New Challenges

Chronic Rejection

At clinic for routine follow-up 4 months ago there had been a very small drop in lung function - made more obvious by having had an all time high the 6 month previously.  I was asked to come back in 3 months, instead of 6 months, just to check. I expected it to be back to usual but instead there had been another drop of about 500ml this time, which was more significant.   My lung function for the 9 years prior has been a steady straight line, never wavering, so we knew something was going on.  I was given another month before review, in which time I had a CT scan of the lungs (showed nothing in particular) and went on holiday with my family.   I returned for review this week and unfortunately lung function had not come back up, and was marginally lower, with me also having lost a kilo in weight.  I was booked in for a bronchoscopy on wednesday and stayed overnight in hospital to get the results yesterday.

They were not the results I had hoped for.   When the doctor said the biopsy had shown evidence of chronic rejection, I did burst into tears.  I couldn't help it.  I have dreaded hearing these words since I had my transplant 9.5 years ago.   I have dreaded them even more since Estelle joined our family, nearly 2 years ago.

However, I guess I also expected it. At some time anyway - it's almost inevitable at some point after lung transplant and I am doing well to be 9.5 yrs down the line before it has reared its head.

I thought it would be better doing a blog to try and explain what this means, as its not necessarily as dire as it sounds, and it is something that is very variable between people.

Acute rejection is something that tends to happen early post transplant -  a process that can be treated with high dose steroids and the decline reversed.   Chronic rejection is a completely different process which causes gradual scarring in the very smallest airways and is not reversible or amenable to steroid treatment.  This causes a progressive decline in lung function.  At 5 yrs post transplant 50% will have this....I'm nearly 10 years so the statistics are closer to 80% (and the survival at 10 years post tx is only 50% anyway, so I'm already beating the odds).   However the speed of progression varies wildly between people - it can be aggressive, very slow or waxing and waning, with periods of no decline.     It tends to be more aggressive when it develops early after transplant and when it starts at a high grade - mines is late after transplant and at the lowest grade just now (e.g my lung function is still high).   What needs to happen now is the process of trying to halt, or at least slow, the decline.    First line treatment is usually a drug with anti inflammatory properties - I am already on this for my sinuses, so we have skipped to the next drug in line.    I will be monitored very closely and the hope will be that the decline will stop or slow.   I will have lung function again in 2 weeks, then 4 weeks at newcastle.   If the new medication is not stopping the decline, then we will progress to radiotherapy - the aim of this is to basically nuke the immune system and try and halt my immune response.

Very little is known about what triggers chronic rejection, and what affects the progression.   Some factors that might trigger this are responses to respiratory viruses (I've had two confirmed this year alone), response to infections,  and acid reflux.   I had surgery to stop acid reflux 9 yrs ago but this will be reviewed if lung function drops again.   There is nothing I can do about the viruses but try and avoid when I can!    I am still waiting on results to see if any infection in lungs (although I think unlikely).   I know my sinuses are heavily infected and might have some surgery for this.

I think all I can do now is keep as well as possible, don't stress too much and eat lots of cake!!  I am disappointed to be facing this, especially at at time when we are enjoying our wee family.  But I will face it with all my strength.  The priority here is Estelle - that she is secure, happy and surrounded by love.  That is my job.   This is what will drive me forward to deal with whatever lies ahead.  I have two angels watching over me, and one angel Estelle making sure I don't lie about dwelling on things!
Life must go on - with as much laughter and cake as I can manage :-)