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: 

http://www.piphealth.com

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

©KAGraham

In the News

There has been quite a bit in the news this week about Organ Donation as it was announced the government are to add opportinities to enable people sign up to the Organ Donor Register on various government forms as well as driving licences - passports, voting registration forms and oyster cards.

I think this is a great idea and a good way forward to remind people about Organ Donation and help trigger them to sign up there and then, rather than them thinking it's a good idea, but I'll do it later. Often people just then put it off, don't think about it again and they don't bother to get round to signing up even though they are quite happily in favour of it.

http://www.skynewsradio.co.uk/story/1409949/online-campaign-to-promote-organ-donation

I believe systems like this really do work. This last week Rob and I visited John Henry Newman School in Stevenage to do a talk to the Year Thirteen groups on Organ Donation. Rob asked them at the outset how many of them were on the register and about 40% of them were already signed up. Many of them explained it was because they had done so when they applied for their driving licences, which just highlights that this type of system really does work.

We focussed our talk about what it is like to wait, and that three people die a day while they wait for a transplant. I told them my story of waiting for two years and what it felt like and then we went on to talk about our dear friend Stacie, who has now been waiting for her new heart and lungs for nearly three years. Stacie recorded a video clip, which really helped. We thought they would be able to identify the situation better with a person similar in age. Stacie had only posted on her facebook page that week how she had been waiting for a 1000 days. 3000 people will have lost their lives during the time Stacie has waited, which really brings it home how tragic a situation it is that only a third of us are yet signed up to the organ donor register.

Because many of them had already signed up to the register, we had a discussion on the importance of chatting to your family about your Organ Donation wishes and the significant impact this has on helping families making those hard decisions when they are faced with such a harrowing situations.

At the end of the session, we had quite a good response and quite a few pupils came and signed up there and then. We will just be pleased if our talk has inspired them to go home and discuss their Organ Donation wishes with their families.

Also in the news was the story about the parents of a new born baby, unfortunately their baby didn't survive, but the brave parents agreed to donate kidney and liver cells,which saved the lives of two other people. Another very significant step has been made to help increase the number of Organ Donors and save lives.

http://news.sky.com/story/1411172/uks-first-organ-transplant-from-newborn-baby

A Good Start to January

It's been one of those quiet weeks getting back to some normality after the Christmas and New Year festivities. Rob and Rose went back to work - I remember how hard that used to be after Christmas, with the cold and dark mornings so don't envy anyone who had to do that this week.

For me it's been trying to get back to my exercise regime and back to healthy eating after all the Christmas excesses. I think that's probably the same for many too. I am prone to gaining weight now with being on steroids, so I'm trying hard to stay at least where I am now and tone myself up a little in the process. I also want to keep my new heart and lungs healthy too. I've been getting back into the swing of doing some gentle yoga and making sure I'm doing plenty of walking and keeping active.

I've also been really disciplined and have spent quite a bit of time on my writing: getting more work done on my book and organising myself with ideas for my next writing assignment. On the writing front I was delighted to get another article I've written published in 'My Weekly' magazine. It is about the morning I got my transplant call and explains about Pulmonary Hypertension, the intravenous medication I was on and its symptoms and then about how my transplant has dramatically changed things. There is also a part on the shortage of organ donors and they have published the links to NHSBT, which is brilliant. Hopefully it may inspire readers to sign up to the organ donor register. 

My Weekly - 6/1/15 Issue

It was also back to medical things too. My problems getting the correct dosage for one of my immunosuppressants, Myfenax, is still going on. It's been over six months of chopping and changing every few weeks now, so it's been back to more blood tests and liasing with the Transplant Team.

Weekly drug diet!

On the Friday evening just before we flew out to New York, the Transplant nurse phoned to say my white blood cell count had gone too high - I had been having regular checks because it had been going too low for months. If they are too high, then it causes rejection, so they wanted to increase my dose of Myfenax this time. The problem was I hadn't got the correct tablets to make the new dose and it was Friday evening and I was going away on the Sunday, so it got a bit stressful.

In the end, after a few phone calls the team decided and reassured me it could wait until we got back and that I shouldn't worry - easier said than done! I was also worried because Rob had had a really bad cold that he hadn't quite got over and as we were going to be so far away from home I was worried I may catch that and become unwell too. As we set off for the airport I had a lot of pain in my eye and when I looked, it had filled with blood. So back home again we went, to check it out, and establishing it was likely to be just a burst blood vessel, we set off once more. I think at that point I would have felt relieved if we'd got to the airport and been told, 'Sorry, but you can't go!' All went well after that though, once I got myself anxiously on the plane, it was a trip of a lifetime and another big milestone, so I'm pleased I didn't buckle out.

Following all that, I started my new dose of Myfenax a few days before Christmas, a prescription from the Transplant Team was ready and waiting in the post as soon as I arrived home thankfully, so we are still monitoring very carefully how things are going. With all the regular checks, I have managed to keep well, while all the adjustments have been going on and that is the main thing. 

I'm not very good at giving blood - third time lucky this week!

I was back at the local hospital for my physio check too and this went well, so I have been discharged and just need to keep my daily exercises up for now.

Lastly, we are getting a grip on the wedding planning front - Sarah and Oli's wedding is in June, so only four and a half months to go - this is all really exciting and getting nearer now! 

It has been another very positive week and a great start to January. 

(This week's pictures are views from Brockhole Visitor Centre in Cumbria - taken during the Christmas break. For more on Brockhole and its gardens and grounds visit my gardening blog below.) 

New Life, New Opportunities, New Year!

The last year was the most amazing year when I started a new life with many new experience and opportunities. It started with Sarah and Oli's engagement and a flurry of searching for wedding venues in January. January also saw me eventually coming off all the inhaler medications I had been on since my transplant - three of them three times a day - and coming off heart failure drugs at long last now my new heart had become stable after my transplant. Late January was the last time I had to have a bronchoscopy after having to have them nearly every week for months. Without all that came new freedom and we visited London to meet friends. We were pinching ourselves and I couldn't believe how much better I felt, how much I could suddenly manage and how far I could walk.

The year continued well and I'd thought I couldn't get much better, but I just kept on getting better and better. Rob and I did quite a few national and local media interviews over the course of the year to help raise the profile of transplant, organ donation and pulmonary hypertension including ones for Hertfordshire Life, Heart Matters (BHF magazine) and The Weekly News. We featured several times throughout the year in The Hertfordshire Mercury, The local Comet newspaper and on local radio Bob FM Home Counties. We also worked with NHSBT during National transplant Week and for their Christmas campaign and were featured on BBC Three Counties Radio, where we were given the opportunity to visit the radio studios in Luton. Speaking about organ donation and pulmonary hypertension is our way of trying to give something back for all the good fortune we have been given.

We seemed to get one wonderful opportunity after another and they just kept on coming. I enrolled on a writing course at the beginning of the year, so I could start writing my own articles for publication and one of my articles on my transplant was published in Cumbria magazine. I won a competition run by Woman and Home Magazine for writing a small article on 'Seizing the Day' and had a full style, makeover, photoshoot and interview published in the September issue of their magazine, plus a thousand pounds prize money for Papworth Hospital Charity. In October I was invited to speak at one of their reader events about my transplant and making the most of life. They were all interesting and new experiences to be part of and at the same time raise more awareness of organ donation and pulmonary hypertension to new audiences.

In July we were asked to take part in a launch of the government's 100,000 Genomes Project and were featured on all the main TV channels as well as getting to visit the Sanger Institute in Cambridge to see how the genetic testing machines work. We also had the wonderful and exciting opportunity to visit Downing St when the project was officially launched and this led to more local press interviews. At Downing St, I was pinching myself because I was able to walk at a fast pace up those famous stairs. It never ceases to amaze me what having my transplant has given me.

I had many small personal goals and dreams to work towards as I recovered including going for proper long walks once more. I set myself a challenge to walk around Tarn Hows in the Lake District in summer, but managed it in March. I wanted to get back in my kayak once more and managed this in June when I kayaked across Coniston Water once again, much to my delight. I travelled abroad once more, after nearly five years and went to Paris by Eurostar. After falling ill I had been deemed too unfit to fly and was unable to anyway being on the transplant list. I made my first flight abroad in October, just a short one to Amsterdam and this gave me confidence to make another dream come true and make a long haul flight to New York in December. It was wonderful to visit some new places.

To a lot of people these are just normal and everyday occurrences, but for me they are massive steps as they are all things I thought I would never be able to do ever again. Achieving these things have been a bonus and the icing on the cake, but the thing that still really amazes me is the fact that I can walk around normally again without using a wheelchair or measuring how far I have walked so I can make sure I can manage the walk back again. It is still special to bath and shower normally without worrying about medical equipment getting wet and it is amazing to run up steps. I run up stairs every day now just because I can! These are the things that healthy people take for granted, having had these taken away from me over the years and then suddenly getting them back is something that still astounds me even a year on.

Standing at the top of the Empire State Building at night time watching the lights of New York twinkling and fireworks going off over the Hudson River was a spectacular moment and more than a dream come true at the end of this year, but it still dazes me and fills me with awe when I walk around Tescos pushing a trolley and filling it with shopping. Extraordinary things and just ordinary things are equally amazing when you have a new chance of life. I had lost my independence and to have that back is a miracle. 

I'm not quite sure how 2015 is ever going to measure up to last year, but I have lots of plans and dreams already, so I'm hoping for it to be even better if that's possible. It is a big year for our family as we have Rose's 21st, Sarah and Oliver's wedding and our 25th wedding anniversary. These are all massive milestones for me to aim for, moments I'm looking forward to that I didn't dare ever hope to have. 

I'm also well underway with writing my book and one of my New Year resolutions is to get my first draft completed during the first part of this year. I'm also continuing with my writing course and hope to have more articles published during this New Year.

I'm not going for all the slimming and exercise resolutions, but after all the festivities I just plan to get back to eating sensibly and healthily and making sure I keep up my walking, yoga and exercise bike. I have got some new yoga DVDs for Christmas and a yoga mat, so I'm planning to step up the yoga a little. 

Of course, it goes without saying that our family will continue to raise awareness of both organ donation and pulmonary hypertension and in this respect the New Year has started well with an article in the Japanese Times, a Japanese national newspaper, about the UK's Genomics programme. The article features an interview I did with Kyodo news on genetic testing. 

http://www.japantimes.co.jp/news/2015/01/01/world/science-health-world/major-genomics-program-underway/#.VKVD9ktMI9e

I know that time has gone on now since my transplant and it is hard to gauge where I would be without it. I cannot thank my donor enough for the wonderful experiences and opportunities that I have had since being given my second chance at life. Without my donor none of them would have been possible including all the new hopes and dreams I have for this new year.

I wish everyone a very happy and healthy New Year and hope that you have many plans and dreams that you can make come true. Here's hoping it is going to be a good one with more new opportunities and experiences.