Isaac has none of these (yet). This is just another bump in the very lumpy bumpy life of CF. And I hate it.
In applying recently for his PIP (personal independence payments) I had the absolute displeasure of describing our day to day challenges in minute detail, and in writing about it (the paperwork is long, painful and laborious) I was reminded of the fact that CF robs us of spontaneity. When acutely unwell, lives are temporarily disrupted, maybe my lack of voice means I have a cold coming on that will annoy me for a few days. But chronic illness means constant and extreme disruption. Because of CF we are unable to plan ahead with any certainty (when will his next course of IVs be?) roam freely (what bugs will he catch next? And will the next one be the one that floors him again?) or to really, truly enjoy the good bits (it’s never an IF he will be sick again, it’s always WHEN).
Isaac is coughing a lot (we’ve introduced a third antibiotic into the mix, but it’s not helping as yet) and the sinus pain is building (ENT backlog means we are still waiting for a scan, let alone a surgery date).
Explaining CF to someone today, I said I would just love, absolutely love, for him to sleep soundly one night, really well. Just that. Is that so much to ask for?
Isaac is not eligible for the wonderful new CFTR modulator drugs you may have heard about in the news (Kaftrio, Orkambi, Kalydeco) as he has two rare, class 1 mutations, meaning he never forms the correct CFTR protein in the first place, so needs a very different kind of fix (read more about his annoying mutations here.). However, there is much research going on that gives us hope (e.g. using other, non CFTR channels to compensate for the lack of CFTR). We need to keep up the momentum (which includes awareness and fundraising). I know it will happen, it’s just whether it will be soon enough for our boy that I worry about.
The PIP process has been difficult, and continues to be. I hate having to explain, again, that CF can somewhat appear to be an invisible disease, but that he is breathing disabled, and that CF affects his bones, his kidneys, liver, pancreas, sinuses, guts, his well-being..... as well as his long suffering lungs. CF is a progressive, genetic disease, and yet they challenge us, year after year, to describe why he deserves the tiny support PIP (previously DLA) provides him. Frustrated doesn’t quite cover it.
Thank you for reading x keep well x
Man Ray ❤️
