Saturday, March 10, 2018

Update to say there is no update!

4 weeks later..

We are another month down the road and I haven't had funding for ECP sorted yet.  In fairness my CF doctor has been on the case since day one, but the wheels of the NHS can be rather slow.  The first funding port of call was a no-go, so we have now made an application to my health board.   It's a formal process with lots of form filling -the patient can add a statement and I spent an entire weekend composing this.  These applications are discussed at a monthly meeting....and unfortunately we just missed this months meeting, so I don't expect any news until April.  Quite a few people have asked me if I can fund this privately - the answer is that I don't know, and wont know until this process has been completed.  If there is no NHS funding, then I will definitely be fighting to fund this myself.  Having now spent time reading various medical studies, I am convinced this is the best chance I have to stay alive longer.   Not everyone responds to ECP....but my type of rejection is the one that tends to respond.....and this could slow decline and improve survival, without putting me at risk of serious infections.   It's a fairly intensive treatment but would be worth it if I was a responder.

In the meantime my ENT appointment was cancelled because of the snow!!  So no further forward with that yet and not sure when it will be rescheduled for.

Estelle has had slapped cheek syndrome (parvovirus B19) -  it's a common and mild childhood illness - however can be more complicated in adults, especially if immunosuppressed.   We think I might have had this as well - I've not been feeling great for a couple of weeks now.   I have had muscle/joint pain, mild fever and exhaustion.  The fever is gone but I am so tired all the time!!   I suspect the whole emotional strain is playing it's role of course.   I've had some bloods taken so we can see if that's the cause.  I'm glad I wasn't having radiotherapy because these are the exact risks I am trying to avoid by pursuing ECP.  I can't expect Estelle to stay away from me when she is not well - it's just not possible!

I am back in Newcastle at the end of this month.   I know my lung function is down a bit again (measured locally) but I was expecting that.  I am trying to stay positive and keep living life as normally as possible, but it's hard when I don't know what's happening and feel very out of control.  I'm hoping that I will get some news about funding within the next month though, then maybe we can move forwards either way.   I will update when I know what's happening myself!

Friday, February 09, 2018

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!

Sunday, December 31, 2017

2017 in Pictures


I went back to work after a years leave.  Estelle started nursery.  We spent the rest of the time inside the playhouse built in the living room..


I was permanently exhausted this month - being back at work (which was pretty full on) and juggling being a mummy and a doctor!   Inevitably things happened like leaving the freezer door open and having to cook its entire contents.....


A mixed month!  Both myself and Estelle had parainfluenza virus - I was off work and Estelle ended up with pneumonia!  She has since taken a keen interest in listening to peoples lungs with her stethoscope!  On the positive, we had a lovely trip to Manchester and Estelle has never stopped talking about her friend Marco since...;-)


Birthday month for me, mum and dad!  Also celebrating 9 year transplant anniversary.  Lots of cakes were eaten.  Then we jumped off all the calories on the new trampoline (Estelle's belated 2nd birthday present!)


Had a lovely family holiday in the Lakes - Estelle had an absolute ball!   Lots of outdoor fun.  Also some lovely weather at home - picnics in the garden :-)  Newcastle clinic noticed a slight drop in lung function, but I wasn't worried as it was still within range at that time.


Estelle loving the Bathgate galaday parade, then cheering Andrew on at the Fathers day 10K.   I had my 15 year university reunion!!!  Poor wee Seb had some health problems and needed blood testing - sporting his wee tractor bandage!


Had a lovely wee mini break in Hawick!  Some great weather this month- lots of garden fun!


Estelles first time on a ride, with her friend Rhiannon.  At Seth's baking party!  Me and Andrew having a day out in Edinburgh for our 13th wedding anniversary.  Another newcastle trip which showed another drop in lung function - I was pretty worried this time.  I was asked to come back in a month so they could do bronchoscopy if another drop.


Fantastic family holiday in Majorca.  Estelle is a total water baby and loved the pool and beach.  This photo was taken on Frances Ann's anniversary - both wearing out butterfly dresses in memory.  Was back in Newcastle at end of the month and unfortunately needed bronchoscopy due to further drop in lung function.   The biopsies found chronic rejection and I started on some new medication.  I was devastated with the news, but thankfully Andrew was with me, so that made it easier.


I was feeling quite stressed with the chronic rejection diagnosis, but tried to make some positive changes - starting pilates and also building up my weight.  Victoria's friend held a fantastic comedy night in her memory.  I finished off jewellery she had started making, which we sold in aid of the butterfly trust, and it was an honour to contribute in this small way.


Early start to Christmas festivities with a visit from the Levinsohn's!   Transplant clinic indicated that although lung function still a wee bit lower it looked to be stabilising slightly.   I made some changes at work to try and make sure I can stay as well as possible!! 


We had a lovely Christmas this year - with Estelle really understanding things for the first time. She loved singing at the vigil mass - clapping along!  Then she had great fun setting out her tray for Santa and the reindeer's.  What she doesn't know is that after she went to bed, Seb helped himself to the pancake and drank half the glass of milk hahaha!  She couldn't quite believe on Christmas morning that Santa had brought her the dolls house she wanted.  It was magical to see!

Looking ahead to the year ahead, I do feel anxious about my health and what might lie ahead.  However, realistically, that has always been the case - it's just that in keeping so well after transplant you almost start to become complacent and believe that you are indestructible!   I am grateful  to have an amazing transplant team who are monitoring me closely and making sure I stay as well as possible.   This coming year will mark 10 years since my transplant and I am forever in debt to my donor for this miracle!   Estelle has enriched my life beyond belief and makes me more determined than ever to stay well and to keep living life to the full.   I am surrounded my family and friends who provide more support than you can imagine.   I am very fortunate indeed and will go forward into 2018 with gratitude for what I have, and hope and faith for what is still to come.

Tuesday, December 19, 2017

Merry Christmas!

I was back in Newcastle for a pre-Christmas check up.  Results were ok-ish.   Lung function was down a little again (2.26 litre from 2.39) which was a bit disappointing...but could have been much worse.  The doctor felt that since I was feeling well and had also recently had sinus infection (which might have caused the drop) then he was happy to leave it for 2 months and review.   He didn't feel it was time to proceed to radiotherapy, which was good!  He said that the pattern of slow decline at moment indicates this is not an aggressive rejection, where things just plummet downwards.  Although the expectation is still for gradual decline, I might also have periods of plateau and they will still progress to radiotherapy if looks like my lung function is dropping to point I am becoming symptomatic.   At the moment it is still 86% (was 109% at start of this year) so I don't notice any difference on a day to day basis - I don't feel breathless at all.   I am very fortunate that I had such a high lung function post transplant as it is buying me more time in this process!

I have ENT appointment this week about my sinuses so will see if there any further thoughts on surgery.   The gastric tests so far have been fine - I've had a barium swallow and endoscopy.  I still have a PH test in January (they put nasogastric tube down for 24 hr which will measure any acid reflux).  I think it's looking like my previous surgery for reflux is still working, which is good news.

I have been feeling very anxious about the whole process though.   I think it's the sense of something not being in my control, and that no matter how hard I try, I have little influence over the progression.   Pre-transplant my CF lungs were unpredictable but my lung function varied day by day, depending on infection levels, so there was always a feeling that it might go back up at the next appointment.  I am finding it hard to accept the irreversible nature of this process.   However, the difficulty with CF lungs was how awful infection made me feel - at the moment my lungs are infection free and therefore I actually feel very well.   So I think all I can do is make sure I am otherwise as fit as possible, so that by body copes with any drop in lung function.  Within my control are the general looking after my self principles - I must remember to stick to these!  

I need to look at this whole process as just another part of the journey.  I don't want to spend the time between appointments getting  increasingly anxious and fretting about what the next result will be. At the moment I do feel well - and I really must make the most of that.   Life marches on regardless and I would hate to look back and think I spent far too long worrying!  It certainly doesn't achieve anything.  I have to concentrate on the fact that things might remain stable for a good while  and I will deal with each hurdle at the time.  

Health aside - it's only 6 sleeps until Christmas!!!!!!!!!   Estelle is very excited - but I think I am still more excited haha!!   She has asked Santa for a dolls house.   I quite like the idea of playing with a dolls house too.... :-)     I am looking forward to spending lots of time with my family over the festive period.   I am forever grateful for my wonderful friends and family who support me at every turn, and I am of course, forever grateful to my donor for giving me another Christmas to celebrate!!

Hope everyone has a  magical Christmas!

Wednesday, November 01, 2017

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!!! 

Friday, September 29, 2017

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 :-)

Saturday, December 31, 2016

2016 in Pictures

I have good reason to have not updated this blog in a year but have managed my end of year update - if only for me to look back on in the future!


We hit the ground running with parenthood.  Estelle is an absolute delight - but she was also a mobile, energetic one year old and we didn't have any slow introduction to get acclimatised.  I don't think either of us had ever felt so tired, and I lost about a stone in weight from just being on my feet all the time!  We faced all the new challenges head on, and Estelle began to settle into her home, her family and it started to feel as if she had always been here.


We were delighted to attend Corey and Monika's wedding as a wee family.  Estelle's first wedding and party!  She coped well with the flight to London, staying at a hotel and meeting lots of new people.  We were glad to have my mum, dad and Sarah with us to help!


Estelle started to climb, balance and get herself into all sort of precarious positions!  This photo is a good representation of that month.  She moved her rocking horse over so she could stand (and rock) while playing on her keyboard.  This was a nightly activity - usually when we mentioned bed time....!!


Here is Estelle with her first pair of proper shoes!  She had been cruising round furniture, standing on her own and climbing for months...but it was around this time she was properly walking.  She seemed to be growing so fast!   It was also my birthday - mum watched Estelle so we could go for dinner.  I think we were back by 6pm and in bed by 9pm ;-)  Our new rock and roll lifestyle!


Estelle's first trip down to Hawick for Ross' 40th Birthday this month.  She was in her element playing with Andrews cousin's children.  She had a ball!  We also had some lovely weather in May, so a lot of time in the garden.   Here she is looking for Seb - who is hiding from her in the bushes! Poor Seb didn't quite know what had happened when this whirlwind arrived.

We also had a family photo shoot courtesy of my friend Kirsty.  Estelle was impeccably behaved -posing away. Seb on the other hand, managed to get into the staff kitchen, ran wild and acted like a complete diva.


We had a week of amazing weather, which coincided with Andrew having a weeks holiday from work.  We did lots of day trips - to Luss, Briarlands farm, Blairdrummond safari park and Loch lomond shores.  The photo is Estelle wading into the freezing cold loch at Luss - because she wanted to be in with the ducks.  There was also much fun in the garden and we ate dinner outside every night, which Estelle thought was great fun.  She had her paddling pool out and was absolutely wild in it!


We attended our good friends Barry and Louise's vow renewal party - Estelle was in her element!  Up on the dance floor and running wild :)  We had a couple of days in Hawick and also managed to attend our friends wedding, while mum looked after Estelle.  That was the first and last good nights sleep of the year haha!


We had a family holiday to Arran, and were lucky to have amazing weather.  Anne and John came with us and Estelle had a fantastic time.  Here she is feeding the ducklings, which was the high light of her holiday!


Estelle's Christening- what a lovely day we all had.  Uncle Hugh baptised her, which made it even more special. Estelle had a fantastic time at her party afterwards, and for the next month did nothing but talk about it!

Estelle also discovered one of her favourite things to do was walking Seb.  Puddle suit, wellies, and puddles to jump in  = one happy girl!


A month of lots of autumn walks, visits to the play park - for the swings and also lots of quieter days inside learning new things.


There is little to  say about this photo, other than her mad aunty Sarah is responsible for the panda suit.  Seb was very accepting of it.  I think he was glad someone else was getting dressed up for once..!


Estelle has loved all the Christmas buzz - the tree, the lights and the parties!  We had a weekend of festive fun with our lovely friends the Levinsohns and also a family day out to santas grotto at lomond shores.  Estelle had an absolute ball on Christmas day, then more celebrations with Andrew's family on boxing day - followed by her birthday yesterday!  She thinks that you get to open presents every day!  What a lovely ending to a fantastic year.   All I want for 2017 is for all my family and friends to have much happiness and joy :-)  For Estelle, I want her to just be herself - the beautiful, funny, clever and kind little girl we all love.