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.

Week 6

At least, I think it’s six weeks since we started isolation? I lose count. 

We are all doing OK. We now have priority access to supermarket deliveries from the government sharing the extra vulnerable shielders lists, we can now start to repay the kind neighbours who have helped us before now. We have had no issues with drug supplies yet (even had the local pharmacist deliver to our door so we can avoid pick up in person). And best of all, Isaac is well. He finished the anti-fungal treatment with no issues with his liver function (but with the dry lips again, but not bleeding like last time). The community nurses came the other day for bloods and post flush, and it felt really odd to have someone else in the house again. 

I’m so thankful that the kids are coping so well with lockdown. No, they don’t study all day, but they create, they write, they get energetic, they play with each other, they redesign their rooms, and best of all, they seem happy. For me, I am more restless. I want, and I miss. And for now, I’m just trying to park that whole question about when this might end, not so much for us, but for Isaac, and all those others more vulnerable. Sending out the biggest virtual hugs to you all. And thank you to everyone who has messaged to ask how we are doing, never ever get sick of all that kindness. Stay well x 

Never felt so lucky to have a garden (and our now completed new pond) and lakes nearby for dog walks; I hope these pictures cheer you, rather than frustrate if you can’t do the same!

 

Who gets treated?

I’ve just seen a CF Foundation (US) update that tells viewers that they are having to urgently advise doctors that CF lives ARE now worth saving, that things have changed, that having CF is no longer a CERTAIN death sentence. It seems that doctors are being forced to choose who to treat, and those with genetic or chronic illnesses may be bumped off a list. I have no words. Sorry, this is a really crappy blog update. But it’s very real. 

Oh, my Obie love! 

Drowning in ‘what if’s’

I can very easily drive myself crazy with ‘WHAT IF’S’.

My endlessly whirring and annoying mind. 

I am forever looking for ways to try and quieten them (...alcohol... sleep?) or shun them (... it is not going to happen head in sand approach, or the fact finding research, choosing only to read the studies which look to disapprove my worries). 

But never more than now are the what if’s trying to escape, run riot, shouting and screaming at me (the little shits). 

And I’m not only worried about Isaac (and others) contracting the virus.

Reading about the exit strategy for Corona Virus lockdown, it seems very likely to be a staged approach, with different categories of potential release based on; a) if you have COVID-19 antibodies b) age group c) geographical location and d) your status in terms of vulnerability. 

Makes sense, it’s never going to go from all to nothing without a sure fire second peak (anything like herd immunity will still be a way off). So, maybe the young, and those in rural areas getting first release? Or schools only (a model I saw today supposes that the school closures only reduce mortality rates by 2-4%)? And those who have already had the bug getting back to work sooner (this assumes immunity lasts sometime, which we don’t yet know)? But in all of the scenarios I read about, one thing remains the same; those on the extremely vulnerable list will be in lockdown for longer - and reports vary on this from being weeks, months, possibly a year... even, until the vaccine is available (likely, autumn 21)?

So, could we be asking Isaac to stay home, shielded for up to 18 months? 

What might the psychological impact of that be, on a fifteen year old? The impact to his physical and mental health? His education? His social skills? His relationships? His sports? His whole life? I can’t even begin the fathom this, and to be honest, I just don’t want to. I know this is worst case scenario, but that seems kind of fitting for 2020 so far. 

Sorry, feeling a bit... depleted. We are all well, and have everything we physically need. Isaac started another round of anti-fungal treatment for the aspergillus growing in his lungs, his fantastic CF team are ensuring we have a supply of all we need. We have community nurses coming over this week for his monthly port flush as normal. We have fish in our new pond, and have been enjoying the sunshine, camping in the garden with the kids. Lots to be grateful for too x 

Home

Sorry, it’s been a while... it has taken this long to get over this last bug. It was evil 😈 First little Rosa bean, then Dan, Anouk, and finally me (it’s a pretty strange reality for us where Isaac is the most well in the house!). 

For Dan and I it was mainly a cough. For the girls, they also had fevers..... So, COVID-19? Who knows. The cough was the opposite of dry, and as any parent knows, kids get fevers all the time with a virus. We did speak to our GP, as Anouk really wasn’t bouncing back. We thought he might consider antibiotics (since it sounded like it had settled on her chest) but he explained that we may have had CV, and that we have to assume we have, so isolation is our only option. We are not convinced. In the UK, they are only testing inpatients in hospital right now, despite the ‘test, test, test’ news we hear. So we may never know (unless we can later have an antibody test).

It’s too early to guess if Isaac has escaped this infection or not (whatever it is) - we can only hope our efforts have been enough, as either way its not the kind of bug he would sail through (without intervention, and he’s so recently had *IV’s anyway) and we want to avoid hospital like the plague. As much as we clean, sterilise equipment, eat apart, keep his tooth brush and towels separate and omit hugs (and I really miss hugging my son, guys) it never quite feels enough. 

In an odd way, the world is experiencing something huge, but part of this new reality for most has been part of ours for so long now.... We are used to alcohol wipes, plastic aprons, gloves and masks. We have been in hospital under barrier nursing for infection control too many times to count. We are accustomed to all those funny looks that people give you when you take a big detour around other people who look unwell, or the opposite, when he is having a very public coughing fit and people scowl at us, assuming he is contagious and should be home (the irony that we endure both of these scenarios is not lost on us). We live with the the eye rolls when people assume we are overreacting to infection risks (seriously, he really should not ever go in a jacuzzi). Perhaps, after all of this, there may be just a tad more understanding in the world, for families like ours? On the flip side, we are so touched by the number of people who have contacted us to offer help and ask how Isaac is at this time. Post Corona virus, will we all be a little more accustomed to (and understanding of) the anxiety that those vulnerable in our society live with each and every day? 

Isaac and I had a good chat the other day about what the virus might mean for him (he is 15, so when I say good, I mean around 6 minutes, and these kind of chats are always better if they are instigated by him, rather than me). Knowing that he has letters coming from the government, putting him on the ‘extremely vulnerable’ list is understandably alarming, but as I said to him, these lists are vast and don’t take into account the individual; so if Isaac does catch it, and we end up in hospital? Well, that is our area of expertise, and we have a first class pass to the best team ever. We would have ended on a high five, were it not for the social distancing. 

In terms of Isaac’s care, we now only have phone appointments with his CF team. We cannot send in sputum for testing in the lab as we would normally (instead, we will need someone to take it to hospital for us, where the team will pick it up from the car at the entrance). We have good access to his medications so far; he is about to start anti-fungals (as we’re coming to the end of his course of extra oral antibiotics, and he does still have fairly fruity cough) and these are being sent by post. The CF Trust have sent us a letter that we can use to explain his status, as well as the government issued letter listing him as extremely vulnerable. We have been able to register with GOV.UK to say that we are isolating and may not be able to get the supplies we need. That was a few days ago, but we’ve not heard back since. Thankfully, we have been able to get everything we need, thanks to the kindness of our family, neighbours and friends - thank you so much all. 

I don’t know about you, but in one way, everyday feels the same, like badly smudged together memories. My appetite for the news is huge, but I’ve stopped taking in information. Everything feels too BIG suddenly. Add to this, the dreaded home schooling (we’ve been too ill to fully get on top of this so far, and you know what? I don’t care. They are learning in experience, through endless games, reading and art, and for now, that’s just fine. They are anxious too, and need some downtime) and working full time, days feel weirdly full and yet... at times empty. Overwhelmed yet lonely.

There are, and there always must be, some positives: Both Obie and Rosa LOVE being home and having us all together. Our garden has never looked better. We have dug and filled a pond (plants and snails have arrived, fish to follow... a great little project for all the family). We are doing well keeping active, projects are planned, we have all of this support around us, and most of all..... Isaac is well. He is well! 

We are thinking of all those unwell, those isolating alone, those without their needs being met (physically or mentally), those unable to contact their loved ones, those watching their businesses go down the drain... and to our key workers, putting themselves (or those they love) at risk for others, thank you so much. Stay safe and keep in touch x 

* When we say IVs, I mean antibiotics given intravenously. These treat bacterial infections, not viral ones. However, as people with CF live with bacterial infections in the their lungs all the time (in progressed disease at least, less so infants with CF) a viral infection allows the live-in bacteria to have a little party in the lungs too, which can be far more problematic long term. On the whole, viral infections are acute issues which reek temporary havoc, but it’s the bacterial after party that is more problematic, hence the antibiotic treatment, even if the acute part might be viral**. The exception here is that COVID 19 is a virus that may be as dangerous as the bacterial infections people with CF face anyway. 

** I am no doctor, but this is my understanding! Always seek sensible medical advice, not the wittering of a slightly mad CF parent with more than a whiff of gin. 

We had to miss the funeral of a very kind and talented friend. Here is the painting he did of our Obie - fly high Gordon. We will miss you x 

Pond under construction. 

Burning lungs and CorinaKindness part 2

My lungs burn. 

Every breath feels like fire. 

My throat tickles again, and again, and again. 

My cough heaves violently against my throat. 

The tissues endlessly being thrown in the bin. 

We clean EVERYTHING. 

We all have this, but Isaac (we think, he is coughing, but CF coughing, not, bug from hell coughing). 

On the plus side, it’s really mucousy, so we’re pretty confident we can discount CV. 

On the bad side?

It’s really frickin mucousy. 

Also not good for Isaac to be around. 

For now, he is on extra oral antibiotics, and is doing OK. 

But essentially we’re trapping him in a house with a bunch of people with disgusting coughs. 

CoronaKindness: Last week we were lucky enough to get one of the last grocery delivery slots for weeks, the driver called first and we explained that we are in isolation not because we are sick (although ironically, we are), but to protect a family member with a lung condition, so we will keep a distance. The guy kindly bagged up all our shopping like an expert, put it into our alleyway, knocking to let me know when he was done, so we didn’t need to have any contact with him. He also told us that next time, they can pack our shopping for us in store, in a controlled environment to further limit the risks. The next day, we received a parcel of goodies from a friend in NZ (thank you Vanessa!). The next, we had a note through the door from a stranger, offering to collect whatever we needed. Our family have us well stocked with food. Friends have shared home schooling resources. We feel like the sad but fortunate isolates; lucky to have so many thinking of us, but missing them all the more. 

In a way, feeling sick right now makes isolation easier... we need rest anyway. It’s going to get a whole lot tougher. Thank you to everyone who is taking social distancing and isolation so seriously, keeping those more vulnerable in our society protected x 

Peter Lanyon ❤️

CoronaKindness

In the last week we have been at the receiving end of all of this: 

• The many neighbours, sending offers of any help they can offer. 
• My dear Mum, dropping off groceries and jigsaws in our side gate, for me to pick up after they have decontaminated for a few hours. Tearful that she can’t help more (she already does more than enough).
• Friends from work, buying and sending me hand sanitisers, soaps and dettol, knowing I had trouble getting hold of these. Along with treats to keep us going! 
• My cousin, sending me the medical wipes we need to clean his medical equipment. Her friend, who doesn’t know us, but went out of her way to drop these off on her way back to London. 
• My brother, who is getting me more. 
• The friend who thoughtfully gave Isaac a PlayStation voucher, to help keep him entertained.
• My sister, printing off work for the kids and offering online lessons. 
• The pharmacist, who took the trouble to order Isaac a months extra supply, so we can worry less about the supply chain breaking down, for now. 

Heartwarming doesn’t quite cover this.... this is family, community... unsolicited kindness. Thank you so much. 

Isaac is not well today, his cough is back with a vengeance, his sputum, dark. We are in touch with his team about next steps. So soon after his last IVs, we imagine the next step will be Cipro, potentially with a side of anti-fungals. Will update soon.

Stay safe, keep talking x 

Man Ray. I’m sure I’ve shared this many times before, but I love it, so have it again.

Social distancing

These are the kind of things you could consider doing now, to help protect the more vulnerable around you.

For us, we are taking life in isolation day by day. Hugs x 

Isolation

This is feeling very fucking real now. 

We are all now in full isolation. There is a confirmed case linked to Isaac’s school. Before that, we had decided to keep Rosa and Anouk home anyway, but it’s making more sense by the hour. Dan worked today, but we’re both feeling ill - most likely with the bug Rosa had last week, and Anouk has now. Nothing more. But since it’s a chesty one, we’ll all be home tomorrow. 

Today I had five meetings on skype. During one, Rosa appeared in the room dressed as an Indian with a curly blonde wig on. By the last, the kids were all fed up and asking me to mute myself to ask questions like ‘I’m bored, what can we do?’ or ‘since the cookers not working, can we have crisps for tea?’. Our standards are already slipping, last night I saw Isaac make himself a bowl of pasta with sauce, but then add squished up crisps on top, as if they were croutons. My heads hurts. I can’t keep up with the work I need to do. And everything single thing the kids want to eat, we have run out of (we still have food, they just naturally want what they can’t have). Each day I drive to a remote lane and walk Obie by the lakes. My only alone time. 

And this is day three.... they are talking about people with underlying health issues self isolating for 12 weeks?! 

CF clinic are only offering telephone appointments, unless you have significant new symptoms. Isaac’s ENT surgeon called me today to see how he was recovering (really well now, thankfully) and told me what we already suspected; had he not had the surgery when he did, he would be waiting a long, long time before planned admissions would go ahead. We are so thankful for that, and for all the kind messages of support from friends, family and community. We have care packages of antibacterial wipes and hand sanitiser on their way. At the same time, I’m frustrated I can’t do more myself for our parents and other vulnerable people around us, who if not yet, will soon be isolated too. 

After being glued to the news for days, I am suddenly exhausted and have information overload. I crave fiction. The kids are coping much better than me, but for how long? This is going to be tough for everyone. Stay well people x 

COVID-19 isolation; strange times

We have been told now to keep Isaac at home indefinitely due to the risk of Corona virus. 

No great surprise; we ended his two weeks of IVs today with his lung function slightly down on when we started (I’m gutted), he is less symptomatic, but there is still a crackle/wheeze to his huff. Not great news - but his team are keen he has a break from IVs and see what happens as he continues to recover from his recent op. 

What we don’t know is what precautions we need to take as a family to protect him. I can work from home (just about) but Dan is a self-employed builder (with a lower risk though; small team, and mostly outside), so does he carry on working? Worse, if schools do not yet close, do we keep Anouk and Rosa home sooner? Do we allow visitors? Do we shop? 

We get individually wrapped Clinel wipes for Isaac’s sterile port/IV care supplied by the hospital, but I also purchase packs of Clinel general wipes (that are frequently used on hospital wards) myself. Clinic don’t usually supply these to us at home, but to clean non-sterile (but still needs to be super clean) apparatus, they are ideal. I use these to clean his nebulisers, the table before starting to prep IVs, and to move from room to room with his IV drugs in hand (I am apron/gloved up, but I need to open door handles etc. to get to his room). Anyway, they come in big packs which last us a month or so, and rather than ask the hospital for these, I simply order them online for around £5 a packet. Today, three packets are advertised on amazon for £375. 

Amid other news that hand sanitiser gels have been stolen from hospital wards, putting those most at risk, at more risk, I am weirdly reminded of the kind of post-apocalyptic/zombie TV series where humans all turn on each other (Dan adores these - I strongly suspect he may have buried emergency supplies in the garden, such is his obsession). 

Someone told me today that he expects police/military on the streets guarding against looting within a month; another thinks it will have blown over by then. I’m not so sure.... I read about some CF families keeping the whole family home and not allowing any visitors, and then in the next breath, those who have been reassured that this will likely not be much more than a cold, so are carrying on as normal. I get the data. I see between the headlines. I know where to find impartial facts (I think).... and.... I have also seen Isaac have a flu virus before that took him from being OK, having IV antibiotics on the ward, to very suddenly being in intensive care on *Optiflow ventilation, reliant on this for high flow oxygen, in a wheelchair for many weeks, and a full recovery which took many months - not fun. 

I think it’s entirely appropriate to have a plan for those more vulnerable in society that is different to everyone else. This is not a one size fits all situation. I get that this is will be a medium to long term issue, but I am keen that we see the government take a proactive stance, not a reactive one. And I’m scared. 

Stay well friends x 

*Having been through our experience on PICU, I am only too aware of how limited resources are (such as Optiflow and our amazing doctors and nurses) .... and which during this pandemic, will be far, far exceeded. We need to support our NHS in any way we can during this time. 

A very little Isaac and Anouk x 

Not for the squeamish...

Isaac’s surgery, thankfully, went ahead today. Not only because he needed it so badly (his headaches, nausea, fatigue have peaked, and the inevitable infection in his poor blocked sinuses will further compromise his lung health) but also that planned surgeries may be on the brink of being postponed to free up beds for Corona virus patients. 

The anaesthetist did um and ahh a little pre-op as to whether he was well enough, but then agreed that this might be ‘as good as he gets’ right now, and only going ahead would give him a chance of bouncing back. Going into theatre knowing there is an increased risk involved is our norm. Thankfully, he was stable throughout, and back in recovery, had good O2 sats. After a good amount of morphine, we were back on the ward, and discharged a few hours later (with the caveat that we would need to go back if the bleeding got much worse). 

Needing to get back for home IVs, we headed off, only on the A14, he begins to feels sick.... I’m stuck between lanes of traffic and roadworks, so single  handedly grab the only thing I can think of, a bag in the back full of his IV drugs I had bought as back up, which spill all over the backseats... he throws up into the bag again and again (all water, as he hasn’t been able to eat) but the action sets off his nose, and the blood starts GUSHING; so there we are, driving along with watery sick and blood pouring out, and he smiles at me, blood smeared across his face, saying ‘I feel so much better now’. We laughed and laughed (because, what else can you do?)...until we realised the bag had a puncture. 

We now have recovery; at least another 8 days of IVs, extra steroids (to reduce his inflammation) and post-op quarantine. After that, he deserves a good long stretch of feeling well. Thank you to everyone who has sent messages of support and have been thinking of Isaac. Love you all x x x 

15th birthday. Blurry as Isaac prefers.