Drug Trial

I had my review and bronchoscopy earlier this week.  The bronchoscopy didn't show anything different (e.g no other cause for lung function dropping other than chronic rejection) but unfortunately my lung function was down again.  1.19 litre to 1.1 litre (46% to 42%) in 4 weeks - so similar drop we were seeing before radiotherapy.  I think its starting to look like radiotherapy has been unsuccessful.....although you never know if its at least slowed it a little.   They managed to rush through the final tests I needed for the drug trial, so that I could just get started.   My details are put into a computer programme, which give pharmacy a random number, to correspond with a bottle of tablets.  So no one knows if this is placebo or active drug.   Here's hoping its active drug so that I am at least trying something useful!!  The drug is called Pirfenidone, and is used in pulmonary fibrosis.  This is the first trial for chronic rejection so it's completely unknown if it will be helpful.  It acts by reducing the scarring progression, so theoretically could slow down decline.  Worth a try at the very least.

I was pretty upset by the result, as I was holding onto the hope of stabilisation.  However I wasn't surprised because in recent weeks I have been noticing an increase how breathless I feel.   Starting with such an amazing lung function has allowed me to almost pretend this decline has not been happening, because it's not been impacting on my ability to do my normal things.  Radiotherapy was really the first time it stopped me in my tracks and I assumed once I recovered from that then I could just get back to normal.   I have recovered from the radiotherapy but my lung function has dropped over that period to an amount that has started to impact on me.  I am noticing feeling breathless going upstairs in my house, if there is an incline outside, or I'm trying to run about after Estelle.   It's funny because at this lung function before I was working, going out and about and coping well.....but I think the decline occurring slowly through my life first time round gave me time to adapt....this time its 109% to 42% in just over a year and I think that's why I'm noticing it.   If it were to stop now, then I would adapt to this level and feel I could get back to work and carry on.   I am however terrified of it continuing to drop at the same rate and becoming increasingly breathless and more restricted month by month.  I think its possibly worse having been there before, as I know what it's like.   At least this time round I don't have the constant CF infections though!  The doctor did say that sometimes it can level out a bit under 1 litre, so this could still happen, and again, I know I would adapt.

I also did ask about being relisted for transplant if things continue going downhill.   Obviously we are not at this stage yet, but I need to feel that I have another option to hold onto.  He said the team would look at this possibility when lung function dropped below 30%, probably needing oxygen and also based on level of functioning.  It's all very individual.   I would need to be assessed again but it would be discussed at the time and there is no glaring reason I could not at least be assessed. 

In the meantime I am still hoping for some late results from radiotherapy and also hoping I'm on the active drug and that is does something!  It's mentally hard work at the moment and I don't feel as if I can get back to work and do my job properly, because my head is elsewhere.   This is frustrating as I actually worked when I was very unwell in the past....but then again I'm not quite sure what I was trying to prove.  At the moment I am trying to stay as well as possible for Estelle, who has to be the focus of my energy.  She is also the reason I will continue to fight hard and stay positive.  That and the fact I'm just a bit bloody minded.... :-)