Turning 40 with CF
In September I reached a milestone birthday and blew out forty candles on my cake. Well, technically, I didn’t as no one wants me blowing my rancid breath over the WKD Blue birthday cake my friend had lovingly crafted for me!
I am a member of some beauty and skincare groups on Facebook. One of my pet hates is when I see someone post about how they are ‘getting old’, followed by a sad face emoji. Aging is a privilege that not everyone has and healthy people would do well to remember this! My aged face is the least of my concerns!
For those of us with CF, or any life-limiting condition, we celebrate birthdays! No getting upset about time running away with us or crinkles on the face. I like to embrace my laugher lines even! It means despite all the crap of bodies have to deal with, we have survived another year and this is to be celebrated, well and truly!!!
Cystic Fibrosis Care in the 1980s
Turning forty is a huge milestone for people with CF. Being born in 1980 we had only a handful of medication available to us.
I wasn’t even diagnosed until I was nigh on three years old, whereas in this day and age most suffers are diagnosed at birth with a heal-prick test. They only diagnosed me because I was so ill all my lungs collapsed and I was very underweight and malnourished. This left my body weakened and my lungs damaged. To the point where some of the lobes of my lungs never reinflated. Imagine if I was diagnosed at birth and was able to avoid all this lung damage! I might have been a neurosurgeon, an Olympic athlete, a contestant on Masterchef even… or I might have just had a slightly easier ride with my lungs!
I’ve just looked up what the life expectancy of a child born with CF was in 1980… Ten years apparently. My parents were told after I was so unwell at three years old I probably wouldn’t make five. Good job I’m a stubborn character and wanted to do what every other child was doing. And teenager… And adult!
Advances in Cystic Fibrosis Care in the Last 40 Years
I have lived through and witnessed amazing advances in treatment, care, and knowledge. Originally being prescribed a low-fat diet before enzymes (yes really!!), having a port at 12, being on the Dnase (Pulmozyen) trial in 1992, and then being allowed to stay on it! (big news then!!).
From hanging out of mixed wards and even going on holiday with other CF patients (could you imagine that now), to the introduction of Tobramycin and other more effective ivs.
When I was a child the only person in the house who could lift my nebuliser was my dad, as it was so heavy and bulky (weighed I’d guess 10kg). The only nebulised antibiotic available was Colomycin (which is still going strong all these years later!), and there were no physio devices (as far as I know) so it was percussion only!
CF Gene Discovery and Modular Advances
In 1989 they discovered the gene defect causing the CF, which so many years on helped to start the work with Orkambi, Kalydeco etc. As a child, I remember the CF Trust always saying that a cure was “5-10 years away”. It became a bit of a running joke in the family!
Now we are spoilt with drugs, medication, and knowledge (we may not like doing all the treatments and physio, but that’s why life expectancies now are so much higher!). These days with modulators such as Trikafta / Kaftrio I really hope a baby born will have a much longer, easier life, perhaps even a normal life-span.
Survivor’s Guilt – The Friends Gone Too Soon
I’ve felt quite emotional getting to this milestone. So many of my CF friends didn’t make it along the way. I had a penpal (c’mon, this was years before Sir Tim Berners-Lee had unleased the Web on us!) who died at 19, and many we’ve lost before adulthood even.
There are still 10% of the CF community which don’t have a modulator that works for their gene type: we still need to get a treatment that will benefit them too. I am still trying to find a way to cope on Trikafta / Kaftrio. It feels greedy to ask for the next generation of drugs – but come on Vertex, let’s iron out the side effects and make them perfect!