Bumps

Isaac is not so great right now. There are a lot of nasty bugs out there, right? Post lockdown bug vengeance, the little shits. I’m sat here tonight, not ill, but with no voice (every meeting I had today started with a squeaky whisper when I opened my mouth, or a slightly off-putting Al Pacino huskiness). My sweet nephew and many others I know have COVID (rates are high around here currently) and there is a cold going round that is wiping people out for days. 

Isaac has none of these (yet). This is just another bump in the very lumpy bumpy life of CF. And I hate it. 

In applying recently for his PIP (personal independence payments) I had the absolute displeasure of describing our day to day challenges in minute detail, and in writing about it (the paperwork is long, painful and laborious) I was reminded of the fact that CF robs us of spontaneity. When acutely unwell, lives are temporarily disrupted, maybe my lack of voice means I have a cold coming on that will annoy me for a few days. But chronic illness means constant and extreme disruption. Because of CF we are unable to plan ahead with any certainty (when will his next course of IVs be?) roam freely (what bugs will he catch next? And will the next one be the one that floors him again?) or to really, truly enjoy the good bits (it’s never an IF he will be sick again, it’s always WHEN). 

Isaac is coughing a lot (we’ve introduced a third antibiotic into the mix, but it’s not helping as yet) and the sinus pain is building (ENT backlog means we are still waiting for a scan, let alone a surgery date). 

Explaining CF to someone today, I said I would just love, absolutely love, for him to sleep soundly one night, really well. Just that. Is that so much to ask for? 

Isaac is not eligible for the wonderful new CFTR modulator drugs you may have heard about in the news (Kaftrio, Orkambi, Kalydeco) as he has two rare, class 1 mutations, meaning he never forms the correct CFTR protein in the first place, so needs a very different kind of fix (read more about his annoying mutations here.). However, there is much research going on that gives us hope (e.g. using other, non CFTR channels to compensate for the lack of CFTR). We need to keep up the momentum (which includes awareness and fundraising). I know it will happen, it’s just whether it will be soon enough for our boy that I worry about. 

The PIP process has been difficult, and continues to be. I hate having to explain, again, that CF can somewhat appear to be an invisible disease, but that he is breathing disabled, and that CF affects his bones, his kidneys, liver, pancreas, sinuses, guts, his well-being..... as well as his long suffering lungs. CF is a progressive, genetic disease, and yet they challenge us, year after year, to describe why he deserves the tiny support PIP (previously DLA) provides him. Frustrated doesn’t quite cover it. 

Thank you for reading x keep well x 

Man Ray ❤️

CF Live!

If you would like to get an update on some truly frickin groundbreaking research, that aims to benefit Isaac and other CF sufferers in that last 10% of CF peeps for which the current small molecule drugs (Kaftrio, Orkambi, Kalydeco) will never work for (he needs a very different kind of fix) and, eeek, weirdly including me on the panel (looking awkward and sounding about twelve) follow this link x hurrah for hope! 

CF LIVE

We're bringing together the cystic fibrosis (CF) community, people 

involved in CF care, researchers and others with an interest in CF 

for a series of online events. Find out more about CF LIVE and 

register for the next event.

Through CF LIVE, expert panels will share the latest information 

and updates from the world of cystic fibrosis. We’ll cover a wide 

range of subjects and you’ll have the opportunity to be part of 

the discussion by submitting questions.

6 October 2021 – Spotlighting the development of new 

treatments for CF, inclusive of all mutations. 

At this CF LIVE you’ll hear about laboratory studies developing 

treatments to target alternatives to the CFTR protein and get an 

update on the (literally) cutting edge world of gene editing. 

If successful these approaches could lead to more effective 

treatments, inclusive of those who are not able to take Kaftrio 

or Kalydeco (like Isaac).

Oh, and I’ll be on the live panel. EEK!  

Register here if you are interested x 

Hey...

Sorry, not posted much of late. I’ve not been feeling quite right for a while now. Most likely my beloved IBS (I hate my own guts) but this week had a gastroscopy and colonoscopy (I mean, why not both ends?). Feel like my internal organs have been through a spin cycle. Or like the consultant played Tetris with my innards. The hospital experience only reminds me further of how much Isaac goes through, again and again, and how well he lives this life. 

He is doing... OKish right now. College is going well, including more walking than he is used to, bus rides, being on his feet in the workshop all day (he is studying joinery and carpentry). He’s missed a couple of days already, which is.... difficult (being such a practical course). His sinuses are giving him jip, and we are chasing up ENT for an appointment, who have a huge pandemic backlog, and then likely surgery (his fifth? I think). This means headaches and nausea. His tummy is not great either, but at least his lungs are behaving well(ish). We are bracing ourselves for a difficult winter of bugs, but he will have his flu vaccine soon, and his COVID booster. 

Anouk and Rosa are doing well. Rosa is completely nuts for ponies, as was I at that age, and is proving to be a gifted rider. Anouk fills the house and garden with all her teeny friends and the laughter echoes through the walls. I wish I could shake off this tiredness, but still, I am soaked in love, and there are much worse things than annoying guts. I dream of their soft skin and giggles. 

Keep well x x x PS: will post soon about a CF Live event, which I will be a part of. Exciting! 

#edit, Isaac came home from college today with a raging fever. Hello autumn. 

Jacek Tofil ❤️

College... tomorrow (eek!)

I’m sure I’ve posted these pictures a few times before. But he hardly lets me picture him anymore (ARGH!). Today we cycled to his new college together - he hopes to mix bike and bus. It is not a short distance (took about an hour today, but that said, I had a slow puncture, and he is very unfit after a summer of gaming and being unwell. Pretty sure he could get this down to 45). I can’t tell you how proud I am of him for trying. He is doing so much better, and the timing could not be more perfect, after a shitty couple of months for him. I’m feeling a mix of excitement and trepidation for him.... but the reasonable part of my mind knows this will be good for him. Sixth form, new friends, a better routine. Renewed reasons for staying as well as he can. That’s the hope. 

My children are so frickin extraordinary, all of them x x x 

Hope for the 10%

Little video clip here about the ongoing work to find a treatment that will work for those, like Isaac, with the rarer CF mutations. It’s been so, so wonderful to hear all the stories of CF peeps benefiting from Trikafta/Kaftrio - it fills my heart with joy and hope. Us next, please! 

Today has been a good day - his chest is settling, the cough receding. He is so much better than he was, and my fluttering heart and funky adrenaline response are slowly falling. My body seems behind my mind by a few days. 

Now, Isaac is looking at starting college. He seems happy. This will be good for him. 

Keep well out there x 

Pintupi artist Wentja Napaltjarri

A little better, day by day

He’s still not right. 

Nor is he pulling his weight, treatment wise. 

Starting college might be just the thing we need. 

A routine. 

A desire to keep well. 

New friends. 

For me, I am taking it easy - after many fretful weeks, I can’t seem to turn off the stress response in my body. 

These gorgeous girls and the beach helped no end (thank you Fountain’s for taking us ❤️) 

Keep well x 

Still coughing

Waiting for the treatment to work. His nights are the worst. It’s typical for him to get worse before he gets better, but this is four weeks now of coughing so much more. Like the superstar he is, his spirits are high ❤️

Love this Lydia Okumura. 

Plans and rosettes

My instincts were right. Maybe instinct is not the right word, since it was pretty plain to see that he was no better. My senses were right (and painfully assaulted). Isaac’s lung function is lower than when we started IVs, and symptomatically, he is just the same (if not a little worse today). 

However, good chat with the doctors today, and we have a plan. Stop IVs (he is now de-accessed and showered!). Start four weeks of antifungals and steroids. 

Antifungals as we’ve tried targeting the bacteria, and that hasn’t improved how he feels, so this is our next best bet (we know he has aspergillus and pseudomonas in his lungs, but initial tests had suggested the pseudo was causing this exacerbation, and the aspergillus blood markers were not as high as they have been recently). Steroids to help turn off his own inflammatory response. 

This is turning out to be a seven week treatment plan, on top of everything he usually does, and the side effects of these drugs are not the best. But he is needle free (we’re trying oral antifungals rather than IV in hospital) and feeling free! He starts college in just a few weeks, and we so want him well for that. Two days until his exam results too (eek!). 

It’s good to have a plan, and for now, I am focussing on that. Sorry, recent posts have been a bit morose. This one has been hard. You are only ever as happy as your least happy child, as they say, and that’s the same for wellness. 

Keep well, and thank you for reading. Please do share if you would like to help raise awareness of cystic fibrosis x 

Rosa bean after her first ever horse show. She has found her passion in life! ❤️

Circles

Isaac is no better. 

But he is no worse. 

Week three of extra antibiotics, and nothing. Nada. 

It’s like circles of drugs and coughing, coughing and drugs. 

Hospital review tomorrow.

Maybe the test results will surprise me? 

Please let me be wrong! 

He says he will refuse to go in if they want to admit him. 

This is hard. 

Kandinsky circles.

Adrenaline junky

We are still home. 

IVs are going OK. 

Doing home IVs always leaves me with this low level anxiety which gives me the constant shakes and no appetite. 

I don’t know why. Since we’re thankful to be at home and not on the ward. 

Adrenaline likely. 

How many times have I done this? 500 hundred? A thousand doses? 

Giving them is fine, but mixing them is no fun. Have you seen the size of those teeny vials against my shaking hands and summer sausage fingers? 

Review with the team tomorrow, and lung function tests. 

I’m not sure how this one will go. 

He is coughing so much still. 

He says if they ask him to come in, he will refuse. 

This is hard. 

Keep well x

Bauhaus love. 

Exhausted from coughing/IVs

Apologies for the slightly morose last post. We are exhausted beyond mumbles. Sleeping only a few hours a night. But... we are also home (thank you thank you thank you). 

It was touch and go for being admitted. Something I had not pre-warned Isaac about because I knew I’d struggle to get him into clinic at all if he knew (he is currently adamant that he will not stay in hospital). Right now, the wards are riddled with COVID, but also normally winter bugs, such as norovirus, which they think have come early due to the pandemic and us staying home more. It’s odd. These bugs are relentlessly cunning. 

For us, this means limited beds - so a reprieve for now. They would also much rather him be safe and home than on the ward. However, we can only stay home if he a) responds to the drugs, and b) adheres to his physio routine, which he struggles with when he feels so crappy. 

We kicked off his usual menu of Ceftaz and Tobi with a side of NAC. Bloods results due in the next couple of days will say if he needs an anti-fungal kicker too. This is his first home IVs in months, which is amazing for Isaac, so we shall not complain. Still, summer timing is always frustrating (not that we had holiday plans). 

Lovely chat with our much loved CF nurse while the drugs were infusing. She said when Kaftrio was finally licensed for use in the U.K., she was tasked with going through the list of patients that they had, to see who would be eligible. When they realised that Isaac was in the 10% minority that this drug would NOT work for, the team were all gutted (being one of the less well on their list). I know they all care, but hearing this bought a tear to my eye. 

Yet again, they managed to get us in to clinic at 24 hours notice, and safely home with two weeks of drugs within hours. Love our team. 

Keep well out there x 

(oh, and did I tell you that his port is bleeding back again, after 18 months of not doing so? Some good news, which my CF friends will understand - a few less stabs!). 

Getting the IV station up and running again. I would pay good money to see them reduce packaging and to provide Tobi in 8ml vials! 

Hospital

He’s not good. 

Like, cough ALL NIGHT not good.

When I do sleep, I have nightmares about hemoptysis. 

I can’t imagine how tired he must be. 

I sneak in to take his O2 sats when he finally manages to doze. 

Still, he is not happy about going in. 

He needs IV’s for sure. 

But a possible admission to the ward? 

That’s going to be a hard sell in the summer holidays, unless he admits how bad he feels. 

Which he never does. 

Emergency hospital review tomorrow. 

July and sad goodbyes

Up until a couple of days ago, I could say that was Isaac was doing really well, for which we are super grateful. Hopefully the last couple of days is a blip he will get over quickly (we’re waiting on lab results to see what he is growing on his chest), if not, he will be reviewed in clinic. I don’t sleep so well when he coughs all night (the worry, rather than the noise, as I usually sleep like a baby stone) but he never complains (that boy ❤️). 

A huge wish of mine was that he would have a good long summer, following the end of school and his (not actually taking) GCSE’s. We await his teacher assessment grades in August. In September, he starts a course for Carpentry and Joinery, following his Dads footsteps into a trade. Not necessarily a career best suited to someone with a lung condition, but his choice, and I know he will excel in this. 

He has had numerous appointments lately, and one outcome was our almost discharge from scoliosis clinic. He has a curvature of his spine which has worsened over his teenage years as he has grown (not necessarily CF related, but may be) but it is mild (14 degrees - only at 40 would they consider surgery). As it is, he will hopefully never notice it, but if he has another growth spurt (not likely now, at 16 and a half) it could become worse, so they will see him one last time before discharging him at 18. 

We’ve also had emotional discussions with our CF team about his impending transition to adult care. This is a team who we have been with throughout. Some of whom have been with us from the very start. They have been witness to us at our very lowest, and sometimes, our most jubilant. Most importantly, they have been there for us always, only a call or email away. I cannot tell you how much we appreciate and adore our team, in particular our longest serving specialist CF nurse, Caroline, whose calm presence has in no small way saved me a million times. We all talk about how our NHS go above and beyond, but Isaac and I witness and personally benefit from this on a very regular basis. We are humbled and beyond grateful, and will pay our thanks to them all before we leave. 

It might not sound like much, since our Adult care team (The lush new Royal Papworth) is now on site with Addenbrookes, so we’re still heading to the same location, but this is an immense change for us. Isaac is now too old for a paediatric ward, so until we can get into Papworth (there is a bit of a backlog due to COVID) he is at risk of being admitted to a general adult ward should he get ill. When we do get to Papworth, the rules are all different (as I understand them, but my knowledge is limited, as due to the pandemic we have had limited transition clinics). I’ll no longer be able to stay with him on the ward (and have never left him before); He doesn’t have to invite me into clinic appointments; I am no longer consulted, or necessarily informed; Critically, they operate a different policy on home IVs. Right now, when Isaac is well enough but still needs IVs, we do the first dose in clinic (in case of a reaction to the drugs) and then I administer the two week course at home and de-access his port myself. The only help we have is the community nurses coming three times during the course for abx blood levels. Of course we are admitted when he is more unwell or if he needs extra tests or treatment, but this home IV regime, when we can, is massively beneficial to us all. He gets to sleep and recover in his own bed, go to school, see friends, and be with his family. In adult care, from what we hear, they keep you in as standard for the first week of IVs, and only allow home IVs for the second. Considering at his worst, Isaac had 10 courses of IVs in a single year, this could be massive for us. 

Both Rosa and Anouk have been isolating lately, due to positive cases in their school classes. Frustrating now we head into school holidays, but it is what it is, and for the sake of others, we follow the rules. Dan, Isaac and I are all now double vaxxed, and thankful. 

Love, love, love ❤️ 

The red balloon. 1956. 

Miss you.

Buckets of freckles.

Isaac is doing super well. Latest DIOS episode resolved, and the Cipro, which rarely works for him these days (often only delaying IVs) seems to have worked its old magic. His chest sounds good, and he was able to enjoy a luscious family and friends camping trip, swimming in the river for hours. Like me, he looks like he’s had a bucket of freckles thrown at him. 

With his LAST EVER day of secondary school behind him, he is free for the summer. Immensely hoping he gets to enjoy this special teeny time and CF effs off and allows him this. 

Have a great day x 

Two, one, two, thirteen.

Two new bugs growing in his chest. 

One new DIOS episode. 

Two new drugs to treat him. 

Thirteen more days of secondary school - ever. 

It’s been a rough week, but he is doing better now. 

Little Rosa had a COVID test (negative). 

We all need some sunshine and light. 

Love my long walks in the woods x 

Hello

Isaac had his Pziser vaccine a few weeks ago now, and will be having his next after the minimum 8 week break. Grateful doesn’t quite cover it. Any worries about side effects were soon put to rest - he hardly noticed a thing. The next few weeks went well. Coughing, yes, of course. We sent in sputum on a few occasions and the lab results are promising, no new bugs (he regularly grows both PseudoA and Aspergillus, that, we now take as a given). Each time we reach that ‘this cough is not getting any better, we need to go into clinic’ point, it improves just slightly, and so we hang between the point of good enough, and we need intervention here. This is a familiar battle in my mind, but the added complexity now is that Isaac wants, and of course, has a right, to say what he wants to do. And he really does not want to go into clinic. 

In the past, CF clinic has been for us all the things; safe haven, respite, dreaded for the news it would bring, exhausting, maddening, heart warming, a place of hugs and support, of pain, sickening.... you name it. But on the whole, Isaac was up for going, if not to feel better, then at least to miss school for the day and a treat from M&S on the concourse. But now.... not so much. 

This change in him has also made me think about this blog. I started it when he was nine, and have enjoyed, and, when I think about it, benefitted from writing about him, us, CF. 

Above all, this blog has massively increased my fundraising ability and helped me raise awareness of Cystic Fibrosis and organ donation. Many people have commented that before reading about Isaac, they had no idea what CF entailed, and if I’ve helped in sharing that knowledge even a little bit, then that is wonderful, as it benefits the whole CF community. CF affects just 1 in 2,500 live births in the U.K. (this varies globally; the U.K. and Ireland have higher rates than most) and so understandably, many will never have come across the disease, or at least, known to have. And for organ donation, if a single person has signed up and (this is the crucial part) told their loved ones of their wishes, then I’m happy. 

It’s doubtless cathartic for me to write, and while I’m always happy when people ask how Isaac is, when he is very unwell, it is easier for me to update all via the blog, rather than individual messages. That way, I send out the news, and receive love and support in response without having to be on my phone all the time, rather than with him. Anyway, all the thoughts. This is not to say I will stop blogging, only that, more so now than ever, I am mindful of the content, as he transitions to adult care. He knows and doesn’t mind that I blog about CF, as long as it doesn’t reach his peers - or centres on him solely. 

These last few weeks have been full of mini GCSE assessments, more nausea, vomiting, more coughing. Last week he took his food and nutrition exam, between two days of vomiting. Mind of matter only works so far with CF, but he managed the whole exam, and I think, did an amazing job (chicken pie and trifle, from scratch, in two hours - more than I would take on). My heart bursts with pride, again and again. No doubt he will need more IV antibiotics soon, and his fourth lot of sinus surgery is in the pipeline. His compliance with treatments continues to vary (taking tablets late, doing the minimal physiotherapy, mostly doing his nebulisers) and these choices he makes feels like little pins being screwed in my heart. On another level, he is a smart, he is sensible, he is sound. I tell myself this is a rebellious phase, and that he will want and need to stay well for all that he wants to do in his life, and when he realises that, start adhering better. Please let that be soon, and my pin pricked heart not leak before then. 

Keep well x 

Rosa in the garden at night, Obie, lovely Anouk, and a very serious freckle face ❤️

Vaccine!

Tomorrow Isaac will have the Pfizer vaccine that we have been so desperately waiting for. This is within a month of his sixteenth birthday (the age at which is is licensed for). He is not well right now. Waiting on lab results and he will be reviewed by his CF team this week, but that he can have this vaccine at last is a huge relief. Fingers crossed for few side effects. 

Have a happy Miro. Tonight we celebrate. Love, love, love x x x

Small molecules, vaccines and a health update

Love this TED talk and so grateful it remembers the 10% of CF sufferers for whom this new generation of treatments will NOT work (these treatments truly are ground breaking - watch the talk if you have the time, this is science at its best). This is not a simple ‘he didn’t get on with that drug’ scenario; the current generation of drugs will not, and never will help Isaac due to the specific mutation of CF that he has. He needs a different kind of fix. Sorry, I feel like I have said that a hundred times... 

These last weeks have seen Rosa, Anouk and Isaac return to school, which felt huge, after so long. I actually forgot when lockdown #3 even began. It is great news for us all, but after so long together, I’ve taken to madly hugging and smelling them when they got home, having missed them being around. 

Isaac got a good week and a half at school under his belt before coming home sick. DIOS issues again. Thankfully this time the plan is to treat at home, instead of on the ward as we did last time. His cough is also increasing, and I lie awake at nights with this echoing around my room and mind. Yesterday we had an ENT appointment, and found out that his nasal polyps are back and he will need more sinus surgery again soon (his fourth). 

If it were me, with all these health issues at once, I’d feel cursed and mega frustrated. Isaac just takes it in his stride. With his (non-exam) GCSE’s upon him and the fact that his last surgery was so recent, the team are hoping to delay the next for as long as possible with extra treatment (doubling the required nasal rinsing, steroid sprays). This is until his headaches become unbearable (right now, he feels stuffed up, but OK). We are very used to PPE in clinic, but this surgeon was in something else, kinda like a hazmat suit but with more pipes; we could hardly hear a word he said (nasal scans would release any COVID infection far and wide... and I was asked to leave the room for my own protection). How they manage to work like that, all day? Our gratitude to all key workers throughout this pandemic is boundless. 

We are busy chasing a COVID vaccine for Isaac. Thankfully, data on CF sufferers having COVID is very positive, likely due to the fact that they are, a) predominantly young and b) were/are shielding. He is now eligible, but the problem is that he can only have the Pfizer vaccine, which is licensed for over 16’s, unlike the AZ one. Both seemed to be widely available just before his birthday (when they would not allow us to make an appointment) but now we can book him in, all we have locally is the AZ. The hospital cannot help; they say it’s the GP’s responsibility. GP’s are trying, everyday, but having no luck, even though we are more than willing to travel. Isaac has a history of reacting badly to viruses; on each occasion that we have been in hospital and he has become unwell enough to need O2 has been when he has also tested positive for a virus (which in turn, allows his PseudoA and aspergillus infections have a little party in his lungs) so however good the data appears for CF/COVID, we remain anxious. 

Have a great day x 

Song for Zula, Phosphorescent. Love this, listen here. ❤️

Some say love is a burning thing
That it makes a fiery ring
Oh but I know love as a fading thing
Just as fickle as a feather in a stream
See, honey, I saw love,
You see it came to me
It puts its face up to my face so I could see
Yeah then I saw love disfigure me
Into something I am not recognizing
See the cage, it called. I said, come on in
I will not open myself up this way again
Nor lay my face to the soil, nor my teeth to the sand
I will not lay like this for days now upon end
You will not see me fall, nor see me struggle to stand
To be acknowledged by some touch from his gnarled hands
You see the cage it called. I said, come on in
I will not open myself this way again.
You see the moon is bright in that treetop night
I see the shadows that we cast in the cold clean light
I might fear I go and my heart is white
And we race right out on the desert plains all night
So honey I am now, some broken thing
I do not lay in the dark waiting for day here
Now my heart is gold, my feet are right
And I'm racing out on the desert plains all night
So some say love is a burning thing
That it makes a fiery ring
All that I know love as a caging thing
Just a killer come to call from some awful dream
And all you folks, you come to see
You just to stand there in the glass looking at me
But my heart is wild, and my bones are steel
And I could kill you with my bare hands if I was free

Isaac is doing, OK.

Our boy, 16?! 

Beautiful Anouk, at the clunch pits

Rosa Isaac huggles

Cake by the amazing Bonny Fountain ❤️

It has been a good couple of weeks. The kids are back at school, and all going well, Isaac will get the COVID vaccine very soon (he can only have the Pfizer one, as the others are not licensed for under 18’s, and we’re struggling to find a place that will take him; but if we need to hassle and/or travel, we will!).  Test results from his annual review are still pending. 

Keep well. Not long now x 

Sixteen

The storms too, they pass. And then you just see the sunshine. 

❤️❤️❤️

Clinical update and vaccines

Today we went to Addenbrookes for what might be Isaac’s last annual review in paediatrics. 

For the last 16 years, around his birthday, we go in for a day long series of tests. This gives the team a good idea of his baseline, and progression of the disease (this is on top of his usual CF clinic visits, which we have on average, maybe 10 a year). The annual review typically includes:

Full bloods

Blood glucose testing (testing for CF related diabetes)

Chest X-ray

Liver and abdomen ultrasound

Bone density tests

Full lung function tests/spirometry 

Port flush

Cough swabs/sputum samples for the lab

Exercise tolerance tests

Psychology review

Dietician review

CF nurse review

Doctor review

Physio review

Pharmaceutical review

Due to COVID (numbers at Addenbrookes are coming down at last, 12 in ICU and 50 more on wards currently) today was a stripped back version. No ultrasound, blood glucose or bone density scans - instead they will review the blood results first, and run these tests later should there be any concerns. 

Today was pretty much my favourite day all month, which seems mad, as annual reviews of yesteryears have always exhausted us, mentally and physically. 

I cannot tell you how much love and gratitude we have for our whole MDT (multi disciplinary team), who go above and beyond for us every time, and even in these most difficult times, take the time to listen, consider and create a plan that works for Isaac and our family. 

I’ve been encouraging Isaac to take the lead in talking to his team for a while now, with limited success. But today he really stepped up, asking me only to review his prescription list. He spoke politely to everyone - beginning to participate in his health care plan. The only downside was that due to his weak and damaged veins, from years of needles, it took four nurses and five stabs to get bloods. He never complains. 

When Isaac was maybe 4 or 5, we had a few years where I dreaded both CF clinic and annual reviews to my core. Not only was the news always mixed, and often disheartening, his behaviour in clinic could be terrible. I was usually on my own, but sometimes with a little Anouk too, and we were confined to a single room for hours due to cross infection risks. All for discussions he did not understand and tests that he did not want. He once threw an orange at our consultants head (still sorry, Richard). Those were the days of sticker charts, rewards for good behaviour, and me silently screaming in my head until it throbbed (OK, I may still do that occasionally). Once I reversed the car pretty hard into a huge concrete post, stressed and trying to escape the damn hospital car park. Little Isaac cuddled me, and, our roles reversed, had to tell me that everything was going to be OK, as I sobbed snottily. 

Today, we laughed, we talked more in a day than we have all week, we joked about, we discussed our hopes for the future, and even agreed on what he could do better, treatment wise. I know I always say this, and believe me, he can be a right pain in the arse too, but always, he is my absolute hero. 

Test results will come in over the next few weeks. For now, no treatment changes; although his coughing is increasing again his lung function is stable, so we await lab results, since he’s only been off IVs for a month. 

Next up.... COVID vaccine as he turns 16 on Monday! That is my advance warning of some baby to teenager picture spam to come ❤️

Have a great day, keep well and wishing you all a vaccine day in the near future too x 

Wish you were here

In January Isaac had two weeks of extra oral antibiotics and anti-fungals, followed by two weeks of IV antibiotics to treat a CF exacerbation. Just a week later, he is coughing more again. Today he felt sick and hasn’t eaten. He looks pale and worn out, despite sleeping all day. He hasn’t left the house in weeks, apart from for hospital. He needs sun. He needs to see people. He needs a break.

Hell, I miss our friends, our families. Spontaneity, the changing of air, aiming for a destination, having a plan.... not fearing hospital trips. 

Things have got to get better, right? 

We were so lucky, having all that freedom. 

Keep well x 

Yesterday’s sky and today’s walk in the hale.