Sunday, 21 June 2015

Hope and Breakthrough News for PH!

I'm still involved with a genetics study to enable a better understanding of PAH and enable better treatments. Although I don't have PAH any longer following my heart and double lung transplant it is still possible for the research team to use my DNA samples, so I'm still giving blood samples from time to time. The genetic thing first became all important to me because I literally had no idea why I had PAH and was scared that my two girls may have it.

My PH team kindly referred me to Addenbrookes and although there was no formal testing for PH genetics at the time, I was lucky because they were about to establish one and establish the research into understanding the genetics of PH in the hope of getting some breakthrough for new treatments.

Filming with BBC Look East

When you're diagnosed with PH it is a very frightening time, no matter what stage your PH is in. For me,  I was in end stages, so I was put on intravenous medication within a few months then referred for a heart and lung transplant. I was told I wouldn't have long to live and perhaps only have two years of quality life left and then in the same breath told it may take up to two years or more to get my transplant. It was a stressful and scary time. With the shortage of organ donors, it is a very difficult to get three organs. Organs can be split to save two or three lives instead of saving one and only a couple of transplant centres still commit to undertaking them - I was lucky - lucky in so many ways.

Sometimes during my life I've complained I've not been in the right place at the right time: the sun shone on me through my illness though - I was being treated in a centre that believes if you need three organs then that's what they will strive to get for you and I was under a transplant team who had a lot of expertise for this rare operation. I was also lucky I was under the excellent care of a superb specialist PH team and they knew exactly what to do every time I met a crisis and saved my life more than once. I was more than blessed on that day I received my transplant, that day a family said 'yes' to organ donation and that my donor had believed in organ donation. It is all a very hard route to go down though, however blessed you are - and I know for some this route is even harder. For many even transplant isn't an option for various reasons.

When you are first diagnosed with PH, it doesn't really matter what stage of PH you are in: mild, moderate, severe ... it is all very frightening. You know it is a chronic and incurable disease and it doesn't matter how well you are doing or how positively you manage things, the fear is always there about the future. You know the disease may progress, then you will need more treatments ranging from oral medication, oxygen to intravenous medication and then for some more complicated and life threatening surgeries such as Pulmonary Endarterectomy, lung transplant or heart and lung transplant. You fear a future of disability and you are afraid of dying and your family are terrified what will happen to you and them next.

Even undergoing the life threatening surgeries to remove PH, a cure as such isn't truly found. Often with a Pulmonary Endarterectomy, there are still remnants of PH and distal blood clots, which require life long management and drugs and with a lung or heart and lung transplant the survival rates are only 50% at two years. I was also informed I would have a 20% chance of not surviving the operation or the first year after my heart and double lung transplant. It is a hard route to be faced with and all any family, who has been affected by this disease, wants is to know there may be a cure - know that somebody somewhere is doing something to help them, something that may save their future.

Last week I was contacted by the British Heart Foundation, who have provided funding for research in the genetics of PAH and was informed there had been a breakthrough in the research. The research is being undertaken by a team led by Professor Nick Morrell at Cambridge, who is both a Research Director for the PH service at Papworth Hospital and a British Heart Foundation Professor of Pulmonary Medicine. 

A protein, BMP9 has been discovered that can counteract the damage that is done by PAH to the cells in the blood vessels of the lungs: it can prevent narrowing of the blood vessels and also restore damage. The research has found that patients with PAH don't make enough of this protein. Tests carried out have shown that it is easy to manufacture and therefore could possibly restore the blood vessels in patients lungs to a healthy condition. 

More testing, research and patient trials have yet to be done and it will take some time yet, but this is a major breakthrough for the treatment of PAH, both inherited and idiopathic and hopefully if things come to fruition, then for patients with secondary PH too. This news brings so much hope for patients who are suffering with PH and facing difficult choices about treatment in the future and so much hope that anyone being diagnosed in the future will have chance to be cured and not face the long and gruelling route that patients must endure now. 

I was asked if I would help with the media launch of this breakthrough, as I've been involved with the genetic research and suffered with end stage IPAH and the difficult treatments. On Tuesday, I was interviewed live on BBC Three Counties Radio talking about what it is like to live with PAH and how this research breakthrough is such a significant step for patients suffering with it. On Wednesday, reporters from BBC Look East visited our home and interviewed me for an article for the 6.30pm and 10.30pm news later that day. I was delighted to help, not only to share the promising news that gives families so much hope, but to raise awareness too of what this cruel disease is like to live with and the difficulties of the treatments we have to face. Also, PH is regularly misdiagnosed, so to be able to explain its symptoms on TV and radio, could possibly alert someone to the condition - the earlier a diagnosis, the better the prognosis.

Cambridge University Research

BBC Three Counties Radio 16th June 2015  (just past half way through)

BBC Look East Report 17/5/15

The hope of a cure is something anyone diagnosed with or affected by this disease hopes for everyday and now things look like they may be getting nearer ... I truly hope so - it would be such a wonderful thing.

Wednesday, 10 June 2015

A Transplant and a Wedding

The last couple of weeks have been absolutely amazing. 

There we were knee deep in finalising all the wedding plans, I kept finding myself a bit overwhelmed and very emotional that this was even all happening. We were just working towards getting all the house organised, drink and food bought in readiness for a house full of guests both before the wedding and for a celebration party on the Sunday afterwards - all this on top of finalising plans for the church and reception, altering outfits, pressing wedding and bridesmaids dresses and veils and all the other last minute things you do. All very exciting and emotional at the same time after all that planning for the last eighteen months. 

We were just about ready to start the final rundown, which started with travelling to Lancashire and back to pick up my mum so she could join in the fun with us all. On arriving back it would then be the wedding rehearsal on Thursday at the church, followed by a family meal and then on Friday, Sarah, the bridesmaids and my sister and family would join us all throughout the day and us girls were having nails done and then another family meal was planned before the big day. 

It was all stepping up a gear and now getting quite emotional. All was going perfectly: busy, busy and then right out of the blue I was roused at 5.30 am by my mobile phone ringing...

I still have my mobile phone by my bed, just like I had during the wait for my own transplant. It has been there every night for a very long time, waiting for yet another all important call. An odd time I will forget to put it there and I cannot sleep until I've gone back down the stairs to retrieve it, as it may just be the 'night' it may ring. 

I know who it will be right away when I wake to the ringing - I have even been half dreaming of her, as despite her own despair she has been supportive and excited about the wedding - she has just shared my last blog about the wedding; she is supposed to be coming to the wedding. Lots of us close friends have been trying to keep her boosted, trying to take her mind off the fact she has had to wait for well over three long years now and her transplant still hasn't happened - only three awful false alarms. Having endured a wait for just over two years, I know how difficult this can be. It is unimaginable to think of a wait that is that long: when you are feeling so poorly amidst what feels like is becoming a hopeless situation. We are all running out of things to say really; frightened of losing hope ourselves even. 

'Hi Kath, I've got my transplant call...' says an animated and excited Stacie - so many of us have been waiting for this day. I'm so excited scrabbling around with my phone and half asleep, it falls from my hands and drops off the bed! It is a long, long day as we wait for news - firstly whether it's even a go ... after three false alarms we wait with some caution ... then before  2pm Stacie phones again - we exchange some 'good lucks', some 'love yous' and then she is gone - there isn't really time to say all we need to say to one another ... another long wait. I expect it to be past midnight if my operation is anything to go by, but no - not our amazing Stacie - we get news on and off that things are proceeding well and by around 8.30pm all has gone to plan and the operation is complete. 

In the early hours of the morning there is more news - all is still well and they are going to wake her from sedation. It carries on - simply amazing - there are no words - bypass equipment is removed, ventilators are removed and Stacie is even trying breakfast and then strawberries for lunch. It is like a miracle ...  We are all thrilled and everyone's emotions are running high ... if you are a similar transplant patient, you tend to relive it all again and feel every step of the way and all the emotions. It is all too overwhelming, as emotions are already running high in our household - we laugh; we cry with happiness; we keep on welling up. Stacie keeps on doing well...

Then back to the wedding, we start the final countdown in between the 'Stacie' updates - she's posting again on social media - her picture on twitter from her hospital bed captioned, 'I'm alive!' goes viral on social media - we are elated at her progress. Just before she posts this on twitter we get a text that says, 'Hi guys, I'm alive! Lol!'  We are sat full of excitement and in tears in the car on a motorway service station en route to get my mum. Then driving back from mum's and there just happens to be a live broadcast of a heart transplant on Radio 5 Live - we listen - it's an incredible experience listening to someone relay what has happened to you - and what has just happened to Stacie -  we are in awe it could possibly have happened to any of us. Emotions of all kinds are stirred up once more. 

The wedding - Stacie - transplant -  the wedding - Stacie - transplant - the wedding.... it is all surreal now - too many mixed up emotions. Every day is incredible - incredible news of Stacie; incredible that I am actually involved in organising my daughter's wedding and fit and well enough to enjoy all the frenetic build up...

Saturday arrives, the sun shines high in the sky and the wedding goes perfectly - an absolutely perfect end to one of the most emotional of weeks. A most brilliant and overwhelming day, one that I wouldn't have seen if it wasn't for my donor and to be able to live this day and celebrate with so many of my family and friends is a truly wonderful and magical experience. 

It has been a dream come true for Stacie too, when she received her new heart and lungs. Her new life has now begun with a great start at long last - again all thanks to the kindness of a complete stranger. We managed to pop and see her just after the wedding and she looked fabulous and was so thrilled and excited she'd had her transplant. 

Organ donation simply changes and transforms lives can never be underestimated: the hope it brings; the priceless moments it enables us to share with our love ones; the future it offers to us.