Adam's story: "Kaftrio has been the most significant change of my life"

What was your diagnosis like?

I was diagnosed not long after I was born, I had a chesty cough which wasn’t getting better, and after several trips to the GP was admitted to our local hospital by which time, I had a collapsed lung and was very unwell. Thankfully I recovered with treatment, eventually being diagnosed with CF at around 10 weeks. Luckily, we live quite near to a hospital which has an excellent CF team and I was referred to them.

What was it like growing up with CF?

I guess that dealing with the enormous load of treatment becomes the norm to anyone having to live with CF but I tried to never let it become a massive hindrance on my life growing up. My mum and my dad are super supportive, and my family are amazing, so that made growing up a bit easier. I also had a solid group of good friends who knew about my CF and never let it become an issue.

Despite regular IVs during my childhood I’ve always been fairly well and able to do most of the things I wanted to. I was always doing sports, playing rugby and swimming growing up, so I think that helped my health. It was challenging because of the level of treatment required, which of course is never pleasant, but I didn’t have to stay in hospital too much as I had home IVs which didn’t interrupt my time at school.  I remember my veins were a total pain and they’d take forever to find a good vein!

As a child, people would never be able to tell and they’d be shocked to find out I had CF. I think people often think of the worst cases when they think of CF so they’re always surprised to meet me. In some ways I think a lot of who I am now is because of my CF.

What was taking Kaftrio like?

Before Kaftrio was available, I’d had a real decline in my lung function to below 40% and was prescribed Orkambi on compassionate grounds.  My lung function did improve which was a relief, but the first few months of taking it was really rough, I was working full time and dealing with constant headaches, nausea and fatigue. The side effects didn’t last thankfully.

Kaftrio has been the most significant change of my life.  I was a bit apprehensive about taking it; I remember seeing posts on Facebook forums from people in America who would post saying they’ve just got Kaftrio and people would be commenting things like ‘your life is just beginning’.  To be perfectly honest I didn’t really believe it at first but eventually I thought, okay, let’s do it, and I started Kaftrio.

I remember taking it and within hours I felt different in my body. I had the initial purge of clearing my chest and after that it was just insane. I’m obviously joking, but also a little bit serious when I say that it was the closest thing I felt to having a superpower - I just felt amazing. I had so much energy, I was breathing easier, putting weight on, and everything felt unreal. My lung capacity went up 1.2 litres in a matter of weeks and I wasn’t even exercising or doing anything significant.

I actually did end up seeing a psychologist for a bit. I was feeling so good that it almost went full circle and I started to struggle a bit with the change in my health and what it meant. She was really great and helped me through it and told me there were a lot of CF patients going to her with the same problems because regardless of the change being a good thing, it was still such a massive change.

What made you start to focus on your fitness?

I think with the success of Kaftrio and feeling so amazing, I started to get complacent. Before Kaftrio, exercise was my physio and I was always running, swimming, surfing or something. When I went on Kaftrio and had such good results I started to drop my exercise and relax a bit, thinking I didn’t really need to do it anymore.

I didn’t feel really poorly for about two years post-Kaftrio but then at the start of this year I got really unwell with the flu, more unwell than I’d ever really been before. My lung function was really low, I couldn’t get out of bed, and I was pretty much wiped out for about three or four weeks. It was quite scary to be honest and I think it was a bit of a wakeup call for me. I kind of thought I was immune in a way, being on Kaftrio, so it was a real reality check.

I spent the next month getting my strength back and recovering and then in June at one of my checkups they said my blood sugar was a bit high, and I was at risk of diabetes. It was another thing that reminded me that I do have CF and I’m getting older and I need to do something to keep myself as well as possible.

In July I started to make a conscious effort to really try and harness the power of Kaftrio and see what was possible.

How have you found your fitness journey?

It’s been insane. In 2013 my lung function was the highest it’s been, it was about 4.05 FEV1 and since then it had been declining for the last ten years. I wasn’t expecting to get back to that level but more just maintain what I do have for as long as I can as I continue getting older. But I’ve surpassed it! I can’t really believe it but I’m working hard and putting in effort, I don’t feel like I’m doing anything over the top but I’m being consistent and doing what’s feasible for me. I’ve now hit 4.13 FEV1 which is an all time best and feels truly amazing. My lung function now hovers consistently around 4.1 FEV1 and 6.9 FVC each day. I didn’t even know it was possible with my age and the decline of my lungs so I’m extremely grateful to have been able to achieve it.

I’ve made a lot of new friends since going to the gym and going to the classes. It’s a good community and everyone really pushes each other and holds each other accountable.

What’s your daily exercise routine?

I do all my usual medications and treatments like my PEP mask once a day and then I take Kaftrio and antibiotics. Then I try and go to the gym and work on my strength, or I’ll do a high intensity interval workout. I run regularly and I surf too and recently I’ve been getting into sea dips, though that’s definitely getting harder now it’s colder!

I like to get up early and walk the dog and then try and do something physical to set me up for the day, then I’ll do a day’s work and try and get to the gym in the evening too.

If you could give one piece of advice to another person with CF, what would you say?

I would say it’s not too late. I feel like it’s easy to think, this is where I am now and that’s that but that’s not the case at all. I think it’s really hard because you get caught in this vicious cycle of trying to adhere to your medication routine and then also trying to exercise and keep fit and those things can be so energy sapping and time consuming, but it’s just about chipping away and being consistent and then you can start to build up your fitness.

You don’t need to push yourself so hard that you’re exhausted and can’t do anything for the rest of the week and you can’t do your job, it’s just about doing whatever you can do. Ultimately, I’m doing this because it makes me feel good and I enjoy it but I find it really helps me in other aspects too, it gives me clarity and makes me more productive and creative.

What are your plans for the future?

I want to continue what I’m doing and see how far I can get in terms of lung function and fitness. I don’t have any set goal but I just want to keep pushing myself. I want to try and do more things now, whether it’s an open water swim event or just being able to go on holiday more. Things are always more enjoyable when you feel good in yourself, so I just want to keep working on myself and my health – and if I’m able to inspire & encourage people along the way to make that positive change, then that’s amazing and I’m here for it.

Please talk to your CF team before starting any new exercise or fitness regime. 

We know that Kaftrio can be a difficult topic for some people. While many people have experienced amazing transformations to their health, other aren’t eligible or can’t tolerate it. Our Responses to Kaftrio factsheet, provides more information or reach out to our Helpline at 0300 373 1000 or 020 3795 2184, Monday–Friday 10am–4pm, or on [email protected].

Cystic fibrosis (CF) is a genetic condition which causes sticky mucus to build up in the lungs and digestive system. It affects more than 10,900 people in the UK. One in 25 of us carries the faulty gene that causes it, usually without knowing.

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