Easter Holidays, Tattoo and Tests!

So ALOT has been going on since i last blogged!

Firstly i fractured my ankle on the 2nd to last day of term, why is it always me?!? I was going my normal speed of 10mph in my chair and the eletric door sensors didnt pick up i was there, so the door closed on my foot, sheering of my £500 footplate and i am lucky to get off with such a small break! Anyways that was nearly 4 weeks ago and i have my foot out of my cast boot and its healing well - the Bone doctor told me this morning :) so yay!! Thats over with now! One Less thing to worry about!

Something more exciting now! I got my first Tatto on Friday! It took about 20-30mins and its amazing! I LOVE IT! I got the word 'breathe' with some butterflys - which always reminds is me that even though life with 26% lung function is hard, you have to appreciate each day as you will never get it back, and life is as delicate and beautiful as a butterflys wings. So while its still beautiful, respect it. Ok enough of that soppy stuff haha. And the word 'Breathe' is a bit obvious....
 So here it is! :)




Yesterday I went to my Muscle hospital - The national, Queens Square in  London. I saw my consultant and we spoke more about what i could have and he thinks its something i have been born with, i had 8 vials of blood taken! Yes... 8!!!
Looking back at my muscle biopsy it had glycogen in it, which suggests Pompe disease but without testing my blood to see if the enzyme is there or not we dont know. So im waiting for that result. If it comes back low, then i will have pompe disease which has been suggested to me and my family in the past. He also took some DNA samples again to check for other inherited conditions like MD and other genetic diseases. I have now been steroid free for 10months and my muscles have not improved at all, infact my hands are now weaker, though i hate to admit it! So this means that my muscle disease is not caused by steroids, and if it is i have had a severe reaction to them that has never been seen before, which is very very unlikly!! All the people who had steroid myopathy diagnosed, when the steroid treatmet stops the muscle returns very quickly, the latest is 6months and i am way past that now. So it is very unlikly to be that. So i have a lot to think about! I just hope one test comes back with a diagnosis for me! It would be so much easier!! I am also waiting for a 2 hour MRI Scan of my neck, full spine and thigh and calf muscles to see if they are built 'normally' and to see if my spine is ok due to my bladder muscle stopping working!! - worst part of my whole condition LOL So yea.... hoping the test comes back with some awnsers :)

Thanks
Chloe

Lil Op happening really soon = happy Chloe

Hello :)

So i am in a happy positive mood today, whoooo :) Its only 2 days until the Easter Holidays! - this means lots of chocolate, time with my sister and family :) AND I'm seeing P!nk LIVE in a few weeks i really cant wait! Im also seeing One Direction  next week.... im sad to say i have to suffer this tourture hahaha but its for my sister so seeing her happy is worth it, i love her so much and knowing i cant be with her for her whole life upsets me a lot. Anyway its a positive post!!!! I had my pre-op meeting with the anesthetist this morning and it went really well i think, we are going for a light sedation so i dont have to be put onto a vent, just my bi-pap but she said if my oxygen levels drop too much then i will wake up in the ICU and get weened off the vent slowly. We chose this so my lungs have more of a chance to not need ICU! I knew this anyway so was not suprised. To be honest i am going to do lots of extra physio, antibiotics and nebs so my lungs are the best they can be for the op and hopefully i can go home the day after or the same day! The only bad thing is that it means i have to wait for an ICU Bed to be free for me to go into afterwards... this could take a while :( But i did say i need the op as soon as cuz i am very uncomfortable ALL the time now :(
So after i will have a tube coming out of my belly just below my belly button, which will be 10000% more comfortable than the tube i have now!! and it means i can wear my skinny jeans again, yay!! haha no more leg bag! :) haha the small things make me happy.

So, yea im hoping to get a date for the op through very soon and i will update when i know anything more! here is a link to some info about what im having if you have no idea... most people dont talk about bladder issues but LOADS of people have them, so yea.... i dont really mind posting to you about it, cuz its part of my disease progression and if you know about it you wont be shocked if you see it one day poking out of my shirt lol

http://www.bladderandbowelfoundation.org/bladder/bladder-problems/suprapubic-catheter.asp


My lungs are quite good at the mo... :) still get the random pain but im used to it now, if you need to you can get used to anything! Im on extra antibiotics cuz i have a joint infection.... random i know and only i could get it haha but its getting better now thank God!

Thanks for reading!
:)

Chloe

Transplant talk - in limbo

So yesterday i saw my main consultant at the Brompton and we talked about transplant....
It was not good or bad news, kinda in the middle.
So basically if my lung function stays stable at where it is now i have over 2 years to live (whooo!) This means its not worth taking the risk of transplating me yet, (in his eyes) but it will be reviewed every 3months and like normal i can contact him anytime if i get sick or want to ask questions or anything. But the thing is, when i get a chest infection i ALWAYS get really sick... like high C02 and lower oxygen levels than normal and it always causes a decline in lung function which i never get back. The risks after transplant are high but for me its quality of life over quantanty. I would rather have 5 years of a great life doing everything i want to do with little restriction than 10 years stuck like i am now needing a venitilor to breathe for me 12hours or more everyday and oxygen just to keep me alive..... In my life time i have already done more and gone through more than most people do in 50years so its definite in my eyes that quality is much better than being a total bum for a whole 'average' life hahahha
My consultant is also hoping to start me on rotated colomycin and tobramycin nebs instead of just the tobi which i think will help keep the lung function!!

I have also been of steroids for a total of around 8months! and from my heaveast i have now lost just over 14kg!! But the bad side to this is that the experiment failed... my neurologist said that if my muscle disease was from my medication, after 3months of stopping it i would see improvement and a good improvement after 6months. Its now over 8 months and i have declined since i stopped the medication. This proves that it is not the cause of my muscle disease and that it is in fact, and in their words 'organic' muscle disease. I am seeing them in April so this is not confirmed but by going by my last clinic letter this is the outcome. My muscle biopsy also showed my muscle had glucogen in it... this may me a clue. But im not saying anymore because thats all i know (well whats in the letter) and i dont know what to make of it.

So if i do actually have a muscle condition it will stop me getting a transplant.... which scares me A LOT now!!

But to be fair everything in life is uncertian and i suppose you just have to live for the day and get on with it - with occasional days of feeling sorry for yourself haha Oh and not forgetting humor! thats how i deal with EVERYTHING!! make it funny and its ok ;)

I went to a UCAS fair thing today, looking at uni's and stuff and i think i want to go to Imperial College London, best in the country for Biomedical science i hear ;) but i need three A's in my A-levels!! OMG!!! but i can always try! i getting A's and B's at the moment so you never know! I might even find a cure for myself and My friends, just maby - you know anything is possible if you try!


Chloe :)

Sorry It's been sooo Long!!

I have not blogged for ages!! So much has been going on, plus my laptop broke so yea!!
Jan was a fab month - because it was my birthday ;) lol it was nice, i had a meal out and saw loads of family that i dont get to see much so that was great! I also was in the hospital, again for antibiotics but it was not for too long so it was ok, but im going to have to have an operation soon to put a tube into my stomach to be able to drain my bladder :( quite nervous lol i know i go into hospital all the time but operations are really risky for people with bad lungs/ low lung function so thats whats scaring me most of all!!! I have to have this op because my bladder has stopped working and i actually cant pee!! So at the mo i have a normal cath so i cant wait to get rid of it!!! This is what it will look like after the op. ( found the least graphic image i could lol) its just below the belly button.




Ok so Feb... i have been in hospital for 2 weeks of IV's AGAIN!!! :( So annoyed with my lungs! they cant really cope off antibiotics which worrys me a bit but i feel great now (i have been off IV's for 4 days ish) But the good news is.... I GOT MY PICC LINE OUT!! Beacuse my port is working again! soooooo happy about this!!!!!!


 This is a PICC Line



This is a port being flushed, its under the skin so no one can see it apart from a scar and a bump under the skin when its not accessed! Its so much better than PICC lines and canulars!

(photos from google cuz cant upload mine for some reason :/  

So its half term... im not going to stress and so any work until i go back to college!! i need a break haha So im gunna go put on my bi-pap and go to bed now :)

Night Night :P

Christmas Eve Eve!

Hello!!!
I have escaped from the hospital yay!!! I did end up going in as an emergency... was drowning in gunk and physio was not working so i was admitted via resus, suprise suprise. I had a really cool nurse though, so that made it a bit better. I was moved to the CF unit which made the stay a lot better, but was not there for long as a CFer had to go in for IV's too :(  Eventually My infection markers went down and i was finally allowed home 7 days later. 
I had a complication this admission which i am very annoyed, puzzled and worried about. But mum has put me at ease so hopefully it will sort itself VERY SOON! Im carrying on with the antibotics at home which i finish on Thursday :) And i feel better! :)
So Christmas should be great! Me and my sister went shopping the other day and it was SOOO BUSY! But we got what we needed and then relaxed in costa while mum got her hair done! :)
I shall leave you with pictures from my admission and a video of my room... haha i get bored as you can tell!!

  Me, my sister and the Nurse LOL

 Me and Bruce :)

 Me.... hahaha - sad face lol

My sister and her POO CHART HAHAHHA

Nails :)
 

 Bored..

 Bored...
 

 Bored.....

SNOWMAN!

Top, second and 5th highest scores on gems... haha

BORED....

Helicopter on the hospital roof!

 

Hope you have a Merry Christmas and Happy Healthy 2013!! I love Christmas! :)

Im so Lucky.....

Hello!
Well the winter is trully here, i have a cold! Well its the weirdest cold i have ever had....... had 2 days with just a sore throat and was fine. Then  woke up with a blocked nose and a belly full of air becasue my Bi-pap must of been trying to force air through my blocked nose and somehow pushed the air into my belly- dunno how that works though! So then i was really out of breath, but have been getting more and more breathless the last few months anyway so just ingored it and thought the blocked nose was an allergy or something. Then came a weird chestyness - wheezy but with crackles at the end of each breath. My mucus is a lot thicker than normal and my normal is thick as so i am completly filled with real thick gunge that does not shift!!! I have intense coughing fits which do nothing and im getting so tired now. I feel spaced out too a lot of the time too and have a temp. Im on this weird antibiotic called Moxiflocacin, which loads of nursers have never herd of - to be fair neither had i until Brompton gave it to me! Im having extra physio and im trying to do active breathing when i can to shift it, i feel so breathless i cant even eat! So feeling crappy :/ Oxygen saturations were down to 87% this morning but cough assist got them up a bit.

My best mutant friend Lucy has been looking after me though :) Love my Luc :) She is amazballs :) haha

So yea - hope i feel better tomorrow, if not i think i will go in for IV's so i can be well / out before christmas, i dont / cant be in hospital for Christmas AGAIN!

On the bright side my letter has come through from my consultant saying they cant stop my physio which is great - hopefully they will have learnt that from this bloody infection; to be honest its probably partly down to that!!!! All that crap building up brewing bugs in the bottom of my lungs that has now been infected by cold germs is gunna make the situation a lot worse for me now. Im so annoyed at them!! Grrrr haha anyways if i end up in ICU mum will get loads of money haha. SUED! :D 

Anyways I Hope you all have a fab christmas!!! and a healthy happy 2013 if the world does not end ;) lol

Unless you get it... you really dont get it.......

What people dont understand, they dont / wont believe...
I Cant say i really blame them, if i were them it would be hard for me to believe too. But then again in this particular situation i would probably look a bit harder to find the truth. Listen to the person going through the daily life with a complex condition nobody really understands, and because no one else has it nobody knows what will happen next.

I have kind of had enough of people just telling me to 'stop giving them all that dying crap' when i say i cant breath and need physio and my ventilator on. Because quite frankly if you dont have 26% lung function, then you dont have a CLUE about how i feel everyday and that when i do ask for help i am actually deep down terrified that it will be my last infection, my last fight. I know it sounds dramatic and stupid; but to me and my family who have been told that i need to fight and keep going when im in ADU / ICU its pretty darn scary. Knowing that a chest infection could potentially make me pop my clogs (lol) is always niggling at the back of my mind. And unless you actually get my disease... you just dont get it. No matter how hard you try you will never fully understand. Understand how dam hard it is to get up when you vent is taken off every morning and takes your breath away and you spend the next hour trying to regulate your breathing. Understand how by 3pm your lungs feel so stiff, painfull and heavy every second but you have to put it to the back of your mind and carry on. Carry on and put a huge smile on your face, forget the struggle and live for that day because you can never get it back. Some days it gets too much, the pain gets so bad you actually cant breathe and the only thing you can do is take morphine and sleep, sleep and hope its over when you wake up. Everynight i wake up multiple times - sometimes i pretend im asleep from the night staff haha and i just think - what if this is all just a dream. Ill wake up soon and be a happy healthy 7 year old - or at least be at that stage of my condition before it effected me much.

I really needed to write this, get it off my chest haha how ironic! Anyways... the last few months its been bothering me a lot. Thinking about transplant and how likley  it is for me to get one, college is getting to me a bit and because im so tired all the time im taking things to heart a lot more. Things that i may be taking to personally, that actually dont mean anything at all. And i am going to write about this person, not going to name them because they know who they are, and they wont read this so why not! Basically one of the people i actually trusted a lot is now avoiding me, telling me to shut up with all that dying crap when i asked for help as i could not breathe... and i am basically quite upset about it. Its like i can see her true personality for the first time. But i dont want her to be like this, i hate it!!! I would much prefer to get on with everyone but in life that just doesnt happen. I thought this person new me, new that i would never and actually could never make up being a bubbley drain pipe lungs! I just dont get it.... as i said im soo tired all the time these days that i am probably taking things way to personally. But its just how i feel and this blog is my way of getting it
 out of my system!! haha even if nobody reads the crap haha!!!

But yea... i do have some amazing people around me, mainly my family but also my friends and actually some staff are pretty amazing too - they know who they are :) So yea..... im really really tired so im gunna buzz to go to bed and maby, just maby i will pluck up the courage to give this blog address to my house manager. I would love her to understand and known how im feeling but i cant say it, i have to type it.

Ok Bye!!! haha
Love you all ;)

And yes im thinking up my next naughty plan to cause mischeif in some way. It makes life more exciting ... mwhahahahahahah :)