Stable Health

Apologies for not updating sooner - I've had 2 Newcastle visits since I posted in September, but have been somewhat distracted and keep forgetting to post.

The good news is that both October and November visits showed the same lung function (around 35%), so that makes 4 months now with similar results.   Makes a nice change from the monthly drop I had before then.   I am feeling the lower function when I'm walking, trying to climb stairs etc but am lucky that it seems to have stabilised at a level where my oxygen levels are ok - so I am not needing any additional oxygen.  This is a huge benefit!  I'm also getting used to this level and knowing what I can and cannot do, which makes it a bit easier to plan and pace myself.   I have no idea at all how long it might stay at this level, and neither do the doctors.  The main aim is to avoid infection, which might set things off again.  It's hard to avoid all the viruses going about but I'm doing not too badly so far this year.

With regards to learning to cope at this current level and get the most out of life - and give the most to Estelle - I have come to the difficult decision not to return to work.  I had been off for a year past September - intially only going off short term for the radiotherapy, but then being unable to return as lung function continued to fall.  It's been a really difficult time making this decision and I've been weighing up options for months in my mind - going over and over the pros and cons.  It was finally a helpful discussion with Occupational health that crystallised my thinking - he spoke about risking the stability by trying to force a return to work, and I actually felt relief that someone was saying it would be ok not to go back.   Don't get me wrong - I love my work and it's a huge part of my identity - but more importantly I am a mum.  I need to be here for Estelle as long as I possibly can, and I owe it to her to look after myself as best I possibly can.  I also owe it to myself to give myself a break and to stop feeling like I have to prove myself worthy somehow.    I made this decision about 6 weeks ago, and although I cried for a week, felt anxious and experienced a great sense of loss,  I also gradually came to terms with where I am at the moment and what it most important in my life.    It doesn't mean that all doors are closed and if I remain in this semi-stable state, then I could consider some other medical interests to keep my mind active.   I don't really think I will be short of things to do!  So far its allowed me to go to every event at Estelle's school and she absolutely loves that :-)

The other reason I have not posted over the past month, is that we sadly lost my wee dog Seb on the 3rd December.  He became unwell with acute pancreatitis about 3 weeks before and it was a turbulent period full of ups and downs as we tried desperately to get him back on an even keel, with lots of admissions to the vets for treatment.  He actually did recover from his pancreas but the whole episode knocked his kidneys off further, which have been very poor for the past few years....the vet said it was remarkable his kidneys had actually coped this long.  He was 15 yrs and 2 months - to be honest, whatever age he lived to was never going to be enough for me.  He was my best wee bud and he was with me through my many life events over the past 15 years.   I loved him more than I thought it was ever possible to love a dog and am completely devastated.  I feel very lost without him.  I used to say to Andrew when we met (and I thought I would never have children) that all I ever wanted was a puppy dog to love me.  And I certainly got that and more.   I will dedicate a post to Seb and his antics, funny ways and total attitude, when I feel more ready to look back.  There are many stories!

In spite of the stresses of recent times, I am still enjoying the run up to Christmas with Estelle and Andrew.  Estelle is just the perfect age for enjoying the magic of this season and hopefully, like me, will never outgrow it.  We have been to see Santa in his woodland cabin, had a day in Glasgow looking at the lights and markets, saw the real Cairngom reindeer that came to our town, pulling Santas sleigh, had two school Christmas parties and tonight we saw the local pantomime.  I still have Estelle's nativity to look forward to - she tells me she is an Angel because there was no way she was being a sheep haha.    Only 10 sleeps until Christmas!

Update

I've had a couple of Newcastle visits since my last blog.   The end of the first 6 months of the study was last month.   That appointment did confirm the Glasgow result, which had been a slight drop from July.   However reassuringly yesterdays result was the same as August, so there hasn't been another drop in the past month.

At the last appointment I was put onto the active drug - having been taking either the drug or placebo for the past 6 months.  Even the doctors don't know what I was on.   However I had to go back to the starting dose and retitrate again.  On the lowest dose, the nausea and fatigue were so much better, then when I went to the middle dose the nausea came back, and on the high dose the fatigue also became extreme again.  It's so overwhelming - a couple hours after taking the tablets I'm ready to go to sleep and feel awful!  The meds are three times a day, so this isn't ideal.   It does confirm to me that I have been on the active drug all along, as the side effects are identical to what I've been experiencing over the past 6 months. 

At yesterdays review we discussed these difficulties.  The doctor felt that overall my lung function has been more stable in the last 6 months.  It has still slowly drifted down but SO much slower than the previous 18 months and some months there hasn't been a drop.   There are a potentially a few reasons for this - it could be the trial drug, it could be late effect from radiotherapy and it could simply be the natural history of chronic rejection, which tends to become more stable at low lung functions under 1litre.   Given that it might not be the drug that is helping and the fact the side effects are impairing me more than my low lung function, we have decided to reduce the dose for this month and see how I get on.   I am anxious about any negative impact on my lung function but I am also really struggling with the nausea and fatigue, and spending a lot of time feeling quite rubbish.  I was determined to tolerate this for the past 6 months but it does start to really wear you down!  They have only had 2 other patients on the drug and neither of them could tolerate taking it.  I need my energy for Estelle and for just living life, so think this is worth exploring.

I'll be back down in another month, so keeping my fingers crossed that I'm coping better with the side effects at this stage, but without any negative impact on my lung function.  Onwards!

No news, is no news!

I'm aware I haven't updated the blog for ages!  I forgot to do it after my last visit and then time just ticked on.   My last newcastle visit was in June and showed no drop in lung function at that time - so that was 2 results the same, and the month before was very similar.  I was feeling cautiously optimistic that we might be reaching some stability!

Instead of travelling to Newcastle for the July visit, I had lung function checked locally.  Unfortunately this showed a drop in lung function from 0.97 to 0.89l (35%).   However in the past I have noticed variability between Glasgow and Newcastle results, so I am very unsure if this is a real drop or not!  I almost wished we had just travelled to newcastle!   I have been feeling more breathless but also wonder how much the weather is affecting this at the moment.  I love hot weather but I'm sure it must have some effect now my lung function is lower.

My next visit to Newcastle in August is for the end of study tests....so I will get the full extended lung function testing and repeat the 6 minute walk test (this shows how far I can walk in 6 minutes and whether oxygen levels drop).   I expect it to be worse than the start of the study but really really hoping lung function is still in same range as June visit and Glasgow was reading low!  They wont tell me whether I have been on the active drug or not, but I will be started on 'open label' drug - e.g I'll know I am on the active drug.  It will have to be re-titrated, so I will know if on the lower dose I stop feeling sick!  Although there was some chat in May about not being on the drug, I really do think I am.  The sickness has been ongoing and very obvious if I take the tablets without enough food - it just seems too coincidental.  The fatigue is also ongoing but I am getting used to the lower energy levels, although find it frustrating, as it wasn't that long ago I felt able to do most things!  

Otherwise I am ok - not back at work and not sure whats happening with that until we have a clearer picture.  Estelle is great and is starting school next month - so I have plenty to focus on other than health :-)

I will update once I have my end of study results :-)

Hope

I was in newcastle yesterday and I felt the news was a bit more positive.  There had only been the tiniest drop in lung function by 1% to 37%.  This is the smallest drop since I was diagnosed with rejection.  There had been previous discussion about some people stabilising under 1litre lung function (mine is now 0.96l) so it could be following this trend.  It could be related to the trial drug - although interestingly the consultant I saw yesterday was less convinced that I was on it.  He thought the ongoing nausea might be explained by one of my anti rejections, which was changed one month before the trial.  He would have expected more stomach upset from the trial drug....but of course not everyone gets this, so who knows!!  He also did emphasise that this is the first trial of this drug for rejection so we actually don't know at all if would have any effect.  Either way, I finish the trial in August and will be put on the active drug at that point so will soon know if I have same side effects.    One of the biggest issues just now is fatigue and exhaustion - we are trying a different anti sickness medication to see if that helps, because the one I have been taking can cause drowsiness.  Fingers crossed this helps.

I saw occupational health regards work and they agreed that I wasn't fit to be at work just now.  I'm finding it hard enough to get through a day doing very little without feeling exhausted.  I'm definitely more breathless now, but of course I've been much worse in the past.  I've not given up on the idea of going back though and hope that if I get some stability, and can sort out the fatigue, then I might be able to work something out! 

I'll be back down in June and keeping everything crossed for only tiny drop ....or even better, no drop. 

Big Birthdays!

I was in Newcastle on 9th April but haven't had chance to update properly since then because it was both my 40th birthday and mums 70th birthday a few days apart!   40 is a milestone indeed - when I was born people with CF were not living beyond childhood, never mind reaching 40!!  I am forever grateful to my donor who has given me the chance to reach this milestone.   I feel like it was only 5 minutes ago I was 18 and leaving school!   It was lovely to catch up with friends and family for my birthday and I am overwhelmed by the love and support :-) 

I appreciate all my wonderful gifts.  One of the most special has to be the necklace my mum got me - it's three interlocking rings with Estelle, Andrew and Jacqueline on them.   It's even more special because unbeknown to each other, I also bought her the EXACT same necklace for her birthday - but with Jacqueline, Frances Ann and Claire.    How spooky is that???   It worries me greatly that we are on the same wave length......!!

With regards the clinic visit, I had another small 50ml drop, taking my lung function down to 38%.  I'm glad it wasn't a bigger drop but I am desperate for it to just stay at one level for a while and give me time to adjust.  I am definitely feeling the drop more and I think it's harder because I am not getting time to adapt.  I have decided to keep going with the trial drug at full dose, even though I'm still feeling quite sick and really tired.   The doctor felt by the summer I might have a better idea if its doing much.  It's near impossible to tell, as we don't know what it would be doing on its own.

I'm still not at work and this is getting me down quite a lot.  There are days (or parts of days) where I think I feel ok and could be at work...then I suddenly feel really sick, or need to lie down and end up napping for hours, then I wonder how on earth I would cope.  The consultant said he thought I was better staying off at the moment, as the most important thing is trying to get some stability.  This is part means avoiding infections etc - I had a chest virus recently which really hasn't helped.  It's near impossible to avoid though!  I am not making any big decisions about work and just giving it more time - I am unsure if the drug trial is causing a lot of my general ickiness but obviously don't want to give up on this too soon.  It's hard to work out a reasonable work plan when every time I visit newcastle I have lost more lung function.     I guess I just have to do what feels right at each stage and not think too far ahead.

I'm back down mid May - and hopefully with some nice spring weather and lots of easter chocolate, it will be a good visit :-) 

Ever Hopeful

So in Newcastle just now waiting on pharmacy to dispense 2nd month of drug/placebo.  I am 99% sure I'm on the active drug because am experiencing significant side effects.  Mostly nausea, headaches and fatigue.  The headaches and fatigue could just be part of life at moment but the nausea seems more surely a drug effect.  I've tried a couple of different anti sickness meds alongside the tablets.  This is taking the worst of the effects away but in all honesty I've not really felt great at all over the last month.  I also keep falling asleep which is not ideal!

Lung function today was down about 50ml to 40%.  This is less than last drop but wee bit more than month before.  It's very hard to know if it's slowing the decline as I've had similar drops in past, then followed by larger drops. Only time will tell! However let's just decide to take it as a good sign..

I was given option of reducing dose of medicine but am determined to keep going at full dose if I can... Even if just to establish whether it might be helping. It's a difficult one as I don't want to feel miserable all the time but I also don't really have options! I'm hoping with time the side effects might lessen or at least I can work around them if I know what to expect and when.

I'm feeling a bit fed up with the uncertainty and just not feeling myself of late. I'm not fit for work which makes me feel a bit lost, but I also know that work would not help the fear of the future and make me more tired. I have a lot to learn about patience!

I shall keep going and see what comes from the next month of treatment and be thankful for the fact it seems I'm on the active drug.... At least I'm getting a chance!

I'm back for review in a month and in the meantime we will celebrate Andrews 40th birthday and try to make the most of the good times!!  Onwards we go!!!

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.... :-)

Belated Happy New Year

I didn't realise it was quite so long since I last blogged!  I didn't do my usual year in photos summary - largely because this blog is currently open (i.e no sign in) so I think its best not to share personal photos :)  It was also hard to sum up the year - 2018 was quite hard going treatment wise and I spent the entire time just praying for my lung function to stop dropping!  I did still have a good year though - some lovely family holidays and the ongoing privilege of watching Estelle flourish and thrive!

I had a review appointment a couple of weeks ago and the results were a bit disappointing, but could have been much worse.  My lung function was down again, but less of a drop than it's been for a while.   Over the past 18 months it has dropped on average 100ml per month (at most 200ml and the least being 50ml).  This time it was a 40ml drop.   So this is hopefully a sign that radiotherapy has done something!  However what I need is it to stop dropping altogether, and the doctor was concerned it could be an ongoing trickle down.  We decided to change one of my anti rejection drugs to see if that could suppress the immune reaction a bit more - so far it's making me quite nauseated but I am hoping that will settle when I get used to it.   The doctor also wanted to enrol me in the trial I had mentioned before I started ECP.  Although I might be on the placebo, I don't really have any other options just now so it's worth a go.  I also believe that after the 6 month period, I could get the active drug for a year.  It's a drug used in pulmonary fibrosis to reduce the lung scarring - which is also part of the chronic rejection process, so may be helpful.  I'm willing to do anything to stop the decline!  In order to assess suitability I need a lung CT scan (done today) and bronchoscopy, which I will get in few weeks  - and also will get further lung function at that time.   So it's back to the waiting, hoping and praying for better results next time.

In the middle of this we also discovered the radiotherapy had induced an early menopause (as ovaries are near the radiation field).  I was glad to get this diagnosed as have been feeling pretty awful and wasn't sure what was going on!!!  I feel a lot better on HRT, which I will need to protect bone health..as well as my sanity!

I'm still not back to work yet.  I had hoped to return after my most recent appointment, as I had been positive it would be a good result.   As several tests needed done I have decided to stay off until these are completed and hopefully I have a better idea of what's next.   Physically I am definitely improving from the radiotherapy - I am less tired than pre-Christmas and generally feel better, although do feel more breathless on exertion now.  Mentally, it's hard to be in a constant state of uncertainty, while watching my lung function dwindling.  I've had much lower in the past and know I will somehow cope if it keeps dropping....but you get used to the amazing feeling of normal lungs after transplant and that's hard to give up.   So I want to get back to work, to feel more normal again....but I am so acutely aware that if my lung function keeps falling, then work will become very difficult to manage.   I know I will need all my energy and focus for Estelle!

I don't have any new years resolutions - why burden myself with extra tasks!  What I hope for though, is a return to normality.  To have stable lung function, to not worry constantly about what's going to happen next, to be back at work, to have something more interesting to talk about than health!   I don't want an exceptional year, or an exciting year or a year of great achievements....I just want a boring, normal, no drama year!!! 

Festive Vibes

I was back down for transplant clinic 10 days ago for my first review since radiotherapy.   My lung function was down from 1.5 litre (59%) to 1.29 L (50%).   This doesn't really tell us anything about radiotherapy success yet, as most of this lost could have been pre-treatment.  The consultant is hoping it might start to stabilise at this level and it will be reviewed again in January.   I'm going to be pretty nervous at that point as I desperately need to see some plateau!

I had been starting to feel better after the cold type virus and was actually feeling not too bad for clinic.  Unfortunately I was only recovered a few days and have not been well since.  It started with what I thought was a terrible migraine last weekend, but then I developed a fever.  I got checked out at clinic and my inflammatory markers in my blood were raised - they thought most likely viral cause as the cells that fight viruses (lymphocytes) were very low.   I've continued to have headaches for the past week but the fever is settled now and my bloods are much better.  It's unclear whether my sinuses might also be contributing but hopefully whatever it is will be 100% gone soon!

I did manage to get my tree up last night and Estelle is getting really excited about Christmas now.  They had a Santa parade locally, with real reindeer pulling the sleigh.  I was more excited than Estelle about the reindeer haha.   I am hoping all these viral things clear for Christmas and I have some more energy to really enjoy the festive period with my family.   It wont be long before I can start counting down the sleeps...

Slowly Slowly!

It's now just over three weeks post treatment.   Overall there have been some improvements in my energy levels - I haven't been napping during the day and sometimes making it past 8.30 before going to bed haha.

Unfortunately I've had a nasty cold virus over the past 10 days and it's taking a while to fully clear - which is not surprising given my immune system is suppressed.   Estelle had something similar for a couple of weeks so it's probably the same thing.  It's left me feeling a bit chesty at the moment and short of breath - so it's even harder to assess how I am overall.    It's horrible feeling short of breath though....brings back a lot of memories.   Hopefully it will clear up over this week and I will get a clearer idea of how things really are! 

I am however definitely doing more around the house and not feeling quite as brain dead.  Estelle announced that she thought I was getting a bit better, so I must be moving in the right direction :-)

Now I just need to be tip top for Christmas!!

Aftermath!

It's been 10 days since my last treatment now and I'm still exhausted.  In fairness the leaflet they gave me did say that the symptoms might be worse for couple weeks after finishing, as the radiotherapy is still acting.  I've still been having nausea and reduced appetite, with things tasting a bit weird, although this is maybe getting a bit better.  Still can't drink cappuccino though haha.

I'm still struggling with the fatigue.  This is the side effect that can last the longest - having spoken to a few people who've had the treatment, it's not uncommon to last a few months.  I've been trying to do a bit more (e.g took Seb along our street last night) but find that even small tasks take it out of me.  I'm also finding my concentration is poor.....as if my brain is tired and fuzzy.  I've not been meeting up with friends or family because of this, and feel quite happy just relaxing on my own at the moment.  I am hoping level of fatigue will start to dwindle soon, even if it might take longer to be back to 100%.

I am back in Newcastle on the 22nd November, although as I mentioned before, we expect the lung function to be down from last review in August.  Hopefully it will give us another baseline to work things out from, but the stabilisation might not be evident until well into the new year.

I'll keep on keeping on!

One week to go...

I have completed 4 weeks of treatment and this is now my 5th and final week!  I can't say how relieved I am.   The last week has been increasingly difficult and I more or less spent all weekend in bed.  The exhaustion is like nothing I have experienced before.... those many nights spent awake with Estelle as a baby don't even come close!  I have developed a new ability to go into coma like naps, where I don't hear the door bell or phone ringing.  My limbs feel so heavy, as if filled with lead weights and I'm finding it hard to think clearly.   It's a very odd experience and frustrating for someone who is used to being a busy bee.   The days immediately after treatment are the worst, so today will hopefully be slightly better, before treatment again tomorrow.  I have spent most of my time watching netflix, but I couldn't even manage that yesterday.

The last 3 treatments have been difficult, as they have been having problems with my alignment.  Procedures are taking over 1 hr and on Friday we had to abandon after 1hr 20min as they just couldn't get me lying in the right position for treatment.  After discussion with the doctor and physics department they concluded that the fatigue was changing the way I was lying on the trolley.  I think during the original scans I was probably lying in a more tense position - holding my spine away from the cold and hard trolley, and could maintain that for the 40min.  Based on original scans, various lines were drawn on me with permanent marker, to help align future treatments.  So basically these lines were now inaccurate as my position was different.  They work in millimetres, so any change throws everything off.   They need the delivery of radiation to be very accurate, so that they hit lymph nodes only (which are tiny) and don't damage nearby organs.  Therefore they redid the scans and markings on friday afternoon, and did treatment after this.  So hopefully these new lines will help the last 2 treatments run smoothly!

I am so looking forward to finishing now.  Although I know it will take some time to recover, at least I know I will be improving from that point.  I have to phone transplant team to sort appointment once finished, but this will probably be another month away as there is no point assessing too soon.  I'm glad there will be a little break, as I am nervous about the next assessment.  It's hard for me to know what my lung function is doing, as everything seems hard work just now....but that might not be my lungs as such.  I haven't been able to walk Seb or do things that would give me a better idea of how my lungs are coping.....hopefully that can start to build up after I finish.   I have managed to maintain my weight so far - despite a lot of nausea.  I am taking some high calorie supplements to try and keep me topped up!  My blood tests have also been good - which shows my bone marrow is coping with the treatment.  I had to get some extra bloods on Friday, but since no one has phoned I assume they were ok for tomorrows treatment to still go ahead.  I also seem to have avoided having any skin reactions, which are apparently quite common.  All little positives to focus on.

Estelle is coping well with all the changes and her mummy being out of action.  She is enjoying the daddy and granny attention!  She was with us at the hospital on friday for 5 hours and behaved really well.....better than I did haha!  It's hard for her to understand what's going on but she is looking forward to me finishing treatment and being back to normal!

Here's to one more week....!!!

Total Lymphoid Irradiation (TLI)

So I have finally started my radiotherapy treatment and this is week 2 (of 5).  It seemed a long wait to get going but I am sure it will be over before I know it!  I think its quite difficult for people to understand what exactly I am having done, as you most commonly hear about radiotherapy in the context of cancer.  So I thought I would blog about what it actually is!

What is Total Lymphoid Irradiation?
It's where radiation is applied to all the main groups of lymph nodes in the body - these are found on the sides of the neck, under arms, chest, abdomen and groin. The spleen is also included.

The lymph nodes are major part of immune system and contain lots of immune cells.  The purpose of nuking them with radiation (basically x-rays at higher dose) is to temporarily destroy immune cells with the aim of suppressing the immune response. 

What's that got to do with chronic rejection?
Chronic rejection after lung transplant isn't really that well understood, but it's to do with my own body recognising the lungs as foreign, and mounting an immune response (e.g essentially attacking them).   This immune response can get carried away, causing ongoing damage to the lungs.  The radiotherapy aims to dial down the immune system and hopefully switch off this errant response.  The balance is to suppress the immune system enough to stop the process which is damaging my lungs, but not suppress it to the point I can't fight infection.

Is it the same as radiotherapy for cancer?
It's the same machine, but with a lower dose of radiation - because the aim is not to kill cancer cells but to dial down the immune function.  Cancer treatment is normally targeted with high intensity at one part of the body, where as this is lower intensity but over a very large area.

What is the treatment like?
I have to lie on a metal trolley - this is not good for bony backed people like me!  I have a mask to wear over my upper torso (this was made before treatment to fit me)  - the sole purpose is to keep me in an exact position.  It is clipped down to the trolley on either side, so you really can't move!  It's not sore, but very snug fitting. 

I also have various crosses and lines drawn on me with permanent marker (covered with dressings) that will remain for treatment - these are for getting me in the right alignment.  They then take scans of all the treatment areas at each session - to ensure everything is lined up to target lymph nodes accurately (and to avoid my organs).   They then they move on to the treatments.   The actual radiation treatment only takes minutes per area, but because of the scans, I have to remain in the same position for about 40 mins total. I am finding this quite hard!  I tend to recite various lists in my head (e.g all the animals starting with 'a' I can think of).   Otherwise I start thinking about the mask being tight, feeling the trolley digging into my back, and getting overwhelmed with the urge to move!  I can't wear headphones as the mask covers my ears, but they did say I could bring a CD and they can play it, so will maybe try this.

I will have 2 sessions per week for minimum of 5 weeks - sessions could be delayed if my immune cells drop too low.  I get bloods checked weekly and see the consultant once a week.

What does it feel like?
I don't feel anything when they do the radiation treatment.  It's just like getting an x-ray.  I am not 'radioactive' afterwards.  I just feel stiff from lying so still!

What are the side effects?
The main side effect is exhaustion and fatigue.  This occurs in all types of radiotherapy but more often in TLI due to the large area being treated.  It's not well understood why it occurs.   I wasn't too bad the first week but am really starting to feel it now.  It's an odd feeling - like the sense that you your battery is drained and I just can't shake it off.   I have a feeling I might be quite wrecked by the end of this!

Radiotherapy side effects also depend on areas of body being treated - because lots of areas are being treated this can be quite varied.  At the moment I am having some problems with nausea - probably worse because of nodes in abdomen being treated.  I've got meds for it but haven't been able to drink coffee.....this is an emergency for me haha!!   My taste has also been really strange (apparently salivary glands are sensitive to radiation). 

What happens after?
It takes a while for the body to recover after and for the fatigue to pass.  The consultant suggested about 6 weeks to recover post treatment, but it varies a lot between people and it will also depend on what's happening with my lung function.

How will we know if it's worked?
The only measure of whether it works will be my lung function.  I have not had this checked since August clinic - because the treatment plan would be the same regardless of the current number.  To be honest it's quite nice not knowing what it is at the moment - it's so distressing watching the numbers fall when I can't do anything about it.    The plan will be to get reviewed by newcastle after treatment ends.  At this point we would expect my lung function to be lower than last measure  - because it will have fallen in interim period.  The actual effects of stabilisation could take 3-6 months post treatment to occur, so I can also expect further decline in this period.   If the radiotherapy works then my lung function will hopefully stop falling (fingers crossed stopping at a level I can still function well) and in some cases might improve a little. 

What if it doesn't work?
I'm not scared of radiotherapy but I am scared of it not working.  If my lung function continues to fall despite treatment, then there are not really other options.   How quickly it falls is an unknown entity - it could randomly slow down/speed up for unclear reasons. Although I am always hopeful that new treatments become available!   People have asked me about second lung transplants.  These are rare but not impossible.   Second transplants are complicated  - the surgery is more difficult, the matching to donor lungs more complicated and it's generally high risk. I have however made it really clear to my team that I would want to be assessed and considered for this option if it came to this. 

However,we are a long way from this stage and at the moment my focus is on radiotherapy working.  It simply has to!!

I will blog more when nearer end of treatment..  but any questions please just ask!

Plan B

I've not updated for ages because we have basically been operating on a wait and see basis for 4 months!   I have now had 16 ECP treatments, over 4 months and was back at Newcastle for review yesterday.   I have been going down every 4 weeks and the last review the drop in lung function was less than before, so we had all hopes pinned on turning a corner.   However yesterday was biggest drop in a while (over 200ml) which takes me to a lung function of 1.5 Litre (59%) - it was just over 2 litre at start of ECP in April (79%) and 3 litres last year (109%). They would expect stabilisation by this point, and quite frankly, we don't have time to sit about waiting now.   Below 1 litre it is likely I will start to get quite symptomatic and it can now take the radiotherapy 3-6 months to take effect, so likely further decline in interim.

So plan is now for Total Lymphoid Irradiation (radiotherapy).   They basically blast the areas that produce immune cells to reduce the amount being released - the idea being to halt the immune process which is causing the rejection.   The immune process going on in my lungs has just got carried away - and it results in scarring of small airways (irreversible) hence the decline in lung function.

The process will be twice a week for minimum 5 weeks.  It can take longer if white cells (immune cells) drop dangerously low, and then need to delay treatments.  It's balance between slowing down immune response without leaving me wide open to infection.   The main side effect is exhaustion.

I wont be able to go to work and will need to avoid as many bugs and germs as I can.   I am meeting with the consultant next week, so will get more information about the treatment and when due to start.  Plan is as urgently as possible.

In meantime I have ECP next week and will get this anyway (as I might be worse without) then that will be stopped and arrangements made to remove the permcath line.  I wont be sorry to see the permcath go!

I am so disappointed that ECP has not worked out....I had many hopes pinned on its success and also hoped it would be the ideal longer term solution for keeping me well.   However at the end of the day, I am also so glad I was funded and at least had the opportunity to try it - otherwise there would always be a huge "what if?!"

Now I need to shift my focus and accept that plan A hasn't worked and be very grateful we have a plan B!   It needs to work - it's not really an option for it not to!!  I am staying positive and hoping everything happens for a reason and it's meant to have worked out this way.   Onwards and upwards...

Kirsty

I still quite can't believe that my dear friend Kirsty has gone.  She passed away on 26th May at age 34 - although I knew she was very unwell, she had been there before and always pulled through.  She seemed invincible.  We used to have morbid discussions about who got to die first, who was the most unwell at any one time and who could outdo the other in their medical dramas  - the kind of humour that comes from a lifetime of being acutely aware that our time here is fragile and all too short.  Also the type of humour that made Kirsty the headstrong, vivacious, fearless and bloody minded woman that she was. 

I knew Kirsty originally through the old CF forums, as we were never allowed to mix in real life due to cross infection.  She was well known in the CF community and part of a group of Glasgow CFers who were all around the same age.  Sadly most of them are no longer here  - Anders, Victoria and Kirsty's dearest friend Nicola, to name but a few.    I remember seeing Kirsty at clinic one day sitting across the waiting room from me.   She was wearing knee high red boots and I remember thinking 'wow, I wish I had the guts to wear those to clinic!'

We were able to actually meet after our transplants - Kirsty having hers the year after mine.  We came together to help Victoria organise the Masquerade Ball, and there formed a fundraising trio not to be reckoned with.   It was when I really got to know Kirsty that I saw behind the dark humour, stunning makeup, outrageous clothes, tattoos, many wigs... and I saw one of the most generous and kind people I have ever known.  She was scatty, always late, head in the clouds half the time, but if you really needed her, she was there.

That first ball was amazing success and one of the most memorable moments was Kirsty singing the song "Hero" and dedicating it to her donor.  She had to steal the show of course!!  You can see her singing it on YouTube  https://www.youtube.com/watch?v=YD9w8OpWWEg&feature=share
There followed a whirl of running organ donor desks, attending advocate weekends in London, speaking at schools, organising another charity ball, halloween night, swap shop and so on.   A friendship forged through the shared experience of CF, transplant and wanting to live life to the full.

I remember one of the first Live Life Give Life advocate weekends.  I was sharing a room with Kirsty and Victoria and they were both set for a night on the town. We were in a pretty rough area and ended up at a dodgy club where a fight broke out (Kirsty totally unaware of course).  My overwhelming memory of that night is that fact that in each photo my blusher got darker and darker...because every 2 mins Kirsty kept getting here blusher out and "topping" me up  because it wasn't drag queen enough for her liking!   I also remember having to stop at a shop on the way back to the hotel because she needed to buy sweeties.  Kirsty was well known for her love of sweeties....diabetes or not!  She could inhale entire bags of haribo with ease.

All who knew her will know about her unique fashion style :-)  I remember she came round to my house to give mum a spray tan for her holidays.  She came straight from her work (mac at the time) and was wearing cat ears - because it was a cat ears kind of day.   She would brighten up the transplant clinic teams day with her outfits and her shoes were something to behold...you never really knew what she would turn up wearing.

Our fundraising came to a halt as Victoria became more unwell, and sadly died last January.  At the same time I became a mummy and Kirsty had become a humanist celebrant, which tied up her weekends.  She loved this job so much and I am sure brought so much happiness to many on their special day.   Kirsty's own special day was a big surprise.   She had invited her family and friends to a local pub for a casual meal to celebrate her engagement.  However, it was in fact her wedding!!!  She didn't want people making a fuss and feeling they had to buy presents, so just sprung it on us!  She wore a short white dress with tartan ruffle at the back and her high top white trainers :-)  She looked stunning as ever and changed her Miss Geddes necklace to a Mrs Harvey one after the ceremony.   I am so glad she had her Dougie -  who saw her through her decline in health towards transplant (piggy backing her everywhere as she wouldn't use a wheelchair), through her life saving surgery, the loss of her best friend Nicola,  and of course with her through the complications in recent times.   I am so glad they got to travel far and wide together, have their fur babies and, honestly, to do more in one short life time than most people ever manage. 

When I was diagnosed with chronic rejection last year, Kirsty was one of the first people I told -because I knew she would just get it.  She phoned immediately and offered reassurance.... but also told me she had just conducted a wedding in the morning and admitted to hospital in the afternoon with sepsis so had outdone me again!  She was strangely proud of this skill..

I was glad that Kirsty was able to get to know Estelle and she loved hearing the funny stories.  Kirsty gave me a family photoshoot as a gift for Estelle's arrival - I had to laugh that she had made sure they accepted pets as well, so that Seb could be in the photo too :-)  Estelle was mesmerised by Kirsty - after we visited Kirsty in hospital a few weeks ago, she came home and asked if I could draw a hello kitty on her neck like Kirsty's tattoo!  She had to settle for a glitter mermaid tattoo at a party, which Kirsty thoroughly approved of.  Estelle also now has the giant fluffy talking unicorn Kirsty brought me one Christmas "I've got your present but I didn't have wrapping paper so it's in a black bin bag!"    For my birthday last year she got me and Estelle matching panda purses - mine is used to store all my daily medicines I need when out and about, so there is always a little reminder of her.    Estelle was with me when I most recently saw Kirsty and little did I know it would be for the last time.   I could see how unwell she was but she was still fighting and determined to get through (and trying to get back on to the liver transplant list, as she also urgently needed a new liver).  My parting words to her were to "stop trying to be more dramatic than me and get fatter!"  Kirsty would not have wanted anything else.  She did not do schmaltz.  She hated being called inspirational, or courageous.  She was dealt difficult cards and she coped the best way she knew how - by steam rolling ahead and not letting anything keep her down.

I find at every turn something reminds me of Kirsty -  she loved Alice in wonderland, flamingos, unicorns, Hello Kitty, sugar skulls, sweeties, dragonflies, tartan, tattoos, makeup, wigs, parties, retro, dancing, music......I think you can simply say that she loved LIFE.   More than that though she loved her parents Sandy and Annette, her sisters Julie and Tracey, her nieces and nephews and her husband Dougie.   Although her dogs might have ranked higher than all of the above! 

Kirsty truly lived a life worth living.

All systems go!

After the last couple of months passed so slowly waiting on funding decision, it's suddenly all systems go!   I met the ECP team on Thursday - the nurses were so lovely and reassuring, so I am starting treatment on Monday!  It will be 2 consecutive days ever 2 weeks for 3 months  - then review response (ie. what lung function is doing) and might be every 2 weeks for another 3 months, or monthly.  It was a bit unclear at this stage because I will be the first lung transplant rejection patient to go through the unit, so the protocol might vary to that for other conditions.   The good news was that because i will have permcath line the process should be much faster than expected - possibly only couple hours (plus the setting up etc) - we shall see on Monday.

I think I understand the process a bit better now.  Essentially they will take out blood, which goes through a machine (looks quite like dialysis).   The blood is centrifuged (spun) to separate out the white cells (immune cells).   They collect a small amount (about 4% circulating cells) and put the other blood components back in to me.   A photo-activating drug is then added to the white cells and UV light is applied (this is all in the machine) - this process will damage those T cells (specific type white cell) and they will die off once back in my body.   The damaged/dying cells trigger the body to eventually release specialised T regulator cells.    These cells are responsible for managing the immune response - they get things under control and dampen down inflammatory processes.   It is thought that people post transplant with rejection have low levels of T-regulator cells - which means the immune reactions towards the transplanted lungs can go unchecked.   The theory is that by increasing these T-regulator cells you can slow down or  stabilise the immune reaction against the lungs.   Since it is only affecting a very specific cell, then it doesn't affect the other immune cells required to fight off infections.    So I wont be more at risk of infection, which is excellent.

The process normally involves putting a wide bore cannula in one arm to remove blood, and another in the other arm to put it back in at same time.   My veins are barely good enough to get blood samples at the best of times.   So this is why I need the permcath.   I had this put in yesterday.   Essentially they make incision in neck to feed tube into jugular vein, to direct access the heart chambers.   The other end is tunnelled under the skin for a few inches (this reduces risk of getting infected) and pops out again on the chest wall (few inches under clavicle) and stitched in place.   There are a couple of access ports at the end - one for blood in, and one for blood out.   It was put in under local anaesthetic and the procedure itself was ok, but it was much sorer than I expected afterwards!   The tube is thicker than I had imagined (my previous ports being very narrow tubes in comparison) but it should settle down and in a couple of weeks be more embedded.   I will have to be really careful it doesn't get pulled out, or infected - Estelle has been warned not to touch!!   She is very interested in the whole thing and has asked to  inspect it several times already.   It's a bit cumbersome but should be ok under my clothes and will make the whole process easier.

I feel nervous about starting - not about the procedure but more about my response longer term.  We wont see any instant changes and can take few months to see improvements/stabilisation.  There isn't a lot of data around but some studies have shown up to 50% people responding, so that's a good chance.   I am unsure about how long effects last (if it works) and what top-ups might be needed.  Will just need to go with the flow and see how things progress.

I am planning to keep working in between.   They said it can make you more tired - particularly for 24hrs after, so I don't plan to go to work the same day as sessions.   I will just have to see how I feel, but hopefully be able to function reasonably normally!

I'll update once treatment starts!

Good news!!

My CF doc phoned this morning to tell me I have been funded for my ECP treatment!!!!!!! The best news I've had in a long time!!  Brought a wee tear to my eye...

The funding is for 6 months initial treatment - this will establish if I am a responder e.g lung function stabilises.   If I am a responder then I will probably need 'top ups' and we can apply for further funding if this is needed.   If I am not a responder then at least I have been given this fantastic opportunity and wont have that awful feeling that I never had a chance at this treatment.

I don't know the details of when this will start, or exactly how often (generally its 2 x 5hr sessions every 2 weeks).  It will be in Glasgow, so much easier for me than having to go to Newcastle.  The ECP doctor will contact me to make the arrangements but I do need to get venous access sorted before we start treatment.  I have poor veins (years of being prodded!) and they aren't good enough for the treatment, so I've to get a permacath (most commonly used in dialysis) put in before we can start.  This will be a less permanent version of the portacath I had for most of my life pre-transplant, so I'm not worried about that.  It might be bit annoying having tubes dangling about my person, but a small price to pay. 

I really needed this wee boost, as have not been feeling great since I last blogged.  It turned out I did have the parvo virus and it's taking ages to fully clear.  I was unwell with flu like illness last weekend, so not sure if it was re-emergence of parvo, or another virus, but I've been really tired since.  I am slowly recovering and ended up taking last week and this week off work.   I realised I haven't actually had to ask the GP for a sick line since my transplant, so I must never have been off more than a week since then!   I hate staying off work as feel like I am letting patients down when I cancel appointments - however I know this time I did need to just try and stop for a while and get on top of this and hopefully I will be back to better health just in time to get started on this next journey.

I will post again once I have more info about when I can get started with treatment and what it will entail.     For now I am just so relieved that I am being given this chance.   

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!

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!

2017 in Pictures

January

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..

February

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.....

March

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...;-)

April

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!)

May

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.

June

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!

July

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

August

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.

September

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.

October

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.

November

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!! 

December

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.