Up The Farway Tree

Health Update

I was back at Newcastle this week and unfortunately my lung function has fallen again  - by much the same volume (from 2.26L to 2.14L).  It's not declining rapidly but, as yet, has not stabilised, and overall I have lost a litre in 6 months -  so it was quite disappointing.  The gastric reflux tests have all been normal, so we know that is not contributing.  I had my sinus CT scan and am due back at the ENT in a few weeks.   I know the scan has shown chronic sinus problems and extensive polyps.   This wont have triggered the rejection, but the transplant doctor thought that getting the polyps removed would probably be a positive for my lungs.  We will see what ENT think.

So we do now need to move to more aggressive treatment.  Radiotherapy is the standard treatment - to effectively blast the immune cells.  However this would leave me very open to infection (at least during the 6 week period) which is not ideal when  you have a germ-magnet 3 year old at home!  I am constantly picking things up from Estelle as it is.   We also discussed a study that is ongoing at the moment, looking at a drug which is currently used for another lung disease (pulmonary fibrosis) and might be useful for chronic rejection.  However its a double blinded study - which means that neither the researcher, or the patient, knows if they are getting the real medicine or the placebo.  It's a 6 month trial and obviously you can come out of it at any time.  However I'm not too keen on spending 6 months possibly being a placebo, while my lung function continues to fall.   Even if I was on the actual drug, we don't know yet if it will be effective.   I really need to try and stop further decline and maintain my lung function as high as possible.     Finally we had a discussion about a treatment used elsewhere in the world (USA and many European countries).  It's called ECP and doesn't have the same immunosuppressive effects, so less dangerous infection wise.   The big problem being that it isn't funded in the UK for chronic rejection.   It is used for some other conditions - so the technology and expertise exists, but chronic lung rejection is not on that list of approved conditions.   However the consultant thought it might be worth investigating the situation in Scotland and whether I could somehow access any special funding.  I have no idea if this will be possible - my CF doc is looking into this.    It's a treatment that really takes at least 6 months to work and doesn't appear to be as effective in the later stages, so would need to be started soon.    Hopefully I will learn more in the coming weeks.    Radiotherapy of course remains a valid option if I cannot have the ECP.   I will keep you all posted!

Otherwise I am doing ok.  I am not feeling breathless yet, so that is fantastic. I have been very fatigued, which  is a known effect of chronic rejection.  It's also a known effect of being a mum to a 3 yr old and working haha!  I am trying to keep a good balance but life has a habit of getting in the way.   I just want to keep life as normal as possible, for as long as possible, so i'll keep on keeping on!