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 ❤️ 

Thinking of others

Last week, on his very last day of a course of IV antibiotics, Isaac called me at work to say 

‘MUM! I PULLED MY LINE, RIPPING OFF MY SHIRT, AND THE NEEDLE IS HALF HANGING OUT IF MY CHEST!’

My usually very cool and calm teen was, quite rightly, a little freaked. I jumped in the car to get home, only for him to call quite calmly two minutes later to say the needle had dropped right out. On reaching home, thankfully his port area looked pretty unharmed. 

The next day we had to head into hospital to be re-accessed, meaning the needle being put back into an already sore port site (I am half-trained to do this, but have not yet done). We have to do this to administer the final line flush he needs at the end of every course of IV antibiotics, to keep the line from blocking. Despite the accidental ripping out, and being re-poked the exact area the next day, he did not complain once. 

Isaac went into this course of IVs feeling pretty well (but needing them to keep his infections at bay and set him up for a better winter) and unfortunately, his post IV lung function tests show a dip in his lung function by the end. It shouldn’t be this way round. But we suspect this is because everything is all stirred up right now, and with some extra physio, we can clear some mucous out, and then he will see the benefits. Dan has also had his long awaited knee surgery and is doing super well. 

To give children with serious illnesses a gift please follow this link to buy gifts that will delivered direct by the wonderful Starlight charity for distribution in hospitals this Christmas. Some in the wish list are just a pound of two, but every gift makes a difference. 

Some dear friends of ours are going through the unimaginable right now, which is always immensely perspective making. Too young, too colourful, too soon, too sad. Hug your loved ones tightly x 

Larks and owls

Today I got up early. Gloved up, apron on, mixed up IV drugs. The smell of alcohol wipes at this time is not kind. I give a very sleepy Isaac the drugs. I only need to nudge him, and he rolls so I can get to his IV line. He dozes, handsome, and I marvel at how he got so big and wonderful. I leave him to sleep a little longer, he looks exhausted even while asleep. Back downstairs I get his nebulisers, physio equipment and tablets ready for his normal routine. I have my first and favourite cup of tea of the day, the sun is still rising. 

Then I wake Isaac up, he feels grotty. Then Anouk (who gives THE best waking up hugs ever) and Rosa, who both tell me they have tummy aches (please no more bugs). 

Isaac does his physio, meds and nebs, and drinks warm milk. He looks pale and doesn’t want to eat (start of IVs is always hard, and he is beginning to cough more). I wash and sterilise the neb equipment while the kids get dressed for school. Argue with all three about whether they are well enough for school (they are). I pull on the least creased clothes I can find. From upstairs I hear Isaac begin to vomit; into the kitchen sink. Sigh. Consider throwing everything that was in the sink into the bin, but the sick is just milk and tablets, so clean up best I can and throw it in the dishwasher. 

I then realise all school shoes are muddy, and scream internally. Rush girls to school, walking Obie at the same time. Shoes get muddier still as I have to venture further into the park than planned to pick up a poop, and Rosa follows me. I stop caring about mud. Rosa cries as the teaching assistant takes her hand and coaxes her into school; this happens everyday still, and it never gets much easier. 

I get back to an empty house. Isaac has managed to go to school, despite feeling rubbish. I look into the games room (our converted garage, a den for the kids) for his discarded clothes, and the bloody door handle breaks off in my hand as I lock up. I can’t then open it. Shit. 

Then I call our community nurses to see if they can come to do the blood levels Isaac needs today. No answer. Just after, our CF nurse calls to say the community nurses can’t come, and we need to take him into hospital this afternoon instead. Community nurses then call to say how sorry they are that they can’t fit us in. So lovely, we know they could if they would. So much of CF care is spent on the phone organising things; Dan and I both work full time, and just the admin of CF can feel like another job on top of that. 

I have a work meeting 1-3 I can’t miss, so call Dan, he will sort it out, thank f**k. Confess about breaking door handle, maybe I don’t know my own super strength? Then I call school to tell Isaac about the change of plan for the bloods, but I can’t get hold of the usual wonderful lady who helps me out with this kind of stuff, so eventually leave a message with the lady on reception who kindly promises to track him down (at lunchtime, in a school of 1200 kids). He is expecting bloods at home, so won’t be pleased to hear he’s got to go up to the hospital again. Feel guilty that I’m not there to explain. 

Home delivery of Isaac’s drugs turns up - 4 huge boxes full of drugs which I need to fit into an already packed meds cupboard. Realise they have sent more saline that I don’t need. Now have 420 vials. Sigh. Drink more tea, staring at the boxes ineptly. 

Then I call into my work meeting (on Skype, thankfully working at home today). One hour in, Isaac walks in. Dan’s dad has kindly picked him up to take him to the hospital, but Isaac is refusing to go. Spend ten minutes pleading with him, whilst also trying to listen to the meeting. Explain that he must have the blood test as his liver and kidneys could fry if the blood levels were too high, and he could even risk deafness. It doesn’t work, but a promise of Sams kebabs tonight does. I get it (not the kebab bit, I’m a veggie) - we were at the hospital for 5 hours just yesterday and he’s tired. 

Then I finish work and run to school for a meeting with Rosa’s teacher. Our darling girl is struggling with school and life in many ways that I won’t go into here. The teacher is so kind. Having spoken to Rosa in a number of different ways, they feel that much of her anxiety and behaviour is down to her worrying about Isaac and her attachment to me, wanting to be with me, always. She is so so loved, but this has not yet given her the skills to embrace life outside of our home. I walk back with Rosa in the grey rain, glad to have my hood up so she doesn’t see that I’m crying. Feel guilty x1000. Get home. Dan has sorted out Isaac’s meds and nebs and he’s already doing physio. This gives me time to make a den with Rosa and cuddle her very hard. 

Then it’s back to mixing up IV drugs for the evening, and I hook him up to the Tobi infusion. I talk Isaac into drinking the liver-saving medicine again that caused his vomiting earlier (this stuff tastes badddddd). Anouk and Dan go to football. I cook (lazily; pasta) and set up IV drugs ready for first thing tomorrow, when we’ll do it all over again. Isaac uses pliers to get into the games room, and I crack open a bottle to toast surviving the day. 

Dan and I are very different in one particular respect; him, the lark, up at sparrows-fart every day for work/football/fishing, never able to sleep much past 6am. Me, the night owl, the bookworm, often found lost in words or music until late. So getting up for weekend IVs is a struggle.... but sinking down afterwards, back in a warm bed, cuddled up with one of my gorgeous kids, ahh.... double the pleasure... then I sleep like an angel. Have a great day x 

Klee ❤️

Sisters ❤️

I will scream my lungs out till it fills this room

Today NHS England finally came to a deal with the pharmaceutical company Vertex that means the 90% of CF patients who have the most common CF mutations will have access (within 30 days) to the first drug that they will ever take that treats the underlying cause of the disease, rather than only its symptoms. Read more here. 

Orkambi, Symkevi and Kalydeco are the first iterations of these small molecule drugs that will limit disease progression by allowing the normal movement of salt and water between cells. These drugs are not a cure, and the long term positive impact of taking them is not fully known, only time will tell, but even newer iterations of these drugs (like Trikafta) are already known to have a more dramatic impact and should be licensed here soon too. Today’s deal should pave the way for these future treatments. For children born today, the hope is these drugs will halt progression before the irreversible damage to their lungs ever occurs. 

This news (after 4 years of intense and passionate campaigning by people with CF and their families) is long overdue, and very, very welcome. 

Isaac is not a candidate for this drug, or others in the same pipeline. Unfortunately he is in the 10% minority that this drug will not work for (see blog below which explains why). But there is other research ongoing and pending clinical trials that may work for his rare mutation, as well as other drugs that are not mutation specific; but these are frustratingly a few years behind. We need to keep him well until these are available, so we continue to fight the progression of this shitty disease. 

If the momentum behind the research seemingly slows, then you will hear my screams. #don’tforgetthe10% 

Today is a very happy day. Next week, we start IV antibiotics again in the next round against the bastardly bugs camping in his lungs x 

I will light the match this morning so I won't be alone
Watch as she lies silent
For soon light will be gone
Oh I will stand arms outstretched pretend I'm free to roam
Oh I will make my way through one more day in hell
How much difference does it make?
How much difference does it make yeah
I will hold the candle till it burns up my arm
Oh I'll keep takin' punches until their will grows tired
Oh I will stare the sun down until my eyes go blind
Hey, I won't change direction
And I won't change my mind
How much difference does it make?
How much difference does it make?
I'll swallow poison, until I grow immune
I will scream my lungs out till it fills this room
How much difference?
How much difference?
How much difference does it make?

How much difference does it make?

Pearl Jam. 93. 

Not having IVs, and time.

Despite a funny week where Isaac’s chest has been up and down (interspersed with some delightful vomiting episodes) his lung function at clinic was surprisingly stable. This is good news, and gave his argument to delay IV antibiotics some pretty punch. In the end, his consultant agreed that he can delay, but only by a week or two. This means our plan to time this extra treatment over school holidays is scuppered (he would miss less school had they been over half term, and we would enjoy a calmer week, balancing treatment and life, as I am on leave for half term anyway). Instead, we’ll being having them very soon anyway, with the added stress of super early mornings before the school rush. 

But I get it. He deserves to enjoy his half term. And while it is a bit scary for us, he does need to be more active in decisions about his own care. We hope now that he stays well enough in the interim that home IVs, when he does start them, are still an option, because if he is not well enough we will end up on the ward again, which no one wants. 

In other news: This week has been filled with incredible news for the CF Community (not that you would know it here, while Brexshit is going on) as a new wave of drugs are gaining approval around the world that treat the underlying cause of CF, and have the potential to dramatically change the course of Cystic Fibrosis in up to 90% of patients. I cried hearing that the US FDA have now approved Trikafta, the stories I have heard from people already on this drug and have felt the impact within mere hours or days (from a pill!!!) are pretty frickin amazing. Each iteration of these small molecule drugs is proving remarkably more powerful than the last. Given that treatments in CF tend to be of a more time or medically intensive type (multiple daily nebulised drugs, and frequent IV antibiotics), the idea that an oral drug each day can have such an impact is pretty mind blowing.  

Then I had a good cry last night, thinking of all those sufferers lost (friends included) that this has come too late for. And I must admit, that as Isaac is in the 10% of patients that these drugs will not work for, a renewed scream for us; the last 10% must not be forgotten (Isaac’s mutation is better explained here.) 

I am so filled with hope, anticipation (mixed with a smidgen of distrust and worry) for what might come for us, and (crucially) when. But bloody hell.... I know this is not a cure by any means, but it is definitely the closest thing to a cure the CF Community have ever come close to! 

So we wait in exciting times. 

It has been 15 odd years now since these kids of ours exploded into my heart... lately I’ve been feeling that time has suddenly seesawed such that I have less time left with them as kids than I’ve already had. I am a sentimental fool at the best of times - but the swing from wishing for more sleep and less worry that they will choke on a grape, or lick a slug... to ‘will they even want to hang out with me much longer?’ has swung too suddenly. 

If you have little ones, enjoy them while you can guys x 

Anouk. 

Rosa (insisting that her baby cardigan still fits as a crop top).

And Isaac. Since he hardly lets me point a camera at him these days, enjoy a mini-Ise x 

Rebel and flu.

We had a double whammy hospital trip yesterday.

Dan went in for a procedure I do not know the name of, but sounded something like cavity wall insulation to me. He had thrombosis earlier this year, which was treated, and then came back, and so they squirted a kind of foam into the now rubbished vein to stop it occurring again. Our total reliance and ongoing adoration of the NHS knows no limits..... right now he is also waiting on a hernia op and knee surgery! 

At the same time Isaac went in for the lineogram to check his portacath, which as suspected revealed he has a *fibrin sheath. The test involved his port being injected with dye while a CT type scanner zoomed in close above his chest him. I stood there in my heavy lead apron, as I always do, making sure that he was OK during the tests. Only now, my murmurs of encouragement are met with a ‘shush Mum’. My not so little boy needs me less and less. 

It was fascinating to see exactly how far down into his chest the port line goes (his port is sited close to his collar bone, but the line reaches near the bottom of his rib cage), to follow the dye moving down the line as they picture it, and to see where it goes wrong. Our consultant still needs to review the images, but initial thoughts are that the port can be used; the sheath means the line won’t bleed back, but the IV drugs are entering his bloodstream properly. We want to use this port for as long as possible, both to avoid more surgery and to prolong the time we use each port-site available to us. 

Another new; chatting to Isaac yesterday about his impending IVs (due to kick off next week) he is now adamant that he doesn’t want or need these. In true teenage style, he won’t really explain his thinking about this, other than...

‘NO, I’m not doing it, I’m fine, I don’t need them’

Only he is not fine. He is not bad, but he is not fine. And yes, we could negotiate with the doctors to delay this course (I’ve left it with Isaac that this is a decision he needs to make with his doctors next week) but I’ve reminded him that delaying further will only increase his risk of getting too sick that the IVs need to happen in hospital rather than at home. Or worse, delaying and he gets so sick and he ends up needing more than a two week course and it drags into Nov, Dec... (Christmas?). A comfortable couple of weeks at home, half of which is over half term where we can just chill - which makes the early mornings, the schedule, the side effects so much easier to manage, seems like the better plan to me. 

But I get it. It means no showers, no football, no swimming. Being tied to a pump. Your Mum coming into your room in the night, rustling in her weird  plastic apron. Waking up to the smell of alcohol wipes. The sore port site. The constant reapplication of tegaderms. The visible lump under your shirt at school. The fatigue. The coughing MORE as a result of the treatment, before starting to feeling better... 

This is the first time Isaac has ever come close to refusing a treatment plan.... yes, he moans at times about the daily grind (six lots of nebulisers, two to four lots of physio and a ton of tablets 365 days a year will do that to you) but ultimately he has always complied after a short grumble, and understood why he needed to (the threat of a PS4 ban usually covers us).

I’m fairly sure we can convince him, or compromise and push IVs back a little, but not much. Plus I know that he needs to become more active in this decision making process as he approaches adult care (in less than two years) and that the push back is understandable... but my worry is he rebels further (from delaying to ‘I don’t need these at all!’). Non compliance is very common in CF, as the burden of treatment is so high. Plus, I rebelled as a teenager. Why would I expect any less from my bright, strong minded boy? I just hope he never stops listening to me. 

One last thought, please consider having the flu jab this year. If you are not eligible for it on the NHS, you can have it at most supermarkets for less than a tenner. You cannot catch flu from a vaccine. It is safe. You are not using up supplies that are better saved for the vulnerable (they will be covered by the NHS). It may save you a lot of suffering. And even better, YOU WILL HELP STOP THE SPREAD which will protect those more vulnerable around you. 

Have a great day peeps x 

* Fibrin sheaths are a heterogeneous matrix of cells and debris that form around catheters and are a known cause of central venous stenosis and catheter failure. 

Portacaths and ports

Hospital tomorrow for this lineogram to check his portacath is working OK. 

Best case scenario; it bleeds back for bloods and takes in IVs perfectly.

Worst case; it’s not working properly for bloods or IVs, and he’ll need more surgery for a new port (also not good because that is one less place to site a port (you can’t use the same vein twice and there are only so many places on a body that you can have a port fitted). Since the life span of a port can be 5-10 years, we could do with this one lasting more than just less than 2! 

Likely outcome is midfield... it will continue to not bleed back, but will happily take IVs, in which case we accept he needs a separate needle for bloods, but happily stick with this port for his much needed IVs.

Isaac is feeling pretty good this week, which feels pretty weird as we will kick off IV antibiotics next week... but knowing this should set him up with the bug-arse-kicking lung health that we want for winter... bring it on! 

No-deal Brexit-shit-lit: What Yellowhammer says:

• Supply chains for medicines and medical products are "particularly vulnerable" to disruption at the Channel ports
• While some products can be stockpiled, others cannot because they have a short shelf life. It will not be practical to stockpile products to cover expected delays of up to six months. 

So just really super reassuring. Thanks. 

Not seeing the woods for the trees.... 

© Pierre Pellegrini

Weekend with little and Obie.

All is good in the woods and sea. 

My heart, it sings. 

Rosa. 

Planning IVs

Lots of great news today at clinic. Lung function is wonderfully, delightfully.... UP. 

Best it has been in months. His team are pleased with his height, his weight, how his chest sounds, his sinuses... pretty much everything! 

We spoke about transition into adult care (this kicks off around now, so he is ready for the big transfer a little over age 16). He will meet his new team soon and have the chance to see the new Papworth Hospital building where his care will be managed. The Royal Papworth has recently moved to the Addenbrookes site where we are treated now; a brand spanking new building with individual rooms for CF patients (hurray!) all the uber mod-cons (state of the art tech, e.g. air changes every minute to reduce infection risks, lifts segregated by bugs that patients grow...). But still, the idea of leaving peads after our long journey together feels HUGE. We have known many of his team from the start and I’ve had more than a few tear stained hugs in that time with them - but Isaac’s attitude to transition today was fantastic (Isaac very much lives for today, worrying very little about the what if’s or the maybe’s). The biggest thing for Isaac, and me, is that from transition onwards, he doesn’t need to invite me. Mind you, he says there is no way he is going without me, simply because he’s too lazy to remember all the details for the many many questions we have in clinic (When did this symptom start again? When did you last have a CT? When did you come off that treatment? What did you eat for dinner last night, and how many Creon did you take for it? Is your cough more of a wheeze or a fruity one today?....)! 

He also had a port flush, and for the third time, his port is refusing to bleed back. He will have a line-o-gram (inject it with dye and X-Ray) soon to check that the port is still working as it should. We can accept it not bleeding back, it means he will need more needles for bloods, but still means it will work for IV drugs going in, which is the main thing. 

The plan is for IVs in October, all going well, which we have scheduled to coincide with school holidays (as he is starting GCSE years, really hoping to up his (circa 80%) attendance record if we can). 

We spoke about the sensitive balance between wanting an IV free quality of life (and limiting the risk of building up drug resistance and the kidney/liver and hearing damage that using toxic IV drugs so frequently can incur) with not leaving it too long that he is so unwell that it means he’ll need to have IVs as an inpatient rather than at home, or worse it will result in long term lung damage and recovery will be so much harder. 

Vigilance is key. Thankfully it is not just Dan and I with an active interest in this now; Isaac too has a much greater understanding and appreciation of staying well and out of hospital as much as we can (the one silver lining of intensive care memories?). This is the first time since 2017 when we first talked about planned IVs that we have actually planned them! Until now he has never lasted the full 12 weeks between courses before needing the extra treatment. 

Today was a good day. Thank you for reading, whoever you are. Have some random pictures! 

Georgia O’Keefe, wave. 

Nan Goldin. 

Persian rug love (a secret obsession of mine!).

What would no deal Brexit mean to us?

Concerns about the supply of medicines in the case of a no-deal-Brexit led me email the pharmacist at our hospital. I got a reply, that while understandable, was about as helpful as an itchy arse when you suspect the kids might have worms; 

An evening dose of meds. 

‘Unfortunately we would not supply above and beyond normal supplies of medicines. If we started to do that, then the current supply chain would fall down quite quickly before we get to Brexit. NHS England are monitoring the current situation to ensure additional supplies are not being made and stockpiles are not being held. The Department of Health / NHS England are working up strategies for supply of medicines in the event of a no deal Brexit’. 

Weekly tablet organiser (add on inhalers, nasal sprays, nebuliser and IV drugs). 

According to a recent white paper published by the Business, Energy and Industrial Strategy Committee, 73 per cent of pharmaceutical imports in the UK come from the EU. This equates to around 37 million packets of medicines every month from the EUworth £18.3 billion.

This is SCARY shit. 

Now, there are some medications people with CF could manage without short-term (the high dose vitamins which they cannot absorb from their diets as healthy people can, the slow release sodium maybe, the acid inhibitors at a push?)

But the Creon (enzyme replacement therapy, which allows the breakdown of fats and proteins which cannot otherwise be absorbed, of which he takes 15-30 a day, and would have agonising tummy aches and no weight gain/growth without) the oral, nebulised and IV antibiotics, the anti-fungals (in Isaac’s case right now, very important) and the steroids....being without these, even for a short time, could be catastrophic. 

Scarier still, delay to flu vaccines (due around the time of Brexit and which have a super short shelf-life), have already been indicated as a nationwide crisis waiting to happen this year, likely to kill thousands at high risk. Not to mention the risk to diabetics and others with chronic illnesses or cancer. 

Our dry meds cupboard. We have a whole shelf in the fridge too. 

Anyway, there is enough catastrophising already (not only in my own head) - what we need now are answers and assurances that our most vulnerable in society will be protected. Assurances that we can actually believe in. Where is that??? 

On a positive note; we are well over our fundraising target for our epic 43 mile trek in June. Check it out here! Thank you so much, again, to my incredible team for walking over 17 gruelling hours, laughing most of the way, and for all the amazing support we have had. This now means our family and friends have raised over £80,000 for the Cystic Fibrosis Trust since Isaac was born. To all our lovely family and friends, this is all down to you, Love, love, love x 

Isaac said to me tonight...

“I can’t believe I’ve been well this long”.

Which is both something to celebrate, but also to recognise. He was last on IV drugs to calm his lung infections down in June. When did two months become a good run? 

Summer.

This summer we are home (having holidayed in May quite expensively). A mix of work and home, day trips, the pool in the garden (we have an over ground pool we set up each summer which Dan has built an amazing deck around it, meaning the kids jump in and out all day) and long dog walks. Yesterday we had a family wedding (thank you so much to Alex and Becky for not only sharing their special day with us, but also for holding a raffle which raised an amazing £560 for the CF Trust). It’s actually been really good to be home. Dan is likely to have three lots of surgery later this year (hernia, vascular surgery due to repeated thrombosis and a knee op for a torn ligament; my poor broken husband) - he’s OK, but it all needs doing, so it’s good to squeeze in some fun before all of that.  Isaac is doing really well.... the anti-fungal drugs really are reducing his symptoms overall (we think), and right now, the only troublesome side effects are his poor cracked lips which bleed. Having last had IVs in June, we’re feeling pretty smug that he is still so well.... if he can last until at least September (or beyond?!) before his next IVs - that would be fabulous. 

Some rare pictures of my lesser spotted teen, and his gorgeous sisters (and cousins Ben and Bodhi). 

Wishing you all a wonderful summer x 

A bloody mess.

Our lovely community nurses visited yesterday to take a blood level for the anti fungals Isaac has been on the last month or so. This is to check the level of the drug in his bloodstream is high enough to be at a therapeutic dose. Kind of different to his normal antibiotic levels which he has on IVs, which are to check for that, but also more importantly that the level is not so high it fries his liver and kidneys. For antibiotic levels, blood is taken from a finger prick as taking blood from his port might mean we have an abnormal level of residual drugs in his port line (in case you are new to this blog, Isaac has a portacath fitted in his chest, which is a little device connected to a major vein in his chest, and implanted under his skin - this enables us to easily access his vein when we need it, by putting in a short (1 inch) gripper needle through the skin directly into the port - making venous access much easier than inserting a cannula or other kind of line, as after years of IV treatment, his veins are shot). However, for a Posaconazole level, he only needed to have blood taken from his port, which needed flushing anyway (every 4 weeks). Great. Only, blood was not forthcoming from his port. It simply wouldn’t bleed back. This is not terrible (for the Posaconazole level, he ended up having a finger prick instead) but it is frustrating, as it’s possible his port has given up bleeding back for good (it happens) meaning he’ll always need needles for outputs (bloods) and his port will only be good for inputs (drugs). 

Anyway, that was quite a long ramble when really what I wanted to say was that he ended up two needles and two finger pricks in, and a bit of a bloody mess at the end of it. But did he complain once? Nope. 

He’s been really well these last few weeks - but his cough is beginning to increase. I had hoped the antifungals might be the magical factor in him lasting longer between courses of IVs, but now I’m not so sure. At least I can be sure of his fantastic let’s get on with it attitude. 

I wish I were half as strong as my fourteen year old son. 

Have a great day x 

Georgia O Keefe 

Life is beautiful.

Clinic this week. Anti fungal treatment will continue for another two months. Lung function was up 3%. Not exactly party time, but a hope filled step in the right direction. Right now, Isaac is feeling well. 

I pass this boat every time I walk Obie down by our river. It always makes me smile. 

Happy summer everyone x 

Cracked lips, home, and well.

Isaac is home and well. The anti-fungal side effects continue to be quite evil, his bottom lip is so cracked it’s almost a crater. The treatment course is three long months. He complains hardly at all, as per his norm. God, I love this kid. His monosyllabic responses to ‘How was the trip?’ ‘Was CERN great?’ ‘What was it like at the UN?’ indicated a typical teenage blah enjoyment of anything vaguely educational. I’m taking that as a positive. 

Have a great day x

Nicola Davison-Reed

Away Away.

Isaac is in Switzerland. Our fears of him not being well enough to go on this school trip were joyfully dispelled - this week he is visiting CERN, the UN, St Pierre Cathedral, the Natural History Museum and enjoying a boat trip on lake Geneva. School trips have changed somewhat since our day, when a dusty day trip to Calais, to order une baguette ci vous plait was seen as a cultural peak.

While away, he is on physio, tablets and inhalers only, as agreed with his doctors. They were keen he take only what they could trust him to do independently, rather than send all the nebuliser drugs, chargers, part and machines that he might not do, and would need sterilising, that he could not easily do. He has back up antibiotics if he becomes less well to hand, and a whole load of sun block to put on for the side effects of his anti-fungals which make him uber photosensitive. His lips continue to crack and bleed on this toxic drug too. That aside, he left feeling good right now (at least, Isaac good). 

I on the other hand feel anxious. The logical 5% of my brain tells me it’s just a few days! He is fine! He can call! If he comes home less well, we can just increase physio! I’m being neurotic! Unfortunately for me 95% of my brain is made up of irrational fear, love and anxiety when it comes to Isaac. I have spent 15 years now, in utero and beyond, trying everything to keep him well. It’s hard to start to let go and trust him to take up the reins. 

But it will be worth it - what an amazing chance, an opportunity we would not want him to miss (but that we would like to see him home well from after) x 

Post IV lung function

Nothing to celebrate here. 

Nothing to cry about either. 

An increase of 4%. 

Not the 20, 30% of my dreams. 

Not the 10% of my reasonable hopes. 

But no stalemate. 

No decrease.

After another two week course we are relishing in IV free times - more sleep, less schedule. Suddenly, everyday treatments feel like a breeze! 

When I deaccessed him, his port site was sore, open and goey (it may be he needs a new needle size, as it looks like a pressure wound). It’s now healing nicely, TF (infection in the port would be very bad news). 

He has started on a three month course of treatment to try and clear this fungal infection. 

We’ve swapped the drug to one which is a little less toxic than the first week. 

He needs to have blood levels taken to check we have the level of the drug at a therapeutic one, but not so high it fries his liver and kidneys. 

The side effects still required an A3 sized drug pamphlet. 

So far, so good. 

New changes to his physio plan to try and shift the mucous better from his lower airways. 

He continues to amaze me - don’t get me wrong, he is a moody teen like the best of them, but how he takes this all in his stride without complaining is incredible. 

He is going away with school for three days soon - he’d lost a lot of confidence about being well enough to go, but these last few days, seems keen, more hopeful. 

Daring to look forward to something. 

He is better. He is. 

Please let him stay well for this, he’s already missed out on too much.

Thank you for reading x 

From our epic trek in Surrey - almost over £5000 now! 

From Rosa. Age 6.

Isaac sometimes is mean to Anouk and me. 

And he asks Anouk to go and get him sweets at the shop. 

It’s hard for Isaac because he doesn’t like doing his nebs, they taste funny. 

Sometimes it is hard for isaac and we try and make him do it. 

Isaac is brave because he doesn’t do anything when he’s in pain. 

I hope that they will get a potion for Isaac to not have CF. 

I love Isaac to death. 

I feel bad for him. 

I hope his IVs will go away. 

Isaac makes me laugh and he plays with me all the time. He always wants to pick me up. It’s annoying sometime, but I like it. 

I hope the Doctors on his birthday give him a potion to make his CF go away. 

I hope everything goes away that he worries about. 

By Rosa Banosa (of the cheeky cheeky nosa) x 

Robin Williams offering toilet paper to The Thinker, ca. 1990s.

Bronchoscopy, IVs and subsequent results

The Bronchoscopy went as well as we could have hoped. It was a long day of nil by mouth (always a frustration point for my ever hungry boy) with delays in going to theatre. But having been bumped off the list by emergencies many, many times before, we were very glad to only be delayed, not cancelled (please never under estimate the huge, debilitating pressure on our beloved NHS). General anaesthetic was fine, he tolerated it really well this time with no big de-saturation’s (his oxygen levels did not dip badly while he was ventilated). I was on my own this time, so spent a nervous hour pacing the corridors (never gets easier) remembering that last look of him, confused, glassy eyed and unreal before the anaesthetic took hold and he fell ‘asleep’ (under GA looks nothing like sleeping by the way, it is very hard for a parent to see). Recovery was OK; usual sore throat, grumpy, disorientated. All IV access was through his port this time, which was great, meaning he didn’t need a cannula as well. He slept and coughed a lot all the rest of the day as his body recovered. 

We kicked off IVs on the ward, but we’re now happily at home. I administer the IV drugs, and the lovely community nurses come every 2-3 days to do bloods (they need to check the levels of one of his antibiotics in his blood stream regularly to check that it’s not too high. Too high could be very hard for his kidneys/liver to process, causing potential and irreversible damage). All good so far. 

That said, Isaac has been sick almost every day since starting IVs, and experiencing chronic nausea despite lots of anti-sickness drugs. Right now, the doctors think this is an unfortunate concurrent viral thing. I’m not so sure, it’s so very hard to know in CF what causes what; it could be his current exacerbation, viral infection, drug side effects, or CF tummy related - and his tummy wasn’t right pre-IVs either. 

Today he managed his first whole day at school in a week or so, so hopefully this is coming to an end. 

While all this is going on, my anxieties build as I know the lab have taken the mucous they washed out of his lungs during the bronch (called a lavage) and are waiting to see what bugs grow on their agar plates to see what is going on in his lungs. Early results (some take days, some weeks) reveal more fungal infection. This is mega frustrating news as he only very recently had treatment for this (early in May). To have it back, again, so soon may mean he will need longer term treatment to attempt to kick this out for good. We meet with his team next week, but I’m aware treatments may include 1) long term anti fungals and 2) steroids - both of which have pretty terrible side effects in his case. And this would be alongside his usual treatment for his chronic bacterial infection (PseudomonasA). Right now, I’m trying not to worry (trying, ha! But not always succeeding). 

The positives: We’re on acute treatment already; We know now what we’re battling (which is much better than not knowing, not treating, and him continuing to lose lung function); This is exactly why the bronch was a good idea - making it all worthwhile. 

We don’t know enough yet about what this reoccurrence means and what his options are. So until next week, we carry on with IVs, we do the new treatments, and we very much hope for improvement. Plus, whilst his tummy has been horrible, his chest is sounding a little better these last couple of days. 

Have a great day x x x 

PS; Fundraising update: We are just £345 away from our £5000 target. Thank you so much! If you’d still like to support us, there is a link on the picture of Isaac to the right (unless you’re viewing on a mobile). Some of my hero team are still wearing medical boots while they recover! x 

Robert Mapplethorpe ❤️

We made it (and hospital)

It was not only 67km.... but 69 (some 43 miles) due to some wee detours along the way. Starting at 6am, with torrential rain and injury time we made it to the end at nearly midnight. During this epic walk I know we all hit walls, felt pain, weariness, love and laughter, but to the end, we were together - guiding each other in the darkness. Thank you so much for supporting us, it means so much to all of us (if you have not yet, you can still sponsor us here) We’re almost at our target (£5000). 

To Jo’s team, Michelle, Jo, Jenny, Cassie, Erika and Tracy, incredible job ladies, and you beat us too! I can’t thank you all enough for joining this bonkers mission and raising much needed funds for the CF Trust. 

To my Holywalkamolies team - I hope you know how much I love you all. 

Shell, Matt and Sarah, it has been so incredibly special to spend so many walk and training hours with you - you were all incredible! 

To Bon, Jennifer, Bodhi and Anouk, you made this all happen. I’m not sure I’ve ever been so happy to see someone as when we saw you all at the checkpoints!

Gem and Annie, you lied! You said you couldn’t possibly walk that far. But you did. And your singing and dancing saved me from despair. Thank you for all the laughter. 

To Gavin, I hope your feet are not permanently damaged. You mad old lovely fool! 

Some pictures for you below of some very special people and some pretty awesome memories. 

We are going into hospital on Thursday for a bronchoscopy, followed by two weeks of IVs, and then possibly a CT scan too. Isaac is home sick right now and feeling pretty rubbish, so we’re hoping he will be well enough for theatre. Sunny side up? It’s good to have a plan. More soon x 

If the sun refused to shine 
I would still be loving you 
When mountains crumble to the sea
There will still be you and me 
Kind woman, I give you my all 
Kind woman, nothing more
Little drops of rain whisper of the pain 
Tears of loves lost in the days gone by 
My love is strong, with you there is no wrong
Together we shall go until we die 
My, my, my inspiration is what you are to me 
Inspiration look, see
And so today, my world it smiles 
Your hand in mine, we walk the miles 
Thanks to you it will be done 
For you to me are the only one
Happiness, no more be sad 
Happiness, I'm glad
If the sun refused to shine 
I would still be loving you 
Mountains crumble to the sea 

There will still be you and me

Zeppelin.