Cystic Fibrosis FAQs

What is a portacath? Isaac had a portacath surgically implanted in his chest wall earlier this year, the aim being easy venous access for regular IV treatment (IV being intravenous, as in, drugs delivered directly into his blood stream). 

Regular cannula/long line/PICC line access over the years has wrecked his veins, and it became harder and harder to find a viable vein each time (veins repeatedly used would just collapse resulting in multiple needles, and often, delayed treatment when he needed it most). We had an agonising wait in HDU (high dependency unit) once, and delayed antibiotics give bugs a chance to fight back. Not good. 

The port meant surgery, always advisable to avoid, but the hope is that this will last him 5-10 years before replacement. Cancer patients often have a port for for chemotherapy. 

He still requires a needle to access it for use, but the key difference is trying to guide a line up a long, thin, windy and collapsible vein, usually blind (they can use ultrasound, but this is unusual) which often fails and needs repeated attempts, compared to a needle plopped right into a kind of rubber bung with a hole in the centre, and ta dah... you have accessed the (pre-accessed) vein. 

What does having a port mean to Isaac? On the whole, it’s great. When his port is not accessed you can see only a smallish bump under his skin in his chest (with one scar nearby and a second by his neck, from the surgery). When it is accessed for treatment, he has a needle in for the whole course (usually 2-3 weeks at a time) and cannot get this wet, so no swimming or showers. The rest of the time, it’s life as normal, aside from avoiding full contact sports like rugby, which might damage the port. 

In between treatments, the port is accessed every month for a flush to keep it clear. So it does still involve regular needles, but compared to cannulas, long lines and PICC lines, overall, it is a helluva lot less stabs! 

What would a Transplant mean to Isaac? CF is a multi factor disease, it affects mainly his lungs, pancreas, liver, sinuses, and digestive system. A lung transplant would only cure the disease in his lungs. But this is also where the biggest risk to life is (90% of people with CF die from lung disease). 

After transplant, people with CF no longer have CF lungs. However, infections in their sinuses may re-infect their lungs, meaning they need to carry on with some traditional CF lung treatments (but not all, and maybe very few). 

Digestive health is unaffected by lung transplant, for example, Isaac will always need to take Creon in order to digest fats and proteins (pancreatic enzyme replacement meds, of which he takes about 50 capsules a day) and will continue to be at risk of CF related osteoporosis, diabetes, some cancers and liver disease. 

Why not transplant now? You will hear me shout loudly on this blog about organ donation and the fact that we have chronic shortage of donors. 

1 in 3 people on the waiting list for lungs dies waiting

I have often been asked why Isaac cannot be put forward for transplant now? The good news is; he is too well. The main test of wellness is a lung function test. His FEV (forced expiratory volume) is measured regularly and forms part of the picture on which his team plan his treatment. I don’t like to post much about his FEV, as I feel it becomes a focus on a number which is only part of a much bigger picture. Plus it fluctuates widely, at his worst (while in intensive care) he blew a 17%. Needless to say, his baseline is never as high as I would like it to be, but also, it’s not near the point where transplantation would be considered (regularly less than 30%). 

More importantly, transplantation is not a cure; the chances of surviving the surgery for a year is only 80% and surviving 5 years 60%. This is a fast changing statistic, as development in anti-rejection therapies improve. Transplant is truly amazing, and life changing for many, but it’s also like swapping one disease for another, and further, a whole new set of not so fun side effects. 

More FAQ’s to follow. Any questions, because medical jargon slips into my vocabulary pretty quickly, please do ask x 

A squash and a squeeze

For anyone who has read this book, the way the old lady feels when she gets her house back? Sums up how we feel post home IVs...

His daily treatments (tablets, nebulisers, physio) which usually feel half defeating to get done, suddenly feel SO simple after two weeks of very early starts, gloves, aprons, mixing up antibiotics and syringe driver pumps....  So here is to feeling thankful, de-accessed, hardly coughing, and our boy getting his summer fun back on track. 

I’m going to post some FAQ’s soon, about CF. All questions welcome. Have a great day x 

NHS England deny Orkambi access

Today NHS England stated, again, that Orkambi is too expensive to fund for sufferers of Cystic Fibrosis, despite a promising pipeline deal proposed which would mean earlier access for people with CF to drugs still under development. See here for more details, and here for how a young girl with CF eloquently puts this frustration across. 

Last year, half of all people who died with CF were under the age of 31. Sorry to be so blunt. Really. If I could avoid the statistics, I would. But in the last year Isaac has had 5 courses of IV antibiotics for 2-4 weeks at a time, 10 weeks in hospital, 4 operations, countless tests and clinic visits, and continues to take nebulised drugs every single day which cost the NHS thousands of pounds every year. I am not saying this is a moany way, we are so so grateful to the NHS for the treatment that he receives, and the life it allows him to live. We really do consider ourselves lucky. 

Orkambi will not work for Isaac’s mutation, but I hope one day there will be a precision drug that will, and while the cost for these initially seems astronomical (Orkambi costs around £100K per annum, per patient), I do believe there is a saving for the NHS in terms of keeping people with CF well and out of hospital, which these drugs really have the capacity to do, and the pipeline deal would guarantee access to future drugs hopefully even more effective than Orkambi. 

I’ve long been gutted that Isaac’s rare mutation means we need to wait longer for these kind of treatments (they are also an oral drug, no IVs, no nebs, Isaac’s dream come true!) in a sad way I’m glad that Orkambi won’t work for him, because knowing that a treatment was there, approved, and waiting on a shelf for him, and not being able to reach it would drive me beyond crazy. I honestly don’t know how I would cope. 

If you are in England, and could find the time to help, please download the template found here and send it to your MP, which you can find here. It would mean so much to me if you could. 

Things here are OK. We had a wobble last night as he had a mild fever and vomited. We have to be very cautious about infection risk in his port on IVs. Thankfully this morning he was back to his usual self. Despite home IVs, we’re just really thankful to not be in hospital for this course, which we hope will end on Tuesday (with a cool swim for him) all going well with lung function tests. 

Have a great day x 

Heat wave and wild woods

Isaac is doing really well, frustrated in the heat, port accessed and unable to swim, but coughing much less. Bloods have been OK (the blood fairies come every three days to check his Tobi levels, as his kidneys can suffer from the toxicity). Angry earlier, he asked why he can’t have a transplant already. He is sick and tired of the treatments; as soon as his hour hooked up to the IVs is done... he still needs to do his nebulisers and physio. There is no holiday from CF treatments. In this heat he walks round, top off, port accessed and IV line dangling on his chest, a very visible sign of disease, but I’m proud he doesn’t care. 

This weekend a little baby in our family gained his angel wings, far too soon. Little lion Leo. We are thinking of you all, Sian, heartbroken. 

Some pictures from Wild Wood last night, basically a rave in the woods. A very welcome blow out, with some beautiful friends, without whom I would cope a lot less well. Thank you lovelies x 

Accessed and ready to go

IVs kicked off. Three doses down, 39 more to go (assuming his lung function increases and we don’t need to do more than two weeks). 

Tonight he is frustrated with his dressing. (CF friends - can anyone recommend a tegaderm that actually sticks?! Please message me if so). I think the issue is exacerbated by the heat, it’s 30 degrees and no swimming or showers to cool down allowed. He is coughing more already. 

Yellow and IVs

We had clinic today - his lung function has dipped again, and he will start IVs next week, but all going well (if he gets no worse before then) we hope to do these at home and avoid an admission (he will have at least a day in at the beginning and at the end, as he needs more tests). He is feeling OK, aside from the coughing. He is sick. But not sick sick as we say. 

After IVs in August, October, January and March, Isaac has had the longest gap between courses in a year. I think it’s been ten whole weeks - which we are bloody thankful for. I’ve said before, the hope is to have planned IVs four times a year, but the last year has been difficult - hopefully this longer stretch is a sign of things to come. We had hoped to eek out this good run, so IVs were closer to our summer holiday, meaning we could be more confident of him being well while we are away, but as always, if he needs it, bring it on! 

Most of his annual review test results are in now. Concerns over CFRD (CF related diabetes), which he will have a repeat test for once he is well enough, and liver disease (again, CF related). But the bloods were mostly reassuring, infection markers down from over a thousand when we were last in, to under 200. No sign of the NTM since the end of last year, but chronic Pseaudomonas infection repeatedly show heavy growth. 

Tomorrow I had hoped to join the protest for access to the new CF drug Orkambi, for those people it works for, this can be a breakthrough treatment. Vertex (the pharma) and NHS England need to make negotiations their priority, and end this! It’s been almost a year since I last attended a protest for this drug in London, and although talks have moved forward, this drug is still not freely available to those who so badly need it. Read more here. However, this also clashes with wear yellow day to mark the end of national CF week, so instead I’m running a cake sale at school, following a non-school uniform day, raising money for the CF Trust. Although we’re always doing some kind of fundraising, we’ve never done any through school, as Isaac doesn’t like the attention. But now he’s at Secondary school, he was happy for us to go ahead at the girls Primary school - Anouk and Rosa are so excited. 

I also attended an information evening for CF, run by the Trust this week. Here are some of the highlights: 

• Cambridge is home to the first CF innovation hub. This will soon be on site at Addenbrookes, when the building is complete, and so we hope, gives us an increased chance of access to cutting edge clinical trials. 
• The hub is focusing on infection and inflammation, the two biggest problems for lung health in CF. As well as drug discovery, they are looking at using smart tech in CF care, and regenerative therapies (its long been thought that once the lungs are damaged, there is little you can do to bring back that lung function, but stem cell regeneration may change that) and precision drugs. 
• Our own Consultant presented a new iPhone app, that we have been involved in trials for, which gives kids a really cool game which responds to their breathing during physio. It can show not only how many breaths they have done, but how deeply, how long, and gives them an incentive to complete the physio (physio can take Isaac up to an hour every morning and every night, mostly due to chronic procrastination). The app even allows kids to play online against other kids with CF (who they could never meet, due to cross infection) which adds a social interaction they may benefit from. The app looks great, and would be especially good for younger kids in creating good habits, and in turn, help prevent much head banging for parents. 
• The NHS is in a staffing crisis; in some areas up to one third of positions are not filled. With the fast growing CF population (CF used to be a disease of childhood only, but now, just over half of those with CF are now adults) this is a massive concern. 
• Dr Charlie Howarth presented the new Papworth hospital building, now on site at Addenbrookes in Cambridge. The old Papworth hospital has a Cf population which has grown from 130 to 330 in 15 years. The new building offers many ensuite clinic rooms, theatres, nuclear medicine, 42 critical care beds, 25 room dedicated CF unit, all with ensuite, fridge and exercise machine, lifts which are segregated by what bugs you are infected with, high tech UV cleaning (kills all the bacteria in a room in 15 minutes), and 15 air changes per hour - all of which are designed to limit cross infection risks. Amazing! 

There was more too, but I have cakes to bake and kids to cuddle. 

Have a great day x

Late walks by the lake, in the lake and summer fun. 

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