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 

It’s Christmas!

We are home. We are well - the best gift I could hope for. We are very merry in the Moly household. 

Thank you for reading, and wishing you all a happy and healthy break if you are having one x 

Have some pictures of Isaac and his lovely cousin Ben. How they change! A constant reminder of the seasons of our lives. We are big fans of cousins in this house, and after years of only a few, the kids now have a new influx of babies to love on, and this Christmas will be all the more special because of them. Merry Christmas babies! 

Happy tweens, grumpy teens! 

Bonds between people who have not, and cannot ever meet.

Isaac is doing really well. One week into IVs and his lung function which was down 20% on his baseline (already not great) had increased 13%, which for one week on is fabulous. So for now, we continue home IVs, and all going well (symptomatically) they will end next week - perfect timing for Christmas. 

Good news for us, but sad news from the wider CF community this week. This is a unique community in that we cannot meet in person without high risk of cross infection (...pretty sure this is unique, as I’ve not heard of it for any other chronic illness? But I could be wrong, t’is not rare!). The bugs which inhabit CF lungs so merrily come in many strains, but from just a few species. They realised back in the eighties that certain strains were prevalent in certain CF centres leading in one case (the Liverpool strain of Pseudomonas) to many deaths. And so came about the end of CF holiday camps, sitting in waiting rooms with other patients, and the start of segregated clinics and beds on the ward so far apart you would never see the other patient. The closest we come to another CF patient in person these days is a knowing smile and a wave across the hospital concourse. As a result, the internet provides those affected by CF our only means in which share knowledge and learn from others who really understand life with CF and what that entails. In those early years, getting over diagnosis, the CF parents forum was my lifeline (shout out to Softie, a kind a generous soul who had CF himself, but spent much of his life advising us parents of newly diagnosed kids from the risks of sandpits, to how to sterilise neb parts, to how to cope with questions from others such as the common ‘that’s the one where you bash them, right?’). 

And as in any community, there are some faces that stand out, the social media ⭐️’s of CF so to speak, and this week we face yet another death of a young activist, advocate, and generally lovely person. In my time as part of this community, I have known far too many die (far too soon) and while CF affects sufferers in very different ways, these losses have broadly fitted into three kinds; 

1) Those who decline in health, usually in their teens or twenties, and are listed for a lung transplant. 

Of these, there are the in the 1 in 3 patients who tragically die waiting for lungs due to a shortage of willing donors (please sign up here ). 

Or those who do get a chance at a new life with new lungs, but die following complications post transplant (as with my friend Jayne. Who I miss so much). 

Or those who initially do well with new lungs, but who later suffer from rejection (as was the case for beautiful Eva, who inspired so many). 

2) Then there are those who decline in health, usually in their teens or twenties, for whom transplant may later become an option, but who tragically die from an infection very suddenly. I am not sure of the details, but in Lea’s last vlog she casually mentions she is starting a new IV drug for her current infection, pretty normal CF stuff, and yet a week or so later, she has passed. 

3) Those who decline in health more gently, and may survive their twenties, thirties, even forties before being listed for transplant (if they even opt for transplant). Or have a transplant earlier, and avoid rejection for many good years. 

Now, it makes perfect sense that when you are at your sickest, you’re much more likely to be home, alone more, and online. Whereas when you are well, you’re more likely to be out and enjoying life too much to be online, and so the internet can skew perceptions. I get this. But one thing is for sure, I know of many, many more in the first two categories than I do the last; and this can be pretty terrifying (‘sudden decline’ especially after our stint on intensive care last year. Too close. Too real). 

While the internet gives us an amazing forum in which to talk about our experiences, and share fears and triumphs with the only people who really understand, it also creates bonds between people who have not, and cannot ever meet. I would not be without my CF community, but with this comes pain and incredible heartache too, despite us never having met.

I shall miss your smile Lea. Sleep well 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 😬 x 

Lakes, walks, coughs.

Today I walked round the lakes with Obie, headphones on, music loud. Cleared my head some, as I was feeling pretty miserable when I woke. Although I’ve gradually moved his IV times so they are now a reasonable 7am and 7pm, I keep waking up at 5am, irrationally fretting until 7am that I might sleep through my alarm (which I never do).  

Isaac is a smidgen better. Still coughing but he hasn’t been sick since Friday (he coughs and coughs and coughs so hard he is sick when he is this ill). Should be a good sign that he is turning a corner, but not as well yet as I hoped he would be by now on IVs. He has repeat lung function tests Wednesday. We need him better by then, otherwise we could be looking at an admission. 

The deal I have with Isaac about this blog is that he doesn’t care what I write (he agrees awareness is a good thing) but he doesn’t like pictures of him looking sick (fair enough), but if you would like a better idea of what a CF cough can be like, you can check out this couple who vlog daily about life with CF. Gives you a fair idea of what Isaac’s mornings look like right now.

My walk today:

The far reaching affects of Cystic Fibrosis; 



Organised me a new IVs cupboard (this makes me very happy): 

Hoping to write a jolly Christmas blog soon x 

No better.

At least not yet. He is still coughing so much he is sick in the mornings. Hoping this is just the bacteria-busting super powers of ceftazidime and tobramycin doing their shit before a much needed ceasefire in his lungs. Pseudomonas, we know you’ve set up a permenant camp in there, but hell, you need to know your place! 

Perspective

Children with cerebral palsy lick milk powder off a bed to feed. Image credits: Lu Guang

Qi Guihua, held here by her husband, fell ill when she returned to the village from Beijing to celebrate the Spring Festival. She died two hours after this photograph was taken. Image credits: Lu Guang. 

Drugs and beds.

Unsurprisingly Isaac started IV antibiotics today. He is no better, and his lung function had dropped a further 20% in two weeks. 

In true Isaac style, despite coughing and spluttering throughout clinic, and giving them a deliciously juicy sputum sample for the lab, when they asked him how he felt, he just replied ‘Alright I suppose. Yeah, fine’. He usually has cold spray (numbs the skin) to access his port, but for the needle today he chose to have nothing, and hardly flinched. If he could have carried on watching you tube he would have. He amazes me daily. 

But the wards are full. There is no room at the inn.... HA! This shouldn’t please us, but it does. We can stay home and I’ll do his IVs here, and this makes us very very happy. They would rather have us in, for the physios to put him through his paces, but we can and will do just as much here (and avoid picking up more bugs on the ward). Administering his drugs myself is tiring (I’ll be up at 5am, to glove up and mix drugs) but for Ise, it means he can sleep through the doses while I creep around his bedroom in my plastic apron. I cannot fully explain to you how much less exhausting, frustrating and life intrusive this is for us than being on the ward. 

Best case, we do two weeks of IVs at home. His lung function improves, and most importantly, he starts to feel better. Then ho ho ho we can hope for a wonderful and well Christmas. However, if he doesn’t improve pretty quickly, feels any worse, or his labs show he is growing something other than his norm (PseudoA) he is guaranteed an emergency bed as that would mean drugs or treatment that we cannot give him at home. He really really doesn’t want to go in, especially so close to Christmas. Please let this work x 

Still home. Still sick.

Apologies to anyone who read the last post and worried. We are still home. Saturday was awful (I can’t quite explain how utterly heartbreaking it is to watch your child cough (and be sick) again and again and again, day and night. To have nothing to soothe their rib pain, the head pounding, the sore throat...) but Isaac stood his ground in light of his many many previous experiences in A&E, and insisted we wait before going in. Credit to him, he knows his mind and body, and Sunday he was coughing a little less, and so by then it seemed best to wait for Monday to be reviewed by his own doctors. 

But come this morning (Monday) we were told the soonest he could be seen would be tomorrow anyway. The inn is full. The wards are packed, and after talking to his new CF nurse it sounds like our only way to be seen by a respiratory doctor in future is on clinic days (Tuesdays and Thursdays) or through A&E. This is new and miserable news for us; we used to be able to call any time Mon-Fri and one of his team would always be able to fit in a review if he was unwell. So short of fitting his disease around clinic days, we need to sit on symptoms or brave the contagious in A&E from now on. 

I’m sorry, I’m tired, I’m angry; I’m not blaming anyone, but I’m sad (and mad) at the state of our beloved NHS. 

Clinic first thing tomorrow. 

The drugs don’t work/It’s beginning to look a lot like....

..... Hospital. 

We’ve come to the end of two weeks of Cipro, and he is no better. He came home sick from school yesterday, O2 saturations around 94 (anything below 93/94 and he really should be on some oxygen). He coughed and slept his way through the night, and this morning I was ready to pack our bags to go. He coughed so much he filled three cereal bowls with sputum and coughing so hard he threw up. 

Since then his sats have drifted between 90 and 98. As it’s the weekend, our only option is to go through A&E. Although he has this ‘open access’ to hospital, it’s kind of meaningless as he still needs to be reviewed by a doctor to be admitted. 

What haunts me is that we had a similar weekend last October when he became much more symptomatic on a Friday, we thought about A&E on the Saturday, he seemed slightly better Sunday, so we eek’d it out until Monday morning to see his own Dr and go straight from clinic to the ward. Whether things might have been different had we taken him in sooner, I don’t know, but by the Thursday he was in intensive care. 

So why are we sat here at home still? 

Isaac is adamant that he doesn’t want to go to A&E, and he increasingly wants to have a say in his care. I half convinced him earlier, and then his sats said 98 and he told me ‘see. I’m fine!’ between coughs. Last time we were there, we sat for hours, with the contagious (so not good) the drunks, and arguably worse? The people with a stubbed toe or a sore throat (thank fuck for triage). Plus, he argues that everyone at school is sick with the same thing, so it must be viral. True, but his friends don’t also have CF, they don’t have extra sticky mucous they can’t clear, and they won’t have an army of Pseudomonas bacteria already living in their lungs, getting ready for the party a virus lays out for them in his weakened lungs. 

I called clinic Thursday and they said they would get back to me. I knew the Cipro would run out tomorrow morning (Sunday) and it already looked like a bad idea to stop the one thing that might just help a bit until he gets on IV antibiotics, so at the very least, I wanted a prescription for more. But despite calling again and leaving messages three times, I heard nothing back Friday either. This is not like our team, and is most likely a sign the wards are full anyway. 

What we need is 24 hour wraparound care with the team that knows him. What we have is a weekend with limited access to consultants, a hugely overburdened A&E system, full of strangers to him and his health. We’ve been admitted before at weekends only for them to put off treatment until he could see his own team on the Monday anyway, as CF is complex, and registrars are reluctant to make decisions.  

He is asleep right now (4pm, and this is the kid who never naps). I’m sat here listening to his every breath/cough, ready to argue that we have to go in when he wakes up. This hurts. I can’t tell you how much I hate CF.