Cystic Fibrosis

WHAT IS CYSTIC FIBROSIS (CF)?

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!