Cystic Fibrosis


No two people with CF are the same, so my experience of living with it may completely differ to another person's - however, here is a quick overview of facts and symptoms from the official CF Trust website, where you can find out a whole host of information: 

  • Cystic Fibrosis (CF) is one of the UK's most common life-threatening inherited diseases.
  • Cystic Fibrosis is caused by a faulty gene that controls the movement of salt and water in and out of the cells within the body.
  • Cystic Fibrosis affects the internal organs, especially the lungs and digestive system, by clogging them with thick sticky mucus. This makes it hard to breathe and digest food.
  • Over 9,000 people in the UK have Cystic Fibrosis.
  • Over two million people in the UK carry the faulty gene that causes Cystic Fibrosis – around 1 in 25 of the population.
  • If two carriers have a child, the baby has a 1 in 4 chance of having Cystic Fibrosis.
  • Over 95% of the UK CF population is Caucasian, but Cystic Fibrosis affects many ethnic groups.
  • Each week, five babies are born with Cystic Fibrosis – one in every 2,500 births.
  • Each week, two young lives are lost to Cystic Fibrosis.
  • Currently half of the CF population will live past 41 years of age, and improvements in treatments mean a baby born today is expected to live even longer.

  • CF and Me

    Just a handful of my daily medicines!

    I was diagnosed at 2 years old after my mum had been fobbed off by other doctors, so CF is all I've ever really known. I'm kind of glad I know no different because the treatments are pretty full on and if it's something you all of a sudden have to take on it would be very overwhelming. When I was about three I had two operations, one was for a portacath, which I wrote about here. The other was for a feeding tube. With CF a good weight is essential to help maintain a good lung function but just to be a pain in the butt CF makes it very difficult to gain weight because the body has to use more energy to fight off infections. So I guess one perk of CF would be that you do get to eat all the fun, fatty things that other people should avoid but honestly, having to eat double the amount of recommended calories a day is actually harder than you think! Plus I have 'lucky' skinny genes so weight gain is so difficult for me but the feeding tube has been a massive help. When I get ill I really struggle to eat so without it I'd get no calories at all and would just get more and more ill! It's basically a tube in my stomach and at night time I attach it to this machine that feeds me nutritional goodness while I sleep. 

    My tube, which I was in two minds posting a pic of. It's something that massively affects my confidence more than I let on, I don't think people really realise how it makes me feel. It's totally ridiculous that I let it have such a negative impact on my life because 1) without it I'd be so unwell and would look like a bag of bones 2) no one can see it and  3) it's absolutely tiny! It's waaay tinier than the one I had when I was younger (why was everything in the 90's so big and bulky?) Something so small shouldn't have so much control over how I feel about myself. I'm working on it though and posting this little pic is a huge step for me. I used to hope that one day I'd be able to gain enough weight and keep it on so that I could get rid of it but because of my genes and having CF I don't see it happening somehow! But I'm sure that once I got rid of it I'd soon find something else about my body that I hated, I'd get that fixed then move onto something else and it'd just be never ending! I'd end up looking like Jackie Stallone haha.

    The white machine is the feed one, minus the actual feed medicine stuff. Next to it is one of my nebulisers. 

     As I said, the treatment regime is pretty full on, An average day for me goes like this:

    • Wake up and do a nebuliser and take my morning pills (about 15 minutes)
    • 1 hour later I do two more nebs, my morning physio and another neb (about 45 minutes)
    • Around 6pm I do another neb (about 10 minutes)
    • 8pm it's time for two nebs, more physio and another neb. Then my evening pills (about 45 minutes)

    That's when I'm well! If I feel crummy then I have to do more nebs and physio throughout the day, which is becoming a more regular occurrence these days, plus I'm on IVs a bit more these days. A nebuliser is the machine you can see in the above pic and there's also one in the first photo too (the little blue machine!) I pour a little vial of liquid medicine into them which they turn into steam that I breathe in, they all help me to breathe easier...without them I don't know what I'd do! Physio is chest physio to clear my lungs of rubbish (CF is not sexy) for me that means breathing techniques although there are many different ways of doing it, you just need to find what works best for you! Nebulisers are fine because you can watch TV, read blogs or whatever whilst you're doing them but physio requires your full attention and it's the most boring thing in the world! I also have to take pills called Creon every time I eat something fatty, for example a big roast dinner will need about 5 Creon and a bag of crisps will need 2. They're basically enzymes that help me digest food because my body has trouble doing so. All my other tablets are for various things, like vitamins etc.

     A few years ago I was also diagnosed with CF related diabetes, I think it's like a mixture of Type 1 and 2 but don't quote me on that! I've been lucky enough that this doesn't cause me too much trouble, all I have to do is inject insulin once at night time and keep an eye on my blood sugars. I take tablets for my liver as I have mild CF related liver disease which doesn't cause me any problems thankfully. I've had surgery on my sinuses as this is also a problem that CF can cause but thankfully for the moment it's all under control. 

    At the moment on average my lung function is around the mid-30's percentage wise, on very good days this has occasionally shot up to the late 40's and when I'm ill it goes down to the low 20's. I think 70+% is considered 'normal' for someone without any health problems just to give you some idea of how my lungs are a bit crappy! I now have to sleep with oxygen and frequently use it during physio and exercise. When I'm ill I can be on it 24/7. My lung function wasn't always so low, it's declined over the years as a result of being damaged by frequent chest infections and illnesses. I don't think CF affected me as much as a child, it was when I got to about 14 I started to miss a lot more school. I had to drop out of university after one year because I'd gone to one an hour away from home which was just too much to try and handle. So then I worked for almost two years part time in retail and loved it but for the time being I'm taking time out to concentrate on getting my health a bit better. But I am running an Etsy store selling handmade greeting cards, you can check out Frojo Designs here!

    Well I'm not really sure what else to say! There's so much more I could write, including the effects CF can have on your mind (but you'll find plenty of that in my CF Posts!) but I'll leave it there for now. Hope it's been somewhat informative and don't hesitate to ask questions if you have any!

    Update: In September 2015 I was told I needed new lungs so I'll be blogging about my experiences with this too. I received my shiny new lungs in July 2016, you can read all my transplant posts here. Also, it turns out I was able to get rid of that tube after all, woohoo!


    1. Josie, what an interesting and informative post. I respect the positive attitude you have towards your health and I really admire your courage. xx

    2. I can't imagine the impact that must have on day to day life, but I admire you for being so upbeat as I imagine alot of people would feel self pity! I think I would. I'm sorry you had to quit uni, but as you said, your health is so much more important! xx

    3. I really admire you for being so upbeat and matter-of-fact about this - I found the post really informative and I wish you all the best with your CF :) I also didn't know that you used to live in Canterbury, that's where I was born and currently still live. It's a small world I guess! xx

    4. Wow I am so glad I came across your blog. It's so inspirational to see someone upfront about illness, I myself suffer from a chronic illness, admittedly a lot less nasty than CF but have never had the guts to do what you do. Having a blog is like a little escape, something so easy to do when you feel too run down to go out! Really admire you!xo

    5. Such an inspiring post and os brave of you to put it all on the internet! It's nice to see someone suffering to not be bogged down about it but happy to explain and talk about it. I know someone with CF and she's spent all of this year in hospital - it really is horrible and lets hope one day you'll be able to get the tube out of your stomach! ox

    6. You are so so inspiring and brave! xx

    7. You are so inspiring! This post has been so very informative and its so great that you seem to be keeping positive. Keep your head up high, you are so brave, and I must say very pretty at that too :) I shall be clicking the follow button at the end of this post.

      Love Becky xxx

    8. such an inspiration, puts my life into perspective! xx

    9. amazing post. you are such an inspiration.
      wow wow wow.
      great blog, so cute!

      Love Anna

    10. I have so much respect for you, I can't imagine living like this, and the fact that you manage to have a blog and a job makes me all the more awed by you. It really puts what I have in perspective. Your health is something that's so important, but a lot of people take for granted. I am currently taking biology AS level, and one of the topics is about CF, so it's nice to be able to think of a person in your mind when you're learning about something to stop it seeming so abstract.
      Love, Katie xx

    11. Wow you're such a courageous and strong person. Reading this I can't help but be totally overwhelmed by how inspirational you are and how positive you are. I am truly awed by your immense strength and determination, you seem like such a wonderful person.

      xoxo Bree
      The Urban Umbrella

    12. You're an inspiration. So positive and level headed xo

    13. I'm really sorry to hear that you have to go through all of this! I had a girlfriend who passed away at 23 with CF, so I have seen the effects first hand. She was truly a beautiful person inside and out, but she neglected her health and unfortunately it got the better of her :( I'm glad you are sharing your story and raising awareness. Keep on, and stay healthy :)

    14. Dear Josie,

      I came across your blog because I was actually trying to find a pari of new balance trainers in burgundy, and your picture came up in my google search!
      You're blog is awesome, and I'll definitely be visiting more often.
      My brother has cystic fibrosis as well and I'm a med student, so can emphatise a lot with this page.
      Glad to hear about your lung function, keep it up!
      Abi xxx

    15. Found your blog quite by chance. You're very brave and I wish you the very best Josie. It's a horrible disease - but keep fighting, little one. I suffer with asthma, so I understand a little of what you go through. Take care and be happy. Andrew

    16. I lost my friend to a mixture of CF and swine flu just after her 20th birthday, you're so brave for keeping a positive outlook on life; I wish you all the best and hope everything goes well for you Josie! Love reading your blog too!

      Beth xxx

    17. It's really brave of you to be so honest about your condition - I've heard how horrible CF can be, and I'm sure posting this will help others with the same condition to feel less alone, plus just make people more aware in general!

      P.S. Just stumbled across your blog, and it's lovely :) All the best!
      Helen at Hels Yeah! xx

    18. I just wanted to say that I think it's firstly so brave to be so open about what you have been through with your CF and the portacath and to give such a good explanation about what CF is to people who may not know what it is! You've definitely done it better than any doctor I know!

      Saskia /

    19. I send you the biggest hug ever. You're so brave and I'm glad people are open about these things. It was super informative, too. I didn't know much about CF, just very basic stuff. You explained everything perfectly. I wish you the very best :)

    20. I'm really glad I can across this blog after you commented on one of my posts (thanks by the way!). An old friend of mine used to have CF but a very mild version of it- she just had a few pills and physiotherapy once a week. Thank you for being so open about this, its good to make people aware of these things. xx

    21. I suffer from chronic illness and have recently set up a blog too, I think it's really admirable that you're so brave and positive in the face of illness and I find it so inspiring to see other sufferers approaching their road to possible recovery with such determination and an open mind. Keep it up, sweetheart!

      Meg | Meg Says

    22. Chronic illness is something many of us deal with, but CF must be a real bummer. I love your attitude though, Josie. You are handling it with style. :) Love your blog!
      Jennifer x

    23. You have an amazing attitude for someone living with CF, I know it comes with it's struggles. Very inspiring!


    24. Josie I think you are so strong to do this every day! You are such an inspiration and thanks for sharing your routine with everyone! I love your blog btw, can't wait for future posts :)

    25. I cannot express how inspiring this piece of writing is to me. I have type 1 diabetes which is an absolute struggle to cope with, giving myself the correct amount of medications to avoid dangers of fits/comas. However you cope with your CF alongside having a form of diabetes and have such a positive attitude with both.

    26. You are such an inspiration! Thank you so much for sharing this and letting me know so much about something I had so little knowledge about. Keep up that positive attitude of yours :) xx

    27. Just read this, and had tears in my eyes reading it! You're such an inspiration, I can't imagine how difficult it must be for you! Thank you for informing us all on CF, I had no idea before I read this, so thank you for being so open:) I love your blog & posts, one of my favourites. Keep it going :) xx

    28. I just found this post. You are really brave to post a picture of your tube. I had a NG tube for a while as a teenager and it really knocked my confidence when I went out. I was going to have a PEG but thankfully I didn't in the end. I think it can only be good that you share your story, it means people don't see it as this big scary thing, if that makes sense?
      I had never heard of a portacath til today so thank you for explaining it. They make sense now I think of it, if you need medicine on a daily basis.


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