Any bright ideas? (Bit of a vent too!)

We’re day 15 of 21 on the children’s ward and I’m scratching my head a bit tbh. The 11yo has cf (chronic pseudo since age 4) and is on 4 monthly elective IVs, and was discharged following a *mostly* planned admission at the end of Feb that included positive culture at the start for the usual PA as well as a ‘new’ cipro and mero resistant PA. With hindsight, we think it’s highly possible that the resistant strain has been present for quite some time given the clinical picture in 2016/17, but she was only out 10 days this time before becoming symptomatic again and culturing her usual strain once more, meaning she’s now had positive microbiology for PA 5 times since October, where before that we hadn’t cultured it at all for in excess of 18 months and it was considered very well managed. We started on cipro but saw little improvement (fev1 down to 58% at day 11 of treatment), and then there was a big ol’ kerfuffle and communication with and between both teams went out the window and the standards of care all went quite spectacularly tits up, culminating in my bringing her in for review at 1am on the 15th with a disgusting chest and raging temp.

Long story short, after a total of 5 weeks out of 6 on orals she came in with a very fortuitous adenovirus (cause of the temp) that led to a chest x-ray revealing changes in lower left (where we know she has permanent damage anyway) and mid right, in keeping with the ridiculous amounts of sputum she was clearing for weeks; it’s a proper put your hand on her chest and you can feel it vibrating jobby, but after 15 days of her normal IV tobi and azlea and 7 days of 40mg pred, she is still abnormally productive, clearing loads with palpable secretions in her right lung that just won’t go away. She came in with an fev1 of 61% and a repeat function today puts her at just 65%, although fvc is up to 79 from 68, with spirometry indicating a restrictive condition. To be fair, weird and inexplicable though it is, it’s not uncommon for her to lose significant lung function over the course of an admission, sometimes as much as 20%, so the numbers on their own don’t bother me, but the ongoing wet right lung is another story. All standard microbiology has been coming back clear for the last 6 weeks despite sputum being manky as hell at times, and we’ve put in for an AFB for the mycos but that’ll take weeks yet to come back. We’ve also requested aspergillus/fungal culture, too, although her IgE and rast are completely fine, but for the umpteenth time in the last three years I just feel like we’re definitely missing something. Her local consultant initially queried whether it was more of an allergic response (we’re pretty sure she has mastocytosis) but if so, I’d expect the steroids to have knocked it on the head by now, where it seems like ‘all’ they’re doing is what I would expect for a lung exacerbation - helping her get the rubbish up more easily by reducing the inflammation. She’s regained the half a stone she’d lost, she’s much better in herself, and what’s coming up now isn’t particularly grim so we’d seem to be moving in the right direction, but there’s still an awful lot of it given that we’re day 15. We’ve done a handful of three week admissions before, but I’ve never known her take this long to properly respond aside from one where she didn’t respond at all until we changed her IVs, and this isn’t that.

Throw in needing to make a formal complaint against the most arrogant and rude doctor I’ve met ever, and this admission has been a bit of a nightmare. We have a planned discharge date of a week today, but at this point I’m by no means convinced...

[sassy59]

Dear me Charlie_G what a worrying time for you all. You are in the right place for caring support and I just want to wish you and your daughter well. Do please make a complaint as you don’t need some a#%e of a doctor being rude.

Take care x. Xx

[Hidden in reply to sassy59]

Thank you. I’ve met some doctors with terrible bedside manner before, but this guy is a real piece of work, particularly for a paediatrician. He started off by aggressively accusing me of refusing tobi levels when I’d only asked why we were doing them day 5 instead of the usual day 8, then failing to provide clinical contact at all for the two days he was in charge of the ward despite being less than a week into an acute admission - we didn’t even see an SHO. He’s consultant in charge this week too, and I think he’s been told I’ve made a complaint, but whilst doing a terrible job of trying to build bridges with me this morning, he proceeded to very loudly imply that our (long-standing, amazing) named consultant is actively lying to me about issues with our specialist centre and isn’t qualified to make decisions about steroid use...our nurse had to peel me off the ceiling when he left!!

[sassy59 in reply to Hidden]

What a disgraceful way for a doctor to behave. Keep your cool and hold your nerve. I’m hoping for better days ahead. Xxxx 👍

[peege in reply to Hidden]

Absolutely disgraceful behaviour CharlieG. I'm glad you made a complaint. You'd have thought a peadiatrician would have more compassion & empathy. Record everything.

I really feel for you and have no idea what to say to help except to give the BLF a call 03000 030555 office hours and read your post or I think you can email them now giving your contact details for a call back - the help Iine wouldn't normally read posts (I think).

Also, do you see a specialist CF centre ie The Royal Brompton?

[Hidden in reply to peege]

Thanks, Peege. We’ve had issues with this guy before, but this time he’s done us the favour of actually misbehaving in front of other staff - there are plenty of people involved in the bod’s care that I don’t like as individuals, and a fair few that don’t particularly like me in return, but we still treat each other with respect because we all want what’s best for Littleun’. I’ve been told he’s already on a warning, but clearly that’s done nothing, and I’ve heard some pretty appalling stories in the last week from staff we’re close to about his conduct towards other families.

We have a specialist centre, and having had a spell of full care from them in recent history, I can tell you that they’re pretty dire. I’d trust them individually as clinicians in a medically acute situation, but the overall care and management for anyone not geographically close is terrible. A lot of the issues are down to communication, or a lack of it, staffing levels (a cf caseload over half that of the Brompton, plus all non-cf specialist respiratory within 100 miles, with substantially less than half the team size) and to a lesser extent, attitude. They might be the experts, but I don’t care if she’s not the worst you’ve got (direct quote 👀), I only care that she’s getting the best possible treatment, you know? It was only 2 years ago they told me I just had to accept her baseline fev1 had permanently dropped to 60% without any valid clinical explanation. I refused to listen to them and accept it if they couldn’t give me a reason why, and sure enough, her best fev1 in the last 12 months was 82%. As a direct result, in 12 weeks time we’re uprooting our lives to move 250 miles so that she falls under the Brompton, and they’ve already started doing the transition work with our local team. It’s pretty astounding just how much care guidelines vary between centres; there are so many things done as standard at the Brompton that aren’t even on the radar here. The guidelines are published online, and having been through them from cover to cover, there’s a whole bunch of things I know they’ll want to do as soon as we move and that I think she should have had done long before now...cf aside, the sad reality is that where we live generally doesn’t work for children with complex needs, and every professional I speak to locally, be that any of our 5 medical consultants, social services, education - they’re all telling me she’s going to get much better care across the board by moving. It’s shameful, and I take my hat off to the amazing individuals we have involved for trying to change the system from the inside out - I’d have got fired by now for completely losing my cool...

[Hidden]

What an awful time you and your cf warrior are having. As you know, I am not an expert on cf but the only things that you haven't mentioned being looked for are NTM and anaerobes. I had repeated exacerbations over a number of years with sputum which could resemble niagara falls in full flow. It could take 9 weeks to grow on a plate and the only ab was horrible metronizadole. Finally I started azith and steroid inhaler and fingers crossed haven't grown it since. I don't have NTM. I know that the treatment I had for anaerobes wouldn't be appropriate for your cfw but if they haven't looked fir them maybe they should.

Having to deal with arrogant doctors is just another burden that you don't need. I do hope that you get to the bottom pf it soon so that she can get onto an even keel. Thinking of you both.

[Hidden in reply to Hidden]

Thank you. The AFB we sent off last week covers NTM and all the other mycobacteria like abcessus etc., but we’re still about 6 weeks off the culture being complete, unfortunately. I’m convinced that there’s something else down there causing problems beyond the cipro-resistant PA (that we can’t prove has been there for years, but probably has) and her usual colony of PA, but unless we successfully culture it...our consultant is going to call in tomorrow and we’ll have a chat then - I don’t think he’s going to be too happy with her progress from when he last saw her on Thursday, but if that’s the case then the question becomes what are we going to do next? And how not to lose my mind if we end up staying in even longer!! 😂

[Hidden in reply to Hidden]

You just get a gut feeling on these things don’t you. I do hope that you both get to leave. As you say, some of these things take so long to grow and even then, what can we throw at them.

[cofdrop-UK]

OMG sweetheart I am so sorry to hear of little E’s struggles and that arrogant medics are exacerbating your problems. In what way he feels it helpful to state that E is not the worst - disgusting and a comment which should not have been made. How unprofessional to make such derogatory statements about another medic. I wish I could say I am surprised you have come across this arrogant git but we have both, along with others on here, all come across these medics who are basically in the wrong job! Well I don’t think he bargained for you lovely. You are so tough and an amazing Mum to have fighting E’s corner.

Well this move doesn’t sound as if it can come soon enough. Any idea of the time-frame? I feel sure, as you obviously do too, the care and treatment little E will receive at the Brompton will be far superior to that she is presently receiving. It really shouldn’t be a post code lottery for medical care when there are guidelines to follow, but we all know it is. As you know even in England care can vary so much and the best care is within one of the CF centres.

I so hope someone can get to the bottom of what is going on at present. Trust a Mum’s instinct, expecially one with so much experience as yourself.

With love to you both

Cx

[Hidden in reply to cofdrop-UK]

Dads too, lovely lady, not just you Mums 😉

Not a good start to the day, as she’s awoken spiking temps and not great from a respiratory perspective despite a *much* better x-ray yesterday. To add insult to injury, the doctor from hell is consultant in charge this week and I’ve just had a third run in with him in the corridor, albeit minor. I had asked that he not be involved unless she deteriorated clinically (at which point I’m not silly enough to not understand he kind of has to be), thinking it wouldn’t happen at this late stage, and of course she does exactly that. Although I wasn’t happy with her condition overall, I suspect she’s caught a virus on the ward and that’s what this is - she’s presenting almost exactly as she did with the adenovirus on admission - but we did discuss yesterday whether pneumocystis was a possibility and we’re trying to get sputum for a PCR to rule that out, but all that’s coming up at the moment is abnormally huge quantities of clear...d’oh!

On the back of the better x-ray, the revised plan yesterday evening was to stop the tobi (due to the ototoxicity concern that tertiary has finally discussed, only 6 weeks after I first told them about it 🙄) and sub for oral doxy to cover any potential atypicals, then continue the course for a total of 14 days, with discharge confirmed for Monday and see how she goes. If you could keep everything crossed, I’d appreciate it, not just because we’ve been here for three weeks already, but because our local consultant has said that he’s pretty much at the limit of the diagnostics and care he can provide; if she doesn’t properly recover over the next two weeks or continues to deteriorate over the next few days, we will be headed for an inpatient spell at the tertiary centre...

[Ergendl in reply to Hidden]

You and your daughter are in my thoughts and prayers. Hopefully she's turning the corner, and your house move will prevent further hospital incidents like these.

[Hidden in reply to Ergendl]

Thank you, Ergendl - I meant to reply earlier in the week, but forgot. Still got everything crossed for discharge on Monday, but not set in stone as she’s still wheezing.

[Ergendl in reply to Hidden]

Bless you both. Hope it goes the way you wish.