This ain’t no lifestyle blog, dude!

Here are some pictures of our kitchen.....

Our front room....

And now the avocado on sourdough toast I had for supper...... 

KIDDING! As much as I do love our house (over the 12 years we have been here we have removed the faint smell of cat piss, re-plastered every wall, moved doors, extended twice, changed every flooring, and renovated the garage to make the kids a games room). We have built a home. It’s covered in art, piled high in books, scattered with discarded shoes (rarely paired) and littered with toys (and the latest ‘in’ toy? Slime). It may be a little grubby around the edges, but it is most definitely a home, a party place, a social hub, music always a tiny bit too loud. Put it this way, you walk into our house, and you get a pretty good idea of who we are and what we love. No beige, no neat piles of magazines, and no loo roll with folded sheets..... 

Anyway, I digress! My point was that this ain’t no lifestyle blog (if it were, I would have to tidy more, and with our lot, that’s not possible). This is the story of our lives with our sons Cystic Fibrosis. It can be merry, but more times than not; it is raw, it involves lots of sputum, it hurts, and as much as I try to put a positive spin on things, I don’t always feel that way. Maybe I’m trying to write ourselves out of the waves of negative, the pain, the nausea....

A year ago this week, Isaac got very sick. Weeks later he was still oxygen dependant, in a wheel chair, and scared that the effort to breathe might get too much. But we got him out of intensive care. We got him home. And he’s spent the last year fighting to get fitter than ever; 

...preparing for the next battle that we know with absolute certainty will come. 

He is so fit right now, and it takes a pretty rocking personality to take the knocks he takes, and get back up running like he does. Our little dude (I say little, but let’s face it, he’s near my height now). 

This other CF dude writes about life with CF in a super eloquent, moving, and very real way. He is post lung-transplant and doing well. So worth a read. His points about CF being an invisible disease are spot on; for every funny look we have been given as he coughs excessively, or when we leave pharmacy with not a bag, but a box load of drugs, jump the queue in X-ray, or when we allow him to add a tonne of salt to his food - please don’t stare people. Disease comes in all shapes and sizes. 

More on us soon x 

Happiness, flu jabs and another loss.

Clinic was pretty frickin positive...! LF was down slightly, but still bettter than it has been most of this year. For now we try extra oral antibiotics (a new one), a new steroid inhaler, and picking up his nasal rinses again (problems with his polyps/sinuses may be contributing to this). So no steroids, and no immediate IVs, for which we are very, very happy. 

It’s flu jab time of year again, and we will be first in the queue. Please remember, if you can get vaccinated, you’re defending not only yourself, but also reducing the spread of the virus and protecting those more vulnerable around you. (note: you cannot ‘catch flu’ from a vaccine, it’s not live, and these days, you can often choose between a nasal spray and the jab). 

Do this. 

Give blood. 

Sign up for organ donation and make your family aware of your wishes. 

At least that’s what I think, but who I am to say?

"Life isn't about being happy, life is a roller coaster of crazy emotions. One second you're fine, and the next second you feel lonely and despair and like nothing is ever going to be okay again. It's not about emotions, it's not about how you feel second to second. It's about what you're making with your life, and whether you can find a deep pride in who you are and what you've given. Because that is so much more impactful, so much deeper than whether you're happy or content or joyful. It's okay to feel pain." - 

- Claire Wineland, an insanely uplifting and beautiful person, who died this month, following CF post transplant complications. This disease is so fucking cruel. Everyone should watch this. x 

Roid fears.

The cough continues, with a scattering of tummy issues thrown in for good measure. CF clinic tomorrow. Hoping for answers and treatment, but please god.... not steroids. I’ve posted previously here about his ‘roid rage. We’re also only six weeks post IVs, so hoping for a longer break from those too.... for his sanity, ours, and his poor battered kidneys. Of course, more than anything, we just want him well. More soon x 

Rhyme or reason

I wrote a long post, and then lost it! DRRRRR. So here is a shorter and much less eloquent update;

Isaac is coughing. 

A lot. 

Like, people stare at you in Tesco, coughing over the veggie aisle, and sleeping is miserable type coughing.

But when we took him in last week for an emergency review, he blew his best lung function in a year!

This, after it being at ‘stable-but-quite-shitty’ level for some months, even when he appeared well.

CF continues to surprise us. 

Occasionally, how symptomatic he appears has no rhyme or reason with his lung function, supposedly the measure of wellness in CF.

Treatment is Azithro and brufen for inflammation, as the doctors feel after a year of intensive treatment of his infections, the coughing now may be due to an overly excited inflammatory response (calm lungs, calm!).  

In CF, infection and inflammation form a vicious circle in the lungs, and if this treatment doesn’t work, he may need steroids (always the last option with him, due to the side effects).

As is his norm, Isaac is taking it on the chin, smiling on, and not letting anything hold him back.

…. Not many people get to live with their hero, but I do. He never ceases to amaze me with his courage, humour and tolerance. 

Some pictures from my travels to Toulouse (holiday) and Madrid (work) recently….

More soon x

Holiday and home.

We made it. He made it! On day three of the holiday, hearing him rattle and cough, I was sure that I would be booking flights home for him and I, to head straight back to hospital. But the next day, things improved, and the next, and the next. Was the Cipro working, when the IVs did not? (they are still treating the same bug, only less powerfully, so no reason why they should). Did he just need that extra time after the IVs? Was the hours of swimming and running around with his friends, the sunshine (the joy?) helping him clear his chest, while the hospital physios could not? Are we even treating the right bug? We know he is colonised with PseudoA, but he may still have something new, that we’re missing. Despite the IVs, a three week break, IVs again, followed by two weeks on Cipro, I’m no more confident now that his lung function will have improved than I was at the beginning of summer. It’s not like BLACK OCTOBER, by any means, but he is not right, and for whatever reason, he doesn’t seem to be shaking this one just yet. A positive test (for a new bug, CFRD or another problem) would suck, but so does not getting any better, without really knowing why. Stable but not great is still no place I want him to be. 

All that said, France was full of love, and fun, and not a sniff of WiFi! Also, lots of art for me, I will share more in later posts. 

Hope you’ve all had a wonderful summer x 

These three 😍; 

Vacances et espoir!

We will go on our holiday to the South of France with our exceptionally lovely friends. His IVs will finish tomorrow, and after his late dose I will de-access him myself (remove the needle from his port). He will be able to swim, have fun with his friends, eat fresh baguettes and pan-au-chocs from the boulangerie, sleep in the villas dorm with all the boys, feast on BBQ every single day... and be a kid. 

I wish I could tell you that all the news was good today, but his lung function was down again, and he’s still not right. 

Normally at this point his IVs would continue for another week, and they would continue to review him every few days. Instead we get to go away on the proviso he swaps immediately to the strongest oral antibiotics he can take. Good news but they do make him super sensitive to the sun (he burns in winter on this shit) so I’ve been panic buying SPF50 rash vests. He will continue with his six daily nebulisers, increased physio, and if he gets any worse; we will come home early. If he remains the same (coughing lots and very productive, which is CF talk for bringing up lots of icky sputum) we call the minute we get back and head straight back in to hospital. 

Our MDT (multi-disciplinary-team) values his quality of life as much as we do, and it’s forever a balancing act between this and treatment. They praised him today on how much he has been putting into staying as well as he can. He continues to complain very little, so how can we?

A cough-free, worry-free two weeks in the sun might not be possible, but we will definitely have as much fun as humanly possible. 

Avec beaucoup d'amour x 

PS. I’ve been asked to add for all those budding burglars, we have dog and house sitters in situ, but thanks for thinking of us 😘

Clinic review tomorrow

This will determine if Isaac is well enough for our holiday. I swing from utter confidence (as his chest is clearer, and he looks well) to total panic (as his huff still rattles). He is better, but maybe not well enough. A normal course of IV antibiotics is a minimum two weeks, but he’s often had three, even four weeks to get well. This will have been just eleven days. Stopping too early may give the bugs the chance to fight back before we fully squash them, but on the otherhand, if his lung function is not improved tomorrow, then we might be looking at a new bug and these might not be the right antibiotics anyway (tests still pending). 

All that said, we are still full of hope. Even if we have to miss the beginning of the holiday, all will not be lost. We can catch them up by train or plane. Without wanting to sound all Starship 1986.... nothings gonna stop us now.....he will get the holiday he so much deserves. We will make sure of that. 

Wishing you all lovely summer fabulousness x 

Little Anouk with her cousins x 

Some news

Good news and bad, or at least the not so good, and for now, I’ll take that 😊

We are home! For the weekend at least. Not much happens on the ward over the weekend. Physios come less. Doctors make few decisions without a consultant, so it always feels like limbo time. Therefore we’re allowed home on IVs again. Either until next Thursday, when he will be reviewed in clinic, or Monday if he becomes any less well. 

His blood infection markers are at 80. Compare this to Black October, when they were over 1000. This is GOOOOOD! 

Although we got home at 3.30 today, he still needs to be back on the ward at 8pm for a blood test (they have to regularly review the antibiotic levels in his blood, as too high risks kidney, liver and hearing damage). Still, should be a flying visit. 

Isaac is very tired following a sleep free night on the ward and needs to catch up. 

His lung function today was no better or worse than when he started IVs on Monday. But worryingly he continues to cough, rattle and roll; a lot, and struggle with exercise. This is not necessarily unusual though, just five days into IVs, and hopefully we will see a big improvement over the next week. And despite him seeming more symptomatic (coughing up lots of gunk) his LF was nowhere near as low as I had feared to begin with. 

They have confirmed that once well enough they will do a three-day continuous blood glucose monitoring for him, to properly access whether his is diabetic too now. He is drinking about 8 litres a day right now, which is not a risk, but could be a sign. 

We’re still waiting for other test results. Frustrating as we can’t be sure until then that he is on the correct antibiotics. But this is not because of any kind of tardiness, it’s simply that his sputum cultures need time to grow in the lab for them to identify what exact bacteria he is growing. 

We’re so so glad to be home, but going away on holiday won’t be a sure thing until we know how he is/what he is growing in his lungs. We won’t know for certain until we’re just about to leave. 

Anouk says things like “why him”, “why us” as she struggles to understand (she is, by the way, the kindest, most empathetic kid you will ever meet). Tomorrow I should have been dancing along to Patti Smith at a festival, but won’t be as I need to be here. But why not us? And above all, we are a team. I will never let him do this all alone. 

Finding the car

Every single bloody time I park during an admission, I swear to myself that I’ll remember where in the seven storey car park I’ve parked, but after days on the ward, never do! I am eventually home for one night with Rosa (Anouk is away with my sister, and I can’t tell you how much I miss her, but I’m glad that she is having fun. She is a worrier and hates our family being apart, so it is wonderful that she has a distraction this time).

Isaac is slowly improving we think. 

I sat in the garden by the gym earlier while he was doing physio and reminded myself that this is not like ‘that’ admission back in October. Let’s call it BLACK OCTOBER. He is not struggling to walk a few steps in that garden on oxygen, he is not in a wheelchair.... he CAN breathe. It was a timely reminder that things could be a lot worse. 

But he continues to cough, a lot. Coughing like that hurts. His throat hurts, his ribs, his back. Only a CF parent will understand this, but I’ve been delighted by the sputum specimens we have been able to send to the lab, the ickiest in a long time - if there is any other shitty bacteria hiding in there, we will find out now, and then aim some big guns at it! 

Isaac, as usual, is mostly in good spirits. He had his six minute test yesterday (they test at the beginning and end of each admission how far he can run between two markers in six minutes) and he tried so hard, but was so breathless. The physios have him in the gym twice a day which helps him shift the mucous from his chest. We normally protest to do IVs at home as soon as possible very loudly, but this time, I know it’s the right thing to be on the ward. The team all continue to aim to get him well enough for our holiday, and I’m feeling more confident now, albeit for the test results. I’ve asked about continuing IVs myself in France but it’s not an option, for risk of sudden reactions and being so far from home. 

As happy as I am that we should (we hope) get to go on holiday, I’d be lying if I didn’t admit that this admission (after IVs in August, October, January, March, July and July again) didn't worry me. Hopefully the tests he is having this week will help us work out why his lungs are having such a bad time. To put it into perspective, that is six (some very long) courses of strong IV antibiotics a year, when previously, he typically had one to two. 

Thank you so much for all the kind offers of help from our nearest and dearest, of which we are spoilt. Good news to follow we hope x 

Hospital and hoping for holidays...

Isaac was admitted to hospital today. He is exhausted and coughing, but otherwise OK. His sputum is noticeably ickier than his norm. As usual, he amazes us with his chilled and accepting attitude, but this time he is worried as we go on holiday soon and so have a time limit to get him better, or risk missing out. Kick arse IVs and intensive physio is our plan A to get him there. I can’t tell you how crushed he would be (our first big holiday in two years) not to mention the girls if they had to go ahead without him. As we are driving (South of France) our plan B would be for me and him to fly down and catch them up when he’s well enough, if that’s even affordable/possible. 

Sometimes I feel like I’m not, not coping, but neither am I coping. Reading that sentence back, I’m thinking this is a sign of some pretty fucking muddled thinking! It’s certainly pretty hard not to get angry that he has to go through so much. On admission today I went through his list of medicines with a doctor, a nurse and then a pharmacist (we always have to do this, I’m never quite sure why so many times). The pharmacist placed her hand on my arm as I listed them off; so many drugs (and that’s without the IVs) why aren’t they keeping him well??? It’s been just three weeks since his last IVs. He needs a break. 

We went to clinic this morning for lung function tests, but as there was no doctor available there, we then had to be admitted via A&E, where we stood in a queue for 45 minutes with a guy in serious chest pain, a drunk woman who vomited on the floor, and a woman with a teeny tiny cut on the top of her head. Thank god for triage. In the end, it was all pointless anyway as the doctor never even came, they just phoned down to A&E to say Isaac was to be admitted as his lung function had fallen significantly and he so obviously needed treatment. NHS I love you, but the crazy bureaucracy less so.

Please send well wishes x 

Sick sick....

The first week of the school holidays, and come with it... a fucking CF exacerbation. 

Coughing. Wheezing. Walking around in a dressing gown, gripping a hot water bottle, in 30 degree heat. Napping. Stairs suddenly feel like a challenge, and huffs with physio sound like they traverse through treacle..... 

This disease holds my son hostage, and I hate it. 

We’re only three weeks clear of his last IVs, I don’t think he’s ever sounded so bad, so quickly. Plus he’s trying to hide how bad he feels as he knows that we go on holiday soon, and he fears another admission. 

We need him better. 

Rothko blue, because I’m blue, and a Miro, because, everyone needs a Miro. 

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.