It’s Christmas!

We are home. We are well - the best gift I could hope for. We are very merry in the Moly household. 

Thank you for reading, and wishing you all a happy and healthy break if you are having one x 

Have some pictures of Isaac and his lovely cousin Ben. How they change! A constant reminder of the seasons of our lives. We are big fans of cousins in this house, and after years of only a few, the kids now have a new influx of babies to love on, and this Christmas will be all the more special because of them. Merry Christmas babies! 

Happy tweens, grumpy teens! 

Bonds between people who have not, and cannot ever meet.

Isaac is doing really well. One week into IVs and his lung function which was down 20% on his baseline (already not great) had increased 13%, which for one week on is fabulous. So for now, we continue home IVs, and all going well (symptomatically) they will end next week - perfect timing for Christmas. 

Good news for us, but sad news from the wider CF community this week. This is a unique community in that we cannot meet in person without high risk of cross infection (...pretty sure this is unique, as I’ve not heard of it for any other chronic illness? But I could be wrong, t’is not rare!). The bugs which inhabit CF lungs so merrily come in many strains, but from just a few species. They realised back in the eighties that certain strains were prevalent in certain CF centres leading in one case (the Liverpool strain of Pseudomonas) to many deaths. And so came about the end of CF holiday camps, sitting in waiting rooms with other patients, and the start of segregated clinics and beds on the ward so far apart you would never see the other patient. The closest we come to another CF patient in person these days is a knowing smile and a wave across the hospital concourse. As a result, the internet provides those affected by CF our only means in which share knowledge and learn from others who really understand life with CF and what that entails. In those early years, getting over diagnosis, the CF parents forum was my lifeline (shout out to Softie, a kind a generous soul who had CF himself, but spent much of his life advising us parents of newly diagnosed kids from the risks of sandpits, to how to sterilise neb parts, to how to cope with questions from others such as the common ‘that’s the one where you bash them, right?’). 

And as in any community, there are some faces that stand out, the social media ⭐️’s of CF so to speak, and this week we face yet another death of a young activist, advocate, and generally lovely person. In my time as part of this community, I have known far too many die (far too soon) and while CF affects sufferers in very different ways, these losses have broadly fitted into three kinds; 

1) Those who decline in health, usually in their teens or twenties, and are listed for a lung transplant. 

Of these, there are the in the 1 in 3 patients who tragically die waiting for lungs due to a shortage of willing donors (please sign up here ). 

Or those who do get a chance at a new life with new lungs, but die following complications post transplant (as with my friend Jayne. Who I miss so much). 

Or those who initially do well with new lungs, but who later suffer from rejection (as was the case for beautiful Eva, who inspired so many). 

2) Then there are those who decline in health, usually in their teens or twenties, for whom transplant may later become an option, but who tragically die from an infection very suddenly. I am not sure of the details, but in Lea’s last vlog she casually mentions she is starting a new IV drug for her current infection, pretty normal CF stuff, and yet a week or so later, she has passed. 

3) Those who decline in health more gently, and may survive their twenties, thirties, even forties before being listed for transplant (if they even opt for transplant). Or have a transplant earlier, and avoid rejection for many good years. 

Now, it makes perfect sense that when you are at your sickest, you’re much more likely to be home, alone more, and online. Whereas when you are well, you’re more likely to be out and enjoying life too much to be online, and so the internet can skew perceptions. I get this. But one thing is for sure, I know of many, many more in the first two categories than I do the last; and this can be pretty terrifying (‘sudden decline’ especially after our stint on intensive care last year. Too close. Too real). 

While the internet gives us an amazing forum in which to talk about our experiences, and share fears and triumphs with the only people who really understand, it also creates bonds between people who have not, and cannot ever meet. I would not be without my CF community, but with this comes pain and incredible heartache too, despite us never having met.

I shall miss your smile Lea. Sleep well x 

To support our fundraising for the Cystic Fibrosis Trust who fund seriously groundbreaking research, please click here. CF is actually one of the areas of medicine where a cure is a realistic idea, but they lack the funding to achieve this potential. As such, myself and a bunch of my amazing friends will walking the Great Strides walk in the Surrey Hills in June 19. For me, a bonkers 67km in a single day 😬 x 

Lakes, walks, coughs.

Today I walked round the lakes with Obie, headphones on, music loud. Cleared my head some, as I was feeling pretty miserable when I woke. Although I’ve gradually moved his IV times so they are now a reasonable 7am and 7pm, I keep waking up at 5am, irrationally fretting until 7am that I might sleep through my alarm (which I never do).  

Isaac is a smidgen better. Still coughing but he hasn’t been sick since Friday (he coughs and coughs and coughs so hard he is sick when he is this ill). Should be a good sign that he is turning a corner, but not as well yet as I hoped he would be by now on IVs. He has repeat lung function tests Wednesday. We need him better by then, otherwise we could be looking at an admission. 

The deal I have with Isaac about this blog is that he doesn’t care what I write (he agrees awareness is a good thing) but he doesn’t like pictures of him looking sick (fair enough), but if you would like a better idea of what a CF cough can be like, you can check out this couple who vlog daily about life with CF. Gives you a fair idea of what Isaac’s mornings look like right now.

My walk today:

The far reaching affects of Cystic Fibrosis; 



Organised me a new IVs cupboard (this makes me very happy): 

Hoping to write a jolly Christmas blog soon x 

No better.

At least not yet. He is still coughing so much he is sick in the mornings. Hoping this is just the bacteria-busting super powers of ceftazidime and tobramycin doing their shit before a much needed ceasefire in his lungs. Pseudomonas, we know you’ve set up a permenant camp in there, but hell, you need to know your place! 

Perspective

Children with cerebral palsy lick milk powder off a bed to feed. Image credits: Lu Guang

Qi Guihua, held here by her husband, fell ill when she returned to the village from Beijing to celebrate the Spring Festival. She died two hours after this photograph was taken. Image credits: Lu Guang. 

Drugs and beds.

Unsurprisingly Isaac started IV antibiotics today. He is no better, and his lung function had dropped a further 20% in two weeks. 

In true Isaac style, despite coughing and spluttering throughout clinic, and giving them a deliciously juicy sputum sample for the lab, when they asked him how he felt, he just replied ‘Alright I suppose. Yeah, fine’. He usually has cold spray (numbs the skin) to access his port, but for the needle today he chose to have nothing, and hardly flinched. If he could have carried on watching you tube he would have. He amazes me daily. 

But the wards are full. There is no room at the inn.... HA! This shouldn’t please us, but it does. We can stay home and I’ll do his IVs here, and this makes us very very happy. They would rather have us in, for the physios to put him through his paces, but we can and will do just as much here (and avoid picking up more bugs on the ward). Administering his drugs myself is tiring (I’ll be up at 5am, to glove up and mix drugs) but for Ise, it means he can sleep through the doses while I creep around his bedroom in my plastic apron. I cannot fully explain to you how much less exhausting, frustrating and life intrusive this is for us than being on the ward. 

Best case, we do two weeks of IVs at home. His lung function improves, and most importantly, he starts to feel better. Then ho ho ho we can hope for a wonderful and well Christmas. However, if he doesn’t improve pretty quickly, feels any worse, or his labs show he is growing something other than his norm (PseudoA) he is guaranteed an emergency bed as that would mean drugs or treatment that we cannot give him at home. He really really doesn’t want to go in, especially so close to Christmas. Please let this work x 

Still home. Still sick.

Apologies to anyone who read the last post and worried. We are still home. Saturday was awful (I can’t quite explain how utterly heartbreaking it is to watch your child cough (and be sick) again and again and again, day and night. To have nothing to soothe their rib pain, the head pounding, the sore throat...) but Isaac stood his ground in light of his many many previous experiences in A&E, and insisted we wait before going in. Credit to him, he knows his mind and body, and Sunday he was coughing a little less, and so by then it seemed best to wait for Monday to be reviewed by his own doctors. 

But come this morning (Monday) we were told the soonest he could be seen would be tomorrow anyway. The inn is full. The wards are packed, and after talking to his new CF nurse it sounds like our only way to be seen by a respiratory doctor in future is on clinic days (Tuesdays and Thursdays) or through A&E. This is new and miserable news for us; we used to be able to call any time Mon-Fri and one of his team would always be able to fit in a review if he was unwell. So short of fitting his disease around clinic days, we need to sit on symptoms or brave the contagious in A&E from now on. 

I’m sorry, I’m tired, I’m angry; I’m not blaming anyone, but I’m sad (and mad) at the state of our beloved NHS. 

Clinic first thing tomorrow. 

The drugs don’t work/It’s beginning to look a lot like....

..... Hospital. 

We’ve come to the end of two weeks of Cipro, and he is no better. He came home sick from school yesterday, O2 saturations around 94 (anything below 93/94 and he really should be on some oxygen). He coughed and slept his way through the night, and this morning I was ready to pack our bags to go. He coughed so much he filled three cereal bowls with sputum and coughing so hard he threw up. 

Since then his sats have drifted between 90 and 98. As it’s the weekend, our only option is to go through A&E. Although he has this ‘open access’ to hospital, it’s kind of meaningless as he still needs to be reviewed by a doctor to be admitted. 

What haunts me is that we had a similar weekend last October when he became much more symptomatic on a Friday, we thought about A&E on the Saturday, he seemed slightly better Sunday, so we eek’d it out until Monday morning to see his own Dr and go straight from clinic to the ward. Whether things might have been different had we taken him in sooner, I don’t know, but by the Thursday he was in intensive care. 

So why are we sat here at home still? 

Isaac is adamant that he doesn’t want to go to A&E, and he increasingly wants to have a say in his care. I half convinced him earlier, and then his sats said 98 and he told me ‘see. I’m fine!’ between coughs. Last time we were there, we sat for hours, with the contagious (so not good) the drunks, and arguably worse? The people with a stubbed toe or a sore throat (thank fuck for triage). Plus, he argues that everyone at school is sick with the same thing, so it must be viral. True, but his friends don’t also have CF, they don’t have extra sticky mucous they can’t clear, and they won’t have an army of Pseudomonas bacteria already living in their lungs, getting ready for the party a virus lays out for them in his weakened lungs. 

I called clinic Thursday and they said they would get back to me. I knew the Cipro would run out tomorrow morning (Sunday) and it already looked like a bad idea to stop the one thing that might just help a bit until he gets on IV antibiotics, so at the very least, I wanted a prescription for more. But despite calling again and leaving messages three times, I heard nothing back Friday either. This is not like our team, and is most likely a sign the wards are full anyway. 

What we need is 24 hour wraparound care with the team that knows him. What we have is a weekend with limited access to consultants, a hugely overburdened A&E system, full of strangers to him and his health. We’ve been admitted before at weekends only for them to put off treatment until he could see his own team on the Monday anyway, as CF is complex, and registrars are reluctant to make decisions.  

He is asleep right now (4pm, and this is the kid who never naps). I’m sat here listening to his every breath/cough, ready to argue that we have to go in when he wakes up. This hurts. I can’t tell you how much I hate CF. 

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