Being Me: Marfan Syndrome

I just turned 13 in February, and I’m 6ft tall. I’m a men’s 11½. I have Marfan syndrome. When I was, like, 5, 6, 7, it was, like, a big thing I got bullied for, because, like, I was a good head or two taller than everyone else, and that was, like, bad, apparently. The last couple of years of primary, I sort of realised that, you know, I don’t really care, because it doesn’t change anything. My mum was incredible. She’s the reason I stopped caring about what everyone had to say. She was very, very protective. I would’ve spent more time with her. I didn’t. Like, I only spent the time I had to spend with her, which was really stupid of me. Captions by James Brown.

Captions were made with the support of NZ On Air. www.able.co.nz Copyright Able 2021 (PEACEFUL MUSIC) – Milly, the Halberg Games are coming up. So, I thought we would drive up this year and take the other kids. – I’m Milly Marshall-Kirkwood. I’m 13 years old. I live with my dad, Rob, my sister, Daisy, and my brother, Archie. – And then what are you entered in this year? – Swimming. – Discus? – Yep. – Wheelchair racing? – I have Marfan syndrome, and it affects the connective tissue in your body. And the connective tissue is like the glue that keeps your body together. In Marfan, the connective tissue is really loose and floppy. Your limbs and everything aren’t very strong or put together that well. Dad’s really strong. He’s just tough. For what has happened, he’s done a really good job. – I guess everyone’s busy, aren’t they? –

Yeah. – And we’re out here on the farm, and a couple of families are in town. Even though they’re only 20 minutes away, you know, just got a few bits of news. – Yeah, it’s nice being able to see everyone in a family gathering. Archie, one day he wants to be a fireman, and the next he wants to be a farmer, and the next he wants to be a police officer. But, you know, he’s 9, and that’s just how he’s gonna be. Daisy — I love Daisy. She’s like a little me. The poor thing will be sleeping, and I’ll just walk in at, like, 10 at night and stand at her door and have a conversation with her. And I’ll wake her up, and she’ll get really annoyed, but she’ll keep talking. – What knee do you tape? – Does it hurt when it happens? – Yes. – What did you do it? – Because I hyperextended it. – It doesn’t look any different than the other one. – With Marfan’s, we’re really tall, we’ve got really long fingers and feet and everything. – Does it hurt when you touch it? – No, it’s not, like, bruised. – It hurts when you walk, though. – Yeah, sometimes. Because of my loose connective tissue, I walk sort of on the insides of my feet. And obviously that’s not very good, because your knees clash together. So I got my feet straightened through two surgeries. I had mild scoliosis at one point. My mouth isn’t very proportioned.

I have a really small jaw. My eyesight’s really bad. It’s sort of like looking through two white toilet rolls. She’ll come home, and she’ll go, ‘Oh, well, Miss kept me in today at lunchtime. ‘Oh! And yesterday. We were talking.’ – I just got kicked out of class today because I was laughing. – Don’t get kicked out of class. – I didn’t really want to read, so… – That’s the problem. (GENTLE MUSIC) I feel like I took on a part of, like, being Daisy and Archie’s mother, which sounds really weird, because I’m 13 years old. No one asked me to. No one expected me to. But it’s just what I think I need to do, to be the older sister. My mum was incredible. I remember the day we were told that she was gonna die. I was 11, Daisy was 9, and Archie was 7. She’d made this rule, when we were 10 we got to go to a concert. And Taylor Swift was coming to Auckland. And I remember sorta like a week before we were going, she went to hospital. – It was October or November of 2018, Paula started getting some stomach pain. Eventually said, ‘I have to go to the doctor.’ The first time, they said ‘You’re constipated.’ Went back to ED about three days later, and then this time, ‘I think you’ve got a stomach ulcer.’ Then went back third time, and there was some concern that it was due to anxiety. So went back a fourth time, and I said, ‘We’re not leaving this time until we get a CT scan.’ Within half an hour, the doctor came in and said,

‘There’s evidence of cancer in your bowel.’ And within an hour and a half, Paula was in theatre having surgery. – I remember going in, and it was all very, like, bleak and high-risk, sort of emergency stuff. And no, just being told she’d had surgery, it sort of all clicked that she wasn’t coming with me to, like, the first big thing. I sat there and I was quiet. And I was sorta looking down. And after she’d hugged everyone, she looked up, and she said to me, she goes, ‘You can’t hold everything in, because when you bottle things up, ‘it just gets so much worse, to a point where you burst, and then it’s just too much.’ And so, you know, I cried, and then we went home. – The colorectal surgeon said it was big surgery, and be prepared for a long, tough year ahead. They classified it a stage four. We spent every second week chemotherapy. You know, eventually we kind of ran out of options. Paula was diagnosed in November, and she died in July. So eight months. 49 years old. – I would’ve spent more time with her. I didn’t. Like, I only spent the time I had to spend with her, which was really stupid of me. – Hello, hello. Come on in. – (DOG BARKS) – This suits you to a T, doesn’t it? – It does. – Hi, Milly. – Hi. – The great thing about Paula growing up in a household of girls is her three sisters have played an important role in Milly’s upbringing. They’ve helped to fill that gap. – Right. Milly. Let’s do your hair for dinner.

  Inside Outside: Henry Stilwell

It’s got so long. – Yeah, I like it long. – It’s pretty. We’re wearing a side part now? – I’ve been wearing a side part for years. – I thought it was a new thing. – So, Milly would have been functioning quite well for the last 18 months, but then just had a time where you could see it really hit her. She would come out wearing Paula’s clothes and wearing Paula’s jewellery, and more photos of Paula on her wall. It’s been really tough for her. – Thank you. – That’s all right. – And Milly wears them and fits them beautifully. – Have a look. See what you think. – And sometimes I actually do a bit of a double-take. I see her at a distance and think, ‘Gosh, that looks like Paula.’ And I know Paula would absolutely be over the moon to see Milly wearing her stuff. Wow! Woop-woop-woop-woop-woop-woop! Pretty good. Paula was a real staunch family member. She would organise things and get the family together. I’d like my kids and I to continue to do that. – Got Mum’s dress on, love. – Yeah. – It’s beautiful. – It’s lovely, eh? – Pretty special. You’ve often got things on I recognise. – Save me buying you more clothes. You can wear Mum’s. – I think it’s really nice when Milly wears clothes that Paula used to wear. – Yeah. – Lovely –

She’d love this, wouldn’t she? – Mm. Oh, yeah. * (GENTLE MUSIC) – We’ve been making a chair for Mum. That’s what she wanted before she passed away. She wanted it to be placed at her favourite spot at the family river on the farm so that we have a place to go and be with her. – So, the kids have been on the end of the belt sander, and oiling it. – Looks nice. It’s pretty. – Mum didn’t do boring. – We were really close, pretty much. Best friends. Because, you know, being the eldest, I spent a lot of time with her. – Grief, to me, does come in waves — comes in enormous waves. I think of it like, you know, when you suffer that big trauma, then you have an open wound. It starts to heal, and it forms a little scab on top. My deep tissue is slowly healing, but occasionally the scab gets ripped off, and it bleeds a bit. Then I patch it up, and then it comes right for a while. I call this post-traumatic growth, where we’ve undergone this enormous trauma and grief but come out the other side much better humans and much better parents and much better people. Paula was always such a great advocate for Milly. She was always looking for ways for Milly to get the best out of her life. And I have their role now, and I absolutely love looking for new experiences and new opportunities for Milly to live her life to the fullest. Go, Milly! Certainly with Marfan’s, you get a lot of hyperextension of joints. Knees can pop out and ankles can pop over and roll back, whereas the wheelchair is great — get the heart rate up. It’s relatively safe, it’s lots of fun, and competitive, which is a good thing. So, when she was born, her feet had a little bit of a turn in. Part of the skull was quite soft, outside of the fontanelle. And she just sorta didn’t feed very well.

And because she was long and thin, we knew something wasn’t quite right. We ended up going to one of the doctors, got a blood sample sent to Sydney, and then within about six weeks it came back saying she did have Marfan syndrome. That just opened up a whole new world for us. Go on, Milly! Go, go, go! One of the biggest risks was cardiovascular concerns. At 9 months old, she had the aortic root the size of a 10-year-old. So Milly underwent a mitral valve angioplasty. Certainly pretty traumatic — a young toddler with open heart surgery, and then watching her recover was pretty traumatic. Since then, her heart has just got stronger and stronger and stronger. It’s been remarkable. – I don’t know how Dad’s dealt with being that, like, single parent. I have no idea. I think he tried probably too hard to be strong. He’s done really well at supporting us through everything. – After Paula’s diagnosis, you know, you start looking at what the future looks like. And for me, I was thinking, ‘How on earth am I gonna raise three kids by myself… ‘and manage the house, manage the land, manage my job.’ – So, you guys, you got a water bottle? – Yeah. – Yeah? Excellent. You got a change of clothes? – Yes. – What have you got? Jandals? – Yeah, I’ve got jandals. – ‘As a solo parent, you don’t get a lot of downtime. ‘So I have this constant battle.’ Do you want a banana or not? ‘I always think any spare time I have, we should be doing something together.’ So you do have to take some selfish time. Because if your bucket’s not filled, then you can’t help anyone else. Have you got your bag, too? So I thought I needed some help around the home. And I’d known people locally had had an au pair. And so I found this amazing young woman, Shenay, who’s a local girl. She’s been living with us for the last six or eight months. And it’s just changed our world. She loves the kids to bits, and she’ll just come over in the mornings and help the kids get ready for school while I go to work, and then is around when the kids get home. Shenay and Milly get on really well. There’s seven or eight years’ difference between them, but Milly’s so mature, they share lots of interests and go riding together and do different things together. It’s really nice. – Shenay is — I wouldn’t say like an older sister, cos I don’t know what it’s like to have an older sister — but I’ve got lots of older cousins that are like her. I’d go to her if someone had said something that didn’t affect me — cos, like I’ve had lots of people say, like, stupid things. My legs are a lot weaker. They’re really strong for what I’ve been through and done and everything, but they’re really weak for family biking, like, activities. Before my heart surgery, I couldn’t bike down the driveway. I couldn’t even get halfway, cos I’d run out of breath and my feet would hurt. You know, I’d be stood in the middle, just in tears, because Archie and Daisy had already done three laps. * (GENTLE MUSIC) – In high school, there are lots of kids taller than me. And I guess I look a lot older than I actually am, so I don’t get that young tall kid. It’s sorta like, yeah, she could be, like, a year 13, and she’s 6ft tall, and that’s sorta normal. It was quite obvious when I was little because I wore big splints up my feet and they went all the way up my legs, and I had casts, and I would always go out of the classroom for doctor’s appointments. I always had a teacher aide, and I wasn’t allowed to go play, or I wasn’t allowed to play rugby. I had to be supervised doing this or doing that. Little kids — like 5, 4, they don’t care. But, like, 9, 10, 11, it was, like, ‘Oh, yeah, you’re not normal. You’re weird,’ you know. And that went on for a while. I just sorta had to learn to not care. You don’t go change for anyone. – I guess the great thing about Milly going to a country school where everyone knows her and has seen her in a wheelchair, seen her on crutches, seen her go away for periods of time, now that Milly’s at high school, not only does she know the people in her year group, and they know her, they know Milly’s medical condition. So if someone says something rude, then she comes back with something equally… profound. Cos she’s so smart and so quick-witted. – She’s not afraid to be herself. – Yeah. She’s very cool to be around. – She’s not afraid to give her opinion, but not in a bad way. – Likes to help. – You know? – Mm-hm. You’re, like, my go-to therapist. – In high school, it’s not a big deal being different. I’ve got good friends, and I’m glad I’m not any other way, because I feel like I’d be a completely different person, and I don’t want that. – Milly, we’ll just go and pot a few pots up. – Fred, can you get the potting mix? – I like my nanna and pop the most. That’s where we always used to go stay when Mum and Dad went up to Palmerston for chemo. – Here we go. – They live in the house my mum lived in when she was little. It just feels, like, right — their house, them. – Milly was 5, at her 5th birthday party. Paula told us all then that it was a wonderful birthday for Milly, because she’d turned 5, and she wasn’t expected to live past the age of 2. And she’d never, ever, ever told us. She had a wonderful mum. Paula made her feel just like any other child, which I think was a good thing. Because Paula was very strong – So, that’s another one to go to the grave site. You have to make big holes. The way she’s going to high school, and she’s enjoying that… – But she’s very confident. Before, when Paula was here, she used to act in plays and that. And I went with them one night, and Paula said, ‘Oh, Mum, you come too, and you’ll see what Milly does.’ Arrived for the first night. Didn’t know a soul. And she soon got in, and she was playing the part of this and playing the part of that, whereas I would never be able to have done that at that age. – That’s the way Paula had brought her up, and Robbie. – Yeah, Paula had brought her up to be very confident. – She’s very likely she’s had such a good grounding. – Milly, that’s yours. – Thank you. – Are you enjoying high school? – Yeah. – And it’s so handy for you, eh? And you’re e-biking home? – Yep. – Yeah. – You haven’t had any crashes? – No. Not recently. – She used to go to lots of things. Paula got her into playing the piano. Yeah, that’s right. – See, Paula maintained that she’d never be able to do contact sport and that sort of thing, so she was really keen— – But we all reckoned she had fingers to play the piano. – She could do other things, and she was always keen on getting her into other things that she could do. – And then she got her playing the violin, which I loved doing that. That was lovely. – It’s been quite a big week for your, Milly, eh? Stressful. – Pop and I will grab it. You guys just grab those boards there. Put the board under here now, love. We’ve got an amazing place on the banks of the Maketawa River. We just call it the picnic ground. What was important to Paula was we would go there, you know, weekly or every two weeks and have a picnic, or light the fire and have some sausages or toast marshmallows. And so that place is significant to the family. So it’s important we continue to go there. Right, we’re good to go. Reverse in. People would say to me, ‘Oh, your kids are so resilient. We don’t see them upset and angry and… confused. You see them when they’re out, amongst others. You know, they’re all hurting. And I think in Milly’s case, it was just time. I see this amazing, young, beautiful woman growing, being proud of her upbringing, being proud of her mum, being proud of what has come since Paula’s death. I just wanna, you know, continue to raise three great human beings that make some great decisions. And I hope that they are connected to the family land. – The river has been, like, a big thing for ages. I’ve got photos of me when I was, like, 4, sitting there with a rash top and a sun hat with my cousins, and swimming. And she loved the river a lot. You could ask any of my family, and you’d go, ‘Yeah, Paula loved the river.’ She was, like, the one that loved it the most. It’s a great seat, isn’t it? It’s come up well, and that’s exactly what Mum wanted. She wanted it perched here, looking down the river. Marfan syndrome now, the life expectancy is no different to any other person. So it’s got a normal life expectancy,… as long as there’s no other complications. So in Milly’s case, she’s got a really strong, stable heart now, after surgery. She’s got no issues with lungs, no other major organ issues. So I would expect her to live a long and full life like the rest of us. I’m super proud of her. She’s really grown. She’s an amazing woman. She’s come a long way. Captions by James Brown. Captions were made with the support of NZ On Air. www.able.co.nz Copyright Able 2021

  Inside Outside: Cory Newman

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