New drugs and hope

We’ve kicked off another course of IV antibiotics, thankfully at home. No improvement yet, but his LF tests in hospital were better than a couple of days prior at home. Now we wait for sputum and blood lab results, and hope he’s on the right drug cocktail. We’re trying two new drugs, one of which is known for lots of side effects, but so far, so good. Six doses a day is keeping me on my toes, but Isaac also had his first training in mixing up and administering the IVs himself (if your wondering how this works, once his port is accessed in his chest, the line from the needle dangles down around 12inches (you can have shorter, or add extensions) which means he can easily reach, twist on the syringes, and push through drugs). He’s not close to doing this solo yet, but it’s a great start. I’ve always been happy to take the whole load, to take all the effort from him, as he has enough on his plate, but he needs to be able to move towards independence too. In my heart, I still hope that research will progress quickly enough, and a treatment might be on the cards that will treat the underlying cause of his disease (as CFTR modulators do for the majority of people with CF, with more common mutations). If that comes soon enough, hopefully his reliance on symptom treating antibiotics will lessen and he won’t need to. Love, live and hope x 

A couple more from our eventful holiday (which we are being (partly) compensated for!) 

(The one when Anouk couldn’t stop laughing 😆)

Fires, blood and drugs.

We flew out for our first holiday in a few years just over a week ago; the kids excited, us hopeful for a relaxing time. The week was, turbulent to say the least - and I’m not talking about the flights. 

We’ve never tried a family package holiday, but given the limited time we had (after Isaac had recovered from his last surgery, but before his next need for IV antibiotics) and funds, we got a late deal and headed out to beautiful Cyprus for the first time.

On arrival, the hotel was…. OK. The pools were fantastic, the beach, less so. The food was good, the rooms, less so. But nothing that we could moan about - and the first few days we swam, ate and enjoyed the luminescent sunsets. 

Until the hotel caught fire. 

The kids and I were lazing in bed one morning, while Dan was at the pool. The first thing we noticed was the Wi-Fi not working (ironic that), and then that the power was off altogether. Checking the corridor, we found it dark and filled with smoke. No fire alarm. No smoke alarm. No emergency lights. No one warning us to evacuate. We stayed pretty calm (Rosa was understandably anxious) and felt our way down the dark corridors and stairs to the pool outside, where everything and everyone was acting …. normally?

I realise you don’t want to panic people, but to leave them unknowing when children could have been left inside asleep, inhaling smoke? It felt like the whole world gone mad. When the smoke could later be seen bellowing from the top floor windows, finally word spread and they started to evacuate the building, but that was maybe an hour later. The hotel manager was trotted out and announced that the fire was under control, had started in the laundry, and while we couldn’t go to our rooms, we could enjoy the pools while they checked the building. With no power, we could only drink from bottles and there was little food. The toilets stopped flushing, and too late, we realised that the pool filters would also be off. I’ll come back to that point later.

An hour or so later, we were told the power was not coming back on, and we would be evacuated to a new hotel for the night. The impeding chaos would have been comical, had it not also been so stressful and hot (…no air-con, hundreds of frustrated or anxious people, potentially hours of waiting). Fortunately, we were in a taxi not too long after, enroute to our new hotel. 

When we arrived, the girls were jumping up and down ecstatic. 5* hotel, a whole suite to ourselves (three bathrooms, three balconies, three huge rooms) - and before long, they were were gowned up and the anxiety of earlier was lifting from our shoulders. 

The second half of the holiday was a mix, best described as anxiety in paradise. 

On one hand, the hotel was incredible, wonderful staff, food, pools, a beach a few steps away (we were in gorgeous Paphos), where we snorkelled for hours. On the other, we had no idea who was paying for this and were threatened daily with a return to the original hotel, which we had serious health and safety concerns about. We spent half the time enjoying the time we had there, and the other with the staff in reception trying to sort things out. The holiday was all-inclusive (our first) so we had no funds for extras, as we didn’t think we’d need any, but this hotel was not. The worry of all of this made it hard to relax and enjoy anything. 

But much worse, Isaac was becoming more and more sick each day. He was vomiting and had a rash (which is when we started to worry about the unfiltered pool he had swam in, which is a bacterial bath for anyone, but particularly risky for people with CF) and his chest was filling with mucous. He was pale, tired, not eating, and despite the heat, curled up on the sofa in covers. We contacted his CF team back home, but couldn’t get hold of them, and knew that seeing a doctor locally would be likely fruitless, unless we could access a CF doctor, or at least a respiratory one. 

We started our extra back-up antibiotic. 

I’m often asked about his intense use of antibiotics. He takes one orally and another nebulised directly into his lungs each and everyday. We also have the option to add a second oral one when a CF exacerbation begins, and IV antibiotics when none of those work. Yes, global overuse of antibiotics is a critical health issue, and one I have posted about before (see here for one) and feel very strongly about. But the breeding ground for bacteria that the CF lungs are, create an exceptional case and should be treated very aggressively, early on, with as many antibiotics that it might take to treat what we know are in those lungs, and possibly those we don’t know are there, but fear might be. Second to that, I am asked about becoming immune to antibiotics, and yes, this is possible, even probable. It is not the person that becomes immune though. It’s the bacteria that the person lives with that can become immune by mutating, and this is too a very critical issue and concern. However, in CF, while we do all we can to reduce this risk, we also need to use the arsenal of antibiotics we have to their best affect, as quite simply, to not do so, could risk death. So if you ever question why we jump to antibiotics so soon, this is a very specific CF treatment plan (right now, our only plan) and not one to be used for healthy people. 

The next couple of days panned out with us finally being allowed to stay at the second hotel (hallelujah) but also having to pay for that stay (we are assured this will be refunded, but still fighting for this to happen). Delayed flight home, we finally arrived home at 4.30am. While we were weary, Isaac was a thousand fold more so. After some sleep, we tested lung function (significantly and alarmingly low), O2 sats (OK) and collected sputum for the lab to test. We’re now discussing with his CF team the best plan, which is almost definitely more IV antibiotics. We are also to keep a new medication in stock to treat lung bleeds, as this can be risky, and his sputum now is blood streaked (likely due to inflammation, rather than haemoptysis). 

If there is any silver lining to this, it’s that Isaac has started to admit that he needs to adhere to all treatments more consistently - for Isaac to even admit that he feels unwell is big, let alone that he agrees to needing treatment - that is a big step forward. I guess he is learning all the lessons that I never wanted, but always needed him to learn. 

My kids are all, simply amazing. More soon x 

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. 

100 incredible miles

On September 2nd 2022, my friend Simon Wright will be running the Millstone 100. 

100 miles over the Peak District. Amazing, or just plain bonkers! 100 miles!!!!

Simon is running this on behalf of both his families charity, The Millie Wright Children's Charity (see Simon Wright is fundraising for The Millie Wright Children's Charity (justgiving.com) for the amazing work that they do), and, very kindly, for the CF Trust. Please give anything you can, every penny really is much appreciated. 

The fact that I have such beautiful friends who think of our cause, on the back of their own tragic loss, is touching beyond words.

You can donate to Simon’s charity here. They are raising important funds for families of children diagnosed with rare, life threatening non-malignant liver, renal and haematological conditions at Leeds Children’s hospital, to mirror those on oncology wards. A gap that must be rectified.

Or to the CF Trust on my fundraising site here.

Good luck Simon, and thank you doesn’t quite cover it. ❤️

Lungs that travel well?

Isaac continues to do really well, post surgery. The headaches have gone, but his cough is slowly building. 

Clinic today, and his lung function is slightly down on where we were just post-IVs, which is disappointing. That said, his quality of life is so much improved. He has started part-time work, with his Dad, as a builders labourer. The fact that he can now do three days a week, lugging things around, learning some carpentry and joinery on the job, and doesn’t come home broken is… massive. 

These changes, we celebrate every day, and an even bigger celebration will be a holiday later this month. Our first in, years? 

Cystic Fibrosis likes to throw spanner’s in the works when it can, and in this case, the search for travel insurance has been painful. Yes, he can walk 100m unaided. But, yes, he takes many more than 8 medications a day for his condition (to keep him well), and yes, he’s been admitted to hospital more than twice in the last 12 months. Many companies won’t even quote when you get that far. Finally found something which covers him, and is less than the cost of the holiday itself. I imagine if the worst does happen, transfer to hospital might be on a blind, constipated, three legged donkey, but needs must. 

I’ve been involved in a few initiatives with the CF Trust lately, and if you’d like to hear me sounding about 12 and inappropriately giggling too much, you can hear a podcast here. 

Right now, we will concentrate on keeping him as well as we can to travel. Have a great day x 

Miro ❤️

The little twiglet

Life after recovery feels blissful. 

Pain free life: never to be taken for-granted. 

We are making the most of every good day. 

Chilling with the little one… we can never get enough of woods or ponies. 

Wishing you all a wonderful and well summer x 

Failed stabbing

I tried (and failed) to access Isaac’s port for the first time today. 

He took it like a trooper. I need to practice more to give him that independence for when our beloved paediatric community nurses leave us when he turns 18. 

While I’ve prepared, mixed and administered Isaac’s IV drugs at home since he was a babe, and de-accessed his port at the end of the course (like popping a cork back out), I’ve never yet done the plunge. Imagine sticking an inch long needle into heavily scarred tissue in your child’s chest? Complete with sterile anxiety? Not fun. 

But it’s OK. We agreed together that I have a few months more to practice. I actually accessed him OK (meaning I hit the spot and got the needle in the right place) but may have nudged the needle while unclamping the line, which is easily done. Magnificent nurses have tried and failed on him three times in a row, for an amateur, I’m taking it as a win! It wasn’t quite as bad as I had feared. 

He is doing well. The post surgery side effects have been significant (you cannot imagine), but he’s so much better in himself. 

Thanks to an unexpected bonus from work, we’ve been able to book our first overseas family holiday in years. I cannot wait to treat them all. We just need to find some affordable travel insurance now, which is challenging. 

We’re also plotting our next fundraiser. Having reached £80,000 in fund raised for the CF Trust since Isaac was born, I have my eyes on that 100k. 

Love to you all x (Pic; from my visit to the Frida Kahlo house (work trip) which I still dream of). 

Thank you

Isaac is doing so much better, following intensive IV antibiotics, steroids, lots of pain relief, and now, some back up oral antibiotics to boot. 

He can breathe, and enjoy headache free days. 

We’re now hopeful for a few months of good health, with the bacteria that camp out in his lungs knocked to a minimum (thank you antibiotics) his inflammation under control (thank you steroids) and his airways cleared (thank you surgery and our heroic NHS). 

Thank you for all the amazing support, anonymous chocolate fudge in the post (thank you if this was you!) cakes our doorstep (from the poet in my heart) and all the love for our darling brave boy. 

I hope you too, can enjoy this summer to the full. Recent losses have reminded me, my friends and my colleagues just how precious each and every day is. As Eva always said, love, love, love x 

Pictures from our Rosa beans 10th birthday. 

Surgery part 2

There was some confusion, being in one hospital post surgery, needing to be discharged from there with new drugs, and yet needing to get back to another neighbouring one for other drugs and care. In that confusion (and partly, Isaac being desperate to get back to his single room and bed on the CF ward) we pushed for an early discharge and a nurse kindly walked us over for handover instead of waiting for a porter. 

Back on the CF ward, with the morphine wearing off, the pain was building. Isaac is terrible at asking for what he needs, despite a high pain score when asked. The bleeding was difficult to manage too. He did cheer up after some cold jelly (all he could really manage that day). 

Exhausted from our 5am start, I stayed until Dan could get there to take over, and we had agreement from the ward that he could stay with Ise that night, his pain and nausea being so bad. For that, we are very grateful. Actually, grateful is not the right word, more, righteous - he is just 17 and navigating adult care for the first year. IV drugs continued and they had a disrupted night with meds, obs, bleeding, pain and coughing. 

All said, we are hugely relieved and thankful that it’s done. The surgery was a success, and the IVs and steroids that he is on now give him the best chance of a good recovery. 

We’re now home, with IVs ongoing. Due back in next week for lung function tests. 

As always, our love and thanks go out to all the amazing NHS staff. Sitting outside theatre for hours, I saw the many patients/loved ones who take out their frustrations on the admin/nursing staff; Moaning about waiting times, delayed surgery, unsure of where they needed to be and when. I cannot imagine working in that environment and being the brunt of that kind of anger and dismay. They deserve medals, not abuse. No, not medals, an appropriate salary rise! 

Thanks for reading, and all the lovely messages. Feeling very hopeful now x 

Part 1a.

We are home. 

There have been some blood and meds coming out of his EYES incidents. 

But we are home. I’ll write more soon. 

Read some more from me here - LOVE working with my friends at the Cystic Fibrosis Trust x 

The op.

Part one (not because this story is exactly worthy of chapters, but because I am both wired and tired):

After a few days of IVs on the ward, and a LOT of questions about how it works between the two neighbouring hospitals (which seem to communicate very little) and very few answers, we made it to Addenbrookes for his sinus surgery on Monday. He was wheeled through by a porter (to his immense embarrassment) through a strange underground tunnel between the two. At one point, in the eerie silence, the lights flickered off and we found ourselves in a flooded section between the two, walls drip dripping. Not for public access that one. Felt all a bit too ‘The Shining’ for me. 

Once there, no one had any idea that we were staying next door at Papworth, and needed to get back there later that day for IVs. Isaac is super smart, but how anyone would navigate this alone is beyond me. 

We were first on the theatre list, for which we thanked the stars and moons (we’ve been there many times before, late on the list, nil by mouth (for Isaac, you may as well say ‘bury me alive with snakes’) only for theatre to overrun and the operation be postponed. 

The anaesthetist didn’t seem overly enthusiastic about anaesthetising him. We face this almost every op, having to list off his many medications, infections, and weird arse medical history. In the end, we pretty much agreed that this was going to be as good as he gets, and we might as well crack on. 

I was thankful to walk him to theatre as normal (where he amusingly tried to pull off all his probes while we wrestled him back as he lost consciousness). 

Pic. Isaac surfing in times of much better health. 

Afterwards, I had to walk (madly pace) eat (sip Diet Coke nervously) and kill time (I went back to his room in Papworth, and for the first time ever, was pleased to see the pharmacist; no joke, each and everyday on the ward we have to list off all of his medications, times and doses to the pharmacist, plus every doctor or nurse who comes on duty, which normally gets a... little tiresome. This time, we had a lovely chat about teenagers and how they all go through a non-compliance stage. I could have hugged her).

I’d like to add here that I could quite easily have called my amazingly supportive friends and family at this point, a number of whom would have driven up the moment I asked and bundle hugged me to the floor. The simple truth is that when stressed and anxious about Isaac/something as big as this, I am an anti-social bastard better suited to listening weird old prog rock/grunge on my headphones. 

The operation was listed to be three, maybe four hours. I was outside asking to see him after two, and back every twenty minutes after that, until five hours had passed. At first I was told I could not be with him in recovery (now he’s in adult care) but the second person I spoke to (also a Mum, I noted) said of course I could, recognising that he is just 17. But they did say that I couldn’t see him too soon, as it would be too distressing for me (this bit I contend; quite sure my morbid, black imagination is way worse. Plus, it’s not about me!). By the time I was allowed in he said he had been asking for me for an hour (mind you, he also asked me where he was four times, so it might not have been that bad). To say that he was in a bad way is an understatement. We’ve been through recovery a dozen times or more, but this was by far his longest anaesthesia and most complex operation. He was confused, loving, thirsty, in pain and really quite bloody. I wasn’t even sure if he was coughing it up, spitting, or vomiting at times (they did say he would have swallowed a lot). 

The surgeon was pleased with the result. He said the sinuses were even more infected than he had feared. He has now removed everything structurally inside that he can (in case you are wondering, this does not change his appearance at all, only his innards; imagine... in a few ops his nasal cavity has gone from Victorian cottage with cramped rooms, to an open plan house, and now been transformed into a huge barn conversion) meaning that this will be his last BIG sinus op. 

The polyps will come back, that’s just shitty CF sinus disease, but with better access for medication, we can prevent them for much longer at least, and polyp removal is, in comparison to this, a walk in a very pretty park. 

The morphine was fun (love the stuff!) but soon wore off. His saliva glands went on strike. Desperate, I suggested the gummy bear taste challenge just trying to get his mouth working again (he won). He was moved to a new ward. Shaky, pale, unable to walk or really eat still. 

The suffering at times felt unbearable to watch, and yet, my boy? He never once complained and was polite to everyone. 

Above all, we/he knows he needed this, to end that constant cycle of infection and the headaches, and so it’s such a relief to finally have it done. Thank you, our darling NHS. We thank you to all the stars, cheese moon and beyond!  Part two to come (getting him home and far more antibiotics). Thank you for all the lovely messages x 

On the ward and prepping for surgery

Isaac finally agreed with the doctors to come in. He’s not happy, but for now, is at the grudgingly accepting stage. Naturally, he feels this is all my fault (default teen state). 

Plan is for two weeks of IV antibiotics (worryingly soon after the last course) to help his lungs (we’re back to endless coughing by night, and grunting chest clearing by day) and to ensure he’s well enough for the anaesthesia on Monday. 

The operation itself, Isaac is more than happy to have. He knows from his four previous sinus surgeries that afterwards he can soon breathe more easily, has way more energy, and the rotten headaches disappear. This we are desperate for. 

Going to theatre is never a jolly, but I don’t usually start to really panic until the surgery day. But this time... feels a little different? I’m not sure if it’s the added complexity of it being managed by two different hospitals (we learned yesterday that there is an underground tunnel between the two, great, but the communication between them, we seem to have to manage? I hope to be proved wrong on this point, but so far, no one seems to be able to tell us how this works); the size of the operation (it’s considered fairly major); or the duration (Isaac has had countless operations/general anaesthetics in his life, but most have been an hour...maybe two? This may be three or more) - all of which make me worry. 

Manically pacing up and down the corridors will at least be good for my step count I guess (I’m really reaching for more positives now!). But I remind myself that this is.... good. It’s really good. We’ve waited for this for months, and yes, the IVs add to the burden of treatment and he is not happy about this (once he is well enough, we hope he can come home and I can finish the course at home for him) - but they give him the best chance of a quicker recovery and lessen the risks - and this much needed surgery will help get him back to a better baseline of health. Hopefully one at which he can fully enjoy his summer - especially as his college is now out (he’s missing the last couple of weeks, but has completed his exams). 

I reluctantly left him there (I can no longer sleep on the ward). Him grumpy and tired already. Me fussing and secretly a bit weepy. The view from his room, filled with fluffy clouds and distant green hills, might help.... were it not for the thought of what goes in those incinerators - which we joked about for a while until it got too obscene and I started to gag. 

More soon. Thank you for caring. Big shout to to my NZ contingency for their massive love and messages of support.... V, I love you! x 

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.

Hospital, if he will.

Hospital busy week. Pre-op and CF clinic, in preparation for his long awaited sinus surgery. His CF team want to admit him to the ward for IV antibiotics before, during and after his surgery. Isaac is not keen. 

Ideally, they want him as sterile as possible before major surgery so close to his eyes... brain....blood stream... and we know he’s hosting at least two bastardly bacterial parties right now, and maybe a fungal fiend too. 

He needs to complete his college carpentry exams this week, so the bed on the ward is booked for later the afternoon he finishes. In the meantime, we try new antibiotics to try and jack up his lung function - which he hopes is a get out of jail free card. I’m not so sure, but will fight my battles, should I need to, nearer the time. 

The good news is that this surgery will improve his QOL significantly. 

Love, love, love x 

Pony mad Rosa bean. 

Finally, a surgery date.

Our partially broken but wonderful NHS have come through with a maybe date for Isaac’s sinus surgery, for which he has been waiting for many months. While he waits, his bacterial infections play volley ball between his lungs and sinuses, and his QOL (quality of life) decreases massively, with headache after headache. I cried with joy on the phone to the admissions team....even though this is not a confirmed date (two weeks time), it is hope. More soon x x x 

A lovely tree

Just back from a weekend at the Suffolk coast, our home from home. I’ve not been well myself lately, so a very welcome getaway. Isaac stayed home, but the girls and Anouk’s boyfriend joined us. Beach, pub, the customary crabbing, meeting up with friends, long dog walks in the woods. Just what we needed. 

Isaac is somewhat better, but struggling headache wise, waiting for this sinus surgery. It’s been six months now, and while he’s on the priority list, the latest date we’ve been given is July. His overall attendance at college is down to 50%, partly due to courses of IVs and hospital, but much more so by these damn headaches and associated nausea. We are still hoping/pushing for a sooner date, but know that his surgeon is also - it’s less so about his backlist, but slots in theatre and the whole NHS backlog.

Sending love and hugs to anyone who might need it, I feel like many do right now x x x 

Sisters and trees x 

A tiny bit broken, but with a beautiful picture

NGL (my teens latest acronym of choice).... the last couple of weeks have been tough. 

Isaac refused to go into hospital, so we’ve been doing IVs at home. The doctors chose to pick their battles wisely and did not insist on him coming in. Any other time, we are the first to request, even beg for, home IVs and avoid an admission to the ward, allowing him a better quality of life. But this is the first time I’ve questioned it. To convince me, Isaac promised to commit to doing all his physio and nebulisers (all the daily treatments that he still needs to do) - but he hasn’t. We argue. I beg. He does a little better. He does a little worse. I collapse and crumple a little more. 

His CF team tell me that they often see this playing out at his age, and he will learn. But while we wait, we risk his lungs becoming more and more damaged - much of which can be irreversible. He says he understand the risks and doesn’t care. I don’t know if either part of that sentence is really true. I show him that I care - is that not enough of a reason to try? 

Today is day 14 - hospital review tomorrow to see if we can end IV’s. I’ve said I won’t do a third week at home unless he pulls his weight too. I’m exhausted, and tied to the house by five daily doses that only I can mix up and administer (he has thus far refused to be trained) and that we cannot be late for. 

I’d never normally complain; we’ve always been in this together. But now, I feel alone in fighting for his health, and it’s a pretty crappy feeling. I’m like a pea without my pod. Sanctions are not working. I need him to try harder - and all the while, his headaches worsen now he’s off the steroids, and we know the next admission is looming for his sinus surgery (if we ever get a date) - so know the next hurdle is heading our way. We need him as well as he can be for anaesthesia. 

My beautiful and talented friend kindly painted me this amazing portrait. Cannot tell you how much I love it! To see more of her incredible art, visit http://www.vollerthun.com/ - thank you so much Rachel ❤️

New drugs and... home

We are home from hospital. IV’s kicked off. Trying a new drug cocktail (Mero and Tobi). 

Symptomatically, he seems worse. The cough is deep, rumbling, reverberating against his ribs. But lung function tests today were better than the last few days have been, which is both surprising and promising. The mucous is on the move, we hope. He has accepted that he needs this intervention, finally, which makes everything, calmer? As per his norm, he’s taking it all in his stride, and although the day was long, we had some giggles. 

Due to bank holidays and staff shortages on the ward, it looks like he’ll be admitted for the latter part of the course, rather than the first week (which would usually happen) for some hardcore physio to help his battered and mucous filled lungs clear. He’s still hoping not to go in at all - but let’s see what the doctors think next week, and test results. Bloods and sputum in the lab now. For now, we treat the bacteria we know he has (PseudoA). 

The house is once again set up like a hospital; drugs stacked everywhere, needles and syringes in boxes, glamorous yellow sharps bin on display. The best thing about home IVs (other than Isaac feeling well, of course) is packing them away at the end and appreciating having home as we know it back. 

Happy to be home with a plan. Have a wonderful Easter break all x

Throw back photo of my sister and I. Thank you Jo, and all of our family, for your support at these busy times, especially with our gorgeous girls ❤️

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 (next of kin) of your wishes, as even when signed up, the decision becomes theirs after death. Thank you. 

  

Hospital, again.

Clinic today was, as Isaac would put it... ‘not the best’.

We don’t know the team at his new adult care hospital well, so we are getting used to them, and them us. New doctor today, who really took the time to talk to us and discuss options. 

Isaac is not doing his physio or treatments as he should, and I’m out of my mind with worry. He is honest with us and his CF team about it now, at least, but his excuse is that he forgets. I disagree as we set out all his treatments each morning, and we remind, beg, tell him numerous times a day. We talk to him, we explain the risks, he makes promises, and does well for a few days, and then.... repeat. 

One problem in CF is that he feels no immediate improvement when doing his treatments. As an asthmatic myself, I know if I forget to take my inhalers twice daily, I’ll be wheezing pretty quickly. Taking my inhaler though, I feel this immediate and lovely relief in my lungs. For Isaac on the other hand, airway clearance and nebulisers make him immediately feel worse (at times, endless coughing ensues). 

But he is smart, he gets it. He understands the need, but still, he is lazy with it, and for me, that is heartbreaking. I remind him that we endured literally thousands of hours of holding a mask to his face when he was a babe/toddler (he first grew the nasty PseudoA around 18 months old) watching Thomas the Tank Engine on maximum volume and on repeat to get these treatments done. Now technology means nebs are so much quicker, silent, portable.... how can it feel harder now than it was then? 

Talking through this with the doctor (kind but insistent), looking at his lung function tests from today (rubbish), and his recent CT scan (worrying) - we’re looking at another admission to hospital for IV antibiotics and some serious physio. This is in addition to impending sinus surgery, for which we still don’t have a date.

Right now, he is refusing to go in, but he’s said this before. We have another week on extra oral antibiotics, and a chance for him to crack on with some proper physio at home while we wait for a bed, in case he can increase his lung function without IVs, but it’s not looking likely. The CT shows too much mucous plugging. 

Home IVs are an option, but the Dr and I agreed, unless this is combined with him committing to decent airway clearance at home too, it won’t work. And right now, I can’t trust him to do this - and that is very hard for me to say. We have always fought for home IVs, although they exhaust me, as these give him a quality of life that hospital does not and we get to keep him close - but right now, maybe hospital is what he needs. This feels like tough love in its rawest form, and that’s not something I cope well with. 

The consultant repeatedly told him that he is just seventeen, and the irrefutable evidence on the CT shows signs that this repeated cycle of infection and inflammation, along with the regularity of his exacerbations and his occasional non-compliance with treatments is resulting in irreversible damage, and that is significantly concerning at his young age. 

This is not a sudden decline. Rather a gradual one which is harder for him to recognise and easier for him to deny; if not for the test results. As the doctor put it -  this is time for intervention, but a two week hospital stay is still a hard sell, however much we try. 

Things feel tough right now. I must say it’s not hugely helped when we are asked by every new CF team member ‘so, is Isaac on Kaftrio?’ and having to explain again and again that he is in that last 10%. To support our fundraising for the CF Trust, who are very focussed on not forgetting that last 10%, please visit my fundraising page here x 

Anouk and random tree ❤️

Genuine highlight of my week...

 ... was collecting a huge, juicy, sputum sample from Isaac to send to the lab. For me, this is gold in a pot.  

Not, of course that he should have so much damn mucous to cough up, nor that it was dark and way too sticky - but because without lab results on what his lungs are actually growing at any given time, treatment is still a bit of a stab in the dark.

Isaac’s lungs have been colonised with the bacterial bug PseudoA for the last 15+ years (I so distinctly remember the hospital call to inform me when he first grew this bug; the cold of the bench that I sat on outside the Edinburgh building, even the scent of the nearby bush... and most of all the awful understanding of what this news meant...). He also has regular fungal invasions (Aspergillus) as well the the odd random growth of NTM family bugs, or Staph, or some other delightful bacteria..... all of which battle it out for Top Dog in his already damaged and scarred lungs. 

Knowing exactly what is his lungs are growing at any given time means we can adjust his treatment accordingly and target the arsenal that we have at the right bugs. This knowledge is powerful. 

We are still waiting for Isaac’s PIP back payments. Worse, we have no sinus surgery date. Worse still, his lungs are really impacted by this cycle of sinus to lung infection. Mega dose steroids have helped a little, but his lung function is dropping. 

He still never complains. 

My heart, my love, my joy x 

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: