'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.

The Arrow of Time

The phrases ‘time marches on’ and ‘doesn’t time fly’ get thrown around fairly often in everyday conversation, but they are indeed very true. Listening to Heart Radio’s ‘Time Tunnel’ (where listeners have to guess which year a selected number of songs played were released in) on Thursday whilst travelling to hospital certainly confirmed this. Several of the songs seemed to me to have been released only recently, but the year in question transpired to be 2002... Some 10 years ago! This got me thinking about my ‘time’ and journey on the organ donation road. 

It has been three years this month since I was first assessed for transplant and two years and ten months of actively being on the list. It has been almost one year since my last call for a potential transplant. Since being listed, I really do feel that my life has been ‘chaptered’ by various passages of time. First and foremost was the ‘average’ waiting time for a lung transplant of 18 months (ha!) I suppose was the first time punctuation mark in this new outlook. During my first 18 months on the list I obviously had an incredibly restricted lung function (18-20%) but I was still able to ‘do’ quite a lot and although my teaching career had wound down substantially, I was still popping into school once a week or so to work with some children one to one or to hear readers. However, over time that inevitably had to stop; it became too much for me to do this. CF is a really complicated disease as one day I would feel good (by my standards) but on the same day the following week I could be completely exhausted unable to leave the sofa. Therefore I did not think it was fair of me to try to commit to any form of responsibility as I did not know until the day itself if I would be well enough to go in and it would therefore be difficult to plan around me. 

Since completely finishing working as teacher (for now...) I tended to live my life by planning key things to keep me busy and by looking forwards to upcoming events; i.e Laura coming over for a coffee; having a date night with Luke, Beth’s wedding, Luke finishing his doctorate... However, since coming out of hospital at the end of September this year, I’ve been going up to the Brompton every two weeks. This has been my new focus and it’s not one I particularly like. I feel like I’m desperately clawing at all of this extra medical intervention (permanent intravenous antibiotics for example) to attempt to remain stable. Now the FEV1 bit of my lung function (how much of the total capacity in your lungs you can force out in the first second of your breath) fluctuates between 12 -18% and the FVC ‘bit’ (volume of air that can forcibly be blown out after full inspiration, measured in litres) fluctuates more dramatically, between 25 and 45%. If you have a low FEV1 it means that the airways in your lungs are quite narrow, caused by inflammation, infection... I had a ‘blip’ a couple of weeks ago and thank God I got over it through changing intravenous antibiotics and by intensifying my physiotherapy at home (Thanks Luke and Mum!). My check up on Thursday was ok, lung function is a smidgen up (FEV1 is back up to about 18% and FVC jumped back up to 45% from 26%) since my ‘blip’ and my oxygen saturation levels were acceptable. The only caveat now is that my liver enzymes are slightly raised meaning that my liver is taking a bit of beating... I don’t blame the poor thing for kicking up a fuss; I’m throwing enough drugs at my body that it has to metabolise! Fortunately the levels aren’t too scary, so I have to get another blood test next week and keep an eye on it... Oh the joys!! 

These days, I feel quite itchy within my own skin; I know this must sound absurd but on my good days I just want to get on with as much as possible, because I don’t know how long my feeling good will last! Then on other occasions I’ll have something planned and will be too tired/poorly/whatever to do it! Luke and I managed to make it out to the cinema last Thursday to see the latest Bond which was well worth the effort! These days we try and capitalise on the good days when simple things, like going to the cinema, are manageable as we can’t predict how I will feel from one week to the next. 

Whilst all this is going on in my own life I am also hearing heartbreaking stories of fellow CF-ers who have either sadly lost their hard battle with this hideous disease, or are becoming more seriously ill as they too await their life changing phone call for new organs. This sharply focuses the notion of how much I need a transplant, and just how quickly. I don’t care if I sound like a broken record, but this really highlights the need for more people to sign up to the organ donation register! 

The arrow of time still marches on, we can’t fight against it (as much as we would want to!) so I must go with it, keeping myself as well as can be. One of my greatest fears is that I will become too ill for transplant before my call comes. This is where my faith helps me. I know that life can be cruel and unrelenting for people, but this verse helps me think of the bigger picture. 

2 Corinthians 4 v 16-18

¹⁶Therefore we do not lose heart. Though outwardly we are wasting away, yet inwardly we are being renewed day by day.

¹⁷For our light and momentary troubles are achieving for us an eternal glory that far outweighs them all. ¹⁸So we fix our eyes not on what is seen, but on what is unseen. For what is seen is temporary, but what is unseen is eternal.

A Day in My Life...

Having been free from hospital (well, aside from the outpatient appointments that is...) for almost three weeks now, life has settled down into a new routine. I have obviously needed a fairly rigorous treatment regime throughout my whole life, I am far more ill and more dependent on various machines and medications to lead any sort of normal life, it has become even more important that I follow a strict treatment routine. I thought that I should give you all a flavour into how my day to day life pans out!

Morning

8.00am – Luke or Mum (depending on whether Luke is at work) gives me my first dose of intravenous antibiotics (IVs) Currently these are Colomycin and Meropenum

8.30am – IVs finish and I come off my Non Invasive Ventilator (NIV), switch to my oxygen through nasal cannula and get washed and dressed for the day.

9.00am – Come downstairs and take my morning tablets (approx 10 drugs including slow release morphine again to combat chronic chest pain) and eat breakfast, I usually try to ensure that my breakfast contains about 700 calories. I also take two types of insulin in the morning; a long acting insulin and a short acting one. If my chest is particularly painful I will also apply a Lidocaine patch to the affected area.

9.30am – DNase; this is a nebuliser which helps to break down the thick mucous in my lungs to make it easier to cough up during physiotherapy. This needs to be taken about an hour before I begin physio.

10am – Exercise time, I use my NIV to do some exercise on the Wii fit. The reason it is necessary for me to use the NIV is to allow my muscles to actually become worked. If I were to not use the NIV, I would become out of breath before my muscles had a chance to even warm up. This means that I can (try) to keep my arm, leg and core muscles in some sort of shape which is really important for transplant.

10.30am – Physiotherapy; whilst using the NIV to help me take deeper breaths and get behind sputum secretions, Luke or Mum will tap and shake my chest to help me cough it up.

11.15am – TOBI nebuliser. This is nebulised Tobramycin which is another antibiotic to help control the pseudomonas aeruginosa that my lungs are colonised with.  At this time I also need to check my blood sugars to make sure that I’ve taken enough insulin with my breakfast. 

Afternoon

12.30pm – Lunch! Again I need to take short acting insulin with my meal and I aim to consume about 1000 calories in my lunch... Got to pile on those pounds!

2.00pm – Physiotherapy, as before

2.45pm – Second dose of IVs of the day.

3.15pm – IVs finish and Mum goes home (if it’s a weekday). Also check blood sugar levels.

4.00pm – Snack time, usually something like a Pot Noodle, or a hot dog as well as a bit of healthiness in the form of fruit. I also try to do some kind of gentle walking exercise during this time, without the use of the NIV.

Evening

6.00pm – Dinner time; again this is usually about 1000 calories as I need to consume about 3000 calories a day.

8.30pm (ish) – Physiotherapy, as before. Check blood sugars.

9.00pm – TOBI nebuliser and evening tablets, approximately 5 drugs this time, including slow release morphine.

9.30pm (ish) – bath; sometimes I need to use my NIV whilst having a bath if I’m particularly tired or finding breathing a struggle. I usually have some sort of snack here too (not in the bath might I add!)

10.00pm - Final dose of IVs of the day

10.30pm - Finish IVs!

11.00pm – Bed, attach myself to the NIV mask. (I also check my blood sugar levels before I go to sleep)

Reading this routine, it may seem somewhat demanding and I suppose it is to an extent. It does mean that I require Mum, or somebody else if Mum isn’t around to come down in the morning and afternoon to help me when Luke is at work. Sometimes it does feel that I am just living this routine and waiting for the transplant call to come. Fortunately it is flexible to an extent and I am able to be taken out for a bit of shopping, or a coffee. However, as is evident, the evenings are less busy in terms of treatment than the day, but after a long day at work the last thing Luke wants is to go out again! This means that we do have to plan outings and such in advance. Also, what I also have to take into consideration is that I get so tired from going out that I need to spend the following day taking things more easily (namely sleep a lot!). Luke and I do still manage to do things, like go to the cinema (well, we haven’t tried it since coming out of hospital, but we do want to go and see the new Bond film when it’s released!!) We also manage to out for meals occasionally. I am very lucky to have many fantastic friends who come round to see me and are able to fit around my routine. The support of my friends and family is definitely what keeps me strong during this time and hopefully, if that call comes, I will be free from this regime and I can enjoy the freedom that I experienced before!

Great Limitations

The old adage is correct - time certainly does fly, another six months have shot past, marked by another half yearly transplant assessment at Harefield. The results of the various assessments were alright in themselves, particularly seeing as I’ve only just been released from hospital and am still trying to build up my strength. However, I definitely noticed that this visit to the clinic was much more physically demanding than previous times which illustrated the fact that I have deteriorated somewhat since my admission to the Brompton.

After the glut of tests and a rather tasty lunch, the wait to see the doctor began. This time I got to see one of the consultants – Dr Carby, who is a lovely bloke and has experience in dealing with Cystic Fibrosis as well as the whole transplant she-bang which makes him really understanding of my condition. The whole team were already aware of the downturn I had taken and the ensuing stay at the Brompton. Dr Carby was able to deem me still fit for transplant, but the urgency of needing one is far greater now. The frustration he feels about my situation (and undoubtedly many others) was palpable. There isn’t really anything that he or any transplant team in the world can do to help me as the lungs that I require are incredibly rare. It is just a waiting game; One that I have been playing for two years and nine months, with no success so far. It is just a fact that small, O negative lungs are hard to come by.  This led him onto discussing with me the use of ‘extended criteria donor lungs’. This group of lungs include heavy smokers, lungs from over 60s, lungs from intravenous drug users and participants of ‘risky sexual behaviour’, donors with brain tumours, lungs from donors with viral infections including hepatitis and other drug manageable blood viruses.  Getting a good pair of lungs, but contracting a long-term viral infection would mean additional drug management and does pose medical risks, but given the state of my lungs as they are, this (whilst not ideal) still represents a better quality of life than I have at the moment and a better chance of a longer life. My window of opportunity of being ‘fit’ enough to survive a transplant operation is becoming smaller since my latest ‘blip’ as the doctors cannot predict how unwell I will become. Any chance of increasing the number of donor lungs available to me is one I’m willing to take. Therefore I signed the consent form (whilst blubbing, obviously) and Dr Carby said he would make a recommendation to the surgical teams. 

Dr Carby also wanted me to think about whether when the ‘time’ comes, would I prefer to be at home with my family and made comfortable or would I like to move to Harefield and receive ECMO (Extracorporeal membrane oxygenation) in last ditch attempt at getting lungs. ECMO in essence is a machine which performs the gas exchange (oxygenate and removes carbon dioxide) that the lungs should do, but outside the body. This is not a very nice procedure and is basically a way of buying me a little more time. I think I would want to fight to the bitter end as I am a fighter – we all have a survival instinct in us. Mine is really bursting out of the seams at the moment. At the moment I feel I would opt for ECMO, but it is incredibly hard to comprehend. 

It saddens me that doctors have to push the boundaries of medicine to increase the number of lungs available. If there were more donors on the organ donation register would it be necessary to put patients at additional risk? 

Having been a bit negative and feeling a bit fed up, I will not let CF define me nor defeat my spirit. CF is NOT greater than God. CF has taken away a lot of things from me, but it cannot take away from me my family, my pets, my friends or my love for them all. 

You, Lord, are all I have, and you give me all I need; my future is in your hands Psalm 16:5 (GNT)

The Great Escape

Over the past two months I have watched many patients come and go on Foulis Ward and on Monday it was my turn to finally leave! Eight weeks was a personal best for me in terms of admissions, by no means a record, but still a long time. To add to the excitement of leaving hospital, we travelled in style... no I don’t mean in a limo, but in an ambulance with the blue lights! (Talk about a getaway vehicle!) This is because I am deemed a ‘high risk’ patient as I need to use my non-invasive ventilator (NIV) during the day sometimes. Therefore the technicians wanted to get me home as soon as possible so I could be settled and stabilised. This meant that it only took us an hour to get home through heavy London traffic and terrible road conditions! 

Drawers of drugs!

A lot of things have changed now. I am going to be permanently on intravenous antibiotics (IVs) – until I get a transplant. This is because the inhaled antibiotics are simply not enough to control the bacteria and fungus that grow in my lungs. The acuteness of the infection during my admission and the bacteria’s ability to become resistant showed that we needed to use ‘bigger guns’ to control the infection. The bacteria in my lungs, if unmanaged, can reach a ‘critical mass’ and can cause me to become unwell almost overnight. These continuous IVs will be cycled, so every two/three weeks the ‘cocktail’ will be changed, to keep the bacteria on their toes! This means more frequent trips up to day case at the Brompton to have various tests and be monitored after my first dose of the new lot of IVs. As a result of this, our kitchen is now akin to a small pharmacy, or drug den, if you will... In order to keep the drugs separate from the potatoes we have dedicated storage to store the abundance of medical kit, as this is now a long term situation. Being on IVs continuously is very different to the odd two weeks blast every now and then, mainly through the way we have to organise ourselves. During my admission fungal infection was also a problem. As I mentioned in a previous blog I was put on an intravenous antifungal drip, but to make this more manageable at home, I now take a more effective antifungal tablet. It had some rather interesting side effects when I had my first ‘loading doses’ I had rather severe visual disturbances. I am pleased to say that these have subsided!  

I am also on much stronger pain medication to help manage the chronic pain I suffer. This entails long acting morphine tablets and ‘break-through’ liquid morphine to manage the pain in between long acting doses. This type of care will be managed locally by the palliative care team at my local hospice. Discussing hospice referrals and the like is not nice to hear. I am under no pretences that my lungs are in bad shape and I am in dire need of a new pair, but to actually have referrals put into place make things seem more real. 

My oxygen requirements have also increased, I now need 4 litres a minute whilst resting and through my NIV and 6 litres a minute whilst exerting myself. I am so pleased that I am able to use nasal specs as I genuinely thought that I would be coming home needing to use a full face mask on rest. I do however need to use my NIV more in the day, probably for about 4-6 hours. I see this as a great achievement seeing as I was on the NIV permanently for a month of my admission and have managed to with some God given strength to rely on it less and maintain decent Sp0₂ levels (approximately 92%). I am slowly building up my exercise tolerance, but at the moment it's quite low. Luckily Luke's mum and dad have given us a Nintendo Wii so that should help me!

Just before I escaped from hospital, as my infection markers had reduced sufficiently I was given an iron infusion to combat the anaemia I recently developed. It takes a couple of weeks for the iron to be converted into haemoglobin, so later tests will establish how successful that treatment was.

After such a rough admission and being so unwell it feels like such a blessing to be home. There were a couple of times when I didn’t think I would be coming home, but praise God, I got through them. Not only by the work of the incredibly talented and caring staff at the Brompton, but through God given strength and perseverance. Having Luke with me when I was really poorly was amazingly helpful as he not only helped me physically with 2am physio sessions and helping me to the bathroom and the like, he was an incredible help emotionally. My faith in God has been vital as there were so many times where I wanted to give up – and even told Luke so, but my faith pulled me through. Although we have a rather busy medical routine now, I am going to do my utmost to enjoy home life! 

Reunited with Azlan the rabbit!

So do not fear, for I am with you; do not be dismayed, for I am your God. I will strengthen you and help you; I will uphold you with my righteous right hand. Isaiah 41:10