We’ve officially hit the 3 week mark. I nearly posted a massive rant at 11pm last night after really vague, unexpected feedback on the CT she had yesterday, but instead opted to wait, so rather than just going ‘argh, what’s going on?!’ at everyone, I now actually have some answers. Not all the answers, but more than we’ve had to this point.
As eluded to at the time, Bod’s LCI on Monday was ‘grossly abnormal’ (direct quote), but exactly what her new consultant expected for her fev1 in clinic and what it is now after 3 weeks’ treatment. She’s entirely satisfied that her fev1 is an accurate reflection of her current lung function, so the bottom line is that she probably does have an absolute max of around 75% fev1, and 95% fvc, which are the main two measures we look at in cf. Her best last year was 86/98, so the rate of decline of her fev1 is a bit crap, but then her infections haven’t been taken as seriously as they should have been by certain previously involved parties, occasionally being left completely untreated. It also turns out as of her CT yesterday that said parties have also failed to detect that she’s developed small airway disease and a measure of bronchiectasis, in part because they wouldn’t accept that she wasn’t ok and therefore wouldn’t do a CT. Our consultant here was actually surprised that the CT wasn’t worse for her symptoms, as she’d expected to see quite a bit of bronchiectasis, but that does also mean that she wants to rule out any additional respiratory diagnoses that could account for her degree of productivity, so we’re going to check for a couple of other things prior to being discharged including doing an NO test for PCD.
We’re going home at the weekend (not sure if we’re physically home Sunday or Monday, but definitely going), and although I still really don’t think we’ve cracked it in terms of getting the bacteria under control, there are some reassuring factors:
1. I really like our named consultant. She calls a spade a spade, but she always seems willing to listen and thinks outside the box. She also seems to respect that I do have a decent degree of knowledge and understanding both of cf generally and small person’s disease particularly.
2. We’ll be on the clinic list for 4 weeks time, and there’ll be a home visit in that time too that will include lung function - if she’s deteriorating, we’ll ‘officially’ know much sooner than we have previously beyond the colour of what she’s bringing up.
3. They’re taking this seriously. How do I know that? They’re commencing 8 weekly IVs for the next 6 months to try and stabilise her health and get a handle on what’s happening. Not denying it’s going to be a long, bloody hard slog being in so regularly, but it’ll be better than the way the last 6 months have been, never knowing if we’d even get oral antibiotics regardless of positive microbiology.
The soul deep vindication I feel is bittersweet albeit with a heavy leaning towards bitter; I asked repeatedly for a scan, her local consultant also wanted one because he thought she might have SAD or bronch complicating things, but tertiary carried on like we were both overreacting and neurotic - the breathtaking degree of arrogance we’ve experienced over the last two years beggars belief, because that’s the only explanation I have for the failings in her care: they decided there was nothing wrong and were completely unwilling to consider the alternative in spite of all the clinical evidence, and the professional concerns of an extremely experienced paediatrician with special interest. They said they had great faith in him to run the cf service, except where it came to my daughter apparently, because they valued his opinion of her condition no more highly than mine as a layperson. The reality of the situation is that they were the ones completely wrong about everything - about the state of her chest, the degree of lung damage, that her fev1 was irrelevant and unreliable - but we’re the ones that have to live with not knowing if the situation would have been different if they’d just listened to the concerns. But then as I was told more than once, they’re ‘the experts’ and bod’s ‘not the worst we’ve got’.
Update
Help please, my sisters ESA benefit has been stopped
Smooth sailing 
