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!

My Wonderful Man

A very quick blog entry, but one I really wanted to share! 

My gorgeous and very intelligent husband officially submitted his D.Phil Thesis for examination yesterday! This is cause for much celebration as a certain someone (me?!) has made his road to completing his D.Phil very long winded! Due to several long stints in hospital over the last few years Luke has had to apply for extensions to his deadline and has spent countless days hunched over his laptop at my bedside. Notwithstanding the opportunities he has missed during the course of his D.Phil, having to forgo conferences in sunnier climes, opportunities to present his work at various functions, as well as the time constraints that being one of my carers has put on the amount of time he could physically spend in the laboratory. Through all of this he has NEVER complained. He is a truly amazing man and an incredible husband. 

We have been so very blessed by the people that Luke works with and for – they have been so supportive and understanding of our situation and one has to wonder whether Luke would be in the position he is in today without them. 

He still has to be examined on his thesis, but I believe that if he can overcome the obstacles he has to get his research written up and submitted; I am in no doubt that he will do well in his viva voce – the oral examination of his thesis! Then we will be Dr and Mrs. Yates!!

I also cried yesterday when I read Luke's thesis dedication page. I hope he doesn't mind me sharing this but he wrote; 'This thesis is dedicated to my wife Samantha the most courageous person I know; and to God; because we are "fearfully and wonderfully made; your works are wonderful, I know that full well' (Psalm 139:14).' I felt honoured to be mentioned in such an amazing piece of work and can't believe that he would dedicate it to me, considering what a mission I made completing it for him!!!

Fit for What?

Reflecting on my visit to Harefield Hospital for a routine transplant assessment clinic, it was overall a positive experience. My x-ray, ECG and ECHO were more or less unchanged from 6 months ago and my blood test results and the results from various other examinations indicated that everything else is ticking along in a stable manner. The staff at Harefield are incredibly positive, speaking of when my transplant will happen, rather than if. However, rather than feeling relieved and satisfied after said appointment, I was instead feeling dejected and anxious. 

The first reason I think I felt this way was due to a change in one of my nebulised antibiotics. I usually nebulise an antibiotic called Tobramycin (lovingly referred to as Tobi!) which the bacteria in my chest are particularly sensitive to. However, this drug has some particularly nasty side effects ototoxicity, which in essence is damage to the ear, being one of them. I was suffering with a fair amount of pain in my ears and low level hearing loss. As I have discussed in previous blogs, other inhaled antibiotics have become less and less effective over time and it’s really only Tobi that has any noticeable effect on my chest. The Brompton sensibly suggested that I try having just 2 weeks off the Tobi and nebulise Colomycin then return to Tobi for a month, and cycle it that way. This would give my little ears a break, as well as stop my kidneys from becoming affected by the drug too. A few days into my Colomycin week, I already started to feel less well. I was tired more of the time, struggled to wake up in the morning, had no appetite and had a more productive cough. I powered through these symptoms and then restarted my Tobi slightly earlier than I was meant to. Within 3 doses I was a ‘changed woman’ (Luke’s words!) and now am feeling perfectly fine (for me!!) and have returned to my usual level of social activity and so on. Whilst I am pleased that I have returned to ‘normal’, I am beginning to worry that potentially Tobi will become less effective and what will happen then? 

Secondly, I have been feeling more and more like a burden to my family recently. Perhaps this was exacerbated by feeling unwell while using Colomycin rather than Tobi. I don’t need to reiterate here how my life has changed over the last few years, I’ve discussed this in depth in other blog posts, but I just have this sense of desperateness stemming from my intense desire to get a transplant and beat CF. I suppose I am just feeling the strain of waiting. According to the NHS Blood and Transplant statistics, the average wait for a lung transplant is 519 days. This is about 18 months. I have been waiting for 2 years and 3 months. I know that many people have waited for a much longer time than me to receive their gift of life. I know of one person who waited 4 and half years! Conversely I have heard accounts of people waiting weeks for a pair of lungs! I also know of many people who lost their fight with CF before receiving a transplant. There is no way of even speculating how much longer I will have to wait. I am very fortunate not to have deteriorated significantly since being listed for transplant. Nonetheless I am not so naive to believe that I can just wait for an indefinite amount of time. According to an article published in ‘CF Today’ ‘between 20 and 30% of people with CF on the waiting list for new lungs will not receive a transplant.’ I have always suspected that my wait might be a bit longer for two reasons – I am of a small stature and my blood type – O negative. O negative is not a ‘rare’ blood type per se, but it is a tricky one regarding transplantation. It is the universal donor, but I can only receive O negative type lungs myself. (Click here for more explanation!) Thus meaning I have a reduced pool of organs available to me.

Me and Beth at my wedding! 07.07.07!

I’ve stopped thinking of keeping myself ‘fit for transplant’ – don’t worry! I’m not giving up! Rather than thinking of this unknown goal, I aim to focus on ‘keeping myself fit’ for more tangible events. For example, a couple of weeks ago I went with my best friend Beth to her bridal dress alteration and to try on my bridesmaid dress! I was so honoured to be asked to be Beth’s bridesmaid, as it meant I could return the favour, as she was my bridesmaid! This is a real goal for me to aim to ‘keep-fit’ for, only 57 days to go! I have many lovely friends who take me out to lunch and for coffee, or even just pop round to see me – these are all little things I can ‘keep myself fit for’. I think of getting a transplant all the time and whenever the phone rings, I always have the small hope that it will be Harefield, but I need to focus on living life now. There are no certainties surrounding transplantation, so whilst I can fantasise about what my life will be like should I be fortunate enough to receive one, I still have to live in the here and now. Yes, I am ‘at the end of a tether’ and have a very rigid treatment routine; I have to get on with it and live my life! It may take a bit of creative thinking and the help of some very considerate and loving friends and family, but it can be done. 

Lastly, I don’t think I would have the positivity I do were it not for my faith and the knowledge and hope that there has to be more to life than this transient period of time we are here on earth for. I’ll leave you this time with a link to the ‘Footprints in the Sand’ poem – not a bible verse, but sums up how I am feeling at the moment!  

CF has got a brand new bag...

The practicalities surrounding living with CF is something that comes naturally as you get older and used to your routine. When I was discharged from hospital I had a few new drugs to get used to. First of all was my new dinner mate, my insulin pen (and the needles with which to administer it!). Secondly, my anti-anxiety medication to stave off panic attacks. The final new addition is pain relief, only bog-standard paracetamol and codeine, but imperative to take regularly to tackle chest pain. This is on top the digestive enzymes I take to enable me to fully digest my food and an inhaler should I become tight chested when out and about.

My Little Stash!

Before I got particularly ill with CF, leaving the house was easy, simply grab a pot of Creon and an inhaler, chuck it into my handbag and I was good to go! Being diagnosed with CF related diabetes (CFRD) added another sheet of tablets to the mix, but it was still manageable, notwithstanding the various pots of Creon and sheets of Repaglinide (the drug I used to take to control my diabetes) that were littered across the houses of my family and friends! The addition of the extra drugs made me reconsider how I managed leaving the house. I didn’t want to be running around searching for various pots of medication – I don’t have the breath to do that these days anyway! So I decided that I needed to become organised! You may think that being a teacher I would have a fairly organised nature – unfortunately for me, my professional organisational skills don’t translate that effectively into my everyday life! The palliative care nurse I met in hospital pointed out that it’s all very well having my anti-anxiety medication, but totally useless if I’m out shopping and feel I’m about to have a panic attack and the tablets are sitting at home! 

Luke had the brilliant idea of getting a little bag which could be for the sole purpose of my drugs. After much searching, I found the perfect little bag in TK Maxx. All my drugs fit inside and it fits perfectly into my three main handbags... It is, after all, a woman’s prerogative to have many bags!  It also has a nifty little strap so that I can simply take it out on its own if I’m only going to either mine or Luke’s parents for tea. Although a little orange bag may not sound like a great medical revolution, finding and using this little bag has really helped me get out and about more easily and without potentially putting my health in any sort of jeopardy. Whilst I have to live with my ‘end-stage’ CF, I am determined not to let it rule me. Therefore anything I can implement to limit its impact on my life – even if it’s just the use of a little orange bag, is a small victory in retaining my independence! 

Convenient sections...

Look how pretty it is...

Body Image...

One thing that has started to get me down of late is, I have to admit is incredibly superficial, but my body shape. To look at, I’m petite at five foot nothing and when I’m well my weight flirts with the 48kg mark. Recently, it’s been a bit lower at around 45kg, but I’m working hard to get it back up, as it is important to have a good body mass index (BMI) when I get called for transplant. 

Annoyingly, eating poorly for just one day can be enough to send me on that downward spiral of weight loss, which ultimately leaves one more susceptible to infection.  Also, when you’ve a lung function of 18%, most of the energy is being expended by simply breathing so not eating enough and having that weight reserve behind you is not great! It also takes such a loooooong time for me to put weight on ... For example I actually lost a bit of weight in my hospital admission, and then put it back on, so irritatingly I left hospital at the same weight as I entered it! I would have liked to have been heavier when I left. Now I am eating like an absolute pig to get me back to my ideal weight. The trouble is, all my weight collects around my middle, so I have really skinny arms and slim legs but a bit of a belly. I am by no means complaining, as my main priority is remaining fit for transplant so having a bit of a belly is a small sacrifice to say the least! 

Shellfish Platter!! (Sharing with my friend James!)

As I’ve been raving about, I was so happy to be home and able to celebrate my birthday exactly where I wanted to... The venue of choice being my local seafood restaurant where I can gorge on shellfish (including oysters) – an odd passion you might think, but am making the most of eating such things as I will be prohibited from doing so after transplant (due to taking immunosuppressive drugs). It is a girl’s prerogative to dress up for such events but one is presented with the age old dilemma of - what to wear? Obviously this called for a trip to Basingstoke town centre to find a new dress. Going clothes shopping is a mission for me at the best of times, due to my need for old ‘wheelie chair’ and getting breathless quite easily. Aside from the logistical obvious, something else makes dress shopping even more difficult for me. This being the lovely gift that Cystic Fibrosis can bestow, the ‘barrel chest’. As CF progresses and the airways become more narrow and inflamed due to infection and scarring, sufferers are unable to exhale all the air they breathe in, which causes the chest to remain expanded (by chest I mean ribcage, not breasts unfortunately!). Over time, this changes the shape of the ribcage and gives the chest a more rounded appearance. Whilst I don’t have a ‘noticeable’ barrel chest, my chest measurements are much broader than they were five years ago.  This gives my body slightly odd proportions, as I am a size 8 on my bottom half, yet I struggle to get a size 12 top to fit (unless it’s stretchy).  We trundled of into town after having researched dresses thoroughly first (so we could be efficient in terms of my energy and oxygen use!) and had my heart set on a cute red dress from Oasis. I don’t usually try clothes on in shops as it requires too much energy, but I felt strong enough to try it on and I didn’t even get breathless doing so on my new flow of 6 litres a minute, which I was pretty pleased with! (Also, if it didn’t fit poor Luke would have to bring it back the next day!) Sadly, the dress looked completely stupid and the size 12 was so tight around my ribcage I couldn’t bear to keep it on, let alone think about sitting down in it. Disappointed, but determined not to be beaten, we perused Debenhams and after a LOT of searching we found a dress that looked like it would be acceptable. This time I needed Luke to help me try it on as I was quite tired from all the shopping but it fit!!! Not only did it fit, but I felt really comfortable in it and it looked nice too! 

Admittedly my body shape and clothes shopping should be by far the least of my worries it can make me feel down, particularly when shopping for dresses. Earlier this week I was having a clear out of old clothes and quickly realised that over half my wardrobe doesn’t actually fit me anymore, due to my changing chest shape. I ended up getting rid of most of my old teaching wardrobe, as well as a plethora of other clothes – shirts, blouses and jumpers that simply don’t fit. Whilst ill-fitting clothes are not major obstacles I still felt despondent, and amongst everything else it simply made me feel further ‘trapped’ by CF. Cystic Fibrosis took my dream career from me (well, for now at least), my independence, my mobility and now it is infringing on something as mundane as my choice of clothes.

Me in my much sought out dress!

I don’t mean to sound vain or completely depressive, but this just illustrates the extent to which CF restricts my life. However, I must be thankful for the fact that I had the strength to clear out all those old clothes and I didn’t get too breathless doing it and I can look forward to amassing a new wardrobe post-transplant!