'I won't give up after my life-long struggle'
Orkambi has transformed lives, and we must fight on despite HSE rejecting its use, writes CF sufferer Daniel McDonald
Published 29/11/2016 | 02:30
Twenty-seven years ago, I made my arrival on this earth. All appeared to be well - until the confirmation of a bowel obstruction indicating cystic fibrosis (CF) brought the world of my nearest and dearest crashing down.
A sweat test confirmed a diagnosis of CF, a genetically inherited disease predominantly affecting the lungs and digestive system. The build-up of mucus due to a faulty gene results in clogging of the lungs - the pancreas is also obstructed. Around 1,200 children and adults live with the condition in Ireland. Average life expectancy here is approximately 38.
Initially, family members found themselves in a state of perplexity. I had five other siblings who were miraculously unaffected by the condition.
Having a terminally ill child was new territory to my shell-shocked parents.
But they rapidly resolved to provide nurture for their newborn bundle of joy - to the best of their ability - with endearing commitment, strength and affection. They thought it imperative that I was provided with a normal, functioning childhood. Upon starting school, they insisted to teachers and school staff that I wasn't to be wrapped in cotton wool.
Whilst aware of my susceptibility to cold and flu, encasing me in a bubble wasn't an option. They actively refused to let my CF define who I was as a person. CF, in their view was not the gross impediment that national media had portrayed it as. Not only was I emboldened by their approach - I embraced it.
Adolescence followed a happy, care-free childhood. But it was at this point that CF would really show its hand. I endured my first hospital admission at 13.
It turned out to be one of the most traumatic periods of my unpredictable life.
One ordinary night, whilst doing my maths homework, I experienced severe haemoptysis (lung bleed), a common complication of CF.
A mild cough suddenly resulted in large volumes of blood from my lungs. It was horrifying - I was screaming aloud for my father to come at once. I was in the dark as to what was happening to me. I hadn't ever anticipated this. My father's natural instinct was to rush me to A&E - providing me with a bundle of tissues en route to help soak up the bleeding.
I was admitted to hospital - and the haemoptysis slowly began to dissipate. I awoke the following morning in a shared ward (prohibited for those with CF due to cross-infection risk) to the presence of five elderly men. Two appeared simultaneously agitated and confused in beds each side of me. My heart was in my mouth. I kept my eyes firmly shut to keep back the tears. My father had to depart the previous night due to work commitments, but I longed for his company like an apprehensive child on their first day at school - I was racked by this vicious new fear. Suddenly, without warning - at just 13 years old - you're forced to confront your looming mortality. For the first time, I had to confront becoming another statistic of CF.
Thankfully, the following years brought prolonged reasonably good health. I had considered myself lucky as the horror stories other CF patients found themselves in were chronicled in the media. It was a damning indictment on our health service.
But I had discovered a new-found equanimity with life and CF in general. I had surmounted my fears and conquered obstacles that lay in my path. I had acquired an affinity for attending hospital appointments, regularly consulting with members of the CF medical team - resulting in a pro-active, positive approach to keeping CF in check.
In September 2015, CF tested me to my limit. The months prior had been turbulent health-wise. I was hit by debilitating flu. The virus penetrated my lungs - my body felt like it had suddenly become a lead balloon. I found myself in a hospital bed, gasping for breath. My lung capacity had plummeted to 23pc. But I was adamant I would claw back my lost lung function.
After this fright, I vehemently vowed to meticulously take control of what I could.
Today, I adhere to a strict routine consisting of various nebulisers with additional airway clearance - along with a mammoth quantity of pills.
Being compliant with secreting mucus from the airways is a fundamental must in cystic fibrosis.
Halting permanent lung scarring for as long as possible is crucial.
A wholesome, balanced diet composed of good fats also applies, and eight hours of adequate sleep.
CF is not the alarming death sentence it was once interpreted as. Yes, it poses a huge challenges and is defined as a life-limiting illness - but I will defy the general consensus that exists relating to the condition. Somehow, you reconcile yourself to a terminal prognosis - taking it in your stride. I refuse to acknowledge that I have a short expiry date. Patients are surviving longer than ever with advancing new innovative medicines.
I had hoped, and will still hope, to access the new drug Orkambi, despite this crushing news.
But I won't give up, not after all this struggle. It has the potential to enhance and prolong the life of those with CF. Despite the odds for all of us, it is critical to endorse a positive and buoyant approach to life as a person with cystic fibrosis.