Having a ball.

My friends and family mean the world to me. At the weekend we attended a fabulous ball in aid of the CF Trust, raising a whopping £15,000. We ate, we drank, we danced. Thank you to these (and other absent) very special people for always being there to support us, and our fundraising x 

Opt in, Opt out.... Give life.

Last year Wales voted to adopt an Opt-out system for organ donation; this means presumed consent unless you say otherwise in your life time. The family of Kirstie Tancock want to continue her legacy in having this change in law come about in England as well. They appear in a short film here talking about this. The change in Wales is now having a positive effect on the number of life saving organ donations going ahead; Its been known for a while that opt-out legislative systems can dramatically increase rates of consent for donation (the so-called default effect) - the UK is years behind in this. 

Consent rates internationally vary hugely, and I am talking about non-living organ donation here; where organ donation is a possibility from someone who is brain-dead. Germany, an opt-in country (as we are currently in the rest of the UK) have an organ donation consent rate of 12%, whereas in Austria, a country with a very similar culture and economic development but which has an opt-out system, the consent rate is an amazing 99.98%. So I fully support the campaign Kirstie began before her death. 

That said, I don't believe an opt-out system alone brings about the best chances for people waiting for organs, a third of whom die waiting here: Spain has one of the highest rates of donations, and the characteristic that enables the Spanish presumed consent model to be so successful includes the fantastic resourcing of transplant coordinators - this role is so so critically important; trained, empathetic people who can work with the family of the donor towards an outcome where a very sick person can benefit from a life changing transplant, and the family of the deceased can benefit from knowing that they have done so. We need better communication and a shift in public support of organ donation. 

Currently in Italy if the deceased neither allowed nor refused donation while alive, relatives decide on his or her behalf; despite an act that provided for a proper opt-out system, and consent rates are lower -this proves that a ‘soft opt-out’ approach alone isn't always successful. Here in England, even if you carry a donor card, on the day of your death consent must come from your next of kin, regardless of your wishes. 

In the U.K. we have the highest rate of family refusal of organ donation in the Western World. Which is why it’s so important to have the conversation with your loved ones now! 

So yes, opt-out here will invariably help, but this must come with better infrastructure, more funding for transplant coordinators, transplant programmes, and more funding for public awareness. In Spain, the opt-out system came into play in 1979, but it took a further ten years for organ donation rates to significantly increase, when they focused resource on this kind of support and education. 

In some countries, an opt-out system alone actually reduced the number of transplants going ahead. Consider this; Instead of two groups; people who opt in, and those that neither opt in or out. The new system creates three groups; people who opt in, those that neither opted in or out (now presumed consent) and now those who opted out (In Wales this is about 6% of the population). The family of the latter group may have agreed to donate, but now the decision was no longer theirs. 

While the debate goes on, sign up here, but please, also tell your loved ones of your wishes. CF is the third most common reason for lung transplantation,  and has the best survival rates (60% at five years post transplant) - probably because people with CF are used to adhering to a strict treatment regime, which is needed post transplant. 

To learn more about CF and transplant, visit here. When I talk to people about organ donation, they often joke that their organs wouldn't be wanted anyway, but the oldest known organ donor was an amazing 107-year-old Scottish woman, whose corneas were donated after her death in 2016. Even cancer patients can donate corneas, allowing the blind to see. This is amazing science, allowing massively life changing things to happen to very sick people. Have a great day people! x 

Joan Miro. One of my kids favourites. 

Haunting and loss.

This blog is hard for me to read, in all its haunting familiarity. I know I have said before, these last few months have left me feeling a little battered and bruised, but the more I feel like that, the more in awe I am of Isaac; he doesn't sail through these things exactly, but he definitely just gets on with it (with a 'not the best' reply, a smile, and a black sense of humour, which I dig). The thought that these events may haunt or leave him traumatised going into adulthood is incredibly sad. 

Today he is feeling much better. He cooked us dinner, and made a pudding, my budding chef. We are channelling his love of food to good effect. 

CF took the life of another friend last week. I met the lovely K, and her sister (who also has CF) many years ago, through fundraising. They went out of their way to support an event I was putting on, manning the children's craft area. They were brilliant, and I was not (I learnt that event organising is not my thing). My love goes out to Emma, her family, and all of their friends. CF is too cruel x 

Surgery.

It's 3pm in the afternoon, and I'm sat in hospital watching Isaac sleep - he is swollen but beautiful. He also is morphined up to his gorgeous eyeballs following his nasal surgery. What they thought was numerous nasal polyps (really common in CF) blocking his airway both sides, was in fact one small polyp, and three huge cysts, completely blocking his airways/cavity. He has come out bloody, swollen and brave. It will take a couple of weeks or more to calm down - but after that, he should feel much much better; he hasn't been able to breathe through his nose in a year. 

They also did a bronchoalveolar lavage, to collect more sputum from his lungs, to test further for the NTM (henceforth known here as; New Twatish Microbastard). 

I look at him going through all this and realise this is like a full time job - he is only twelve; but just to stay well each day and keep CF at bay includes countless medications, physio, endless nebulisers, IV's, hospital stays, added extras like surgery, intestinal blockages.... but everytime we come in, he does it without complaint, thanking everyone who cares for him. If he feels scared, he never lets anyone (but me) see it. He watches as the needles enter his skin. He knows which are his good veins, and which are now useless from so many lines. Today he looked around the huge theatre, eyes wide, but acting calmly. We don't usually see the inside, as they put him to sleep just outside, but today was different. I hate the way he shakes, like a fit, as the gas puts him to sleep. Today they made it strawberry scented for him. The staff are amazing. 

Hopefully this will just be a flying visit, and he will recover at home (he will miss another 10 days of school, because of infection risk - his attendance this term will likely be under 50%, a worry for another day). In the meantime, we wait nervously for the test results to come in. 

Have some more cool pictures x 

I

The good, the bad, and the... twirling thoughts around my maddened mind.

Clinic was a mixed bag. On one hand, he is so much better now. His chest sounds good, he is still coughing and productive, but he no longer sounds like he is breathing through treacle. He absolutely aced lung function - and for that we are so so glad. He surprised us all.

However, the bronchoscopy results that continue to creep in have revealed that he does have a new bacteria growing in his lungs - along with the PseudomonasA (bastard bacteria) and the Aspergillus (flagitious fungus) that we know already reside in his precious lungs - this bug is known as a Non-TB-Mycobacterium (NTM, a cousin of tuberculosis) - but there are many strains, one being much worse than the others. Until we know for sure which strain it is, there is nothing we can do, crucially (and terrifyingly) some are multi-drug-resistant. All of this information twirls and bounces around my mind and stops me sleeping. 

We are also preparing for surgery on Monday - to remove the Polyps growing in his nose and widen his airways. This should just be day surgery. Isaac will have IV antibiotics throughout, to help prevent his bugs having a little party down there while he is weakened by the general anaesthetic, and they will likely bronch him again while he is under, to hoover out more mucous to test further for the NTM. I'm packing an overnight bag just in case! I don't think he is aware yet quite how rubbish he is going to feel when he wakes up, poor kid - super sore throat and a punch in the nose. 

Have a great weekend guys. Here are some very cool pictures: 

To build a home

Anouk (age 8) wanted to write a post: 

CF splits our family up. It makes me feel sad. My brother has to take lots of medicine and tests. He stays in hospital a lot. I miss my mum and dad, and Isaac when they are gone. Me and my sister don't have CF because my mum and dad decided to put us in a freezer and made lots of eggs and then they picked us. We have to stay with our grandma sometimes when mum and dad are in hospital. My mum does fundraising for cystic fibrosis that helps a lot. They will make a cure. Isaac is a bit annoying sometimes, but I love him. I felt scared when Isaac was very poorly in intensive care. When we visited him he had machines all around him, tubes up his nose, and lines in his arms. But it was still nice to see him. It was hard for our family and especially hard for my mum and dad.It was scary for us when he was in hospital because we didn't know when he was coming out. 

And so Rosa (age 5) had to follow!: 

I hate it when Isaac goes into hospital. And he doesn't like to stay in this long, and it makes him feel bored and sad from home. I hate when he is sick. It feels sad when he's at hospital. I miss the people that look after him and definitely Isaac. We see lots of poorly people in hospital. Some have no legs. But they have cool toys. When Isaac gets home from hospital we get to go on special moments. We went to Center Parcs. It was happy. People that got CF they feel really bad and sad from home. And Isaac has lots of tests and he has cuts, and he throws up in a special bowl. It's fun to see him. We feel sad when we're at home without him. Even more happier when he comes home.

We build a life around our own, exhausting, funny, heartbreaking, loving and cosy reality. CF does not define us. Family does x 

There is a house built out of stone
Wooden floors, walls and window sills
Tables and chairs worn by all of the dust
This is a place where I don't feel alone
This is a place where I feel at home

'Cause, I built a home
For you
For me

Until it disappeared
From me
From you

And now, it's time to leave and turn to dust

Out in the garden where we planted the seeds
There is a tree as old as me
Branches were sewn by the color of green
Ground had arose and passed it's knees

By the cracks of the skin I climbed to the top
I climbed the tree to see the world
When the gusts came around to blow me down
I held on as tightly as you held onto me
I held on as tightly as you held onto me

And, I built a home
For you
For me

Until it disappeared
From me
From you

And now, it's time to leave and turn to dust. 

The cinematic orchestra - to build a home. 

This life.

We've just got home from a weekend away with a very Christmassy feel, full of swimming, feasting, water slides, tree top adventures and pony rides. The kids had a ball, and Isaac was really pretty well, this last week his health has come on in leaps and bounds. They all so deserved this. 

This week we are back at hospital for clinic and the all-telling-lung-function-tests - massively hoping for an improvement on his last results. Next week we are back in for his nasal surgery, which will likely set him back again. But only a little we hope. General anaesthetics are always a risk, and so he will have IV antibiotics throughout the surgery to give him some extra cover. Those nasty bugs like to party given half the chance. 

As close to a nice picture of the grumpy teen as we are allowed these days.... 

For me, this last week has been full of sadness too; The world has lost a funny and charming soul. Another friend is coping with the impending loss of her husband. And news of more earthquakes, the worst yet. Loss seems all around us, and I admit, at times it is hard for me to see the light amid the darkness. 

That said, I'm happy to be back at work, although busy, and trying to make better plans for future hospital admissions. As we now know we will be going in for regular, planned courses of IV antibiotics (therefore spending more time in hospital) we're trying to think of any ideas which will help us provide more stability for the kids, and enable us to work when we can. Work have been really supportive of all of my ideas, for example, allowing me to have a loan phone for admissions (so I can use the data for wifi on my laptop if I can't get to the office - Hospital is fortunately is just a ten minute cycle ride from my office, so my hope is to flit more between the two), and fully flexible working. 

In between all of this, and extra treatments for Isaac, we have been busy having as much fun possible. My little Halloween dudes: 

Have a great day. Be safe x 

Frustrations.

I want to tell you is that Isaac is well, back at school, and loving life; I'd love to tell you that Rosa is settled and behaving again, and her rage and tantrums are a thing of the past; That Anouk is getting all the love and attention she deserves; That I am sleeping well and when I fart, rose petals fall to the ground behind me....

But it's just not true. Recovery is slow; Isaac is vomiting daily, and missing more school. This could be a) cough-til-your sick vomiting, b) CF digestive problem related vomiting, c) side effects from the steroids/anti fungal drugs which he is still on or d) a bit of everything, with a little post-hospital anxiety thrown in for good measure - we just don't know. Plus, we think he has been teased at school about his moon face (known side effect of steroids) which makes me feel mildly, well, murderous. 

Rosa swings from loving me so hard, to hitting me in rage. I am trying to get back into work, which is a welcome distraction to everything (I love my job and colleagues) but my heads not quite there - I want to be there, but my *anxiety still come in waves. Still trying to figure out how the time I have taken off work will affect us (unpaid?) and at the same time planning how we will manage this better in future, as we are facing more regular, planned hospital stays. 

My anxiety stems from seeing Isaac become so sick, so quickly; a glimpse of a future that we hope never happens. 

We are waiting for a date for the CT, while results from the bronchoscopy come in slowly - and will do for some weeks yet. We also have to prepare ourselves for surgery in a couple of weeks; shit, that had better go well.

* Anxiety is an emotion characterized by an unpleasant state of inner turmoil, often accompanied by nervous behavior, such as pacing back and forth, somatic complaints, and rumination.^[1] It is the subjectively unpleasant feelings of dread over anticipated events, such as the feeling of imminent death.^[2] Anxiety is not the same as fear, which is a response to a real or perceived immediate threat,^[3] whereas anxiety is the expectation of future threat.^[3] Anxiety is a feeling of uneasiness and worry.^[4] It is often accompanied by muscular tension,^[3] restlessness, fatigue and problems in concentration. ^<Wikipedia)