What is Cystic Fibrosis?

What is Cystic Fibrosis? Well, where do I start?! Even after 23 years, I still struggle to find the words to explain this very complex illness, I don’t know whether I am the only person who feels the same?!
 
So here goes…Cystic Fibrosis (CF) is a genetic illness (I’m not a fan of the word ‘disease’) which affects a lot of our body. Every person with CF is different so we are effected by it in different ways, we all have different genotypes so no person with CF is the same. It affects everything from our lungs, to our digestive system to the liver and sinuses. I could go on about all the ailments that CF gives us so here’s a picture courtesy of CF Aware :
 
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If you want to find out more facts about Cystic Fibrosis, then please visit the Cystic Fibrosis Trust’s website

My Life with CF

One thing I make sure I don’t let CF do in my life – define who I am. I know that CF is a huge part of me but I make sure that I don’t let it take control of my life too much.
CF also involves me having regular clinic appointments at the Cystic Fibrosis Adult Centre in Llandough Hospital in Cardiff, this involves me doing a range of tests including a lung function test, X-ray, blood tests and also seeing a multi-disciplinary team, which includes a doctor, CF specialist nurse, physiotherapist, dietician and possibly the psychologist and social worker. Clinic appointments usually last for about 2-3 hours at a time, but that can differ.
 
The way I describe Cystic Fibrosis is this – it’s like being on a 24 hour shift at work with no breaks in between. From the time I climb out of bed in the morning to the time I climb into bed at night, I am doing treatments, taking different medication, doing exercise and physiotherapy just to keep my body as well as I can. At the moment my dining room table is covered with my medication and spare IV medication:
 
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The ‘I’ Word

Yes, the I word… IV’s! IV’s (intravenous antibiotics) are every CF person’s worst nightmare. IV’s are needed when your lungs are feeling a little on the rough side. Every person with CF has a different experience when it comes to IV’s, but on the whole, it’s not a nice experience!
 
You always need a minimum of two weeks of IV’s, but it may last longer depending on how unwell you are. When I have IV’s, I have a needle inserted in my portacath. A portacath (port) is a little device that is placed underneath the skin, this can be placed under general anaesthetic or keyhole surgery. Attached to the port is a long line which is fed through a large vein which leads to the top of the chest.

I had my port fitted in when I was 11 years old as my veins were completely broken because of the catheters and long lines wrecking them when I was on IV’s. Having the port fitted was the best decision I ever made, it meant having less stressful and less traumatic IV stays in hospital, my port is over 11 years old and is still going strong (touch wood!).
 
So, the needle is injected in the port and it needs a lot of dressing and tegarderm (clear plaster) to cover it to make sure the needle stays in place and to protect the needle from getting any infection in it.
 
I usually have two IV medication, Meropenum (Mero) and Tobramycin (Tobi). I used to be on Ceftazidime but my doctor felt I needed to change to Mero. I inject Mero three times a day along with saline to help flush the line and I have Tobi through a pump once a day which lasts for an hour.
 
When I moved to the Cystic Fibrosis Adult Centre in Llandough Hospital in Cardiff, I made it my mission to learn how to do home IV’s. I managed to learn in my first hospital stay in Llandough and i’m so glad I did learn, it’s nice to IV’s with all your home comforts around you.
 
Even though it’s great to do IV’s at home, my dining room table takes the brunt of having all the IV meds on it for two weeks!
 
IV’s is a very stressful and trying time, especially as IV’s make you feel constantly tired, and you have to work twice as hard to get yourself back to your best, so it’s never easy.

 Are You Allowed To Eat That?

Probably one of the most common questions I get asked in relation to my diabetes. Yes, I am a diabetic. I got diagnosed with Cystic Fibrosis related diabetes (CFRD) when I was 11 years old.

Now, CFRD is neither like type 1 or type 2 diabetes. Even medical professionals outside of Llandough Hospital look at me with a complete vacant look when i tell them that I have CFRD.
 
CFRD has the components of both type 1 and type 2 diabetes. So it’s important that my blood sugars are within a good range otherwise it could lead to a hypoglycemia episode (hypo), that’s when the blood sugars go down below a certain level, for me personally when it goes below 4.0. But it’s also important to make sure that the blood glucose level doesn’t go too high, for example above the level 10.0. Trying to get your blood sugars on the right level is like trying to get the air bubble in a spirit level in the middle.
 
In order to do that, I have to inject myself with insulin (Lantus – 11 units) every morning before I eat breakfast. I’ve been doing that every day for the last 11 years, when I first got diagnosed with diabetes, I never fully understood how serious it can get, but thankfully I’ve managed to keep it under control for so long now. I also inject a fast acting insulin called Novorapid before every evening meal to help bring my blood sugar level down. Injecting yourself everyday always comes at a price – bruises. If you inject in the wrong place, you can guarantee there will be a bruise on my belly the next morning. It’s important to inject in different parts of my stomach everyday, or it could lead to little lumps in my stomach called Lipo’s (Lipohypertrophy). Without injecting insulin I would become very ill and end up in a diabetic coma.
 
But to make sure that I’m getting the correct amount of insulin, I need to do blood sugar tests every day. So I use my glucose monitor, do a finger prick, alternating between different fingers for each test I do a day, otherwise that results in more bruising.
So that’s how my diabetic life works, as well as fighting CF, CF decided to give me diabetes and I’ll be battling them both for years to come now.
 
So there you have it, this is what Cystic Fibrosis is! If you have any questions please feel free to message me.