Hey you.

Things are good here. Isaac is on some extra oral antibiotics (his lovely Cipro, which makes him so sun-sensitive he has a cute glow even in autumn) while we try and eek out some extra time before his next IVs. His lung function at clinic last week had dropped, but when we scrutinised the figures more, while his predicted % had decreased, his volume had increased, and it was only when we realised he had grown a whole 2cm in two months (!) that we figured out why (predicted lung volume/force is based on a number of factors; gender, age, height, weight). 

He is changing before our eyes, from our cute curly boy to our handsome man. And with these physical changes, come surprising mental ones too; I have found that my usual routine is becoming somewhat void of the levels of nagging previously required (um, wow!).... I make up his nebulisers, get out his tablets, prep his bi-pap and aerobika, he wonders down sleepy eyed, and now... he just kind of... DOES IT. There is still the occasional prompt (Deep breaths! Huffs! Neb!!!) but nothing on the scale of the last few years, and when I do prompt, it doesn’t result in a barrage of angry denials and arguments. In other words, he is getting on with his treatments and my life is feeling inexplicably easier, as feeling like a nag is a necessary but really quite shitty part of the role that we play. 

Honestly, however long this lasts, it’s like the best early Christmas present ever. When we holiday with friends and they saw his previous unlimited levels of procrastination, they would always ask us, why doesn’t he just get it done so he can get on with his day? And we would say, hell only knows! But finally, he seems to be getting this. So, CF friends, know that it can and does get better (at least at times). 

Some pictures from our recent happy adventures with some very lovely family. Love you so much guys x 

Feeling fortunate right now ourselves, but sending my love to so many friends who are having a much shittier time ❤️

Vaccines and herd immunity.

Vaccinations against life-threatening diseases are one of the greatest public health achievements in history. No shit, literally millions of premature deaths have been prevented, and yet while I am actively seeking out any kind of vaccine Isaac is eligible for (with CF, he can have more) I’m still reading crazy anti-vaccine rubbish in the media. 

The wonderful herd immunity that we have gotten used to in the developed world is now breaking down in some areas, because of these inaccurate stories of associated side effects. In reality you are ten times more likely to be struck by lightening than you are to have any kind of adverse affect from a vaccine that a doctor would actually diagnose it as such. 

If you are turning down childhood vaccines for your children based on these crackpot theories, rather than trusting the hundreds of thousands of expert doctors and scientists that endorse them? Then I’m not sure what more I can say to you. Sorry. 

Not quite on the same scale as rejecting the MMR for your baby, but you also meet the odd person who doesn’t just not worry about the flu jab, but who is actually against it, stating they have no need, or that it’s another cost the NHS doesn’t need. Yes, it’s the less well in our community, the diseased, the young, the elderly who are more likely to die from flu. But if you still think flu is a few days in bed watching Hallmark movies with a box of tissues, think again. Flu can be pretty random in who it kills. Getting vaccinated, if you can, will not only protect you, but helps provide herd immunity for those less well around you. And no, you cannot catch the flu from a jab, and yes, vaccine programmes cost, but not nearly as much as anti-virals or admissions for flu do.

Of course, I’m pro choice and all that. I mean, look how well Brexit is turning out for us, right? 

Mental health.

I meant to post during mental health awareness week (just a little late then...). If I had, I would have written about the fact that I too have suffered from mental ill-health, and it’s not something I shy from telling people. I no longer feel that shame that I did once, and that’s partly because I have seen and been inspired by a number of close friends and family who have suffered much worse (breakdowns, crippling panic attacks, stints in hospital sectioned) and come through that AND continue to be the funniest, smartest, and most wonderful people I know (that is fact, no exaggeration at all). 

My mantra when faced with the kids, how can I put it, challenging behaviour (in the case of Rosa this is a big fat fricking AHHHHH!?!! at times) has always been ‘everything is just a phase’, and occasionally, that can be true of adults too. It is for me, and I still now need to remind myself that how I am feeling one day won’t be forever. 

I hope that mental-health awareness week goes a long way to help others understand mental ill-health, and accept and embrace this in the same way they do physical ill-health. My own experience has not been a particularly dramatic one, but I respect my mental health now and try to take better care of my mind and body to keep myself well. 

In my hard partying twenties I once found myself sobbing, literally paralysed at the top of the stairs in a pub, irrationally feeling 100% sure if I delved down into the basement toilets I would be attacked (anxiety induced paranoia). 

I became pretty agoraphobic at college for a while, alongside a weird addiction to watching Dallas repeats on UK gold. 

Even now, I can understand the addictive lure of self harm to release stress (I resist now, but didn’t always). 

Today I rely on a daily dose of Citralopram to keep my anxiety on an even keel (for me, this works, and if it ain’t broke, don’t fix it!); 

I use alcohol to self medicate for stress on a pretty regular basis....(not recommending that one). 

Yet ask any of my friends, and I’m pretty sure they would tell you that I am one of the happiest, most chirpy people they know, most of the time. I think I balance hiding it well, whilst also confiding in my right people, when I need to (find your right people). At my saddest, I want to dig a hole, talk to no-one, listen to sad music very loudly with headphones on, non-stop, maybe walk to the river with my dog and morbidly stare at twigs and leaves for hours. 

But having a big family means I cannot hide for long, and this is both THE most annoying and yet THE most wonderful thing. It no doubt saves me. 

BUT this is me; a healthy Mum of three absolutely gorgeous children, with a job I love and some pretty kick-arse friends and family to boot. When I feel down, I feel guilty too, knowing all I know about how hard life with CF can be; I have to be able to pick myself up to show an example to Isaac, whose challenges, stresses and pains are a hundred-fold to any of mine (albeit I am his Mum, and I love him so much, his pain hurts me too). 

I am no expert, and but the one thing that I can share that always helps me is music. I have eclectic tastes (I make no apologies for a little prog-rock in there!) but in those moments, these are the kind of songs that help me most. I hope you like them. I’m not quite sure how this sharing playlists works, so if you click on the link and get loads of weird hip-hop, that will be Isaac, as we share an account. Anyway, let me know what you think, and also, what helps you. Because one thing is f’sho.... sharing helps peeps! Much love x 

PS: Spotify sometimes adds random tracks they think you might like to the end of playlists, to stop this, go to settings, and turn off auto play, I think that works. Unless you like random suggestions that is. I just don’t want them to add some Cliff Richards and for you to think it come from me 🤨

67

Next trek is booked! I will be walking a full 67km around the beautiful Surrey Hills in June 19, supported (in relay) by some fabulous friends and family to cheer me on. After Brecon Beacons, I said 50km was my limit, but what the hell, what’s another couple of hours with peripheral neuropathy so bad your feet feel like they are no longer housed in skin? 

You can sponsor us here (more on that later) but this post is to say we would LOVE more people to join us; so if you fancy challenge, a chance to get fitter, with THE best company, and all for great cause, PLEASE LET US KNOW. To find out more, and sign up to this or a number of other events in support of the Cystic Fibrosis Trust, see here.

Today in the sunlit woods. Happy weekend all x 

Lakes and growing up.

Isaac is having a really good week, much better than he has been in a long time. I post a lot less when he is well, always mean to, but we’re usually busy enjoying life. That said, I had a few weepy moments today; we cleared out his room of toys to give away or sell. Long overdue, as he hasn’t really played with Lego and the likes for ages, but hoobah doobah, the memories! My little boy is one big strapping lad now, and that is hard to get my head around. 

Here are some pictures (must confess, not mine, stunning aren’t they?) of some of the lakes where I walk Obie, just ten minutes from our house. 

Happy autumn all x 

A gene treatment?

Isaac has a fairly rare (severe) mutation of CF, which means he will not respond to the small molecule drugs that you may have heard about in the news of late, which should work for a wonderful 90% of people with CF. So what hope have we? 

“About 10 percent of all CF mutations don’t allow for any CFTR protein — working or not — to be produced at all. Compounds can’t correct or potentiate a protein that doesn’t exist, so an entirely new approach is needed — one that might fix the CFTR gene itself. Vertex may be “the CF company,” but gene editing is not its expertise. So, it teamed with two companies: CRISPR Therapeutics and Moderna Therapeutics. Scientists discovered in 2014 a way to harness CRISPR/Cas9 — a mechanism used by some bacteria to prevent infection by viruses — to cut unwanted DNA from organisms, raising the possibility of doing so in people. The field has exploded since, with researchers worldwide testing CRISPR’s limits against genetic and other diseases in a kind of “scientific frenzy,” according to The New York Times. Vertex sees CRISPR as a possible pair of “molecular scissors,” acting to remove a dysfunctional CTFR gene before it is replaced with a working one – effectively curing the disease.
But CRISPR trials in CF patients aren’t likely anytime soon. “[W]e’re up for the challenge. We are working on it,” Altshuler said. “But anyone who’s serious about it realizes that there’s a multi-component aspect.”As Altshuler explained, “The DNA piece, or the correcting-the-gene piece, is the easy part.”  The hard parts include editing to only affect targeted genes, while aiming at those in both mature lung cells and lung stem cells — which give rise to new cells — because cells in the lung turn over frequently. CRISPR is still in its infancy, and some scientists and bioethicists have already raised concerns about a limited understanding — and the possibility the tool might be more unpredictable than thought. A study in mice, published in the journal Nature Biotechnology in July, reported damage to genes beyond those targeted.A separate three-year research partnership was started in 2016 with Moderna Therapeutics, which specializes in a gene therapy approach targeting messenger RNA. Messenger RNA (mRNA) is involved in the transcription of genetic information, the first step in producing a protein.
“We hope it’s sooner, but we’ll work for 10 or 20 years to develop genetic therapies — or any other type of therapy — to treat those people living with CF who cannot take benefit from our small molecule or oral therapies,” Altshuler said. So, while its triple combinations appear to be zooming ahead, Vertex is with gene therapies about where it was in the early 1990s, screening for the small molecules that eventually became Kalydeco, Orkambi, and Symdeko.“I want to express … our deep commitment to completing our CF journey. We’ve made a lot of progress. We’re very proud of that,” Kewalramani said. “But we’re not going to rest until we get to all patients … our commitment is really unrelenting.”

It’s encouraging that they say they are committed, it really is. But also knowing the frustrations that come from NHS England and Vertex having yet to agree a deal which will allow people with CF access to one of their existing drugs (Orkambi) and the fact the ‘gene therapy’ was the buzz phrase when Isaac was born and yet still bears no fruit; it’s also a huge (head banging) frustration. These are as close to a cure that we can hope for right now. 

We were told when he was born that the earliest we could hope for access to a treatment that would work on the underlying cause of his disease (rather than just the symptoms) was about five years. We’re 13 years and counting now, trying to keep his lungs as healthy as we can so that the treatments, when they do come, may still work for him. 

So, I’m working on my next fundraising plan; we are bloody in this for the long term! Have a great day x 

This ain’t no lifestyle blog, dude!

Here are some pictures of our kitchen.....

Our front room....

And now the avocado on sourdough toast I had for supper...... 

KIDDING! As much as I do love our house (over the 12 years we have been here we have removed the faint smell of cat piss, re-plastered every wall, moved doors, extended twice, changed every flooring, and renovated the garage to make the kids a games room). We have built a home. It’s covered in art, piled high in books, scattered with discarded shoes (rarely paired) and littered with toys (and the latest ‘in’ toy? Slime). It may be a little grubby around the edges, but it is most definitely a home, a party place, a social hub, music always a tiny bit too loud. Put it this way, you walk into our house, and you get a pretty good idea of who we are and what we love. No beige, no neat piles of magazines, and no loo roll with folded sheets..... 

Anyway, I digress! My point was that this ain’t no lifestyle blog (if it were, I would have to tidy more, and with our lot, that’s not possible). This is the story of our lives with our sons Cystic Fibrosis. It can be merry, but more times than not; it is raw, it involves lots of sputum, it hurts, and as much as I try to put a positive spin on things, I don’t always feel that way. Maybe I’m trying to write ourselves out of the waves of negative, the pain, the nausea....

A year ago this week, Isaac got very sick. Weeks later he was still oxygen dependant, in a wheel chair, and scared that the effort to breathe might get too much. But we got him out of intensive care. We got him home. And he’s spent the last year fighting to get fitter than ever; 

...preparing for the next battle that we know with absolute certainty will come. 

He is so fit right now, and it takes a pretty rocking personality to take the knocks he takes, and get back up running like he does. Our little dude (I say little, but let’s face it, he’s near my height now). 

This other CF dude writes about life with CF in a super eloquent, moving, and very real way. He is post lung-transplant and doing well. So worth a read. His points about CF being an invisible disease are spot on; for every funny look we have been given as he coughs excessively, or when we leave pharmacy with not a bag, but a box load of drugs, jump the queue in X-ray, or when we allow him to add a tonne of salt to his food - please don’t stare people. Disease comes in all shapes and sizes. 

More on us soon x 

Happiness, flu jabs and another loss.

Clinic was pretty frickin positive...! LF was down slightly, but still bettter than it has been most of this year. For now we try extra oral antibiotics (a new one), a new steroid inhaler, and picking up his nasal rinses again (problems with his polyps/sinuses may be contributing to this). So no steroids, and no immediate IVs, for which we are very, very happy. 

It’s flu jab time of year again, and we will be first in the queue. Please remember, if you can get vaccinated, you’re defending not only yourself, but also reducing the spread of the virus and protecting those more vulnerable around you. (note: you cannot ‘catch flu’ from a vaccine, it’s not live, and these days, you can often choose between a nasal spray and the jab). 

Do this. 

Give blood. 

Sign up for organ donation and make your family aware of your wishes. 

At least that’s what I think, but who I am to say?

"Life isn't about being happy, life is a roller coaster of crazy emotions. One second you're fine, and the next second you feel lonely and despair and like nothing is ever going to be okay again. It's not about emotions, it's not about how you feel second to second. It's about what you're making with your life, and whether you can find a deep pride in who you are and what you've given. Because that is so much more impactful, so much deeper than whether you're happy or content or joyful. It's okay to feel pain." - 

- Claire Wineland, an insanely uplifting and beautiful person, who died this month, following CF post transplant complications. This disease is so fucking cruel. Everyone should watch this. x 

Roid fears.

The cough continues, with a scattering of tummy issues thrown in for good measure. CF clinic tomorrow. Hoping for answers and treatment, but please god.... not steroids. I’ve posted previously here about his ‘roid rage. We’re also only six weeks post IVs, so hoping for a longer break from those too.... for his sanity, ours, and his poor battered kidneys. Of course, more than anything, we just want him well. More soon x 

Rhyme or reason

I wrote a long post, and then lost it! DRRRRR. So here is a shorter and much less eloquent update;

Isaac is coughing. 

A lot. 

Like, people stare at you in Tesco, coughing over the veggie aisle, and sleeping is miserable type coughing.

But when we took him in last week for an emergency review, he blew his best lung function in a year!

This, after it being at ‘stable-but-quite-shitty’ level for some months, even when he appeared well.

CF continues to surprise us. 

Occasionally, how symptomatic he appears has no rhyme or reason with his lung function, supposedly the measure of wellness in CF.

Treatment is Azithro and brufen for inflammation, as the doctors feel after a year of intensive treatment of his infections, the coughing now may be due to an overly excited inflammatory response (calm lungs, calm!).  

In CF, infection and inflammation form a vicious circle in the lungs, and if this treatment doesn’t work, he may need steroids (always the last option with him, due to the side effects).

As is his norm, Isaac is taking it on the chin, smiling on, and not letting anything hold him back.

…. Not many people get to live with their hero, but I do. He never ceases to amaze me with his courage, humour and tolerance. 

Some pictures from my travels to Toulouse (holiday) and Madrid (work) recently….

More soon x

Holiday and home.

We made it. He made it! On day three of the holiday, hearing him rattle and cough, I was sure that I would be booking flights home for him and I, to head straight back to hospital. But the next day, things improved, and the next, and the next. Was the Cipro working, when the IVs did not? (they are still treating the same bug, only less powerfully, so no reason why they should). Did he just need that extra time after the IVs? Was the hours of swimming and running around with his friends, the sunshine (the joy?) helping him clear his chest, while the hospital physios could not? Are we even treating the right bug? We know he is colonised with PseudoA, but he may still have something new, that we’re missing. Despite the IVs, a three week break, IVs again, followed by two weeks on Cipro, I’m no more confident now that his lung function will have improved than I was at the beginning of summer. It’s not like BLACK OCTOBER, by any means, but he is not right, and for whatever reason, he doesn’t seem to be shaking this one just yet. A positive test (for a new bug, CFRD or another problem) would suck, but so does not getting any better, without really knowing why. Stable but not great is still no place I want him to be. 

All that said, France was full of love, and fun, and not a sniff of WiFi! Also, lots of art for me, I will share more in later posts. 

Hope you’ve all had a wonderful summer x 

These three 😍; 

Vacances et espoir!

We will go on our holiday to the South of France with our exceptionally lovely friends. His IVs will finish tomorrow, and after his late dose I will de-access him myself (remove the needle from his port). He will be able to swim, have fun with his friends, eat fresh baguettes and pan-au-chocs from the boulangerie, sleep in the villas dorm with all the boys, feast on BBQ every single day... and be a kid. 

I wish I could tell you that all the news was good today, but his lung function was down again, and he’s still not right. 

Normally at this point his IVs would continue for another week, and they would continue to review him every few days. Instead we get to go away on the proviso he swaps immediately to the strongest oral antibiotics he can take. Good news but they do make him super sensitive to the sun (he burns in winter on this shit) so I’ve been panic buying SPF50 rash vests. He will continue with his six daily nebulisers, increased physio, and if he gets any worse; we will come home early. If he remains the same (coughing lots and very productive, which is CF talk for bringing up lots of icky sputum) we call the minute we get back and head straight back in to hospital. 

Our MDT (multi-disciplinary-team) values his quality of life as much as we do, and it’s forever a balancing act between this and treatment. They praised him today on how much he has been putting into staying as well as he can. He continues to complain very little, so how can we?

A cough-free, worry-free two weeks in the sun might not be possible, but we will definitely have as much fun as humanly possible. 

Avec beaucoup d'amour x 

PS. I’ve been asked to add for all those budding burglars, we have dog and house sitters in situ, but thanks for thinking of us 😘

Clinic review tomorrow

This will determine if Isaac is well enough for our holiday. I swing from utter confidence (as his chest is clearer, and he looks well) to total panic (as his huff still rattles). He is better, but maybe not well enough. A normal course of IV antibiotics is a minimum two weeks, but he’s often had three, even four weeks to get well. This will have been just eleven days. Stopping too early may give the bugs the chance to fight back before we fully squash them, but on the otherhand, if his lung function is not improved tomorrow, then we might be looking at a new bug and these might not be the right antibiotics anyway (tests still pending). 

All that said, we are still full of hope. Even if we have to miss the beginning of the holiday, all will not be lost. We can catch them up by train or plane. Without wanting to sound all Starship 1986.... nothings gonna stop us now.....he will get the holiday he so much deserves. We will make sure of that. 

Wishing you all lovely summer fabulousness x 

Little Anouk with her cousins x 

Some news

Good news and bad, or at least the not so good, and for now, I’ll take that 😊

We are home! For the weekend at least. Not much happens on the ward over the weekend. Physios come less. Doctors make few decisions without a consultant, so it always feels like limbo time. Therefore we’re allowed home on IVs again. Either until next Thursday, when he will be reviewed in clinic, or Monday if he becomes any less well. 

His blood infection markers are at 80. Compare this to Black October, when they were over 1000. This is GOOOOOD! 

Although we got home at 3.30 today, he still needs to be back on the ward at 8pm for a blood test (they have to regularly review the antibiotic levels in his blood, as too high risks kidney, liver and hearing damage). Still, should be a flying visit. 

Isaac is very tired following a sleep free night on the ward and needs to catch up. 

His lung function today was no better or worse than when he started IVs on Monday. But worryingly he continues to cough, rattle and roll; a lot, and struggle with exercise. This is not necessarily unusual though, just five days into IVs, and hopefully we will see a big improvement over the next week. And despite him seeming more symptomatic (coughing up lots of gunk) his LF was nowhere near as low as I had feared to begin with. 

They have confirmed that once well enough they will do a three-day continuous blood glucose monitoring for him, to properly access whether his is diabetic too now. He is drinking about 8 litres a day right now, which is not a risk, but could be a sign. 

We’re still waiting for other test results. Frustrating as we can’t be sure until then that he is on the correct antibiotics. But this is not because of any kind of tardiness, it’s simply that his sputum cultures need time to grow in the lab for them to identify what exact bacteria he is growing. 

We’re so so glad to be home, but going away on holiday won’t be a sure thing until we know how he is/what he is growing in his lungs. We won’t know for certain until we’re just about to leave. 

Anouk says things like “why him”, “why us” as she struggles to understand (she is, by the way, the kindest, most empathetic kid you will ever meet). Tomorrow I should have been dancing along to Patti Smith at a festival, but won’t be as I need to be here. But why not us? And above all, we are a team. I will never let him do this all alone. 

Finding the car

Every single bloody time I park during an admission, I swear to myself that I’ll remember where in the seven storey car park I’ve parked, but after days on the ward, never do! I am eventually home for one night with Rosa (Anouk is away with my sister, and I can’t tell you how much I miss her, but I’m glad that she is having fun. She is a worrier and hates our family being apart, so it is wonderful that she has a distraction this time).

Isaac is slowly improving we think. 

I sat in the garden by the gym earlier while he was doing physio and reminded myself that this is not like ‘that’ admission back in October. Let’s call it BLACK OCTOBER. He is not struggling to walk a few steps in that garden on oxygen, he is not in a wheelchair.... he CAN breathe. It was a timely reminder that things could be a lot worse. 

But he continues to cough, a lot. Coughing like that hurts. His throat hurts, his ribs, his back. Only a CF parent will understand this, but I’ve been delighted by the sputum specimens we have been able to send to the lab, the ickiest in a long time - if there is any other shitty bacteria hiding in there, we will find out now, and then aim some big guns at it! 

Isaac, as usual, is mostly in good spirits. He had his six minute test yesterday (they test at the beginning and end of each admission how far he can run between two markers in six minutes) and he tried so hard, but was so breathless. The physios have him in the gym twice a day which helps him shift the mucous from his chest. We normally protest to do IVs at home as soon as possible very loudly, but this time, I know it’s the right thing to be on the ward. The team all continue to aim to get him well enough for our holiday, and I’m feeling more confident now, albeit for the test results. I’ve asked about continuing IVs myself in France but it’s not an option, for risk of sudden reactions and being so far from home. 

As happy as I am that we should (we hope) get to go on holiday, I’d be lying if I didn’t admit that this admission (after IVs in August, October, January, March, July and July again) didn't worry me. Hopefully the tests he is having this week will help us work out why his lungs are having such a bad time. To put it into perspective, that is six (some very long) courses of strong IV antibiotics a year, when previously, he typically had one to two. 

Thank you so much for all the kind offers of help from our nearest and dearest, of which we are spoilt. Good news to follow we hope x 

Hospital and hoping for holidays...

Isaac was admitted to hospital today. He is exhausted and coughing, but otherwise OK. His sputum is noticeably ickier than his norm. As usual, he amazes us with his chilled and accepting attitude, but this time he is worried as we go on holiday soon and so have a time limit to get him better, or risk missing out. Kick arse IVs and intensive physio is our plan A to get him there. I can’t tell you how crushed he would be (our first big holiday in two years) not to mention the girls if they had to go ahead without him. As we are driving (South of France) our plan B would be for me and him to fly down and catch them up when he’s well enough, if that’s even affordable/possible. 

Sometimes I feel like I’m not, not coping, but neither am I coping. Reading that sentence back, I’m thinking this is a sign of some pretty fucking muddled thinking! It’s certainly pretty hard not to get angry that he has to go through so much. On admission today I went through his list of medicines with a doctor, a nurse and then a pharmacist (we always have to do this, I’m never quite sure why so many times). The pharmacist placed her hand on my arm as I listed them off; so many drugs (and that’s without the IVs) why aren’t they keeping him well??? It’s been just three weeks since his last IVs. He needs a break. 

We went to clinic this morning for lung function tests, but as there was no doctor available there, we then had to be admitted via A&E, where we stood in a queue for 45 minutes with a guy in serious chest pain, a drunk woman who vomited on the floor, and a woman with a teeny tiny cut on the top of her head. Thank god for triage. In the end, it was all pointless anyway as the doctor never even came, they just phoned down to A&E to say Isaac was to be admitted as his lung function had fallen significantly and he so obviously needed treatment. NHS I love you, but the crazy bureaucracy less so.

Please send well wishes x 

Sick sick....

The first week of the school holidays, and come with it... a fucking CF exacerbation. 

Coughing. Wheezing. Walking around in a dressing gown, gripping a hot water bottle, in 30 degree heat. Napping. Stairs suddenly feel like a challenge, and huffs with physio sound like they traverse through treacle..... 

This disease holds my son hostage, and I hate it. 

We’re only three weeks clear of his last IVs, I don’t think he’s ever sounded so bad, so quickly. Plus he’s trying to hide how bad he feels as he knows that we go on holiday soon, and he fears another admission. 

We need him better. 

Rothko blue, because I’m blue, and a Miro, because, everyone needs a Miro. 

Cystic Fibrosis FAQs

What is a portacath? Isaac had a portacath surgically implanted in his chest wall earlier this year, the aim being easy venous access for regular IV treatment (IV being intravenous, as in, drugs delivered directly into his blood stream). 

Regular cannula/long line/PICC line access over the years has wrecked his veins, and it became harder and harder to find a viable vein each time (veins repeatedly used would just collapse resulting in multiple needles, and often, delayed treatment when he needed it most). We had an agonising wait in HDU (high dependency unit) once, and delayed antibiotics give bugs a chance to fight back. Not good. 

The port meant surgery, always advisable to avoid, but the hope is that this will last him 5-10 years before replacement. Cancer patients often have a port for for chemotherapy. 

He still requires a needle to access it for use, but the key difference is trying to guide a line up a long, thin, windy and collapsible vein, usually blind (they can use ultrasound, but this is unusual) which often fails and needs repeated attempts, compared to a needle plopped right into a kind of rubber bung with a hole in the centre, and ta dah... you have accessed the (pre-accessed) vein. 

What does having a port mean to Isaac? On the whole, it’s great. When his port is not accessed you can see only a smallish bump under his skin in his chest (with one scar nearby and a second by his neck, from the surgery). When it is accessed for treatment, he has a needle in for the whole course (usually 2-3 weeks at a time) and cannot get this wet, so no swimming or showers. The rest of the time, it’s life as normal, aside from avoiding full contact sports like rugby, which might damage the port. 

In between treatments, the port is accessed every month for a flush to keep it clear. So it does still involve regular needles, but compared to cannulas, long lines and PICC lines, overall, it is a helluva lot less stabs! 

What would a Transplant mean to Isaac? CF is a multi factor disease, it affects mainly his lungs, pancreas, liver, sinuses, and digestive system. A lung transplant would only cure the disease in his lungs. But this is also where the biggest risk to life is (90% of people with CF die from lung disease). 

After transplant, people with CF no longer have CF lungs. However, infections in their sinuses may re-infect their lungs, meaning they need to carry on with some traditional CF lung treatments (but not all, and maybe very few). 

Digestive health is unaffected by lung transplant, for example, Isaac will always need to take Creon in order to digest fats and proteins (pancreatic enzyme replacement meds, of which he takes about 50 capsules a day) and will continue to be at risk of CF related osteoporosis, diabetes, some cancers and liver disease. 

Why not transplant now? You will hear me shout loudly on this blog about organ donation and the fact that we have chronic shortage of donors. 

1 in 3 people on the waiting list for lungs dies waiting

I have often been asked why Isaac cannot be put forward for transplant now? The good news is; he is too well. The main test of wellness is a lung function test. His FEV (forced expiratory volume) is measured regularly and forms part of the picture on which his team plan his treatment. I don’t like to post much about his FEV, as I feel it becomes a focus on a number which is only part of a much bigger picture. Plus it fluctuates widely, at his worst (while in intensive care) he blew a 17%. Needless to say, his baseline is never as high as I would like it to be, but also, it’s not near the point where transplantation would be considered (regularly less than 30%). 

More importantly, transplantation is not a cure; the chances of surviving the surgery for a year is only 80% and surviving 5 years 60%. This is a fast changing statistic, as development in anti-rejection therapies improve. Transplant is truly amazing, and life changing for many, but it’s also like swapping one disease for another, and further, a whole new set of not so fun side effects. 

More FAQ’s to follow. Any questions, because medical jargon slips into my vocabulary pretty quickly, please do ask x 

A squash and a squeeze

For anyone who has read this book, the way the old lady feels when she gets her house back? Sums up how we feel post home IVs...

His daily treatments (tablets, nebulisers, physio) which usually feel half defeating to get done, suddenly feel SO simple after two weeks of very early starts, gloves, aprons, mixing up antibiotics and syringe driver pumps....  So here is to feeling thankful, de-accessed, hardly coughing, and our boy getting his summer fun back on track. 

I’m going to post some FAQ’s soon, about CF. All questions welcome. Have a great day x 

NHS England deny Orkambi access

Today NHS England stated, again, that Orkambi is too expensive to fund for sufferers of Cystic Fibrosis, despite a promising pipeline deal proposed which would mean earlier access for people with CF to drugs still under development. See here for more details, and here for how a young girl with CF eloquently puts this frustration across. 

Last year, half of all people who died with CF were under the age of 31. Sorry to be so blunt. Really. If I could avoid the statistics, I would. But in the last year Isaac has had 5 courses of IV antibiotics for 2-4 weeks at a time, 10 weeks in hospital, 4 operations, countless tests and clinic visits, and continues to take nebulised drugs every single day which cost the NHS thousands of pounds every year. I am not saying this is a moany way, we are so so grateful to the NHS for the treatment that he receives, and the life it allows him to live. We really do consider ourselves lucky. 

Orkambi will not work for Isaac’s mutation, but I hope one day there will be a precision drug that will, and while the cost for these initially seems astronomical (Orkambi costs around £100K per annum, per patient), I do believe there is a saving for the NHS in terms of keeping people with CF well and out of hospital, which these drugs really have the capacity to do, and the pipeline deal would guarantee access to future drugs hopefully even more effective than Orkambi. 

I’ve long been gutted that Isaac’s rare mutation means we need to wait longer for these kind of treatments (they are also an oral drug, no IVs, no nebs, Isaac’s dream come true!) in a sad way I’m glad that Orkambi won’t work for him, because knowing that a treatment was there, approved, and waiting on a shelf for him, and not being able to reach it would drive me beyond crazy. I honestly don’t know how I would cope. 

If you are in England, and could find the time to help, please download the template found here and send it to your MP, which you can find here. It would mean so much to me if you could. 

Things here are OK. We had a wobble last night as he had a mild fever and vomited. We have to be very cautious about infection risk in his port on IVs. Thankfully this morning he was back to his usual self. Despite home IVs, we’re just really thankful to not be in hospital for this course, which we hope will end on Tuesday (with a cool swim for him) all going well with lung function tests. 

Have a great day x 

Heat wave and wild woods

Isaac is doing really well, frustrated in the heat, port accessed and unable to swim, but coughing much less. Bloods have been OK (the blood fairies come every three days to check his Tobi levels, as his kidneys can suffer from the toxicity). Angry earlier, he asked why he can’t have a transplant already. He is sick and tired of the treatments; as soon as his hour hooked up to the IVs is done... he still needs to do his nebulisers and physio. There is no holiday from CF treatments. In this heat he walks round, top off, port accessed and IV line dangling on his chest, a very visible sign of disease, but I’m proud he doesn’t care. 

This weekend a little baby in our family gained his angel wings, far too soon. Little lion Leo. We are thinking of you all, Sian, heartbroken. 

Some pictures from Wild Wood last night, basically a rave in the woods. A very welcome blow out, with some beautiful friends, without whom I would cope a lot less well. Thank you lovelies x