Wednesday, September 11, 2019


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!

Wednesday, July 24, 2019

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 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 :-)

Wednesday, May 15, 2019


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. 

Wednesday, April 17, 2019

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 :-) 

Tuesday, March 12, 2019

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!!!

Thursday, February 14, 2019

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.... :-)

Monday, January 21, 2019

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 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!!!