Happy Halloween.

Hello. We are all coughing. Nothing new for autumn, in a house where we are systematically exposed to walking, spluttering, gunk dribbling bio hazards (small children).

Isaac is due back in clinic (just 2 weeks after the last one) due to his drop in lung function (LF). I don't imagine we'll see an improvement yet, despite the extra antibiotics, but my hope is this is viral rather than his pseudo infection (further reading here, but it might put you off hot tubs for life) rearing its ugly spiteful head any further. Still, I am so thankful that he made a whole half term at the start of Secondary School without a full day off, can't really complain. He is settled and happy, and that means the world to me. 

My exciting read tonight was this article which is music to my ears. I imagine (quite possibly incorrectly) a biofilm to be like a slimy film around something (like a slippy seaweed around a rock in the ocean) which sounds quite inpenitrable, due to its oozyness, protecting its evil bacterial friends. So let's kill kill kill!!  

Please also watch this if you have two minutes, and sign up here if you have one more. 

Have a great day x

This blog is about Cystic Fibrosis. (Yep, that's the chest infection one where it's legit to bash your kids on the back. People always ask me that).

So it's been a CF kind of week. It's not always like this, despite how this blog must sound. Maybe it's a bit like reading a Jeremy Clarkson column in the Times every week..... You'd be forgiven for thinking that his only mode is angry ranting.... Actually, that might not be the best analogy, I think he probably is just an angry prick. Anyhow.... our lives are NOT all about CF - but this blog is, hence the dwelling on the subject somewhat. But fear not, we laugh, we dance, we sing (poorly), and mostly CF is very much the annoyance in the corner, like an unwanted house-mouse knawing through your cereal (we get those too). But this week I've had a CF parents evening, followed by CF Clinic. The headlines are; 

The Ataluren Trials continue. You may remember me blogging here about this drug trial previously. I was heartbroken when we couldn't join the clinical trial because of one very good lung function result, meaning he was TOO WELL to participate (as predicted, never repeated, drrrr!!!). I've been watching the progress of this drug for many years, as it's the only small molecule drug in promising trials that would help Isaac, as he has two rare (class 1) mutations (Classes of mutations explained better here). The results so far have not been outstanding, but as these drugs treat the underlying cause of CF (rather than its symptoms) the full efficacy may not be fully known for many years. The other funny thing about Ataluren (as opposed to the more well known small molecule drugs Kalydeco and Orkambi, which will treat the majority of people with CF with more common gene mutations) is that this drug is suitable for other genetic diseases, specifically for 'Stop (or nonsense) Mutations', which includes Duchennes Muscular Dystrophy. Ataluren has recently been approved for use in DMD for children up to the point where they are wheel chair bound. I have no idea why treatment stops there. This is a limited time (as I understand it) and the cost is circa £220K per annum, per patient. Yep. Really. The question is, if approved for use in CF, would the cost be lowered as the time frame would be longer for each patient? The problem is always the same; 

The investment cost for the pharmaceutical company + the small number of patients who require the treatment = a high cost drug. 

Unless the number of patients is very very small, and the efficacy high, in which case the overall cost is manageable. I wonder if the problem with CF is that it's pretty rare (1 in 2500 live births, 10K pop in the UK) but not so rare that it doesn't add up for the big pharma's. Orkambi was recently refused by NICE at a cost of £104K pp pa. Heartbreaking for some who have high hopes for this drug. 

I did though catch up with some other lovely CF parents, and our amazing team (can't tell you how much I love the Addenbrookes CF Team) and there was good news about the Adult CF Centre Isaac will attend. We've known since he was small that Papworth hospital (local adult CF centre) would eventually move to the Addenbrookes site, which is perfect for us; access to everything he might need on one site, which we live 20 minutes from (how lucky are we?!)  and we have a date now; Spring 2018. His transition to adult care will be from 14-16 years old, so not that far off, and the new purpose built CF Ward will have private rooms, ensuites, and fitness facilities in each room. If you read this blog or know CF at all, you'll know why all of those are so important, these are not perks, but essentials due to cross infection, and exercise being so important to lung health. 

Then clinic... Lung function was down, again, so we're starting some more antibiotics (oral) to try and set him up for a better winter. His chest is not yet bothering him, so good to do this now before it does.  

Tummy wise, we are trialling him off one of his 5 tummy related drugs to see what difference this makes. He is still not right, but we're talking about many, sometimes conflicting, factors here; DIOS (blockages), side effects for all the drugs he takes for this, and his pancreatic enzymes (Creon, or specifically, lack of). Knowing where one starts and another ends is pretty hard to figure out! 

He is learning the hard way about not taking his Creon properly (we suspect he has been skipping it at school, as he dislikes taking them in front of people) and will try out a new Creon dose which means instead of taking 10 capsules, he can take 4 larger ones, which he can do more discreetly and see if that helps. 

It's been a long day, but we got to see one of our favourite nurses before she retired. Thank you Fiona.... We will miss your friendly face more than you can know. 

Clinic is a weirdly bonding time for Isaac and I. We always make it fun and have some pretty deep chats (usually followed by pranks and giggles). He then came home and threw up all over the rug. It's hard not to feel that it's a bit bloody unfair at times, he deserves a break, surely? But he doesn't complain, and I couldn't love him more. 

We had letters from school about forthcoming school trips he can go on, which for us create two issues, one being the cost (£1000 together, EEEEK!) and the other being, can/could he start to manage his own treatments for a week to travel without us? I have to see the school regarding travel insurance too. We refuse to let CF hold him back in any way, so we have to make this work somehow. 

Have a great day x 

Updated Friday to say; Today we celebrated because I found out I have passed some recent exams (Professional qualifications)! Took the kids out to eat and be merry. Isaac is tired but feeling better 😊

All the pretty things you see outside.

Yesterday was Jayne's birthday. I can't explain how much I miss her. But I do.

Isaac was sick again today. It seems to come in phases. I hope I'm wrong. 

Another young CF friend is back in ITU, as if she hasn't already been through enough already (two double lung transplants not enough?).  

A very kind friend of mine is raising money for CF, having met Isaac and I, by riding over 200 miles (from Scarborough to Norwich).
There is much sadness in the world, but also, so much kindness. To support the Cystic Fibrosis Trust who do so much amazing work in research and support for those affected by CF, please follow this link. Thank you so much James!

Listening to this (lovely tune), and remembering all that....

"Don’t talk to me
Just walk to me

I’m distant

Sure I’m your man

Do what I can

But don’t stand by my side

Stand inside

That’s my good girl

My whole world

Turning on the couch

Close that cute mouth

And kiss me

Like all the pretty things you see outside

Imma gonna make you satisfied, and

All the silly things inside my mind

Imma gonna make you satisfied

Imma gonna pick on my guitar

Baby 'til we feel it, future [?]

We gonna live in a house together

With me on the couch and my guitar, singing

“Oh my god I love you, I love you”"

The sadness that surrounds us.

I must admit the last few days have knocked me a little. Lots of sad news. Young lives lost. Other CF friends struggling. Isaac coughing again. Clinic next week. A favourite nurse leaving (it's things like this that make a real difference).

This blog is not about Me. It's about CF, and all that goes with it. But I am so affected by all of that, it's hard to separate the two. To leave the 'Me' out of this. 

It's been a hell of a summer; Lots of highlights (France, New York, our fabulous new family caravan in Dunwich, Isaac and Rosa starting new schools, the arrival of fab family babies) and other challenges (new role at work, training and 4 exams to hurry through qualification to keep said job). Put it this way, the house has never been messier. But it's all been doable (just) because Isaac has been so well this summer, until now. For which I feel so bloody fortunate. He has had the great start to Secondary school that we hoped for. Like any other kid. Which is So Important. 

It's also all too easy to dwell on the worries; like this about the risk nuts may pose to people with CF.  I mean, hell, there's not much to worry about already is there? It used to just be jacuzzi's, rotten onions, compost, stagnant water, other people with CF, greenhouses, inadequately chlorinated pools, rotten coughing people.... The list goes on. It's crazy making stuff for CF parents.

 .......but then this, which makes me smile again.  It's a funny old up and down life, isn't it?
Merriness will resume shortly x 

Remarkable New York

I chose to stop believing in jinxing your luck, so here goes; This is the longest time that we have not been admitted to the ward in.... I don't know exactly, but a long time! The start to Secondary school has gone really well. So far, he says 'it's OK', which is a big improvement on the last year of Primary. We just have to hope that he is taking his Creon when he should. We have come to the age where we can only sit back and trust that he chooses to do the right thing for his health, in some respects. Big step for us and him. It looks like his last cough swab was lost in the lab, which is annoying, so we're waiting again for lab results to see how his Pseudo infection is. But he feels well, and that is the main thing for now. We so wanted him well for this start at school. 

I have just returned from a fabulous week in the Big Apple. I don't travel often for work, and this was the longest I have ever left the kids for (1 whole week, eek!). But both professionally and personally, I couldn't have asked for a better time. I found New Yorkers to be so kind and friendly, and the art and architecture incredible. Some pictures of lovely NY below, and some very happy girls with their presents. 

A few people have told me recently that I am 'so strong', 'remarkable' or even 'inspiring'. EEK! Little old me! I am blushing just thinking about it. I guess this is something to do with the fundraising, the working, the juggling 3 energetic kids, one of whom has a chronic illness.... But I always find this kind of comment odd, and argue that I'm not. Because I really am not. So here are some reasons why I really am not very remarkable after all (but thank you); 

If it were your child, you would do the same. At least, every friend I have in this world would. You just do. You too would do anything. You just cope in the tough bits (and privately crumble afterwards when you have time to cry). 

I don't iron. At all. I have no explanation for this other than pure laziness and desire to not waste a minute of life. 

If you had met the many remarkable people I know suffering with CF, you too would feel quite unremarkable in comparison. They are the inspiring ones. 

I rarely brush my children's hair. At least until dreadlocks start appearing. I'm the one in the school queue trying to smooth down the frizz with the spit on my hands, wiping the Nutella off their faces and hoping no one is judging me. I feel this is a very non-inspiring parental trait. 

Staying on children's wards, is always VERY HUMBLING. There are always many people much worse off than yourself (and they manage to brush their children's hair and have clean, ironed clothes). 

I usually have some miscellaneous, child related material on my top at work, and only discover it half way through the day, this might be toothpaste, lactulose or snot. Inspiring or (s)not? It once took me a whole day to realise I had some Lego poking out of my bra. 

I am not coping alone. I have the most excellent family and friends, nearby and far away, who help us when we need it. I am not strong, I am very, very fortunate. We didn't choose this life. They are the remarkable ones for choosing to help us. 

I'm just a Mum, to three fabulous children (and a bloody handsome dog). 

I could go on, but it would be neither remarkable nor inspiring, so I'll leave it there. Have a great day x 

PS; welcome to the world little Bodhi! I can't wait to meet you. Auntie Lizzy x 

National Transplant Week.

It's that week again. My endless apologies to my FaceBook friends as I will post links all week boring them all. But the only one I will post here is this, and the link to the register.

I promised to be honest in this blog (I am often too honest, to my detriment, this is either a character flaw or a blessing, depending on the audience). I've been signed up to donate for as long as I can remember, but these are the very real reasons as to why it's so important now to me personally that people to sign up to the organ donation register; 

It's more than likely that my son, my little Isaac, will need a lung (and/or liver) transplant in his lifetime (yep, CF is pretty shitty like that). We just don't know when. I can't tell you how much this scares me. 

1 in 3 people on the waiting list die waiting due to a shortage of donors. When I think about this I feel like I am falling into a black hole of despair. 

Regardless of being on the register, the ultimate decision comes from your next of kin, who, at that tragic moment, can override any decision made by someone in advance. You need to have that conversation with your loved ones. I know, it's not ideal teatime conversation, but nothing about transplantation or dying is ideal. 4 in 10 opportunities for organ transplantation are missed because of this. 

Think; You are more likely to need an organ donation than you are to give one. 

I miss Jayne. I miss her texts, and her blogs that made me laugh so hard I wet myself a little. I wonder if her transplant had come sooner if she would have been strong enough to survive? I miss Eva, and Toria, and Anders, and Emily and so many more. It's all feels so bloody unfair. And this is just within the CF community. 

One person can save up to 8 lives, and change countless others. 

What greater gift is there? 

Please sign up here. 

With love on National Transplant Week x 

Organ donations don't just prolong lives, they save lives.

I read a recent article in the paper about someone receiving an organ donation, and how this 'prolonged their life'.

The whole article kind of bugged me, and I've finally worked out why; 

Consider this; you will never make it onto a transplant waiting list unless your doctors feel that you have no other option. That most likely, you have less than 2 years to live with the damaged organs that you have. It's no easy thing, making it onto this magic list; it involves countless tests, counselling, and heart wrenching decisions. And after the HF that it is to agree to go on the list (Am I ready? Am I sick enough? Will I be too sick later if I delay?) you may still not be accepted if tests reveal you to be too risky for transplantation (and I get that doctors have to make incredibly hard decisions based on the evidence and experience they have, as organs are scarce, but still, it makes me so unbelievably sad.... because then what? Home, and wait for the inevitable? This is a reality for many). But anyway, I digress, say you do get to join the magic list, and you're not one of the 1 in 3 who die waiting (due to a massive shortage of people signing up as organ donors and crucially telling their loved ones about their wishes) and you receive those so vitally needed organs from a generous stranger; then is this saving your life, or prolonging it? 

I mean, we all die, right? If I grab a child about to run across the road, rather than allowing them to run right in front of a balding Audi driver with an inner rage issue; am I saving their life, or prolonging it? If that child lived then, but later tragically died 5 years later in another way, would my saving them initially be any less worthy? 

What worries me is that telling people that organ donation 'prolongs' rather than 'saves' lives might mean less people sign up. Will it make people think their decision is any less valuable, important, worthy, vital, altruistic, generous.....kind? 

Lung transplantion in CF is not a cure. I get that. So you may say that technically, the 'prolong' bit may be in some way correct. If Isaac had a lung transplant he would be swapping one set of treatments (treatment for lung infections and inflammation) for another (anti rejection), and he would still have CF in his other organs. It would be no easy ride, of that I am sure. There would be huge risks; side effects; unknowns. And possibly - limited life expectancy still. But do we measure life in days? Or happiness,  passions, friends, family, quality of life, potential, love? Is he not worthy of that chance, however much longer that chance would give him? 

And what of the donor? And their family? Would the fact that the recipient only lived a few more years make their gift any less valuable? I don't think so. 

We have a lot to learn yet about chronic rejection after transplant, and many continue to fall victim while we do, but there are organ recipients living decades post transplant. Seriously, decades! How great is that? 

Live life then give life. Please sign up for organ donation here and remember, if you don't tell your loved ones of your choice, then signing up alone is meaningless as the decision remains with them at that tragic time when you shuffle off this mortal coil. 

With love x 

Ma belle vacances

Greetings from the South of France.

Clinic went OK. His lung function was down 9%. Not good. Over 10% and they like to action more treatment. Hoping that it was just a bad day, and that we are not heading towards IV's (he starts Secondary school in 2 weeks, so that would be the worst timing). 

Lab results are back and his PseudoA infection is rampant again, despite 3 weeks of extra treatment recently for this (Cipro). Really not good. 

We are away, and have Cipro with us to treat him again, but a side effect of this drug is serious sun sensitivity. He gets burnt in an English Winter on this stuff - so it's not compatible with 30 degree + sunshine in Montpellier. So we have to wait until we get back. Or he gets more symptomatic, in which case we may have to come home early. 

This place is beautiful, and the kids are having a ball. It's so hard sometimes, the balance of life and what is best for his health. 

For now, we will enjoy every day we have here. And hope that the car gets us home when we do leave (it was not a happy bunny on the way here, it took 21 hours with numerous stops to cool engine and add oil galore). 

The good news; Rosa is swimming without buoyancy aids. Anouk is loving French markets and new dresses. I passed my recent exams. We are 17 people here, we eat, we drink, we swim, and listen to Kurt Vile. It's wonderful. 

Bonne nuit et une bonne santé x 

Note to burglars; we have house sitters and an over enthusiastic dog 😜 

Drugs, money and mucous.

Hard on my heart, but true.... this. Great blog.

Clinic this week; He is well. Wishing for fabulous lung function results. It's always just A NUMBER, and I know we shouldn't just look at the numbers. But it's ALWAYS a worry. Worry is my forever companion. I love him so much x

Sensationalism.

Dismayed to see sensationalist stuff in the paper today (see here) about PReP, a drug which prevents HIV infection, and how a ruling which enables widespread prescribing of this highly effective drug to be 'preventing children with CF having life saving drugs'. The problem is exactly the same, decisions have to be taken as long as the NHS has a limited pot of money, and big pharmaceuticals insist they need to recoup costs on new drugs to fund research into the next wonder drug. What is the government doing about this? To create a level playing ground that helps all? This playing off of one drug/condition against another helps no one - there has to be a middle ground here. Why is there not more in the news about the work that could be done to build bridges between those developing these life changing drugs and those funding them? (Meanwhile Theresa May's leopard print shoes make other exciting headline news). 

ANYHOW! A health update as promised. Isaac is having a great month, tummy never altogether great, but not awful, and relying a lot less on the anti-sickness meds. I think it's helps that it's the holidays and he gets to sleep a bit later. Chest wise he is really good right now; this feels like the longest stretch out of hospital in a long long time (I have decided to cease to believe in luck and jinxing... So feel safe to say this now, without fear that it will all go wrong tomorrow. Luck is what we make it). People with CF tend to need more salt in the hot weather (the basis of CF is the abnormal movement of salt and water across cells) and Isaac takes salt tablets all year round because of his digestive problems, but in summer especially he gets very tired unless he snacks on salty stuff too. The CF diet is very much the antithesis of ours! 

The kids are all enjoying the holidays, lots of paddling pool action, and we have lots planned, before the BIG school start in September (Rosa to Primary, Isaac to Secondary). We are busy working on the house (converting the garage into a games room). So for now, life is good. And I am feeling very lucky. One thing about a life with CF, or similar, is that it makes you really appreciate the good bits. Always smelling the roses. 

Good night all, and thank you x 

Eva wrote that before her death she wanted to leave;

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

Read this to see how Eva's family continue her legacy. 

Kids enjoy another amazing starlight day.

I've been working, but the kids have had a ball. Thank you again Starlight (to support this fabulous charity, read stories about the work they do for seriously ill children, and read about Isaac's granted wish, visit here.)

Have a great day all, proper health update soon I promise! X

More excitement in Merry Moly World....

OK, so this one is for the more geeky CF parents like me! I read this tonight. As ever, my belief is that there will be no 'One Cure' for CF (although very happy to be wrong on that front, such as gene therapy) but that a thousand small advances will, in affect, be a cure for CF as we know it. This might be one of those.

Good night all x

Who knows where the time goes...

All is well in Merry Moly World. The latest lab results are good! Today my gorgeous boy started Secondary school. This is quite incomprehensible to us, as it's surely not been 11+ years?! Here are some pictures of our amazing boy. Have a great day x 

First of Primary school. And yes, we let him go with that hair do. 

 

When Isaac and Anouk got on.... 

Laughing at my attempt at a cake (head previously fell on floor and was later reattached). 

Mummy love. Before I shrunk the lovely hat Ju made (nightmares still). 

Our little star. 

He no longer permits pictures being taken, so for now you'll forever be shown old ones! 

There were your eyes in the dark of the room...

Today has been a good day.

The kids are well. My head is well. Work is manic in a kind of 1001 things to do but I actually want to do them kind of way. And I am working with the loveliest people, and that means a huge amount to me. I am all about the people. My head may be less well soon as I am heading into 8 days of programme management training. Last time I had to do that amount of solid learning was way back for my degree (Psychology, if you're wondering. I am still wondering how I managed to pass). I'm not sure I can sit for that long, let alone listen, and understand, and retain any actual information! But I badly need to well in this. 

Isaac is well enough to postpone clinic this week, while we await his next lab results. If the PseudoA has not cleared, we'll need a new plan, but for today, I will think positively. I will post soon about his attitude and compliance with treatments, as we head into these teenage years. It's been emotional shall we say. We badly need him well for holidays and the start of secondary school in September. 

In the meantime, interesting article here on the use of inhaled Ibuprofen. Considering the widespread use and benefits of Hypertonic Saline (to us lay people, salty water) in CF now, when these already developed drugs come to light as a new therapeutic option, this is very welcome news in a world where NICE are refusing to fund precision drugs like Orkambi. For now at least. Which reminds me, if this petition can reach 100,000 signatures (...so about 80,000 more) it will be raised for debate in the commons. Any ideas on how to highlight this issue and gain that kind of support? Please message me with ideas! 

Have a great day. Listen to this or anything from the Epic album . It always cheers me up x 

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.