Last year, half of all people who died with CF were under the age of 31. Sorry to be so blunt. Really. If I could avoid the statistics, I would. But in the last year Isaac has had 5 courses of IV antibiotics for 2-4 weeks at a time, 10 weeks in hospital, 4 operations, countless tests and clinic visits, and continues to take nebulised drugs every single day which cost the NHS thousands of pounds every year. I am not saying this is a moany way, we are so so grateful to the NHS for the treatment that he receives, and the life it allows him to live. We really do consider ourselves lucky.
Orkambi will not work for Isaac’s mutation, but I hope one day there will be a precision drug that will, and while the cost for these initially seems astronomical (Orkambi costs around £100K per annum, per patient), I do believe there is a saving for the NHS in terms of keeping people with CF well and out of hospital, which these drugs really have the capacity to do, and the pipeline deal would guarantee access to future drugs hopefully even more effective than Orkambi.
I’ve long been gutted that Isaac’s rare mutation means we need to wait longer for these kind of treatments (they are also an oral drug, no IVs, no nebs, Isaac’s dream come true!) in a sad way I’m glad that Orkambi won’t work for him, because knowing that a treatment was there, approved, and waiting on a shelf for him, and not being able to reach it would drive me beyond crazy. I honestly don’t know how I would cope.
If you are in England, and could find the time to help, please download the template found here and send it to your MP, which you can find here. It would mean so much to me if you could.
Things here are OK. We had a wobble last night as he had a mild fever and vomited. We have to be very cautious about infection risk in his port on IVs. Thankfully this morning he was back to his usual self. Despite home IVs, we’re just really thankful to not be in hospital for this course, which we hope will end on Tuesday (with a cool swim for him) all going well with lung function tests.
Have a great day x
