I'm home!

I managed to get out of the hospital on Saturday, which was great because nothing happens in hospitals on a Sunday so I probably wouldn't have got out till Monday. There was so much stuff to fit in the car as the huge delivery of medication was sent to the hospital so I could start the drug. We somehow managed to fit all the boxes of drugs and syringes in the car along with my suitcase and all the stuff I had accumulated while I was in hospital and with my wheelchair taking up the boot. Honestly, I have no idea how we did it! 

As well as those huge boxes there were three smaller ones which we ended up putting in bags so we could fit them in the car a little easier. When we got home it was time to sort it all out. 

Yes, that is a lot of stuff! And the best thing - that's only one months worth! We get a delivery every month from bupa healthcare, which the NHS use for IV meds as they're much more reliable than the people who delivered my iloprost and it's a bit more serious if I run out or a pump breaks. We've actually found we don't have enough of a few things so we're probably going to have to get an extra delivery this month. 

The line itself isn't nearly as annoying as I thought it was going to be. To be honest I don't really notice it at all. The wound is still a bit painful because I still have my stitches in and if I move or stretch a bit too much the stitches pull at my skin but I think the actual line is pretty painless already. I'm getting my stitches out on the 20th June at my Bristol hospital so I think it will be a lot more comfortable after that. I've had stitches out loads of times so I'm not too worried about having them out. I could feel everything when she put them in and I know having them out isn't going to be as unpleasant as that! The pump doesn't get in the way much and it's not really a pain being attached to something all the time. Even at night I'm not tangling myself in tubing, which I thought I would be. Overall I'm pretty happy and I actually prefer it to the nebuliser I was on before. Having a bath is slightly more effort but even that is getting easier, I just hang the pump on the shower curtain and clip the line out of the water with a bit of plastic wrap on the connections so they don't get too wet when I wash. I'm being extra careful about infection, you're supposed to be able to shower, get water all over the water-proof dressing, over the line and the connections but I really don't want an infection so I'm doing as much as I can to prevent it. (I may have recently placed an order at Lush so I'm definitely not giving up on my lovely baths!). Minus the horrible operation, it's been a pretty smooth ride and I'm happy with how things are going. 

Here's the pump. It's pretty small, a little on the heavy side, at least a bit heavy to hang around the neck for too long, so I usually just carry it around and rest it on my lap when I'm sitting. The buttons are really stiff so you don't have to worry about leaning or sitting on it. You'd have to press two buttons at once to turn it off and it starts alarming if you do manage to do that. 

Making up the meds is getting easier every time and isn't really too much of a bother. It takes about 20 minutes in the evening to make up two batches of the drug, then I attach one to me and the pump and the other one goes in the fridge so I can do a much faster change over in the morning when I'm all half asleep! It only takes a few minutes to put on the cold one. It has to be changed every 12 hours because it's a time sensitive drug that starts to loose it's efficiency after 12 hours and the cold one will only last 12 hours in the fridge if it's then got to last 12 hours attached to me. It's important to change it on time but in a disaster I would only start feeling ill after either the drug had lost all it's potency or I ran out of the drug in the syringe. However they said if I did get stuck somewhere, in traffic or something, I'd have to phone 999 and get an ambulance or police to take me home so I could do it because the pressures in my lungs can rocket up and then putting the new one on late will cause them to plummet again and that's obviously not very good for you.

I'm not getting any side effects at the moment from the actual drug but when they were upping the dose in hospital I was getting pretty bad headaches and hot flushes, which I assume will come back every time they up the dose. But seeing as it wears off that's not too much of a worry. I feel like there's less pressure in my head since I've been on the new drug. Before, I couldn't lie down very comfortably as it felt like all the blood was rushing to my head, but that's definitely better now so whether that's the drug or not I don't know. I'm also starting to feel a bit hungry again, which is actually annoying at the moment because my tummy is swelling like mad and I'm getting full super quick so I can't actually enjoy all this food I'm finally feeling like eating. They told me at the hospital it would be three months till I started feeling any better and that'll probably be true for the breathlessness and the pressures in my lungs going down but I think because the drug causes the blood vessels to dilate (which is why it causes headaches and a hot flushing feeling) it might be already helping a little bit with things that aren't really symptoms of my disease but have turned up because my blood vessels are under so much strain. Who knows? But I feel like it's a good sign. 

There's not much to see at the wound site at the moment because there's a patch on it that's supposed to prevent infection and because I have all the stitches it's not sitting around the line like it would normally so all you can see is the patch but I'll probably post a picture when the stitches are out and it's healed a little and isn't a bit gross with dried blood. I realise some people don't like wounds but I'll be wearing a transparent dressing so it's what everyone will see when they see me and like I've said before, we mustn't be ashamed or our scars! 

Thanks for reading! 

One Year On and Groshong Line Insertion

I was feeling a bit tired on Thursday to write a good blog post but Thursday was my one year blog anniversary. To be honest I though I'd missed it ages ago, for some reason I thought I started my blog in March. An awful lot has changed in the last year. Back when I started I was on new medication and was starting to feel better. The effects wore off pretty quickly but I still enjoyed a fairly healthy summer and I was at least feeling good at Christmas. All in all not a terrible year. 

It's pretty much all gone downhill from there though, which I guess makes this blog a lot more interesting at least! It's been great writing a blog. Especially these last couple of months as it gives me an opportunity to let my loved ones know how I'm doing and opens up their ability to support me. There are lots of people I'm very close to that I don't see every day. I feel like it's a really easy way for people to find out what's happening with me. I hope I've been of some inspiration to people and I'm really flattered by the amount of views I'm getting. I feel like it's a good time right now for ill people to get their stories out there and I think this is probably down to the social media buzz around things like the no make-up selfies for cancer and the amazing story of Stephen Sutton. I think it's lovely how humans latch on to these personal stories and it's great social media can spread them so quickly and to such a wide audience. I feel like when you can't make a difference doing what you'd like to do then sharing your story is all you have. Our stories are the only things anyone is going to have in the end. (Deep!) 

Currently I'm still in hospital having just had my line fitted. This week has been great as I got to meet Stacie who is another blogger around my own age who also has CHD and PH. Have a look at her blog here: www.stacie-lifeisworththefight.blogspot.com 

Our stories are pretty similar and we're both really positive and it was great chatting with her. She's already got a line and she's been on the medication they're going to put me on so it was extra helpful to have a chat. She's also waiting for a heart and lung transplant so hopefully we'll meet again at Papworth as I don't come to Hammersmith very often. 

On the subject of the line insertion. I'm sorry if you've found this post because you are currently waiting for a line insertion because I have to say my experience wasn't very nice at all but as far as I've been told my experience was unusually bad. In all honesty it was an awful experience. I've not had the smoothest run up to the line insertion either, it having been cancelled twice and then up in the air for a while. It's really bad luck that everything that could go wrong did and it all happened to me! It was very painful, something I really wasn't expecting. I was already feeling stressed out as I'd been waiting an hour and a half in the waiting room just to find out if it was still being done because they were worried about my bloods. Then I got in there and surrounded by a big X-Ray machine and my face covered with a sheet I was feeling really claustrophobic, and then the pain! It didn't feel like it was under local anesthetic. I felt a lot of what was happening. And then half way through the guy says, "is your anatomy unusual in any way?". For those who've lost track I actually have a completely back to front heart. So I was obviously pretty stressed out after that and I was terrified that something was going to go seriously wrong. I had a lovely nurse holding my hand and stroking my head throughout and I think without her I couldn't have done it. So it wasn't good, all in all. I've never been that scared, stressed out and upset over an operation. I don't know if I made myself ill with the stress or I was reacting to the local anesthetic and radioactive dye they used, probably a bit of both. But I felt pretty rough for the rest of the day, had quite a bit of sickness and tiredness and everyone kept saying how awful I looked. My doctors were really sympathetic and they're looking into why it was so awful for me as they said they haven't heard of as bad an experience as I had before. 

I will stress that it is unusual for it to go so badly, it was a lot of little things that just made it so horrible and I'm sure it's probably just really bad luck that it all just happened to the same person and of course that person had to be me. Well it's all done now at least and so far I'm only getting little headaches from the new meds so hopefully that carries on going smoothly as they start upping the dose. I'm at four nano grams at the moment. A nano gram is a unit that equates to your weight so it's different for everyone. For me four nano grams is an infusion rate of 1ml per hour. They want to get it up to six nano grams before I start using my own pump (I'm on a huge hospital one at the moment) and up to eight before I go home. At the moment I'm praying they send me home at the weekend because I'm confident with the meds now and the nurses are really happy with how I'm doing. It feels like I've been here forever and I'm so desperate to get back to my own bed and to have a nice hot bath! Just to get out of this room would be great. I'm pretty sick of looking at it! When I get on my little pump hopefully they'll let me get out of the ward for a bit of change of scenery. I feel I am slowly going mad! 

Hammersmith Hospital

It's strange being in London and not being anywhere near the hustle and bustle of the shopping centres and business buildings. Hammersmith is next to the prison Wormwood Scrubs and is slightly in the middle of nowhere. It's all residential and little corner shops, though admittedly not shops that I'd feel safe walking into. It's such a contrast to where I live in the country but when I'm in hospital I might as well be anywhere as all I can see from my window in a brick wall and metal structures and that's usually what I see from every hospital room I stay in. 

My admission yesterday was awful. Asked to get here at 9 we felt guilty for deciding to get some more sleep and arrive at 11 (although when we phoned them, they said that that was fine) and then they said they wouldn't have any beds till 2 and put us in a waiting room. We ended up going to the restaurant and having a bit of early lunch but I must admit I was feeling pretty abandoned and not cared about after the procedure has already been cancelled twice and this was the third date we'd had to come into the hospital. I don't function very well on lack of sleep and was desperate for my bed so I could lie down and rest. Well we got there eventually at 2.30 but by that time the hospital day is pretty much over so I was feeling pretty annoyed that we'd come in and wasted a whole day. 

Things did start picking up though and my specialist nurse came for a long visit to explain everything about the line. It being the second time it was cancelled she wanted to admit me because the doctors are much more likely to do something if I'm in hospital rather than at home. Not the best reason to spend two weeks in hospital but I'm so desperate to get this line in and over with that I don't care how long I have to stay here as long as they do it. Unfortunately I've had a chest infection and I'm on antibiotics so they're being a bit hesitant to do anything while there's any infection so we're waiting for blood results to come back to see when I'll get the line fitted. Meanwhile they're going to teach me how to make up the medicine, set up the pump and care for the line once it's in so at least I'm not sat here doing nothing. 

The good news is because this is a university hospital I can sign into the wifi with my student login from my university and it works really well. I've got Netflix, iPlayer, 4oD and even an app which means I can watch live tv so boredom shouldn't be too much of a problem. In other news the food is much better here than usual hospital food. I had a curry tonight which was pretty tasty. I thought the chicken looked a bit dry so I just had the sauce and the rice. For a hospital to successfully produce a curry is pretty unusual. The food at my Bristol hospital is terrible and Papworth isn't much better. We also got some meal vouchers for the hospital restaurant in case I don't like the hospital food or I just fancy something different because they really can't have me missing meals. The restaurant is really good too and that'll save us a lot of money because I'll probably get quite a lot of food from the restaurant just because it's always going to be nicer than the stuff we get on the ward.

I've been trying to mix up my meds today so I know how to do it but I ended up with the drug all over my bed so that was a great start. But I'm here to learn and I've got plenty of time to practise before I have to be able to do it properly. I'll be doing it twice a day eventually so I'll get used to it. 

Looks like I'm going to be here for a while and in view of the good wifi I hope to be getting some blog posts up over this week. I'm all settled in as you can see:

Thanks for reading! 

5 Things That Made Me Happy This Week!

1. Baths

The simple cure for cabin fever is a good bath. I can't explain how much I'm loving baths at the moment. After a bad day a good lather of soap and a scrubby sponge is all you need to wash away the worries of the day. After a good day, what better way to relax than a sweet smelling bath? Sitting around all day feels a bit icky and dirty and no matter how ill I feel, I can always have a lie down in a bath. With my liver being so swollen it's the only time of the day I can get it to stop hurting. The warmth and boyancy of the water really takes the pressure off. It makes a lot of difference having that time without any pain. My liver isn't agony by any stretch but being uncomfortable 24/7 isn't pleasant and having that hour off makes it pretty easy to deal with.

I love a good smelly bath and I don't get much luxury in my life at the moment so I feel more than justified using up all of my lush products. I love lush for many reasons. They're a lovely green company started by a married couple with a great sense of humour. And their products smell amazing and do great stuff for your skin. Even the bubble bath makes my skin all soft and lovely. I was never that interested in moisturising until I smelt some of theirs. Nice smells and nice skin is the way to keep a girl happy! 

2. Swimming

On a similar subject, I went swimming on Thursday! I say swimming in the loosed sense of the word, what is actually did was float around. But my muscles feel well exercised and I didn't get too puffed out, which is great. The reason I went is that once I have my line put in I won't be able to go swimming anymore. Firstly the infection risk is high when your submerging something that has direct access to your heart in water that people have probably peed in. Yes the dressings are waterproof but they're not really designed for submerging, more a light shower or a quick wash with a damp flannel. There's also a risk bacteria could get into the line through the open end. For me there's extra reason because I'll be on a pump that can't be removed for more than a minute or two while refilling the medicine and I imagine it wouldn't appreciate being submerged in water. I know what your thinking but I can thankfully still have my baths. (That was the first thing I googled! Even before, how much does it hurt?). As long as I don't have it super deep I should be able to sit and lie in the bath with access site out of the water and I can just put the pump on a stool next to the bath to keep it out of the way of the water. Though it's a good idea to use clean water from the tap to wash my chest rather than the bath water. The good news is they're no longer putting the line in this week so I can go swimming again! I'll be having the line in the week after now.

3. Sunshine

I have a bit of a love-hate relationship with the sun. Or rather, I like the sun but the sun doesn't seem to like me. I come out in sore and itchy rashes after as little as ten minutes under the sun. So I have to coverup with a shirt and stay in the shade when it's hot. I used to only get it three or four times a summer when I was a child but the last two years it's really gone mad and it's like I'm completely allergic to the sun now. I also burn really easily. Last year I burnt my arms sitting in the shade with factor 30 sun cream on. So I can't really enjoy the sun to the fullest but it's just so lovely to see it shinning out there and relaxing in the shade with a cold drink is lovely. Everyone's so much happier when the sun is out and I feel my mood lifted too. As long as I avoid the dreaded heat rash, I very much enjoy a hot summer's day. 

4. BBQs

Like every other British person, we had a BBQ this weekend. In fact we've been having them all month. My mum is brilliant at BBQs. Everything is chargrilled and tasty, rather than burnt to a crisp and raw in the middle. They're just so good! We probably use the BBQ more than we use the oven in the height of the summer. There's just nothing better. We had my grandparents over on Saturday for a BBQ and it was the most perfect day. I felt really good, I usually start getting tired an hour into any kind of social gathering but I felt good all day apart from being insanely full after we'd eaten! It's always nice when I'm feeling well when I see my Gran because I know she worries about me. We've always been really close and I just love her to bits. It's not nice having everyone worried about you when you're ill but at least you get to see how happy they are when you feel better again. 

5. Papworth

The last that made me happy this week was going to see my transplant team at Papworth hospital because they were really positive about everything. Obviously I'd had a bit of a rough patch and I wondered if they were going to freak out about it. But they just said it was great how well I'd recovered since being in hospital. They want me to gain weight like everyone else but they weren't really pushy about it. They trusted that I was doing all the right things. Sometimes I feel like doctors think I'm a complete idiot. I have to assure them I'm looking after myself. As if I wouldn't be trying my hardest to stay alive long enough to get my transplant call! So I was really happy with that. And the appointment was really fast too so I was back home by 5 0'clock. I was expecting to still be at the hospital at that time! 

These are all the things that made me happy this week! It's been a good week! 

Weight loss, hospital and upcoming excitement!

I've had a bit of a rough time of it since my last blog post. My abdomen started swelling more, my breathless got worse, I couldn't eat and I had zero energy. After a few days spent entirely in bed we decided to go to the hospital to sort everything out. The main problem was I didn't eat so the weight was dropping off me and bare in mind I my swelling problem meant that I should have been gaining weight. You can really see how the weight has gone from my upper chest and shoulders. I've found bones I didn't even know existed. They didn't really do much at the hospital. I'd already doubled all of my water tablets but perhaps they took a long time to kick in as I started feeling better after being admitted. I started eating a bit better and they decided to let me go home after a few days as it would be a lot easier to eat lots of fatty foods at home rather than in hospital. They've threatened me with a feeding tube if I loose more weight. I'm just hovering over a severely underweight BMI at the moment and even though I've been stuffing my face since I left hospital and eating between 1,800 and 2,200 calories every day I haven't actually put on any weight. Although I haven't lost any either. According to my dieting app I should be gaining 1.5kg a month so fingers crossed that happens soon!

I'm feeling really insecure about the way my body looks now. I suppose if I was big and everyone kept saying "oh my god you've put on so much weight" and "have you lost weight yet?" and every time I tried to eat people said "why are you eating that? Don't eat!" I would feel the same way. I guess it's enivitable to feel self conscious about something everyone keeps talking about and you have to keep thinking about in order to accomplish weight gain or loss. I feel exceptionally angry at those posts that crop up every day on Facebook. Those "before size zero there was something called beauty" and "no one wants to hug a stick" posts. I find these really horrible. I wouldn't say, "no one like a fat person" or "oh these plus size models are disgusting". But FYI models are skinny because it's cheaper to make sample sizes of clothes in smaller sizes because it uses less fabric. Models all have to be the same height and size as the mannequins so that fashion designers can make clothes and know that they're going to fit whatever model they employ to wear it. It wasn't like an industry got together and decided to define beauty as skinny. There's no need to be cruel to thin people! I know people say they'd kill to have trouble gaining weight but I assure you if you were in my position you would be complaining too! 

I'm slightly concerned that I'm going to look like a drug addict after my transplant being so thin and with the medications making your hands shake. At the moment I look like some kind of child refugee who's got some serious bloating issues. Practically I'm constantly really uncomfortable with my bones being so close to the surface of my skin. I'm sleeping on a foam mattress and I still wake up with either my shoulder bones or my hip bone feeling seriously bruised. Size zero isn't all it's cracked up to be. I have a renewed sympathy for people with eating disorders. 

On to some good news, I have actually been feeling loads better since leaving hospital. I've had a run of really good days, which is brilliant. As long as I have a reasonable nights sleep I can pretty much garentee that I'll be able to get out of the house if someone invites me somewhere or I make plans, which is a huge improvement. Of course I will have the odd bad day but at the moment I'm enjoying my run of good days. 

My upcoming excitement is that I'm going to have my Hickman line fitted next week. Okay, so I'm weird to be excited about this but just the act of doing something that might possibly help my disease is much more preferable than sitting around waiting for the next lot of bad days to land me back in hospital on a feeding tube. Although I swear I'm going to be so annoyed if the new medication makes me sick cause I can't be not eating again! Mostly I've heard good things about it and hey it's something to do, mixing up medications and changing dressings, which is much more exciting than sitting breathing into a machine every two hours. Even though the medications is continuously pumped you only have to refill it every 12 hours and change the dressings on the line once a week so it's a lot less effort on my part. Sure having the line fitted isn't going to be much fun and I've heard they're really painful and bruise up a storm when they're first fitted but I'll deal with that. I've had a lot of catheters in my time and I can't imagine it being more painful than that. They both involve sticking tubes into arteries at the end of the day. 

The only annoying thing is that because it's changed every twelve hours you have to go to bed and wake up at the same time every day because if you suddenly stop the infusion your body can go into shock and people have died from sudden withdrawal. So I think I'm gonna go for 11am and 11pm as I like a good lie in because I wake up a lot in the night and although I go to bed early I don't get to sleep till 12 usually. I'm never going to stay out later than 11 so I'll always be home. The morning one might be a bit more problematic. I'll probably have to get up at 10.30 to mix up all the meds in time to change it over at 11 but if I have to go somewhere. Most likely a hospital appointment I'm going to have to change it on the road, in a cafe or at the hospital, which might be a bit annoying. But I don't think there's going to be a good time to do it really. If I did it earlier in the morning I'd have to do it earlier at night, which might interrupt meals out and visiting family and friends, which will probably be more annoying. 

So you'll probably be hearing from me after I've had my line fitted or before if I have any more thoughts. Thanks for reading! 

Living on the List

This has turned out to be the hardest post I've ever had to write. Which makes absolutely no sense whatsoever because I'm really happy at the moment. I think the problem is that I usually write down my thoughts but to be honest I don't really have any. The only times I really think about the transplant is when I'm having a bad day and just can't wait to feel better. I think I'm in a really good place at the moment because I'm ill enough that I really want that call to come but I'm level headed enough to not spend my nights tossing and turning, wishing and hoping. I know the call will come it's just a matter of waiting and keeping myself well enough for surgery.

So in view of the fact that I don't really have many feelings towards my transplant, other than a strange sense of calm, I've decided to write this post about what it's like being me at the moment. If I had taken the time to imagine what it might be like at this stage of my illness I probably would have imagined myself a lot sicker than I actually feel. Considering what's going on inside my body and how badly my heart and lungs are failing, day to day I feel okay (until I try and walk anywhere that is). Though it varies considerably. It's definitely a case of good days and bad days. It's mostly good but the bad days are really horrific and I do get quite down on those days. Everything just seems to get worse all on the same day and of course tiredness always makes everything harder to deal with so when I'm more tired I generally feel really rubbish. On bad days it's a case of trying to while away the day as fast as possible so I can get to bed and hope for a good day the next day. But the good days are okay. I can go out in my wheelchair, enjoy a bit of food, have a laugh and feel quite happy about everything. 

My main symptoms are tiredness, sickness, palpitations, painful swelling in the tummy and a lot of phlegm. But on a good day nothing is too much bother. The breathlessness however is horrible whatever day it is and I avoid it as much as possible because it feels so horrible, it can't possibly be doing my body much good. And I really hope to avoid an exploded lung or god knows whatever else could happen as my whole body feels like it's ready to throw the towel in. At the moment I can't walk much more than 5 steps without feeling out of breath and I physically couldn't do much more than 10 steps but that varies a lot too. My tummy pain has been under control up till now but it's been getting very painful recently, to the point where I can't sit still. I'm waiting for my water tablets to start making a difference because at the moment they don't seem to be doing much. I'm loosing weight like crazy too, which means my body shape is looking abit weird at the moment as I'm skinny as anything but my stomach and lower back is swollen so I'm looking slightly pregnant at the moment but hopefully everything will go back to normal after the transplant. 

In other news I'm off to get my central line fitted in two and a half weeks. It'll take months for the actual medication to start making a difference so I want to get the ball rolling as soon as possible. I'm not too worried about it as it's not under general anesthetic or anything so it's quite simple and then I have to learn how to mix the medication and hook everything up, keeping everything clean and stuff like that so that'll be quite interesting. 

Other than all that nothing much is happening. I've been out for the odd outing to stop me going crazy from being inside all the time. I've had lots of visitors, which has been nice. I like having something to look forward to like that. We're having a girls meal out this weekend for my Gran's birthday and the week after my boyfriend James is coming to visit so we'll probably do something nice then. I always think it makes a lot of difference getting out for an hour or two twice a week. 

A very inquisitive lamb I visited when I was at my Gran's last week! 

I'll update you all if something interesting happens but I'm really struggling to write anything at the moment. Perhaps watching constant reruns of Friends has frazzled my brain! 

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! 

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!)