All is merry in Moly world

Kids are all well (ish) and excited about school holidays. Apparently 'baby cheeses' will bring chocolate eggs because somebody died. So that's good, hopefully I won't have to buy the chocolate this year, only spend the next few weeks picking up the little teeny tiny bits of wrapper that's scattered all over the house. 

Brexshit continues, but I'm not allowed to say that now, because it makes me a 'remoaner', apparently. Anouk (age 8) asked me what all this means, and I was tired, so it seemed easiest to just say 'immigration built this nation, but sadly some kids still don't like to share', and she replied 'jeez, some adults are silly'. Too true baby! She is a clever one that kid. 

Sent Rosa (age 4) to school in flares today. Her bottom is so small she looked like a triangle. So cute! 

Trialling Isaac (age 12 - Can you tell I'm a Programme Manager with those age gaps - scheduled to perfection!) on packed lunches. Since he started Secondary School he has been buying lunch there, but frequently not taking his medication with his food (which results in malabsorption - in other words, not digesting his food properly, resulting in tummy aches, runs, and gaining no goodness from his food). This has had mixed results, as he is taking his medication more often, but also helping himself to school dinners after his packed lunch, and so under dosing anyway! 

Have a great day x

Spring.

Sorry for the silence. It's not because we've been away in hospital, thankfully. The steroids and extra oral antibiotics seem to have done the trick, and he seems better now than he has all year. The 'roid rage I blogged about before has calmed, and we're getting back to our normal. I can't tell you how relieved we all are. 

We're having mixed luck on the dry-powder inhalers that he is trialling again, which we hoped would shorten his treatment times. One (TobiPodhaler) he quite likes, but the other (Colobreathe) he can't tolerate again. He alternates months on Tobi/Colomycin to try and trick the bugs a bit, as they work in slightly different ways, so it's likely he will now alternate Tobi podhaler and Promixin through the Ineb (will make sense for my CF friends). So, better than before, but not as good as we had hoped. He also seems happier in himself, and is beginning to take his Creon in school (hallelujah!) so is feeling much better tummy wise. Meeting with ENT next week about his sinus surgery, which can't come soon enough. He's had months now of not being able to breathe through his nose which is a bit poo. 

In other news; We had an explosive cistern water leak in which I was sprayed with toilet water for ten whole minutes; our games room is complete - from garage to pool room and the kids love it; we have a new car, like a 'proper grown up car' on which the wing mirrors are not held on with duct tape and body is beautifully dent free - amazing; my best friend is having a baby boy!; and summer is coming...

Have a great day x 

'roid rage.

We're in the frustrating and somewhat odd position of choosing whether to go into hospital or not. We were in last week for his annual review, which involves tests galore; Bloods, blood sugars (diabetes test), urine, X-ray, lung function, bone scans, ultrasound, along with his usual o2 sats, weight, height, CF nurse checks, psychologist, physio, dietician, pharmacist and his consultant. 

Some of the results are immediate, some take longer, and considering his cough right now, things didn't look too bad. But on the otherhand he has been symptomatic since Christmas, with only the odd day off (quickly ruined by a pesky virus). His cough is wet, fruity and productive. The worst kind.  

Before CF I imagined medicine to be a more precise science; Diagnosis, prognosis, treatment. But I've learnt that there is a whole frickin array of Farrow and Ball greys inbetween... His lead consultant (who we love) took the time to debate the various options open to us, even bearing in mind that he has a birthday treat in two weeks time that he would hate to miss. Knowing that a course of IVs is usually two weeks long, but anything from 10 days would be acceptable, we opted for a weekend at home on high dose steroids to see if this could be an inflammation reaction rather than rampant infection (we already know he has pseudomonas lung infection, but how rampant that is right now? Still up for debate). We also agreed that as he's had a bad run of things, and has missed a lot of school already, an IV course could be done at home (For that, we love her even more!). 

Home IVs typically means; admitted to ward, cannula, first dose, long line, second dose, check for reactions to the drugs, I revise my nursing skills, then home in a couple of days to complete the course. Excellent. We do need to go back in every 2-3 days for blood tests, and immediately if we have any problems with his line, but we're incredibly lucky to live 20 minutes away. We used to do this more often when he was younger, but they changed the policy a couple of years ago, and now seem to keep us on the ward longer, at least until lung function is increasing, but we feel we can do more at home (physio/fitness wise) and reduce the risk of picking up more bugs by staying home. Plus its better for the whole family. 

Anyway, here we are, with our hyper, raging, ravenous boy..... Did I mention before the side effects of high dose steroids? I can vouch for the majority of this list....

 

So this weekend, we headed to my Dad's caravan in Norfolk for fish and chips with wooden forks, sea air, walks, rest, and family time. It's been as lovely as it can be with the crazy beast that steroids presents (constant mantra being 'it's not his fault, it's the drugs, it's not his fault, it's the drugs....) and as agreed we'll make a call in the next couple of days if his cough is improving (superb) or not (IVs). These steroids are short term; he has previously had months of steroid treatment, and the side effects are always the same, so feeling that we're kind of getting off lightly at least. 

On the way home today, Rosa asked me, 'when you're a Grandma Mum, can Isaac be my Daddy?' Sibling love ❤️ X 

 

Clinical disappointment.

In tears tonight reading this. My hopes once ran sky high for this drug, that could potentially be the first to treat the underlying cause of Isaac's CF. 

For my beautiful boy and others with class 1 (stop/nonsense) mutations we must continue to campaign for research and treatments that target these rare (and reportedly more severe) mutations so that we are not left behind as the *untreatable few. 

*Only 10% of the 70,000 CF population worldwide have these kind of mutations. 

Plan Be.

We spend much time second guessing when Isaac will next be admitted; too much time. Currently we're on Plan Be, as in, what will be will be. He had a good 48 hours of feeling better after weeks of coughing, only to come down with a tummy bug, which has set him right back again. Five days of fevers, and although he is no longer feeling sick, his chest seems worse than before the Cipro - those bugs that camp out in his lungs are opportunistic little bastards. 

The question now is whether we can eek this out until Thursday, when we're due into hospital anyway for his annual review (full day of tests galore), or if we need to take him on sooner. Even Isaac has resigned himself to thinking that he'll need to be admitted, which is not like him. 

For all of us, being admitted is our worst case scenario. Isaac, Dan and I know this as a necessary evil, but it's not so readily accepted by Anouk and Rosa who are too young to completely understand. Even with the most supportive and wonderful extended family in the world, the impact of hospitisation hits wide and hard, in many aspects of our lives. 

I am just back from a short trip to Madrid for work, and for now, I'll leave the suitcase out just in case, awaiting ideas from our fantastic CF team. 

Good night all. More soon x