CF Myths, part three

This follows on from my previous CF myth blogs.... Which you can see here and from there to there. 

I probably don't want to talk about it, so you don't ask: Not at all. I am always happy that people ask how our boy is getting on.... It shows people care, so please ask away. I find writing this blog cathartic in some way. It gets it out if my head somewhat, reordering things in my mind so I can focus on what really matters. 

But I can't talk for Isaac; it is for him to decide how much or how little he likes to disclose about his health, and for now, that is not much (mostly he just talks to me about it). If things are really not great, he might say more (and oddly I go quiet and say a lot less). 

But please never ask me about him when he is there; you wouldn't believe the comments I have had in the past, serious questions about his health, all whilst he is stood right in front of me. He has CF; he's definitely not stupid, deaf, or ignorant to the fact that you're talking about him. 

Remember, he is the only person with CF that he has ever properly met.... 

A lung transplant is a cure for CF: Sadly no. A lung transplant is only ever offered as a last resort scenario, where living more than two years is unlikely with your current CF lungs. It CAN give people years of good health that they may not have experienced since childhood. It can also bring more complicationso (problems due to being immune suppressed, and chronic rejection) than it solves. It will mean you would no longer have CF in your lungs, but you would still have CF in the rest of your body, meaning the digestive issues remain and the risks of liver disease, CF related diabetes, cancer, sinusitis, osteoporosis all persist, and can affect your new lungs. That said, transplantation is still a relatively new science, if average life expectancy after transplant is five years now.... but some are living twenty years or more, we can have high hopes for the future.

He looks so well, so he must be fine, right?: I wish. Like my friend Jayne says 'It's my innards that are fecked, not my outards'. Isaac looks great, most of the time. But he is one handsome little dude which may dazzle you to not notice that he can look kinda tired and pale at times. He can have relatively cough free days, but with physio sound like his chest is full of jelly. All the same, it's good to know he looks well, just don't forget how hard he works to stay that way. 

Wow, he takes how many tablets? OK, not a myth as such, but I never get how on CF documentaries, or when you have similar diseases on t'telly, they focus on how many tablets that person takes. Isaac takes around 40-50 tablets a day (for a start, he needs some with everything he eats just to digest fats and proteins), and yes, it's a bit of a pain if you've left them in one room and you're eating in the other and you have to go find them, or like the one time we forgot them, got the train to London, then spent ages in Boots trying to get some dispensed (they did, without a prescription, which we were eternally grateful for), but it's not like he spends ages swallowing down each one in painful gulps... On the contrary, he scares me by swallowing 10 at a time. Taking tablets is not hard (OK, granted, it was more of an issue when he was three, and some people are tablet phobic). But apart from 3 years olds and fuss pots, it's really not that hard. 

What they never explain though, is how the SIDE EFFECTS of those drugs, and drug interactions, might effect those people. Now that really can be a bummer. Isaac can barely stand near a low voltage table lamp before getting sunburnt when he is on Cipro. He gets a rash on Ceftaz. He gets grumpy and aggressive on Prednisolone. He gets a tummy ache from too much Creon. He gets a tummy ache from too little Creon. He has some drugs that he can't take one hour either side of milk. Or food, and others he has to have with food, otherwise his stomach burns...... Taking tablets is great, it's the side effects that suck. 

CF is a terminal illness: No way Jose! I once heard a parent describe CF as such, and was appalled. I believe the term 'terminal' describes a disease where you are expected to live less than one year. CF was later described by the Trust as 'Life threatening' and now 'Life limiting', which is more accurate in my opinion. No one knows what is round the corner. Science changes things so painfully slowly when you are full of hope, but it is changing things. We were told when he was a baby that his lungs would be irreversibly scarred by the time he was 5 years old, and we hoped that gene therapy would become a clinical option by then, before it was too late. He's almost ten now, and gene therapy is still a dream, but you know what, he's still doing OK. The life expectancy quoted now doesn't take into account new therapies. I don't believe in one single cure, but as our consultant once put it, rather there would be many small pieces that put together will improve outcomes greatly, and then one day, we will look back and think, wow, CF is a treatable 'condition' rather than a life limiting disease. We truly believe that all these small pieces we are doing now will keep him well enough to benefit for all those others to come. 

Have a great day.

It's a class thing, init.

So here is some science for you. There are 5 classes of CF mutations, class I being seen as the most severe and five as the least, but with lots of variance in each (you can have kids with a supposedly severe mutation who do really well, and others with "mild mutations" do quite badly, as well as twins with the same mutation who have very different outcomes...). Best analogy I can think of to describe these classes is a baking one I'm afraid, but I'm sure you'll all agree, a very realistic, factual account of the problem (CF friends may well want to correct me!); 

You really need to make a cake and get it to a party.... 

Class I; you've got some ingredients, but not all of them. You're fecked basically. 

Class II; the most common problem bakers have, you have all the ingredients, you make your cake, you just don't put it together too well, it flops in the middle. 

Class III; you make the cake, woohoo, it looks OK, but it tastes, well, not great. Maybe the slightly out of date butter wasn't such a good idea. 

Class IV; you make the cake, it's pretty much OK, but on the way out the door you trip, fall, and the cake goes everywhere. 

Class V; you bake a lovely cake, but just before you get to the party to share it, you realise the dog has eaten it in the car. The little shit. 

So, all pretty annoying, and maybe if you were in class five, you'd wish you had never started making the bloody thing in the first place. But in CF terms, the worst position to be in is class I, which means the correct (CFTR) protein is never correctly made, which in turn means a fault in the way water and salt move between cells, resulting in the sticky mucous that those blimin bacteria love to holiday in. 

There are some amazing things happening in CF research right now, and in the last couple of years, the first drug was licensed that actually treats the underlying cause of CF (until now, all treatment has been symptom treating). This is aimed only at people with a certain kind of (class three) mutation, of which there are very few (about 5% of the CF pop). Coming up soon, assuming it gets the astronomical funding that it needs (...which if it does, as the rumoured price stands, would cripple the NHS.... but that is another blog) will help the vast majority (who have class two mutations). This really is exciting times, and makes all the fundraising that we, and other families like ours do, feel so worthwhile. 

These new drugs, if they make it to the people who need it (and I have every faith that they will, eventually) will leave only a small minority of people with other mutations that won't benefit from them because of the different problem they have in the baking of that cake. I just hope that the pharmaceuticals (hopeful) success with the majority won't mean a lack of funding on other research for the minority left behind. 

... Like Isaac - who has two class one mutations. 

In other news, we had an appointment this week to discuss his rather rubbish immunology results, and the news was.... mostly reassuring. Waiting on further blood results now, but every reason to think that this requires no further treatment (which if it had, would mean monthly IVIG transfusions, which we REALLY don't want to add to an already full regime!). And Isaac is feeling great right now and was fab for his bloods. He celebrated with an M&S cottage pie (the one thing he misses from being on the ward). 

And just two weeks until puppy Obie joins us! So excited. In the meantime, one ex chicken this morning, resulted in a trip to get three new ones. Welcome Goldie 2, Floella and as yet unnamed white one. 

Have a great day x

Farewell friends

CF, it is said, is an isolating disease. Cross infection means that people with CF are excluded from things like conferences, where I might meet up with other parents from time to time. It's one of the harder things about CF, much more so for those suffering with it. 

Technology means that we can still share our stories though, and when you have a query about that bloody plastic part in your iNeb which pops out all the time, or you have that sinking feeling when the sputum is distinctly green one morning, these are exactly the people I need to talk to. So there are forums, and these tend to be either for people with CF, or parents of those with CF (I never ever thought I would be a forum kinda person!). Although the two can mix.... they don't always do so nicely; There are parents who write things like 'We could cope with another child with CF, so we're trying for a baby!' Or entitle posts 'My child's preschool allowed them to kick through autumn leaves!' shock horror (see previous blogs on these subjects). This tends to piss off some people with CF, as they are better positioned to recognise that having CF is about them, not their parents, and that physically and psychologically being able to enjoy life (particularly as a child when they are well enough to) is so important (just using some common sense about infections risks, as you would with any child). Equally, someone with CF might post about how annoying their Mum is about making them do their nebs (I'm sure Isaac will be soon...). 

I don't know why, but I often share the views of more adults with CF, than I do some other parents. I have tried to learn from their experiences with CF, and ask them for advice on how best to handle things at times. And while I haven't met many of them, I do count many as my friends.

But having friends with CF comes with some significant problems, in that they get sick; And sometimes they struggle for a really long time. Sometimes for a short time. And sometimes.... they don't make it.

Emily was one such person. She was a great character on the forums years ago (when I was terrified mother of a poorly baby and needed them more). She was very very sick at that time, and had a double lung transplant at age 22. She did amazingly well after that, and co-founded the charity Live Life Then Give Life (read more here) with her friend Emmie, to raise awareness of the serious lack of organ donors. She was a funny, bubbly and caring lady, and I am so pleased that she got to experience the joy of motherhood (through surrogacy) before she died. She suffered at the end from chronic rejection, pneumonia, and a second transplant came too late.

If you want to help at all, sign up to the organ donor register. Spread the word that you are more likely to need a organ transplant than you ever are to give one. And to make sure you tell your family your wishes before it's too late. Emily had 7 great years thanks to her donor, her legacy is that she has raised awareness so that others might too (as well as her lovely baby daughter). 

An absolutely amazing blog.

On the edge of sleep last night I thought of an amazing blog to share with you all. 

Forgotten all about it by morning. Sorry about that. 

All OK in Moly world right now x 

Happy New Year, but not for all.

Isaac is well. We are all well! Christmas has been pretty wonderful after all. I know it sounds cheesy, but I have spent much of it thinking about how lucky we are. We have three amazing children. A supportive family. Fun friends. A house that we have worked hard on to make beautiful (and it is our kind of beautiful). Jobs that we enjoy. Things to look forward to....

At the moment, it is a new addition to our household. That's right, we are having another boy! Albeit a four legged version this time. His name is Obie, and we are in love already... Pictures to follow. He is a German Shorthaired Pointer, and we will have him home in a few weeks. Kids are UBER excited. Of the litter of ten, he was the one that came right over to Isaac and sat on his lap the whole time... Like it was meant to be. We are all looking forward to another someone to love and to running around with him having fun.

And while we have been enjoying our festivities, presents, food, friends and family, all soaked in wine and love, my thoughts have also been with the daughter, friends and family of Emily Thackray, who has been so kind as to advise me about life with CF when Isaac was young, and has done so much for both the CF community and in raising awareness of organ donation for all who benefit from it (see here). I will miss you Emily. It all seems so fucking unfair.

Good night.

A Merry Moly Christmas after all....

Merryness resumes. We are all well for Christmas! At least, we were all well all the way until Boxing Day when Dan woke with a dicky tummy, but we shall blame the cheese for that. We have had a fantastic few days of festivities.

Wishing you all a healthy and merry time too x

A sad film, a happy note

a sad film to watch here

Isaac is feeling better today, hospital put off for a few more days at least. Looking forward to feeling festive now. Goodnight all x

Sick of sucky sickness

I am sat here listening to my boy coughing and I want to cry. 

I am feeling crappy too, just a nasty cold thing, but it doesn't help. How can I be the one looking after him when I can't stop coughing and spluttering myself? 

We have just been away for the weekend with friends, which was great, he managed really well, but home again he looks thinner, pale, and exhausted. He is already on the big guns (Cipro), we have upped his hypertonic saline nebs, physio, and added inhalers. Nothing more we can do right now but hope that he turns a corner very soon. 10 days til Christmas. That should be 10 days of fun filled Christmassy things with his friends and family.... Not home doing more treatments. The mission now is to get him well. And part of that is hoping that we are all well.... It feels like an endless game of pass the bug in our household this winter. Bleughhhhh. 

Merriness to resume shortly. 

Almost festive.

It hasn't been the best of weeks. The Molys need some merriness back! Isaac's chest sounds a bit yuck again, which we dread any time, but all the more so before the holidays (I can't remember a year where we haven't been a teeny bit worried about spending Christmas on the ward). Work has been stressful, but as people who bang on about their jobs are about as entertaining as Ed Milliband, I'll stop there. I will just say that I had to fire someone last week, for the first time ever, which was no fun at all. I know I need to take a step back from all the work stress before I implode... It's just so busy and the pressure is huge. It's not normally this bad, I love my job, it's just.... A LOT right now.

Christmas Break is what we all need. I just can't wait. Dans back is hurting again (he had surgery on it last year) which is always a worry (he is self employed, so bad back means no earning). He works so hard despite it. Went to Anouk's nativity today (our little Elf) and it was surround sound coughing there are so many germs at school right now, it makes me want to keep them at home just to avoid it. Only little Rosa is having a good week, finally getting the whole potty malarkey, woo hoo!

I want to smother myself in family and friends, and yummy food and drink, and loud music, and dancing around the table, and kisses and cuddles, and tickeling fights, and bitter cold walks followed by the fire and hot food.....and and and.....to forget everything that ever worries me. Actually, just writing about this has made me feel better. Roll on Christmas!

Merry Molys

Clinic this week bought good news. His lung function is up another 2%. Doesn't sound like much, but it's going in the right direction, even though he is off the steroids. He is a little rattely again, so we're upping his nebulisers. Five a day instead of four. On a good day this would take him 50 minutes, but in reality, it can take anything up to 2.5 hours, mostly down to his lack of concentration (his neb is breath activated, and it only releases the drug when he has breathed deeply enough for the drug to reach his small airways. Clever things nebs these days). But overall, a good visit, and as always we are so thankful to have such a fantastic team at Addenbrookes.

I'm even beginning to feel Christmassy. We have much to look forward to this month, and beyond. I am one of those people that has to have a project or plan to look forward to.... Be that a baby, an extension, a party, a fundraiser.... and our next plan is something entirely new for us. More to come on that soon! Happy days all x

PS; and just in case she reads, Happy Birthday to the most amazing Mum ever. Last weekend she organised (yet another) fantastic CF fundraiser, and there washing up, I found myself helped not just by her, but also my step dad and my dad. Our family has changed in so many way, but we all come together when it counts. I am very lucky to have them all.

And then there were three...

My children are all playing together. Yes. That's right. All three. And for a whole 42 minutes now there has not been one 'Maaaaammmm' from their den. OK, they have every cover in the house in my room, they have probably stolen some chocolates from the jar, and the den will take a while to deconstruct when they are in bed, but still.... It's so rare these days that they all get on. It's the elder two mainly at each other's throats. Everyone finds Rosa Banosa so funny you just can't get mad with her.

I don't think about it so much these days, but it's moments like this that I do. How lucky are we? How the odds were humongously stacked against us having a second child, let alone a third. So thank you for science, and to a bloody amazing NHS that gave us this chance, our complete family, our everything.

And breathe....

Isaac is doing really well right now, so I have less to blog about. His little moon face is going, and he is back to normal. His kind of normal at least. Clinic next week, and hoping if his lung function is OK, he might be able to stop the hypertonic saline nebs. Four lots a day is taking him a couple of hours, the speed he does them, so one less would make a big difference, and might make getting to school on time more likely. He has appointments in December with an immunologist too, to check out some long standing issues. Hoping for reassuring news before Christmas on that.

I had a lovely weekend break in Norfolk with two of my best friends, eating, drinking, walking, and laughing so much my tummy muscles still ache. I really needed that, I think we all did. Love you guys.

The girls and I are finally getting over our coughs, bit of a bugger that bug, and the toe I think I may have broke stubbing it on the box under the bed whilst ever so slightly under the influence seems to be less purple and throbby. Oh, and I was promoted at work, which makes all the extra hours I am doing because we are so busy that bit sweeter. So all good in merry Moly world right now. Hope you're all good too x

Feeling ill, but not for long.

It's my turn to cough this week. My lungs feel like they are on fire, like a huge itch on the inside. It's funny how when you're well, the idea of a sofa day, while your husband takes the kids out and you have rare custody of the remote, seems quite welcoming. What you forget is just how sucky it feels to be ill. 

For me, this is just a little insight. I slept for 12 hours last night (albeit it coughing the whole time, my body kindly allowed me to sleep in-between). Tonight I will have inactivity induced insomnia even though I still feel tired. But I know others who live this feeling, day in, day out. When climbing up the stairs becomes a struggle, and drug induced nausea makes even the small joy of eating an enemy. 

I think I allow myself this little fantasy where all the love, and treatments, and family, and creativity, and fun, and friends, and action, and fresh air, and hoodies with that soft fleecy inside, and hot chocolate...  all of this and more will somehow really save him from ever feeling that bad. But I guess even I must concede that just occasionally he will feel pretty damn crappy, and what I feel right now is just a fraction of that. 

I know that the chances are I will wake up tomorrow and feel better. I'll get outside and breathe down that bitter cold air into my lungs, and feel cleansed. If not, I'll feel rubbish a couple more days, get down the doctors, who may or may not give me some antibiotics, and I'll recover at a slightly slower rate. But I will recover. Even if this bug leaves me any long term damage, lowering my lung function, I'll probably never know nor notice. Whereas for people with CF, just one more infection might be the one that chips off that extra 10% that they couldn't afford to lose. 

And while they live their lives, and do their treatments, and go to school, or work, and all with the knowledge that their current infection may be irreversibly damaging their lungs, they will be doing all that feeling pretty damn crappy at the same time. 

So no, to those people that have said to me, 'oh I know how you feel, my son/daughter had flu last week and coughed for days' it is not the same (But I still feel for you, flu sucks too). 

On a brighter note, the kids are featured on the Starlight website right now. Which you can read here.

And here  is a link for my CF friends, which has cheered me up today. I hope it does you too.  

Good night all. Stay well x 

Merry Moly World on TV

Did I ever tell you about the time we were on TV? We were contacted through Guys hospital, where we had the IVF and PGD to conceive the girls. Reluctant at first, after meeting Adam, the writer/producer, we agreed. Loved him, and all the questions that he was raising. This was 4 years ago now, around Christmas time, and just before we got married (at home, with the kids and all our family and friends around us) so here you can see a mini Isaac and baby Anouk (make sure you watch to the scrambled egg bit).

To see the full program, search on you tube 'should I test my genes'. It's well worth watching, as well as Adam Wishharts other documentaries.

For the little segment including us, click HERE!

PS; Isaac is doing great right now, thank you to everyone who has asked x

Our crazy lovely boy, our gorgeous girls.

Just sitting here, looking back at our year.

Things have been a bit up and down.

We've had some wobbly moments.

But we're still smiling (with our cousins). 

And we've had some beautiful moments. 

And we've shared these moments with our amazing family and friends. 

Love you guys. 

OK, I may have had a teeny drink tonight and be feeling a bit soppy. So shoot me x 

Nutty kids - love em.

Had a totally ROSA'd couple of days. Bliss. Our childminders extraordinaire (aka, Grandma and Grandad Joe) are away on holiday so I am enjoying a few days off, doing all the school runs and spending some serious one on one with my littlest lovely. She is just so funny right now ('Mama... I'm taller. Me taller, but baby me Rosa, yes?'). I usually only have time off in the school holidays, to be with all three, or covering hospital visits and stays, so this is a nice change. I spent two hours on a boat (sofa) saving her from shark monsters while she bravely made expeditions for supplies (raisins) to enable us to fight the terrible monster (large cushion).... Unfortunately we both died and she insisted we lay tongues loping on our sides for a while. Much fun. 

I have been working my butt off this last year, during a huge period of change at work. I just want to do a good job, and to enjoy it along the way, but at times the pressure is ...... Blimin high. I wouldn't mind, but right now the team I am working in is not a team at all. Rather there is resentment about changes, and stress from the overload. It makes me sad. These are good people, doing a great job. We need to come together, and remember that human factor when coping with what we have, rather than complaining and whining. 

I feel like the only glass half full amongst a whole bunch of half empties.

Isaac too is having a few issues right now, mostly anger ones. Coming off the steroids seems harder than him first being on them.... He can be full of rage one minute, and then soppy the next. We had a good chat in the car (good chats with him always seem to be in the car, it's the only time he's not fidgeting or playing). We still talk all the time, about everything. I hate the thought of ever losing that. Hopefully he'll manage off the steroids and we'll get our Isey back soon. 

Had many a heart melty moment tonight at their school parents evenings. Above all, both their teachers say they are an absolute delight to teach. A sweet, clever girl who just needs a little more confidence, and a twinkly eyed boy who everyone likes and who tries so hard to please. We must be doing something right. It's a nice reminder, as often we feel so tired, trying to fit everything in, we must be letting them down.... after food, treatments and exercise, school work can sometimes be the thing that waits. Plus we think they're kind of nuts, so it's a relief that the school don't. 

So glass of bubbly tonight, listening to some lush Sharon Van Etton on the headphones while Dan geeks himself out on the Walking Dead (his favourite zombie fest). Cheers everyone. 

New heart for New York

A lovely little animation for you to watch Here. 

To sign the UK organ donor register click here!

Have a great day my lovely readers x 

Nature, nurture, and a pile of leaves.

I've always been heavily on the side of nature in the whole nature nurture debate. I honesty believe I could have fairly accurately described my kids personalities, broadly speaking, by the time they were just a few weeks old. Isaac is active, cheeky and smiley. He is very quick to forgive and forget and never holds a grudge. He fidgets, non stop. Anouk is sweet, tactile and thoughtful, and cautious physically, but very very loud. Rosa is cuddly and crazy stubborn (and so hilarious with it). They are all so different. 

I don't believe I have treated them very differently, although I suppose there is a difference in the time I get to spend with each, as his treatments insist. We do try and make it a family thing as much as we can, and all three have been known to lie down for 'patacakes' (percussion physio). But one way we have most definitely not treated them differently is in terms of fun and freedom. 

There are certain bugs which live in soil, stagnant water, compost, hot humid environments like greenhouses.... Which wouldn't be a problem to someone with healthy lungs, but these bugs love a CF lung. Therefore there are certain activities that are best avoided. When you have a baby, this is easily done, but less so as they get older. So, like all parenting decisions, you find a balance that suits your child, your family, your level of sanity. 

We avoid the obvious risks; jacuzzi's (full of pseudomonas, jacuzzis are responsible for many an ear infections in anyone); stagnant water around the house (vases of cut flowers left for days); pond dipping in stinky ponds; playing in composts or greenhouses; damp and mould; mucking out stables. 

We don't stop him; playing in the garden; swimming; playing football and rugby in mud; going to farms; canoeing...... After all, exercise is hugely important in CF health too, and psychologically, so is being allowed to feel normal. In other words, we make a judgement call. Pseudomonas and other bugs like it are everywhere; They are in the air we breathe and the grass we sit on. We cannot protect them from everything. Exercise and enjoying the activities that he shares with his friends are vital to his physical and mental health. 

One of the saddest things I heard at a CF conference a few years ago was a Dad who stood up and said that he had never let his child kick through leaves in the autumn, and not just that, he was also struggling to explain to his son WHY he was not allowed to, as he had yet to tell him that he had CF. The boy was 9. 

Isaac has grown up around the words Cystic Fibosis, and although his understanding about his condition comes in stages, I'm so glad that he never has to go through a sudden realisation about what he has. To me, a sudden realisation later in childhood is much worse than a childhood always knowing about your condition. For those diagnosed late this is unavoidable, but to hide it, and yet limit his life at the same time.... ? I don't agree with that. 

I know this is about fear. And it is crippling (I was much more protective too when he was smaller). But in nurturing them, we also need to let them free a little too. 

Nature or nurture, I think that Isaac's personality will help him through his life with all the extra things he has to do to stay well. That's not to say he doesn't get hugely pissed off with it at times, and he certainly lets us know when he feels like that. But he gets over it pretty quick. He doesn't dwell. I could have told you that when he was a baby. He is every bit as fabulous as I ever dreamt he would be. They all are. Our cheeky boy, our thoughtful girl, and our fiercely funny baby. 

Have a great day. 

Autumn update.

Isaac is feeling so much better. Or at least, he's back to his normal. Better than he has been since the start of the summer, which is so so good to see. 

We are still tapering off his steroids. He has the full moon face now, and has a rounder tummy than he has ever had before (he gives an extra comfy huggle now). I guess he will revert to normal slowly when they stop, he is getting a teeny bit self conscious about his face. The question now is if his lung function will drop again off the steroids, and if so, whether he will need the Broncoscopy still. He's also still on all the other extra medication, which includes 4 nebulisers a day, which is pretty full on, but the advantage to being in hospital is that he is being a bit more compliant now; He got used to doing extra work to stay well, and complains less while actually doing more. We're not yet sure if or when he'll stop the extra drugs. We're also waiting on a referral for another issue (will blog about that if and when we know more). 

Anyway, those are worries for another day! 

Right now, I am focussing on the girly girls, especially Anouk, who has been very fretful since we were on the ward. She is having a hard time, says that she can't even bear me to be an arms length away (...heartbreaking when you are a working Mum, and you have to leave them crying for you...).  

But aside from that, we are enjoying autumn, we've have had lovely friends to stay, cold mornings on the rugby pitch, evenings cuddled up on the sofa as a family with the fire lit and the diary is filling up fast for the rest of this year .... Life is pretty good. 

Have a great day x 

Transplant

One of the funniest people I know, who also happens to have CF, has recently had the scary news that she is unwell enough now to warrant a lung transplant, because her own lungs are no longer behaving (read, really quite crappy). 

She has a lovely husband, a fantastic son, and parents...and friends... and a three legged dog..... and she bloody deserves every single second more that a transplant might offer her, when she is ready to take it on. 

I can't imagine what she is going through right now; Go on the list now, and go forward for a transplant, with the risks of not surviving the operation? Or wait, and risk getting so ill before the chance of a transplant, and not survive? Or to go on the list now, only to find you're in the 'one in three' that does not survive the (too long) waiting list? ....Or go on the list at some point, survive, and go on to enjoy some fantastic good health with new lungs....the generous gift of a complete stranger. This is what I would summarise as a HEAD FUCK (excuse the swearing, but this one really warrants it). 

I'm sorry, that last paragraph seems too crude and is simplifying an issue that is anything but simple.... But this really is a life or death kind of thing. I can only guess at the questions I would have if it were me. 

I don't know what decision she will come to, or when, or what is going through her mind right now, I only know this;

That if she missed out on the chance of a transplant just because someone thought to sign the organ donor register, and forgot, or didn't let their wishes known to their family before they died? What a waste that would be. Sign up, and let your loved ones know your wishes. My friend, and hundreds others waiting for an organ, deserve that chance. 

Click Here to sign up. Thank you x 

A cure? Yes please!

People often say to me "oh, there are so many medical advances, I'm sure a cure is just round the corner" which is lovely, and I certainly hope they're right. I am a total CF geek, reading all sorts of medical journals and following clinical trials, so if there was any wiff of a cure on the horizon, I would know about it. And I'll tell you now why I don't think there will be 'a cure' for us.

The underlying cause of CF is the CFTR gene which normally creates a protein that moves salt and water out of a cell. If the CFTR gene is defective, it results in a build-up of thick, sticky mucus in the bodies passageways.

Up until very recently, treatments for CF were solely focused on the symptoms of the disease. In 1989, when they first identified the defective CF gene they hoped that a cure was just around the corner, but it didn't quite pan out like that. I guess knowing what the fault is, doesn't always mean you can fix it.

Anyway, new treatments are finally being developed which correct the CFTR defect, they can help move or correct the protein and create some normal movement of salt and water out of the cells. The hope is that these treatments will help kids whose lungs are not already damaged by disease, or hold off further damage and stabilise those whose are.

But these new treatments work only for a small minority of people with CF with a certain class of the disease (class 3). They are hoping to develop another drug which will target the class of CF that is most common (class 2) - these are in clinical trials right now.

Unfortunately these drugs will not work for Isaac, who has class 1 gene mutations (associated with more severe disease) because in his case the protein does not need help folding correctly, or getting to the right part of the cell, it's just never made properly in the first place. Bugger.

Have I lost you already? I told you I was a CF geek!

However, that's not to say we are not HUGELY positive about his future. There are some lesser known trials happening on drugs which may help his kind of nonsense mutations, and that's just the small molecule drugs; There is also hope in gene therapy, which is going on in the UK, and is truly groundbreaking - this kind of research is not happening anywhere else in the world.

Plus there is something else we have high hopes for. Since the 1940's median life expectancy has risen from a few months, to the mid thirties. This is largely down to the use of antibiotics, physiotherapy, pancreatic enzymes, and a whole lot of other medication that he takes every day already. I believe that the closest we will come to a 'cure' is a jigsaw of a whole lot of different pieces. Take DNase, which he nebulises daily, this is thought to save 5% lung function. Hypertonic saline, thought to do almost the same... So that's 10% already, and bit by bit, piece by piece, we hope that put together, the myriad of treatments on offer, or to come, this will be our 'cure' - albeit one where he has a lifetime of treatments and medications. It will be a long lifetime.

I hope that gene therapy, or Ataluren, comes along and makes all my dreams come true. Every birthday candle blown out, every Christmas cake stir, every penny in a fountain, wishes come true. But if I'm right, we'll be OK too. Bit by bit we will beat this disease. We just need major pharma's to invest in new antibiotic research - this is a subject that will not just affect the CF population, but us all, if we don't do more about it soon (read more here ). Imagine all those evil little bacteria laughing at us, the big people, at their power over us, to make us ill, to kill.... Forget the Middle East, there are wars going on in our bodies, and we need to help arm against them!

Update on our boy soon x

IV's OV-er.

I am trying to resist the urge to say that it is just one thing after another right now. Clearly I have failed. I also tried to resist the urge for vodka tonight, and this too failed. 

Yesterday his line came out, and the IV's are no more. It's great to get the time back as well as the full use of his bruised and punctured arm. Mixing up the drugs when pharmacy had mistakenly given me 5ml ampules of sodium chloride when we needed 50ml for his Tobi took too long (Being uber sterile means numerous gloves, aprons, needles, syringes, bungs, meds, sharps bins.... The house looks like a dispensary). We get home, he goes straight out on his bike, only to wobble back to me with his chain cog thingy having taken a lump out of his leg. Straight to the doctors for a clean up and steri strips to hold him back together. No tears. It really was quite a chunk - my boy is either mad or very very brave! 

Clinic was OK. We are continuing with the steroids (which have him up and down and everywhere in between) and the hypertonic saline nebs for now. Hoping that the HS might be the reason he still sounds pretty fruity. It would be so good to have a little break from his cough after the truck loads of medication that they are throwing at him, but no (he doesn't cough all the time, mostly with physio right now, so not so bad). Lung function is a little down from last week, but not dramatically so, and that might be down to the steroids working to reduce his inflammation and therefore allowing him to cough up more gunk, which in turn affects his ability to do the tests well. There is still a broncoscopy looming if his chest doesn't dry up soon, but I'll worry about that another day. 

He is also being referred to specialists for two other issues that may or may not be issues at all. I'm not quite ready to talk about this yet, need to get my head around it first*. 

Get home to find that a regular job that Dan and a good friend of ours have been doing for one company for quite a few months now is over, just like that. He is a self employed builder, so it's not his sole job, but it was a good, regular income, and one that he has turned down other work to do. Now it means a last minute scramble for more work, leaving our income, more.... uncertain. Not great timing. 

On the otherhand, Isaac was on great form all day yesterday at the hospital, and we have laughed so much. Rosa did her first number two on the toilet, and black permanent marker does wash out of blonde hair (oh the joys of being a parent), and my Mum managed to fix the big lump of hair that I cut out when I decided I could possibly maybe trim the back of my (short still) hair myself. And it's good to be back at work, school, and some normality. Now time to plan some autumn fun (I love autumn). So lots of Moly merriness too. We're not quite OK, but we're getting there. 

Have a great day x 

*I do always check with Isaac that he is happy for me to blog about his health before doing so. He is old enough to know what he would like known and what he would not, and I absolutely respect that. He absolutely never has a smelly bottom for example. Never. 

Smiling on.

Isaac just about to go into his clinical trial last week. Smiling on. Love that bed hair. 

Nurse Anouk helping Mummy out with the home IV's. 

Hospital schmostimal no more!

Two posts in a row, because I had no wifi earlier.... What a difference a few hours makes! 

We are home! The steroids have done the trick - he is like a fire ball of energy right now, mood swings galore and eating for England (all known side effects), but his lung function is up at last! In fact, it's the best it's been FOR A YEAR! And we are home, woohoo! Continuing the steroids and IV's at home until Tuesday, when we go back for a review and more lung functions tests. They need to see him stabilised, but we are feeling much more positive. His fantastic response to the steroids means he may need to continue you with them long term. (CF friends; Perhaps inflammation rather than infection is his main problem. What do you think? Any experiences of long term steroid use to share?). 

He was so sad and feeling defeated just before the test, it was heart breaking. I am so proud of him, it's not been easy. Now I need to get back to my lovely family for some serious cuddle time. More soon x 

PS: Sorry, got to do a massive shout out to Ben, Wills, Sonny and Matthew for visiting so much (the parentals accompanying them were OK too ;0) it seriously made each day knowing that he had that to look forward to, and the biggest thanks to Auntie Bronte, who came everyday, and sat with him to give me a break. Love you. And again for everyone who helped with our gorgeous girls, who I have missed so so much. We are so lucky to have you all x 

Hospital schmostipal.

We have become super saturated with each other. It's been almost two weeks, and we're still not sure when we'll be home. Today he is grumpy and fed up and angry and frustrated and happy and sad and everything in between. I had a little cry in the garden. One minute he is sweet and cuddly, the next he is refusing to come when the doctors ask him to, and yelling at me. 

Sometimes it just feels so HARD. The day is one long list of things I have to ask him to do (nebulisers X 4, IV's X 4, physio X 4, clean your teeth X 2, go to hospital school X 1, let the doctors examine you X 2, nurses obs X 4, take your meds X 54,  wash your hands properly X 23.....). And I have to do it all with a smile on my face, holding it all together. How can you ask a kid to do all that and not sound like a nag? 

The good news is, he has put on 3kg since coming in, which actually makes me worry that he might actually pop. Isn't that how Elvis died? I checked this with his doctors, but they seem very pleased. His cough is slightly better, in that he coughs less often, and is sleeping better. The long line is lasting well, with no sign of infection. And most importantly, he feels OK. (It is very odd being on the ward where kids are coming in seemingly more poorly than your own, but the next day they are the ones going home, whilst day after day, we stay).

The not so great news is that he is still rattley. With physio he is very productive still, and a huff sounds less like a whoosh of air, and more like a chest full of jelly. This doesn't sound any better to me than when we arrived. He is still breathless with exercise. 

Everything hinges on today's lung function tests. The bottom line is, they won't let him go home until they have seen some kind of improvement in his lung function. On one hand I am so glad that they are committed to seeing is come back up, and not willing to accept that we might have lost that 20%, but on the other I am worried that we might not see an improvement YET (I'm sure we will see it, but listening to his chest, I'm just not sure it's gonna happen today, and if not today, then we'll be in over the weekend at least. It might only sound like a few more days, but it feels like an eternity to us). 

I think now that he feels OK, the only advantage of staying in is the weight gain (not because we feed him less at home, but just that out of boredom and inactivity, he is eating more here). Not wanting to knock anything they are trying to do here, but we were meant to be staying in for intensive physio, but I know he is doing less here than we could do at home. This morning the physio could only give him 5 minutes, and then when I ask him to do more, he argues that he's had all he needs (when I know by his cough, he hasn't). We have half an hour at the gym, but this is nothing compared to the exercise he could be getting at home, even on IV's he could be going on long bike rides. I can do everything at home, IV's, nebs, more physio, that we do here. He is due to finish the IV's in 3 days, but they have mentioned carrying them on for another week. We just really want to see that rise in lung function today so we can do that extra week at home. 

I think I'm sounding a bit desperate now, sorry. Our girly girl is crying before school every day, missing me and having the new school term to cope with at a time when she only ever gets to see one parent at a time, and when she does, they're pooped and worried about everything. We miss our family, our home, our beds. It's hard to be positive all the time. Hospital seems to have zapped it all up. Wish us luck. 

PS; When I started this blog, I promised myself that I would be honest, else what would be the point? So this is an honest account of how I am feeling right now. This blog is about life with CF..... It is not an illustration of our lives as a whole, so if you find it a depressing read, and I sound like I am moaning all the time, maybe find a blog about cats or something. I hope for our family and friends it is a good way of keeping up with how he is, and that it also raises awareness and funds for CF, and names on the organ donar register. We are currently experiencing a CF blip. Happiness will resume shortly. Have a great day x 

Staying in....

We won't be coming home today as we had hoped. There is no improvement in his lung function yet, it's just kind of static, and they want him to gain some 20% before we get out, to get him back to his baseline (really hoping we're not looking at his new baseline).

Started some mega dose steroids to put the heeby jeebies into his inflammation problems, and hope this improves things enough by his next test (Friday) otherwise we could be looking at a broncoscopy (camera down into the lungs for a little scoot around, and a wee hoover out while they're down there, which means theatre, general anaesthetic, and most importantly to him, some time NIL BY MOUTH (his most dreaded phrase, along with 'Hospital School' - oh yes, he is now having a hour a day with a teacher too - he is not impressed!).

Good news is his long line seems to be lasting well, no problems as yet, and he contunues to feel OK(ish), but for the coughing. Chest X-ray showed no pneumothorax.

So again we are thanking all his visitors, family, a friend who kindly sent him a Costa voucher for his hot chocolate addiction (thanks Gabe) and everyone who is helping out with the girls while we stay in. Dan is back to work tomorrow so I am in full time for the next few days, just in time for the steroids, which always have him grumpy and bouncing off the walls. Wish me luck!

Will update when I can (wifi access only in some parts of the hospital). Have a great day x

When the drugs don't work....

I'm not sure I've explained what IV's actually are, have I? In case you're not sure, by IV's we mean a course of intravenous antibiotics. When the drugs don't work (by mouth or nebuliser to his lungs) then IV's are the heavy artillery of bug killing - by feeding the antibiotics directly into his blood. At first he had a normal cannula (a short line, in his hand) but this was then swapped to a PICC line (which is a long line, from his elbow right up to his chest). The advantage of this is that it should last a lot longer, hopefully for the whole two week course, whereas a cannula would only last 1-3 days using it as much as we are. It's also much more comfortable for him. The disadvantage of a PICC line is the risk of infection, which is why have to be so careful when mixing and giving his IV drugs. We have had PICC lines fail before (becoming blocked, or tissued) so we are really hoping that this one lasts the whole course. He was very brave having it inserted on gas and air, but it took two attempts, and it's a long and sometimes painful procedure, so he would be very disappointed to have to do it again. 

For my CF friends reading, he is on Ceftaz and Tobi, for assumed Pseudo again, although his last culture was lost in the lab so we're still waiting to hear what he is currently growing. We've started hypertonic saline again (which he is tolerating much better than when we last tried it), salbutamol, and may start steroids next as his LF is actually down from when we first came in. He is really productive with physio - so we assumes it's just lots to shift. We are just swapping his three times daily Ceftaz to twice daily, in the hope we can get home in a few days and he can get to school some of the time. 

We had some weekend leave today, so took him home for a few hours. I felt OK until then, but crashed in the afternoon. Napped for an hour or so. Think it's all catching up with me. 

It's odd being on the ward when he is outwardly very well. I mean, he is coughing a lot, but is eating fine, and running round with the physios. He tires quickly, but he feels, kind of OK. You have other kids come in with acute problems, or post op, who seem so poorly, but then they're home in a day or two, while we are still here. It's also funny how quickly you become institutionalised. It feels normal now to be popping down to Costa in my slippers, microwaving M&S ready meals for my supper and lying awake at night to the sounds of hushed voices, machines beeping, and babies crying. 

We need to see an improvement in both his cough and his lung function by Tuesday (next test). I don't want to accept that we might slowly be losing some LF with every infection that we're not ever going to get back. He's just too young. 

I'm sorry, I think I'm over tired. Will be back to CF butt kicking positivity in the morning! I'll leave you with a picture of my boy on the beach (can that really just be a couple of weeks ago?) surfing on, despite the thunder clouds looming over. 

Huge thanks you's to our visitors for keeping him going, our parents for helping with the girls, Dan for always being there and coming to watch us sleep (love you) and biggest thanks of all - to the AMAZING NHS Trust with whom we sleep tonight. 

Sweet dreams. 

Hospital stays.

I am home for the night! This is a mixed bag for me, it is the first time I have ever left him on the ward, and the first time his Dad has stayed with him. It's not been an intentional thing, just the way it has always worked out. So I am home, with my gorgeous girls for the night. Little Rosa sang to me as I rocked her to sleep 'let it go, let it go' and now Anouk is staying up late (she needs some special Mama time) and we're watching... Yes, you guessed it, Frozen. I am both delighted to be home, and yet somewhat sad - I think I need to learn to let it go a bit too. 

The good news is he is feeling OK - the physio's have him in the gym daily doing all sorts, we fill our days with giant chess and connect four in the hospital garden, super mario and endless nebs, IV's, and physio. We have both made friends on the ward, and being in hospital is always hugely humbling. We come away feeling very lucky for our lot. 

The bad news is, his cough is about the same, and his lung function has dropped again. Damn it. Early days. He may be started on steroids if there is no improvement soon. 

This admission has been unusual for us. In the past we have stayed just a day or two, before going home, where I complete the two weeks of IV's at home, with community nurses input where needed. It's only been when he is very unwell where we have had to stay in longer. This time they want him to stay in for the first week or so, which we were not expecting. They are trying both intensive physio and hypertonic saline, both of which we can do at home, so hoping we can get him home soon. I have refreshed my IV training (he has a PICC line in, which can be a big infection risk, so we have to be super dooper sterile in mixing up and administering the drugs). We just want to get him home now. Better sleep.. Better food...  Our family all together. 

Being home without him.... Just feels, wrong. 

I made it home from the the hospital in ten minutes. Which tells you two things; we are very lucky to live so close to an amazing (CF Specialist) hospital, and that when I am tense and need to see my lovely girls, I may speed. 

Things that I have learnt (again) this week; My boy is so brave. Hospitals are crazy hot. Every hospital hour = three normal hours. Children's wards are not for light sleepers. People can be so lovely.

When I ask him how he feels about staying in, he said it is OK, painful, fun, tiring and good for snacks (as well as three pretty good NHS meals a day, his dietician drops off extra snacks as he need to up his weight...  This and the Costa hot chocolates and M&S extra breakfasts, which are costing us a small fortune, keep him very happy). He is not unhappy about missing school either. Yet. 

Hoping for better new to report soon. I'm sure we will x 

IV's it is....

Just waiting for a bed on the ward. His cough seems worse. IV antibiotics, steroids and intensive physio. 

More soon x 

Thank you for your support.

Canoe trip went wonderfully - huge thanks to everyone, in particular the Campbell's, who helped come up with the whole idea and made the event such fun. Also to the Fountains, Pledgers and everyone else who joined us, and helped feed and cheer the boys on. Special thanks to my fantastic father in law for setting up camp (and, embarrassingly, saving me when I got the car stuck in the mud miles from anywhere) - don't know what we would do without you.

You can still sponsor the boys by clicking Here

Our family and friends have never failed to completely overwhelm us with their support. However large or small a donation, we know the thought, hopes and generosity behind each one. THANK YOU so much. For this event we are over £1800, and since Isaac was born, up to £45,000 raised for the Cystic Fibrosis Trust. Woo hoo!

PS; Hospital tomorrow. IV's or not IV's... That is the question. Wish us luck.

Escapism and holidays

We all do this, don't we? Use one thing in our lives to distract from the other, scarier shit. The papers always like to talk about women 'having it all'. Either not being able to, or some celebrity showing off that they can and do. I work part time, in Publishing, which I love. I enjoy the work, and really enjoy being with the great people I work with. It is my escape. Since becoming a Mum, I dropped to three days a week, but this year I've gone up to four days (for various reasons). And they are long old days.... I am up before 6am doing physio, nebulisers, meds, and because I do the morning school run, I'm not usually home until around 7pm, for bedtimes. I know a lot of people have longer days - but juggling it all.... it is a struggle sometimes. I either feel like I'm doing well at work, or home, but rarely both. Torn is the best expression of how I feel, and I am guessing how best most working mothers would describe themselves. You either have a great meeting at work, only to realise after that you had baby snot all over your shirt the whole time, or a great morning reading with the kids, only to get to work and realise you have their library book, and they have your tomato salad. 

By working four days a week, I can usually manage all the hospital appointments by either swapping the days I work, or taking annual leave. Next week he is going in twice, so it's a bit of both. I can't miss his appointments - my coping mechanisms involve me knowing as much as I can about him and his CF, the unknown terrifies me, and not knowing exactly what his doctors have said would drive me crazy. On occasions when we have stayed in and I miss the morning doctors rounds while in the shower, Dans hazy reports usually have me asking all day 'but how did he say this' or 'was she smiling when she said that' driving him crazy too. 

I will work for as long as I can keep up this juggling act, and if and when I can't anymore, I'll give it up - which in itself will create a new juggling act with both money and maybe my sanity. 

We have enjoyed a week of laughter away in Wales with fab friends and his cough is much improved, but not altogether gone. It's been a fantastic week of swimming, surfing, beach action (dad's new nick name; Dan-Van-Dam after his expertise at beach dam building), Cipro sunburn (despite all the sunblock and very little actual sun) and non stop playing with his best friends. Really hoping all this exercise, salt air and Cipro have helped his lung function improve for when we go in on Tuesday, otherwise he'll need more treatment. 

So annoyed at myself. I managed to pack everything except his sodium chloride, which meant we had nebuliser drugs, but nothing to make them up with. To have a doctor prescribe him this involved Dan going to a pharmacy, an A&E department, being turned away, but with an out of hours GP's number, and finally a second visit to the pharmacy. In 9 years it's only the second time I've ever forgotten anything, and what a polava it causes, especially frustrating when it is essentially some SALT WATER! 

Keeping his weight up is an ongoing issue - in CF good weight and lung function are known to be positively correlated, and his drop in both lately is a concern. He needs to consume around 50% more calories than you would normally at his age just to keep up, so when infections affect his appetite, the weight falls off him very quickly. His appetite is still uncharacteristically low (for him anyway, he usually eats like a horse on steroids). Big thanks to Mrs F for filling him up with yummy pancakes and homemade hot chocolates on holiday. 

Two new things recently of concern; 

1; he was spotted throwing away some tablets (his least favourite) when camping. What else might we have missed? Back to supervising everything again. 

2; he showed awareness and concern for the numbers he was reaching on his tests at clinic. This is good I guess, but I'm just sad that he has to, and to know that he does worry about things. 

So on one hand he is testing his boundaries and cutting corners, but at the same time, he is worried about his health. How to get that balance between making him realise that his treatments are very important without worrying him is really hard. 

We watched 'Kids with cameras' on TV last week, which featured a kid with CF. He was quite unwell with his CF (30% lung function) and both the sound of his cough, and his rounded shoulder posture were sadly familiar thing to us. On one hand I am so uber positive about our future with CF, but on the other, I worry that maybe I am being naive - is my head in the clouds or the sand? 

Goodnight all, hope you have had a great summer too x 

*I should add, I could not cope without the amazing support I have from my family, in particular my mum and stepdad, who have always had the kids while I work, and my lovely husband who works much harder than I do. Love you D.V.D x 

The big canoe challenge

They kick off tomorrow! He is not altogether well, but we think he is improving. We'll take each day as it comes, but he is a little soldier our boy, not much will hold him back. Fingers crossed the big guns are doing their magic. 

The trip will take 3 days, numerous snacks, one fantastic Grandad who is ferrying the camping gear around and setting up camp, some big cooked breakfasts, some eager visiting sisters, a small amount of beer or wine before bed, quite a few fantastic supporters who are either waving on route or getting in boats themselves, and one frazzled mum who will no doubt get lost in the car trying to follow them. Thank you so much to everyone who is helping us do this, it is a complete team effort. 

Please, if you can sponsor them, click Here and then have a great day with that warm and fuzzy feeling that YOU have made a difference x 

Pictures to follow. 

New model army

Today we had an emergency appointment with our favourite consultant. We are so lucky, we have 3 amazing consultants on the team, and have a long relationship with each, and admire them all in many different ways. Anyway, I digress. His cough isn't improving at all, so we had to go in. He has started to feel pretty grotty now too, and just lieing on the sofa his breathing sounds like the crackling of an open fire. 

The bad news was that his lung function (the main measure of wellness in CF) is down 15% from last clinic - and it wasn't great then. He really struggled doing the tests too, but tried so hard (love him so much). Blowing into a machine both forcefully and for as long as possible induces lots of coughing, so the graphs come out looking like the outline of the Alps. He's also lost weight, which they are concerned about. Good news is the lab results are coming back clear so far, but that doesn't mean there isn't infection down there, only that the sputum he produced for that sample, from that cough, didn't contain any, but hopefully it goes some way to saying there is no heavy growth yet (more tests today, so wait on those results now). 

The plan is to try a new oral antibiotic, Cipro, which is the dude of all oral antibiotics for 3 weeks (problematic side effect; it makes him super sensitive to sun, so almost always looks burnt, even in winter, so bring on the red face). If he is no better (or worse) by week 1, we go back in, start IV's immediately. If he is a bit better by week 1 we carry on with the Cipro, but if not completely better after week 2 we go back and start IV's. If he gets better, just complete the 3 weeks of Cipro, Bobs your uncle. 

The other change, and the one he is very unhappy about, is swapping back from Colobreathe to Promixin. He was on Promixin for years until just a few months ago. It is a twice daily nebuliser, which usually takes him about 20 minutes a pop. We were very excited when Colebreathe was licensed as this is a dry powder inhaler; just breathe in, hold and go. But when we finally received our new toy, it wasn't quite as great as we had hoped. It's REALLY hard for him to hold down, and makes him cough and splutter all over the place. He has tried so very hard, because we all wanted this to work so much (the mixing up and refrigeration of neb drugs, and sterilising the parts, and problems with the machine itself all make nebulisers very frustrating at times, and make things like sleepovers much more complicated). So now, along with the DNase he nebulises daily, he is back to 3 nebs a day. So potentially an hour day (he could do them in less time if he focused more on his breathing, but he chooses not to, sadly leading to lots of nagging on our part). Sadly he blames me for this decision, but really it was a joint decision lead by his consultant and we have to put his health above convenience. 

So, not altogether great, but another chance to kick bug butt before we cave into the last resort - IV's. Isaac and I have an ongoing analogy on everything to do with his chest infections; 

The bugs have set up a small camp in his lungs. They have recently allowed further visitors in and expended the site without permission, they litter all over the place and are generally a dirty smelly bunch. The last lot of antibiotics were arming his white blood cell army with handguns. The Cipro arms them with machine guns. If that doesn't kick them off the site, we will send in the heavy artillery - the IV army. 

We live in this 'will we or won't we be going in for IVs' uncertainty not infrequently, and we usually manage at home, so hopefully the Cipro will be enough again this time. 

In other news; we had flash floods last week and the kids were able to canoe down our road. Very cool; The boys have just 3 days to go until the Canoe to the Coast challenge (you can still sponsor them, see blog below for link); Rosa's (the toddler, must stop calling her baby) new phrase is 'shub up Mama, luff youuuu' (as she shoves me over in bed), melty heart!; We are soon off to Wales with the lovely F's. Lots to stay well for. Well wishes please (and thank you for all the lovely comments people have sent on FB). 

Have a great day x 

Invisible disease

Over the last couple of weeks we have enjoyed the start of the school holidays by going out loads. We have spent lots of time with lovely friends, been to parks, blackberry picking, bike rides, swimming, BBQ's, he's been to a football party this weekend (thank you L, you are a great friend). Once over the tonsillitis, he was up and about and lots of people have said how great it is to see him looking so well. In fact, we always have comments from people about how well he looks - it's great. 

However, all is not always well. His CF team list him as 'colonised with pseudomonas'. This is a bug which adores the CF lung, and would not affect other healthy people. This means he lives with this bug in his lungs all the time. But at times it affects him only a little. He inhales antibiotics and takes others by mouth which keep it at bay. Occasionally the infection flares up, and this is called a exacerbation. 

As well as the Pseudo, he grows some other bugs from time to time, and will also catch as many viral bugs as we all do. Viruses are not treatable, and leave less damage in their paths, but they do make him more susceptible to secondary bacterial infections - they help prime his lungs ready for an invasion. We hope that if someone has any kind of chesty cough they have the forethought to avoid contact with us to limit this risk, but understand that it's not always possible, especially with kids (if they had a known bacterial chest infection, then I am sure they would avoid us like the plague).  

I guess my point is, having a chest infection when you have CF is not the same as having a chest infection when you are healthy. He can sometimes present without fever, without fatigue, without achy bones, but with a nasty old cough. This might sound great, at least he feels well much of the time, but we can't ignore even the slightest cough as this invisible enemy is down in his beautiful pink lungs, setting up camp, and causing irreversible damage, inflammation and scarring. 

So although he is feeling much better, running round having fun in the paddling pool, we will worry until all the test results come back clear and the cough subsides completely. With physio today he was coughing and coughing and coughing. It is heartbreaking. In the night I even heard him going cough cough huff, which is what he is taught to do in clinic (a huff clears sputum more effectively than a cough) - He is learning how to better manage his cough on his own, I'm just so sad that he has to. I'm pretty sure my cough radar is more acute than most, you might hear an occasional cough, I hear every stinky one like a poke in the eye. 

We know that he is on the full whack of medication that they can throw at him orally and inhaled - if the cough is no better, or the lab results show that he is growing something that is not sensitive to the drugs he is already on, or in fact worse, it is, but they aren't working, then he'll need more IV antibiotics, which means going in to hospital. In the summer holidays. That will suck.

So, this is why we worry, and why when you might say he looks great, that's lovely, thank you very much, but we can't always share your joy entirely. Come lie in bed next to him at nights, he gives a lovely hug but you won't get much sleep. 

Goodnight all. 

No better. No worse.

He is still coughing. So we await the results from the lab, and in the meantime add a fourth antibiotic to his daily count in the hope that this one does the trick. He's feeling better in himself despite the coughing, and is still up and about. Clinic soon if no better though. He can't carry on like this, as untreated infections, if he has one, cause long term damage in his lungs. I hope to report back with better news soon.

He was in the local paper the other day which you can read Here.

Good night x

A few days in pictures

Started and (almost) finished a project that we've been meaning to do for years (just needs grouting). The kids all wrote their own names. It was a whole family project with Dan building the patio around us. 

Tonsillitis is slightly better, so we head on a 10m bike ride. Anouk is only 5 and new to being allowed to ride on pavements....she did so well. Stopped for a sweetie break on the way home by the lakes. Beautiful. Stayed up late in the garden afterwards with friends. Perfect end to a great day. 

Headed to the Science Museum, just me and the big two. They started out arguing as usual, but ended up carrying each other round, and sharing a seat on the tube. There is hope for them getting on better this holidays! Love love love. 

His tonsillitis is much better, although he still has that typical tonsillitis voice. But his chest is worse. It's the usual way, I guess if his immune system is busy fighting something new, it takes the brakes off the chest infections which are waiting for their opportunity to thrive. Nasty bugs. Hoping that we're not going to clinic early this week. Well wishes please!