Home.

We are home! Can't tell you how happy we are. Discharge day is always the hardest for us; once you have your hopes up, the delays in actually getting out the door become mega frustrating. No one is to blame for this (but Jeremy Cunt certainly hasn't helped the situation), so you don't get mad at anyone, just at the situation. To get out, we need a discharge letter, drugs from pharmacy, and medical equipment in order for me to do his IV's at home for the next week. This took eleven hours. We arrived home more exhausted, both mentally and physically, than when we went in. 

Home IV's on top of all his usual treatments is a pretty full in schedule, but we are so thankful to be home, we don't care. 

The ward at night. 

Our home IV station, and a card from his sister. 

One evenings medications. The girls still call his long line a wiggly (just as he used to). 

The empties after making up IV's. 

The final IV doses for the night all made up, gloved and plastic aproned, administered by moi! 

Part six and seven.

Sorry for the lack of updates; we've been enjoying ourselves too much. Due to the bank holiday weekend, and there being an absence of doctors, Isaac was able to come home for a few hours each day. Some home time makes all the difference. We are now back on the ward, in our third bed of the week, and in a much nicer room, with huge windows overlooking the garden. This makes me very happy. I feel like we have room to breathe. 

Tomorrow we will stay here to have lots more tests, and results, in the hope that they will agree to let us go home on IV's for the next week. To get home, I need to demonstrate that I can mix up and administer the drugs  (uber sterile, gloved and gowned up) three times. One done tonight, another later, and then again at 6am. Although his long-line has been getting stiff to push the drugs through, it seemed OK tonight, so we just have to hope it lasts the next week. They always aim for a course of 14 days with IV antibiotics, but we've previously done anything between 10 and 21, due to either the line failing early, or him still being unwell after two weeks. 

He is still cheerful and is coughing less. The not so good news is that his O2 sats are very low after exercise (moderate hypoxia), I need to speak to his doctors further about this, to understand what this means. Really hoping for some better news tomorrow. Had a very long and lovely hug from my dear Mum earlier. We both needed that. 

The view from our room. See that door down the very very very far end? That's where I have to go for my cup of tea. Helps pass the time x

Part five.

Isaac came home today! At least for a few hours between physio and IV's. He got to see some of his friends and bounce around on the trampoline for a while (having an IV line in doesn't stop him doing much!) and we got to be home together as a family for the first time in two weeks (as I was travelling for work before we went in to hospital). 

Its been a great day, but watching his friends going off to the park while he had to return to the ward was another teary moment, for me. As always Isaac is smiling, and I couldn't be prouder. 

Part four.

CF, the gift that never stops giving....Results we've had back this week; 

His bone density scans came back as low. Meaning he is more at risk of osteoporosis and general breakages. This despite being on the maximum vitamin and calcium supplements he can be. We need to increase weight bearing exercises. 

His lung function has dropped. But this isn't altogether surprising, this early on IV's. 

Still waiting for blood and sputum lab results. I'm worried about these. 

Some concern has been raised about how many good veins he might have left, given the number of long/PICC lines he has had in the past, and a portacath been mentioned again. These are permanent implanted IV devices that are inserted into the chest (or arm) in surgery, that should last many years, and can be easily accessed every time he needs IV's. We've been pretty lucky to last without one all this time, purely because he is very good at having lines inserted and has no fear of needles (mostly due to his love of laughing gas - did I ever tell you, Isaac means 'laughing one' - so apt!). There are only so many places on a body they can site ports, before they run out of veins they can use too, so in some ways, the longer he can put this off for, the better, but it may be happening sooner rather than later at this rate. 

Today I have been at home with the girly ones, watching Flicka movies and making slime (their latest fads). Isaac is in good spirits still. I miss our family all being together under one roof, but I'm thankful that my boy is still smiling, to be here for now, and that everything is going well. Thank you for all the lovely messages x 

Part three.

Some other hospital observations; The parents room always has that slightly unlived in smell, and includes shared mugs that look clean, but that you will always pre-wash before using. Food is labelled in the fridge, which smells despite it being checked and emptied of old food weekly. Although Isaac is fed here, I am not. The hot food on the concourse is so expensive, I live on M&S salads and posh microwave meals. Whatever time of year, the ward is always hot. 

You can't help but parent watch in here. You can't help but overhear when all at divides you is a curtain. When others express sympathy to me about our incarcerations in hospital (usually about two/three times a year right now) I always explain how humbling hospital stays can be. Most of the time, we are in hospital to keep Isaac well, which is quite different to coming in to make you well, or for respite care, or worse, end of life care. 

As well as some heart-aching stories of support and love, and many many loving and incredible parents, you also see other parents using nurses as child-care, leaving for  hours or even days at a time. You see young parents not coping. You see quiet toddlers, seemingly afraid to cry. You see couples arguing. You see children with carers, whose parents never visit. You see distraught families.  

Being on the ward also reminds me of Isaac being young. We spent a lot of his first year in hospital, as he caught Bronchiolitis twice. It wasn't until he was much older that Dan and I would share the nights on the ward. As a baby it wasn't an option because I was breastfeeding. Although I feel almost traumatised by some of these memories (the failed PICC or long lines, that took hours to get in while he screamed, only to fail again later that day... the oxygen and still his O2 saturations dropping below 90...). I also have lovely memories of rocking him to sleep in the big wooden rocking chair, him speeding around the corridors in a baby walker, giggling all the way as he dipped in and out of rooms (long hospital corridors rock for vehicles on wheels!). 

Tonight Dan and I have swapped over, so I can have a much needed night with the girls. It's so lovely being home, with them, on our own sofa, with Obie  sitting on my feet. So why does it feel so wrong? 

Part two.

Tea. Lots of eye rubbing. Attempt to tame bed hair. Nebuliser. Physio. Oral meds. Shower. Breakfast. IV's. Nebuliser. Gym and physio. Nebuliser. Lunch. Oral meds. Nebuliser. Visitors arrive (thank you so much Bon, Sonny and Asher). Gym and physio. Visitors leave. Dinner. Nebuliser. IV's. Bloods. Dan and the girls arrive (hell, I have missed them). IV's. Nebuliser. Oral meds. Dan and girls leave (there are tears). IV's. Bed. Babies crying. Machines beeping. Finally sleep. 3am IV's.... 

It has mostly been a good day. Isaac is in good spirits, and his friends visiting cheered him up no end. He struggled at the gym (breathless, coughing) but it's normal to feel worse on IV's before you start to feel better. His tolerance and humour amaze me always. Tomorrow night Dan and I will swap over, so I can have some much needed time with Anouk and Rosa too. 

My main problem is that the Toblerone remains wrapped and elusive. It's killing me. What kind of hell is this? 

Our hospital garden. My some time sanctuary; 

Hospital diary part 1.

I'm going to try and write a daily update of this hospital admission, to give you an idea of hospital life with a handsome, lovable, funny, but often grumpy teen. 

Day one: Today started with the usual will they or won't they question over availability of a hospital bed. When you have your bags packed and mind set on going in, it's understandable but frustrating when they don't have room. Today we are lucky, and have a bed by lunchtime. 

Arrive to find that it is the WORST BED EVER. The middle bed of a 6 bed ward. This means we are surrounded by curtains. Even a window view of the opposite wall would be better. The main problem is we have only a bed, a fold down bed for me, and one small cabinet for our stuff. This is impossible with the amount of medical equipment we have with us. While we are in, I still need to do his usual treatments, which include five nebulisers a day, which means bringing two different machines and all the paraphanalia these involve. We're told we will be moved as soon as something bigger is available, but it's still likely to be on a mixed ward rather than a side room. 

Worse still, there is another kid with CF on the ward, and he's an older child too. This means we can't go in the teenagers room, due to cross infection risks between people with CF. The kind of opportunistic little bugs which love the CF lung are not the kind that usually affect other healthy people. But between CFers, these bugs would spread rapidly given half the chance. The teenagers room was our life line last time we were in. He's too old for the kids playroom, so this leaves us only the garden. This only increases our feeling of claustrophobia..... and it's only day one. 

After a few hours, the doctor makes it up from clinic and we have his long line inserted. This is simply a cannula with a longer tube which threads up his vein from his elbow towards his shoulder, to administer his antibiotics directly into his blood (intravenous). Thankfully this time it goes in first time. It still takes about 25 minutes and he uses entinox (laughing gas) throughout. Once he is all stuck down and bandaged up, I guide the drunken teen back to his bed. 

An hour or so after this, he has his first IVs, and we spend another hour or so doing his usual stuff. 

Move beds, to the corner of the same room. At least we have room now to do his drugs properly. We have a couple of toddlers and a baby in the room, and a young girl whose Mum has gone home. It will be a long night for us all. I miss my girls, Dan, Obie and my bed. 

I fall sleep about 10pm, still jet lagged, and mildly annoyed that he hasn't opened his Toblerone so I can't possibly steal a chunk undetected. 

Wake up at midnight to find that he's still on his laptop. Oops. 

Nurse comes at 3am to do his second lot of IVs but we both sleep through it. Pretty good start all in all. 

Screaming.

I have just walked sufficiently far down the fen with Obie (the handsome dog) in order to scream out loud. 

Clinic today and it turns out we're heading back into hospital for a stay. I say back in, but to be fair, we have had a good stint out, so shouldn't complain. We have sent in a few more sputum samples than usual lately, as his cough has been worse (the highlight of our lovely CF nurses day must be opening the post to a juicy pot of mucous for the lab). The results show that despite two long courses of extra antibiotics (Cipro), his Pseudo infection is rampant, and has been joined by another bug for good measure. The decision was made before we'd even been reviewed; Two week course of IV antibiotics with a whole heap of physio thrown in for good measure. 

It's not unusual for people with CF to go in for IV antibiotics as a kind of 'tune up' to dampen down the infections in their lungs, regardless of how symptomatic they are. Isaac, on the otherhand, has managed up to now on adhoc courses (usually twice a year, ish) when he is less well, his lung function drops, or the lab results show rampant infection, but it feels like we might be heading towards a more regular tune up, typically every three months. 

The extra stress right now is that I am due to fly to Mexico City in two days time for work. We have agreed to wait until I return to go in. Our consultant has reassured me that this is in no way putting Isaac at risk. His lung function is stable, and he can have more Cipro for now, which we know holds the infection at bay. The problem is that it runs rampage in his lungs every time the Cipro ends - which we hope the IVs will put an end to. For now at least. 

To add to this, his surgery on his nose has been delayed - despite asking to go on a cancellation list (we live so locally to the hospital and can run him in anytime) they failed to actually do this, and it turns out the waiting list is 5-6 months, and he is at the bottom. He has polyps and massive inflammation, meaning he can hardly breathe through his nose at all, and is most likely infected there too, which in turn may be reinfecting his lungs. Despite this, he was given no priority on the list. Thankfully today, our consultant will write to explain why this is urgent, and we are to write ourselves and complain, which will allow this issue get to the powers that be, and she is confident that we can get bumped up the list. I'm sorry, but... child...nose... lungs.... life threatening condition.... it's not hard to figure out. 

I have yet to tell my little girls that not only will they miss me for a week for work, but then I'll be heading straight to the ward with Isaac. Another heartbreak. 

And so I scream. And cry. And then feel a bit better....

Isaac on the otherhand is absolutely fine about going in; Looking forward to the laughing gas he will get to have his IV line inserted; the ward chef at his beck and call; fun in the gym; and extra time gaming as the drugs infuse. His only gripe is that it will be the last two weeks of his school holidays, rather than missing school. Gotta love that kid. How can I complain when he doesn't? 

This Klee pretty much illustrates how I feel right now (especially as I was hit round the back of the head quite ferociously playing dodgeball at a trampoline park yesterday.... did not feel funny at the time, frickin kids!). Goodnight all x 

Disinformation annoyance.

This kind of shnizzle really annoys me. One study, widely publisised on the news last week, which means many will now cut short courses of antibiotics as soon as they start to feel better, now believing they might be doing us all a favour. 

Standard antibiotic treatment calls for taking the medications for a definite period – even if symptoms clear up. The sustained dosage is needed to make sure that all the bacteria are killed. However, when treatment is stopped early, some bacteria survive and mutate into super bugs with enhanced resistance to the antibiotic. This is why we must always complete prescribed treatment, even if symptoms clear up. Otherwise not only do we avoid killing present bugs, we may be helping stimulate the development of drug-resistant bacteria. So fine, it's good that we recognise that an 8 stone granny with a toe infection might not need the same dose as a 16 stone builder with the same - but this kind of reporting does not illustrate that point well, it just gives protagonistic headlines which will speak to many who won't read the small print, and will believe it. 

Sorry, I know I have blogged about this many times. But the cumulative effect of our misuse and overuse of these drugs is undermining a once-powerful treatment tool. Today, when you go to the hospital with a serious infection, there’s no certainty that it can be controlled - Particularly with vulnerable patients – a person with CF, a child, an elderly person or someone with a compromised immune system. 

I am no expert. Maybe I am wrong. But nor are these journalists reporting on a single study. Please follow your doctors advice. And likewise, if you are eligible for a flu jab this autumn, remember that it's not just for you, it helps protect the less well around you too. 

Big love x 

The grumpy but handsome freckle teen ❤️

Going away.

I am going to Mexico City for a week fairly soon. I've had the joy of travelling a little for work over the last 14 years (I started the job thinking I would stay a couple of years, and never left, the people are too lovely!) maybe once every couple of years, but more often in the last few years. Visiting printers and our other publishing offices around the world. As someone who never went travelling after uni and regretted it, it's a wonderful opportunity to see the wonderful sights of the world, and work in other cultures. 

But the idea of a week long trip, to so far away would have filled me with fear when Isaac was younger. To in no way belittle Dan's role as a parent (he is a fantastic Dad), when you're young and poorly, sometime you just want Mum. I would worry no end that he would get sick while I was away and I wouldn't be there for him, not to mention the cost to the company if I had to suddenly come home. I had to pull out of a trip to Milan the day before we flew once. But now... things do seem easier. 

There is nothing harder than your child being in pain, unwell, or symptomatic without knowing why when they can't call and tell you how they feel. There is something very special about being the one to wrap your arms around your kid and know that they feel somewhat better just because it's you. There is something very intuitive about a Mum and child, especially when they're sick; I can feel and hear the difference between normal cough and a crackle coming from his lower right lobe. I would often wake at night when he was little, knowing he needed me before even he did. 

But now, thanks to a wonderfully supportive family, Skype, and a fantastic kid who is happy for me to go away, and can tell me exactly how he feels on the phone, I can go away in peace and enjoy my adventure...

OK, admittedly I WILL worry, but then I always do. But only a little more than usual from afar. Frida Kahlo Museum and Teotihuacan Pyramids - here I come! 

I hope you have wonderful travels too x 

Antibiotics will not cure viruses!

"The increasing prevalence of bacteria that are resistant to antibiotics is a potential problem for everyone. However, for people with cystic fibrosis (CF) it is a matter of life and death. Long-term and acute bacterial infections cause damage to the lungs of people with CF, resulting in gradual respiratory failure and the eventual need for a lung transplant, or even death. Antibiotics are essential for fighting these infections, preventing or delaying damage and prolonging survival. Over the coming years it is essential that antibiotics are used appropriately and with care to reduce the number of opportunities for bacteria to develop resistance.

There needs to be a global reduction in the use of antibiotics. They are frequently used in agriculture, and are often prescribed for conditions that are not treatable with antibiotic drugs. As understanding of the problem increases, steps need to be taken to reduce these practices. If you don’t have cystic fibrosis, you can help by not asking your doctor to prescribe antibiotics for conditions for which they won’t work, such as flu or the common cold."

I have blogged about this quite passionately before here. Please spread awareness that insisting on antibiotics for what may only be a virus puts others (who really need them) at risk. 

Have a lovely picture to brighten up a more negative note x 

Home with the boy wonder.

Isaac came home from camp full of stories of fun, late nights, fresh air and new friends. Just what we wanted for him. Unfortunately, he also came home full of chesty cough. We heard later that the canvas tents they stayed in were damp and may have been mouldy. The worst kind of environment for a kid with lung problems. That said, he was on reduced treatment while away, to avoid taking two nebuliser machines (which require mains power, sterilising of equipment, and an hour or so to complete) so whether the chestiness is due to the dampness or just doing less treatments (all though he was also on added Ciprofloxacin) we can't say. A week home and his chest is much clearer, and he made it through his last week of school without missing a day, despite also being sick on a couple of mornings. Things are looking up. 

My dad (THE most generous man to ever live) has a caravan at the coast, which we all visit often. It's a family and friends site, strictly no renting out, in a beautiful woody field on a cliff edge, not far from Southwold. It's a far cry from holiday rental sites, where the vans are packed in, all white picket fences. It's surrounded by woods, the sea, loads of green space, and we're beginning to know our neighbours. It's like a home from home - just way more relaxing.  This weekend Dan has taken the girls down with a friend, so Isaac, Obie and I are home alone. A time to bond, I thought. And then I remembered that he's strictly teeny these days. His idea of us spending a weekend together means him playing PS4 and me reading my book in the garden. This might do for tonight, but I'll force myself on him tomorrow, hopefully by kicking some serious Isey butt at pool (that said.... he is starting to get pretty good... ). To do anything together I have to remind him of the IRL (in real life) way in which some people still converse. Imagine! 

School is out, and I'm only working three days a week over the holidays. Lots of long weekends to look forward to, both at the caravan with friends and family, and home, which I need, and which Isaac really needs. First year of secondary school done. Phew. 

Sending big love out to my cousin, who will also have a son given a shitty hand in the genetic lottery of life - we're thinking of you Sian, and wish you all love. 

And also to baby Ned, who should be putting in an appearance this week - happy birth-day - we can't wait to meet you. 

Happy holidays all x 

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