It’s Christmas!

We are home. We are well - the best gift I could hope for. We are very merry in the Moly household. 

Thank you for reading, and wishing you all a happy and healthy break if you are having one x 

Have some pictures of Isaac and his lovely cousin Ben. How they change! A constant reminder of the seasons of our lives. We are big fans of cousins in this house, and after years of only a few, the kids now have a new influx of babies to love on, and this Christmas will be all the more special because of them. Merry Christmas babies! 

Happy tweens, grumpy teens! 

Bonds between people who have not, and cannot ever meet.

Isaac is doing really well. One week into IVs and his lung function which was down 20% on his baseline (already not great) had increased 13%, which for one week on is fabulous. So for now, we continue home IVs, and all going well (symptomatically) they will end next week - perfect timing for Christmas. 

Good news for us, but sad news from the wider CF community this week. This is a unique community in that we cannot meet in person without high risk of cross infection (...pretty sure this is unique, as I’ve not heard of it for any other chronic illness? But I could be wrong, t’is not rare!). The bugs which inhabit CF lungs so merrily come in many strains, but from just a few species. They realised back in the eighties that certain strains were prevalent in certain CF centres leading in one case (the Liverpool strain of Pseudomonas) to many deaths. And so came about the end of CF holiday camps, sitting in waiting rooms with other patients, and the start of segregated clinics and beds on the ward so far apart you would never see the other patient. The closest we come to another CF patient in person these days is a knowing smile and a wave across the hospital concourse. As a result, the internet provides those affected by CF our only means in which share knowledge and learn from others who really understand life with CF and what that entails. In those early years, getting over diagnosis, the CF parents forum was my lifeline (shout out to Softie, a kind a generous soul who had CF himself, but spent much of his life advising us parents of newly diagnosed kids from the risks of sandpits, to how to sterilise neb parts, to how to cope with questions from others such as the common ‘that’s the one where you bash them, right?’). 

And as in any community, there are some faces that stand out, the social media ⭐️’s of CF so to speak, and this week we face yet another death of a young activist, advocate, and generally lovely person. In my time as part of this community, I have known far too many die (far too soon) and while CF affects sufferers in very different ways, these losses have broadly fitted into three kinds; 

1) Those who decline in health, usually in their teens or twenties, and are listed for a lung transplant. 

Of these, there are the in the 1 in 3 patients who tragically die waiting for lungs due to a shortage of willing donors (please sign up here ). 

Or those who do get a chance at a new life with new lungs, but die following complications post transplant (as with my friend Jayne. Who I miss so much). 

Or those who initially do well with new lungs, but who later suffer from rejection (as was the case for beautiful Eva, who inspired so many). 

2) Then there are those who decline in health, usually in their teens or twenties, for whom transplant may later become an option, but who tragically die from an infection very suddenly. I am not sure of the details, but in Lea’s last vlog she casually mentions she is starting a new IV drug for her current infection, pretty normal CF stuff, and yet a week or so later, she has passed. 

3) Those who decline in health more gently, and may survive their twenties, thirties, even forties before being listed for transplant (if they even opt for transplant). Or have a transplant earlier, and avoid rejection for many good years. 

Now, it makes perfect sense that when you are at your sickest, you’re much more likely to be home, alone more, and online. Whereas when you are well, you’re more likely to be out and enjoying life too much to be online, and so the internet can skew perceptions. I get this. But one thing is for sure, I know of many, many more in the first two categories than I do the last; and this can be pretty terrifying (‘sudden decline’ especially after our stint on intensive care last year. Too close. Too real). 

While the internet gives us an amazing forum in which to talk about our experiences, and share fears and triumphs with the only people who really understand, it also creates bonds between people who have not, and cannot ever meet. I would not be without my CF community, but with this comes pain and incredible heartache too, despite us never having met.

I shall miss your smile Lea. Sleep well x 

To support our fundraising for the Cystic Fibrosis Trust who fund seriously groundbreaking research, please click here. CF is actually one of the areas of medicine where a cure is a realistic idea, but they lack the funding to achieve this potential. As such, myself and a bunch of my amazing friends will walking the Great Strides walk in the Surrey Hills in June 19. For me, a bonkers 67km in a single day 😬 x 

Lakes, walks, coughs.

Today I walked round the lakes with Obie, headphones on, music loud. Cleared my head some, as I was feeling pretty miserable when I woke. Although I’ve gradually moved his IV times so they are now a reasonable 7am and 7pm, I keep waking up at 5am, irrationally fretting until 7am that I might sleep through my alarm (which I never do).  

Isaac is a smidgen better. Still coughing but he hasn’t been sick since Friday (he coughs and coughs and coughs so hard he is sick when he is this ill). Should be a good sign that he is turning a corner, but not as well yet as I hoped he would be by now on IVs. He has repeat lung function tests Wednesday. We need him better by then, otherwise we could be looking at an admission. 

The deal I have with Isaac about this blog is that he doesn’t care what I write (he agrees awareness is a good thing) but he doesn’t like pictures of him looking sick (fair enough), but if you would like a better idea of what a CF cough can be like, you can check out this couple who vlog daily about life with CF. Gives you a fair idea of what Isaac’s mornings look like right now.

My walk today:

The far reaching affects of Cystic Fibrosis; 



Organised me a new IVs cupboard (this makes me very happy): 

Hoping to write a jolly Christmas blog soon x 

No better.

At least not yet. He is still coughing so much he is sick in the mornings. Hoping this is just the bacteria-busting super powers of ceftazidime and tobramycin doing their shit before a much needed ceasefire in his lungs. Pseudomonas, we know you’ve set up a permenant camp in there, but hell, you need to know your place! 

Perspective

Children with cerebral palsy lick milk powder off a bed to feed. Image credits: Lu Guang

Qi Guihua, held here by her husband, fell ill when she returned to the village from Beijing to celebrate the Spring Festival. She died two hours after this photograph was taken. Image credits: Lu Guang. 

Drugs and beds.

Unsurprisingly Isaac started IV antibiotics today. He is no better, and his lung function had dropped a further 20% in two weeks. 

In true Isaac style, despite coughing and spluttering throughout clinic, and giving them a deliciously juicy sputum sample for the lab, when they asked him how he felt, he just replied ‘Alright I suppose. Yeah, fine’. He usually has cold spray (numbs the skin) to access his port, but for the needle today he chose to have nothing, and hardly flinched. If he could have carried on watching you tube he would have. He amazes me daily. 

But the wards are full. There is no room at the inn.... HA! This shouldn’t please us, but it does. We can stay home and I’ll do his IVs here, and this makes us very very happy. They would rather have us in, for the physios to put him through his paces, but we can and will do just as much here (and avoid picking up more bugs on the ward). Administering his drugs myself is tiring (I’ll be up at 5am, to glove up and mix drugs) but for Ise, it means he can sleep through the doses while I creep around his bedroom in my plastic apron. I cannot fully explain to you how much less exhausting, frustrating and life intrusive this is for us than being on the ward. 

Best case, we do two weeks of IVs at home. His lung function improves, and most importantly, he starts to feel better. Then ho ho ho we can hope for a wonderful and well Christmas. However, if he doesn’t improve pretty quickly, feels any worse, or his labs show he is growing something other than his norm (PseudoA) he is guaranteed an emergency bed as that would mean drugs or treatment that we cannot give him at home. He really really doesn’t want to go in, especially so close to Christmas. Please let this work x 

Still home. Still sick.

Apologies to anyone who read the last post and worried. We are still home. Saturday was awful (I can’t quite explain how utterly heartbreaking it is to watch your child cough (and be sick) again and again and again, day and night. To have nothing to soothe their rib pain, the head pounding, the sore throat...) but Isaac stood his ground in light of his many many previous experiences in A&E, and insisted we wait before going in. Credit to him, he knows his mind and body, and Sunday he was coughing a little less, and so by then it seemed best to wait for Monday to be reviewed by his own doctors. 

But come this morning (Monday) we were told the soonest he could be seen would be tomorrow anyway. The inn is full. The wards are packed, and after talking to his new CF nurse it sounds like our only way to be seen by a respiratory doctor in future is on clinic days (Tuesdays and Thursdays) or through A&E. This is new and miserable news for us; we used to be able to call any time Mon-Fri and one of his team would always be able to fit in a review if he was unwell. So short of fitting his disease around clinic days, we need to sit on symptoms or brave the contagious in A&E from now on. 

I’m sorry, I’m tired, I’m angry; I’m not blaming anyone, but I’m sad (and mad) at the state of our beloved NHS. 

Clinic first thing tomorrow. 

The drugs don’t work/It’s beginning to look a lot like....

..... Hospital. 

We’ve come to the end of two weeks of Cipro, and he is no better. He came home sick from school yesterday, O2 saturations around 94 (anything below 93/94 and he really should be on some oxygen). He coughed and slept his way through the night, and this morning I was ready to pack our bags to go. He coughed so much he filled three cereal bowls with sputum and coughing so hard he threw up. 

Since then his sats have drifted between 90 and 98. As it’s the weekend, our only option is to go through A&E. Although he has this ‘open access’ to hospital, it’s kind of meaningless as he still needs to be reviewed by a doctor to be admitted. 

What haunts me is that we had a similar weekend last October when he became much more symptomatic on a Friday, we thought about A&E on the Saturday, he seemed slightly better Sunday, so we eek’d it out until Monday morning to see his own Dr and go straight from clinic to the ward. Whether things might have been different had we taken him in sooner, I don’t know, but by the Thursday he was in intensive care. 

So why are we sat here at home still? 

Isaac is adamant that he doesn’t want to go to A&E, and he increasingly wants to have a say in his care. I half convinced him earlier, and then his sats said 98 and he told me ‘see. I’m fine!’ between coughs. Last time we were there, we sat for hours, with the contagious (so not good) the drunks, and arguably worse? The people with a stubbed toe or a sore throat (thank fuck for triage). Plus, he argues that everyone at school is sick with the same thing, so it must be viral. True, but his friends don’t also have CF, they don’t have extra sticky mucous they can’t clear, and they won’t have an army of Pseudomonas bacteria already living in their lungs, getting ready for the party a virus lays out for them in his weakened lungs. 

I called clinic Thursday and they said they would get back to me. I knew the Cipro would run out tomorrow morning (Sunday) and it already looked like a bad idea to stop the one thing that might just help a bit until he gets on IV antibiotics, so at the very least, I wanted a prescription for more. But despite calling again and leaving messages three times, I heard nothing back Friday either. This is not like our team, and is most likely a sign the wards are full anyway. 

What we need is 24 hour wraparound care with the team that knows him. What we have is a weekend with limited access to consultants, a hugely overburdened A&E system, full of strangers to him and his health. We’ve been admitted before at weekends only for them to put off treatment until he could see his own team on the Monday anyway, as CF is complex, and registrars are reluctant to make decisions.  

He is asleep right now (4pm, and this is the kid who never naps). I’m sat here listening to his every breath/cough, ready to argue that we have to go in when he wakes up. This hurts. I can’t tell you how much I hate CF.