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