A coughing good Christmas.

It is me coughing this Christmas. A 'can't lie down for spluttering, lung screaming, throat angering' kind of cough. But being ill is always a reminder of what it might be like to feel rubbish daily, as some people are, others who are suffering in ways I almost never do. Feeling normal is criminally underrated, and I am as guilty as the next person for taking that forgranted. And so I hope my empathy and this cough makes me a better Mum in some small way, for my boy who almost never feels 'normal'. That is my coughing Christmas silver lining. 

We have had a lovely Christmas. The kids have had fun. We are relaxing with family, surrounded by music, and soaked in gin. 

Wishing you all a happy holidays too x 

With family this Christmas?

Looking back, I think I was a wonderful mum of two. There was baking and homemade costumes aplenty. Heavens, even homework was tackled and hair brushed. In comparison, I fear I am a pretty crappy mum of three. In this run up to Christmas I've fallen into that classic parent mode where IT'S ALL TOO BLOODY STRESSFUL. I never believed parents suffering from this phenomenon before, I mean, how hard can it be to order presents online and make a Saxo stuffing? But I am, and it is. So, forgive me, no Christmas cards this year. I am making a donation to the CF Trust instead. 

And thinking of all you lovely people, who I hope are looking forward to a family Christmas too, can I suggest a light after dinner chat? Even if you are signed up to be an organ donor, your next of kin can (and surprisingly often do) override your wishes in that tragic moment. 

You are more likely to need an organ transplant than you are to donate an organ, and so it makes sense that more than half on the list die waiting due to a shortage of donors. Please sign up, and please, have that conversation while you can. Sign up here! 

For us, we are all well. Isaac's extra treatments since his admission have now come to an end (aside from the extra nebs which he will remain on now). He has a few tummy problems again, but is mostly well and very happy to be home and off school.  

Wishing you all a very merry time this holidays and a healthy new year too x 

Christmas cheer

Huge thank you's to my wonderful Cambridge University Press colleagues who supported our annual Christmas Raffle in aid of the CF Trust today. This raffle is now in its 11th year, and used to consist of all the supplier donated Christmas goodies sent in. However due to compliance regs, suppliers send in a lot less these days, if any, and so the raffle now largely consists of prizes donated by staff. Despite this, the funds raised increase with every year.... Which by my books means my colleagues grow kinder and more wonderous each year! 

£500 raised today, and more to count up next week from cakes sold too. I've created a new fundraising page which you can see here, and this has prompted me to think about my next fundraising challenge too.....Ideas on a postcard please! 

Our family and friends have now raised over £53,000 for the Trust since Isaac was born, and a large part of this is down to my colleagues, never seemingly getting sick of me asking for sponsors. It means more to us than you can know. Thank you. 

Amazing Anouk.

Our little Anouk turned 8 this weekend gone. She has always been the sweetest little thing, the most beautiful baby, and continues to fill us up with love. A miracle baby, born following PGD treatment (which you can read more about here), so new that she was the 23rd baby born in the world following that kind of scientific test. She even has tree planted for her in a woods for this reason, she is so special. From day one she was full of love, grace and kindness. She mothers all children, a prolific love-letter writer, a gift giving creative, an arty soul....

Our little Nanook, Babooska, Shnuks.... we love you so.
As you like to say 'I flew right into your heart'.

Whenever I'm alone with you You make me feel like I am home again Whenever I'm alone with you You make me feel like I am whole again Whenever I'm alone with you You make me feel like I am young again Whenever I'm alone with you You make me feel like I am fun again However far away I will always love you However long I stay I will always love you Whatever words I say I will always love you I will always love you Whenever I'm alone with you You make me feel like I am young again Whenever I'm alone with you You make me feel like I am fun again However far away I will always love you However long I staye I will always love you Whatever words I say I will always love you I will always love you

Young lives lost.

Another young life lost. Not lost, cruelly taken, by Cystic Fibrosis. A family heartbroken. 

Kirstie was 27, and blogged in her usual candid and ever positive style here when she knew that she was dying. Kirstie featured in a documentary about CF which you can see a clip of here. She went on to have not just one life saving double lung transplant, but two. These gave Kirstie five more years of life. She was an ambassador for the charity Live Life Give Life founded by the lovely Emily (also sadly no longer with us). 

People sometimes ask me what they can do to help? Well, there are ways.... 

Fundraise for the Cystic Fibrosis Trust. It doesn't have to be shaving your hair off, or climbing a mountain as I do, a cake sale at work can be wonderful too, or a non-school uniform day in fancy dress. The Trust fund research which WILL extend the lives of people with CF. And not just extend them, but also improve the quality of that life. 

If fundraising is not your thing, then campaign to get more people signed up to the Organ Donor Register. Remind people; you are more likely to need an organ donation than you are to give one; Half of those listed die waiting; You must tell your next of kin your wishes, otherwise they can over ride yours; And that Transplants can and do SAVE lives. 

If you haven't already, please sign up here.

Here is to those extra five years during which you lived and loved life to the full. Sleep tight Kirstie x 

Sunday Roast and A&E

Today we celebrate my wonderful Mum's Birthday, and my favourite Nephew, Ben's. I can never say it enough, but my Mum is my ultimate hero. As well as being the hardest working Mum, she is the most incredible Grandma, who along with her husband Joe, have looked after mine and my sisters kids in their early years while we worked, and even now they are all in school, having them in the holidays when we can't, picking them up from school, and coming to our rescue with hot meals and fresh veggies from the allotment all the time. She is the most selfless person I know, only ever wanting to help others. 

So today it was our turn to spoil her a little, but just a few hours before they and family arrived for a roast dinner, Isaac and I were having to head into A&E to get him checked out. His longline is hurting him now, which it shouldn't. Thankfully they agreed it was not blocked, but likely his vein is irritated. Not surprising really, having housed a tube for the last two and a half weeks and pushing through pretty toxic drugs 3 times a day. It would be great for it to last until Thursday..... but it's still not comfortable for him tonight, so I'll be calling clinic in the morning. The team in A&E were fantastic, and Isaac as brave as ever, we made it home in time to celebrate. 

I promised a summary about IV lines, so here is my very non-medically trained summary; 

A cannula is the most common IV line, typically inserted in the back of the hand. A needle is pushed into a vein and a short flexible tube is pushed up the vein. The needle is removed, but the line stays in. On the end of the line is a port, which syringes (or drips, depending on the amount of drug and how slow this needs to be delivered) can be attached to, to then deliver drugs directly into the blood stream. 

Essentially, all lines follow this same principle, just in different veins in the body, in different lengths, and to last different durations. 

A longline is the same, but as the name suggests, longer. This is usually inserted just below the elbow, but Isaac has also had them in his ankles, and some veins in the groin may be used. These typically last longer than a cannula, usually up to 3 weeks. 

A PICC line (peripherally inserted central catheter) are similar to a longline, and Isaac has had these in the past too. Only they are longer again, and usually inserted under general anaesthetic. The length is typically from his elbow right up to his chest cavity, or from his ankle to his abdomen. These can last many weeks, or months. 

Isaac has not yet had any other kind of line, but Central, Femoral and Hickman lines are often used for drug delivery over a longer period, often in oncology. 

In CF, a more permanent IV device can be surgically implanted, called a Portacath usually in the chest or upper arm. Isaac is likely to have one when he needs more regular IVs as this makes drug delivery much easier. It's like a rubber bung, sitting under the skin, which can be accessed easily when needed for IVs, but at other times, is fairly invisible. It's also often used in CF due to the number of courses of IV antibiotics which damage his veins, meaning they cannot be used again. 

Our IV station; 

Happy birthday Mama and lovely Ben x

Pudding honey?

Ceftaz, Tobi, flush, with a side of Hepsal perhaps?

Home blimin sweet home.

At last. Two weeks in hospital feels like 3 years 4 months and 29 days when you are there. I was kind of hoping to get out and find that the whole Brexit/Trump thing was a whole Dallas type dream, Bobby would wake up in the shower again and none of it would have happened.... no? Hell.

Isaac has gained 15% lung function compared to admission, which is good, no.... it's great, but not quite as high as we would all like it. I am so thankful for his excellent CF team who like us won't settle for less. If we can get him better, we will! Therefore we are planning a third week of IVs, which is unusual, but may help his LF further. But I'll do these at home, which is so much closer to a normal family life. So we loaded the car with our many many bags, meds and equipment, and escaped. If his longline fails sooner we will rethink (I will post soon to better explain cannulas, long lines, and PICC lines, all of which he has at times, as I realise it is very confusing)

The fresh night air was exhilarating,  played the music so loud and sped home to be a family again, smiling all the way. Talking to another CF parent earlier who is in the same situation right now, about how you get home, and it's amazing, and you celebrate...... but unlike many other people leaving hospital, it's not over, it's never over for us. And so it's often when you get home that you crumble, after holding it together inside so well. I am not yet crumble. I am still smiling, but soon....

Huge thank you's to the Grandparents as always, coming to our rescue. We can never thank you enough. The Cayley's for always being there, Bronté for always visiting, Lautaro, Ju and Jess (so great to see you guys), Lemon, Lizzie, Gem, Jin, Hamish, James, Bon, Sarah, Gavin and Jennifer and many others for the soup, the wifi, the help, the kind words.... it really does mean the world to me, and helps Isaac pass the time.

And to the amazing staff on D2, nurses, our unsung heroes x

Hospital life part 2.

It feels like we have been here for weeks already. It is such a mix of emotions. On one hand he is relatively well, we feel like the lucky ones. He bounces around, eats non stop, goes to the gym twice a day, and is more than happy to have needles as it means more laughing gas. We see so many poorly children here, but many of them come and go. Patch them up and get them home. Whereas we stay. He looks so well, but his lungs are not.

I am with my boy, and I miss my girls, my dog, the cosy life we have made for our family in our little cottage. Dan comes to my rescue, but then it feels wrong not being with Isaac. He is brave, and he is brilliant company, but he is also at times angry, bored and frustrated, and it's me he lashes out at. Because that's safe. Unconditional. And I know this, I understand. I understand him. But it's still hard sometimes.

CF affects all of us, Anouk especially gets very sad when we have to stay in, and she's beginning to question why the poorly looking kids get to go home, but Isaac doesn't ('Is Isaac more sick than them?' She asks).

Midweek it looked possible that we would get to come home early to finish the course at home. I've retrained, so I am all set to do his IVs myself, but his test results just weren't good enough. As much as we tried not to get our hopes up, we did, and it was a big disappointment. Then we were told he might even need a third week of IVs, which he has not needed in years (two weeks is the standard length for IV antibiotics). We agree with the plan, which is focussed on getting his lung function up as much as we can, but it's tough.

This weekend he has been allowed home for the day inbetween IVs. Tomorrow we are back on the ward full time to resume the extra physio sessions too. Last night he was up until 4.30am doing his IVs which had been due at 7pm. His longline had blocked, and he has another's cannula in his hand now. They will try and rescue the longline again tonight, otherwise he will have a new one tomorrow morning. He is looking increasingly pin cushioned, and the cannula (in his hand) limits him much more than the longline (in his arm).

On the plus side, we escaped for a few hours the other day and we had some fun, and the Christmas Spirit came to the ward early this year, when every child was given a book on Astronomy, a piece of Meteorite, and a Telescope. Amazing.

He is getting better, just not quite better enough yet. Merryness will resume shortly x

All is well.

It's been a good weekend. Isaac's IVs are well timed, we get a few hours free in the afternoon, and today he came home for that short time and went out to play with friends. His chest sounds worse, but this is likely good news; The gunk is on the move. Tonight he has returned to the ward with Dan, and I'll take over in the morning for the next 4 nights. I'm enjoying time with Anouk and Rosa, we sleep in a soft heap of girlieness. But while I know he is in safe hands, it is still very hard for me to not be with him. We sleep in his bed to feel close to him.

Monday means the return to proper doctors rounds, and a whole lot more tests to see if these antibiotics are having a positive affect. We know that we are in the right place, and fighting against CF aggressively like this is right; we welcome the battle as it will keep him well longer. But it's not easy to explain to a 4 and 7 year old who just want their family to be together.

Thank you for all the lovely messages. People have said in the past that they haven't contacted us because they didn't want to say the wrong thing, or ask what might be intrusive questions. But in my experience, I would always rather people did ask, however silly they might think their questions are, that you are thinking of him enough to ask shows that you care.

Hoping to have good news for you soon. If his lung function increases enough, we may get to finish off the course at home, once I've had my refresher training as 'nurse Lizzy'. Keep everything crossed! Good night all x

Hospital life...

You know when you have settled in to hospital life again when you're in M&S buying sushi for breakfast, in your tea stained pyjamas at 11am, and you don't care. It's freezing outside, but you're in flip flops because the ward is a sweaty 35 degrees all year round. You play chess, Xbox, cards and watch a film, hoping the hours are passing, only to find it's still only 10am. You go to bed at 8pm and stay in bed until 6am, to have 4 whole hours sleep. You have lovely cuddles, pranks, laughter, and then strops and shouting, all within an hour.

That said..... It's all going well. Cannula in (uncomfortable, and in his favoured hand) the first day, long line the next. Went in first time perfectly, on gas and air (which I had to wrestle off him in the end!). This is so much much better for him, he has great movement, and should last the whole 2 weeks. He was so brave. He has a full on routine; 4 lots of IVs. 5 nebulisers. 4 lots of physio. We have been in the gym twice today. On one hand it seems like an endless cycle of treatments, but on the other, time passes so very slowly. He was very happy to see two aunties today, and for us to all eat together as a family tonight. His cousin Ben is visiting tomorrow.

All going well so far, and I couldn't be prouder.

Hoping for good results from the lab and better spirometry results (early next week) when we will know more. Good night all x

Hospital stay.

We are heading into hospital tomorrow for 2 weeks of IV antibiotics. No huge surprise. He feels OK, but his cough and lung function are worse. We are confident this will kick some serious CF butt, and we'll have him fighting fit again for Crimble season. Don't you worry! 

More soon x 

Compliance.

I've been meaning to write for a while about CF and compliance to all the treatments CF entails. To illustrate this better for those less familiar with CF, this is Isaac's typical day (when he is well);

Morning; 

Anti-sickness medication when he wakes up

Inhalers (to open up his airways), nasal sprays

*Nebuliser 1 (DNAse, which is an inhaled enzyme that helps thin the mucous in his lungs)

Breakfast (with Creon, his enzymes which allow him to digest food properly)

Morning tablets (antihistamines, salt tablets, plus three medications for his tummy issues)

**Physiotherapy to clear his mucous

Nebuliser 2 (Antibiotics, Colomycin or Tobramycin, to fight his infections)

Afternoon; 

Lunch with Creon again

Evening; 

More inhalers 

Nebuliser 3 (Hypertonic saline, to draw more water into his lungs, to again loosen the mucous)

Physiotherapy again

Nebuliser 4 (Antibiotics again, to fight his infections)

Evening tablets (vitamins, oral antibiotics, many more tummy related medications)

Night;

Possibly more Physio, depending on how he feels

*A nebuliser is an electronic device which breaks down particles of medication into smaller blobs (sooo scientific, me) which are then better delivered to and absorbed in the lungs. Each nebuliser takes him between 10-30 minutes, and he has two different machines, because of the different drugs he nebulises. We call these 'nebs', and anyone walking past our home at any given time would have a pretty good chance is hearing us yell 'neb Ise!' as his concentration on these is lacking. 

**CF Physiotherapy can be of different kinds. He has small devices which he can breathe through against resistance, which help shake his lungs. Some of these he can do hooked up to a nebuliser at the same time, which help him clear his chest on exhalation, and then deliver the medication on inhalation. He also has chest percussion when unwell, which is the classic 'pat him on the back' physio that most people remember about CF, we call this patacakes. 

His current prescription list is 22 medications long, which we need to ensure we have in stock at all times. And due to crazy NHS budget rules, only some of these can come from our local pharmacy, the others we need to get from the hospital pharmacy. In addition to this, he needs to take enzymes (Creon) with everything he eats that contains fats or proteins (pretty much everything aside from plain fruit or veg) because of a rubbishy pancreas he produces none of his own. This varies according to what and how much he eats, usually around 30-50 capsules a day. 

There is a strict order to his treatments; for example, we want to loosen his mucous first, then help it come up, and finally throw antibiotics in to make war on the bacteria, when they have clear access. Also, many of his drugs have interdependencies, side effects, or just... effects, which mean you need to take them at a certain times of day, with or without food, or avoiding something else. This can be mighty hard to get right all the time (try giving babies antibiotics four times a day which can't be taken one hour either side of milk...!). 

Reader, I can't imagine how that reads to you..... Is it a lot? Less than you thought? 

To us, this is our norm. This is our good day. Our minimum. It can be much more when he is not so well; for example, he has occasional courses of IV's (Intravenous Antibiotics) - this might be because he is more symptomatic than usual, or because his lab results reveal something new (they culture samples from his mucous to see what bacteria are growing in his lungs). This means two weeks+ with an IV line in; antibiotics and physio galore. 

But we're lucky, IV's are an occasional occurrence for us still; many other kids and adults with CF have regular IV's - two weeks every three months (at least) simply to keep the infections at bay. There are also other complications of CF which we don't have to deal with yet - diabetes, liver issues, problems in maintaining weight (resulting in PEG feeding directly into the tummy to cram in the calories overnight).... The list goes on, and each of these would add significantly to the treatment burden. 

There is not a day that goes by where I don't think how fortunate we are to live in a time of such great medical advances, to have such a wonderful NHS, and such a supportive CF team. I even pass by our ever expanding meds cupboard sometimes and admire the neat pharmaceutical packages, so pristine and white, the syringes, the wipes, the nebuliser (retail cost £3K), all lined up and ready to join us in battle against those pesky bugs. We don't moan about these treatments, we are thankful to have them. 

But it is so hard for Isaac. On a good day, the above will probably take him one to two hours. On a bad day, three to four. On IV's? It's pretty much like a full time job. Just to stay well. He is 11 years old. He wants to be out playing with his friends..... he wants to be like everyone else. He doesn't talk about CF with his friends, and even taking Creon in front of people is an issue for him. Anyone who remembers Secondary School can understand that. When I was 11 I too wanted to be like everyone else (when I was 21 I wanted to be like no-one else). Right? I am ever mindful of how he must feel, but also need to get him to do all this, because the alternative is him being less well, and likely resulting in long term lung damage. That is not an option to me. He has an understanding of how important his treatments are, I tell him, his Dad tells him, his CF team tells him, and yet he still cuts corners wherever humanly possible unless we watch him the whole time (which with two other smaller children is difficult to say the least)...... The nebuliser which he insists he finished properly while I was in the bathroom, the pathetic blows of a mouse doing his physio, the 'forgotten' Creon, which then gives him a rotten tummy, and makes doing effective physio the next morning even less appealing..,.. 

I get it, I really do. I get him. I know him better than anyone. And if it were me? I'd have probably done the same at 11. But it doesn't make it any easier to watch, and getting him doesn't always make me any better at reasoning with him. We have this closeness in hospital where we can talk calmly about it, and I do anything to not sound like I am nagging him, I've drawn blood biting my tongue so much. But  when he's frustrated, and angry, and feeling like his life is so unfair, what to do? So I agree - CF does suck; or I tell him how many more people are worse off; I bribe, I bargain; I think up wacky new ideas to put the fun back in treatments; but sometimes.... I have nothing. I'm just mad too, not at him, but at CF, and how does that help the situation? Not one iota. 

Some days there is no issue at all, he just gets on with it. 

Other days I know we could have done better. 

On the worst days, I'll walk the dog for hours, just procrastinating, knowing the next round awaits me when I get home. 

Some nights this keeps me awake, thinking, have we done our best by him today? 

To support the Cystic Fibrosis Trust who fund research into CF please click here. 

I'm sat in front of my fire, with the dog on my lap, listening to this beauty, put it up loud, it's a grower, I promise. Goodnight all x 

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