Monday, 24 March 2014

Big decisions!

I think I finally feel ready to write about what's happened the past couple of weeks. If you've read my last post you'll know I had quite an eventful birthday, well after that I went home for a while and was just too tired to get out of bed most days and standing up was exhausting. So we phoned my specialist and within a few days I was in hospital. Due no doubt the the vast amount of blood I lost on my birthday my haemoglobin levels were down so I had a blood transfusion and some more water tablets to keep my liver a bit more comfortable. They explained that the reason I'd been feeling so grotty and breathless was because of a sudden worsening of my Pulmonary Hypertension, (which has a bit of a habit of doing that) which caused my heart to struggle resulting in heart failure. In short my heart just isn't gathering up the strength to push blood around my body. This causes a backlog of pressure and blood, which is pooling in my liver, making it swell and because the liver is enclosed in a kind of capsule containing a lot of nerves, it's making me pretty uncomfortable. However the water tablets are helping a lot with the pain even though my upper abdomen has swollen like a balloon. The liver is then pressing on and possibly inflaming my stomach, which explains why I had been feeling sick and generally not the slightest bit hungry for the last few weeks. Unfortunately I'm still unable to eat meals but I'm trying to keep up my weight by snacking as much as possible. 

It isn't great news that my PH is getting worse and that my heart is failing as not much can be done about either of those. There are pills that can help with heart failure but as long as my PH is putting so much pressure on my heart, the problem is going to be hard to manage. I'm through all of the PH medication they can use so the only option now is a double lung and heart transplant. I felt a little numb when they told me that. I found myself only shedding one or two tears before suddenly feeling very calm. At that's point, although I'd been to Papworth hospital before, I didn't really know what a transplant would mean for me so I tried not to think about it too much as the only thoughts I had were questions that couldn't be answered until I met with the team at Papworth.

After a week in hospital, I went home to await the call from Papworth. My doctor must have done a good job at pressing the urgency of the situation because it was only a few days until we had a call to say they'd fitted us in to a cancellation the next week. Meanwhile my lower abdomen had swollen and I was actually in A&E when we got the call from Papworth. I had started retaining fluid in my abdomen, making it swollen and sore. Yet another problem to add to the list! Fluid retention is a symptom of pulmonary hypertension so it's just another thing to deal with. It's uncomfortable but it looks like I'm pregnant, which I don't like. It's typical of me to be loosing weight and getting fat at the same time! Only me! 

We had a nice drive in the sun down to Cambridge and stayed in a guest house for the first night as we had to be at Papworth at 9.00 the next day. The next two nights I spent in hospital. The first day I had some of the smaller tests, x-ray, bloods, the usual and then we went to see the post-transplant nurses to discuss all the problems of the operation and aftercare. And boy are there a lot of things that can go wrong! Okay, so I knew transplant surgery is probably the most dangerous kind of surgery around but I didn't really expect so much bad stuff. She'd just finished talking through the side effects of all the pills, which include kidney failure, osteoporosis, diabetes - to name a few! And then she said, "right now onto the bad stuff". What?? That wasn't that bad stuff! Then it was all doom and gloom, the possibility of your airways closing up after surgery, the almost certainty of getting acute rejection in the first year, which requires hospitalisation and a major amount of medication, the infection risk of having a suppressed immune system. There was so much bad stuff I was feeling quite flat after the first day and pretty sure that unless the surgeon was sure I would live a lot longer after the transplant that I wasn't going to have it done because it just wouldn't be worth all the pain and horror of the surgery and then the aftercare. 

And - wait for it - I won't be allowed to eat cheese! What is life without cheese? I can have cheddar because mostly that's gone through all sorts of processes so it isn't high in bacteria, but no Brie! Baked Brie with cranberry sauce has to be one of the best things to come out of a kitchen. Also no salad bars, takeaways, any kind of red meat that isn't really well cooked (I mean what is the point of a steak without the yummy blood in the middle!) and runny eggs! (You literally can't eat eggs unless they're hard boiled - ruined in other words!), You've got to be so careful about what you eat. I mean going to a restaurant and having a salad, you have to worry about whether they've washed it properly, whether the salad dressing has raw egg in, if you have a pasta - is there raw egg in that, what cheeses have they put in it? I'm in love with food - can you tell? Joking aside, it probably will be a massive pain in the bum but I'm happy to trade in cheese for feeling healthy.

Back to the serious stuff. I had another day of tests before I saw the surgeon. These were the tests I was most worried about as they included breathing and exercise. The two things that had become extremely difficult in the last few weeks. However the nurse testing me was really lovely and understanding and gave me lots of rests during the lung function tests, which involves blowing into a machine (easy for you but my lungs really don't like it!) and he didn't push me too much on the walk test either. I managed to walk a little bit just to show them how bad it was. At this point I was seriously doubting they'd say I was fit enough to survive a transplant because once you get too ill the risks of the surgery are just too much for a sick body to handle. 

The next day was by far the best of the three days. We saw the surgeon and he was really confident that they could do the surgery and that the results would be good for me. He said the death rate of the surgery would be 15% but that once I got through the surgery and the first few weeks we were looking at 10-12 years of life. It had become clear by that point that I'd be extremely lucky to make it that long considering how ill I am now and how quickly I my condition has deteriorated. In fact the doctors are slightly concerned that the 2 year waiting list for transplant will not be long enough for me. But I've been known to be a bit of a wild card where health is concerned and I've beaten the odds many times before. I think I'll be fine. Most things are in my favour. I'm a common blood type, I'm small (small heart and lungs come up more often) and I'm bloody lucky! I have some weird antibodies, which means that a few heart and lungs won't be suitable for me but mostly the odds are in my favour for getting a transplant within the next two years. It varies a lot. The shortest time anyone has been on the list is four hours but a lot of people die on the list too, so it just all depends who happens to die to become a donor and whether or not you're the person who is a match. 

Some other good news was that, even though my heart is really deformed, they reckon they'll only have to recreate one artery in order to get a normal heart into my body. This is mainly because the heart and lungs come out of the donor as one organ, all connected up and ready to be attached into the patient rather than two separate pieces that need to be fitted into the jigsaw. All the arteries they need happen to be in the right place in my body except this one big one. However they said they can take an extra artery from the donor and do a bit of clever stuff to make it work and it shouldn't add too much time to the surgery, which was the big problem in the past as the longer the surgery the worse the survival rates generally. They have a lot of problems with electrical malfunctions in the brain if you're on a heart and lung machine too long. Obviously the less time you don't have two major organs missing from your body the better! 

After that I was feeling pretty confused as I was so against the idea of transplant after the first two days. I think the nurses could tell I was unsure of what I wanted because they offered to find me someone who'd had a transplant so I could chat to them and ask any questions. As luck would have it, someone who'd had a transplant to combat PH was there at that moment and free to talk. It was good really that she hadn't had the smoothest of transplant experiences because she said even after all the horrible things that happened to her, and she had a lot of problems most transplants don't involve, she said it was completely worth it for the quality of life she has now and even if it doesn't last the ten years they predict, the quality of life makes it worth it. Of course, everyone goes back to dying eventually, and most of the symptoms are the same as PH and heart failure, and there's a certain level of "delaying the inevitable" when it comes to transplants, but hearing that it's worth it to feel healthy again, even for a short time really swayed me when deciding what I need to do. The way I see it there's a, if not 100% then a good 99% chance that I'll die and in the next couple of years if I don't have the transplant, so suddenly that 15% (which works out at around 1 in 7 people) doesn't seem such a huge risk. I know 15 is a low number but that's pretty much how risky operations get. It's considered really high risk and usually they won't carry out any kind of surgery if it's over that number. And when you take it down to 1 in 7 people you can understand how big of a risk it really is. And when I think the chances of me actually having PH in the first place is 2 in a million people, 1 in 7 starts looking pretty scary. And that's just one of my diseases. I like numbers. I always get doctors and surgeons to give me numbers because it helps me make sense of things. In this situation though, I'm trying not to think about the 15% too much and go with my gut. I know the transplant is the right thing to do, so I'm going for it, because for the chance of 10-12 years (possibly more if they come out with some swish new drugs in the meantime) it's worth the risk. I think positivity is really important. If your mind and your body want it enough then you've got a much bigger chance of making it happen than someone who has given up. I'm sure it works. How many times have you heard of dying people hanging on until some long lost relative comes to visit, and then dying moments after they've seen them. I'm sure it's possible to will yourself to live! 

So I've officially made my decision. I rang Papworth this week and as soon as they have all my blood results back, I'll be making the trip over to Cambridge to sign the papers and then within a week of that they'll ring me up so say I'm officially on the list and my transplant can happen at any time from that point. Someone has actually waited only four hours before now! But it's most likely going to be around the 18 month to 2 year mark. They only do around 5 double lung and heart transplants every year so it really just depends on what donors come up. That's compared to over 50 heart transplants they do each year and I think the number for double lungs is around the same. So it's not a huge number! Which is slightly scary but I guess it's the same thing as doing them separately really, it's not like the surgeons are going to be out of practise! I have made the decision to not accept organs from over 55 year olds and the reason for that is because there are always new treatments coming out and if they suddenly say, oh you know what we've come up with this amazing new drug that completely cures rejection, I'm going to have a limited lifespan because I have organs that are already old and likely to give out within 20 years. Obviously if nothing changes then that's fine because you'll be lucky to get 20 years but I live in hope that they will figure something out for rejection and I'd hate to die thinking, if only I'd had young heart and lungs, I could have 50 or more years to live. They rarely use heart and lungs from people this old because it's rare for older people not to have damaged their hearts or lungs so I'm not cutting out many donors and I think it's a sensible decision because you never know what's around the corner what with stem cell therapy looking so promising. 

So my plan for the time I'm going to be on the transplant list is to sit around, watching films, doing some sewing, taking up knitting, writing, maybe trying to get my writing published, getting out in the countryside as much as possible, perhaps go to a few museums or zoos or castles that are on my doorstep but for some reason have never been too and maybe a weekend away in the summer and just generally chilling out. I'm definitely not going to be sitting around feeling sorry for myself! I'm also going on a new IV medication that unfortunately means I'll need a Hickman line but will hopefully make me feel generally more comfortable and stop my PH getting any worse because the transplant will be off and I don't want that. Hopefully I'll get the call to go up to Hammersmith in London to have my Hickman line put in (the ward nurses at my local hospital won't be familiar with this particular drug and how to mix it and hook it up to the line so my doctors decided it was safest to send me to London where they're really used to dealing with it and most importantly teaching the patients to do everything because a new infusion needs to be attached every 12 hours so I need to know how to do that at home). Until then, fingers crossed I remain stable, because if I get much sicker the transplant will be too dangerous and I won't be able to have it. And hopefully the transplant comes quite soon! Although I think 4 hours is a bit too quick! 

So a lot has happened this month! And a lot has changed but I'm still here, little old me and my blog. It would mean a lot to me if you all took a moment to appreciate the ability to walk and to breathe, and to live with the prospect of time stretching out in front of you. Not to feel sorry for me, but to feel good about your own lives. And maybe if you're deciding whether or not to buy those diamond shoes, this might give you the push to go for it. You only live once! Now have a nice cup of tea and put your feet up. I'll write again soon! 


  1. Hey Sara, I'm so sorry that you've been so sick recently! But I'm so pleased theta you've decided that transplant is the way your gonna go, it's definitely not a easy decision and one that takes a lot of courage. I certainly hope you won't have to wait as long as I have been waiting :) xoxo

  2. Thank you Stacie! It's a big decision but I'm sure I've made the right one :) Oh I know, you've waited such a long time now. I hope you get your call really soon! x x

  3. Hi Sarah! My name is Serena and I was diagnosed with PH not too long ago and stumbled upon your blog while looking for things. I'm very sorry to hear about your hardships lately. You are incredibly strong and brave, and that will certainly help you in your upcoming journey. You are in my thoughts and I wish you all the best xo

  4. Hi Serena! I'm sorry to hear you have PH but glad people with my disease are finding my blog. Thank you for your kind comments and I wish you luck with your PH for the future! If you need to chat about anything I'll be here. Best wishes to you too! x

  5. Hi Sarah!

    I don't want to be a bother a but I have been thinking about you, and I think some of the stuff I've come across might be interesting to you too. I hope this comes across with the best intentions oppose to anything else.

    I'm not sure if this articles are helpful for you at all, but this young women had a second double lung transplant after rejection and she has maintained her career as an opera singer I have talked to her on a message board for PH and she is very kind if you wanted to reach out to her.
    I know your situation is different, but I find it inspiring to find stories of success because that is what we need to focus on for ourselves.

    I have also read a lot of stories by Dr. Bernie Siegel, he believes that positive attitude and mindset help with healing. I know it sounds kind of strange, and I promise I wasn't this kind of person before my diagnosis, but I truly think his suggestions have had some kind of impact on me. I have also read Mind Over Medicine, which was written by another doctor. I enjoy Dr. Bernie Siegel's books more, but Mind Over Medicine has more up to date science and facts to back up the idea that one's thinking can impact their healing. Dr.Bernie was a surgeon so he talks about what kind of mindsets help with recovering faster and so on.



  6. Thanks for the info Serena! I definitely believe in mind over matter. Even the nurses at my transplant hospital say that the people who are really positive recover so much better than those who are unsure that they want the surgery. Hopefully it'll serve me well! x



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