The court has ruled.

Finally got the letter from court today, following days of butterflies. 

The judge and panel ruled massively in our favour! Not only is Isaac awarded PIP for daily living restrictions, he was given the enhanced rate (we thought after the initial rejection that he might only be awarded standard rate, if at all), as well as standard mobility (which I had not thought we even applied for!). On top of this, the award is not only backdated from when his DLA payments ended last year, but covers five years (some are only given for two years) meaning we don’t need to reapply in all that time. 

We nervously opened the letter together. I felt sick with worry that I may have let him down. The relief is palpable. Isaac is delighted. I truly cannot thank Sangeeta and the CF Trust enough for their invaluable support, advice, and immense knowledge on this subject. 

I could bang on for hours about how and why Isaac deserves this support, but I expect I don’t need to here.  

Thank you for all the love and support that we have received. Love love love! 

Some happy photos x 

I saw the light fading out

Tribunal was today. A blur! 

Felt almost like a job interview, but one where you describe the worst job in the world. The one that no one would ever want. 

I was questioned for an hour, which is much less than I had feared or expected (I was told some tribunals last 2-3 hours). My representative from the CF Trust, Sangeeta, was not only amazing on the legalities side, but also gave me the strength to carry on. I cannot thank Sangeeta and the CF Trust enough for their incredible support. 

For any CF peeps applying for PIP; I strongly recommend gaining free advice from the CF Trust before applying. Had we done so, we may have been able to avoid this proceeding altogether. To support the amazing work that the CF Trust do to support the CF Community, alongside all the essential research that they fund, please donate here, thank you. (And for the generous Anon donation this week, which I can only assume is a close friend, thank you so much!). 

Isaac wasn’t well enough to attend (laid out in bed with sinus headache, cough, and a bad tummy from an incorrect creon dose with his pizza the night before) but I think of this now as a blessing; he doesn’t need to hear the difficulties of his everyday life articulated, spelled out, and interrogated in this incredibly detailed way, for strangers that are judging his (largely invisible) disease and how it impacts him individually. This is his reality and he’s lived with this all his life. He has two perfectly well siblings; he fully understands the difference between their lives/health and his. 

The panel was made up of a judge, a GP and a disabilities expert. Mostly the questions were well meaning and felt...alright. Only the GP did query why Isaac was not yet on CFTR modulators (such as Kaftrio), despite the fact that I had explained why Isaac was in the 10% of people with CF for whom the current generation of drugs would never work very clearly in his paperwork, as had his CF nurse, and the CF social worker; suggesting he had not fully read our case. Still, it gave me the chance to explain this fully which was good. The supporting letter from the CF Trust and his ENT surgeon also proved powerful, I think. 

I felt sick in the days before. Thinking I might let him down when he needs me most. And I felt sick going into it. 

Isaac, on the other-hand was actually sick in bed, but gave me a huge hug the day before, and after, thanking me.... but he shouldn’t have to thank me, should he? We shouldn’t even have this fight on our hands.... CF is a progressive, genetic disease, for which he has already been classified by the DLA as being breathing disabled *and this review only came about as he transferred from DLA to PIP after he turned 16. 

Despite that fact that they make the decision immediately after the case, we now await the decision by post.... POST! A few anxious days at most, we hope. 

Thank you all so much for the kind messages of support. My stress levels are still stars high, but I can see the light fading now x 

*T’was the Twattish Tories that introduced PIP as an alternative to DLA, and despite a rise in population, it was all about cost savings in social care;

“Although PIP was expected to cut costs by 20% over the longer term, costs were forecast to rise by £1billion to £15.4billion in 2015-16, partly due to a rise in mental health issues and learning disabilities.”

“The Multiple Sclerosis Society of Great Britain produced a report about its members experiences of the test in September 2015. 1,780 participated. 42% of those who had a face-to-face [PIP) assessment said the hidden symptoms of the condition had not been taken into account. More than a third said face-to-face assessments had caused their condition to relapse or deteriorate.” Wikipedia. 

Willem de Kooning, Untitled IV, 1982. Hope to share some amazing Ukrainian art next time. 

Tribunals, surgery and heartache

My heart actually aches. Can that be right? Certainly my body does. 

Isaac is suffering. His surgeon called me today (I almost sobbed with gratitude). Although Isaac is on the priority list (because of CF, he is treated as priority P2 alongside cancer patients) due to NHS backlogs, the wait could still be up to three months. 

The fact that his consultant took the time out of his day to call, to explain, to care, and to complain about the state of the NHS (which leaves people in pain this long) along with me, is, beyond touching. He told me how he feels bad for taking annual leave at times, his backlog is so long (I told him not to). Something is broken and it’s not only my heart and sanity. When you vote next, please consider this. 

I’m often asked if we can go private? Not an option unfortunately. We would beg, borrow and steal to do so if we could, however no surgeon would operate on him in private care due to his CF lungs and associated risks with infection and anaesthesia. He needs easy access to ICU. 

So for now, we wait. We pass the ice packs. Keep the painkillers in stock. Smother him in love. He’s not currently able to go to college (the headaches are too bad) and the impact of this additional time off....? Well, we’ll figure that out later. Worryingly the longer we wait, the higher the risk that the sinus issues further impacts his lungs, and we’re only now getting over the last admission and IVs. 

Alongside this, we are preparing for court next week - a tribunal to appeal against his Personal Independence Payments (PIP) decision. I’m not sure I have it in me to write much about this just yet, only that it’s like baring your bloody and beaten soul; having to articulate everything that is hard about daily life with CF; acknowledging the prognosis; detailing the very mundane but personal challenges to strangers, and fighting for something that is the very, very least that he deserves - with a ton of bureaucracy thrown on top (his case is over 120 pages of documentation so far; I’m going to my Dads caravan this weekend to work through this and prepare statements simply because I need to be alone to get through it and I’ll be rubbish company in that time). 

CF is a genetic, progressive disease, for which there is no cure or even treatment for the underlying cause of the disease in his case. And still they challenge that he needs extra support?

I cannot thank the CF Trust enough for the support that they are giving us through this process - I consider myself pretty resilient normally, but this is close to breaking me, and without them, I’m not sure I could continue. I’ll update on this after the case, as I’m fast learning about the process and maybe others can benefit from our experience. 

I hate writing this kind of post, I really do. I’ve been putting it off for weeks. But then I talk to the kids all day about being open about mental well-being. In truth? They are so much better at it than I am (which I’m so glad of). 

We are also thinking of, angry for, and sending funds and love to those in Ukraine 🇺🇦 

It is hard to see all the beauty in life when the suffering takes such a violent forefront. 

Love x 

That smile.... ♥️

No peace, but war

Rosa has COVID. She is doing fine now, after a weekend of high fevers, but has been very anxious.

Talk of WW3 worries her all the more (Anouk and Isaac see all sorts on social media and have questions). 

And what to say? We are all so upset and outraged for the people of Ukraine. 

We are chasing Isaac’s next sinus surgery date, as his headaches are back in full force. 

The ice packs and painkillers only go so far. 

We have a date for his tribunal, where I will need to argue in a court for his right for independence payments. This makes me want to poke my own eyes out. 

I don’t know what to say. I feel a bit like this, if I’m honest. 

Miro. 

Finding our way in adult care.

This brand spanking new hospital, with a dedicated respiratory/CF ward, is, for us, pretty life changing. 

The care we have received in paediatrics from our dedicated CF team has always been second to none. We always felt safe, cared for, and so incredibly grateful. However, once admitted to the ward, we were cared for on a general children’s ward (at Addenbrookes, this was mainly C3 for 0-3 year olds, D2 for 0-16 year olds, and another newer ward for day cases and overflow should they need the beds overnight). Although we still saw our CF doctors at times, the rest of his care was with general respiratory doctors and ward staff, not the dedicated CF team. 

Of the many, many stays we had on children’s wards, we were mostly cared for in shared bays (we only had a room when he was known to be infectious to others with a virus, not a CF bacterial infection) often six children to a room (with parents staying overnight, that meant up to 12 in a room) very little room for the equipment we needed to bring with us, all trying to sleep and keep our little ones settled. For as many times as I worried that others were kept up by Isaac’s cough, or his IV pump beeping, we would be kept awake hearing children in pain, anguish, and their panicked parents concerns. I often got up to give someone a hug in the night. 

Worst was the worry that he would pick up more bugs on the ward than we had come in with, or that we would infect others. We were once opposite a very poorly child reliant on regular suction to clear her airways for days, only to then find out that Isaac also had a nasty respiratory virus which he no doubt passed to her. 

We will always be so grateful for all the care Isaac received on these wards, but with the facilities that they were restrained with, it was at times difficult, heartbreaking, and painfully exhausting, often meaning he came home infection treated, but less well overall.

On this new ward, he will always have a room to himself. It is so, so quiet! He has a TV, an ensuite, a fridge for his meds, and an exercise bike - and does not leave his room at all, but for certain tests. We feel a tiny bit spoilt... but this is simply what is required to keep him safe in hospital. Due to cross infection risks that come with a shared respiratory ward - CF patients especially are known to have easily transmissible bugs that only others with the same lung impairments will be at risk from, he needs this closed environment to keep not only himself but also others safe. 

The staff are amazing, and seem calmer and a lot less stretched than we have seen on other wards (that said, we are only a few days in, and with the closed doors and quiet, maybe we just don’t see the stress as much?). They often check on him, and treatments arrive incredibly close to the times they should do (on a children’s ward, IV doses have often come as much as a few hours late, despite being needed at very regular intervals). This is no criticism, only an observation and concern that children’s wards are inadequately staffed and funded. Or perhaps the improved facilities in a brand new hospital create efficiencies that allow for better care? Or both? I don’t know the answers, I am simply a caring observer and longtime NHS fan.  

Today I was signed off to do his IVs again. A few of the nurses asked me if and how many times I had done this before (countless over the last 16 years) - still, there are a few differences (the doses seem more... rounded up in adult care (as opposed to mixing up 8ml from the 2g vial and 5ml in the 1g vial, then draw up all of the 2g vial but only 3.42 of the 1g vial kind of accuracy, this is more - the dose is 3g, use 10ml of saline to mix - super easy), and we can push through more drugs rather than use a pump, so quicker; and they don’t even require me to wear gloves and aprons..so weird for me!). It was good to be observed and have them confirm that I am doing it all correctly. On our next admission, Isaac will also be trained, to offer him the independence to do them himself, should he choose to. Another change is that the doses are much more frequent, meaning five doses a day, which will be quite full on for home IVs when we do get home. 

Isaac has been amazing. He’s bored but agrees that it is a good opportunity to get to know the staff and ward. His cough became initially worse on IVs as usual, but already seem to be improving. He’s on steroids too, which may be helping his boost. Tests tomorrow, and hoping to squeeze in a cheeky sinus CT while we are in, to speed up the process to get him a sinus surgery date. Then hopefully home sometime this week to continue the IVs at home. Today we revived a long dormant shithead tournament, is there anything better than a pack of cards for quick and easy entertainment? I love that I get to spend so much time with him, just us. 

We are feeling positive. I am on the ward with him all day, and then home with the girly ones at night. It’s at times exhausting (I’m never quite sure why that is, since it’s really a lot of sitting around and eating cheese sandwiches), and I’m equally as pained to leave him as I am happy to be home to see the girls, which has meant happy and sad tears all at once. One thing is for sure, they are all growing up so fast, and I love them all so much. OK, that was two things, sue me! 

One blue eyed blonde, one hazel eyed brunette, and one very curly dude happy with chocolates on the ward ♥️

Cystic Fibrosis is a life limiting, genetic condition, affecting some 10,000 people in the UK alone. CF sufferers often, as a last resort, need to trade in their damaged CF lungs for the lungs of a generous stranger. To sign up to support organ transplantation, please visit https://www.organdonation.nhs.uk/ and tell your loved ones of your wishes, as even when signed up, the decision becomes theirs after death. Thank you. 

COVID, hospital, surgery, and being a bit more Isaac

Nothing out of the blue. 

While we are pretty sure we had COVID at the very beginning of the pandemic (all of us but Isaac, amazingly) we’ve been fortunate since, but last week Anouk tested positive. Being the amazing human being that she is, she stuck to her room without complaint, and we cleaned after she used the bathroom. With her phone, Netflix and chucking her the odd snack, she was happy. Last two days she has tested negative and is now back at school. The rest of us have tested negative all week, but today there were a few confirmed cases in Rosa’s class alone, so still bracing ourselves. It’s just everywhere. 

Separate to the COVID malarkey, Isaac’s lung function has fallen significantly after a change in his cough this last week or so (we don’t think this is anything new, just a typical exacerbation of his CF lung infections, and not responding to Cipro); today in clinic they decided to admit him onto the ward to commence IV antibiotics. This is also off the back of his lung CT results, which were.... not fab. 

Not unusual. What is new, is that this will be his first in adult care, the differences being; we cannot stay the night with him (and we have never left him before); he will have his own room on a specialist CF ward (rather than being on a general paediatric ward); at his new hospital it is standard to start IVs with a stay in hospital (whereas we are used to doing the first dose in outpatients, and doing the rest at home); I need to retrain to do his IVs before they can sign off on me doing them at home (after 15 odd years of doing them); that we need to stick to actual visiting hours (in paediatrics there is no rule for parents); and,  because of COVID, only one person can visit throughout the whole stay (which will have to be me, as I’ll be retraining for home IVs) and this is limited to just one hour per day. 

I’m taking that last bit with a lump of frickin salt the size of my arm - not only will I need longer than that to demonstrate that I know what I’m doing IV wise, but this is his first visit, to a new ward, new hospital, with a new team.... and he is just sixteen. I’m lucky that he is a mature sixteen, but still, that is my boy. 

We were also told by his ENT surgeon today that his next sinus surgery will be a major one. This confuddles me muchly, as he’s already had the ‘major’ sinus surgery (effectively converting his airways in there from a traditional house to an open plan one) - I wasn’t aware there was anything more major that they could do! Sigh. So now we await another CT (surgeon was hoping this could be done while he is an inpatient, but the possibility of two separate areas of two separate hospitals (all be them on the same Cambridge site) being able to coordinate this at such short notice seems unlikely to me, but let’s see) and then a date for surgery. This will be his fifth round of sinus surgery alone, in what? Three, four years? 

Isaac is taking it all in his stride and doesn’t seem fussed at all. 

I wish I could be more Isaac. 

In fact, I think we could all do with being a bit more Isaac ♥️

Gerhard Richter love: 

Nonsense mutations, tribunals and test results

Of Isaac’s two weird arse (rare) mutations (one from each of us, unknowing that we were in the 1 in 25 people in the UK that carry the faulty gene) my gift was what is known as a stop or nonsense mutation (written out, this mutation ends with an X). This is effectively a stop sign in his coding to make the correct CFTR protein. If only we could throw a blanket over that stop sign, the protein might be formed correctly, fixing the underlying cause of his CF. These trials, and others, give us and others in the last 10% of sufferers for whom the new CFTR modulator drugs will not work for, much needed hope; read more here and here. 

Isaac is keeping quite well right now. His lung function is stable (which is good, but I still dream of improvement, rather than stability). However, his recent ultrasound confirmed continued progress in his liver disease (despite treatment) and the CT scan showed bronchiectasis and mucous plugs in various areas of his lungs. In addition to this, his ENT appointment keeps being pushed back month on month (likely due to COVID) and his sinus issues are becoming much worse (yesterday his sinuses on one side were so fully blocked, his meds came out of his eyes).

I also learned that we need to go to a tribunal to appeal his PIP (personal independence payments, which replaced disability living allowance) decision. They sent me a 120 page dossier to read through, in which I’ll need to contest any points I don’t agree with. It makes me want to scream, weep, hit walls and stuff. The CF trust are kindly advising, but today, I just can’t face it. 

On a happier note, I am still on annual leave, and have been enjoying a mix of sleep, kiddie huggle heaps, long dog walks, reading (I’m going to start adding book recommendations at the bottom of blogs, in case anyone is interested) and a mass house sort out. I’ve loved that. 

Stay safe out there. We’ve had a handful of bugs in the house these last weeks, but so far, no COVID. Main problem now is access to the damn LFT’s x 

Book recommendation: The hearts invisible furies, by John Boyne. I loved this so much. A little like Elena Ferrante (who I adore; look up the Neapolitan series, if you are not familiar) in that it takes place over a number of years and includes tragedy and injustice, but with the odd giggle too, and a narrator you come to love. I’ve gone on to read a few more of his works recently. He is author of ‘The boy in the striped pyjamas’ - but his repertoire is so much wider than just that. 

Pics: Anouk and I at Christmas (she takes a much better selfie than I can ever do!). Showing my old man and sister about filters (♥️). And seven years of our gorgeous Obie (German Shorthaired Pointer love). 

Feliz navidad

It’s Christmas, and.... Isaac is well!!! Not sure what 2022 will hold, but I’m taking this one as a win. 

As I walked the dog home from visiting the grandparents earlier, they took a detour simply to pass me shouting ‘bus wankers’ at the top of their voices with their Dad (...terrible TV joke, but had me in stitches). 

Wishing you all a healthy, happy Christmas. We miss many of you aplenty. Apologies for the lack of cards, yet again. I’m saving trees but sending love x 

Our beautiful, funny, cheeky... and frickin adorable ones. I am hugging them extra tight this year, thinking of those who sadly can’t. Love, love, love x 

A merry little Christmas

.... we wish you all! 

Isaac is COVID boosted, which is a present enough for me this Christmas. 

* Thank you to everyone who has been vaccinated, again, and again and again, not only for their own health, or their families, but to help protect those more vulnerable in society. It makes a huge difference to people with CF, and many other conditions. If not for yourself, then consider it volunteering for those in need, via needle * 

Isaac’s third booster, despite being Pfizer again, like his first two, resulted in fever and sickness. It seems to be waning now, on day four.... unless of course, it’s not side effects, but CF infection peaks... always hard to say with Ise. Anyway, today has been better, and we still hope for a well and happy Christmas. While Anouk and Isaac are fully fledged teens, they, like us, still enjoy little Rosa’s Christmas innocence and energy, which adds a certain sparkle to Christmas which I miss as they grow out of it. 

Our annual raffle at work raised over £4,400 for the cystic fibrosis trust. This is our 16th year. From very humble beginnings of a few gifts from suppliers... to a huge event, including highlights from team scavenger hunts to a draw for prizes including noise cancelling headphones, a Hotel Chocolat Velvetier, Echo dots, to massage guns. Sophie and Gem... how far we have come!!! Thank you for helping me launch this when I still felt hollowed out. I hope you are as proud as I am of what it has become ♥️

Christmas lights 2021 with the kids, and our newest baby cousin, little Lyra ♥️

Adulting

My child. Officially in adult care, at age 16. 

First day was exhausting, tests from 8am- 5pm, and then an emotional farewell to our beloved paediatric team when we dropped off presents and IV pumps. Isaac was great - speaking so much more to the new team, and being more honest about the treatments he is less compliant with (a decent amount of physiotherapy, mostly). Lung function was a smidge better. All the other tests (diabetes bloods, CF etc.) we now wait on. 

Our day in pictures... (apologies for the lazy post... more soon). 

Thank you for reading x 

New hospital, new CF ward, private rooms with ensuite. Not so bad. 

Caroline, our CF nurse for the last 16 years, and one of the kindest people I have ever met. 

Same site. New views. 

Doodles galore in hospital. 

Exhausted! 

Quick update

Lung function - down, but not abysmal. 

Anti-fungal drugs - underway. 

Tummy - unforgiving.

Plan? - transition to adult care and make a new plan. 

Next post will likely be an epic ode to our amazing paediatric team, many of whom who have been with us all sixteen years.  We are going to miss them immensely, but also know, it is time to move on (sob!). First appointment this week, including tests 8am-5pm, including chest X-ray, CT, glucose testing, lung function, bloods....the lot. Long day, but looking forward to seeing the brand spanking new hospital that will become our new home from home x 

PS. And wow, just re-read a post from five years ago, pre-portacath (read here). If anyone is needing but doubting getting a port, we can only say that it’s been miraculous for us. Isaac has so few viable veins for access now, and the ease of starting IVs quickly and easily, is such a huge relief. 

November

Quotes from me on the CF Trust website..... I cannot tell you how much the Trust do to support us and the CF Community. Read more about gene therapy progress here. Talking of the Trust, the annual festive raffle that we organise at my work is underway, thanks, as always, to my amazing colleagues - and it feels so good to be fundraising again after the break that the pandemic has enforced.

Isaac is... much the same. Not great, and fighting the battle he wants to fight right now. He is not improving on oral abx (Cipro) although his pseudoA infection should be sensitive to this drug. He wants to delay IV’s (I get this) and try anti fungals first (I get this too, but worry that we are delaying action and this might be causing long term damage). He has a point, since his last exacerbation saw him only better after the anti-fungals (and steroids), but the typical path would be to try IV’s first, to treat the bacterial infection we know he has. Anyway, CF team have agreed to a blood test to see his infection markers for Aspergillus (his fungal infection) first, to see if that might be the best path. It’s good that he is wanting to participate in these decisions about his care, but also, bang-head-on-wall-with-worry-frustrating at times. Hopefully we will have a plan by tomorrow. 

Thank you for reading x x x 

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