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 

Fleeting thoughts

Yesterday, my Mum and Stepdad (have I told you that they look after the kids the days I work? And how wonderfully amazing they are?) decided to take all three kids (it's school holidays) to the beach for the day. Dropping them off, they all stood waving goodbye to me from the drive way, and I froze for a moment. This terrible thought danced cruelly across my mind - what if they never came home? 

Immediately I craved for the softness of Rosa's skin on my cheek, and Anouk's kisses, and Isaac's hugs.... and for that horrible fleeting moment, just a second, my mind allowed me to imagine a life without them... No life at all. I spent the day worrying, calling, and asking a lovely friend of mine if she believed in omens (thank you for making me feel better Gem). 

This is not like me at all. Really. I'm so glass full usually, I'm overflowing. And of course they all had a lovely day in the sun, swam in the sea, enjoyed their picnic, and drove very safely home again. 

There is nothing like those fleeting moments to remind you of how lucky you are, and just how wonderful things really are. We have these three beautiful souls to share our lives with, and a supportive and loving family around us. We are the lucky ones. 

Feeling better

It's been a bit up and down of late. But he IS feeling better. And I am feeling much more positive again. He did not have tonsillitis, but he is growing pseudoA again (nasty little CF bug) which explains the cough. He is improving again on some extra oral antibiotics (for now). Also, we hope to hear more news on the Ataluren clinical trial this week, which I'm excited about. More hospital next week. Hoping the DIOS problems of late are behind us (but not completely convinced). 

More soon. And in the meantime, have a lovely summer, and please do add comments/send me questions if you want. I love to hear from everyone x 

If you have nothing nice to say, don't say anything at all.

My mum was always full of sound advice. And that's kind of how I've been feeling this week. Not really wanting to update until I had something positive to say.

But....  Isaac is still not well. 

During the Gastrograffin explosives of last weekend, he came out with a rash, all over. Our best guess at the moment is that it was due to the new medicine (we hope side effect, rather than allergic reaction, as we'd like to be able to keep this drug in our arsenal for future use). He's also been tested for other possibilities, and we await blood test results for those, none of which would be good news. 

His tummy is much better (lots of other extra drugs to help avoid more DIOS) but the best thing about the last few weeks is that his chest has been great (and sorry guts, but you are a poor distant relation to those regal organs that we call lungs. In other words; Lung health is the booyaka of CF). 

But now he is coughing. Really coughing. And, I can't quite believe this, but it very much looks today like he has tonsillitis again. That in itself is not strange, but the fact that he has had tonsillitis for the past two years at exactly the same time (as in, he breaks up from school, we plan to go away, and boom, he gets tonsillitis, which in turn gives him a crappy cough and we end up in for more IV's). I so don't want the same summer again! Please let me be wrong. Is tonsillitis even seasonal? 

Some happy news. Spent today at a party that Starlight throw every year (read here about our  experience with Starlight, an amazing charity who makes dreams for kids living with illness come true). Was so much fun. Isaac threw up in the car on the way down, coughing too hard, and struggled all day, but still wanted to stay and even played a little football. The girls got the most amazing face paint, and had a ball. 

Tomorrow we were due to go camping for a week, with some of our bestest friends. But after months of good weather, the forecast for the next week is just terrible, so we're not sure what to do. It makes me all the more sad for the kids, as the last few weeks has been difficult enough, for all of them in different ways. And we can't afford to do anything but camp right now. Isaac may not be well enough to anyway (back to the Dr tomorrow), so it may be beside the point, but the saying it never rains but it pours seems annoyingly apt right now. 

I know deep down that none of the above is insurmountable. We can and will cope. He will be OK. Things will get better. But also, I must admit I'm struggling. Missing a lot of work (so spending evenings catching up), feeling worried all the time. It just feels like the breaks in between the problems are getting fewer and far between. 

Merriness will resume shortly, I'm sure x 

ps; People have asked about the clinical trials that I spoke about previously; no news yet. But still hopeful for something to happen this year, it's a question of where right now. It looks likely that we will have to travel outside of Cambridge, and unfortunately not in London either. 

Back in....

Isaac illustrates what his medicine tastes like; 

And how he keeps on smiling anyway; 

And some happy pictures from last weekend; 

Home again soon (we hope) x 

DIOS... Again. Back in hospital? Maybe.

Isaac has this (sorry for the wiki quoting, but kind of sick of people comparing this to normal constipation. It is not!)

Distal intestinal obstruction syndrome (DIOS) involves blockage of the intestines by thickenedstool and occurs in individuals with cystic fibrosis. DIOS was previously known as meconium ileus equivalent, a name which highlights its similarity to the intestinal obstruction seen in newborn infants with cystic fibrosis. DIOS tends to occur in older individuals with pancreatic insufficiency. Individuals with DIOS may be predisposed to constipation. Features; Symptoms of DIOS include crampy abdominal pain, vomiting, and a palpable mass in the abdomen. X-rays of the abdomen may reveal stool in the colon and air-fluid levels in the small bowel. Management; Surgery is often required to relieve the obstruction. However, if there is no sign of bowel rupture, a more conservative approach may be attempted. Restricting oral intake, placement of a nasogastric tube for decompression of the stomach and proximal intestines, and administration of laxatives and enemas may resolve the obstruction without the need for surgery. Individuals prone to DIOS tend to be at risk for repeated episodes and often require maintenance therapy with pancreatic enzyme replacement and stool softeners and administration of laxatives and enemas may resolve the obstruction without the need for surgery. Individuals prone to DIOS tend to be at risk for repeated episodes and often require maintenance therapy with pancreatic enzyme replacement and stool softeners.

There were no beds on the ward, so we're home, with some meds, but may be back on the ward tomorrow if he is no better/they have room for us. 

Need to add, we have the most wonderful CF team. Especially our fantastic CF nurse Caroline, who we caught on her way home, having just been told that the pharmacy could not prescribe us the drugs he needed. She went out of her way and spent the best part of an hour sorting us out, as everyone else went home. We can't thank them all enough. 

Thanks also to Jin, for listening to me moaning today, and my work colleagues who didn't mind Isaac sitting in the office for a bit while we waited for his doctors to call (that's a first!). 

After that I reversed into a large post and scratched all the back of the car. 

And worse, the best Mum in the world is also in hospital overnight with a bad knee, and I can't even go and help her as I need to be here. 

Today can just piss off (sorry for the language Mum) x 

New lungs new life

Hate this paper, but This article is pretty good. Check out the GOSH documentary too (Great Ormond Street: Fight To Breathe will be broadcast on BBC2 at 9pm on Tuesday, July 21). 

Pretty hard to imagine that the only difference between Isaac and this gorgeous, brave and courageous boy is one year and a really horrible infection. 

Thankfully new lungs were found for Charlie. Please consider signing up, which you can do Here. 

Have a great day. 

CF fame

 

I often feel a bit mixed after CF is in the news or on TV. It's either portrayed in drama as a 'terminal illness' where sufferers are pale, poor skinny souls without a hope. Or in the news as the NEXT BIG BREAK THROUGH disease. A cure is imminent! We can all throw out the nebulisers and look back on these days and laugh at the hours of physio endured! 

 

The truth is, it is mostly neither.

 

Gene therapy is ground-breaking science, it really is. And like others in the CF community, I am hopeful that one day this may be a therapeutic treatment for cystic fibrosis. But I also believe we are many years away from this, and after the science, the funding will also delay this further. I'm also not hugely excited about a 3% increase in lung function. Isaac's can vary by 15% from one clinic visit to next. 3% and lung function stable? That would be, promising, but not exactly epic (as Ise would say), is it? I believe this is only the beginning of the long story of gene therapy. It is progress, and I am hopeful, but I'm not yet excited. And people saying to me how fabulous this is doesn't help much - I must look a bit deflated when I am less enthusiastic than they are. Sorry guys. But we've been here before. 

Gene therapy was the buzz phrase when Isaac was a baby. We asked our consultant about it then, and she looked kind of flat in the same way. She surmised it might only work well in healthy lungs, not already destroyed by infection, inflammation and scarring. We asked at what age Isaac might have signs of this, and she replied age 5, maybe. When he way a baby, 2013 was talked about as a time when we might hope gene therapy drugs to be in the clinical setting. 2015 and we're still many years away. And yes, he probably does have all these signs already, but the trial showed positive results for those less well, so it is far from hopeless even though the wait continues.

 

A consultant once told us that they did not believe in a cure for CF. Which sounds like a crushing blow, but I think I agree. Rather, there will be many new therapies (small molecule drugs, new antibiotics….) that put together will hold off decline.

 

So great, the gene therapy results have raised the profile of CF (which is always good, helps raise funds, as well as awareness) and people are excited for us. Thank you. I'm just not sure that Press publicity accurately reflects what we should actually be excited about. Other trials results are a lot less sexy, but no less important. 

 

In other Moly news;

 

One puppy, one less chicken. Oops.

Fibrogel is the evil cousin of Movicol - can anyone really drink that stuff? Back to the Movicol battles.

Summer holidays are fast approaching, and the Moly's will be going camping soon. Obie's first trip. We may be home sooner than we think.

My baby is 3! And starting pre-school soon - where did all the time go?

Isaac won most improved player of the year for his football team, and Anouk lost her first tooth and has her first horse show soon. Not sure which she is most excited about.

 

Have a great day.

Home

We are home. 

Happy Father's Day men folk x 

Hospital survival guide

While we are here, and it's fresh in my mind, here is my official hospital survival guide, should you ever be so unlucky to need it; 

Bring flip flops or slippers. Wearing shoes on the ward feels, just wrong. Pyjamas and shoes? Not a good look, and you don't want to be venturing in toilets bare foot. 

Take your own pillow/blanket. Home comforts to make things feel a lot less cold and sterile. Plus hospital pillows are plastic coated, the crinkley sound as you roll over just reminds you of the thousands of other people who have once dribbled on them too. 

Books, magazines, games, cards. I may remind you here that every hour in the real world, is three hours in hospitaland. You will need entertainment. And at least here at Addenbrookes, the kids wards still have no Wifi. *** note to NHS, this is probably the single biggest thing you could do to improve the hospital experience for kids and parents alike *** No need for toys, the playrooms are great. 

Bring money. We rejoiced when M&S food opened on our concourse, but between that and Costa hot chocolates (complete with cream and marshmallow) it is haemoraging our bank balance. Not to mention the parking and TV/films (... £10 for 24 hours). 

CF families, I suggest you bring all your own medicines from home, everything. You know all the doses, routines, cleaning and preparation required, and just explaining that to the nurses would take as long as doing it. As fantastic as nurses are, if they do all the normal drugs as well, everything will be late, in the wrong order, or somehow, just-not-quite-right. Plus, as parents on the ward, we have jobs too. The nurses have enough to do. 

Invite visitors. Make sure you ask for help, most people (I hope) are happy to be asked. Grown up company for you, but the kids want kids, so ask their best friends/cousins. 

*** Note to visitors *** No need to bring anything, just come ready to play, he wants to be distracted and Mum wants a break from getting her butt kicked at Mario Kart. But don't stay too late - no one sleeps well on the ward, so we need our early nights. Also, best time to come is early evening, the days are mostly filled with doing or waiting for the next treatment and visits from numerous doctors and what feels like hundreds of medical students. If you come in the day, you risk not finding us as we're out at X-ray, lung function, ultrasound..... 

Treat yourself to nice pyjamas. Almost worth having a 'Sunday best' pair just for coming in, if you come in as often as we do. The consultant that you see all the time in clinic is soon going to see you in your nightwear. Would feel a bit odd in your old grey granny pants and your husbands old t-shirt. 

Use the parents kitchen. I used to live on sandwiches and rubbish when Isaac was little on the ward. Now we have M&S I have microwave spinach and ricotta cannelloni, or have soups and salads. Much more fulfilling. Also lots and lots of tea. Helps pass the time. Tea helps everything. 

And a word of warning. Prepare to manage in hospital, but have a meltdown the day after you get home. Happens to me every time. You hold it together when you need to, but it's got to come out sometime. 

Back in hospital

We are in again. This time on the baby ward, not because he has shrunk, but as that was the only ward with a cubicle available. He needs privacy as this time it is tummy related.... CF friends will understand. DIOS hell.

I am feeling tearful as we spent a lot of our first year here together. Many memories of C3.

Hoping this one will be a briefer visit. More soon x 

Third time....

Jayne has had two calls now. You can read about the second Here.

This is no bog standard, call from your Mum, or the local neighbourhood watch to tell you about the latest stolen hanging basket. This is, we may have new lungs for you, so you can like, BREATHE, and be well, and get one with your life with your lovely family.

Can you imagine? Each time, it's been a no-go. This time, the lungs were no good (but thank you donor for at least offering).

Having known others on the waiting list for new lungs, this is; to-be-expected-but-still-bloody-annoying. I know people who have moaned more when their dentist has cancelled an appointment. I know people with CF who have had almost a dozen calls like this before getting new lungs. 

I'll give the lovely, funny, wonderful Jayne a little twist on a saying she will almost definitely be hearing right now; 'third time fucky?'. 

Love you funny lady. It will happen. I can feel it in me creaky old bones x x x 

Our roundabout.

Our week.

1; Wakes up with tummy ache. Pain. Folded over. Vomits. Eats. Feels better. School. 

2; Wakes up with tummy ache. On the loo all morning. Can't eat. Braves school. 

3; Wakes up with tummy ache. I vow to call dietician. Doctor. Start the dreaded 'food diary'. Is it not enough Creon. Too much Creon? Looks pale and sad walking into school. 

4; Wakes up, feels fine! Panic over. 

5; Wakes up, tummy distended. Movicol, Movicol, Movicol. DIOS again? Can't leave toilet. No school. 

6; Wakes up, tummy ache. Vomits. Bug at school. Is it tummy bug? Or side effect of new meds? Or not enough Creon? Too much Creon? Last nights curry? 

7; Wakes up, feels fine! Panic over. 

And repeat. 

Feeling helpless. 

Super bugs are taking over the world.

Or, to put in a slightly less hysteric manner, multi-drug-resistant organisms are spreading — like klebsiella (a bug Isaac was chronically infected with when he was younger for some years) or MRSA, the multi resistant form of staphylococcus aureus, which commonly affects CF lungs. Even infections that used to be easy peasy to treat, like gonorrhoea, are becoming incurable. Tuberculosis alone kills 1.4 million people a year. One reason for this crazily high number is that most people in the world are too poor to pay for medicines, but another is that strains have become resistant to the array of drugs we have. Even after two years of toxic treatment, drug-resistant tuberculosis has a fatality rate of about 50 percent...

Add to this the ease of access, in some parts of the world, to buy antibiotics over the counter – which people are over using, and not finishing courses, which only helps breed super bugs. I saw an experiment once where they spread bacteria on an agar type table, and added the correct amount of antibiotic, which killed the bacteria. Take the same table and add a little antibiotic, but not quite enough, and the bacteria not only overcame the antibiotic, but it mutated and got all the way over the table and had a massive super bug-party. 

Asking your doctor for antibiotics when you don’t really need them is adding to this problem. As is not quite finishing the course she gives you when you do need them…

Old antibiotics are being phased out at twice the rate that new ones are being developed. We are losing the most important kind of medicine ever discovered, and why?

Duh duh dah… MONEY SCHMONEY! Historically, the drug industry loved antibiotics. But if an antibiotic is useful against only one type of bug then relatively few people need it during its patents life (patents run for 10 years from invention, I think?). And if an antibiotic is “broad spectrum,” meaning it works on many different types of bacteria, wider use shortens its life because resistances develop faster. Also antibiotics are designed to cure an acute disease, so only used for short periods. So not a great money spinner compared with drugs that are used for years and years to treat widespread conditions like asthma. 

But CF is one exception…. People with CF are on daily antibiotics, usually numerous ones (oral, nebs and IV’s). It’s the single biggest thing in our extensive arsenal to keep Isaac well. 

The other problem is how blimin CLEVER bacteria are. Alone they are pretty feeble bunch, but collectively they gain intelligence. They learn together how to survive antibiotics. And the more they work out how to survive, the harder it is to find drugs that can beat them.

So how do we get drug makers to want to try? Addressing these two problems might be a start… 

Monopoly protects the ability to set price for profit. Patents allow monopoly – can they not allow longer patents for new antibiotics for a start? 

Like bacteria, who share what they learn and get smart, could drug makers operate in the same way: academic and government scientists and other drug companies working together to share what they learn… collaborate and innovate? I know this is happening in a small way already, thanks to philanthropists, which shows that even competing research teams can share knowledge, risk and avoid costly redundant efforts. 

Or maybe rewarding drug makers for products in proportion to their IMPACT? The lower the cost of a lifesaving antibiotic, the greater the number of people who could use it; the more lives protected, then, the greater the reward. If common infections start to kill again, due to super bugs, then surely the impact of a new antibiotic would be huge? This is already happening people! 

If we don’t create new antibiotics, the super bugs will win. They were around before us, and they will be around after us. Little shits.