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. 

Annual review

We had Isaac’s annual review today. While we go to CF clinic regularly throughout the year, this is the one day in which we do a whole bunch of tests/reviews and look in more detail at how his baseline is changing with progressive disease. Some results we get are immediate, some, like the vitamin levels in his bloods, take several weeks. What we know now: 

• Most blood results are outstanding; but we do know the glucose fasting test (looking for CF related diabetes) was fine. 
• Lung function; down significantly, but as expected since he has a current exacerbation, and he struggled to do the tests while coughing (they were better later in the day when repeated; mornings are not friends with his lungs). 
• Psychology review; yep, confirmed that he gets pretty cross whenever talking about CF. 
• DEXA bone density scan; results outstanding.
• Sputum lab results; outstanding. 
• Dietician review; his diet is great, but he needs to up his creon dose (pancreatic enzyme replacement) and to take it more reliably.
• Chest x-ray; not too bad, considering current infection. 
• Portacath (his implanted venous access device); looked OK on X-ray (there were fears that it might be broken, as he’s had pain during flushes) but he was accessed today fine. We think the pain may be related to the scar tissue he has around the site from a while back when it got quite sore. 
• Physiotherapy review; all fine, but his adherence to treatments when he’s left alone to do them is lacking (e.g. lower last week, while on half term). We will also drop one of his six nebulisers a day, as they would rather he did five really well, than six badly. Plus the sixth is using DNAse twice daily, which it is not licensed for, but he had prescribed after black October (read more here) as his secretions were so ridiculously thick. 
• Ultrasound; enlarged spleen and fatty liver, indicating CF liver disease. He will start a new treatment for the liver, the spleen we already knew about, and is associated with the number of infections he has had. 
• Doctor review; the fact that his lung function has picked up as much as it has after black October, is great, and this year has been better than the previous year. They praised his hard work to stay well. Despite his current exacerbation, they are really pleased with him. 
• He may be referred to Orthopaedics to check what appears to be a curvature of his spine. He was scanned for possible scoliosis a few years ago, and if memory serves me right, it was said then that he may have a very mild case (no action required) but apparently this is something that can become more significant in teenagers. His consultant will refer his X-rays to see what they think. 

Plan; We’ve started IVs today and he had the first dose in clinic. Bloods tomorrow (and twice more next week) to check antibiotic levels in his blood. He will start treatment for the liver issue next week (just tablets). They hope he will be well enough for his sinus surgery next week, and they will do a BAL while he is under GA (hoover up some more sputum from further down, to test in the lab for infection). They may also do a bronchoscopy at the same time, subject to availability on the day. Await further test results. 

Sorry, nothing particularly poetic or insightful here. The day was full on, and he came home feeling sick and has gone to bed. I feel like I have no edges; I’m all blurry. Like most years, it was a mixed bag, but it wasn’t awful either. So proud of him. 

Have a great day, if not today, then tomorrow x 

Two weeks of IV drugs, a stocked meds cupboard, and a dogs bottom. 

Smells like smelly teen spirit

His chest is sounding worse day by day. While we had hoped to do his next IVs post sinus surgery, it’s looking increasingly likely that he can’t wait that long. He coughs all night, and while he is sleeping through most of it, I’m not. The worry in the night is always amplified to screaming point. Him, coughing his lungs out, me, silently screaming mine. 

Last Thursday we arranged for an emergency appointment to have lung function tests and see his doctor. Dan and I were both working, so Dan’s Dad kindly offered to pick Isaac up from home and drive him into Dan, who would take him in to clinic. Only Isaac refused to open his bedroom door, insisting that he felt fine and that he could wait a week until his full annual review appointment instead. I went home mad, knowing his team had gone out of their way to squeeze him into clinic, and worse, that if he leaves it too long, he risks not only getting worse, but also delaying surgery as they won’t anaesthetise him if he’s that unwell. Despite his pleading that he felt better, he then coughed all night long. 

Friday his consultant phoned to speak to him (this never happens) and explained the same thing... that if he needs IVs, he needs them ASAP to have any chance of not delaying the op that he so badly needs. He grumbled a bit, but then conceded that his decision to delay might not have been the right one. As it was then too late to take him in, we’ve started extra orals instead in the hope they tide him over to this week. If he can make it to Thursday, we can kick off IVs while we’re in for his annual review. This would give him 6 days of IVs pre-op and (at least) 8 post-op (post-op infection is a risk, so as long as it takes). If he gets any worse before Thursday, we’ll be in sooner. We’ve been warned already that they may want to keep him in a few days, considering his chest and the significant surgery, but they are balancing up that decision with the fact that they have flu on the wards right now, and that is the last thing he needs. 

More than the delay, the not knowing just how unwell his lungs are right now and the worry about delaying the op, it’s that he is increasingly taking the reins in terms of how and when he wants treatment, and not always for the right reasons; Refusing to go on Thursday was much more about the fact that it is half term holidays and he wanted to stay home and play PS4 than because he really didn’t want to start IVs. Had it been a school day, he would have happily taken the morning off school to go. 

For fifteen years now we have put his health above everything, and knowing that he might not do the same is heartbreaking. He’s going to make a lot of wrong decisions in the next few years I fear, and sitting back to watch that, as a parent, is really hard. When he was grumpy with me for being mad with him (after refusing to go to hospital) I told him that unfortunately for him, I will always love him way more than he can ever hate me for wanting him to to stay well. So ner ner, ner, ner ner (because we are that childish) x 

Isaac, 2019. 

Complaining and coughing.

After a CT scan, the follow up appointment shortly after, and then pre-op the very next day, we were pretty confident of the surgery date being very soon. After a week of waiting, I called, only to find that yes, he is on the urgent list, but this would still mean AT LEAST a two month wait. 

When I explained to the kind but slightly ineffective lady in admissions that he has constant headaches and nausea, is off school a lot because of this, that he also has CF, and that his infected sinuses cause issues in his already infected lungs, she struggled to reply (I was very polite, I always am, I get that this is a strained NHS, and that they do their very best), the only reassurance she could offer was that she would schedule a reminder for herself to check with the doctors again in TWO WEEKS. We cannot sit back and wait that long while he suffers this much. He has days where he walks round with either an ice pack or a hot flannel on his head all day, despite 24/7 pain relief. 

So I reluctantly called PALS and explained the situation. Within 24 hours, we had a surgery date two weeks from now. 

It’s so sad that we have to do this, yet again - we had the same with his last sinus surgery; after months of waiting our CF consultant actually advised us to complain through PALS to have him bumped up the list, as it was the only thing that would work. It seems being under the care of different teams creates a breakdown in care for the individual (at times Isaac has been under the care of both his CF Team (which includes his Doctors, Specialist nurses, Dietician, Physiotherapy, Psychology and Pharmacy as standard) but also Gastro, ENT and Immunology, and it feels like all these teams see only the aspect that they treat, without looking at him holistically; it doesn’t take a brain surgeon to get that blocked and infected sinuses will further hinder someone with lung disease, right? PALS help see the bigger picture across all of an individuals needs and prioritise appropriately. At least I hope that’s what it is, because otherwise it’s just about ‘he who shouts loudest’ and that would be sad too. Anyway, we have the date. 

A few days before surgery, he is also having his annual review (his full CF MOT; chest X-ray, bloods, bones density scan, ultrasound, full suite of lung function tests, diabetes test, dietician, physio, psychology, CF nurse and doctor reviews, all in a single day) and since he is coughing significantly more each day, a likely action plan will be to have two weeks of IVs post surgery.

But our worry is twofold; one that his lungs will need these IVs sooner - which could delay surgery, and two that because he is having surgery and his chest sounds so shitty that they will admit him to the ward, rather than allow home IVs. Corona* fears aside, the hospital is already amidst a flu outbreak, and we would much rather keep him safe at home. Plus, you know, being in hospital kind of sucks.

*Despite the very small rate of Corona infection rates here in the U.K, they are predicting that this could still unfold into a full epidemic here, and if that happens, up to 50% of people may be affected. Stay safe people! x 

Above, pictures of Rosa bean (her, a willing model and me, playing about with my camera). 

Below, little Isey. This was us festivaling it up, almost ten years ago. He will turn 15 very soon. How can that even be? ❤️

Corona virus

If Isaac were to contract this kind of virus, he would be at such a disadvantage from the off, it would be a significant risk to his life. 

We have already seen a virus take him from being quite well, having IV antibiotics on the ward, to intensive care needing breathing assistance - in just a matter of hours. That illness necessitated the use of a wheel chair, 24/7 oxygen, and a physical recovery which took many weeks (I’m not sure our mental recovery is even complete now). Mostly we just feel lucky that he recovered at all. 

However you feel about the risk (or not) of Corona virus, and wherever you are in the world, please, please practice good hand hygiene (this is much more powerful than wearing masks alone) - like having the flu jab, this is more about protecting those more vulnerable around you, than protecting yourself. 

If this does become a pandemic, and it sounds like it will, every one of us must take responsibility in trying to limit the spread of the virus in any way we can. If this means working at home more, if you can; do. If this means battening down the hatches and avoiding all non-essential socialising, if you can; do. If this means avoiding your GP surgery or A&E, if you possibly can; do. 

If you feel unwell, call for advice instead and keep the vulnerable (the very young, the elderly, and those with pre-existing conditions) in mind. Cough into your elbow, not your hand. Use disposable tissues and dispose of them well. As well as washing hands well, use alcohol gels in-between. Avoid public transport. Stay home. What might feel like a mild flu to you, may kill someone else. Take to the sofa, watch films, get well! 

My thoughts are with those in the eye of this storm, and those that fight everyday to help them x 

Sinus pain, surgery, and miracle drugs

CT scan done. The doctor said it was as he expected (since he’s already poked a camera up there anyway). Our CF nurse gave a less guarded review ‘looks ghastly, no wonder he’s feeling so bad’. 

The follow up which was to be a month later, I had moved to 3 days later (happy kick arse Mama moment), and the ENT team have been great, seeing him for pre-op the very next day, so we are all set for surgery, just waiting for the date now (we will keep the pressure on). 

His CF team will liaise with ENT, as while he is under anaesthetic they will do a lavage (hoover up some sputum from his lungs, to test in the lab), and depending on lung function test results, he will likely start a course of IV antibiotics at the same time. He is coughing more each day, and his last course was in late November, so the timing is no great surprise. He will need two weeks off post-op due to infection risks anyway, so tying in IVs to this time off school is no bad thing. 

Our hope now is that the op comes first, and the IVs second. If he becomes any less well, and needs IVs sooner, that could mean he wouldn’t be well enough for surgery, but at the same time, the sinus pain/infections continue to impact his lungs too. Bit chicken and egg that. 

Our beautiful, spirited, Rosa bean.

In typical Isaac style, he is taking it all in his stride. He is not able to exercise much; he is in pain and sick often; he is missing a lot of school - but he still laughs with us everyday. We got his school report, and despite his low attendance (about 80%, so a day a week on average.... and shall we say, a rather lack lustre attitude to homework in general) he is still doing well. I don’t know how he does it. 

In other news, myself and some very good friends are beginning to plan our next big fundraiser... so excited about this! No trekking, hair shaving or throwing ourselves out of planes this time... but an EVENT. More details to follow. 

Best of all, the CF community is awash with incredible stories about how Trikafta (the third generation of these new *small molecule drugs) is having such a crazy positive impact in the quality of lives of the 90% of CF sufferers for whom this will work.... in some cases, it’s mind blowing (e.g. being listed for a lung transplant due to end stage CF, to coming off the waiting list entirely with a 20% increase in lung function - in a matter of weeks!). Many happy tears hearing all of this news, but with it, I’m reminded of all those we have already lost, for whom this comes too late. I can’t help wondering, if it could have come in time for Jayne, would she still be here? For those most recently lost, this seems all the more cruel. In some countries, these new drugs had been developed, even manufactured, but they sat unlicensed in pharmacy warehouses due to cost and frustratingly slow negotiations and process. This must end. Trikafta needs to be made available in the UK now, before more lives are lost. Please sign the petition here.

Have a lovely weekend all x 

*I am asked about these new developments often, so just to reiterate, the current drugs that are licensed will not work for Isaac who is in that last 10% who have a class of mutation where the protein cannot be fixed (as it can be for the majority) as in his case, the protein is never formed in the first place, so we need a whole different approach. But it brings us a lot of hope too. 

Bassin de lumieres ❤️ (need to go): 

Headaches and nausea

Heublein. And my mood today. 

Isaac needs this surgery now. He is missing school, dosed up on painkillers 24/7, walking around with ice-packs or hot flannels on his head, and feeling sick. He has a pre-surgery sinus CT scan next week, but the follow up appointment is a month away. This cannot wait. The lioness in me is poised to fight for him. Again. This can be so exhausting x 

Pain and ports

Hello. 

Isaac is dealing with a lot of pain right now. His sinus issues of a couple of years ago have reappeared with a vengeance. I managed to bring forward an ENT appointment that he has later this month to this week, as his symptoms are worsening by the day; sinus pain makes his head hurt, and the potential (probable) infections in his sinuses make his lungs worse, and a combination of all of this results in a very vomity Isaac. It’s like a vicious triangle of mucous and pain and inflammation and retching. Maybe that is more of a square than a triangle? Anyway, I hope it makes some sense. It basically sucks. 

His last sinus surgery included removing polyps (very common in CF) and shaving off some bone (are your toes curling right now?) to widen his airways. We were warned then that this may not be the last time, and today we we found out that not only will he need this surgery again imminently, he’ll likely need it yearly, until he is 18 and he can have a surgery which is a bit more major that might resolve the issue for a longer time (or forever? I’m not sure). As he was last time, Isaac is enthusiastic about this painful surgery, simply because his day to day pain is so rotten. 

As well as this, his IV access (via his portacath) is troubling us too. Our community nurses (akin to Mary Poppins) came last week to do his normal monthly port flush at home, but the flush immediately entered his tissue around the port, rather than his blood stream as it should. It began to sting, Isaac alerted them pretty quickly, and the area looked obviously red. No harm done, but it raised concerns that his port was not working at all, and since we’ve had recent issues with a fibrin-sheath (causing stiffness in the line), this was not a good sign. Thankfully our CF nurse worked her magic, seeing him outside clinic time, re-accessing his port and managing to flush the line successfully. Possibly the community nurse may not have hit the right spot the first time (imagine feeling his chest, finding a a circular disk under the skin, and trying anchor that moving disk to stab a needle into the centre of that small area blindly? Not easy) but the stiffness of the line is still apparent and an ongoing concern. 

The hope would be for a port to last 5-10 years, and Isaac has had this one just two. If it fails, he will need a new one, which not only means more surgery, but also one less possible place on his body that they can site a port - which are limited, and could mean problems with long term venous access later on. 

That said, it’s a worry for another day! Thankfully his lungs are behaving right now.

Have a great day x 

Trikafta joy

To see an accurate glimpse into life with CF, and then the renewed optimism that one new drug, Trikafta, the fourth drug licensed so far that treats the underlying cause of CF, can bring, click here. If this does not bring you a warm fuzzy feeling in your heart, nothing will. From the brink of needing a lung transplant, to significantly less exacerbations, less IVs, and a much, much better quality of life. 

On average, Trikafta has been seen to increase lung function by 18%. That doesn’t sound like a lot, but if it maintains that increase in people with progressed disease, like Mary, that might prolong that time before needing a lung transplant, for, who knows how long? And for children without progressed disease, it might mean that they never reach the point of progressed lung disease at all. 

As I have said before, this generation of drugs will not (sadly) work for Isaac. These drugs fix a protein fault, but in his mutation, that protein is never formed in the first place... nonetheless, it brings us much hope, and for the 90% of people with CF that these drugs do work for, it really is life changing. Babies born today, with these more common mutations, may never know a life like Isaac has. This brings us much joy. 

So to all of you who have donated to CF research, thank you, and please don’t stop giving until these kind of treatments are available to everyone with CF. A worry of ours is that the big pharma’s will be less interested now - so our fight continues. 

New Year pictures (theme this year; pub names). 

Thank you to all our family and friends, we love you so much x x x