Annual review

We had Isaac’s annual review today. While we go to CF clinic regularly throughout the year, this is the one day in which we do a whole bunch of tests/reviews and look in more detail at how his baseline is changing with progressive disease. Some results we get are immediate, some, like the vitamin levels in his bloods, take several weeks. What we know now: 

• Most blood results are outstanding; but we do know the glucose fasting test (looking for CF related diabetes) was fine. 
• Lung function; down significantly, but as expected since he has a current exacerbation, and he struggled to do the tests while coughing (they were better later in the day when repeated; mornings are not friends with his lungs). 
• Psychology review; yep, confirmed that he gets pretty cross whenever talking about CF. 
• DEXA bone density scan; results outstanding.
• Sputum lab results; outstanding. 
• Dietician review; his diet is great, but he needs to up his creon dose (pancreatic enzyme replacement) and to take it more reliably.
• Chest x-ray; not too bad, considering current infection. 
• Portacath (his implanted venous access device); looked OK on X-ray (there were fears that it might be broken, as he’s had pain during flushes) but he was accessed today fine. We think the pain may be related to the scar tissue he has around the site from a while back when it got quite sore. 
• Physiotherapy review; all fine, but his adherence to treatments when he’s left alone to do them is lacking (e.g. lower last week, while on half term). We will also drop one of his six nebulisers a day, as they would rather he did five really well, than six badly. Plus the sixth is using DNAse twice daily, which it is not licensed for, but he had prescribed after black October (read more here) as his secretions were so ridiculously thick. 
• Ultrasound; enlarged spleen and fatty liver, indicating CF liver disease. He will start a new treatment for the liver, the spleen we already knew about, and is associated with the number of infections he has had. 
• Doctor review; the fact that his lung function has picked up as much as it has after black October, is great, and this year has been better than the previous year. They praised his hard work to stay well. Despite his current exacerbation, they are really pleased with him. 
• He may be referred to Orthopaedics to check what appears to be a curvature of his spine. He was scanned for possible scoliosis a few years ago, and if memory serves me right, it was said then that he may have a very mild case (no action required) but apparently this is something that can become more significant in teenagers. His consultant will refer his X-rays to see what they think. 

Plan; We’ve started IVs today and he had the first dose in clinic. Bloods tomorrow (and twice more next week) to check antibiotic levels in his blood. He will start treatment for the liver issue next week (just tablets). They hope he will be well enough for his sinus surgery next week, and they will do a BAL while he is under GA (hoover up some more sputum from further down, to test in the lab for infection). They may also do a bronchoscopy at the same time, subject to availability on the day. Await further test results. 

Sorry, nothing particularly poetic or insightful here. The day was full on, and he came home feeling sick and has gone to bed. I feel like I have no edges; I’m all blurry. Like most years, it was a mixed bag, but it wasn’t awful either. So proud of him. 

Have a great day, if not today, then tomorrow x 

Two weeks of IV drugs, a stocked meds cupboard, and a dogs bottom. 

Smells like smelly teen spirit

His chest is sounding worse day by day. While we had hoped to do his next IVs post sinus surgery, it’s looking increasingly likely that he can’t wait that long. He coughs all night, and while he is sleeping through most of it, I’m not. The worry in the night is always amplified to screaming point. Him, coughing his lungs out, me, silently screaming mine. 

Last Thursday we arranged for an emergency appointment to have lung function tests and see his doctor. Dan and I were both working, so Dan’s Dad kindly offered to pick Isaac up from home and drive him into Dan, who would take him in to clinic. Only Isaac refused to open his bedroom door, insisting that he felt fine and that he could wait a week until his full annual review appointment instead. I went home mad, knowing his team had gone out of their way to squeeze him into clinic, and worse, that if he leaves it too long, he risks not only getting worse, but also delaying surgery as they won’t anaesthetise him if he’s that unwell. Despite his pleading that he felt better, he then coughed all night long. 

Friday his consultant phoned to speak to him (this never happens) and explained the same thing... that if he needs IVs, he needs them ASAP to have any chance of not delaying the op that he so badly needs. He grumbled a bit, but then conceded that his decision to delay might not have been the right one. As it was then too late to take him in, we’ve started extra orals instead in the hope they tide him over to this week. If he can make it to Thursday, we can kick off IVs while we’re in for his annual review. This would give him 6 days of IVs pre-op and (at least) 8 post-op (post-op infection is a risk, so as long as it takes). If he gets any worse before Thursday, we’ll be in sooner. We’ve been warned already that they may want to keep him in a few days, considering his chest and the significant surgery, but they are balancing up that decision with the fact that they have flu on the wards right now, and that is the last thing he needs. 

More than the delay, the not knowing just how unwell his lungs are right now and the worry about delaying the op, it’s that he is increasingly taking the reins in terms of how and when he wants treatment, and not always for the right reasons; Refusing to go on Thursday was much more about the fact that it is half term holidays and he wanted to stay home and play PS4 than because he really didn’t want to start IVs. Had it been a school day, he would have happily taken the morning off school to go. 

For fifteen years now we have put his health above everything, and knowing that he might not do the same is heartbreaking. He’s going to make a lot of wrong decisions in the next few years I fear, and sitting back to watch that, as a parent, is really hard. When he was grumpy with me for being mad with him (after refusing to go to hospital) I told him that unfortunately for him, I will always love him way more than he can ever hate me for wanting him to to stay well. So ner ner, ner, ner ner (because we are that childish) x 

Isaac, 2019. 

Complaining and coughing.

After a CT scan, the follow up appointment shortly after, and then pre-op the very next day, we were pretty confident of the surgery date being very soon. After a week of waiting, I called, only to find that yes, he is on the urgent list, but this would still mean AT LEAST a two month wait. 

When I explained to the kind but slightly ineffective lady in admissions that he has constant headaches and nausea, is off school a lot because of this, that he also has CF, and that his infected sinuses cause issues in his already infected lungs, she struggled to reply (I was very polite, I always am, I get that this is a strained NHS, and that they do their very best), the only reassurance she could offer was that she would schedule a reminder for herself to check with the doctors again in TWO WEEKS. We cannot sit back and wait that long while he suffers this much. He has days where he walks round with either an ice pack or a hot flannel on his head all day, despite 24/7 pain relief. 

So I reluctantly called PALS and explained the situation. Within 24 hours, we had a surgery date two weeks from now. 

It’s so sad that we have to do this, yet again - we had the same with his last sinus surgery; after months of waiting our CF consultant actually advised us to complain through PALS to have him bumped up the list, as it was the only thing that would work. It seems being under the care of different teams creates a breakdown in care for the individual (at times Isaac has been under the care of both his CF Team (which includes his Doctors, Specialist nurses, Dietician, Physiotherapy, Psychology and Pharmacy as standard) but also Gastro, ENT and Immunology, and it feels like all these teams see only the aspect that they treat, without looking at him holistically; it doesn’t take a brain surgeon to get that blocked and infected sinuses will further hinder someone with lung disease, right? PALS help see the bigger picture across all of an individuals needs and prioritise appropriately. At least I hope that’s what it is, because otherwise it’s just about ‘he who shouts loudest’ and that would be sad too. Anyway, we have the date. 

A few days before surgery, he is also having his annual review (his full CF MOT; chest X-ray, bloods, bones density scan, ultrasound, full suite of lung function tests, diabetes test, dietician, physio, psychology, CF nurse and doctor reviews, all in a single day) and since he is coughing significantly more each day, a likely action plan will be to have two weeks of IVs post surgery.

But our worry is twofold; one that his lungs will need these IVs sooner - which could delay surgery, and two that because he is having surgery and his chest sounds so shitty that they will admit him to the ward, rather than allow home IVs. Corona* fears aside, the hospital is already amidst a flu outbreak, and we would much rather keep him safe at home. Plus, you know, being in hospital kind of sucks.

*Despite the very small rate of Corona infection rates here in the U.K, they are predicting that this could still unfold into a full epidemic here, and if that happens, up to 50% of people may be affected. Stay safe people! x 

Above, pictures of Rosa bean (her, a willing model and me, playing about with my camera). 

Below, little Isey. This was us festivaling it up, almost ten years ago. He will turn 15 very soon. How can that even be? ❤️

Corona virus

If Isaac were to contract this kind of virus, he would be at such a disadvantage from the off, it would be a significant risk to his life. 

We have already seen a virus take him from being quite well, having IV antibiotics on the ward, to intensive care needing breathing assistance - in just a matter of hours. That illness necessitated the use of a wheel chair, 24/7 oxygen, and a physical recovery which took many weeks (I’m not sure our mental recovery is even complete now). Mostly we just feel lucky that he recovered at all. 

However you feel about the risk (or not) of Corona virus, and wherever you are in the world, please, please practice good hand hygiene (this is much more powerful than wearing masks alone) - like having the flu jab, this is more about protecting those more vulnerable around you, than protecting yourself. 

If this does become a pandemic, and it sounds like it will, every one of us must take responsibility in trying to limit the spread of the virus in any way we can. If this means working at home more, if you can; do. If this means battening down the hatches and avoiding all non-essential socialising, if you can; do. If this means avoiding your GP surgery or A&E, if you possibly can; do. 

If you feel unwell, call for advice instead and keep the vulnerable (the very young, the elderly, and those with pre-existing conditions) in mind. Cough into your elbow, not your hand. Use disposable tissues and dispose of them well. As well as washing hands well, use alcohol gels in-between. Avoid public transport. Stay home. What might feel like a mild flu to you, may kill someone else. Take to the sofa, watch films, get well! 

My thoughts are with those in the eye of this storm, and those that fight everyday to help them x 

Sinus pain, surgery, and miracle drugs

CT scan done. The doctor said it was as he expected (since he’s already poked a camera up there anyway). Our CF nurse gave a less guarded review ‘looks ghastly, no wonder he’s feeling so bad’. 

The follow up which was to be a month later, I had moved to 3 days later (happy kick arse Mama moment), and the ENT team have been great, seeing him for pre-op the very next day, so we are all set for surgery, just waiting for the date now (we will keep the pressure on). 

His CF team will liaise with ENT, as while he is under anaesthetic they will do a lavage (hoover up some sputum from his lungs, to test in the lab), and depending on lung function test results, he will likely start a course of IV antibiotics at the same time. He is coughing more each day, and his last course was in late November, so the timing is no great surprise. He will need two weeks off post-op due to infection risks anyway, so tying in IVs to this time off school is no bad thing. 

Our hope now is that the op comes first, and the IVs second. If he becomes any less well, and needs IVs sooner, that could mean he wouldn’t be well enough for surgery, but at the same time, the sinus pain/infections continue to impact his lungs too. Bit chicken and egg that. 

Our beautiful, spirited, Rosa bean.

In typical Isaac style, he is taking it all in his stride. He is not able to exercise much; he is in pain and sick often; he is missing a lot of school - but he still laughs with us everyday. We got his school report, and despite his low attendance (about 80%, so a day a week on average.... and shall we say, a rather lack lustre attitude to homework in general) he is still doing well. I don’t know how he does it. 

In other news, myself and some very good friends are beginning to plan our next big fundraiser... so excited about this! No trekking, hair shaving or throwing ourselves out of planes this time... but an EVENT. More details to follow. 

Best of all, the CF community is awash with incredible stories about how Trikafta (the third generation of these new *small molecule drugs) is having such a crazy positive impact in the quality of lives of the 90% of CF sufferers for whom this will work.... in some cases, it’s mind blowing (e.g. being listed for a lung transplant due to end stage CF, to coming off the waiting list entirely with a 20% increase in lung function - in a matter of weeks!). Many happy tears hearing all of this news, but with it, I’m reminded of all those we have already lost, for whom this comes too late. I can’t help wondering, if it could have come in time for Jayne, would she still be here? For those most recently lost, this seems all the more cruel. In some countries, these new drugs had been developed, even manufactured, but they sat unlicensed in pharmacy warehouses due to cost and frustratingly slow negotiations and process. This must end. Trikafta needs to be made available in the UK now, before more lives are lost. Please sign the petition here.

Have a lovely weekend all x 

*I am asked about these new developments often, so just to reiterate, the current drugs that are licensed will not work for Isaac who is in that last 10% who have a class of mutation where the protein cannot be fixed (as it can be for the majority) as in his case, the protein is never formed in the first place, so we need a whole different approach. But it brings us a lot of hope too. 

Bassin de lumieres ❤️ (need to go): 

Headaches and nausea

Heublein. And my mood today. 

Isaac needs this surgery now. He is missing school, dosed up on painkillers 24/7, walking around with ice-packs or hot flannels on his head, and feeling sick. He has a pre-surgery sinus CT scan next week, but the follow up appointment is a month away. This cannot wait. The lioness in me is poised to fight for him. Again. This can be so exhausting x 

Pain and ports

Hello. 

Isaac is dealing with a lot of pain right now. His sinus issues of a couple of years ago have reappeared with a vengeance. I managed to bring forward an ENT appointment that he has later this month to this week, as his symptoms are worsening by the day; sinus pain makes his head hurt, and the potential (probable) infections in his sinuses make his lungs worse, and a combination of all of this results in a very vomity Isaac. It’s like a vicious triangle of mucous and pain and inflammation and retching. Maybe that is more of a square than a triangle? Anyway, I hope it makes some sense. It basically sucks. 

His last sinus surgery included removing polyps (very common in CF) and shaving off some bone (are your toes curling right now?) to widen his airways. We were warned then that this may not be the last time, and today we we found out that not only will he need this surgery again imminently, he’ll likely need it yearly, until he is 18 and he can have a surgery which is a bit more major that might resolve the issue for a longer time (or forever? I’m not sure). As he was last time, Isaac is enthusiastic about this painful surgery, simply because his day to day pain is so rotten. 

As well as this, his IV access (via his portacath) is troubling us too. Our community nurses (akin to Mary Poppins) came last week to do his normal monthly port flush at home, but the flush immediately entered his tissue around the port, rather than his blood stream as it should. It began to sting, Isaac alerted them pretty quickly, and the area looked obviously red. No harm done, but it raised concerns that his port was not working at all, and since we’ve had recent issues with a fibrin-sheath (causing stiffness in the line), this was not a good sign. Thankfully our CF nurse worked her magic, seeing him outside clinic time, re-accessing his port and managing to flush the line successfully. Possibly the community nurse may not have hit the right spot the first time (imagine feeling his chest, finding a a circular disk under the skin, and trying anchor that moving disk to stab a needle into the centre of that small area blindly? Not easy) but the stiffness of the line is still apparent and an ongoing concern. 

The hope would be for a port to last 5-10 years, and Isaac has had this one just two. If it fails, he will need a new one, which not only means more surgery, but also one less possible place on his body that they can site a port - which are limited, and could mean problems with long term venous access later on. 

That said, it’s a worry for another day! Thankfully his lungs are behaving right now.

Have a great day x 

Trikafta joy

To see an accurate glimpse into life with CF, and then the renewed optimism that one new drug, Trikafta, the fourth drug licensed so far that treats the underlying cause of CF, can bring, click here. If this does not bring you a warm fuzzy feeling in your heart, nothing will. From the brink of needing a lung transplant, to significantly less exacerbations, less IVs, and a much, much better quality of life. 

On average, Trikafta has been seen to increase lung function by 18%. That doesn’t sound like a lot, but if it maintains that increase in people with progressed disease, like Mary, that might prolong that time before needing a lung transplant, for, who knows how long? And for children without progressed disease, it might mean that they never reach the point of progressed lung disease at all. 

As I have said before, this generation of drugs will not (sadly) work for Isaac. These drugs fix a protein fault, but in his mutation, that protein is never formed in the first place... nonetheless, it brings us much hope, and for the 90% of people with CF that these drugs do work for, it really is life changing. Babies born today, with these more common mutations, may never know a life like Isaac has. This brings us much joy. 

So to all of you who have donated to CF research, thank you, and please don’t stop giving until these kind of treatments are available to everyone with CF. A worry of ours is that the big pharma’s will be less interested now - so our fight continues. 

New Year pictures (theme this year; pub names). 

Thank you to all our family and friends, we love you so much x x x 

Christmas, bugs, gratitude

Isaac’s cough continued. We gave the Cipro a few more days, to see if it just needed a little more time. On the downside, his chest sounds wet, and his huff, crackly. But on the plus side, his lung function is stable, and most importantly, he feels well in himself. So to bide us some time, and see if we can clear this cough without more IVs, he has started a mega dose of the dreaded steroids. Dreaded because they can affect his mood, his appetite (ludicrously so) and face (look up moon face). Also because once he is on such a large dose (40mg) we can’t just stop them suddenly, we need to gradually taper the dose back down to nothing over time. There are many other side effects too, so they don’t put him on them lightly. But all that said, they work, so ho ho ho. 

Christmas holidays has bought with it our annual dose of bugs. The girls have both been ill - but forever looking for silver linings, we’ve had a lovely couple of days on the sofa watching films with our little fevering and sleepy roast potatoes, and we hope to have the bugs out the way before a big New Years party (this years theme; pub names). 

To everyone who reads this blog; helps me to spread CF and organ donation awareness; supports our fundraising: thank you so much. We wish you all a fabulous break, and an even better new year x  

My present to you, a genuine, award winning photograph, showing two mice seemingly wrestling at a tube station. 

BEST THING EVER! 

Coughing and monopoly

Yesterday we held our annual raffle at my work in aid of the CF Trust. Some friends and I started this in 2006 (I have been there a looong time, and when you read more about my amazing colleagues, you will understand why). In those days, we used to receive gifts galore from suppliers; chocolates, booze, hampers of goodies, even a PS4 one year. We agreed that rather than try and divvy these up between us, we would make it both fairer for all, and fundraise at the same time, by holding a raffle. I can’t remember how much we used to make in those early days, but I guess it was around £200-300; which seemed incredible! Then we added cakes; each year, the same dear colleagues would bake, and we’d sell them on the day. Over the years, due to much stricter compliance rules, we have less and less from suppliers, and yet the raffle gets bigger and BIGGER. 

Not only do my colleagues donate prizes to the raffle, they bake to sell cakes, and buy tickets to win prizes back - all for CF, and yesterday we made over £1,000! 

Every year, I try and pluck up the courage to say thank you in person at the raffle, but I know I cannot do this without tears. it means too much and I know I wouldn’t be able to articulate my gratitude sufficiently. I will try and make up for that now; a huge thank you to Heather, Charlotte, Beth and Debbie for selling and sorting tickets, and to Richard and Paul for being my voice (and to the many others over who have been raffle compere over the years), to Gem, Sophie, my Mum and many others for baking, and to everyone who buys tickets or cakes. What I would really want to say if I could do so without crying is that CF sucks, truly, there is no getting round that, but thanks to all of you, we have more HOPE, and for that, I thank you all from the bottom of my heart. 

Today Isaac went to hospital for tests, as his chest is getting progressively worse each day. He is coughing so much he is sick at least once a day now. We are only two or three weeks post his last IV antibiotics, and having to start a course now would mean a certain home IV Christmas (or worse, being in hospital) - but we feel like we may have been given a get-out-of-jail-free-card; he’s had bloods and results on Monday will show if his markers for aspergillosis are up (previously referred to here as the f**king fungus) in which case, oral anti-fungals may help. Also, he responded well to salbutamol on his second lung function test, which suggests he is wheezy; which suggests this might be more down to inflammation than increased infection; which suggests he might respond to oral steroids. Again, less intensive treatment than IVs for our family Christmas at home. 

Don’t get me wrong, having the aspergillus back and/or further inflammation (which causes the scarring in his lungs) is not something we’d wish for, but we also wish for him to have a lighter treatment load and less trips to hospital over the holidays. He deserves that. We need him well, and quick. 

.... And then we need a well funded and resourced NHS; access to European research trials; and quick access to new drugs that come out of those trials (not the three years it took for NHS England to agree to fund Orkambi). We need Trikafta (the next of these drugs) NOW and then we need a new generation of these drugs in the future which will also work for Isaac and the other 10% of CF sufferers that these drugs will not yet work for. I will never stop until Isaac has that chance, but today was a sad day in that the election results, I think, hinders rather than helps our chances of this happening any time soon. 

Have a great day x 

A blog by Rosa, age 7

For Christmas I want to give presents and get presents. I already done my presents for my family. My dad, mum, my brother and sister. I’m going to get my dog, Obie, something too. He likes toys. But most of all, I want Isaac to be well. Because I don’t want him to go to hospital at Christmas or cough all the time. This is some pictures of me, my sister, and my cousin. Isaac doesn’t like pictures taken of him, because he is a smelly teenager. Love Rosa. 

PS: We have started extra oral antibiotics to try and knock his current exacerbation on the head, hoping this will be enough to see us through Christmas.  Lizzy x

Cough, rattle, roll... and a new blog.

*Warning, this post will mostly be uber medical, factual and scientific, with high quality data and graphics to support my descriptions. 

Just a week or two post IVs and Isaac is coughing much more. This is not supposed to happen.

It’s hard to describe, but much of what I know about how he is feeling comes from 14+ years of listening to his breathing, holding his chest, feeling the rattle, hearing that crackle, asking him to huff and hearing the resistance. He can, and occasionally will, also tell me with words, but I still have to do a lot of filling in between the lines, as he would much rather gloss over whatever he can, to put off extra treatment. That only works so long, and my job is to ensure it’s not too long, and he’s too ill. Tonight he actually asked for percussion physio, which is telling of how he feels. 

The timing is not good, and we’ve been here many times before before, doing endless calculations; If we try orals first, and they don’t work, we risk being on IV antibiotics over Christmas. Jump to IVs now, and he won’t be happy, having only just come off them, and there is still a chance this is viral and he could physio the arse out of the mucous and gets through this. But leave anything too long, and he risks getting ill and needing to go into hospital just before Christmas... 

The below graph illustrates what we can expect for lung function over time with progressive CF disease. 

Breathing we know to be a pretty handy thing to be able to do, and CF disease is not massively conducive towards this; 

Below is how we might expect maintenance IV antibiotic treatment to help reduce this decline; he dips, we start IVs, he gets worse before he improves, and then we see have a good spell of wellness until we repeat again, and again, and again...

Only this is what it actually feels like; 

The added stress at this time of year is always that we have a BIG FAT LUSCIOUS HOLIDAY that we desperately want to be home and well for, both for Isaac and his little sisters. 

Our lovely Anouk has asked to start a blog about girls and football, please support her idea by clicking here! She would love some followers. My budding blogger ❤️ 

Thinking of others

Last week, on his very last day of a course of IV antibiotics, Isaac called me at work to say 

‘MUM! I PULLED MY LINE, RIPPING OFF MY SHIRT, AND THE NEEDLE IS HALF HANGING OUT IF MY CHEST!’

My usually very cool and calm teen was, quite rightly, a little freaked. I jumped in the car to get home, only for him to call quite calmly two minutes later to say the needle had dropped right out. On reaching home, thankfully his port area looked pretty unharmed. 

The next day we had to head into hospital to be re-accessed, meaning the needle being put back into an already sore port site (I am half-trained to do this, but have not yet done). We have to do this to administer the final line flush he needs at the end of every course of IV antibiotics, to keep the line from blocking. Despite the accidental ripping out, and being re-poked the exact area the next day, he did not complain once. 

Isaac went into this course of IVs feeling pretty well (but needing them to keep his infections at bay and set him up for a better winter) and unfortunately, his post IV lung function tests show a dip in his lung function by the end. It shouldn’t be this way round. But we suspect this is because everything is all stirred up right now, and with some extra physio, we can clear some mucous out, and then he will see the benefits. Dan has also had his long awaited knee surgery and is doing super well. 

To give children with serious illnesses a gift please follow this link to buy gifts that will delivered direct by the wonderful Starlight charity for distribution in hospitals this Christmas. Some in the wish list are just a pound of two, but every gift makes a difference. 

Some dear friends of ours are going through the unimaginable right now, which is always immensely perspective making. Too young, too colourful, too soon, too sad. Hug your loved ones tightly x 

Larks and owls

Today I got up early. Gloved up, apron on, mixed up IV drugs. The smell of alcohol wipes at this time is not kind. I give a very sleepy Isaac the drugs. I only need to nudge him, and he rolls so I can get to his IV line. He dozes, handsome, and I marvel at how he got so big and wonderful. I leave him to sleep a little longer, he looks exhausted even while asleep. Back downstairs I get his nebulisers, physio equipment and tablets ready for his normal routine. I have my first and favourite cup of tea of the day, the sun is still rising. 

Then I wake Isaac up, he feels grotty. Then Anouk (who gives THE best waking up hugs ever) and Rosa, who both tell me they have tummy aches (please no more bugs). 

Isaac does his physio, meds and nebs, and drinks warm milk. He looks pale and doesn’t want to eat (start of IVs is always hard, and he is beginning to cough more). I wash and sterilise the neb equipment while the kids get dressed for school. Argue with all three about whether they are well enough for school (they are). I pull on the least creased clothes I can find. From upstairs I hear Isaac begin to vomit; into the kitchen sink. Sigh. Consider throwing everything that was in the sink into the bin, but the sick is just milk and tablets, so clean up best I can and throw it in the dishwasher. 

I then realise all school shoes are muddy, and scream internally. Rush girls to school, walking Obie at the same time. Shoes get muddier still as I have to venture further into the park than planned to pick up a poop, and Rosa follows me. I stop caring about mud. Rosa cries as the teaching assistant takes her hand and coaxes her into school; this happens everyday still, and it never gets much easier. 

I get back to an empty house. Isaac has managed to go to school, despite feeling rubbish. I look into the games room (our converted garage, a den for the kids) for his discarded clothes, and the bloody door handle breaks off in my hand as I lock up. I can’t then open it. Shit. 

Then I call our community nurses to see if they can come to do the blood levels Isaac needs today. No answer. Just after, our CF nurse calls to say the community nurses can’t come, and we need to take him into hospital this afternoon instead. Community nurses then call to say how sorry they are that they can’t fit us in. So lovely, we know they could if they would. So much of CF care is spent on the phone organising things; Dan and I both work full time, and just the admin of CF can feel like another job on top of that. 

I have a work meeting 1-3 I can’t miss, so call Dan, he will sort it out, thank f**k. Confess about breaking door handle, maybe I don’t know my own super strength? Then I call school to tell Isaac about the change of plan for the bloods, but I can’t get hold of the usual wonderful lady who helps me out with this kind of stuff, so eventually leave a message with the lady on reception who kindly promises to track him down (at lunchtime, in a school of 1200 kids). He is expecting bloods at home, so won’t be pleased to hear he’s got to go up to the hospital again. Feel guilty that I’m not there to explain. 

Home delivery of Isaac’s drugs turns up - 4 huge boxes full of drugs which I need to fit into an already packed meds cupboard. Realise they have sent more saline that I don’t need. Now have 420 vials. Sigh. Drink more tea, staring at the boxes ineptly. 

Then I call into my work meeting (on Skype, thankfully working at home today). One hour in, Isaac walks in. Dan’s dad has kindly picked him up to take him to the hospital, but Isaac is refusing to go. Spend ten minutes pleading with him, whilst also trying to listen to the meeting. Explain that he must have the blood test as his liver and kidneys could fry if the blood levels were too high, and he could even risk deafness. It doesn’t work, but a promise of Sams kebabs tonight does. I get it (not the kebab bit, I’m a veggie) - we were at the hospital for 5 hours just yesterday and he’s tired. 

Then I finish work and run to school for a meeting with Rosa’s teacher. Our darling girl is struggling with school and life in many ways that I won’t go into here. The teacher is so kind. Having spoken to Rosa in a number of different ways, they feel that much of her anxiety and behaviour is down to her worrying about Isaac and her attachment to me, wanting to be with me, always. She is so so loved, but this has not yet given her the skills to embrace life outside of our home. I walk back with Rosa in the grey rain, glad to have my hood up so she doesn’t see that I’m crying. Feel guilty x1000. Get home. Dan has sorted out Isaac’s meds and nebs and he’s already doing physio. This gives me time to make a den with Rosa and cuddle her very hard. 

Then it’s back to mixing up IV drugs for the evening, and I hook him up to the Tobi infusion. I talk Isaac into drinking the liver-saving medicine again that caused his vomiting earlier (this stuff tastes badddddd). Anouk and Dan go to football. I cook (lazily; pasta) and set up IV drugs ready for first thing tomorrow, when we’ll do it all over again. Isaac uses pliers to get into the games room, and I crack open a bottle to toast surviving the day. 

Dan and I are very different in one particular respect; him, the lark, up at sparrows-fart every day for work/football/fishing, never able to sleep much past 6am. Me, the night owl, the bookworm, often found lost in words or music until late. So getting up for weekend IVs is a struggle.... but sinking down afterwards, back in a warm bed, cuddled up with one of my gorgeous kids, ahh.... double the pleasure... then I sleep like an angel. Have a great day x 

Klee ❤️

Sisters ❤️

I will scream my lungs out till it fills this room

Today NHS England finally came to a deal with the pharmaceutical company Vertex that means the 90% of CF patients who have the most common CF mutations will have access (within 30 days) to the first drug that they will ever take that treats the underlying cause of the disease, rather than only its symptoms. Read more here. 

Orkambi, Symkevi and Kalydeco are the first iterations of these small molecule drugs that will limit disease progression by allowing the normal movement of salt and water between cells. These drugs are not a cure, and the long term positive impact of taking them is not fully known, only time will tell, but even newer iterations of these drugs (like Trikafta) are already known to have a more dramatic impact and should be licensed here soon too. Today’s deal should pave the way for these future treatments. For children born today, the hope is these drugs will halt progression before the irreversible damage to their lungs ever occurs. 

This news (after 4 years of intense and passionate campaigning by people with CF and their families) is long overdue, and very, very welcome. 

Isaac is not a candidate for this drug, or others in the same pipeline. Unfortunately he is in the 10% minority that this drug will not work for (see blog below which explains why). But there is other research ongoing and pending clinical trials that may work for his rare mutation, as well as other drugs that are not mutation specific; but these are frustratingly a few years behind. We need to keep him well until these are available, so we continue to fight the progression of this shitty disease. 

If the momentum behind the research seemingly slows, then you will hear my screams. #don’tforgetthe10% 

Today is a very happy day. Next week, we start IV antibiotics again in the next round against the bastardly bugs camping in his lungs x 

I will light the match this morning so I won't be alone
Watch as she lies silent
For soon light will be gone
Oh I will stand arms outstretched pretend I'm free to roam
Oh I will make my way through one more day in hell
How much difference does it make?
How much difference does it make yeah
I will hold the candle till it burns up my arm
Oh I'll keep takin' punches until their will grows tired
Oh I will stare the sun down until my eyes go blind
Hey, I won't change direction
And I won't change my mind
How much difference does it make?
How much difference does it make?
I'll swallow poison, until I grow immune
I will scream my lungs out till it fills this room
How much difference?
How much difference?
How much difference does it make?

How much difference does it make?

Pearl Jam. 93. 

Not having IVs, and time.

Despite a funny week where Isaac’s chest has been up and down (interspersed with some delightful vomiting episodes) his lung function at clinic was surprisingly stable. This is good news, and gave his argument to delay IV antibiotics some pretty punch. In the end, his consultant agreed that he can delay, but only by a week or two. This means our plan to time this extra treatment over school holidays is scuppered (he would miss less school had they been over half term, and we would enjoy a calmer week, balancing treatment and life, as I am on leave for half term anyway). Instead, we’ll being having them very soon anyway, with the added stress of super early mornings before the school rush. 

But I get it. He deserves to enjoy his half term. And while it is a bit scary for us, he does need to be more active in decisions about his own care. We hope now that he stays well enough in the interim that home IVs, when he does start them, are still an option, because if he is not well enough we will end up on the ward again, which no one wants. 

In other news: This week has been filled with incredible news for the CF Community (not that you would know it here, while Brexshit is going on) as a new wave of drugs are gaining approval around the world that treat the underlying cause of CF, and have the potential to dramatically change the course of Cystic Fibrosis in up to 90% of patients. I cried hearing that the US FDA have now approved Trikafta, the stories I have heard from people already on this drug and have felt the impact within mere hours or days (from a pill!!!) are pretty frickin amazing. Each iteration of these small molecule drugs is proving remarkably more powerful than the last. Given that treatments in CF tend to be of a more time or medically intensive type (multiple daily nebulised drugs, and frequent IV antibiotics), the idea that an oral drug each day can have such an impact is pretty mind blowing.  

Then I had a good cry last night, thinking of all those sufferers lost (friends included) that this has come too late for. And I must admit, that as Isaac is in the 10% of patients that these drugs will not work for, a renewed scream for us; the last 10% must not be forgotten (Isaac’s mutation is better explained here.) 

I am so filled with hope, anticipation (mixed with a smidgen of distrust and worry) for what might come for us, and (crucially) when. But bloody hell.... I know this is not a cure by any means, but it is definitely the closest thing to a cure the CF Community have ever come close to! 

So we wait in exciting times. 

It has been 15 odd years now since these kids of ours exploded into my heart... lately I’ve been feeling that time has suddenly seesawed such that I have less time left with them as kids than I’ve already had. I am a sentimental fool at the best of times - but the swing from wishing for more sleep and less worry that they will choke on a grape, or lick a slug... to ‘will they even want to hang out with me much longer?’ has swung too suddenly. 

If you have little ones, enjoy them while you can guys x 

Anouk. 

Rosa (insisting that her baby cardigan still fits as a crop top).

And Isaac. Since he hardly lets me point a camera at him these days, enjoy a mini-Ise x 

Rebel and flu.

We had a double whammy hospital trip yesterday.

Dan went in for a procedure I do not know the name of, but sounded something like cavity wall insulation to me. He had thrombosis earlier this year, which was treated, and then came back, and so they squirted a kind of foam into the now rubbished vein to stop it occurring again. Our total reliance and ongoing adoration of the NHS knows no limits..... right now he is also waiting on a hernia op and knee surgery! 

At the same time Isaac went in for the lineogram to check his portacath, which as suspected revealed he has a *fibrin sheath. The test involved his port being injected with dye while a CT type scanner zoomed in close above his chest him. I stood there in my heavy lead apron, as I always do, making sure that he was OK during the tests. Only now, my murmurs of encouragement are met with a ‘shush Mum’. My not so little boy needs me less and less. 

It was fascinating to see exactly how far down into his chest the port line goes (his port is sited close to his collar bone, but the line reaches near the bottom of his rib cage), to follow the dye moving down the line as they picture it, and to see where it goes wrong. Our consultant still needs to review the images, but initial thoughts are that the port can be used; the sheath means the line won’t bleed back, but the IV drugs are entering his bloodstream properly. We want to use this port for as long as possible, both to avoid more surgery and to prolong the time we use each port-site available to us. 

Another new; chatting to Isaac yesterday about his impending IVs (due to kick off next week) he is now adamant that he doesn’t want or need these. In true teenage style, he won’t really explain his thinking about this, other than...

‘NO, I’m not doing it, I’m fine, I don’t need them’

Only he is not fine. He is not bad, but he is not fine. And yes, we could negotiate with the doctors to delay this course (I’ve left it with Isaac that this is a decision he needs to make with his doctors next week) but I’ve reminded him that delaying further will only increase his risk of getting too sick that the IVs need to happen in hospital rather than at home. Or worse, delaying and he gets so sick and he ends up needing more than a two week course and it drags into Nov, Dec... (Christmas?). A comfortable couple of weeks at home, half of which is over half term where we can just chill - which makes the early mornings, the schedule, the side effects so much easier to manage, seems like the better plan to me. 

But I get it. It means no showers, no football, no swimming. Being tied to a pump. Your Mum coming into your room in the night, rustling in her weird  plastic apron. Waking up to the smell of alcohol wipes. The sore port site. The constant reapplication of tegaderms. The visible lump under your shirt at school. The fatigue. The coughing MORE as a result of the treatment, before starting to feeling better... 

This is the first time Isaac has ever come close to refusing a treatment plan.... yes, he moans at times about the daily grind (six lots of nebulisers, two to four lots of physio and a ton of tablets 365 days a year will do that to you) but ultimately he has always complied after a short grumble, and understood why he needed to (the threat of a PS4 ban usually covers us).

I’m fairly sure we can convince him, or compromise and push IVs back a little, but not much. Plus I know that he needs to become more active in this decision making process as he approaches adult care (in less than two years) and that the push back is understandable... but my worry is he rebels further (from delaying to ‘I don’t need these at all!’). Non compliance is very common in CF, as the burden of treatment is so high. Plus, I rebelled as a teenager. Why would I expect any less from my bright, strong minded boy? I just hope he never stops listening to me. 

One last thought, please consider having the flu jab this year. If you are not eligible for it on the NHS, you can have it at most supermarkets for less than a tenner. You cannot catch flu from a vaccine. It is safe. You are not using up supplies that are better saved for the vulnerable (they will be covered by the NHS). It may save you a lot of suffering. And even better, YOU WILL HELP STOP THE SPREAD which will protect those more vulnerable around you. 

Have a great day peeps x 

* Fibrin sheaths are a heterogeneous matrix of cells and debris that form around catheters and are a known cause of central venous stenosis and catheter failure. 

Portacaths and ports

Hospital tomorrow for this lineogram to check his portacath is working OK. 

Best case scenario; it bleeds back for bloods and takes in IVs perfectly.

Worst case; it’s not working properly for bloods or IVs, and he’ll need more surgery for a new port (also not good because that is one less place to site a port (you can’t use the same vein twice and there are only so many places on a body that you can have a port fitted). Since the life span of a port can be 5-10 years, we could do with this one lasting more than just less than 2! 

Likely outcome is midfield... it will continue to not bleed back, but will happily take IVs, in which case we accept he needs a separate needle for bloods, but happily stick with this port for his much needed IVs.

Isaac is feeling pretty good this week, which feels pretty weird as we will kick off IV antibiotics next week... but knowing this should set him up with the bug-arse-kicking lung health that we want for winter... bring it on! 

No-deal Brexit-shit-lit: What Yellowhammer says:

• Supply chains for medicines and medical products are "particularly vulnerable" to disruption at the Channel ports
• While some products can be stockpiled, others cannot because they have a short shelf life. It will not be practical to stockpile products to cover expected delays of up to six months. 

So just really super reassuring. Thanks. 

Not seeing the woods for the trees.... 

© Pierre Pellegrini

Weekend with little and Obie.

All is good in the woods and sea. 

My heart, it sings. 

Rosa. 

Planning IVs

Lots of great news today at clinic. Lung function is wonderfully, delightfully.... UP. 

Best it has been in months. His team are pleased with his height, his weight, how his chest sounds, his sinuses... pretty much everything! 

We spoke about transition into adult care (this kicks off around now, so he is ready for the big transfer a little over age 16). He will meet his new team soon and have the chance to see the new Papworth Hospital building where his care will be managed. The Royal Papworth has recently moved to the Addenbrookes site where we are treated now; a brand spanking new building with individual rooms for CF patients (hurray!) all the uber mod-cons (state of the art tech, e.g. air changes every minute to reduce infection risks, lifts segregated by bugs that patients grow...). But still, the idea of leaving peads after our long journey together feels HUGE. We have known many of his team from the start and I’ve had more than a few tear stained hugs in that time with them - but Isaac’s attitude to transition today was fantastic (Isaac very much lives for today, worrying very little about the what if’s or the maybe’s). The biggest thing for Isaac, and me, is that from transition onwards, he doesn’t need to invite me. Mind you, he says there is no way he is going without me, simply because he’s too lazy to remember all the details for the many many questions we have in clinic (When did this symptom start again? When did you last have a CT? When did you come off that treatment? What did you eat for dinner last night, and how many Creon did you take for it? Is your cough more of a wheeze or a fruity one today?....)! 

He also had a port flush, and for the third time, his port is refusing to bleed back. He will have a line-o-gram (inject it with dye and X-Ray) soon to check that the port is still working as it should. We can accept it not bleeding back, it means he will need more needles for bloods, but still means it will work for IV drugs going in, which is the main thing. 

The plan is for IVs in October, all going well, which we have scheduled to coincide with school holidays (as he is starting GCSE years, really hoping to up his (circa 80%) attendance record if we can). 

We spoke about the sensitive balance between wanting an IV free quality of life (and limiting the risk of building up drug resistance and the kidney/liver and hearing damage that using toxic IV drugs so frequently can incur) with not leaving it too long that he is so unwell that it means he’ll need to have IVs as an inpatient rather than at home, or worse it will result in long term lung damage and recovery will be so much harder. 

Vigilance is key. Thankfully it is not just Dan and I with an active interest in this now; Isaac too has a much greater understanding and appreciation of staying well and out of hospital as much as we can (the one silver lining of intensive care memories?). This is the first time since 2017 when we first talked about planned IVs that we have actually planned them! Until now he has never lasted the full 12 weeks between courses before needing the extra treatment. 

Today was a good day. Thank you for reading, whoever you are. Have some random pictures! 

Georgia O’Keefe, wave. 

Nan Goldin. 

Persian rug love (a secret obsession of mine!).

What would no deal Brexit mean to us?

Concerns about the supply of medicines in the case of a no-deal-Brexit led me email the pharmacist at our hospital. I got a reply, that while understandable, was about as helpful as an itchy arse when you suspect the kids might have worms; 

An evening dose of meds. 

‘Unfortunately we would not supply above and beyond normal supplies of medicines. If we started to do that, then the current supply chain would fall down quite quickly before we get to Brexit. NHS England are monitoring the current situation to ensure additional supplies are not being made and stockpiles are not being held. The Department of Health / NHS England are working up strategies for supply of medicines in the event of a no deal Brexit’. 

Weekly tablet organiser (add on inhalers, nasal sprays, nebuliser and IV drugs). 

According to a recent white paper published by the Business, Energy and Industrial Strategy Committee, 73 per cent of pharmaceutical imports in the UK come from the EU. This equates to around 37 million packets of medicines every month from the EUworth £18.3 billion.

This is SCARY shit. 

Now, there are some medications people with CF could manage without short-term (the high dose vitamins which they cannot absorb from their diets as healthy people can, the slow release sodium maybe, the acid inhibitors at a push?)

But the Creon (enzyme replacement therapy, which allows the breakdown of fats and proteins which cannot otherwise be absorbed, of which he takes 15-30 a day, and would have agonising tummy aches and no weight gain/growth without) the oral, nebulised and IV antibiotics, the anti-fungals (in Isaac’s case right now, very important) and the steroids....being without these, even for a short time, could be catastrophic. 

Scarier still, delay to flu vaccines (due around the time of Brexit and which have a super short shelf-life), have already been indicated as a nationwide crisis waiting to happen this year, likely to kill thousands at high risk. Not to mention the risk to diabetics and others with chronic illnesses or cancer. 

Our dry meds cupboard. We have a whole shelf in the fridge too. 

Anyway, there is enough catastrophising already (not only in my own head) - what we need now are answers and assurances that our most vulnerable in society will be protected. Assurances that we can actually believe in. Where is that??? 

On a positive note; we are well over our fundraising target for our epic 43 mile trek in June. Check it out here! Thank you so much, again, to my incredible team for walking over 17 gruelling hours, laughing most of the way, and for all the amazing support we have had. This now means our family and friends have raised over £80,000 for the Cystic Fibrosis Trust since Isaac was born. To all our lovely family and friends, this is all down to you, Love, love, love x 

Isaac said to me tonight...

“I can’t believe I’ve been well this long”.

Which is both something to celebrate, but also to recognise. He was last on IV drugs to calm his lung infections down in June. When did two months become a good run? 

Summer.

This summer we are home (having holidayed in May quite expensively). A mix of work and home, day trips, the pool in the garden (we have an over ground pool we set up each summer which Dan has built an amazing deck around it, meaning the kids jump in and out all day) and long dog walks. Yesterday we had a family wedding (thank you so much to Alex and Becky for not only sharing their special day with us, but also for holding a raffle which raised an amazing £560 for the CF Trust). It’s actually been really good to be home. Dan is likely to have three lots of surgery later this year (hernia, vascular surgery due to repeated thrombosis and a knee op for a torn ligament; my poor broken husband) - he’s OK, but it all needs doing, so it’s good to squeeze in some fun before all of that.  Isaac is doing really well.... the anti-fungal drugs really are reducing his symptoms overall (we think), and right now, the only troublesome side effects are his poor cracked lips which bleed. Having last had IVs in June, we’re feeling pretty smug that he is still so well.... if he can last until at least September (or beyond?!) before his next IVs - that would be fabulous. 

Some rare pictures of my lesser spotted teen, and his gorgeous sisters (and cousins Ben and Bodhi). 

Wishing you all a wonderful summer x