Growing up with Cystic Fibrosis

It all starts at a diagnosis, many children and adults with Cystic Fibrosis failed to thrive after entering the overwhelming reality of life; but there is some exceptions. Many babies like myself may have struggled to catch breath or gain weight, which sent doctors and nurses frantically looking for answers.

I was diagnosed very early on as I failed to thrive and gain any weight. After numerous tests the doctors did a sweat test which determine my fate (Cystic Fibrosis ΔF5080)

I can’t begin to imagine what my parents must of thought, after being bombarded with information and questions. Nothing really prepares you for bringing up a child with Cystic Fibrosis. It’s a condition with no guidelines but your full of questions with little answers.

It’s a learning curve none of us are prepared for because Cystic Fibrosis affects everyone differently. So whilst you listen to the facts and statistics; just remember no one explains the different mutations. So how can you base your child’s life on a number or percentage?

Growing up with Cystic Fibrosis (CF) had its ups and downs. My parents always tried to make life normal as possible. I never allowed my condition to to define me or stop me from growing up normally. At school I was known as the girl who ‘ate bird seed’ which was eight year olds referring to my creon capsules. My parents organised a routine with school which allowed me to leave class fifteen minutes early and take a friend for support. So I could go to the school office and take my medications. I was everyone's best friend when it came to deciding who to choose, hahaha. Whilst being at primary school I managed to avoid being in and out of hospital except for one occasion. Which helped me to continue living a normal lifestyle. Rarely people asked questions and I just continued to swallow tablets like it was normal, however the foul taste still haunts me. As a child I had a fear of swallowing tablets so all my prescriptions were requested in liquid format which caused my complications leading up to my diagnosis with ABPA.

At ten years old I became extremely ill with what everyone describes as a ‘sea lion cough.’

You ask why it is called that? Medically speaking I was diagnosed with ABPA after spending two/three months in hospital. I had a dreadful cough which sounded like a sea lion bark, x-rays showed significant changes on my lungs and the hospital wanted to act fast. After several intravenous antibiotics (iVs) which didn’t improve my symptoms. The doctors could only conclude one explanation... my weight. They pinpointed the lack of improvement down to my weight loss. Until I was referred to a specialist CF team in Leeds St James Hospital, who tried several tests including a bronchoscopy which finally gave us some answers. This was when I was diagnosed with Aspergillus (ABPA) which finally started to make sense. Aspergillus was sensitive to a drug called Voriconazole which I had never had before and concluded why I failed to improve from intravenous antibiotics. Unfortunately voriconazole was only available as a tablet, something that always seemed very daunting. For months I showed no improvements on voriconazole, little did everyone know I was hiding the tablets and pretending to swallow them. Until I was eventually found out and this resulted in me having to overcome my fear. From then onwards, I did improve and I returned to living my normal life with a few added treatments.

As I grew up friends got more inquisitive about my condition but it was something I could barely explain. Yes, I lived with CF but no one sat me down and said ‘this is Cystic Fibrosis.’ For years I blew off my condition, playing it down as a bad cough or cold. How was I supposed to explain CF when to me it was normal? Like most of us do, there became a point where I tried to do some online research. Worst mistake ever! Imagine being told at twelve years old you’re not expected to not live past your thirties. That information hit me like a ton of bricks, all of a sudden I felt like I was living on borrowed time. At that age no one explains that CF is a progressive disease that eventually gets worse. Every hospital appointment is to track your progress and manage infection. As a twelve year old girl the most important thing to me was leaving the hospital knowing for once I could leave not being admitted for intravenous antibiotics. My fear had been settled until next time. Little did I realise back then, this was my life and the hospital will start to feel like my second home.

Something I have learned whilst growing up is that life will take you on unexpected journeys but one thing it will never prepare you for is Cystic Fibrosis. Everything I know about CF has come from my own experiences and no two stories are the same.