Sophie - Interview 15

Age at interview: 19

Age at diagnosis: 8

Brief Outline:

Sophie was diagnosed with cystic fibrosis when she was 8 years old. She was taught by her parents to do all her own medications and treatments and encouraged to do sport. She is the Social secretary for her university Netball Cub. Sophie has a positive approach to her condition and says that she lives life to the full.

Background:

Sophie is a second year university student reading Psychology. Lives in shared accommodation with other female students. Sophie enjoys doing sport as it cleans her chest and makes her feel well. Ethnic background/nationality' British.

More about me...

Sophie was diagnosed with cystic fibrosis at the age of 8 when she got pneumonia. She was not able to understand all the implications of her condition at that time but her parents gradually started to teach her how to do her own medications and treatments.

Her experiences at primary and secondary school were very positive. Her attitude has always been to tell others about her condition and explain what cystic fibrosis is. In her experience she has found that her peers are curious to start with but soon they get used to her taking her tablets and doing her physiotherapy. 

Sophie comes from a very sporty family. She has always been encouraged by her family to take up sport and exercise. At secondary school she was captain of netball and athletics. Sophie enjoys doing sport as it cleans her chest and makes her feel well. She is now the Social Secretary for her University Netball Club. Despite having problems with haemoptysis (coughing up blood) she tries to exercise whenever possible. Sophie says that cystic fibrosis patients should be encouraged to exercise.

She attends a special cystic fibrosis unit and finds that the medical care she receives there is brilliant. She knows that if she has any problem she can talk to a consultant the same day. When she travelled abroad she was given all sorts of written information, medication and a contact number back in the UK. 

At university she initially found it difficult to get the balance between managing her condition and her independent life as a first year undergraduate. She has learned to give more attention to her diet and tries not to let herself get run down. But she always does her physiotherapy and nebuliser everyday.

Sophie has known about the life expectancy of a cystic fibrosis patient right from the beginning, and, which at present, is 41 years of age*. But she does not think of her life as a countdown, but rather sees cystic fibrosis as a challenge and does not allow it to stop her from doing the things she wants to do.

Read Sophie's guest blog to find out what she's doing now.



*The average predicted survival for someone with CF currently stands at 41 years old. This means that according to the statistics currently half of those with CF will live to over 41 years old, although a baby born today can be expected to live longer- Cystic Fibrosis Trust May 2014

She was feeling healthy but started to cough blood. Her doctors decided to conduct a procedure...

And as we were walking back I got really bad haemoptysis just out of the blue completely. I was just like walking down the street. And one second I was just talking to my friend fine about something and the next second I, I was hyperventilating, my breathing went really irregular. And quite a lot of blood came up which was quite shocking 'cause, 'cause I was just saying how well I. The day, just that day one of my friends was asking me how my chest was feeling. And I was like, 'To be honest it's feeling really, really good. I know I've been stressed out about my essay but I should really be happy 'cause my health is really good. And everything's really good.' 

Yeah [laugh] so just as I thought everything was going great I had had the odd bit of haemoptysis from now and again which was to be expected. It just, yeah just as everything was going fine blah, blah, blah. And then I started to have haemoptysis more regularly. 

Can you explain to us what haemoptysis is?

It's coughing up blood. So when it started to happen it was happening when I was feeling my most healthiest. And it kind of felt weird that I was feeling so well yet this was happening. It wasn't like I had an infection or anything or anything extra. It was just. I found it quite like hard to understand. Like why is this happening when I'm feeling so well? And it happened for a few days and then it stopped and it happened for another few days and my consultant said to me. Well we had discussed what would happen if it continued. And he said, 'Well we're going to give it another, you know, another few weeks or whatever. We'll see how you get on. We'll see how things go but if it does start to happen again more regularly'. 'Cause I've been on some transexamic acid which helps to reduce it. 'But if it, if this doesn't work and if it does keep happening, you know, we might have to do something a bit more severe to help it stop. But we'll discuss that if, if you need it.' And he explained that it would be a procedure that would seal off the blood vessels that are causing the problems. And he said that it's not actually a pleasant experience but it's something that will help and something that will stop it happening. And I went away that day and I really thought well I, I really hope it doesn't come to that because it's not something I really want at the moment. You know, I don't really want to have to have any time off uni and everything. And I went away that day and I didn't have anymore haemoptysis for a good three, four, a good month. It just, it just didn't happen again, not even a little bit, nothing. And I, I was like, 'Wow like whatever he said must have scared my body to stop and it, it's not happened again'. 

And it was my friend's birthday and we went out and had a brilliant night. We ended up going to the Curry Mile on the way home which is the famous place where all the curry restaurants are in [name]. We went and had a curry at 4 am and then came back and everything was really fine. And the next morning I woke up and I started to have haemoptysis again but it was, it was worse than before. It was quite a bit more blood and it was quite scary. 

And the next day it happened again and I spoke to my consultant and he said, 'You're going to have to come into hospital and have this procedure done. So that week I went in and I had bronchial artery embolisation which is where they inserted a catheter into my groin which went through it, my main like vein into my, which led through my, heart into my lungs.
 

On a busy day she would do her physio before leaving home and use her inhalers on the bus. Says...

Sometimes you feel tempted to do more than you should?

Yeah I've, sometimes I feel tempted to enjoy myself more than I should and maybe put. Well it's kind of hard but like for example in the summer when I was travelling for the first time in about ten years I didn't do my physio, just for one day. I woke up late and it was a really hot day and the air conditioning wasn't working so I was fed up and I was like, 'Oh I don't want to spend the next hour in this hot room doing my physio. I'm just going to, I'm just going to go to the beach. And I'll do my physio later.' And that's what I said. But when we were on the beach one of my friends, she wasn't being mean or anything. She said like, 'Do you want me to help you with your physio?' And I was like, 'Oh I'm not in the mood, like I'll, I'll do it later.' And the day got later and then it ended up that we were going out for a meal at about 7 o'clock or something and I hadn't actually had time to do my physio. And so I said, 'Well I feel fine, like my chest doesn't feel that bad like. I will just leave it for today. I won't bother doing any.' And I didn't. 

And the next day when I woke up in the morning just like my chest was so tight and my breathing was so much more difficult and it was so like. It was just really. It just, everything felt so much effort just to like do little things. I thought, 'Gosh it just shows what missing one, for me personally what missing one session of physio can have an effect on'. And it took me a good two or three days to get my chest back to normal. So at uni, like my main priority in the morning, I would never wake up and go and do something different. I always wake up and do my, my physio because I could never, ever. I know what happens if I miss it.

So I always put my physio first and almost always put my nebulizer first. There will be the odd time when I'm rushing to go out somewhere or do something. I haven't had a chance to do my nebulizer and I will literally just think, 'Well I'll just miss one dose of that for now'. So it's kind of almost prioritising. You know what's good for you but it's, it's almost prioritising, getting your right priorities right. I mean is it essential to do a nebulizer when everyone's waiting for you and you're in a rush and it's just one dose. Well I think sometimes it's not 100% essential. Other times, I mean because I've got this nebulizer because it is so small and so portable I sometimes do it on the bus on the way to university. And people might look at me and think, 'What's she doing?' But I don't care. I mean it's saving me ten, fifteen minutes which in a morning when I'm in a rush is, is a, is brilliant.

Explains her condition and her daily care routine and says how hard it is sometimes to get a good...

Sometimes I'll just say, 'Well I've got cystic fibrosis and it affects my pancreas so I can't drink.' And some of them will go, 'Alright ok.' Another people will go, 'What you've got what, what, What tell? What's that? Tell me about it.' So then I'll just say, 'Well you know it's a condition which affects my lungs and my digestive system by for it, producing excess mucus. And it makes your lungs like prone to infection and it makes it difficult to digest food because it blocks the pancreatic ducts which secrete the enzymes. So I have to take pancreatic enzymes to help digest my food. And I have to take various antibiotics to help reduce infection in my lungs and do different nebulizer's. And on a average at the moment I'm taking about 50 tablets a day of various kinds. And I have to do physio every morning when I wake up I usually do about an hour depending on how I am. But usually it averages out to be about an hour which helps to reduce the secretions and I bring up the mucus which would otherwise remain there to catch infection. 

Then every day I have to do well I don't have to but I choose to do a form of exercise if possible. For example jogging or cycling or  playing netball or some form of physical activity. Which I find when I run I, it makes me cough which helps to release all the mucus. Or if I don't do that then I'll do some more physiotherapy depending on how I am.' 

And I do enjoy keeping fit and well with my sport and exercise. And it also makes me feel like, almost it makes me feel like I am a healthy and well person despite having CF. And some people say to me, 'Oh you're fitter than me even though you've got CF'. And it was our friend's birthday last week and we were trying to blow out these balloons. And one of my friends couldn't blow up the balloon properly 'cause it was like a funny shape and I just did it without even trying. And she was like, 'Hang on a minute you've got CF lungs how can you do that?' And it was kind of, we were just joking and laughing about it. 

But after my City of Manchester 10K like I did the run. Everything was well and I went back and I was working in a restaurant in the afternoon. And then after that I went home and I was kind of exhausted. It was like, 'Gosh been a busy day'. And then I had some more haemoptysis and stared coughing up a bit of blood which was a bit annoying. It was kind of. I was kind of disappointed more than anything because I was thinking how well I was and I managed to do the City of Manchester 10K and despite having CF. And I'm really, I'm really healthy. 

And then when I started to cough a bit of blood I was, I was more disappointed in my body like, 'Why can't you support me? Like why can't you?' That's what, one of the things I get annoyed about. Like because I sometimes find it hard to get a balance between everything. I find it, I find myself getting a bit disappointed with my body when it doesn't support me all the time. 

I just sometimes find it really hard to get a balance between my social life, between my medication and like managing CF. Between my diet and making sure that I eat a good high calorie diet to keep my energy levels up and between my studying. And I just find all of it sometimes hard to balance. And just as I think I've got it all balanced and I think everything's going really well something will happen and then I'll be like, 'Oh gosh' like and I have to reassess everything. And I kind of learnt through what I can and can't do and I've learnt through you know, the experience of it all. But I still feel like, even though I've experienced, even though I feel like I know what I can and can't do I still feel like something, sometimes things happen and I think that I

She thought that alcohol didn't affect her but one time she drank a lot and ended up in hospital...

What does it mean for a person with CF to drink alcohol? I mean can you do it? What do the doctors advise you? How do you feel?

Yeah. Well I first got drunk or not drunk, I was first sick of alcohol, whether I was drunk or not I don't know, when I was quite young. When I was like, with some family friends at like a weekend away and we like snuck some bottles of Bacardi Breezers and I ended up being sick off a top bunk. And that was enough to put me off alcohol until I was about 16 like. And I started to drink socially at parties out with friends and then when I started going out round pubs when I was like 16, 17. I'd drink at the weekends. Like I wasn't drinking an excess amount of alcohol. I was just drinking about the same as all my friends and just getting tipsy and having good fun. And it was, it was, it was absolutely fine. Like it was fine having CF for me and drinking alcohol. It was. It wasn't a problem. It didn't really affect, it didn't affect me any more than it affected my friends. 

And everything was fine. And then when I was 17 it was one occasion when I had more than I'd ever had before. And that was when I ended up in hospital 'cause I'd been at a party for about twelve hours. It was a party that started. It was like started at like 4 in the afternoon and went on 'til like 4 in the morning. And I drunk an excess amount that I've not, I'd never drunk that much before. And that was when my body really reacted to it. And that's when I ended up in hospital. And that's, I've never drunk since then. And I'm not sure if anyone else has ever had that sort of experience but I don't really know 'cause I can't drink anymore.

I have had the odd drink since and I've always got the same sort of pain that I had when I had acute pancreatitis. And I just thought well it's not worth it because acute pancreatitis is such a serious condition and is so painful it just wouldn't be worth drinking for the risk of that happening again. So now I don't drink.

I luckily, I manage to have a good time without it and it doesn't really affect me too much. The only time it affects me is when everyone else is really, really drunk and I'm not really hyper. 'Cause I get quite hyper and quite full of life when I go out anyway. But if I'm tired or not, not really with it and everyone else is really drunk then I get a bit fed up. But that doesn't really happen very often. So I have a. I always manage to have a really good time without it.

She is generally open about her cystic fibrosis and thinks that is it better to tell others the...

I'm always very open about my CF. And I just told if anyone asked like for example the reason, obviously I don't drink alcohol. If anyone ever said to me, 'Oh, oh why don't you drink?' Sometimes I would just say, 'Oh because I don't want to.' But then other times I would actually explain it to them 'cause I think it's good for more people to be aware of CF. And if I feel like I don't know, explaining it to them if I've got the time and energy then I will, I will explain it to them. And I think it's good that more people are aware of it.  

What do you, what do you say?

What do I say?

Yeah.

Well say for example last night I was out with all my housemates' and all my friends and then there was another group of people there. One of the, one of the people we knew and they kind of came over and we all introduced them and then said 'Hi'. And then someone was like, 'Oh I, I'll get the drinks, like what do you want to drink?' And if I just said, 'A coke' they'd be like, 'What a coke and vodka?' And I'd be like, 'Oh no no, no just a coke'. And they were like, 'Why?' And I'd be like  'Because I don't drink.' And sometimes they'll go, 'What?' Like 'Why don't you drink? That's just weird.' Like that. It depends like different people have different responses. Some people are like, 'Alright, ok you don't drink.' And they kind of like, 'Oh like why don't you drink if you mind me asking?' And I kind of say, 'Well do you want the short story or the long story?' [laugh].

And then if they say, 'Well how does that affect your pancreas?' I'll say, 'Well when I was 17 I had got acute pancreatitis  and it's, well it's left my pancreas quite delicate and I'm worried that if I drink again then it could. If I drink alcohol then it could set it off because alcohol is, obviously affects the pancreas.  And then people always go, 'Oh right.' [laugh]. Sometimes they ask more questions and sometimes they don't but usually it shuts them up when I say I've got CF.

Last, not last week, a couple of weeks ago I was in I was in Leeds staying with one of my friends and we were at a Halloween Ball. And he was helping to organise it so I said to him, I said, 'Oh well I'll serve out like. I don't really know many people here so I'll like help you if you want'. So I said, 'Well I'll serve out the punch'. And he's like left me to it and a group of guys walked past and I gave them the punch and they were like, 'Oh do you want some?' And I was like, 'Oh no I'm ok thanks.' And they were like, 'Oh have some, have some.' I was like, 'No' and like 'I'm, I'm alright'. Thanks like I'm fine I've got another drink. And they were like trying to make me drink this punch. It sounds daft but it was, it was kind of like they were just joking around. I was like, 'No, no, no.' And, and one of them went, 'Why, why don't you want it?' I was like, 'Oh well I can't drink alcohol'. And another one went, 'What?' And I, so I didn't say I can't, I said, 'I don't drink alcohol.' 

And on of them was like, 'What you don't drink alcohol? Are you a weirdo? Are you a freak? Is there something wrong with you?' And I was kind of like, 'Well actually [laugh] yes there is something wrong with me.' But my friend that, that was with me he knows that I'm really open about it. So he was like, 'Oh she's got CF.' And it was just, everyone kind of went quiet and kind of looked at me. And I just felt really, it was the first time in a long time that I've felt really kind of I don't know, like almost a bit like touchy about it like I didn't really want everyone to know. And like I just felt that everyone kind of looked at me and was like, 'Oh right'. And

At university she found it hard to get the balance right and ended up not eating the high energy...

But when I'm, started at uni I think it wasn't the whole balancing. Well it wasn't the whole doing my medication and preparing it myself that I found  to be much of a problem. It was because I was like cooking for myself and kind of it was getting the balance between. 'Cause, 'cause everything I did was kind of, I could do what everyone, whenever I wanted I could have mealtimes when I wanted. I could eat when I was hungry and not when it was ready. I could go to bed at whatever time you wanted. Like no, you know you could do whatever you want.

And I found it difficult like managing, getting a balance between everything and like. Anyway after about six weeks I got, I started to get quite run down. I hadn't been eating that well because I was. I wasn't in catered accommodation. I was in self-catered which is what I chose 'cause that's what I wanted to do. But I didn't really enjoy cooking that much and I wasn't eating a good diet. So I started to lose a bit of weight and I wasn't getting enough energy from my food and I was still doing a lot of sport and exercise so I started to get quite run down.
 

Nurses from the adult clinic visited her at her paediatric clinic and at home before she...

Yeah I was really happy and really settled at my paediatric centre. I know, I knew all the staff there. I got on really well with my consultant and my physio and I felt. I just felt like really happy there and when we started talking about moving and I was like, 'Oh do I have to go? Do I have to go?' And they were like, 'Well yes, you are an adult now.' And it was a gradual process. Like we talked about it with my CF consultant at my paediatric centre and then we had a, we actually had a visit by two of the CF nurses from the adult centre. They came to see me at my hospital in one of my clinics. And then they actually came to visit me at my home as well and showed me some pictures of the hospital and told me what the set up was there. Like what the appointments are, the different medical staff that work there, what was likely to happen. And then I had my first appointment in the September just before I, just before I started at university. 

And although I was quite apprehensive and thinking I didn't really want to move, right from the first appointment I knew that I was going to be absolutely fine. I was shown round the clinic and explained everything. And I had my first appointment with Professor [name]. And they, like I'm really, really happy with the care I receive there. And I settled in quite well. And now it's just like it was before. I just, it's just like kind of being at the paediatric centre but it's a bit different. So yeah everything kind of is, is. Although I was quite worried about it I, I do feel like I receive excellent care there and I, I'm like happy there. And it's also quite near to where I'm at university. So when I have clinic appointments I can literally just get the bus there which is quite handy. So that's good.
 

When she was 14 her doctor explained that women with CF could have children. Feels she was a...

Yeah. When I was  in my paediatric centre in one of my Clinic appointments my doctor just said to me, you know like we can just talk about when your. He said I'd just like to have a little chat with you just because like about when you, when you're older. Just about like having children. And he was, he was really nice about. He said that you might read things or you might like see articles about people with CF getting pregnant and having babies. And he said that I'd just like to like reassure you  that there have been like many happy ending stories of CF people having children. And he just explained that it depends on whether the lung function and the health status of that patient at the time when they're thinking of having children and it totally depends on the situation and the circumstances of each individual person. So you can't just generally say that CF people will all have happy, healthy, well pregnancies. And you can't say that well CF people shouldn't have children. So he said. He explained that you can't make generalisations about that and you can't say, 'Well I don't think someone with CF should have a child'. But he explained that obviously pregnancy does put a lot of strain on your body and already having CF puts strain on your body so pregnancy could be quite difficult maintaining all your treatments and things like physio. And he just briefly explained about that. And just mentioned that it obviously depends on how, how well you are at the time. And what the consequences of it could be 'cause if you are well then hopefully you'd like to think that you'd get through the pregnancy fine and that everything would go well. But if you are feeling. If you are like not quite so well or you've got an infection or your lung function's reduced for some reason it might not be a good time at that time or in the future to have children. So he just explained all that to me and I've never really spoke to a doctor about it since. But that was when I was probably about 14. So.

And how did you, how did you take that?

At first I was quite shocked that he was talking to me about it. I was, I'm only 14. I'm not going to have any babies yet. But then I think it, I really appreciate him telling me about that because from a young age I was aware of it and if I read an article or saw something saying like CF people shouldn't have children. I'd have been like, 'What'. But because I'd, I was aware of both sides of the story then I think that was really good talking to me about it from a younger age and making me aware of the different, you know, the distant situations and circumstances.  

Her social life at university was hectic and she began to feel run down. She has learned what she...

After about six weeks of partying during my first few weeks of university I got quite run down. It was the first, being at uni was the first time I'd ever lived independently. It was the first time really I'd have to do everything for myself. And when I was at home like it was a kind of a gradual process, kind of me getting my independence with my treatment. It wasn't like one day my parents said to me, 'Right from now on you're going to do everything yourself. It became more like, of a balance like. I'd start to do, get my nebulizer ready myself from when I. Not sure how old I was but in. I'd just like start to do, you know, my nebulizer myself and start to get my own tablets out instead of my mum putting them me on the table. And it gradually throughout the years I would just start to take on more and more of treatment. And at one point, like before I left for uni I think the only thing that my parents would actually do for me is like wash my nebulizer's, mouthpieces. That's the only thing they'd actually do for me. 

One of the things I find, like I absolutely love being at university and I love the student life. I'm really happy in my house with my friends and I'm settled on my course now although I find it quite difficult but I suppose everyone finds their course difficult. But overall I'm like really happy with the uni life. I just sometimes find it really hard to get a balance between my social life, between my medication and like managing CF. Between my diet and making sure that I eat a good high calorie diet to keep my energy levels up and between my studying. And I just find all of it sometimes hard to balance. And just as I think I've got it all balanced and I think everything's going really well something will happen and then I'll be like, 'Oh gosh' like and I have to reassess everything. And I kind of learnt through what I can and can't do and I've learnt through you know, the experience of it all. But I still feel like, even though I've experienced, even though I feel like I know what I can and can't do I still feel like something, sometimes things happen and I think that I haven't really got the right balance but it can be quite difficult. But overall like I do enjoy uni so it's worth it [laugh]. I hope.

And I think that people, like if I had to give advice to anyone going to university 'cause, I would have loved someone to have said to me when I go to university. You know, you can do this, you can't do that, you can do this, you can't do that. If you do this this will happen. If you do that that'll happen. So as long as you do none of them things you'll be ok. But I honestly think that the only way to learn is to learn from experience. So if you're going to start going to university, if you're going to a, a course or something or you're going to go to uni and you want to know like, what's the best way to go about it because you want to make, remain in as best health condition as possible. I would honestly say you can only learn your own boundaries and you'll learn that through experience. Like I know now that I can't have five nights out a week staying out 'til 4 am, getting up and going on a run before lectures and not eating a proper diet. But obviously each person is different and they'll all learn through experience.
 

Look at things from the individual patient perspective. Not all CF patients are the same.

I'd advise doctors and nurses and all people that care for people with CF and any medical condition to be realistic and to look at things from that individual patient's perspective. So say you've got a teenager who has CF, try and look at things from that teenager's point of view like the experiences they could be going through in their life at that time, the type of friendship groups they may have. 

And when giving them a new treatment or considering a new type of treatment for them is to consider it in the way that it will affect them in their situation, so to make sure that you treat them as an individual and not just as a general CF patient. While for example you might say, well this is advised to give to CF patients in this situation. Well not just to look at it from that way but to think, well in this situation we've got this individual with these symptoms and this situation. And make sure that you, you look at things realistically and for that individual patient. So to treat each patient with CF not just as a CF patient in general but as an individual person who has individual needs and priorities.