Floods and drugs

Hoping you’re all have a wonderfully restful Christmas. Ours has been just that - we miss others, but have made the most of the time we’ve had, sleeping in, watching movies, long dog walks in the floods, house stuff, and playing with the new toys (Rosa’s hoverboard and cart being a winner this year). I even saved a kayaker from the river in the floods (I suspect, just in time to avoid hypothermia). This, and seeing six fire engines, two police cars and two ambulances today racing down the lakes locally show just how dangerous floods can be (it sounds as if a car got in trouble in floods, possibly not knowing where floods actually become lakes, or the river, and how strong the currents can be). Yet I’ve never seen the fens busier. This once tranquil and quiet area is now a bustling attraction in lockdown. 

Fields not lakes.

Kaftrio, the new treatment for CF, has been in the news again and is undoubtedly the best thing to come out of 2020 in my eyes. Today the Observer detailed the funny year that CF patients have had; on the one hand COVID was expected to be a huge threat to all with chronic illnesses, but this hasn’t proven to be true in CF. Mostly, I expect, because COVID turned out to be so ageist, and CF sufferers are mostly young. But also because the CF community is well versed in infection control already, mask wearing, avoiding the ill, being uber clean, and seeking medical help as soon as symptoms progress. I read blogs, watch vlogs and speak to friends who have been able to try Kaftrio often, and it never ceases to amaze me. An oral drug, that can treat the underlying cause of CF (the CFTR mutation affects the movement of water and salt in and out of cells) - it really is incredible science. And the results have gone far beyond what many dreamed of; children and young people with end-stage CF are being removed from the transplant list, no longer needing new lungs from a generous stranger. In many cases, quality of life has increased dramatically. Children who take this drug may never have the inflamed, scarred and damaged lungs that Isaac has now. Families may avoid the pain, the stress, and the heavy burden of treatments that has been the norm for others all this time. 

The lesser spotted teen, on camera! 

Friends contact us when reading these stories... excited as we are in these exciting developments. However, Kaftrio will never work for Isaac. It’s not that it needs a slight tweak to make it work for his specific mutation, he needs a whole new recipe. To understand why, you can read more about his rare mutation here. That said, the success of these new drugs fills us with hope, and while we know it’s going to take a while (years, not months) we think that one day we will able to access a clinical trial for something that will work for him, and the other 10% of CF patients who are still waiting. 

Right now, Isaac’s lungs are struggling. Lung function dropping, cough increasing. We’re waiting to make a plan with his team as to our next steps, hoping above all to stay away from hospital, as they struggle with rising cases of new variant COVID patients in this tier 4 area.  

Christmas Day Bellini breakfast 

I will be walking 500km over the darkest month of January, and some wonderful friends and family will be pledging their own active challenges in the theme of 500 to support my fundraising further. There is no Christmas break from Cystic Fibrosis, and more than ever, charities need your donations. To read more, and sponsor us, please click here. 

Keep well x 

Obie, the most handsome dog

Wishing you all a well Christmas

Walking in a winter wonderland? Rather, Walking into tier 4 viral-war. 

Silver linings: We are home. Isaac is better (tummy wise, but cough is building up to an exacerbation again). Our family and friends are well, or getting there. We are not living on an airfield, in a haulage lorry cab, eating cold bacon baps, hoping to get back home to family for Christmas. We are not working over the holidays on Brexit deals, or as key workers. There have been no delays to accessing Isaac’s medications. We are warm, dry, fed, and together....

We have enough, more than enough. We are the lucky ones, but I think it’s also OK to admit; fuck this is shit! 

Mostly, I just miss people so, so much. I miss dancing. I miss hugs. I miss pubs, and soaking in a new atmosphere. Hearing the laughter of strangers. People watching. I miss meals with friends and family. I miss chaos with the kids and their friends. I miss feeling safe, and breathing freely. I miss excited anticipation, and spontaneity. I miss being early, or late. Having plans! I miss dressing up. I miss waking in the night, still giggling from the nights fun. I miss the jaw and rib ache you get from having laughed so much. I miss other peoples houses, and others feeling at home in mine. I miss being able to tell the kids of all our festive plans. It’s just one year, we think. This is not forever. But it is pants. 

I will be walking 500km over the darkest month of January, and some wonderful friends and family will be pledging their own active challenges in the theme of 500 to support my fundraising further. There is no Christmas break from Cystic Fibrosis, and more than ever, charities need your donations. To read more, and sponsor us, please click here.

Keep well x 

Hospital, transplants and walking

We just got home from a quick admission to the ward. The pain, vomiting and nausea hit a peak, as his abdo X-ray was pretty bad last week, and the initial treatment did not work, they called us in on Monday for another kind of treatment aiming to kick start his digestive system again properly.

For this, the plan was NG tube for 3 litres of medication (and IV fluids through his port). We were told we might get home in 3-4 days... but escaped early today on the proviso we go straight back if he has any pain or vomiting again. Getting out of hospital earlier than we thought...? Pretty sure that is a first! 

I can only imagine how Isaac feels....battered and bruised from within I guess (36 hours nil by mouth, an uncomfortable NG tube, and vast quantities of meds and fluids that bloat you out...) - and there was some fairly significant cursing when we arrived; he hadn’t been able to eat for 14 hours before we got there due to nausea, and when he finally mustered up an appetite, only to be told, no, sorry, you need to be nil by mouth for the duration (they didn’t tell us that bit)..!! But he was, in his usual Isey style, understanding, if not enthusiastic. 

As with any admission to a children’s ward, I’ve come home feeling raw, humbled, emotional; especially at this time of year, seeing so many kids on the ward who will not get home for Christmas, or maybe, at all. 

Wonderful short film here about life, CF and lung transplantation if you’re interested, here. 

This week has been an uber emotional one; a family funeral that no-one can attend due to COVID, hospital stay, test results, and then yesterday, our annual raffle at work, in aid of the CF Trust. I can honestly say, I don’t think there was a dry eye in the house after watching videos of the many festive activities that my colleagues got involved in leading up to this fundraising event, a hugely touching speech from our much loved MD, a fantastic and moving poem from a very good friend, and a fundraising total of over £4,000. I can’t thank enough everyone who helped organise all this, and all who donated X 

I will be walking 500km over the darkest month of January, and some wonderful friends and family will be pledging their own active challenges in the theme of 500 to support my fundraising further. To read more and sponsor us, please click here.

A Christmas Rosa and a hospital spot the difference! 

Hospital and birthdays

Clinic today, to try and get to the bottom (crappy pun intended) of his digestive problems; pain, nausea and vomiting.

He immediately had and X-RAY and bloods to test for pancreatitis (rare, but serious) and an emergency surgical consult. Unsurprisingly, the X-RAY showed likely DIOS, again. Relief that it’s not something new, but also....sigh. I mean, yes, better the devil you know and all that - but a frustration nonetheless, we’ve been round and round with this years before.  The good news is we are back home, with Gastrograffin again, to treat this over the weekend. But this is not a long term solution. 

It is not right that he is needing pain relief and anti-nausea drugs most days. The vomiting has no rhyme or rhythm, but at the same time, it’s a constant and unwanted companion. It doesn’t appear to be linked to what he eats, how his lungs are, what treatments he takes (as far as we can gather), or his physiology (in that, Gastroparesis has previously been ruled out, and he’s eaten radioactive egg on roast (really) to check his digestive track, albeit a few years ago now, and that was all fine) - it’s just always there, making him throw up, on a average, maybe 3 times a week? 

Isaac’s digestive problems rarely make it to the top of our priority list to focus on; because he has both bacterial and fungal lung infections to battle with concurrently, these preoccupy most of our time with the doctors. Also, Isaac is atypical in CF in that his weight and height are very good now. He was underweight as a babe, and we had years of supplementing his diet to boost his weight, but for the last 5 years, we’ve had no growth worries at all - perhaps because of this, his digestive problems rarely make it to centre stage in his medical reviews, and play second fiddle to his lung problems. But in the last few weeks he has missed probably 50% of school time due to vomiting, pain and nausea, and that is no quality of life. When we do get him into school, his is often sent home, looking pale and nauseas, and promptly throws up and then curls up in bed. We will be picking this back up with his team again, once we’re over this episode, looking for some longer term solutions. 

In his usual style, Isaac took it all in his stride, pointing out his good veins for bloods, and we had a good giggle about a new dietician that he saw, who talks to him like he’s a pre-schooler (‘so Isaac, you able to swallow your tablets whole, how wonderful!’). Not quite sure how he would take the 50 odd tablets a day if he weren’t able to swallow them! 

Today is Anouk’s 12th Birthday - our lovely Shnuk’s, could not love her more ❤️❤️❤️

Frida, fundraising and health

Wowsers. Feeling pretty overwhelmed; from as far away as Mexico City, New York, India and Madrid, my amazing colleagues at the Press have been buying raffle tickets and donating money for my 500km walk challenge in droves. 

Can’t tell you all how much we appreciate all of this. These donations not only fund important research into Cystic Fibrosis, provide much needed grants for medical professionals to become specialists in CF, but also support people with CF, many of whom have been in isolation during this whole pandemic. 

Again, many hugs and thanks to Heather, Charlotte, Kay, Beth and Megan for organising the scavenger hunt at work, which leads us into the raffle celebration, and all the teams who are participating, we’ve been crying with laughter watching some of the videos coming in! And to our MD, Paul, for wanting this to still happen this year, bigger and better, despite all the complications. I am so, so grateful. Also to Vicky for the help with internal comms - I can’t quite believe that over the last 14 years we’ve gone from raffling off a few gifts from suppliers, to this huge event, that many of us in ELT look forward to each year. 

• For CUP friends only, to enter the raffle, please see internal comms, and remember not to include gift aid, as that contravenes gambling laws.
• For everyone else, to sponsor our 500 challenges, you can do so here (and add gift aid if you are a UK taxpayer). 

Right now, Isaac is stable. Lung function is no better, but no worse, and he continues to have problems with nausea and vomiting. But we have options, including another anti nausea drug to try, and all that said, he’s just completed what I suspect to be his first full week in school this term. He’s had a good stretch, for him, without IV antibiotics which is both reassuring.... and yet, feels risky going into the festive period. Being on home IVs for Christmas would be a shame, but an admission to the ward? Much worse; and we always have that risk at the back of our minds. For now, every good day deserves a mini celebration (tonight with kebabs). 

We’re not quite sure where on the priority list Isaac will be for COVID vaccine yet. Adults with CF are on the priority list, but as kids are doing so well, he is no longer identified as critically vulnerable according to his team. Which is a huge relief, but we still hope he can receive this vaccine soon, and we have no doubts about its safety, only fears that anti-vaxxer propaganda will mean people will not take it, and continue to put the vulnerable at risk. Having lost our Mamgu (Welsh Grandma) to COVID this week, who died in a care home, without her family, I urge everyone to read the science, not the sensationalist news and associated trolls. 

THANK YOU AND KEEP WELL x 

Feeling the Frida today; like our Mamgu, a strong and wonderful woman. Pictures also from her home, that I feel honoured to have visited. 

500, because research can’t wait.

Today Isaac went back to school. having missed another three weeks, including pre and post op isolation. Today is a good day; He has finished the angry steroids; the extra antibiotics (those on top of the two he takes daily anyway, prophylactically); and, best of all, is weaned off the pain relief (....mostly. It is really frickin hard to watch your 15 year old being reliant on morphine, fentanyl, brufen, and paracetamol so much). 

I was about to write that it’s been a bumpy month, health wise. But that could be said for the last year, or even... three years if I’m honest. This time three years ago we were arriving home after weeks in hospital and being on intensive care (read more here), and in my more realistic moments (me being a born optimist, I seldom take off my rose tinted glasses) I concede that this was a pivotal point in his health declining. Just as we feared. 

He won’t feel the full benefits of this third sinus op for weeks yet, but he is seeing some, and it’s reassuring to think of that PseudomonasA infection being cleared from his sinuses, if not from his lungs. He is home, he feels well, and for that, we are super grateful. 

Pseudo first infected his tiny pink lungs when he was around 18 months old, and have never left - the little bacterial bastards. I remember with a mix of sadness and awe those days; holding a nebuliser mask on his face, while the machine rattled and grinded, noisily aerosolising the drugs to reach the far off parts of his little lungs, for 30 minutes at a time, with Thomas the Tank Engine on full blast - he took it all in his stride, even then. But that’s not to say it was easy for any of us. When we first moved to this house, we camped downstairs for months while we renovated the upstairs (little cottage, low ceilings, tall husband...), sleeping together on a mattress on the floor, making physiotherapy fun by jumping off packing boxes, piling cushions on the floor for percussion therapy, making bubble pep by blowing through straws into soapy water to teach him breathing techniques, community nurses coming round amid the chaos of building work to help with bloods during IV antibiotics, and yes, I remember it was hard, but also, weirdly, the best of times. Time does that though, doesn’t it? Only the Pseudo stayed put (as is typical in CF, the damn bacteria love a sticky CF lung) like an unwelcome guest. Technology has thankfully moved on, so today, while he still needs to do six nebulisers a day (one to draw moisture into his lungs for physio, another to soak the bugs in antibiotics after physio, and finally an enzyme to help break down the sticky mucous inbetween the physio, all twice a day) the machines are now more mobile, quieter, and quicker. Right now, his lung function (the main measure of wellness in CF) is frustratingly low, and we know as well as the pseudo he also has fungal infection invading his precious airways. But we’re hopeful that this is a post op lung function dip, and it might bounce back still - before we have to climb back on the IV antibiotics train once more. One positive from the pandemic is having home spirometry now, meaning we can test his LF as often as we feel we need to. 

But today is a good day. Today he feels well, and we have learned to celebrate each and every one of those. And now, I am delighted to announce the launch of our most recent fundraiser for CF! 

Alongside a whole LOAD of things that we are doing at my work (we hold a raffle each Christmas, which this year will, clearly, be different - but amazingly bigger and better than before thanks to the most generous support I have from friends at Cambridge University Press) my new personal challenge is to walk... 

500km over the month of dark, cold, dreary January

This is some three to four hours a day on average (and I love to walk, but that far? Everyday? In the mud...?!). In addition to this, some very special people are creating their own challenges on the theme of 500 too (see website for more details, and if you’d like to get involved in fundraiser 500 with your own challenge, whatever that might be, please let me know, the more the better). 

I appreciate this year has been challenging, for us all, but if you can sponsor us even a little, and support the incredibly important work that the CF Trust do at this time when charitable donations have almost completely dried up, and the research cannot wait, please visit our fundraising site here. I will post updates along the way. 

HUGE thanks to Heather, Kay, Charlotte, Megan and Beth for organising all the fundraising at work, I could never do all this without you! Also to all the kids that are pledging their own 500 challenges. BIG love to you all x 

Have some lush Gerhard Richter ❤️

I was thinking earlier...

That if you could only include three statements on my gravestone (OK, I was walking in the rain, feeling ill, and listening to Graham Greenes’ ‘The end of the affair’ so this kind of thinking was very much in context, I am not normally so maudlin) that they might be; 

Loved her children obsessively. 

Knew how to party. 

Had THE best friends and family ever. 

Thank you Sarah ❤️

Recovery is hard, but Isaac is beginning to feel better. We had two emergency trips back to hospital on days one and two at home, and needed to have one stent removed (which may cause more scarring). He is also at the angry end of twelve days on steroids, and is still dosed up on pain relief, but each day is getting a little easier. The rest of us are germy and coughing, which I wouldn’t mention at all, only we worry that he will get this next too, and a virus would almost certainly set off a CF exacerbation, which he really doesn’t need. 

Lockdown #2. Sending love to you all x 

My mantra, now in neon lights! 

Operation

Isaac had the sinus surgery yesterday. In his surgeons words; imagine the sinuses like a house, with a kitchen, living room, bathroom, study.... by removing bone reaching as far as his eyebrows, Isaac’s house is now open plan. This doesn’t change the CF disease in his sinuses, but it’s opened everything up which means the drugs can do their work. 

The Dr said he will most likely still be treating Isaac until his own retirement (and he is not old) but the hope is that he will not need such a big operation again, if at all. The surgery went well (it was three hours... in which I paced around, ran to the toilets feeling sick, drank tea, paced some more, stared blankly at walls, couldn’t face calling anyone, soaked in the misery of a hospital amid pandemic...). He predicts that once recovered, Isaac will feel like a new person as it was worse than he expected inside, full of infection. He said the impact on his quality of life in these recent months would have been huge. 

In recovery, I found Isaac delirious, shaking so much that I had to hold him from falling out of bed, and scratching like crazy. 

The nurse called the anaesthetist back, who said it could be a reaction to the anaesthetic or the pain drugs, and prescribed IV antihistamines. These worked almost immediately. Only his pain score was rising (Isaac rarely complains, so when he says his pain is 9/10 I know it to be true), so they prescribed IV fentanyl, saying that if it was the fentanyl that he was reacting to, it would be fine now he had the IV antihistamines. Only, while the fentanyl sent him back off to cloud cuckoo land, I peaked at his chest and back, and he was covered again in a raised, angry red rash. It took a few hours to calm, and it’s one to add to his notes for future reference (although, they say they would not rule out using it again, as it’s such a powerful drug, but they can treat with antihistamines at the same time if they need to). 

Neither of us are squeamish, so the bleeding, which lasted maybe 12hours, was manageable, but while he was floating on the morphine high, blood was coming from his nose and mouth in clots. At times he would retch or cough and wasn’t even sure where the blood was coming from. On the plus side, he needed little oxygen post op, and his sats remained OK throughout (big positive for him). 

We got back to the ward a few hours later, where he rested, and we tried to remove the dried blood so he looked a little less like he’d been in a fight or accident. We had the option to stay, but his Dr was happy for us to go home and call if we needed anything, so we came home for the night, and he slept and slept. On crawling onto the sofa myself, the exhaustion hit me and I knew with absolute certainty that I when I woke up, I would be ill. Sure enough, I am full of cold, and so is Anouk. Now we must try and avoid passing this on to Isaac - it’s all he needs. 

He is now back at the hospital to be checked over, as he feels that one of the stents in his nose (which need to stay there for three weeks) has dropped down. Because I’m not well, I can’t be with him, which I hate. We just want him home now, warm, safe and recovering. For once, it’s not such a bad time to be locking down. 

Sorry, not the most cheerful of posts. I feel like someone mopped the floor with me. But under this snotty exterior, I am relieved as fuck and so, so glad. The surgery is done; Isaac can see! And yes, it’s going to take a few months to feel the full results, but he will, and this in turn will help his lungs. 

And I know I always bang on about this... but, that boy? He’s a fricken hero! ❤️

PS: and just to be clear, this was all micro surgery, up through his nose. So no scars, and no peeling his face off as someone had asked, thankfully! To see amazing surgery like this happen at our very own Addenbrookes and the Royal Papworth, including lung transplantation, I urge you to watch ‘Surgeons, the edge of life’ on the BBC (pictured below). Amazing stuff, for the non squeamish. 

Pictures from Addenbrookes, where he is treated now, and the Royal Papworth, where he will attend next year when he leaves paeds, which is now next door and the grounds of my pacing up and down. 

Surgery

Today, start pre-op antibiotics and steroids. Friday, pre-op assessment over the phone. Sunday, we all go into isolation to reduce COVID and other infection risks. Monday he has a COVID test. Monday/Tuesday, I try and juggle a busy work schedule, with three children who are not allowed out of the house, can have no visitors, in their half term holidays (they would have gladly taken isolation should it have been in school term!) who are high on early Halloween party treats that I have bribed them with. Wednesday, on the ward for 7.15am, hoping we get an early slot in theatre, but mostly that it all goes ahead (we been through pre-op nil-by-mouth before and had it cancelled at the last minute for emergency surgery coming from ED. Me anxious, and Isaac nil-by-mouth = not a great mix). Thursday, home, we hope, all going well. He is much safer here, so if we can get his pain under control, and the discharge drugs in time, we will run as soon as we can. After that, we spoil him and his sisters rotten. 

This is how I hope it all goes down, but I feel like I am walking down a corridor of poised spanners, just waiting to be thrown at us. His DIOS is not under control (he went to school today, but ran home and vomited immediately). This, or his chest infections could get worse before then, meaning they would not operate. Or COVID (cases are now rising rapidly in our area too), either for him, or that cases rise so much they delay planned ops. 

It’s such a weird feeling.... longing for a surgery that scares us, is very painful, and has a long recovery time with no guarantee that he won’t need it again? But then we see him with his beloved ice pack on his head, the nausea, the cyclical infections, and we know this needs to happen, and it needs to happen now. 

Hoping to write next on the other side of the Op. Keep well everyone, and take care x 

PS: Fundraising news coming very soon, watch this space! ❤️

Kandinsky ❤️ This one always makes me feel Christmassy. 

A date.

CT scan this week, surgery before the end of the month. Cannot tell you how pleased we are (I may have cried a little when they called). 

However, it’s an oxymoron; we know/he knows he needs this to feel better longer term, but at the same time it is risky, scary and painful surgery. It’s a relief to have a plan and no more delay, but something we also absolutely dread to our bones. For now, we keep our fingers crossed that the second CV peak does not prevent planned operations again, as that would undoubtedly be worse at this point. His lung health continues to be impacted by his sinus disease, and that’s not going to change until surgery resolves this cycle. 

Today I went back to work. At least, two days a week in the office (three days at home). I NEED this. I feel bouncier already - I appreciate that many are working from home and loving it; we all manage in different ways, but lockdown has only highlighted to me how much I need to be with people (possibly some of that is to distract myself from the day to day stress of CF). Anyway, it may be short lived; should the R number rise in this area I will stay home, but for now, I will take that monitor, that desk, that comfy chair, and seeing ACTUAL PEOPLE (in 3D!) and I’ll enjoy it while I can. The data on people with CF contracting COVID remains super positive - and he no longer needs to shield any more than we do. What a weird disease?! I think we’re only now beginning to gain any understanding of it. 

I am thinking of all that are struggling with the restricted pandemic life, it is not easy, and I know I am so much luckier than most to even have the options I do. 

Keep well x 

A weirdly empty office.

Back again.

DIOS came back with a vengeance. Another hospital visit, X-ray, more drugs. We have him home; more gastrograffin, and two other drugs to add to his usual regime, trying to ward off further incidents. He is in less pain already. Just when I thought he might manage a whole week at school, it ended up just being two days before the vomiting began again. Some good news, we have the CT scheduled for this week, after which, we can hope for a surgery date. Hopefully before damn CV prevents planned surgeries taking place again. Life feels like one big, chaotic mess. 

These lot keep me smiling. Take care, keep well x 

Isaac is doing much better.

Thank you for all the well wishes. 

Next steps... get the CT and surgery scheduled. I am on the case! x 

Bassin de lumieres ❤️ - still need to go. One day! 

Wishing I had better news

Bacterial chest infection. 

Fungal chest infection. 

Coughing. 

Sinus pain. 

Headaches. 

Vomiting. 

Headaches while coughing or vomiting.... the worst. 

... and now DIOS too (read more about what DIOS is here). And no, it is NOT the same as normal constipation. 

Isaac had an emergency review in hospital today. Abdomen X-ray. Bloods to test for pancreatitis (results pending). 

X-ray confirmed moderate to severe DIOS. They have allowed us one night to treat it at home with 100ml of Gastrograffin (possibly the worst tasting drug ever), lots of rest and fluids. If he is sick again, cannot consume enough fluids, or is still in as much pain, he will be admitted tomorrow for more treatment and IV fluids to help his poor kidneys. If he becomes in any more pain, we head to A&E (as there are risks of intestinal rupture, which really is as fun as it sounds). Isaac is being his usually stoic and wonderful self, laughing about how full of shit he really is (literally). DIOS is not new, he suffered a lot from this a few years back,  which is why we’re allowed to try and treat at home first, but it’s frustrating as we’d hoped it might be an issue of the past. 

Sinus, lungs, digestive system; all separate issues, but undoubtedly each issue impacts his health overall, and consequently, each other. 

We discussed his pain management more generally with his doctor, and agreed that what he ultimately needs is the surgery. They will continue to lean on the ENT team, and we will do the same. He needs the CT first, but they even have a backlog for those (damn COVID). 

We will get him well... but shit, it feels like one thing after another sometimes, and if anyone deserves a break, it is him. 

The next fundraiser plans are coming together... more soon on that x 

Do’in better

As bleak as things looked late last week, come Saturday night.... Isaac slept well, and wow, how sleep can heal. The many, many drugs are starting to do their magic, and our boy is starting to feel better. Hoping now to put off IVs for a little longer. 

Although more IV’s and surgery are pending still, we celebrate these little victories. Thank you all, for the love and support x x x

Clinical update (not the best).

Hey. 

Isaac is sadly no better. In fact today was pretty horrible; endless, violent coughing. 

He manages the odd day at school, but other days, the sinus headaches, the nausea, the vomiting, the coughing, and exhaustion from all of these, and bought about by both bacterial and fungal chest infections and the toxic treatments needed to fight these, all making his mornings pretty impossible. 

It is very hard to hear, to watch, to feel so helpless in the face of it, let alone to endure yourself, day in, day out. 

We do have new treatment options, and a fabulous team only a phone call away. We have back up antibiotics in stock at all times. This is so when he becomes more symptomatic we can quickly add in this third antibiotic (he already nebulises Colomycin and Tobramycin daily, and takes oral Azithromycin, which helps reduce the inflammation in his tired and angry lungs as part of his daily and ongoing treatment plan). We will now keep in stock a back up anti fungal, so we can jump on exacerbations quickly for both bacterial and fungal infections - since this aspergillus seems set to stay put. Bastard. 

For now, he is completing his back up antibiotic (Cipro) and a months course of the anti fungals (Posaconozole), and we’re monitoring his lung function and symptoms closely (lung function is down 10% further right now). After that, options include nebulised and IV anti fungals. The biggest downside being IV anti fungals are highly toxic, so must be done via infusion in hospital. If this becomes a standard treatment option, and he requires these IVs as often as he does IV antibiotics right now, we could be looking at four or more two-three week stays in hospital a year. That is pretty life changing, since we’ve managed the majority of his courses of IVs at home for years now, giving him a better quality of life. However wonderful the care is, long periods of time in hospital is no quality of life for anyone, not least for a child (the child is actually bigger than me now). 

We’re hoping the three remaining weeks on the anti fungals will help and he might get a break before his next IVs, but with his sinuses and pending surgery (and considering how bad he is today) that might be unlikely. His CF team are adding pressure on ENT to see if we can get him bumped up the waiting list. Should the pandemic mean they stop planned surgery again, this would be terrible, leaving him in this cycle of pain and infection. As always, Isaac’s standard response to people asking him how he is? ‘Not the best’. However bad he feels. How he copes I don’t know. 

My knee jerk reaction is to start planning my next fundraising (watch this space). Thank you in advance for your wonderful support. 

Hope I can deliver better news on my lovely boy soon x

Little festival Ise.

Strange looks from cows

Isaac needs more sinus surgery, and this time, it’s going to be an much bigger op than the last time (...think bone being chiselled away and a three month recovery time... sob). On finding this out, I walked Obie deep into the green fens and had a little scream, tore at the grass, and stomped my feet like a toddler. The cows stared but seemed quite used to this kind of behaviour. Who knows what else they witness? 

This is his third lot of sinus surgery in the last three years. The polyps which seem to love populating his poor sinuses are more common in CF patients, and he is one of the unlucky ones in this respect. We knew after the first surgery that they may come back. And after the second, that a bigger op would be required if they came back yet again. But this soon? His last op was just before lockdown. That sucks. 

Isaac is his usual stoic and heroic self. He knows that from now the headaches will only build and build, and daily life goes slowly from hard, to intolerable. But there is no choice to make, really. Only surgery can help now. 

His surgeon from the last two ops wants to do this himself, as he knows Isaac’s anatomy (scarring from previous ops included) but due to COVID, waiting lists are longer than ever, and planned surgery may go on hold again should there be another peak. We do not have the luxury of going private, as due to his CF, he is high risk for anaesthetic and post op infection, so needs his CF team to be there too - so we’ll likely need to fight for him to get up the list again. 

As with any surgery, they must outline the risks, and now Isaac is old enough to decide for himself, they described these to him: Death and the potential to go blind (since they operate so close to the eyes). Super. 

Imagine being 15 and taking all that in? But also knowing, you have no choice? 

It is like a dagger in my heart that my son has to face these things, again and again and again. And now, three days back into school he came home coughing like a trooper. Day four, he is off sick. COVID? CF exacerbation, despite his last IVs being in July? The vicious cycle of lungs and sinuses infecting each other does not help either. Can he not get a break? 

Everyday I wish I could take it all away from him. 

I’m frustrated, but also thinking of all my friends having a super tough time right now; grieving for loved ones, coping with cancer diagnoses, my fellow CF families, and my friends struggling with both physical and mental ill health in these tricky times x 

J

Walberswick, Suffolk UK. 

Thank you Sarah for a wonderful weekend away, love you loads ❤️

Compliance (or rather, non compliance)

I think it is mostly an Isaac thing. His character. His medical history. His age. Possibly it is somewhat a boy thing too? Speaking to other CF adults and parents, I believe teen boys tend to rebel against treatments more often than girls.

Is rebel the right word? No, he is not actively NOT doing treatments; It’s the INACTIVITY in which he does all the treatments that he ACTIVELY needs to do. He says he just doesn’t care. I reply that I can’t stop caring. Mostly we reach a compromise, but often, after a teary standoff. 

Each day, I set out his days worth of physio tools, tablets, inhalers, nasal sprays and nebulisers. I can say with confidence that on the most part, he begins each of these treatments. Not always on time (e.g. some doses should be 12 hours apart, but when you sleep most of the day, it’s difficult to get the timing right). But he does them. But NOT with the motivation that they require; physio requires him to actively breathe through a tool, huff at intervals, and spit out sputum. When he argues that he’s done everything that has been set out for him, it’s very hard to kind of agree, but then argue that he need to do them BETTER. 

How can I prove or argue that he’s not taking the nebulised drugs deep enough into his lungs to kill the bugs that have colonised in his lungs? When I can’t see that? How can he prove that he’s doing blows with physio with the gusto they need to help shift the infected mucous in his lungs, when he isn’t always able to cough it up anyway? It’s all subjective. Only my love, is obsessive. 

He is facing likely sinus surgery again soon, for the third time. And this time it will likely be a bigger operation than the last two. This is probably inevitable, but not doing the recommended sinus rinses won’t have helped (he refuses these most of the time). Today, Anouk helped me fill his weekly tablet folder, she likes to do this to help me, while Isaac never has. I asked her how she thought life would be, if it were her, rather than him. Her simple reply was ‘I would do everything to stay well’. But the point is, we will never know. These are his shoes. How could we ever know how we would be, wearing them? 

I’m not coping super well with semi-lockdown life right now. Too much time to think. To worry. In a funk. I miss people too much. So bad. Too much time at home, and for Isaac and I, a fair proportion of that time is made up with asking him, pleading with him, to do his treatments. Being able to test his lung function at home, for the first time, is incredible. The fact that in the last six months, we have visited the hospital just twice; unprecedented! We feel safe with the amount of monitoring he has, and reassured by the access to his team that we are always subject to. We can sleep longer, without the need to be out of the house by a certain time (mornings are always the hardest part of his day). We laugh, we dance, we see friends (on a limited basis) when we can. So why does this feel so hard? 

Next summer, he will transition to adult care, at the Royal Papworth Hospital, and then, more than ever, the emphasis will be on him. HIS wanting and needing to stay well. Is he ready for that? He has no idea what he wants to do after GCSE’s. Does he have any idea of how active he will be in keeping himself well? Am I ready for that? After 16 years of his health being my priority? Watching him take the reins, and make his own treatment choices?

We’re returning to face to face clinic this week and next (CF and ENT). I suspect this might be short-lived, once we hit another peak. But for now, we’ll take it - we think he’s doing OK, but a face to face chat from the professionals might help him take things a bit more seriously. As a parent, it sucks to be the messenger of endless rules, bad news and health woes, when really we’d rather spend the time just lovin him. 

My plan is to complete my port-access training now, so we can avoid hospital more, and help the community nurses out, who currently come monthly to flush his port for us. It is one thing to administer the IV drugs through his port at home, and even to remove the needle following the two/three week course on antibiotics (which I have been doing for years now), but it is something else to use your fingers to anchor an invisible lump below skin, and plunge an inch long needle into a very small, scar covered spot on your sons chest, hoping you make it in first time... EEK! Wish me luck.

Keep safe people x 

Summer by the river, and random tile love ❤️

Look at all the peaches

Tonight is all about the music. Listen.

All is well. Happy summer all x 

Recent pictures from Paris, Dunwich, and home with the handsome Obie ❤️