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.

Until recently, the treatment for Alport syndrome was just like the treatment for any other kind of kidney failure, which was to control the blood pressure, look after general health, encourage people to lead a very healthy life, and then prepare for dialysis and transplantation if it became necessary. And then it was picked up a number of years ago, that some medicines that were used to control blood pressure seemed to be particularly useful at slowing down the disease in Alport syndrome. And these are the ACE inhibitors, which have been quite widely used in other kidney diseases where there’s protein in the urine. And it was first found in experimental animals with Alport Syndrome, that it slowed down their disease. And then Oliver Gross and his team in Germany pulled together a lot of European patients, and showed that those who’d had ACE inhibitors seemed to get much later kidney failure than those who hadn’t. So that is a really important part of treatment now.

And can you explain in sort of layman terms what ACE inhibitors are, and what they do exactly?

So they’re blood pressure medicines. One of about four of the common blood pressure treatments that we use. They all work in slightly different ways. And ACE inhibitors seem to have a particular effect in the kidney, that protects the filtering units, the glomerulus. And in patients with proteinuria, they seem to slow down the deterioration of, of, of kidney function and loss of filtration capacity. The patients who benefit are the ones who have protein in their urine, and that is usually the case in Alport syndrome.

I was thirty nine. Well twenty four they started failing when I had the pancreatitis, but I was always between kind of nine G, the GFR of nineteen to twenty two, around that level. At that time I was actually put on high blood pressure tablets and after the information day, obviously now I know that that was the best possible thing that they could have done for me because they’re the ones that prolong, you know the kidney. So, I think that is probably why I went those kind of eighteen years staying at a similar level. I think if I hadn’t have gone on the blood pressure tablets it probably would have collapsed a lot sooner.

So, when were you put on those tablets?

Almost immediately because my blood pressure was really high because of, you know because of all the, the kidney issues and they liked to keep it under a hundred and twenty over seventy I think it is for kidney patients. So, I was put on them immediately when they failed. So that kind of, listening to the consultant at the information day, that kind of explains why I went so long really you know, with that kind of function and not having to go on dialysis. So, I was quite lucky cos they didn’t have any knowledge of that then that I just happened to be put on the right type of blood pressure tablets.

And do you feel they worked then; did you feel different, was it?

Well obviously it reduced my blood pressure and it kept that down, cos if, if your blood pressure goes over that kind of level it’s going to affect your kidneys. So, by keeping my blood pressure down and level it obviously prolonged the life of my kidneys, and delayed me, you know my failure.

But the results that they’ve been getting look really promising in terms of the earlier in the process, the earlier in the sort of symptoms the patient is given the ACE inhibitors, the longer the delay before the symptoms progress.

Yes

And it looked, so I mean the geneticist is saying you know it’s early days and it’s very early to be drawing firm conclusions. It does look encouraging but it, you know tread with caution. But I was really encouraged by some of the results which seemed to show that if people were given ACE inhibitors before they showed any symptoms those patients are still healthy.

Which is why I asked the question of the consultant about whether [my son] could be put on them.

Before he showed symptoms and he said, “Well, you know in terms of cost benefit then – , then we wouldn’t do it,” and I was slightly disappointed but you have to put your hands in the, you have to put yourself in the hands of the professionals, you can’t

You know they, they’re not going to make a decision that isn’t for the best of the patient are they?

So, he’s acting on the, he’s acting to the best of the knowledge he has at the moment and that’s all we can ask of them and, you know the specialists have the up-to-date knowledge so it’s not like, it’s not like going for a, to a GP and asking them what they think. This is, you know this is somebody who does know where the research is heading and what the conclusions are so far.

Yeah well I mean it’s kind of from two different angles really. With Alports they’re talking about using ACE inhibitor drugs, like a blood pressure tablet type things, to slow the progress of the damage to the kidney, which means it could last until much later in life. So, whereas the kidneys damaged and could fail at a young age, they can push that back so that they can get through school, build their social life, get through university without having to worry about missing out because of kidney issues which people generally do. They lose a huge chunk of their young adult life to it, or teenage years to it. So that, that, you know already there’s, it looks like that can change. And from the other side of things, from the transplant side, there’s so much work being done with organ donation, and it’s in the news quite a lot more often these days than it, that it has been about successful tests in transplantation, you know in animals currently with a -. They’re getting working kidneys that have been kind of made from, created in stem cells of animals to help them produce, you know artificial organs that work to a certain degree. But it’s promising anyway.

Yeah did you have like medication?

Yeah I was on medication daily at the time I think I was on three lots per day just because of the tablets I was on and so it was getting used to that as well, getting used to taking them a lot of the time on time and planning as well which when you’re 21/22, it’s not top priority to plan things it’s most things are fairly spontaneous and yeah so it was getting used to that side of things and to prioritise that over everything else. It didn’t really get in the way of life.

And can you say a little bit more about the medication that you took back then? You said you were quite bad about taking it, could you say a little bit more about it?

I think it was just with being, being younger, and not as responsible as I am now. I don’t think I realised the damage that it could do if I didn’t take it regularly. And perhaps maybe had I taken it regularly, I wouldn’t have needed to start dialysis as early as I did. I don’t think it was really explained to me either, how important it was to take the medication. I think if that had been stressed, then I probably would have got into the habit of taking it regularly. Another thing that frustrates me as well, is – might not have helped at all but, when I was younger, before I even knew of anything to do with Alports, I suffered from headaches from time to time. So I’d mentioned to my GP this, and we tried several things – keeping a food diary, that type of thing. But I’d mentioned to them that I take ibuprofen. And then for years I just continued to take ibuprofen, even once I had been diagnosed as having kidney problems. But I wasn’t aware that the ibuprofen shouldn’t be taken if you have any kidney problems. And it was on the GP records that I suffer from headaches, and I’d said on several occasions I take ibuprofen. So I wonder if that, you know, speeded up the, the kidney failure and that.

And what medication were you on?

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.

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.

Yeah it’s completely normal, you know, so I go for four month check-ups have my, take my tablets twice a day and once you get used to that it’s not, you know, it’s not really a big thing, people say taking them for life is, you know, it’s a big a big deal but it’s not really cos it keeps you alive, so if I didn’t take them then there’s a chance that my kidney might reject.
So it’s a small price to pay just to swallow a few pills a day, that’s it.

So medication is an interesting one. At various times, various points I’ve had very high blood pressure and very low blood pressure. The first transplant I had to do with the nature of the organ I received caused me to have very, very high blood pressure, seriously high blood pressure. So I got treatment for that, not entirely satisfactory treatment for that but I would say more to do with the limitations of the treatments that were available more than any lack of attention to it. So that was fine.

This calcification issue causes a lot of problems and a lot of medical. It caused a stomach ulcer, caused my tendons to rupture, all kinds of stuff over the years and so that the. I’m taking, I take stuff like a medicine now for stomach, keep the stomach settled, that kind of stuff.

That’s improved over the years as with the side effects. The medications you take to keep phosphates in check is improved a lot. It’s much easier to take. It used to be a glass of water, mixed a powder, mixed up in a glass and that was your fluid intake gone for the day. So now it’s now a chewable tablet so that’s easier. The biggest, the biggest thing are, the biggest improvements for me have come around treatment for anaemia which the anaemia has, yeah it reduces your energy, it reduces your appetite. So the fact that that can now be dealt with very satisfactorily through medication is a massive improvement for me. And it’s all dealt with intravenously by dialysing so there’s no injections or anything. So that works very well. So that’s a massive improvement. I’m not taking very much. The, I had very low blood pressure for a while and the treatment for that was a dosage of a particular pill that my consultant was able to prescribe. But my GP was not actually licensed for use here. So that was a bit of a hassle. So my GP couldn’t prescribe it. My consultant had to prescribe it and when my consultant came down to the unit every three months she’d get some from the. Save me having to go up to the hospital to go to the pharmacy she’d bring it down with her. That’s, that’s service as far as I am concerned. That’s, you know, absolutely marvellous. But that wasn’t licensed here so she was able to give it but the GP wasn’t, that’s a bit of a business but it did what it needed to do. And so that’s ok. But generally speaking I think the only other observation around that is that all my interactions are with my consultant. I don’t have really much interaction with my GP. That’s, that’s something that happens. I probably see my GP every couple of years. Your prescriptions can all be done online now so I do them online. And it’s all done automatically and so.

And what medication are you on? Can you explain? Are you on any sort of medication?

Yeah, well I take phosphate binders with each meal, that’s for the phosphorous levels. And then I take aspirin. And I take lisinopril, which is, to keep – it keeps my blood pressure down, but also it’s to help with the kidneys in some form. Does seem to be helping with them as well. So I take that. I’m on amitriptyline, because I have serious arm pain and hand pain. I’ve had operations on my hands because I had carpal tunnel. And I’ve got Raynaud’s as well. And then that plays up, so I’m on medication for that. It’s not too bad really, medication-wise.

Do you get any side effects from any of those medications at all?

Tiredness. Which is a bugger, because I’m on amitriptyline for my arm pain and that adds to the tiredness as well. And codeine as well, so.

Yeah, what’s the codeine for?

That’s for the arm pain. Yeah. Yeah. So I’ve had operations on my hands, which has helped a lot. But at the same time I’ve still got pain. Which is bad because when I had the operation, I had to set my machine up and things, but my daughter comes and helps me with a lot of things like that, so that’s good.

Yeah, only a low dose, 5mg. And I take nifedipine, that’s for the Raynaud’s, for my hands. Most of my medication actually is for my arms and my hands. For the kidneys it’s just lisinopril, aspirin, phosphate binders, and then Lactulose, which is a laxative which I have to take regularly because – the main thing with peritoneal dialysis, sorry, it’s a conversation you probably want to avoid, but it’s being constipated. That can affect adversely with the dialysis. Because it’ll push the tube out of synch. Obviously, can you imagine if your bowel gets filled, it’ll push the tube out of synch, got to make sure you’re, you’re regular. So I take that every day, twice a day.

But apart from – no, I feel okay. You know? I really can’t – and I think my son does as well, generally. Does, does suffer quite a lot of headaches at times, and I have mentioned this to the consultant, saying “You know, he really does have really bad migraines.” Touch wood, he really hasn’t had them in the last couple of months, but there was – I mean, I’m going back since he was first diagnosed, that – you know – once a week, twice a week, he was having these severe migraines, that I had to actually pull over the car, and he was vomiting out of the car, and. He has to go to his bed and sleep. And they were saying, you know – they’d sent us along to the consultant saying “Well could it maybe like something that he was eating, or? You know, just keep a wee note of what he’s eating, like that.” And there was nothing different from any kind of normal, so like that. I had went onto the Alports UK Facebook page, and basically just put on a wee status, just saying, you know ‘my son’s been suffering with these headaches, and it’s really quite concerning because they’re saying it’s not Alport-related, and there must be something wrong, I don’t understand – you know – why this is happening. And someone come forward saying it could possibly be the medication, and that maybe you need to think about changing the type of medication. So I had went back to the consultant and said, you know, “I put this on the Facebook page, and this is what had come back.” And they were like “No, never heard of that.” And “Well, we’ll monitor it and we’ll keep a wee eye on it.” And things like that. So, don’t get me wrong, I think different makes of – he’s on enalapril. And I think different makes, different manufacturers, there is different taste to the medication. And because he can’t swallow tablets, he used to crunch them. So some of them were absolutely disgusting, and it was really hard for him to get it. So we kind of stuck to this certain make that he, he didn’t mind the taste. So I was kind of thinking ‘well maybe, maybe we just need to stay away from that make, he’s a wee bit older now, I’ll just need to say to him well get it behind you, you’ll be fine, get a drink of water you’ll be alright. So now I don’t kind of restrict on that type and make of the manufacturer.