This article is more than 4 years old
We won't stop until everyone has access to the best treatments
When I was a little girl, I remember my consultants and nurses talking about a ‘miracle drug’ that would eventually help ease the burden of treatments, hospitalisations and more regarding cystic fibrosis. It wasn't something I really thought about until I transitioned to adult care... but then the prospect of a ‘miracle drug’ came sooner than I imagined it would.
Before I explain anymore of my whirlwind journey, I would just like to say that these are my personal experiences. I would hate for anything I have experienced to deter anybody’s decision to take this drug, but hope that patients and loved ones alike resonate with at least something.
Getting the news
At first, I believed that I was not eligible for any of the modulator therapy drugs (Symkevi or Kaftrio) due to my CF-related liver disease. I was a little upset at first, but hopeful that something would be out there for me at some point. I mean, look how far CF treatment has come in 10 years right?
It was a summer’s day in Lockdown 2020 (1.0!) when I got the call to say I was eligible for Kaftrio! I was gobsmacked and speechless – believe me, that does not happen very often!
'A burst of energy'
The day came – October 3rd. A day I had been waiting for all my life without even realising it. A beacon of hope quantified in three little pills. It was completely overwhelming. I remember taking the two pills in the morning alongside my mum as I held her hand. Although I was excited, I was somewhat walking into the unknown! felt this burst of energy, my chest almost felt light weight. I was breathing so much deeper and lighter… all in a couple of HOURS! I was in disbelief. Weeks turned into months and this continued.
Around Christmas, I decided to go for a run. I came back and my mum’s eyes were welling up with tears. I turned to her and asked if she was ok. She replied, ‘I am ok because you are more than ok. Look at you go’. In that moment, every hospital stay, every sleepless night, every cough and every painful tear somehow became worth it. I am not naïve, but it felt like I had made it. I felt safe and a little bit free from cystic fibrosis.
'Hero to zero'
I wish I could say that things only got better from here… but unfortunately, it didn’t. I’m quite a complex patient, but I don’t think my CF team, nor I anticipated, what would happen in the next few months. From January to April, I was in and out of hospital. Due to raised liver function levels because of Kaftrio, I had to adjust my dosages. This meant that my chest was no longer stabilised, and I somehow experienced an aggressive and constant purge. I had to go on 24-hour oxygen for the first time in my life after reaching 83% lung function. I was coughing all day and all night; I lost a huge amount of weight, then gained it back all at once; I had to go on two different IV antibiotics, one IV antifungals a huge increase in steroids… I think you get the drift.
Physically, I had to adjust again. Admittedly, I think I am a bit of a pro at adjusting to unfortunate situations – but this just felt cruel. I had gone from hero to zero in a matter of months. I felt useless, like I had let my body down. Was I not capable to handle this drug? Was it something I did? Has my body become too reliant on certain drugs that I am now not on? I was constantly playing a game of 20 questions with myself!
Undoubtedly, this affected my mental health. As I slowly began to build my physical health back up, my mental health started to decline. I began to experience low moods. I began to experience physical anxiety – heart palpitations, feeling nauseous and panicking all the time. I felt angry and resentful of Kaftrio. I soon realised that these emotions had slowly been creeping in since starting Kaftrio, but the stress of the last few months had made them rise to the surface. I felt empty within myself despite crying almost every day. I was crying because I was so unhappy – my body was changing, and I didn’t know how to process the physicality of weight gain and hair loss coupled with the mental challenges of being able to do things I have always wanted to do but feeling stuck and scared to.
From the outside looking in, it may seem like I am being ungrateful. I promise you; I am not. If you live your life a certain way for 22 years; knowing your limitations and your capabilities regarding your life – it is the most foreign adjustment to realise you can do what you thought you couldn’t. Personally, this ranged from sleeping on one pillow to acknowledging I can leave home and live independently! Bizarre. I think a lot of people assume this adjustment is easy and an overnight process – it is very much ongoing.
Understanding the negatives as well as the positives
After more hard work and determination, I’m glad to say that physically I am back on form. As someone with CF, I know that it can sometimes take time to return to a comfortable physical condition but for me, I know I am luckily able to reach my ‘normal’. I’m pleased to say my lung function is back to 80%, my liver is stable, my exercise tolerance is amazing, and my energy levels are at an all-time high. I am in regular talks with my health psychologist which helps massively. I would implore anybody with CF on their kaftrio journey to speak to a psychologist through the good times AND bad times.
I think my hope for the future is that health professionals and individuals begin to realise that this drug is not a guaranteed fix. In my opinion, I believe that the positives were very much over highlighted whilst the negatives were massively unexplained. I do understand this – but the psychological and social side effects need to be explored and explained in my more detail. I would hate for another young woman or man to experience what I have mentally, but I am glad I have so I can try and help people on their journey. In the same vein, I wholeheartedly want any person with CF to experience the joy of breathing deeper and living life to their fullest. Living by their own means, as opposed to the constraints that CF can impose on them.
I truly believe that Kaftrio has helped our physical conditions and has highlighted what more needs to be done psychologically and socially. Cystic fibrosis does not just affect our bodies, but our minds and souls too. It is important this is recognised within the community so we can achieve a fulfilled, engaged and healthy life.
After a year which has affected the CF community so deeply, we want you to know that we remain totally committed to improving the lives of everyone with CF – that's why the theme of this year's CF Week is all about how we won't stop. Help us continue our work by getting involved in with CF Week or making a donation to support people affected by cystic fibrosis.
Please do talk to us if you are struggling with any emotions around the subject of Kaftrio – we are here to help. You can speak to your CF team about how to access more support with this if required, or you can contact the Cystic Fibrosis Trust Helpline.