High tech care.

We are still in PICU (Paediatric intensive care unit) and Isaac is having help with his breathing. This sounds more invasive that it actually is though; he is still breathing for himself, this just helps by increasing the flow and the oxygen to his lungs so he doesn't have to work so hard. He still needs a whacking amount of O2, between 15 and 20 litres. To put this into perspective, he was on just 3 litres to begin with. The hope now is to gradually decrease this, but for his sats to remain high. This may take a few days, and attempts to lower it today have failed. He is on some new treatments, and is having lots more physio (using a device called a Bird, which helps force his airways open by giving him high dose O2 under increased pressure). The best news is that he is sleeping better at last, and is a lot more comfortable. This is our spot in PICU; 

Despite everything, Isaac thanks everyone that comes to treat him. He can't leave his bed area, so has accepted things like bed baths, commodes and pee pots without complaint. Whereas a few days ago (sorry, I really can't remember what day it is now, what happened when, or my own name anymore) he was battling with us; pulling off his O2 all the time, and finding physio so hard he didn't want to even try, he now actively wants physio, to help shift the gunk on his chest, and becomes distressed without O2 even for a short time. He desperately wants to feel better. He has also wanted both Dan and I to be here with him, which he's never asked for before. It's not PICU that has been scary for him (it's so reassuring to be up here, in this super high tech environment, with amazingly calm one to one nursing care) - but the breathlessness and gasping for air has been traumatic beyond words. 

There are no beds for parents on PICU like we have on the ward. After a few hours here last night they bought me this chair; 

Which is THE MOST COMFORTABLE CHAIR EVER - a fully reclining piece of medical-blue faux leather sumptuousness of great splendour and magnificence, like a bed of roses for a tired and worried parent. I may have cried a little. 

We also have access to a house on site here - managed by the Sick Children's Trust, and run by volunteers. While Dan is here I've been able to go over there for a nap. Again, I may have cried. A few days ago I felt kind of numb, emotionally deadened. Unable to process everything that was happening so suddenly. But today I pass a wee baby on PICU every time I go to the loo, and this has me in floods every time. Isaac is nearly my size now, but in my mind, he will forever be like this. 

He is asleep again now, and this is the best thing for him. He is being an absolute hero. I can't tell you how proud we are, or how much we love him. As we have told him a hundred times, he is not alone in this, we are a team, and CF can do one! 

Thanks so much for all the love, messages, and to everyone helping with Anouk and Rosa, as this is scary for them too. As always, knowing they are happy means we can concentrate on looking after Isaac, so this means the world to us x 

PICU.

Quick update to say we are now on PICU (kiddie intensive care). Isaac is getting a little help with his breathing, and is on 20 litres of oxygen. He is more comfortable already, and I'm hoping he'll get some sleep soon. 

Exhausted.

We have been moved to a new bed right by the nurses station for closer monitoring. I am sat opposite a cheerful sign that says 'Smile each day!' - and I'm bloody trying to, but Isaac is more poorly everyday. His oxygen saturation levels are monitored constantly, and you fast become obsessed with the numbers. Yesterday I was willing it to stay above 90, and did a little celebratory dance at 94. Today he is struggling to get to 88 on 5 litres of oxygen, and 78 is his new low. He preferred the nasal specs he used for a while last night, but really needs humidified O2 which means a mask instead. This is warm, wet and very noisy. He is exhausted from coughing so much, as well as having very very little sleep. He can no longer have even short breaks from the O2, going to the toilet leaves him gasping and dizzy. When he does eat (very little) he has to hold the mask near his mouth while he chews. Apple juice through a straw in the gaps of the mask are our best way of keeping his energy up. 

They have added a new IV antibiotic to the mix, to make it three different drugs (Ceftaz, Tobi and Meropenem) in seven separate doses. Strangely my job has become easier, as he's temporarily dropping four of his usual nebuliser drugs, as they simply aren't cutting through the gunk on his chest. He wheezes, crackles and sounds like he is breathing through treacle - this is endurance breathing; like running a marathon in a spacesuit. Tired doesn't quite cut it. If he continues to stuggle we will be moved to the high dependency unit so that he can use a C-PAP machine; 

"Continuous positive airway pressure (CPAP) is a form of positive airway pressure ventilator, which applies air pressure on a continuous basis to keep the airways continuously open in people who are able to breathe spontaneously on their own."

This will assist his breathing and he won't have to work so hard.

His job is to do all the physio, cough even when it hurts, take his IVs and keep his mask O2 on. Mine is to watch his numbers, give him his normal meds, chest percussion, foot rubs and encouragement. This won't last forever, but to him it feels like it might. 

Oxygen.

Isaac is now on oxygen. His saturations have dropped at worse to 79, but most the time hover around 88-92, and this is with 3 litres of oxygen supplementation. Ideally he should be at 94 or above on just air. Anything below 92 is a worry. He hates it. He needs humidified O2 to keep his mucous moist so he can cough it up, but this means a mask, and warm wet air. At night we struggle; he falls asleep and pulls off the mask. The alarm goes off. I wake and put it back on. He curses at me. We fall asleep. And repeat. This, along with a ward full of people and machines and endless beeping, equals very little sleep. He has been on O2 24 hours now, and still no sign of improving enough to come off it. 

I have wanted to cry a thousand times. But I only have twice. OK, maybe three. Part of my role here is to be the one he lashes out at when things are this bad. He is polite with the nurses, and for that I am proud. (NB; that is not to say I let him walk all over me, or that he often lashes out, but just that I understand that things are so frustrating for him, it is him going through this, not me, and I am his safe place, so it's a kind of back handed compliment). 

I've stood by the nurses station as 8 or more staff walk around me, me like a mute rabbit in headlights, they avoid all eye contact, all going about their work around me, but never actually acknowledging that I am there. All I need is a sick bowl - but everyone is too busy. I do every thing I possibly can to not take up any more of their time, but the damn sick bowls are in the sluice, locked away from me. The care, when they have the time, is faultless, they just don't have the resource to give the time and care they want to. This is the real state of our NHS. 

The good news from today is that we have a plan; He can drop the nebulised hypertonic saline, as he is coughing too much (I'm quite surprised he didn't produce a whole lung). But double up the DNAse, which helps thin his mucous. Thankfully a chest X-ray ruled out any pneumothorax (collapsed lung). So we can increase the physio, but not at the gym (he is not well enough to leave the ward now)  we'll go back to chest percussion and something called the Bird, which is a big retro looking machine that pushes air into his lungs. We will continue with the IV drugs he is on, awaiting lab results which may show something new, until then, we assume it's a flare up of his usual Pseudo infection. 

Tomorrow will be better. It must be. To all who have called, helped with the girls, sent food or love.... THANK YOU. x 

The 'Bird'. 

Not the best.

Two failed long-line attempts means he needs to put up with cannulas for longer. To insert a long-line, they put in a cannula, and through that thread a long thin wire up his vein, then thread onto the wire a very small tube, and push that up into the vein, and finally remove the wire; Voila, you have a very long thin tube in your vein, which lasts much longer than a normal cannula. Only his tube kept getting stuck at a valve, meaning a small cutting of skin to try and push it up, a fair bit of blood, and a big fat fail. Despite the laughing gas.... this was no laugh. He did not complain and we will try again tomorrow (he is a frickin hero our boy!). 

Physio at the gym was a non-starter; he was pale, clammy and coughing so much, he looked like he might pass out. Lung function was terrible; huge drop. LF is the measure of wellness in CF, but we're assuming today's reading was meaningless, as he was coughing so much, he just wasn't able to do the test well (to think otherwise is too depressing). He is eating very little, and the coughing zaps all of his energy, so he is not up for games. His oxygen blood saturations are too low, and I think might be causing his headaches. He has not been this unwell for a long time. 

Today was not a good day, but Isaac's response to anyone who ever asks him how his day has been is; Not the best. So let's go with that. I like his version better. 

Hoping I'll have better news for you soon. 18.40 on the ward, and we're already in our PJ's. Tonight will be a long night x 

Confinement.

Fours weeks ago today we left the ward, and today we return. Isaac is feeling rotten. Like, cough so much you're sick rotten. Again and again on repeat. His O2 sats are low, he is wheezing, he breathes with grunts, and his sputum is extra icky and green. None of these are good signs. Cannula is now in. IV's tonight. Tests tomorrow... then a plan will be formulated. He has not complained once. It doesn't take us long to get cosy and settle in. 

More soon x 

And I'll tell it and think it and speak it and breathe it.

Isaac is never one exactly keen to go to school. His mornings are mired with nausea and pain way too often, not to mention always being on catch up work from the days he misses. But today was his school sponsored walk, and he wanted to get up early, eager to go. But his chest was audible with crackles before he even entered the room. Cough after cough after cough. Productive enough to get a juicy sample for the lab (dropped off first thing today. Results won't be ready before Monday earliest). Until then, we have to rely on his usual antibiotics, extra physio and Cipro (his most powerful back up anti-bot). Clinic had advised last week to delay Cipro as it is too soon after his IVs, but he is now too poorly to put it off. We will give this a few days, and if no better, take him in. He is feeling so bad, he actually asked to go to hospital (which never happens). But he made it through all 16 miles of the walk. How amazing is that? Battling through with crappy, angry lungs, coughing all the way, he still won his own personal battle. 

CF is all consuming. It affects him in countless, immeasurable ways. Laying awake at night, listening to my boy go through this, is utterly heartbreaking. I can't describe it. I wrap my hands round his chest for physio (we do chest percussion when he is coughing this much, on top of his physio he does with his Aerobika device, as he gets so tired) and I don't just hear his rattle, his wheeze, his crackles, I feel them. 

At times like this, CF is all I can think about. I like to think I do all the worrying so he doesn't have to, but as he gets older, I'm not sure that's always true. 

A stormy autumn.

The kids are back to school, but already Isaac is coming home sick. Yesterday was not a good day. Today, Rosa was sent home after spontaneous vomiting in class too. This time of year is often not good for us. A new round of viruses to pick up and pass round the family. At the moment I'm hopeful that we can stay home and send in samples to the lab, which they grow to show any new or rampant bugs. All being well we stay home and put off CF clinic longer. He has had too many rounds of Cipro (the most powerful oral antibiotic that his bugs are sensitive to) this year to begin another so soon after IVs. Although he has other daily oral and nebuliser antibots, we have to reserve Cipro to occasional use on top of these, else his bugs will fast develop cunning ways to overcome it. He is still waiting on a date for his nasal surgery (which will involve removing CF related polyps he has grown, and widening his airways.... not nice, but very necessary) but his consultant is on the case. It's likely he will have more IVs at the time too, as the surgery and associated aneastetic will leave him open to infection rampage. I don't want to say the 'C' word in September, but I bloody hope it's well before that busy season, and we have him well and home for the holidays. 

The other night, unwell in bed in the early hours, he said 'Why me?'. 

It's times like those where I feel so helpless. 

And so I try and turn that helplessness round and start thinking about my next fundraiser..... core team (you know who you all are!)... anyone up for a skydive? 

This blog and home IV's.

I am often asked why I write this blog, and do I find it cathartic? The answer is absolutely yes, it feels so good to get things down in words, and I am a very open person generally, so happy to share (although of course I keep some things back that are not mine to share). 

I began this blog to raise awareness of Cystic Fibrosis, and Organ Donation. Although 1 in 25 people in the U.K carry the CF gene mutation (unknowingly usually, as carriers are healthy) only 1 in 2500 babies born have CF, and this amounts to around ten thousand people in the U.K. The most common, genetic, life threatening disease is still pretty rare. And increased awareness really does help, not only in raising more funds for research, but also to dispel myths, and reduce the number of crazy questions sufferers deal with. The organ donation part is linked as people with CF make up a large proportion of people on the waiting list for organs. The chances are, at some point, Isaac's lung health will be so low, transplantation will be his best option. Although this does mean swapping one disease, for another in a way (a lifetime of suppressed immune system and other drugs, along with their own special side effects). Currently half of those people on the waiting list for new lungs die waiting. 

The one thing I never wanted this blog to be about was pity, or to attract sympathy. I really hope that it comes across in the way I intend - to share, inform and give a glimpse into family life affected by a serious genetic disease, in a positive way, while still being very real. One thing to emphasise is that the treatment regime for CF is to treat infections very aggressively. This is why Isaac takes daily antibiotics, and occasionally has these courses of high dose (treatment doses in CF are much higher than for other people) IV antibiotics, which kick some serious bug butt. So whilst the treatments sound, and really are, pretty full on, this is all aimed at keeping him well, rather than because he is currently very un-well. As I am often told, you'd never know Isaac has CF to look at him. 

We talk very openly at home about CF, we never wanted to make Isaac feel it was something he should be secretive about, and he knows he can ask us anything freely, and without worrying about upsetting us. Anouk and Rosa talk quite proudly about their brothers CF, and that he has medicines into the 'wiggly' in his arm. They all understand that the many treatments that Isaac does, very much part of our everyday life, are doing just that; keeping him well, as such, we never refer to CF as being a sickness (although you will regularly find us all agreeing; CF sucks!). 

Some observations about home IV's; 

We very much prefer home IV's to staying in hospital (this study supports the idea too). It is no doubt easier for our family as a whole, and protects Isaac from the risk of picking up extra bugs in hospital. And whilst the physiotherapists do an amazing job, trying to increase his exercise and clear his chest, we can do more at home. He can't swim or do contact sports with a line in, but he can do most other things. 

If we do this more often (many kids with CF have regular courses of IV's every 12 weeks, rather than adhoc as Isaac does, maybe one or two times a year) we will need another blue bin. The amount of discarded packaging is immense! 

I am happy and willing to mix up and administer the IV drugs at home, which enable Isaac to spend less time in hospital. That said, I would be lying if I said it was easy, and I always felt relaxed doing it. Mixing up the drugs to give him includes many vials of drugs, various mixers, sterile wipes, needles, bungs, syringes, and making sure various parts don't ever make contact with other parts.... in hospital each dose drawn up by one nurse has to be checked and signed off by another; the patient, the dose, the use-by dates, whereas at home it's just me (thankfully with just a single patient mind). After all, I am pushing these powerful drugs up a line directly into his bloodstream. Problems can include spontaneous anaphylaxis, infection and the vein popping and the drugs going into his tissue instead of his blood. Scary stuff. 

My son is amazing. 

And so, we have come to the end of yet another course of IV's (have lost count how many he has had now), feeling pretty lucky. A single long-line, which came out today, has lasted the whole time; He's had very few side effects; We got to spend half the time at home, rather than the full two weeks in hospital; and most of all, he is feeling better. Plus I am the reigning queen of our new card game, Exploding Kittens. 

Huge thank you's to our parents for all their help, our visitors in hospital, Dan for surviving the long weekend on the ward while I got to spend some much needed time with our girls, and for all your lovely comments on FB. Isaac 1 - 0 CF x 

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