Today I was up at clinic to have my port flushed and pick up some drugs that have proved a bridge too far for my *ahem* crap *ahem* pharmacist to supply. More about him another time...

This was the third port flush that Brad has been attending as part of his learning process for being able to access my port himself at home. We both feel it would be an advantage for him to be able to do this as it means that if we are on holiday he can flush it and also that we don't have to do a 3-hour round-trip to the hospital every 4 weeks. At first I think he found it difficult to get his head round the idea of pushing a two inch steel needle straight down into his wife's chest. I've assured him it hardly hurts but I think its a strange psychological situation to get used to when you are not medically trained!

This appointment helped him quite a bit because the nurse gave him two ports to feel, hold and play with, along with a port needle. One of them was cut in half down the middle which was really good as it enabled us to both see clearly what is inside a port and how the needle needs to hit the metal back plate of the device when it is inserted. I think he can now envisage what he is dealing with under my skin better.

He still watched again today but I've told him that next time he absolutely has to have a go himself. I expect it won't go right the first time (even the nurse missed at first today because my legendary rhino-skin caused the needle to bend in half!) and I am expecting that he won't have anticipated quite how hard he is going to have to push down on the needle to get it through the skin, muscle and silicon centre of the port, but I'm happy to grin and bear it while he gets his confidence and I'm sure in the long run he will be doing it just fine without even thinking about it!

During the procedure one of my consultants came in and told me some good news. Well it may not sound like good news but it is to me...I am seriously anaemic! Why the positive response to this news? Because it seems that I have been anaemic for quite some time, which is exactly what I had suspected for the last 18 months but which I have never been treated for until now. I've struggled with tiredness for a few years but it has been particularly bad for the last 18 months-2 years...so much so that I have become more dependant on my wheelchair, have had to plan each day with meticulous precision so as to ensure I "spend" my meagre allowance of daily energy wisely (e.g. resting all day if I'm going out for the evening etc) and have been generally feeling so utterly exhausted that I can barely function a lot of the time.

About 18 months ago (when I was at my previous CF centre) I tried and tried to get the doctors to take this exhaustion seriously. Unfortunately I think they had already made their mind up that it was all in my head (my history of depression appeared to hang like a cloud over my notes every time they opened them) and after running various tests they as good as said so. Well they said that since the blood tests were all fine there must be a "psychological element" that was causing it, though when I asked what that was (taking into account that I was no longer depressed) they had no suggestions. Winner.

When I tentatively asked if having a Serum Ferritin level of 12 when the normal level is 15-200 could be an issue, I was told that although it was below normal, I didn't need any treatment for it and it was irrelevant. I tried to suggest that at least a trial of iron supplements might be worthwhile, as a final attempt to find a solution, but again was told this was simply not necessary and that actually iron supplements could lead to susceptibility to pseudomonas. Bad idea, please close the door on your way out.

I moved clinic a few months ago and things have been so different that sometimes I feel like crying with relief after visitng the team at my new hospital. They were determined that they were going to find ways of combatting my chronic breathlessness, exhaustion and constant infections. After running some specialised lung function tests my consultant identified that my smaller airways were functioning very poorly and he put me on a new type of inhaler called Spiriva that is usually used in patients with COPD.

Within about a week I noticed a massive difference in my breathlessness. I went from getting hugely out of breath just having a shower (and then collapsing exhausted on the bed and needing help with dressing) to being able to walk round the shops without my wheelchair and without experiencing any noticeable breathlessness. I could have kissed my consultant! The effect wore off a little with time, but I am still so much less breathless than before that it is amazing.

Next on their list of problems was my constant infections . A week after finishing a course of IV's (with minimal results) I would be back heading downhill again. My lungs were also bleeding more and more regularly. After 6 courses of IV's in one year, the team and I decided that an option other than yet more IV's was needed. They decided to try me on nebulised Tazocin, a powerful antibioitc that I am sensitive to but which is not licensed for use in nebulisers as yet (though has been trialled at one other centre before). The difference this has made to me in the last 8 weeks is phenomenal. I haven't needed any IV's or orals and my chest is better than I can remember it being for the last 2 years!

However the one thing that just didn't seem to shift was the exhaustion, barely altering with variations in my chest symptoms. With hindsight I have had the classic symptoms of anaemia - debilitating exhaustion, breathless, looking very pale, flaky nails, a constantly dry mouth and occasional heart palpitations. My team, determined that they would eventually find the cause of my fatigue went back through my recent blood tests results since becoming their patient. When they asked to do some blood tests two weeks ago, I suspected they might be on to something.

Today it turned out my iron levels are skulking around with a miserable expression on their face at a paltry 7ng/ml (the normal scale is usually taken as 15-200 nl/ml in the UK, although one research report concluded iron deficiency was a likely possibility with results below 50ng/ml)). No wonder my poor body has been throwing major strops for a considerable length of time! They can't work out why the levels are so low but I am now popping my iron tablets with gusto and have high hopes of being powered through the ceiling by tomorrow morning with my new-found energy levels. May be a little optimistic, but at least I feel we are finally starting to work out the conundrum of my complicated little system!

Introducing Sybil!

Apologies for the lack of blogging over the last couple of weeks. My excuse is that I have been spending my time dressed as a slut, being drunk and loud and copulating with old men. Some excuse hey? Even better, Brad has amused himself during this time by showing his bum to 200 people in our local village…which was nice.

May be I ought to explain…..

Three months ago I joined PVADS (Pewsey Vale Amateur Dramatics Society) and auditioned for their modern comedy “Denim” by Jay Saunders. Originally I was cast in an unspeaking role as a member of a girly-type Birthday party. Meanwhile Brad was cast as…a stripper….which made me laugh hysterically and think “Thank god I got away lightly!” whilst feeling highly smug that I didn’t have to do anything so embarrassing.

However at the second rehearsal the Director came up to me with some interesting news. One of the cast had dropped out and he wanted me to play the part of Sybil. SYBIL???? Oh SH*T!!! That will be the Sybil who has a relatively major part as a drunken nymphomaniac and who has to simulate sex with a pissed old man (played by a 73 year old chap from the village)? NOOOOOOO WAY!!!!!!

I very nearly decided there and then that I had never really wanted to be a thespian and that the role was rather too complex for my liking. But then I stopped myself from turning and running out the door by thinking that this was make or break. I have wanted to act my whole life. As a little girl I spent hours writing little plays in my bedroom and then performing them to my gathered family (not easy when you have to play all the parts by yourself and there can only be one of you onstage I can tell you…)

I remember so clearly the day I was told, at the age of 7, that I had been cast as an angel in the Sunday School Nativity play. I was beside myself and ran out to my Mum afterwards on cloud nine, full of thoughts of wearing a white sheet with tinsel in my hair and angel wings. When my Mum gently reminded me that we would be away for Christmas that year, in the exotic location of the United Arab Emirates (my first ever trip abroad) I absolutely wailed and pleaded with her to cancel the holiday!

At secondary school I had auditioned for the annual musical every single year without fail and never even got a non-speaking part. My school was exceptionally high-achieving and many of the girls there had private singing lessons in the lunch-hour. For some reason they never picked musicals with anything more than a small cast so the only people who ever got to take part were those who were very gifted singers and who, presumably, would make the school look extremely prestigious. Every year I would get so excited when the details went up on the notice board of who had got a part and I’d run up the stairs and stand desperately scanning for any sign of my name – but nothing. The let down was terrible.

When I went to my sixth-form school I joined the drama club and auditioned for the first play that they were putting on. You can imagine my amazement when the teacher became slightly overexcited by my audition and immediately cast me in the leading male role (that’s the trouble with all girls schools!). However this was a bit of a shock to the system after having been turned down for so long and I took one look at the pages and pages of lines I would be expected to learn and panicked.

Fortunately, or unfortunately as the case may be, whilst panicking over how to extricate myself from what seemed an impossible task, I was struck down with an appendix abscess which went undiagnosed until I was so ill that I was rushed to hospital with peritonitis and my parents were taken out of the room to be gently told I might not make it through the night.

Still, it got me out of the play nicely….

So anyway, back to the role of Sybil. I knew that if I turned down this opportunity then I could kiss goodbye to any dreams of acting in the future. I made up my mind there and then that I would overcome my natural inhibitions and play this role as well as I possibly could. Even if it did involve being on centre stage drunkenly singing “Happy Birthday to you, Have a shag cos you need too, shag him twice on the sofa and suck him off till he turns blue!” Seriously! Not to mention being grabbed from behind by an old man and oohing and aaahing as if we were at it. Eeeeek!!!!

Actually within the first few rehearsals I felt so at ease with the wonderful bunch of people there that it really didn’t matter anymore and we all laughed along together. The PVADS members are some of the nicest people ever and are a really fun and genuine bunch. They made me feel so welcome and were very understanding about my CF-limitations. Many of them are smokers but once they understood my problems they all agreed amongst themselves that they would only smoke outside and well away from me during rehearsals which I thought was really sweet since I hadn’t directly approached them about it.

Anyway, after 3 months of rehearsals the play finally opened on Thursday and ran for 3 nights. I’ll write more about it in my next blog entry in a few days time....

I can't stop eating.

No really. I've spent my whole life without an appetite but its been particularly bad over the last 6 months. Until now. Until the time that I have a gorgeous dress to fit into for the Breathing Life Awards. Suddenly I've gone from being someone who pushes all her food round her plate before scraping 3/4 of it to the side, to someone who is asking (yes asking!) for treacle pudding and custard after clearing her plate. My husband has been getting more and more stressed over the last few months, vainly trying to tempt me with something to eat or encourage me to drink my (disgusting) Calogen...and now suddenly he's running down the shop to stock up on ever-diminishing packets of crisps and Haribo.

This evening I was actually hungry an hour before dinner and polished off a bag ofHaribo and a raw carrot (a somewhat random mixture), then ate my dinner, then desert and now some savoury snacks. It's like a TV diet show in reverse. Picture the scene...Gillian McKeith stands before a long trestle table, her face a mixture of delight and revelation. She turns to look at me with a glowing smile. "Emma, just look what you ate in the last week!" The table contains a handful of plates with tiny helpings, a few apples and a bag of salad. You are amazing! You've lost half a stone in the last month! I can't wait to catch up with you week, you're a national inspiration....

Cut to the following week. Only this time Gillian has on her familiar scrawny, smacked-arse face and she's looking at me with a mixture of disgust and shame. The table this time is groaning with food. Bags of Haribo are split open everywhere, their contents spilling over into bowls of crisps and pretels. Snickers bars compete for space with sponge puddings and custard, fried chips are lined up side by side. And standing behind it all is me, wide as a house and wobbling about like a jelly.

Well, I may be exagerating just a teeny bit, but I have actually put on half a stone in the last 6 weeks (since going on nebulised Tazocin which has really helped me chest-wise). It's great! It's amazing! No struggles to drink Calogen, no sticking out ribs, no friction between me and Brad about my diminishing weight. BUT WHY NOW??!!!! I NEED TO FIT IN MY DRESS!!! Isn't that just Sod's Law?!! Ah well, I'm not going to stop eating so if worst comes to the worst I'll just have an excuse to go dress-shopping again hehe. Oh and please do tune in to watch the Breathing Life Awards on Five Life, Saturday 2nd May 2007. I'll be the one doing the impression of a space hopper with legs.....