Finding our way in adult care.

This brand spanking new hospital, with a dedicated respiratory/CF ward, is, for us, pretty life changing. 

The care we have received in paediatrics from our dedicated CF team has always been second to none. We always felt safe, cared for, and so incredibly grateful. However, once admitted to the ward, we were cared for on a general children’s ward (at Addenbrookes, this was mainly C3 for 0-3 year olds, D2 for 0-16 year olds, and another newer ward for day cases and overflow should they need the beds overnight). Although we still saw our CF doctors at times, the rest of his care was with general respiratory doctors and ward staff, not the dedicated CF team. 

Of the many, many stays we had on children’s wards, we were mostly cared for in shared bays (we only had a room when he was known to be infectious to others with a virus, not a CF bacterial infection) often six children to a room (with parents staying overnight, that meant up to 12 in a room) very little room for the equipment we needed to bring with us, all trying to sleep and keep our little ones settled. For as many times as I worried that others were kept up by Isaac’s cough, or his IV pump beeping, we would be kept awake hearing children in pain, anguish, and their panicked parents concerns. I often got up to give someone a hug in the night. 

Worst was the worry that he would pick up more bugs on the ward than we had come in with, or that we would infect others. We were once opposite a very poorly child reliant on regular suction to clear her airways for days, only to then find out that Isaac also had a nasty respiratory virus which he no doubt passed to her. 

We will always be so grateful for all the care Isaac received on these wards, but with the facilities that they were restrained with, it was at times difficult, heartbreaking, and painfully exhausting, often meaning he came home infection treated, but less well overall.

On this new ward, he will always have a room to himself. It is so, so quiet! He has a TV, an ensuite, a fridge for his meds, and an exercise bike - and does not leave his room at all, but for certain tests. We feel a tiny bit spoilt... but this is simply what is required to keep him safe in hospital. Due to cross infection risks that come with a shared respiratory ward - CF patients especially are known to have easily transmissible bugs that only others with the same lung impairments will be at risk from, he needs this closed environment to keep not only himself but also others safe. 

The staff are amazing, and seem calmer and a lot less stretched than we have seen on other wards (that said, we are only a few days in, and with the closed doors and quiet, maybe we just don’t see the stress as much?). They often check on him, and treatments arrive incredibly close to the times they should do (on a children’s ward, IV doses have often come as much as a few hours late, despite being needed at very regular intervals). This is no criticism, only an observation and concern that children’s wards are inadequately staffed and funded. Or perhaps the improved facilities in a brand new hospital create efficiencies that allow for better care? Or both? I don’t know the answers, I am simply a caring observer and longtime NHS fan.  

Today I was signed off to do his IVs again. A few of the nurses asked me if and how many times I had done this before (countless over the last 16 years) - still, there are a few differences (the doses seem more... rounded up in adult care (as opposed to mixing up 8ml from the 2g vial and 5ml in the 1g vial, then draw up all of the 2g vial but only 3.42 of the 1g vial kind of accuracy, this is more - the dose is 3g, use 10ml of saline to mix - super easy), and we can push through more drugs rather than use a pump, so quicker; and they don’t even require me to wear gloves and aprons..so weird for me!). It was good to be observed and have them confirm that I am doing it all correctly. On our next admission, Isaac will also be trained, to offer him the independence to do them himself, should he choose to. Another change is that the doses are much more frequent, meaning five doses a day, which will be quite full on for home IVs when we do get home. 

Isaac has been amazing. He’s bored but agrees that it is a good opportunity to get to know the staff and ward. His cough became initially worse on IVs as usual, but already seem to be improving. He’s on steroids too, which may be helping his boost. Tests tomorrow, and hoping to squeeze in a cheeky sinus CT while we are in, to speed up the process to get him a sinus surgery date. Then hopefully home sometime this week to continue the IVs at home. Today we revived a long dormant shithead tournament, is there anything better than a pack of cards for quick and easy entertainment? I love that I get to spend so much time with him, just us. 

We are feeling positive. I am on the ward with him all day, and then home with the girly ones at night. It’s at times exhausting (I’m never quite sure why that is, since it’s really a lot of sitting around and eating cheese sandwiches), and I’m equally as pained to leave him as I am happy to be home to see the girls, which has meant happy and sad tears all at once. One thing is for sure, they are all growing up so fast, and I love them all so much. OK, that was two things, sue me! 

One blue eyed blonde, one hazel eyed brunette, and one very curly dude happy with chocolates on the ward ♥️

Cystic Fibrosis is a life limiting, genetic condition, affecting some 10,000 people in the UK alone. CF sufferers often, as a last resort, need to trade in their damaged CF lungs for the lungs of a generous stranger. To sign up to support organ transplantation, please visit https://www.organdonation.nhs.uk/ and tell your loved ones of your wishes, as even when signed up, the decision becomes theirs after death. Thank you. 

COVID, hospital, surgery, and being a bit more Isaac

Nothing out of the blue. 

While we are pretty sure we had COVID at the very beginning of the pandemic (all of us but Isaac, amazingly) we’ve been fortunate since, but last week Anouk tested positive. Being the amazing human being that she is, she stuck to her room without complaint, and we cleaned after she used the bathroom. With her phone, Netflix and chucking her the odd snack, she was happy. Last two days she has tested negative and is now back at school. The rest of us have tested negative all week, but today there were a few confirmed cases in Rosa’s class alone, so still bracing ourselves. It’s just everywhere. 

Separate to the COVID malarkey, Isaac’s lung function has fallen significantly after a change in his cough this last week or so (we don’t think this is anything new, just a typical exacerbation of his CF lung infections, and not responding to Cipro); today in clinic they decided to admit him onto the ward to commence IV antibiotics. This is also off the back of his lung CT results, which were.... not fab. 

Not unusual. What is new, is that this will be his first in adult care, the differences being; we cannot stay the night with him (and we have never left him before); he will have his own room on a specialist CF ward (rather than being on a general paediatric ward); at his new hospital it is standard to start IVs with a stay in hospital (whereas we are used to doing the first dose in outpatients, and doing the rest at home); I need to retrain to do his IVs before they can sign off on me doing them at home (after 15 odd years of doing them); that we need to stick to actual visiting hours (in paediatrics there is no rule for parents); and,  because of COVID, only one person can visit throughout the whole stay (which will have to be me, as I’ll be retraining for home IVs) and this is limited to just one hour per day. 

I’m taking that last bit with a lump of frickin salt the size of my arm - not only will I need longer than that to demonstrate that I know what I’m doing IV wise, but this is his first visit, to a new ward, new hospital, with a new team.... and he is just sixteen. I’m lucky that he is a mature sixteen, but still, that is my boy. 

We were also told by his ENT surgeon today that his next sinus surgery will be a major one. This confuddles me muchly, as he’s already had the ‘major’ sinus surgery (effectively converting his airways in there from a traditional house to an open plan one) - I wasn’t aware there was anything more major that they could do! Sigh. So now we await another CT (surgeon was hoping this could be done while he is an inpatient, but the possibility of two separate areas of two separate hospitals (all be them on the same Cambridge site) being able to coordinate this at such short notice seems unlikely to me, but let’s see) and then a date for surgery. This will be his fifth round of sinus surgery alone, in what? Three, four years? 

Isaac is taking it all in his stride and doesn’t seem fussed at all. 

I wish I could be more Isaac. 

In fact, I think we could all do with being a bit more Isaac ♥️

Gerhard Richter love: 

Nonsense mutations, tribunals and test results

Of Isaac’s two weird arse (rare) mutations (one from each of us, unknowing that we were in the 1 in 25 people in the UK that carry the faulty gene) my gift was what is known as a stop or nonsense mutation (written out, this mutation ends with an X). This is effectively a stop sign in his coding to make the correct CFTR protein. If only we could throw a blanket over that stop sign, the protein might be formed correctly, fixing the underlying cause of his CF. These trials, and others, give us and others in the last 10% of sufferers for whom the new CFTR modulator drugs will not work for, much needed hope; read more here and here. 

Isaac is keeping quite well right now. His lung function is stable (which is good, but I still dream of improvement, rather than stability). However, his recent ultrasound confirmed continued progress in his liver disease (despite treatment) and the CT scan showed bronchiectasis and mucous plugs in various areas of his lungs. In addition to this, his ENT appointment keeps being pushed back month on month (likely due to COVID) and his sinus issues are becoming much worse (yesterday his sinuses on one side were so fully blocked, his meds came out of his eyes).

I also learned that we need to go to a tribunal to appeal his PIP (personal independence payments, which replaced disability living allowance) decision. They sent me a 120 page dossier to read through, in which I’ll need to contest any points I don’t agree with. It makes me want to scream, weep, hit walls and stuff. The CF trust are kindly advising, but today, I just can’t face it. 

On a happier note, I am still on annual leave, and have been enjoying a mix of sleep, kiddie huggle heaps, long dog walks, reading (I’m going to start adding book recommendations at the bottom of blogs, in case anyone is interested) and a mass house sort out. I’ve loved that. 

Stay safe out there. We’ve had a handful of bugs in the house these last weeks, but so far, no COVID. Main problem now is access to the damn LFT’s x 

Book recommendation: The hearts invisible furies, by John Boyne. I loved this so much. A little like Elena Ferrante (who I adore; look up the Neapolitan series, if you are not familiar) in that it takes place over a number of years and includes tragedy and injustice, but with the odd giggle too, and a narrator you come to love. I’ve gone on to read a few more of his works recently. He is author of ‘The boy in the striped pyjamas’ - but his repertoire is so much wider than just that. 

Pics: Anouk and I at Christmas (she takes a much better selfie than I can ever do!). Showing my old man and sister about filters (♥️). And seven years of our gorgeous Obie (German Shorthaired Pointer love).