13 April marks Cystic Fibrosis Ireland’s annual fundraising appeal, 65 Roses Day.
The appeal, rather sweetly, gets its name from the way children with this condition often mistakenly say the words “cystic fibrosis”.
Ireland has the highest incidence of CF per head of population and some of the most severe types of the condition in the world. According to the most recent Cystic Fibrosis Registry of Ireland annual report, there were an estimated 1,339 people living with CF in Ireland in 2016.
That's roughly one person per every 50 people affected with the disease. For a time in history when most people's Facebook friends often rise into the thousands, that's a lot of people.
To find out more about what could often be considered a secret chronic disease, JOE spoke to Brian McCarroll, aged 29, from Walkinstown, Dublin.
Brian has cystic fibrosis and he was lucky enough to be on the clinical trial for Orkambi for six months not too long ago.
The Orkambi effect
JOE spoke to McCarroll about the process of being chosen for the Orkambi trial and how the aftermath affected his life.
"Yeah, well I was in hospital at the time I was asked to take part in the trial anyway, and so I had to take a trial to get onto the trial if you know what I mean," McCarroll began.
"Anyway, after all that I was accepted as a candidate in St Vincent's Hospital after passing a load of tests. It was brilliant being told I was accepted – a great day for my whole family, since you know that even if you do get the placebo, you know you'll have an answer in six months."
Brian's enthusiasm is palpable. Speaking about the way the trial changed the way he lives his life is as encouraging as it is a joy to listen to. The trial made a huge difference to his life in that he has far more energy and less infections.
"I knew right away from when I started the trial that I got Orkambi, because straight away I felt better," he continued.
"I was bursting with energy, no tiredness, didn't have that groggy feeling waking up in the morning. Family members noticed that I wasn't coughing, my weight went up, I was able to do more stuff in the gym, eat more food. Straight away I was flying from it. My body accepted it really well."
Within six months, life had improved dramatically for McCarroll; his lung capacity, previously at 40% at the beginning of the trial, had increased to 72% on Orkambi.
CF is an inherited chronic disease that primarily affects the lungs and digestive system.
A defective gene and its protein product cause the body to produce unusually thick, sticky mucus that clogs the lungs, leading to life-threatening lung infections and obstructs the pancreas, stopping natural enzymes from helping the body break down and absorb food.
Day-to-day life
Brian has to spend about three hours a day managing his cystic fibrosis. This involves everything from taking nebulisers, digestive enzymes and vitamins. However, this doesn't stop him from really living life.
McCarroll spends a lot of time in the gym trying to look after himself and is looking forward to undertaking a triathlon soon. Diet is also hugely important to him too – he eats a whopping 4,000 calories a day.
"I spend about two hours every day solely clearing out my lungs and chest. I'd spend a lot of time preparing and eating food, as I need the nutrients and find it hard to put weight on. As someone with CF, you need to take tablets constantly to help you absorb the nutrients in food," Brian tells JOE.
"I'd eat twice the amount of a normal person and would have to rest quite often. However, it's not just taking a tablet and you're okay, it's a whole process, CF treatment. I'd spend between two and five hours managing it daily but generally I'd take everyday as it comes."
The difference the Orkambi trial has made on Brian's life is quite outstanding. From the get-go, he noticed a vast improvement in the way his body reacted to the drug.
"I just wouldn't be groggy in the morning time. That's usually what CF feels like, even though I'd be fit enough. My neighbours even noticed, as I was walking up the road, that I'd colour back in my face and had a look of health about me.
"Since the Orkambi trial I've been in hospital twice in five years. Before that it was about two or three times a year for about three or three weeks at a time, so it's a huge improvement."
Cystic Fibrosis and mental health
While CF is primarily a disease which affects your physical health, mental health also comes into play when something as simple as waking up in the morning comes more difficult than it should be.
"I felt a bit of a lift because I knew myself in my mental state that I'm gonna have this drug even for six months, and even if I'm not on it, they'll know more about it very soon.
"The thing with CF is that you can't plan. With Orkambi I could start planning for things and look forward to things people take for granted like events or holidays."
What really helps Brian keep things under wraps is fitness and exercise. An avid gym-goer, Brian also, rather incredibly, completed the mammoth 602km Malin2Mizen cycle for Cystic Fibrosis Ireland in May 2017 – a huge achievement for anyone.
However, not too long after this, he was admitted to hospital for two weeks. But it was worth it, he says – “you got to live your life”.
"After Malin, I spent two weeks in Vincent's on a course of IV antibiotics. I was told to rest and recharge, as well as getting my exercise in. Although as a recurring patient, I was able to exit the hospital independently once I got the go ahead from the doctors, so I'm lucky enough to be able to do that when I'm in.
"[After the cycle] I had an appointment booked, I knew myself that I'd need to go in. It wasn't a scare or anything, it's just normal enough for me. I was sweating at nighttime which is usually a sign that my body is brewing infection."
"The test results I got [afterwards] weren't actually too bad. I did the cycle but I was also training beforehand so that helped."
Brian then tells us cheerily about his plans for the future, reminding us that it's the simple things that people take for granted which are top of his list.
"I've never let [CF] affect me once – I always have a smile on my face and wouldn't say I suffer or anything. My sister has CF as well, she's 36. I also have two other sisters [who don't have CF] and there's never been a difference with how we treat each other.
One of his ambitions is to have his own place to live and to have a family. However, with cystic fibrosis, access to a mortgage is very difficult. Brian is on the medical housing list and very much hopes that, one day, he will be granted the key to the door to the next phase of his life.
Over the past year, he has enjoyed travel, in particular island-hopping in Greece, so much so that he and his girlfriend, Jen, intend to see some more islands later this year.
His hope for the future is “to be able to take less medication and to limit the hours managing my CF each day. I also want to see younger children with CF have the best treatment and facilities.”
