The first series of What The CF! A Cystic Fibrosis Podcast has been an amazing and emotionally exhausting project and I don’t regret it for one second. The support and messages that have been shared with me since I began this podcast journey have all been so heartwarming, encouraging and positive for the outlook of PWCF. One such message really touched me as the writer shared so much of their life with me while reflecting on the journey their parents must've gone through.
I asked permission to share this message anonymously as I felt it would be so useful to both parents of PWCF and adults with CF. You can read it below and I hope you receive it with the same warmth, support and kindness that I did.
“Your podcast is an excellently raw (in a very good way), witness experience almost, of your journey and fascinating to hear. It will be a great resource to share with any families with a new diagnosis of CF in the family. Your ‘What the CF Moment’ is a clever and humorous concept!
As a person with CF, it helps me understand more comprehensively the journey my parents would have travelled, and are still travelling I suppose since I was born. My parents are now in their late 70’s and they’ve been great all through my life. I have very typical CF - double delta F508 - and I’ve been relatively fortunate with my health over the years even despite a rocky start in early childhood as well as constant colds and flu during the winter months. I’ve been lucky enough to only require IV antibiotics on a very infrequent basis over the years.
Even though I haven’t seen my parents since last June and only saw them twice in total in 2020 due to the COVID-19 lockdown, my parents are always looking out for me, more so than my siblings. They’ve never stopped. I think it’s an understandable form of guilt complex with them. My mother insists on baking for me whenever I call to visit even though her hands are crippled with arthritis now. I have told her she doesn’t have to feel so guilty. But it’s her way of trying to support me still. She knows and remembers how much food I need and how hungry I would always be. I used to get angry trying to refuse this pampering out of concern for her and to give her a break, but now, I just accept it in the hope that it lets her feel she can still contribute to my care.
I am a father now myself, with two daughters aged 9 and 7, and every day I think how my parents would have had to deal with all the same responsibilities – how they would have approached learning, educating and discipline - as my wife and I try to cope with these ourselves as parents. But, then I think of adding CF into the mix as was the case for their child (me) and try to reconcile my own childhood memory of these things. My parents were protective of course, but they were also very much live and let live in their approach. I didn’t feel so different as a child. I knew I had CF and took medicines and attended hospital when my friends didn’t need to. But I never felt very different - well, maybe just a little different.
My early school attendance was affected by my CF. I was always so hungry. I couldn’t eat all the same foods that my siblings ate. I would get very cold so quickly in summer evenings when friends were still running around in shorts and t-shirts. I took heaps of enzyme tablets and bathroom breaks were much different for me compared to others. But apart from these things, I felt normal enough. I can’t fully appreciate how my parents would have felt seeing and knowing these effects of CF on me along with the prognosis the doctors told them to expect for me.
Even being aware of these effects on my health from early childhood, I still knew certainly from age eight onwards that I was fortunate with my CF compared to other children with CF. I could see at clinics how ill some of the other children were and I always felt that I was a bit different to them too. Over the years, I’ve constantly learned of individuals with CF who are late diagnosis, who are at retirement age and have been “well” with their CF. So it definitely is a very individualistic experience.
I started Kaftrio (Trikafta) about three months ago and even though prior to this my ability in my day to day life was not significantly impacted, other than the time to manage the very demanding treatment burden, I’ve seen many improvements. With Kaftrio, this burden has eased a lot and my day is a little more normal. I cough much less than I did and my mucous production is dramatically reduced. I also hear of many really transformative experiences from people who have commenced on Kaftrio.
Unfortunately, I know of less fortunate outcomes too. My previous relationship was a happy and loving long term one with my fiancée Mary who also had CF. Her journey was very challenging for her day to day and she had advanced illness from early adulthood. She was actively listed on the lung transplant list for 2 years. She never got that second chance though, and unfortunately passed away the year we had been hoping to get married. I was so lucky to later meet my wife Gemma with whom I now have a family of my own.
I grew up in a world of cigarette smoke- my mother smoked, my father smoked a pipe and cigars for years. The outpatient clinics used to be full of smoking parents. The bus or train I used to take when I transitioned to adult CF clinics were always full of smoke too. The pubs and clubs I went to in my college days were thick with smoke and it would always take a day or two for my lungs to settle back after such exposure. But that has all changed. I am no longer exposed to smoke. Such factors along with treatment improvements and cross infection prevention in clinics mean that children with CF now have a better chance than when I was a child. My childhood outpatients clinics were openly mixed with other families with CF.
I survived a childhood of second-hand smoke, of cross-infection clinics. I played competitive team hurling until I grew too old to continue. All before CFTR therapies were available. I know you hear stories of people with CF who have not been as fortunate, and I know also that when you hear of people who are alive well into adulthood this stirs mixed feelings for you as Orson’s path (and anyone with CF’s path) is so unpredictable and individual. CF presents difficulties that must be managed every day and hearing of adults with CF doesn’t really help you or Orson with his CF right now.
All this is really just another CF story for you and I can imagine your podcast has prompted many others to share personal stories with you. I suppose this message is to say the world of CF has changed so much for children born now. It really has. This is not to say Orson won’t have his challenges, his emergencies and won’t have his CF to manage day to day. It’s just to say he has a chance of staying well despite these challenges.
I think time will prove the undeniable cost-benefit to healthcare systems of introducing Trikafta and I would expect and hope for you that New Zealand will fund this therapy very soon, for all the PWCF in New Zealand. There will be other drugs too, next generations of Kaftrio and likely gene editing treatment will come on stream in the near distant future as a treatment alternative. I know this is a double-edged message to you when these treatments are not immediately available right now to you. But it is much reason to be hopeful.
I could go on all day! But, thank you for sharing your podcast -it is a fascinating insight. Well done putting this together and I hope Orson stays well and lives a long healthy happy life with continued love and care and support of his doting parents.”
If you would like to share a story with me for our blog or podcast please email on wtcfpod@gmail.com.
Thank you all for your ongoing support.
Ingrid xx