What is Cystic Fibrosis and why should more people know about it?

Growing up, trying to explain what was happening to my sister, was always challenging. Mostly because I always had to start by explaining what Cystic Fibrosis (CF) is, which is not the easiest challenge. Even if you try to explain it scientifically, there are a variety of elements to tackle and not everyone finds it palatable. The thing with CF is that there’s never just one issue, it comes with a mix ’n’ match of other issues that like to stack and create their own unique concoction. I’m going to break down the elements of CF as someone who, for nearly two decades, had to cherry-pick the elements which were relevant to those who didn’t understand.

The Basic Definition

The NHS website defines it as ‘an inherited condition that causes sticky mucus to build up in the lungs and digestive system. This causes lung infections and problems with digesting food.’ which, upon first glance, doesn’t really appear too serious, painful or debilitating. It sure doesn’t sound good, but by and large sounds like a bit of a chest infection for people who need to just eat a bit more food. It’s important to note that every CF person is different, some people haven’t been diagnosed until later in life, and some only start needing to go to hospital in their forties. Others, like my sister, die at 24 after a long, constant and painful battle with the condition.

The problem with CF is that can invariably be worse in certain individuals than others, the pancreatic function can have severe implications for weight gain and this combination allows other infections to affect the individual — for example, my sister contracted Pseudomonas, a bacterial infection known for causing other infections such as pneumonia and other chest infections.

Why Should You Know About Cystic Fibrosis?

According to the Cystic Fibrosis Trust, CF affects more than 10,800 in the UK, with 1 in 25 people carrying the faulty gene. While 10,800 probably doesn’t sound like loads when you look at statistics of other illnesses, it is, according to NHS England, the ‘most common, life-limiting, recessively inherited disease in the UK, with 7,700 of those living in England’. It affects around 100,000 people worldwide.

CF is a genetic, lifelong condition. It means from the day that person is born their body is battling a brutal condition that is going to kill them. Since there are over 2,000 identified genetic mutations within CF, not everyone suffers in the same way. Yet, my sister suffered from an aggressive rare mutation and a common mutation. After watching my little sister die in one of the slowest and most painful ways I can imagine, I know there is more we can be doing about that — and it starts by raising awareness.

What Will Raising Awareness Do To Help?

The more people know about Cystic Fibrosis, the more people can work toward helping those suffering from the condition prevent exacerbations. How can you do this?

A little understanding goes a long way. An excellent example includes educational establishments and teachers. Wearing a coat in the winter and colder months can be the difference for someone with CF between hospitalisation and being well.

Without deep-diving into Sophie’s primary school years, I think a great example is actually when she was in College as an 18-year-old — legally an adult. Sophie wore her coat into class because the classroom was too cold for her, and she didn’t fancy a trip to the hospital for IVs and a chest infection — she was not humiliated in class but asked to leave unless she took it off. Sadly, it wasn’t the case that the teacher did not know she had CF, but I do believe that they didn’t sufficiently understand the significance of it. It would be quite dark to assume that the teacher wanted Sophie to get sick. Yet, this was certainly not a unique scenario, this happened throughout secondary school for the sake of proper uniform wear.

Sophie went to the Conquest Hospital which was directly opposite her secondary school, called Helenswood Lower at the time. She would often miss class when she went into hospital because, well she had full admissions for several weeks for her health. One time she decided to ask the hospital if she could go in, she missed her friends and wanted to go to class — but she didn’t have appropriate footwear. She had white battered shoes she took into hospital with her that weren’t quite up to standard, but she did have the rest of her uniform. The attendance officer, in front of Sophie’s class, decided to issue a charity slip so that Sophie could ‘afford’ appropriate shoes and not to come back until she had them.

You must be thinking that this must have been a communication issue of some kind, otherwise, how could things like these regularly occur? I would come to the same conclusion except that, I’m two years older and attended the same school — they knew all about my sister down to my own attendance issues, and our mother was well-versed in school politics and making sure they knew exactly what they needed to know. The issue wasn’t that they didn’t know something was wrong with Sophie, but that they didn’t really understand what it meant. Sophie lived with her sickness, so when her body was under immense pressure and stress and pain, she looked strong and happy to those who didn’t know better.

Why Understanding Matters

Of course, it’s impossible to know about and understand every condition out there, but given the lifelong nature of CF, the precedence of it in the UK, and that understanding the condition can lead to prolonging the life of the person living with CF. Acting on that understanding can mean preventing a kid from getting a chest infection which will, in turn, exacerbate their lungs, and slowly chip away at their lung capacity.

The (Brief) Take on the Reality of Living with CF

So, why is CF so bad? Which part kills the bearer of the condition?

As previously mentioned, it chips away at lung capacity. The rate depends on the person, the conditions they put their body through (i.e. smokers, drug users, alcohol), their genetics, their rate of exacerbations, how rigorous they stick to their physio and more.

To give one example, my sister had pretty good lung function up until she turned about 12, which is when we started to see more significant drops. When she went to Reading Festival and didn’t have a good enough tent, got wet and cold, she suffered a bad infection and exacerbation and it dropped further. A year later her lung function was 30%. Lung function is irretrievable. When she had her double lung transplant her lung function was less than 10%. This means being on oxygen 24/7, and potentially having a machine that does the breathing for you such as CPAP or BIPAP. It means being in a wheelchair, not being able to wash yourself, struggling to talk, laugh, eat, or otherwise do anything.

Watching someone you love decline like that, lose weight, gasp for breath, choke on their own lungs — it’s painful. So it seems like a small thing to do to raise awareness.

References:

NHS. Conditions — Cystic Fibrosis — https://www.nhs.uk/conditions/cystic-fibrosis/

Cystic Fibrosis Trust — What is Cystic Fibrosis? https://www.cysticfibrosis.org.uk/what-is-cystic-fibrosis