Showing posts with label PAH. Show all posts
Showing posts with label PAH. Show all posts

Friday, 7 October 2016

Happy Retirement

Retirement. It's what many of us eventually strive towards, once we've reached all those career orientated goals and are beginning to think it's time to spend time on ourselves more, do those things that we may have always wanted to do, but can't ever fit in between our busy work lives and other commitments. It's something we may think of when our children have flown the nest and are happily established in their own adult lives and we can relax and let go a little. 



The retirement situation has been a little different for Rob and me, but yes, Rob has now finally retired from work. This is after quite a bit of stopping work and then starting again because of my ill health over the years and that's how things have been a little different. Of course, we tried to make the most of all the time he's taken off work, but during those years our time has been peppered with some extreme health issues. It's been a series of high highs and low lows. A rollercoaster until I received my transplant and transplant still does not come without its ups and downs and trials and tribulations. Having transplanted heart and lungs is a condition that needs to be permanently managed and does usually come with a shortened life expectancy. We are forever aware of this. 



We decided at the last minute to go on a city break to Budapest to celebrate Rob's retirement. We had a few reasons for deciding to do this, obviously his retirement being a big factor and being yet another excuse to celebrate, as life is so precious nowadays and we don't need much excuse. It's a case of when the going is good, try and reach for the stars while you can and while they're there in front of you. 



I don't think Rob was that bothered about travelling abroad though, when I first mentioned that maybe we should do something special to celebrate his retirement. We'd been very fortunate and enjoyed a few lovely weekend breaks and holidays in England this year. We've had so much beautiful weather too, which seemed to be continuing on and on. 



I felt quite strongly we should mark it with something special though, as Rob was forced to quit his career when I fell poorly and became my carer almost overnight following my diagnosis of Pulmonary Arterial Hypertension (PAH).  I literally had to walk out of my career too. There were no 'leaving', 'retirement' or 'moving on' celebrations, the usual get togethers with colleagues for a farewell drink or meal, we merely walked out on our usual routines and daily lives and careered head on into a battle for survival, uncertainties and the unknown. 



On retrospect, we couldn't ever halt or change how things happened and the course of what was to come and I've managed to survive and have improved health so that's all that matters for us both. I've always felt guilty and saddened that Rob had to give up so much to live with an uncertain future and I was pleased he'd been able to return to work since my transplant, albeit being part time and contract work, which suited our situation. I felt it was good for him to have his work, the opportunity to be in the real world and not always being in what has become our other world of hospital and transplantation. 



This time around Rob has decided for himself to retire and I'm delighted he's been able to have this opportunity without it being forced upon him; that he's been able to make his own deliberate choice. It feels like the more normal and planned for retirement I think he always deserved. That's the main reason I felt his retirement should be marked somehow; to make some sort of stance that we got there in the end; that we've both reached retirement together although we went a long way round to achieve it. 



It's been a lovely time for Rob during his last few days at work, enjoying celebrations with his colleagues and being able to leave in what has been a more usual manner. I've felt so pleased, after all the years of him struggling with work on and off because of my needs, that his working life has eventually finished on more of a high and more how he would have planned and dreamed of before I ever fell ill.




Another reason why I wanted to travel abroad again was because of my health. It's been a year since we travelled abroad and I know that I've been really fortunate to be able to do lots of travelling since my transplant and especially visiting several countries abroad during my second year post transplant. My health has been more challenging this year though. 




It started with a difficult bout of CMV virus last November, which resulted in being hospitalised for weeks and over Christmas, being unwell for a few months and has been what seems an ongoing battle to keep it under control, prevent it reactivating and dealing with the resulting havoc it has caused ever since. This manifests itself with stomach and colon inflammation and ulceration and the associated pain and severe fatigue. I've worked hard to try and overcome this with diet, exercise and pacing myself more. My transplant team have also been more than helpful and are still monitoring me very closely and regularly, adjusting my medications so things are kept in good control. I'm still having monthly CMV tests and clinics more regularly than I had been, which I'm happy about, as it gives me the reassurances I need.



I'd had to cancel quite a lot of things we'd been looking forward to during some of these difficult periods, so I could recharge myself, help myself better and get myself back to the good health I'd been enjoying since my transplant. Regaining my health was the most important thing, so it didn't matter so much to have to cancel in the scheme of things. It's always good to plan things to look forward to, but if things don't go as planned, it really doesn't matter. We accept this happening as part of our lives now. Attempting to go abroad again though, felt like it would be a big step. A step that would be a small victory against all this year's health problems and show myself I can overcome them and still make the most of everything life offers when the going is good. 



We love city breaks and seeing new cultures and places and this time we chose Budapest -  we've drawn ourselves up another list of cities and places we'd like to see and this was a favourite. We managed to have a wonderful time, exploring yet another new place, seeing new sights, enjoying more beautiful sunshine, having fun and celebrating retirement and life. It was another chance to live life to its fullest before we reached the end of my third year post transplant and it always goes without saying, all thanks to my donor.



Of course, we don't take this period of 'retirement' for granted. Reaching this stage in life is an enormous bonus, it's something many people don't have the chance to have, so I see growing a little older as a complete priviledge not something we should moan about. I often laugh how the government keep changing the state pension ages - I know it's not a laughing matter really. State pensions are becoming ridiculously out of reach for the healthiest of people. Everyone worries about how they will manage, but I know if I'm ever lucky enough to reach the sixty seven years it is currently, then it will be a nice problem for me to have. In the meantime, Rob and I plan to keep ourselves busy with things that mean the most to us and keep on enjoying life to the full. 








Monday, 25 January 2016

Breakthrough for Pulmonary Hypertension Treatment

Last year there was a significant breakthrough in the genetic research into Pulmonary Arterial Hypertension (PAH) being led by Professor Morrell at the Cambridge Biomedical Research Centre. Professor Morrell is both British Heart Foundation Professor of Cardiopulmonary Medicine and Research Director, National Pulmonary Hypertension Service at Papworth Hospital.



A protein was discovered that targets the effects of and reverses the damage caused by a faulty gene BMPR-11 - this faulty gene is the main identified gene for causing inherited Pulmonary Arterial Hypertension. I have put the links for more detailed information on the research project and on the breakthrough discovered last year below.


The Genetic Research Project


BHF Press Release 2015


This week it was announced that Morphogen-IX, a new Cambridge based biotech, has gained seed funding to develop a new treatment for PAH, following on from the findings last year. This drug will directly target the disease and has the potential to provide the first disease modifying approach to the serious life limiting effects of PAH. The current therapies for PH and PAH help to alleviate symptoms, but as yet there are no medical options to modify the course or outcome of the disease. The company will be solely dedicated to developing a therapy that will tackle the disease rather than just manage its symptoms and they have received grants of 1.5 million to develop the drug. The link below gives more information on Morphogen-IX


Morphogen-IX


This is marvellous news for the Pulmonary Hypertension community - we always live in hope that a breakthrough will happen and that eventually a cure will be found. When a person is diagnosed with PH it is both shocking and life changing. Whatever stage of PH you are in, you know that you face a long journey of hospitals, doctors, tests and treatments. The treatments for PH vary depending on the type of PH and the stage of severity that the PH is in - they vary from oral medications to complex inhaled medications and intravenous medications, which are very daunting to face. There are some surgical alternatives for some patients such as Pulmonary Endarterectomy, Lung Transplantation or Heart and Lung transplantation - high risk surgeries that again can feel terrifying to face. For some there are no surgical alternatives, just therapies that manage the symptoms, although the disease progresses. There is also the knowledge that PH can be a life limiting disease and that you face a future that is uncertain and with some disability.



To know that there is research and development of a drug which may reverse the effects of this devastating disease is wonderful news. These new developments bring so much hope to so many. Hope that they may not have the future they are presently having to face - one of complex drugs, surgeries and one that is potentially life limiting - and hope for a future that could change for the better.

The announcements that this new drug is going to be developed demonstrates great progress has been made with the genetic research. The new drug will then need to be put through the various clinical trials. We wait in hope for more announcements when developments reach this stage. In the meantime there are other drug trials in progress, where it is hoped that symptoms in patients may be helped and controlled better and again, even modified. One is a trial for Tocilizumab, which is currently used for arthritis and it is hoped it may modify PAH too. Again more hope. A link for more details on this is below.

New Clinical Drug Trial

On a personal note, although I don't have PAH any more after undergoing a heart and double lung transplant as an end stage treatment, I'm delighted there are these new developments to help PH sufferers and that there is some hope for the future. I have a keen interest in the genetics research as I underwent genetics testing and took part in this genetics research at the very beginning of the project. I still take part in the research and last year gave more DNA as the project moves on forward and I was given information about the possibility of second generation testing, so it is still of great interest to me. It was an honour to take part in the publicity process about the genetic research and the breakthroughs that are happening over this last week.


















My story and involvement with genetic

This week I had the opportunity to take part in an interview with BBC Look East and on a fun note the pictures show my new puppy, Ted making his TV debut in his first ambassador role for PH! Visit my author Facebook page and scroll down to find the whole interview with BBC Look East, Professor Morrell, the research team, Ted and me.

Life is for the Living Facebook page


The 1.5 million funding raised for the new drug will now build on the work of the project funded by donations from the British Heart Foundation. The funding from BHF is vital in developing this research. Their link is below.

British Heart Foundation

We live in hope!

Saturday, 28 February 2015

March, a Dechox and Genetic Research








When I arrived at clinic the other week I was greeted at reception then asked if I could sit and wait instead of going off for all my usual rounds of tests. I was advised that someone was going to chat to me about my blood tests. Feeling a little confused I sat and waited and wondered what it all might be about. At first I was worried something was wrong with my last blood tests I'd had at the doctor's that had now come through, then I decided they must have me muddled up with someone else and when 'the person' came to chat to me they would realise they'd got the wrong patient!




Eventually it turned out the PH Research Team wanted a chat about the Genetic Research they are currently undertaking, which I had taken part in at the outset a few years ago. Although I don't have PH, my DNA is still the same constitution so they can still use it and I am able to continue in the research project. They asked if they could take some further blood samples and obviously this could be done alongside the blood samples that were being taken for transplant clinic, hence trying to see me before I wandered off and had my tests done! 



The study is planned to include relatives if it determines the need in the future. It includes looking for the presence of known mutations in genes that can cause PAH - which in my case has already been done and fortunately I'm clear - and it also includes looking for other new mutations that may cause it and this is where my DNA will still be useful. PAH patients taking part will go through other regular testing, which will run alongside their usual clinic tests, but as I have been transplanted, I will only have blood tests. There may be a time in the future when my daughters will take part, but only if they wish to. The studies will involve part or whole genome sequencing. 


No more cakes until April!
The study is being supported and funded by the British Heart Foundation and this brings me on to March and the Dechox challenge. The Dechox Challenge is being run by the British Heart Foundation so that they can raise funds to enable them to continue supporting vital research such as the 'National Cohort study of Idiopathic and Heritable Pulmonary Arterial Hypertension', which is the study I'm taking part in. They also support research studies into various aspects of heart transplantation too, so as their research could potentially make a massive impact on both mine and my children's future I've decided to take part in the challenge and help to raise some much needed funds.


Last chocolate was 5th February!

The challenge launches on the first of March and I registered to do it a couple of weeks ago just after my clinic. I haven't eaten chocolate since, so my Dechox will go on for over six weeks if I manage to stay the course. I am also going to give up sweets, biscuits, cakes and puddings just for good measure too! If you can help support a good cause, please click on the 'Dechox' link, otherwise wish me luck as the chocolate and cake cupboard is constantly calling and if you know me don't forget how I love Cadbury's Creme Eggs at this time of year!


A little group of us - all involved with heart transplantation - have joined forces so we can motivate each other along the way - click below to see all our stories:


Thanks to everyone who has sponsored us already and please help us raise these much needed funds, any amount, however tiny is welcome!


This week's photos (first two) were taken on a beautiful winter walk around Elterwater in the Lake District. 






Thursday, 29 January 2015

Pulmonary Hypertension Survey





Last week I was contacted by PIP Health - a patient healthcare company -  and asked if I would take part in an interview that was being undertaken by Atlantis Healthcare to help understand more about PAH patients' unmet needs and challenges. It is hoped a series of interviews with PAH patients will help to develop methods that may improve and support patient care.

Interviews were being undertaken over a couple of days and I had my interview which lasted for around forty five minutes or so and was able to highlight a few concerns that I had had when I was a PAH patient. I discussed how one of the hardest parts was the lack of understanding within the medical profession and how this impacted on visits locally to the GP surgery, A&E and admissions to local hospitals. 



PH is very complex with varied specialist treatments according to the classification and types of PH. I found no one outside my specialist centre who had even heard of IPAH or my specialist intravenous drug Flolan. I always felt the doctors at my GP surgery should have had better understanding of my drug should I ever have been in an emergency situation, which often threatened. I felt just some basic things should be marked on my file: 1) that the IV drug needed to run twenty four seven at all times 2) that it should never be disconnected and used to administer other drugs 3) what the drug was for and what it did. The GPs involved in my care were only really concerned with my general drugs. Obviously I did inform a doctor at my surgery, but it is a surgery with several doctors including locums and you are not allocated any specific doctor.



It was even harder on admission to another hospital other than my PH centre, which happened on several occasions and my husband had to come in and make the drug up instead of the professionals. I had to be very possessive about my line, pump and drug, as I couldn't really trust anyone would have a clue what they were doing with it. Most would remark they hadn't seen anything like it before or heard of Flolan. I also had doctors trying to take me off various oral medications -  because they didn't understand the condition properly -  I knew that would put me in an even more life threatening state. Every single time I had to visit a GP or hospital I had to stand firm in my own knowledge of my condition and medication. It leaves you feeling completely vulnerable and unsupported however hard you try. You live in dread of an emergency happening - and if one happens -  I always found it was on a weekend, evening or Bank holiday, which exacerbates that vulnerable position as locum doctors are normally on duty and A&Es are pushed to the limits. 



We also discussed the need for wellbeing, emotional and financial support locally, but it seems to be a bit hit and miss varying on where you live or which GP surgery you attend. Our centres are usually excellent and can provide us with some of this, but I recall coming out of hospital with a Hickman Line and on my intravenous drug and again feeling very vulnerable, unwell and trying to find my feet on my own.



I had my eyes opened following my transplant, a letter from my physio to the local hospital triggered things into action locally. A heart failure nurse came to the house to assess the help I needed, although it was very nice to be offered help, support and advice and it was welcomed, it also made me really cross. Where was this help when I really needed it? It's no good when I have a brand new heart that beats strongly - what about when I actually had to live with heart failure? Advice on physio, diet to get my strength back and aids around the house would have been most welcome. When I first arrived back home after a long hospital stay with deteriorating PH and poor prognosis, there was no offer of any help or home visits from anyone and we were left floundering on our own locally. We just muddled through and used disabled websites to buy equipment such as bathing aids and wheelchairs.


Those were the biggest 'gaps' that I found in my care, mainly this lack of  'joined up' thing with my PH centre and GP. I did stress, however, that my centre had always offered very strong support and the PH nurses used to phone me regularly and I could phone them whatever time of day. I had a twenty four hour emergency card with numbers for the hospital, however on some occasions staff in A & E had refused to use it. I was always grateful for the support given by my centre, but they weren't the ones near home should I have an emergency. 



The psychologists from Atlantis Healthcare put forward several ideas and suggestions to help improve support for patients, which I felt were appropriate and very good ideas. One was to create an online 'symptom tracker', where patients can record their daily symptoms including drug side effects. This tracker would also be available at the patients PH centre, so medics there can track how patients are doing. I thought this was a great idea as it is our symptoms that can often indicate when things are going wrong or deteriorating. I thought this could be even better if it could be extended to our GP surgery too: it would help put that joint care into place. 

Drug side effects, which I suffered quite badly from, would perhaps be taken more seriously too. We know we have to be on the drugs as we probably wouldn't survive, but sometimes side effects can be as debilitating as the PH. 


We also discussed having a facility for recording all the medications patients are prescribed on some online system too. This could be updated and monitored by patients and their centres. This would give historical and current information at a glance and perhaps have necessary warnings about interactions and safety measures included - eg. IV meds aren't to be disconnected. I thought this was another good idea. 

All in all I felt it was a very good discussion and I felt the concerns about the support PH patients need is being taken very seriously. Hopefully there may be some developments from this in the near future. 

You find more information about  PIP Health on: 








This week's photos were taken at Wrest Park, Bedfordshire, an English Heritage site. 

©KAGraham