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: 

Drugs and rivers.

A week into IVs and his lung function had increased a measly 2%. Enough to not be admitted (for now) but much less than we had hoped for one week in. This means an increased course - instead of finishing on day 14, his next test will be on day 17, and if we don’t see a significant increase by then, I imagine we’ll be looking at third week. He is still coughing (a lot at times) so either we’re looking at something new, these drugs are just taking their time, or his inflammatory response is on overdrive. He is mostly in good spirits still, going to school when he can. He sleeps through the morning dose (at sparrows fart) and I get to count his beautiful freckles while I push high dose toxic drugs directly into his bloodstream. He is so handsome. 

Today by the river with my gorgeous girls.

Hello spring, we are very glad to see you x 

In this world nothing can be said to be certain, except death and taxes...

.... and getting a super itchy nose when you’re mixing up IV drugs and need to stay sterile. Fact.

Port accessed. Bloods done. Lung function tested (about the same as last week). Four days in and he is feeling....well, ropey...this is pretty standard, he tends to get more symptomatic before he starts to feel better. He feels generally pants, headachy, has low grade fevers and was violently sick again last night. No idea if these are side effects or his chest infection being disturbed by these killer antibiotics, or just something new. Hoping he will feel better tomorrow and improve enough in the next three days to not have to be admitted for the second week.

It is very, very hard to see him suffering like this - but what makes it bearable is him; his ‘let’s crack on’ attitude, his lack of complaining, his cheeky smile, his ability to vomit and then immediately ask ‘what’s for dinner?’ - I swing from tears, to laughter, to utter sadness that it’s him and not me, to feeling like I might burst with with pride and immense love - a thousand times a day. 

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.

Some say CF is the most curable incurable disease - let’s prove that to be true x

Kandinsky ❤️

Crackles and coughing

We’ve gone from resigned acceptance that he needs to start IVs this week to step up his treatment against Pseudomonas, to a much increased crackle and cough, and a desire to bring it on as quickly as possible. He needed percussion physio last night (on top of the physio he does independently) which he doesn’t ask for often. The Cipro is having no effect, despite his Psuedo seemingly appearing sensitive to it. It’s looking like Tuesday before they can fit us in for his port access and for the first dose (he always has to have the first dose in hospital, in case of a reaction).

When he is like this I simply have to listen, to hold my hands round his ribs to feel the vibrations, the crackles, to look at his eyes, to hear the wheeze... and I just know that his daily nebulised and oral antibiotics, the mucous thinners, the lung shaking physio (his everyday treatments), they’re just not going to cut it this time. He needs these IVs. 

In true Isaac style, he is taking it all in his stride.

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

Man Ray love ❤️

No place like home/Brexshit.

We will be starting IVs again early next week. 

Isaac is feeling no better, no worse, but a review of his lung function, xrays, bloods and symptoms have led his team to this decision, which makes sense to us. His last IVs were actually 12 weeks ago, which is a pretty good run for him of late. We will go in, pick up mountains of drugs, access his port, have the first dose, and (all going well) come home. I will do his IVs here (home, glorious home) and we only need be admitted if he gets much less well/his lung function has not increased after the first week (nothing like a deadline to get him to do his physio super well!). 

For that chance.... to stay home and snuggly.... we are super grateful. 

Have a great day x 

PS: I half wanted to write a ranty brexshit post about how Brexit/no deal may affect our availability to medications/and not attract talent/resource to meet recruitment needs in the NHS/and further negatively impact medical research (the UK is very successful at conducting clinical trials, sponsoring around 1,500 trials right now in collaboration with other EU countries – half of which will happening this year. Or won’t be happening). Sigh. But right now I think we have enough Brexshit. 

MOT

Today was Isaac’s annual review (a kind of CF MOT) and as we find every year, the immediate results are (*sigh*) mixed. Most other results are still pending - meaning days or weeks of wonder, but amongst those immediate results; 

Chest X-ray, not so great. 

Lung function down, again. 

Blood glucose test (for CF related diabetes) was.... normal (a MASSIVE relief after a borderline test last year). 

He started the day in hospital at 8.30am feeling pretty good. Port was accessed early and bloods all went well. But later, the lung function tests themselves stirred up his congested and inflamed lungs, and we still had X-rays (chest and abdomen this time, for his GI issues) and ultrasound after that, before returning to the main clinic, by which times he was tired, coughing and looking pretty peaky and ill. He coughed up some pretty spectacular looking blood streaked sputum for the lab just as we left. The blood is indicative of inflammation, not good, but it is good to have a juicy specimen for the lab - we cannot treat what we cannot see. 

As we wait for the team to review this news, and await the sputum and many blood results, we’re back on Cipro for a few days, with the view of probably starting IVs next week. We’re also working with his dieticians and doctors to find a way of helping with his GI problems (abdo X-ray not good either). 

After a long wait in pharmacy (brightened massively by bumping into a wonderful friend; love you Laura!) we got home just past 6pm. Isaac spent the journey home with his face in a bag, nausea taking over his weariness. Annual review is something we both dread but feel hugely reassured by. He absolutely needs all of these tests, and taking a step back and looking at the changes from one annual review to the next is a useful (but at times painful) exercise. Isaac is never enthusiastic about talking to the CF team psychologist, but we keep trying. We are so grateful for the amazing service the NHS provides us - but still, days like this can be very hard. 

Lucky for me, I have the best company x 

Fourteen and five feet apart.

‘Five Feet Apart’ is a Hollywood film due out this spring starring some young famous hunk (the fact that I still use the word hunk explains perfectly why I have never heard of him). I don’t know much about it yet, I’ve not seen the movie much less read the book, but the trailer looks a lot ‘the fault in our stars’ish, but this time around the subject of two Cystic Fibrosis patients who fall in love, ultimately risking the cross infection of bugs which could prove detrimental to their health - unless they stay five feet apart at all times (as hospital guidelines dictate about limiting cross infection risks in CF). 

A number of CF bloggers/vloggers have responded to this already.... and it’s proving pretty divisive. I can relate to the negative attitudes to the film; seeing CF depicted in medical dramas again and again in the past has always left a bitter taste in my mouth - too inaccurate, too cringy, and you’d need an Oscar worthy performance to get that CF cough right (they never do). Why can’t they do their homework, actually meet a CF patient and ask decent questions? 

On the other hand - this is Hollywood, and with that comes big time awareness. People will google CF. People will learn about CF; that alone can be invaluable to a sufferer. Hell, they may even donate to fund research.... so that has to be good, right? And, I also know Clare Wineland was involved in the film before her death, so maybe this time we can be hopeful for a more accurate depiction of the disease too. 

From our own perspective it worries me in terms of the attention Isaac may receive from his friends at school...

All of them will know he misses a lot of school. 

Some will know that is because he is in hospital a lot. 

A few will know that is because he has CF (his life, his choice). 

But right now, even fewer will really understand what that might mean for him, and a film aimed at his exact demographic might change that. 

More on this later as I’ve been asked (via the CF Trust) by the film makers to provide a response to the film (waiting to see what this entails exactly). 

Today Isaac turned 14. My darling boy. Every one of Isaac’s birthdays have been emotional for me (today was part celebration, part hugging baby pictures and snotily weeping about how time goes too quickly, part producing a soggy banana cake which cemented my reputation as the terrible birthday cake baker that I am). As my first born, perhaps I am not just celebrating him, but also my leap into motherhood fourteen years ago (best decision I ever made).... x 

Little Isaac. 

Give life.

Don’t stop asking.

This blog  rang pretty true to me, even as only a Mum of a kid with a chronic condition. I always, always appreciate when people ask me how Isaac is doing, but the one thing I would add is this; 

Please only ask if you are willing and able to listen if the answer is not good. 

Many times I have been asked, and partly because they asked, and partly because I am a ludicrously honest person (a quality that has got me into some pretty awkward situations in the past!) I have told people how it really is, only for them to awkwardly stumble over their words, clearly keen to get away. Leaving me just feeling awkward and kind of sad. 

Janeil’s experience of trying to balance her public persona as an honest account of the bitterness of life with CF, whilst also wanting to show that life is not all about CF - sadly familiar too. 

People are often surprised to hear that Isaac regularly plays football for a team and goes to CrossFit as much as he can, but our reality is that the very next day he might be in hospital on IVs to help him battle his live-in bug residents. Even in hospital his physios have him in the gym for an hour a day. What he can do each time varies incredibly; a spectrum all the way from walking just a few steps from his wheelchair using portable O2 to supplement his breathing, to press ups and sprints on a treadmill. Exercise is massively important to his lung health, and so we encourage him to do his best, whatever that might be that day. 

CF is an incredibly complex disease, the prognosis might suggest a slow decline of lung health over years, but we also see huge dips and peaks in symptoms along the way too. Add to that, the number of other organs affected by CF (for Isaac, this is mostly his GI problems right now) and side effects from his many medications, sometimes when I’m asked how he is, the answer might also be;

‘umm, kind of OK, not sure, bit mixed, he threw up coughing this morning, and then bench pressed his best weight, might be on the brink of another admission or a fantastic apperance at football’ - we can’t always tell. 

So if you ask, be happy with us when it’s good news, and listen when it’s not. But thank you for caring enough to ask, all the love and support we receive never ceases to amaze us. 

This week has been a funny one. Dan had a football injury a couple of weeks ago, and his leg later became inflamed. The GP diagnosed an infection in his vein, and prescribed an antibiotic. And then another one as it was getting no better. And then a third. After that didn’t work, he was advised to go to A&E - where today they told him it’s not an infection at all, but thrombosis - He has a bloody blood clot from his mid calf almost to his groin! It’s massively inflamed and painful, but a painful ultrasound revealed it’s a superficial vein, not deep vein thrombosis which is more dangerous. He is now home, and hopeful that a 45 day course of blood thinners will sort him out. Got to love him, but he is a plonker, he’s limping around but wants to work tomorrow! 

Isaac, on the other hand, is having a good week. His GI problems persist, but it’s manageable. Next week he is having some hi-tech tests in hospital to assess how well his body tolerates exercise. More on that soon. 

Was so nice to see some sun this weekend. Rosa and I took a walk in the woods - how gorgeous is this girl? Roma time (the name we call ourselves together, Ro and Ma 😊). 

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

Have a great day x 

Lurgy lurking and hope.

We still have lurgy lurking in our house (Dan is ill now too), but Isaac is back at school, and we are tentatively hoping he may have successfully escaped my flu (I have decided that believing in jinxing our luck is a pile of old pants and to go ahead and outwardly hope). 

Stay well my friends this bleak winter... spring is on its way x x x 

"Which of us is not forever a stranger and alone?"

Wings of Desire (1987) dir. Wim Wenders

Live life, give life

In the UK more than 80% of adults support the idea of organ donation, but only 24% have signed up. UK friends, can sign up here.

Please also share this video, and your own thoughts on organ donation. It may one day save Isaac’s life, and many others like him suffering chronic disease. Thank you. 

Just a chest infection please.

Isaac first cultured Pseudomonas aeruginosa when he was around 18 months old. Since then, despite intensive treatment (colomycin and tobramycin nebulisers daily, dozens of courses of Cipro, and many courses of IVs) he has never been more than a few months between positive cultures - thus he is considered colonised with this bug (one well known to enjoy the extra icky mucous of a CF lung). This means that while he has the odd negative culture (negative cultures sound good, but are actually pretty meaningless) we can assume the infection is always there, lurking in the depths of his lungs; an uninvited guest that nobody likes. 

Symptomatically, living with a constant chest infection can vary very much in CF - unlike healthy folk with a chest infection, he has few fevers, and if you are a regular reader here you will know he plays football, goes to CrossFit, has friends, attends school on a semi regular basis, and has a social life. But when this bug decides to have a little party in his lungs (CF exacerbation) he also spends a lot of time at home or in hospital having IV antibiotic treatment to try and keep this bug at bay. It doesn’t always go to plan. Pseudomonas is like a rude, unruly child - an invador, trespassing on his health. I hate it. 

That said, right now, having had flu in the house last week - we’d take an Isaac typical pseudomonas party over that any day - *flu would floor him more than it did me, and likely give his bacterial infections free rein to party all over his precious lungs like never before. 

Isaac has had a funny few days, up and down, certainly coughing more, on/off fever. Today he has been sick a few times. I’m terrified he might have flu - but nothing is straightforward in CF. Unpicking what is new (flu?) and what is his norm is not easy. 

What is normal is his crazy amazing attitude to just getting on with things - we could all learn from him. More soon x 

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

* we all have the flu vaccine every year - it only protects you from the predicted top strains that year. Looks like we got unlucky. 

Flu.

It started with a pounding head. 

Fevers. Shivering. 

My limbs ache, my bones hurt. 

Are these even my legs? 

Feeling so cold in three jumpers, two quilts, lying in front of the fire. 

Incredible thirst. Sip water, feel sick. 

Can’t stand bright lights. 

Vision blurred. Can’t watch TV, can’t read. 

The whole world has dark edges. 

Head pounding. 

I sleep and sleep and sleep. 

My head tells me to eat, but food has no taste. 

I eat, I feel sick. 

I cough so much it hurts. 

I cough and then I’m sick. 

Fevers, fevers, fevers. 

I need to be horizontal, always. To live in a dark world under covers. 

Day five and I manage to take the girls the five minute walk to school, but it takes an hour lying down to recover. 

Day six and I’m still sleeping all day. 

My head pounds, but all I can think is ‘what if Isaac catches this?’ on a loop, before I fall into a restless, cold heap of sleep, waking only to cough up more crap.

I will be fine.

Today Isaac came home with a fever. 

I called the hospital, they won’t consider anti-virals as he has had the flu jab (so have I) and until he is more symptomatic. 

Please please please don’t let him have this x 

Rene Magritte. 

Orkambi. Still waiting.

Orkambi is a drug which should be available right now for the many throusands of people with Cystic Fibrosis for whom it will work. 

“Children & young adults with CF endure lifelong suffering & early death. They need Orkambi and other precision medicines as they are developed. Sufferers in the EU, US & Australia can access the drugs, but not the UK. Hundreds have died in the 3yrs since these drugs were licensed”.

To urge Vertex, the pharmaceutical who developed Orkambi, to negotiate further with NHS England, please click here, it will take just a minute. 

To sign the petition urging NHS England to never give up, please click here. 

CF is unfair enough already without having to fight for drugs which might save your lungs from further decline. 

Miro... with love

I

Can we get off, please?

Seems like we’re on an endless ride that we never queued up for in the first place.

IV antibiotics for two to three weeks at a time, at home if we’re lucky, on the ward if we’re not. Or a mix of the two....

On IVs he feels worse to begin with, but his lung function steadily increases (massive relief)....

Following IV’s he enjoys a few weeks of good health. Maybe six weeks if we’re lucky? Just two or three if we’re not...

But sooner or later the symptoms increase and his lung function falls (massive disappointment)...

We might try oral antibiotics (these used to work more often, why not now?) but more often than not, we’re back on IVs and the ride continues.....

During an exacerbation (CF talk for when his live-in bug, Pseudomonas, has a little flare up and makes him much more symptomatic than he usually is) of course he coughs alot, but how he feels can vary from being able to go to school, CrossFit, football.... to lying on the sofa all day, pale and exhausted,  or in hospital, needing supplemental oxygen. 

Right now, he is exacerbating. He missed football today, but he is well enough to stay home, try two lots of extra antibiotics and wait and see where this one takes us. Yesterday his doctor was saying that even the density of bacteria growth that the lab find in his sputum is not indicative of how poorly an exacerbation might make him - people with CF can have a light growth and be very poorly one week, or a heavy growth the next, but be able to stay home and cling on to a normalish life. I’m endlessly looking for rhyme and reason where there might not be any. 

In the meantime, increased physio, and they are looking at his bloods for aspergillosis levels (a fungus that he has previously grown in his lungs too) and to see how well his kidneys and liver are coping with not only all this treatment, but the amount of painkillers he also takes (he takes some sort of pain relief most days, mainly due to his GI problems). He was given a small lecture about adherence to his Creon (the pancreatic enzymes he needs to take with food, which we think he ‘accidentally’ forgets at school), but he took it well. It’s not awful to miss it, the main consequence being pain and malabsorption, but it doesn’t help his overall well-being. 

For a number of reasons (other family stuff included) this week has been a very hard one. But I have also been reminded of all the support we have around us, and we’re so thankful for our family and friends that wrap us up with love. Thank you x 

Lucio Fontana - Concetto spaziale, attese 1967.

All that glitters is not gold.

I said I would write about this last year. For Isaac and for us, it was one of recovery and adjustment. For me, one of fear, anxiety, sadness, hope, laughter and love. 

But that’s just life, right?

Isaac’s time in intensive care gave us a glimpse of how bad things can be; dependency on oxygen, wheelchairs, a complete and utter reliance on the healthcare system that wrapped itself around us. A profound insight in actual breathlessness, the impossibilities of walking,  getting to a bathroom, to wash, to eat, to do anything normal that a lack of O2 entails.  

Overcoming that exacerbation, getting home, getting back into life after a month in hospital... amidst all of that I never could have told you how stressed out I was. It’s only now, looking back, that I can see that I shook for months without really realising why (do stress hormones linger in your body that long?). Literally shook. And that was just me - what must Isaac be thinking? 

Then adjusting to a new norm. Instead of hospital and IVs once or twice, max three times a year, now to six courses a year - two to three weeks at a time. That is a whole lot of life to live with a needle in your chest, maybe days per year, hooked up to a drip/pump to administer drugs directly into his blood stream, fighting a battle with a bacteria that he won’t really win (he is considered colonised with PseudoA, so the fight now is to keep it in check, rather than eradicate). 

Not to mention two lots of surgery, one very painful and the other for a permenant IV device in his chest. This portacath was an addition that at first he was adamant he did not want. But he came around to slowly as his veins became shot and it became harder and harder each time to get a line in. A permenant reminder of disease. A marker that he never wanted, but that now we are so thankful for (makes intravenous access and a speedy IV kick off so much easier). 

When Isaac is sick, there are clear obstacles to his everyday life. Six nebulisers a day takes up a lot of time. Add IVs to that, and he’s house bound for 2-3 hours more at least, he can’t swim or shower, and to bath he needs to be wrapped in cling-film. His exercise options are limited, sleep is difficult, not to mention that he can feel terrible. But I was thinking today about all the smaller, everyday things that being chronically ill with CF might mean; 

When you cough that much, would you enter a small shop, a lift, a cinema for fear of those LOOKS? The jokes about giving up the twenty a day habit (so not funny)? People running for cover thinking you’re the contagious one? The fear of the dreaded cough-cough-vomit? Would you want to go out for a night in winter, when the cold air/hot air transitions make you cough more? Would you want to cook/eat out, when cough/treatment induced nausea might mean you can’t eat anyway? Would you want to join a gym, when exercise induces just more coughing? And you’re surrounded by peers that don’t have the invisible disadvantage of reduced lung function meaning you can’t ever keep up? Do you carry on accepting invites from friends and family, if you so often have to cancel last minute if you’re unwell? (Or worse, do they stop inviting you?) Would you apply for a job when you knew that it would mean not having the time/energy for all your treatments? Would you wish for that promotion if you knew you might be absent due to hospital stays more and more often in future? What about relationships, when your condition affects your fertility, your ability to travel, your being able to PLAN ANYTHING are all affected? 

The list goes on, and OK, so I may have drifted off the ‘everyday’ theme there, but my point (that I meanderingly get to) is that on the most part, CF is an invisible disease, but it affects sufferers in a thousand different ways, a thousand times a day. I hope by raising awareness here I can increase mindful consideration of life with this and other chronic disease. 

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

All that glitters; listen here x 

“

Gorgeousness from Hilma af Klint. 

Smuglessness and a new year

Happy new year lovely people! I may have spoken a teeny bit too soon in my last post, smugly saying how well we all were. I was feeling a lot less smug on Christmas Day eve, when poor Anouk had a thumping headache and a fever of 39.8, and positively smugless over the next week, when we all passed round such a stinking head cold/tickly cough thing that we couldn’t sleep for spluttering and no one knew where one bug ended and another started. 

Thankfully Isaac stayed well, although his junkiness is definitely picking up again day by day (junkiness/fruitiness/rattleiness are all genuine medical terms in our eyes). After IVs so recently, and the fact that there are so many viruses around right now, it’s too soon to call clinic. Instead he was back at CrossFit today trying to physio the junkiness out of his lungs with sweaty panache. 

2018 felt like a year of recovery and adjustment for us, following black October of 2017, and facing more regular courses of treatment than ever before (I’ll write more about this in my next post) - but looking at where we are now, since this time last year? It ain’t all bad - just look at these three x