Isaac is now the proud owner of a shiny new portacath in his chest, which will make blood draws and IV antibiotic treatment so much easier. He has been in a fair amount of pain post-op, the incisions are larger than we expected, and he’s had all the usual fun stuff post anaesthetic (throwing up, massively sore throat from the intubation, lips so dry they crack and peel). But it is in, and it is working! He remains in hospital, and his cough is still worrying me, especially as we are coming to the end of two weeks of IVs, the point at which we’d expect his chest to be the best it can be. Hopefully this is just post-anaethetic-cilia-need-to-wake-up-effects. This is an actual thing, I didn’t just make that up. We have lined up samples for the lab, so have the usual wait now for all the test results. But for now, we just want him home. His sisters need us all home.
Welldone and thank you to all those that will or have been attending NICE meetings around the U.K, campaigning for the right for people with CF to access Orkambi, a life changing drug; 96-week data shows that Orkambi can slow decline in lung function – the main cause of death among people with cystic fibrosis - by 42%. There are 2,834 people in England, 243 people in Scotland, 118 people in Wales and 101 people in Northern Ireland who could benefit from Orkambi. Sadly Isaac is not one of those (due to his rare and quite shitty mutation) but I can completely relate to just how maddeningly painful and frustrating it must be for other parents and adults with CF to know that this drug is there, it’s bottled up, ready to go, and your doctor wants to prescribe it for you..... but can’t. What price can you put on a life?
Thank you for reading x
