It's a Long Hard Slog!

I don't wish to bore you with a long detailed update, but I know that many of you like to know how I'm getting on!

Last weekend was a bad one. Things took a turn for the 'worse' on Friday. My chest pain had become so unmanageable that I was put back onto a morphine syringe driver. However, the good news to accompany this is that subcutaneous anti-sickness drugs can also be added, so all my feeling of nausea are gone (yay) and I am generally able to eat well. My sputum remained thick and stubborn, so we decided to increase my usage of a drug called DNase which breaks down the sputum at a molecular level, making it easier to cough up. I also began using saline to hydrate my airways. In addition to this, physio was increased from three sessions to four slightly shorter sessions. Needless to say this was exhausting, so time spent not doing nebulisers or physio was spent sleeping on the NIV. We got through it! I felt so much better by Monday which was good, as it meant I could enjoy spending time with my brother, Ian, when he came up to visit!

However, I did continue to feel tired and when Mum came to visit on Tuesday I spent quite a lot of the time asleep (sorry Mum!). However, it transpires that there was an explanation for this... You know my lovely CRP infection markers had come right down to 40? Well Tuesday's tests showed them to have jumped right back up to 140! Grrrrrrrrrrr!! Stuuuuuuuuupid CRP/CF/body! Therefore I had another change of IVs on ward round (only one of the drugs, so I'm now on Meropenum, Colomycin and now Septrin). Again it's going to be a case of waiting and seeing whether this combination works. Obviously I'm annoyed, but we'll have to just wait and see if this Septrin IV kicks some bacterial butt! Oh, and nebulised Tobi has decided to make me tight chested, so that's a no-go for the moment!

Another recent development is that Luke has now started staying with me (glamping in Chelsea, you know!). This has helped me immensely and he just travels to work from the hospital as opposed from Eimi's. Hopefully towards the end of my admission when I am feeling stronger he can go back and enjoy spending time with his friends! An illustration of how helpful Luke is here is that last night I had a particularly poor night, for some reason my sputum decided to mobilise itself at about 2.30am. It just meant with Luke being here, he could help me clear and just generally comfort me and I hacked up about half a sputum pots worth of joy! Obviously had Luke not been here I would have been wonderfully looked after by the nurses and on call physios, but Luke knows my chest so well (sounds dodgy!) that we could just get on with it efficiently and as quickly as possible.

On a bit of a different note, my diabetes is being a bit of a pain. Obviously my steroid dose is still high, so that plays havoc with blood sugar levels, but I've been getting high readings first thing in the morning which isn't great. It's an indication of the progression of my CF related diabetes. So I will need to now take some long acting insulin at night and see if that helps. What worries me about other things going 'wrong' is that is I so desperately need new lungs, I don't want anything else to start failing before I (hopefully) receive my transplant!

I'm so overwhelmed by people's responses to the ITV Meridian news piece that I featured in on Wednesday, as it makes you feel quite vulnerable putting all your emotions out for the general public to see! Thanks for all the lovely messages and I just hope and pray that people are encouraged to sign up to the organ donation register and tell their loved ones their wishes as a result of this huge ITV From the Heart campaign. The last figures I read were that 100,000 people have signed up since the beginning of the week!

I must also urge all of you to visit Rhod and Caroline's 'Team End of my Tether' fundraising page! These amazing people are running the Reading half marathon to raise money for the CF Trust, so any donation will be gratefully received!

I am still hoping that my call for my double lung transplant will come very soon. It just makes me sad that all this fighting and effort to beat this exacerbation is basically what my life is now. Just treading the water until I get that call. But I will do it with a smile on my face and as much positivity as I can muster!

Time to Press Reset...

One week on and nothing much has really changed (unfortunately). It has been necessary to play the role of the 'patient patient'. Usually I do not play the role well, but as I have been feeling a rubbish-y it has been a bit easier! It is really difficult to put into words my hospitalisation and recovery. As I have said in many previous blogs my admission is not going to make me 'better' per se. The main aim is control my infection, as I am never going to be infection free due to the nature of my lungs, but the level of infection can be dramatically reduced and managed. Therefore the aim of my treatment is to reduce infection markers (CRP provides an indication of the magnitude of infection, whereas white blood cell count is always elevated as I am always colonised with Pseudomonas) and to increase my lung function and other clinical observations.

Unfortunately my lung function is being uncooperative and is currently about 13% despite two and a half weeks of IVs. My sputum is also still being really stubborn, thick and an effort to move. This means I have been attached to IV fluids 24 hours a day to help keep it moving and much easier to cough up and spit out. Another issue which has (unsurprisingly) reared it's ugly head is my chest pain. I suffer from chronic chest pain due to the constant inflammation and infection, but when I have an exacerbated infection the pain obviously increases, not particularly helped by super intense physiotherapy! Luke says that I am a different person when I am in pain - drained of energy, irritable, very tired and it affects my sleeping, eating and obviously my breathing. Everything is a constant effort, even taking a simple breath. Hence my increased need for the non-invasive ventilator. It also knocks my confidence a bit. So the doctors have increased my background morphine dose as well as my 'breakthrough' doses. They have also said that if I want to I can go back on a continuous subcutaneous morphine pump to ensure the pain is continually managed. I am a bit reluctant to do this, but the higher dose of oral morphine is making me a bit drowsy and nauseated, so perhaps it may be the best option just for the short term. Another little niggle is that my oxygen saturations are playing around a bit in the early hours of the morning, sitting at around 87% to 91% despite being on the ventilator... Hoping that this will get better once the infection subsides further!

As my infection markers have reduced a little, but nothing else has really improved, the doctors have decided to completely change all my IVs. So now I am back on Meropenum and Colomycin but they have also added Chloramphenicol (which is four times a day, boo!). They have stopped the IV anti-fungal and 24 hour Ceftazidime (one less 24 hour appendage, yay!). This means i can also restart nebulising Tobramycin which I've always found effective. In essence we have hit the 'reset' button so I am assuming I will be in here for another two ish weeks to see what this cocktail does to me!

We are playing a long game here and the main thing is that I remain fit for transplant. Harefield are fully aware of my current condition and who knows, perhaps I may get a call whilst in the Brompton! I'm still living with that hope of receiving that life changing and saving phone call. My whole family are.

Oxygen tubing and fluids

Fluid drip

Ceftazadime infusion... no longer!

An Update From the Brompton

Lukey and Me!

This last week has been pretty difficult. Since being in hospital my lung function has actually declined a bit, but that can be explained by many different factors, which I hope will be reduced when the infection begins to subside further. At the beginning of this week the Meropenum intravenous antibiotic (IV) has been replaced with a continuous Ceftazidime infusion, which delivers the drug constantly. This is to basically keep the bacteria-killing levels high throughout the day, rather than it peaking and troughing with having the dose three times a day. I'm still on tobramycin and ciprofloxacin IVs as well as IV caspofungin (antifungal).

All of these IVs are wreaking havoc with my electrolyte levels, so have to have regular magnesium and potassium infusions to replace what has been depleted by the medication. I also need to have quite a lot of extra fluid to keep my sputum lose enough to cough up with the least amount of effort. This means I am attached to three different drips all day and night.

Sorry if you're squeemish, sub-cut terbutiline

As I have still been quite tight chested, I am also having terbutaline (a drug which opens up the airways) subcutaneously (a little needle underneath the skin of my stomach). However this could cause me to become more tachycardic (fast heart beat) as I'm quite tachycardic naturally, so that's yet another thing to keep an eye on!

I'm relying quite heavily on my non-invasive ventilator (NIV) as you can imagine, with my infection markers being high and so on. It's a bit disheartening to be so reliant on so much machinery, but at the same time I feel incredibly blessed to have such expert help available, to help keep me as fit as possible for transplant. I am feeling very frustrated that although some of my infection markers have dropped a bit (CRP is now 145) they are still far from ideal. Mornings are also so hard for me, as my chest just feels so tight and heavy with sputum. It basically takes forever for it to wake up. However, I must not let myself slip into the spiral of self pity and negativity, otherwise things are ten times as hard to face! I keep praying and hoping that my transplant call will come soon and although it's going to be another hard road to travel, it's a lot more hopeful than the one I'm on at the moment.

Me and my wires!

I did have a very exciting start to the week! ITV came in and filmed me as part of an organ donation campaign they are going to be running in February. I was very pleased to be able to do it, I felt incredibly unwell on the day of filming, so I don't think I looked my best at all! However, vanity aside, I really hope and pray that the message about organ donation reaches many during that week and people make the decision to sign up to the organ donation register!

Please remember to visit Rhod and Caroline's CF fund raising page!! Any donation gratefully received for a good cause!

L'hotel Brompton... It's the Place to be...

Just thought I would update you all briefly as to how I am doing. I arrived at the Brompton on Friday despite the weather being as awful as it was! I'm just glad we went with hospital transport as there is no way we would have had enough oxygen to last the journey! 

Upon arrival my various and observations were not too horrendous, one of my lung function levels (FVC - how much air I can blow out in my total forced expiration) was markedly down from 45% to 33%. Thankfully however, my FEV1 remains stable at 18%, which is good as this is the result which would indicate a more acute increased inflammation. At least I am not battling from such a low as last time! My oxygen sats are pretty similar to that of home (I.e. horrendous, but no more so than at home!). I am able to walk around the ward for physio with a high flow oxygen mask, not solely on the Bi-pap. Obviously I still spend a large amount of time on my Bi-pap (non invasive ventilator) but I'm not on it permanently which is good. I am on two different intravenous (IV) antibiotics, Tobramycin and Meropenum. I am also on an IV anti fungal, as the doctors thought that it was likely to be a fungus irritating my airways. My prednisolone (steroid) has been increased to 30mg from 10mg to help open things up too. As well as lots of IV fluids to help loosen sputum. It all seems to be helping and at ward round today, my doctor said my CRP is now 170 from 224 and I could hope to be discharged next Tuesday if the CRP continues to drop nicely. We will also need to make sure I'm stable on the IV antibiotics they send me home on. I have also had an abdominal x-ray as I've been having a lot of stomach problems, with being on morphine and various other medications. Fortunately there are no big 'obstructions' (lovely!!) so I just need to keep on  drinking plenty, and taking senna and movicol (yum!) .

I am really pleased that this should be a relatively quick turn around... Less than two weeks is a record for me, even on paediatrics! However it also kind of hammers the point home that there isn't too much more they can do for me. I desperately need new lungs. It's just a case of maintaining the status quo at the moment. I'll never be without needing bi-pap again, or high flow oxygen, never be without infection, so will need IVs permanently until I'm lucky enough to get a transplant. However I am thankful that I am allowed to have so much done at home and hospital transport can take me backwards and forwards from my very regular appointments. I hope not to sound despondent, this is just how it is now. Sometimes people can wrongly associate leaving hospital with being completely better, which is not always the case. 

Could you also spare a thought for Luke? As I'm not too ill he's been commuting to Oxford from our lovely friend Eimi's house and coming to me in the afternoon/evening, then tubing it back to Eimi's later in the evening. He's exhausted. In addition to this he is desperately sad that I'm at hospital in the Brompton as opposed to being on ITU in Harefield should my call last Sunday have been successful. I just feel like I am such a burden to him, but I know he doesn't think of me like that and he wouldn't want to be away from me.

On a positive note, my friend Laura has finally had her liver and pancreas transplant! Cystic Fibrosis affects most of the organs and Laura was in desperate need of a new liver and I thank God she got hers today! Here's a link to her blog. It's really interesting to see other ways CF affects the body. 

Thanks to all for reading and please, please, please consider signing up to the organ donation register if you haven't already and tell your loved ones of your wishes regarding it too! Also, here is a link to Rhod (Eimi's boyfriend) and Caroline's fundraising page, they are running the Reading half Marathon to raise money for the CF Trust! Any donation will be greatly received! 

 

All Gowned Up and Nowhere to Go

The last couple of weeks have been very manic to say the least. Since Christmas and the new year, I've been feeling a bit 'iffy' health-wise and shortly after Christmas we changed my cocktail of home IVs to Colomycin and Ceftazidime. Whilst Meropemum works really quite well, it's never a good idea to stay on the same antibiotics for a sustained period of time. Notwithstanding the issues Meropenum causes my liver, the docs and I decided a change would be a good thing. However, it was always going to be a guess as to what to change to. It's clear now that poor old Ceftazidime just isn't cutting the mustard anymore, as my chest is not quite back to baseline in terms of sputum production and I just feel 'icky'.

Also, how can I forget to mention my third unsuccessful call for transplant? The emotional trauma of getting the call on a lazy Sunday evening, spending all of Sunday night and the early hours of Monday morning awake (I've not pulled an all-nighter since scrambling to finish a uni assignment!) has really taken it's toll on my body. When I was assessed at Harefield on the Sunday night prior to the possibility of the transplant they found my infection markers to be high (very high to be honest), but they would still have operated on me. They assess cases on an individual basis and as I have been waiting for so long, having had so few calls and balancing all the risks, in my case, the decision to operate was taken. However, this turned out to be irrelevant as at 3am we were told that the lungs were unfortunately unviable (as is the case with four out of five of all donated lungs). With heavy hearts we trudged home, emotionally wrecked and as I previously said, I was and still am physically drained!

Although I said Harefield took the decision to operate despite my high infection markers, I decided that it would be best to get on top of this, as if I am fortunate enough to get another call I don't want to continue to test the boundaries of CRP/white blood cell levels! At hospital yesterday, surprisingly my lung function remained stable at 18% and all my other observations were stable too. The decision was taken to add an oral antibiotic into the mix. However the doctors said that due to my clinical symptoms to only take them if i felt worse. We didn't get the blood test results until my doctor phoned me on my way home and unfortunately my CRP had spiked massively (from 160 to 224) so I was to take the oral antibiotics immediately and to call if I felt worse. Annoyingly enough I felt horrendous this morning, so up to Hotel Brompton I go tomorrow! I'm hoping that as my clinical observations aren't yet diabolical, I will avoid a lengthy admission, but I'm just praying and leaving that in God's hands (what else can I do?).

Thanks to everybody who sent me messages on Facebook, Twitter as well as text messages on that Sunday night. Although I couldn't reply to them all individually I appreciated each and every one of them. I also want my church family to know that I was deeply touched to know that you opened up the church and many of you prayed for me on the Sunday night. I know it's so frustrating that I'm still desperately ill and that the transplant didn't go ahead, but I just trust that God has a plan for me and I still have hope that the call will one day come.

How could I possibly not take this opportunity to thank the potential donor and their family. Although my transplant did not happen, other organs may have been viable and I thank the donor's family for being so selfless in such a horrific time for saying yes to organ donation.

Me at Harefield ready and waiting...

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.