New trials

Good ones this time. 

It looks likely he will be accepted onto a new clinical trial. We try and keep our hopes in check, as he has screened and then failed to be accepted onto trials before. There are a hundred different variables he needs to meet, including being well enough, but also sick enough for them to prove efficacy to pass. Sometimes this is based on his lung function, others, his microbiology. Keeping hopes in check is not one of my strong points, the curse of being an optimist. 

This current trial, if he gets on, is fundamentally different from the last two, which were both early stage gene therapy trials, looking to treat the root cause of his disease. Both were open label, meaning no placebo group. This trial, which I can’t go into details about, looks at treating a specific bacterial infection (that set up camp in his little lungs when we was just a toddler, and never left) in a new and innovative way, rather than relying only on rounds and rounds of antibiotics. Not gene therapy, but still, exciting stuff. The less positive part is that there is a control group. One third of patients get a high dose of the drug, one third a low dose, and one third, placebo). That is new to us, and something to get our heads around. Treatment will last one year, which is a long time to wonder for, and potentially see no effect at all. So we hope and hope, in layers. 

This week has been filled with vomiting, crackling lungs and problematic blood glucose levels, when what we need more than ever some stability. As always, he is calm. Me, a little less so. But hope and plans help enormously. 

We have kicked off our annual festive fundraising, culminating in the raffle that we hold at my work, which tops up my heart. I have the best friends, family and colleagues. Love, love, love x 

Cystic Fibrosis is a life limiting, genetic condition, affecting some 11,000 people in the UK alone. CF sufferers often, as a last resort, need to trade in their damaged CF lungs for the lungs of a generous stranger. To sign up to support organ transplantation, please visit https://www.organdonation.nhs.uk/ and tell your loved ones of your wishes, as even when signed up, the decision becomes theirs after your death. Thank you. 

I offer riches

 Donna Tartt

The Goldfinch, The Secret History, The Little Friend (Probably my favourite author of all time—The Goldfinch is the book you can bury me with).

Hanya Yanagihara

A Little Life, To Paradise, The People in the Trees (A Little Life tore my heart out, wrung it dry and left it in pieces. Somehow, I loved every devastating moment).  

Barbara Kingsolver

The Lacuna, Demon Copperhead, Flight Behaviour, (the new one, Partita, less so) (One of my favourite authors, though nothing else of hers has struck me quite like the first two. I read The Lacuna soon after visiting Mexico City and Frida Kahlo’s house, and fell completely in love with it. If I could, I would quote it daily. Demon Copperhead feels painfully of our time: tragic, urgent and far too real).

Khaled Hosseini

A Thousand Splendid Suns, The Kite Runner (So heartbreakingly real).

Jeffrey Eugenides

Middlesex, The Marriage Plot (I came late to Middlesex, but it was more than worth the wait—truly wonderful).

Elena Ferrante

The Neapolitan series, and everything else (An author I return to without hesitation. I love everything she writes).

Kristin Hannah

The Four Winds, The Nightingale, The Women (All incredible. A modern Steinbeck, with women’s lives and suffering at the very heart of her stories).

Sarah Winman

When God Was a Rabbit, Still Life, Tin Man (When God Was a Rabbit has a special place in my heart, love love love).

John Boyne

The Heart’s Invisible Furies, The Boy in the Striped Pyjamas, All the Broken Places, The House of Special Purpose, A Ladder to the Sky, The Absolutist (Please start with the first, brilliant). I love him, but some of his books are hard. 

Gabriel Tallent

My Absolute Darling (Incredible debut)

Delia Owens

Where the Crawdads Sing

Chimamanda Ngozi Adichie

Half of a Yellow Sun

Lauren Groff

Fates and Furies

Marlen Haushofer

The Wall (I could not put it down. A seemingly simple story of resilience and the practical business of survival, yet it stayed with me long after the final page. Still does really). 

George Orwell

Animal Farm (Of all the Orwell I read at school, this is the one that stayed with me—my first doorway into sociology, the Russian Revolution and the idea of revolution itself)

Miranda July

All Fours (Kinky, troubling, unbelievable and yet somehow not)

Sarah Waters

The Paying Guests, Tipping the Velvet…. (I’ve read a lot of her books, all good)

Kazuo Ishiguro

Never Let Me Go, The Remains of the Day

Elisa Victoria

Oldladyvoice

Miranda Cowley Heller

The Paper Palace

Helen Cullen

The Truth Must Dazzle Gradually

Lara Prescott

The Secrets We Kept

Henri Charrière

Papillon (I read it as a teenager).

Jeanine Cummins

American Dirt

Jonathan Franzen

Crossroads, Freedom, The Corrections (Complicated, infuriating and extraordinary in equal measure).

Eliza Clark

Boy Parts (Definitely not for the faint-hearted, very cool). 

Yael van der Wouden

The Safekeep

Danya Kukafka

Notes on an Execution

Hernan Diaz

Trust

Anthony Doerr

About Grace

Ann Napolitano

Hello Beautiful

Bonnie Garmus

Lessons in Chemistry

Alice Winn

In Memoriam

Michael Ondaatje

Warlight

Hervé Le Tellier

The Anomaly

Andrew Miller

The Land in Winter

James Baldwin

Giovanni’s Room

Hans Fallada

Alone in Berlin

Maggie O’Farrell

The Marriage Portrait, Hamnet

John Steinbeck

The Grapes of Wrath, East of Eden

Kelly Rimmer

The Things We Cannot Say

Tara Westover

Educated

Philip Pullman

His Dark Materials trilogy (Fantasy is rarely my territory, but the idea of a daemon companion utterly won me over)

Taylor Jenkins Reid

Daisy Jones & The Six, The Seven Husbands of Evelyn Hugo

Louis de Bernières
Captain Corelli’s Mandolin, Birds Without Wings (I think this is where my love of stories about war began)

Alex Garland
The Beach

Celeste Ng
Everything I Never Told You

Elif Shafak
The Island of Missing Trees

Anna Sewell
Black Beauty (The book that started it all: my first love as a reader)

Florence Knapp
The Names

Nickolas Butler
Shotgun Lovesongs

Charlotte McConaghy
Wild Dark Shore

Not an exhaustive book list by any means, but some that I can recall. I’ll kick myself later for missing some out. Reading keeps me going, always. 

We have a lot going on, and right now, Isaac is well. Proper update soon x 

Rare amongst rare.

The first gene therapy trial that Isaac took part in (in which he was the very first patient worldwide to receive multiple doses) has now been discontinued due to adverse reactions in patients. We couldn’t talk about the details at the time, but the latest press release can be found here. The fact that Isaac was the sentinel patient was, pretty frickin huge. 

I couldn’t stay in the room during the treatment (via nebuliser, his team were masked up carefully) and stood shaking in the corridor watching through the gaps in the blind. Lately, one of the team told me that it was just as scary for them (they are always cool as cucumbers at the time). Hearing that reassured me somehow, knowing not only how much they care for their patients, for him, but also that it was as significant to them also. This is new, groundbreaking science, and with that comes risks. 

Knowing that this drug will not be progressed further is a blow, but we go in knowing that very few phase 1/2 trials progress to the point where they are actually licensed for clinical use. The learning that is gained will benefit future developments, and every clinical trial participant has contributed to that important fact. Beyond proud of our boy. 

Furthers news that the Lenticlair trial was halted after just five patients being dosed is a stab to this wound. We had talked to the Brompton hospital, London, about joining this trial some time ago. At that time, again, he had the possibility of being the first patient dosed in the trial. Only with a trial for gene replacement rather than gene editing, the effects could be much longer lasting. At that time he opted for gene editing instead, because of the potential risks, but also that he would likely be ineligible for further gene therapy trials if he took part. Now that the trial has ceased, at least for now, we are so glad of his decision. Gene replacement in CF has been the ultimate goal to hope for since the early 2000’s, and now, what? 

CF is a rare disease, and Isaac is now in a rarer group still. Only 10% of people with CF are left ineligible for CFTR modulators which are transforming the life of so many patients for the better. It’s not a great club to be in, and with trials being pulled it can feel isolating even further. Hope is everything, and any knock to that, for me, is hard to bounce back from. 

His lung function has been on a downward trajectory all year, he finished IV antibiotics recently, and is starting new nebuliser treatments. In a week, his lung function climbed 10% which is, epic! He is on an urgent list for sinus surgery once more, and the headaches have been much worse than normal. Pain, heatwave, and a needle in your chest don’t make for the best summer, and that’s hard to watch. Really fucking hard. 

I always want to be able to tell friends and family something positive, problem is, chronic illness rarely gifts us that. So instead, here are some pictures which say a thousand words. Rosa’s birthday, hospital with my boy, dancing with the best of friends, and getting out to listen to my book in a park x

Life, loss, research hopes.

Read an article by me, here!

And a picture of family love after loss. Despite the sadness, in the celebration of life, in our togetherness, our hearts boomed. Love you all. 

More soon x 

My whole world, right there

Today’s headline: CF sucks, but CF + it’s many implications + additional chronic illness, sucks even more.  The impact of new diagnoses on our souls, new symptoms and new treatments and the myriad of overlapping effects this means further amplifies our need to live in peace with ambiguity and the need to take each day as it comes. I am trying.

It seems at to me that we live in a world where wars are waged by elected twats with less sense than the people who voted them in; where famine is ignored in favour of celebrity gossip. In this climate, simply being aware of your own privilege - warmth, safety, freedom - and wishing those things for others makes you a ‘woke lefty’. Everyday starts with a dose of anger and melancholy, just from watching the news. I feel exhausted at times, living in a world that so often has forgotten how to care. On top of that, life with chronic illness in the family, where you can only manage, never cure, it still amazes me that we find laughter in every single day. 

We have one new positive; a new clinical trial to focus on, hopefully in the next month or so. That, we so need. As ever, he is my hero, and increasingly takes the lead in every way, making our team stronger. At home, our funny, bright and beautiful girls and kooky dogs make everything seem brighter for us and nature helps ground me further. More soon x 

Doggy

Suspicious dog. Puppy dog. You know I am right dog. 

They rule my heart dogs. Two dogs. Too handsome x 

The lived story

These last weeks have been confusing, to say the least. We are grappling with new diagnoses’, confusing and overlapping symptoms, additional meds, and multiple clinical teams (between which communication is not always the best). Today I offloaded a whole bunch of worries on a CF friend. Just talking it through helped clear my muddled, cloudy thoughts. Having friends in the CF community to talk has proved vital to my balancing of emotions, thoughts and learning. It’s not all clinical, the lived story is complex, meandering and never black and white. 

Thankfully Isaac’s lungs are stable right now, following recent IVs, and he’s currently away visiting his girlfriend. Getting him well enough to live life to the fullest of full is always our top priority. Travel with chronic illness takes forward planning and preparation, but when you win, it tastes all the sweeter. 

Due to these new complications he cannot, for now, take part in the gene therapy trials we had been on track to join and had pinned our hopes on. While we wait (somewhat impatiently) I’ll continue to shout about the needs of the 10% of CF sufferers for whom the new modulator treatments will not work, who cannot be left behind.  This clip sums it up pretty well. Love love love x 

Swings, roundabouts, and plummeting slides

The CF cough is different. Hard to explain if you’ve not heard it. I would say guttural, but I just looked it up and it says harsh and from the throat. This is deeply from the chest. From every muscle and fibre of his being. Violent. Vomit inducing. Loud. Rumbling then escalating. Exhausting. 

These last weeks have been tricky. As a Mum, the stress of a CF exacerbation, plus issues with blood sugars, unknown seizures and tests, culminated in physical, mental and pretty weird ways. Minuscule in comparison to what he has to go through, but hard nonetheless - not only for me, but for those around me. I wept for an hour at a petrol station recently, unable to fill up because the hire car we had was like an escape room puzzle that I could not fathom for opening the fucking petrol flap. Dan had to drive a hour to rescue me, hug off my silly tears. This is not normal for me. Then an old Irish tune that Dan used to sing Isaac as a baby crawled in as an ear worm and I found my cheeks wet with tears before I realised it. 

CF is not only a disease of the lungs. It affects most of his organs, most notable his digestive system, kidneys, liver, pancreas and his fertility. As a child he experienced the gastric issue’s extensively (DIOS) but these have gladly lessened over time. His liver and kidneys suffer predominantly because of the aggressive antibiotic treatments that are essential for his lungs. His pancreas suffers because the enzymes which should be released via a duct to digest food are unable to, as that duct never formed in utero, blocked by mucus, and so slowly erode his pancreatic function from within. This, in turn, impacts the pancreases other job; to produce insulin. Leading to issues with blood sugars or CF diabetes. Seizures, epilepsy, the brain? That is not part of the CF package. That’s all new and confusing, and feels very unfair. 

He was not accepted onto the clinical trial, as his health is not deemed stable enough. We had, by then, assumed it was a given. The clinical trial team were all set to go, schedule ready. Disappointed doesn’t quite cover it. 

As we approach his birthday, we focus on getting him well (he started IV antibiotics today) and looking forward to easier times, and as this post describes, we enjoy the life amid the noise. Love x

Hazy lush nights with amazing friends ❤️

To think is not always to see.

We had a fantastic Christmas, all home and well. At times like that, it’s easier to shut CF into the back room and ignore it for a while. Even better, Isaac enjoyed his first solo trip (to Germany, to visit a girlfriend). This felt like no small win - CF so often scuppers plans at the worst possible time it can feel like the whole worlds shitty fate is against you. He had a brilliant time, came home loved up, and is planning his next trip back in the Spring. Despite the rampancy of flu and other viruses going around, he has escaped most of this, but his lungs are starting to protest more now. He’s also experiencing more of the confusing episodes that he had late last year. For that, we’re waiting on consultations with other teams and further test results. If I dwell on this new challenge, it’s easy to feel bitter, he already has too much to contend with - but my sunny side says we will work it out. 

I’ve said before, that while we have a pretty good idea what his lungs are like at any given time now (fruity, rumbling, wet, dry, wheezy, clearing, plugged…) we still occasionally surprised by either good lung function results when he is symptomatically much worse, or vice versa. Right now, we have the former, and so wait and hope he can avoid another course of IVs as we’re only a few weeks out from the last lot. Excitingly, he has been invited to screen for the next phase of a gene editing clinical trial, only one of four patients in the UK. I can’t say too much due to confidentiality- but suffice to say we are keen as custard. Hoping to start next month, which is another reason to hope he won’t need IVs anytime soon (he cannot start a trial within 30 days of extra treatment, that is, beyond his daily routine). 

Organ donation is something I’ve always felt strongly about, but even more so since I unwittingly joined the CF community. I have lost friends waiting on the list, and one shortly after (her body likely too weak after end stage CF to survive other complications). I’ve also known friends to be enjoying a full and healthy life many years post transplant. This can and does truly change lives, and yet despite changes to the law that were supposed to increase the number of donations, the reverse is happening https://www.organdonation.nhs.uk/get-involved/news/international-experts-advise-on-action-to-support-more-families-to-say-yes-to-organ-donation/ Please don’t only sign up, but tell your next of kin of your choice, as whatever you say now, it becomes their choice after your death x 

Lee Miller ❤️

 

10%ers

For the 10% of CF sufferers who cannot benefit from modulator therapy, the wait continues. And it’s hard. See some reasons why here. 

Isaac had another episode last week that we cannot understand, the tests continue. For now, we hope that he can enjoy his first independent trip away from home that is planned for before Christmas, the festivities that will follow (no doubt including my happy tears on his safe return) and then some answers. Aside from these worries, we have promising news on gene therapy trials he may be able to join soon - I try to manage my excited desperation x 

Ready for more

Since my last post, Isaac’s health has been more stable. By stable, I really mean more of the usual cycle of infection, inflammation, treatment, side effects, a few weeks of better health, and repeat. IV’s complete as of yesterday. All went well. At 20, Isaac is now pretty independent in his treatments; able to mix up and administer his own drugs (thanks to the much needed portacath in his chest). It’s a funny kind of pride, watching him do this, after so many years of it being me or the nurses. He amazes me in a way that leaves me gazing into space and smiling aimlessly. Hoping this course of bug busting antibiotics will keep him going through a trip he has planned in December (to see a girlfriend) and Christmas. Best of all, he has been invited to join the next stage of a clinical trial (gene therapy) that he participated in last year. Changes have been made, which again, I cannot disclose due to confidentiality reasons, but which have us excited. Hopefully starting again in the NY. 

Today is a good day. Love x

Emergencies and curveballs

I’m not quite sure how to explain these last few weeks. 

Ahead of planned sinus surgery, Isaac started IV antibiotics again. Partly because it helps protect his lungs from the stirring up of infections that surgery brings, and partly because his lungs were struggling a little more than usual. (I was sick on holiday, and Isaac caught the same after. Turned out to be the new strain of COVID - shitty little bug, wicked sore throat and evil headaches). He recovered well and surgery went ahead, which was a huge relief as the sinus headaches were worsening (ice packs and painkillers stage, but not quite the ‘under the duvet stage where the air is still’ kinda symptoms he has reached before). He asked me to film him in recovery for the first time (where Fentanyl should only ever be prescribed). We laughed a lot after the pain and he got to see for the first time the stage in recovery that he never remembers. For me, general anaesthesia never gets easier, holding his hand as his eyes go hauntingly vacant, but at his age, I’m thankful that he still wants me there. 

It’s not for me, in a blog, to share the details of Isaac’s health, and all the details of how that impacts him. But I can explain, somewhat, how chronic illness affects us, our family, and me. Isaac is proud of the work that I do with the CF Trust, the fundraising and awareness, but there is, of course, a wordless agreement that we keep a lot to ourselves too. 

A week after surgery, and a few days after we de-accessed his port following IVs, we had an emergency admission due to sudden collapse in the night. Thanks to his pup Remy, who woke Anouk upstairs, we found him and quickly called an ambulance. 

We have yet to figure out why this happened, and Isaac himself remembers nothing. It was a confusing and terrifying time for us all. The paramedics and the many teams that we were cared for in A&E and in the days after were incredible. This included emergency doctors, his ENT team (who had performed the surgery a week before, in case it was related to that), a Neurology team (new to us), Cardiologists, and of course his CF and Diabetic nurses. He is still having tests, many of which have been reassuring. His bloods are confusing. Nothing new there. 

We are accustomed to CF exacerbations; his symptoms tend to worsen over a period of days or weeks, or more quickly if he catches a virus. But it’s never been an emergency situation. Infection is our domain, our norm, and we know exactly what to look for. That could be a new infection, or a flare up of his existing bugs camped out in his lungs, or worse, something like a blood infection in his port line, or following a surgery. That we can look for, test for even. But this was entirely different, and I for one felt like the rug had been pulled out from under me left me in a crazy breathless free fall, the air from my lungs sucked out. A stark reminder of his time in ICU, I realised how much of that trauma and pain had been buried, both over time and in confusion. The pain and fear was so much worse than I even remembered. 

Since then, he has been much more stable, and we remain hyper vigilant. Remy and Anouk have been hugged to death for their parts in finding him (it was 3am). We want to understand this, I feel like I need that, but may need to accept that it was a one off and we may never know why, while also being prepared for it to happen again. 

In conclusion, CF still sucks, and curve balls can also be thrown in your face at any time, hard. Please consider organ donation, the waitlist is real. 

Cystic Fibrosis is a life limiting, genetic condition, affecting some 11,000 people in the UK alone. CF sufferers often, as a last resort, need to trade in their damaged CF lungs for the lungs of a generous stranger. To sign up to support organ transplantation, please visit https://www.organdonation.nhs.uk/ and tell your loved ones of your wishes, as even when signed up, the decision becomes theirs after your death. Thank you. 

Plans, holidays, IV’s, surgery

Happy to say we squeezed in a five day holiday in the mountainous, beautiful, hot and sometimes smoky (spotted the wild fires before we even landed) Montenegro. With chronic illness, a plan that actually happens is a huge feat. Back home and we are starting IV antibiotics once again. His chest is moaning, the bugs shouting, and we need him well for more sinus surgery next week. Today has been full of fevers and headaches. For all of Isaac’s care and surgery we rely on our amazing NHS; with any surgery he is high risk to anaesthetise so private health care is never an option (even if we could afford it). Again, we navigate a CF team plan for IV treatment from one hospital and his ENT care in another, which always makes for interesting talks with his teams. We make it work, and we are always so very grateful for what we have available to us in the country we live in. Many do not x

Cystic Fibrosis is a life limiting, genetic condition, affecting some 11,000 people in the UK alone. CF sufferers often, as a last resort, need to trade in their damaged CF lungs for the lungs of a generous stranger. To sign up to support organ transplantation, please visit https://www.organdonation.nhs.uk/ and tell your loved ones of your wishes, as even when signed up, the decision becomes theirs after your death. Thank you. 

We did it!

This was a phrase much used on late Saturday night and Sunday in our cute little cottage near the Jurassic Coast. Sometimes in wonder, some in disbelief, many in pride. 40km is not the longest any of us have trekked while fundraising for the CF Trust (we did 67km in a day in the Surrey Hills once), but this was tough. Steep, beautiful, sometimes scrambling. Doing this kind of event as a group is bonding in ways I cannot explain. Sarah, Michele, Jo, I hope you know how much you all mean to me, always. 

We have raised over £3,800 with this trek, plus gift aid (link here if you would still like to donate). No matter the amount, we appreciate every sponsor (and spend a lot of time delightfully debating who the very generous anonymous donations may be from!). Thank you so, so much. 

Today Isaac completed his latest course of IV antibiotics. He’s feeling much better, and coughing so much less. The feeling he describes to me after having his port de-accessed following two weeks with a needle in his chest sounds wonderfully liberating - especially so in the heat of summer. The packing away of the IV supplies back into the cupboard, which dominate the kitchen while he’s doing them, feels cleansing and cathartic. Today was a breezy happy day, until I see the children of Gaza starving and I weep again. I am supporting the Red Cross in the small way that I can (https://donate.redcross.org.uk/appeal/gaza-crisis-appeal) and plan to join protests. This cannot go on x 

More drugs please

Lots has been happening lately. We are talking to our team about new clinical trial options. Hope is everything. 

Sorry - it’s tricky as we can’t yet say much; as gene therapy research is all still very new and commercially super valuable to the pharmaceutical industry we are not able to share what trials he has taken part in (two) or how he found them (mixed) until the data is published. We are tied into his last trial until autumn, but excitingly we have options other than gene therapy now too. One example is phage therapy. I LOVE phage. We should all love phage. 

In my very simple layman’s terms, phage is a virus whose sole purpose in life is to destroy the bacteria Pseudomonas. Isaac is colonised (meaning these bugs have taken up permanent camp in his lungs) with three bugs; two bacterial, one fungal. But the most prevalent in his lungs by far is the PseudoA. I still remember the call from the hospital to say he had first grown this. He was 18 months old and I sat, head in hands, on a mossy bench outside the office. This news changed our lives forever, resulting in daily nebulisers and frequent courses of IV antibiotics ever since. The idea is that by inhaling phage directly into his lungs, and if that can reach the parts of his inflamed and damaged lungs, we might be able to destroy the unwanted Pseudo camps, or at least, damage them. The stuff of my wildest dreams!

Isaac has asked to start IVs next week, as his symptoms are worsening. That he has matured so much in these last couple of years, and now plays an active role in his treatment is, I don’t know how to describe it really, just, brilliant, and relieves the pressure on me massively. 

In less than three weeks, my sister Jo, friend Sarah, cousin Michele and I will be trekking 40km of the Jurassic Coast. This will be a 9-10+ hour slog with difficult climbs, probably during a horrible heatwave. To help fund vital research that can lead to real life treatment options for people with CF, like those I describe above (especially for the 10% like Isaac who have no other options) please consider sponsoring us here. Every sponsor, small or large, is truly appreciated. Thank you thank you thank you! X

Anouk’s prom ❤️

Cystic Fibrosis is a life limiting, genetic condition, affecting some 10,000 people in the UK alone. CF sufferers often, as a last resort, need to trade in their damaged CF lungs for the lungs of a generous stranger. To sign up to support organ transplantation, please visit https://www.organdonation.nhs.uk/ and tell your loved ones of your wishes, as even when signed up, the decision becomes theirs after your death. Thank you. 

Winning

Things are pretty groovy right now. 

Our amazing Anouk is calmly thriving during her GCSE exams  - this girl will go far, and always full of heart. My pride bubbleth over!   Our days off are often filled like this…. walking through meadows with Isaac, Obie, Remy and our Rosa Rocket on the lovely Skyfaxa…

“You can look at a picture for a week and never think of it again. You can also look at a picture for a second and think of it all your life.”

― Donna Tartt, The Goldfinch

Stability in health is something we never take for granted. Health and happiness coexist like the river is to the sea. That’s my way of saying, comparatively? Things are good right now.  While we enjoy this welcome reprieve from hospital, Isaac is back working part time, for the first time this year, which is fantastic. 

Meanwhile, our training is getting well underway for the next fundraiser. I have to admit, my bones feel so much older now than when my sister and I began with Ben Nevis (pictured below) when our boys were still so small and the diagnosis was still so raw (and we looked so much younger!) x 

Our 40km Jurassic Coast trek will take place on July 19th and you can sponsor us here. Every donation, small or large, is welcomed by warm fuzzy hearts by us all. Huge thank you to all that have donated so far. 

Set backs and training

It is always harder to write without good news. 

Even harder when you cannot divulge details of the clinical trials that we are part of due to non-disclosure agreements. I think all that I can say is that… it’s been emotional. A soon as I can write more, I will. 

For now, lung function is down and we will concentrate on getting him well. I can’t lie; without advances in research, we have no option but to treat only the symptoms of this disease, which is progressive, shitty and cruel. He’s currently being scheduled for yet more sinus surgery too (we have lost count now), another hurdle. 

And so, again, I throw myself into the fight once again; the only way I know to keep going and feel proactive. I’m doing a few things, but my favourite by far is the Jurassic coast trek with my core crew coming up in July. This will be a challenging trek, likely taking us 10+ hours, over a single day. Type2 fun we like to call it! If you can support, in any small way, it will be so much appreciated by us, and of course, the Trust and the whole CF community that they support so fantastically. 

Training pics below. Love you my ladies, more than you will ever know x x x

 Our Jurassic Coast trek will take place on July 19th for which you can sponsor us here. 

Health update and the Jurassic Coast

Exciting news! For us at least. We have planned our next fundraising trek which will take place on July 19th and you can read more about here. Please consider sponsoring Jo, Shelley, Sarah, Matt and I on this next challenge. I’ll post training updates along the way. 

Why this is so important to us? Why do we trek again and again, punishing our feet? Why so many of our dear friends organise gigs (thank you James), collect money in lieu of birthday gifts (big hugs Jimbo), help arrange our yearly raffle (Heather, Paul, Richard to name just a few), and all the other crazy things we have done along the way that have meant that we have now raised over £100k for the CF Trust…..

In blunt terms, twenty years on from Isaac’s diagnosis, we are still completely reliant on fighting only his escalating symptoms, not the root cause of his disease. 

Yes, we have some new antibiotics (not nearly enough, which you can read the dangers for all of us about here), we have new techniques and tech for drug delivery and physio to help try to clear his chest of mucous, one new mucous thinning drug for him to inhale, and a handful of new variations of existing meds that help keep him plodding on; but ultimately? Isaac takes only the same old treatments that were available to him as a child. 

Right now we are pursuing the best option open to us; gene therapy trials. I’ve written about this before, and added some FAQ’s here. We have the best team around us at Papworth hospital. We have an incredible consultant, who over this Easter bank holiday weekend, while not working or even being on-call, has called us three times to check how he is and give us test results. And we’ve been lucky enough to participate in truly groundbreaking ‘first in human trials’ (I cannot write more about this yet, as the information is commercially sensitive). 

However, even participating in trials can only give us limited, time-bound hope. The treatment period is short, after which he stops the drugs and goes back to only his usual routine. The data crunching and endless bureaucracy that brings a drug to patients takes years, and realistically, even if these turn out to be both safe, effective and affordable, they are probably years from being licensed. 

But we do this to further the research for all, which is incredibly important to us. And it comes with fringe benefits; we are seen by THE best doctor (as principal investigator in the trial) with 121 care and lots of extra tests and consultations (which so far have revealed other new treatments we can try in his day to day care), and possibly, if these drugs prove themselves, early access on compassionate grounds may be given if his data prove that they work for him,  if he was very unwell and licensing was closer. 

We feel like we’re taking the most proactive way forward, and Isaac has grown magnificently into his role as a patient. He advocates for himself, can now list to every medication he takes and why, is clear and can communicate his preferences (bloods from port today, or venepuncture buddy?) and is building up a much clearer story of his own medical history (allergy to Fentanyl? That was 2018. More sinus surgery? Yep, ok, despite the pain, it’s worth it longer term). We are a team in all of this, and enjoy our time together, no matter what. Quite possibly me more than him. 

Right now, his lung function is down, and we’re having ongoing issues with his liver. He’s had mild liver disease for years now (very common in CF), and it’s an unhelpful complication, despite being something we’d not know about were it not for all the tests. But, he is a few months from his last IVs (which give his liver a welcome break). We are back and forth to the hospital a lot, with another admission planned this week. He makes the most of the food in bed and internet. I sit in fear that complications might prohibit his participation in trials, and feel thankful for every day that means we can continue, and for the chance this might give him and many others in future. 

It feels like winning every time we get home and his dog bundles him with love and gross face licking. 

We desperately need funding to continue for the last 10% of CF patients, for whom, like Isaac, new CFTR modulators do not work. All sponsors, however small, are so much appreciated. More soon, love love love x

Image: Dog love when you feel like shit ❤️

Why lung function and symptoms don’t always match

Oh my bananas, I cannot tell you how many times we have got this so wrong. Coughing until he is sick, and then LF is stable? Feeling really quite good, and LF is terrible? 

These days, we always try and guess ahead of testing in clinic (we also have home spirometry, but testing too often can be soul crushing and anxiety fodder). Often we can predict within a 5% window. But not always, and truly, I’m not sure what way round is worse… Shitty LF when he seems quite well is always worrying (and usually a prompt to start IV’s which he may argue against). But good LF when he’s feeling so crappy? The course of action feels less clear. Right now we seem to seesaw between on and the other. Confusing. Thankfully we have an incredible team who we have now built a relationship with (over three years in adult care) and who listen to Isaac and take a more holistic approach, and we always find a treatment path that we all agree on. 

Sorry, writing is weird at the moment. We still can’t talk about the last gene therapy clinical trial that Isaac was a part of (until the pharma publishes the data) and we now can’t talk about the next one. But we can say that thangs are happening, and we hope they happen soon. Annoyingly his diseased sinuses are causing headaches and nausea once more. When we last saw his (lovely) ENT surgeon, he said that Isaac can pretty much choose when to schedule his next surgery (schedule that is, waiting lists dependent!), and we need to do this, but can’t during trial participation, which is difficult. 

Otherwise, things in Molyworld are grand. The bulbs are sprouting. Anouk is acing her GCSE exam revision, and Rosa and I are loving our pony time. Love x 

Obie, Remy and Skyfaxa, our dæmon companions ❤️

Forgotten posts

I realise that writing might be deeply unfashionable these days, I should be offering bite sized chunks of content plastered all over social media. But I’ll always love words, lyrics, idioms, editing down the random ramblings from my mind, books, getting lost in the landscapes that stories build in my mind. As much as I’m told to get on social media more, or worse, vlog (ha!), I think I’m sticking around here, in my geeky comfort zone. I don’t really do any social media now (can’t be arsed with the ads!). Although I’m mostly too busy or tired, I still occasionally get the urge, maybe need even, to write here. Often to offload. Sometimes to inform friends and family so we don’t have the repeat health updates again and again. Maybe because it builds up a timeline of events that I can look back on. But mostly in the hope of raising awareness of Cystic Fibrosis, a largely invisible disease that is so often mistaken or misunderstood. A GP that I had an appointment with asked me recently ‘when did he get it?’. Christ. 

So while I’ll stick to words, you can watch this vlog which captures what life with CF can look like better than I could ever describe. Words are not always enough. Please do take a look here. 

Isaac is currently, um, stable? I guess? Hard to say when things change daily. He’s had recent IV’s, a plethora of lovely viruses (he is currently on anti virals, a fourth daily abx and steroids) and the random fevers and joint pain persist. Our participation in gene therapy trials, which we so desperately want, is delayed for as long as he is on extra treatments for an exacerbation (he needs to be at least four weeks clear of meds that are additional to his usual regime). It’s frustrating to say the least. Too many delays, and he could miss out altogether. 

But things could be so much worse. A good health day is walking the dogs together and him doing the cooking. A bad day is only ever the one before a whole new one. 

I often start writing, and then forget to ever post them, so do look back on previous entries if you’d like to. Hard to believe that this little old blog has had almost 100K views. Crazy. Love love love x

Pictures: Christmas at home.

War on bugs

This week Isaac wisely decided that he needed IV antibiotics, and that he wanted them now to be well for Christmas. We had a call with his consultant, who praised his maturity of mind, agreed a treatment plan (Ceftaz and Meropenum with a side order of steroids). Yesterday, Dan and Isaac headed into clinic. Had his port accessed. Did the first dose while chatting to the team. Picked up the drugs, bins and the masses of required paraphernalia to complete the course at home, and headed back. 

It reminded me of a video I once watched where another CF patient was in a similar position; lungs protesting, extra physio and usual meds not nearly cutting the mustard. For many CF patients, IV antibiotics are inevitable when lungs scream. At some point you must concede defeat and just get on with the war on bugs. Certainly in Isaac’s case, however much of a pain and chore IVs are, they are a fait accompli, and a tool we rely on and are grateful to have in our arsenal. 

So, a familiar story that we can empathise with. Only after watching this video, I accidentally wondered into the darkest depths; the dreaded comments section (mistake, troll city) where the patient was criticised for her ‘privileged conceit’ expecting her ‘doctors to be on speed dial’ and seemingly ‘choosing her own treatment plan’. 

Whilst I rolled my eyes (as I often do at the fucking audacity of people who moan through the anonymity of their icky keyboards) and took breath, it reminded me of why CF awareness is so important. Because, as absurd as this obviously sounds to some people; this is our reality. 

People with CF know their bodies better than anyone. They know when their lungs will respond to nothing less than the big guns (IVs). There is no way, on any planet, that anyone with CF would ever, ever, ever, choose to do more treatment than what was absolutely needed - no one thinks, ohh, what a great idea, let me pop a needle in my chest and inject toxic shit that makes me feel crap into my blood stream multiple times a day! 

Moreover, CF teams know their patients. They know the bugs that are growing in their lungs. They know what drugs those bugs are sensitive to, or not. They know their patients medical history. Critically, they know that CF lung exacerbations escalate. Bugs don’t just give up, so nor can we. Proactive is the name of the game we must play. A game we never chose. 

The relationship between CF patient and clinical team is probably a fairly unique one. CF is a chronic, lifelong condition. In paediatrics we had the same two consultants for the whole 16 years we were there. Now, we are building new relationships in adult care, and I can confidently say that we spend way more time with our CF team than we do most of our even closest family. 

So yes, as crazy as it might sound, we can make a call, make a plan over the phone, and take action. This might sound like a privilege, but that is because this is needed. CF requires aggressive, timely treatment. Without that, infections take hold, worsen, and lead it irreversible inflammation and scarring of the lungs. 

We don’t have the same for other co-conditions (we wait for many months for much needed sinus surgery, on an almost annual basis), but lung infections? This is like our BAU, our bread and butter, we know this, we get it, we arm ourselves quickly and appropriately before the little feckers cause any more long term damage - and ultimately, this keeps us out of hospital as much as we can, saving essential NHS resources by caring at home as much as we can. 

The relationship that we have with our CF team is one that we are so, so grateful for x