Autumnal news

Isaac is well. Chest. Tummy. Everything. Best start to Autumn yet I think... 

Little Rosa less so. She has just started pre-school after a life of mainly being at home or with Grandparents, so we can pretty much write off the next year for her bug wise. It will be one after the other for a while. She is such a darling, and despite being the clingyest babe ever, settling into this new world of hers has gone better than expected. 

Anouk, our gorgeous girl. If there was an award for sweetness, she would be a gold medalist. Examples being these notes and gifts left on our pillows (just one of many sweet things...)

I am really excited about how National Transplant Week went. I posted daily about this, and the buzz on my FB feed was so much greater than other years. 

We have escaped hospital for a month, and following my huge disappointment about the clinical trial, this is very good timing. I know it sounds ridiculous (as he can't join the trial as he is currently TOO WELL) but this is still bittersweet for me. It would just be so good to feel like we were fighting CF, rather than just managing it. Which this drug could do. Maybe. So the wait continues. 

Tonight I am sad. It's not my sadness to share. But a friend is going through the unimaginable. Knowing so many people now who are going through such hard times (because of CF usually) I sometimes feel a little numb to it all (I think I have to). But this is raw, and feels so so unfair. 

I hope you are having a better day. 

Clinical disappointment

So the good news. Isaac hit a 93% FEV at his last clinic appointment. This is a measurement of lung function, forced expiratory volume, that is, how much air he can blow out in one second, and is one of many ways in which doctors measure wellness in CF, and often, the most predictive. 93% was a really good day for Isaac, as in the past couple of years he has declined to, at times, 70-80% (which is not life changing in itself, but is a decline we would rather not see, at this, or any age). At times this decline is because of known infections, at others just unknown, which is worse.

The bad news is, this recent good result means that he won't now be eligible to be assessed for the Ataluren clinical trial. 

To say I am disappointed would be an understatement. I had a little cry (most unlike moi!). Clearly we're delighted at his good results. But how ironic that they come at this time, when the drug that I have been following the progress of for 5 years or more has eventually started recruiting for trials, not only in the UK, but just down the road from us, in London. 

This is not just any drug. This would be the first drug that Isaac could have taken which could, potentially, treat the underlying cause of his CF, not just the symptoms. And yes, it may not have worked. He may have even been on the placebo. And even if it had worked, would it ever be approved by NICE for the clinical setting? Would it be affordable for the NHS? I don't know. But I sure would have liked to have tried! 

CF friends will understand, Ataluren is very important to us, as Isaac has rare mutations, class 1 (supposedly the most severe) and so Ivacaftor and Lumicaftor will not help him. And gene therapy, which would work for all, by my estimations is still 8 or more years away.

I know this doesn't mean that our love affair with Ataluren is impossible, only we have to wait longer to find out. I'm just disappointed that they don't look at average FEVs rather than the most recent, or assess him in London and then decide? Sod's law his FEV will have dropped next time he is tested! 

Sorry. Rant over. Feeling disappointed. 

Merriness will resume shortly x 

Transplant week

This week is transplant week, and I will decorate my Facebook page with 'inspirational' messages enticing people to sign up. Hopefully they will be in an old fashioned font and have a sunset image in the background.

Here, I can point you to REAL LIFE stories, should you want a realistic view. Transplant is not easy, and Piper writes here about her second transplant with her usual wit and realism.

I can point you in the direction of so many more stories; those friends who have died waiting; those friends who have had a successful transplant but later died following complications or rejection; those who have lived, and lived, and loved life.

The one common theme of the stories I know is that not one of them have regretted having transplants (or regretted hoping for one).

Live life then give life (after all, what you gonna do with them?).

And please remember, more importantly than just signing up for organ donation, please tell your loved ones. Your families wishes override any donor card you carry or register you may be on. It is just one conversation, but one that could save 9 other lives.

Sign up here.

Thinking of Jayne, who is still waiting for her new lungs, and who never loses her humour. Love you funny lady.

Some summer pictures to brighten up this old place...

Life is good.