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!

Eighteen Months Post Transplant

I'm now officially eighteen months post transplant! I don't know where the time has gone, but it just seems to go faster and faster. I haven't posted for a good few weeks as life is getting busier and busier now things are much more normal, but here's just a quick catch up. 

On the health front things are good. I've been back to clinic for more blood tests and they were fine this time, so no more clinic until May if things keep going well. I'm waiting to see my GP next to sort out the blood pressure monitor that the Transplant Team want me to have, so everything is going as well as can be.

Socially, we've been busy getting out and about: we've been to London a couple of times meeting up with friends and we got the wonderful opportunity to go to a reception at St James's Palace, hence this week's pictures taken in London around St James's Park and the Mall. We've been up to Papworth to the PH Matters Support Group, where Rob and I did a talk about my transplant journey and we travelled to both Hemel Hempstead and Birmingham this week to see more friends. Life is different than before my illness, but we've got back to being just as busy somehow, which is fantastic and something I never could have believed. 

Opportunities keep on coming to raise more awareness about the issues around Organ Donation. Following my day at the House of Commons with Transplant 2020 earIier this month I was contacted by the local press and managed to get another article in the Stevenage Comet with an interview from me and one from my local MP, Steven McPartland. 

Stevenage Comet Article

I've also just completed my Dechox for the British Heart Foundation and managed to raise £270 passing my target of £250! Thanks to everyone who sponsored me. British Heart Foundation are helping to fund the genetic research for Pulmonary Hypertension that I'm taking part in, so it's a cause that is close to my heart. I'm looking forward to Easter now and maybe a few chocolate treats! 

Dechox Challenge

I'd just like to wish everyone a very happy Easter, I hope you get chance for some peaceful time with your family and friends and enjoy the break. 

And to finish, never a day goes by without me thinking about the person that gave me the chance to thoroughly enjoy this last eighteen months and all this precious time with my family and friends. They are always in mine and my family's thoughts. 

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!

Kath's DECHOX

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:

Team Chocoholics Anon

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. 

February Clinic

On Wednesday I went back to clinic for my three month review - well it was supposed to have been three months since my last visit back in November, but in reality I was back just a week after and have been having my white cell blood count monitored with regular blood tests and adjustments in medication.

The white blood cell thing has been going on since last May when it became too low. With adjustments in my medication I was getting back to normal. Then I was taken off a drug - valganciclovir - which I'd been on for my first year after transplant and it went all awry and went too low again.

When your white cells get too low there is nothing to fight infection with so it can be dangerous, therefore I had more adjustments in my medication. Next thing, just before Christmas they got too high - when they are too high, there is a chance the white cells may start attacking your new organs as they react as though your new organs are an infection in your body, so more adjustments. 

I was thrilled at last week's clinic as they managed to get my blood tests back before I left and the consultant checked my white cell count and at long last it was just where it should be, so that meant no changes in my medication.

We've have also been monitoring my blood pressure closely as the majority of patients with transplanted hearts go on to develop high blood pressure, which is obviously a risk factor for heart disease and strokes. It is usually inevitable that patients require medication for high blood pressure. My blood pressure is gradually getting higher, so I have to arrange to have a twenty four hour monitor with my GP next so they can look what it is doing and decide whether it is time to start new medication. 

My consultant told me that 100% of Papworth heart transplant patients are on medication for high blood pressure after a year, so seeing I am 16 months post transplant now, I've done really well to avoid it so far. I've always known that this would probably happen so I am not surprised, I'm more surprised that I've got this far without needing it! 

We had an enjoyable and sociable clinic because we got to meet some friends old and new at the clinic and then when we went across to the canteen we ended up bumping into one of our transplant friends Jacqui, who just happens to work at Papworth. We'd just finished speaking with Jacqui when we spotted our 'PH' friends, Kathy and Michelle - so it was an enjoyable morning. That's what I love about Papworth! 

I was delighted to be given three months until next clinic this time with just the blood pressure test to organise in the meantime. This was short lived though and when my other blood tests were checked, the team phoned to say I need to have more tests because of my Tacro levels and kidney function, so I'm now back in a month's time. 

Luckily, I feel really well thanks to all the careful monitoring of my transplant team and hopefully it is just a question of maintaining the correct balance of the drug combination I need.

I was in the local news again the other week after a reporter phoned from the Hertfordshire Mercury following Grant Shapps' anouncements that the government is going to put organ donation on more forms such as passport applications and oyster cards. They had read an earlier blog of mine and picked up on it because Grant Shapps is a local MP in Welwyn Garden City, Hertfordshire and it was another great opportunity to get people thinking about organ donation.

We have had Valentine's Day too. Rob and I didn't really celebrate Valentine's Day in my pre illness days, as it is a bit hyped up and paying for flowers and cards can be a bit of a rip off. Now we use any excuse to celebrate and I was lucky, Rob bought me a new Pandora charm - the latest one in support of the British Heart Foundation -  so at least some of the cost went to charity and one we fully support at that.

Talking of the BHF - I'm taking part in their DeChox during March to help raise funds for them. My link for sponsorship is below.

Kath's DECHOX

Just returning to Valentine's Day, I cannot let this week go by without mentioning my dear friend Stacie, who has worked tirelessly - although she is poorly - to do everything she can to promote organ donation. She was on ITV's Good Morning Britain, on her local radio and in her local papers. You can check out Stacie's blog with all the links below. Our friend John Fisher supported and enabled Stacie to do this. John runs a transplant charity, 'To Transplant and Beyond', which provides support for anyone touched by transplant and they funded the Valentine's campaign she fronted. Hopefully it has got many people motivated to sign the organ do not register. 

Life is Worth the Fight

To transplant and Beyond

This week's photos are taken on or near London's Westminster Bridge. ©Kathryn Graham

Show Love Today

My friend Terry, who has had a heart transplant, has made a track called 'Show Love Today' with his band, The Street and Mark Moraghan. He has done it to help raise funds for the British Heart Foundation and to raise awareness of organ donation. He has also managed to get the support of all the Coronation Street cast in the process too!

Coronation St is currently running a story featuring Les Dennis, who plays Michael - Gail's boyfriend. Michael has been struck down with a genetic heart condition. The British Heart Foundation are currently funding much work on genetic causes of heart disease. They gave funding to help the genetic research currently being undertaken by Professor Morrell, at the Cambridge Centre of Research Excellence, on the genetics of Pulmonary Hypertension, so the BHF's research is something close to my heart.

To watch the video and listen to the track click on the link below: it features Terry, myself and some of my transplant friends - some of those we have lost while they waited on the list; some of us who have been lucky enough to receive a transplant and have our lives changed dramatically and those who are still waiting. 

https://www.youtube.com/watch?v=qvR_hIgjWIo&feature=youtu.be

We chose this picture for my picture in the video, as it just about sums up what transplant can do to transform someone's life - from using a wheelchair to kayaking once more, something I thought I would never do again! This moment was one of the highlights of my year post transplant!

There are still 3 people a day dying while they wait for a transplant, so come on, 'Show Love Today' and sign up to the organ donor register. Click below to order the track and support BHF: 

https://itunes.apple.com/gb/album/show-love-today-single/id945497551

The link to sign up to the register is listed below. 

Genetics may be the Heart of the Matter

It's May already and although the first of May has started off wet and cool it can often be a glorious month, so I am really looking forward to it. The forecast for the first bank holiday of the month and this weekend is looking good. I'm now seven months post transplant and still doing incredibly well.

I had a bit of a blip with my lung function levels during the Easter period, which went a bit haywire and dropped a lot more than they should, but as everything else showed up totally fine I just had to keep a careful watch on things to make sure they didn't continue declining. It was put down to my reduction in steroids, which the team had reduced on my last visit. I ended up missing the PH conference because of it, but decided it safest to err on the side of caution, as I wasn't sure if I was coming down with something or not and my immune system is already very low. I didn't want to risk the chance of infection as the conference is very busy with lots of people, but I was disappointed to miss my friends. My lung function readings are now much better again and back to where they were, so I think I did the right thing.

This month there is a feature on the genetics of Pulmonary Arterial Hypertension in the British Heart Foundation magazine, 'Heart Matters' and Professor Morrell, who is heading the research, explains all about it. I was also interviewed for this feature, having had Idiopathic Pulmonary Arterial Hypertension before my transplant and having been tested to see if I carry the known genes that can be an indicator that it may be in the family. The research work is a continuing process, but there is hope that there may be a breakthrough, which will enable the development of future drugs to help all PH sufferers. The British Heart Foundation have given generous funding to enable this research. Below are the links to the articles.

http://www.bhf.org.uk/heart-matters-online/may-june-2014/research/pah/kath-graham.aspx

http://www.bhf.org.uk/heart-matters-online/may-june-2014/research/pah.aspx

A cure or breakthrough cannot come too soon. Some of my friends are really suffering with this disease. Several are waiting for transplants and are having to face long waits because of the shortage of organ donors and then they will have to go through the process of the transplant and the ongoing care and drugs that are required afterwards. If a cure could be found or better drugs to control the condition then patients wouldn't have to face these options. 

Some of my friends are coping with other diseases, such as Lupus, which has also caused their PH. Others have PH through having bloods clots on their lungs and although they have had their PH improved through having the Pulmonary Endarterectomy operation to remove the clots, some of the clots are too distal for removal, so they still have to cope with PH. Many PH patients are not eligible to have a transplant, so the biggest hope is that a cure can be found or a breakthrough that can help the treatment of PH more efficiently. 

We all live in hope for this day and for a breakthrough to come soon. 

Moving House, Moving On

It's been another busy, busy week with lots going on.

Waiting to unload!

Monday started with Sarah and Oli getting the keys to their new house. They started the process of buying the house just before I had my transplant, so I was hopeful I would see them get the keys and see them settled in their new house, although I knew I wouldn't be able to help them in the condition I was in with my pulmonary hypertension.

And we are in!

I'd forgotten how stressful buying a house is, how you have to keep chasing solicitors constantly and keeping up to date with what stage things are at and then chasing again and again. It always feels like everyone is sitting about doing nothing until you keep pushing and then another little thing gets done, small steps at a time. I'm sure it could all be a lot quicker and efficient, but nothing seems to change over the years. So it hasn't surprised me that it's taken four months to complete the sale, with the little blip of my transplant and Chistmas thrown into the mix as well!

Most important job - getting the internet sorted!

Because of my transplant, what has been really amazing is that I was actually able to help out with the house move. Of course I couldn't do any of the lifting boxes and heavy furniture, but I was able to help unpack boxes and put things away. So I was left in charge of sorting out the new kitchen and making the tea. I had plenty of energy for both, not like old times when just getting to the new house would have been an effort, let alone trying to do much to help.

That was a lot of boxes - tea break needed!

It felt another big milestone in my journey since being unwell - to see my daughter buy her first home with Oli and help them move in. It is something that as a mother I have always looked forward to doing. We are planning a shopping trip or two next to buy some nice bits and pieces for the house and I'm looking forward to helping them transform the garden in spring. There seems so much to look forward to now, especially as I'm able to do much more than I could.

Kitchen looking good!

Tuesday was time for the transplant clinic again, both for blood tests to check my Tacro levels and to collect more drugs as my Prograf drug dose has been increased. It was a quick visit for once and we had time to visit Sarah and Oli on the way back - they are nearer to Papworth than us now - exactly 38 minutes to be precise! That may come in handy!

At last - all unpacked!

Next up was a visit to the dentist, my first one since my transplant. I've been going to my dentist for 22 years now, so he was interested to know all about my transplant. He needs to know all about the medication I am on and keep a very watchful eye on my teeth. My medication can cause problems with my teeth and gums and it is one of the stipulations of going on the transplant list that you visit the dentist for check ups regularly and continue to do so. I was pleased that all was fine, especially in the light of my operation and being on a ventilator and having various tubes and cameras in my mouth,  throat and windpipe, which can also cause damage to your teeth - in the process. I also had to rely on other people cleaning my teeth for me during those first weeks after the operation and no matter how hard someone tries it isn't the same as doing it yourself, so I was a bit bothered my teeth had taken a hammering. 

Smile please!

This morning it was time for a photo shoot and a photographer working for the British Heart Foundation came to visit to get some photographs of me for their article on Pulmonary Hypertension. I was interviewed for this article earlier this month and it is going to be featured in the May edition of 'Heart Matters'. Now I was a bit nervous about this one as I got a whole itinerary from the Art Director about what to wear, what they want to achieve and how it may take a few hours - all the other photographers for the newspapers and radio that have visited usually just take a few quick shots and it's all done and dusted in a few minutes. So I found myself worrying about what I should wear and chopping and changing my mind every five minutes about it. A typical woman, I suppose! It turned out to be lots of fun though and hopefully there might be a few good pictures! 

Oops think we need another!

You can sign up to the 'Heart Matters' magazine on line, it is a useful and interesting magazine both for people with heart problems who want to learn more and for those who just want to be healthy and look after their heart health. Click on the link for more information and to sign up to get the magazine - it is free. Look out for the May edition if you do sign up!

www.bhf.org.uk/heart/how-we-help/information/heartmatters.aspx

Another smile...that's better!

January Bits and Pieces!

This has been a busy week with all sorts of bits and pieces going on, wedding planning being one of the main activities amongst them!

Firstly, it was back to reality and a visit to the GP to finalise which drugs my surgery will prescribe and which they won't and to also update the doctor on my changes in medication. My surgery is only able to prescribe the 'cheap' drugs and not the more expensive ones. These will come from Papworth. It is all a bit of a rigmorole, as at the end of the day it will be the same Primary Care Trust that will pay for them anyway. 

It seems to be a big lottery over drugs and who will pay for them: I've met other transplant patients whose doctors are willing to prescribe all the drugs and then others like me, who have been refused the expensive ones. According to the newspapers it seems to be a similar situation when it comes to cancer drugs and there are even reports of drugs being refused because they are seen as too expensive, although they have been approved by Nice. At least I am going to get my drugs one way or another, so I can't complain, but it seems wrong to me that there isn't just one straight forward system for all. 

We celebrated an early 'Burn's night' on Tuesday evening with haggis, tatties and neeps - we always celebrate 'Burn's night' - Rob was born in England, but his late parents were Scottish, so we like to carry on traditions! It's a quick and tasty meal, proper winter comfort food! And all washed down with a wee dram of whisky if you fancy one! 

We were recently interviewed for an article that is going to be featured in Hertfordshire Life, the article is going to be about living in Knebworth, but they had seen our transplant story in the local news and wanted to include it too, together with our views on living in Knebworth. We are always happy to try and spread the word about organ donation, so on Wednesday a photographer visited to get some pictures to go with the article. We are beginning to get used to this!  The feature will be in the March issue, which will be on sale in February. 

We have been exploring a few wedding venues this week too, for Sarah and Oli's wedding, just shopping around really to see what there is available and when and getting an idea of costs. This is all very exciting for me, and even more special in the light of my transplant. They hope to get married in spring next year and now I've had my transplant, I'm looking forward to enjoying every minute of it -  planning, preparations and all! It's a good excuse to get out and about and have a few afternoon teas - as Oli said on Saturday, after seeing a venue that didn't quite meet our needs, 'Well at least we've had a good afternoon together and enjoyed a nice afternoon tea!' It's going to be a lot of fun: I'm going to make sure of that! 

Just when we had thought we had finished with media interviews for the time being, on Friday I was asked to do another interview with the British Heart Foundation for their magazine. I was more than pleased to help with this, as it was about living with Idiopathic Pulmonary Arterial Hypertension and the importance of Professor Morrell's work and research on genetics, which aims to help develop new treatments for Pulmonary Hypertension. Hopefully the article will help raise awareness of Pulmonary Hypertension. His team were awarded a two million grant last year for this important research, which we are all hoping will lead to some targeted new therapies for PH and even a cure. 

We are still catching up with friends we haven't seen since my transplant and spent a lovely day on Saturday when friends came from Lichfield, near Birmingham to visit us. It all felt a bit surreal at times, as last time we met up was just before my transplant and now I've recovered a lot from the operation it was a bit like I had just blinked and it had never really happened! At other times I noticed how much more energy I had and how I can concentrate on conversation for longer. We are slowly managing to meet up again with many of our friends since I had my transplant and seem to have a busy calendar over the next month or so. 

After a lovely weekend, just as things feel like they are getting back to some normality after my transplant and Christmas, it's time to start packing a case ready for my next hospital visit - transplant clinic and yet another brochoscopy are looming again. Hospital always brings it home to me, the seriousness of all that has happened and reminds me that there is always some uncertainty about the future. I always begin to get worried about what they might find and why they need to keep checking my lungs over and over again. On the other hand it is always a relief to be going back as there is always something niggling - this time my never-ending cough feels worse this last few days - so it will be reassuring to be checked. It's always a massive relief to get home again and be reassured that all is going well. So I'm bracing myself ready and hoping I'll be home again fairly quickly again. 

My late father once gave me a lovely silver cross and disc engraved with a prayer called, 'The Soldier's Doxology', which had belonged to my grandfather. My grandfather wore this during the Battle of the Somme and survived it. I always keep it with my transplant bag and bring it to the hospital every time I stay in or visit. It is my lucky charm, I reckon if it worked for my grandad, then it could work for me! So far, so good, it will be coming with me yet again! 

Today I'm also sending off my first writing assignment for the writing course I've just enrolled on. I've decided it's time for some new challenges and activities now I'm getting my life back again and writing is something I can do from home and can also manage even if I feel a bit under the weather so it is a perfect choice for me. It feels good to get started on a new challenge and I am mainly doing it just for my own pleasure, but who knows where it may lead ... we will see! 

So a busy week with lots of bits and pieces going on - life seems to be getting busier and busier now, which is good news, because busier and busier means getting better and better!