1 (INSPIRING MUSIC) Captions by Starsha Samarasinghe. Edited by Faith Hamblyn. www.able.co.nz Captions were made possible with funding from NZ On Air. Copyright Able 2017. (POIGNANT MUSIC) Life right now is, on good days, pretty good, just being able to wake up and having Skylar every day and... I wanna live for as long as I can to be with her. I wanna buy a house and go on holiday and do all that kind of family stuff. So trying to just think positive that I will be round long enough to do all that. (EXHALES) Nikki has cystic fibrosis,... a genetic mutation that has slowly eroded her lungs. Now when I'm really bad I get quite breathless, and I need oxygen quite a lot at home. Overnight you have a lot of phlegm and really breathless and tired. (COUGHS) Hey, Skylar. How are you? Hey, Nikki. Hello. How are you? Good. How are you doing? I'm all right. Nikki's sister, Kristie, also lives with cystic fibrosis. Even though she's my sister, she'd be my best friend, and I probably look up to her. I'm the oldest, but I probably look up to her more. (COUGHS) How was your night? Oh, it was so bad. I left Hayley's bag here with all my medicine in it. I was up all night, couldn't lie down. Cystic fibrosis affects the lungs. All the little airways get clogged up with mucus, and it just infects them and destroys your lungs. So you just need to keep having lots of medication to stop the infection. And then later in life, you can develop liver and heart conditions as well. Mummy's gonna go to her appointment again next week with Daddy. Are you gonna come this time? Do you remember why I go to my appointment? What am I gonna get new ones of soon? Gonna get new...? Lungs. (LAUGHS) She goes, 'Mum, when you get new lungs, you'll be able to chase me and catch me, eh?' Gonna pour these into here. That's good. With lung transplant, you don't know long you'll wait for a donor, cos it can happen any time. And cos I'm quite small, they think I could wait quite a while. Cos I possibly could need a paediatric donor. Do you know what I'm gonna get done in Auckland this time? No? Yes, you do. New lungs? New lungs? No, I'm gonna get some medicine to get my lungs better. Kristie is not yet on the transplant list. Do you reckon they're gonna turn out yummy? Yeah. I hope so. Not many people know about cystic fibrosis, so we want to get the awareness out there, and we came up with the idea together of doing good deeds, to do nice things for the community and other people. And everything that we did, we'd just leave a note saying it's from Cystic Fibrosis Sisters and explain what CF was. Done! High five. High five. Yes. We came up with the idea of doing 65 days leading up to CF Week for cystic fibrosis, so 65 days of good deeds. Where should we take them? I reckon we take them to some organisation. Women's Refuge? Women's Refuge or Life Saving? Yeah, I like the Surf Life Saving. Surf Life Savers? Yeah. It makes you feel good, and more people need to do stuff like that. Not many people do it enough. Hello. Hey, girls. Um, we've got cystic fibrosis, and we're raising awareness for it. And we know you guys do an awesome job, so we thought we'd do a nice deed and bring you guys afternoon tea. Lovely! Dish 'em out, girls. When you tell people that you have CF, and they get the basic of it, what it is. Good knowing that you've got awareness out to at least someone else that didn't know what CF was. You cough a lot, and then people think that you're infectious and got diseases and stuff and wanna stay away from you. And then they don't understand why you're tired. And, 'You look healthy. Why don't you come and do this?' But they don't actually get how you're feeling, cos you look well. (COUGHS) Well, at least they must have been eatable, cos they all ate them. (LAUGHS) (MACHINE WHIRRS) (COUGHS) Kristie's early years were pretty horrific, really. She had problems right from birth. Unfortunately, she wasn't diagnosed until she was 3, so her first three years were, like, um, repeated hospital visits. The doctor sat us down and explained how disaster it was that we mightn't have her at 16. It was just devastating. The outcome they told Mum and Dad, 'You'd be lucky to meet her` make her teens.' But as time went on, obviously, treatments and stuff. And I'm still here today, so... (COUGHS) (COUGHS) To have a CF child, both parents have to be carriers. And both have to have that C` CF gene, um, for the child to actually have CF. So you have one chance in four of your child having CF if both partners have got the gene. I was told after I had Kristie that I couldn't have any more children. So we just didn't worry about it after that, and then eight years later, I got pregnant... somehow. (LAUGHS) So we just hoped and prayed that she would be OK. But no, she was born with CF as well. Unfortunately, she was a little bit worse than Kristie was, so the` at least the only thing we could do was just to sit back and say, 'OK. Let's enjoy what we've got.' Do you guys want a drink? Up the Mount, down the Mount, up the Mount, and then around the Mount. That's pretty good going for you. I was gonna go for a swim after, but then I was too tired. Yeah. 'I met Craig through school days. And then we, kind of, were just together. 'Left home at 18 and flatted with him.' Did you make the stuff for Dad? Yes, last week. Brendon's my husband. He's really good. When I am unwell, he takes over and does everything I can't do so I can just look after myself. Tablets, Kristie. Thanks. Two, please. I fell pregnant at 18 and dealt with that. We just didn't think it was possible to fall pregnant or have kids, so it's a bit of a shock. Uh, having Skylar was a big surprise. Me and my husband didn't think we'd ever actually have children, so we were, um, so happy when we found out we were gonna have her. 'And it's definitely made me stronger and push a lot harder to stay alive and keep fit.' She's a really good mum. None of the hospital stuff really scares me or anything. The only one thing that scares me is just losing her, basically. And that's the only thing. And just for Skylar. Main thing is, I think about her. Just cos they're so close. Just that's the one thing that really freaks me out. (CHUCKLES) Wasn't till I had children that our kinda outlook changed a bit. Knowing that that could be taken, it sucks a whole lot more. (CHUCKLES) The oldest, I've just kinda told him that I've got CF, I might need new lungs, and then it'll make me healthy again and I'll pretty much be around forever, so... Could happen, so... Oh, yeah, exercise pretty much got me to where I am today. Pretty much what I live for is physical activity ` getting out there and keeping me as fit as I can. I find it hard now, cos I push myself and get really fit for a week, and then I get sick, and then go into hospital for two weeks, and I've lost everything that I've worked for in that week, so then I've gotta try and redo it the next week. And I have bad days where I'm, like, 'There's no point even doing it, cos next week I'm gonna be sick,' but I know that it's gonna help in the long run, so I just push myself to do it. (GENTLE MUSIC) Shall we start with your front hook? Yep. So inside, and your inside knee's going to hook round the pole. Right round. Squeeze it tight. OK, you're gonna fall forward. Feet together. Knees wide. And I want you to have pointed toes when you do it. I know a few other people with CF who are a bit more relaxed, and they might smoke or drink and think they'll be all right, but me and Kristie are quite, um... we wanna be healthy and strong, so we try do everything the best we can and look after our bodies, just so we can stay healthy or healthy as we can for our kids and family. Well done. Any form of exercise is really good, so anything that we're interested in they encourage us to do. She's lived with, like, having no lungs for quite some time. So her, probably, she's got used to not being able to breathe. And she thinks what she's experiencing now is normal, but it's not anywhere near normal. Like, what she's coping with now ` probably you and I could not live like that. We would think we were dying, can't breathe. But she's learnt how to control it and how to live with it. So although her lungs have had it, she's still coping with that. I used to be able to run round with Skylar. I always say to her, 'I'll be able to chase you, and I'll actually be able to catch you now.' Just having energy to do daily stuff, just little things. Like if we go the pools or to the beach, and I can't really go out there and do it with her, cos I get too puffed. So Brendon or other people generally do it; I just have to sit and watch. So it'd be good to actually do things with her. So that's the physio done. Massage is one of the things Nikki does to try and make her lungs last long enough for the transplant. Quite strange having to do this again on you. If she does get new lungs, cystic fibrosis will eventually infect them too. Hope that the transplant will give me better quality of life and that I can be around for as long as I can and watch Skylar grow up. Three to five years is the average, so that's just what they go on by how many transplants they've done and how people go. They can't tell what you'll be like, cos everyone's completely different. Is that enough? It's another trauma for us to get through. We're just positive that she's gonna get through this and lives for another 10 years. Cos it, um... it won't cure the disease ` the new lung will get destroyed by the CF gene again. So, yeah. So we just hope we can get another 10 years out of our little girl. (LOCK CLICKS) Ever since Skylar was born, I've written in a book every day and a` another book just of advice and things for her to have if I'm not around, sort of write to her about how much I love her and to be strong and happy. READS: Sing, dance, read, write, learn everything you can. Play with kids, listen to the elderly. Watch everyone else learn from their mistakes, so you don't make as many. And if you do make a mistake, just roll with it. Learn. You were meant to make it. My advice ` what I try to teach you ` is only a small part of the knowledge and experience you will figure out all on your own. 'I think having CF has definitely brought us all closer and thought that we wouldn't be around as long, 'so we've really stuck together, and if we didn't have such a tight family unit, 'I don't think we'd be where we are today. It's made us keep going and fighting.' 'At the end of the day, the most important thing is family and just living in the moment. 'Cos you really don't know what is gonna happen round the corner.' 'I think the most important things in life is just to be happy with your life and family and fun.' You're here to have fun and enjoy life, so do it with the people that love you and support you the most. Mummy's going to hospital for two weeks. Are you gonna help me pack my bag? What do I need? My inhalers? Get my other inhalers. Yep. What else do I need? Those are my Creon. Put them in. Are youse gonna come visit me when I'm in hospital? Yeah? Two weeks is a long time. Are you gonna do all your homework when you start school when I go to hospital? Let's go. Let's go out the door. Cos I'm pretty much my lungs are sitting at 30%, and they're not really coming up much with treatment, the next step is to do all my treatment in hospital to see if staying in hospital can prevent me from needing the new lungs for as long as possible. It's kinda the last step. (SOMBRE MUSIC) I normally come up to Auckland every month for two weeks of IV antibiotic, so just staying in the hospital for two weeks. (MUSIC CONTINUES) They think that just the combination of everything in the hospital works better than doing your treatment at home. You get the intensive physio from the physios and their help, and the rest of the antibiotics in your body can heal with that. You don't really get that at home with three kids. (SOMBRE MUSIC CONTINUES) I've reluctantly agreed to it, but it's what has to happen at this point of my life. So I just gotta suck up with it and deal with it, but it's pretty hard. Hiya, Kristie. How are ya? OK. Good. Hi, Kristie. Hey. How you doing? Yeah, not too bad. Not too bad? Yeah. Yeah. Do you think that anything we've done, in terms of the last few times, has changed the amount of benefit you get or how long it lasts? Uh, not really. Um, so just the same with steroid versus without. Yep. OK. All right. Few` (COUGHS) Few big breaths for me. (BREATHES) OK. Good. (COUGHS) So, how's things been in terms of, you know, activity at home? Uh, before I got sick, I was going up the mountain, swimming about 70 lengths a day and` Wow. ...lots of walking and running. Yeah, that's fabulous. Yeah. So, your lung function's come up nicely. Um, it's probably as good as it's been in quite a while, so that's really good. Um, so I think we, um, will stop today and let you get out of here. Cool. (LAUGHS) All right. You happy with that? Yeah. (LAUGHS) Um, so, and then we'll see you back, um, in a month in clinic, um, and see how things are then. (GENTLE MUSIC) Every three months I come up to the transplant clinic, and they usually just do bloods, X-ray. Cos I'm on the list, you're kinda at the end ` there's nothing else they can do for you. Before you're listed, they're trying to keep you off the list. Once you're on the list it's kinda like, 'Well, that's it.' Uh, number 19? Hi. Hi. Now, we're just down in room number two. Grab a seat there. Uh, blood tests ` I've had them my whole life, so I'm used to being pricked, and I've got quite... I haven't got very good veins, so it usually takes them two or three goes to finally get blood. Right. I'll get you to press on there. Well done. Thanks, Skylar. (GENTLE MUSIC CONTINUES) 'Course there's always moments where I feel down and sad and think everything's unfair and, 'Why me?' But if I stay like that, then I'm not gonna be able to live and be very happy. So I always pull myself out of that and just keep on living and be happy. Clinic's at 1.30. Psychologist is... What's the psychologist for? They just make sure that you're coping with everything and understand everything. Oh, of course. (GENTLE MUSIC) I'll get you to have a seat on the green chair over there for me. So, Nicola, what we're gonna do today is a test called spirometry, so we're gonna be looking at your airways and how well they work at moving the air in and out of your lungs. And I'm gonna ask you for a big, deep breath and as much as you can. (COUGHS) So right up. I'm gonna yell, 'Blow!' And I want you to blast it out as fast and as hard as you can. And keep squeezing all the way to the very end. So, there's your nose clip if you wanna pop that over your nostrils. And then hold on to that white part, sitting up nice and straight. Right on. Seal your lips. Blow! Good. Squeeze it out. Squeeze, squeeze. Keep going, keep going, keep going. Squeeze it out, keep pushing. Keep going, keep going, keep going, keep going. OK, good. Come off, and have a rest. (COUGHS, GASPS, COUGHS) (COUGHS) (COUGHS) Hi, Nicola. Hello. How are ya? Come on in and have a seat. Hi, Mum and Dad. BOTH: Hi. Come on in. Come on in, have a seat. All right. How you doing? Pretty good. Pretty good? Yep. Yep. So, how's things been? What, did I see you... November? Much changed? Uh, I have felt a little ` probably last couple months ` worse than I have, just with breathlessness and doing stuff a bit harder. OK. Yep. Yeah, we're all convinced it's the right thing to be on the active waiting list. As you know, we don't have any control over it, so it can happen any time. Um, the outcomes from transplant are still, you know, not as good as what we'd want them to be. Uh, the average survival post-lung transplant would now be between, um, seven and eight years. Yeah. And that's one of the reasons to try and defer it for as long as possible. Yeah. And of course we've done that with you. Um, so, you know, what, did we first think about transplant, when you were 14? Yeah. Um, and you're now 25. So we've done quite well in deferring it a decade. Yeah. (ALL LAUGH) Um, but I think that, you know, the nature of CF is that it ultimately, you know, will always progress and` Yep. Um, and I guess that's where we're, or why we're, you know, um, in the position we are now. The principle reason that you have a transplant is because of the improvement on your quality of life. Something like 80% of our people will, um, you know, go back to basically being able to do the things that you wanna do, um, and not be constrained by, you know, all the treatment requirements. She's amazing. She just copes with, life itself. I know she has moments. I went home the other day and found her just in a real mess down at the clothes line,... bawling her eyes out cos she couldn't cope. She couldn't hang the washing out on the line, and she just got beside herself, and I want her` She wants to do things, certain things, and she just can't do them sometimes. And that was one of those days. She just... just wanted to cope, and she couldn't. (POIGNANT MUSIC) She realises now she can't keep the lungs. She's not going to live another two years with these lungs. It's, you know, sort of like, put down in black and white. In the end, this is what you've got. There's just nothing left, so she's got no choice. Nikki's really only got one chance at a new set of lungs, and she's gotta be well enough to actually go through all this and come out the other side. Coming out the other side is the hard bit. We've got three months of` of rejection of all her organs that gonna have to go through, and that's gonna be` I think that's gonna be the hardest road for us when that happens, yeah. To me, I feel that that's putting her life on, like... With her own lungs, you don't know how long you've got. But once she's got that transplant, you can put years` so many years on it. Last couple months I had a struggle getting my head around being on the active list, even though the doctors said it was the right idea. Just when I feel good for a few weeks, I feel like I'm too healthy to be on it. But I now feel like it's the right decision and it's better to go into the operation strong than sick. (POIGNANT MUSIC) There are so many things in life that I won't get to do. READS: All of the things I wanted to be used to upset me, but now I have you to do and see everything for me. You'll have your own dreams and things you want to see and be, but I can guarantee that you will do and see and be just like me. Officially on the transplant list ` I'm scared, worried and wishing it was in 10 years' time. But mainly just want to be here forever with you, Skylar and Willow. Every time the phone rings, I panic. It's going to be a long journey, but Skylar, your face and smile will get me through. (POIGNANT MUSIC) (MUSIC CONTINUES) I have notified Nicola that we have, um, some exciting news for her ` that we thought we may have some new lungs for her tonight, So she is making her way to Auckland City Hospital, where I will then be responsible for preparing her for theatre. So how`? It was bad traffic, was it? BOTH: Yeah. So, Nicola, what will happen now is that Chris Lewis ` you've met Chris? Yep. He will come and see you. We're both at home, pretty much planning a good deed that we're gonna do, and then she` her phone rung, and she was on it. And then my phone rung, and I was on it. And then she kinda went all quiet. And then all of a sudden, she said, 'I've got my lungs.' And we both kinda stared at each other, and we were both shaking and just didn't know what to do. Hi, Nicola. Hello. Hello. How are you? I'm good. So, I just need to have a little listen to your chest. All right. That three hour trip was, um, pretty hard for us,... just sorta talking about things and talking about our daughter, and Nikki wrote a couple of letters to us, just on the way. Just bec... Didn't know what's gonna happen. You'll probably be kept asleep for several hours. Yep. Might be up to a day or so, just to let everything settle down` Yep. ...and just to let your body recover from all the surgery. Yep. Um, so you won't remember much about that ` good for your husband and for the family. It's just probably a little bit of a shock when they first see you on the ICU with the tube in the mouth, um, some, uh, lines in the neck for medications, you get four chest drains ` it's all quite, sort of... little bit, uh, little bit of a shock when they see you for the first time. Well we've been expecting ` not expecting it, waiting for it ` for well over a year. So, you know, I think you get to the stage where you get, 'Oh, it's never gonna happen,' sort of thing. I was a mess when I was told at work. I was stacking leeks in a shop, and next thing, I was told, 'Your daughter's going into Auckland to have lungs in.' Wow. Yeah, it was a... it's here now. How you going, Kristie? Yeah, not too bad. (CHUCKLES) It's quite a big operation. There's always that fear that it might not work or it won't work for very long. Worried about waking up and what I'm gonna wake up like. I have so much to look forward to, like watch Skylar grow up. (GIGGLES) (SOMBRE MUSIC) While Skylar goes home to sleep, her mum is about to begin an eight-hour operation. (MUSIC CONTINUES) This is where you'll say goodbye` Yep. ...to your family. And then you'll go through to theatre. (POIGNANT MUSIC) OK. If you can help ` the brakes. (SNIFFLES) (MUSIC CONTINUES) (ACOUSTIC GUITAR MUSIC) The operation was for eight and a half hours. Each day I've just been feeling better and better. When I breathe now... (INHALES) I feel like I'm actually getting air in. So, I didn't realise how bad I was before. I can't wait to get outside, see what they can do. (LAUGHS) (SERENE MUSIC) Just six weeks after double lung-replacement surgery, Nikki is back home with her family. Now I'm back home in Tauranga, waking up every day and doing what I want, which is a bit weird. But the successful operation has separated the sisters. I can't be within 4m of Kristie. She's still got her CF bugs, which I could catch. Cos I've pretty much got no immune system, I can pick up bugs easily. And then if I did get sick, I've got no immune system to fight it, which could potentially kill me. I think it's harder for her than me, cos my life has changed. Nothing's really changed for her. You'll wake up in the morning, and then you get up, and you cough for half an hour, and then have to sit down. Just even making the bed and hanging up the washing, I get really puffed and struggle a bit with that. For her, that's all gone. I would love the feeling of what Nikki has, but I know there's a big.... I've seen what's ahead, so gone a bit, 'Don't want it to happen,' but you know that it's gonna be better. Hopefully, when I've got mine, we'll be just as close again, and be running round with all the kids and having big family barbecues and being just as close as we were again. It hasn't taken long, and I've started to forget what I used to be like. Completely new life ` more independent and not relying on people and having more energy and just doing whatever I want, really. Breathe. Captions by Starsha Samarasinghe. Edited by Faith Hamblyn. www.able.co.nz Captions were made possible with funding from NZ On Air. Copyright Able 2017.