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.
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.