Why lung function and symptoms don’t always match

Oh my bananas, I cannot tell you how many times we have got this so wrong. Coughing until he is sick, and then LF is stable? Feeling really quite good, and LF is terrible? 

These days, we always try and guess ahead of testing in clinic (we also have home spirometry, but testing too often can be soul crushing and anxiety fodder). Often we can predict within a 5% window. But not always, and truly, I’m not sure what way round is worse… Shitty LF when he seems quite well is always worrying (and usually a prompt to start IV’s which he may argue against). But good LF when he’s feeling so crappy? The course of action feels less clear. Right now we seem to seesaw between on and the other. Confusing. Thankfully we have an incredible team who we have now built a relationship with (over three years in adult care) and who listen to Isaac and take a more holistic approach, and we always find a treatment path that we all agree on. 

Sorry, writing is weird at the moment. We still can’t talk about the last gene therapy clinical trial that Isaac was a part of (until the pharma publishes the data) and we now can’t talk about the next one. But we can say that thangs are happening, and we hope they happen soon. Annoyingly his diseased sinuses are causing headaches and nausea once more. When we last saw his (lovely) ENT surgeon, he said that Isaac can pretty much choose when to schedule his next surgery (schedule that is, waiting lists dependent!), and we need to do this, but can’t during trial participation, which is difficult. 

Otherwise, things in Molyworld are grand. The bulbs are sprouting. Anouk is acing her GCSE exam revision, and Rosa and I are loving our pony time. Love x 

Obie, Remy and Skyfaxa, our dæmon companions ❤️

Forgotten posts

I realise that writing might be deeply unfashionable these days, I should be offering bite sized chunks of content plastered all over social media. But I’ll always love words, lyrics, idioms, editing down the random ramblings from my mind, books, getting lost in the landscapes that stories build in my mind. As much as I’m told to get on social media more, or worse, vlog (ha!), I think I’m sticking around here, in my geeky comfort zone. I don’t really do any social media now (can’t be arsed with the ads!). Although I’m mostly too busy or tired, I still occasionally get the urge, maybe need even, to write here. Often to offload. Sometimes to inform friends and family so we don’t have the repeat health updates again and again. Maybe because it builds up a timeline of events that I can look back on. But mostly in the hope of raising awareness of Cystic Fibrosis, a largely invisible disease that is so often mistaken or misunderstood. A GP that I had an appointment with asked me recently ‘when did he get it?’. Christ. 

So while I’ll stick to words, you can watch this vlog which captures what life with CF can look like better than I could ever describe. Words are not always enough. Please do take a look here. 

Isaac is currently, um, stable? I guess? Hard to say when things change daily. He’s had recent IV’s, a plethora of lovely viruses (he is currently on anti virals, a fourth daily abx and steroids) and the random fevers and joint pain persist. Our participation in gene therapy trials, which we so desperately want, is delayed for as long as he is on extra treatments for an exacerbation (he needs to be at least four weeks clear of meds that are additional to his usual regime). It’s frustrating to say the least. Too many delays, and he could miss out altogether. 

But things could be so much worse. A good health day is walking the dogs together and him doing the cooking. A bad day is only ever the one before a whole new one. 

I often start writing, and then forget to ever post them, so do look back on previous entries if you’d like to. Hard to believe that this little old blog has had almost 100K views. Crazy. Love love love x

Pictures: Christmas at home.