It’s been quite some time since I posted about Bod despite intending to, and quite a few people have mentioned her in comments or replies to me. As always, I remain fairly useless at replying to comments, but I’ll do my best, and I do really appreciate everyone’s support and interest in what’s happening for her. It means a lot.
The two big bits of news are that it took almost 12 months since our move and a tonne of pressure on my part, but she’s finally been placed at an amazing, autism specialist secondary school. We also got a new social worker who came from outside of the department and within 4 weeks had done our needs assessment, taken it to panel, and got us 2 overnights of respite each month, although there’s no sign of identifying an appropriate foster family as yet. You may recall that at the time of my last update we were being told she didn’t even warrant being under the disability team, never mind respite care, so loud cheering and party blowers on both fronts as I’m sure you can imagine. It went somewhat under the radar, but paediatric shielding guidelines changed in June and most kids with cf officially came out of shielding at the start of July, meaning she’s been able to physically go to school 2 days a week for the last 3 weeks: it’s been a bit nerve wracking for a number of reasons, including covid, but her class is only 3 other kids and 2 members of staff, and they’ve been operating in a pod system that means they have no contact with anyone else whilst in school. They also maintain strict social distancing within the pod and reinforce hand washing. I then slather her hands in antibac gel the minute she gets in the car, she strips off when she gets home, and so far we’ve stayed covid free. It’s done her a lot of good mentally to go in, though, and I was very proud that she not only got a special mention at the end of year assembly today for her resilience and adaptability (both big deals for a kid on the spectrum, no matter how academically able!!), but also came first in a whole school short story competition.
Her mental health has taken a massive dive. It wasn’t great before lockdown by any stretch and we were begging CAMHS to get involved since Christmas, but she’s now ended up on antidepressants whilst waiting for therapy to materialise. The wait is currently at least 12 months and what they’ve initially offered isn’t what she needs, but we have at least got a foot in the door and a psychiatrist that seems decent overseeing her care. Unfortunately, one of the recent manifestations of her poor mental health is a refusal over the last fortnight to do nebs and physio as a means of harming if not actually killing herself. It’s heartbreaking that she feels so low, but also hugely frustrating in turn when I’ve worked my butt off for 12 years to keep her as well as I possibly can, fighting against the old medics who wanted me to accept things that we now know were completely wrong and actually caused her harm, only to have to sit on my hands whilst she actively encourages her lungs to get worse. Her team have ‘reassured’ me that this kind of refusal is common anyway in the early teens, but my problem with that is her degree of lung damage and decreased fev1 isn’t that common anymore. It’s not like we’re starting at 90% and have some wiggle room. I can only hope that she mentally picks up soon and we can make treatment a regular feature at either end of her day again.
On the plus side, her chest is much more stable generally, although still not right. She was really good through Jan, declined in Feb, and actually worse at the end of IVs that month. Hugely frustrating, but once more reinforced that I’ve done absolutely the right thing in moving us all the way here: by March there was agreement that fungus was probably still an issue even though we hadn’t grown it since the month course of posa back in November, and after another course of oral antifungals that both improved things and proved the fungal theory, they gave her IV amphotericin as an eradication attempt alongside Piptaz and colomycin in May. The end result of that in combination with the covid situation is that I’m now qualified and signed off as competent to do home IVs (exhausting, but it turns out being exhausted at home is infinitely preferable to exhausted on the ward), and bod is significantly improved from March/April if not particularly close to where we were in Jan. The pseudo remains a more regular feature, persistently showing up on almost every other culture, and I know this is just par for the disease course, but remains frustrating when it appeared so well controlled for years. Other than that, we had the one very unusual bacteria in Feb I think I posted about (a. beijerinckii), another growth of the e.dermatitidis (fungus) in March, and a first culture of candida dubliensis to keep her airway colony of c.albicans company. As with albicans, dubliensis isn’t considered pathological in its own right, but is considered a marker of lung disease severity, and is thought to correlate to quicker decline in fev1, particularly when cultured alongside both albicans and pseudo. One of my questions for the team at some point is what they would actually consider ‘reasonable’ annual decline in fev1. I suspect it’s not what we’ve had over the last couple of years, although she’s currently still sitting around the 75% mark when well. We’re due in for IVs again the last two weeks in August, where I’m hoping they may have a definite answer for the interesting adventures we’ve been having with home spirometry during the covid outbreak and remote clinics. To the best of our current knowledge, she’s the only child in the UK, possibly even the world, that has their lung function consistently over-estimated by all the new home spirometers to a tune of 15 to 20% for both fev1 and fvc. 🤦♂️
End stage copd update 
UPDATE ON TIGGER
Lung transplant Update
Daughter update
Just a quick update
