Playing catch up

First off here's the article about my special gift to the hospice (here).© Manchester Evening News

Christmas Eve

I've always believed Christmas is as good as you make it as with other special events, days, making the best of what you have makes the day what it is. This year I didn't expect Christmas to be an easy going day and it wasn't but we still made it a special one and enjoyed it. Of course Santa was very good to me and spoiled me.
Not too long after that article had gone out in the local paper I got a call from the Manchester evening news. They said NHSBT had contacted them over the 'help save my life' article and Channel M interveiw to say that as a result 3000 people in Manchester signed up to the organ donor register. As I was told this over the phone my eyes filled up. At the time when it came out I thought, it's quite good maybe 1 or 2 people might sign up and even if its just 1 or 2 that’s enough for me, that's my job done. But to hear that 3000 signed up took me by surprise, the thought popped in my head '3000 people? And 1 person can save upto 7 lives?! That's alot of lives saved!' The journo asked for a few comments and said they would run another article over Christmas to report the response. And sure enough on Dec 29th I was again on the frontpage here. That cheered me up alot but to hear that Jessica Wales received her lungs after over 4yrs of waiting cheered me up more. A couple of days before my article came out I saw on facebook that Jess had taken a turn for the worse, in no uncertain terms when your ill enough to be on the transplant list that often spells out that time is up. Knowing this I texted a couple of friends trying to find out exactly what was going on and received details that that was in fact the case. Jess was clearly out of time and I thought it was more than likely within the next day or so I'd probably get a message saying time was up and Jess had passed away. But a few hrs after hearing Jess was critical rumours started to fly around facebook that she had had a call and it was a go. Just after midnight on Dec 28th it was confirmed Jess was in fact in theatre getting new lungs. The feeling of relief was over whelming. Of course not forgetting the donor family, they have lost a loved one over the Christmas period and during that time have been selfless enough to think of others and agree to organ donation and because of this Jess' life and probably the lives of a few others have been saved. Jess was so poorly going into transplant so there's no doubt her recovery will be lengthy and probably a little complicated, it's early days but Jess has been given her 2nd chance, at last. Jess' friends handled how ill she was and documented details about her transplant so well that it spurred me on to do my final part of planning which I'd been meaning to do for a while. My group on facebook, Rachael Wakefield's Transplant Plea has a following of over 8000 people now and is not really used anymore so I've made a couple of close friends admins and they're going to update the group for me when I can't because I'm hopefully getting new lungs. The group, twitter and my blog will be updated by friends and will be the most upto date forms of news. I will (situation permitting) update right until I go to theatre then my phone will be switched off until I'm able to use it. Mum will contact 3 close friends with updates and they will keep on top of facebook, twitter and blog. Now those final plans are laid hopefully it won't be too long before they are put into practice.
New Year's Eve was manic so more on that later.

long week

What a week I've had! When people say to me "what do you do to occupy yourself? You must get bored?" Ha! Chance would be a fine thing! So I'll start from last week.
Last Thursday as usual I went to the hospice, felt perfectly fine in the morning had some reflexology, had my NIV all set up beside me and was comfortable. By mid afternoon I got a strange feeling, I had palpitations, my heart felt like it was racing and I felt sick and tired. The hospice doctor prescribed some extra diazepam and said it'd probably pass. We waited it out for 5hrs before I was finally sent to a very busy, very noisy A&E. In A&E everything looked fine so I decided I was safer at home and signed myself out. I contacted my consultant the next day and arranged to see him the following Tuesday. That day I went to a support meeting at my transplant unit. Fantastic idea, they allow pre and post transplant patients to get together, share thoughts, ideas and tips on surviving (quite literally) transplant. It was a quiet meeting with just a few of us one very inspirational post transplant patient and I did also meet one lady, Ruth who was pre transplant, waiting 16mths. She mentioned the new start Christmas party that was taking place the next day at the hospital. I knew nothing about it so she pointed us in the direction of J's. J is lively, bubbly and lovely. She works in the new start office at wythenshawe and from what we could gather works hard! She sold us some tickets for the party for the next day.
I didn't really know what to expect, J had said she would introduce us to Sky and her family. By chance I suppose, Sky was leaving the ITU whilst I was being admitted for my transplant assessment back in August, she is the 16yr old girl I spoke of back then. She is now 5mths post heart transplant, in 9mths she's gone from feeling a bit poorly with a stomach upset to having an ultrasound scan and medics stumbling across her very diseased, failing heart, to urgently needing a heart transplant which she got within 4 days of being listed. There is only one word to sum up that story, amazing! Sky was inspirational and we got some pics together.

Pics with Sky

Also my surgeon (hopefully) was there and consultant. It was strange to see them as 'people' in normal clothes outside of their medical roll. It reminds you that at the end of the day, underneath the stethoscope there's actually a person under there. In the past I have not had the best relationship with my consultant and many of the staff at the hospital for that matter but now it almost feels as it should do and for that I couldn't feel more thankful and well, lucky. It was a good day I got a few snaps and it was great to meet staff and patients involved with newstart and transplant. People, mainly high up in the charity, were coming over wishing me good luck. You know you’re famous when people know you but you don't know them!

Handing a cheque to the chair of new start (right) and J (left)

I exchanged numbers with Ruth, we agreed to call each other if we got calls and I agreed to find Sky on facebook. A day later, around 7pm in the evening I got a call from Ruth. They had lungs for her, at that moment I grabbed my transplant phone, Ruth said they were calling in reserves incase she couldn't have it. At the thought I shivered. I sat thinking of her most of the night and wondering what I'd do if it was me now? It made it all hit home a little and gave me the push I needed to at least sort out my transplant bag! Unfortunately for Ruth the transplant didn't go ahead and at around 10.30pm she called back and told me about it, the lungs were in good condition but her antibodies were not compatible, I can't imagine how disappointing that must be. Fair enough if it's determined that your not a good match, or the lungs aren't good enough but to hear they are good and you were good match must be heart breaking. The day afterwards she just simply said "I keep thinking I could be transplanted by now :-("
On Tuesday I went to see my consultant. I wasn't looking forward to seeing him so much, I knew what was coming. I have gone downhill some what in the last couple of weeks. I've gone from needing NIV a few hrs over night to plus a few hrs in the day, to most of the day, to all day. It's a worrying trend that I don't like. He didn't have much to add just to highlight I need some lungs now and we're getting pushed for time. He promised he'd write to my transplant consultant and make sure he knew what was happening. I cried that day.
I will write about Wednesday’s ventures when I have pictures up, that was an exciting day.
Thursday I relaxed at day hospice and yesterday I went to the hospice party. The staff always bend over backwards to make it enjoyable and this year was no exception. Tinged with sadness as I gazed in the direction I sat last year with friend W who died of breast cancer this year. Remembering her family, her husband and three children that will be spending their first Christmas without her this year. There were laughs though too as always and I laughed (NIV permitting) until I had tears in my eyes and my cough forced me to stop. The hospice have seen me through some of the lowest and highest points of my life in the last 12mths and I gave them a special Christmas present also but more about that later when the article is online!
All in all a busy week so for those of you that think I lounge around doing nothing there you go!

Just breathe

Something I always wanted to do when on the transplant list I got the chance to do last week. The day after I was listed Sarah Milne from the charity LLTGL wrote a press release and my story went down pretty well. Being able to raise the profile of organ donation via the media for me gives me a focus, it gives me a voice when there's nothing to say or do but wait. For me it keeps my mind busy and if I'm honest it takes my mind of what's really happening to me and what I'm facing.
On Thursday at day hospice when the media hype had died down a little I was left to my thoughts and feelings I'd blocked out both physically and emotionally. The fact is over the last week or so I've been more unwell, I've tried to ignore it but by that point the fact that I was knocking back 20mls of morphine in one go so I wasn't in tears with carbon dioxide headaches was being questioned. I talked to a hospice nurse and we both agreed it couldn't be anything other than carbon dioxide. An appointment with an NIV nurse today confirmed this. This meaning either A) my VPAP was broken or B) my lungs had deteriorated and were less able than before to do their job. We figured seen as there was nothing to point to the VPAP not working it was the latter. I have occasionally used NIV in the day but it wasn't a complusory thing and I would just use it in the day if I felt a bit under the weather.
Since the day I went on NIV I always said it would never restrict me, I would not be stuck on an NIV in the house for the majority of the day watching life pass me by, over my dead body. Now I've arrived at that point, I'm dependent on NIV more than I ever have been before, to at least keep pain free and feel 'normal' I need to be on it 15-20hrs a day. Carbon dioxide headaches are not something you can ignore as much as you think you'd be able to in a 'oh its only a headache!' style, they bring you crashing down to earth, at times I have fallen to my knees had in my hands screaming and crying. High carbon dioxide affects everything, concentration, energy, memory even down to physical appearance.
So what am I supposed to do now? I need to stay alive and wait for these lungs and that, now, involves alot of time on a ventilator but how can I sit back and watch the world outside my window carry on without me? leaving me behind.
I always wondered, would I get on the transplant list and just think 'ah at last, the battle is over' and as soon as I think that would I physically give up before I got to where I wanted to be? Which as gruesome as it sounds, is an operating table.
The fact is this latest blow has hit me hard, of course I saw it coming, anyone with a progressive disease on NIV sees it coming but how do you prepare for the fact that for the majority of the day, for the majority of your life here and now you can't even breathe for yourself?

normal conversations...

Tonight I talked to someone on instant messenger, although we've met on a couple of occasions we don't know each other that well and very rarely talk. He is a normal 20something guy going about a normal life, the conversation is pretty self explanatory, to me it was eye opening as to just what 'being normal' is and how important it is to get the message across about organ donation. I'll leave you with conversation we had:

P says: ahhhh cool erm where do you actually fill in the forms for being an organ donor?
Rachy says: I take it that means you aren't on the organ donor register then?
Rachy says: http://www.organdonation.nhs.uk/ukt/default.jsp
P says: not yet been thinking of going on to it for a while now but was busy with job hunting
Rachy says: whilst your busy job hunting the hospital are busy trying to find me an organ donor in time to save my life, just think about that one?
P says: opps my bad major guilt trip
Rachy says: I'm sorry it wasn't intended as a guilt trip, organ donation is a personal choice
P says: its ok, no worries I'm not guilt tripped to be honest because I know there's a few people out there who don't even consider signing up
Rachy says: 96% would if they could just get round to it, imagine if they or you were in my position, I'd bet if you needed an organ to save your life you'd accept one but would you take the time to sign up to give one? it seems the answer to that is already obvious doesn't it? *gets off soap box*
P says: Yeh, I see your point now, I'm in the middle of signing up
Rachy says:
:)

I did it!

Well I've waited quite along time to write this blog and now I have no idea how to word it?

I did it? I made it onto the transplant list, I'm now active on the transplant list waiting for a double lung transplant. It doesn't even seem real to type that, I don't think it's actually sunk in yet, that I will actually get new lungs and I will live. That is if a donor is found for me in time.

My news came yesterday afternoon after a tearful morning. I discovered that one of my friends Joanne had passed away in the night, Jo was on the transplant list waiting for lungs. She had had a tough time getting listed herself , I supported her through that. Just a few hours after discovering she had passed away I went active on the transplant list, life at times puzzles me so much.So off I went to clinic yesterday, we got there early and stopped in the cafe for a coffee then almost got lost in the corridors trying to find our way to clinic. My co-ordinator was hanging around in the waiting room, I don't actually get to see her that much, she doesn't do my consultant's clinic that often so it was nice to catch up with her I asked if my tissue typing was back and she said it was. It was at that point the penny dropped and I thought 'oh god, there is actually nothing stopping me going on the list now...' But I put it out of my mind and got talking to a lady beside me who had Alpha 1. She had been on the list 2yrs already.

I went in to see my consultant and he went through last minute details mentioning my gallstones could be due to weightloss and so on. But that there was no inflammation in my gall bladder and it looked ok, I did, if I admit, zone out a bit, as a watched the goings on out of the window a stark comment brought me back to my chair "shall we just do it then? Shall we go for it and get you on the list? stop dragging our feet ah?" before I could collect myself to think of a sensible reply I just raised my eyebrows and simply said "erm yes please?" and that was that. The appointment was no longer than 10minutes, I bumped into my co-ordinator in the corridor and she shouted "Rachael?" and put her thumbs up and I nodded and put mine up. The co-ordinator that was in with us took my 'transplant line' number. I have put an unused sim card into an old phone and I'm reserving that number just for transplant calls. Just so I don't jump everytime there's an unknown number flashes up on my phone!
Which at the moment is happening alot because the lovely Sarah Milne kick started the LLTGL Christmas appeal today with a press release on my story (click here to view it). My mobile has been ringing off the hook! The MEN have snapped up my story and are coming round for a photoshoot tomorrow.
So that is it now, now I wait, wait for a donor, for a hero to save my life. My story is now also on the LLTGL website, go have a look here. If you sign up to the organ donor register you could be my hero so go sign up here!

I'll leave you with a picture that Sammi took last week during the photoshoot, many more to come.

Crazy life!

My life at the moment seems to be all or nothing. Literally for the past week I have sat twiddling my thumbs doing nothing, then the last couple of days it seemed to all come at once.
Yesterday I called the transplant co-ordinators to check they had my ex-vivo consent forms and also for a bit of a catch up. No sign of my blood/tissue typing results yet, I can't go on the transplant list, officially until they come back. I've been waiting 3 weeks now. Whilst on the phone I asked for the number for the transplant dietician. I met her during my assessment and she had alot of understanding. I have struggled with my appetite for the past couple of weeks, then to top it off I caught a sickness bug last weekend and wasn't even able to keep fluids down properly for a couple of days. That combined with pretty poor intake as it is has meant I've dropped another 7lb that I didn't really have to drop in the first place and haven't bee able to regain. Being a bit on the small side is something very new to me, as a child I was always a good weight then as a teen I went on prednisolone and well from that point on there was never any worries about me being underweight (those on prednisolone will know what I mean ;-)
I had a chat with the dietician anyway and she advised going to my GP for better anti sickness medication. But it isn't just sickness and nausea that are the problem, after I've eaten pretty much anything now I feel bloated and unable to catch my breath, it is a battle at the moment and it isn't one I ever anticipated. And 'little and often' really is of no use to me.
So following my chat with the dietician I got a call today from the co-ordinators, my consultant now wants to see me ASAP before I go on the transplant list, I have an appointment for about 10 days time. Hopefully around that time my blood results should be in as well. So that didn't worry me too much, the part where she said "to check your well enough for transplant now" did. Weightloss in ILD is a sign of progression. I can't not be well enough to be listed for a transplant, I've come too far so I intend on doing something about it. I put as much effort in as I can into eating and it's getting me nowhere alone, it just isn't easy anymore and it's not even something that comes natural, I see food now like medicine, something I have to take but there's no enjoyment in taking it, my lung disease has taken that enjoyment away. So now I'm seriously looking into nasogastric feeds as an option, they could run overnight whilst I’m on my bipap and breathing wouldn't be so hard then. If anyone has any info about this I'd appreciate it.

Next week, hopefully, I have a photoshoot at the hospice with the beautiful Sammi Sparke for her photography degree. For some reason she thinks my mug shots will win her a prize haha!

I've also agreed to do a media feature at some point in the next week for the charity Breathe On UK. With the case of baby RB being all over the media this week Breathe On have had alot of coverage, they support families affected by long term ventilation. So watch this space.

On Wednesday I also have a CT scan to get to the bottom of these lung bleeds which are yet to subside.

Thursday, I have day hospice, I've been helping out with the St Christopher project there, which came about at the hospice thanks to a lovely lady who I was great friends with (click here and she's mentioned at the bottom of this blog along with why the St Christopher project is vital to school children) she passed away just a couple of months ago. Basically the project myth busts over what hospices are actually for. And tries to get the message across to the public that they are actually a place of life.

Busy times ahead!

This week saw the launch of the powerful new campaign by NHS blood and transplant in bid to highlight the need to act on good intentions.
''The campaign aims to increase significantly the number of people on the organ donor register, asking what we would do if someone we love needed an organ: if we would accept an organ, shouldn't we be prepared to give one?'
More to come later, for now I shall leave you with the very powerful advert which we should be seeing more of on TVs soon.
Link to YouTube.

What a rollercoaster the last few days have been. I had the pleasure of meeting my surgeon on Wednesday at transplant clinic. Great man, in the respect that he does actually save lives, with organ donation, yet he does not come across as thinking he is some god, which is a fatal mistake of some doctors in my opinion...
I sat in the waiting room wrapping my oxygen tubing round my fingers, a bag of nerves. After a while one of the co-ordinators came out with my consent forms which she asked me to sign to which I replied "what? Now?!" and I signed.
She came back out and led us through to a room with several people in it, two students that were interested in the field of transplant, the consultant anaesthetist, the surgeon, she took a seat and my parents sat beside me and the room wasn't actually that big! The appointment was over an hour long. The surgeon went over the basics of the procedure, what was important pre transplant, survival rates, post transplant complications. He stressed how important keeping physically mobile was to recovery, survival rates increase by over 50% if your able to do at least something every day, even if its just pottering round the house and next week I'm going to talk to a local physiotherapist and see if I can get access to pulmonary rehab. I got a sense of 'this is going to be no walk in the park, this is big...' and I've never felt like that before. I've focused so long to actually stay alive long enough to make the list I don't think I'd appreciated, fully, what being on the list actually meant.
Survival rates, I knew roughly but to have them directed at you face to face is a bit shocking, on average 50% of double lung transplant (bilateral) patients are alive in 5yrs, for single lungs its slightly less at 46%. Those are averages, no one can say for sure how long a transplant will actually last and they have patients celebrating their 18th, 19th and some 20th years post transplant. Only 10% don't make it through the actual operation and post op period, which when you consider how big the procedure actually is and whats involved that is a good figure. They can't say how long you'll be in hospital because no one can for see what complications there could be, 3wks is the minimum.
There is a whole host of post transplant complications which he went through but the basic things I needed to know we're weight control post transplant is very important, the medication can make you gain weight, they do not have enough data for post lung transplant but they know in heart transplant patients that if the BMI goes over 30 75% do not even make it beyond 2yrs. That, to me, is shocking, it really does make that much of a difference. And antirejection drugs. Without those he explained, you don't breathe, they are the reason you keep breathing post transplant, they come before anything else in your life, even food and drink. You must not miss a single dose, the immune system can be switched back on very quickly and if you miss doses rejection starts to take place almost straight away. I could not ever be imcompliant post transplant, I would feel too guilty on the donor family, the transplant team and my parents and family that are going to see me through this. We then discussed the pros and cons of bilateral then single lung. Basically bilaterals are harder to come by, I am small framed now, the size has to be 100% perfect or the lungs won't fit, I'm 5'4 and a size 6, my donor would have to be my size or smaller, I'm hoping that isn't as difficult as it sounds. Bilateral means the wait will be longer because of the sizing and the fact that sometimes one donor lung is good and another isn't for whatever reason, the operation will be bigger and longer, the recovery will be harder and longer but the survival rates are better. Single means the size doesn't have to be perfect so the wait is shorter, they're easier to come by, the op is shorter so it's easier to recover from but the survival rates aren't as good. On Wednesday he was undecided about which was our best option and said it was up for discussion in a meeting to be held yesterday. The surgeon said he can only advice on what he believed was best and if I came back to them and said it had to be one or the other they would be guided by me, he explained it was my lungs, my op, my call, my life. I'm not used to being able to have so much of a say in things. I talked to alot of online friends that are on the transplant list (thanks to those that listened to me waffle and ask questions!) and waited for some feedback from the meeting. The team decided bilateral was our best option, I'm young for a lung transplant patient at a smaller centre, the survival rates are better and due to the fact I don't have a 100% accurate diagnosis it's a safer option. Blood was taken and when tissue typing and antibody screening is done I will go active on the transplant list, this will take anything from 3-6wks. In the hope that it may shorten my wait for a match I have decided to sign up for the ex vivo/lung reconditioning study. I will talk a bit more about that in my next blog.
Thankyou to everyone that's supported me over the last 12mths, I couldn't have done it without you :-)

I am learning all the time, sometimes even when I don't realise it. The lessons I learn are sometimes hard to swallow, they don't always come in the easiest form but the hardest lessons I learn are the most valuable one's. A comment, or a few recently from a post transplant patient made me sit back and think for a second and if I'm honest upset me more than I even realised. They made me question if what I was doing by going down the road of transplant was actually the right one. After a chat with other post transplant patients I'm now left wondering why I let those comments get to me so much. They taught me a lesson, they taught me I love life and always will, it may not always be easy but it is worth every second. Transplant may not be the right thing for everyone, its a personal choice, my mind is made up, I want to get better, I want to live, if it that means having a transplant then so be it. I will tackle it like I do with everything else that life throws at me, to the best of my ability, I will never take one single breath for granted because I know I'm lucky, every day I wake upto is a bonus.
My transplant co-ordinator called this morning. They have a multidisciplinary meeting once a week, involving surgeons, co-ordinators and my consultant, it took place this morning and by the sounds of it my case was the topic of conversation. I called earlier in the week for an update and was told my case was being discussed today and that if the surgeons are happy I will be sent an appointment to see them. When I asked how long this would take I was told 4-6wks, I was a little bit deflated but happy to know this. So this morning after the meeting my co-ordinator called, the surgical director and head of the transplant program wants to see me on Wednesday. My co-ordinator seemed keen to state that at this point it is still an 'if' I go on the list situation as ultimately the decision lies with the surgeons. The paperwork which I'm required to sign should the surgeon agree to transplant has been sent to me in the post, this will then be sent to Transplant UK and they will contact us when I go active on the transplant list. My consultant as promised has fast tracked my case and I haven't had to wait to see the surgeons. I'm told he will spend about an hour talking with us, discussing the surgical details of the procedure, potential problems and will answer questions which I must get writing down. I am quite looking forward to meeting the surgeons, even if at this point it is a no (everything looks good, everyone is confident, I've got this far but it's still a possibility) and hearing the details of transplant, people seem believe its a case of old lungs out, new lungs in but I'm inclined to think it must be a little more complicated than that!
Any positive thoughts would be greatly appreciated :-)

Time for a catch up. The infection I talked about in my other blog got worse and I gave up and went in hospital for IVs. I pushed it and pushed it though and before I knew it a week had passed since my clinic appointment. I was so very worried about the blood results, I'm terrible for over assessing things and worrying. The thought of potentially having to go through the process of seeing kidney doctors and haematology before transplant could be reconsidered was terrifying. Even though my doctor promised he would rush it through if that was the case it would still take months, I knew that. I became very down through worrying, then on the Friday before I was due to call for the blood results on the Monday something arrived in the post. I hadn't ordered anything so I didn't know what it could be. I opened the envelope and there in 2 neat little bags were 2 bracelets, no note and no explanation. Then I realised what they were and my eyes filled with tears. A friend of mine who was awaiting a lung transplant passed away on April 6th after a long battle with cystic fibrosis. Before she died and whilst very unwell she started making bracelets to raise money for the CF trust. I ordered the bracelets March 21st and after not receiving them and then hearing the worst a few days later I presumed she hadn't had time to make them and that was ok, my donation had gone to the CF trust and was now part of her memory, I didn't expect to receive them. Suzy always spoke so warmly of the NHS and the treatment she received from her CF team, she sympathised with my struggles with doctors and my fight for life whilst I was in the hospice. I like to think the bracelets just arriving out of the blue like they did were her way of saying it WILL be ok. Later that day I went into hospital to start IV antibiotics.
On the Monday I phoned the transplant co-ordinators, my co-ordinator answered the phone and she brought up my results on the system. Both blood results came back within normal limits, no further intervention was going to be needed. That was the boost I needed having spent a difficult weekend in hospital. My case was to be discussed at their weekly meeting on Friday and things would go from there.
That week in hospital was not easy and to make thing worse, my NIPPY which I had been relying on almost 24/7 because I had an infection overheated and broke. I should have seen it coming, NIPPY ventilators are old, noisy and recently it had been stated that mine was not fit for it's job anymore, it was providing enough support. After a few hours of being off it I felt the affects my carbon dioxide starting to raise. The doctors began to worry and asked the physiotherapists to see me to try and help me clear my lungs. The consultant physio who I know well came to see me. She knows the situation and sorted me out with a hospital non-invasive ventilator (NIV), lucky for me the NIV service had been set up just a week ago, if I wasn't able to have NIV on the ward eventually I would have had to have gone to the high dependency unit. The NIV I was set up with was called VPAP (click for explanation!). It was so much better than my NIPPY and helped a great deal. My consultant contacted the regional NIV centre (which also happens to be my transplant hospital) to ask them to sort me out with a new NIV.

On VPAP with my quilt

Whilst all this was happening I received a very special gift from a company called quilts for adults USA. They had been working on my quilt for months and it arrived whilst I was in hospital I snuggled up to the quilt and imagined it as a big hug! It includes cross stitched squares of my favourite things.
A week or so later I was fit enough to travel to chest clinic and meet the NIV team, I have met the doctor before, under different circumstances which I'm not going to go into here but he had a look at my blood gases and we had a chat. I had been off the VPAP for 3 hrs and my carbon dioxide levels had already risen. He was happy to set me up with one of their VPAP machines, he adjusted the settings a little and asked me to do overnight pulse oximetery to check the settings were ok. I was so relieved, VPAP,is smaller, quieter, portable and now I have an expert NIV team on my doorstep, I couldn't be more grateful. I returned to the ward at my local hospital with the VPAP, decided seen as my IVs had stopped and I was able to use the VPAP I had been using on the ward at home I was ok to go, I ok'ed this with my consultant who was happy. I called the co-ordinators to let them know I was home and asked for an update. My consultant had spoken with the surgeons, their happy for things to go ahead, pleased with the blood results, don't see the need for a total lung capacity and they're now waiting on my consultants’ referral. Once that is written I'll go and see the surgical director and hopefully be offered a place on the list. For the moment I'm on strict rest due to a couple of lung bleeds, will update again soon.

Transplant update (long one!)

Well despite some worries that I may need some IV antibiotics I made it to transplant clinic yesterday. I've never seen so many patients on oxygen in one place, obviously I knew there were going to be patients there in similar positions to me but it made me feel slightly less 'unique'. I over heard some of the conversations, unfortunately I wasn't in the waiting room long enough to actually join in (which is unusual!). A patient opposite me (I had read this article about him some months ago but he looked so well I didn't even know it was him) was telling another patient he received his lung on christmas day 2008, after 11 false alarms! 11!
Not so long after my consultant called us in, we got seated and he began to run through some of the assessment results. Based on the information they have obtained about my lungs he is as sure as he can be that a lung transplant is now our best option. He spoke positively and admitted he was very keen in light of a couple of the results to get me on the lung transplant list as soon as possible. I may have waited a while for the assessment results but that time has given my doctor time to talk to my London team about my results and about what is going on in my lungs in general. The assessment has given them confidence that they're doing the right thing by me. But, there are a couple of buts. A couple of my blood results came back abnormal. One of my clotting factors came back low. If this is low the risk of excessive bleeding during transplant is increased which with such a huge procedure could be a problem. The condition can be genetic but has never been picked up before so I had more blood taken and we're hoping, as is sometimes the case the result was just a bit out. It isn't impossible to transplant with such a condition but the opinion of haematology alot of caution is needed. My consultant reassured me they did a lung transplant on a patient just last week with a more complex clotting condition and the surgeons were able to replace the missing factor during the transplant and the patient continues to do well. Also my creatinine clearance came back high. Creatinine clearance is global kidney function, the higher the level the less efficient the kidneys are at cleaning the blood. Kidney function for transplant is vital, the anti-rejection drugs post transplant can hit the kidneys hard and because of this it isn't unheard of for transplant patients to later need kidney transplants. He advised me poor kidney function can be treated with dialysis but in the transplant recovery period this isn't ideal. Again this has never been a problem for me and no signs of a decline in kidney function have ever been spotted. I had more blood taken to check this as during the assessment when my blood was taken I was nil by mouth waiting to go to theatre for the RHC and was probably dehydrated so we're hoping it was just that. I have to call the co-ordinators Monday and check the results. If this time they're normal my case will be passed to Dr Yonan and I will be offered a place on the list. If they're the same discussions between the surgeons, haematology and the renal team will need to take place and I may need to see both teams for further investigations. Overall my consultant was very positive that at some point I will get on the transplant list, he praised me for my hard work, the amount of hoops I've had to jump through to get where I am and told me I had been through more than enough. This time last year our doctor-patient relationship was none existent so I was a little taken a back by the praise but I'm now thankful my hard work to get where I am and just stay alive is being acknowledged by the right people.

Late night rambles

After the shocker of a bad few days last week, I finally gathered from my lungs it is infact infection. I was happy about this, why? Well because infection is something that can get better and go away, it isn't just my lungs deteriorating, it is infection that is 'fixable'. I'm on lovely big doses of antibiotics at the moment and coughing green gunk with every bit of energy I have but I am getting there. And I managed to fit in an awesome few days away on a spa break last week. It was at times hard work, ok so sitting around in a spa for a few days may not seem like it but I do have less than 15% lung function now, just the sight of a flight of stairs makes my toes curl up. But it was so worth it. It gave me much needed head space to get things into prospective. I looked out over the balcony in the spa and just felt relief, I felt lucky, I felt lucky to be me, to be where I am and to lucky I'm still here.
It amazes me in the face of so much adversity, facing so much uncertainty only then do we really appreciate who are, what we have and in my case what I'm still able to do. And it was then I thought 'if I don't make this transplant list then I was never meant to and I can accept that now'. 12 months ago I didn't even come close to accepting that.
Fact is I've learned to love my life, it may not be perfect but it is my life and it is the best I can make it. Being offered a chance at transplant would be amazing but I think I can accept it now if I don't get the opportunity to go on the list.
Tonight my thoughts turn to Jessica Wales. She's been waiting for new lungs for 4yrs, 4yrs too long.

The machine currently keeping Jessica alive...

The prospect of losing Jessica because she can't hold on any longer for a donor horrifies me, the fact she's held on for 4yrs already horrifies me even more and to think her wait could now well be in vain is just horrible. The fact that I could also be potentially facing this myself, well I think I would need tips on how to cope because I don't know how I would, my mind is yet to even get that far ahead. Nobody deserves to wait that long for an organ donor, someone to give their organs after their death when they're no longer alive and no longer need them. Not difficult really is it? The really sad thing? Jessica is one of many desperate for a transplant tonight, why would you not want some good to come out of the bad? Why would you not want to give someone (or more than one person in many cases) the chance to live after you've died? Would you not want someone to do the same for you? Nobody likes talking about dying but I believe facing upto our fears makes us more human. Think about it, talk about it, do something about it.

It takes alot to scare me now. Having been through what I've been through. Right now I'm scared. My mentality, with transplant in particular has always been 'hold on a bit longer and you'll get there, just a bit longer to the next appointment'. I'm so stubborn that that has not failed me yet. As silly as it may sound, seen as I was given just days to live in a hospice exactly 12mths ago its only recently that I've really realised that its going to take more than my stubborn mind set to keep me alive. And that as much as I desperately want to cling onto life and the chance of a transplant my body might just fail me before that's possible. Right now things are bad, I'm determined I will not fall before this (hopefully) final hurdle. I'm not religious but I talked to a family member recently that is and since then I've been praying, I don't quite know to who or what, just praying. Please keep everything crossed that my clinic appointment on the 22nd goes well.

Pics

It's times like these that make me appreciate my life and help me to realise just how special it really is.

On Monday I'm going away to spend time with my family in Wales, I'm looking forward to the break and spending time with the people I love.

News

So there's news. It was what I expected I guess, Pre Transplant clinic 22nd September. It just puzzles me where 'urgent' comes into that. I understand its holidays, everybody is away and it's a multidisciplinary meeting, involving co-ordinators, dieticians, social worker, physiotherapy, my consultant, so everyone has to be gathered together. Alot of people will say "wow 4wks to wait for an appointment is nothing". But it will be a year since I was in the hospice where I was given just days to live. I don't have the luxury of being able to wait around for 4, 5, 6wk appointments anymore. I've waited upto 8mths before for appointments and there was no way around that and I just had to wait and that was that and it looks like this is the same. I have done my fair share of waiting and I understand why you have to and I'm not an impatient about it, I just know that time isn't on my side anymore and should I get listed I could be in for a anything upto 4yrs wait for a donor so I'm conscious of that also. 4wks is the best, the earliest they can do, I accept that and I will wait, you know what they say about good things?...
I think my mum was more upset about it than I was. We had expected earlier but I had been told it would be weeks before we hear anything so I had braced myself for around the 8wk mark. I think more than anything I'm just grateful I know when it's going to be and knowing that things are happening without me having to poke the stick. Something I've always done and something I believe in the NHS that at times saves you from being just another file they brush to one side. Also the plan had been to see the surgeons after the review in clinic so I may not actually go active (provided there's no medical reason I shouldn't) until after I've seen them. If it's a yes I can go on the list that is, we don't know for sure because the results are still being collected from the assessment. And then I will have to wait for another 6wks+ to see the surgeons, to do chest measurements and final paperwork etc.
So there you have it. I could be looking at another 12 or so weeks for everything to actually be tied up and done with.

Waiting to wait...

As the title says, I spend my life waiting. Right now I'm waiting for the results of the assessment to come through which ended now well over 10 days ago. The story of my life! Then I'll have to wait to see the team in clinic for a discussion of the results and hopefully an answer.
I have a few things going on at the moment and I'm overly, stupidly tired. I haven't been able to keep up with physio not because I'm too out of breath just because I haven't got the energy. I have never felt fatigue like it but I think its just of those things that comes in the package. I am waiting (yes also) for a referral to the regional non-invasive ventilator (NIV) team to go through because the NIV I use is over 15yrs old, sounds like a helicopter and just isn't upto scratch anymore, its so old that nobody knows what to do with it or how the settings work. I have been getting the distinct morning headaches and nothing compares, I think overall my symptoms would decrease a great deal if I was getting a good nights sleep on an NIV that was doing its job affectively but I'm not... This could also be the cause of how stupidly tired I've been of late.
I've also had a case of sinusitis (we think) that's lasted weeks. It was so bad at one point I was hitting my head against the wall and clenching my teeth. I've been popping Sudafed like smarties. When I went into hospital the transplant team put me on strong antibiotics for MRSA. Rifampicin and Trimethoprim, I'd been taking Ciprofloxacin at home for a few days but it hadn't really had any affect and if it's MRSA it wouldn't have, I guess I was hoping it would be a simple infection. I've had MRSA for many years now and never been clear of it since I caught it, the sinusitis is still there a little but is alot better so I'm starting to wonder now if I have it in my sinuses. It's in my nose, throat and has been grown in my lungs before so it wouldn't be surprising if it was. I may talk to the transplant team about staying on the antibiotics a bit longer because they have definetely helped. I also have some crap going on with a previously MRSA infected finger right now as well but thats another story. So that's my life right now, in terms of how long I'm going to be waiting for the transplant assessment results I really don't know, the blood results should be back this week then I have to wait to see the team so we can have a chat and decide...

Reading the 'lung transplant patient information booklet' given to me by a co-ordinator

Well I finished my assessment Thursday and was discharged afterwards. I had 3 tests on Thursday, lung CT scan with dye, an abdominal ultra sound and an oxygen study. I didn't hear anything about the lung CT because a radiographer will have to have a look at it but the abdo ultrasound I got to see on screen. The doctor doing the ultrasound was nice and pointed everything out to me and then said "ah, you have gallstones!"
"What?! Seriously?" The student nurse that was accompanying me to my tests that afternoon jumped up out of her chair for a look. The doctor pointed out the tiny white masses on the screen and explained they were gallstones and there were a few of them. I panicked and asked "will they affect the transplant?!" I was reassured no but it was something my doctors had to be aware of and was told 80% of people with gallstones don't even know they have them. That came as a bit of a shocker, I wasn't expecting anything out of the ordinary to come back on an abdo ultrasound, it was the least of my worries! To my relief everything else looked normal.
Next I went to lung function for an oxygen study, the oxygen nurse took one look at me and said "errr yeah how far can you walk?" I told her roughly and she took a blood gas from my ear based on the results she said that was all she needed to do and I could go back to the ward... I was expecting the oxygen study to be difficult and it was far from it.
Later that afternoon, once back on the ward I saw the transplant dietician, very understanding, very nice woman. I told her what an average days food was for me and she was concerned over protein. Protein is one of the main things post transplant that patients need to rebuild muscles and gain strength and its one of the main things my diet is missing. She checked my protein stores using a small hand held device and said despite not getting much from my diet my stores were good, she took note of this and said post transplant I may have to stay on protein shakes a bit longer than their average patients because I don't get enough of it from my diet but that's no problem. She told me to watch my weight and warned if it got any lower I'd need to consider nasal gastric feeds, I've had them before and considered they maybe in my future so I wasn't surprised by this.
That evening I was discharged. Now once again I wait...

The above picture was how I started my day at 6am... I'm not a morning person and by the time breakfast came at 8am I couldn't stomach it and was whisked off for a chest and mouth xray anyway. Soon after a surgeon in blue ITU scrubs came and introduced himself. He worked for my consultant and came to explain the RHC that he'd be doing. I asked a couple of questions and consented. He explained I'd have to lie flat for a while and asked how I was with that, I don't do completely flat on my back very well so he asked me to take my NIV with me to theatre. I was nervous to the point of shaking in theatre, the staff were fantastic though and really put me at ease, they put on some cheesy music and talked me through everything they were doing. The only painful part of the procedure was the local anaesthetic being injected into my groin, I've had local more times than I can even remember and it's one of those things you grit your teeth through knowing it'll be over in a few seconds. Once that was done with the line was inserted and threaded up a large vein and into my heart. I could feel a few strange sensations in my chest and back then when it reached my heart it caused a few extra beats which I could feel. The line fed data about the pressures in my heart back to a computer and after a few minutes it was over. I had got myself worked up over nothing and if I had to go through it again it wouldn't bother me in the slightest. I had to lie flat on my back throughout and then for an hour when I got back to the ward.
Not too long afterwards the transplant physio came for a chat. She went through what her role would be post transplant how important exercise and mobility are post transplant and what I can do pre transplant to ensure I go into the operation in the best possible shape. She explained people that do exercise pre transplant recover more quickly post transplant. Exercise has a huge impact on recovery, it can make the difference of a recovery that takes a few weeks to a recovery that takes a few months. Despite my failing lungs exercise is something I enjoy doing, I can't do all that much to help myself but exercise is one of the things I can do to help myself so I make sure I do it. If I want to walk somewhere or do something for myself it may take me a while and I may have to stop and rest but I make damn sure I do it in the end. The physio was impressed by my attitude towards this and left me with a few simple exercises I can do to keep my muscles strong. Later the co-ordinator came back to see me, she went over a few last details, what tests I'd already had and what I needed and explained they'd gather the results from this week and present them to my consultant on this coming Tuesday, based on them he will either see me in clinic and explain why I can't be transplanted or pass my case to the transplant surgeons...

The gift of life

Well Monday was a busy day. My mum called the transplant unit and they still had no beds. I was pretty upset by this point and just wanted the assessment I had been promised. I had lung function at 11am so they told us to bring a bag incase a bed was free afterwards but it wasn't looking likely. They had a 16yr old girl who had had a heart transplant ready to leave the transplant (tx) ITU and she needed a side room.
So we could get there in good time we left the house pretty early, I was tired and in a bad mood. Not a good combination. I tried my best at lung function but the machines were giving a poor picture so for a few of the tests I was taken off the larger machines and put on an old style smaller machine on wheels. I then had blood taken from my ear to check my blood oxygen levels, they turned out to be very poor on my 8litres of oxygen so I was also a bit upset with that. After I'd done lung function they called the transplant unit to find out if a bed had come up and if I could go home. They said no I couldn't because one had become available but it would be in the afternoon so we had to kill time in the hospital from 11.30 until I got to the unit...
Once I got there I met some of the staff, one of the doctors clerked me in and one of the co-ordinators came for a chat. She was going through a list of things including if I got on the list how I'd get to the hospital, contact numbers and after a few minutes her bleep went off. She went to go answer it and it was a heart being offered for transplant. She then obviously had to dash off to arrange the transplant but left me with the lung transplant handbook to read through, she said "this will be your bible, most of what you need to know you'll find in here but I will come back and talk to you more". I hadn't expected for things to be so busy and intense on the unit.
The co-ordinator came back today and took me through what would happen step by step from now to life post transplant. She highlighted the sad fact that some people don't get their call in time and in light of this life must carry on as normal as possible because life sat beside the phone waiting for it to ring could be a wasted one. She also stressed that transplant shouldn't be seen as the cure, it is an extension of life. She used emphysema patients as an example she said "they could be ill but tick along for 10yrs or so. If we transplant them they may only get 5yrs but the hope is that those 5yrs would be better quality than they could ever hope for." She then spoke about donor families and post transplant contact and how they encourage recipients to write to thier donor families. She used an example of a card she passed to a family recently, a heart and lung transplant patient wrote and thanked her donor family on her 16th transplant anniversary and how over whelming it is to think a decision they made to help other people live by choosing to donate their loved one's organs 16yrs ago is still making a difference today. There is no bigger gift than the gift of life, organ donation is a miracle. We then moved on to post transplant life and she explained about the drug regime and general health and the fact that not only do you owe a good, compliant drug regime to yourself you also owe it to staff that have saved your life by giving you a second chance and also to the donor family that had to make one of the hardest decisions of their lives at one of the most difficult times of their lives. That for me puts it into perspective, a selfless family say yes to donating a loved one's organs to strangers they may never meet but wanting to save thier lives anyway, how can you not want to look after yourself and your new organs post transplant? I would feel very guilty if I didn't.
Tomorrow I have one of my biggest tests, the right heart cath, I am nervous but I think its more because I don't know what to expect than anything else.

There was no bed for me on the transplant unit. I don't know what's going to happen now, I'm going to the tests that are booked and that I can go to as an out patient which is only a couple. They tell me I'll still a priority and they hope a bed will come up over the next couple of days, I'm not holding my breath.

One down

Yesterday I completed test one of the transplant assessment. I want to say I was a little surprised by what I saw yesterday but I wasn't really. I'm not stupid, I know I'm not going into this transplant in the best of condition but still what happened yesterday took me by surprise a little. I was sat in the TDC waiting area waiting to see the dentist. Transplant requires you have good teeth, I have recently seen my own dentist but its a case of having the boxes ticked so I had to see the transplant centre's dentist also. Whilst we were sat chatting and waiting a lady in a chair and dressing gown was pushed in front of us, she said thankyou to the porter and off he went. A nurse approached her and verified who she was and that she'd come from the transplant unit. After a few minutes she looked at me and said "I hope you don't mind me asking but that oxygen you have in that bag (a back pack) do you find it heavy?" I said "yep that's what I have my carer for" whilst looking at mum! She then replied "yeh I have oxygen too but I don't use it when I'm supposed to because I find it too heavy to carry".
I sat and thought for a minute, this lady was in her early 60s, was probably in for a lung transplant assessment, yet she didn't look out of breath and could pick and choose when she used her oxygen. I tried to get my head round how different our situations actually were and just couldn't. I knew I was going into transplant in a not so great state but that was a shocker. Anyway, the maxillofacial department seemed to think I was in inpatients also and as a result my notes were on the transplant unit, a nurse came out and said "is it the cystic fibrosis (CF) unit your under or the transplant unit?" I looked puzzled "neither at the moment, I'm on the transplant unit next week". A common mistake alot of medical staff make, they presume you have a condition before you even open your mouth, because I'm young, on oxygen, quite small and going into transplant they presume I have CF. You know what they say about books and covers? Well that couldn't be a better example.
I got called in to see the dentist, he looked terrified and so was very accommodating, didn't bother to push the dentist chair back let alone do an xray, gave me basic oral hygiene info and said all was fine. Thumbs up there!
The rest of the assessment takes place next week, I'll be taking my netbook so I will be blogging probably late at night when I have allsorts flying round in my head and no I don't mean the sweets... I'm not worried about the tests themselves, apart from the RHC they're all tests I've had before, I've had so much bad news lately the assessment just has to go well...

Just a quick note. Even though I have been prioritized on the list for a right heart catheter (RHC) as urgent the list is long. They carry out two cardiac catheters/angiograms a week and there's 8 people ahead of me on the urgent list. I've been given an admission to date to the transplant unit for August 2nd and will be there for 4-5 days depending on how things go. The RHC will be carried out on August 5th and I've been receiving appointment letters for that week from other departments for other investigations. In true 'Rachael's Luck' style I received an appointment for oral clinic for July 30th... Yeah that would be the week before I'm admitted, god damn.

Today out of anxiety more than anything I called one of the co-ordinators. I didn't want them to think I was pestering, my appointment was only on Monday but the amount of concern my consultant showed at the appointment has been playing on my mind. Well not really the concern but the sense of urgency and the 'oh dear although this is what I've been fighting for it is really happening now and I don't know what to do about it' feeling. Just a feeling that I want to help myself as much as I possibly can and if that means being a pest then so be it. The co-ordinator was extremely understanding and put my mind at rest. They had received a fax from my consultant and he has ordered an urgent cardiac catheter, he mentioned this would probably be needed. Now we wait for Cardiology to free up a slot when they do I go onto the transplant unit and complete the rest of the tests. As the cardiac catheter has been marked as urgent and the request has already been sent it shouldn't be more than a week. Things are moving forward, that, to me, is rather novel and something I'm not that used to.

Anyway although the co-ordinator had received the fax by the sounds of it, it was a brief one because she didn't know anything about me and began asking questions, most of which I struggled to answer. This led me to think back to some of the turning points I remember about my lung disease. Some of the main things that stick in your mind as sort of flash backs. Really not major events in reflection but just situations that never leave you.

She asked what my diagnosis was and I said I didn't have one, I didn't compare what I had to another more common lung disease which I and people around me have fallen into the habbit of doing. But then I thought how must that have sounded? I'm 21 in end stage respiratory failure due to some random lung disease I developed age 14 that I've had tests for for the last 6yrs and some of the top respiratory teams in the UK cannot even fully explain the disease or what it is, why I developed it and why its suddenly picked up pace and become hell bent at taking my life. For the first time in along time I allowed myself to relate to what my doctors or the doctors facing me must feel, Frustrated. Frustration has never really come to the front of my mind like it did today. Its always been there but never really a major feeling most of the time I've had to much other stuff going on to actually take some time out and try and assess what I'm actually feeling or even care about what they, my doctors might actually be feeling. I've always thought when it comes to my doctors 'how dare they feel frustrated when from what I'm feeling frustration would be an improvement'. And for some reason that just hit me today, that what I've learned might not actually be right, that contrary to what I might actually believe my medical teams aren't the enemy...

Confused? Me too...

So you'll probably recognise the new url and the altogether new blog!
Since me and Jamie split and as my situation with transplant becomes more tricky (and the blog was becoming about just me) I thought about starting a new blog. I have so many followers on facebook (34 comments on a status last night!) and so many on here that its just not going to be possible to keep everyone in the loop with goings on so rather than starting a new one I thought I'd use this one.

So those of you that are managing to keep up, here goes.

Yesterday I had an appointment to see the transplant consultant. Last time I saw him I didn't fit criteria and he refused to put me forward for an assessment. This time round problems had been ironed out on both sides, there was no reason why I shouldn't be put forward for an assessment, we all agreed on this, the consultant was pleased I was a good candidate and has passed my case to the transplant co-ordinators and surgeons. I will need to be admitted to the transplant unit for a few days whilst the team check my other organs, mainly liver and kidneys because the post transplant drugs are most harmful to those organs. And run a few other tests to bring things upto date then they will all sit down at a meeting and decide whether or not to offer me a place on their list. I'm not at this point on an active transplant list. The consultant has given the go ahead for the assessment to take place, it looks extremely positive but nothing is certain yet. My london team have been in contact and are 'delighted' with the fact the consultant was happy to put me forward for the assessment. I am now just waiting for the co-ordinators to call me onto the transplant unit to complete the final tests as I said that will be within the next 2 weeks.

On the subject of my London team, a recent summary has revealed they understand what is happening with my disease, what is going on but not really why and I don't have an official diagnosis. I have a lung disease so rare it doesn't even have a name. It is similar to several conditions but doesn't fit into one label. As the blog title says, anything but ordinary!

The assessment will hopefully take place soon, I will be taking a laptop into hospital with me so I'll keep this blog upto date daily.

Rachy

Update from Rachy's mum

Hello, don't worry Rachael is ok. She told me to come on here and update, she hasn't abandoned her blog completely but is taking a break at the moment. Her health is continuing on a slow decline which unfortunately we can't do anything about. Not much has happened recently with transplant, we still don't know which way things are going to go. We're still waiting to get the ok to get her listed be it in the UK or America. This waiting has affected all of us a great deal. If we had a definite answer we could deal with it either way but the not knowing is the worst thing. Rachael hasn't really been herself recently and I can see this has got to her alot. She's taking time out at the moment to find some head space, enjoy what she has and make the most of the things she can still do. Rachael hasn't had much support recently either, unfortunately you can only spend a limited amount of time at day hospice and her time there was up some time ago. Her local doctor sees her on an as needed basis, as her decline is a steady one there isn't really a point in seeing her unless the situation becomes acute i.e with infection and other wise her other support systems seem to have disappeared which hasn't helped.
We want to thank everyone that has donated and the people that continue to donate to Rachael's transplant fund. I hope to be able to update you soon with some positive news.
Lynne x

long update...

I'm sorry for the lack of bloggage and lack of contact lately, I've had emails, facebook messages, texts and I just haven't replied to any of you, I am so sorry! Things have been a bit rocky. Due to a poor appetite I've not had much energy to do anything. When that happens or I'm feeling worse than usual it affects everything including my mood. I've not had motivation to do anything and have spent alot of time lounging in pyjamas. My lungs have been, I think the best word to describe them is 'ratty'. They just feel angry. I've seen the doctors at the hospice and my nurse practitioner has been out to confirm it isn't infection. My nurse practitioner advised I increased my morphine and diazepam. It is the only thing that can be changed when things aren't going well, it is the only 'fix'. That's resulted in more dovet days than usual in the past week or so. On a transplant front, I know alot of you have been asking about this so here's your up date. In my last blog about it I made it sound very simple, for those of you that know me you'll know I'm not that simple! It's been a case of one step forward and one backwards. The reason or the main reason anyway that I couldn't get onto the UK transplant list was because I was told there was problems with my respiratory muscles, they didn't move in the right direction and so that would make recovery from a transplant too dangerous. Plus I was told that the problems with my respiratory muscles were the biggest issue anyway and new lungs wouldn't fix that. Tests in London, invasive, more accurate tests on my respiratory muscles have come back 100% clear... There is nothing wrong with them. That's the step forward...
The step backwards. London have reason to believe the secondary condition I've got (the condition I've got as a result of having diseased lungs for so long) has got worse. I've got secondary pulmonary hypertension (SPH), a condition that affects the heart and blood vessels and the London team believe the blood vessels around my heart and lungs have become narrower and are going into spontaneous spasm. When this happens my oxygen levels can drop by upto 50% as it literally cuts off the oxygen supply to my body. This is what they believe is happening. There is only two ways to diagnose this, one being a lung biopsy which I am not fit enough for the other being a right heart catheter which doesn't come without its risks. At the moment I am waiting to hear if this can go ahead in the intensive care unit. If it can't be done or if when given the risks I decide against it (and that has crossed my mind because I know what the risks are) I don't know where that is going to leave me. They have said if the SPH is stable I will be able to go onto the transplant list for a single or double lung transplant and I could just be listed for whatever comes available first. If it turns out to be progression in SPH there are treatment options but they don't hold much hope for them at this stage and it will eventually mean I will need a heart and double lung transplant. So that is where things stand at the moment...
As some of you may know I've just become the Good Will Ambassador for the charity Breathe On UK. I will hopefully be attending my first conference in April to launch a parent support pack at Portucullis House, Westminster, London. I have to say I'm rather excited!

A small clue (click here!) as to what I have been upto recently... More to come later!

update

A long over due update from London. I have had to give myself time to process this info so I'm sorry for the lack of blogging.
My week turned into a bit longer as is usual here, if it weren't my 21st birthday this weekend I would be staying a lot longer I can tell you. Having said that they haven't done any tests that are out of the ordinary. I had a lung CT, which was pretty unchanged, lung function, which I think was around 12%, my lung function is quite up and down between 10 and 30% so that is not an abnormal figure for me. I was just having a bad day. Unfortunately the lung function techs still panic on my 'bad days'. I did well on the capillary gas on air, they take off your oxygen for as long as you can stand it and take blood from your ear to check your oxygen levels. It took a full 2 minutes for my oxygen levels to fall to 81% and the tech took the gas at that point. It came back as oxygen 79% and carbon dioxide a little on the high side but again not higher than what is within normal for me. And I've been on over night ventilation since age 17. Bloods have been ok as far as I've heard. On Tuesday went to the muscle labs. This was always going to be the biggest hurdle for me. Muscle tests are brutal, how they can put patients through them and even call them 'tests' is beyond me. They insert two NG type tubes into your nose, one sits above your diaphragm the other below, they have balloon sensors which are inflated when they're in place and they feed data back to a high tech computer. The tubes are thick and wiry unlike normal NG tubes. I always have difficulty passing them even with anaesthetic and jelly. They tried and tried until my sinuses bleed, I was in tears, my nose was pouring with blood, jelly and god knows what else. It hurt anyway, alot, but they did eventually manage to get them down. I sat in the lab with these tubes down my nose tape all over my face, really pissed off, I felt like someone had punched me in the nose, more than once. And one of the techs passed me a newspaper... I'm not going to write on here exactly what I wanted to do with it... but it wasn't read it anyway. I held a tissue under my nose catching the blood whilst they got their much needed data. But then to every one's surprise the results were 100% normal... None of us had planned on them coming back anywhere near normal but they conclude that I have NO DIAPHRAGM problems or 'funny breathing patterns' of any kind. The reason or one of the main reasons I was turned down for transplant in the UK was because of the diaphragm issues. They said they would make recovery post transplant almost impossible and a transplant wouldn't be the answer because I'd still be out of breath anyway. The diaphragm disorder no longer exists... so I will leave you to ponder that one as I don't want to say too much at the moment but I don't think I need to spell out what that probably means...
*grins* :-)

transplant update...

Well I poured my heart out in an email to my professor last night in London. I am not really someone who does that often, especially to professionals incase they think I'm like emtionally unstable or something but I really made sure my email painted a good picture of how my life is now and how much my lungs are affecting me physically. I explained how my consultant had said in his opinion this is an 'urgent situation' now.
I went to see a friend today I met at the hospice. She hasn't been for a couple of weeks as she's just had an operation and is recovering at home. We sat and had a catch up, which was long over due! And we just spent the afternoon nattering. Then my phone rang 'unknown' I went "oh god better answer this". It was the interstitial lung disease nurse from the Brompton she said "hi Rachael do you know your coming in on Monday?" I just went quiet "THIS Monday?"
"Yeah we've arranged transport and bed manager will be in touch, ok? see you Monday!"
Just like that... No explanation of what they will actually be doing, how long it's going to take or even why all of a sudden they have just decided to admit me. I don't know where the bed is coming from because I can usually wait anything from 3-6wks for a bed for tests. I expected a reply to my email explaining all when I got home... Nothing. I phoned my consultant he said "ah jolly good, this will complete your transplant assessment, call me as soon as you get home".
Now to clear things up a little... Why after all this time has it taken them so long when transplant was first mentioned over 3yrs ago to me? Well 3yrs ago I had the assessment and they found I had a problem with my diaphragm, they said it would make transplant dangerous and as long as my lung disease remained stable the risk really wasn't worth it. My lung disease did stay fairly stable apart from the odd 'blip' until late August last year. It was at that point I was referred to the hospice and my lung disease really started to pick up pace and progress. I started a heavy Morphine and Diazepam regime in September when I was admitted to the hospice, at that point I didn't actually now if I'd leave there alive but 5wks later I did. In late November I caught severe Pneumonia and I will admit I wrote letters incase it was the last time I was to catch pneumonia. Since then I have been living on a knife edge with my lungs, I have been seeing my main consultant weekly and the physical side affects of everything my body is struggling to cope with has become very obvious. Everything from darker eyes and thin hair to weightloss and fatigue. We're hoping now (me and my consultant) that although we know there is a risk the diaphragm problem could still be there that they now feel that I will benefit more from transplant than I stand to lose. Until today I had not even thought ahead to what 'life on the list' could be like, I was focusing more on actually getting on a list, that has been my main focus. And although I still don't want to think that far ahead incase it doesn't happen it actually crossed my mind today that I could soon be joining the other 7,910 people on the UK transplant list...

I have stayed away from my blog recently. Mainly due to how difficult things have been health wise. I have not wanted to blog about my bad days but I guess thats what its for... The last week or so has been tough. The fatigue and tiredness has been the worst I've ever known it. I have been sleeping so much I have sort of not had chance to sit down to proper meals. This is obviously not a good cycle to get into because I don't really need to lose anymore weight at the moment. The weightloss has led to muscle wastage in my legs which is making that bit or walking I could do even more difficult than it already is. I saw my consultant today he is really trying to push things through with the assessment, he said the physical deterioration recently is concerning. To hear him say that makes me worry even more! He is someone who when they're a bit worried they will kept it hidden and have you believe everything is going well to hear him say "3mths have gone by and we have got nowhere, we can't afford to sit back and watch another 3mths go by..." Is not exactly what I wanted to hear but then again I would rather I was told the truth.