Never give up.

Has summer finally arrived? Oh no wait, it's only for 3 days then it's smelly rain again! Come on British summer, get your arse into gear, some of else want a tan! However, I am currently sitting in the glorious sun while writing this so maybe by the end I won't be looking as pale as a ghost! It's been pretty manic recently with appointments up in London, literally up and down like a yoyo! starting to feel like a commuter these days! ...which isn't a good thing with train strikes every other day, I don't know how people do it everyday! Anyway, about three weeks ago I saw the bladder surgeon at Queens Square, London. As some of you know I also have Fowlers Syndrome which means my bladder doesn't work, due to the muscles and nerves not doing what they're meant to do...cheeky buggers. At the moment I have to self-catheterise myself, not all the time but there are some days I could go all day without going to the loo, or I'll go to the loo but not everything will come out. I've had to self-catheterise for about two years now and they wanted to give me a few options to try and help. They have suggested having a pacemaker put onto my bladder, very similar to a heart pacemaker. This would make my bladder work and hopefully a better quality of life, as I then wouldn't have to catheterise and they could control things better. However, they have done studies of this procedure with people with Elhers Danlos Syndrome and it hasn't been successful...typical. Also, after discussing everything I do feel at the moment I am coping with catheterising and I feel I'm maybe not yet at that stage? Especially as i don't have to catheterise 24/7. So they have said I can come back in one year and we can see how things at going then. As they said I have a lot of my plate at the moment and a lot of surgery coming up, so maybe one less thing to worry about at the moment is a wise decision which I couldn't agree more with!

A few weeks back I saw the nutrition team at UCL. These guys look after everything to do with my nutrition and my TPN. Of course as soon as I walk into the room I have to get on the scales and unfortunately I have lost a bit of weight recently since I last saw them which is frustrating. Even though I'm on the TPN, I used to have a little bit more orally too each day, however due to my pylorus now completely closed (opening into my intestine) I have been unable to take much in orally apart from liquids/mousses, jellies etc, meaning consuming less calories meaning weight loss.  so it does make sense. They have suggested I have a supplement drink a day...oh the joys of them again, GRIM. They don't really want to increase my TPN as they're worried about my liver. If you know much about TPN, I'm on lipids which is basically got everything in it and a lot of fat too, because they want me to weight gain for surgery. However lipids aren't good for your liver so they're having to keep a close eye. They're also worried about my heart as its been struggling recently and I've been getting chest pain. Also on that day I had a lot of pain around my PICC line so the vampires took a load of blood to check everything out, so we'll wait and see.

The same week I had to go to UCL to have my three-month Lignocaine infusion...can you now see what I mean by me becoming a bliming commuter! Anyway, the Lignocaine infusion is to help with my pain. I was two months late this time due to them not sending me a letter of the original date, so I annoying I missed that slot. Anyway, if you've been offered this treatment I really recommend it! What it basically is doing is numbing your body, of course only a tiny little amount. Its the same stuff they use for numbing your gums when you go to the dentist! ...yes really! But they infuse it into your blood steam to help pain. At first, and I know other people who's said the same thing and that is at first, you don't think its helped but when it starts to run out you really notice the difference. Recently I've been getting a bit of leg pain too (on top of everything else!) and this really helped it so if you're offered it, do it!

Lastly, last week I saw another surgeon again in regards to my upper GI surgery this time. He called me up to see him as they have decided what they are going to do in regards to the operation on my pylorus. What they're going to do is open me up, (grim) unfortunately it can't be done key hole but they'll stitch open the pylorus (opening into my intestine) meaning that it will be permanently be open. This should then mean I should be able to take things orally again. Still on a limited diet though due to my intestine failure but more then just fresh air like at the moment. However, this has happened to the pylorus because of the nerves dieing around that area, and he is worried about what the other nerves are doing and if my stomach is working and if it will be able to pump things through. But with the pylorus being permanently open, gravity would get things through so keep EVERYTHING crossed it works. Also, he's all going to fit me with another JEC (feeding tube into my small intestine) and the TPN will stop...yayyyyy! (feeding infusion straight into my veins). I can't wait to see the back of TPN. TPN saves lives but it doesn't give you a life. You have to be so careful with it and it takes a lot of time. I was only meant to have this for 3-4 weeks but 18 weeks later...yes really! Also then they'll be able to take my PICC line out (line going into my main vein, up to the top of my heart, a bit like a long term cannula). I have received the date of this op and it's in middle of September. They have said I should hopefully only need to stay in hospital for one week after, and I'll be holding them to that as I am SICK of hospital nights! Im not going to lie, I am pretty scared but I've just got to think of how much this is going to help! Stay brave and get through it.

This week...again, I have to go to UCL to see my consultant on Thursday. My bowel is in a pretty bad way at the moment, so the surgeon I saw who's doing my upper GI surgery also wants to be at that appointment too so we can discuss everything. Things are tough at the moment and I'm really starting to struggle with  pain and sickness. It is so draining and some days you just want a break! But you've just got to keep you're chin up and keep trooping on because things are changing and for the best!

Bring it on...

Well what a busy few weeks its been since my last blog, so I apologise in the lack of typing... nothing ever goes straight forward thats for sure! But one must keep their chin up and keep pushing on because being negative doesn't help anyone. I'm still on the TPN, and still have nurses come around everyday however only once a day now, as they've taught me how to disconnect from the TPN in the morning. Which is nice as I can finally have a lie in and not be up by 7am 7 days a week! Hello lovely bed! I must say I've had no problems with the nurses and they're all really friendly and helpful however, the first dressing change of my PICC line, the line moved a bit but the hospital was happy for it to still be used. Then...one week later on my second dressing change and the line come out quite a lot, causing me to having to get in contact with one of my consultants locally. Unfortunately this happened on a Friday meaning I was unable to use the line for TPN over the weekend and then on the Monday I had to go to East Surrey hospital for an X-ray, where they concluded that the line had come out too far and I had to have a new one put in. A little frustrating as the nurse should have been a little more careful. But apart from that I wouldn't say a bad word about them as they've been so good apart from the particular circumstance. I don't know if anyone else reading this has Calea nurses and think the same?

I've had two appointments since my last blog. The first one was with the nutrition team and they wanted to make sure everything with the TPN was going well etc...and it is so thats all good. The second appointment was an important one and that was with the surgeons. They have come to a conclusion that I need to have surgery on my Pylorus (opening into the intestine) as the nerves around mine have died causing me to get really bad sickness and nausea and not being able to eat solid foods. In total I've had five dilations and two botox's to try and help the opening stay open however these only last a couple of weeks now. There's two ways they can do the op firstly, doing a bypass and connecting my stomach to the small intestine missing the Pylorus, or the other way is by slicing the Pylorus in half and stitching it the other way making the opening permanently open. Unfortunately they can't do this via keyhole and have to open me up, which I'm a little upset about as it'll be another scar to add to the list, but when you think how much this is going to help me a scar is nothing. Also while they're in there they're going to put in another JEC (feeding tube into the intestine) meaning that the TPN will be stopped...Yay! A JEC is so much easier to look after, and I can do all the setting up etc myself as I've been taught before when I had a JEC. So I won't need the nurses to come around everyday which will bring a bit of normality again. So a few horrible weeks coming up to say the least but I've just got to stay positive and keep going.

On a brighter note, this week ed took me down to devon for three nights. The hospital gave me a couple of days off the TPN which felt amazing as I was free and wasn't waiting around for nurses! We were blessed with the weather and it was the most perfect rest-bite for me. After going to the peaceful, beautiful beach, I feel my mind is ready to bring anything on again! So bring it on!!!

So on that note, I hope everyone is having a lovely bank holiday weekend and remember to enjoy every second because you don't know whats around the corner! x

5 Weeks Later...Home, Sweet Home.

There really is nothing better than your own bed...especially after being in a rock hard, boring, cold hospital bed! I've been home now for a week one, just over and it wouldn't feel more like bliss. I was in hospital for nearly 5 weeks...so when they said I could go home, I didn't hang around that was for sure! Even though I'm home, I am home with the TPN which I have nurses came around twice a day. Once in the afternoon to connect me up, and again in the morning to disconnect...bit of a bore, but I've just got to think its only temporary and then its done...and I'm holding the doctors to that too!

Before I was discharged I saw the surgeons who have told me the plan. Firstly, after 2-3 months on TPN and my weight is higher then they can go ahead with the surgery. Firstly, they want to put another JEC in (feeding tube directly into my intestine). I've had one before which I had for just over year and half, but then is become dislodged and fell out and and this was the start of this story! Also they need to take another chunk of my intestine for a biopsy as they last one they took came back abnormal. Lastly, not sure if you've read any of my previous blogs before, but I have mentioned about having dilations of my pylorus as the nerves around this area have died causing this section (opening into the intestine) to be closed and very tight. Causing oral foods great difficulty getting through and a lot of nausea. However after having another endoscopy while I was in, they found it had got really bad again and dilated it again. Making it in total 5 dilations and 2 botoxs in the past 5 months. So they have now decided they want to do a permanent solution to this. This will make it permanently open and I won't have to have dilations anymore! ...yaya...they're so horrible. So its turning out to be quite a big op, but all these things need to be done and they will make things slightly bit better. I haven't got a date through yet but I'm seeing the consultant in a couple of weeks so I'm hoping they'll book the op then.

who says you must rest when you come out of hospital, because I for sure haven't been sticking to that really! ...oops. Well...to a degree, Ed has just brought a new apartment which he moved into when I was unfortunately in hospital, so now I'm out we've been putting that little homely touch to the place.

Hope everyones had a good bank holiday weekend, and I'll keep you all up to date when I know more!

Today's picture is shortly after coming around from sedation after having a anther dilation and I get a camera forced in my face by brother Matt and boyfriend Ed...say cheese!

2 Weeks In...

Day 14 of being in this hell hole also known as UCL hospital. I have been here for two weeks now and todays the first day I've been well enough to finally to get on my laptop and do a bit of typing away for a couple of hours. I'm not going to lie, these past four weeks have been pretty dreadful and I have finally ended up back here in my specialist hospital for more treatment. If I take you back to a couple of months, when my JEC also known as a feeding tube into my intestine become dislodged which caused huge amounts of pain, but then nature took control and it eventually made it fell out. Since that day I was waiting for surgery for a new one to be put in and another chunk of my intestine taken for a biopsy, due to the last one coming back abnormal. However...long story short, but I waited and waited and unfortunately a couple of months went by, without no nutrional feeding which eventually made me very poorly and lost so much weight that they are now unable to do the surgery, as I wouldn't make it through...yes, proper rubbish luck right there. I then saw my consultant in clinic who couldn't believe the mess I was in. She said I had to be admitted as soon as possible because I was in a critical state and my organs were at risk of starting to shut down. I finally got a bed up here at UCL where they put a PICC line into my arm, (a more permanent line into my blood stream) and I've been started on TPN. (Intravenous Parental Nutrition) basically getting fed straight into my bloodstream. It's very high risk, and when I had this a couple of years ago I got an infection from it and made me very poorly, so this time I'm sleeping wth my eyes open! My bloods were all other the place and my white blood cells were low but they believe this has happened due to maturation and hopefully with TPN they should improve. So I'm currently attached to a stand pretty much every second of the day, but pushing it around is a work out in its self! The plan was to be on the TPN for four weeks and then to be should be strong enough to have the surgery however since being in here the situation has changed, and they're talking about possibly going home with TPN for a couple of months and then coming back for the surgery. I must admit I a bit annoyed as going home with it isn't straight forward, as they have to involve district nurses etc but if it has to be, then I don't have a choice and I've just got to get on with it and come out the other side! Also since being in here they're trying to get the pain under control as well as the sickness as they've both got really bad the last couple of weeks. Apart from that, thats all to report at this stage. Still on a very long road ahead but I've just got to keep positive and keep going! Friends and family have been amazing, and they've kept me going. Below is my brother Matt and I standing by the only good thing about this place and thats the view, being on the 13th floor has its perks!...if thats even possible! Have a good week everyone and I shall keep you all up to date as best of possible x

January Blues

Well... this year has got very ugly very fast. January blues really are true for me...or should I call it January is doomed. The only positive is that this time last year, I was a inpatient at UCL and was in hospital for 5 weeks starting on New Year Eve...couldn't have been more of a horrible time. However don't get you're hopes up... i have already annoying been in hospital this year but thankfully for only a couple of days...still just as bad though. I'm sure each time I have to go in it gets worse and worse. Maybe its because I know how rubbish it is in there...24 hours feeling like a month and the worst bit I find is that is I hate feeling stuck in there...such a horrible feeling. But you've just got to keep going and hold your chin up because being negative gets you nowhere!

Now let me bore you with updating you on everything thats going on...ok this could sound confusing, so be prepared. After coming back from an amazing couple of days away in Austria in December, the next morning I found myself sitting outside my consultants room...which couldn't of felt more different from the previous days. My JEC had become extremely painful and I couldn't even flush it without screaming the house down...and thats no exaggerating! I was told it was infected and I was put on a 2 week course of antibiotics. The following week, 4 days before Christmas and I had an appointment with my surgeon for a follow up of the surgery I had 4 weeks prior. By this point I had a very odd lump appearing underneath my skin next to the JEC. He took one look at it and straight way said it was definitely not right, shortly followed by him giving it a good push...typical doctor of which he was lucky I didn't slap him for as it was so painful. He came to the conclusion that the JEC had become dislodged, meaning that the flange (hate that word) had come out of the bowel and was digging into the skin wall from the inside. I was told this meant I had to have surgery again...my heart sunk to the bottom of the floor. what he would do is to take the old JEC out, sort out the bowel and put a new JEC in. It seemed like I didn't have a choice and I just had to just keep going as it needed to be done. At that stage he said stop using it...unknowing I was unable to use it anyway making it 2 weeks without feed from that point. If you're like me, and you have to rely on it nutritionally because feeding is the main source than you know 2 weeks is a difficult time. I am also very limited on what I'm aloud to eat orally at the moment with the unsuccessful dilation of the pylorus I had, all I can have is fluids/jellys/fromage frias/soup and supplement drinks. This all couldn't have happened at a more worse time of the year because it meant that everything had slowed down to a snails pace with which felt like every doctor going on leave over christmas. However,  a couple of days later on, on Christmas Eve and nature had taken control and the JEC had actually fallen out...you could say I gave birth to a JEC? ...we couldn't believe it! I luckily got an appointment with the surgeon 4 days later, where he obviously said I still had to have surgery but now just to put a new JEC in. This was classed as an emergency and I was put on his following Friday list. He enforsized that I needed to have 2 or 3 supplement drinks a day because each one is 300 calories. This had to be a must because I had to be strong enough to get through the surgery and also I wouldn't heal too. One week later and I unfortuently was admitted into hospital. I had not been a feed for 4 weeks, was very dehydrated and my body was really struggling and I had no choice. However things had unfortuantly got too bad already and I was told I couldn't have the surgery because I had lost too much weight and wasn't strong enough to get through the op...I was gutted, and was so upset because all I wanted was for the op to be done and everything to be sorted so I could get back on the road. I was fitted with an NJ feed (a tube down my nose)... which I hated even more than the time I had one before...if that was anymore possible? I was told I had to have one for a couple of weeks or until I was strong enough again to get through the surgery. Thankfully I was aloud to go home with it, which was amazing to be free again however I hated having a ugly tube on my face. So many people on the outside world really do have a good old scare at it and I know I should just ignore them but its so hard. I don't think they even realise they're even doing it...really I should say 'yes was there something you wanted to say?'...I would be a millionaire for every penny someone looked. But you must try and not worry about what people think and hold your chin up and keep going. I just kept thinking its only temporally. Two weeks on and things have gone down hill even more. I saw my consultant in London who firstly, has said she wants to try another dilation. This time for it to be done at UCL and to be done by a consultant who has successfully done it before, so keep everything crossed that this time it is successful. Secondly, she said she wants me to have the surgery for a new JEC and another biopsy taken as soon as possible. Frustratingly the last section of bowel they removed for a biopsy was 'blurred' from inflammation caused from the dying bowel they removed. Making this section of bowel its 3rd biopsy! Currently, I'm in constant contact with UCL because ok, I admit things really have gone down hill and I couldn't feel anymore poorly than I do. Everyday life has become such a struggle but I'm trying my best to keep going. Everyone is being so kind and caring and I couldn't get through each day without them. In times like this, you must really try your hardest to stay positive because negativity makes everything even more of a challenge. If you're going through a difficult time or experiencing anything I have mentioned then please don't feel like you're alone. I'm always free to chat or help with anything you may need.