Plan B

I've not updated for ages because we have basically been operating on a wait and see basis for 4 months!   I have now had 16 ECP treatments, over 4 months and was back at Newcastle for review yesterday.   I have been going down every 4 weeks and the last review the drop in lung function was less than before, so we had all hopes pinned on turning a corner.   However yesterday was biggest drop in a while (over 200ml) which takes me to a lung function of 1.5 Litre (59%) - it was just over 2 litre at start of ECP in April (79%) and 3 litres last year (109%). They would expect stabilisation by this point, and quite frankly, we don't have time to sit about waiting now.   Below 1 litre it is likely I will start to get quite symptomatic and it can now take the radiotherapy 3-6 months to take effect, so likely further decline in interim.

So plan is now for Total Lymphoid Irradiation (radiotherapy).   They basically blast the areas that produce immune cells to reduce the amount being released - the idea being to halt the immune process which is causing the rejection.   The immune process going on in my lungs has just got carried away - and it results in scarring of small airways (irreversible) hence the decline in lung function.

The process will be twice a week for minimum 5 weeks.  It can take longer if white cells (immune cells) drop dangerously low, and then need to delay treatments.  It's balance between slowing down immune response without leaving me wide open to infection.   The main side effect is exhaustion.

I wont be able to go to work and will need to avoid as many bugs and germs as I can.   I am meeting with the consultant next week, so will get more information about the treatment and when due to start.  Plan is as urgently as possible.

In meantime I have ECP next week and will get this anyway (as I might be worse without) then that will be stopped and arrangements made to remove the permcath line.  I wont be sorry to see the permcath go!

I am so disappointed that ECP has not worked out....I had many hopes pinned on its success and also hoped it would be the ideal longer term solution for keeping me well.   However at the end of the day, I am also so glad I was funded and at least had the opportunity to try it - otherwise there would always be a huge "what if?!"

Now I need to shift my focus and accept that plan A hasn't worked and be very grateful we have a plan B!   It needs to work - it's not really an option for it not to!!  I am staying positive and hoping everything happens for a reason and it's meant to have worked out this way.   Onwards and upwards...