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. 

5000 THANK YOU’S

Before lockdown, I pledged to walk 500km over the month of January for sponsorship in aid of the Cystic Fibrosis Trust. Unfortunately, lockdown here in the U.K, home schooling, and then Isaac becoming less well again, and subsequently needing two weeks of intravenous antibiotics meant that this was not possible, however hard I tried to make it happen. Mostly, this was due to time... an average of 17km a day would take me around three hours. Gutted, I wrote that I would need to push back the challenge until after lockdown (whenever that might be). 

However, the lovely Claire White suggested instead make this a group challenge, and suddenly flocks of family and friends volunteered to join, walking separately, but in heart, very much together..... from as far away as Mexico, New Zealand, Poland and the Netherlands! And so the group challenge was born; to collectively walk 1000km over the month. I set a new target of 300km for my part, only part way through the month, so many people had volunteered, we upped the group ante to 2000km..... and what happened? 

My total: 300km

Group total: 2650km!!!

Total raised for this walking challenge and our fundraising at Cambridge University Press: £5110 ❤️

To read more, or sponsor us still, please click here....and to everyone who walked, donated, thought of us, thank so much. 

Special thanks to Claire, my Sarah, Camila, Anna, my sister Jo, my lovely Mum, Vanessa, Sam, Yasemin, Jo T, Steph, Jess, Hannah, Debbie, Chloe, Kay, Erika, Mieke, Jon & Flynn, Dilys, Gavin, Lemon, Kasia, Fran, Sarah OD, Tilly & Heather x (so sorry if I missed anyone!). 

It means the world, really, and 5000 thank you’s doesn’t cover it x 

Walking for CF

Amazing what you can do, when you come together ❤️ 

Myself and a group of AMAZING friends and family are walking 2000km over January with me, to raise much needed funds for the Cystic Fibrosis Trust. Due to the pandemic, charitable giving has almost completely dried up, and yet charities need the support more than ever, and we NEED a cure for CF. 

To read more, and sponsor us, please click here. Walking team - thank you all so much! 

My total so far:  254km

Group total so far: 1675km

How far we are off our £5,000 target: £250

Update on Isaac: Yesterday we completed a two-week course of IV antibiotics at home, and I de-accessed his portacath last night... Needle free life is good people! Symptomatically, he is doing really well. Lung function results? Not quite so great, but we can still hope for an improvement from the treatment over the coming days. Regular readers will know, we much prefer doing treatments at home than be admitted to the ward; more so now than ever, but .... it’s still exhausting, for us both. Today we celebrate with extra sleep, and for him, a long hot shower. 

Isaac complains.... hardly at all. The odd groan and eye roll, of course. I mean, he definitely has his moments in other ways (...ask him to come off his PS4 before he has finished a game, and you get an ear full...) but how he just gets on with things like IVs, that are imposed on him, on top of his already aggressive daily treatment regime? Especially when he knows he may not feel any immediate relief; will need blood tests every three days; putting up with your old Mum in your bedroom, early hours and every evening, poking and pumping you full of drugs while you doze; the endless beeping of the syringe driver.....? He never ceases to amaze me. Our wonderful boy. Big shout out to Dan and our gorgeous girls too, Anouk and Rosa ❤️ Home-schooling, working full-time, home IVs, keeping the home in a semi-fit state, fitting in the walks.... this is very much a team effort. They have been wonderful, they really have x x 

Mieke and Daan, adding their KMs in Rotterdam! 

From Vanessa in NZ. One big global hug! 

1,600km and counting...

Amazing what you can do, when you come together ❤️ 

Myself and a group of lovely friends and family are walking 1000km over January to raise much needed funds for the Cystic Fibrosis Trust. More than ever, charities need donations, and we need a cure for CF. To read more, and sponsor us, please click here. 

My total so far:  235km

Group total so far: 1635km - NEW TARGET 2,000km BY THE END OF THE MONTH!!! 

How far we are off our £5,000 target: £270

Trekking through snow and ice; so much better than mud! 

Camila x 

Little Evie and Isla! 

1,400 kilometres

Amazing what you can do, when you come together ❤️ 

Myself and a group of lovely friends and family are walking 1000km over January to raise much needed funds for the Cystic Fibrosis Trust. More than ever, charities need donations, and we need a cure for CF. To read more, and sponsor us, please click here. 

My total so far:  232km

Group total so far: 1472m

How far we are off our £5,000 target: £335

Walk from the lovely Jess.

Snowy walk today, with my sister Joby. 

Jo and Erika, mud wrestling. 

And just because he’s perfect. Obie. Photo by Rosa. 

I’ll post personal and group totals at the end of the month. 

Health update: Isaac is doing really well, ten days into home IV antibiotics. We’re exhausted, but hopeful x 

One thousand splendid suns

Myself and a group of lovely friends and family are walking 1000km over January to raise much needed funds for the Cystic Fibrosis Trust. More than ever, charities need donations, and we need a cure for CF. To read more, and sponsor us, please click here. 

My total so far:  176km

Group total so far: 712km

How far we are off our £5,000 target: £405

Not jealous at all, I need to walk this with you one day Vanessa! 

Steph and Louis

Sarah and I kicking some serious mud butt! X 

IV update and 1000km pictures

Quick health update: Isaac had his port successfully accessed Thursday, and so home IVs are now underway. Bloods indicate this is a bacterial exacerbation (his usual unwanted lodger, PseudomonasA) rather than fungal. Therefore plans for nebulised or IV anti fungals can go back on hold, which is a relief at this time, as either would necessitate time in hospital. Right now, he is coughing more, but this is typical of the start of IVs, clearing out the infected mucous. Otherwise, it’s going well. We have reasoned, there are worse times to having home IVs than in lockdown! 

Myself and a group of truly amazing friends and family are walking 1000km over January to raise much needed funds for the Cystic Fibrosis Trust. More than ever, charities need donations, and we need a cure for CF. To read more, and sponsor us, please click here. 

Pictures all the way from NZ (the lovely Vanessa) to the UK (the lovely Kay) and my foggy, flooded 10km today.

Keep well. Take care x x x 

River, flood or fog? 

IVs & Failed access

Hospital today. Isaac is stable, but no better after almost two weeks of extra antibiotics and anti-fungals. Side effects of the latter mean his poor lips are cracked again (and I don’t mean dry, chapped lips, but big old welts that bleed). The plan was for bloods, lung function tests, and then kick off IV antibiotics while we wait for the blood results (if the fungas is rampant again, they may consider nebulised and or IV anti fungals as well).  But, best laid plans and all that... after three failed attempts to access his port, we have come home, many hours later, with no venous access. Bloody annoying seems an apt phrase. Although frustrated, and in a little pain (the third attempt, they thought they were in, but the flush tissued under his skin, rather than going straight into his blood stream) Isaac was his usual stoic and wonderful self. Despite everything, we laughed on the way home, reminiscing on other medical fails in his history, and how much better things are now, than then (for one, although the port hasn’t helped today, this is the first time we’ve not been able to access it, and yet pre-port days, we had many a failed cannula, PICC or long line issue - funny how we can now laugh about these things, at the time I think we were all traumatised). The hospital was eerily quiet, being closed to visitors and all but emergency outpatient appointments. The wards are anything but quiet, and staff look exhausted. Weirdly, while there, we heard and felt a sonic boom as some fighter jets were scrambled to meet an incoming plane that was had lost comms. I think I am starting to be unshockable this year, amid the politics, pandemic and weird weather. Anyway, for now we’ll complete the extra meds he’s on, and will return on Thursday when his usual CF specialist nurse (and all time wonderful human being, C) is back in, hoping she may have better luck, knowing him and his port well. Portacaths can last anything up to ten years, but as he was 11 when he had this, five years ago now, he’s grown and how this child sized port sits in his chest now might be causing issues (as I understand it). It would be frustrating but understandable if this port were coming to the end of its little life. For now, we’ll keep our fingers crossed that this is a hiccup, not a port issue. It’s been a long day, and not an easy one for Isaac, but I reminded him that this has been the longest stretch between courses of IVs in a couple of years, or more. He smiled, said ‘oh yeah’, and just got on with it. It only cost me two M&S curries and a packet of yum yums. 

Today was not a walking day for me, but the group effort and sponsorship is growing. Thank you all so much x 

#Stay home, protect the fucking NHS, please. 

I’m not allowed to picture him much now, so have some ickle Ise instead ❤️ 

(PS. Before you report me, I don’t really give him beer)