Friday, 16 November 2012

4 months today since started Kalydeco!

Bonjourno all!!

so today it has been 4 months since i started the wonder drug that is Kalydeco!

Ive not been feeling my best at the moment, but in the world of CF the whole lot of us always need a bit of TLC before Xmas, with all these bugs around and obviously none of us want to be in hospital for Xmas, we avoid it like the plague!! nothing more depressing than that!! Ive done it a few times or managed to escape Xmas eve!!
Anyway even with special k threes not escaping this!!

so realistically Ive been feeling unwell for about 3 weeks but with the wedding and everything Ive been putting it off, only this last week Ive resigned to the fact its time to get an mot! lol

i informed my team before coming up that i was unwell and needed to be admitted, and just on the off chance they might have a spare bed for me today i went to clinic prepared with an over night bag with the essentials!

got to clinic and signed in with my new name of Mrs Compton (still odd) haha everyone congratulated me, as they saw my name had changed :) and i was put into a room.
Had my weight done which is by my surprise has stabilised for months now without making any effort of 38.6kg, considering my appetite has been rubbish and sometimes eating one meal a day its pretty clear that Kalydeco is working its magic!

then the physio came in, new lady id not met before, sats were 88-91% so not great but could have been worse i wasn't on oxygen or anything and had been walking around.
next lung function and last time i was in clinic was 2 weeks post starting Kalydeco and i was on a massive high then feeling the best id ever felt and my lung function was 23%
did my blows today and almost felt like dying throughout it, but too my surprise it come out at 23% :S
couldn't believe it, mum and i had guessed before it would be about 15-18% because i was so breathless walking around and just felt awful!!
i was shocked... messes with ya head to think i felt that unwell yet my lung function was coming up at its best since K...

so off she went, then the dietian, was the usually bla bla bla you gotta eat more do ya ng feeds bla bla bla i know all this but im rebelling on doing feeds at home because i don't want to cough and chuck up everywhere on my lovely carpet in bedroom in case i don't make it to the toilet...so i just said il promise to do feeds in hospital, don't care if i chuck up there haha nurses can clean it up for me! plus even more reason to get them to try get me in asap!

then dr came in...was just telling how i feel, tired, out of breath, needing oxygen more, hadn't left the house all week, having a bath tires me out, just the usually chest infections symptoms...so I'm on the list to get a bed...but realistically it will be at least a week but no longer than 2 weeks before i get in!! which is crap, i have my harefield appointment in two weeks and wanted to be at my best for that so i can talk to the team and not make any rash decisions etc... i explained this and she understood.
i also spoke to her about the transplant and my feelings about it.

my minds in a bit of a fuzzle, this time last year my lung function was 16% id had a rough year after my lung collapsing and all i wanted was to get on that transplant list and get me some new lungs, i kinda went in knowing nothing about it as well, naive really....a year on having dipped so low and gone so high after  being given this new drug and not needing to be in hospital since its all a bit crazy. without kalydeco whos saying i would even be here now without a transplant. but i know and have heard so many stories about transplants now the good, the very lucky and the bad and im terrified now, i just literally dont know what i want! and the fact my lung function is so good (for me) even though i feel so unwell just makes me think well what will i be like after 2 weeks of ivs in hospital with all the best help around me, get my feeds inside me to improve my weight just max me out on the best quality of care...could i reach my target of 30%??
if i was 30% why would i put my life at risk of the chance of a transplant going wrong when my quality of life is stable and good?? you got to remember ive been bad, really bad so although you might read this and think ok what about having lung function of 100% surely thats better.... yes its the obviously choice but like all these things it comes with other problems etc, i dont think im ready to take that risk again yet.
plus once i have a transplant i cant take kalydeoc anymore because of the anti rejection drugs, so although lungs would be good, cf could start affecting the rest of me, ive been lucky so far in that sence!!

also people who have had transplant have never experianced kalydeco, when i sit back and think about it, its not just helped my lungs. this is naughty of me but i want to be honest...im rubbish at remembering to take my vitamins and some tablets some times and before special k if i forgot to take them i would get sores on side of my mouth.....i havent had that since starting k!!
dont have any tummy problems...

my doctor also told me about a cf confrence they attended in america in october and the main subject was obviously Kalydeco (ivacaftor) and she said they showed a slide show of cf lung damage before starting k and then after being on k and the damage started to go...and once k was stopped the damage started again.

so once ive been admitted as an inpatient were going to do some research into me!! lol xrays, sweat tests and trying to get them to do a ct scan just to see what the old lungs are really like....might have to fake a chest pain for that one ;) hahaha

yeah so just waiting for a bed now and no doubt once i get in il have plenty to moan about ha bordem and the smallest things annoy you in that place :)

laterrrrzzz lol

xxx

Monday, 12 November 2012

hello winter!!

Ok so now all the exciting stuff has happened in my life its time to get back to reality....and quite frankly it sucks at the moment.
My lungs have turned on me this week and im finally admitting to myself that im not well and defo need to be admitted into hospital!

typical signs, no energy, sleeping more, needing oxygen most of the time, cant just walk around the house without struggling to breathe, the cold weather literally wipes me out so trying to avoid going out at the moment, sleepless nights where i wake up in the middle of the night coughing my guts up then literally takes me hours to settle back to sleep again...theres no such thing as laying flat to go to sleep at the moment...and 9 times out of 10 i tend to wake up coughing because ive slipped down off the pillows and lungs cant handle me laying flat!!

oh the joys of CF!! even with kalydeco i dont think i can hide away from the lovely winter infections!!

i dont want people to read this and feel that kalydeco doesnt work because it does, i am one of many that is extremly lucky enough to get on this drug, however i was at a point where i had no other choice but transplant to save my little lungs i wasnt going longer than 17 days out of hospital and so far ive done 4 months.....but kalydeco has stablised me and improved me from what i dropped too. in the end of the day i still have cf and there is alot of damage in my lungs, 22 years worth of infections after infections....if someone had given me kalydeco when i was 6 years old then i most probably wouldnt be in this situation now but thats life!!

this is why we need to support all the petitions and fight for this drug so that kids born now wont end up like me at the age of 22!! they will most prob live a life as near possible as any normal kid out there without cf!!

now that wedding and summer is out of the way, transplant is on my mind again. will be a year ago on friday that i had my first assesment at harefield and i remember feeling nothing but eager to get on that list and get some new lungs.... alots happened in the last year ive had my very very low points and then massive massive highs!!

ive learnt and spoken to alot of people who have gone through transplants and seen alot of close friends loose there life to cf aswell! ive seen people get there calls and do amazingly and ive seen people have a really rough time and struggle to get through the recovery stage and come out with other problems....
quite frankly im terrified about transplant now, this time last year i was scared because i didnt no how long i would last without a transplant but i was unaware of the ins and outs, yet now im scared because i dont know if im strong enough to handle it all! i know too much about it all now and its not always a good thing!

my heads in such a whirlwind at the moment. i have harefield appointment again in december so i think i need to see what the results come back with tests etc. and speak to them and my cf team and maybe make a decision in the new year when my heads clearer. i cant make a decision when i dont know how i feel about it all!!

crazy year!!

thats all xxxx

(119 days)3 months 27 days on kalydeco
 

Friday, 9 November 2012

first blog as...MRS COMPTON!!

Ive been meaning to blog for weeks now and kept putting it off and off, my life has literally been running at 100mph.

lots to catch up on!!

Firstly

Lungs have been a bit up and down at the moment, i think the stress of the wedding hasnt helped and i really could have done with admitting myself into hotel brompton for i.vs to give myself a boost but stubborn george refused to let CF get in the way, plus didnt wana loose my tan and every time i have my port acsessed it swells up and couldnt be having that before the wedding etc, so i opted for the less sensible option of just cracking on with things, but like a trooper i coped just fine ;)...

so my hen do was mad- i was in my own little world that night, we were drinking straight from 3pm until 1am but i was dedicated and lasted the whole night!! we went to dreamboys in london and dressed up in burlesque outfits...mine was more the chavi styley haha but was such a good night!!
heres some piccys.....



 
was good night! so thats was the weekend before the wedding...
 
then 

well i started feeling more chesty the week before and i knew i needed to take it easy but i ahd left so much til last minute there wasnt much time to chill.
im a true believer of "everything happens for a reason" and i think it was the tuesday or wednesday before the wedding on saturday i twisted my ankle!! - worse pain ever and i honestly thought i had broken it!! haha i literally stepped outside my mums front door and missed the door mat and my ankle twisted and i just dropped to the floor! haha i didnt no weather to laugh or cry i couldnt believe it, was in total shock. mum carryed me into the living room and was too scared to take shoes off incase i had a bone hanging out or something (ok little dramatic) well i did anyways and had a weird lump on the side of my ankle and freaked out it was hard and i couldnt move my foot so yes i thought it was broken, my sisters burst out laughing and mum and i just burst into tears i think i was mainly crying cause it was my wedding day at the weekend and after everything thats happened and being well enough to walk down the aisle without a wheelchair and oxygen now i just thought im gna be in a moon boot and look like a twat!! haha
Then my lungs turned on me as i got into the car i think it was the shock and the fact it was so cold, so off we went to A&E with oxygen and my wheelchair.
i was such a wimp i couldnt stop crying it was so painful and i felt sick at the thought of how stupid and minor i had hurt it.. so angry at myself!!

anyways was seen and as i calmed down whilst in waiting area i began to try wiggle my toes and it was a bit painful but i could do it so i knew i hadnt broken it!! so saw nurse doctor etc had xray and was all ok just a sprain, but was told to keep off it for 2 days and not put pressure on it and there was no medical reason why i couldnt where heels on the saturday other than it might hurt...will power got me in them heels!! lol

but although my ankle is still very painful i think it was a sign to stop pushing myself and rest up before wedding, i literally had no choose to i could barely walk!

always a drama it seems in my life haha but its actually felt nice to go to hospital for once instead of it being CF related....a definate first in my life!!

So that leaves me to my wedding day....

 i am now offically Mrs Georgina Compton! still trying to get use to that, as of the 3rd November 2012!
They say its meant to be the best day of your life- and it really was for me!! it felt amazing not only marrying my soul mate (sorry for the cheese) but my best friend too!! but i did it all without oxygen hanging off of me and not a wheelchair in site!!! although at times with a little hop!!  haha

the day was beautiful we were soooooooo lucky with the weather!! was a little chilly but the sun was shinning and was quite warm when on us!.. we had our little angels in heaven there with us looking out for us!!

i was so worried about my chest that day as i started getting quite emotional before arriving at the registry office, when i laugh too mcuh or cry my chest goes so tight and generally have a massive coughing fit!!, i thought alot about our loved ones who wouldnt be there but should have been there which got me all chocked up, i think i kept it under control and just wanted to see lee and get that bit over and done with. i hate talking infront of people.
it was all ok i changed my tears into giggles and laughed basically the whole way thru...i think the reg got a bit annoyed at one point, oh and lee called me his awful wife instead of lawful!! hahahaha

then we had a little buffet and got ready for the evening party which was so much fun and lee surprised me with a photobooth which was incredible and made the night everyone seem to have so much fun! would highly recomend people to get one for partys weddings or any excuse etc!!

www.photobooth.co.uk

now that life has calmed down, im going to concentrate on gettign myself well for christmas, going up to hospital for appointment next week, get myself booked in for a stay and a sprog mot lol and my next dilema of deciding what to do about transplant now wedding is out of the way!
my heads not in the right place to write about all that now so il save that for another day!
on a brighter note...
so heres some piccys i have so far waiting for all the proper ones from the photographer so no doubt il bombard my blog with that another time!! :) il try not to leave it so long next time!!

signing registry

 
our cake

 
Mr & Mrs Compton x
 
 
Me with my mum & dad x
 

 
My In laws x

 
jager bombs ha

The Compton & Petrie Kids

me and the hubby

 
groom bride bridesmaid and bestmen