Floods and drugs

Hoping you’re all have a wonderfully restful Christmas. Ours has been just that - we miss others, but have made the most of the time we’ve had, sleeping in, watching movies, long dog walks in the floods, house stuff, and playing with the new toys (Rosa’s hoverboard and cart being a winner this year). I even saved a kayaker from the river in the floods (I suspect, just in time to avoid hypothermia). This, and seeing six fire engines, two police cars and two ambulances today racing down the lakes locally show just how dangerous floods can be (it sounds as if a car got in trouble in floods, possibly not knowing where floods actually become lakes, or the river, and how strong the currents can be). Yet I’ve never seen the fens busier. This once tranquil and quiet area is now a bustling attraction in lockdown. 

Fields not lakes.

Kaftrio, the new treatment for CF, has been in the news again and is undoubtedly the best thing to come out of 2020 in my eyes. Today the Observer detailed the funny year that CF patients have had; on the one hand COVID was expected to be a huge threat to all with chronic illnesses, but this hasn’t proven to be true in CF. Mostly, I expect, because COVID turned out to be so ageist, and CF sufferers are mostly young. But also because the CF community is well versed in infection control already, mask wearing, avoiding the ill, being uber clean, and seeking medical help as soon as symptoms progress. I read blogs, watch vlogs and speak to friends who have been able to try Kaftrio often, and it never ceases to amaze me. An oral drug, that can treat the underlying cause of CF (the CFTR mutation affects the movement of water and salt in and out of cells) - it really is incredible science. And the results have gone far beyond what many dreamed of; children and young people with end-stage CF are being removed from the transplant list, no longer needing new lungs from a generous stranger. In many cases, quality of life has increased dramatically. Children who take this drug may never have the inflamed, scarred and damaged lungs that Isaac has now. Families may avoid the pain, the stress, and the heavy burden of treatments that has been the norm for others all this time. 

The lesser spotted teen, on camera! 

Friends contact us when reading these stories... excited as we are in these exciting developments. However, Kaftrio will never work for Isaac. It’s not that it needs a slight tweak to make it work for his specific mutation, he needs a whole new recipe. To understand why, you can read more about his rare mutation here. That said, the success of these new drugs fills us with hope, and while we know it’s going to take a while (years, not months) we think that one day we will able to access a clinical trial for something that will work for him, and the other 10% of CF patients who are still waiting. 

Right now, Isaac’s lungs are struggling. Lung function dropping, cough increasing. We’re waiting to make a plan with his team as to our next steps, hoping above all to stay away from hospital, as they struggle with rising cases of new variant COVID patients in this tier 4 area.  

Christmas Day Bellini breakfast 

I will be walking 500km over the darkest month of January, and some wonderful friends and family will be pledging their own active challenges in the theme of 500 to support my fundraising further. There is no Christmas break from Cystic Fibrosis, and more than ever, charities need your donations. To read more, and sponsor us, please click here. 

Keep well x 

Obie, the most handsome dog

Wishing you all a well Christmas

Walking in a winter wonderland? Rather, Walking into tier 4 viral-war. 

Silver linings: We are home. Isaac is better (tummy wise, but cough is building up to an exacerbation again). Our family and friends are well, or getting there. We are not living on an airfield, in a haulage lorry cab, eating cold bacon baps, hoping to get back home to family for Christmas. We are not working over the holidays on Brexit deals, or as key workers. There have been no delays to accessing Isaac’s medications. We are warm, dry, fed, and together....

We have enough, more than enough. We are the lucky ones, but I think it’s also OK to admit; fuck this is shit! 

Mostly, I just miss people so, so much. I miss dancing. I miss hugs. I miss pubs, and soaking in a new atmosphere. Hearing the laughter of strangers. People watching. I miss meals with friends and family. I miss chaos with the kids and their friends. I miss feeling safe, and breathing freely. I miss excited anticipation, and spontaneity. I miss being early, or late. Having plans! I miss dressing up. I miss waking in the night, still giggling from the nights fun. I miss the jaw and rib ache you get from having laughed so much. I miss other peoples houses, and others feeling at home in mine. I miss being able to tell the kids of all our festive plans. It’s just one year, we think. This is not forever. But it is pants. 

I will be walking 500km over the darkest month of January, and some wonderful friends and family will be pledging their own active challenges in the theme of 500 to support my fundraising further. There is no Christmas break from Cystic Fibrosis, and more than ever, charities need your donations. To read more, and sponsor us, please click here.

Keep well x 

Hospital, transplants and walking

We just got home from a quick admission to the ward. The pain, vomiting and nausea hit a peak, as his abdo X-ray was pretty bad last week, and the initial treatment did not work, they called us in on Monday for another kind of treatment aiming to kick start his digestive system again properly.

For this, the plan was NG tube for 3 litres of medication (and IV fluids through his port). We were told we might get home in 3-4 days... but escaped early today on the proviso we go straight back if he has any pain or vomiting again. Getting out of hospital earlier than we thought...? Pretty sure that is a first! 

I can only imagine how Isaac feels....battered and bruised from within I guess (36 hours nil by mouth, an uncomfortable NG tube, and vast quantities of meds and fluids that bloat you out...) - and there was some fairly significant cursing when we arrived; he hadn’t been able to eat for 14 hours before we got there due to nausea, and when he finally mustered up an appetite, only to be told, no, sorry, you need to be nil by mouth for the duration (they didn’t tell us that bit)..!! But he was, in his usual Isey style, understanding, if not enthusiastic. 

As with any admission to a children’s ward, I’ve come home feeling raw, humbled, emotional; especially at this time of year, seeing so many kids on the ward who will not get home for Christmas, or maybe, at all. 

Wonderful short film here about life, CF and lung transplantation if you’re interested, here. 

This week has been an uber emotional one; a family funeral that no-one can attend due to COVID, hospital stay, test results, and then yesterday, our annual raffle at work, in aid of the CF Trust. I can honestly say, I don’t think there was a dry eye in the house after watching videos of the many festive activities that my colleagues got involved in leading up to this fundraising event, a hugely touching speech from our much loved MD, a fantastic and moving poem from a very good friend, and a fundraising total of over £4,000. I can’t thank enough everyone who helped organise all this, and all who donated X 

I will be walking 500km over the darkest month of January, and some wonderful friends and family will be pledging their own active challenges in the theme of 500 to support my fundraising further. To read more and sponsor us, please click here.

A Christmas Rosa and a hospital spot the difference! 

Hospital and birthdays

Clinic today, to try and get to the bottom (crappy pun intended) of his digestive problems; pain, nausea and vomiting.

He immediately had and X-RAY and bloods to test for pancreatitis (rare, but serious) and an emergency surgical consult. Unsurprisingly, the X-RAY showed likely DIOS, again. Relief that it’s not something new, but also....sigh. I mean, yes, better the devil you know and all that - but a frustration nonetheless, we’ve been round and round with this years before.  The good news is we are back home, with Gastrograffin again, to treat this over the weekend. But this is not a long term solution. 

It is not right that he is needing pain relief and anti-nausea drugs most days. The vomiting has no rhyme or rhythm, but at the same time, it’s a constant and unwanted companion. It doesn’t appear to be linked to what he eats, how his lungs are, what treatments he takes (as far as we can gather), or his physiology (in that, Gastroparesis has previously been ruled out, and he’s eaten radioactive egg on roast (really) to check his digestive track, albeit a few years ago now, and that was all fine) - it’s just always there, making him throw up, on a average, maybe 3 times a week? 

Isaac’s digestive problems rarely make it to the top of our priority list to focus on; because he has both bacterial and fungal lung infections to battle with concurrently, these preoccupy most of our time with the doctors. Also, Isaac is atypical in CF in that his weight and height are very good now. He was underweight as a babe, and we had years of supplementing his diet to boost his weight, but for the last 5 years, we’ve had no growth worries at all - perhaps because of this, his digestive problems rarely make it to centre stage in his medical reviews, and play second fiddle to his lung problems. But in the last few weeks he has missed probably 50% of school time due to vomiting, pain and nausea, and that is no quality of life. When we do get him into school, his is often sent home, looking pale and nauseas, and promptly throws up and then curls up in bed. We will be picking this back up with his team again, once we’re over this episode, looking for some longer term solutions. 

In his usual style, Isaac took it all in his stride, pointing out his good veins for bloods, and we had a good giggle about a new dietician that he saw, who talks to him like he’s a pre-schooler (‘so Isaac, you able to swallow your tablets whole, how wonderful!’). Not quite sure how he would take the 50 odd tablets a day if he weren’t able to swallow them! 

Today is Anouk’s 12th Birthday - our lovely Shnuk’s, could not love her more ❤️❤️❤️

Frida, fundraising and health

Wowsers. Feeling pretty overwhelmed; from as far away as Mexico City, New York, India and Madrid, my amazing colleagues at the Press have been buying raffle tickets and donating money for my 500km walk challenge in droves. 

Can’t tell you all how much we appreciate all of this. These donations not only fund important research into Cystic Fibrosis, provide much needed grants for medical professionals to become specialists in CF, but also support people with CF, many of whom have been in isolation during this whole pandemic. 

Again, many hugs and thanks to Heather, Charlotte, Kay, Beth and Megan for organising the scavenger hunt at work, which leads us into the raffle celebration, and all the teams who are participating, we’ve been crying with laughter watching some of the videos coming in! And to our MD, Paul, for wanting this to still happen this year, bigger and better, despite all the complications. I am so, so grateful. Also to Vicky for the help with internal comms - I can’t quite believe that over the last 14 years we’ve gone from raffling off a few gifts from suppliers, to this huge event, that many of us in ELT look forward to each year. 

• For CUP friends only, to enter the raffle, please see internal comms, and remember not to include gift aid, as that contravenes gambling laws.
• For everyone else, to sponsor our 500 challenges, you can do so here (and add gift aid if you are a UK taxpayer). 

Right now, Isaac is stable. Lung function is no better, but no worse, and he continues to have problems with nausea and vomiting. But we have options, including another anti nausea drug to try, and all that said, he’s just completed what I suspect to be his first full week in school this term. He’s had a good stretch, for him, without IV antibiotics which is both reassuring.... and yet, feels risky going into the festive period. Being on home IVs for Christmas would be a shame, but an admission to the ward? Much worse; and we always have that risk at the back of our minds. For now, every good day deserves a mini celebration (tonight with kebabs). 

We’re not quite sure where on the priority list Isaac will be for COVID vaccine yet. Adults with CF are on the priority list, but as kids are doing so well, he is no longer identified as critically vulnerable according to his team. Which is a huge relief, but we still hope he can receive this vaccine soon, and we have no doubts about its safety, only fears that anti-vaxxer propaganda will mean people will not take it, and continue to put the vulnerable at risk. Having lost our Mamgu (Welsh Grandma) to COVID this week, who died in a care home, without her family, I urge everyone to read the science, not the sensationalist news and associated trolls. 

THANK YOU AND KEEP WELL x 

Feeling the Frida today; like our Mamgu, a strong and wonderful woman. Pictures also from her home, that I feel honoured to have visited.