1 Captions by Amy Park. www.able.co.nz Captions were made possible with funding from NZ On Air. Copyright Able 2016 UPBEAT MUSIC UPLIFTING MUSIC KRISTIE: Even though she's my sister, she'd be my best friend. NIKKI: We spend pretty much every day together. As soon as I wake up, the first thing I do is I text her and say, 'What are you doing today?' We share so much in common and go through the same sort of thing every day. We know what each other thinks and feels, really. We can always tell when the other one is not feeling that great. Cos we've got each other to talk to about what's happening, and I don't think anyone that doesn't have CF doesn't really understand what you're feeling. Having someone else that has CF makes you feel a bit more normal; someone to relate it all to. INHALER HISSES GIRL: Hi, Skyla! (COUGHS) Hey, Skyla! How are you? Mwah. Hey, Nikki. Hello. How are you? Good. How are you doing? I'm all right. 'Every day, we normally go to my sister's house, and then decide what to do from there. 'We do something fun with the kids every day and just hang out, pretty much, every day together.' How was your night? > Oh, it was so bad. I left Hayley's bag here that had all my medicine in it. I was up all night. Couldn't lie down. Had no Ventolin. Yeah, I saw the Ventolin when I woke up and wondered how you'd got on. I'm here to get it all. Hey, guys, what do you want for breakfast? (COUGHS) INHALER HISSES Cystic fibrosis affects the lungs. All the little airways get clogged up with mucus, and it just infects them and destroys your lungs. LIVELY CHATTER 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. INHALER HISSES When I'm really bad, I get quite breathless, and I need oxygen quite a lot at home, and overnight I have a lot of phlegm and really breathless and tired. There's a lot of tablets. Before we eat meals, we've got to take tablets` three or four tablets before we eat anything. And then you've got the antibiotic tablets, vitamins, and we do physio twice a day, blood test every` before and after meals, testing the diabetes, insulin if needed. 3.2. Low! I get to have some sugar on my rice bubbles. INHALER HISSES Every single morning, we struggle to breathe. Everything we do is hard work. And because you're struggling to breathe and working on that, you get tired really fast. It takes me a good three or four hours in the morning just to get up and get ready. I feel real yuck in the morning and cough. I don't stop coughing. MACHINE CHUGS AIR HISSES (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, doctor's visits. Nearly every week, actually. She would be on antibiotics. But nobody picked` sort of, like, picked up the CF. So she was in` had a lot of pain; had a lot of discomfort for years. We didn't know anything about this, cystic fibrosis. So we just` we were pleased that we knew what was actually really wrong with her. But then the doctor sat us down and explained how disastrous 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 make her teens.' But as time went on, obviously, with treatments and stuff, and we're still here today. So... (COUGHS) (COUGHS, SNIFFLES) To have a CF child, both parents have to be carriers, and both have to have that CF gene 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. GENTLE MUSIC 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 only thing we could do was to just sit back and say, 'OK, let's enjoy what we've got.' LIVELY CHATTER So, what have you done today? We went 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` we're just together. Left home at 18 and flatted with him. Did you make this stuff for Dad? Yes. Nice work. Brendan's my husband. He's really good. When I'm 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 that was it. We just didn't think it was possible to fall pregnant or have kids, so it was a bit of a shock. Kristie and I met at school with groups of friends, hang out together and got to talk, and, yeah, one thing led to another. Having Skyla was a big surprise. Me and my husband didn't think we'd 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 Skyla. The main thing is I think about her, just because they're so close, just` that's the one thing that, really, freaks me out. It wasn't till I had children that my, kind of, outlook changed a bit. Knowing that that could be taken away sucks a whole lot more. The oldest, I've just kind of told him that I've got CF, I might need new lungs, and then it'll make me healthy again and pretty much be around forever. It could happen, so... I just want to spend every day with them and do as much fun things I can and give them heaps of cool memories to remember and hope that I can be around for as long as possible to see all their milestones. SNIFFLING We're gonna go and get the chickens. INDISTINCT CHATTER GENTLE, STIRRING MUSIC COUGHING Life right now is... on good days pretty good. Just being able to wake up and have Skyla every day and Brendan and all the family around me and friends. I think I'm pretty lucky to have all them. We want to buy a house and go on holiday and do all that kind of family stuff. So I'm trying to just think positive that I will be around long enough to do all that. Oh, I don't even know how I would deal with it if anything` if she did die or something before me or anything. We spend so much time together, I'd be completely lost. I just wouldn't know what to do. Let them out. Good boy. It's definitely hard to see her, how much worse she is than me. I always feel stink and think, 'Why did she get the shitter lungs?' and stuff. COUGHING Was it hard for you to walk up today? It was all right. It made me cough a lot, so a little bit of physio for me this morning. It was all right for me today. Yeah. (COUGHS) Having CF means I've got to do a lot for the chest, a lot of physiotherapy, a lot of tablets, um, antibiotics, nebulised antibiotics. And vitamins. A lot of vitamins, inhalers. And then I've got complications with my sinuses, so I have to do a lot of sinus rinse every day in the shower and stuff. I had a lot of operations and procedures with that. < I take a lot of steroids. So, a prednisone is a steroid for the inflammation of the lungs. < And then just the diabetes and the insulin. And then I've got quite a strong component of asthma with the CF, so I get all the wheezy side of that. So that's just more nebulisers of Ventolin. For me, it's a lot of the same stuff ` all the chest therapy and nebulised antibiotics, oral antibiotics. I've always struggled to keep my weight on and gain weight, so I drink, um, like, supplement drinks to help keep weight on. When I'm unwell, I drink two or three of them a day, cos I don't really eat. Part of the food is your pancreas produces the enzymes to digest the fat, and our pancreases are blocked with phlegm, so it doesn't make those enzymes, so we take them before we eat so it'll break down the food. Otherwise, if we ate a sandwich, we'd only get about a quarter of it. Hi. < Hey, Zack. Take your shoes off. < Say hi to Zack. Take your shoes off. Hey, Nikki! Hi, Skyla! How are youse today? Good? Rahhh! Mwah. MIXER WHIRRS You reckon it all looks good? You want to stop? It's really hard. MIXER POWERS DOWN 'Skyla's nearly 5, so she sees me quite sick.' Mummy's gonna go to her appointment again next week with Daddy. Are you gonna come this time? 'We don't hide anything from her.' Do you remember why I go to my appointment? What am I gonna get new ones of soon? I'm gonna get new... lungs! She goes, 'Mum, when you get new lungs, you'll be able to chase me and catch me, eh?' 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. Not many people know about cystic fibrosis. So we wanted to get the awareness out there, and we came up with the idea together of doing good deeds. So doing 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. CLUNK! Done! 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. We've explained to the kids that we've got, um` we're doing random acts of kindness to other people because it's a nice thing to do, and they've kinda gotten on board and they know all about it and get all excited to help. Where should we take them? I reckon we take them to some organisation. The Women's Refuge? The 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. See you later, girls. We'll be back soon. Hayley! See you later. We'll see you soon! Love you! Bye. GENTLE GUITAR MUSIC Hello. Hello, girls. 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. Wicked! That's awesome. How's that, guys? Lovely! Dish 'em out, girls. Here you go. We tell people we have CF, and they get the basic of what it is. It's 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 people think that you're infectious and got diseases and stuff and want to stay away from you. And then they don't understand why you're tired, 'You look healthy. Why don't you come and do this?' But they don't actually get how you're feeling, because you look well. Well, at least they must have been eatable, cos they all ate them. BOTH CHUCKLE BREEZY MUSIC 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. Then I've got to try and redo it the next week. And I have bad days when 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. Should we start with your front hook? Yep. So inside` your inside knee is going to hook around the pole, right around. Squeeze it tight. OK. You're gonna fall forward. Feet together, knees wide. Tuck your knees, draw them up, 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 want to be healthy and strong, so we try to do everything the best we can and look after our bodies, just so we can stay healthy, or as 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 she's probably 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 if we 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 just want to be able to run around with Skyla. I always say to her, 'I'll be able to chase you, and I'll actually be able to catch you now.' Just having the energy to do daily stuff. Just little things, like if we go to the pools or to the beach, I can't really go out and do it with her, cos I get too puffed. So Brendan or other people generally do it, and so I have to sit and watch. So it would be good to be able to actually do things with her. That's the physio done. Yeah. With a lung transplant, you don't know how long you 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 and they don't come up very often. I hope that a transplant can give me a better quality of life and I can be around as long as I can. 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 you what you'll be like, cos everyone's completely different. 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. We just hope we can get another 10 years out of our little girl. Ever since Skyla was born, I've written in a book every day, and the book is just of advice and things for her to have if I'm not around later on. I, sort of, write to her how much I love her and to be strong and happy. 'Sing, dance, read, write, learn everything you can. 'Play with your 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 and 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'll 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. 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 going to happen around 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. Can you get my other inhalers? Yep. What else do I need? Those are my crayon. 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? ZIP! Boom! Let's get out the door. Cos I'm pretty much` my lungs are sitting pretty much 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 kind of the last step. POIGNANT MUSIC I normally come up to Auckland every month for two weeks of IV antibiotic, so just staying in the hospital for two weeks. They think that just the combination of everything in the hospital works better than doing the treatment at home. You get the intensive physio from the physios and their help and the rest of the antibiotics, and your body can heal with that. You don't really get that at home with three kids. POIGNANT MUSIC CONTINUES I've reluctantly agreed to it, but it's what has to happen at this point of my life, so I've just got to suck up with it and deal with it, but it's pretty hard. I just sit here in the room for two weeks, thinking about the kids and all the things that I'm missing at home. PUMP HISSES SOFTLY I saw you and your sister in the, um` in the paper last year, about what you guys were doing with the... Oh, the deeds? Yeah. That was awesome. How long did you guys do that for? THERMOMETER BEEPS 65 days. Oh. And did anyone, like, just randomly join you? Yeah, they did, like, a day here and there, yeah. Helped us out. Yeah. Yeah. (COUGHS ROUGHLY) So, what does Dom say you have to do when you get home? Carry on with the exercise. Yeah. All right. There you go. All done. Thank you very much. Cool. Hiya, Kristie. How are ya? OK. Good. (COUGHS GENTLY) Hey, Kristie. Hey. How are 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. Ah. So just the same with steroid versus without? Yeah. OK. OK. All right. A few` (COUGHS ROUGHLY) A few big breaths for me. (PANTS SOFTLY) (BREATHES RAGGEDLY) OK, good. (COUGHS) So, how's thing's been in terms of activity at home? Um, before I got sick, I was going up the mountain, swimming about 70 laps a day. Wow. Lots of walking and running. Yeah, it's fabulous. Yeah. So, your lung function has come up nicely. It's probably as good as it has been in quite a while. Yeah. So that's really good. Um, so I think we'll stop today. We'll check you out of here. Cool. You're happy with that? Yeah. And then we'll see you back, um,... in a month in the 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 a list, you're, kind of, at the end. There's nothing else they can do for you. Before you're listed, they try to keep you off the list. Once you're on the list, it's kind of like, 'Well, that's it.' Number 19? Hi. Hi. Now, we're just down in Room Number Two. I'll go and grab some gear. Blood tests, I've had them my whole life, so I'm used to being pricked enough. I've got quite` I haven't got very good veins, so they usually take two or three goes to finally get blood. Right. I'll get you to press on there. Well done. Thanks, Skyla. GENTLE, HOPEFUL MUSIC '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. HOPEFUL MUSIC CONTINUES I'll get you to have a seat on that green chair 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) Fill 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 the fin up, pop it over your nostrils. And then hold on to that white part, sitting up nice and straight, bite on, seal your lips. Blow! MACHINE WHIRRS 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 up and have a rest. (COUGHS ROUGHLY) (WHEEZES, COUGHS) (CONTINUES COUGHING) (WHEEZES) Hi, Nicola. Hello. How are you? Come in, have a seat. ALL EXCHANGE GREETINGS Come on in. Come on in and have a seat. Right. How are you doing? Pretty good. Pretty good? Yep. Yeah? So, how's things been? When did I see you, November? Much change? I have felt a little, probably last couple months, worse than I have, just with breathlessness and doing stuff a bit harder. OK. 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 all over it. So it can happen any time. The outcomes from transplant is still not as good as what we'd want them to be. The average survival post-lung transplant would now be between seven and eight years, and that's one of the reasons to try and defer it for as long as possible. And, of course, we've done that with you. So, you know, we first thought about transplant when you were 14, and now you're 25. So we've done quite well in deferring it a decade. LIGHT LAUGHTER But I think the nature of CF is that it ultimately will always progress, and I guess that's where we're` or why we're, you know, in the position we are now. The principal reason why you're having a transplant is because of the improvement in the quality of your life. Something like 80% of our people will go back to, basically, being able to do the things that you want to do and not be constrained by all the treatment requirements. She's amazing. She just copes with life itself. I know she has her moments. I went home the other day and found her just in a ball of 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` I want to` She wants to do things, certain things, and she just can't do them sometimes. And it was just one of those days. She just wanted to cope, and she couldn't. SOMBRE 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. But she's got no choice. Nikki has, really, got only chance at a new set of lungs, and she's got to be well enough to` to actually go through with 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 of her organs that she's going to have to go through. And I think that's going to 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 of months, I just struggled getting my head around being on the Active List. Even though the doctor said it was the right idea, 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 READS: 'There are so many things in life that I won't get to do. '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 will 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. 'I'm officially on the transplant list. I'm scared, worried and wishing it was in 10 years' time. 'But mainly, I just want to be here forever with you, Skyla, and Willow. 'Every time the phone rings, I panic. 'It's going to be a long journey, but, Skyla, your face and smile will get me through.' POIGNANT MUSIC CONTINUES Captions by Amy Park. www.able.co.nz Captions were made possible with funding from NZ On Air. Copyright Able 2016