Thank you for agreeing to share some of your personal reflections with us Sally. Can you please tell us a little bit about your background?
I’m currently 47, diagnosed with CF in 2009 at age 34. I grew up knowing nothing about CF as no one else in my family has it. A healthy kid, I started developing asthma and getting chest infections from about the age of 10, often diagnosed as pneumonia. I also suffered with bouts of acute pancreatitis as an adolescent (I am pancreatic sufficient).
My mum knew there was something wrong that always went unexplained and I had many hospital admissions as a teen and young adult, but in those days gene testing wasn’t yet developed and I didn’t display classic CF symptoms. In my late 20’s I was becoming increasingly unwell and that was when I first started coughing up blood, which was the catalyst that led to eventual diagnosis via gene testing some 5-6 years later.
What has been the biggest adjustment that you have had to make since being diagnosed with CF?
Life as I knew it completely changed after diagnosis. All of a sudden, I was faced with daily treatments, a shortened lifespan and risks with having children. My marriage broke down because of it, and I’ve been on my own ever since. As anyone with CF knows, it’s a very isolating condition and I’ve become more and more solitary over the years, to the point that I no longer seek relationships or invite many people into my personal space. Though I have had to learn to accept help because I can’t manage maintaining a house on my own.
How did your road to modulators start?
When Symdeko was added to the PBS and my genotype was made eligible, I commenced taking that and I was then changed to Trikafta in 2022 as part of the clinical trial for Trikafta. The trial finished in May 2023 and then I was approved to continue the drug trial for the modulator that is being developed to follow on from Trikafta.
Is there anything you wished was discussed more when it comes to modulators?
I wish there was more understanding and knowledge of the weight gain that comes particularly being a pancreatic sufficient patient. I am now reasonably overweight and now have issues with hypertension and high cholesterol. There was really only the glorified data of people having amazing results from modulators with huge increases in lung function, which hasn’t been my experience at all.
I am so happy for all the people that have had some life-saving results, but equally as disappointed for those of us like me that haven’t had great results or aren’t eligible to take them. I’ve actually been quite unwell since commencing Trikafta. I caught a virus and ended up very unwell and spent 15 days in hospital. And I’ve had the worst chronic sinusitis of my life so far.
What are you most grateful to your past self for doing?
I’m grateful that I made huge adjustments to my life around 5-6 years ago where I cut back on work, housework, and socialising to increase the amount of rest I give myself. It’s allowed me to add so much quality to important moments and not feel so guilty for not being able to do everything.
Do you have any advice for other adults in a similar situation when it comes to modulators?
I think while it’s important to be hopeful that modulators are going to improve your life beyond measure, it might come in ways not associated with increased lung function. It might only come with less coughing, reduced fatigue, or fewer bacterial infections. Try not to be too hard on yourself if you find that not much has changed at all, because one day you might realise that actually some aspects of your health have improved.