Friday, 23 March 2012

A Quiet Lull, A Chance to Reflect Spring 2012

Well that brings my story up to date now, so now I can blog freely about all the things that happen to be in my next leg of this journey.

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No hospitals for a little while. Help is never far away and I have just  been telephoned by my specialist nurse to see how I am. If I leave them alone, they don't leave me long or forget about me. I have a few queries about the medications I have been given by the dermatologist for my allergy, which she will check out for me. She also thinks that my next visit which is planned on the day ward in July is too far away and will sort out an appointment for earlier, perhaps admitting me to Duchess Ward following my next planned visit to the Lung Transplant Team in April. That afternoon I have a CT scan booked as well and they might try and squeeze in the PH tests they need to do the following morning. I'm not sure what the scan is for: routine monitoring or to do with the domino transplant that has been discussed. I could phone them and ask, but it is nice to be hospital free. I need to phone in each month to update the Transplant Team on my current health so they have no surprises to contend with should I receive 'the call'. I have three appointments for the patch testing at Addenbrookes Hospital around this time too.

I have been feeling quite well and wonder if I still really need the anti - sickness tablets I am on. I worry that if my drugs are ever increased, they may not be that effective. I decide to just try and do without the one I take in the morning. No such luck that I can do without it, I'm back to where I was twelve months ago and hastily take it again by late morning. Worth a try. I think my medication regime is as good and balanced as it can be now.

Easter flower
In the meantime we have Easter and my fifty first birthday looming. My dad is very sick now and will not recover, it looks like just a matter of time and at Easter sometime we plan another trip to the Lake District and Lancashire so we can visit, all being well. We have the builders back in, this time to spruce up the bathrooms, which are getting very tired. Again, we would like this done before my transplant operation because of the infection risk the dust and dirt may pose. It puts a little strain on me, but there is plenty of room downstairs out of their way to rest, so it is not half as bad as last time.

I feel like I am nesting like I did when I was pregnant with Sarah, keeping busy, getting things ready pending the new arrival, although it will be a new heart and lungs this time and not a baby. It feels strangely similar to a first pregnancy,even including the nausea, which I have worse in the mornings. There is that waiting, itchy feet feeling, that feeling of anticipation of what is to come. You know you can read all you can about it, but it will never really prepare you enough when it does happen. When it does happen you know you will have to fumble through it and it might be one step forward and ten steps back. You don't know what the pain may be like and if you will cope with it, you will have to learn a new regime all over again, this time with drugs, not a baby. You will have a new and very different future ahead of you. It will be a new era for our family.
It is only really now and by pouring my story out in my blog, that I have had time to stop and reflect about all that has happened. It has felt like a roller coaster and now it has just stopped. But it has only just stopped for a little while and I'm not allowed to get off it yet as it is all going to start again, I just don't know when.

Perhaps this is the time I've got to take a breath and be ready for phase two, as I believe phase one has at least gone quiet for now. Somehow I know that phase two is going to be the hardest and I haven't really gone through anything yet, it has only just begun and the roller coaster is going to do a few more rounds yet before I am allowed to take that first step off it.

I do not think I will ever get over the shock of being diagnosed with this illness and then being told I need a heart and lung transplant, and I don't think Rob will either. He sees my illness as his too and is with me every step of the way, he calls it our illness and always has and always insists and is adamant that we will go through whatever is to be gone through together.

Pansies in the garden
Although our match has always been perfect from the very beginning and has continued so throughout our marriage, it is only now that I have really had the chance to appreciate how deep love can be. It is when someone remains devoted to you whatever happens; when someone is happy to wash, bathe and dress you when you are too unwell to do it yourself; when someone will get up at the crack of dawn to make up your medication, day in and day out; when someone shares all your illness every step of the way and comforts you when you are at your worst and in the depths of sickness and despair; when someone is tuned into your movements while they sleep so they can still help you even while they sleep; when someone still sees you as their equal partner and finds you attractive despite everything; when you are able to ask someone to do these things for you and not feel a burden. People say love is tested in these situations, well not ours, for me Rob's love has been the strong bedrock beneath me, never budging whatever the rollercoaster does. Of course the medication has played its major part in my recovery, but my well being and good mental outlook  is down to him and of course my girls. Added to this strong support is that of  the Papworth teams and the several support groups we have joined, and of course those friends, who show compassion and understanding and help me get on with some normality in all of this.  

I feel I am now in 'no man's land', I have overcome 'the enemy' so far, but I'm still aware 'it' is there, waiting, lurking in the depths of me, but I am waiting and ready, head and face on ready for the next attack, when and if  'it' tries to claim me back. Help is on the horizon, will it get to me in time and make me safe once more, free of these burdens? Will the troops come?

2012, A New Year, New Hope and a Bundle of Hospital Appointments

January snow in the garden

January was dotted with hospital appointments for this, that and another. A monitoring appointment and tests with the PH team; a monitoring appointment and tests with the Lung Transplant team; an appointment for the results for my Holter Monitor test at the local hospital; an appointment with a genetic consultant at Addenbrookes, as I had asked about risks for my children given that I had Idiopathic Pulmonary Arterial Hypertension and this can be caused by a muted gene; I also had an appointment for early February with the dermatologist at Addenbrookes for my problems that I was having with my dressings. Also I still had my usual two weekly warfarin clinic visits to check my INR range.

This barrage of doctor and hospital visits is probably quite typical for somebody with ongoing medical needs and probably it will be worse for some.

Snowdrop week at Bennington Lordship
The appointment was cancelled at my local hospital and rearranged for the end of February, that would now be nearly four months after having it fitted and nearly five since the main incident that caused the need for the test. Never mind, I had been back to Papworth Hospital twice and been reassured in the meantime, so for me, not a major worry. I wonder about other patients who do not have my luxury of a specialist centre or someone who is needing a specific diagnosis in order to get treatment. They would probably be dead by now if they have something serious and are not being treating properly until they get these results. It seems ironic that I have two teams at Papworth bending over backwards to make sure I stay well and enabling me to stay on the transplant list, yet another part of the NHS cannot even come up with some straightforward test results for at least four months now. Never mind, I shall just be ringing my specialist centre should I have problems and will avoid this hospital altogether unless it is going to cost me my life. Even then I wouldn't feel so confident I would get the right help, it would depend if its Saturday night or a week day and whether it was a consultant happy to refer for help or whether it would be one who thinks he is an expert on everything and refuses to ring my specialist centre because he knows best, as happened to my on my first visit to A&E at this hospital.

Sherrardspark Woods Welwyn
Off we went to Addenbrookes for our meeting with  the genetics consultant. This was a very interesting meeting. We found out that this consultant had been brought in to the genetics team here to try and establish a united programme for genetics testing for families with Idiopathic PAH and other rare diseases. At the moment there is no united front on this testing and no specific funding. He explained that my PH could be caused by a mutated gene and there are several he could investigate first, if these were not found then there were more he could still look at. If nothing was found, it would not rule out that my PH might be caused by a genetic disorder, it may just be one that is unique to me that they may never find.  He was working closely with my team at Papworth and working closely with a leading genetics researcher, who was researching genetic cures for PH. The whole thing appeared very cutting edge and we were at the very start of it, we were the second PH family he had seen so far. They took blood tests for samples of my DNA so they could get me tested and store it in case of future breakthroughs and for their research.

 Duxford after a visit to Addenbrookes
He explained it may take several months to get the results, as there was no funding available for these purposes at the moment, although they hoped to acquire some in the near future. Whatever, he would try his best to get the DNA test done and contact us in a few months. This is important to us as if I do carry the commonly mutated genes for PH, then my daughters will have a fifty per cent chance of having inherited the gene; however, this does not automatically mean they will get PH if they have got it, something needs to trigger the gene first and the triggers are unknown, but childbirth is considered a possible trigger. If I don't have the common mutations for PH, then they may have less chance of inheriting PH.

It would be helpful just for me mentally to identify where this awful disease came from, useful to find out for my girls sake, I was also happy for my blood to be used for their future research, as helping anyone in the fight against this disease will be a bonus.    

Rob performs in King lear at The Barn Theatre 
Next stop was the PH team. By this time, the allergy to my dressing was in full force and the rash had spread  all around it, as well as underneath it. This was the first priority when I went back to clinic and I was prescribed another dressing to try. Apart from this ongoing problem, after walk tests, lung function tests and blood tests, I was confirmed as being stable, which was great news and I do not have to go back for at least three months. I hoped that I would be able to manage this this time and was really pleased.

The following week, I had my visit to the Lung Transplant team. The usual routines followed: height and weight and blood pressure measuring, meeting with a consultant and then blood tests to finish. Yet again the Transplant Team had a little surprise hidden for me when it came to discussions. I saw a consultant from the surgical team, who I hadn't met before and we went through all the usual updating.

The consultant then paused and asked me if I had considered donating my heart to someone else once I got my new heart and lungs. I was a little taken aback, because I had thought that my heart was probably too damaged to be of use to anyone. He explained how they had a lot of data on my heart that they do not usually hold for a donor and that my heart may be relieved when it did not have the high pressures from my lungs to contend with and could possibly go back to a normal state. My heart could be a good donor heart, with its potential to beat normally again, the vast information they hold about it and the carefully controlled position they would be in if it was suitable to use.
Rose celebrates being 18

Obviously the heart would be of no use to me once I get a new one, so I agreed this would be a good idea. Well a wonderful idea really, the thought that I could save a life as well as having improved health myself - well it just doesn't get any better than that if all the outcomes are successful. My donor too, although they will never know, would be able to save an extra life as well as mine and all the others. They promised to look into the idea further. This would be called a 'Domino Transplant' and is only performed rarely in the UK now, only a few per year, if that.

Next stop was back to Addenbrookes again, this time to see the dermatologist. In this waiting room they have a big screen, which tells you whether the consultants and doctors are running to time. My appointment was at nine thirty, so a difficult and early start for me to get myself to Cambridge this early. I reasoned we shouldn't have to wait too long seeing that it was quite early and they wouldn't have had a lot of time to get too behind. The big screen continually flashed up everyone was on time, it was about twenty past ten when I got to see someone. Why can't they just tell the truth? They are very clearly running over three quarters of an hour late. What is the point of winding up people even more while they sit there waiting? They are already wound up because they have been sat there for nearly an hour and they are stressed already because they are there because they have a problem they are stressed and worried about. What a waste of NHS cash on a machine just designed to wind people up!

It got even better, I am seen by a medical student to start with whose first question is, 'so when is your heart and lung transplant booked for?' After we go through my history, she omits to ask me one of the most important questions of her session and has to come rushing back in to do so, 'what is actually wrong with you?' Rob and I cannot look at each other as we will start laughing. Anyway I am not wanting to criticise this hospital, I don't  mind having to wait for appointments at all, I am used to it and I know that some patients take longer than others, I just bring my kindle along and read and wait and sometimes enjoy the entertainment of the waiting room. The 'gem' questions by the red faced student doctor have given some good entertainment value for my friends too and she probably learned a lot that day!

Wrest Park in January
Unfortunately I will not have the patch testing done that day, that will have to take three more visits and both the consultant and doctor that I went on to see, tried to give me some medication which may help the rash in the meantime. Unfortunately it is not aseptic medication and I'm pretty certain I will be unable to use it and know I will be wise to check with my specialist centre first. I don't want to give myself an infection. They had at least tried their best to help me and fortunately the new dressing was helping the situation by now.

Although the doctor had marked my case urgent, I would have to wait until the end of April for the patch testing. I just hoped I could hang on long enough for this with the new dressing I had been given.

Next came the appointment at my local hospital for the Holter Monitor results - what a surprise - cancelled again. This time I lost my patience for the first time ever since first feeling ill. I told them I would be dead by now if I had to rely on them to help me. After my rant, I apologised to the girl as I know she was only the messenger. Surprise, surprise, I got an appointment for the next day.

Here I was told I shouldn't have had to come to outpatients for the results and they would send them on to Papworth, but it was their policy to see all patients. That is fine, but why did it take all this? Surely somebody, somewhere must have looked at my notes? Three hospital visits, three cancellation letters and two phone calls later I found out that the ECG had recorded a 'tachycardia' in my heart rhythm. Nothing of concern to them, but for my specialist hospital perhaps a piece of important information in the jigsaw of all the comings and goings of my condition. Anyway at least the incident back in October has been laid to rest.

March is hospital free and I only have two warfarin clinic appointments during this month. I feel free and steady and stable at last, the dressing allergy has also eased up with the new dressing.

The Snowdrop Walk at Benington Lordship


Thursday, 22 March 2012

Yet another December visit to Hospital

Milnthorpe Parish Church
Once we had got ourselves back to the Lake District safely, the weather and traffic was horrendous, we began enjoying our break. The weather remained wet and it did not seem to come proper daylight now we were in winter time, but we enjoyed just relaxing in our home, taking it easy and going out for lunches to some of our favourite pubs and some new ones. We have been going to the Lake District all our lives, but there are always new places to be discovered. All the pubs had their roaring log fires blazing and Christmas trees up and everywhere looked very festive. The rain and the dismal overhanging clouds seemed to emphasise the twinkly lights and made everywhere look even more sparkly,warm and welcoming.

View from Bowness

After a day or two I noticed my skin under my dressing was looking red and inflamed again and I had developed two big rashes on each side of my neck, which were itchy and irritating. We were not too concerned, but as usual we kept a very close eye on things. A day or two later, my skin under my dressing was getter redder and the wound had started to weep. This was not looking good and we were beginning to be anxious, so we immediately made a telephone call to my specialist centre. The specialist nurse advised me to go to A&E or at least see a doctor. It would need to be swabbed and I needed blood cultures taking. I knew it may be a bit of a risk, but we decided to just pack our bags and abandon our holiday and get ourselves back home.

We both could not face yet another A &  E department and decided it would be better to at least get home and then review things again. If things had worsened by then, we would go to A&E near home, if not I would get an emergency appointment with my GP the following morning.

I will hasten to add that the redness was not at the entry site and that this, from my previous experience, did look like another allergic reaction, otherwise we would have taken the precaution of going straight to A&E. An infection in the line is always a danger when having intravenous drugs, blood poisoning is always a threat. I was more concerned about being stuck up in the Lake District for longer should I be hospitalised and things deteriorating and me not being near enough to my experts at Papworth should things get worse. Although I felt ninety nine per cent that it wasn't an infection, Christmas was only a few days off and I didn't want this allergy travelling to the wound entry site and then getting infected over Christmas, this was my main concern.

I had had allergy tests taken at Addenbrookes Hospital in Cambridge during autumn, following my bad reaction to the site dressing in summer. They had found I had quite a lot of allergies, latex had been a worry, but it was found I only had a mild allergy and decided that I should really have patch testing for contact allergies next to see if they could find what it is that makes me react to the dressing. I had been referred and had received an appointment for February, so was having to wait on this one in the hope that things didn't really get any worse in the meantime.

We packed in a whirl and made the long journey home. Nothing looked any different when we got home, so I decided I would be better getting a good nights sleep ready to brace myself for a long haul at A&E and the doctors in the morning.

My first port of call was going to be the doctors and I did expect from here that I would be referred to A&E, I have found now from experience, its better to be referred to A&E by a doctor rather than just turning up unannounced. I went down to the doctors for eight thirty in the morning and managed to get an emergency appointment for later that morning. By the time I had arrived home, Papworth were on the telephone, wanting to admit me to the ward so that the PH consultant could check me. Relief. I would be back in the hands of the team who know me, my condition and its problems.

Off we drove, hospital bags in tow, I was hugely relieved not to have to go through the rigmarole of A&E and I would be quite happy to stay in hospital if that was what was needed. Thank goodness we had got ourselves home, but yet again, I felt some disappointment that my body had let me down.

I was given a single room on Baron ward, I'm going to have been on most beds in these wards one day! I was admitted and thoroughly examined, blood cultures and swabs taken as a matter of routine. Everybody agreed it did not look as it should be, but also it did not appear to be infected, although the tests would tell. Some staff wanted to just come and observe what had been happening, just because there are not many of us with this type of intravenous drug and it useful to see what the problems patients endure actually look like when they happen. While all this was being done, it felt a bit like we had come back home again, my door was open to the corridor and lots of staff spotted us as they walked past and popped their heads in to say hello.

As it was Christmas time, it was decided to let me go home and give me a supply of antibiotics, only to be used on further instruction from the ward doctor, should the rash get any worse. We had chance to thank the staff for all they had done for us this year and then off we went home, pleased and relieved I had been checked, relieved we had a back up plan for over Christmas and the consultant working over Christmas was now aware of my condition and had seen it first hand. We would obviously receive a phone call should tests reveal  any further problems.

A day later and Papworth Hospital was headlining the news. It was on every TV channel. Now my second home is in my lounge again, goodness me! Prince Phillip has been admitted straight from Sandringham, where he has recently travelled to be ready for the Royal Family's traditional Christmas celebrations. He was having a stent procedure following chest pains. I knew he would be in the right hands. The press and TV coverage is full of praise for Papworth and quite rightly so I want to tell them. I feel sorry he has had to miss his Christmas and be in hospital, but know if he is anything like me he will be a good judge of when it is better to be safe than sorry. He will know he is in the safest place albeit he will be itching to get back to his usual routines. I'm a tiny bit jealous I am not there when the royal princes visit him on Christmas day and Rob is not on the TV  parking his car in the car park when he comes to visit me, the TV presenters are stood in front of the car park we use! Some people are moaning he is getting five star treatment, but I know better, we all do, whoever we are, when you go there!

We didn't get any phone calls and the rash did not worsen over the Christmas period, neither did it disappear, so we were still monitoring it carefully. We were all ready for Christmas now and had a wonderful worry free Christmas day, except for all the usual drug regime and my usual problems including chest pain. Rose even managed to make a phone call from Uganda to wish us Merry Christmas and Sarah and Oli came and had an evening buffet with us and stayed over for Boxing Day.

I was delighted I was here to enjoy another wonderful Christmas with my family. I had always planned to be, but you never know what is round the corner with this terrible condition. During the Christmas period we also had lots of friends round and again it was lovely to catch up and enjoy the more normal things in life. I can manage eating and drinking (well soft drinking) just as easily as anyone else!

Wednesday, 21 March 2012

Autumn and a Wonderful December and Christmas 2011

On my discharge from hospital, my GP was asked to arrange for me to have a Holter Monitor via the local hospital. This is just a device that monitors your heart rhythms and throws up any irregularities and can be useful to pick up any problems that might occur while you are going about your normal activities. I didn't really hold out much hope that anything sinister would be revealed on this, I had had countless of these in the past year or so and twelve years ago had even had a device implanted near my heart to try and get a reading of what was occurring. I had this device in my chest for two years. Nothing substantial was ever found, despite the growing disease in my lungs and the dilation of the right side of my heart.

This was duly arranged and six weeks later I had the monitor attached. Unusually they took an ECG recording first as a base line and just as I laid down, my heart began to race quickly. I had been sleeping more or less sat upright and propped up by pillows since the dizziness began and I don't think my body liked having to lie flat. Well at least something might be recorded for the first time ever!

Nothing else happened while I had the monitor on and it was duly returned, more car parking, car parking fees and a traipse through a maize of long hospital corridors- well for Rob at least, it wasn't necessary for me to go with him this time, so I didn't.  I was sent an appointment for the follow up for early December. All this was taking a long time in my ideal world and the event had passed and gone really, but it probably was reasonably quick by NHS standards and in the real world of hospitals.

Xmas shopping in Stamford
Autumn came and went and I spent the next three months or so sleeping sitting up, propped by pillows, I was still getting dizziness and was having some incidents with palpitations occurring when I needed to get up in the night. They felt relatively mild and I didn't relish the thought of being whisked back in hospital only for nothing to be found, so I was frightened when they happened, but they always passed after a moment, so I decided to just monitor the situation. At my next outpatients appointment, I mentioned this was happening and it was explained that when I have been asleep and laid down for a few hours, it is a big strain on my heart when I then try to get up and it will make it beat harder as though I have been exercising. I felt reassured to a point, but these loud throbbing heart beats and my heart racing began to remind me of when I used to pass out.

At times I felt extremely weak when getting out of bed and moving, making me feel unsteady on my legs they felt so weak, and then the heavy beating would start up again, making me scared it was going to end in a faint, which would mean my heart wasn't coping again. I worried in the back of my mind that the PH is beginning to deteriorate. So during Autumn and over Christmas I went to bed every night, having to sleep almost sitting up and being frightened of the night, because all these symptoms seemed to be at their worst then. It was a good feeling when I woke to daylight in a morning and I had got through what seemed yet another long dark night. At night time, Rob is always sensitive to my movements. He wakes without fail if I am awake and he is always there to help me. It reminds me of the days when the children were little and we were always tuned in to hear them if they roused at night. Rob offers his never ending support to me twenty four hours a day. It is a great relief to me, I don't think I would even sleep at night if he wasn't with me every step of the way.

There is always a sense that I have made it through yet another twenty four hours as well, when I wake up in the morning, having had no phone call from the Transplant Team. We are trying hard to act normal and do normal things and make the most of life, planning outings to look forward to and planning for Christmas, but never far away is the thought of the actual transplant, what it will entail, what the outcome might be. What we will have to go through is never far away, and could be as near as a few hours away or as long as two years or so away or might ever even get to happen.

Uncertainty is our future at the moment. Uncertain when the transplant may happen - will it be sooner or later? Uncertain if it may happen at all - can I live long enough if it takes a long time? Uncertain if my PH will stay controlled - can I remain stable and maintain this same quality of life? Uncertain how the transplant may affect me - will I be a lucky one, who manages and copes with things with minimal problems or will I struggle and end up with a worse quality of life than before? Uncertain where all this will end - will I have the worst case scenario and not survive or even make it to transplant? All of this and then there is going to be the burden of knowing that someone else has lost their life to help me, some other poor family will have to suffer grief and loss, while my family may have huge relief and a new lease of life. How will I handle this?

The only thing I can do to cope with all of this is just take one day a step at a time and get through it and make the most of it. At the moment I do not have a clue what will happen in the future, so I can only control what is immediately happening, so that is the best thing to focus on. I find it better not to dwell on the transplant, but at the same time remind myself of it and what is going to happen so that I will not go into complete shock when the time arrives, if it does. I have a transplant folder, which describes the procedures and drugs and possible outcomes, from time to time we just browse through this to refresh ourselves, all of us, so we are prepared when it happens. It is inevitable that waking up feeling unwell every single day, day in day out, dealing with the drug regime and all that brings, together with the uncertainties ahead, brings moment when you feel sad, depressed and down.

For me, I feel its important to have a little cry here and there and have a little wallow now and again as it is important that these feelings are released. But it is equally important to me that I don't waste this borrowed time I am on and it is best spent making most of what I have and enjoying what I have got, so most of the time I am still able to pick myself up and get back on with things. I know that I manage this with the close support of Rob and my girls, who are full of understanding and love. Some of my friends are never far away either and come to see me or meet up regularly with me. With all this support, it is difficult to stay down for too long.

Winter in Knebworth 
It concerns me I still have a lot to go through yet and a lot more illness and hospital ahead of me, so I do not discount having some professional help at sometime to help me through. The palliative care nurse at Papworth always sees me on my visits there to offer help and support and I know she will be there if I need her. Just knowing that there is this support is available helps me too. I also try and focus on all the positives having the transplant may bring and day dream about swimming and travelling again. When people ask me what I will do when I've had my transplant, the reality is that all I really wish for is wake up in the morning and feel well, all I want is to be able to fulfil a day as a fit person, that I will tire from being busy not sick, the rest of the dreams will follow and become a reality if this ever happens.

During this autumn period, the PHA Conference is being held. This is both a fun and sociable event as well as being informative about PH and the developments being made to treat it. Anyone whose life has been affected by PH is welcome to attend and Rob and I have booked to go. Funnily enough it is being held in the Cotswolds at Heythrop Park, which used to be our staff training centre when we both worked for NatWest. We are curious to go for this reason as well, we both had fond memories and it is a stunning building in beautiful grounds. It will be interesting to see what it is like now. We are also looking forward to meeting people again we have met through Papworth PH Matters Support Group and friends I have made on PHA Forum.

Unfortunately, the conference is only two weeks after my wedding escapades in Lancashire and I have not long been discharged from Papworth and still feel dizzy and unwell, I decide I would rather not be away from home again or have to make the journey there. I'm still not fully recovered from it and mentally it is going to take a lot of strength to be away from home again. I do not relish the though of visiting yet another A & E so soon. It is disappointing, but as we said to the conference organisers when we rang up to cancel, it is part and parcel of the job of being ill, sometimes you just have to be disappointed and then think of new plans you can make.  

Following the conference, we had a sad day for our PH community, the inspirational young man, who had supplied me and many of us with our specially designed bags, which conceal our pumps and catheters, died at the age of thirty. He had been diagnosed with PH at aged nineteen and had survived eleven years with his Hickman Line and pump. He had been assessed for lung transplant some time ago and at that time he was too well, which was probably good news for him then, but he deteriorated and was sent for assessment again. Unfortunately this time he was too poorly to be listed. His story really brings home to me how important and short the window is between being 'too well' or 'too sick' for transplant and timing is key.

Whenever I question that perhaps I'm doing all right as I am for now and should I really be risking putting myself through transplant, I remind myself of him and know I am in a lucky position to have been given this opportunity. I know that had he been given it, he would have grasped it to the full and so would my other two friends who had lost their lives and battle with PH. I have met five other people on the Epopostrenol  medication so far, making six of us. To date, three people have died and to my knowledge the other three of us are alive. This awful fact seems to resonate with the data I have been given by the Transplant Team on survival rates: only fifty percent of patients surviving on diagnosis and treatment. I know that I have to try and stay in the correct fifty per cent, therefore transplant has to be my only option.

What have they done?
We had building work planned for November, which was a little arduous for me when I felt unwell. We had new electrics and doors fitted throughout the house, which creates lots of dust and mess in every downstairs room. Everything had to be redecorated and the whole house was completely upside down. I think it may have been easier to move house. The dust and mess being created wasn't really ideal for my lungs, but we had reasoned it would be better to get it all done now as when I have had my transplant, I have been advised to avoid building dust and mess due to the infection risks they pose. We reckoned better to attempt now or it would be years before we might be able to get things sorted. This time felt like extra pressure, pressure and stress due to the builders invading our home and added to that if I got the phone call during the beginnings of the work, I might be coming home to a lot of mess, which wouldn't be possible. There would be massive burdens on Rob and the girls if this so happened. So a self inflicted gamble that the call will not come in November.

House wreck
As we got half way through the mess of it all, I felt more relaxed that if the call came now, the work would be done before I was back home again. There would still be added pressures for the family though if it happened. Many people may think what on earth were we doing planning this when we have my illness to contend with, but I believe life has to go on as normally as possible and I was able to do lot of resting in between the chaos, which helped me cope. It was a great relief when it was finished though and well worth it with the downstairs newly decorated. We have to spend a lot of time at home because of my illness and again, I am fortunate and blessed that I have a lovely home and can enjoy my comfortable surroundings while I feel unwell.        
December arrives and it feels quite an emotional time for Rob and I, it was at the beginning of last December  that I was rushed into Papworth as an emergency and I was put on the intravenous drugs, I had been very unwell for most of that month and only discharged on Christmas Eve. This year I was counting every day I was at home in December as a blessing. The whole house was decorated for Christmas early, me having a main helping hand this time with all the preparations and we had lots of friends round to visit. This year was going to be a perfect Christmas.

Celebrating an early Xmas with Rose
The only person missing would be Rose, who was off to Uganda for a fortnight with school. This was an incredible opportunity for her, one she shouldn't miss for anything in the world, so I was perfectly happy at the prospect of a Christmas without her. When school broke up, Rose got off to and arrived safely in Uganda. We decided the time was right for me to try again and go away for a few days back to our cottage in the Lake District. It is home from home, so I knew I would be fine as long as I stayed well. My follow up appointment for the results of my Holter Monitor recording at the local hospital had been cancelled until January, so there was nothing stopping us now.

Deer on Dalham Estate
Christmas in the Lakes is always beautiful, we have gone every year at some stage over the festive period and this time we were going to stay a week, until just before Christmas Eve. Sometimes, it is snowy and icy here at Christmas and the stunning scenery can take your breath away. If it isn't, it doesn't matter as it is all roaring warm log fires and twinkly fairy lights in the pretty villages and you can relax in beautiful surroundings with some delicious food. There are lots of pretty gift shops and organic food shops to do that last minute bit of Christmas shopping.

My excitement overrides my fears and anxieties about the trip.    

View on a drive to Coniston

Tuesday, 20 March 2012

Back to Hospital Again! October 2011

I was pleased to be going to back Papworth again, this time we felt we had had a real scare and it would be a relief to get some reassurance or get to the bottom of what had happened.  I was put in a room by myself because I had recently been in another hospital.

I had used this room before, but since then much had happened. This was the room where my friend had spent her last few weeks before losing her battle with PH. It made me feel sad, I would always see this as being her room now,  my thoughts were on her and her brave family. I know I have try and put my sad thoughts of this wonderful lady out of my mind for now, so I can remain positive. Sometimes you have to think of yourself and nothing else so you can stay focussed to be able to muster up your strength to face what is going to be ahead, this can feel hard and selfish and an unnatural thing to do; but I have to stay positive and bright and clear my sad thoughts so as not to bring myself down. My friend had been brave, dignified and courageous in her last weeks and this will always be a real inspiration to me, so I inspired by her, instead of feeling sorry, I was able to put my fears to one side and put a smile back on my face and find my own courage again.

Here, it was a very different story to my visit to A &E, firstly I know everybody and everybody there has some expertise on Pulmonary Hypertension, then the atmosphere is always calm and efficient giving me a great sense of security and trust that what can possibly be done for me will be done and done at its best. Its as though worry and the weight of what has happened to me is lifted from my shoulders for a while. I am back in a five star hotel compared to having just come out of a squat.

There is some hustle and bustle while I am admitted and blood tests and chest  x-rays are done. I was in a room on my own and I cannot see what is going on outside of it. By late afternoon I have the full attention of two doctors and a consultant, who go through absolutely everything that has happened and are determined to eliminate various possibilities. In the meantime I am wired up to a heart monitor. I mention in passing the idea of an ear disorder, which had been flagged during my time at the last hospital. I'm to be kept in overnight for observation and to wait for test results to come back. I'm still having the dizziness when I move my head.

After the doctors had gone, I realise that all the patients have been discharged home on both the men's and women's wards and I am on my own. This is a highly unusual event and unheard of normally, it just so happened, tests had been completed, patients had been treated and been able to go home early, a random, one off situation. So I wasn't getting five star treatment anymore, it became seven star! I remember thinking of the hotel in Dubai, Burj AL Arab, which claims to be the world's only seven star hotel, I'm now having one to one attention from the experts and I've now been upgraded to a seven star hospital compared to where I had just been.

I know I am one of the lucky ones compared to other very sick people in having this specialist centre to support me. Sometimes it feels like I've drawn  the short straw in having a rare lung disorder that people do not generally understand, even people in the medical profession, but somehow there are enough of us PH patients and the expertise available to merit having the nine Specialist Centres that we have in the UK. And for that I am eternally grateful. Our country is world renowned for its PH care, so in this respect I have got lucky.

Many people are terminally and chronically sick with even rarer diseases where there are no specialist places to go to and they are literally passed from pillar to post without any focused support. Many have more common diseases that are so prevalent that they are dealt with as a job lot at their local hospitals, which are usually much bigger and busier with longer waiting lists and longer waiting times at clinic appointments. All these patients have much to contend with and challenges to face before they even get in front of a doctor, then the doctor knows nothing of your history and is reading the notes as they deal with you. The hospitals have bigger overwhelming buildings to find your way around and its a battle of wills to land yourself a car parking space and major achievement if you do. Car parking costs are double or more than the going rate for town centre car parks, another pleasure of being sick, that is on top of you and your partner having to abandon any hopes of working for the future through no fault of your own.

All of this can add dearly to the stress of being chronically or terminally unwell as you face hospital visit, after hospital visit, relentlessly with no respite. It can make patients want to give up in the end and make them more ill, because they have resolutely just had enough of it all. I can say this with first hand experience, some from my own visits to hospital and while this is all happening to me, my father is terminally ill with COPD, a more common form of lung disease and is now at complete breaking point and ready to give up. He just cannot face yet more of this outpatient business, getting himself to appointments on time and finding his way to yet another clinic with yet another medic who knows nothing about him. All this when he cannot even walk anymore from one room to another, not only housebound, but room bound, but he has to face the overwhelming contest of just getting himself in front of a doctor or nurse, just to be told there is nothing more that can be done for him anyway.
I count myself lucky indeed when I consider the plight of others.

Most things are eliminated such as infection and deterioration in my condition. They try and test out whether it is an inner ear thing, by putting my bed in the middle of a room and tipping my head down the back of it, they are trying to bring on the dizziness and watch the heart monitor at the same time. I am surrounded by the whole team during this exercise, the consultant, three doctors, the specialist pharmacist and a nurse. I could not help feeling that this was a bit unreal and I must look funny. I thought my friend, whose room I was borrowing, would be looking down having a real laugh at this one! I do get some dizziness and fortunatley nothing untoward happens on the heart monitor.
It is thought most likely that I may have some viral infection or inner ear disorder, although infection cannot be traced. It is explained to me that as my heart is weak, it may have struggled to cope when the virus first hit me, hence the strong palpitations, the dizziness may be an inner ear disorder. Obviously if I have further problems, I can phone up the ward or the specialist nurse immediately, which is always of great comfort to me.

I was discharged after a comfortable night and back home feeling more assured. Before I am discharged one of the nurses and I have a laugh, there are not many people who are absolutely delighted that they have an inner ear disorder! But for me it is good news; if it was my heart and lungs deteriorating that may spell removal from the Transplant List or increasing my medication. Increasing my medication is a big worry to me, I know I will lose the balance I have now with my illness and my drug side effects. I will probably spend weeks in hospital being sick and unable to tolerate food and drink and the balance I have now will be lost forever, the side effects overriding the quality of life I have established for myself now and the disease reaching new heights.  Removal from the Transplant List, tells me there is no hope left and the doors back to possible good health are firmly shut.

I have managed to stay where I am, for now.

Friday, 16 March 2012

Home Again

I gingerly went to bed that night, wary of how I may wake up in the morning. The dizziness was still bothering me, especially laying flat, so I went off to sleep, completely and utterly exhausted, following my second day in hospital. I could still hear that thrashing rain and wind outside. I had to sleep nearly sitting up or the dizziness is too much, even with my eyes shut.

While I slept, Rob and Rose watched a programme on organ transplants, in it a person goes to the brink of death and eventually gets a heart transplant in his last hours, it brings it home to them, the realities of what may happen. It is strange, there seems to be a couple of TV documentaries about transplant at the moment and we had only watched a programme the week earlier, the story about a brave donor and the five lives she had saved. It was a very moving programme.

I can't think of anything better you can give to someone than the gift of life. It must feel marvellous to be a transplant surgeon and part of a transplant team to be able to be that bridge between donor and recipient, supporting a donors wishes to give new life to someone who has no hope left. I am only sorry these programmes were screened at ten thirty at night and not at peak viewing time, but hopefully they may have encouraged donors to register, even possibly my future unsuspecting donor.

I had a good nights sleep and I could still move without my heart racing, although I still had dizziness, we decide to make an early start home. I felt a little apprehensive about making the journey, but I really wanted to get back to my own bed and the safety and comfort of being in my own home. We phoned the transplant team to inform them of our movements and when we expected to be home.

It was still throwing it down and blowing a gale outside and packing was a bit of a muddle and confusion, there was stuff from my hospital bags and stuff from the other bags and we ended up just throwing everything into whatever case it would fit in, to sort out later. Rob's wedding suite, my wedding suite and hat and coat, our flowers, our evening ball gear, new shoes just got wrapped back in their bags. Unused, never needed, deprived of their intended use. Worst of all Rob had to brave the weather yet again to get the riot of things we had with us, back in the car. He was sopping wet through again and I was more than helpless, too weak, dizzy and exhausted to do anything.

We made the journey home in one piece and he had to go through the rigmarole again at the other end. He was soaking wet and cold again. Bags were duly sorted and the transplant hospital bag repacked and restocked and put away.

We sat down resting and all we could do is just look at each other, we were speechless and totally numb. For us this had felt the hardest experience of the lot. We really had thought on that wedding morning that things had turned for the worse and our hopes of making it to transplant were drifting rapidly away, floating out of our reach. We had really believed  when we dialled 999 that my heart was in terrible distress and possibly final distress; we had been totally desolate.

Now we felt completely devastated, emotionally drained and physically exhausted. The fact that everyone else had been at a wedding having a wonderful time, while we had endured one nightmare after another was difficult to bear. It just felt like such a stark contrast. What with the trauma of the wedding morning and then the trauma of a being terrorised by a drug addict, feeling ill in the first place anyway despite all this, and now feeling even worse than ever with this distressing dizziness, we both suddenly hit rock bottom, together with one big loud thump.

This was compounded by all the roller coaster of events that had happened to us already during the last twelve months and more and further bound by the fact we had been looking forward to this event for such a long time. But for us, we never got there, in one single tortuous moment we had missed the lot, but for everyone else the party carried on, not only on the day, but the whole weekend. The hardest bit to bear was that after the night before the wedding, we didn't see anyone again, we were only fifteen minutes away from the festivities for the whole weekend, but too far away. We had blinked, missed it and felt nothing but isolated.

Devastated and desolate are a poor excuse for words to describe how we felt at this moment. Going through events like we have had to manage over the last year or two is difficult, stressful and traumatising and sometimes life often feels like it is happening in slow motion, while the rest of the world wakes up every day and just carries on as normal. All you want to be is normal again, but you know it is not going to happen. I don't think you can ever get used to this, it just begins to live alongside you and becomes part and parcel of your life. How your life is now, how your life will now always be.

When you tumble to rock bottom, the only way is back up again. We have each other still; we have our gorgeous girls still; we have some lovely friends still; we have Papworth Hospital to support us still; we have plans still; we have hopes and dreams still; I am alive still. So off we go again on the climb back up.

Blessings now counted and first things first, Papworth Hospital wanted to admit me back in to check out what had happened and check me over. Help and support is always on hand from them when you need it.  

Better still, the sun has begun to shine outside again. The miserable rain and wind has died down and things feel brighter.        

Wednesday, 14 March 2012

Are A & E and Acute Assessment Units the Right Place for This?

It soon emerged that our fourth lady was the one who had needed the urgent medical attention that I had had to wait for while she was sorted out. It transpired she had a sore throat and had needed some antibiotics. She had a sore throat. Strange, a sore throat? It didn't seem to stop her from screaming loud when she first arrived in the unit; it didn't stop her from shouting and bawling for the entire afternoon and evening; it didn't stop her from wailing and bellowing out constant strings of  four letter words to get attention literally every ten seconds from the minute she was brought on the ward. I was extremely tired now after the days events and was still dizzy and  I began to feel exhausted and worn out, not least from all the constant commotion that was going on. The was a stream of overworked staff constantly attending to this woman.

I'm not one to feel sorry for myself, but I couldn't help but wonder why this person had had the immediate medical attention when I was first brought in and I had had to wait. Something seems wrong here: I've a rare and complicated heart and lung condition and am waiting for a heart and lung transplant and had had a real scare this morning. This person has a sore throat, yet she was brought in after me and given immediate attention while I waited. I do not wish to appear that I think that I am more important than others, but it does not take a lot of common sense to see which case should have been given priority. With all the comings and goings as she demanded more and more attention, it seemed evident that if you make a lot of fuss, then you get a lot of attention. This is really not my style and I expect both for myself and for others that the most needy should be given attention, not the most loud.    

During all this continual commotion and demands on staff, it emerged that the sore throat had been acquired because this person was a drunk, an alcoholic and a drug addict and she had little immunity. She was after drugs and trying to manipulate staff to this end. Of course, she has a very pressing medical need with these problems, but is the A & E or an acute assessment department of a hospital a place to be dealing with this sort of problem? Are emergency medical staff, who have to deal with burst appendices, heart problems and the rest, the ones who should shoulder all this?

The other two dementia patients were also becoming agitated and distressed with the woman's behaviour and doing their own shouting as well as bed wetting and again, I wonder should these medics have to be burdened with this as well? I am not saying that needy groups of people should be neglected and be bottom of the list, we are all needy if we end up at A & E, and I know I am quite needy with the problems I have got too and my problems are no greater than theirs.

The alcohol and drug problems seem to be growing in number and the demands on staff make them overburdened. It is well known to avoid A&Es on a weekend because of the drunks and if its not the drunks and addicts, then it is the poor old dementia patients, who nobody wants or cares about. It seems there is a great need to be addressing these facts and the staff in A & Es are strained because of it , as well as patient care suffering through no one's fault.  

A nice surprise happened in the middle of all of this, when the girls and Oli came to visit. The girls had kept their bridesmaids dresses on just to show me and they looked beautiful. I will always remember it and it cheered up what had been a rotten day. I hadn't expected any visits from the wedding party, but they really made my day and I was delighted at the effort they had made to come and brighten me up. Unfortunately that was all of the 'wedding' I managed to get a glimpse of that weekend and I was unable to give my good wishes to the bride and groom personally.

The consultant came to see me with the doctor, they wonder about an inner ear disorder, but cannot find any signs of infection or see anything on examination. I rather hoped this was the cause, it would be better than my heart being in trouble. They are unable to pinpoint what has been the problem and what may be still causing the dizziness.

The commotion across from me continued into the night and when lights are dimmed at eleven o'clock our dear little bed mate decided she would get dressed and come and scream and shout in all our faces, then scream and shout up and down the corridors and then back again to have a good shout in our faces. Fed up with this, totally emotionally and physically exhausted and needing to feel safe, after all I came in here to get safe, I ask if I can move wards. I now felt unsafe having a drug addict screaming in my face in the night, with no staff in the room, as well as having my medical problems. This is duly done, without hesitation and I was safe again, now amongst five dementia patients, but they are all behaving, they might have been having a shout here and there, but they were in their beds. I managed a few hours sleep.

Rob arrived early to make up my medication. It is an unknown process to the medical staff here and they were grateful. He had to find me first though, as I'd been playing musical beds in the night! He also spoke to my dear friend, she was in an assessment room by this time, being guarded by a policeman because she had hit a member of staff. She was shouting at him for cigarettes. Don't know whatever had happened to the sore throat, obviously a cigarette or two is a good cure!

The girl in the next bed, who had just arrived,  had taken an overdose and attempted suicide. So here I was fighting with all my will and determination to hang on to my wonderful life, waiting for a heart and lung transplant and wishing for it sooner rather than later, next to someone who wants to just die and give up on life, because its so bad. What a contrast if anyone was looking down on us. The consultant came to her talking about suicide, then to me talking about how transplant would save me!

It was time to get out of there, I'd had enough now. It was still lashing down outside and the heavens just seem angry. It was late into the afternoon and near dark when I was discharged out into the rain. Rob was already soaked wet through as he has had a struggle to park in the frenetic car park and we are both now soaking wet, along with the bags and the wheelchair back at the car.

Thank goodness I had my wheelchair; thank goodness I had my hospital bag; thank goodness we had booked the cottage for an extra night and we had somewhere to go in this weather; thank goodness for Rob and my girls and Oli, who helped us all lots; thank goodness I was out of there; thank goodness I am still alive. Again, I felt lucky, albeit cold and wet!

I'm felt in no fit state for the drive back to Hertfordshire, but I feared waking up in that cottage the next morning in case it happened again; I feared the long drive back home. I was still a bit dizzy and felt quite anxious, how I wished I was near to home and could be in my own bed, or better still have been checked by Papworth, just so I could have had some reassurance from the experts.          

Adventures in a New Hospital October 2011

I recall being lifted from the stretcher onto a bed in a corner of one of the A&E wards. Here the paramedics handed over the information Rob and I had given them about my medical condition and the ECGs they had taken. The 'cottage sheet' was taken and put with my bags. I was then wheeled to a single assessment room, where we went through all the information again. By this time I was absolutely desperate to go to the loo and a commode was brought and I was actually able to move myself on to it without being dizzy. At this point I began to hope that things may be better than I thought they were. Blood tests were taken and then not before too long  I was wheeled through to the Acute Assessment Unit. Here I was left in a bed at the back of the waiting room for a little while until an assessment room became free.  

Getting ready after the drama 
We went through our story again with a nurse and then waited for a doctor. While we waited I could hear another patient being brought into the next room, who sounded in a terrible state and in real pain and agony and I began to feel that maybe I wasn't in so bad a state after all. It seemed clear this patient needs were a major emergency and I settled down for a long wait while she was being dealt with. I remember still fretting about the 'cottage sheet' and Rob was amused, he hadn't even noticed how they had got me on the stretcher and he folded it and put it safe to go back. He left me for a short while to go to the loo and while he did this the dizziness started again when I moved my head and I had to shout for help. At this point the doctor came in to see me and took all my details again. Eventually I was settled onto a ward of four beds and linked up to a heart monitor. There were two elderly dementia patients in two of the beds - nothing new here then, although I'm in a different hospital. There was one empty bed still left.

My heart seemed to be calm now and ECG readings were showing good rhythm, so that was good news. I was still having the dizziness without the palpitations though, so they wanted to try and do some tests to get to the bottom of things and help me. They also wanted to phone Papworth for advice too, which I was pleased about. Rob had already phoned the PH Team for advice and informed the Transplant Team of my whereabouts.

It was almost twelve o'clock, so there was no way I would make the wedding. Rob had told the girls I had stabilised and they had got ready in their dresses and gone on ahead to the wedding. There would be nothing for them to do here at hospital with me and they would only be hanging around in the holiday cottage feeling useless, so after a traumatic start to the day, off they all went. Rob had been unable to get hold of my sister, but had managed to speak to her husband to be. He had also spoken to my brother, who took over doing the speech he had prepared, so all was in hand for the wedding. The staff at the hospital kept saying my sister would probably turn up afterwards, but I knew they had a busy day ahead of them and there would not be time that day for hospital visits.

I want my mum!
I was going to be kept in overnight and then the consultant would liaise with the consultants at Papworth on Monday morning. I got myself settled in my bed, Rob by my side and although it was devastating to have missed the wedding and all the celebrations and especially our girls being bridesmaids for the first time ever, I knew I was in the right place. I felt calm and settled and best of all I had survived to tell the tale. I was not out of the woods yet, this PH is a threat that hangs over us always and that threat has been heightened yet again today, but I am now full of hope that I will get out of here, which was contrary to my thoughts earlier this morning. We had well and truly missed the wedding now and I wasn't going anywhere tonight.    

All was calm, except for one of the dementia patients desperately trying to find her slippers, shouting at anybody and everybody, but at least the other one was too busy picking her nose to cause a riot. Oh well, I would be having a different kind of entertainment for the rest of the afternoon and tonight, instead of the wedding, a type of entertainment I was beginning to get used to.

A fourth patient was wheeled in to fill the last bed on the ward. Let the entertainment begin.                

An Early Wedding Morning Disaster October 2011

I had thought long and hard about how I was going to cope with the wedding day itself, the actual ceremony was at twelve o'clock, followed by a meal and in the evening there was to be a Venetian
Sarah and Rose bridesmaids for the first time ever
masked ball, the whole wedding theme was Venetian. It was going to be quite a gruelling day for me and we had brought my wheelchair with us, so I would always be able to sit down, especially during the photographs if they were outside and we planned to come back to the cottage after the afternoon events, so I could sleep and do my medicine before the evening events kicked off. I planned to take my diuretics as early as possible in the morning and drink very little, but I was anxious about how well I was going to feel, especially following an early start to get ready. My experience told me, I would probably feel pretty terrible and would probably not feel that well until after lunchtime and lunchtime was going to be fairly late that day. It was going to be a struggle to start washing my hair even and get my glad rags on, so we would be ready by late  morning, we would probably have to be ready to set off by eleven o'clock. I only usually venture out in the morning for doctors or hospital appointments, and on these occasions don't need to bath and wash my hair or get dressed up and I usually take the diuretics later, after the event. So, despite looking forward to the event so much, it was going to be a difficult one and I would just have to go with the flow and manage as best as I could with a smile.
Mum and dad and the bride and groom

On the morning of the wedding the alarm rang as usual at six o'clock and Rob got up to make up my intravenous medication, ready to swap the syringe at six thirty when needed. There is no respite from the drug routines whatever the event, there are no days off! I was awake and could hear him preparing my drugs, I decided I needed the loo and raised myself to get up. On doing so, the whole room span around and my heart began to pound rapidly. This did not feel right and I lay back down straight away, I had barely lifted myself from the pillow. I could still hear Rob happily making my medication and I decided to stay calm then give it another try, I was completely dizzy and my heart was racing fast and now thumping so hard it felt it would come out of my chest. I lay back down again, now absolutely petrified about what was happening to me.

Rob came into the room and I calmly told him as I lay still what was happening. I asked him to change my medication over and to stay calm, which he duly did. He felt my chest and my heart pounding, well he could actually see my chest pounding and was getting alarmed like me. I decided to try moving again, but no, the dizziness started as soon as I moved and my heart raced even faster. It felt as though 'this was it', I thought my heart had taken a turn for the worse and was giving up, although I wasn't actually fainting, it felt like I would collapse if I tried to move. There was nothing for it other than to dial 999.

So instead of waking the girls and Oli to get them up to get ready for the wedding, Rob had to wake them up to tell them there was ambulance on the way for me. It was horrendously distressing for them all at this time, I was still lying as still as I could, so I wouldn't pass out and lose consciousness and my heart was pounding through the roof. Rob and Sarah were very upset. Someone needed to be ready at the door to greet the ambulance and get all my medication and hospital bag ready - Rob and Rose went off to do this to keep busy. Once done they were back at my bedside.

Sarah and Oli stayed by my bedside holding my hands, I remember feeling so bad and poorly, I had to ask them to stop holding me and just stay by me. My thoughts at this time were that I was going to be lucky to get through this, if at all; that the transplant couldn't come fast enough and that it hadn't been fast enough and that was the end of all that; that I just needed the ambulance to come now to take the stress of my family, because they all felt so helpless and couldn't do anything for me and neither could I; that I was going to die and it was my sister's wedding day and this should not really be happening. I had often pondered what it may be like when the time came: would my heart just give up randomly and out of the blue and quickly and that would be the end of it or would it fail slowly and I would become sicker and sicker. Well that question seemed answered now, it was giving up quickly, randomly and out of the blue. I was just desperate for the ambulance to come, which seemed at the time to be taking an age. I lay as still as I could and although my heart was going fast, it had stopped pounding now I wasn't trying to move.

The ambulance arrived very promptly, although in the circumstances, every waiting minute felt like an hour. They decided to get me onto the stretcher without my having to move, so they lifted me in the sheet from the bed. I remember thinking I would have to make sure this sheet got back to the cottage somehow, but if I died the owners probably would not mind in the circumstances. It is funny the sort of things that go through your mind during these types of incident.

My heart seemed calmer now, although I still hadn't moved. Rob came in the ambulance with me and had to leave the girls and Oli waiting for news. My ECG showed good heart rhythm, but it was fast, well that was encouraging. I immediately felt safer being with the paramedics, my thought was, they could try and resuscitate me, if it comes to that and although I had tried to remain very calm through what was happening, I was now relieved of the stress.

When they took me outside to get me in the ambulance, the rain was still thrashing down in a cold, wild wind. The dark, dismal weather seemed a good reflection of what was happening to us. I arrived at the local A&E around about seven in the morning and it looked as though it had been a hectic Saturday night, I remember the strong wafts of  stagnant alcohol, the blur of fluorescent yellow police jackets and sounds of walkie talkies. Was I in a hospital or had they got mixed up and brought me into a police station or was I just totally out of it? Wherever I was, there were more police in the corridor than medical staff. Welcome to the A&E of a large busy town on a weekend!

A Wet Weekend and Pre Wedding Dinner October 2011

Its still September and I had my routine monitoring appointment with the PH team, this includes a walk test and blood tests and a check on my Hickman Line and site entry for it. All is stable and for the first time since I had been diagnosed I was told I didn't need to come back for three months. Hurray - something to celebrate at long last, my next appointment was sent through for end of December, but as it was during the Christmas period and I didn't know whether we may be busy with family, I changed it and got an appointment for early January. Three whole months off from hospital including Christmas! This felt like a good result, after more than twelve months of hospital visit after visit and test and after more tests, I was stabilised at last and I was beginning to feel I was.

Early October and it was my sister's wedding, which was taking place in the Ribble Valley was imminent. The girls were to be bridesmaids and I had spent a lot of time sorting out and altering their dresses to fit and sorting out my own outfits to wear. Rob is going to do one of the speeches on behalf of my father. We were really looking forward to it.

We had rented a cottage nearby the wedding for three nights, big enough for all of us to have our own space and relax before and after. Sarah was bringing her boyfriend and they were staying for three nights, we were going to stay for two as we needed to get Rose home to go back to school on the Tuesday, she has been given permission for the day off on Monday. Rob and I decided to make the journey up first and stay at our cottage in the Lakes for a few days beforehand, so I will be rested after the long journey ready to cope with the rigours of the wedding day. Sarah and Oli planned to go onto the Lake District after they had finished using the cottage we had booked in the Ribble Valley for the wedding. So there had been a lot of planning logistics for the days around the wedding and a lot of preparations. We were are all looking forward to and were excited about a long wedding weekend together and had been building up to it for months.

We enjoyed a day or two in the Lake District first, just Rob and I and the weather was glorious. I even managed a lovely boat trip round my favourite lake, Coniston Water. The wedding was to be on a Sunday and we travelled down to the Ribble Valley on Saturday and found the cottage we are staying in. It was lovely and spacious and best of all nice and warm, it also had a disabled bathroom in our room, so bathing should be quite easy for me.

When we woke up on Saturday morning it was absolutely lashing down with torrential, driving rain and this was forcast for the whole weekend unfortunately, but never mind we will still have a great time, you cannot plan the weather, however meticulously you plan. Poor Rob had to load the car and then unload it again in all the rain at both ends of the journey. We had mountains of stuff, bridesmaids dresses, suits, flowers, wedding hats as well as usual luggage, my wheelchair and mine and Rob's 'just in case' transplant bags, so he was pretty wet through and soaked to the skin by the time he had finished and I was helpless as usual when it comes to heavy lifting and carrying and walking up and down.

Many of the wedding party were also staying nearby including my sister and husband to be and their children and we planned to meet up with them for a pre wedding dinner that evening. Oli drove our girls up in the driving rain during the afternoon and the plan was they would meet us at the Three Fishes pub, where we were having dinner.  We set off to the pub, a little late, because we needed to do my intravenous medication first. It was a cold, dark drive and the rain was still pouring, we were splashing through flooded country lanes and not sure if we were going in the right direction, it was so bad and visibility so poor. We made it to the pub at last and met up with Jayne, Rob and some of their friends and Rob's family and the girls and Oli soon arrived as planned.

We enjoyed a lovely dinner. I had decided I would like to give my sister and Rob their present that evening, as we had spent a lot of time thinking what to buy them and we had had a bespoke piece of word art, with lots of their personal wedding and family details on it, made for them. I really wanted to see their faces when they opened it and I knew they would not have time on their wedding day perhaps to open it in front of us. They were really pleased with it and it was a lovely evening. None of us wanted to be too late to bed, because of the big day ahead, so after the meal we braced ourselves back into the driving rain and dark night and drove precariously with Oli and the girls following.

We ended the evening catching up on X factor and just enjoying being all together in the lovely cottage and feeling excited with the anticipation of the wedding. It was so lovely for us to be away from it all, all together and looking forward to something that had been planned for ages.