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! 

Thursday, 20 March 2014

Iceland and Birthday Adventures

I hope you'll excuse my lack of posts recently, it's been quite a busy month, to say the least! I actually wrote this post a couple of weeks ago so I hope you won't mind being a bit behind the times until I feel like I can write about what has happened to me more recently.

As I hinted at in my last post I had a rather special birthday week planned. I was off to Iceland to see the Northern Lights! Well, 'best laid plans!' but the main thing is I managed to get there. I went on holiday and had a brilliant time! Better than that, we saw the Northern Lights three times! The bad news is that I was unfortunately not feeling very healthy for the majority of the trip. But in a few months time, I won't remember how ill I felt, I'll only remember the brilliant things I saw and above all the Northern Lights, which I've been dreaming of seeing since at the age of thirteen I read Philip Pullman's Northern Lights and read his description of the phenomenon. We saw them first from the plane just as we were approaching Iceland, I had the most perfect view of them from my seat and being so close I could see exactly how the green bands of light were quivering in the sky. It was beautiful! You can certainly see where Philip Pullman got the idea of the Northern Lights being a portal to another world. The next day we went on a special trip to see them, and after the tour guide spent twenty minutes telling us we probably weren't going to see them, I shouted out that they were right ahead of us. We stopped on the top of a mountain and managed to watch them for a few minutes before we were blown away by the bitter wind. It looked particularly special with the silhouette of the snow-capped mountains on the horizon. Then we went off outside of the city, to a dark hillside where we could sit in the couch and see them from the warmth, which was brilliant for me as standing out in the cold and the wind was starting to have an effect on my chest. We were there for three hours and the lights were showing the entire time. We were very lucky to see them so much. We didn't manage to get our own photos of the lights because you have to have your camera set up just right to be able to capture them and we decided it was a better use of our time to sit and watch them rather than fiddle about with cameras in the dark. The tour guide took lots of photos and she emailed them over to us so you'll be able to see how wonderful they were.

As you can see, they are pretty incredible!

The next day, we were only there two full days as we landed late on the first day and set off very early on the last day, we went on a trip around the Icelandic "countryside" where they have a distinct lack of greenery. It's mostly desert land at this time of year but apparently the whole landscape is covered in green moss in the summer but they don't have much in the way of flowers or trees. We had a private tour in a taxi as I wasn't feeling too well it would be a lot easier than a whole day couch trip, which would have worn me out a bit too much. At this point my breathing was very bad and I couldn't stand up without getting breathless and all my muscles were floppy and useless. We saw the main cites, the geezers (I stayed in the car for that one) and the waterfalls, which were amazing.

On the plane back though my breathing was getting ridiculous so we went straight to A&E as soon as we got back, which was about four 0'clock in the afternoon despite leaving the hotel at five in the morning. I mentioned in my last post that my 'women's problem' had gone mental for the last few weeks and while it slowed down just before I went on holiday, it started again on the first day in Iceland. I had lost such a vast amount of blood that I was very anemic and my heart was really struggling to keep up, which was causing fluid to build up in my lungs and causing me to feel pretty run down and breathless. So they wanted to keep me in overnight. I wasn't brilliantly happy with this as the next day happened to be my 21st birthday. But there wasn't much I could do about that, I needed to be in hospital so my mum and boyfriend brought all my presents into hospital in the morning and arranged a steady stream of visitors for the day. Unfortunately my body had other ideas for the day. I'd just managed to open all of the lovely presents and cards my mum and boyfriend had brought in when my stomach started to ache and I was starting to feel a bit odd, lightheaded and just not quite myself. I thought maybe I just needed the bathroom but after sitting on the loo for ten minutes the pain just seemed to be getting worse. By the time I was back in bed it literally felt like my stomach was about to split open, or like someone had stuck a vacuum into my stomach and was sucking up all my organs. It was the most pain I've ever had, much worse than both my Pulmonary Embolism (which had previously held the title of most pain ever) and my Lung Haemorrhage (which surprisingly didn't hurt much, but sounds quite extreme) and the times I've had my sternum broken open during open heart surgery, not to mention all the chest pain I get, I would say I have quite a good pain threshold but I was screaming like a baby with that stomach pain.

I must say doctors are surprisingly rubbish at dealing with ill people, considering that's quite a big portion of their job. The doctor they got to take a look at me just said, 'stop screaming and tell me what's wrong' as if it was that easy. My mum and my boyfriend were standing right there and he didn't think, oh she seems a bit preoccupied at the moment maybe they know what's going on! I think he was a bit out of his depth as a cardiac doctor and called the gynecology doctors who were in the middle of surgery so couldn't come down straight away. They gave me tramadol, oramorph and IV morphine, which I can't say had much impact on the pain but made me so exhausted I couldn't scream as loud. They moved me to a private room (funny how those rooms suddenly become free as soon as you start making a noise) and kept me drugged up too much to know what was going on. Then the gynecology doctors turned up and did what I will describe no more than 'examined' me (ouch!) and said there was so much clotted blood in my womb that my body had started having child-birth like contractions to try and push it out. Even though the pains weren't coming and going like contractions, all the muscles involved were doing the same thing as they would with contractions. They went up and pulled all the blood out (ouch again!) and it instantly felt a whole lot better and I was able to rest a little before effects of the morphine started making me feel extremely sick and drowsy. I managed to console my Gran for a while, who had turned up most unluckily at the height of my pain and then had to listen to me scream from down the corridor while the doctors were sorting things out. I opened some more presents and cards before falling asleep. I spend the rest of my 21st birthday dozing, throwing up and crying with happiness that I wasn't in pain anymore.

The people on the ward were lovely. The other patients banded together and bought me a birthday cake and then came into my room in their dressing gowns and sang happy birthday to me. When I got moved to the gynecology ward the patients there too were lovely and wished me happy birthday, one of them even came up and consoled me when I was upset because the nurse had just completely ignored me when I was throwing up and crying. The nurses were a bit mean really. It's a shame because so many of them are lovely, but I had a run of three not very nice nurses and a few useless doctors who couldn't really deal with me when I was in pain. I think they should focus more on the caring aspect of a nurse, I feel like the hire people as long as they know their stuff, but it really harms your recovery if you're constantly upset because of how the nurses are treating you. It particularly bothered me that the last hour of my birthday was spent being upset at my nurse rather than a real, unavoidable problem.

As you can see I was pretty chipper while opening my presents! And my lovely bear came in handy for hugging while I was in hospital. 

I'm still weak and breathless at home now and am in contact with my specialist who is a bit concerned at how quickly they let me go home, only the day after I'd had all my pain and bleeding. So I might end up in hospital again this week as my chest pain is worsening and I have no energy at all. And I still can't stand up without feeling out of breath. It's a massive effort to get up to go to the toilet, I actually have a seat with a bucket in my room now because getting across the landing is too much of a drain on my energy and my lungs just don't have the capacity for that kind of exercise. I'm not going to lie - it's really horrible. It feels like my whole body is screaming at me when I get out of breath. It's not like when you go for a run and do a bit too much and have to stop to get your breath. It's like I've just jumped into a pool of ice and every nerve in my body is screaming "what are you doing???", when all I've done is put a pair of leggings on. I hope that makes sense, I'm just trying to say it's not like normal breathlessness, it's like my whole body has gone into shock. So obviously I'm avoiding getting out of breath as much as I can because the feeling is really not very pleasant at all. I'm managing okay though. Watching a lot of tv and staying in bed some days with my iPad, looking on YouTube and catching up with tv on BBC iPlayer. Say what you want about technology ruining the world but it sure does come in handy when you're disabled and ill and can't get out and about. 

So life is on pause for a while, no doubt I'll be back in hospital soon and finding out whether this breathlessness is going to be a permanent thing or whether this is just how life is going to be now. I hope I haven't depressed you too much, maybe read the nice first paragraph about the northern lights again before you go, then grab yourself a nice cup of tea and your favourite biscuit! 

(I'll be updating you on the rest of what's been happening as soon as I can find the words! I'm hoping inspiration will come in the next few days!) 

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