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 ❤️

Tortoises

Isaac finished IVs today, feeling so much better. The last couple of weeks have been really rocky; firstly coughing SO much more, then so exhausted from the double treatment of anti-fungals and IV antibiotics at the same time. Then feeling better, but followed up with a couple of days of nausea, chills, and sickness. He has bounced back miraculously and his lung function today was good. Hopefully he is set up now for a summer of good health. He really deserves a good break.

If you would like to support the amazing work the CF Trust do in funding research into life changing treatments AND have a chance of winning money back, please enter this online raffle - it really only takes two minutes. Thanks so much for reading, and happy summer all. We don’t have many plans, since we thought we would still be shielding, but now feel like tortoises, slowly poking out our heads to see what is what. There is so much fun to be had. Life is beautiful x 

Gerhard Richter, one of my favourites. 

PPE, exacerbations, and the NHS

Yesterday Isaac had an emergency admission to hospital. He is sick, cough, sick, cough, sick, sick now. My poor boy. 

We were met at the hospital doors by a nurse and escorted the back way to lung function labs. After that, we were led to a triage COVID Ward. We know our way around the hospital more than most, but this is a precaution due to the pandemic. Last week, when we came in for just an X-ray, we were offered a security escort, had we not known the way. It is so well controlled. 

We never thought it was COVID, but with any respiratory illness, they have to assume it could be. On the COVID triage ward, we sat in a sealed room. Everyone we saw had to wear full PPE and discard it as they left the room, sometimes the same people, changing multiple times, each and every time they leave and come back in the room. Forget one thing for his port access? De-gown/glove, and put a whole new set on just to collect what you missed. It must be exhausting. 

We were both tested for COVID (me, only as I was with him). Isaac had bloods, and sputum was already in the lab (we now know he’s growing aspergillus again, so a fungal infection on top of his live in PseudomonasA bacterial infection). To cover all bases and to avoid coming into hospital again soon (despite all the amazing measures they take, and really, all they do to protect the spread of the virus is frickin spectacular) they decided to start the full shebang immediately; two weeks of IV antibiotics AND anti-fungals. This is perhaps more than they would usually do at this stage (at this point, we were still pending the blood results, so it was a good guess), but it helps us avoid going back in for a second assessment, tests... and further exposure. So all good with us. 

Isaac has had a very hard and sleepless week, but we’re hoping that this plan will have him bouncing back very soon. I have to acknowledge the irony that as soon as we are told we can unshield, and he can return to school, that this latest exacerbation has kicked off. Just bad timing. He started lockdown before us (due to sinus surgery and an exacerbation) and now coming out of the most stringent rules, he hits another CF bump. Anyway, we’re getting back into the rhythm of home IVs, and he is sleeping lots to regain his energy. 

We are so, so thankful; to be home rather than on the ward; to our fantastic CF team for enabling this; for our community nurse team that come for his bloods every few days; and for our amazing NHS as a whole, for all that they do, especially at this difficult time x 

Trikafta/Kaftrio tears and X-rays

This week has been an emotional one. 

 

Four years of campaigning, protesting outside Westminster and writing to MPs about the immense and immediate need for these new small molecule drugs (that treat the underlying cause of CF, rather than only its symptoms, as Isaac does) that have been licensed but remained unavailable to patients across the UK, due to COST. From Kalydeco, Orkambi, Symkevi and now Trikafta (the UK brand will be called Kaftrio) - yesterday a deal for the whole portfolio of drugs has been agreed with NHS England. 

This will prolong lives; this will improve the quality of lives; and for young children with CF, this should prevent the lung scarring, colonised infection and chronic inflammation the CF brings about. 

I am so proud of the CF community and the CF Trust that have helped bring this about. There is no doubt that the campaign and publicity have driven this decision in a way that the government and NHS England have not. 

So many happy tears for those who can now start this amazing treatment. But also tears for those that this drug comes too late for. There are too many. 

For us in that last 10% that these drugs will not work for (I explain about this here) we hope not to be forgotten, and that these breakthroughs in medical research will open up new avenues for treatments that will work for the minority too. When Isaac was born, we were told that gene therapy was our best hope, but that it may come too late to help him. Still waiting on that! That these treatments have come to fruition at all gives us incredible hope for a better future. 

Isaac is not well right now. Chest full of treacle. Hoping oral antibiotics may help, if not, will need to kick off IVs shortly. Tomorrow we head into hospital for the first time since lockdown began (which is an amazing stretch for him!). Mask up. Drop off sputum. X-ray, and get out of there. Follow up by telephone. So thankful for our incredible CF team that have enabled us to stay safe at home so long x 

Local art. Life is beautiful. 

Being well

To support the incredible work that the CF Trust do, both in terms of funding ground breaking medical research, and supporting people with CF, and for a chance to win some cash, please enter this raffle - you can play online, and it takes two minutes, really. Top prize is £2,000. 

It has been a long time since Isaac has been so well, for so long...

Although his cough is now increasing and his huff is fruity, his last IVs were in March, so this is a very good run for him. Our community nurses have told us they see the same with many of their patients - shielding from CV is naturally also protecting him from the usual barrage of other viral bugs (which make his bacterial infections think they can have a pretty rave in his lungs). Day by day, his lungs are sounding worse though, meaning we will need to brave hospital soon, but we have escaped the higher risk period, and for that, we are super thankful. 

On his first day trip out of the house: Norfolk, June 2020. Despite being sick on the way down, he still managed to piggy back little Rosa on the long walk to the beach, and give his shoes up for Anouk, who had blisters. The fact that he soldiers on and never complains constantly amazes me 🥰

Happy 8th birthday our little Rosa 🌹 

Keep well x 

What Cystic Fibrosis really looks like

He will awake (late).... groggy. His sinus infections (for which he has already had surgery for twice) can fill his head with a pressure that never leaves him. Just getting out of bed begins to shift the sticky mucous in his chest, and the coughing begins. 

With the coughing fits come retching, and often, some pretty spectacular vomiting. The mucous he coughs up may be blood stained (from inflammation in his poorly lungs). 

His digestive system (also clogged with mucous and reliant on synthetic enzymes to help break down all fats and proteins) causes more pain, nausea. Breakfast may be a delightful cocktail of anti-nausea drugs and pain killers (if he can keep them down). Curling up in a ball helps until it eases, but treatments need to be done. Many toilet visits. 

Walking, talking, laughing, anything can bring on the coughing fits. Despite the nausea, he must swallow down the dozens of pills he needs, many of which are simply drugs to offset the side effects of other drugs he takes. Inhalers and nasal sprays are taken. 

Then nebulisers; the first is hypertonic saline, the ‘salty neb’ - this helps draw water into his lungs to loosen the mucous (and causes yet more coughing). This is interspersed with breathing techniques through a physio device (to help him shift the mucous and cough it up). Can you imagine coughing tons, but having to do treatments that actively bring about more coughing? When your head, throat and ribs ache already? Rounds of deep breaths and huffs, followed by a ton of coughing (and more retching, and possibly more throwing up). Sputum sometimes patterned with thick plugs of mucous. The darker the mucous; the more evil the infection. But better out than in. 

After that, he needs to rain antibiotics on his now clearer lungs, with a second nebuliser. Trying his best to breathe this down into the deep crevices of his poor angry lungs. A third nebuliser is an enzyme that works to break down and thin the mucous over the day, making it easier to clear later. This will all take an hour or more, and it might be hours before he can eat anything. 

During the day, exercise will bring on more coughing, and fatigue may mean he won’t want to, but exercise is so important, to help keep his lungs clear, that he must try. As the day goes on, the cough will likely settle some. With any luck, he may manage a chuckle without fear of it bringing on another coughing fit. The audible sound from his chest is like breathing through treacle, it rattles, crackles. He will feel exhausted all day; breathing like this consumes a huge amount of energy, and his weight plummets with each exacerbation. 

The day may also be filled with IV antibiotics. In his case, roughly every 8-12 weeks; Ceftazadine and Tobi, with a side of NAC to help protect his poor and battered liver and kidneys. And dozens of Creon capsules with everything he eats.  

Come evening, he needs to repeat the same morning routine; physio, nebs, meds. 365 days a year. There is no holiday from CF. And the nights can be the worst, endless coughing, needing to do physio in the night to help clear his lungs. Exhausted is not a strong enough word for this. We become wordless, numbly doing anything we can to help him.

When we can, we manage this all at home, rather than in hospital (I am trained to give him IV drugs myself, not all families are able). Some years are worse than others, with many admissions. Some admissions requiring oxygen supplementation, surgery, HDU, ICU. And there is never a time that we are not awaiting test results of some kind; lung function, sputum results from the lab, chest X-rays, CT or MRI scans, blood sugar tests, ultrasounds, bloods for infection markers, liver and kidney health, care for his surgically implanted intravenous device in his chest (portacath). Appointments not only with his core CF team, but with ENT, Gastro and Immunology...

What I describe above is what a typical ‘CF sucks day’ can be like for Isaac. We are very, very fortunate that inbetween exacerbations, he is currently able to bounce back to a level of lung function that means he can still play football, gain back the weight, run and swim, so it is not always like this, for us. The relentless treatment regime keeps him well enough that he can lead a near normal life, but only until the infections become rife again in his lungs, that he becomes more symptomatic and we climb back on the IV train (for two to three weeks at a time), hoping each and everytime that we can manage this at home and not need to be admitted to hospital (more so now than ever). But also knowing that each and every exacerbation can chip away at his lung function that bit more, maybe permanently, and that is scary. Every new infection could be the one he doesn’t bounce back from. 

For many others with CF, they get no bounce back, no respite. This is their everyday. Unable to walk up the stairs without O2, or laugh without the coughing taking over...

I am not telling you this for sympathy, I am writing about this in anger, as right now, there is a drug, manufactured and sitting in big pharma warehouses that is proving to be very, very effective at improving the health, lung function and quality of life for the 90% of people with CF that this will work for (not Isaac unfortunately, due to his rare mutation) and yet it is STILL not getting to those who so badly need it in the UK. Because of DELAYED DECISIONS, and ultimately, COST. 

This drug is amazing, and the delay must end.

“Trikafta changed my life. Since I started taking it in November 2019 my Pulmonary Function Tests have improved by more than 40%. I can exercise at the gym 5 times weekly, do daily activities with more energy and efficiency than ever before, and I’m about to finish a university degree. I have hope for the future, for the first time I dream about what it’s like to live to be middle aged, I can breathe like I have never been able to in my life. Every cystic fibrosis patient deserves to know what it feels like to take a deep breath.”

To support the campaign to get this drug into the hands of people who need it NOW, please click here. Alternatively, copy and edit the below text and send it to your local MP. Every day people are living through and dying of end-stage cystic fibrosis. I have no doubt that this drug will be approved, they will agree a deal, it will be licensed, it will get to patients, but every day they drag their heels, it saves money and lives are lost in the process.  

Thanks so much for reading x 

Letter for MP’s: 

Dear X 

 

I am a constituent of yours who is a parent of a child affected by cystic fibrosis (CF). I’m writing to share my experience of cystic fibrosis and the desperate need for urgent access to life-saving drugs. A new CF treatment, the triple combination therapy, could treat up to 90% of those living with the condition – including 40% who currently do not have the eligible mutations for the existing drugs, Orkambi and Kalydeco and Symkevi. 

 

This drug, known as Trikafta in the US, has already been licensed by the Food and Drug Administration and shown to make a marked difference to the lives of those with CF.

 

Currently, the drug is being assessed by the European Medicines Agency (EMA), where it needs to receive a licence before it is appraised in the UK. The NICE appraisal process had begun alongside this, but disappointingly there has been a delay, and it has been pushed back to January 2021. Appraisal bodies in the devolved nations have yet to announce a date for their appraisal of the drug.

 

CF is a genetic life-threatening condition that affects over 10,500 people in the UK. The median age at death is just 31 years old. 

 

My own son, Isaac, is unfortunately in the 10% of people with CF who these drugs will not work for, due to his rare, severe mutation, but these amazing new drugs give us much hope for a better future for him. We have already lost too many friends from this cruel disease, please support us in this fight to give him, and his friends a chance of a future. 

 

The newer triple combination therapy has the potential to be even more effective for those who can’t benefit from existing drugs. While conventional CF treatments treat the symptoms of the condition, these new drugs target the underlying cause. It is not a cure, but it has been shown to significantly reduce decline in lung function – the leading cause of death for someone with CF.

 

The pandemic has caused disruption to usual CF care, whichrisks irreparable lung damage. This makes access to this drug even more important at this time.

 

We have already seen a lengthy campaign for Orkambi and Symkevi and we cannot afford to see this repeated when it comes to the triple therapy. The charity Cystic Fibrosis Trust is urgently calling on all parties to work together to make sure that the triple therapy is made available in the shortest possible time to those who need it, and I ask you to lend your support to this.  For more information please contactpublicaffairsteam@cysticfibrosis.org.uk for a full briefing.

 

Yours sincerely, Dah dah dah... 

Restlessness

Hey. I haven’t posted much of late. I think it’s because, ultimately this is just too big for my little head. Too much to think about. Too much to despise (Trump, BoJo, Cummings...!). Too many damn unknowns...

Isaac is physically well right now. Chest sounds good, eating well, tummy not so great, but little nausea. But on week 11+5 having not left the house a single time, he is understandably frustrated, and that shows. He is 15, cooped up at home with me and his sisters 24/7. On his own terms, he wants to sleep all day, and play online with friends all night. On my terms, he needs to meet me half way... get up by noon, and share some kind of resemblance of a day with the rest of us - and get his treatments done at a time that I can properly supervise them.

We had CF clinic (by phone) again Thursday, and were told that for the first time, he could actually leave the house!! The data on people with CF having C19 is (quite unbelievably)..... positive. In one international study, 44 patients positive, none under 18, only one needed ICU care, and all survived - it’s way to early to speculate why this group (with already massively compromised lungs) might be doing so well, but ideas include; they are more closely monitored and will seek help sooner; that they are used to airway clearance techniques; that they are on antibiotics daily anyway, giving them some protection from secondary bacterial infections; genetic reasons; but also that people with CF are also - well.....mostly young. 

On hearing that he could go out, his eyes widened with joy, until we repeated what his consultant had told us - he was only to go out with those is his household, and must not meet up with others. He is gutted. Seeing a group of his friends (or, friends of friends at least) down the river today, flaunting the rules, drinking beers, I didn’t feel annoyed, just kinda sad - Isaac’s options are so much more limited. Again. And again. 

Isaac’s CF team also gave us the go ahead to ‘unshield’ the rest of the family, with extra care. This means that as well as Dan being back at work, Anouk (in Y6) will return to school in June. She will need to strip off clothes for me to wash when she gets in, and shower. She is a smart kid and she gets it.

We continue to have weekly priority slots at a supermarket which covers most of our needs (but 80 items a week, for a family of 5 is soon eaten up!) so family have kindly topped us up. Access to Isaacs medications has so far not been affected (but devastatingly, I know medical funding/research to find a treatment/cure for his mutation will have been upended by this pandemic too). We are hoping that we can avoid hospital until July - when he is due his first appointment to review his (possible) scoliosis. If we must go in for this (as it will require X-rays) we’ll hope to combine it with lung function at the same time - the one thing we cannot as yet monitor at home. 

For all my restlessness and sharing Isaac’s frustrations (not to mention bearing the brunt of them) I appreciate how lucky we are x 

Banksy and us. 

Three thousand flips!

Anouk and Ellie both completed their challenge of 1,000 front flips each on the trampoline. To support their fundraiser, the handsome Sonny Fountain joined the challenge from his own garden, doing some frickin impressive back flips! 

Our first lockdown fundraiser! They have raised more than £1,200 for the CF Trust, at this critical time when charitable donations have dried up due to cancelled events. Thank you so much for all the support, our amazing, beautiful, generous family and friends. It means the world to us. 

Couldn’t be prouder of this special girl, and Rosa didn’t want to be left out, so did 1,000 seat drops at the same time! 

You can still sponsor them here if you’d like to x 

Anouk and Ellie are fundraising

Due to the pandemic, charitable fundraising for the CF Trust has almost completely dried up. At the same time, the Trust is funding vital research and supporting CF families in isolation with well being grants. Now, more than ever, we need to raise funds.

On May 9th (or soon after, if the weather is bad) Anouk will be taking on the challenge of completing 1000 front flips on the trampoline in a day. Anouk is just 11, and has a heart of gold - She would love it if people can support her in this..... Her hope is to raise £500. Further to this, my gorgeous niece, Ellie Cayley, will do the same from her garden a village away! 

To donate, click here.

Thank you so much. Keep on keeping well x 

Little Anouk and Ellie. Cousins and best friends. 

NHS love

Our home from home, up in lights. Special thanks to our fantastic paediatric CF team, who have been there for us, every step of the way, and continue to keep our boy well from afar x 

Addenbrookes Hospital, Cambridge, UK. 

Photo credits; A Cambridge Diary.