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! 

Worry list

He has tonsillitis, again. Fevers persist, and his throat is nasty. Especially annoying at the start of his summer holidays. But I suppose he has had a long stretch of good health, so I shouldn't complain too much. He doesn't. His chest is pretty bad too tonight, despite the three different antibiotics he is on. Extra physio seems to be helping. 

Like all mums, I worry, but because of the things I REALLY worry about, I have learnt to worry a whole lot less about some things that I just can't fit in my head. Here is my list of worries today (I reserve the right to re-order at any given time) - if you would like to purchase any worries, please do contact me, I seem to be over stocked right now.

Things that terrify me:

Losing any of my family and friends

Cepacia

mycobacterium abscessus

Transplant

The words 'sudden decline'

Things that worry me a lot: 

Pseudomonas 

CF related diabetes

Aspergillosis

Not having enough of me left to do my best at work and letting people down

CF liver disease

Not having the time to do all the things that I want to do with the kids

Syria, Gaza, Ukraine.....

Being there for my girls as much as I am there for my boy, when CF takes me away

The teenage years 

Have they cleaned their teeth properly tonight? 

Being the best mum, wife, daughter, friend, sister I can be

Things that I don't have time to worry about (sorry):

Ironing

Being anyone other than who I am (used to worry me in my twenties, couldn't care less now)

Getting the kids to sleep in their own beds, rather than diagonally across ours

Travelling, reading, exploring, seeing as much art in the world as I possibly can (because while I can't do it all now, one day I will catch up, I will immerse myself in all the beautiful things in the world..... you won't see me for years!). 

I feel kinda better now for writing that down. Good night all x 

Evenings in the garden...

Just too nice....

Struggling lately, just a little.

I've been struggling a little lately. The boy has been really well (until waking up with a sore throat and fever today) but has seemed out of sorts these last few weeks. He is pushing for more and more independence, like playing out with friends, but instead of pleading nicely, he is grumpy and rude, and then the next minute cuddly and quiet. Tired and pre-teeny I guess. On top of this our big girl has chicken pox, I have ongoing work stress, end of school-term mayhem, more sad news in the CF community (23 is far too young), staying up in the garden a little too late every night (it's so hot right now) and a toddler who never sleeps in = tired and slightly withered Mum. We wait now, 24-48 hours, and if the sore throat doesn't improve, start him on a back up antibiotic (he already takes two daily, but one more might be the ticket) to try and nip it in the bud before it hits his chest. 

In other happier news, I went to Madrid for a short work trip, and fell in love with the place. I could have spent weeks rather than hours in the Reina Sofia, and need to go back to explore more. This is all made possible by my amazing family - a husband who encourages me to go despite all the hassle of managing without me, and parents who are willing to come round at sparrows fart to get the kids ready for school while he works. I am so lucky. 

Some lovely friends of mine are having problems too, B with her own health, and another two friends with relationship problems. I'm glad that I can be there for them. What goes rounds comes round, and I have great friends, and try to be the best friend I can be too. So a few people struggling, but together we laugh and cry and get through it. I am so lucky. 

Have a great day x 

Live life then give life.... Again.

It's National Transplant week. I have previously posted on this topic before, which you can read Here. I feel both lucky and yet also immensely sad that I have known so many stories of people either waiting for an organ that never came, or receiving that most amazing gift from a generous stranger. Some of those stories have affected me deeply; I miss some of those people very much, while I celebrate the new lives that others have gained.

I just urge everyone to think, you are more likely to need an organ donation, than you are to give one.

Eva lived only 3 years after her lung transplant, too little by far, but I know they were 3 amazing years and she did so much to raise awareness of these important issues. When she knew she was dying, she wrote;

a mark on the world
a difference
some proof that i had been here
something to say that i mattered
that when my body left this world my soul had made its imprint

She did matter. Very much, and she has left her heart shaped imprint on me, and no doubt thousands of others across the world. Organ donation can mean a whole new life, a new chance. Sign the register Here.

Read more about the amazing Eva here. Goodnight all. 

Canoe to the coast? Well, almost.

I am most excited. Our next fundraiser for the Cystic Fibrosis Trust is planned! It's the boys turn (I am still growing my hair out after the last fundraiser) and Isaac and his friend Matthew have decided to canoe from our home village to the coast. Only you can't quite get to the coast (due to muddy banks and strong currents towards the Wash, Kings Lynn) so they are doing the next best thing, going to the last navigable part by boat, Denver sluice. This is still a huge trek; 60k, numerous locks, setting up camp, and paddling over 3 days. They are only nine. How great is that? Their Dad's will be with them (no doubt enjoying the odd pint at pubs along the way). The girls and I will be driving the support vehicle, and I hope we can get other friends and family to either join them on the river, or camping along the way with us, or even cheering them over the finishing line (please do contact me if you'd like to join us). Most importantly, you can sponsor them by clicking HERE. It will be very much appreciated, the boys are very excited about how much they might raise. 

Have a great day x

Pre teen and teens

The teenage years scare me. He already acts pre-teeny. The giveaway is the answering back and the overuse of words like epic and awesome (pronounced awwwwwwsum). He makes me laugh so much, it's hard to get cross with him even when he is being cheeky. I imagine like a lot of kids his age, getting him to clean his teeth, hang up his coat, not have crap all over his bedroom floor and haribo stuck to the car window requires some nagging. But add onto that everything else he has to fit into his day, and it can feel some days like nag is all I do.

And yet, right now, his compliance with meds, nebulisers and physio is good. We have a zero tolerance policy on treatments, so he knows no amount of arguing will change the fact that he has to get on with it in the end. But what will he be like in a few years when you add in more homework, friends calling for him to come out, more medication, more physio, more nebulisers....?

Recently one of his nebulised drugs (a nebuliser is an electronic device that turns a liquid medication into a fine mist, better absorbed in the lungs) has been replaced by a super dooper dry powder inhaler, Colobreathe. We celebrated! Instead of 3 lots of nebs a day (10-20 mins each), for now he has just one, plus these inhalers twice a day. But the inhaler isn't quite as perfect as we had both hoped..... It is FOUL. It's like trying to breathe down salty, burning lumps of sticky powder. He has to extensively rinse and spit out after each breath, meaning we have to do it at the sink, and even then he manages to soak the fruit bowl, the eggs, and anything else in the area. Trying to get him to hold this down for the recommended 10 seconds is next to impossible. He really tries. But the older he gets, I worry he tries a tiny bit less. This morning he took mouse sized puffs, so we had to repeat again and again and again, until done. The old nebuliser might have been quicker (and better tasting). So I get on at him, he gets cross with me, I get on at him some more, and then we both laugh. We always end up laughing because I can never stay cross at him. We laugh about how yuck it is (I try it too, to try and prove I can hold it down longer than him.... I can't). But imagining how this and everything else might be a few years down the line... When he's a grumpy teen and we just have more to do?

Maybe I'll be blessed with a lovely, health conscious, polite, hard working and compliant teenager...... they do exist, right?

PGD

This is a post I wrote many years ago when hoping to conceive our second child.  We have gone on to have two children thanks to PGD.

This is for other CF parents thinking of having more children. I thought I'd re-post it here as I know others who are thinking of trying PGD too who are reading. If you are, very best of luck, and thank you for following the blog x


''I hope this might give a useful insight into Pre Implantation Genetic Diagnosis (PGD) should anyone be thinking of following the same route to conceive:

We started by asking our CF clinic to refer us to the genetic councillors at our own hospital. There, we discussed PGD and we felt that this was the right choice for us (as opposed to CVS). As we both carry rare CF mutations (neither DeltaF508) we knew that we would need PGH (Pre Implantation Genetic Halotyping) - a very new test which wasn't available until 2006, so we felt lucky that we could follow this PGD route at all.

We were referred to Guys (the only UK hospital offering PGH at this time). They discussed the same issues with us, and also gave us more information about the test / process. Over the next 3 months they carried out blood works (on samples from both us and our son). Only then could they confirm that the genetic markers they would be looking for in the embryo would be identifiable, and that they could indeed offer us PGD.

Guys then applied for funding for the treatment from our local Primary Care Trust (PCT) on our behalf. This took months. 1 cycle of PGH at Guys costs between £6000-£7000. We were told that in our position (we only had one child, and he had CF) that the chances of getting funding were about 50%. Fortunately for us, our PCT granted to the funding - for one shot.

We then waited for a second appointment with Guys, where we signed consent forms and learnt more about the treatment. I had a scan to check all was well with me, and partner had a sperm analysis to check for any other problems. We were told that at the beginning of a cycle we had about a 1 in 5 chance of success, but if we got to the stage of having healthy embryos to implant, the chance will have risen to 1 in 3. We then went away, and told to call Guys on the first day of my next cycle. There were more delays (at Guys end) which was very frustrating, but we were delighted to finally start in Jan 08.

The treatment begins on day 21 of your cycle (3 weeks after the first day of your period) with a down regulation medication (which stops you ovulating naturally, and so losing the eggs they want to collect themselves). For me, this was simply a nasal spray taken twice a day. About 2 weeks into this I had scan to check that this medication had worked (it had) so I was then able to start the stimulation phase (whilst still taking the down regulation med daily). This was once daily injection which I did myself (into my tummy). The self injecting is much easier than it sounds, you hardly feel a thing and get used to it very quickly. Apart from feeling a little bloated and ovaries feeling tender, my spirits lifted as we counted down the days.

About 10 days into this I had another scan, and they found that I had responded very well to the stimulation (a little too well) and had many follicles (the little sacs that they hope will contain an egg) and blood tests showed I had high Oestordial blood levels meaning I was at high risk of a side effect of IVF called Ovarian Hyper Stimulation Syndrome (OHSS). They warned me that to go ahead with the cycle at this stage could be risky (the OHSS could get worse) and so I was taken off the stimulation medication and had daily bloods and scans for a few days in the hope that the Oestodial levels would come down, and the cycle could go ahead. Fortunately they did.

The last medication taken is called a trigger shot which is a one off injection late at night. It has to be a specific time as the eggs need to be collected exactly 36 hours after this. This is like natural ovulation surge and helps mature the eggs ready for collection. We went back to Guys for the egg collection operation (they empty the follicles with a needle). I was sedated for this op and remember nothing, just feeling a little groggy and uncomfortable afterwards, but nothing too bad. The eggs are then injected with the sperm (called ICSI).

We were very happy to have got this far (because we had feared that the OHSS I had would mean the cycle would be cancelled) and knew that the next part was out of our hands - we could only now hope for good news. We had 27 eggs collected. This is an amazing amount (more typical if you have over stimulated). The following day we had a call to tell us 24 of these eggs had fertilized (again, very good). We then had to wait 2 more days to see how many of those embryos made it to the biopsy on day 3 (the PGD test). Of our 24, 14 made it to this point.

In typical IVF, embryos are transferred to the womb at 3 days old, but in PGD they can only test at this stage (they remove one cell from each embryo with a very fine needle). Whilst testing the removed cell, they hope to grow the embryos on to 5 days old, when they are called blastocyst. This has the advantage that those embryos that make it to blasto stage are probably the stronger ones, but the disadvantage that fewer make it to this stage (but if they didn't make it to this stage in the lab, the chances are they would never have made it anyway) so if you have few embryos to begin with, you could have none make it to blasto, and no transfer will take place. So to have as many embryos make it to this stage as we did was fantastic.

We waited until day 5 to hear how many of our 14 tested were healthy, and were relieved when we were told that only 4 were affected with CF. So on day 5 we had the strongest of the healthy 10 transferred (this is the easy part, much like a smear but also being scanned at the same time, and is quite moving as you get to see the embryo go in on screen). We were also told that as the embryo was classed excellent, they put our chance of success at about 50%, so we were delighted. 10 minutes later I was going home to take it easy and just wait and see.

On day 6 we had a call to say that of the 9 embryos we had left, 4 were suitable to freezing, so we now have 4 little snow babies (frosties :o) waiting for us on ice. A frozen embryo cycle (FET) is much cheaper (about £1000) than a complete fresh cycle, as the expensive part of PGD is in the tests and the egg collection op. So we hope to use these frozen embyros to grow our family in the future. We then had to wait 11 anxious days to do a home pregnancy test to find out the outcome of the whole cycle.

At this point our story goes slightly off track as the mild OHSS that I had been suffering with since the stimulation phase became worse. Up until then I had just felt mildly bloated and a little uncomfortable. But by day 16 my tummy was hard and distended and I went to my local hospital to get checked out. I ended up being admitted for 2 weeks, and looking about 6 months pregnant, I was so swollen (fluids were leaking into my abdomen and my ovaries were 3 times there normal size). The only silver lining at this point was that I had been warned that moderate/severe OHSS (which I now had) was associated with a positive pregnancy test, as the pregnancy hormones add to the problem.

Indeed blood tests confirmed that I was pregnant, yay!

The worst part of the treatment for me was the amount of time I spent away from home. At times I was travelling to London daily (door to door at least 5 hour round trip). The OHSS was obviously an unfortunate (and rare) side effect, and it was at times painful, and definitely frustrating (and ongoing, with blood tests every 3 days for the first 3 months of pregnancy, and I have to inject myself daily to thin blood, and this will continue for another 11 months). But when I compared this to the time our son has already spent in hospital in his 3 years, I would never complain. The chance to have another child out weighed any of these short-term down sides for us. Fortunately our son has been very well in this time, otherwise this would have been all the more difficult. Actually for me, the most negative times were not in the treatment itself, but in the waiting to start. I'm not the most patient of people, and it can feel like your whole life is on hold when you want something so much.

The best bits were that it was an exciting adventure. The science of it all amazes me, and I feel so lucky to have been given this chance at all. A cycle is generally just 6 weeks from start to finish, so whatever happens in that time, you can remind yourself that its is not forever, but a lovely baby is. I am now 8 weeks pregnant, and although it is early days, we are beginning to share our good news (hard not to when we have obviously been up to something for weeks so people are suspicious!). We feel very lucky and blessed, and we're glad that we chose this path (although I understand that it's a very personal decision, and we were very lucky that it was available to us). Had we not become pregnant, we would be attempting a frozen cycle as soon as we could. We've come this far, and we'd have no regrets for trying PGD. Good luck to anyone thinking of trying in future. One thing I would suggest to anyone even considering this is to get the ball rolling early. It took around 2 years from our first request for a referral to actually starting treatment, by which time our son was 3, a larger age gap than we had hoped for, so I do wish I'd asked when he was younger. You can always choose to delay start of treatment later x''