Love bombs

Two weeks of Cipro, and he seemed less symptomatic, but just a few days off, and he has been coughing again today. Hoping this is just a blip. Ideally, we want to put IVs off until July now, so we can go away afterwards while he is at his best lung health. 

Cipro and sunshine? Really don’t mix. This is OLD NEWS but he  learnt the hard way after jumping in and out of a pool all day last week. Hell, he gets a sunburn in winter on that antibiotic! He is so grown-up in many ways.... but we can’t rely on him caring two hoots about his health just yet. 

We have more information about CFRD now (CF related diabetes) and it seems he has a very high chance of developing this, and we’re to try and lower his carb intake to delay onset. We have yet to have any advice on how to do this exactly, so will be working with his Dieticians. To add diabetes to his long list of treatments would be, I fear, pretty traumatic for him (regular blood glucose testing and insulin injections a few times a day), but in true Isaac style, he’s not worrying about it until it happens (and currently has no interest in a low carb diet at all!). As well as the added treatment burden, CFRD will negatively impact his lung health, so definitely something we don’t want for him (expletives removed). 

His latest bloods also show a rising sensitivity to aspergillos (the flagitious fungus) that he also grew in October last year, and he continues to have heavy growths of PseudomonasA (the bastard bacteria).... all the while I read about the antibiotic crisis in the news (which I have blogged about here) a very real crisis that very few people seem to be talking about. 

On the flip side, reading other CF stories, I realise how fortunate we are that he has recovered most of his lung function following his time in ICU - not all are so lucky (and phrases like ‘sudden decline’ continue to chisel away at my mental wellbeing). So like him, I try to live more in the present day, and today.... things are OK. I have a few days off and plan to lay a love bomb on all of my kids in this time. Happy May to you all x 

Leggy, freckles, smiles.

Isaac’s coughing has been increasing day by day. 

His test results come in on a rolling basis (we are almost never not waiting for results); his infection markers from his latest bloods are looking OK, but he is still regularly growing (antibiotic sensitive) PseudoA, NTM first checks look promising, but take six weeks to prove clear, lung function is stable. His nasal polyps are back already, and he will likely need further surgery in the future. 

As we’re just six weeks clear of his last IV antibiotics, we’re trying a two week course of Cipro (orals) hoping to eek things out a bit longer before the next course. He is doing everything he can to stay well; meds, physio, nebulisers, extra exercise. He has stepped up his game.... will his lungs? We’re trying steroid nasal rinses to see if we can shrink the polyps. 

His latest ‘fasting blood glucose’ test result was abnormally high, which is new for us. This raises concerns of CF related diabetes (CFRD), a very real condition which has aspects of both type 1 and 2 diabetes, and is insulin dependant. In Isaac’s case, the duct from his pancreas to his guts was blocked in utero due to his sticky mucous. This meant he was completely dependant on synthetic enzymes (Creon) to digest any fats and proteins from birth, as his enzymes cannot escape his pancreas. In turn, these trapped enzymes damage the organ from within. And the pancreas’s other job? Insulin creation - hence the high likelihood of CFRD. This is something that will be monitored closely. Right now he doesn’t show any other obvious signs of CFRD as his height and weight are both good, so we wait, and hope. 

We go away this summer with friends again, and the hope is to schedule IV antibiotics in before we go, to ensure his lungs are tip-top before we go, reducing the chance of him getting another exacerbation while we’re away. Timing wise, this can be tricky, either we squeeze in two courses, by doing one soon, or hope he can wait, and have one course, but later. 

Always so much to think about. 

I got home from work tonight, and the kids were all playing football in our sunlit garden. I watched them for a moment. Isaac, all curly dark hair and freckles, Anouk all leggy and tanned, Rosa so dinky and all smiles. I am so, so lucky. 

£70,000 and counting....

Our skydive has now made over £6,000 (my employers will match what I make at work up to £500 per fy, and huge thank you’s to the Vargas Brothers for the most amazing fundraising BBQ) which means our running total; and this is by no means just me, but our whole family and bloody lovely friends, is now over £70,000 raised for the CF Trust  since Isaac was diagnosed in 2005.

Ahhh, words fail me.... Just thank you thank you thank you! It really does mean the world to us to feel so supported in this fight for a better future for Isaac and others with CF. 

Some pictures from our skydive. Shell, Jess and Bon.... there is literally no one I would rather throw myself out of a plane with. Love you all. 

CF update to follow, CF clinic and ENT review tomorrow. Cough slowly increasing x 

I am not dead.

Skydive complete! Pictures to follow. Can’t thank everyone enough for the support.... over £5000 for the CF Trust. Means the world to us all. 

For Shell, Jess and Bon, my fellow jumpers.... I love you so much, thank you thank you thank you x x x 

Spring.

The sun started to shine, which for me means padding around barefoot, and reading in the garden. 

I am utterly obsessed with my new book, My Absolute Darling (Gabriel Tallent); be warned, this is DARK (I love my books, music and art quite dark)... but unputdownable; in a jeez, this is frickin sick kind of way. But the sun seems to have gone back into hiding these last few days. Still, in the small window of early summer the kids spent a weekend in the paddling pool, which is pretty damn good for April in the U.K., and Isaac is doing..... OK. The results from the bronch trickle in... Pseudomonas infection; check. No surprise there, but symptomatically he is doing better, so we’re feeling good. Roll on summer for real... (and a few hospital free weeks please). 

Just the small matter of throwing myself out of a plane first....on Saturday. To say I am nervous is an understatement, but needs must. Everyone with CF deserves the chance of a longer, healthier life, so there is not much I wouldn’t do to help this cause. Please, please support our skydiving fundraising by clicking here.  We are so close to our target now! Post to follow on the amazing friends jumping with me, and all the support we have received. THANK YOU! x 

Bronchoscopy results....

.... were mixed. While they look at his lungs with a camera, they also flush the mucous out with saline, and these samples are sent to the lab. Results for these are outstanding (normal bacteria is grown in 3-7 days, but the NTM ones take up to 6 weeks), so no news on these yet. But the good news is that infection wise, his lungs looked good. This is in stark contrast to his last bronch in Oct/Nov last year, where we were told his lungs looked like ‘one of the worst CF lungs’ that the consultant had ever seen. Sorry if I repeat this often, I’m still haunted by it, and if I’m honest, a little pissed off. He was so sick back then that I think this was merely a snapshot of his lungs at that horrible time, rather than an indicator of his lung health more generally, but that was not what was implied (a later CT looked better also). 

Anyway... things look better now, infection/inflammation wise. But with good news, often comes bad... the bronch was performed by our favourite doctor, and she came straight from theatre to tell me that he has areas of ‘malacia’ which basically means some of his airways are too floppy. This is normal for little ones, but not for a teen. For a more medical description; 

“TM (Tracheomalacia) is significantly more common in infants and children with CF than in the general population and is associated with airway obstruction (lower FEV) and earlier Pseudomonas acquisition. TM is identified as a common co-morbidity of infants and children with CF. It appeared most commonly in children with other severe manifestations of CF such as meconium ileus and was not observed in pancreatic sufficient patients. TM was associated with some features of disease progression.” And “These disorders both tended to occur in children with more severe CFTRalleles, and our most severely affected patient with TM had meconium ileus and two Class I CFTRmutations (G542X and 3659delC).”

Kind of fits what we know already (Pseudo infection: check. Those exact class 1 mutations: check) and not great news. Only reassuring part is that the kind of physio that Isaac has at home, he uses BiPAP, is ideal in this condition as these kind of interventions stent open proximal airways and help relieve respiratory distress. 

So... Another worry to add to the list (to be discussed further with his team next clinic). On the positive side, he is recovering well from the bronch, his cough is calming, the sun is shining, and we’re hoping for a stretch of coughing-less, hospital-free time. 

For me, I am getting increasing scared by the idea of throwing myself out of a plane.... in less than two weeks! If you could sponsor me here I will be so grateful. I’m also coping with an Isaac-fiddled auto correct which means when I type ‘Hi’ it turns to ‘Daddy’, ‘love’ is ‘hate’, and ‘Hello’ is ‘nipples’. I have sent some seriously dubious text messages of late. Got to love that kid x 

Obie’s latest sleep pose. 

A closer look at his lungs.

Less than two weeks since his IV’s ended, and Isaac is coughing. His consultant rang me at work today to discuss this (she is so brilliant) and has suggested we hold on more antibiotics, and instead do a bronchoscopy to get a better picture of his lung health, and hopefully figure what the hell is going on (they hold on the extra antibiotics now because they want to suction off a mucous sample for the lab, and giving him Cipro now might just mask the problem). 

There are numerous ways in which they piece together a picture of his lungs; lung function tests, exercise tests, chest X-rays, listening to his lungs, sputum samples which they grow in the lab to see what infections he has, how he feels, CT scans, and bronchoscopies (a camera inserted into his lungs, and they flush out secretions with saline while they are down there). Each give a slightly different illustration of what is wrong. As his last bronch was so bad (“one of the worst CF lungs I have ever seen” the doctor said. Still haunts me) we are feeling both apprehensive, but also hopeful that this time, things will be different. Thankfully they can fit him in on Monday, so not long to wait, and it is our favourite consultant performing it this time. Yet another anaesthetic and theatre trip, but hoping this will be a fleeting visit to hospital unlike last time. 

He is not super unwell. He’s just not quite right either. 

In the meantime, have I mentioned lately that I’m throwing myself out of a plane soon? Everyone with CF deserves the chance of a longer, healthier life. Please support our skydiving fundraising by clicking here. Thank you x 

In Bristol. Cousin love 💕 Anouk and Bodhi. 

Needles and big fat fails.

After two weeks of IV antibiotics, Isaac was blowing better for his lung function tests, but not quite well enough to finish the IVs while his chest still sounded wet with huffing. And so the course was continued for an extra four days to top him up, bringing us up to the bank holiday weekend. This meant no clinic, and no community nurses, and although we have open access to the ward should we need it, we agreed that I could remove the needle from his port myself. Simple I thought....

Only, delivering drugs through a line into his blood stream is one thing, but actually inserting or removing that line (and gripper needle, which is in his port for the duration of the course of antibiotics) is kind of something else - his skin grows over his port, so it’s not like simply removing something from his port alone..... it’s pulling a needle through his flesh, my closest experience is home piercing ears as a teenager (not something I would recommend). 

So I braced myself; all I needed to do was flush the line with saline, followed by an extra strong Hepsal (like a very strong plug unblocker, to ensure the line in his chest remains clear for next time). I got my gauze and plasters ready for the bleeding. Isaac laid down. I gloved up, and got ready to pull. I anchored his port with one hand, and with the other pulled up hard. The needle made a popping, suctiony sound as released, and I stemmed the bleeding. I’d done it! My first time from his port....

Only as soon as it was out I realised that I’d forgotten the bloody Hepsal flush!  

Now I can tell you that there is not much worse than feeling that you have failed your child like this, especially one who endures so much already. I had been so focussed on the needle part, I had forgotten the simple flush. 

In tears I called the ward.... who were hugely reassuring. His line still has Hepsal in it, from the last dose of IVs I had given, only in a weaker solution. This means his line will be fine, only it’s likely he will need to be reaccessed on Tuesday now (the needle put back in) only to give him the correct flush, to ensure his line will be clear for next time. It’s not the end of the world, and Isaac is laughing about it, but kicking myself is an understatement x 

Drug mix up

This is tonight’s mixing up of drugs for Isaac’s IV antibiotics. Excuse my feck (attractive face/neck merger 😂).  If only it were this quick in real life! 

We are so so thankful to be doing home IV’s rather than in hospital again. It is day 11 of 14 (all going well with his tests on Tuesday, hoping it won’t be more than a two week course). Blood tests every three days show his kidneys are coping well with the toxicity of the drugs. We had one dash to hospital as his gripper needle dislodged, but this is most likely due to his port being new, we’re still sussing out what size needle is best for him. It’s all going well otherwise. Today he is coughing a lot more again, but I’m hoping this is just a big clear out (no more blood tf). 

Look at me, my first VLOG! I am totz down with dis, FML. 

Have a great weekend! 

Parliamentary debate.

Watching the parliamentary debate (here) tonight on Orkambi, a life changing precision drug which is only the second drug made available that treats the underlying cause of Cystic Fibrosis rather than just the symptoms. The stories are heart wrenching and all too familiar. Feeling super proud of the CF community who have petitioned and campaigned relentlessly for this debate, and have been protesting outside Westminster today. This CF community and the Cystic Fibrosis Trust together are well known to punch way above their weight politically. I so wanted to be there today, but as Isaac is currently on home IV’s, and I am the only one trained to give these, I couldn’t.

The debate raises so many interesting points, not least the question of what price you can put on a life? It also highlighted that middle ground that CF exists in; it is neither so rare that a high cost drug in a small population would be affordable, nor so common, like acute conditions, where a large population drives down the cost per patient. 

What is apparent, is that NICE must change the way in which they perform cost benefit analysis on drugs for chronic conditions: Every night we spend on the ward costs the NHS more than £400, and in PICU, more than £2000; more than one of Isaac’s daily drugs cost £8,000+ per annum... and at every single clinic visit he requires the care not only of his CF nurse, his CF consultants, but also a dietician, physiotherapist, psychologist and the pharmacist. He has also at various times been under the care of ENT, the Gastro team and Immunology, all related to his CF symptoms. He has multiple tests each year, including X-rays, CT’s, ultrasounds and bone scans, not to mention the regular bloods and sputum samples the lab deals with..... what this adds up to I can’t imagine. 

.... Orkambi has been proven to reduce admissions to hospital and reduce the decline in lung function by 42%. NICE agree the drug is effective, but not cost effective for the NHS (the original offer was over £100k per patient, per annum). I know none of this is simple, but the debate suggests to me that NICE is not fit to make that decision as they don’t fully understand the bigger picture in chronic conditions. 

There are around 70,000 people with CF in the world, and the U.K. and Ireland have a much higher prevelance of the condition than normal (10,400 in the U.K. alone). Orkambi is available in Ireland, but not in England. Government intervention between NHS England and the manufacturer, Vertex, is urgently required. I have no doubt an agreement will now be found, but everyday it is delayed, children are losing their lives. Half of sufferers will lose their life to CF before the age of 31 and imagining how those at that end stage of the spectrum feel about this delay is simply crushing. Yes, the cost is high, and it’s easy to blame that on the pharmaceutical company, but the portfolio offer to NHS England (Vertex have newer, more effective options to Orkambi already in development, and this portfolio would guarantee future access to these drugs also) is better than deals made in other counties that are already prescribing Orkambi. 

I’ve said before, Orkambi will not work for Isaac, nor will the next drug in the pipeline, Symdeko, nor even the one after that, as he has a rare and shitty mutuation of CF which will require a different kind of precision medicine to enable the correct movement of salt and water through his cells (the root problem in CF) but that doesn’t mean I’m not hugely passionate about access to the drug, and we are super excited about the paths it will open for further research that may one day help him, and move CF into a category like diabetes; a condition that you live with, rather than one that you die from. 

In the meantime, have I mentioned lately that I’m throwing myself out of a plane soon? Everyone with CF deserves the chance of a longer, healthier life. Please support our skydiving fundraising by clicking here. Thank you x 

That long black cloud is comin’ down...

“The friends and family who care for someone with a rare disease really become experts and the ‘voice of care,'” said Nicole Boice, the founder and CEO of Global Genes. Ninety-four percent become involved in care communication and advocacy work. Eighty-nine percent educate healthcare professionals about their care recipient’s condition, and 84 percent help with medical or nursing tasks. Caring for those with a rare disease comes with a lot of stresses and strains. Seventy-four percent of caregivers report struggling with a sense of loss about what their care recipient’s life could have been. And 67 percent report emotional stress —  twice as high as that for those caring for people with other conditions.”

Taken from an interesting article here. CF represents 9% of those rare diseases. 

Today we picked up his IV drugs and he had his port accessed again; a small needle called a gripper is pushed into his port, which the drugs will go through. This needle will stay in place for the whole course (hopefully just two weeks, depending on how he responds). 

Silver linings: at least we are home and not in hospital this time; he is sick, but not too sick; and not having to wait for a long-line thanks to the port is brilliant. 

Need to keep thinking of these positives. I miss Isaac being well. I miss Jayne, I have been thinking about her a lot lately. I admit I’m struggling a little more than usual right now...  the last six months have been tough and I’m not sure my muddled mind has fully caught up with everything. Working full time and raising three kids doesn’t allow a lot of time for contemplation. I’ll be OK. What I’d really like, is when friends ask how Isaac is, is to be able to say ‘He’s doing fine. Thank you for asking’. It feels like its been a LONG time since I’ve been able to do that. Things have got to get better. 

Picking up two weeks of IV drugs today. I bought my huge (and really damn cool) old lady shopper, thinking that would fit everything in. So wrong; 

BD, 1966.

Mama put my guns in the ground
I can't shoot them anymore
That cold black cloud is comin' down
Feels like I'm knockin' on heaven's door.

With a lot of tests, come lots of results.

Today was ‘not the best’ (my favourite of Isaac’s quotes, however hard things get). 

His lung function had dropped some 10-15% since his last test (January) and so he needs his fourth course of IV antibiotics in the last 8 months, his worst run yet. After the really shitty time he had in October last year, the plan was to switch from ‘IVs as required’ to ‘planned IVs every 12 weeks’.... only he hasn’t been well enough to last more than 8 weeks since then before needing more courses to help battle his infections. His doctor thinks this may be because he is still recovering from the October exacerbation and hopefully this will improve and we can start to stretch out the good bits inbetween over time. 

That wasn’t the only bad news: The data from his nebuliser was downloaded in clinic, and disappointingly showed that while he is doing all his treatments, he is not always completing them. His ineb beeps when it’s finished and shows a smiley face screen; sounds straightforward to monitor, right? But as the duration varies depending on how well he is (how deeply he can breathe) and how much he focuses (the data showed that each neb was taking anything between 2 and 41 minutes, and he does six nebs a day) with noisy family life, trying to cook dinner, check homework and pick random bits of school uniform off the floor, we don’t always, 100%, definitely hear the beep ourselves, even if we’re in the room; we rely on Isaac being honest, only it seems he is not always. 

; Cue some pretty emotional and honest conversations about his treatments, his health, and his future health. He has promised to do better, and I think he understood how serious this was. In the meantime, we’re reverting to old school ‘you sit in front of us until it’s done’ method. Cruel to be kind. It breaks my heart at times, we do so much to keep him well, but we need his buy in. 

His liver ultrasound was also worrying (further results and bloods to follow) and demonstrating his physio in clinic today, he coughed up the biggest bit of sticky blood streaked sputum* I have ever seen..... CF truly sucks. 

Anyway, there are no beds available on the ward, and there are no signs of the winter bugs abating. Our beloved NHS is cracking at the seams. This doesn’t sound ideal, but does mean I can administer his IVs at home, instead of spending two weeks on the ward. When he was younger we used to do more home IVs (I trained to do them when he was a toddler) but over the last few years they seem keen to do at least the first week in hospital, arguing that intensive physio will help. I get this, but also know that wards are rife with.... like, sick people (who knew?!) which is not ideal for him to be exposed to. That is no criticism about cleanliness on the ward, the cleaners are amazing, but there is no doubt that people cough all over the place, and we are using shared bathrooms, doors and communal food areas. 

We’re confident that we can keep him active here, do the extra physio, avoid further bugs, and have a more normal family life, which helps us all and keeps him safe. He will continue with his personal trainer (three times a week) and can go to school, even with the needle in. Only missing the football. This will be my first time doing his IVs through his port rather than a long line, but his flush today went well, so I’m sure it will be fine. It just means being even more careful (sterile) than before, as the port is a permenant IV device and so close to his heart, infection in the line could prove fatal. 

Plenty more results from todays tests still to come over the coming weeks, but for now we have a plan, and we will fight for every single percentage of lost lung function that we can. There was good news too, he is growing well, his dietician was very happy with his diet, and they have a new Clinical Trials nurse starting, and a joint venture with Cambridge University, which we hope will result in more access to clinical trials, which are hard to come by for his rare mutation (for my CF friends, he has two Class 1 mutations). Thanks for reading. 

Everyone with CF deserves the chance of a longer, healthier life. Please support our skydiving fundraising by clicking here. x 

*Blood in his sputum is a sign of lung inflammation, and not the same as hemoptysis, where you cough up fresh blood. 

A muddy and knackered Isaac walking the dog. We are so lucky, these lakes are just minutes from where we live. 

This weeks tests.

Glucose tolerance test (for CF related diabetes). 
Bone density scan (for osteoporosis and other bone density diseases associated with CF). 
Chest X-ray (to look for lung damage and areas of infection). 
Ultrasound of abdomen (to check his pancreas, kidneys, bowels, and most importantly, for CF related liver disease). 
Lung function tests (main measure of health in CF). 
Sputum tests (what bugs he has growing in his lungs right now). 
Blood tests (for infection markers and vitamin levels, as he needs daily super high dose vitamin supplements (not the kind we buy over the counter) as his CF prevents his body gaining these from his diet alone). 

Followed by... 

Dietician analysis (reports on what his diet may be lacking). 
Equipment tests (his nebulisers and other equipment are tested at least yearly). 
Physio review. 
CF nurse review. 
Psychology review
Pharmacy review. 
And finally, the doctor review.

All in a day, thanks to a wonderful NHS. 

Please support this petition to have a voice against the privatisation of the health service we so greatly rely on in the U.K. x 

Got to scrape that shit right off your shoes.

Isaac has missed about half of the school days this year, through no fault of his own, and hospital school does very little to make up for this absence as he is usually too unwell in hospital to actively participate. But at parents evening this week they had nothing but praise for how well he is doing this term. Yes, he is a little behind in a few places, but nowhere near as much as his absence suggests. Along with this, he now up to doing CrossFit three times a week, and football twice a week. This, the same kid who was in PICU four months ago, wheelchair bound, on 24hr oxygen. He is my frickin hero! He is not feeling hugely great right now, and we’re due back in hospital for tests next week, but for now we’re feeling happy and super proud. 

"Sweet Virginia" The Rolling Stones; 
Wadin' through the waste stormy winter,
And there's not a friend to help you through.
Tryin' to stop the waves behind your eyeballs,
Drop your reds, drop your greens and blues.
Thank you for your wine, California,
Thank you for your sweet and bitter fruits.
Yes I got the desert in my toenail
And I hid the speed inside my shoe.
I want you to come on, come on down Sweet Virginia,
I want you come on, honey child, I beg of you.
I want you come on, come on down, you got it in you.
(honey child)
Got to scrape the shit right off you shoes.
But Come on, come on down Sweet Virginia,
Come on, honey child, I beg of you.
Come on, come on down, you got it in you.
(honey child)

A beginning.

I started this blog almost four years ago. My intention was to raise awareness of Cystic Fibrosis, because it is so often misunderstood, I even had a GP once ask me; so when did he ‘get’ CF?! Also, to raise awareness of organ donation; one in three seriously ill people die waiting for transplants, and this is entirely avoidable, and that just feels nonsensical to me. My final reason was to update friends and family on Isaac’s health, so everyone could come here to see how he was, without us having to repeat the same story many many times; that is not to say that we don’t like people asking how he is, we do, but for more detail, and more regular updates when he is unwell, this is a great tool for sharing our news. 

Anyway, I jumped right into blogging, and it’s been fun. I love to write, share, I’m a bit of an open book as my friends will know, and it has helped us to raise more funds for the CF Trust (we have raised over £55,000 since Isaac was diagnosed) and I hope we have urged people to sign up to organ donation, as well as tell their loved ones of their wishes (that conversation is vital, as signed up or not, it is their choice after you die), more about that here. But diving into blogging, I never really introduced us, and shared our CF story..... so here goes (some facts are a little hazy, smudged with age, worry, and perhaps a little gin, but I’ll do my best):

Isaac was born on a cold and snowy day, with perfect pink and healthy lungs. We had hoped for a home birth, but after three days of labour, I wanted, like, ALL THE DRUGS, and so we had a hospital birth, and although it was long, everything was fine. We couldn’t believe how perfect he was. Despite vowing that I would have him sleep in a cot next to us, and never in our bed, on that first night in hospital, it felt so wrong. I pulled him into bed with me, and promised that I would love him forever and would always keep him safe. Less than 24 hours later we were home, tired, but excited about our new life. Family visited, and he was happy and content, and for that first day, I think we kinda thought, hey, how hard can this be? All you need is love, right? And we had that by the bucket full.... But that night he cried, and cried, and cried. All night. A screaming cry, with hints of pain and exhaustion. We had no idea what was wrong, and although we felt like something must be amiss, we convinced ourselves that we were being neurotic young new parents. We had no idea what normal was. We were just pretend parents, nothing felt real. We called the midwives as soon as dawn came, and Mary Poppins arrived by 9am. Just before she did, Isaac had his first dirty nappy. We’d been told that the first would be black, like tar, but his meconium (first poop) came out like a golf ball and rolled out of his nappy. No wonder the screaming. Mary Poppins reassured us, and by now he seemed content again. 

For the next few days we had our only experience of a ‘*normal’ family life that we would ever know. 

But then he started to lose weight. A lot of weight. I was breastfeeding, and soon the midwives and health visitors were coming daily to weigh him. He was having a dozen or more dirty nappies a day, so I was clearly producing enough milk, and he fed for hours, typically for an hour, every other hour. We were exhausted, so much so I found it hard to string together a sentence (that is no exaggeration, I clearly remember not being able to remember simple words like ‘much’ or ‘meal’). I could never have got through it without Dan. We playfully called Isaac chicken legs, as the skin started to hang off his little limbs. We were worried, but there was talk of food intolerances, a tummy bug perhaps? We sent off stool samples for tests and assumed that everything would be OK. On day 5 he had the heel prick test as all babies do. On day 10 this was repeated. They told us that he probably had a false positive test, and that this sometimes happened, and that they just needed to repeat the test to be sure... I asked ‘Repeat the test for what?’ And they replied ‘Cystic Fibrosis, but you don’t have any family history do you? So it’s very unlikely’. 

And I just knew. It felt like our darling, sweet, innocent baby had a life sentence. The words, WHY HIM, AND NOT ME??? ran on repeat through my mind. 

And they were wrong anyway, the vast majority of families in our position are the same, CF seems to strike out of the blue much more often than it does run in families knowingly; CF is genetic, both parents need to be carriers of the mutation, and in the U.K.1 in 25 of the population are carriers, when two carriers have a child, that child will have a 1 in 4 chance of being affected, and so generations of fortunate carriers may never have a child with CF, and many others may never have realised; how many of us have a great grandparent or relative that died many years ago of ‘pneumonia’ (CF related?) as a baby? 

¡

Anyway, I felt like the floor had just disappeared beneath me. I dreamt of black holes, and felt like I was grieving for the life Isaac would never have.  We spent the next 11 days waiting for confirmation, where I fed, slept, and researched CF on a loop. The waiting was the worst, the more I read about CF, the more certain it seemed; his nappies were green, and often oily, as if he was not digesting the milk at all, and he continued to lose weight. But we also clung desperately to hope, and this just made it worse. On Easter Sunday I was home alone with Isaac when the health visitor called to say they wouldn’t be coming to weigh Isaac that day, as they were coming round on Tuesday with a CF specialist, I screamed down the phone; was that how the diagnosis was confirmed, over the fucking phone? (I probably didn’t swear really, I am way to polite in real life, and I’m adding it here for dramatic affect). She tried to back-track, as if a specialist nurse would be coming if the results were negative?! Worse, I knew now, but we still had to wait to see a doctor and start treatment, while he continued to lose more weight. My reaction must have caused a stir, as an hour later, the CF specialist, Joan, arrived on our doorstep. We asked a million questions. She answered them honestly, without giving us false hope, nor no hope at all. Compared to what I had read online, the prognosis seemed less bleak. By the Tuesday he was on treatment; daily vitamins (which his body was unable to absorb from his diet), antibiotics, to protect his lungs from infection, and most notably Creon; replacement enzymes which enable him to digest fats and proteins, which his body cannot, due to a blocked duct from his pancreas to his guts. He remains on these to this day (he takes around 30-50 capsules of these a day, depending on the fat and protein content of what he is eating) and most likely, always will. He gained a pound in the first week, and started to sleep for more than one hour at a time. He was no longer constantly hungry and in pain from malabsorption. The hospital were great, but gave us no written information (nowadays parents get a pack of information from the CF Trust) and so the CF Trusts parents forum online became my family, fraught with sadness at times, but also the only people who really understood. 

From then on, Isaac went from strength to strength. We went to CF clinic every single week for many months, to learn how to do chest percussion for his physio to keep his lungs clear, and for the many tests he would have, and will always need. All went well, until he was 8 weeks old, when he caught bronchiolitis, which was just bad luck. He became very ill very quickly, and was still at this stage very underweight. He were admitted immediately, and it was here that he had his first two week course of IV antibiotics, and given oxygen to help his breathing. My absolute favourite memory of Dan (husband) is texting him in the night, asking for him to bring something in for me when he was to come the next day, and adding at the end how little sleep I’d had. Half an hour later, seeing a tall, bandy legged guy walking through the door at 4am made my heart weep with gratitude. Sleep deprivation is one of the oldest forms of torture. 

Little Isaac: 

But he got over bronchiolitis, and he got over it again at 8 months old, although we were in hospital then for a lot longer. He had his first broncoscopy during that admission, where they look at his lungs with a camera, and flush them out with saline at the same time to collect mucous for the lab. Only he was quite poorly going in to this, and he gave them all a scare when they tried to wake him from anaesthesia. Being told that afterwards will forever haunt me. 

Isaac continued to have problems catching up with his weight for a few years, he tracked on the 0.8th centile on the charts, and we had to supplement his milk with sticky maxijul powder that we’d mix up into an icky paste and squirt into his mouth after feeds, pure glucose! Later he had prescribed milkshakes, and we would add cream, butter and sugar to everything he ate. Age two he was still small in height too, and I asked about his predicted height, as if he stayed on the centile he was on in the charts his adult height would be very small. His Dad is 6ft5, so it seemed likely that his lack of  height was a result of his CF and his being so underweight in those early years. We were told he probably would always be very small, but he is now above average and needs to avoid extra calories rather than add them. Take that CF! 

Over the next few years he was only admitted about once a year. He even had a couple of years without any IV antibiotics, his best stretch yet. Things started to feel more *normal, whatever that is. We relaxed a little, and just enjoyed him. He was a happy, active little soul, with dark eyes and blond curls. He had regular cough swabs taken, and these showed that his beautiful pink lungs were beginning to be infected with bugs, and so by the age of 18 months, he was taking daily nebulised antibiotics, which in those days was a big noisy machine, and Thomas the Tank Engine on full volume was our saviour. There are typical ‘CF bugs’ which love the sticky mucous of a CF lung, and Isaac mostly grew Klebsiela for his first few years, but by the age of 3 (I think) this was driven out by a big CF baddie, Pseudomonas Auriginosa, which still inhabit his lungs today. Bastards. 

Otherwise, these years were mostly taken up with extensive house renovations. We bought a small Victorian cottage with a large garden, and not a single wall, floor or ceiling was left unturned. We knocked low ceilings up into the attic space, extended (twice) and lovingly restored all the character that we could. Dan is a builder, the only way we could have ever afforded all of this. I filled the house with art, books and music, and the kids filled it with shite, sorry, I mean toys. 

Dan (and no, he did not catch that tuna, only in his dreams): 

The other major thing that preoccupied these years was choosing to have more children. We always thought we would have 3 or 4, but now we faced the chance that our next child would have CF too. For us, this was not an option. No one would ever choose to have this disease, and to knowingly pass it on, now we knew we were carriers? That didn’t feel like our choice to make; it was about that child that we would be bringing into the world, not us, and our desire for another baby. Besides, having a sibling with CF could negatively potentially affect Isaac’s health as people with CF are encouraged never to meet, as the bugs that love their lungs are easily transmissible between CF patients. Naturally siblings can’t avoid this risk, so would likely share bugs throughout their lives. This is a hugely emotive subject, and one debated on CF forums regularly, and I do not sit in judgement. Many families choose to risk those odds, or conceive and then test, but for us, IVF became a possibility and we chose that route. 

The hardest thing about the IVF was the waiting to have it; Although we were not having IVF for fertility issues, I can identify with that desperate longing for a child, and that feeling of it being out of your hands. For us, the year or so waiting was mostly because we needed to secure funding. As well as IVF, which meant stimulating my egg production, taking the eggs when ready, and injecting the sperm into the egg to make an embryo (ICSI) when the embryo was five days old and around 8 cells big (a blastocyst) one of those cells was removed and tested to identify the CF mutation, to ascertain whether that embryo was affected by CF. This is called pre-implantation genetic diagnosis (PGD), and a single cycle costs around £7000 (probably more now). We were told we had about a 1 in 5 chance of success. 

Things didn’t go quite to plan, I overreacted to the IVF drugs and developed ovarian hyper-stimulation syndrome (OHSS) which left me looking 6 months pregnant overnight, in hospital for 10 days, and needing to inject myself daily with blood thinning drugs for 13 months..... but it was worth every second. Anouk was THE most beautiful baby, with dark hair and the sweetest temperament, and Isaac adored her. After the blur that was Isaac’s first few months, Anouk’s was peaceful and happy (full post about our PGD story can be found here.). 

Baby Anouk: 

We had our challenges; at one point we had no kitchen wall, a shell of an extension built, a chicken wondering into the house, baby Anouk, and community nurses coming round whilst Isaac was on home IVs. At times chaotic, but always happy. Three years later, knowing we had three more healthy embryos in a freezer at Guys hospital in London, we thought we would have a final go at IVF, this time self funded, but without the expensive PGD part as the tests were already complete, we had our little frosty implanted, knowing we had just a one in four chance of success, and feeling like we’d pretty much already won that lottery. 

Our feisty, funny Rosa was born four years after both Anouk and her were conceived, our twins born at different times. We said ha! Our family is complete, but a few years later that yearning came again, but this time we added Obie to our family, the worlds most handsome and loving dog. 

We have amazing science and the NHS to thank for our family. What words could cover that? 

Baby Rosa: 

I’m not sure quite when, but probably around the age of 8 Isaac started to suffer with his digestive system more often. He was diagnosed with episodes of Distal Intestinal Obstructive Syndrome (DIOS) which saw him admitted to hospital many times. For maybe 2 or 3 years, he had pretty much continuous problems with nausea, vomiting and DIOS, and developed a deep hatred of the many medicines he needed to take to prevent this; Ironic when the medicine you need to help your digestive system is the same that also makes you sick. It wasn’t always awful, but came in waves, but he started to miss more school as a result, often not being able to make it in first thing as he’d been sick. He was referred to a gastroenterologist, and had many tests, including eating radioactive egg on toast and being popped through the CT scanner, and dozens of X-rays. Thankfully during this time, chest wise, he was stable. But seeing him eat and then throw up most days, I think we started to resign ourselves to this being his norm, after so long. Until one day, it just wasn’t. He does still have problems with nausea, and takes anti-sickness drugs for this most days, and CF digestion is never normal (Creon dosing is an art!) but he hasn’t been admitted with DIOS for over a year now, which is fantastic. I hope this is welcome news to other families affected by regular DIOS, things can get better! 

Frustratingly, as things were looking better tummy wise, he started to have more exacerbations (flare up of the bugs living in his lungs, causing more symptoms), and in the last few years, he has had many more admission for IV antibiotics to treat these chest infections, ultimately leading to him being very unwell last year,  spending time in intensive care, and him finally accepting that he needed a partacath fitted as his venous access was so pants. Anyway, you can read all about within this blog. 

So that’s us really, in a happy, shiny nutshell. I am probably forgetting about a million things inbetween. The biggest being that we have amazing friends and family who help, love and keep us sane. And also that CF can be very much in the background of our lives when it’s behaving. We laugh, we dance, we have fun. And even when it is not so great, and we are stuck in hospital, we are mostly there to keep him well, so it’s worth it. 

Everyone with CF deserves the chance of a longer, healthier life. Please support our skydiving fundraising by clicking here. x 

* “If you are always trying to be normal, you will never know how amazing you can be.”

― Maya Angelou. 

You want it darker.

If I am a parent, am I a carer? 

And if I am a carer, can I also just be a parent? 

 

And if I am a carer, is that because I order his prescriptions? I sterilise his medical equipment? I mix up his IV drugs, and administer them directly into his bloodstream? Or because I give him chest physio in the night when he can’t sleep for coughing? Because I stay with him when he is in hospital? Am I a carer because I nag him each day, until he has done all of his nebulisers and tablets and my throat is dry and aching? Or because we dedicate a quarter of our kitchen storage to his many medications, in which stock rotation is a full time job? Because I measure his temperature, his oxygen saturation levels and analyse the colour of his sputum? Am I a carer because I know the names of all his drugs, his medical team, the tests he has had, his list of diagnoses? Because I take him to every appointment? I capture his sputum and send it off to a lab to grow the bugs in a petri dish? Because when he has a PICC line, I need to wash his hair for him? .... 

.... Or am I a carer and not just a parent because one terrible month last year, he needed my help to simply get out of bed and into a wheelchair, his breathing was so impaired?

 

I watched this TED talk the other day. I do all the above, but I’ve never really considered myself a carer. I am just a Mum. Would I feel differently if he were so ill I couldn’t work? (I would be entitled to financial support for caring then). Or if it were my parent, my sister, my friend, my spouse and not my son who was unwell? Either which way; we should be celebrating every carer or caring person out there, supporting the less well in our society, because there are millions of us, and ultimately, caring for someone you love is a privilege fraught with sorrow. 

“82% of carers report feeling more stressed since they took on their caring role, with 61% facing depression.”

Isaac is doing OK: His problems with nausea and vomiting are declining; His chest sounds good; His pain better; The scar from his portacath op has healed beautifully - only his neck bothers him, where he can feel the line under the skin, tugging where is dives into the vein towards his heart; He will have his first flush in a couple of weeks, the line will need this every 4-6 weeks to keep it working (to see a video showing a port being accessed click here.); He is busy choosing the subjects he wants to take for his GCSEs, but as his attendance is so poor, his choices are limited (damn CF); But he is regaining the fitness he lost in October (through football and CrossFit) and for now, we are home and thankful. 

Everyone with CF deserves the chance of a longer, healthier life. Please support our skydiving fundraising by clicking here. x 

Working and Richter.

This week has been one of professional disappointment for me. A few years ago, my ambitions were teeny-tiny micro ambitions..... having happy children with vaguely brushed hair, haribo picked off the car windows, being dressed by ten in the morning, and a house filled with art, music and dancing round the kitchen table was plenty. But as we came out of the sleepless baby years, and my role at work has changed, I started to NEED to work. 

I guess my fear is that without work, my focus would be 24/7 on our battle against CF, and I realise now, that would do Isaac no favours; While his health will always be my top priority, and I will fundraise, and shout loudly for CF and organ donation awareness, for now, our balance in life is much healthier if I have that other string to my bow, to distract and keep me sane. 

That said, it’s not an easy balance. We have spent two of the last six months residents of Addenbrookes Hospital. I sleep there in the week, and try to work from the ward when I can in the day, and make it into the office for meetings I can’t really do by Skype. Still, most of my annual leave is taken up with hospital - and that can feel so unfair for all the kids, as it limits what other time we can have together. 

I’ve also taken two professional qualifications in the last year or so, and I’m studying for a third now. I admit, I wonder why I do this sometimes, especially weeks like this (being turned down for a challenge I would have loved, following an interview I took after a sleepless night on the ward). But I like my work, and I LOVE my colleagues and friends there. 

And, quite simply, I half agree with the adage that money does not buy happiness, but on the other hand, seeing Isaac, Anouk and Rosa playing in a pool in the South of France with our best friends kids a couple of years ago, I have to concede, it kind of does! Plus, who knows what kind of employment Isaac’s health will allow him? We need to build our lives up for his future. 

So, despite my disappointment this week, I will carrying on working for as long as Isaac’s health permits, and while I can, we will pack in as much fun as we can. 

I leave you with some brilliant Gerhard Richter. For a really spooky insight, check out this. Freaked me out slightly!

Everyone with CF deserves the chance of a longer, healthier life. Please support our skydiving fundraising by clicking here. Thank you x 

This isn't flying, it's falling with style...

On May 5th 2018 I will be jumping out of a plane along with my amazingly supportive and slightly crazy friends and family, Bonny, Jess and Shelley. Luckily I am good with heights, however I do have a mortal fear of rickety old flying machines, and, well, death generally, so this will be no small feat. 

We are doing this to raise funds for the Cystic Fibrosis Trust. Our family and friends have now raised over £55,000 for the CF Trust since Isaac was diagnosed.... such incredible support, we can’t thank everyone enough! 

In 2005 when Isaac was born, he could only be prescribed medications to treat the symptoms of his disease, and sadly, despite dreams of gene therapy, that is still true today. But we are on a brink of a whole new way of treating CF...tackling the underlying cause of the disease, which is all about abnormal movement of salt and water between cells. One drug (Kalydeco) is already available to those few with a CF mutation it will work for, and another (Orkambi, which will treat many more people with CF) is available in other countries, but not yet on NHS England, due to cost (thanks to an overwhelming response to the petition the CF community have been sharing on every social media platform going, we hope this will be debated in parliament very soon). 

Unfortunately, neither of these drugs will work for our boy (he has two class one mutations, which is really crappy) but it fills us with hope for the future; however a huge amount of research needs to happen in order to deliver this kind of treatment and ensure that everyone with CF has access to these groundbreaking small molecule drugs. 

We can personally vouch for just how shitty CF is: 

It means my son has to spend 1-2 hours a day doing treatments, even when he is well; 

It is the reason he has a bacterial chest infection all of the time, and why daily antibiotics only help keep the bugs at bay; 

It is why he has spent two of the last six months in hospital; 

Which means his attendance at school this term has been less than 50%; 

CF is why my daughters worry, lash out and have to cope with us prioritising their brothers health over everything; 

It is the reason he has had two painful operations recently;

....And why that he ended up in intensive care for six awful days; 

It is why he takes dozens of tablets each day, five nebulised treatments, along with rounds of physiotherapy to shift the mucous from his lungs; 

It is why we worry about diabetes, liver disease, deafness, osteoporosis and cancer, all of which can be linked to the disease or its treatments;

CF has resulted in his lack of venous access, after years of blood tests and IV treatments, and is why he now has a permenant IV device in his chest for easier access;

Cystic fucking Fibrosis explains how he was left wheel chair bound, on oxygen 24/7 and unable to even stand without becoming breathless for a time.... 

Everyone with CF deserves the chance of a longer, healthier life. Please support our skydiving fundraising by clicking here. Thank you x 

Petition, for young lives!

Please, please take one minute to sign & share this petition. It has received more than 10,000 signatures in 24 hours and we need it to reach 100,000.... 60K and counting! 

You could help save the lives of thousands of young people with cystic fibrosis. Thank you.

Making our own sunshine.

Isaac is home from hospital. All is healing well. All is as it should be. A happy video for you here!

Have a great day, and thank you for reading x 

Some of my favourite from Man Ray; 

Operation.

I used to love that game, growing up in the eighties.... we never had it, so it forever remained an elusive and fun thing that we only ever played at family and friends. Ditto my neighbours barbie oven which had lights and actually hummed like an oven. Oh how I lusted over that oven aged six! 

Isaac is now the proud owner of a shiny new portacath in his chest, which will make blood draws and IV antibiotic treatment so much easier. He has been in a fair amount of pain post-op, the incisions are larger than we expected, and he’s had all the usual fun stuff post anaesthetic (throwing up, massively sore throat from the intubation, lips so dry they crack and peel). But it is in, and it is working! He remains in hospital, and his cough is still worrying me, especially as we are coming to the end of two weeks of IVs, the point at which we’d expect his chest to be the best it can be. Hopefully this is just post-anaethetic-cilia-need-to-wake-up-effects. This is an actual thing, I didn’t just make that up. We have lined up samples for the lab, so have the usual wait now for all the test results. But for now, we just want him home. His sisters need us all home. 

Welldone and thank you to all those that will or have been attending NICE meetings around the U.K, campaigning for the right for people with CF to access Orkambi, a life changing drug; 96-week data shows that Orkambi can slow decline in lung function – the main cause of death among people with cystic fibrosis - by 42%. There are 2,834 people in England, 243 people in Scotland, 118 people in Wales and 101 people in Northern Ireland who could benefit from Orkambi. Sadly Isaac is not one of those (due to his rare and quite shitty mutation) but I can completely relate to just how maddeningly painful and frustrating it must be for other parents and adults with CF to know that this drug is there, it’s bottled up, ready to go, and your doctor wants to prescribe it for you..... but can’t. What price can you put on a life? 

Thank you for reading x

Floods, Op’s and home IVs

We are home.... still on IVs, but home for much needed family time for the weekend. I am back into the swing of administering the IV drugs; it’s all aprons, gloves, endless alcohol wipes and checking the measurements and dilutants obsessively, but my confidence grows quickly each time. His chest is  sounding much better (the sound and feel of his lungs is a great measure of wellness, from a crackle to a wheeze, to a vibration or a gurgle). 

Tomorrow we return to hospital for his portacath Op, subsequent recovery, and to hopefully finish off the course of IV antibiotics (they will also use the opportunity under anaesthetic to suction up more sputum from his lungs to test for New Twattish Microbastard), Thursday marks the two week point, the usual length of the course, and unlike last time, I’m confident his lung function will have increased enough to finish on time. 

This admission has been nothing like last time. Still a little way to go, but we are feeling fortunate and pretty damn chipper; We need this operation to go just as well, I’m sure it will. Have a great day x 

My flooded fen dog walk at dusk: 

Sleeping with other people.

As chirpy as we try and be about hospital, there is one thing that rarely gets any easier, and that is sleeping with other people. Because, let’s face it, a thin blue curtain does nothing to change that fact that you are sleeping mere feet from others, and while I enjoy my own pretty hippy and carefree attitude co-sleeping, life on a hospital ward is something else, and we’ve had some mixed experiences... 

The whole light etiquette: come evening, we try always retire early; knowing our night will be interrupted in a hundred different ways, we have to go for quantity rather than quality. But when is it polite to turn off the main lights when you’re sharing? I’m all for bed at 8pm on the ward me (so frickin rock’n’roll!). We once slept next to a family who every night angled their bed lamp so it was actually touching our curtain, facing our direction, giving them a lovely dimmed light, and us, the full beam. I silently wept and tried to knock it away, as if by accident, with an outstretched yawn. 

Space: The children’s ward we frequent is split into a few individual rooms (which we only get if he is contagious, or if there are a number of CF kids on the ward all of whom need to be kept apart), and other rooms with three, four or six beds. The more beds, the less comfortable you will be. Fact. On the six bed ward, I once had a parent push their chair so far up against the curtain dividing us, his chair was touching my back as I laid on my side. We were next to each other for two whole weeks. 

Crying: The worst was the sweet baby that came in for cleft palate surgery, who cried all night; a shrill, gargling, piercing, and hungry cry. I honestly think Isaac had about an hours sleep. I assumed the babe was nil by mouth pre-op, and having been there myself, it wasn’t hard to empathise with the poor mother. But when the dad came in the morning after, I heard him ask the mum, ‘have you tried feeding her?’ And the mum replied ‘no, I thought she was just tired’. I’m telling you.... ALL BLOODY NIGHT, and she stopped as soon as she was fed - I think it’s sometimes possible people see the illness first, and the baby second. If that makes sense. 

Night arrivals: There are the families that come up in the middle of the night from A&E, who have probably have had some kind of BIG LIFE EVENT of sorts, and it is a known fact that people talk around 22% louder during such episodes than they normally do. Kids often come in with parents, sometimes with siblings and grandparents, maybe aunts and uncles too. And all of these worried and loud people are right next to us as we try and sleep. You understand, but sometimes it doesn’t make it much easier when you’re tired. 

Privacy: This week, we had the 14 year old who was on her phone all the time and tried to have her 18 year old boyfriend stay over (the nurses kicked him out). I felt like I knew her by the time she left without hardly talking to her, we’d accidentally overheard stories of snapchat screenshots going viral round school, her social worker, going back into care, her mother calling social services on her father, her sister being in the pub all day, her warning friends to back up her story to the police (really).... how can you not listen when you’re close enough to hear each other roll over in bed? As she kept rolling back in on her wheelchair smelling of fags, I desperately wanted her to go, but I also wanted to give her a big hug. Equally everyone knows exactly what is going on with us, be it an intentional or accidental invasion of privacy, I get it, but sometimes that is hard to deal with (even for me, who writes a blog about this life. Kind of ironic I suppose). 

Environment: Then there is the inevitable snoring; the heat (wards are always so hot); the hospital hum (the noise which I imagine comes from his generators, boilers, air conditioning units? I hope it’s not the incinerator, I don’t like to think about those); the wondrous smells of all varieties; and of course, the forever, incessant beeping of machines. 

Anyway, I could go on and on, but feel bad for moaning. There are positives... occasionally at least. You can meet amazingly interesting families, and make friends even. We are definitely in the regulars club now; We know all the nurses, and the nurses know what Isaac needs: space, more than most, only because we have to bring in so much medical equipment with us (two nebulisers, bi-pap machine, all of our usual drugs) so where possible, they do try and get us a good bed. And while the majority of people we meet here we only ever see that one time, there are others regulars like us. Sadly I can’t meet up with a good friend of mine, whose daughter also has CF. We are often in at the same time, and text each other from our respective beds, but cross infection is even possible from *patient to person to person to patient* (if that makes sense!). So we support each other from afar and wave if we see each other (good luck for today A&G! Much love to you both). 

Today has been a little difficult for Isaac. His port op has been delayed again, and should happen next week now, unless his longline fails sooner (in which case he should get on an emergency list). He had quite a sleepless night worrying about the op, I think mostly because he had two very recent general anaesthetics, and for both, his experience in recovery was rough (initially that is), so he is understandably frustrated for it to be delayed a second time. Then he had his hopes up to go home for a few days, on home IVs, as his lung function today had increased nicely. However the doctors are not quite ready for him to come home, and will review him on a daily basis until they are. That said, things are otherwise going well. 

I, on the otherhand, have come home for a good nights sleep and a cuddle with the girly ones while Dan stays with Isaac. It’s always bittersweet for me though, I feel like I am missing a limb when I’m not there with him (despite the fact that he hit me round the back of the head with a football earlier in the gym. Little shit). Love him x 

The long escape route from hospital....

Chronic illness.

Chronic illness is very hard to explain sometimes. Isaac’s baseline ‘well’ is almost definitely not the same as your ‘well’, but life is no competition on suffering. I honestly don’t mind when people have a grumble about a particularly bad cold they have been suffering with for a while, but people often apologise for doing so. This last couple of weeks I have had a broken or bruised coccyx (I say broken or bruised because I decided not to have the X-ray to check, as either way, the treatment is the same; try not to sit on it, anti-inflammatories and painkillers). And jeez, did I moan! Only the diazepam/tramadol/naproxen cocktail shut me up (mostly because I was an incoherent wreck crawling around on the floor...I’ll stick to the mojitos in future).

When someone expresses concern about a blood test while Isaac has had more needles than hot dinners in his lifetime, I don’t compare their situation to ours. When people have effortless pregnancy after pregnancy, I don’t bemoan the fact that we had to have IVF. Coping with chronic illness in the family does not diminish your empathy for others. For someone with near perfect health, a new health experience can be a very scary thing, whereas for Isaac, we are used to tests and subsequent results on a rolling basis (and some will always bring bad news) and I guess in some ways this desensitises us to some aspects of hospital life. 

This week we met a particularly useless surgeon who proceeded to inform us that Isaac would have his port fitted in the side of his chest, rather than at the front, because it would leave less of a scar and be less visible. Isaac protested that he wanted it at the front because he doesn’t give two hoots about a) the scar or b) the visibility, as it would still be below his T-shirt line, and that for him, accessibility was his priority. The surgeon just really couldn’t get it (he also called him ‘James’ throughout the appointment, which never fills you with confidence). When we explained that depending on what IV drugs Isaac takes, he might be hooked up to drugs for some hours each day, so to pull down his T-shirt, and have the line pop out his neck, leaving his arms free to go wherever, is a lot better than having it on his side where he would need the line to come up, down or out his sleeve. Plus, one day we hope he’ll be independent and able to do his own IVs, this is only possible (I think, or at least it’s a lot easier) if the port is on your front. The surgeon still looked at us, mouth agape, before finally conceding that Isaac was right, and then proceeding to talk us through the surgery; 

Dr Useless; And we make one incision here (pointing to childish drawing he is doing for us). 

Me; Really, Just one incision? 

Dr Useless; Yes, that’s right. 

Me; But don’t you also need an incision in his neck? 

Dr Useless; Oh yes, that’s right. Two incisions. 

Me; But you’re not the one doing the surgery on Isaac, right? 

The flip side of chronic illness is that yet another admission to hospital, when it is expected, even planned, is a very different context to a first or emergency admission. This is our reality, and for the most part, that’s OK. Last night I stayed in with Isaac, and we gossiped and giggled about the people on the ward (I have a lifetime of shocking stories about the families we see on hospital wards, and please know, this is not us snooping, we are in a four bed ward, and last night, this meant a room full of maybe 15 patients, visitors and nurses - only divided by curtains, you can’t help overhear, and all I can say is that last night was like an episode of Jeremy Kyle!). We stayed up too late, we ate chocolate after cleaning our teeth, we overslept. Went to the gym for physio as soon as we woke up and raced each other on the bikes. Then I swapped over with Dan to spend some time with the girls. We shopped, went to a funfair, and I laughed my head off on the dodgems. Then we snuggled up with the dog in front of the fire and watched Dumbo. 

I think sometimes, because we do carry on, we laugh a lot, we throw a lot of parties, and we make the most of every day, and yet we still prioritise Isaac’s health, we can be labelled as (eek!) inspirational. And this is NOT a word I have come up with, people say it to me all the time. One CF blog I read said this: 

“Frankly, many people treat the chronically ill as inspiration porn. So when we express any type of grief, they act as though we’ve rudely interrupted their televised programming for something unscheduled and unwanted”

But before I very publicly fall off any hypothetical pedestal you might put us on, can I tell you that we do moan, we do cry, we do have tantrums, throw weetabix on the wall and slump against the fridge crying. I can certainly tell you that I myself feel anything BUT inspirational when I’m tired and usher the girls up to bed with their iPads because I JUST WANT TO HAVE A GIN IN PEACE. And Isaac has his little paddies too. 

Sorry, this post has no real outline, no conclusion. Things are going well in hospital. Isaac is coughing more, but that is usual for early on IVs. The physios are pushing him hard, which is great. And best of all, we think his port surgery will be bought forward to Tuesday now, so we just need his line to last until then. Depending on his recovery, we hope to get home on home IVs soon after if we can. I guess what I am trying to say, is that if it was your child, you’d be doing the same too (only probably better, or at least with less gin), and that mostly, life is not so bad. It was a completely different ball game last time, when he was so sick, but right now, we’re doing OK. 

Thank you for all the kind wishes x 

Georgia O’Keefe love tonight.