You've got renal failure." And I was, 'okay'. He said "The blood test that he'd done, sent away, came back with renal function at eight percent." And he'd only just got the results back from the bloods. And so I was - I guess I felt fine. Maybe looking back now, I think that it could have been such a slow steady decline that naturally by body's become used to how I felt, and that becomes the new normal. So, shock. Massive shock. And feeling a little bit sorry for myself, but also guilty that I'd not followed up my clinic appointments yearly, because there may have been something that could have been done to decline that, reduce that loss of function. Because the only thing I was told at my initial visit was that with Alports, you're expecting around about 50 years old to start suffering from problems. And that's - the guys at the hospital, that's all they'd ever seen people with Alports, in their fifties, that's when they had problems. And so that's probably why I had this feeling of 'I'll be okay until I'm 50 and then we might have to take some tablets'. Obviously it's not the case.
Richard Y describes how he and his partner came to the decision to conceive naturally.
Richard Y describes how he and his partner came to the decision to conceive naturally.
Richard Y hopes when his daughter is older that medicine will have made advances.
Richard Y hopes when his daughter is older that medicine will have made advances.
Richard Y describes his experience of taking medication after his transplant.
Richard Y describes his experience of taking medication after his transplant.
I'd had an anti-rejection cocktail of - and when I said 'no problems', we'll get back to that, actually - you might be interested in this bit. I'd had prednisolone, and two - which are steroid. And two anti-rejection medication. One was Adapore? [Adoport], and the other one was mycophenolate. So they helped control my immune system and prevent rejection. Everybody's dose differs. So, when I first started we were talking in clinic with people who'd just had transplants, "What do you take? When do you take them?" And I noticed that everyone, everyone differed. And they were like "Well I take more than you." Or "I take less than you." And I just told "Look, it's the absorption rate. If you're taking twenty or one, it doesn't matter, as long as your body absorbs the right amount." They said, "So it could be some small old woman needs one, a big bodybuilder needs one, you might need ten. So, don't worry, we'll make sure." And they have a ideal range to work in, and they sort of explained that to me. So now when I go into clinic I'm looking round at the computer and trying to see from the consultant what's, what's going off, and I'm starting to recognise the little symbols they're using, and little how they're shortening up on them. So, everything - everything was okay. I said earlier I had no problems. But in that first year, I went back to work. And I was on quite a high dose of steroids to start with. And I found that I was - well, I didn't - I didn't realise it until people started mentioning it, that I was quite aggressive. Short with people. And generally not very nice. Which is something you don't realise, I guess.
Richard Y describes doing home dialysis for the first time.
Richard Y describes doing home dialysis for the first time.
Absolutely terrifying [laughing]. The first night that I set up at home, the dialysis nurse came home with me. I'd been setting my own machine up, and she watched at the hospital. And then when I got home, we set the machine up, we found a clean area, and I set the machine up all ready for when I wanted to connect later on. Could leave it for an hour or so. That was okay. The following night, myself and my partner got everything out, laid it all on the bed, and we just had it on a, a bedside table. It's that convenient, the machine. And we got like a drip stand for extra bags. And we got everything out ready. And we sat there, reading the instructions to each other. Done it half a dozen times, with staff watching me, but this time it was - you're on your own. There were numbers we were given if there was any problems, somebody would come out. But it was - I needed about ten hours for the machine to cycle through. And so I'd want to try and get on it before no later than ten o'clock. So I'd then be able to disconnect at a certain time, get to work. So we like started this at seven o'clock, and it took us about an hour just to make sure we were pressing the right buttons, the right connectors. There was a set procedure that I was given, about washing hands. So I'd wash my hands. I changed my taps so I could have little elbow things, so I wasn't switching taps off. And then as I walked out of the bathroom I got the light cord and switched the light off. And then it's go back and start again, because. I washed my hands, the proper procedure you see in the hospitals. And so for the first week doing it on our own, it was - it was scary. But I think not always being on the phone gave us the confidence that if something went wrong, or we missed a step we could go back and we wouldn't panic, we'd just - right, we know where we are, let's reset and start again. And we got into that. And then after a week or so, this thing that'd take us an hour got us down to ten minutes. And then I started to look logically at going to and from the bathroom, washing my hands and stuff. And I was like 'okay, can do different - move these steps around, and just wash my hands once, do everything, leave it, and then before I connect again, just wash up and then connect'. So, yeah. I gave that back to the nurses, and they just said "Okay, we're still not using that, but you carry on. Whatever's comfortable for you." During that time, my results for clearance on dialysis on that type weren't very good. I was retaining a lot of fluid. And I think that was partly my own fault as well, that I wasn't as strict with my fluid intake. So I suffered with my blood pressure.
Richard Y describes his emotional state after having a transplant and how an incident at work prompted him to get CBT.
Richard Y describes his emotional state after having a transplant and how an incident at work prompted him to get CBT.
So that was in that first year. So we're looking at 2013. It was early 2014, just before my donor got married. I was at work, and I'd spent a day on a project. And something went wrong. And they have these like blocks of post-it notes that companies give out. And I'd got one, because I was writing something on it, and I got that annoyed with what I'd lost a day's sort of work - that I threw this block of post-it notes unfortunately to one of my mild-mannered colleagues. Not at him, but in his general direction. And I threw it. And as soon as I had, as soon as it left my hand, I thought 'oh dear'. Not 'am I in trouble with anybody here?' Because I think they, they must have known, and noticed that I'd changed a little bit. So I apologised. Well, I walked straight out, I was so embarrassed. I sat on the toilet, cried. Because probably that was the realisation that maybe in the back of my mind I'd known things weren't right.
But that was the realisation that probably I'd turned into a little bit of a Jekyll and Hyde character here. So my colleague, I took him to one side, I apologised. He said, "Don't worry about it, it's no problem." I said, "No, I am worried about it, and it is a problem. It won't happen again." I went into clinic a few weeks later, mentioned it to my consultant. And there's not - not really any support network for once you've been transplanted. There is pre-transplant, there's dialysis social workers, and there's renal social workers, and loads of help. But I was just kind of - not discarded, but it's - I guess people think you've got other things to worry about, medically. And for me, that wasn't the problem, it was mentally. So I asked for help. And thankfully my transplant centre had psychologists, psychologist team. So I made an appointment. Well, they made an appointment for me to have an initial assessment. We sat down. And if I'm honest with you, I thought all this - you know - I'm just, 'I'm a guy who's 35 years old, pull yourself together', is what I would have told somebody else. And I got to that point where I sat with somebody in a room, and we talked about my fears, everything. And I just found out that I'd held a lot in, I'd not really told family and friends how ill I ever felt, how frightened I'd felt. My fears for the future. You know, if the transplant fails. My problem, I'd had like nearly a year of this on my mind. I just think - the analogy I gave, it's like a pressure cooker. And it'd just finally gone. And so we started an eight week programme of writing down what happened through the week, triggers. And I was asked at the first one, "Do you want a tablet to sort this out?" And I was like, "No." And the person who was helping me, she said "Fantastic. Because I don't want to give you a tablet, I want to find ways to help you cope. So, using cognitive behavioural therapy. And find ways of, for you to identify when things are gonna get too much for you. But also what you can do to diffuse those situations, and help relax as well." So we had about eight weeks working on this. And it's an open - not an invite, appointment - then to, if I ever feel that things aren't going well again, or I've got new fears, concerns, that we can revisit that. So I found that that helped massively. And yeah. It helped massively.
Richard Y describes how he felt after his transplant operation.
Richard Y describes how he felt after his transplant operation.
I'd got two nurses helping me out, trying to cover me back up again, and get me to drink a little bit of fluid. And I'm trying to guzzle fluid, and they're trying to give me it through this tiniest little straw. I remember high-fiving in some weird anaesthetic state, high-fiving anybody who came past. Shaking people's hands. Maybe a bit euphoric, maybe a little bit high on painkillers, and everything like that. So it was quite funny. And then went back to the ward. Went past the day room, saw my family [laughing]. Apparently I tried to get them to phone [my cousin], because he was in a different part, to find out he was. What I didn't realise is that when they removed his kidney they'd pumped him full of air, and he'd had quite nasty surgery, and he was in some discomfort. And he was on a morphine driver, trying to press that button like it's a fruit machine or something. So he really struggled, from my understanding. At that time I was a little bit out of it. And then we're looking at - so that was a Monday. Tuesday. Probably early afternoon. What time would it be? I'd started to sit up, and they'd got me out of bed quite early. It was about seven o'clock I came back from theatre the day before. And it was about eleven o'clock they'd helped get me out of bed. And I was sat there. And I heard a shuffling. And this drip stand came round the corner. And he's hunched over, he'd - they take the kidney out similar to how they put my dialysis access in, so they cut just below the belly button, and then they use keyhole surgery to go in and cut it out, and then this guy puts his little hand in and pulls the kidney out. So he came hunched over, holding his stomach. Probably one of the best things I've ever seen [laughing]. Him in pain is not what I wanted to see, but to see him up on his feet. Because they pumped him full of air, he just kept burping, trapped wind, and complaining about his shoulder. And then the following day - so he had the surgery Monday morning. My partner took him and his partner home. Because he wanted to see the kids. He left on a Wednesday evening at six o'clock. So he'd had surgery Monday morning to take a kidney out. Probably a little bit too early, but he wanted to be at home.
Richard Y felt he got a lot out of seeking help from “outside”.
Richard Y felt he got a lot out of seeking help from “outside”.
And so that was for eight weeks. And then? What happened then?
That was it. The person I spoke to felt that I'd got the techniques in place. And "Did I feel comfortable with them?" She asked me that. I said, "I feel okay." She said, "Look, I don't think there's anything, anything more we can do." With this session, I don't know if it was like that was the kind of session, it was planned out or anything. But she said, "If you've got these strategies, methods to try and cope with it, you can identify it, then go away. If you have problems, then please speak to your renal team, they can re-refer you again, and we'll happily see you again. But we feel that you're okay."
Richard Y avoided telling his partner he needed dialysis because he thought she might panic.
Richard Y avoided telling his partner he needed dialysis because he thought she might panic.
Richard Y is careful about which websites he uses, and has found it useful to also attend Alport UK information days.
Richard Y is careful about which websites he uses, and has found it useful to also attend Alport UK information days.
Yeah, try and search out, but look - the NHS website is, for any sort of renal problem, is quite good. Anything dot gov. Be careful of being lured into anybody trying to sell you a cure, or a save, or eat some turmeric and you'll be okay. Yeah. I looked at them to start with, and then realised that if something - if that's, if that was true, then the NHS wouldn't be spending millions and millions on renal medicine each year. So, go to places like Alport UK, find out when the next meeting is. Go along. I went along, not really knowing what to expect, and there's such a good mix of people. People who've been transplanted, people who are - kids have Alports. People who are, who certain stages of renal issues, or kidney disease with Alports. So you're gonna meet somebody who's in a similar situation to you, possibly locally to you as well. I went to one about two years ago, we got information on - for my daughter, or who was on the way. But in a year or so's time as she's a little bit older, we could probably go along. Been told there should be no concern in these early four or five years. And about five - four or five - they might start taking blood. But in the meantime if they notice anything, to do that. So for me, it's that. And then there's a few people who've had transplants as well. And looking to try and see people who've had transplants for twenty years and thirty years, and forty years. But it's so uncommon that it's difficult. Or people have had transplants maybe because of Alport, I don't know, they've just had shrivelled kidneys, or it's been diagnosed as something else, so. I'd just say to do that. Get as much information as you can.