Keeping on keeping on

What is chronic rejection?

So I am a month down the line of being diagnosed with chronic rejection and I was back at transplant clinic yesterday.    Overall it was a good result - my lung function was marginally lower at 2.39 L(as comparison it was 2.43 last time, 2.7 before that and 3.1 L at highest).  So this much smaller drop can be within normal variation.   This is good because they think it probably means it's stabilising just now - but frustrating in that it could also still indicate a very slow trickle downwards.  I so wish it had just been exactly the same!   However I need to be very thankful there was no drastic fall and even if it is trending down, it's very slow.   I think I need to just get my head round the fact it's not going back up!  The doctor said the natural pattern of chronic rejection is a dip, plateau then another dip - so at the moment I just need to be monitored more often so that any dip is caught early on.   I asked more about when they would think radiotherapy would be needed and he said if drop was more than 150ml (this drop was 50ml).  I also asked about how long it could plateau- he was suitably vague.  I asked if maybe it could stay the same for a year - he said theoretically it could, but the biggest problem is exposure to  respiratory viruses and the drastic effect this can have on the progress of chronic rejection.  I know people who have been stable for years though, which is something to hold onto.  I guess the bottom line is: its unpredictable and there are no clear answers. That's not so good for my logical mind - but it does also allow me to maintain more hope - if there are no clear answers then I need to write my own ;)

I know friends and family are finding it difficult to understand what chronic rejection actually is.  It's hard to explain because in reality very little is known about the process and exactly why and how it occurs.   The technical name is Bronchiolitis Obliterans Syndrome (BOS) which really just describes what it looks like in the lungs.  The tiny bronchioles (smallest airways that attach to the little air sacs in lungs) become fibrosed and scarred - causing them to narrow to the point the little air sacs collapse.  Oxygen is passed into the blood in these air sacs so you can see how this process causes a gradual loss of lung function.   Unfortunately it's also apparent why the changes are not reversible - you can't undo the scarring in these airways.  This means I can't get any lost lung function back - so I need to try and preserve it as best possible.  I am lucky that is was so high to start with!

The reasons why the airways start scarring is poorly understood.   The immune system is one of the most complicated areas of medicine and the body will still recognise the lungs are not 'self' despite the immunosuppresion.  Multiple episodes of acute rejection increase risk of chronic rejection in the longer term and exposure to viruses are thought to play a role: the damage they cause in airways cause various inflammatory chemicals to be released - it might be that these chemicals can trigger the process - it also means that further exposure to respiratory viruses can speed the process up.  The lungs are exposed to the environment (compared to other organ transplants) so are at risk of many different insults.   The medication I am on (montelukast) targets a specific inflammatory chemical and I am already on another medication (azithromycin) which can also reduce inflammation in the lungs.   I mentioned before that gastric reflux can also be associated.  The thought is that acid can reflux into lungs and cause an inflammatory reaction and damage.   For this reason I have various gastric tests lined up this month - barium swallow, endoscopy and PH testing (they put nasogastric tube in for 24hrs and measure acid levels).  I've had all these in the past so not worried about any of them - hopefully they will give us more information. 

So at the moment the current plan is to wait on the gastric tests, have lung function review in about 2 months and reassess.   I have worked hard on my weight and already put 2kg on!  Wee fatty me!  I have started a pilates class and looking at what else I can do exercise wise (a little limited by sore CF joints!)   I have had my flu vaccine - as have all my close family.  I have to try and avoid respiratory viruses if possible - this is near impossible with Estelle.   I came home last night and she is full of the cold with a high fever!   You couldn't make it up!

However on a huge positive note, I still feel fine - my lung function is still high so I haven't really felt any different:-)  So I am still working, doing everything as usual and keeping busy.  I probably do need to pace it a little better and avoid getting 'run down' - it's a hard balance.   Life goes on and I am not sitting back and watching it pass by!   Onwards and upwards - the next lung function result will be stable.....because I have decided it shall be.

Now to start getting organised for Christmas!!!