CF dominoes

Isaac was diagnosed with CF when he was three weeks old, following a standard heel prick test at five days old. The test was repeated at 10 days old and we were told this was because of an unusual result, which was probably a false positive. All would be groovy. But those days that fell between were the hardest days of our lives so far; The not knowing was hell; I was 27, and Dan a year older. We were first time parents, and really had little to no experience of babies full stop. We spent days second guessing symptoms that we felt he may or may not have, but had no idea of 'normal' to compare this to. People often assume there must be a family history of CF, but this is rarely the case; it affects one in 2500 live births, one in 25 people are carriers of the recessive mutation, and if both parents are carriers, there is a one in four chance of the child having CF. Genetics lesson over; In the end, I think we knew before we were even told (by accident by a health visitor, over the phone, while I was home alone and Dan out playing football. I understand how we use the word 'crumble' now, because that's exactly what I did, in a heap of tears on the floor). I remember very little of that whole time but this horrible sense of falling into a dark hole. Heartbroken and yet completely in love with our little boy, and a huge sense of WHY HIM? AND NOT US? And then, a little later, an OK, bring it on! Let's get him well, learn all we can, and be his biggest champions. 

As people often ask me (especially those terrified few, awaiting a diagnosis like we were once), his early symptoms were; No weight gain, salty skin, green and oily stools, and his very first poo (which we had been told by other parents would be like tar) rolled out of his nappy like a golf ball after many hours of screaming (this was our first night home with him, and we had no idea how to console him, and assumed we were just rubbish parents. A midwife we only refer to as Mary Poppins came in the morning, and when she reassured us that he was OK, I wept in her lap. Only he wasn't OK.) And later, a persistent cough. 
At 8 weeks old he caught Bronchiolitis, a viral lung infection. This was not due to his CF, just shitty luck. But his CF did mean that it took him longer to bounce back, and he needed more aggressive treatment to recover. He was so so tiny, terribly underweight, and needed IV antibiotics and O2. We were in hospital for three weeks, and then needed to be seen in CF clinic weekly for some months. At 8 months old he caught it again. This time it took even longer, and we were in and out for some months, after a Bronchoscopy (camera into the lungs) made him more unwell. 
But the point I am (very slowly) getting to is that apart from the Bronchiolitis, and a milk protein intolerance when he was a baby, which were just bad luck, Isaac has had a numerous other problems, and people often ask me if these are CF related, and the answer is, probably yes. 
The gene affected by the Cystic Fibrosis mutation controls the movement of salt and water in and out of cells. People with CF experience a build-up of thick sticky mucus in the lungs, digestive system and other organs, causing a wide range of challenging symptoms affecting the entire body. For Isaac, this has led to problems with; 

• Delayed growth (no longer a problem, in fact, we're currently rationing his Easter eggs!)

• Many lung infections, and most notably, colonisation of the evil bug Pseudomonas Auriginosa (meaning this bug has set up camps permanently in his little lungs... little bastards)

• Reflux (which in turn, infects his lungs)

• DIOS (Distel Intestinal Obstructive Syndrome)

• Nausea and vomiting issues

• Pancreatic Insufficiency (the duct from his pancreas was blocked by mucous in utero, and so the enzymes which breakdown fats and proteins in his food never reach his intestines, meaning he needs to take medication with everything he eats, while the pancreatic enzymes themselves attack his own pancreas)

• Decreased bone density

• Nasal polyps and sinus problems

• Immune deficiencies

• Posture problems

• The many weird and wonderful side effects of his many medications

Also, due to the number of medical tests that he has due to his CF, he has had investigations into scoliosis, heart scans, spine scans, simply because if doctors look at a body long enough, they'll spot a potential abnormality (all thankfully negative in his case). 
The things he has yet to experience, but probably will at some point include; infertility (he can have children through IVF only), liver disease, diabetes, osteoporosis, hearing and kidney problems (due to huge antibiotic use), and most likely at some point....lung transplantation. 
I guess the point I am eventually going to make is, that if you have a problem at such a level, in each cell, then the domino effect of that one mutation can be vast. Add to that the side effects of the many medications (high dose antibiotics and steroids especially), then you might as well sign up for an unfeasibly expensive annual pass at your hospital car park. That one tiny mutation, causing a thousand small problems. Like falling dominoes. 
And this is why we need treatments that are directed at the actual underlying cause of CF; that movement of salt and water between cells, rather than treating only the symptoms of the disease. 
There are two such drugs available to people with CF, Kalydeco and Orkambi. Each drug works only for a certain class of mutations (there are over 1000 CF mutations, but some are very common, others, very very rare). Kalydeco is available to the 5% of patients it works for in the UK now, but Orkambi is not, due to the cost. This would help delay the progression of the disease in some 40% of patients. Please sign this petition  to help make it available to all who need it. 
For us, our wait continues, as Isaac has some rare, class 1 (the most severe, they say) mutations which neither drug caters for, but it will come, and hell, we need to make it affordable for the NHS when it does. 

Freckles and noses.

It's freckle season around here, when the first few rays of sunshine splatter them over our faces. They have not inherited my auburn hair, but they did get the pale skin (poor kids) and freckles - Isaac and Rosa especially. I do love a freckle face. 

We're trying to get summer ready; packing the sun cream, planting the veggies, and exercising more. Dan and I are going for a very rare kid-free weekend soon to see a friend in Croatia and celebrate his 40th. This involves a boat trip around the coast.... the stuff of parental dreams! In my mind I see tanned, bikini clad women in nice sandals, sipping cocktails, dancing, and diving effortlessly into the sea. In reality I will be more white/blue skin, covered in factor 50, in an oversized T to hide the squishy belly, in tatty flip flops (but I'll take the cocktails, dancing and swimming anyway, 'cos I'm that kinda girl). I have a month to get (kind of) boat ready, and that involves getting the kids even more active too. Great stuff!

Isaac is doing well. He spent the first week of holidays at a CUFC day camp. He has a CT scan scheduled in a couple of weeks. The problems with his nose (CF related Polyps etc) are somewhat worse than I had hoped, and surgery is the only option. But he is keen, and the consultant was very encouraging. He is confident that Ise will have a massive improvement (as in... be able to breathe through his nose again). We're just hoping this can happen sooner rather than later now. It will no doubt mean more time off school (his attendance this year so far is at 83%). I must confess a hugely unprofessional (if being a CF Mum were a job) giggling episode watching the camera up his nose. From zooming into his face to a nasal cavity.... yikes! Thankfully we share the same humour, he wants me to record it on my phone next time. 

Found this blog powerful, scary, and very moving.
Also just finished a book by one of my all time favourite authors, Khaled Hossieni, And the Mountains Echoed, and actually sobbed on the train, much to the distress of the only other passenger in the carriage (it was late), poor guy! 

Happy holibobs all x
PS; And no, still no idea who our mystery donor is. Ever thankful x 

Something amazing has happened

....really truly amazing!

We haven't decided on our big fundraiser for this year (possibly Isaac and I are thinking of doing something together) but we always have a few things on the go (I sell my own candles, my Mum organises Bridge days, my sister is always doing runs....). We will be raffling off a case of bubbly at the 40th birthday party a couple of friends and I are having next month, for example. As such, I always have a couple of fundraising pages on the go at any time, and add to these as we go along. Anyway, you can see my latest page here. And it is here that something amazing happened yesterday.....

We received an anonymous donation for ....... £1000!!! 

I am quite literally gobsmacked, delighted, shocked, and a little bit in love with this nameless donor! I don't know what to say, other than Thank You so so much.

Re-watching this debate in Parliament tonight reminds me of how much more we need to do to find a cure, and yet also how much closer we are. Drugs that actually treat the underlying cause of CF rather than its symptoms, were just a pipedream when Isaac was born. Now they are a reality for some people with CF, and we need to keep the momentum going until all classes of CF mutations have that same opportunity, both in developing the drugs which will work for all, and working with pharmaceuticals to make them accessible without crippling the NHS.

ONE THOUSAND POUNDS CLOSER! I hope, like me, this news leaves you with that warm fuzzy feeling you get when you're reminded of all the kindness and hope in the world x

All is merry in Moly world

Kids are all well (ish) and excited about school holidays. Apparently 'baby cheeses' will bring chocolate eggs because somebody died. So that's good, hopefully I won't have to buy the chocolate this year, only spend the next few weeks picking up the little teeny tiny bits of wrapper that's scattered all over the house. 

Brexshit continues, but I'm not allowed to say that now, because it makes me a 'remoaner', apparently. Anouk (age 8) asked me what all this means, and I was tired, so it seemed easiest to just say 'immigration built this nation, but sadly some kids still don't like to share', and she replied 'jeez, some adults are silly'. Too true baby! She is a clever one that kid. 

Sent Rosa (age 4) to school in flares today. Her bottom is so small she looked like a triangle. So cute! 

Trialling Isaac (age 12 - Can you tell I'm a Programme Manager with those age gaps - scheduled to perfection!) on packed lunches. Since he started Secondary School he has been buying lunch there, but frequently not taking his medication with his food (which results in malabsorption - in other words, not digesting his food properly, resulting in tummy aches, runs, and gaining no goodness from his food). This has had mixed results, as he is taking his medication more often, but also helping himself to school dinners after his packed lunch, and so under dosing anyway! 

Have a great day x

Spring.

Sorry for the silence. It's not because we've been away in hospital, thankfully. The steroids and extra oral antibiotics seem to have done the trick, and he seems better now than he has all year. The 'roid rage I blogged about before has calmed, and we're getting back to our normal. I can't tell you how relieved we all are. 

We're having mixed luck on the dry-powder inhalers that he is trialling again, which we hoped would shorten his treatment times. One (TobiPodhaler) he quite likes, but the other (Colobreathe) he can't tolerate again. He alternates months on Tobi/Colomycin to try and trick the bugs a bit, as they work in slightly different ways, so it's likely he will now alternate Tobi podhaler and Promixin through the Ineb (will make sense for my CF friends). So, better than before, but not as good as we had hoped. He also seems happier in himself, and is beginning to take his Creon in school (hallelujah!) so is feeling much better tummy wise. Meeting with ENT next week about his sinus surgery, which can't come soon enough. He's had months now of not being able to breathe through his nose which is a bit poo. 

In other news; We had an explosive cistern water leak in which I was sprayed with toilet water for ten whole minutes; our games room is complete - from garage to pool room and the kids love it; we have a new car, like a 'proper grown up car' on which the wing mirrors are not held on with duct tape and body is beautifully dent free - amazing; my best friend is having a baby boy!; and summer is coming...

Have a great day x 

'roid rage.

We're in the frustrating and somewhat odd position of choosing whether to go into hospital or not. We were in last week for his annual review, which involves tests galore; Bloods, blood sugars (diabetes test), urine, X-ray, lung function, bone scans, ultrasound, along with his usual o2 sats, weight, height, CF nurse checks, psychologist, physio, dietician, pharmacist and his consultant. 

Some of the results are immediate, some take longer, and considering his cough right now, things didn't look too bad. But on the otherhand he has been symptomatic since Christmas, with only the odd day off (quickly ruined by a pesky virus). His cough is wet, fruity and productive. The worst kind.  

Before CF I imagined medicine to be a more precise science; Diagnosis, prognosis, treatment. But I've learnt that there is a whole frickin array of Farrow and Ball greys inbetween... His lead consultant (who we love) took the time to debate the various options open to us, even bearing in mind that he has a birthday treat in two weeks time that he would hate to miss. Knowing that a course of IVs is usually two weeks long, but anything from 10 days would be acceptable, we opted for a weekend at home on high dose steroids to see if this could be an inflammation reaction rather than rampant infection (we already know he has pseudomonas lung infection, but how rampant that is right now? Still up for debate). We also agreed that as he's had a bad run of things, and has missed a lot of school already, an IV course could be done at home (For that, we love her even more!). 

Home IVs typically means; admitted to ward, cannula, first dose, long line, second dose, check for reactions to the drugs, I revise my nursing skills, then home in a couple of days to complete the course. Excellent. We do need to go back in every 2-3 days for blood tests, and immediately if we have any problems with his line, but we're incredibly lucky to live 20 minutes away. We used to do this more often when he was younger, but they changed the policy a couple of years ago, and now seem to keep us on the ward longer, at least until lung function is increasing, but we feel we can do more at home (physio/fitness wise) and reduce the risk of picking up more bugs by staying home. Plus its better for the whole family. 

Anyway, here we are, with our hyper, raging, ravenous boy..... Did I mention before the side effects of high dose steroids? I can vouch for the majority of this list....

 

So this weekend, we headed to my Dad's caravan in Norfolk for fish and chips with wooden forks, sea air, walks, rest, and family time. It's been as lovely as it can be with the crazy beast that steroids presents (constant mantra being 'it's not his fault, it's the drugs, it's not his fault, it's the drugs....) and as agreed we'll make a call in the next couple of days if his cough is improving (superb) or not (IVs). These steroids are short term; he has previously had months of steroid treatment, and the side effects are always the same, so feeling that we're kind of getting off lightly at least. 

On the way home today, Rosa asked me, 'when you're a Grandma Mum, can Isaac be my Daddy?' Sibling love ❤️ X 

 

Clinical disappointment.

In tears tonight reading this. My hopes once ran sky high for this drug, that could potentially be the first to treat the underlying cause of Isaac's CF. 

For my beautiful boy and others with class 1 (stop/nonsense) mutations we must continue to campaign for research and treatments that target these rare (and reportedly more severe) mutations so that we are not left behind as the *untreatable few. 

*Only 10% of the 70,000 CF population worldwide have these kind of mutations. 

Plan Be.

We spend much time second guessing when Isaac will next be admitted; too much time. Currently we're on Plan Be, as in, what will be will be. He had a good 48 hours of feeling better after weeks of coughing, only to come down with a tummy bug, which has set him right back again. Five days of fevers, and although he is no longer feeling sick, his chest seems worse than before the Cipro - those bugs that camp out in his lungs are opportunistic little bastards. 

The question now is whether we can eek this out until Thursday, when we're due into hospital anyway for his annual review (full day of tests galore), or if we need to take him on sooner. Even Isaac has resigned himself to thinking that he'll need to be admitted, which is not like him. 

For all of us, being admitted is our worst case scenario. Isaac, Dan and I know this as a necessary evil, but it's not so readily accepted by Anouk and Rosa who are too young to completely understand. Even with the most supportive and wonderful extended family in the world, the impact of hospitisation hits wide and hard, in many aspects of our lives. 

I am just back from a short trip to Madrid for work, and for now, I'll leave the suitcase out just in case, awaiting ideas from our fantastic CF team. 

Good night all. More soon x 

Good news.

We do have good news sometimes, forgive me for not always sharing this. I know I post more when he is less well. I'm too busy enjoying him being well to post! He seems better, a lot better, and the lab results were good this week. He will continue on the extra antibiotics this week; annual review next week (heaps of extra tests he has yearly). Let the Birthday celebrations continue (I say continue as we started early at the weekend)...

Our handsome, funny, lovely boy is almost 12. And we couldn't love him more x 

Invisible disease.

I hope I'm wrong, but it feels suspiciously like we're hurtling towards another course of IV antibiotics (which inevitably involves an admission to hospital, of various lengths). 

The typical reasons for IV's are.... 

He is more symptomatic than usual (coughing, wheezing, rattley chest). 

His sputum has been sent to the lab and has grown a new bug, or a heavy growth of his usual suspects (Pseudomonas and Acinetobacter). 

His extra oral antibiotics (Cipro) to which these bugs should be sensitive to, has not reduced his symptoms. 

His lung function drops (FEV/FVC). 

Me saying this might seem mighty weird to people around him.... I think if you bumped into Isaac today, or most other days, the only thing that would cross your mind was what an unbelievably handsome kid he is.... not how he is fighting chronic lung infections every day. It's not inconceivable for him to come from the football pitch, trying to give it his all for 60 minutes, but then go straight onto the ward. 

To me, it's not so weird. I developed an uber sensitive 'cough radar' soon after his diagnosis at three weeks old; Give me a whole bunch of kids in a mile radius, and I'll be able to say if it's him coughing (I get right funny looks as I wince when I hear him coughing from three rooms away - And I do literally wince, it pains me so).  

He certainly doesn't fit the 'horribly ill' description you might think CF means. Typically when we get to hospital, he looks like the healthiest kid on the ward. But he's not. Plus he's very unlikely to tell you that he feels unwell, because that just not his style, and this, frustratingly, is his norm, so he wouldn't think to. 

It's his 12th Birthday next week, so I'm very much hoping that I am wrong. If I'm not, this would be his shortest gap between IV courses ever, which hints of a disease progression that I am not ready to accept. 

Big fat pharmaceuticals and the depleted NHS.

Picked up some of Isaac's drugs from clinic today. Due to crazy NHS budget issues his inhaled drugs have to be dispensed at the hospital, while the rest we can get locally. Here, lovely readers, is £4,500 worth of medications. Seriously; 

 

Crazy I know. Two of these replace two of the drugs he currently inhales through a nebuliser (an electronic device which aerosolises drugs into smaller particles to better deliver them into his lungs). Instead these are dry powder inhalers, although not exactly the kind you see for asthma. With these he cracks open each capsule in a device and breathes it in until empty, which can take a few goes, and he needs 8 capsules a day. But still, much quicker than the 20 minutes per neb that he does currently. Test dose went really well, so hoping this is something he can tolerate long term. This reduces his nebs from five to three a day, so he is delighted. 

But what of these profiteering pharmaceutical companies? Who regularly make a profit margin of 20% or more? With one drug costing £10m per patient, and a drug that targets the underlying cause of CF in a minority of CF pateints (Kalydeco) costing £180,000 pp, per annum, with a manufacturing cost a tiny fraction of this, you can see how. Drug companies justify the prices they charge by arguing that their research and development costs are huge. On average, only three in 10 drugs launched are profitable. Many more do not even make it to market. The industry argues that the overall value of the drug needs to be considered; some high cost drugs do save the NHS money over all, as they reduce other needs for intervention. But just because you can charge a high price for something does not mean you should, especially when it comes to health. 

Big pharma companies then argue they only have a limited time in which to make profits. Patents are generally awarded for 20 years, but 10-12 of those are typically spent developing the drug. This leaves eight to 10 years to make money before the formula can be taken up by other pharma's, which sell the medicines for a fraction of the price. Sales then fall dramatically because there is no brand loyalty, but how can there be when the NHS struggles as it does? How could they continue to pay more for the same drug? A drug which might be made for just pennies? 

Ultimately the profit line speaks for itself...in the news they regularly bemoan energy companies making a hell of a lot less profit at our expense, and this is about health. People's lives. It sickens me. 

The WHO has talked of the "inherent conflict" between the legitimate business goals of the drug companies and the medical and social needs of the wider public, which is also rife with bribery. Isaac is prescribed one drug which costs the NHS more than an identical drug because they give the patient the incentive of a free nebuliser (worth £3,000) - if he stops that drug, but we still need a nebuliser (as is the case if this trial on the Tobi podhaler and Colobreathe goes well) will they demand the neb back? Another drug he takes is preferred by the NHS because they throw in free filters which protect pregnant women from the drugs which the patient is taking. One of his drugs costs £500 per week. Another £8,000 per year. Another still, about £8 per year. Seriously. 

In other news..... Sinus surgery sounds more invasive than we first thought; Difficult to read and moving article here about life on O2; I am still appreciating my own functional internal organs, but not altogether right just yet; And frustratingly Isaac is more symptomatic towards the end of his course of Cipro (extra course of Pseudo fighting anti-bots) than when he started, and will continue on them for another week. Being no better after Cipro usually means IV's, so hoping for a better week to come; He was cheerful at clinic today and his sinus pain means he is actually quite keen for the surgery, and extra marsh mellows on his hot chocolate had its usual positive effect on my brave and handsome boy x 

The good, the bad, and the damn right annoying.

The good; 

Isaac trialling two dry powder inhalers again (Colobreathe and Tobi Podhaler). If he can tolerate them this time, this would mean two less nebulisers a day and much quicker treatment times. 

Having my dream job and fantastic colleagues. Off to Madrid again soon - one of my favourite cities. 

Rosa performing the Gingerbread Man with her class, and not having a paddy about it. So cute. 

Dan and I going to Croatia for a weekend to celebrate a 40th birthday with some very old friends. Thank you GP x

Planning a joint party for my own 40th this year too. Be warned friends, there will be fancy dress shenanigans! 

The bad;

Pseudo-bloody-monas infection exacerbation. More antibiotics. Poor kid.

Isaac likely needing sinus surgery. But waiting on ENT appointment...

....Over stretched NHS ☹️

Angry Rosa (Nice Rosa's nemesis, who throws glasses of water on the floor, rips all the pages from a book, and runs away from doting grandparents). Scary.

Annual review looming, tests aplenty, but feeling less and less confident that they will deliver good news this year. 

The annoying; 

Pre-teeniness, and trouble in school. Uh oh. 

Unhelpful pharmacy dispensary staff (yes, I really do mean 14 boxes of Creon per month, it is not a mistake. Oh look, despite having this conversation three days ago, you have still given me just four, and yet you're insistent that this is my fault? Wonderful). 

Relentless treatment regime, but always worrying you could do more. There is always more. 

Him trying harder at treatments and being more compliant, and yet being more symptomatic rather than less. DRRRR! 

My mojo seems to have moseyed off track a little today.... but who knows what tomorrow will bring (summer would be nice). If I believed in luck I'd be expecting a truck load for my boy to arrive; I wish I could give him all of mine. He complains a hell of a lot less than I do. 

Have a great day x 

It mocks me now!

After two and a half weeks my bladder finally forgives me.... in fact mocks me by peeing upon request, as if we never fell out. 

I won't count all of my chickens yet, as I came home last weekend without a catheter, and ended up going back in for another... but I do feel much better this time. Still some weirdly high white blood cell counts... but hopefully the antibots are doing the trick and we can all get back to some normality. I feel as free as a bird without the damn tubes! Thank you for all the lovely messages peeps, its been emotional x 

PS; CF related blogs to follow I promise, Isaac is doing well, lung function is stable.... but clinic has left us with a few bits to digest first. 

 

Miro always cheers me up x

To pee or not to pee....

Sorry, cheesy I know, and sadly it is a no pee from me. I must admit, it's been a little harder than I thought. After a few days trying without a catheter late last week, I now have one back, and an infection to boot. 

I'm thinking of it like this; my bladder was put into an induced coma for a week to let it rest, and then awoken not feeling happy to say the least. And so a further induced coma is required (and antibiotics) to let the catheter do all the work the bladder normally would. After which we hope it will play ball. I cannot describe exactly how it feels to sit on a toilet, desperate to go but unable to.... if you've ever had a UTI you'll kind of understand, although the pain is different. The opposite of urinary incontinance.... It's a sulking, stubborn, bladder tantrum, refusing to let go to spite me, with some kicks in the abdomen for good measure. 

As if I needed it, the words 'disposable self-catheterisation kits' gave the incentive to keep trying more weight; I'm back in hospital later this week to give it another trial, and try my very best I will. Huge kudos to people living with long term issues of this kind. It's not the worst thing in the world, but it's not much fun either. I can't go to work or walk my dog, but my sock drawer has never been tidier. 

We have CF clinic and a few Isaac related issues to tackle next.... so I need to get well asap. Read something promising here about Pseudomonas and biofilms. I always imagine these biofilms like those slimy green sea weeds that make you slip when you're rock climbing - in Isaac's lungs biofilms help shield the bacteria from the antibiotics that we throw at them, so if we can tackle these, our arsenal could get a whole lot more powerful.... 

Anyway, the whole debacle with my own health has left me pretty much house bound at times, and we couldn't have managed without our families and friends, helping, visiting and calling to keep me sane, taking the piss (not literally, but I suppose they could help with that too now...), and keeping the humour in everything. But most of all to Dan and the kids, thank you for letting me rest when I've needed to, and for not tripping over my pee bag. Love you all, my A-team x 

Listening to this, it's weird one, but it's a grower. Beautiful album. Have a little picture of Obie; 

 

A&E and the amazing NHS

We spent last night on A&E with our little Rosa. 

Let me first say, there is not much more that I hate than people who clog up A&E departments with non urgent cases, least of all those who use ambulances when they could drive themselves. In the next cubicle was a mother and daughter who were there because the little girls heart rate 'seemed a bit fast'. Could be worrying, sure, but as the story unfolded, both the mother and child admitted that this happened quite often, and only when she was feeling a bit panicky. Sure, not a great situation for either of them, but if it had happened many times before, and was related to stressful situations, why did this become an emergency on this random Tuesday night? After a heart trace and some reassuring words from the doctors they were sent on their way. Because this is what the NHS does, it cares for A&E, anything and everything. 

Despite our regular visits to hospital, we've never made the trip in an ambulance, but for our dramatic little Rosa (now 4), it would have to be her vehicle of choice. She fell down the stairs, hitting the back of her head all the way (we think, we only heard the duh duh duh duh duh...). Poor tink. I thought I could manage her tears, and Dan was about to take the big two to swimming class so I ushered them off. However the crying soon turned to sleep, blurred vision (I can't see you Mama!') and then vomiting. So I calmly (my arse) called 111 to ask what I should do, but given the two warning signs of concussion (blurred vision and vomiting) they immediately despatched an ambulance, despite my insistence that I could drive. By now, Rosa only wanted cuddles, darkness and sleep. We spent a few hours in (we were almost allowed home at one point, when she hadn't been sick for two hours, but as we got up to go she was sick into Dans eyebrow) before being allowed home to just keep a watchful eye on her. I spent the whole time there in my PJ's (I have no clothes that fit over a catheter bag of pee strapped to my thigh), whoozy on Tramadol (I'm still on these, post Op, but cutting back, unfortunately I'd just taken one when she fell). Glamorous look I tell you. 

Anyway, I just thought I'd share this tale with you, my lovely readers, as yet another example (as if I need one) of the endlessly amazing NHS. They keep saying the NHS is at 'breaking point', and yes, I see the tiredness in the faces of our fantastic nurses and doctors, and don't doubt that the Trust is in financial crisis, but let's not forget that they continue to provide the most amazing care, with the least amount of complaining. I bloody love our NHS. 

In other news, from a very good friend with a lump in her breast, another whose Dad may be dieing and who has her own health fears, a baby back in hospital again due to Bronchiolitis, Isaac who is back on extra antibiotics for his chest, another friend who has had cancer treatment and a hip op and who is still struggling, and a pregnant friend who is feeling frustrated and upset....  I think we can be forgiven for feeling a little bit cursed right now.... If I believed in luck I would be thinking that surely things have got to get better? They will, they have to! We are overdue a bloody party. My friends, I am here for you, as you are for me (so often). Love you all x 

Our beautiful, feisty little Rosa; 

 

  

Internal organs are so overrated anyway.

I recently read in the paper that the internet makes us over-share. As in, we wouldn't normally tell people all that we do online as we would to peoples actual faces. I guess in some way this probably true of me too. I certainly try not to bore people with endless pictures of the kids, or tales of our worries, but I do all of that in this blog quite regularly (sorry about that!). But on the otherhand, those people who know me will say that I have a long history of over-sharing in real life too (usually when least appropriate), a habit of ruthless, unapologetic truth-telling, perhaps to a fault. So this here follows a tale of how I have ended up with a bladder strapped to my leg and asking everyone who visits if I smell of pee ... (It's no big deal really, I mean, internal organs are just soooo passé, no?).... 

In case you are new to this blog, I write it to raise awareness of Cystic Fibrosis and Organ Donation, because to put it bluntly, my 11 year old son has CF, and may well need a lung transplant at some point in his life due to this. 

Cystic fibrosis (CF), a genetic condition which he was diagnosed with at 3 weeks old. In his lungs thick sticky mucus obstructs the airways and forms a breeding ground for infection, and each infection causes progressive lung damage. Isaac keeps relatively well on the most part, but the relentless daily regime that CF demands is staggering. At the start and end of each and every day he has to be plugged into nebulising machines to clear the mucus from his chest, and inhaled antibiotics to help manage infection, as well as chest physio (PT) and currently a prescription list 23 meds long. He has an unwanted live in companion (a bacterial chest infection called Pseudomonas) and when he coughs, it's not like ours, it rattles and wheezes and deeply reverberates in his ribs. Otherwise, he is typical cheeky 11 year old, who, above all, wants to be normal and not to be defined by his condition. CF awareness helps raise not only vital funds for the charity we support (the Cystic Fibrosis Trust) but also understanding of this chronic condition, and hopefully reduces the number of really quite stupid questions he is asked (So when did you catch CF? Can't you just take cough medicine? When will you be better?). Raising organ donation awareness we hope will increase the number of people on the donor register, and reduce the number of people on the list for organs (who all too often die waiting due to the shortage). 

So, all of that, plus, I really quite enjoy writing. And although I never usually re-read blogs, occasionally I do look back to the same date a year or two before, to see what we were doing then, and so it serves as a kind of journal for me. It also means I can keep family and friends up to date when we are in hospital and I can't face talking. 

All that said, this blog is mostly about Isaac and his health. He is almost 12 now, and I have the utmost respect for his privacy, despite my own habit of over-sharing. I would never write anything that he might not want others to know (and I do keep back somethings because of this). When it's about him, it's not my story to share (although if I had been writing this when he was a baby I'm pretty sure it would be much more poop orientated). If I'm not sure, I check with him, and on the most part he is happy for me to write about his health, as my blog is boring, dull and not at all epic or boss, he can't imagine anyone being interested (the girls on the otherhand love it, and always ask me to post pictures of them pouting). 

And so, in the spirit of being open and honest; I went in for a simple Op last week, which went fine. Not interesting enough to let anyone know about. However, because I had a general aneasthetic, I had a catheter (presumably so you don't wet yourself during surgery). After the Op, everything was fine, but when the catheter was taken out, I was told I could go home 'just as soon as I could pee', sounded simple enough.... 

But pee I could not. Not with running water. Not with deep breathing. Not with wetting my neck. Not even with my lovely nurse Marcella doing a little pee pee dance and thanking the gods of the rain. Nothing. My brain has seriously fallen out with my bladder and they are refusing to talk. Never before have I wanted to pee so much. 

And so I am now home, with my new handbag. Not exactly the kind I had hoped for, but hell, worse things happen! I am now resting my bladder for a week, and will then have the catheter removed and hope by then the fall out will be over, and my bladder will again talk to my brain. I am assured I do not smell of pee. 

Two observations about this whole debacle; Firstly, I have medical insurance with work, so had my Op privately. Since we spend so much time in the bosom of the NHS (CF is so specialist, it's likely that all his care will be with the NHS) this felt really odd to me. It was not the room, the nice TV, or ensuite that got me though, it was the time that the nurses spent with me. I so wish we didn't stretch our NHS nurses so much, and we could let them do the kind of caring they would like to do, given the time. Secondly, when the tables are turned, Isaac is every bit as caring for me as I am for him. He has been amazing, and perhaps this is a good thing for us, we both get to appreciate the flip side of the coin. Only for me it's short term (we hope). I won't have a pee bag strapped to my leg for much longer, whereas he will always have CF to contend with. But we are a team and we will do it together. 

This is also a breeding ground for bad toilet humour, our favourite kind. 

Have a great day x 

Balance

Getting over my cough took longer than expected, still shaking it off now. One thing I never have time for is going to the doctors myself. Never mind though, we enjoyed the break very much, so much so that the night before going back to work, I felt almost bereft at the thought of leaving the kiddles.... and I love my job. It seems the more time we have together, the more I want. But the plan is for me to work for as long as Isaac is well enough for me to do so, while I can still juggle work and family life, so we can gain some financial security. 

Isaac is not quite well (but had a good Christmas). It is both frustrating and deeply saddening at times; He has been back at school three days, and had to come home early for two of those. Clinic soon, if he can wait that long, if no better this week I'll take him in early. We have something called 'open access' at the hospital. Which essentially means we can call anytime and talk directly to the on-call registrar. This also helps us bypass A&E sometimes and go directly to the ward, but during normal hours it means we can go directly to the CF team in clinic (outpatients). The problem is knowing when to go. The relentless conversation in my head usually goes something like this....

He is in more pain than usual, it could be another bout of DIOS, I should take him in.... 

Or, he may not have taken his Creon in school, and he might just be malabsorbing? Better stay home, wait and see. 

He has headaches and nausea that his anti sickness drugs aren't helping, I should take him in....

Mind you, there's lots going round, we've all been ill, it's probably viral, better not take more germs into hospital. 

He's still not right, needing painkillers everyday is not right. Are these side effects from one of his many medications? Has he had enough salt today? I'd better take him in.....

Ahhh, the hospital has winter vomiting virus, better stay away. Don't want to catch that! 

His chest doesn't sound good, is that a wheeze? A crackle? A new chest infection, or an exacerbation of his existing live-in bugs? I should take him in.... 

Actually, sending in a sputum sample to the lab would be better, then we'll know what we're tackling. Extra physio at home while we wait for the lab results, that'll do the trick! 

No, he's really getting no better, I'm taking him in.... but he has a (insert fun kid thing) today, he would be gutted to miss it, can we put it off one more day...? 

I don't think I am alone (in the CF world) in feeling like this. It can often feel like you're damned if you do, damned if you don't, and judging exactly when the right time is to start extra antibiotics, or take them in to clinic (or worse, A&E) is a mindbend. At the stage we are at, there are only rare occasions where CF can require emergency treatment, but inaction could cause long term damage. On the other hand, I've been that Mum that rushes in on many an occasion too, and been sent home after no more than a cuddle and a sympathetic look. And that is no fun for him, nor is it reassuring that this life is any way 'normal'. 

While I juggle my own work/life balance, I'm finding at this age with CF, for Isaac, it's the health/life balance. He doesn't want to spend ages every day doing treatments, but he doesn't want to be unwell either. He doesn't want to be 'different', but he is. Right now it feels like a constant struggle to keep the many factors of his life balanced, as so many of them are important, and there are only so many hours in the day; 

1) CF treatments (to stay well), 

2) sports (to keep fit, and see 1.)

3) the things that make him happy (I'd love to say spending time with family, but PS4 might be slightly higher up his list!)

4) feeling normal (very important to him psychologically) 

5) education. 

Due to the amount of school he has missed, number 5 seems to be taking a hit right now, which is unsurprising and not his fault at all, but the extra work he needs to do to catch up hits numbers 2, 3 and 4. And it's vexatious to think that even if he puts in the extra work to catch up, his next admission to hospital might set him back all over again. 

So in my fuddeled up way, I think what I'm saying is, we're all feeling a bit unbalanced, and 'CF sucks' features highly in our home vocabulary right now. 

In other news, Dan and I are enduring 'dry January'. Dull. I've started my new job, and nothing has gone terribly wrong so far. Little Rosa becomes evermore fiery, feisty and funny by the day and Anouk said to me the other day (after a chaotic morning school run where they each couldn't find something before we left) 'Jeez Mum, there is no way I am having three kids.... it's AWFUL!'. Only sometimes sweetcheeks. 

Happy New Year x 

A coughing good Christmas.

It is me coughing this Christmas. A 'can't lie down for spluttering, lung screaming, throat angering' kind of cough. But being ill is always a reminder of what it might be like to feel rubbish daily, as some people are, others who are suffering in ways I almost never do. Feeling normal is criminally underrated, and I am as guilty as the next person for taking that forgranted. And so I hope my empathy and this cough makes me a better Mum in some small way, for my boy who almost never feels 'normal'. That is my coughing Christmas silver lining. 

We have had a lovely Christmas. The kids have had fun. We are relaxing with family, surrounded by music, and soaked in gin. 

Wishing you all a happy holidays too x 

With family this Christmas?

Looking back, I think I was a wonderful mum of two. There was baking and homemade costumes aplenty. Heavens, even homework was tackled and hair brushed. In comparison, I fear I am a pretty crappy mum of three. In this run up to Christmas I've fallen into that classic parent mode where IT'S ALL TOO BLOODY STRESSFUL. I never believed parents suffering from this phenomenon before, I mean, how hard can it be to order presents online and make a Saxo stuffing? But I am, and it is. So, forgive me, no Christmas cards this year. I am making a donation to the CF Trust instead. 

And thinking of all you lovely people, who I hope are looking forward to a family Christmas too, can I suggest a light after dinner chat? Even if you are signed up to be an organ donor, your next of kin can (and surprisingly often do) override your wishes in that tragic moment. 

You are more likely to need an organ transplant than you are to donate an organ, and so it makes sense that more than half on the list die waiting due to a shortage of donors. Please sign up, and please, have that conversation while you can. Sign up here! 

For us, we are all well. Isaac's extra treatments since his admission have now come to an end (aside from the extra nebs which he will remain on now). He has a few tummy problems again, but is mostly well and very happy to be home and off school.  

Wishing you all a very merry time this holidays and a healthy new year too x 

Christmas cheer

Huge thank you's to my wonderful Cambridge University Press colleagues who supported our annual Christmas Raffle in aid of the CF Trust today. This raffle is now in its 11th year, and used to consist of all the supplier donated Christmas goodies sent in. However due to compliance regs, suppliers send in a lot less these days, if any, and so the raffle now largely consists of prizes donated by staff. Despite this, the funds raised increase with every year.... Which by my books means my colleagues grow kinder and more wonderous each year! 

£500 raised today, and more to count up next week from cakes sold too. I've created a new fundraising page which you can see here, and this has prompted me to think about my next fundraising challenge too.....Ideas on a postcard please! 

Our family and friends have now raised over £53,000 for the Trust since Isaac was born, and a large part of this is down to my colleagues, never seemingly getting sick of me asking for sponsors. It means more to us than you can know. Thank you. 

Amazing Anouk.

Our little Anouk turned 8 this weekend gone. She has always been the sweetest little thing, the most beautiful baby, and continues to fill us up with love. A miracle baby, born following PGD treatment (which you can read more about here), so new that she was the 23rd baby born in the world following that kind of scientific test. She even has tree planted for her in a woods for this reason, she is so special. From day one she was full of love, grace and kindness. She mothers all children, a prolific love-letter writer, a gift giving creative, an arty soul....

Our little Nanook, Babooska, Shnuks.... we love you so.
As you like to say 'I flew right into your heart'.

Whenever I'm alone with you You make me feel like I am home again Whenever I'm alone with you You make me feel like I am whole again Whenever I'm alone with you You make me feel like I am young again Whenever I'm alone with you You make me feel like I am fun again However far away I will always love you However long I stay I will always love you Whatever words I say I will always love you I will always love you Whenever I'm alone with you You make me feel like I am young again Whenever I'm alone with you You make me feel like I am fun again However far away I will always love you However long I staye I will always love you Whatever words I say I will always love you I will always love you

Young lives lost.

Another young life lost. Not lost, cruelly taken, by Cystic Fibrosis. A family heartbroken. 

Kirstie was 27, and blogged in her usual candid and ever positive style here when she knew that she was dying. Kirstie featured in a documentary about CF which you can see a clip of here. She went on to have not just one life saving double lung transplant, but two. These gave Kirstie five more years of life. She was an ambassador for the charity Live Life Give Life founded by the lovely Emily (also sadly no longer with us). 

People sometimes ask me what they can do to help? Well, there are ways.... 

Fundraise for the Cystic Fibrosis Trust. It doesn't have to be shaving your hair off, or climbing a mountain as I do, a cake sale at work can be wonderful too, or a non-school uniform day in fancy dress. The Trust fund research which WILL extend the lives of people with CF. And not just extend them, but also improve the quality of that life. 

If fundraising is not your thing, then campaign to get more people signed up to the Organ Donor Register. Remind people; you are more likely to need an organ donation than you are to give one; Half of those listed die waiting; You must tell your next of kin your wishes, otherwise they can over ride yours; And that Transplants can and do SAVE lives. 

If you haven't already, please sign up here.

Here is to those extra five years during which you lived and loved life to the full. Sleep tight Kirstie x 

Sunday Roast and A&E

Today we celebrate my wonderful Mum's Birthday, and my favourite Nephew, Ben's. I can never say it enough, but my Mum is my ultimate hero. As well as being the hardest working Mum, she is the most incredible Grandma, who along with her husband Joe, have looked after mine and my sisters kids in their early years while we worked, and even now they are all in school, having them in the holidays when we can't, picking them up from school, and coming to our rescue with hot meals and fresh veggies from the allotment all the time. She is the most selfless person I know, only ever wanting to help others. 

So today it was our turn to spoil her a little, but just a few hours before they and family arrived for a roast dinner, Isaac and I were having to head into A&E to get him checked out. His longline is hurting him now, which it shouldn't. Thankfully they agreed it was not blocked, but likely his vein is irritated. Not surprising really, having housed a tube for the last two and a half weeks and pushing through pretty toxic drugs 3 times a day. It would be great for it to last until Thursday..... but it's still not comfortable for him tonight, so I'll be calling clinic in the morning. The team in A&E were fantastic, and Isaac as brave as ever, we made it home in time to celebrate. 

I promised a summary about IV lines, so here is my very non-medically trained summary; 

A cannula is the most common IV line, typically inserted in the back of the hand. A needle is pushed into a vein and a short flexible tube is pushed up the vein. The needle is removed, but the line stays in. On the end of the line is a port, which syringes (or drips, depending on the amount of drug and how slow this needs to be delivered) can be attached to, to then deliver drugs directly into the blood stream. 

Essentially, all lines follow this same principle, just in different veins in the body, in different lengths, and to last different durations. 

A longline is the same, but as the name suggests, longer. This is usually inserted just below the elbow, but Isaac has also had them in his ankles, and some veins in the groin may be used. These typically last longer than a cannula, usually up to 3 weeks. 

A PICC line (peripherally inserted central catheter) are similar to a longline, and Isaac has had these in the past too. Only they are longer again, and usually inserted under general anaesthetic. The length is typically from his elbow right up to his chest cavity, or from his ankle to his abdomen. These can last many weeks, or months. 

Isaac has not yet had any other kind of line, but Central, Femoral and Hickman lines are often used for drug delivery over a longer period, often in oncology. 

In CF, a more permanent IV device can be surgically implanted, called a Portacath usually in the chest or upper arm. Isaac is likely to have one when he needs more regular IVs as this makes drug delivery much easier. It's like a rubber bung, sitting under the skin, which can be accessed easily when needed for IVs, but at other times, is fairly invisible. It's also often used in CF due to the number of courses of IV antibiotics which damage his veins, meaning they cannot be used again. 

Our IV station; 

Happy birthday Mama and lovely Ben x

Pudding honey?

Ceftaz, Tobi, flush, with a side of Hepsal perhaps?

Home blimin sweet home.

At last. Two weeks in hospital feels like 3 years 4 months and 29 days when you are there. I was kind of hoping to get out and find that the whole Brexit/Trump thing was a whole Dallas type dream, Bobby would wake up in the shower again and none of it would have happened.... no? Hell.

Isaac has gained 15% lung function compared to admission, which is good, no.... it's great, but not quite as high as we would all like it. I am so thankful for his excellent CF team who like us won't settle for less. If we can get him better, we will! Therefore we are planning a third week of IVs, which is unusual, but may help his LF further. But I'll do these at home, which is so much closer to a normal family life. So we loaded the car with our many many bags, meds and equipment, and escaped. If his longline fails sooner we will rethink (I will post soon to better explain cannulas, long lines, and PICC lines, all of which he has at times, as I realise it is very confusing)

The fresh night air was exhilarating,  played the music so loud and sped home to be a family again, smiling all the way. Talking to another CF parent earlier who is in the same situation right now, about how you get home, and it's amazing, and you celebrate...... but unlike many other people leaving hospital, it's not over, it's never over for us. And so it's often when you get home that you crumble, after holding it together inside so well. I am not yet crumble. I am still smiling, but soon....

Huge thank you's to the Grandparents as always, coming to our rescue. We can never thank you enough. The Cayley's for always being there, Bronté for always visiting, Lautaro, Ju and Jess (so great to see you guys), Lemon, Lizzie, Gem, Jin, Hamish, James, Bon, Sarah, Gavin and Jennifer and many others for the soup, the wifi, the help, the kind words.... it really does mean the world to me, and helps Isaac pass the time.

And to the amazing staff on D2, nurses, our unsung heroes x

Hospital life part 2.

It feels like we have been here for weeks already. It is such a mix of emotions. On one hand he is relatively well, we feel like the lucky ones. He bounces around, eats non stop, goes to the gym twice a day, and is more than happy to have needles as it means more laughing gas. We see so many poorly children here, but many of them come and go. Patch them up and get them home. Whereas we stay. He looks so well, but his lungs are not.

I am with my boy, and I miss my girls, my dog, the cosy life we have made for our family in our little cottage. Dan comes to my rescue, but then it feels wrong not being with Isaac. He is brave, and he is brilliant company, but he is also at times angry, bored and frustrated, and it's me he lashes out at. Because that's safe. Unconditional. And I know this, I understand. I understand him. But it's still hard sometimes.

CF affects all of us, Anouk especially gets very sad when we have to stay in, and she's beginning to question why the poorly looking kids get to go home, but Isaac doesn't ('Is Isaac more sick than them?' She asks).

Midweek it looked possible that we would get to come home early to finish the course at home. I've retrained, so I am all set to do his IVs myself, but his test results just weren't good enough. As much as we tried not to get our hopes up, we did, and it was a big disappointment. Then we were told he might even need a third week of IVs, which he has not needed in years (two weeks is the standard length for IV antibiotics). We agree with the plan, which is focussed on getting his lung function up as much as we can, but it's tough.

This weekend he has been allowed home for the day inbetween IVs. Tomorrow we are back on the ward full time to resume the extra physio sessions too. Last night he was up until 4.30am doing his IVs which had been due at 7pm. His longline had blocked, and he has another's cannula in his hand now. They will try and rescue the longline again tonight, otherwise he will have a new one tomorrow morning. He is looking increasingly pin cushioned, and the cannula (in his hand) limits him much more than the longline (in his arm).

On the plus side, we escaped for a few hours the other day and we had some fun, and the Christmas Spirit came to the ward early this year, when every child was given a book on Astronomy, a piece of Meteorite, and a Telescope. Amazing.

He is getting better, just not quite better enough yet. Merryness will resume shortly x

All is well.

It's been a good weekend. Isaac's IVs are well timed, we get a few hours free in the afternoon, and today he came home for that short time and went out to play with friends. His chest sounds worse, but this is likely good news; The gunk is on the move. Tonight he has returned to the ward with Dan, and I'll take over in the morning for the next 4 nights. I'm enjoying time with Anouk and Rosa, we sleep in a soft heap of girlieness. But while I know he is in safe hands, it is still very hard for me to not be with him. We sleep in his bed to feel close to him.

Monday means the return to proper doctors rounds, and a whole lot more tests to see if these antibiotics are having a positive affect. We know that we are in the right place, and fighting against CF aggressively like this is right; we welcome the battle as it will keep him well longer. But it's not easy to explain to a 4 and 7 year old who just want their family to be together.

Thank you for all the lovely messages. People have said in the past that they haven't contacted us because they didn't want to say the wrong thing, or ask what might be intrusive questions. But in my experience, I would always rather people did ask, however silly they might think their questions are, that you are thinking of him enough to ask shows that you care.

Hoping to have good news for you soon. If his lung function increases enough, we may get to finish off the course at home, once I've had my refresher training as 'nurse Lizzy'. Keep everything crossed! Good night all x

Hospital life...

You know when you have settled in to hospital life again when you're in M&S buying sushi for breakfast, in your tea stained pyjamas at 11am, and you don't care. It's freezing outside, but you're in flip flops because the ward is a sweaty 35 degrees all year round. You play chess, Xbox, cards and watch a film, hoping the hours are passing, only to find it's still only 10am. You go to bed at 8pm and stay in bed until 6am, to have 4 whole hours sleep. You have lovely cuddles, pranks, laughter, and then strops and shouting, all within an hour.

That said..... It's all going well. Cannula in (uncomfortable, and in his favoured hand) the first day, long line the next. Went in first time perfectly, on gas and air (which I had to wrestle off him in the end!). This is so much much better for him, he has great movement, and should last the whole 2 weeks. He was so brave. He has a full on routine; 4 lots of IVs. 5 nebulisers. 4 lots of physio. We have been in the gym twice today. On one hand it seems like an endless cycle of treatments, but on the other, time passes so very slowly. He was very happy to see two aunties today, and for us to all eat together as a family tonight. His cousin Ben is visiting tomorrow.

All going well so far, and I couldn't be prouder.

Hoping for good results from the lab and better spirometry results (early next week) when we will know more. Good night all x

Hospital stay.

We are heading into hospital tomorrow for 2 weeks of IV antibiotics. No huge surprise. He feels OK, but his cough and lung function are worse. We are confident this will kick some serious CF butt, and we'll have him fighting fit again for Crimble season. Don't you worry! 

More soon x 

Compliance.

I've been meaning to write for a while about CF and compliance to all the treatments CF entails. To illustrate this better for those less familiar with CF, this is Isaac's typical day (when he is well);

Morning; 

Anti-sickness medication when he wakes up

Inhalers (to open up his airways), nasal sprays

*Nebuliser 1 (DNAse, which is an inhaled enzyme that helps thin the mucous in his lungs)

Breakfast (with Creon, his enzymes which allow him to digest food properly)

Morning tablets (antihistamines, salt tablets, plus three medications for his tummy issues)

**Physiotherapy to clear his mucous

Nebuliser 2 (Antibiotics, Colomycin or Tobramycin, to fight his infections)

Afternoon; 

Lunch with Creon again

Evening; 

More inhalers 

Nebuliser 3 (Hypertonic saline, to draw more water into his lungs, to again loosen the mucous)

Physiotherapy again

Nebuliser 4 (Antibiotics again, to fight his infections)

Evening tablets (vitamins, oral antibiotics, many more tummy related medications)

Night;

Possibly more Physio, depending on how he feels

*A nebuliser is an electronic device which breaks down particles of medication into smaller blobs (sooo scientific, me) which are then better delivered to and absorbed in the lungs. Each nebuliser takes him between 10-30 minutes, and he has two different machines, because of the different drugs he nebulises. We call these 'nebs', and anyone walking past our home at any given time would have a pretty good chance is hearing us yell 'neb Ise!' as his concentration on these is lacking. 

**CF Physiotherapy can be of different kinds. He has small devices which he can breathe through against resistance, which help shake his lungs. Some of these he can do hooked up to a nebuliser at the same time, which help him clear his chest on exhalation, and then deliver the medication on inhalation. He also has chest percussion when unwell, which is the classic 'pat him on the back' physio that most people remember about CF, we call this patacakes. 

His current prescription list is 22 medications long, which we need to ensure we have in stock at all times. And due to crazy NHS budget rules, only some of these can come from our local pharmacy, the others we need to get from the hospital pharmacy. In addition to this, he needs to take enzymes (Creon) with everything he eats that contains fats or proteins (pretty much everything aside from plain fruit or veg) because of a rubbishy pancreas he produces none of his own. This varies according to what and how much he eats, usually around 30-50 capsules a day. 

There is a strict order to his treatments; for example, we want to loosen his mucous first, then help it come up, and finally throw antibiotics in to make war on the bacteria, when they have clear access. Also, many of his drugs have interdependencies, side effects, or just... effects, which mean you need to take them at a certain times of day, with or without food, or avoiding something else. This can be mighty hard to get right all the time (try giving babies antibiotics four times a day which can't be taken one hour either side of milk...!). 

Reader, I can't imagine how that reads to you..... Is it a lot? Less than you thought? 

To us, this is our norm. This is our good day. Our minimum. It can be much more when he is not so well; for example, he has occasional courses of IV's (Intravenous Antibiotics) - this might be because he is more symptomatic than usual, or because his lab results reveal something new (they culture samples from his mucous to see what bacteria are growing in his lungs). This means two weeks+ with an IV line in; antibiotics and physio galore. 

But we're lucky, IV's are an occasional occurrence for us still; many other kids and adults with CF have regular IV's - two weeks every three months (at least) simply to keep the infections at bay. There are also other complications of CF which we don't have to deal with yet - diabetes, liver issues, problems in maintaining weight (resulting in PEG feeding directly into the tummy to cram in the calories overnight).... The list goes on, and each of these would add significantly to the treatment burden. 

There is not a day that goes by where I don't think how fortunate we are to live in a time of such great medical advances, to have such a wonderful NHS, and such a supportive CF team. I even pass by our ever expanding meds cupboard sometimes and admire the neat pharmaceutical packages, so pristine and white, the syringes, the wipes, the nebuliser (retail cost £3K), all lined up and ready to join us in battle against those pesky bugs. We don't moan about these treatments, we are thankful to have them. 

But it is so hard for Isaac. On a good day, the above will probably take him one to two hours. On a bad day, three to four. On IV's? It's pretty much like a full time job. Just to stay well. He is 11 years old. He wants to be out playing with his friends..... he wants to be like everyone else. He doesn't talk about CF with his friends, and even taking Creon in front of people is an issue for him. Anyone who remembers Secondary School can understand that. When I was 11 I too wanted to be like everyone else (when I was 21 I wanted to be like no-one else). Right? I am ever mindful of how he must feel, but also need to get him to do all this, because the alternative is him being less well, and likely resulting in long term lung damage. That is not an option to me. He has an understanding of how important his treatments are, I tell him, his Dad tells him, his CF team tells him, and yet he still cuts corners wherever humanly possible unless we watch him the whole time (which with two other smaller children is difficult to say the least)...... The nebuliser which he insists he finished properly while I was in the bathroom, the pathetic blows of a mouse doing his physio, the 'forgotten' Creon, which then gives him a rotten tummy, and makes doing effective physio the next morning even less appealing..,.. 

I get it, I really do. I get him. I know him better than anyone. And if it were me? I'd have probably done the same at 11. But it doesn't make it any easier to watch, and getting him doesn't always make me any better at reasoning with him. We have this closeness in hospital where we can talk calmly about it, and I do anything to not sound like I am nagging him, I've drawn blood biting my tongue so much. But  when he's frustrated, and angry, and feeling like his life is so unfair, what to do? So I agree - CF does suck; or I tell him how many more people are worse off; I bribe, I bargain; I think up wacky new ideas to put the fun back in treatments; but sometimes.... I have nothing. I'm just mad too, not at him, but at CF, and how does that help the situation? Not one iota. 

Some days there is no issue at all, he just gets on with it. 

Other days I know we could have done better. 

On the worst days, I'll walk the dog for hours, just procrastinating, knowing the next round awaits me when I get home. 

Some nights this keeps me awake, thinking, have we done our best by him today? 

To support the Cystic Fibrosis Trust who fund research into CF please click here. 

I'm sat in front of my fire, with the dog on my lap, listening to this beauty, put it up loud, it's a grower, I promise. Goodnight all x