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.