My sister

My sisters biggest fault and her greatest strength is that she is a people pleaser. She will sign herself up to every challenge and committee going, despite already doing too much. 

She is generous to her core. 

She is almost always late. 

But will take 52 minutes saying goodbye before she actually leaves. 

She is sociable and bubbly. 

She knows everyone, and everyone knows her. Probably because of the 34 commitees she is signed up for. 

She has had her heart spectacularly broken, but she got through it. 

She is stronger than she thinks. 

She is fantastic teacher, friend, daughter, Mum.

She is married to someone lovely but so completely different, but between them they have produced two of the best kids ever made. 

I am the proudest auntie. 

She does too much and I worry about her sometimes. 

Unlike me, she can play musical instruments. 

Unlike me, she has really naff taste in music. 

She is always there for me, for us all. 

She is always the first visitor at the hospital, and the last to leave. 

The day after our wedding, she turned up first thing in the morning, hungover, to clear up the party mess before we woke. I'd never loved her more. 

She has joined me on every single fundraiser for CF so far (although not the hair shaving oddly). 

She is the best sister I could ever have hoped for... (apart from when she repeats the same cringey stories from the past about me). 

I hope my kids to grow up able to lean on each other in the same way. 

Knowing you can count on someone so completely is priceless. 

I am one lucky much much younger little sis (he he).... 

Love you Joby-Jo. Happy belated Birthday x x x (sorry, I did write this then, but failed to post it!). 

Big plans.

Last week we had Isaac’s annual review, and lots of good news. Lung function, chest x-ray, ultrasound all looking reassuring. He did great on the exercise tolerance test (looking at his heart rate and blood o2 sats as he runs up and down a zillion times). In his words he ‘bossed’ it. Whatever that means. Other results (blood work etc) are to follow, but we’re feeling good. It's a whole day at the hospital, but he is the best company, it's great to spend that time with him without the distractions of home. We are both as silly as each other and it's a great people watching place. 

 

This week we met with the gastro specialist team about his tummy issues. They are not keen to label his problem, as the management of it would be the same regardless. Bottom line was he is already taking everything they can offer. The full arsenal. The anti-sickness meds are working so well right now, they are happy for us to continue, although we can't rule out future bouts. Surgery to stop his vomiting is a possibility, but even if he has one day a week where is not sick, this proves mechanically things must be working, so for now, we continue as we are, tentatively hoping that he continues to improve.

We see his Immunology team again in May, but have sent in bloods ahead of that to avoid another stabbing. 

 

So, all in all, reason to celebrate – another glass of wine me thinks! Although I have come to the conclusion that alcohol is just a way of borrowing the next day’s happiness… but I'll feel sad about that tomorrow. 

 

Talking of sadness, Jayne's passing creeps up on me when I least expect it. Usually when I laugh, and I think ohh, Jayne would like that and I think to text her. A good reason to think of someone I think, laughter. If I miss her this much, how can her family be managing? 

I’m trying to focus on the things that we have to look forward to. This year feels like a BIG year –

 

We have a proper holiday planned. Two whole weeks! 

But first I am joining Isaac on school camp.... Not because I am a stalky Mum, but because of his nebulisers, physio and meds. Bit much on how own yet. 

Jo, Sarah, Jane, Erika, Lemon, Jess and I will 'boss' our 50km in the Brecon Beacons and raise loads of dosh for CF (please please sponsor us here!)

Isaac and Rosa both start new schools (Secondary and Primary). I'm not sure this one can be true, but I'm told it is. 

We have more plans for the house renovations (we never stop, can't help ourselves).

....But work have announced redundancies – so we might have to put those renovation plans on hold.

.... At the very least, the redundancy risk is making me think about my options. That might not be a bad thing. 13 great years at the Press, I have been lucky. 

We are going to have a new baby in the family for me to squeeze (not mine before you ask, huge congrats to my lovelies, you know who you are!). 

And Isaac will be well. He will, he will.... he must. 

 

Have a great day all. Tell the people you care for how much you love them. X 

Flood walking.

Goodbye to a lovely friend.

Sorry, it’s been a while. 

 

My lovely, funny friend has gone. I guess looking back, perhaps I should have seen it coming? But I didn’t, and it was such a shock. Is it always that way?

 

I first got to know Jayne 11 years ago. Isaac had just been diagnosed with CF and that whole time is like one black hole. I remember some things very little; like telling anyone about his diagnosis. But I remember others like it was yesterday; the smell of his milky breath, the softness of his skin, his tiny little chicken legs, the horrible way that we found out. I felt very scared, overwhelmed by all we had to learn, and this huge sense that it was so bloody unfair (why him, and not me?). 

One of the more shitty aspects of CF is that people with the condition are not advised to mix. In the seventies and eighties charities would organise CF camps, and they had CF kids wards in hospital where they were all free to cough all over one another. Families all knew and could support each other. But it later became apparent that the particular strains of nasty bacteria that wallow in the lushness of a sticky CF lung were ones that easily spread from one CF patient to another, and even mutate to become super baddies. The Liverpool strain of PseudoA was a famous example (in a CF world kind of way anyway). And so, we are now segregated. On arrival at the hospital we are ushered to a room and all staff visit us, rather than the other way round. On the ward we have a side room with ensuite. If we’re lucky. Otherwise, we are on a mixed ward, but never with others with CF or anyone with an infection. We see plenty of concussions, broken bones, cleft lip surgeries, and kids who have swallowed strange objects. There was one toddler who had swallowed a Ping-Pong ball, and his mother told me (on her way out shopping for the day as she left the nurses to look after her son…. cos that’s what they’re there for, right?) that it wasn’t the first time either.

 

Which explains why Isaac has never actually met anyone else with CF. If we bump into someone in the corridor we hurry past keeping our distance, us parents exchanging knowing and sad glances.

 

While I was in that very black hole, I sought solace on the forums that the CF Trust managed, and Jayne and I became friends. The forums meant I could sound off my worries to the only people who really understood. At that time, Jayne was a single Mum, and whilst we became friends because of CF, it never defined her. She seemed so full of life. Wickedly funny, she had the darkest sense of humour I have ever known. She laughed in CF’s face (‘fancy not being able to breathe properly’). She hated people who called her inspirational or brave. She was just living. We shared views which were sometimes at odds to others on the forum (like those CF parents who would say 'I am ready to have another child, I can manage it if they too have CF’ – but it’s not about YOU, is it? It’s about that child). Jayne would rant. And she always made sense. She had the greatest blog, or blogs, which would have me snorting with laughter. She loved her son, and later her new husband, ferociously. She made silent mad videos with her son worthy of an Oscar. She always asked about Isaac, even when her health was very poor, and our own troubles so small in comparison. We talked about our love of watching bats, and flashing boobs at our husbands/decorators (one of those was a mistake). Her texts and emails always seemed to come at the right time, and I could trust Jayne to find the appropriate swear word for every occasion.  

 

I never got to meet Jayne, but we talked about it many times. But I didn’t need to meet her for her to have had a profound effect on my life. And isn’t that what life is about? When you leave your imprint on the world, is it your job, or your house, or what you have learnt that matters? Or is it the effect you have on those who will remember you? I think Jayne’s legacy is her fabulous family and her ever memorable humour. 

A goodbye Jayne would have liked….

 

Dear Jayne, I am very fucked off that you have died. Bloody shitty plum sacks. I love you, and thank you for being my friend x To T&A, I am thinking of you. I am so so sorry x 

 

New breathers.

Very cool transplant video here. Transplant is one of the hardest things I (poorly) attempt to explain to people. My mumblings go something along the line of;

Your lungs are knackered.

You need new lungs to live.

But there is a waiting list due to a shortage of donors, and because not everyone makes their wishes known to their loved ones, who have to make a hard decision at a terrible time.

You hope you're not one of the 1 in 3 that die waiting due to this shortage.

You get new lungs! The op alone is a huge risk, but not having the op means certain death.
You still have CF, but your new lungs don't.

But because your body is so clever, it detects this foreign organ and your own immune system attacks the new lungs (rejection).
Doctors suppress your immune system to limit rejection.
Due to your suppressed immune system you're at greater risk of infections, among other things.
You hope to get many more years from your healthy new breathers. A gift from a generous stranger. 

Watch the video though, says it so much better. And then sign up here. 

Have a great day x

This week in two pictures.

Isaac's new anti sickness drugs; 

Equal the....

Good night all. Things are looking up! x 

PS; Big love and well wishes for my friend Jayne who is back in hospital with post transplant complications. I'm thinking of you x 

The price of life?

I'm sorry, have had some lovely messages asking how Isaac is. My update is very late.

Good news; it is not Gastroparesis! So a mechanical tummy issue is ticked off the list, which is wonderful. For more news we have to wait for his referral to the Gastro team, which is coming, but we have some ideas of what might be causing his vomiting. He has also been prescribed some anti-sickness medication used in chemotherapy, and so far...  so good. 

Had such a lovely long chat with our favourite consultant (apologies to any other families in clinic that day, we totally hogged her) and I'm feeling much more positive. 

We know he has chronic problems with DIOS (CF related) but it's not right that he is sick so much too, although one may well affect the other. In fact, when you consider all of the what if's ....  too little Creon, too much Creon, the many drugs he takes and all the possible interactions of those drugs, the DIOS episodes.... Trying to work out what causes what is frustratingly chicken and bloody egg. 

For now, we are immensely thankful for a vomit free 5 days. 

Interesting article on Orkambi here (a new drug that will treat the underlying cause of CF rather tham just the symptoms, which will be suitable for the majority of CF patients (not Isaac, you can read  here as to why)). And you can support the campaign for access to this and other life changing drugs by signing here. Please. 

I have so much more to say about this, and how I feel about big pharma's and the Governments need to work with them to gain fair access, but for tonight, I am tired. 

Training for our 50k; a very hard couple of weeks at work (they have announced extensive redundancies and a big restructure); and making up lost time with my little girlingtons.... I am weary. Hopeful but weary. I suppose the bottom line of whatever it was I wanted to say is; if your loved one had a chronic, life threatening condition, would you not move heaven and earth in order to access any treatment that could change their lives? And how would it make you feel to know that such a drug existed, but your doctor could not prescribe because of the cost? FYUIGYUIGHknkhjcrtudtyu!!! 

Goodnight all x 

Radioactive egg and toast at last.

Things have been a bit crazy for a while here in Moly World. Dan's 40th rolled on for some time. We've just got back from a weekend in Budapest, just the two of us. We haven't been away without the kids since 2010, and the biggest thing that struck us was the deafening silence. It was so good to read ferociously, sight-see, visit old thermal pools, walk to our hearts content.... sleep, eat and drink. I had a massage from a huge Hungarian guy who smelt like pine needles. I spent half the time wobbling up and down on the bed trying not to giggle. And no, that is not a euphemism. 

But for all the laughs, jeez, we missed our little Moly's too. 

We are so so thankful to all the kids five Grandparents that make us ever going away a possibility. Isaac was sick on day one, which is never nice to see, and his treatments are a lot to take on and manage if you're not used to them. Thank you Mum and Joe. 

Today was back to earth with a bang for some radioactive breakfast and 5 hours of scans to follow. We played games all day and made what fun we could. 10 years in, and the old 'don't step on the cracks' game is still rocking. I quite possibly look like I've had a stroke mind. And i'd like to add that I won Shed 7-1, and no, it's not just a game of luck Isaac :o) 

We have no firm results yet, should know more later this week, but we know it's not good news; they needed to add a fifth scan as he was still showing nuclear powers from within after that amount of time, which is not right. More on that soon. This is him during the scan (claustrophobics look away); 

And after his fasting, him enjoying a special lunch out for being so brave and utterly gorgeous; 

We saw a man at the hospital who carried all his possessions around in an old Sainsbury's shopping basket. As always, being in hospital is so humbling. 

Have a great day x 

Quick update

Things continue to be very up and down. 

I continue to be very up and down. 

He is being sick again, on and off, and the stomach pain continues. No change from the new meds, apart from an increased appetite. Egg and Scan next week. Can't come soon enough. 

Little Rosa is coughing too, poor pudding. Very much hoping he doesn't catch this too. 

In between all of this we have been celebrating Dan's 40th including a surprise party, a coffee machine, Anouk making him a sign and about 50 handmade cards (she is so sweet). All going well, we will have a weekend soon in Budapest (our first in 6 years without the kids). Happy Birthday honey. Don't know what I'd do without you x 

Pilbox passion

This one is for my CF friends and anyone else who takes large amounts of meds. Isaac's Pilbox has arrived, and we love it...

This is our messy meds cupboard (we have another for the equipment and IV stuff). A lot of stuff, and along with his inhalers, Picolax suspensions, nebuliser drugs, Creon and supplements, he takes 19 other tablets a day right now. It's a full time job for us, his CF nurses and our pharmacist to keep us in stock; 

But this leather bound bundle of loveliness, a weekly 'Pilbox' organiser, is about to make things much better;

We can fit in all the tablets he needs for the day, over 4 compartments. The modules all slide out of the case, so can be used individually for on the go; 

No room for Creon as well, as he takes 30-50 a day, depending on how much he eats, so we got an individual case too. Managed to squeeze in 32 capsules into this, so not enough for a whole day, but great for school lunches and shorter days out; 

To serve his drugs we always use little bamboo bowls, easy to clean and scoop stuff out of; 

We have loads of these and they're usually found all over the house. He also likes metal screw up pill pots, which he attaches to his trousers for school and help with discrete Creon popping; 

Kinda cool for boys (be warned, you can buy these super cheap online, but some are very small, so won't fit in enough capsules for a CFer). I'd love to hear from others how they manage and present their meds. I am an organisational geek!  Sorting out the week ahead in one go will save us so much time, and we'll better see when we're low on something. Plus it's something Isaac won't mind carrying round with him. Really pleased with it, it's so hard to find things like this that don't look and smell purely medical. 

....It's been the one highlight in a not so great week - he is back to daily vomiting. Boo. 

Please, if you can, sponsor me HERE for a 50km (most likely 12-14 hours) trek over the Brecon Beacons in June. Training has commenced with vigour! In aid of the CF Trust. Have a great day x 

Egg, toast and jam, with a side serving of radiation.

We are heading back into hospital soon for his next scan, which includes that delicious breakfast menu. I am trying hard to sell it to him, but I have been told it is pretty replusive.... So I suspect it may take some bribing. He is then scanned over the following 5 hours (5 hours? That is a whole lot of card games!). This will tell us just how broken his tummy is. 

You see, what you should never do after being told something might be wrong is GOOGLE. But if I had been tempted I may have found that his last scan, which revealed 'significant delayed emptying and a distended stomach' might be something like 'Gastroparesis'... and if I had read further I may have found that this is otherwise known as 'broken stomach'. Problem is, the symptoms kinda fit... (Stomach aches, vomiting...). Hence the new scan and radioactive breakfast. 

He has already started on some treatment for this condition, funnily enough, more antibiotics which coincidently help make your tummy contract, and his appetite really does seem to have picked up since, which is great. The worry that I have is that the recommended diet for someone with a broken stomach is 'low fat, low fibre, low volume'. 

Now anyone who knows Isaac will know that he needs a high fat diet (because of his CF he has an inability to break down fats and proteins without medication). And due to his DIOS (an unfortunate complication of CF) he needs more fibre. And because of both of these reasons (and the fact that he is his fathers son) he normally consumes very high volumes (whilst gaining little weight). So you can imagine the worry here. 

That said, we take each day as it comes. Today has been a good day. I hope yours has been too x 

ps: I got stupidly excited earlier over a new tablet organiser. I am always looking out for new, non-medical looking pill pots (suggestions welcome). Isaac loves anything that can he can discreetly carry his Creon in - he takes about 30-50 per day, with his food, and just finding something to fit in the dozen he needs for a school dinner is difficult. But this is a weekly organiser for his other (21 currently) medications... A bundle of leather bound loveliness. I'll post picture soon for CF friends. It's by Pilbox. It's all labelled in French, but I think that just adds a certain je ne sais quoi! His new medications are 4 times daily, so this will really help. 

Early New Year Cheer

I bring early New Year Cheer to you good people! I am excited to tell you that I, along with six fabulous ladies will be entering what we hope will be our biggest fundraiser for the CF Trust yet....  

Almost eleven years ago.... When Isaac was first diagnosed at three weeks old, I set a goal in my mind of raising £50,000 for the CF Trust by the time he was ten. Our family and friends have been wonderful and so far between us we have completed the following; 

10k, half and full marathons 

Clmibed Ben Nevis and completed the Yorkshire Three Peaks

Shaved off hair

Skydives

Organised a family fun day

Raffled off wedding dances and asked for donations in lieu of wedding presents

Held charity numerous Bridge days, and many work cake sales

Zip slides across Old Trafford

For Isaac's first personal challenge he canoed from where we live to the coast

Campaigned for our employers to have the Trust as their Charity of the Year

Held a Christmas Raffle annually at Cambridge University Press 

Got all made up in fancy dress to complete sponsored walks

Made and sold over 500 scented candles 

And yet, I am not quite there..... I failed my target :o( 

.... But they say, better late than never, right? If I'm being kind to myself a few pregnancies and many hospital stays have somewhat thrown a spanner in the works, but I am determined to make up for it. And fortunately for me, I have some fantastic friends to help me as always. So we (my ever supportive sister Jo, and my friends Lemon, Sarah, Jess, Erika and her friend Sarah) will be taking on the next challenge together - one much harder than our past efforts... For we will be trekking 50km in a day, over the very steep Brecon Beacons (the training grounds of the SAS). Jeepers! 

I am only about £5000 off reaching my goal. I would really appreciate every single donation, big or small, towards that goal.... So please visit our fundraising page here and continue to read and support this blog.... the whole point of which is to raise awareness of CF, organ donation, and share our own news with friends, family and other CF families (who I LOVE hearing from, so please do drop me a message). 

Thank you SO MUCH for your support - it means the world to us. Lizzy x

Festive pics from Christmas past....

Angelic Isaac*. 

Adorable Anouk. 

Rosy cheeked Rosa.

*Clearly I have an unsafe obsession with allowing small children to wrap Christmas lights around their necks. Don't try this at home kids. 

A Christmas CT scan

Merry Christmas one and all. 

Kids had a ball yesterday. Presents galore, family and friends round. It was more G&T's and prosecco than Christmas TV and charades this year, so today is very much about family, films and securing the best spot on the sofa (although Obie and I had a lovely long walk by the river too. I needed that). 

Isaac had his CT* scan two days before Christmas, and we found out some results that day (no news usually is good news after a scan, so a call this soon is gonna set off alarm bells). A registrar called to say they had a positive result, which at first I thought was just good news, all was positive, right? But sadly no. It was positive as in; they positively found something amiss. 

We're not quite sure what this problem will mean for him yet, but most likely he will be scheduled for another, more detailed scan quite soon. He is also being referred to a new Gastro team. More on this when we know ourselves. This issue may be completely separate to his having CF and the DIOS episodes he has been having; a whole new thing. 

It's always horrible finding out that something is wrong, and hell, life really has dealt him some pretty shitty cards lately, but on the brightside we've discovered what is causing him all this pain, and that's gotta be good. He has had a good few days, less sickness, but more nausea and stomach aches, and he continues to eat about half the amount he usually does (I hope the ample festive chocolates will help maintain his weight, but he has lost a lot already). We can't go on as we are, it feels like an endless cycle of the same symptoms. Plus because of all of the GI problems, his body is having to work harder on less, which means his chest is bound to take a hit at some point. 

We feel frustrated, and hopeful, and worried, and festive, and lucky and just about everything in between. But we are home, we have much to look forward to. 

Happy Christmas all, especially the fabulous Jayne who is now HOME after 11 long weeks recovering from her double lung transplant, saving her from end stage CF. If you support the idea of organ donation, please use this time now with your families to tell them about your wishes should you die. An organ donation WILL NOT go ahead, regardless of whether you have signed up to donate, if your next of kin does not agree. 

Have a wonderful holly-days x 

* I've had a lot of questions about the CT scan. For this one, Isaac had to have a cannula, drink some Gastrograffin for contrast, and had contrast through the line during the scan. He lies on the bed, and the bed zips in and out of a ring doughnut shaped scanner. It takes just seconds, and gives the doctors a detailed cross sectional image of his abdomen (as opposed to an X-ray which is a flat image). It a fairly hefty dose of radiation, so they have to avoid frequent use. A CT is different from an MRI which uses magnets, in a huge enclosed scanner. A CT is a lot less claustrophobic. 

This sadness inside of me.

Many head in hands moments this past week. We are still waiting for the CT (I think there was a mix up with the booking). He is a lot less vomity, but still having stomach aches everyday. I hate that he needs painkillers so much. He also has a worrying new symptom which means that we need to take him into hospital tomorrow regardless of when the CT is scheduled for. Christmas looms ever closer, and we have so much planned. I just want him well.

I spent an hour on the phone earlier today (Sunday) trying to speak to an on-call registrar at the hospital about this new problem, only to be passed from department to department. In the end the reg was found but refused to take my call, saying that consults over the phone were not the norm, and I'd need to bring him into a&e - the exact thing I was trying to avoid. They have the winter vomiting virus going round, and we need that like a poke in the eye. So I had to take the plunge and text his consultant directly - I hate bothering her out of hours, and have never needed to before, I'm not entirely sure how I even got her mobile number. But I needn't have worried, she called me shortly after and was so wonderful. This means we can stay home today, just need to rush him in if anything changes (she explained exactly what to look out for, all OK for now) and tomorrow we'll head in and see our own team. She is going to chase up the CT and bloods he needs, more urgently than ever. She also reassured me that I did the right thing, and that the registrar should have taken my call (as per our written instructions from the team about out of hours advice). It is SO HARD to always know the right thing to do.... We've spent hours in a&e with the drunks and the flu-ridden before, only to be sent home wishing we had waited until clinic hours - other doctors are usually reluctant to make any treatment changes without consulting his CF team anyway, so it's just a waste of time. On the other hand, we need to be vigilant about so much more. Especially lately. 

This wonderful blog by an adult with CF really struck a chord with me; 

'Life’s been really wonderful recently. *knocks on wood'. But there’s also a sadness I can’t shake. The older I get, the harder it is to not become my disease. It’s incredibly important to me to be more than cystic fibrosis, but with each big life event I feel more and more trapped by the hard-to-swallow truths of living with CF. The big one? Knowing a shortened life – one riddled with scary health obstacles – is a strong possibility. As people get older, life gets more complicated, no matter who you are. And when you throw in a life-threatening illness, the complications seem to grow exponentially. It’s a daily struggle to live my life outside of my disease – to not let it seep into each moment of my day. My biggest fear is that it’s changing me. The loneliness… the fear… the what ifs… I’m scared they’re slowly chipping away at who I am. In many ways, having cystic fibrosis has helped me become who I am. It’s made me better, stronger, more empathetic. And I wouldn’t change any of that. But it’s also made me more fearful, less spontaneous, and always worried. There’s this HUGE part of my life that very few people are able to understand. With cystic fibrosis, there’s no break, no day off, no vacation. Every single day there are pills to take, treatments to do, neb cups to wash, scary thoughts to try and ignore.' 

It's not me suffering here, but there is a sadness in me because of it, and I'd do anything for it to be me, rather than him. I don't always feel this sad, but it is always there inside. 

Please send well-vibes for my boy this Christmas! 

Isaac update

Things continue to be very up and down. A lot of throwing up and laying down to be specific. But whilst he has bouts of pain and nausea, in between he is not feeling 'ill', if that makes sense. So if he can get past the feeling sick, and keep down a meal, he can still do things (he went to football training mid week, and tonight has gone swimming). But he only managed two days at school this week. It's really that up and down. When he does feel well enough, we have to seize the opportunity to enjoy what he can. 

We headed into clinic yesterday, and it looked likely that we were heading for another admission, but have been given a reprieve for the weekend. Halla-bloody-Luyah.... It's Anouk's birthday weekend, so very glad that we didn't have to change plans and disappoint her. Instead we are going in early next week and he'll have a CT scan to see if we can find out what the hell is going on in his innards. He has lost 3kg in these last few weeks. These tummy issues, DIOS related as far as we can guess, have been going on for months now, but this lack of appetite and daily vomiting is new. Isaac can tuck away twice his body weight in food on a good day normally, whereas now his appetite is so hit and miss. For this reason alone, we know something is not right. 

So for now, feeling frustrated and sad that he still feels like this, but hopeful that we will know more soon and get him sorted out, and as always, feeling hugely proud - he complains so little, and takes it all on the chin. He literally picks himself back up off the bathroom floor and gets on with his day.... How many 'well' people could do the same? I'm sure I couldn't. 

And finally, Happy Birthday to our beautiful funny dancing Anouk-a-Shnuk. 7 today! I love her I love her I love her I love her I love her x x x 

All I want for Christmas....

Is for Isaac to be well. 

Back to clinic Thursday - lots more sickness this week. I'm feeling frustrated, as we seem to be going round and round and round in circles over these tummy issues. I know there are so many worse off..... But, this is just, so, so, heartbreaking at times. 

Looking good, and a deeply uncool confession.

Things are looking good in Moly World. Isaac's tummy is better than it was. Not right, by any means, but improving. He has a new drug for this, which is the last in the line of drugs that he can do at home. The next one up from this will have to be on the ward, so fingers crossed this continues to work for him. He is also tolerating Gastrograffin again, which we keep as back up. He has previously had rashes from this, and we have to be very mindful of allergies, which can start at any point. We want to keep this back up, so it's really good news that he seems to be tolerating it again. 

We have been in clinic a lot lately, and I think he has probably missed more school this term than any other one. Its all be pretty shit if I'm honest (pardon the pun). We are very much hoping that we can keep away from hospital until after Christmas now .... But taking each day as it comes. We are also seeing a new team psychologist and hoping to work out some coping strategies around his pain, nausea, and yet still having to go to school. 

The girls are as gorgeous as ever and getting excited for Christmas already, as am I. In fact, I have a confession. One which is truly, deeply, terribly uncool...... Are you ready people? 

I have finished my Christmas shopping. And worse (du da da....) I've wrapped most of it too. 

I know, I know. Don't worry - no decorations up, but I really love December and want to enjoy it all; the Christmas plays, kids parties, carols in the village, ice skating by moonlight, and have friends and family over every weekend... All without the worry of all the presents. I started doing this when I was pregnant with Anouk who was born mid December. After blitzing the shopping while pregnant, those weeks after her birthday were wonderful, I could focus on the kids and enjoying everything I had organised already. Its kinda stuck since then. With three kids and lots of Birthdays (Obie included) at this time of year too, we are soooo busy right now. 

So, while I am out sipping mulled wine and singing Fairytale in New York, I'll think of you suckers pulling your hair out and fighting over that last toy in the shop. Ha ha! 

I have just got home after a night away for work. Nothing like a horrible drive home to make you appreciate just how lovely being home is. I'm going to sleep in a mountain of girly cuddles tonight. 

It's also our 5th wedding anniversary this month. Happy day my love, I really don't know what I would do without you x 

This is us looking a little bit tipsy after the wedding. After a small ceremony, we had a big party at home. Best party ever! You can fit a lot of people and love in a little house if you really want to. 

Classy picture. Making the Moly World M x 

Why you need to tell the people you love how you feel....

Lovely little film here. 40% of potential organ donations don't go ahead because of that missing conversation. Communicate people!

Sick people don't need grapes and sympathy, they need company.

However he feels; ...... sick, in pain, tired, Isaac has never once declined company. He makes do with me most of the time, or his Dad, sisters, the dog. We play cards, rub his back, talk rubbish, get beat at video games....  And that might be what he both needs and wants when he's throwing up, can't leave the bathroom, trying to keep down the terrible medicine. But the rest of the time (give us a call) the single nicest thing people could do is come and see him; parents with kids especially. 

It can be a lonely old business sometimes, feeling crappy. I might like a glass of wine and an adult conversation, or a chance to walk the dog and leave the house/hospital for the first time in days, and I know he likes to kick back and just be a kid for a bit. His friends have him giggling in a way that is unique to clusters of kids. 

You see, this is not like an acute illness that knocks your kid out for a few days. We don't know that this treatment will work. We don't know if we'll be back in hospital next week trying something else. And even if this passes, that something else won't take its place soon after. It's not been a bad year, but he hasn't been completely well all year either.... 

Having friends and family who make the time to come see him when he's been off school, or send him a card in hospital so he knows people are thinking of him, that really makes a difference. 

We don't need grapes, just people to play with (and chocolate maybe). And that includes his sisters too. 

His tummy seems a little better. He has eaten very little these last few days, looks a bit worn out, but hasn't complained at all (thank you Minecraft). 

He did say to me this morning 'why do I have to have this Mum?' and I was just about to reply, 'Oh Isey...' When he replied in an American drool 'is it cos of DEESE BALLS' and laughed his head off. I have no idea what that's from or means, but it perfectly sums up him, laughing in the face of a lot of crap. 

Boy done good. I love him so so much x 

Clinic today

As I have said too often recently, Isaac is having ongoing tummy issues. This never used to be his main problem. He has grown a nasty little bug called pseudomonasA for many years now, so has fairly regular exacerbations (imagine a little bug-campsite in his lungs, with all-year-round campers who, whilst occasionally in trouble for things like littering, on the most part behave and keep themselves to themselves. Problem is they occasionally invite their friends, drink too much, and have a right old party, spilling out of their own campsite and causing all sorts of havoc). This keeps us pretty occupied CF wise... But 2015 is mostly definitely the year of the tummy (fortunately the campsite has been quiet recently). 

X-ray today revealed his DIOS is back and worse than ever. He's actually been quite bright this week, but at clinic today looked pale and in pain. He did a fantastic job of talking about his symptoms, and when later looking at the films I felt so proud, as he was absolutely spot on. So none of this is good, but Isaac and I are both feeling positive. We were given the option to try another drug at home (the explosives) which we will do this weekend - will not be leaving the house for 3 days now, but much prefer that to staying in hospital again. We also have a plan B (the big guns) and a longer term plan too (troops on the ground). 

He has had acute episodes of DIOS in the past, but nothing like this. This has been on/off, but never completely better, for months now. Enough pain. Enough nausea. Enough pain killers for my ten year old. We can beat this now! 

Other news; he has lost a little weight (understandable) and his lung function was down, but he is growing well. We agreed the drop in LF could be tummy related (pretty hard to blow furiously into a machine for as long as you can when you don't feel great). We'll be back in clinic next week, so will worry about that then. I can't thank our CF team at Addenbrookes enough. They go above and beyond in my opinion, and I'll be eternally grateful to them. 

So we are home with our arsenal of new drugs...(bringing his prescription list to a tidy 20). Bring it on he says! I honestly think my heart might pop out of my chest I feel so proud of him sometimes. 

In other good news, Jayne has left the ICU 5 weeks after her double lung transplant, and is back on a normal ward. This makes me very very happy. BIG love to Jayne, Tim and Adam. I can only imagine how hard it has been, and I'm so excited for your new life Jayne. 

Have a great day all x

Hello.

Isaac is super well today. Best he has been in ages. That's about all I have to say. I guess I feel bad that I post more when he is sick. Really CF is very much in the background of our lives when he is well. To others it might seem like taking 50+ tablets a day, and me forever shrieking ......NEBS ISAAC that CF dominates our lives... But that is just our norm. We really don't think about it so much these days. We are too busy doing whatever it is we all do, which is a lot. 

We have clinic this week, but I'm going in feeling confident that he is going to continue to kick some serious CF butt.

Have a great day all x 

This is my bed

This is MY bed. 

OK, fine. This is my bed. 

To be continued with the chapter 'dog on the sofa'. 

Happy day all. Big shout out to Jayne, making a good recovery, hopefully leaving ICU soon x 

Whoosh.

I used to cycle along, little wheels, little legs, felt like so far, the wind always blowing hair in your face. Then from behind, a push from a parent, and whoosh. A helping hand to get that bit closer to home. 

Isaac is in trouble at school. He's in trouble at home too. All at once. Glowing reports every year, then suddenly he's like this. Teenage angst before time. He gets SO ANGRY at times, which I can understand. I really don't believe that we let him get away with anything just because of his CF, apart from chores perhaps - since he spends a couple of hours doing nebulisers, and learning how to mix up his own drugs, it hardly seems fair to make him empty the dishwasher too. Not yet anyway. He is relatively well right now, so no obvious reason as to why he is acting out so much suddenly. Secondary school looming perhaps? His PE teacher said she had never had to really tell him off in all 6 years of teaching him, until these last weeks, she can't understand it either. I mean, don't get me wrong, he's no angel at the best of times, he is a strong personality. But he has good bones. Right now, he's just acting out of them. 

This (amongst other things) has been making me feel like I must be somehow failing him. And if I'm failing him, then I must be failing them all. Have I bitten off more than I can chew? Three kids, dog, husband. Work is full on. Constant restructures leave everyone unsettled. We need the stability of my work to offset Dan's self-employedness. But maybe my work is no longer a sure thing. Should I work more, while I can? Or be home more? I've always said I would only work for as long as he is well enough for me to do so, but as well as physical wellness, there is the mental. Mine and his! 

A few times this week, I feel like the rug has been pulled out from under me, at least small tugs. Thinking of a friend who lost her little one, and Jayne, on the painful road of recovery. Immigrants getting colder on the streets as winter comes, the children.......... 

And then people are kind, and reassuring, and wise, and these gestures help pick you back up, whoosh! There is so much sadness in this world. But much kindness too. 

Clearly I am feeling soppy tonight. Forgive me. 

Doing well

Jayne is doing really well. I can't tell you how happy this makes me. Organ donation, transplantation, it is all truly incredible. Just to think about the scientists, doctors and nurses that make it all possible.... Our NHS is beyond amazing. 

Since I'm in a happy mood, I'll leave you with some more happy pictures... Have a great day x 

My handsome husband, Dan. 

Our little Rosa. 

My best friend and I. 

Isaac. Superhero day. 

Anouk the shnuk and I. She loves a group selfie I'm afraid! 

Jayne has new lungs!

My lovely, funny friend Jayne had her third call for new lungs, and this time it was a YES! Surgery has gone well. Pleassssse make recovery go equally as well! 

Can you even imagine it? A call in the morning to say 'we may have lungs for you?' And hours later, waking up with those new lungs sustaining your life, breathing thanks to someone else. Jayne waited almost a year for this chance. One in three die waiting (please sign up, and let your family know your wishes). 

Thank you donor, and the donors family, for the most amazing gift of life. She is most definitely worth it! I think of you tonight too x 

Feeling excited, thankful, and a little shocked still! 

Autumnal news

Isaac is well. Chest. Tummy. Everything. Best start to Autumn yet I think... 

Little Rosa less so. She has just started pre-school after a life of mainly being at home or with Grandparents, so we can pretty much write off the next year for her bug wise. It will be one after the other for a while. She is such a darling, and despite being the clingyest babe ever, settling into this new world of hers has gone better than expected. 

Anouk, our gorgeous girl. If there was an award for sweetness, she would be a gold medalist. Examples being these notes and gifts left on our pillows (just one of many sweet things...)

I am really excited about how National Transplant Week went. I posted daily about this, and the buzz on my FB feed was so much greater than other years. 

We have escaped hospital for a month, and following my huge disappointment about the clinical trial, this is very good timing. I know it sounds ridiculous (as he can't join the trial as he is currently TOO WELL) but this is still bittersweet for me. It would just be so good to feel like we were fighting CF, rather than just managing it. Which this drug could do. Maybe. So the wait continues. 

Tonight I am sad. It's not my sadness to share. But a friend is going through the unimaginable. Knowing so many people now who are going through such hard times (because of CF usually) I sometimes feel a little numb to it all (I think I have to). But this is raw, and feels so so unfair. 

I hope you are having a better day. 

Clinical disappointment

So the good news. Isaac hit a 93% FEV at his last clinic appointment. This is a measurement of lung function, forced expiratory volume, that is, how much air he can blow out in one second, and is one of many ways in which doctors measure wellness in CF, and often, the most predictive. 93% was a really good day for Isaac, as in the past couple of years he has declined to, at times, 70-80% (which is not life changing in itself, but is a decline we would rather not see, at this, or any age). At times this decline is because of known infections, at others just unknown, which is worse.

The bad news is, this recent good result means that he won't now be eligible to be assessed for the Ataluren clinical trial. 

To say I am disappointed would be an understatement. I had a little cry (most unlike moi!). Clearly we're delighted at his good results. But how ironic that they come at this time, when the drug that I have been following the progress of for 5 years or more has eventually started recruiting for trials, not only in the UK, but just down the road from us, in London. 

This is not just any drug. This would be the first drug that Isaac could have taken which could, potentially, treat the underlying cause of his CF, not just the symptoms. And yes, it may not have worked. He may have even been on the placebo. And even if it had worked, would it ever be approved by NICE for the clinical setting? Would it be affordable for the NHS? I don't know. But I sure would have liked to have tried! 

CF friends will understand, Ataluren is very important to us, as Isaac has rare mutations, class 1 (supposedly the most severe) and so Ivacaftor and Lumicaftor will not help him. And gene therapy, which would work for all, by my estimations is still 8 or more years away.

I know this doesn't mean that our love affair with Ataluren is impossible, only we have to wait longer to find out. I'm just disappointed that they don't look at average FEVs rather than the most recent, or assess him in London and then decide? Sod's law his FEV will have dropped next time he is tested! 

Sorry. Rant over. Feeling disappointed. 

Merriness will resume shortly x 

Transplant week

This week is transplant week, and I will decorate my Facebook page with 'inspirational' messages enticing people to sign up. Hopefully they will be in an old fashioned font and have a sunset image in the background.

Here, I can point you to REAL LIFE stories, should you want a realistic view. Transplant is not easy, and Piper writes here about her second transplant with her usual wit and realism.

I can point you in the direction of so many more stories; those friends who have died waiting; those friends who have had a successful transplant but later died following complications or rejection; those who have lived, and lived, and loved life.

The one common theme of the stories I know is that not one of them have regretted having transplants (or regretted hoping for one).

Live life then give life (after all, what you gonna do with them?).

And please remember, more importantly than just signing up for organ donation, please tell your loved ones. Your families wishes override any donor card you carry or register you may be on. It is just one conversation, but one that could save 9 other lives.

Sign up here.

Thinking of Jayne, who is still waiting for her new lungs, and who never loses her humour. Love you funny lady.

Some summer pictures to brighten up this old place...

Life is good. 

A proper update

I feel I should update you on Isaac's health. Afterall, this is a blog about CF rather the random ramblings of my mind.... 

His tummy issues are still very much up and down. He still has pain. Which pains me. If not better soon, he will have a CT scan to look for  abnormality in his guts, for which there is a small chance of needing surgery. In the meantime, we are reintroducing the big guns for DIOS treatment, Gastrograffin, which last time (we think it was that) gave him a spectacular rash. We still hope this is a side effect rather than an allergy, and we can keep using it, as it works, and he just can't stand Movicol and the other 3 drugs they have prescribed him which are basically Movicol with a different name and in a different packet. They do that a lot with drugs. 

Chest wise he is great. We tend not to freak out about positive cough swabs these days, when we know the bugs at least. We know from experience to focus more on the symptoms than just the lab results. He is classed as 'colonised' with pseudoA (which basically means the bugs have set up camp, and plan to stay, but they may only be small camps, and it's only when they invite friends and have a party that it causes a big problem; which in CF they call an exacerbation). It's crappy that he has campers at all. But right now, he feels great. And I'm happy with great. 

We have news about the clinical trial (Ataluren) and very much hope to join (and in London, woohoo; before we were faced with travelling to further, less exciting cities!). But I must say it is a frustrating wait. We so much want to be part of something that may help him, and others. And for the first time, potentially taking a drug which targets the route cause of CF, rather than just the symptoms. It feels like a positive step, but we still feel no closer to knowing if we can join or not. 

That's about it really. There is always SOMETHING going on, but it's not all bad. Have a great day x