The Listing Meeting September 2011

Over lunch we tried to take in the enormity of what had been said to us and what we had committed ourselves to. We had agreed in the meeting with the surgeon, that we thought transplant was the best way forward. He had gone through the risks entailed in the surgery, which are complex and many.

These included: infection, bleeding, blood clots, failure and rejection of the transplanted organs. I had then signed the forms consenting for the operation, which felt frightening enough without all the problems that had been explained that may happen to me post transplant. Post transplant there has to be a lifetime commitment to medication, which helps control infection and suppresses the immune system to avoid rejection. There are many side effects to the drugs, some very risky and there is always the risk of acute rejection and chronic rejection.  This is why transplant is offered as a final treatment, it is not to be taken lightly and there is no going back once it is done. One set of problems is swapped for another, but there is no guarantee that things will be any better and in fact there is a big risk, they could get a whole lot worse.

But the balance is still  a fifty per cent chance of not surviving the next three years and fifty per cent chance of living five years or longer post transplant. The odds are in my favour for transplant and I have been told I am a good candidate. It is strange to sign forms for an operation when you have no idea when it will be done, but it is different than a planned operation, everything will be an emergency when it happens and it is better to have things explained to you when you are calm and sign the forms then. I will have things explained again and sign again when it happens too I believe, but goodness knows what I will be taking in when that moment comes.

I have also signed for various research projects that are being undertaken investigating a variety of things regarding transplant such as rejection. And I have signed that my old heart and lungs may be used for research purposes, well they will be of no use to me and it may help someone else.
 
After lunch we go back to meet the transplant co- ordinator to go through what I can and cannot do while on the list. Surprisingly the 'cannot dos' are only one: I cannot travel abroad as I will be too far away for them to get me should a donor be available. However, I can travel anywhere in the UK as long as I inform the team if I go anywhere further than three quarters of an hour away from home and I am given twenty four contact numbers to phone for this reason or to phone in emergencies. I am even allowed one unit of alcohol a day if I like, this is really easy for me because I haven't really been drinking alcohol at all in the last year. It had been very easy to give it it up, although I did use to enjoy a glass of wine or two (or three!!!) in days gone past.

We had a lot of questions to ask, which were duly answered such as: how does 'the list' work? Well you are not top or bottom of the list or working your way up it, it purely depends on a 'match' coming in and then your condition at the time. If there is any other competition for the organs in your region, then each case will be considered and the most suitable patient decided upon: there may be no competition at the time so you get the goodies. A match may come into another region and if they have no takers, then, again, my luck may be in, if it is offered to my region. A whole team of  specialists from Papworth will go out to the donor's hospital to do a thorough check on the organs, then the organs will be brought to Papworth and be assessed by the team there. While all this goes on, I will be brought in and they will start getting me prepared ready for the operation. I may be brought in by ambulance or helicopter, depending where I am at the time. While I am being prepared and all this checking and double checking the organs is going on, it may be found that the organs are not suitable for me after all and if there is any question over this, the operation will not go ahead. It was explained that a few false alarms like this may happen and I need to be prepared for this.

I was also very surprised to find out I would be heart and lung transplant patient number four on their list at that time. I don't know why, but I'd always imagined there would be hundreds! There are only twenty odd patients waiting for lungs and thirty odd patients waiting for hearts, or was it the other way round? Whatever, I was surprised again at this.    

Well I think that was everything and more than enough for one afternoon, back to Wimpole Hall Estate for that much needed cup of tea and a slice of cake, where we sat in the garden once again, watching the early September wasps getting on everybody's nerves and children enjoying the last day of the school holidays.

The sun always shines at the end of the school holidays, it always used to sunshine when I went back to my new class after the summer. I didn't have to worry about that now, I miss that feeling at the beginning of a new school year, the excitement, the fresh start ....... but there was a feeling in the air for me that late afternoon, that this will be the right way forward, that everything will work out, that I will be one of the lucky ones, that I might have to start worrying again about that pension, that I might hit seventy after all. The turmoil of the decision that had been hanging over us since February had ended, the decision had been made. It was done. The door was open wide.

I had to wait now for confirmation from the team that I had gone on the live list. There is a substantial amount of data collated then input into the national register and this may take a few days.
Another important decision had been made by Rob, he had given up work now and we were on permanent holiday now, so we decided to plan a short trip away, while Rose was away on her biology trip.


Norfolk and Suffolk here we come.    

PH Team Again and Back Again for the Surgeon September 2011

We returned from our trip to the Lakes and my problems with my Hickman Line site had worsened. I was getting concerned because it was very red and blotchy and some of the skin was raised. I was frightened that its going to escalate and get into the entry area, cause an infection and then things may get serious. I telephoned the specialist PH nurse and after speaking to the consultant, I had to go back to Papworth for the afternoon to get it checked.

After a lot of deliberation, they decided its probably an allergy to the dressing and they changed the dressing type I will need to use each week. As they are not totally sure sure, I have to have a series of blood tests including blood cultures just to rule out the possibility of infection and have the entry site for my Hickman line swabbed.. I will need to continue monitoring it and phone back straight away if there is any deterioration.

I got a phone call from the transplant co- ordinator, who says there may be a possibility I can see the surgeon later on that week. She arranges a time for me to come back to Papworth, but nothing is definite as the surgeon may get called to an emergency and might not be able to see me, as I live close it may be worth us coming up to to see though.

We manage to see the surgeon. He brings it home to us once again that I am in a delicate state and we are brought back down to earth with a bump once more. He goes through some of the things that are in danger of happening to me should I continue as I am for too long, amongst these are heart failure, my heart giving up and pulmonary aneurysm. We have this conversation in a calm manner, as though we are speaking about the weather.  He had a lovely, caring nature and was very kind. I always have that feeling when I am with the transplant team that I should be turning to look behind me to see who it is they are talking to, because it cannot possibly be me. The harsh reality though is that it is me, and I know this deep down, but it is so difficult for this to sink in and register properly in my mind that this is really happening and that things have come so far, so fast. Twelve months ago I hadn't even got  a proper diagnosis and eighteen months ago I was racing round as a full time teacher and busy mum, blissfully unaware of how my life was going to change in just one moment.

The surgeon confirmed he thought that a heart and double lung transplant was the best plan for me at the moment, although plan B would be a double lung transplant should the situation become more urgent. The size of my dilated pulmonary arteries are the issue and matching them to donor blood vessels in the new lungs could present a more vulnerable situation with more risks. It would not be an impossibility though and as lungs can be available more quickly, then I could be listed for just lungs on the transplant list too. At the moment, the plan was to put me on the live list for a heart and double lung transplant. The fact that I am going to be listed for transplant also paves the way for a third specialist therapy should I deteriorate while I wait.

The surgeon had had all the test results back from my assessment stay by then including my blood tests. My blood group is Group A and my antibodies are very low. He explained that there is a quirk, that cannot be medically explained that many donors tend to be blood group A, so this would work in my favour in getting a match for my new heart and lungs. This quirk in blood groups, plus my low antibodies and my size was a good indication that I could get a match. As usual in the transplant unit, they like to spring a shock. When Rob mentioned the average 'two year' wait for heart and lungs that had been explained to us on our last visit, the surgeon commented that he would be confident he could get me donor heart and lungs in six to eight months time. This was because of what he had already explained, my blood group, my antibodies, my size. I think Rob nearly physically fell of his chair at this point and I was thinking he really is talking to someone else now.

After the surgeon left, the transplant co -ordinator confirmed what the surgeon had said, she had been doing this job over twenty years in various transplant units and told us to be prepared for it to happen even this side of the six months. She asked us to go for lunch and then return so she could do the 'listing' meeting with us. This would be about all the inn and outs of being on the list and waiting for transplant.

We went off to lunch, quite dazed I think, with a lot to absorb.  Heart and double lung transplant operations are not commonplace, the surgeon had explained how only five had been done in the UK in the last twelve months, he also added that he had done them all, in a very humble and unassuming manner. Well I was in the right hands then - he better not be on holiday when my time came!

While we are waiting in the waiting room, Papworth Hospital is featured on the news because they had just fitted one of their patients with a pioneering artificial heart. It is rather strange to see my hospital on the main news. The patient is being interviewed just outside the transplant centre, where we are and we have just walked past that very spot.

At home, we are reminded again as Papworth is featured on both the six o'clock news and the ten o'clock news because of the artificial heart. I didn't really need reminding, but there was the hospital now in my own home, bringing it home to me and reinforcing to me that transplantation is the route I will now go down.

The transplant operation was now not a glimmer in the far future, but an imminent and very real prospect. At this moment in time Rob and I were both absolutely stunned.

Getting Out With a Serious Illness

We enjoyed our bank holiday break in the Lake District. As I coped so well staying in a hotel on our last visit, we decide to give it another go this time. We booked into a hotel for an evening in the North Lakes, near Keswick and planned to spend an afternoon there and then drive to the Solway Coast the following day. We spent a lovely afternoon in Keswick and round about, then got to the hotel.

Fishing boat at Whitehaven

We were pleased with our room and we noticed there was a nice indoor swimming pool. I suggested to Rob that he may like to have a swim, although I can't because of my Hickman Line and pump, I went with him. I braved it in a t shirt covering my extra bits and my bikini bottoms and sit with my feet in the pool, and enjoyed watching Rob have a swim. It felt very relaxed and I felt like I was on a real holiday being by a pool. A lovely moment. A moment though unfortunately that I think was the trigger for a very irritating problem. It was very hot and humid in there to keep the pool temperature warm and inviting and of course I couldn't swim to cool off  like others were doing. Soon afterwards, I noticed my skin becoming red underneath my dressing, which covers my Hickman Line. This can signal life threatening problems if the entry site becomes inflamed it may be an infection. The line goes direct into my heart and blood stream and infection can cause septicaemia, which has a rapid onslaught and ends in death if not caught swiftly and treated promptly.

I needed to watch the site closely now, it was just a bit red under the dressing and the redness was not around the actual line entry site, so we were not too worried.  The dressings used are clear so that the site can be checked regularly for problems.

We enjoy our hotel stay and drive up to Cockermouth, I planned to take my diuretics when we got back to the cottage later, so the daily 'weeing for England' does not impact on our day out. At Cockermouth I had quite a  long walk and we manage to visit Wordsworth House, a National Trust place. I needed to rest with a cuppa  after this, because I had probably walked too far and my legs ached and I was tired. We headed up to the Solway Coast and had a picnic lunch next and a small walk. We were near the car and I felt my legs would not take my any further, I was exhausted.

The Gondola, Coniston

A busy day the day before, and quite a lot of walking that day, however I do like to test how far I can push myself. It is always in the back of my mind, do I really need a heart and lung transplant? I stumbled into the car, completely exhausted and fell asleep straight away for an hour. We needed to make sure we got back in good time for my medication too, I do carry spares all the time in the event of an emergency, but would choose to do the Epopostrenol in the clean conditions of my own home when possible. When we have stayed in hotels, we have managed to do it safely using a tray we have purely for this purpose, lots of Dettox wipes and sterile gloves, so we have found a way round it, so we can live life a little more to the full.

There are lots of extra challenges when you are sick and trying to live normally and go out for the day or go out at all, for me its things like: how far can you walk without dropping or your legs ache too much? How fast can you walk when there are people trying to get past you and pushing? Can you manage those unexpected stairs and still live to tell the tale when you get to the top of them? When you go for a walk, constantly checking how far you have walked as you need the energy to walk back. Fatigue is a major problem, even just having a conversation can wear me out at times. Remembering to take the tablets, I take eleven or twelve tablets in stages throughout the day, as well as twenty four seven IV drugs that are changed twice daily.

Warfarin thins my blood and I get cold easily, some of my other drugs make me flush and my heart just doesn't like extremes of cold and heat. I wear layers all the time, always taking an extra layer even if its hot and I have to take great care when sitting in sun, in fact its difficult to sit in sun, my Epoprostenol is light sensitive and my dressing on my Hickman Line tends to get sticky and cause a rash if I'm not careful.

The diuretics feel like a big bug bear, on a day when I actually might feel well, I still feel restricted on when I can go out, if I take them as normal in the morning, then its a struggle to go out before lunchtime; if I do go out before then, then I have to take them when I get back and suffer having to run to the loo all evening. On top of the diuretics side effects the nausea is often worse in the morning, so mornings are always difficult. My day in the real world usually starts at lunchtime.

Eating out is something I like to do, but is always challenging, will your tummy cope and the sickness and diarrhoea tablets work? Your tummy is somersaulting with nausea and cramps, will it go away or get worse? Will we have to leave the pub or restaurant when we have only just sat down. I struggle with food and what I can manage to eat as my Epoprostenol and Sildenafil upset my stomach. I also find it hard to go too long without food, I think its the drugs going straight to my stomach sometimes and this can sometimes pause a problem if we are out and about and I can feel very ill and feel light headed, then I worry I might faint and if I faint it will mean all sorts of problems. It is an achievement for me every time we eat out successfully.



Timing is of the essence, Epopostrenol cannot be made up late, PH symptoms can come back with a vengeance if there is a blip with timing or a blip on the line, it can be life threatening; it is always there in the back of my mind, have we got all the spare equipment should an emergency happen with the line? Are my emergency cards handy so medics would know there is an existing problem and my Epopostrenol supply must not be shut off at all costs. Time needs putting aside to change the dressing each week and change the line connector and extra time allowed in the daily process every other day to change the line.

A lot of these things are manageable when you are with your partner who knows all the ins and outs of the problems and lives with them everyday like you. When you are company of other people, then it becomes harder.

There is often an expectation that you are now feeling a lot better and that you are out and about, you can be how you always have been and unfortunately people are deceived by the situation, especially as this disease is invisible. Sometimes it can be a bonus that I do not look ill as it allows me to go about my business in a normal manner most of the time. The truth is though that it is an effort and a strain to be out and about, as we manage all the difficulties, but I love to get out and about if my illness allows. I want to be in the real world not laid on the settee or in bed feeling sorry for myself.

Luckily we have some brilliant friends, who do seem to understand and do make allowances for me without being patronising.

Anyway that is just a flavour of what it is like for me, for everyone with a chronic illness there is a lot to endure that others just do not see and I know for a lot of people things are a whole lot worse than what I have to endure, so I count my blessings for now.

Back to the Lake District now though and Rob and I are on our own, enjoying our own company and managing to work around this PH and its problems.  

       

Holidays, More Tests and more Holidays August 2011

Following this visit for assessment, we were left with important decisions to think about: was this definitely the way forward for me? If so, how were we going to manage the waiting period?

Gardens at Sharrow Bay, Ullswater

Rob had now got two weeks annual leave from work and Sarah and Rose were going off on holiday to Greece, so as I was feeling quite well and side effects of the drugs improved by my recent additions to medication, we decided that it would be good to escape up to our cottage in the Lake District again for a week or so. We had to give the transplant team these dates as they wanted to make arrangements for me to see the surgeon soon.

 Before we went I decided it would be easier to get my mammogram and smear test over and done with in one go and quickly and avoid getting into NHS long waiting lists for the mammogram. I really wanted a bit of control now where I could at last and I didn't want to be waiting another three or four months before I could get back to the transplant team and continue the discussions on my suitability  for a transplant. I therefore booked myself into the Bupa Well Woman clinic, where they undertake these two procedures for you as part of the process, as well as giving advice on breast examination and lifestyle. I had always intended to do this last year, when I cut my hours to part time work and felt I needed to start the process of getting fitter. I had been stopped in my tracks on this front, with the shock of my unexpected illness, and although I didn't really want to find out I had cancer as well, that would definitely be a death sentence for me in my position, chemotherapy or no chemotherapy, it was something that I thought I needed to do. So in a strange way, I was glad to get round to it, although I had had my fill of hospitals in the last twelve months. The appointment was booked for two weeks time and off we went to the Lake District.

Gardens at Sharrow Bay

We had a nice time here, visiting some of our favourite places, visiting new places and me getting plenty of rest and relaxation around my illness. Rob surprised me and booked us into a beautiful hotel on Lake Ullswater for an overnight stay, he thought I was doing well enough again to manage this. The weather was atrocious on the day we went and it was quite hairy negotiating the Kirkstone Pass to get there. The hotel was beautiful and the bedroom had the most fantastic view of the Lake and so did our table at dinner. I can clearly remember the waiter apologising for the view being spoiled by the torrential, driving rain and we just smiled and said it was still nice. In my mind it was the most beautiful view ever, I felt lucky I was sitting there looking at it and felt grateful I could still enjoy all of this, the ambiance of the evening; the Michelin starred meal; the spectacular view, despite the weather; the beautiful, exquisite environment of the hotel and its grounds and most of all my caring, doting husband. I would have liked to turn round to the waiter and tell him he really has nothing to apologise for!

When you are sick with an invisible illness, there are often moments like this, people don't know there is anything wrong; what you have been through; what you are going through and what you will need to go through next. You find yourself 'acting' a lot of the time, trying to 'act' like you are 'normal' having a 'normal' life.  Whatever is normal? I feel a long way from it and keep trying my best to get back there.    

View from Sharrow Bay accross Ullswater

While we are away we heard the devastating news that my friend, who had been moved to the hospice, had passed away. It truly saddened us and saddened the PH community too. She had done so much to raise money for PH awareness and research and it was a shock to lose her. She had been so brave. This terrible news I think and the rapid loss of my other friend in February and the death of the lady who I met on my first visit to Papworth, probably helped sway us even more in favour of transplantation. The outlook that doctors had spoke of, became a grim reality. These lovely ladies who I had had the pleasure of meeting had lost their lives to PH, they had all recently been put on Epopostrenol like me. I felt an urgent need to be getting that door to transplant opened as quickly as possible. We both did, Rob even more so I think.    

When we got back home, I went off to yet another hospital to have my smear and mammogram, these are always a bit stressful and daunting for a woman in ordinary circumstances, but I had extra burdens and I did feel apprehensive. The bottom line was, if they find cancer I would not have the physical strength with my weak heart and lungs to get through the treatment and the door to transplantation would be closed forever. It was a bit strange to going to a well woman clinic when you know you are already seriously ill. I am still a woman though; I'm still susceptible to all the health risks other women face; I still have to face the run up to the menopause; I still have elderly parents to worry over; I still have my lovely children to mother; I still have to go through all the ups and downs of real life just like well people do; life does not stop because you are ill.

It is a bit traumatic having these tests, having to explain my situation time and time again to the doctors and nurses and radiographers, especially when I am sitting in a well woman clinic! We had to be there early - well what is early for me now, I used to be an early bird before all of this - I felt very unwell. So a huge relief when its over; two weeks later and an even huger relief when the tests are negative. Now I'm really getting on track towards going on the transplant list.

We informed the transplant team of the test results and then went back to the Lake District for the August bank holiday. Another little break and escape away in our favourite place. Rob had decided, after much turmoil and pondering and with transplant becoming more of a reality that he was going to leave work for a while, so that we can spend some time together while I am well, we really do not know how long this stable period will last. He wanted to be with me so he can care for me both pre and post transplant and do so without the pressures and worries of work.

Of course, there is the small matter of making ends meet while he does this and his employers were helpful in this respect, giving him redundancy, which would help a long way to getting us through the next two years, when Rob could then draw an early pension if needed.

View to Fellfoot Park from Lakeside

Again, I know we are lucky and blessed that we are able to do this and for a lot of people in my position, money would be a massive issue and a huge stress on top of all the other horrors that they have to face and go through.

I had had to give up my work as though it had been the flick of a switch and I had worked so hard in the last ten years to achieve all I had in quick time. I had done a part time degree in three years, the time usually needed to undertake a full time degree, I had successfully completed my teacher training and my teaching induction year and I had spent two further years back at University yet again to achieve an Advanced Diploma in Education. All this while working part time or full time and trying to be a good mother and wife. I was in my seventh year of teaching and really hoping for a management role soon. Of course I would not have achieved anything if it hadn't have been for the loving support of my husband and girls. Rob was also giving up probably one of the best career opportunities of his working life and a chance to really make us financially secure for our retirement that had been being planned for ten years time!

So a very big deal; but nothing in the scheme of things.    

Still we were in the Lake District now, enjoying a lull in our world of sickness and hospital, escaping for a while. Our cottage has always been a retreat: when we worked, a retreat from deadlines and work pressures; now a retreat from hospital, tests, waiting for appointments and follow up letters and worst of all waiting for the next drama that could happen at any time with this life threatening illness.  

Waterhead, Ambleside

We were back in a cosy bubble for a few days, resting, recuperating, important decisions made and on holiday indefinitely now. Rob did not need to go back to work.

The Transplant Assessment August 2011

We arrived early back at Papworth Hospital and I was admitted back on Duchess Ward again. This is becoming like a second home. We had waited and waited as usual for the appointment, it had come at the right time, just before we went on holiday back to the cottage, so we could try and get away from it all, once we were done.

Gardens at Sizergh

We were greeted by one of the transplant co- ordinators first and he explained what would happen over the next few days. A tranplant doctor then saw us and examined me and took my history. I had a walk test and lung function tests. In the afternoon we met another of the transplant co -ordinators who went through a lot of the ins and outs about the transplant operation, what would happen before, during and afterwards and she explained all about the medication.

The whole process was daunting. I was quoted by my consultant that there is a twenty per cent chance of not surviving this operation and a lot of possible complications, plus only a fifty per cent survival rate after five years. The drug regime that follows sounds daunting and even the drugs pose some very serious risks such as diabetes, lymphoma and certain cancers. This is on top of other side effects such as nausea, sickness, headaches, shaking and others. It sounds like I will really be swapping one set of problems for another, but if it keeps me alive and with my family for a few more years then this is all that matters. Both my PH Consultant and Transplant Consultant thought I was a long way down the diagnostic pathway for my PH.

Fuscia, Sizergh

On the second day, I had an echocardiogram, which was in line with my last one, showing that my heart was improved. I then met with the consultant again. He went through a lot of the things I had been told before about the risks of the operation and the statistics for survival after transplant and the stastistics for my survival in my current condition. All in all, he felt I would be a patient who may benefit from transplantation, I might get another five years if I am lucky as opposed to the two and half year prognosis I had now. Of course, no one really knows and I can only be advised on the statistics they have got and my medical history to date.

The next bombshell was then dropped, this was becoming a bit of a routine in the transplant unit. It was looking like a heart and double lung transplant would be a safer option for me. The consultant explained that my pulmonary arteries are markedly enlarged, he even showed me a picture of them on screen and highlighted how dilated they had become by sizing them down to what they would be in a normal patient. A bit frightening all of this. Matching these to the donor vessels would be tricky, although not impossible. It was explained that there is usually an average two year wait for heart and lungs, but this may be in my favour as I was quite stable and well at the moment. Two years sounded far away enough to get my mind round it, but near enough to ease my worries about my condition.

I was asked to make sure I am up to date with my dentist visits, I was, so no problems there. This is because infections can be passed through the gums, which could pause a problem post transplant if I had gum problems. I was also told I would need to get both up to date mammogram and smear tests. Again, they need to rule out any other major disease before putting me on the transplant list. I was lucky to have no other medical history prior to this dreadful PH, other medical conditions can also affect whether you are suitable or not to be listed. So all looking good so far.

I had to have a whole host of blood tests, including HIV and an antibody check again. So far my antibodies were showing very low, which is good for matching to a donor and good for rejection problems. All in all I was a good candidate and the consultant just wanted to go through my case with the transplant team the following morning and check with the surgeon about his thoughts on heart and lung transplant versus lung transplant. As we lived quite near, he said I could go home for the evening and then come back in the morning.

Solway Coast

As I had had some problems the week before and been taken to A&E, the PH team wanted to check me out as well. When I had this check, I mentioned the nausea and diarrhoea I was having still and I was prescribed domperidone to take daily for the sickness and loperamide for the diarrhoea. They felt my drugs were keeping me stable now, but wanted to help me overcome some of the side effects now I had been on them for a few months. We also had to be observed again making up the epopostrenol medication - no rest for the wicked! Eventually I was discharged and was given a time to come back after lunch the following day for the verdict. I should have met the surgeon, but this had not been possible due to his work commitments.

An awful lot to take in again.

While I was on the ward this time, there were a few things happening that probably influenced my decision in favour of transplantation. The lady next to me had turned down a lung transplant at an earlier stage and now she had deteriorated, her heart in a poorer condition and she really hoped she could be accepted for a heart and lung transplant now, although she feared she would not be well enough. I feared if I turned the offer down, then I may become too ill to be listed.

One of the ladies I had met on my last visit was still on the ward and she was losing her fight against PH and had become too unwell to have her heart and lung transplant assessment. Our stories up to now had been similar, but I had now been offered a second chance.

 Rob and I walked out of the hospital feeling very sad and I felt very humbled that I was being given another chance and another door was opening for me. Again, I felt blessed and lucky.

Coniston Launch

We returned the following day and it was recommended that I have a heart and lung transplant. I would need to meet the surgeon and have a listing meeting with the transplant co -ordinator before I would be put on the live list and it was also subject to my mamogram and smear tests being clear. I would need to arrange these next. The other lady having her assessment was in the waiting room too and she had been offered a heart and lung transplant also. We were both pleased and overwhelmed that we had been given this chance. I remember feeling how surreal everything was, it was a strange thing to be delighted about really and I felt overwhelmed with sadness for the other lady, who had not been so lucky. She was a similar age to me, with two children and a devoted husband. This disease is really so cruel.  

Rob and I left the hospital and called at Wimpole Hall, a National Trust place nearby for a cuppa. Again, it all felt strange, our conversation about heart and lung transplants, in the summer sunshine amongst the families eating their ice creams in the middle of the school holidays. We used to bring our children here a lot when they were little, at the time I didn't know I would become a teacher and be bringing my Year One classes here to visit the farm and the garden. I remember thinking I could never have even dreamt this one up, that I would be sitting here a couple of years later, drinking tea and discussing my heart and double lung transplant.

The world was going on all around us, while our world was spinning and spiralling more out of control and into the unknown.

Fun in the Acute Assessment Unit July 2011

When I arrived at hospital, I was whizzed through A & E, through lots of corridors and straight to the Acute Assessment Unit. Here I was dealt with promptly and efficiently, my story listened to sympathetically and in detail. Blood tests were taken and an X ray arranged. As my last visit to this hospital, I had to have the blood test that indicates a heart attack, which is followed by another blood test several hours later. The staff were all too pleased to be given the twenty four hour number for Papworth I have been given in the event of an emergency, and made a phone call to them straight away. I recall thinking, remember to go to the doctors first again next time, this was a totally different experience from last December at this hospital. I do not have the answers why: is it better quality staff? Is it because its during the day and a weekday? Is it because the doctor sent me and I didn't just turn up? Is it because I came in an ambulance? Or is it a combination of these things? Whatever luck was on my side today, I was very frightened, wary and anxious about my illness and the staff were doing all they could to make me feel safe again. When things go wrong with an illness, all you need is to feel safe and secure and today I was in the right place.

The staff on this unit have a lot to cope and contend with, I am in a small bay, only part of this unit, with six other patients. One patient is, from what I can gather a cancer patient, having some secondary problems and he was soon discharged home, but he had been there a while from what I overheard and been dealt with well. He looked a similar age to me or a bit older. They had me, who had just walked in with a rare heart and lung disease, none of them had never seen a PH patient before, let alone heard of the intravenous medication I had, with the Crono Pump and Hickman Line, but they appeared to be taking things in their stride.

The other four patients alongside me were thirty years plus older than me and appeared to have a variety of problems, but one problem they all had in common was severe dementia. So not only were the staff having to cope with their urgent medical conditions, they were having to deal with the dementia problem as well, which appeared very testing. They were all incontinent and were shouting out like young babies, the whole time they were on the ward. They were all hard of hearing and could not really understand what was being said to them or give coherent answers.

One lady was shouting out for her horse that she thought she had lost, in between needing the loo every five minutes and she was very distressed. Another old gentleman had brought in a carrier bag; as the nurse went through what valuables he had with him, it transpired he had hundreds of pounds in his wallet and in his pocket and a carrier bag full of cash. He had only brought in most of his life savings by the looks of it. It is frightening that old people are just so vulnerable and it was heartening to see the staff doing their best for them.

As for me, I don't really know whether I'm in the right place here or not, it is quite disturbing when you are feeling so ill to have to listen to shouting, howling and complete mayhem going on around you and I have got a rare condition that the staff are having to speak to another hospital about. Still, I know I am safer here, if my heart is about to start packing up again, at least they can try and resuscitate me or try and get a reading of what is happening by ECG and then pass me on to Papworth.

It can be argued are the elderly in the right place here? They obviously have urgent medical issues on top of all the other problems they have to face and are completely vulnerable as they haven't really any idea what is going on. Staff are having to deal with their extra demands, because of dementia, but at least they are in the right place to access the urgent medical attention they are needing.

All this is going on, complete mayhem, the staff have to talk to the elderly patients in a loud voice, not because they are shouting, they are speaking loudly, but gently to help them understand. Of course you hear every word of it, not because your nosy, but because everybody is shouting!

I lie there calmly, somewhat bemused by all this carry on, contemplating my escape from all this: if I could just catch that woman's horse that is roaming around somewhere, I could make a quick getaway with the old gent's life savings and ride off into the sunset! Then I'm not feeling that calm, the nurse is trying to take some blood cultures from my arm, but for the second time is having to drop everything because the lady next to me has wet herself again. I do feel sorry for the lady and the nurse, but I'm getting a bit bothered whether those plastic gloves the nurse is wearing are same ones that she had on when she started. I can only put my trust in people at this point.

In the end they can't find anything disturbing causing the chest pain. Discharged and home safe again. I feel grateful and lucky again. I have a caring husband, doing all he can for me; I've got my lovely girls; I've got my friends, who visit and take me out;  I've still got my wits about me and understand what is going on; I'm not on my own, vulnerable and afraid. Better still, although they cannot pinpoint what the chest pain is, other than muscular and because my heart is under strain, at least it doesn't look like I'm having a heart attack and my heart is in good rhythm still. I now have some peace of mind, after all my worrying, which is a lot to be said when you walk round and sleep with a life threatening disease twenty four hours a day.  


The plight of the elderly is not going to go away, it will only get worse as the elderly population increases. The systems we have now are not really geared to cope. From what I could see, staff were doing the best they could, giving their all and more, but it was not enough, although I don't think they could have given any more. In my ideal world, these A & Es should be staffed with carers, who can help reassure, calm and attend to some basic needs, while the nurses and doctors get the urgent medical needs either sorted or continued in the appropriate department.

And back to the Story: Fun with Doctors and Nurses

I was now waiting for a phone call or a letter from the Transplant Team to let me know when I was going to be admitted for my assessment. Waiting becomes the norm when you are ill: waiting for appointment letters; waiting for doctors appointments; waiting for hospital appointments; waiting for blood test appointments; waiting for consultant and doctors reports; waiting for prescriptions; waiting for test results; waiting, waiting, waiting in waiting rooms. Your patience is tested to the limits and back again. I ponder often is that why we are called 'patients', is it derived from the word patience?

Over the last six months I would often get chest pain and sometimes it would be painful enough to wake me at night and sometimes it would be quite worrying. I had had some strong chest pains over the weekend, they had been continuous, sometimes a dull ache and sometimes sharp and stabbing pain. It seemed to radiate out around my heart and left area of my chest and I had some tingling down my arm. I did not feel so happy about these chest pains any more and was getting quite concerned, although I had mentioned them on several occasions, both in hospital and at outpatients clinic and the doctors had not seemed too bothered or interested.

It was a strange coincidence that day that the PH nurse rang up to see how I was getting on. I explained to her that I wasn't too good at the moment and it was chest pain that was worrying me. She immediately told me I must go to the GP and get an ECG done. That was easier said than done at my doctors surgery, where appointments with the nurse are usually booked for a week or two, but I gave it a try, explaining the situation and was lucky to be given an appointment at the other surgery they have for that afternoon. Rob needed to come home from work early so he could drive me there. I wasn't well enough to be going on my own.

When my turn came to see the nurse, she was a little put out by the fact that there was no paper work to support my having this ECG, but I explained the situation and she seemed satisfied to go on with the procedure.  At this point she told me I should get completely undressed to the waist down. Having had more ECGs than hot dinners at this point, I knew that this was totally unnecessary and I had also worn loose, buttoned clothing to accommodate this test. However, she seemed in no mood to be questioned, so I did get on and get undressed. There were two windows in this room and they were fully exposed, so I was not impressed at this stage, but kept quiet because I still felt coming here was probably the better option than turning up at the A & E at the local hospital, considering my bad experience back in  December. She had drawn a curtain to my amusement round the bed I needed to lie on, I suppose this was so that no one would be able to see me, a bit of a joke stood there topless on the outside of it. I didn't know whether to laugh or cry, but I have learned that laughing to myself at how pathetic or bizarre a situation is usually the better option.

I knew as I clambered round the bed and found my way through the curtain that I would have the upper hand next, and I purposefully and gracefully got onto the bed and laid down with my Hickman Line displayed very nicely, sprouting out of  the wound in my chest. Ten - nil to me! Her face was a picture and I have never seen an attitude changed so quickly. Suddenly she could not do enough for me. And that is how it should be when you are walking round with a life threatening disease every day, knowing that one day your illness may get the better of you. Many days are spent coping without any extra support and getting on with things; when you really need the help, because you know something is not quite right and possibly this may be the day all the treatment fails, you need people to be at least respectful of you and at best, sympathetic and helpful. Mission achieved, I was told the heart rhythm looked fine and she copied it for me in case I needed it for the doctor.

The next stop was to get an appointment with a doctor, the pain was twinging a little, but was more intermittent and calm. Again, doctors appointments are always booked days in advance, but an emergency appointment can be booked if you visit the surgery at eight thirty in the morning - too late for today, but next plan would be to get to the doctors by eight thirty in the morning. I felt I still needed a chat and check with the doctor and had been advised by my PH nurse to do so.

I got my appointment easily enough and Rob decided to go in to work later and come with me to the doctors. In fact he walked round with me in his suit and brought his briefcase with him so he could carry on round to the train station after. I usually take my spare pump with me everywhere plus a full set of spare medication just in case of an emergency, as I had been told to do when I first started the Epoprostenol. The doctors is only across the road so I didn't feel it was necessary to do so today.

We were lucky, we saw the doctor who I had been to see about my condition when I was first put on Epopostrenol, so she knew my history. I explained what had been going on and about the ECG, the doctor was not happy about what I had been experiencing and before I knew it a blue light ambulance was rung for     and I was put on oxygen. Blooming typical, I was being rushed to hospital and I hadn't got my spare pump or medication with me, the only time ever I have not taken it with me. That will teach me. Rob was not supposed to leave me alone, the doctor had left him sitting with me while she carried on her surgery until the ambulance arrived. He ran back home and got it before the ambulance arrived and the doctor didn't know he'd disappeared for a while.

So instead of leaving the surgery and tootling off to the train station for another day's work, Rob was now with me in the back of an ambulance, whizzing with a blue light, off to the local hospital. I remember him saying, 'never a dull moment with you!'



   

Hospital Fun and Games July 2011

When you are seriously ill, it is inevitable that you visit hospital regularly and sometimes a variety of hospitals. When I first found out that I had a problem I had used our private medical insurance to get tests that were needed done quickly, so we could get to the bottom of things quickly. On reflection later, I am relieved we had had this now and the ability to go privately for tests, as my PH was so severe by the time I got to Papworth, it is quite clear I would probably not have survived waiting months and months for this test or the other, given the waiting times on the NHS; not only the waiting time to have the tests done, but the waiting time for the follow up appointments to actually find out test results. Having said that, once tests were done and there was sufficient information to be referred to a specialist centre, all the specialist centres for PH are NHS. Papworth Hopsital has proved over and over again so far to me that it is a centre of excellence and absolutely 'five star' and 'premier league' and the NHS should be proud. It is the NHS at its best. I can only say in my experience, once you have been diagnosed with something pretty serious, the NHS can be second to none in providing the appropriate care.  

During the time awaiting diagnosis, I visited a few private hospitals and the local hospital and I have to say had frustrating experiences at both.

The local hospital is not geared up for patients who have unusual diseases and is really geared to treating patients who have a variety of more common problems. This is quite rightly so, they cannot be expected to know about and have the ability to treat every single rare disease or condition that is going, however, it can be easy when your symptoms are of a more general nature to be swept along by general experts and general assumptions. In many cases of PH, patients take years to get diagnosed  for this reason.

My CT scan at the local hospital looked as though my lungs had scaring and the conclusion was the general reason for this, possibly blood clots. As I now knew that there was definitely a lung problem, I began to research on the internet all the jargon that had been put in writing about my lungs. It was amongst this research that I kept finding reports saying that it is only a very specialised eye that can interpret what scarring is caused by in the absence of blood clots. I had been told that the cause was probably just several mini blood clots, but I was reading about vascular disease, which presented very similar results on CT scans.

I had joined the PHA by this time, knowing that I had PH, and was becoming increasingly aware that this seemed to need treating at a specialist centre. Rob and I researched where these centres were located in the country and were coming to the conclusion that this was where we needed to be going, pretty quick. We also concluded that Papworth was probably the best one to go to, as they are the only hospital that performs the operation to remove blood clots from the lungs, they also perform lung transplants if it ever came to that.

Armed with this information we had a discussion with my cardiologist, who at this time also thought that things had become beyond his remit, he found himself unable to prescribe the specialist drugs I needed, and it was agreed that I be referred to Papworth. As it was, we picked the right place, but at the time it was furthest in our thoughts that it would be for a transplant! Before we got to this point though, I was told as I had feared that my PH was Idiopathic and that I had not got blood clots. I am glad we trusted our instincts on this one. My only regret now is that I didn't ask for this referral earlier or the opportunity for referral wasn't offered earlier, but everything is easy to do on hindsight and we were swept along in shock in those first few months. I had gone six months from PH being first mentioned to actually getting treatment that worked for me and during this period my PH was at its most severe.

I believe, I am blessed to have a specialist unit looking after and caring for me, there are many stories of patients with rare diseases that have nowhere to turn and they are passed from pillar to post or have to raise money for treatment in other countries to help them.  

I also found that the private hospitals I visited were very lacking at times. You feel quite secure in the knowledge that you have private medical insurance when you are well and believe if anything happens to you then you can get quickly sorted. This may be the case if you want a few tests and a routine simple operation or procedure, but it is the NHS that steps in when you need more and have a chronic or terminal illness, the ability to have yet more tests to monitor the condition and any associated problems is no longer there.

These private hospitals can be very lacking in the service they provide too. I needed an angiogram undertaking quite urgently in those first few scary weeks on finding I had a problem and as private angiograms could not be done at the local hospital, the nearest place was Harley Street in London. Here I was attended to by a male nurse, which I found quite embarrassing every time I had to have my groin checked,which seemed like every two seconds following my procedure. I was new to all of this and wasn't quite as inhibited as I have become now I'm a 'seasoned hospital goer'. Being a 'seasoned hospital goer', I find at NHS hospitals that you are asked beforehand if you are happy to have a male nurse, when there are embarrassing things to be checked, even just to have an ECG you are asked. I don't usually object as I trust it is their job and they have to do this all day long. In this early stage,however, I would have much preferred a female nurse to be looking after me, especially as I was paying for the privilege.

The experience at this hospital went even further downhill when a work man decided to barge in and start putting up some shelves in my room. My room was meant to be a private place and I was laid there feeling anxious, my life turned upside down and in a whirl and terrified of what they were going to find. Now I was laid down in bed, in a strange place, feeling very vulnerable and had this bizarre situation of a man in blue overalls coming in and out with a work box and a drill, drilling in my room. I did not really need this at this moment in time. This situation was exacerbated when the nurse and consultant, who was to undertake the procedure came into my room to explain the procedure and he still carried on. At this point I signalled to my husband to stop any further talks and get rid of him. I couldn't really believe this was being allowed to happen, let alone pay for the pleasure! I was really angry, but another part of me wanted to laugh so much,as it looked like some cartoon sketch and you couldn't really make it up.  


These hospital traumas were to be the first of many to come.    

Transplant Continuing Care Again June 2011

Things seemed to be fairly stable and at the end of June, my next visit to the transplant team loomed close. This was really still hanging over us, but as things were stable, we thought the outcome would probably be that I would be monitored closely again. The same routine happened, a visit to speak to the nurse, height and weight measuring, medication updates and then in to see the consultant. This time it was a different consultant, the previous one had been a locum consultant.

We went through my history again and where we had got to with the current drug increase, plus we discussed the drug side effects and my limitations. I hadn't had any more faints, that was the big plus for me, but I was still limited in how far and how fast I could walk, although my walk test had improved since my previous visit; I had chest pain both on exertion and at rest; I got tired very easily and needed stop and rest frequently; the calves of my legs ached terribly after I had been standing or walking for fifteen minutes and the jaw pain was still quite bad. Plus I was still experiencing nausea and diarrhoea, which had worsened when Sildenafil had been added to my drug cocktail.

The consultant went through my history and he discussed the severity of my PH and how my PH appeared very aggressive. His thinking was the PH had been diagnosed late and on diagnosis was at end stage. He explained gently that when I had been fainting my heart had been giving up and this was of some concern still, he was also concerned that I had had fluid in my heart and the right side was dilated. He also had an x ray or CT scan of my lungs up on his computer screen, which showed how damaged my pulmonary arteries are, which are markedly dilated and scarred. Although my fainting had stabilised for now and my latest echo showed much improvement, he was concerned about my past history and the severity of my PH on diagnosis. He believed my heart had been under a lot of strain and possibly for a long time without treatment, considering my fainting started years ago. There is no way of telling whether my earlier fainting was PH or not, myself, I am totally convinced it was, without any doubt.

He went through some of the statistics again with us on survival rates after transplantation and survival rates for PH. For transplantation it is fifty per cent after five years and for PH it is fifty percent after three years following diagnosis and treatment. He considered that given my late and severe diagnosis and the aggressiveness of my PH that the three year outlook for me may not be that  positive. I was already on end stage treatment and given my drug side effects, may struggle with much further increase if any.

All this considered he felt it was time that I should be admitted back into hospital and have a full assessment for a double lung transplant.

He confirmed our worst thoughts really, although we had tried to be positive about the situation, this scenario had not escaped our thoughts. So another blow and another step on the journey of this illness, another route, another path to go down. All quite scary. It would be so nice to get some good news, but being positive, this was another option for treatment, with the possibility of a better quality of life.

I could not really ask for more care in these circumstances, now under two very specialised teams in a world renowned hospital. Much more than a lot of people get opportunity for.

It was at this point that Rob really began to consider his position at work. What would he want to do if they put me on the transplant list? Would he be happy commuting from Hertfordshire to Canary Warf every day, working a ten/ eleven hour day with a commute of an hour and a half either side and having to give total commitment to his work while we waited for the phone call? Did he really want to work like this and me be at home not feeling that well, only for me to be rushed into hospital in emergency circumstances and then possibly face a long recovery? Precious time that may never be recovered for either of us. I think he made up his mind there and then really, if I go on the transplant list, he was going to stop working and we would have some quality time together while we still can. Time that is free of work commitments and pressures. Time for us and the family together. We did our sums, could we stretch our savings for two years? At least by then he could draw some pension early to help us if we were still not out of the woods.    

We would need to see what the outcome of the assessment would be.

A Beautiful June 2011

Rob had got himself back to work He found his new role challenging in different ways compared to his last role and it was demanding in time and commitment. I think he found it hard to separate himself from the ongoing saga at home with me and the total dedication needed to get a demanding job done, however he was giving it his best shot and things were going quite well. He was very concerned about the transplant issue hanging over us and really, after the rollercoaster last twelve months, it still felt like a delicate situation. He had hated having to go back to work on the day I was admitted back into hospital, but had no choice really, we had to start getting on with our lives or at least trying for the time being before events took over again if they did.

It was very difficult for me that first week when I came out of hospital, I missed him dreadfully and the mix of Epopostrenol and Sildenafil along with everything else began to take its toll again, the side effects seemed more heightened again. Despite this, I began enjoying the period of relative stability that I was in and started learning to live with the side effects of my drugs.

I found strategies around the problems caused by my drugs; restful mornings and any plans for the day taking place only from lunchtime onwards seemed the answer. I always seemed to perk up after lunch. When I used to teach, the children used to buy me lovely bath time presents and I had accumulated quite a lot of bubble bath, lotions and potions. I started using all these lovely presents to make what I called the 'bath time palaver' more of a treat than an endurance. Thank you  to all my lovely children, you don't know how much you helped me! If I needed to go out at night, I would need a sleep in the afternoon to be able to cope and usually just one outing a day was enough to manage. I often use the quiet mornings for my guilty pleasures of facebook, emailing, texting and reading the papers, and now don't need an excuse to sit and read a good book and do little else!

The spring weather continued into early summer and the garden was stunning and Rob worked really hard at weekends to make it lovely for me. I also tried to potter about around it and try and do easy lightweight bits I could manage. Gardening had been a passion of mine before my illness. I was meeting friends for tea and lunch in the sunshine, many of my friends happened to have cut their hours to part time or have retired altogether, which had been a bonus for me in all of this, they were around a lot and really supported me. All in all, life did not feel that bad again, and I was actually really enjoying being at home, life was pretty perfect had it not been for the illness that lingered over me, but I knew compared to other people who are seriously sick too, I was still very lucky to be enjoying all this and enjoyed the good moments to the full.

I think as a family we were beginning to learn to live with my illness and adjust to its ups and downs and I was becoming a seasoned PHer! The only trouble is that when you are ill with no cure, it never leaves you and you go to bed with it, sleep with it, wake up to it and live every day with it. I think feelings you go through are probably similar to the feelings of bereavement. There is shock, anger, desperation and great sadness and sorrow. All the plans you have for the future are blown out of the window and you know you will have to follow a very different route to what you thought may happen. Somehow, if you are lucky like me, you manage to find an uneasy path where you muddle a way through, although you never know where it is going. You find ways to cope and strategies to help you manage so you can go out and live life to the fullest you can achieve within your limitations. The short term future is the one to focus on, taking each step as it comes and making the most of each day. Thinking long term any more is a difficulty as goodness knows what will happen, there are too many 'what ifs' and unknowns. It is hard when you have been told your life span will be shortened. If you do start thinking longer term, it is best to think in a positive way and imagine that all is going to work out well for you, you are going to be the one who defeats all the odds. There is no alternative really, otherwise you will start to slip down what I call 'the black hole' and I stubbornly refuse to do that. Really that's what most of us do anyway, if we haven't been struck by illness at all, we make plans for the future and think we are going to live until we are about ninety without too much bother, these thoughts are just more heightened and focused when you find out you are ill.

These future matters come with some bonuses though: I'm not going to end up being ninety and all alone in a nursing home; I don't have to worry too much about the increase in pension age, especially being a woman and now having to wait until I'm sixty eight, with threats of this ever increasing, I've been given my private pensions now; I don't have to worry about my teacher's pension being devalued any more and about striking, as I've already got it and got quite a good deal; I don't even have to go to work any more and definitely wont be doing if I get better, that long term plan is definitely to catch up on even more good times and fun; I don't have to drive in the snow and worry about getting to and from work and getting stuck in it; I can visit places that are busy at weekends when they are nice and quiet during the week; I can take advantage of all the week day meal deals offered in pubs at lunchtime; I don't get plagued with that Monday morning feeling any more, everyday is a weekend; I can have a pyjama day if I want to and nobody will blink an eye, because I need it; I have lots of time for indulging in simple pleasures such as watching the birds and flowers in the garden and reading endless books; I'm now a blue badge holder and I can park much more easily, my family and friends just love taking me with them so they can park!  


All in all, there are some advantages to being ill and I'm making most of them all at the moment when and while I can! You have to look on the bright side of things amidst all the disappointment!

Success with Drugs at Last! June 2011

Following this, I waited patiently to hear when I was going to be admitted back into hospital to try Sildenafil and have more tests to see how things were going.

When you are a seriously sick patient, you find that a lot of time is spent having to be patient and wait for phone calls about blood tests, appointments and admission into hospital. Obviously to you these things are the most important things going on in your life, but to a busy medical profession they are only a matter of daily routine and an every day, even every minute occurrence. You find yourself getting impatient, wondering when the phone is going to ring or when the letter may drop through the letter box.

While we waited for this, we took another short break in our cottage in the Lake District and I managed quite well, home from home, usual routines with medication and coping with the side effects and some nice visits out in afternoon. We were still blessed with gorgeous weather. I was also in touch with other PH patients that I had met through the PHA forum, which helped a great deal.

After what felt like a long wait, but in reality probably not that long, I was admitted back into hospital at the end of May to try the Sildenafil again. Rob had just completed his three months career break looking after me and was given a new role with his company. As luck would have it, on the first day of this new job I was admitted back into hospital! Sarah took me in that day and when I got there and settled in, I was lucky enough to meet two members of the PHA forum, one girl was in the bed next to me and the other girl was visiting her. We had a long chat together and it was nice to be able to meet them. They had both done lots of fundraising for the PHA and also helped to run the Papworth PH Matters support group and I decided it would be good to try and attend this sometime. The lady in hospital had similar problems to me and was on Epoprostenol and Sildenafil like me, so at six o'clock each evening during our stay, our husbands were busy making up our medications for us.

They started me on a very low dose of Sildenafil to minimise the side effects and I coped with this well. I also explained about weakness in my legs I was experiencing a lot and they looked at my iron levels, which had been low and prescribed a course of iron. I really hoped this would improve my walking ability. They ran some tests and my heart looked much improved on the echocardiogram and my walk test had improved. The consultant still wanted to increase my walking ability and there was talk of increasing Epopostrenol at first, rather than trying Sildenafil. I wasn't very keen on going through 'death by Epopostrenol' as I now called it and was going to fight this one, I would rather walk more slowly than my quality of life dragged down by even stronger side effects of this drug. Fortunately, Sildenafil was the option decided upon, I think with a little ear bending from my specialist PH nurse. Thank you, thank you, thank you!

The Sildenafil was increased slowly over a few days and after five days and tolerating it well, I was allowed home. Normally Sildenafil is given to a patient and if there are no adverse side effects, they are allowed to go home the same day. After all the sickness I had endured, it was decided to keep me in for observation and monitoring just in case. A positive visit this time, but quite stressful for Rob, starting a very demanding new job and having the worry of me in hospital to contend with.    


I was now feeling quite pleased and although the drug side effects were heightened again (I don't think the Epopsotrenol really wants to share me with the Sildenafil), at least I had now achieved tolerating most of drugs that had been planned for me back in February. I felt I was stabilising at long last, only more time would tell, but things looked like they were on the up. I thought this would be good news on the transplant front also, I was now on the dual therapy and responding well, so when I went back to see them, which was fairly imminent, they would be pleased and hopefully say too early again, we will see you again in six months!

Welcome to Transplant Continuing Care Unit April 2011

Today was my first visit to meet some of the transplant team to see what they were going to say about my referral for lung transplantation, and this was quite daunting for me. I still could not quite believe it had come to this in such a short space of time. I didn't really know whether I should be pleased this was happening or totally disappointed with what was happening. There is somewhat of a conflict of emotions going on here, both for Rob and myself.  Should we be delighted another door was being opened for me and there would be another avenue and future hope, because things were not looking that brilliant at the moment? Should we be feeling  disillusioned and demoralised because things did not look very bright for the future at the moment. My PH was still being classed as WHO class IV.

At the clinic we were promptly attended to by the Transplant Co-ordinator, who took us into a comfortable room, offered tea and then went on to ask my lots of questions about my past medical history, lifestyle and the run up to my ending up here in transplant. I was then taken by a nurse and had my height and weight measured and went through my medication details.

Following this, I was then seen by the Transplant Consultant.When we went into the room, it all felt a bit daunting for me, not the least because couldn't quite believe this was happening and was still shocked I think and because I was frightened really about what they may say. I have to say up to this point Rob had continually asked doctors and consultants about my life expectancy and we had always been quoted the same facts and figures by my consultant, I will quote from a letter she wrote for me regarding my ill health pension as I think this may be a questionable point for some people, there is varied information on life expectancy on the internet and appears to be varied information on what people are told by their consultants when this issue is discussed on the PH forum.

My consultant wrote this: 'Historically patients with haemodynamics such as Mrs Graham, have a very poor survival rate, with only 50% surviving less than 3 years. With current medication we are improving the survival and currently the five years survival is 60%'. This information was given to me verbally also on several occasions when we asked for it in those early days too. I obviously trust my consultant implicitly, as she is one of the country's leading PH specialists and is a Director of National Pulmonary Vascular Diseases Unit. Also, because of the high pressures in my pulmonary arteries and my poor cardiac output,classifying my PH as Who Class IV, my consultant thought that it was quite likely that I fall into 'the poor prognastic category' of these figures.     

At first it had seemed really important to know and then I could gauge how much time I might have, but as time went on and we began to go through the hoops of my illness, it became a less significant issue, we realised we just had to make most of any good times and each day as it comes. There were four or five people in the room, and I was pleased Rob was with me to help me.

We went through my medical history again and the consultant then explained some of the details regarding lung transplantation, he tried to keep this simple and explained it would be too much for me take in, if he went into too much detail at this point. He did, however, touch on the operation itself, the medication regime and then the risks,compared to what I faced now. For lung transplantation there is an eighty five per cent chance of both surviving the operation and the first year, then fifty per cent chance of survival after five years. There were also risks of complications remaining from the operation itself, developing some cancers and diabetes because of post operative drugs required. There were also issues discussed around quality of life as it was now and how it may be post transplant. He then asked me how I felt about having a transplant and pursuing this line of treatment. At this time I replied, I would be grateful if we could explore the option, as it opened another door for me, when options were beginning to run out.

The consultant explained that the PH team had been right to refer me for transplantation - there is a very delicate balance with my condition and indeed for any other patient facing transplant - you can be too well to put yourself through all the risks that are attached, you can be too ill to face going through the gruelling operation and survive. He thought in my case it may be too early to actually put me on the list as other lines of medication were being explored and dual therapy may give me at least two more years perhaps if  medication could stabilise me.

The consultant then went on to say he would keep me under the transplant unit now and monitor my progression alongside the PH team. I remember being quite relieved at this, but grateful that we had had a frank talk at least with the consultant and begun to open up another 'door'. Rob and I were then shocked when he said he would like to review my situation again in two months time, we had expected him to say six months. Before I left, there was another round of blood tests, these seem to be standard, wherever I go! I think any sick patient will tell you this!

I was not out of the woods yet.

Really, I should not have been surprised at this outcome, when we were last at the PH outpatient clinic, Rob had had a frank talk with the specialist nurse and she had stated to him that I was not what I looked and I was deceiving, inside I was a very sick person. I knew this deep down, I had known this from the first day I found out I had PH, but part of coping when things keep going the wrong way is to put a brave face on and keep trying and hoping for best outcomes. It also helps to keep yourself informed so you can be prepared for the next onslaught.  It is for me, the only way to go forward,as well as to focus on all the things I can still do and not what I am unable to do.

Yet More Tests and a Big Celebration! April 2011

Before my birthday, I needed to go back for my follow up appointment following my discharge from hospital. This was also timed with some further tests that I had agreed to have to help research for treating and testing PH. I had already been involved with some research when I came to Papworth on my first visit: I agreed to take part in a blood sampling project for PH patients and also I took part in a test which involved blowing into some apparatus, which could give some measures needed to determine the progress of my PH as an alternative to getting them by having right heart catheter procedure.







































































 This time the tests included a walk test, which is standard in measuring PH symptoms, but as well as walking, I had to blow into a tube linked to a computer beforehand and after I had finished as well. I also had a bike test, where I was wired up to heart monitor, computer and had to blow into a tube while I exercised on the bike. This would also give some accurate information on my PH. I did not perform too well on my walk test, not achieving even three hundred metres, but I still felt so unwell since my last visit to hospital. The bike test was not too good either, I hadn't long eaten lunch with some struggle and the tube in my mouth made me gag because I was nauseous before I even started. The peg on my nose had also been put on in such a way that it really hurt me. All in all I felt a bit stupid as well as unwell, peddling on a bike, all wired up with my shirt unbuttoned, being observed by a doctor, a physiotherapist and my husband with my mouth in a tube and a peg on my nose. To be honest I felt too unwell still to be having to be bothered to do all this, although I did want to help others if I can and on this day that's the only reason I did it.
There is no cure at the moment for PH and it can still only be controlled to a certain extent by drugs, although in the long term the outlook isn't so good, only a three year survival rate for fifty per cent of patients is predicted, from diagnosis and first treatment. PH affects all age groups, genders and race. It is important for me to try and help others in the fight against this disease, especially the children, for whom I hope against hope there will be a cure one day other than transplantation. I will be surprised if a cure will be found in my lifetime, but there is a lot of research and advancements are being made all the time.

Following the tests I saw a doctor. Here it was discussed how poor the tests were and also the problem of my nausea and the need to increase my medication still. It was decided that I would be admitted again in the near future to try again with the oral drug, sildenafil and also to have some tests to measure how I was progressing.

My fiftieth birthday came and I really enjoyed myself, we had planned to go out for some lunch with the girls, but I didn't really feel up to it and I wasn't bothered about this, I was just so pleased to be at home with my family. They made a huge effort, lovely presents, balloons, flowers and Sarah had had a special cake made for me. Really, I felt privileged to still be alive and loved every minute with Rob and our girls. The sun shone, as it had done for a few weeks now and I enjoyed my new summerhouse. We did manage the meal together a few weeks later, which was a lovely occasion again.  

The only thing that really marred my birthday was the fact that my dad was really very poorly again, I had a phone call to say he was back in hospital and again in a terrible state on the eve of my birthday. I had spent months hoping I would stay alive and well myself, looking forward to this day and it hadn't crossed my mind that dad may get worse again, they didn't expect he would live the night. Now the worry was swapped to whether my dad would survive my birthday. Luckily he did, but it was nerve racking each time the phone rang, until we got the call that he had picked up.

Two days after my birthday a visit to meet the Transplant Team back at Papworth was looming.
Happy Fiftieth Birthday, Kathryn!