Christmas, bugs, gratitude

Isaac’s cough continued. We gave the Cipro a few more days, to see if it just needed a little more time. On the downside, his chest sounds wet, and his huff, crackly. But on the plus side, his lung function is stable, and most importantly, he feels well in himself. So to bide us some time, and see if we can clear this cough without more IVs, he has started a mega dose of the dreaded steroids. Dreaded because they can affect his mood, his appetite (ludicrously so) and face (look up moon face). Also because once he is on such a large dose (40mg) we can’t just stop them suddenly, we need to gradually taper the dose back down to nothing over time. There are many other side effects too, so they don’t put him on them lightly. But all that said, they work, so ho ho ho. 

Christmas holidays has bought with it our annual dose of bugs. The girls have both been ill - but forever looking for silver linings, we’ve had a lovely couple of days on the sofa watching films with our little fevering and sleepy roast potatoes, and we hope to have the bugs out the way before a big New Years party (this years theme; pub names). 

To everyone who reads this blog; helps me to spread CF and organ donation awareness; supports our fundraising: thank you so much. We wish you all a fabulous break, and an even better new year x  

My present to you, a genuine, award winning photograph, showing two mice seemingly wrestling at a tube station. 

BEST THING EVER! 

Coughing and monopoly

Yesterday we held our annual raffle at my work in aid of the CF Trust. Some friends and I started this in 2006 (I have been there a looong time, and when you read more about my amazing colleagues, you will understand why). In those days, we used to receive gifts galore from suppliers; chocolates, booze, hampers of goodies, even a PS4 one year. We agreed that rather than try and divvy these up between us, we would make it both fairer for all, and fundraise at the same time, by holding a raffle. I can’t remember how much we used to make in those early days, but I guess it was around £200-300; which seemed incredible! Then we added cakes; each year, the same dear colleagues would bake, and we’d sell them on the day. Over the years, due to much stricter compliance rules, we have less and less from suppliers, and yet the raffle gets bigger and BIGGER. 

Not only do my colleagues donate prizes to the raffle, they bake to sell cakes, and buy tickets to win prizes back - all for CF, and yesterday we made over £1,000! 

Every year, I try and pluck up the courage to say thank you in person at the raffle, but I know I cannot do this without tears. it means too much and I know I wouldn’t be able to articulate my gratitude sufficiently. I will try and make up for that now; a huge thank you to Heather, Charlotte, Beth and Debbie for selling and sorting tickets, and to Richard and Paul for being my voice (and to the many others over who have been raffle compere over the years), to Gem, Sophie, my Mum and many others for baking, and to everyone who buys tickets or cakes. What I would really want to say if I could do so without crying is that CF sucks, truly, there is no getting round that, but thanks to all of you, we have more HOPE, and for that, I thank you all from the bottom of my heart. 

Today Isaac went to hospital for tests, as his chest is getting progressively worse each day. He is coughing so much he is sick at least once a day now. We are only two or three weeks post his last IV antibiotics, and having to start a course now would mean a certain home IV Christmas (or worse, being in hospital) - but we feel like we may have been given a get-out-of-jail-free-card; he’s had bloods and results on Monday will show if his markers for aspergillosis are up (previously referred to here as the f**king fungus) in which case, oral anti-fungals may help. Also, he responded well to salbutamol on his second lung function test, which suggests he is wheezy; which suggests this might be more down to inflammation than increased infection; which suggests he might respond to oral steroids. Again, less intensive treatment than IVs for our family Christmas at home. 

Don’t get me wrong, having the aspergillus back and/or further inflammation (which causes the scarring in his lungs) is not something we’d wish for, but we also wish for him to have a lighter treatment load and less trips to hospital over the holidays. He deserves that. We need him well, and quick. 

.... And then we need a well funded and resourced NHS; access to European research trials; and quick access to new drugs that come out of those trials (not the three years it took for NHS England to agree to fund Orkambi). We need Trikafta (the next of these drugs) NOW and then we need a new generation of these drugs in the future which will also work for Isaac and the other 10% of CF sufferers that these drugs will not yet work for. I will never stop until Isaac has that chance, but today was a sad day in that the election results, I think, hinders rather than helps our chances of this happening any time soon. 

Have a great day x 

A blog by Rosa, age 7

For Christmas I want to give presents and get presents. I already done my presents for my family. My dad, mum, my brother and sister. I’m going to get my dog, Obie, something too. He likes toys. But most of all, I want Isaac to be well. Because I don’t want him to go to hospital at Christmas or cough all the time. This is some pictures of me, my sister, and my cousin. Isaac doesn’t like pictures taken of him, because he is a smelly teenager. Love Rosa. 

PS: We have started extra oral antibiotics to try and knock his current exacerbation on the head, hoping this will be enough to see us through Christmas.  Lizzy x

Cough, rattle, roll... and a new blog.

*Warning, this post will mostly be uber medical, factual and scientific, with high quality data and graphics to support my descriptions. 

Just a week or two post IVs and Isaac is coughing much more. This is not supposed to happen.

It’s hard to describe, but much of what I know about how he is feeling comes from 14+ years of listening to his breathing, holding his chest, feeling the rattle, hearing that crackle, asking him to huff and hearing the resistance. He can, and occasionally will, also tell me with words, but I still have to do a lot of filling in between the lines, as he would much rather gloss over whatever he can, to put off extra treatment. That only works so long, and my job is to ensure it’s not too long, and he’s too ill. Tonight he actually asked for percussion physio, which is telling of how he feels. 

The timing is not good, and we’ve been here many times before before, doing endless calculations; If we try orals first, and they don’t work, we risk being on IV antibiotics over Christmas. Jump to IVs now, and he won’t be happy, having only just come off them, and there is still a chance this is viral and he could physio the arse out of the mucous and gets through this. But leave anything too long, and he risks getting ill and needing to go into hospital just before Christmas... 

The below graph illustrates what we can expect for lung function over time with progressive CF disease. 

Breathing we know to be a pretty handy thing to be able to do, and CF disease is not massively conducive towards this; 

Below is how we might expect maintenance IV antibiotic treatment to help reduce this decline; he dips, we start IVs, he gets worse before he improves, and then we see have a good spell of wellness until we repeat again, and again, and again...

Only this is what it actually feels like; 

The added stress at this time of year is always that we have a BIG FAT LUSCIOUS HOLIDAY that we desperately want to be home and well for, both for Isaac and his little sisters. 

Our lovely Anouk has asked to start a blog about girls and football, please support her idea by clicking here! She would love some followers. My budding blogger ❤️