The house feels so empty.

Isaac has never been away from us before. 

I know (no, I hope) he is having a wonderful time. 

I miss him so much. 

We get a break from the relentless CF treatments, but he does not. 

It's been raining all day. Please let him be warm and dry. 

Talk about tugging the heart strings.... 

Cheers!

I haven't updated about Isaac's health properly for a while. Thank you to everyone who asks after him, it's always nice to know people care, or at least that I'm not boring you all senseless with this blog!  

His lung function is stable - not fantastic, but definitely not awful at all either. 

Still waiting on a date for his surgery. He can't remember what it feels like to breathe through his nose. Bloody polyps. 

He is currently on extra oral antibiotics (three different antibiotics, rather than his usual two), for his usual pseudomonas infection. However he is really well right now - we're doing this just to give him a little boost before he goes away on school camp. 

On school camp he will not do his nebulisers. This is wonderful for him (think bulky machines, having to sterilise neb parts, needing electric (on a camp site), and having to do these treatments with his new friends present five times a day). But still worrying for me.... he has been on regular nebulised drugs since he was 18 months old. BUT, his team is supportive, and the extra exercise he will get there, and the extra oral meds will provide extra cover. This is his first time away without me or Dan. Eek. 

His tummy continues to give him jipp. It's mostly manageable at home. But he is missing too much school. His attendance is around 80% this academic year, which is slightly up on the year before. We're in talks with school now to see if he can drop a non-core subject in year 8. This would give him time to catch up/do homework in school time. The school are really supportive of the idea (which came from a CF parent friend of mine - thank you G!). I hate that he struggles so, and after treatments at home, sometimes the last thing you want to ask him to do is catch up work... So we're feeling positive about this change. 

We've been out and about having fun at the coast, canoeing on the river, BBQ's galore, sitting in the garden so late that the mozzies have a field day on my legs..... Everything is sunny groovy. Have a great summer people! x 

Handsome Obie. 

Trying out some new, and pretty effective, parenting techniques with the pre-teen. I can recommend certain types of tape. 

Rosa. Looking like butter wouldn't melt... don't be fooled! 

Lovely Anouk! 

Now 8, 5 and 12. How did that happen? 

Orkambi.

Exhausted but finally home after a day at Westminster to protest about Orkambi - a ground-breaking drug that could save the lives of thousands of young people with Cystic Fibrosis. It's available in the USA, France, Germany & Ireland, but not in the U.K. This has to change. Read more here. 

Today was great, excellent company, and something I am proud to be part of, and whilst it's not a drug that will directly help Isaac (due to his rare mutation) I believe it will open other doors. Orkambi's UK list price is some £104K per patient, per annum, which NHS England simply cannot afford, but these protests (we hope) will restart negotiations with the pharmaceutical company. 

Many young people who could benefit from this drug will lose valuable lung function the longer this goes on, or worse still, die waiting. 

 

Crashes and Coughs.

After a really good spell with his chest, we've hit another rough patch. It's hard to explain, but his cough sounds and behaves differently to ours. Deep and reverberating. Coming in waves. Infection without fever. This means extra physio, samples to the lab, and CF clinic this week. He is not far off his school camp, so really hoping his lung function tests go well. 

He is also cut up and bruised from a bike accident. On the way home today from a 10m ride to a nearby town for a drink and pasty by the river, he flipped my fixie bike over. It's all elbows and knees, but as I saw it all play out in front of me, hell my heart burst out of my chest! In his usual good humour he picked himself up and carried on. 

I absolutely love our time alone together, he is the best company. His CF dictates that we spent more time together than most sons and mums, but I can't think of anyone more wonderful to do this with. Bloody love that boy. 

This weekend was also our little Rosa's fifth birthday. We surprised her with a disco dome bouncy castle in the garden for her party, lights and music included. Such fun, and some pretty cool physio for Ise (bouncing is great for clearing mucous). She had the best time, our little Banosa, and Anouk made it the best party by mothering all the littlies, she is so wonderful. We partied until late. 

I am off to the Orkambi protest at Westminster tomorrow. Read more about this here. I am looking forward to venting some energy in this great cause, hooking up with other CF parents, and fitting in a swift Tate Modern visit on my way. 

Have a great day x 

All the worlds troubles.

I'm sure I'm not alone in feeling like the news everyday, both political and tragedy filled, is overwhelming at times. Feeling sombre seems like the national BAU these days. In Moly world, we can't help but be humbled and reminded of how lucky we are. Isaac has been well, despite his ongoing problems with his nose (for which we are still waiting for a surgery date) and tummy (including some pretty spectacular projectile vomiting when the kids and I were home alone last week - think sick on the walls, skirting boards, rugs, dog.... It's both a little sad and also a point of pride that the kids all knew their roles in this, little Rosa got towels, Anouk got the water, and Isaac managed it in his usual good humour). But the best news is his chest is great, which is probably the one thing keeping my glass the right side of half filled right now. 

I'm working with a teacher at his school and his CF team to work out a way that he can go away for 4 nights in July without me (a first for us), hopefully on a reduced treatment regime that he can manage on his own, with supervision. I know my anxiety about this will be far higher than his, which is how it should be. 

Today marks the first day of the UK CF week. I have always supported and promoted this, as I can say for sure that more awareness of CF would benefit people with CF, it is still so often confused with other conditions and misunderstood, and this is really difficult at times. I've even had a GP ask me how long Isaac has had CF....! So please, share my posts and encourage people to sign up for organ donation. Some relevant ones can be found here and here about CF myths and here about giving life. Thank you. 

Colour run and other stuff

Isaac, along with his Dad, cousin Ben and Auntie Jo completed his first 5km colour run today, and had a great time. This is a run with 20 obstacles and a whole lot of colour. Sounds easy, but not so much when you have CF and his current nose/tummy problems. Thank you so much for the sponsors so far, and if you'd still like to, click on the image of him on the right in the red t-shirt. 

 

Isaac and Ben, best friends and cousins. 

 

 

Ben. First one over the line. 

 

My lovely and ever supportive sister, Jo. 

I have not been feeling so great lately, which is weird, as Isaac has been more stable. When I was a teenager myself I swore that if I were ever a parent I'd remember exactly how it felt, and therefore I would totally ace the teenage/parent relationship, right? I thought I'd be SO much better at this than I am. Most of all I never wanted to be a nag. But the hand dealt to us by the genetic lottery means our son has so much more to do each day than most, and at times that feels like all I do...

Even when well his day consists of physio therapy, nebuliser one, nebuliser two, nasal sprays, inhalers, medicines he hates but has to drink, tablets, even more tablets, more physio, nebuliser 3, nebuliser 4, nebuliser 5 (hell yeah, CF includes a LOT of breathing treatments!) more tablets, medicines he hates but has to drink, and many more tablets whenever he eats, all interspersed with a Mum checking he feels OK, requests for coughing up sputum samples for the lab, a good 'hands round the chest' huff to check his lungs, O2 saturations and temperature checking, plus all the usual homework, teeth brushing, eating both vertically and with cutlery (this request still surprises him) that we have to ask of him. 

Now we can ensure he does his treatments every single day, 100% of the time, but what we cannot ensure is to what effect he does these, as he needs to put the effort into his breath to do breathing treatments effectively, and we can't measure that at home. Therefore, he insists he has done enough (the required number of breaths, huffs and coughs) but with effort of a geriatric mouse, and so we can't be sure that his chest is cleared of the infected, inflammation causing mucous that clogs his airways. The teenage years are about giving our kids the responsibility for their own lives little by little, and we have to allow them to make their own decisions, however good or bad. The only difference for us is the potential impact of those bad decisions. We have fought hard to keep his lungs as well as we possibly can for 12 years, and seeing him resist, cheat, and lie through treatments leaves us heartbroken. 

People often ask me how we cope, with three kids, both working full time, and with CF to boot. And the truth is, I don't always, at least not very well. And just lately I have felt the balance between coping and not has been kind of, well.... wonky. You know how they say people think about sex every 7 seconds or something? I doubt people look back on their day and think, wow, I thought about sex 376 times today! It's a back of the mind kind of thing, and that's how I feel about CF too. It doesn't dominate our lives exactly, but we sure as hell live alongside it. It is always there. (edit; actually, the whole 7 second thing I might have confused with a goldfishes memory? Either way, hopefully you catch my drift). 

In a crisis, I am a lioness for my cubs; I generally do OK in hospital, even when it has been very hard. And at times, it has. It's when I get home that I crumble (typically by popping open the gin and listen to The Cure or Patti Smith on my headphones very loud and having a little cry). It might surprise a lot of people who know me that I take antidepressants for anxiety,  and have done for many years, and I don't mind saying that, as I feel passionately about mental health awareness. Why should I hide this fact, but openly talk about physical problems? (And everyone knows that I'm a chronic oversharer on health generally (see previous blogs from earlier this year about living with a catheter bag!). It's all the rage anyway.... Prince Harry is talking about mental health, as are other celebs, so we must too, I think its great and does go some way to dispelling some perceived shame about feeling a bit shit. 

I asked my best friends to describe me in three words, and they said; positive, loving and empathetic; creative, honest and generous; bubbly, fun-loving and thoughtful; Spirited, loving and imaginative. This was not me fishing for compliments (although it did cheer me up some, as I like to think I am all of those things to my friends, as I love them dearly) I'm trying to illustrate the point that you can suffer from anxiety, in truck-load of worry proportions, which at times can feel quite debilitating, but also be optimistic, happy, and be a good Mum. And I really am happy, and I do feel like a really lucky person. It's just OK to be anxious, sad, and admit that you're not coping so well at times too. For me, this means focussing more on doing what I like most, hugging my kids until they squirm, spending more time with those closest to me, and figuring out how to get that balance better between work and play. And since many people with CF suffer from poor mental health at times, due to symptoms, limited life expectancy or the burden of treatments, I hope I am showing Isaac that it's OK to not be quite so OK always too. 

Have a great day, but if you don't, that's OK too x 

Thank you.

I've had a wonderful week long celebration for my 40th birthday. Thank you so much to everyone who donated online and at the party, raising £400 for the CF Trust on the night, this is the best present! My feet ache from dancing, my ribs from laughing; the usual signs of a good party. 

Isaac, Dan, Auntie Jo and cousin Ben will be running the 5K obstacle colour run on Saturday - you can sponsor them here. Thank you so much for your support friends and family - it really will make a difference, and means the world to us x 

She doth protest!

I will be joining the protest in London (read more here) to campaign for the rightful access to the life changing drug Orkambi for the 40% of CF sufferers that this drug will be suitable for; the first that any of them will have taken that treats the underlying cause of cystic fibrosis rather than just the symptoms. This drug, a so called precision drug, can have a huge impact on quality of life by reducing the amount of time spent in hospital and slowing the decline in lung function experienced by people with CF.

Sadly this is not a drug which will benefit Isaac (due to his class of mutation, which you can read more about here) but this fight is no less personal to me. 

CF friends, as it stands, I am going alone to Whitehall, so if anyone is in the same boat and wants to meet up, please PM me through FB. I'd love some company! 

Have a great day all. You can read more about Orkambi here x

Dreams of you all through my head

We've had a busy few weeks. Isaac is.... OK. We think. Pretty hard to tell. His tummy troubles rumble on, from one extreme to another. Some days we're all loving cuddles and family closeness. Others it's shouting and angst; all otherwise known as the teenage years. Yep, he's only 12, but he's always been one step ahead of most. We can laugh through most anything, but I'd be lying if I said it never made me cry.

It's never easy to explain, but he ALWAYS has a chest infection. In his case, BPA (Bloody Pseudomonas Auriginosa). But this isn't how you might imagine it. Chest infections in people with CF are rarely associated with fever, like we might have. The BPA set up their little camps in his lungs, and it's only when they decide to have a little party down there (known in CF as an exacerbation) that he gets more symptomatic, but that can come on pretty quickly. Right now, his BPA is revealing itself quite clearly in his sputum lab results (doing a little bug twerk in our faces) but chest wise, he is feeling OK. It's like the lull before the storm. All we can do is extra physio, and have extra antibiotics at the ready. We can't always jump on them straight away, as we can't afford to overuse antibiotics for fear of resistance. 

In the meantime, we're out and about having fun. Please, if you can spare a little, sponsor Isaac for his 5km colour run by clicking on the image of him looking handsome and moody to the right. He is so chuffed with the reponse so far. Have a great day x 

 

   

Looking good.

Isaac has had a fairly rough few weeks, but we're hopeful things are beginning to get better. He continues to miss too much school, deals with pain and nausea most mornings, and we're still not sure if he is taking is medication while he's at school (much to my endless frustration and heartache). It's very hard to explain to people sometimes, as within a day or so, he can go from being sick of a morning, to hospital for emergency X-rays, and then back to the football pitch for a training session.... this blog sums up this CF roller coaster pretty well. We take things hour to hour, minute to minute, if he is well enough that moment, he's going...Seize the moment! It is music to my ears when people say he looks well - if only he always felt the same. 

Please please sponsor him for his colour run here, we need a cure to this shitty disease x 

My girlies, Rosa and Anouk (I really don't know what I'd do without these two ❤️). 

 

  

Colour Run

On June 3rd Isaac and Dan will be completing their first 5K run together, including obstacles and a whole lot of colour (details here in case any friends would like to join them?). Isaac is just old enough to do this; the course would be a struggle for any child, let alone a kid with CF with ongoing chest and tummy problems, so please please support him and the Cystic Fibrosis Trust by sponsoring him here.

Thank you so much! x

Flying visit

We are home from a quick admission (DIOS related) where everything went pretty OK. We had hoped his tummy problems were somewhat behind us, as he hasn't been admitted for this in over a year now, but hey ho. He has a CT scan this week, and a DEXA scan and CF clinic next week, so it's still pretty full on CF-styley right now. But the main thing is he is feeling much better. Plus he is dropping three medications (which seem to no longer work for him, at least for now, he may just need a rest from them) and gaining just one new one. It's very rare for us to reduce his prescription list. We really need this one to work (the alternatives are a lot more invasive).... It does sometimes feel like we've patched him up, but only for now. Anyway, it's not all bad being in hospital, we always have some fun too. He is the best company! This time, he was hooked up to an IV line all day, which for a change had some pretty sturdy wheels on it, so he skated down to the concourse on its wheels to a series of funny looks (we both find commotion he causes pretty amusing... yes, sick people have fun too!). He loved making a short film of our day, and here are some stills from that. We are feeling hopeful and happy. Have a great day x

    

Spinning plates

My week; Doctors appointments for two of the kids. One blood test. One lovely friend leaving work. One parents evening (during which the first teacher I saw kicked off with 'Isaac misses a lot of school....'. Yeah? No shit Sherlock). One mad dash to the hospital for X-rays and to see Isaac's consultant.... resulting in one admission to hospital (tomorrow). Most busy at work I've been all year, Monday deadline looming. Did I mention that I also work full time now?

I walked out of school in tears; mostly Isaac does OK, but he misses so much he struggles. I feel both proud that he manages to keep up as much as he does, and gutted that he has to just keep up.  Now my tears have turned to frustration, my head is spinning and screaming 'Why do people vote UKIP/Tory/Trump??? Why the hell do people fly-tip? Why do people walk/cycle/drive SO slowly whenever I'm in a hurry? Why do people wear trainer sandals? .....but mostly, why is it just one thing after another for our lovely boy?'. Tonight is very much a gin night.

My eternal adoration goes out to our amazing CF team, especially our consultant, who saw us first thing, out of clinic time, and came to our rescue once again. She spent a long time with us talking about our options, and checking first with her colleagues from Gastro for a second opinion, and chasing up ENT for his surgery date. Also X-ray who got us in and out within 10 minutes, and the ward which rang me shortly after with a check-in time for tomorrow morning. We hope this admission will be a flying visit. Less ranty updates to follow. Whatever way you vote in this general election, I urge you to check their policy on the NHS and consider this very high up on your agenda; It's at the top of mine. We will all need it one day, and we need to protect it.

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