Powerless.

No electricity today (don't ask, let's just say, old house/over enthusiastic builder/not a trained electrician). So rescue plan by Super-Ran (love you Dad) followed by lecy and very late food; An emergency visit to Sam's kebab van. But check out how many meds Isey will need to digest that amount of kebaby fat.... makes me glad to be veggie! 

 

Good night x 

You put your hands into your head. And your smiles cover your heart.

Rosa is well again, and loving life in a leotard it seems. Day and night... 

I am excited about new travels, and so thankful for a supportive husband. 

Anouk is a beautiful soul. She is not so excited about my travels. 

I am almost crying listening to Daughter (who I can't wait to see at Latitude very soon). 

Isaac is kicking some serious chest infection butt. My soldier. 

With GiftAid we are well over £6000 for our Trekfest. Amazing! 

I am thinking of a friend in pain from loss today also. Susan, my love and thoughts are with you x 

The lovely Bonny found this and sent it to me earlier. Had forgotten all about it. Never do local news now, since they always make CF sound like a sob story rather than a life so enriched, where we dance everyday, we laugh, we have the best fun. And that's because we have the best family and friends. It's all about the people! Always. Love, love, love x 

Flying.

The wonderful Starlight Charity have been so kind as to take us flying, in both a helicopter and small plane. We can't thank them enough; as well as granting Isaac an amazing wish in 2014, they invite us for parties every year too (read here about his wish).

HUGE smiles all round....

To support the truly amazing work Starlight do for seriously ill children please click here.

Also, if you have a minute to spare, and care that desperately ill people will be denied access to precision medicine due to cost, please sign THIS petition. If it were your child/parent/loved one, you would, wouldn't you? 

Isaac continues to be pretty up and down. His tummy pain and nausea are still not great. He's been full of virus too, which never helps, and means that he needs to continue on the extra antibiotics that he's taking right now, all with the hope that the nasty little PseudoA bacteria don't set up further camps in his lungs. Despite all this, he is ever cheerful and always making us laugh. 

Have a great day all x x 

Holy fuck balls.

Apologies Mum for the swearing, but I know you share the sentiment. 

Are we ever stronger apart than together? 

European to my core. Love love love x 

I'm so far in the remain camp I just found myself coming out the other end....

...the lovely Madrid, where I have been for work (It's a hard life sometimes). I don't really understand why others wouldn't want to be part of this European Union, it's so fascinating, wonderfully multi-cultural, and beautiful. It was a fantastic trip with some very good company. I am still smiling. Leaving the EU would affect us in immeasurable ways. Interesting little article here, for example. It's not too late to vote yet people! I for one am thankful to be part of the EU and have so many friends from all over Europe whom I might not have known otherwise. 

Good night x

Unwanted visitors.

PseudoA are rearing their ugly little heads from the small camps they have set up in his lungs yet again. Command and conquer they will not. Little shits. 

Thanks to a fantastic CF team, hospital labs and pharmacy, a few emails, lots of calls from work, a fab sister in law who went in to pick up the drugs from hospital for us; tonight we have a plan, Cipro and Tobramycin (to mix it up from the Colomycin nebs which the bugs seem to be escaping right now) plus a new regime for his tummy to try, involving emergency Gastrograffin and more Picolax.... All without even seeing a doctor. This is modern medicine people! 

All in a day's work. Fingers crossed it works x 

Pictures of the summit, the Brecon Beacons.

Trekfest.

We made it. It's been emotional. The bottom line is; I have such amazing friends.... 

In no particular order, huge thank you's to my sister Jo, who always joins me on these mad adventures and more, and Jo Elliot who walked through the pain to make it with her. Lemon and Jess who completed the 25km in an amazing time. Bon, who couldn't walk this time but has before, and was the glue that held us together. The gorgeous Sarah Cater Skater who walked over the finishing line with me despite the worst blisters all over the soles of her feet. Jane who kept the conversation going for those last few painful hours and never complained. Erika who also climbed the Yorkshire 3 Peaks with Jo and I previously and whose feet I couldn't even look at the next day for cringing, and her friends Tammy and Sarah who also finished the 50km. Tammy, was so good to meet you. Sarah, sorry we never caught you up! 

We finished the 50km at 10pm after 12.5 hours of walking. The climb to the summit was incredibly hard, as was the steep climb down on our feet, but the views were amazing (sorry the English and Scots, but Wales wins in my opinion!) and the company even better. The weather was very kind to us; 18 and cloudy on the day of the walk, and hot and sunny for our day of celebrations the next day. Little Anouk joined us for the weekend which made a lovely change. She misses me terribly when I'm away with Isaac, so was a treat for her, and me. 

I thought a lot about Jayne up there. Tim and Adam, if you are reading, love love love. 

Isaac was ill most of the weekend too, a timely reminder of why we were doing this. I am glad to be home with him now, my soldier. He goes through so much and complains so little (how many of us can say the same?). 

And so I feel happy, and sad, and proud, and lucky, and tired... And hopeful. We have now raised over £52,000 for the CF Trust since Isaac was born thanks to this and other things we and our wider family and friends have taken on. This can and will make a difference. I have to believe that. 

Thank you for your support. For our amazing family and friends who donate again and again, it means so much to us. You mean so much to us. 

Here are some pictures from our weekend x 

I will never stack a dishwasher in the same way again (at least not without laughing). I'm still waking up in the night snorting with giggles! 

PS; still not too late to sponsor us, see link below in blog x 

BLISTEROILS!!!! 

This kind of shnizzle

In three days time a group of lovely ladies will be walking 50km over the Brecon Beacons with me in aid of CF. I'd love to come back to find that we have made our target. To support us you can sponsor us here. We're so nearly there. Thank you so much for the support so far.

Some half termly observations;

'Do as I say, not as I do' is not an effective parenting option.
Feisty three years old can be even harder than teens to reason with. Who knew?
Indoor surfing is a thing. And it's blimin fantastic!

All children at the age of eleven inexplicably come into possession of a machine-scatter-gun with which they load dirty clothes, wrappers and football cards, sit on a bucking bronco and fire repeatedly around their bedroom. 

People actually have business meetings in which they say things like 'conversate' and 'getting ducks in a row' in a non ironic way.  

The mountainous range we will be covering on Saturday is over 3500ft and includes 'Fanny Big'. I kid you not 😉

Good night all x 

Back from the school trip.

I wanted to add some pictures from our week away at Isaac's school camp at PGL (parents get lost) but due to his pre-teenyness the only face he wears for pictures right now make him look like he's smelling an especially unpleasant fart. But he had a great time, and stayed well throughout. A week of climbing walls, kayaking. quad biking and the like... I stayed mostly in the background, so as not to cramp his style (the only kid with a parent in tow) so used the time to work/walk/read and sleep more. Missed my girls terribly. We're both glad to be home.

Less than two weeks to go until our 50km, sponsorship is adding up, over half way to our target with gift aid. It's my birthday in a couple of days too, and I'm asking for donations towards our target if anyone would like to be so kind, click here. Training going well.

Have a great week peeps x

All good fun

Things are going really well right now. I have started an exciting new job (secondment from my normal one, not a new place) which is just the thing I needed - a fresh challenge to get my mind off other things. Working with some really great people, which makes going in every day interesting. 

I'm thinking of starting a sideline blog on cooking. It's a kind of antithesis of cooking blogs; more about just how crappy my food choices can become, a kind of 'how low can we go' of meal trackers... Dan and the kids eat without me the days I work because I get back so late, and as they eat meat and I don't, it's often easier if he feeds them, and I feed me. It's not that I eat BADLY, it's just that I don't care that much, so just eat whatever is first out of the fridge. Last night it was a bag of spinach, steamed (microwaved in its bag) with a tin of baked beans.... I love my food, I just don't really mind what food it is. Fortunately for me Dan was once a chef, so I get a bit more variety on other days. But I wonder if such a blog might attract other cuilnary deliquants? Perhaps there is a whole raft of stressed out workers/lazy cooks like me who eat for hunger rather than pleasure? This is not a diet, it's lack of time and inclination! 

Isaac is doing fab! Sick only once this week. Next week he has his first ever school residential camp (5 days, lots of different activities, staying in dorms). Poor kid has to put up with me tagging along though, as he needs me there to do his meds and nebs... But I plan to stay very much in the background and will use the time to train more (did I mention that I'm doing a 50k trek over the Brecon Beacons? Well, you can sponsor me here if you could be so kind!) and read. God I miss reading more (I read loads, reading/music/art are my things, but just not as many books as I'd like, I make do with devouring newspapers and all the supplements). 

Girls are great too. Anouk is the best company these days. She is still at an age where she actually wants to hang out with me still, and is my dog walking partner a lot of the time. She loves her art and list writing, an apple not so far from this tree. Rosa has really come out of her shell and is beginning to enjoy life outside of home/grandparents. She was THE MOST CLINGY BABY EVER, she struggled to even make eye contact with people once, but the other day I managed to go order coffee in a playbarn leaving her chatting to a friend of mine she doesn't know well. That is huge progress for her! She so cute, my feisty little monkey. 

Today I met someone I have wanted to meet for a long time, a friend of Jayne's too, who also has CF. I feel like I have met a kindred spirit, of the blackest humour. We had the loveliest walk and chat, I'm so so glad we made the time, sad though it was that it was without Jayne. 

That's us really. Feeling pretty positive and excited about our summer to come. I hope this good cheer spreads to you, lovely people, whoever you are. Seize each day x 

Fun running for fundraising...

Pre-teen with the obligatory 'moody face for all photos despite hidden joy' look. They did great! 

Big love to Jo, Ben and Ellie too for doing so well! Very proud mum/sister/auntie moment x 

All running towards our sponsorship target, which you can support here. Thank you so much for all the support so far! Love love love x

Annual review letter.

So this is the dreaded and yet eagerly awaited letter with all of his results from the many tests he had last month. Not a huge amount of surprises... Mostly about his tummy issues, low immunoglobulins blah blah, things we are only too aware of. As I do every year, at our following clinic appointment I'll ask a million questions about the many results (honestly, the blood work alone is 2 pages long). 

Anyway, one thing that did stand out was in the Psychologists review, who wrote 'Isaac's responses to the survey show signs that his CF causes him emotional distress'. 

Now fortunately for us, Isaac is the most chilled out dude ever, who tends to worry very little. Yeah, we often agree that CF sucks at times, and how it feels unfair. But he's never really been sad about it, just frustrated. But worried that I may have underestimated his feelings I asked him about the survey tonight; 

Me; So Ise, you know that survey you did in clinic last time, did it ask about how you feel about CF honey? (Giving him a loving hug) 

Him; oh yeah.... but I mostly just ticked any old thing (followed by roars of laughter and him pulling his underpants up to his chest giving himself a huge wedgie...). Ha ha, I'm dad, I'll give you EMOTIONAL DISTRESS (more laughter). 

No worries on that front just yet. And while he is happy, so am I. 

Goodnight x 

PS; my auntie Margaret's kindness in life has raised more than £850 for the CF Trust in her passing, in lieu of flowers. Ian, Helen & Sian, love love love x 

CF doesn't need to be so lonely...

Video to watch here.

CF friends, parents, partners; please stay in touch. And for those I have yet to meet, please contact me through FaceBook or similar.

Much love x

Spent the evening reading about cocktails

Unfortunately it was bacteriophage cocktails to treat Pseudo aeruginosa infections in CF where the existence of biofilms and multi-drug resistant bacteria are increasingly problematic. 

Science is so sexy. 

Goodnight all. We have lots to hope for thanks to all those sexy scientists x 

Trek on

Its taken a while, but we're all feeling better. Although Isaac continues to never quite make it through a whole week at school.... the anti sickness meds are fantastic, but only if we get them into him as soon as he starts to feel nauseous. If we're too late, once he starts, he can't stop. And going to school having just been sick everywhere and its going to take a while for your tummy to calm down is not always possible. ANYHOW... It's much better than it has been, so can't complain. He doesn't.

We are training hard now for the trek, less than 7 weeks to go (please please sponsor us here, this will be 12+ hours of hard slog!). I managed to get my laces tangled together and totally face planted last week, hurting my shoulder and hand. But while my legs are still going, so shall I! Feeling quite excited now, our team is now 10 lovely ladies (THANK YOU ALL!). 

This week my thoughts are with my uncle and cousins who have just lost my aunt so sadly, suddenly and unexpectedly. Despite their tragedy, they have thought of the CF Trust and are asking for donations rather than flowers. So very very kind. 

The CF trust are doing such important work in research, support and awareness. And how lucky are we to live in a time where cause-treating drugs are a real possibility that could one day make CF stand for Cure Found? Oh, and did I mention, you can sponsor us here?😜

For Margaret, Edgar, Tod, Penny, Jayne, Anders, Toria, Emily and Eva... Party on up there. Miss you x 

War on bugs

I have decided not to talk about feeling ill as it makes things sound so glum (which they are generally not, we have a lot of fun in this house). I hate glum. Instead we will discuss health in terms of wellness;  

 

Rosa is less well. Poor pudding. She has had 3 weeks of fevers, a water infection, coughing, cold. Small people are simply bio-hazards that wonder round your house firing germs at every available surface.

Dan is not so well. He has had a couple of weeks of fevers and headaches (although admittedly, he has also gone cold turkey off the Tramadol, which he has been on for some time for his back, so not sure if its viral or withdrawal). 

Anouk is getting better, my little lovely. She is so caring and helpful, I just want to squish her with cuddles most of the time.

And I am getting better too …. just have that ending cough which drags on.

…. But Isaac is most definitely not quite really properly well at all. Saving the best for last, he has fevers (40+), vomiting (his speciality), headaches and dizziness (almost hitting walls and the like), and was hilariously delirious last night (apparently we 'lost' the whole house). So we will throw an extra antibiotic at him to limit the risk of secondary infection and hope that does the trick.

 

If you’re anything like me, pre-CF, you might not be very familiar with the difference between viral and bacterial infections. I explain them to the kids in terms of size… viruses are teeny tiny little buggers who squeeze their way into poor unsuspecting cells. Small, nimble, but tough... antibiotics will do nothing for a virus. But thanks to our amazing immune systems (which Isaac and I refer to as his white blood cell army), you can only be infected with the same virus once. This is almost definitely what Isaac has right now, and is what most coughs and colds you ever have will be. 

Bacteria are big fat oafs in comparison, slower to grow, but cunning. They like to roll up after a virus has caused havoc in our bodies, or post op, and seize the opportunity to set up camp. Now this is where antibiotics are our saviour. Bacterial infections are nasty, but thanks to the handful of antibiotics we have readily at our disposal, we can beat most of them. 

But one day this might not be the case…. Because of widespread misuse of antibiotics, particularly in the East where you can buy them over the counter for a cold or a stubbed toe (which they cannot treat!), we are creating our own worst enemy - super bugs. Bacteria that we almost kill with a few days of antibiotic treatment (but not quite), will re-group, recover, mutate, and infect again, and again, and again. Simply by surviving the onslaught of antibiotics, they are developing more clever ways to overcome the most sophisticated and advanced antibiotics. Some of the most resistant infections are caused by Gram-negative Acinetobacter, and by certain strains of Klebsiella and Pseudomonas species - all of which Isaac has cultured in the past at some point. And while infectious agents are becoming more and more resistant to the medicines that are currently in use, not enough drugs are being developed to combat them.

People often ask me, if Isaac is on maintenance doses of a few different antibiotics all the time, will he become immune to them? But it’s not people who become immune, it’s the bacteria. And if we go on creating super bugs, and not-investing enough into research to find new antibiotics, this could be the next world war we all face… And that is the kind of shit that keeps me up at night.

 

People with CF are no more susceptible to viruses than people with healthy lungs, but they are much more likely to develop bacterial infections following them, which is why he needs daily antibiotics, both by tablet, and inhaled into his lungs, and occasionally by IV (intravenous, as in, into his blood) to prevent them setting up camp, or flaring up where they already have set up camp. This is also why we ask people to avoid him if they have a cough themselves, and why I wish more people would get a flu jab; to help limit the risk not just to themselves, but for those less well around them. 

So why is there such a shortage of new antibiotics being developed? One is scientific; the whole low-hanging fruit has been picked kind of thing. It’s not easy, I get that. But another big reason I fear is commercial. Antibiotics have a poor return on investment because they are taken for a short period of time and cure their target disease. In contrast, drugs that treat chronic illness, such as high blood pressure, are taken daily for the rest of a patient’s life. The solution may lie not only in scientific discovery but also in the economic incentives for developing drugs. Separating research and development costs from drug pricing and the return that drug companies receive on investment could turn this problem around. 

I believe the rewards should be higher for those drugs developed which make the most difference; in other words, what is more important? A small difference to many people, or a huge, life saving chance for fewer? 

This year the World Health Organization is devoting World Health Day on 7 April to raising awareness around the issue of antimicrobial resistance. More information is available at: http:/www.who.int/world-health-day

PS: On a lighter note, Isaac called me into the room the other day to ask me ‘Mum, did you know there was a man called Marvin GAYE?’. Snigger snigger. Not growing up so fast then. 

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.