What CF Was Like Growing Up

Due to my late diagnosis as a teenager, I cannot talk about multiple hospital admissions, endless physiotherapy or the smell of disinfectant or missing school during my early childhood.  On the contrary, my school attendance record was quite good, there was no hospital visits and the only smells of disinfectant were from our kitchen or bathroom after my mother had cleaned them, and playing football everyday was my only physiotherapy! I was a regular normal kid on the face of things, going to school, playing with friends scraping knees and falling off bicycles. Cystic fibrosis to me back then was a dangerous couple of words used to describe sick people, that was all I knew about the condition pre 1999.

Painful stomach cramps and a consistent cough, particularly at night, the only signs that something wasn’t fully right, but back then in Ireland you just got on with it, the cough became more my trademark and nothing really to worry about.  Multiple doctor checks over the years failed to find anything seriously wrong. On many occasions I given antibiotics and sent on my way. At one point an inhaler was prescribed but a stubborn ten year old me saw no need for it. They were for the guys in school short of breath after climbing some stairs, not for me who could run as fast as the wind and barely break sweat.  As I was approaching my seventeenth birthday a local GP suspected something more serious when I presented for an appointment during my school summer holidays, that persistent nightly cough becoming too much for my family as I barked frequently during the night. A few hours later I was being treated for a chest infection in hospital and monitored with testing. Some days later my life would change with my diagnosis of cystic fibrosis after a routine sweat test.

Related Story:
  → → → My Irish Childhood

The Predictions

Many people with CF grew up hearing life expectancy statistics. I had thankfully missed out on that stage but at seventeen what would my future look like?  I don’t know exactly what the doctors told my parents, I just remember them chatting while the next steps were discussed. There were no numbers, no promises, just a plan of my future care.  For me there was no planning ahead, just to get out of hospital and enjoy the rest of the summer.  I would be passed onto the care of the specialists at St. Vincent’s University Hospital, Dublin and they would be able to give me more information from start to finish, all this as I prepared for my final year in school and the road to adulthood.

The Emotional Impact

It is really hard to quantify the emotional impact, at the time I really wasn’t too worried. Taking medication wasn’t really an issue, just a couple of seconds to pop a few pills while I was eating. Reading books about the condition probably brought and raised some concerns, particularly the life expectancy which was about thirty years old at the time. Even though it was a very young age, it was still a lifetime away from the mind of a seventeen year old.

I feel I would have been the same person regardless of the diagnosis but at that stage I didn’t look too far ahead and done what I wanted to do at that particular moment in time. My first real eye-opener that life could be different, was my first admission to hospital four months after my initial diagnosis. Just a routine check with my specialist ended up with a two week stay in hospital. Two weeks out of school, away from friends and family and stuck in a hospital ward.  It certainly was a bit of a shock to the system and I started to feel like this would be the new normal now, I had only gone for a routine three month check up and now I would be admitted attached to a drip several times a day!

Looking back at those early days in hospital they were simply crazy. What was happening back then in Ireland wouldn’t be accepted these days but they were strangely fond memories and the start of gaining real knowledge into the condition. Today we are more aware about infection control and the importance of isolation away from other patients. Back in 1999 there was a common room in the hospital where all the patients with CF would meet, hang out, eat together, watch tv and chat! It would be unheard of these days but back then it was normal. Through these interactions with patients I got to know more about the illness and how to cope. I was worried that every cough or sneeze would mean another hospital admittance but the other patients put my fears at rest. Between treatments some of the patients used to go across to the local pub for a few pints, it sounds mad but that was the attitude, just get on with it and try lead as normal a life as possible.  In addition, I shared a ward with cancer patients, those recovering from surgery and those recovering from flus and pneumonia, certainly a world away from the isolated rooms of today with private bathrooms, shower and TVs.

Diet was another unique aspect of the illness. We were encouraged to eat high fat meals when possible and take on as many calories as we could to maintain weight and fight infection. Rather than frowned upon, fried chicken, chips and calorie dense treats were encouraged. Today we have more nutritional information and at times the high, fat diet is necessary, but with advances in food transport and supply, we can now fuel the body more carefully and nutritiously to ensure we get all our daily requirements. Having cystic fibrosis has actually made me more aware and studious, with food science and nutrition a particular interest of mine. Finding ways to improve the functionality of the body without the need for medications a real goal of mine.

Related Story:
  → → → A Day in the Life with Cystic Fibrosis

Part Two: The Revolution – Treatments Changed Everything

To be honest, as the years progressed I was lucky, if there is such a thing as being fortunate with cystic fibrosis! I didn’t require any hospital admissions for over ten years after 1999 and was fortunate to hold down a full time job, travelling and living in Australia for a year and backpacking the US, and Asia. By the time of my next hospital admission in 2010, I was now twenty eight years old and fast approaching that thirty year old life expectancy. By that stage, the life expectancy with the illness had increased due to advanced treatments and research, but still there were many dying too young with the condition around the world.

Up until that point, my treatments had remained almost the same from my initial diagnosis, I had been on long term antibiotic use for years but I made my own decision to come off them around 2010. Having researched more with the internet now well up and running I decided long term antibiotic use was not ideal, particularly for my gut health and with increasing antibiotic resistance in healthcare settings quite evident. I turned more to exercise and hitting the gym as well as playing football.  More research into nutrition and diet led me to cut out many foods which I thought were benefitting me, but on reflection were not, and overall listening to my body and not necessarily relying on pharmaceuticals.

Soon after, I was being tested for a new breakthrough drug, ivacaftor, which had the potential to ease many of the symptoms, particularly the lung infections.  Research teams from the US came to my hospital in Dublin and a battery of tests were performed. The first day taking the drug was really exciting, I couldn’t wait to see if there would be any differences. The road to obtaining the tablet was not easy for the 120 Irish people with CF who were deemed suitable for treatment. It is estimated that treatment of a single patient with the new ‘wonder drug’ costing around €234,000 back in 2013. Now don’t get me wrong, I am grateful that the “government” covered the costs, but frankly that’s what we elect them for, to protect their citizens. I am more grateful for the hard work done by sufferers and the families of the sufferers themselves who tirelessly worked hard for many years to find a cure. I am also thankful for the gifted and talented medical professionals who made this wonder drug possible, maybe that college education isn’t such a bad thing after all! I understand research and development costs a lot of money but I still think it is disgusting to charge nearly a quarter of a million euros per person for a life changing drug. It is because of these high costs that many governments around the globe rejected the drug with campaigners fighting to bring about another life changing drug, Orkambi for the many other gene types.

The main, almost immediate benefit of the drug was the reduction of my daily cough and increased lung function. Now I could sleep soundly without waking to cough my lungs up, a joy for me and my wife. Before the breakthrough I was probably on course for more progressive lung damage but now there was hope the damage could be slowed down or stalled.

Related Story:
  → → → The Road To Kalydeco The Little Blue Pill

Part Three: The Present – Learning How to Grow Old

New Problems, Better Problems

So here we are 2026, I know I am reaching older age when I start reflecting on the past and believe things were better in my day! Certainly they all were not, particularly the treatments for cystic fibrosis as discussed. I am not overly happy about modern life and the direction people are going in the world, but throughout history patterns sadly seem to repeat. I don’t like to focus on politics and take news headlines with a pinch of salt. If you were to believe everything on the news there would be no point in leaving the house each day! In a world with more and more fake people it is hard to stay real, but I try and won’t sugarcoat or bullsh*t.

Getting older with cystic fibrosis has posed challenges I never thought possible back in 1999.

• Joint aches.
• Careers.
• Mortgages.
• Retirement.
• Looking after ageing parents.

Things many people with CF never expected to face, certainly not me.

Identity Shift

Questions such as:

• How do I plan for a future I never expected? This perhaps has been the biggest challenge. Not expecting to live into old age perhaps shaped my plans in the early days. I had no real interest in going to university or college but probably with the expectancy that a long successful career was out of reach, due to my condition, the motivation wasn’t there. I didn’t want to waste more years in a classroom only to ultimately not enjoy the job I had studied for.
• Forming relationships. This was another potential minefield, having girlfriends breaking up and meeting new potential partners. Do I open up early about my illness? Do I persist with long term relations if I don’t think I will be around? And children, what if a new partner wants children. Do I want them? Can I have them? Do I pass on my defective gene?  All issues you need to take in your stride and learn on the job.  Fortunately, I was quite open early on with my now wife about what life with me may be like. In the end, her support, big heart and encouragement has been more beneficial than any drug on the market. Having someone to be by your side at doctors appointments, be there when I am not feeling so well and being my biggest cheerleader is a blessing.
• What does success look like now? Success now is getting up out of bed in the morning and feeling energized. Success is completing my daily workout at the gym, success is training and playing football with my team, and success is seeing another country on my bucket list.  All quite simple really, financial success comes and goes but health and life experiences truly are what motivates me, you cannot bring the cash to the grave after all!

The Joys of Ordinary Life

Celebrate ordinary things:

• Although I have no children of my own, watching my nephew grow up from a newborn who I held in my arms to a teenager has been incredible. Despite not living in the same country and talking everyday, I still love to hear how he is doing and am proud of the young man he is becoming. It is remarkable to see how much he is like his mother and father and taking on their different physical and character traits. Seeing he has an interest in the world and countries shows there is definitely something in the family genes and it helps that he supports Manchester United too!  It has been a privilege to be there by his side at his communion and confirmation and I hope in the future I’ll be there for his 18th, 21st and perhaps his own wedding.
• Grey hairs. To be honest, this has been the biggest issue. Call it vanity, but it is not until you start losing your hair colour that you really see how good you looked in the past and how father time catches up with you. Now going grey wasn’t exactly a shock for me. All my family from my father, to aunties and uncles had full heads of grey hair throughout my young years. It would always be my fate but not expecting to live to old age meant I thought I would probably die with my black hair intact.

Call it a coincidence, I got married at thirty and that’s when I noticed my first grey hair! Seriously though, it was only one or two and as the years went by I had a little help from some shampoo that slowed the process down. Full on hair dyes I found were just not going to work with the fake look not really doing it for me.  As my thirties progressed the greys kept coming but not at a rapid rate, I figured that if I can last into my forties that would be fine with me, a fair deal.  Having reached forty with my black hair intact I was pleased, but father time was doing it’s thing. Fast forward to today, I am forty four and now grey is taking hold at a rapid rate. To be honest I am embracing the change and enjoying the new look but it’s hard not to look back with fondness for my dark hair on my head and on my face.  Hair dyes look too fake and don’t really help the structure of the hair so I don’t want to follow that path. I am at peace with the change and focusing on the privilege of this new chapter in my life, certainly one I didn’t see back in 99. Grey hair, wrinkles, bring it on, all signs of a life lived.

Part Four: The Future – A New Kind of Hope

Hope for the Next Generation

Back when I was diagnosed in 1999, the last century! There have clearly been a lot of changes for me personally as well as many changes in the world. I’m not going to say it was a simpler time, maybe it was in many ways but there are no easy times in life. Everyday, decade and era providing different challenges, nobody escapes with an easy life. The first main difference is the life expectancy from 1999 to now, approximately 30 years back then to approximately 65 years today! That is a major improvement and offers huge hope to those diagnosed daily.

The internet was available back in 1999 but again it was nothing in comparison to what we can do on it today. It gives patients the opportunity to connect with each other without compromising their health and infection control. We can now support each other and share our experiences which wasn’t readily available years ago.

What life looks like for children diagnosed today? I can’t personally comment fully on this as I don’t have children, but I would like to think that the opportunities are there and that a diagnosis today, while traumatic, doesn’t have to mean the end of the world.  Earlier interventions, better treatments and potential gene therapies in the future mean the world is a lot more brighter than it was in the old days.

Personal Hopes

For now, more travel is firmly high on my agenda. Making more memories with my wife and family and simply living each day is what I seek.  Birthdays can come and go, they are just a milestone and a number but it’s about the quality of life lived, not the years you have on the clock. I know some people who have lived more life in twenty years than some who have lived eighty, but then what is living a life? We are all bound in some ways by society and what is expected, going to college, getting a job, getting married, having children, the mortgage, pension fund retirement and death.  We are told what to eat, how to behave and rules to obey, but what truly is right or wrong? For me, as long as you don’t harm anyone intentionally and have respect for all living things, then that is a good start. Some will soar in their careers, others won’t, some will have children and property, others won’t. Some will find success in the shiny new car or big house, some will be happy to have the sun on their back and little else.

It’s a road we all need to navigate and some paths will be less bumpy than others. Life doesn’t come with a manual, and if it did most of us wouldn’t read it anyway! Take each day as it comes and go from there.

Conclusion: The Privilege of Growing Older

Many people complain about getting older, but for those with CF it can feel different.

Every wrinkle tells a story. Every grey hair is evidence of years I wasn’t guaranteed. Growing old isn’t something I fear. It’s something I spent a lifetime hoping I’d get the chance to do.