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. 

Antibiotics, bronchoscopies and CT scans.

Thursday we popped into CF clinic for an emergency review, as his cough is building. We saw the registrar as many of the team are away at the European CF Conference, but he was great, really took the time to listen and understand where we are right now. The relationship we have built with our MDT (multi disciplinary team), which is made up of CF/Respiratory doctors, CF specialist nurses, physios, dieticians, pharmacists and psychologists, is an essential part of Isaac’s healthcare, and it’s both important and valuable for Isaac and I to feel part of the decision making, not simply passive participants in his care. Anyway, his lung function was (as predicted) down, and he had more bloods to check his infection markers (TBC). 

We discussed that for six months now his LF has been creeping down steadily (as lung function is considered a vital measure of wellness in CF, this is major concern). The doctor suggested that as Isaac had been so poorly in Oct 17 (read more here) this may have contributed to his overall decline, but he also agreed with me that we don’t want to simply accept that this is his new baseline, without really understanding why this might be, and nor will we stop trying to regain lost LF. 

It’s also clear that he is exacerbating right now (despite recent IVs, anti-fungals, and steroids) and we need to deal with this now. Therefore, tentatively the plan is a week on Cipro (extra strong oral antibiotics, started last night), with a review in one week. If his LF has not improved by then, IV antibiotics will be kicked off in hospital, with the idea of doing a bronchoscopy (a looksee in the lungs with a camera, plus a flush out of his mucous to test in the lab) at the beginning of the course, and potentially another CT scan following the course (to have a more detailed look at his entire lungs; beyond the point they can bronch, essentially). It’s a tentative plan for now, his MDT will discuss at their meeting next week. It’s incredibly important to know we’re not missing anything, and bloods, xrays, medical reviews and culturing sputum samples can only give us a partial picture; a bronch and CT will ensure nothing is escaping us that might explain why he’s declining. 

Not great news by any means, but a reassuring plan of action, and in true Isaac style, he took it all in his stride. There is good news scattered in each clinic visit too... his weight and growth are great (something many people with CF struggle with), his exercise tolerance is amazing (he does CrossFit and is in a football team) and his gastro problems are much, much less frequent and severe than they were a few years ago (a cycle of DIOS and problems with nausea that for a while I thought we would never get out of). Lots to be thankful for, heaps to hope for. 

Have a great day - and please think of us, walking for some 13,14 hours tomorrow!

Picasso ❤️

Blisters, love, and gratitude.

Holiday was wonderful. The kids had a ball, swimming dawn until dusk. I’m not sure if I explained about the holiday? If you’ve been following this blog for a while you may remember that Isaac had a wish granted 5 years ago by the wonderful charity Starlight. His wish was to go to the biggest water park in the world (he is part fish) and amazingly they really, truly did this! We spent 5 breathtaking days in Dubai at Atlantis (he is still featured on the Starlight website here if you want to read about his dream holiday). At that time, our little Rosa was just one, so we made the difficult decision to leave her at home with the grandparents as it would be too hot, and having her there would mean we could do less with Isaac and Anouk. So ever since then, we have promised Rosa that we would go again, and revisit everything we did then - as a family of five. A good deal gave us our chance - only four days this time, but every second counted, and our fears that Isaac would not be well enough to go were proved gladly wrong. 

Following the Voriconazole (to treat his evil lung munching fungus) Isaac was really well for a couple of weeks. Side effects made his poor lips blister so much they bled at nights (he looked kinda goth in the morning, lips black with dried blood) - but his chest cleared. 

Now? Coughing again. A low, deep, rumbling cough. Clinic have squeezed us in this week on an emergency list. It’s a worry+ situation; an increase in symptoms is always a worry, but gets a plus whenever ‘other life’ factors (Christmas, birthdays, holidays, epic fundraising walks....) are imminent, as I’m away this weekend for our walk. In fact, the walk is a worry generally as I’ve hurt my hip (for a couple of years now I’ve been able to click my hip in and out of the joint - true and, yes; yuck) and the last training broke me slightly... but I’m still very much planning to make it round (even if my wonderful friends joining me have to wheelbarrow me!). 

Thank you SO MUCH for all the support so far - it really means the world to me, our family, and our whole Holywalkamolies team. You can still sponsor us here - I hate to ask (at least, as often as I do), but CF research absolutely needs these funds, and Isaac is sadly not one of the 90% of people with CF who will benefit from drugs which are soon to be licensed which treat the underlying cause of his disease. He unfortunately has a rare, severe mutation. This means a longer wait - but I’m confident a cure will come, in time. Thank you for helping in any way you can x 

Huge thanks to my friends and family Jo Cayley, Gavin Dytham, Michele Dytham-Ward, Jennifer Dytham, Sarah Cater, Matt East, Gem Wilkins, Annie Marriot and Bonny Fountain. Michelle kavanagh, Jo Elliot, Jenny Hays, Cassie Flack, Erika Pyne, and Tracy Coulton-weir for joining me on this epic 67km trek.

And then smiles cover your heart, again.

Isaac is doing really well. 

His cough is better. 

He hasn’t been sick in a couple of weeks. 

Thank you, our toxic friend, Voriconazole. 

Descripti

DescriptiVoriconazole

We love you. 

Hope you’re having a great day x 

Lee Miller love x 

Chase away those restless fears.

I said I would write about Isaacs prognosis and our treatment options going forward, so this is my wine stained attempt at just that. Now, I can set out some likely scenarios, but really, truly? We just don’t know; 

There are many stories of people living well with CF for decades, but more about those who don’t, or do but whose quality of life is greatly impaired.  There are examples of siblings with CF (who have the exact same genetic mutation) where one is super well, while the other is not. I remember reading about a family in the US who had seven children, when one of their children fell ill, presenting with suspicious symptoms, all siblings were tested and of the seven, five were diagnosed with CF. Of those five, two died very young, two in early adulthood, while another was still living well in his fifties. This perfectly illustrates how the disease affects sufferers in incredibly varied ways, and how his genetic mutation alone is not indicative of his prognosis. Part of this might be due to other genes we all have and how they interact with the CF gene; some might be exposure to infections; and some might be lifestyle and adherence or access to treatments. 

The one thing we can say for sure is that Isaac’s live-in infections continue to attack his beautiful, once pink lungs, and because of this, we will see decline in his lung function over time. 

90% of CF sufferers ultimately die from lung disease. 

So here are some thoughts (in no particular order);

1) Ever the optimist; I’ll start with my favourite. A cure is found! This might be gene therapy, or a small molecule drug that will work for his Class 1 mutations. These drugs would halt the progression of his disease. It won’t win him back lung function that he has already lost, but if he had access to these now, he could lead a long and healthy life with only mild CF complications (...pancreas, liver, bone and digestive problems). While there are promising small molecule drugs newly licensed that will treat the underlying cause of CF for many people with CF (CF affects how salt and water move through our cells, and these drugs correct a fault in this) these will not work for Isaac who has two rare genes for which nothing is yet through successful trials. I have written before (here) about this, but since then, there is new hope in various trials. I spend an enormous amount of time reading about research and trials, but don’t often write about it here simply because progress is slow (think ten years from concept to market) and there are so many hurdles at which promising drugs can fall; I have learnt over the years to not get too excited. 

It’s not going to happen this year, or the next. But one day - I am certain of this. 

2) His current treatment options (in particular the oral, nebulised and IV antibiotics he takes, plus the mucous thinning drugs and intensive physio to help him clear the mucous) continue to work for him and despite the progressive nature of the disease, we keep it at bay as much as we can. Our consultant once described his regime as a jigsaw puzzle - no single aspect of this will be a cure, rather these many jigsaw pieces will come together to help him as a whole. As we are seeing now, this would likely mean a slow decline in lung health over years. 

3) New drugs are developed to add to our arsenal - and by this I mean symptom reducing treatments rather than those I include in part 1 which would treat the underlying cause of the disease. Most critically, this could be new antibiotics as his chronic infections can and will become resistant to current options at some point. 

4) Other CF related problems raise their ugly head; CF related diabetes, liver disease, osteoporosis, gastrointestinal problems, even some cancers - and alter the course of his disease. And more oftenly ignored and maybe even more significant; mental ill health. CF is no easy ride. 

5) His disease progresses so much that lung transplantation becomes an option - maybe our only option. This would only be considered when his lung function is about a third of what it should be on a regular basis. He has been at this level before, but only during an acute exacerbation - thankfully post exacerbation it has always recovered enough and it’s not on our radar right now. 

5a) He is accessed for and is turned down for transplantation due to complications (this happens for a number of reasons, certain infections are one). Or he makes the choice himself that transplantation is not an option he wants to pursue (it happens; it’s a huge and very risky operation). 

Go back to 1, 2 and 3 before you pass go. Do not collect £200. 

5b) He is assessed for, and listed for a lung transplant. But due to the shortage of organ donors, he is one of the 1 in 3 people who die waiting. Please sign up here  if you haven’t already - and remember to tell your next of kin as regardless of your choice in life, it becomes their choice after it. 

5c) He is assessed for, and listed for a lung transplant. A selfless person dies, donates their precious lungs, and he is transplanted. He will no longer have CF in his lungs, but he will still have CF in his sinuses, liver, pancreas and other organs. He swaps one set of treatments for another, and begins a life of anti-rejection drugs and the side effects of having these is a comprimised immune system. A successful lung transplant will be absolutely life changing; it could mean many years of breathing freely. But it comes with considerable risks too, and average life expectancy after transplant is somewhere in the region of five to ten years, so something we hope to put off for many years to come. 

6) My most feared; a sudden decline from a serious exacerbation (see blogs from Oct17) which might result in emergency listing for transplantation, or worse. Not one I let myself think about, or really believe will happen again. 

7) All of the above, and maybe more. 

8) This is all a dream! CF is all make believe. Like in Dallas, when Bobby dreamt that Pam had died and married Jenna? I like this option too. 

Clinic today was mixed, as always. His lung function remains low - just less than his last test at the end of his recent IVs, but higher than I expected, as he’s been seriously coughing today. His infection markers are high for a fungus he has previously grown in his lungs (Aspergillus) - and while the markers are much lower than they have been previously, they are rising significantly. Not good. But given that his last IVs did not increase his lung function as expected, and having had recent steroids which would normally give him an uplift too but haven’t, it’s actually reassuring to have another treatment option to try right now. Maybe these anti-fungals will be the thing to pull him out of this blip. So despite this, and him being markedly more symptomatic (so much so, the coughing around the supermarket on our way home got us some pretty serious stares) we’re actually feeling hopeful. Maybe we’re also relieved not to be facing IVs again so soon, but whatever, we’ll take it.

Thank you for reading. I’m forever unsure how this blog really resonates with everyone - I very much want it to be real, honest, informative and not at all depressing and sympathy-seeking sounding. I don’t plan blogs, I write very much from the heart and I hope it comes across in the way that I imagine when I write. Please, please remember, that our lives are full to the brim with OTHER LIFE STUFF; dancing round the kitchen table with the music much too loud, love and huggles, roll-around tickle fights and fulfilling work lives... this blog focuses in on CF, on Isaac, and it is undoubtedly a struggle at times, but we sing, we laugh, we prat around as much as anyone. Sending big love to all, especially to those carers and families juggling-with and affected-by chronic illness of any kind x 

I would love to tell you how much better he is....

But his lung grumbling cough continues. Sorry, but fuckedy fuck fuck shit. 

Still, we had a laughter filled weekend with some much loved friends at the coast (thank you guys). Lots of food, soaked in gin, warm sea air, exercise, blood moons and early sunshine (latter being much needed since his latest bloods show he is deficient in Vit D again; Isaac cannot absorb vitamins A, D, E and K through his diet as we can (the fat soluble ones) and despite some hefty supplementation already, this will now be increased). 

Spending the weekend in closer contact with him (no PlayStation, ha!) I’m increasingly aware that he is suppressing his cough in front of others. I get this. Who wants to cough all over people? Who wants the questions? The jokes about giving up smoking? But suppressing his cough is not good at all; he needs to get it off his chest (literally) to stop the mucous build up. We’ve bought forward his next clinic review to see if he needs further treatment, despite being only a few weeks clear of his last IVs, and recent steroids. It’s disheartening to take on these treatments - not so much his usual day in-day out ones (which already take up 1-2 hours of his day and he takes as a given) but the extra burden of IV antibiotic courses every couple of months and the yucky steroid side effects, but then still not see any improvement. 

I’ve just been looking around for medical insurance for a holiday we just booked, which is about as fun as poking chilli dipped cocktail sticks in your eye. Even looking at a company who have worked with the CF Trust to better understand the condition (and not panic by the vast amount of medications taken) it’s so expensive, and I’m not sure it will 100% cover us - In the hundreds of questions it throws at me, if I say yes to every one the policy will be astronomical. But I’m not sure if saying no is even true at times (example; Does he have an enlarged spleen? - um, I think they mentioned this on an ultrasound once, but it’s not a significant enlargement, so can I say no? Does he take drugs for asthma? Yes, but he doesn’t actually have asthma, we don’t think, just the symptoms can overlap at times, so can I say no? Have we visited A&E in the last year, well, technically yes, but only because our doctors were not available and we need to be admitted to the ward by being seen - in fact, we were wheeled up to the ward after just a phone call up to the ward anyway, no doctor review, so it’s just a technicality, right?). I just don’t know - any wrong answer and if something did happen it could invalidate the policy. Anyway, I daren’t apply yet as he may need IVs again before we go, which would change things - so a worry for another night. 

We smile on - the main thing is we have a holiday to look forward to soon, and the kids are going to LOVE IT (more on that later). We will make it work somehow. 

Today I had to play catch up on training - our walk is just a few weeks away now, and I need to up my game. I’m walking one long walk between 15 and 25km once a week now - but this is a far cry from the 67 I need to complete in a single day! 

Please, if you can consider sponsoring us for this epic walk, that our Holywalkamolies team (Jo Cayley, Jennifer Dytham, Michele Dytham-Ward, Sarah Cater, Gem Wilkins, Annie Marriot, Michelle Kavanagh, Jo Elliot, Jenny Hays, Cassie Flack, Erika Pyne, Tracy Coulton-Weir, Bonny Fountain and I) will be completing in June, please follow this link to donate to the Cystic Fibrosis Trust. They fund groundbreaking research into CF - known by some as the most curable, incurable disease (next post I plan to write about all of our treatment options for Isaac, assuming he continues to decline, so watch this space). Thank you so much for the donations so far. I can’t tell you how much it means to us all x 

More Gerhard Richter ❤️

Flat Stanley

Tonight we are feeling a bit FLAT (Stanley, a very favourite book of ours), or, in other noises, just a bit BLEUGH.

Post IV clinic today and his lung function had increased..... not at all. 

Why is this equation not working for us? The infection we know (his unwelcome guest of 12 or more years now, PseudomonasA). The drugs that these bacteria are sensitive to, we also know. He’s had a two week starter of Cipro, followed by a two+ week main course of IV Tobramycin and Ceftaz - he is treatment fatigued. Infection + treatment = improved lung function, right? But it isn’t, it hasn’t, it sucks, and therefore he started today a hefty dose of Prednisone for dessert. 

Long time readers of this blog may remember that Pred (steroids) are our least favourite treatment option due to the side effects which are plentiful on as high a dose as he needs to take - we have been here many times before so know how it affects Isaac (reading the pamphlet; moon face, aggression and suicidal thoughts anyone? Super!), but the thinking is that the cycle of infection and subsequent inflammation means his inflammatory response has gone into overdrive (the blood in his sputum is indicative of this). So Pred it is. Hoping a short sharp course is all he will need (any longer and he will have been on a high dose long enough that he would then need to taper down the dose over many weeks, which is where the side effects really kick in). 

We arrived at clinic today en masse (minus Dan who was working) as it’s school holidays. After lung function tests he was coughing up a whole lot more, but despite this we had a yummy lunch out and then went to a climbing wall. Making the most of it.

To sponsor us for our latest fundraiser, in which I will be walking 67km in a single day (by current training standard, will likely take me 14ish hours) alongside some amazing friends (shout out to my team Holywalkamolies!) raising funds for the CF Trust who fund groundbreaking research into CF treatments, please follow this link. Thank you so much for everyone’s support so far, donations large or small, every penny counts and means so much to us all. Have a great day x 

A very rare picture of the lesser spotted teen. 

 And with the gappy toothed Rosa Banosa. 

Large, small and medium today: