Happy New Year!

It is safe to say that 2012 has been a year of ups and downs. Seeing many of my friends get married and being maid of honour for my best friend were certainly some of the best times I saw this year. 2012 also saw the completion of Luke's doctoral studies which I was unsure I would be around to celebrate with him. I was also really pleased to be involved with my local paper (The Basingstoke Gazette) in a campaign to raise awareness about organ donation as well as Cystic Fibrosis. I was also fortunate to be included in a BBC national news piece about ex-vivo lung perfusion transplantation. It means so much to me that I can use my circumstances to raise awareness about organ donation and even one person signing up after reading my blog could potentially make a huge difference! Oh and how could I forget the addition of Azlan the rabbit to our little family?

The bad times? Spending several long stints in hospital, the first back in January/February and then spending two months in hospital over the summer. Being so desperately ill was so frightening and feeling my health decline generally makes you feel very powerless. I am very lucky to have come through that admission, albeit a bit weaker and in more need for my transplant now than ever before, but at least I'm here.

I have two lovely friends called Rhod and Caroline who are running the Reading Half Marathon to raise money for the CF Trust. It would be fantastic if you would take the time to visit their fundraising page and sponsor them, no donation is too small! Click here to visit the page. The CF Trust does some amazing things with the money they raise from providing support in many ways to individuals with CF and their families to investing in high quality research to bring forwards new treatments for CF. Hopefully with new treatments and perhaps even a cure one day being discovered, the need for organ transplants to extend the life of Cystic Fibrosis patients will be no more! One drug that is currently making a big difference in some CF sufferers lives is Kalydeco. This drug works specifically on the CF mutation G551D. Although the CF Trust did not fund any of this particular drug’s research, they are working hard to campaign for it’s availability on the NHS. To see what a difference this drug can make, please read my friend George’s blog.

For me a cure for CF will not undo the damage sustained by my lungs, but could potentially keep me stable. What I really need for an improved quality of life and for an extended life is a double lung transplant. Perhaps in 2013 someone will be selfless enough to decide to donate their organs should the worst happen to them. If I were to benefit from such an act of generosity and get a second chance at life, it would be truly humbling. I look upon 2013 as a year of hope.Wishing all of my blog readers a happy new year!

'Tis the Season...

I'm a real lover of Christmas time. Obviously my faith plays a big part in why I enjoy celebrating Christmas, but I also really enjoy the coming together of family and friends. I enjoy our little family traditions which Luke and I have adapted and adopted as our own, such as the choosing and naming of our Christmas tree (sad, I know!), decorating it in a coordinated manner (Luke's input - I'm all for random colours and madness!). I love all the decorations and the lights and the tacky but somehow strangely watchable daytime Christmas films! I love attending various carol services and church services. Unfortunately I am not strong enough to attend church on Christmas day itself (as I would probably need to sleep for the rest of the day to recover!). In addition to this comes the excitement of choosing of presents for people and wrapping them up... Whilst ensuring the Cole family tradition of making the presents as difficult as possible to get into is observed! I was really hoping that this year I would be standing up and decorating the tree independently, but unfortunately that wasn't meant to be as I'm still waiting for those new lungs. However, I was fortunate enough to be well enough last Saturday to walk around our lovely local Christmas tree farm, although it did tire me out significantly. 

The most frustrating thing about being so restricted by my poor lung function is that everything has to be planned and done in little stages. For example, when decorating the Christmas tree  (Luke obviously does all the heavy lifting and putting the decorations on the tree) I first have to sort through the various ornaments and tinsel. Rest. Then attach the tree hanging bits if needed. Rest. Pass them to Luke to put on the tree. Rest... Luke and I wrap presents together, but I obviously wrap Luke's presents on my own. Again this involves planning to ensure I don't have to make multiple trips around the house. I hope that I don't sound self pitying, but I just wanted to give people an idea of simple festive tasks which have now become quite cumbersome for me (and to be fair, I took them for granted when I was well). I am however, very grateful to be able to do some things with some degree of independence, but I just yearn for simplicity again. 

Not only do I have to plan the tasks step by step and think through where things are geographically in the house, I also have to make sure that if I've had a busy day I take it easy the next. I have been in a lot of pain this week; this is largely due to the fact that I was very busy last week. I went Christmas shopping with my dad on Thursday, went to the opening of the restaurant that my brother in law works in on Friday, Saturday saw the getting of and decorating the Christmas tree and Sunday was roast at mum and dad's. I also went to my GP for a blood test on Monday. Whilst all these small events don't seem like much, my annoyingly weary body just needs so much time to recoup after each event. So with complete rest and maxing out on my pain relief on Tuesday and Wednesday I felt much more human by Thursday. I just feel so blimmin' useless and burdensome, which my family insist I'm not. I still can't help feeling like it though! I feel like for the last three years I've been holding onto this hope that one day my call will come and I'll get a degree of normality back. I'm just hoping and praying that by next Christmas I will have had my transplant and be able to enjoy the festive season in a much less restricted manner. 

I have a hospital appointment on Thursday and I'm praying that I am 'well' for it - in as much as my lung function, oxygen saturations and other various observations are stable. I know I am silly to worry so much, but I just want to stay at home for as long as possible with my family and friends and I don’t want to wreck their Christmas by being admitted into hospital for whatever reason. Hanging onto stability is great, but I also know that without a transplant things aren't going to improve. I am hopeful though, and despite being told 3 years ago that with my current lung function I had a life expectancy of 2 years, I'm still here- a bit wearier, but still with that fighting spirit. I am in desperate need of a double lung transplant, but it is a waiting game with no guaranteed results. All I can do is live the life I've been given as well as I can and enjoy what I have. If you want to do something amazing this Christmas, sign up to the organ donation register if you haven't done so already! If you have, encourage others to do so too! It could be the most selfless and generous gift anybody could ever give.

Team Yates!

Luke and I celebrating!

It has certainly been a hectic week for me. Luke has finally finished his doctorate which I am so proud of. I know those of you who follow me on social networking sites have seen me go on and on about Luke’s doctorate ad nauseam, but there were many times over the last four years where I truly questioned whether I would be here to see him complete it. After feeling a little unwell earlier in the week, I fortunately felt well enough to be driven up to Oxford (by Mum and Dad) to meet Mat, one of Luke’s colleagues, who drove me to the lab (and taxied us about Oxford much of the evening!). Here we enjoyed a champagne reception and some speeches, after which we returned to Magdalene College, were Luke’s supervisor had booked us a rather sumptuous suite of rooms to spend the night! It was here we met our only trouble of the day... Despite booking holiday oxygen weeks in advance and receiving a confirmation letter detailing the equipment we could expect to receive, no oxygen had been delivered. Upon calling the company there was apparently no record of our booking on the system! Very strange. Nevertheless the company sent out an ‘emergency’ concentrator, back up cylinder and portable cylinders, so all was resolved in the end! We were then able to enjoy Luke’s celebration meal which was delicious; unfortunately Luke wouldn’t let me take any photographs of my food... of course it’s not the done thing! I’m so grateful to Luke’s supervisor and colleagues who went out of their way to make it possible for me to participate in the celebrations! 

On Lind Ward, getting my line in

After all that excitement it was back to life with an exhausted and ill-felt bump. It was CF business as usual and I was seen in clinic very shortly after the revelry – talk about sublime to ridiculous. Despite feeling grotty, I am very pleased to report that my team at the Brompton did not wish to keep me as an in-patient after my clinic appointment on Tuesday. However, we did decide that I should have a course of intravenous antibiotics as I am beginning to show the symptoms of a good old chest infection... Annoyingly I have lost a bit of weight, so I’m down to 46.2kgs, which although isn’t too awful it takes my BMI down to 19.7 which is creeping too far away from my ideal of 20! Surprisingly my O₂ sats were 91% which I was pretty happy with, as they usually choose to dwindle around the 88% mark! My lung function was down ever so slightly in terms of the individual numbers, but as a percentage it remains stable at 17% (FEV1). The doctors didn’t think there was anything that could be added to my routine that ‘Team Yates’ (as we’ve been dubbed!) can’t provide at home. 

My 3 times daily IVs

We had a discussion about what the next steps regarding maintenance inhaled antibiotics should be and came to the conclusion that any testing would be obscured by the chest infection and would therefore be pointless. As I have previously mentioned, Aztreonam makes me incredibly tight chested, inhaling TOBI causes high levels of the drug to build up in my system and Colomycin is just not as effective in its nebulised form for me anymore. We will readdress that problem at clinic on the 31st July, when hopefully my chest will be behaving itself a bit better! It may even be that we can revisit Aztreonam, as I could have become irritated by it due to an infection; it’s really going to be a case of try it and see. (Obviously in an observed and monitored manner!) The intravenous antibiotics that I have been given are Meropenum and Colomycin. Although Colomycin doesn’t seem to work for me in a nebulised form, it should work much more effectively in an infused form as my lungs don’t have to deal with it. I am also on an oral antibiotic called Co-Trimoxazole. Hopefully this three pronged attack will be effective. Having home IVs is no picnic though. It requires a high level of dedication as these potent IVs need to be given three times a day. Luckily one of them (the Colomycin) is delivered to us in a pre-drawn up ‘bladder’ which infuses itself over half an hour. The Meropenum however, needs drawing up from scratch and it is a real nuisance to dissolve! Also, it is so important to keep all surfaces, hands etc. Incredibly sterile, as these drugs are going straight into my veins – we do not want to risk getting an infection that way! 

Meropenum

Luke giving IVs to an exhausted Sammie!

Thinking back to ‘whether I will be here or not in x years/months time...’ even when I was well with my CF, I was never the sort of person who made ‘five-year-plans’, but these days I do question whether I will ‘see’ certain events, next Christmas for example, my next birthday? However, you just CANNOT live like that. For one thing, nobody can truly be certain of their future, without wanting to sound macabre, who can know what is around the corner? Just because I think I have some vague idea of the time I’ve got left I can’t let it rule me. That is why I get so excited by celebrating the things in my life – Luke’s doctorate, our 5 year wedding anniversary to name just two! It’s at times like these I really value my faith in God and although it’s far easier said than done, I try not wonder why some prayers get ‘answered’ and some remain seemingly ‘unanswered’. 2 Corinthians 4: 17-18 ‘For our light and momentary troubles are achieving for us an eternal glory that far outweighs them all. So we fix our eyes not on what is seen, but what is unseen. For what is seen is temporary, but what is unseen is eternal’. I am thankful for what I have been blessed with and take each ‘milestone’ that I reach as a blessing. If I am meant to have a transplant and do eventually get my call I will be so very thankful, but I’m going to try my hardest not to dwell on it and as I always say, live my life, as it is, to the full! 

My Best Friend's Wedding!

Over a year ago now, my best friend of 20+ years approached me and asked me if I would do her the honour of being maid of honour at her wedding to her then fiancée, Ed, this June. I of course agreed, as she was mine back in 2007 and I was so honoured to be able/alive to return the favour! Having been on the transplant waiting list for almost a year when Beth asked me, I was naively hopeful that I would have received my transplant by the time the big day came around! Well... things didn’t exactly turn out that way! Two false alarms occurred, but not a lot else! As the wedding crept nearer and nearer I began wishing for something that I never thought I would wish for... That Harefield WOULDN’T call! Please don’t think of me as stupid or worse still, ungrateful  - I would of course have trundled up to Harefield had I received a call, but when there is something so exciting and un-CF or hospital related to look forward to, it really makes a change! (Also, there would have been the possibility of the call being another false alarm which would have just added insult to injury!) 

Luke and I were very fortunate to be given access to free (and incredibly sumptuous, might I add!!) bed and breakfast accommodation within the farm on which Ed works and the wedding reception took place. I cannot begin to express my gratitude towards how accommodating people were to ensure that I could take part in Beth and Ed’s day. It goes without saying that I was nervous as to how I would ‘cope’ physically with the day but it transpires that I need not have worried! Aside from the fact that I was pretty well (by my standards) Beth and Ed’s family, as well as Luke and Kate (Beth’s other bridesmaid), did more than enough to make sure that all I had to do was be there for Beth, walk down the aisle and enjoy the day! Even with an oxygen canister in one hand I was able to do my maid of honour duties (the removal of the veil and being responsible for the bridal bouquet!)

Wedding Oxygen!

The bridesmaids and the bride!

The gorgeous happy couple!

Whilst my CF obviously limited my ability to boogie the night away on the dance floor and party into the small hours, the huge amount of support and loving care I received from my friends meant that I still managed to participate fully in Beth and Ed’s day. I was worried that all of my extra demands, such as machinery and needing a bit of an extra helping hand would somehow ruin the occasion (stupid, I know!!) However, that didn’t happen in the slightest, the wedding and the reception were beautiful, with a real atmosphere of love and happiness. I am completely thrilled for the new Mr and Mrs Bagshaw, everyone had an amazing day and although I was pretty wiped out for the next two days, I would do it all again in a heartbeat! Thank you Beth and Ed for including me in your special day!

I did it! 

IVs... Emergency Blood Tests and A Hen Weekend!

I apologise profusely for my lack of blogging... I usually tend to go quiet when I either ill or busy, I am happy to report that my current quietness is due to the latter and not being ill (well more ill than usual!). It has been a real blessing to be so busy largely due to a number of weddings Luke and I have been invited to. As well as planning and attending the hen-do of a very special friend of mine!  When the hustle and bustle fades however, I am left alone with my thoughts and the one thought that dominates all others is how desperate I am to receive a transplant. I know that I should focus on the fact that I feel generally well at the moment but I do panic and think that it doesn’t take a great deal to make me feel poorly – the slightest cold could potentially go to my chest and worsen my already poor lung function.

Therefore I thought that it would be an exceedingly good idea to get myself up to the Brompton Hospital as a day case and start a course of home intravenous antibiotics (IVs), to keep me feeling as well as I have been feeling and to ensure that I am really really really well for Beth and Ed’s wedding on the 9^th June (only 17 days to go!!!). I went up and started my IVs with very little hassle... my lung function was it’s usual low but steady self (Fev1 18%, FVC 45%), weight was a little down, but still acceptable, x-ray unchanged since last time, O₂  Saturations were a bit iffy, but eventually decided to climb to 89/90% which they decided was ok (for me!). 

After one dose we trundled home and commenced the usual routine of administering the IVs (Ceftazadime 3 times a day and Tobramycin once a day). I felt a bit grotty for the first few days, as they are pretty potent chemicals, I felt a little sick and tired. I had to send a Tobramycin level up to the Brompton before I took my 3^rd dose, to check that the drug wasn’t building up in my body and potentially causing damage. Having taken Tobramycin as an IV fairly regularly for longer than 10 years, and always had normal levels this was just another bog standard part of the routine. However, I got a bit of worrying news from the Brompton the day after I sent my Tobramycin level back to the hospital. The level that should be registered before the next dose (at least 18 hours after the previous dose) should be less than 1. However my level was 9, almost 10 times higher than it should be. I had to immediately stop the IV Tobramycin. The Brompton were very concerned as firstly, I am a remote patient, so I’m not at their fingertips to nip a quick blood test down to the lab and secondly they desperately needed to check my renal function as the high levels could indicate kidney damage as this would suggest why the level was so high; it was not being removed from my body efficiently. It was also mean that my other antibiotic would be damaging my kidneys further. Getting a blood test done locally is a very merry dance to lead!! I had to ring up my GP and plead for an emergency test, which they fortunately allowed me to do. Mum (aka Supermum) then had to drive me with the blood sample to the pathology lab in Winchester hospital so it could be tested and then the results communicated back to the Brompton. I am pleased to report that the test on Friday indicated normal renal function, thank God, but I am having it repeated tomorrow to ensure that it is still ok. The doctors are really unsure as to why my level was so high, so I’m not to continue with the IV Tobramycin and to just have it in it’s nebulised form (TOBI).

Currently I have to say I’m feeling a bit on the poorly side due to not being on any form of Tobramycin for nearly 5 days (and an incredibly hectic weekend)... But since being allowed to nebulise it again, I’m beginning to pick up again. 

All of this aside, I had a really lovely weekend... It was the long awaited and highly anticipated hen do of a certain Miss Beth Avis!! We had a lovely meal at ‘The Shapla’ restaurant in Alresford and then we spent the next day completely relaxing at Forest Mere Health Spa. The only thing to mar this lovely weekend was my STUPID portable oxygen unit.  As I have discussed in previous blogs, I have a Dewar of liquid oxygen which I use to replenish a small portable unit which I can take around with me. On ‘demand’ setting (which releases O₂ when I breathe in) using 3 litres a minute will last approximately 9 hours. Filling the portable unit is pretty simple – you push the portable unit onto the top of the Dewar and under pressure the liquid O₂ shoots into it and fills it. Once filled, you leave it to temper and then 20 minutes or so later you are good to go... Or so one hopes!!! Occasionally it misbehaves and I am not quite sure why it does this, but from time to time once it is filled, the portable unit will not dispense oxygen ‘on demand’ (or at all sometimes) meaning that my 3 litres per minute usage will only last about 3 hours.. Scuppering any hopes of leaving the house for more than that time! On Saturday, the night of Beth’s hen meal of course it decided to play up, but it wasn’t too much of a problem as I knew I wouldn’t be out for all that long... However, it really threw the spanner in the works for me when it decided to misbehave on the morning of the spa day... Firstly we filled it and then left it to temper, then after it still not working almost an hour after filling, and further time spent searching for the English language part of the maintenance manual, we were instructed to lay it on its back to temper. We followed these instructions, and suddenly about 5 minutes later there was a loud cracking noise from the kitchen... The liquid O₂ had completely leaked from the unit! As liquid O₂ is incredibly cold (it’s boiling point is approx -183°C) it had cracked part of the paintwork on the windowsill!! Thank goodness it was only superficial damage, and can be fixed with a bit of paint! So the unit was now empty! After a second attempt at filling, the unit was dispensing O₂ at a slow rate, we decided just to go. There was no way on earth I was going to let CF ruin Beth’s spa day, besides this, I was really looking forwards to it! Nevertheless, after much frustration and anguish, it started to pathetically dispense oxygen! Luke (my real life Superman) very kindly drove me to the health spa where I met up with the lovely hens and had an amazingly relaxing day! I was a bit deoxygenated as I’m meant to have a higher flow rate when I move around, but on the whole it was absolutely fine! 

I’m a little bit anxious about my blood test tomorrow; I’m really praying that the kidney damage hasn’t occurred after the last test. Despite this I am firstly really thankful to have such an accommodating GP surgery, as there is usually such a long wait for a blood test... Secondly that I have such a great carer in mum; she is willing (and sometimes able) to drop everything to drive me here there and everywhere when Luke can’t. Lastly, but by no means least, I have such a wonderful group of friends who take me as I am and wheel me here there and everywhere! I used to be so self-conscious about wearing my oxygen in public but now I really don't care about it... People will either accept me for who I am, or they won't! So whilst my life is difficult and trying, it could be so much worse, and I am so grateful that it isn’t!