Latest (day 17)

Eh.

Plan is still to discharge on Thursday. Her fev1 is 68% (down from 74 start of week 2, but that’s still good), but she’s still really productive and wet, even if what’s coming up is on the whole off-white or slightly yellow rather than full-on canary and lime.

My gut instinct is that we haven’t cracked it.

I very strongly believe that the Aztreonam is no longer effective, and where we’ve yet again given that as the second line, I think that’s why she’s been so slow to improve and why we’re still not home and dry now. The difficulty I have is the docs are maintaining it’s just where she’s been ill for so long and that she’ll dry up in time, and I have no evidence to refute that other than experience and what’s happened over the last 48 hours. Long story short, her IV colomycin was inadvertently (and incorrectly) stopped on Wednesday night, and I only picked up the error today, meaning that if I’m right and the Azlea is no good, she’s had no effective anti-pseudomonal IV for 48 hours: she’s cleared almost a full sample pot today, which is a lot more than yesterday and yesterday was twice as much as the day before, some of it thick, blobby and starting to look yellow again. Exactly the same thing happened when her IV tobi was stopped in July: within 48 hours her sputum was worsening. Her second line on that occasion was a close relation of a drug she has resistance to, and whilst she’s significantly more well now after 14 days of treatment than she was then, I just don’t think we’ve got the situation under control, but I also don’t know what to do for the best.

If they kick us out on Thursday as planned - and tbf to the medics, other than the anecdotal/observational/sputum based stuff, there is no clinical reason not to - I’m absolutely convinced we’ll be back at square one within a month at best. Don’t get me wrong, I’ve done 17 days straight with no respite, and I am absolutely desperate to get out of this sodding hospital to my own bed and a stiff drink or two, but the thought of taking her home only to repeat the same cycle for a fifth time? Genuine nightmare. But how do I convince the docs there’s an issue without looking completely neurotic and potentially poisoning relationships that are only just beginning?

Argh!!!

[sassy59]

How very frustrating Charlie! I really feel for you as, to be perfectly honest, you know more about your dear daughter and her health issues than any doctor. Your instincts and past experiences tell you exactly what will happen next.

I just hope that someone will reply who has more knowledge than I do. I just want to say I’m thinking of you and your daughter. Xxxx

[cofdrop-UK]

A parent’s instinct is usually right but I appreciate your dilema re the docs at such an early stage. They will get to know your daughter’s patterns in time but that is probably little consolation to you now Charlie.

I appreciate too you feel 68% is good but I would have thought they would want this to be up to the 74 she was at week 2, following the IVs, although I note what you say about the Astrea.

OMG ommision of the Colo IV - how did that happen?

Does your little one ever dry up, even at her optimum best?

I hope there is further improvement and you can get home on Thursday, as planned. You must both be so fed up and it will be good for you both to get home to some ‘normality’ and I can hear your frustration.

I was wondering how you were both getting on. Thank you for the update.

Really hope you Both get a good respite run.

Love cx

[Hidden in reply to cofdrop-UK]

The colomycin error can be explained in 3 words: electronic prescribing system. Whenever they prescribe a drug they have to assign a treatment duration for the system to accept it, but once that duration has ended, it removes the entry without anyone having to confirm that it’s to be stopped - the initial treatment plan was 14 days, so it wiped it when we went past that. The reg who came to explain it to me said they were going to do a datex, but included in that complaint will be that staff should have picked the error up. I hate the system with a passion anyway though - to be on SAMS 3 (self-administration level whereby I keep everything bar fridge meds and controlled in the room and administer as we would at home) all the labels and records have to match, but the EPS won’t allow a dose outside of the BNF, meaning a couple of her entries didn’t match up and caused havoc because she was on 3 times the adult dose 🤦‍♂️

See, that’s part of the problem, too - I keep referring to ‘normally, she does x, y, z’, but it’s so long now since she’s been at her well baseline, how do I actually know what her well baseline even is anymore? I’ve decided to start saying previously to differentiate. Prior to February, completely well looked like a very small amount of clear sputum up with morning physio, and that was pretty much it. No daily cough, no real breathlessness, no wheeze. Even at her most well in months she’s clearing buckets by comparison, still struggling with exercise, and her sats are quite often 93/94 where she would previously be 98+. They also started talking to me yesterday about how her tracheomalacia means we shouldn’t get too hung up in her numbers, and after all the crap we had with her old team (I was told her spirometry was more or less irrelevant, good or bad) that’s just a red rag to a bull, not going to lie. Even if you accept that her genuine fev1 could be up to 18% higher (which was a figure that a retrospective study in Iowa came up with regarding kids with cf and TM/TBM), the trend is still entirely relevant: when she’s well, her numbers are good. When she’s symptomatically not well, her numbers reflect that. I’ve had occasions where she’s scored better than I expected for the state of her chest, but never the other way. I’ve never, ever seen crap numbers when asymptomatic. On the plus side, they’re going to do a Lung Clearance Index with her on Monday; if that comes back higher than normal, at least we know that her fev1 is in keeping with the degree of lung damage she actually has.

I think I’ll just have to bite the bullet and speak to the consultant. At the end of the day, if I’m right and they discharge, I can just say I told you so when she ends up back in. If they choose to listen to me, they’ll keep her in, change tack and hopefully we can get her sorted. But if we go just home without me explicitly making my case and it turns out I’m right, I’d be letting her down....

[cofdrop-UK in reply to Hidden]

Electronic Prescribing System - progress is not always a good thing. It sometimes throws the baby out with the bathwater!

No wonder you are frustrated by what is littleun’s norm following the less than perfect service you were getting over a long period. It may take RBH time to get your daughter’s status quo. I hope it won’t be long.

I think you are right to discuss with the consultant and express what you feel. Charlie you are an amazing Dad who fights your daughter’s corner and could never let her down.

Cx

[CDPO16]

While I have few if any suggestions, I am so very sorry for the situation you find yourself in and I can understand your feelings as you have the day to day experience and knowledge of your daughter's problems because you live with them.

I can also understand your concern about the need to fight your daughter's corner while worrying about alienating her doctors but I thought that patient and carers were seen as part of the team now in achieving results . Is it possible to have a sit down meeting with her doctors to voice your concerns prior to any discharge? I would have thought that this is a reasonable request and that her medical team would understand and be willing to have such a meeting given that you will be caring for her when you are back home.

Thinking of you and I hope that your fears will be eased soon. xx

[Damon1864 Volunteer]

I hope someone can give you some advice that can help both of you. Try to keep smiling and stay positive, I hope your daughter is well enough to go home soon and not before. Have a good night and take care of yourself 😊 Bernadette xx

[HungryHufflepuff]

I have nothing useful at all to say, but sending love and best wishes to you and your daughter.

[Ergendl in reply to HungryHufflepuff]

Here too.

[CDPO16 in reply to HungryHufflepuff]

Love, best wishes and support very often mean as much as suggestions x

[Corin1950]

Hi Charlie

17 days is a long time for both of you and although you are longing to get home I can understand your concerns.

You have clearly raised all your concerns for your daughter with us and I think you will have no difficulty expressing them to the consultant and having a discussion with him or her on what the best options going forward are.

Any reasonable consultant worth their salt will see that not only do you speak from the heart but from a comprehensive day to day knowledge of your daughters symptoms, condition and medication etc and that your opinion is essential in establishing the best course of action for her.

Hope you get the situation resolved and find the best option.

Sending best wishes to you and your lovely daughter

Stay strong

Let us know how you get on

Corin

X

[Izb1]

Sending love and best wishes to you both, hope you get to the bottom of things before they send you home x

[Caspiana]

Hello Charlie, 👋

I'm new to Azlea's story but reading this as a mum of two girls, my heart aches for you. I hope they don't kick you out yet, because it seems she isn't quite ready to go home. The best you can do is be frank with the medical team and tell them your fears. I am sorry I can't be of more help but I am thinking of her and your family at this time.

Sending you both a gentle hug and you a stiff, but virtual drink (or two) . 🍸🍹🍷

Cas xx 🙋