If you’re a reader of my blog or follow me on social media, you’ve probably seen me talk about my life with Cystic Fibrosis (CF).
Lately CF has been in the news quite a lot but there are still a lot of people out there who don’t know what exactly it is or what it actually means to live with CF.
In this post I want to change that.
I really hope by the end of this post you’ll understand what CF is and if you ever meet someone with CF you’ll be able to understand a bit about them and their life.
What is Cystic Fibrosis?
I’m going to quote a CF website here as it explains it much better than I ever could…
Cystic Fibrosis is a progressive, genetic (you are born with it and it’s not something you can catch from someone) disease that causes persistent lung infections and limits the ability to breathe over time.
In people with CF, a faulty gene causes a thick, sticky build up of mucus in the lungs, pancreas and other organs.
In the lungs, the mucus clogs the airways and traps bacteria leading to infections, lung damage and eventually respiratory failure.
In the pancreas, the mucus prevents the release of enzymes which help to break down food and absorb vital nutrients.
People with CF are at greater risk of getting lung infections because thick sticky mucus builds up in their lungs allowing germs to thrive and multiply. Lung infections mostly caused by bacteria are a serious and chronic problem for people living with CF so minimising germs is a top priority!
Here are some facts about CF which might be a bit easier to digest:
- Cystic Fibrosis is one of the UK’s most common life threatening inherited diseases
- CF is caused by a faulty gene that controls the movement of salt and water in and out of the cells within our body
- CF 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 CF
- Over 2 million people in the UK carry the faulty gene that causes CF – that’s 1 in 25 of the population
- If 2 carriers have a child together, there is a 1 in 4 chance of their child having CF
- Over 95% of the UK CF population are Caucasian but CF affects all ethnic groups
- Every week 5 babies are born with CF that’s 1 in every 2,500 births
- Every week 2 lives are lost to CF
- Currently half of the CF population will live past 41 years of age (this was 30 when I was born!) and improvements in treatments mean that a baby born today is expected to live even longer!
I found this picture online and it just highlights how CF isn’t just a one trick pony…
As you can see, there’s a lot to handle. A lot of people say having CF and managing it daily is a full time job and I totally agree.
There is so much involved daily to just stay stable and even then you’re not guaranteed you’ll escape an infection…
I will be posting a ‘Day in the Life’ type post later this week so you can understand just what I have to do every day to stay alive.
What symptoms are there?
People with CF have a wide range of symptoms but no two people with CF are the same. One person could suffer with a lot of the following symptoms and another could just suffer with one or two.
- Salty skin.
Woe to that child which when kissed on the forehead tastes salty. He is bewitched and soon must die.
This is a quote taken from European folklore and is a very early reference to CF! I myself don’t really taste salty (yes, I’ve licked my arm and nah, nothing) but others I know with CF claim they do. I’m not going to lick them to find out though haha!
- Persistent coughing.
This is annoying AF as I have constantly have a cough, doesn’t matter if I have treatment for it or not. Mucus is always waiting to be brought up off my chest and I cough to get it out!
I found it most annoying when taking my school exams in the exam hall. Do you remember how people would fake a cough then their mates would fake cough too then you’d have a hall full of fake coughing going on? Well some people thought I was doing that when I coughed in exams and then they would fake cough… But yeah, it’s very annoying in quiet places for a long period of time.
- Frequent lung infections including Pneumonia
The build up of mucus traps germs and then the germs develop into infections. The key is to keep coughing the mucus up so it doesn’t get chance to develop into these infections.
Normally I would get an infection approx. every 3 months. I could try fighting it off with tablet medications but repeated use of them now means they don’t work much anymore or I go into hospital for antibiotics given through a vein for two weeks.
- Wheezing/Shortness of breath.
I get this occasionally when I am ill but it is a very common symptom of CF.
- Difficult bowel movements.
Yep CF can affect my toilet habits… What a great thing you now know about me hey? Because my food cant get digested properly due to mucus clogging up my pancreas I would have constant diarrhoea if I didn’t take artificial enzymes (which are actually pig enzymes!!)
People with CF can also go on to develop several related conditions (CF really is the gift that keeps on giving right?!). They include; Diabetes, thin/weakened bones – Osteoporosis, infertility in men and liver problems.
Is there a cure?!
There is no cure for CF out there, only treatments to help manage it.
There are a couple of treatments that have been developed quite recently which have shown very promising results in slowing down the progression of CF but they are VERY expensive. The government here in the UK aren’t keen on funding the costs of it…
People are trying to fight this decision as in the long run it would actually save the government money on NHS costs such as inpatient stays in hospital.
CF patients wouldn’t have to need as many antibiotics…
I have read great things about this drug and pray that I get it one day, ’til then I have to keep working hard to keep my health as stable as possible.
When a person with CF’s lungs become so damaged and scarred from years of infections they can be listed for a double lung transplant. This in itself is not a given and there is no guarantee they would even make it onto the transplant list to get lungs as there are many tests to go through to determine if that person would be suitable for a transplant.
Transplant truly is the gift of life and I’ve recently seen how much of a difference it can make to a person who has struggled to breathe for many years.
One of my friends with CF 2 months ago received that gift of life and now she can run with her dog and plan her wedding. She can sign up to a beauty course at a local college and start to actually LIVE again whereas before she was virtually housebound and needed oxygen to breathe.
Signing up to the donor register is the most amazing thing you could ever do in life. Live life then GIVE life!
My CF Story
I was diagnosed with Cystic Fibrosis when I was 18 months old so basically I don’t remember life being any different to what it is now…
My mum took me to the doctors as I kept having diarrhoea (niiiiiiiiiice) and wasn’t putting on weight. They decided to do a test that had only recently come out to see if I had CF. It was, and still is, called the Sweat Test. This measures the amount of salt in the sweat of a person, in people with CF it is abnormally high.
Along with that test they also did a genetic test to see if they could find the faulty CF gene in my genetics.
It is possible to be a carrier of Cystic Fibrosis but not actually have the disease. It can run in families and there is a one-in-four chance the carrier can pass it onto their own child if they have children with another person who also carries the disease.
This may explain it slightly better…
If you are worried that you could be a carrier (there are no symptoms so you wouldn’t know) and are planning to have children in the future with your other half then you can ask for a genetic test to see if either one of you are a carrier.
I always encourage friends and family members to get tested as I wouldn’t wish this disease on my worst enemy… Please consider it.
Back to me…
I was very lucky throughout my childhood, I only had to go into hospital once for antibiotics but I know of people who spent their childhoods on children’s wards. I’m forever grateful that I didn’t.
I still had to be seen by my team of doctors/nurses every 2 months to check I was doing okay but other than that I was very lucky to have led a relatively ‘normal’ life.
Things started changing when I was 13, probably because I was in high school now with a lot more people. More people = more germs and what do more germs make? Yep, INFECTION. Good… you’re learning!
I started needing antibiotics every 3 months for 2 weeks at a time. When I look back at that time now I don’t know how I did it… A child being cooped up in a room on a ward for 2 weeks (I was allowed to go home for a few hours at the weekend), it’s just WRONG. Glad to say things have moved on and children with CF are encouraged to have that treatment at home via their parents who are trained to administer it.
I did that all through my teenage years, sadly, up until I passed my driving test and I was free to come and go from the ward as I pleased. I cannot tell you the difference it makes…
I had my first operation at 15 to put in something called a portacath.
My veins were/still are wrecked from the thousands of needles I’ve needed over the years to pump antibiotics through so this was a more permanent fixture. It is connected to one of the main arteries.
It honestly is my best friend so when it needed replacing years later I wanted it doing asap.
You cant see the box, it’s inserted under the skin so to look at me, you wouldn’t even know I had one.
My second operation was to fit a feeding tube in my stomach. This was something I’d been dead against for YEARS but my weight at the time was rock bottom and this was the only solution.
I was awake for the whole operation and it wasn’t pleasant, they’d cut into muscle so every time I moved afterwards it was SO sore. But it turned out to be one of the best things I EVER did.
This is it below. It’s placed just above my belly button and it’s a total lifesaver when I’m really ill and I find it difficult to eat.
I just connect it up to a pump and it pumps a liquid filled with HUNDREDS of calories into me overnight as I sleep. And no, I haven’t ever tried putting alcohol down it, I cant tell you how many people have asked me this over the years!!
I was diagnosed with CF-related Diabetes about 4 years ago now. I knew it was coming as my test results had been showing signs of it developing for some time. It didn’t really faze me to be honest, it was just another thing I had to learn and get on with.
I do have to inject insulin every time I eat a meal which is annoying when you are starving and just want to eat all the delicious food straight away. But if I don’t inject, I’m absolutely exhausted for hours after.
But then I can go the other way where my blood sugar goes too low… This is extremely dangerous as I can go into a coma and die if I don’t treat it by eating things that will bring it back up again – hellooooooooooo jelly babies!
So there you have it, I really do hope that you understand a bit more about my life now and the things I talk about over on my social media!
Life is hard with CF but I am determined to not let it take over!
If you want to learn a bit more about Cystic Fibrosis click HERE