Thursday, 29 August 2013

The pha UK

Imagine being sat down after what should have been one simple routine test and expecting them to find nothing and then being told your heart is damaged, the pressures in your heart and lungs are abnormally sky high and then in the next breath you are told that you probably won't have long to live anymore.

Where do you turn? What do you do next? How do you cope with the enormity of what you have just been told?

Well if you are like many people these days, you will probably fumble and stumble your way back home and then sit down on a computer and start googling. This is exactly what Rob and I did on the night I was first diagnosed by a cardiologist at my local hospital. The results of the searches we made just got worse and worse when we already knew things were pretty bad, but then with a massive stroke of luck, amongst all the doom and gloom we came across a website for the phaUK. We had been frantically trying to find some reliable and detailed information about Pulmonary Hypertension so that we could begin to understand and make sense of all that we had been told by the doctor. It was something we had never heard of being such a rare disease. So that is how we first came across the phaUK and the organisation which has very solidly supported us ever since. We were so relieved to find an organisation that could help us make some sense of everything.

The phaUK is a small charity and was set up to support and provide information for people whose lives have been affected by PH and to help fund research to assist with the many aspects of managing PH such as new drug treatments, finding the causes of PH, and supporting the PH Specialist Centres (there are only 9 in the UK).

During those initial first weeks when we were full of angst and fear over the future, the phaUK provided us with a whole range of handbooks and booklets, which helped inform us on every aspect of the condition, from all the treatments and surgery, about the specialist centres, to all about living with and managing PH. We began to learn what we were dealing with and what we might expect in the way of future treatments during a period of great turmoil and this helped us try and carry on and go to work each day and try and function as normally as we could manage when all our family's world had been blown upside down.

At this stage I was being treated locally by a very kind and understanding cardiologist and although he knew enough about PH to diagnose me and do further testing, he had no means or expertise to give me the specialist treatment needed or for that matter to even begin to classify my PH so I could get the right treatment. We realised through the literature supplied by the phaUK that I needed to be referred to a specialist centre, who could classify the type of PH I had and how severe it was and that is how I ended up being referred to Papworth. The doctor who had been treating me, although an expert in cardiology, said at the time that such a rare disease was out of his depth. So we were left worried and frightened once again, not knowing what would happen when I got to Papworth, knowing my disease was being practically untreated and that I was deteriorating by the day.

Again, the phaUK came to the rescue during that period when I was absolutely beside myself and feeling pretty desperate, this time in the shape of the patient online forum they ran. At first I found it useful to read the threads that patients were chatting about, it gave me some consolation and was comforting to see that others had been in my situation and were experiencing the same and many were stable again and coping with their disease. I eventually began to join in the chatter and as a result met some friends who have supported me from the very beginning of my disease, these friends really understood every step of what we were going through and reassured me right from the start. Many have become lifelong friends now.

The phaUK organise conferences and family weekends for PH sufferers, where there is chance to meet and socialise with other patients, take part in lifestyle and recreational workshops and learn about all the up to date research and developments for PH that they are helping to support. PH affects all ages from young babies to the elderly and can be a very isolating disease as people haven't heard of it and therefore have no idea about it. Mention cancer to someone and there is an automatic, unspoken and clear understanding of what that can mean; mention PH and you will usually get, 'oh my brother has hypertension'. There is no idea of the implications and that it is chronic and progressive and in its late stages a terminal disease. So, you can imagine the chance to meet other sufferers is very important for patients.

The phaUK do a lot of work to educate both the medical profession and public about the disease. Because of its rarity, patients are often misdiagnosed in the early stages, as I was myself, and then it is too late when the PH has been identified as damage has already been done. They are working hard with the medical profession to try and address this problem. In these worrying times of NHS budget cuts, they are also working hard to provide a strong evidence base to keep the Specialist Centres that have been established for us. I can honestly put my hand on my heart and say if it wasn't for my specialist centre, I would not be alive today.

I have been discharged from A & E as being perfectly well on several occasions and when my PH centre has stepped in, my heart has been actually been packing up; I have had doctors who do not understand the importance of some of the treatments and had them trying to alter some of my routine prescriptions, which would have sent me straight into heart failure and no doctor or nurse I have come across outside my PH centre has any idea about the IV Epoprostenol drugs. They are always relieved when I am in a hospital outside my PH centre that Rob volunteers to come in and make my IV drugs up; they are usually frightened to death of it and won't dare to intervene. So you can see the importance to patients of maintaining the PH Specialist Centres in the future, they save lives everyday.

The phaUK are also working hard to maintain the budgets for our specialist drugs and the development of new ones; one of our oral drugs has now come off patent and has become the cheapest drug and so obviously this is the drug that the health executives will want to be used first compared to pricier drugs on the market. Our PH centres will have to be able to evidence more than ever the need for the more costly drugs and justify the cost of new drugs. I was put on some of the most costliest drugs from day one as they were what was required to save my life. This is a big focus of the PHA's work at the moment to develop and maintain the treatments available to us and is so important for the future.     

So that's just the tip of the iceberg of some of the many things that the phaUK do for us patients and after three years of living with PH and being supported by the phaUK, it is time for our family to try and give something back.

I am able to take take part in the phaUK'S major study of 'Living with Pulmonary Hypertension' (IMPHACT study) and have completed my first questionnaire, which will be done every 6 months and Paul from the phaUK is coming to visit me to undertake an interview about living with PH. It is an 18 month project, with 240 patients and 120 carers involved and will be the first in-depth research looking into the long term impact of living with PH. I am so pleased to be able to be involved with this project.

Rose is going to take part in the 'Run to the Beat' half marathon in London to raise some much needed funds for the phaUK: I hope you can see now why Rose chose the phaUK as her charity for this run and how important it is to our family.  I would like to say a massive thank you to all my friends and family who have already sponsored her, we have been overwhelmed by your generosity.

You can sponsor Rose through her Just Giving page below: 

You can learn more about the phaUK and their work below: 


Friday, 23 August 2013

Summer in the Lake District

As a family, we have always enjoyed part of August in the Lake District, we have done this for years and this summer was no exception. It was a good time to get away from it all and try and leave all the transplant and illness stuff behind - well as far as we could, it is always there physically, but we were hoping that maybe mentally things would feel better for being away - and they were. Everything began to feel just that bit better for being away from it all.

We went to lots of our favourite places including Sizergh Castle, Sizergh Barns, Levens Hall and Grange Over Sands and met up with family and friends. We spent a few days with my mum in Lancashire and took her out and about and Rose and David joined us in the Lakes for a while too. 

David had never been to the Lakes before so we tried to show him a few of the beautiful Lake District landmarks and some of our favourite pubs and eateries: Coniston Water, Windermere, Arnside and Grizedale to name but a few! 

We spent a lovely day on Lake Coniston picnicing and Rob, Rose and David went kayacking; it is a dream of mine that I will be able to do this once again some day. We bought our kayacks six or seven years ago and I used to love taking them out on Coniston Water, the view from a kayack in the middle of the lake has to be one of the best views in England. I know I will do it again one day. 

I would also dearly love to get walking properly again, there are so many fabulous walks at every turn. I've managed to do a little bit of walking though as well as use the wheelchair, because I'm trying hard to keep my muscles working so they don't lose too much strength before my transplant. I know they are not as strong as they should be now and it is not helped by the muscle pain that is caused by my specialist PH drugs. 

For now though I'm happy I'm well enough to travel here and still manage to get around and out and about, the sights are dazzling and spectacular and that is great therapy, which is second to none! 

One of the highlights of our holiday was meeting up with Bente and Les again. We last met them back in October at the PH conference and have kept in close touch every week since, so it was fantastic to enjoy their company for the day. We had lunch in a lovely pub in the countryside near Ambleside and then had tea and cakes by the river at Skelwith Bridge, the sun shone and it was a perfect day. 

The one thing that I can say about this awful illness is that I have met so many wonderful, brave, inspiring and lovely people along the journey and that is something I can honestly say I wouldn't change or swap for anything.

It has been a calming and enjoyable holiday, a calming oasis for a while. I think we're ready now for another busy spell in September with preparations for Rose's half marathon run for the PHA UK, which is coming up soon and getting everything ready for her return to University. We're also feeling positive for the future, enjoying what we have and what we can and when we can and trying not to dwell too hard about what may or may not be ahead of us.

You can find out more about the work of the Pulmonary Hypertension UK on the following link:

You can sponsor Rose through:

Friday, 9 August 2013

The influence of a Soap

Last week an episode of Holby City was broadcast by the BBC, which featured organ donation. The episode was watched by more or less five million people so there was a fantastic opportunity there for the BBC to help the NHSBT in their cause to raise awareness of organ donation and transplant issues. We do not usually watch this programme, but switched on in anticipation and expectation that some of the issues surrounding organ donation would be explored and explained to the general public, which would then help towards all the hard work many people and organisations have done to promote awareness.

Soaps can be very influential in this day and age as millions of viewers tune in to them regularly and they can potentially influence a lot of people. They are often used as a vehicle to raise awareness of important causes and can help disseminate any controversy that may surround them.

Unfortunately this particular episode portrayed the organ donation process inaccurately on so many levels, irresponsibly jeopardising all the hard work undertaken to raise awareness of organ donation and increase the numbers on the organ donor register. There are still only a third of the population registered as organ donors, so there is still a long way to go in getting people to understand about organ donation and transplantation and informing the general public so people can make that important decision whether to be an organ donor or not. 

In the programme a potential donor was treated as a commodity and the donor family was bullied into agreeing to organ donation against their wishes, this was done by the actual doctor who wanted to perform a heart transplant to save another one of their patients. The mother of the donor also asked the doctor if she could go to have a look at the the recipient and she got to see her and meet the mother so she could check them out first. A heart transplant then ruthlessly took place when the donor's  mother had said she didn't want to donate her daughter's heart after all and after she was allowed to burst into the operating theatre.

All a very dramatic stuff, and you would expect some big elements of drama in a soap; however, one of the biggest fears that people have when considering organ donation and not understanding the processes and protocols involved, is that should they be in such a state where they may donate organs, doctors will be overzealous in their keenness to get their hands on them and their families feelings may be ridden over and not be considered in all this zest.    

Now a lot of these these myths and concerns have been addressed during recent campaigns such as ITV's 'From the Heart', so people have been able to overcome these ideas and sign up to be organ donors. In reality there is a specialist team and nurse both supporting and informing the family during every single step of the process, starting with making sure the family know and understand fully the condition their loved one is in. Agreement about every step of the process is sought throughout and nothing whatsoever is undertaken if the family feels it is inappropriate for their loved one or doesn't feel right for them. No family would ever be bullied into transplant and no part of the transplant process or a transplant would take place against the families wishes.

So most unfortunately, the BBC has now put these myths about organ donation back into the forefront of people's minds, swaying people to believe it is possible for doctors to break the protocols surrounding both the donor and recipient. I know some will say that it is only a soap opera and a drama and that's what they are all about, but unfortunately people are very easily influenced by them, especially nowadays when it is quite common for soaps to home in on current issues and use them to write their story lines.      

I was pleased to see that NHSBT felt this programme was so far off the truth they needed to issue a statement to clarify matters to potential donors and to people who have already registered. They even reported that some people were taking themselves off the register, because of the programme. Again, people will argue that these people coming off the register must be fickle, but people are genuinely frightened that some of things that were broadcast may happen to them and they begin to wonder if there is truth in them if the BBC, a supposedly world renowned organisation, has broadcast them.

There are strong and watertight protocols surrounding the whole organ donation process so that a situation like this would never happen. Both the donor and recipient have very separate medical teams and our organ donation system is built on complete trust between patients, families and medical staff. 

The BBC responded that they will be following the story up in future episodes - the consultant taking the rap for breaking the transplant protocols. The trouble is many people may not even see this and really it is very damaging to portray every protocol in the book being broken when it would just not be possible to happen in the first place. In this case their storyline is so flawed, it will be difficult to follow it up in any satisfactory manner.

This screening has gone a long way to break down the trust between patients and what actually happens and it was so disappointing to see when you are in the position of waiting for a transplant like me, especially when a soap reaches out to 5 million people and could really help our cause, rather than damaging what we have been trying to achieve. 

I am pleased the NHSBT responded as they did, some people may say it's an over reaction to just a soap story, but when a soap can influence millions and lives are at stake the record needs to be set straight and they have done the right thing. I've also been pleased to see that many complaints have been made to the BBC and that there has been much media attention stirred up, all in the cause of putting the facts straight and none of it good publicity for the makers of Holby City and the BBC.

Of course our expectations of soap operas may be too high and there has been some opposition to the transplant communities' views, one view was printed in a newspaper from a Holby City fan saying, 'it is a soap, soap is all made up, get a life!' Well my answer to that one is, 'I will one day when some very special person enables me to, but thanks to the BBC 'getting a life' could be potentially much longer!' They would feel very different I think if they or any of their family or friends had been affected by organ donation and so would the people involved in the making and broadcasting of this programme.

For our part, we have complained to the BBC and Rob was interviewed by the Welwyn Hatfield Times, here is the report they put in the paper:

I was also interviewed too by our local radio Jack FM and they broadcast their radio interview throughout the day on Wednesday.

Both the Welwyn Hatfield Times and Jack FM were more than too happy to help put the record straight and support us in doing so. Hopefully all publicity is good publicity as they say!

NHSBT statement:

The BBC replied to our complaint yesterday and in a nutshell they did not give any apology, just stated that they did not mean to upset anyone and that they had consulted specialists on the issue. The same bog standard reply went to out to many others in the transplant community. So we will just have to carry on campaigning as we have done and try and undo all the damage they have done.  

It is two years since I stayed in hospital for my full transplant assessment and coming up for nearly two years soon since I was listed for transplant. 
If you want to sign up to the organ donor register click on: