You really need to make a cake and get it to a party....
Class I; you've got some ingredients, but not all of them. You're fecked basically.
Class II; the most common problem bakers have, you have all the ingredients, you make your cake, you just don't put it together too well, it flops in the middle.
Class III; you make the cake, woohoo, it looks OK, but it tastes, well, not great. Maybe the slightly out of date butter wasn't such a good idea.
Class IV; you make the cake, it's pretty much OK, but on the way out the door you trip, fall, and the cake goes everywhere.
Class V; you bake a lovely cake, but just before you get to the party to share it, you realise the dog has eaten it in the car. The little shit.
So, all pretty annoying, and maybe if you were in class five, you'd wish you had never started making the bloody thing in the first place. But in CF terms, the worst position to be in is class I, which means the correct (CFTR) protein is never correctly made, which in turn means a fault in the way water and salt move between cells, resulting in the sticky mucous that those blimin bacteria love to holiday in.
There are some amazing things happening in CF research right now, and in the last couple of years, the first drug was licensed that actually treats the underlying cause of CF (until now, all treatment has been symptom treating). This is aimed only at people with a certain kind of (class three) mutation, of which there are very few (about 5% of the CF pop). Coming up soon, assuming it gets the astronomical funding that it needs (...which if it does, as the rumoured price stands, would cripple the NHS.... but that is another blog) will help the vast majority (who have class two mutations). This really is exciting times, and makes all the fundraising that we, and other families like ours do, feel so worthwhile.
These new drugs, if they make it to the people who need it (and I have every faith that they will, eventually) will leave only a small minority of people with other mutations that won't benefit from them because of the different problem they have in the baking of that cake. I just hope that the pharmaceuticals (hopeful) success with the majority won't mean a lack of funding on other research for the minority left behind.
... Like Isaac - who has two class one mutations.
In other news, we had an appointment this week to discuss his rather rubbish immunology results, and the news was.... mostly reassuring. Waiting on further blood results now, but every reason to think that this requires no further treatment (which if it had, would mean monthly IVIG transfusions, which we REALLY don't want to add to an already full regime!). And Isaac is feeling great right now and was fab for his bloods. He celebrated with an M&S cottage pie (the one thing he misses from being on the ward).
And just two weeks until puppy Obie joins us! So excited. In the meantime, one ex chicken this morning, resulted in a trip to get three new ones. Welcome Goldie 2, Floella and as yet unnamed white one.
Have a great day x
