How Things Change In A Year!?

Helllloooooo!!

so im sitting here in my hospital room and cant sleep so what better time to blog!

In my last blog i was waiting for a bed in the lovely hotel brompton!
iv now been an impatient for 1 week! :) and i have to say this is one of the quickest turn arounds i think i ever had in my whole life!! i was a completely different person after 2 days!
i almost felt like i was a massive "faker" but thats not the case my infection levels were 112 so there defo was an infection.
my lung function starting ivs was at 24% which is new score for me since starting Kalydeco! i havent used oxygen at all this admission...i dont even have the connections in my room for it, my sats have been 96% and my resting heart rate has been 74!! thats incredible, all this considering i have an infection!

knowone knows who i am, hardly anyone has seen me since starting kalydeco...there use to seeing a frail little girl in a wheelchair and on oxygen and who barely left her room the whole admissions...now they have me bopping around the ward at 100mph without oxygen or getting out of breathe, a different surname....and someone also poitned out ive changed my hair colour! so unless im face to face with people or i say hi first knowone reconises me or comes to say hello!

its been a massive eye opener for me really, i mean i know how much better i am but when i hear it form other people and see there shock on there face to see me looking so well you dont really realise it! someone said to me, they have never seen anything like this before...someone get so unwell and see them gradually decline to then improve sooooo much, without a transplant, but even then they dont see them post tx! so i really am a rare species now ;) lol

i always said i would prove them wrong and im proud that i have, and this is only the beginning!

i saw my consultant for the first time since starting special K and ive been wanting to see her since two weeks post starting k! thats when i felt so good straight away and showing off my new lung function, but even then everyone was a bit unsure about how long this would last or if that would be it...
she didnt give much emotions away but i knew she was so pleased, her attitude towards me had changed esp reguarding transplant, before i was convincing them that if i improved i didnt wanna risk a tx yet....but talking to her about it the other day and saying how i feel now about not wanting to go back on the active list was one of the easiest conversations i think ive ever had, she totally agreed with me, she can see my improvements and as my lung function continues to increase, even if its not 10% at a time every little improvement makes the biggets difference, it would be mad to go back on.
they said to me before starting this drug that it was here to stablise me and not take me off the transplant list, im so glad i proved them wrong!
(bit of rank mucus talk but if your interested in the changes of kalydeco its interesting information!)
i also told her how ive been bringing up lots of plugs, thick hard bits, she thinks now that my little air ways that have been clogged up with really thick mucus are now starting to move and unclog because the cloride or something is losening it up. so if that is the case my lung function should increase or thats why my lung function has increased and why i can breathe alot better.

i find it facinating how this drug is working in my little body, and im so thankful that ive been given this chance, its basically saved my life, without this drug who knows if id even be here right now after the way i was declining....my lung function had gone to 12%..thats 12% of my lungs i was using...makes you wonder how that is even possible? how could i survive with such little use... one more bad infection and it could have killed me...i cant even imagine that...and even knowing that this information is true i would have laughed at you if you had told me back then, im very strong minded and would never have given in!!

thinking back to this time last year i was going through my assesment and i was still adjusting to life, i spent alot of time indenial about my decline in health, from an outsiders point of view it prob looked like i dealt with it really easy, but i really didnt.
i use to cry myself to sleep at night worrying about what would happen and the risks of transplant, sometimes i just wanted the floor to open up and drag me in, i didnt let on because i wanted to be strong and didnt want anyone else close to me or anyone to worry, and if i didnt cope well then how would they?...but i just wanted to be a normal 21 year old, i went through a stage where i wouldnt go out the house. i didnt want to be in a wheelchair or on oxygen, people would stare at you and give you that feel sorry for you look- i HATED that, if i wanted to go shopping i would get my mum to drive us miles away out of the area where i didnt no anyone just so i would feel a tiny bit more relaxed and no i wouldnt bump into anyone, it wasnt even the fact i hated having it all so much, i just didnt want people to feel sorry for me or treat me differently or ask what had happened. just wanted to be normal george!
then other times i would just rebble and not use oxygen which would make things a million times worse, putting strain on my lungs didnt help and one 10 minture trip out with nothing on would end up making me house bound for days after.
everything was such a struggle and the hardest part was getting my head around it all. it all seem to happen so quickly, my decline was rapid form my lung collapsing in summer 11 to being on the list, scary really looking back- i eventually gave in and just went out with it all on, gave up even caring what people would think! still felt major awkward when i saw anyone i knew though!

then there was other parts of my life i wasnt happy with, i hated living in those flats, it was such a struggle trying to get from the carpark to my front door...and i refused to wear oxygen which again didnt help! i wanted a dog which i couldnt have in the flat, i wanted a little gardeni wanted my own front door, wanted to make my house my own and decorate and i couldnt do any of this. cause i was so unhappy with that, it didnt help with health either!

so looking back over this year my life has changed more this year than it has in the last 22 years!! lol everything i wanted in life i got in the last 12 months!
i got a beautiful bungelow
i got a gorgeous puppy
i got engaged
i got married
i got to go on holiday again
and obviously most importantly i got this amazing drug which has improved my life
and now im off the transplant list :)...FOR GOOD

all of this aside wanted to explain the realistic side of things too with kalydeco or not so much the drug but my life style.

people might think oh how comes shes in hospital again if she on this new drug that makes her well.
some people can be quite niave sometimes. dont get me wrong as you all know from me saying how amazing i feel which is true. but theres also the fact that i still only have 24% lung function compared to the normal 100% i could have if i was normal lol although im alot healthier than i was, i still have Cystic Fibrosis and unfortunately there is alot of damage already there that no matter what amazing drugs they make i cant undo 22 years of constent infections. so i do still get days or times when normal day to day challenges are hard like walking around or washing, and even if ive been out all day walkign around fine and coping fine...it means in that evening or next day im totally wiped out!
and im never the person to let on how much im struggling....even when its completely ovbious i am, you ask me and il reply "im fine" ha its like a reflex reaction lol

anyways just thought id add that little bit because i dont want to think its all a complete transformation because there are still days that are bloody hard work and i get the days where i think sod iti cant cope, but i wouldnt change my life for anything and if i can carry on like this how i am now for rest of my life without declining, then id rather be like this than have a transplant! because this life style im use to and it comes with alot less risks than tx :)

thats all from my little head tonight.

im having a ct scan on monday to compare my lungs from before kalydeco to now, i asked to have it caused im interested to see if theres obvious changes, hopfully il get some piccys of it, il persuade the nice drs or night staff to print it off or email to me :) oh and got to keep an eye on my sugar levels, had a little hypo yesterday my own fault hadnt eaten much and they dropped to 2.6...dotn really understand all these sugars etc but the nurses told me off for not letting them no, but they were busy and the only nurse that was free i hate with a passion so id rather suffer than get her help! haha

anyways laterzzz



 

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

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
 

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

 

Long time no see!??

Been a while, seems like blogging tends to be more interesting to me when im more ill, so i suppose in that sense no news is good news??

Been quite busy inthe world of sprog!

celebrated a mates birthday a couple weeks ago, we went to essex for the night, visited a club called sugarhut! heres a few piccys, was good night...doesnt take much to get me drunk these days as iv learnt...cheap date ;) lol

So i had a lovely holiday! forgot how amazing it was to be away! sun, sea and sand! the feeling of the sun on my skin was so much better than 10 mins on a sunbed ;) haha

I felt like i was back where i belong! the place we stayed at was a beautiful little town called Puerto De La Duquesa, Andulucia Spain! between gibraltar and malaga airports. Iv been going there since i was 13 years old as my auntie and uncle own an appartment there!
once we arrived i couldnt wait to get my first Gambas Pil Pil, for anyone that doesnt know what it is or has never heard of it or tried it, wellyour simply missing out on life!! haha
its the perfect Cf dish and great for taking Kalydeco with!
its prawns with garlic and chilli in oil and got to have chips to soak in the oil and bread to dip! pureeee heaven! and i made the most of the week eating it at least once a day!
heres a pic

the last time i visited Duquesa was the end of may 2011 just before my lung collapsed! it was the first holiday i had used a wheelchair to get around and first time using a wheelchair in public really. i was at he beginning of my decline in health.
whilst at my favourite beach bar opposite our appartments the little spainish man who works there "spongebob" as myself and family liek to call him, he said to me as best as he could it went somehting like this....

"whena i see you", "ermmm" "you aaaa" "make meee" and he smiled and pointed to his face.
"when i see you before, you aaaa in" and did a wheelchair motion...

im guessing by this conversation he was basically happy to see me up and about walking around and not in a wheelchair like last time!!

after this comment esp it got me thinking about things and how lucky i am, id never had thought at the beginning of this year there would be any chance of a holiday this year...cause even if i had had a transplant you have to wait a year to go away.
And as shallow as it sounds tanning is a big part of my life and thinking of going on holiday esp back to Duquesa and not to be able to sit in the sun would be very strange...

So considering iv been able to not only come off the transplant list but to also travel to spain on holiday id say yes that Kalydeco really does work and has changed my life!! heres a few piccys from hols!

the compton family and me x

me in Gibraltar

 

me and the hubby to be x

again x

 

Now that holiday is over and back to the norm, its offical countdown till the wedding!! and yes im starting to stress and panic!! 19 days now till i get married! thats crazy!! this years been one eventful unexpected year! one i wont be forgetting in a hurry!

so much to sort still, need to hand forms into reg office tomorrow, choosing what vows we wanted...choosing wasnt the problem it was pronouncing the bloody words... and also for the smallest girl in the world i have the longest name in the world, so im garenteed for a few giggles and piss takers at the ceremony...manly bianca no doubt hahaha!!
had to choose songs we want, were very unorganised both me and lee haha.
also sorting out cake tomorrow, explain what i want! ha
and picking up dress on wednesday! so fingers crossed it fits perfectly its all been altered so hoping it does or im f**ked basically lol
oh and my lovely husband to be is still terroising me about turning up in an iron man suit, so wish me luck with that one! :S

then theres the hen do, gotta get an outfit.....

for a small low key wedding theres still loads of things that have to be arranged! nothings simple!

ah well all will be ok on the day aye!

health wise im good...the plane well and truely dried out my lungs on the way home, i have oxygen obviously whilst flying and normally cope ok until i get to about 2 hours into the flight then i start to suffer, thankfully its only 2 n half hour flight to and from spain so not struggling for long, but for a couple days after we got back my chest was giving me alot of trouble!

also air con in the appartments, have it on at night time, wake up in the morning and my lungs feel like they been infront of a fan all night! haha

but you gotta take the good with the bad, and the good out weighs the bad ;)

planning on booking myself into hotel brompton after the wedding for a good MOT service lol and relax after the stress of it all! and give my hubby to be a break haha and get myself good for xmas and new year cause wanna be out partying seen as though i missed out last year!!

so lots to look forward to before the year is out!! and a new life to begin as mrs compton!!

love love
xxx

90 days since started kalydeco
80 days since off transplant list
 

Trying to be positive!

Well as you may have noticed I'm not on the "kalydeco high" I want it back! Feeling a bit beaten at the moment, pretty tired a lot of the time, chest feels so full!

It sometimes crosses my mind weather the hospital have made a mistake and maybe I don't have the G551D gene...I do snap out of this train of thought!

I just feel pretty let down at the moment from K and I feel stupid for even thinking like this as my lung function is still 20% and that's way better than it has been before!
Just want that burst of energy, I want that high, I want to not constantly be coughing up, it's secretly getting me down a bit now! Wanna rewind to the first two weeks of K!

I started using my oxygen again at home sometimes, and at times it has given me that burst of energy to witch then Iv completely blitzed the house clean... When I write stuff like this I then feel stupid for complaining and being negative cause lets face it I couldn't tidy the house before even if I had been on oxygen!

Think my expectations are a bit high, lets face it my lung function is still only 20% I think I just need to adapt and go with my body instead of feeling let down by it! However I refuse to settle for this! I want to be running around like a loon again, and I'm on a mission to get to the gym!!

Think the stress of the wedding isn't helping...god knows what I'd be like trying to organise a massive wedding!

Holiday next week so maybe a good bit of Spanish clear sea air and lots of sun will do the trick! Also need to put weight on I'm looking skinny at the moment and don't feel good about myself at all!

A pretty depressing blog!

Sorry haha il try be more upbeat in the next one....

plodding along...

So my last blog turn into a complete rant..sorry about that, back into reality now and not focusing so much on being the "ill person" means things begin to pee me off again! haha

So i finished my two week course of ivs end of last week and was up at the hospital on friday to have lung function and fit to fly tests done!

well my appointment was at 9.30am which was hard enough as it is being up that time, let alone being in london and about to basically do a work out for my lungs!

i havent done proper lung function with all the fancy machines since may 2010, i find them so much harder than the usual ones you get in clinic, and they always push you soooo much its a killer!

my results from that came out that my lung function was 20% which was a bit disapointing, considering after two weeks being on kalydeco my lung function was 23% and pre ivs it was 21% so its not exactly going in the right direction at the moment..but it could be so much worse therefore i cant complain too much!

im hoping im just having a rough patch at the moment and this will pass and il continue to start increasing again, im still bringing up Loads than i did before K and the whole point of this tablet is to thin the mucus and mine is no where near thin yet so im guessing my lungs are still in the clearing out stage! its not even been 3 months yet! and lets face it, im not on oxygen and not in a wheelchair so that alone is a mega plus!

I caught a cold over the weekend thanks to mr Lee passing me his lurges!! haha so thats been a bit of a knock and still feeling pretty rubbish from that, poxy dry cough, headaches, feeling exhausted- no fun!

Had some really sad news also this week, a childhood CF friend sadly passed away peacefully in her sleep early hours of the morning. this came as a bit of a shock to me as i was unaware that she was so unwell and hadnt seen her for many years, we use to go to the same hospital as children but as adults i went to the brompton and she went to frimley, so the last time i saw her was in our local shopping centre where she use to work in mc donalds! she was always a bundle of fun and as a child myself her and bianca would get up to mischef on the childrens ward...running away from physios, hiding in cupboards, squirting the nurses with seringes full with water and throwing water balloons made with gloves at the nurses and staff!! and not forgetting the wheelchair races at early hours of the morning because we would refuse to go to sleep in our rooms!!
quite frankly we were little shits!! haha lots of good memories!
I think she got quite ill after having her little boy this year and never really recovered as well and had spent the last 3 months in hospital. my heart goes out to her family and her beautiful little boy which her memory will live on through.

This last 18 months has been horrible with friends losing there lives to CF, all young girls from the  age of 18-26! its been so heart breaking and just shows how awful this illness can be!
how ever this gives me the drive to be more positive and fight harder!
i never thought 6 months ago id be feeling how i am today without a transplant or if id even be alive and no1 nos what the next 6 months will bring!

onto some less depressign news!.... finally wedding plans are coming together and i finally got my engagment ring! which i loveeeeeeeeeeeee so me!! and its so tiny, litterally the size of a 5p lol
heres a little piccy

 

also have my wedding dress just getting that altered this week cause im a midget lol, and then just to bring on the 3rd november!!

 

less than two weeks till im off to spain which i think im more excited about than the wedding hahaha cannot wait for gambas pil pil and sunning it up in the beautiful costa del sol!

 



think thats all thats going on in my little life at the moment.

xxx

54 days off the transplant list.
65 days on kalydeco



 

Frustrated!!

Bit fed up at the moment. Don't seem to be on such a high from special K at mo! Feel so tired, no energy, no motivation!
I went up to the brompton the other day in my head to start ivs, as feeling like this made me think I might have an infection, it's so hard to tell these days as kalydeco brings up so much and gives me a few mixed signals...all so new to me..so knowing if I had a chest infection coming on was a bit tricky.

Well got up there and everyone still said how great I'm looking (health wise) and I have to remember that they been so use to seeing me with oxygen and in a wheelchair for the past year that although I was feeling bit rubbish that I'm a million times better than what I was months ago.
This was first time in years id come up to the hospital on my own...not that my mum neglected me lol I wanted to do it on my own like old times!
That again was a bit of a shocker for everyone think they were expecting me to say "she's gone to get a coffee" or "she's parking the car" lol

So did sats and blood pressure which was all good resting sats we're 95% on air ;) weight was 38kg lol stabilising could be better I suppose...

Then dr came to do me lung function it had dropped slightly from 23% last clinic to 21%....hardly worth worrying bout seen as though I was only 19% pre K so all good. I wanted to do home ivs as orals never work for me and wanna be best I can, and needed port flush anyway, so started on cef and Tobi home intravenous antibiotics (ivs)

They didn't bother doing x ray for some reason said they never bother for home ivs...bit of a shock for me as I always have x rays, did try get her to request one as I was intrigued to see what the difference was from now to pre k but she weren't haven't it, so gave up had bloods done though!

After a few hours waiting around for drugs and having first dose and getting assessed on how to do it even though I've been doing home ivs on and off since god knows how young they have to check each time or every 6 weeks! Lol I got my nurse to check my crp (infection levels) and to my shock they were only 20!! I couldn't believe it! After having ivs in hospital before I started kalydeco my crp was 70 odd when i finished ivs and I think for a normal well person they have to be between 4-11, so I was pretty over the moon about that...which made me doubt my decision for starting home ivs now...kalydeco is clearly working and maybe I should have held off the ivs until I over came this "bad patch" but I went with my natural instinct....

Although im feeling very zoned out at the moment weird how it can affect me the older I've got it happens a lot now, they make me sleepy and dazed and there not even strong ivs just standard normal ones! Also first time in a long time I've not had anti sickness iv with it!-So far so good so fingers crossed it stays that way!

I'm hoping il start feeling a bit brighter in next few days...feel like a drugged up breathless tired George at the moment and not particularly enjoying it...want my special K high back!

Something that's really starting to piss me off at the moment and can't help but write about it is certain CF people (mainly boys/men with cf) are talking about how fitness is the key to not being I'll and never having ivs or EVER being admitted into hospital....don't want to dig or come across as jealous...well to be honest yeah I am pretty jealous who wouldn't be in my situation...living a life without hospitals, admissions, needles agg after agg, of corse I envy that...but the thing that REALLY pisses me off is when they come out with comments like, "people who don't do fitness that's why there in hospital etc etc"

For the record I was in and out of hospital with endless infections before the age of 2 years old.... Now please inform me what child of that age or shall I say "infant" does exercise?? So how could I have prevented myself from these chest infections?

It's bollocks they get all high and mightly expressing these opinions and say how we must not have looked after ourselves, purely because they haven't experienced a life of cf like myself and many others...it's insulting and makes it look like we don't try or take care of ourselves or that we choose to be "I'll"
Plus it's a known fact that once cf girls hit teenage years with our hormones etc that it affects girls a lot more and can make them a lot iller.(sorry bad English)
One thing that's very hard to understand ESP as there is so little in the public eye about cf as it is, but there are sooooo many different levels of cf, not every person is the same and not everybody's experiences are the same.

so for those of you who have made stupid ignorant remarks, if you haven't lived a certain way...then you shouldn't comment about it or have such strong opinions!

Rank over! I'm off to sleep feel better now haha

39 days off tx list
49 days on kalydeco

First night out!!

I'm getting rubbish at blogging these days. Not had a good week this week, I mean I'm still better than I was months ago so I can't complain too much and i am still very grateful to feel as good as I do.

Barely had any energy since Tuesday feel so tired all time, soooo much stuff keeps coming up and becoming quite tight chested at the moment and breathless, haven't used oxygen and haven't checked weather I've needed it or not...it's so easy to slip back into old habits, got back into a routine of not using oxygen or needing it that maybe I might be neglecting my body of it or not recognising when I do need it...
So maybe I should keep an eye on that!!

Also this week Iv been living on paracetamol, been getting the worst headaches ever, ones that literally sting when lifting my head!! Very much disliking that! But hey Ho things can't always be perfect!

I've started taking my tablet with bacon now to make sure im getting lots of fat with it, also heard that taking kalydeco In between eating fatty foods can make it work better!? Just trying everything to get the most out of it! But funnily enough since doing that, that's when the headaches have started so god knows!

Something that has crossed my mind is I might have an infection? But I really don't no how I can tell the difference at the moment. I mean normally i know when I have an infection because I struggle more, or bring up more mucus, or cough more but since starting kalydeco thats most of the symptoms lol so it's a tricky one!
I have a hospital appointment at the end of this month I think but I think if I carry on and don't feel 100% il get myself to hotel Brompton, don't wanna take any chances anymore.

Anyways onto a brighter note!! Was a big brave girl last Saturday and took to a night out in my local town of Weybridge with a few of my mates! First time out since match 2011-and I loved every minute of it! Haha

When it got to 10pm and time to take my kalydeco tablet I ordered a nice cocktail and a packet of nobby nuts to take with it! Think the barman thought I was a bit of a weirdo ordering nuts-lol sod him, it was the only fatty thing I could think of having with it, was a bit early for a kebab wasn't drunk enough yet! Haha

So yeah I did it my first night out! I was Sooooo nervous going out and felt like a little kid just turned 18 again and never been out before! There's no way 6 months ago I thought without having some new lungs I'd ever go out again! I got all emotional about it that night thinking how scary bad things had got and how much life has changed again! Just too thing a little blue tablet could work such miracles!

That's all
Here's a few piccys!
Love love xx

39 days since started Kalydeco (5 weeks)
29 days off transplant list

Thinking mode!

Laying in bed having a little think to myself... Been on my laptop this evening looking over pictures of myself this past year and thinking back over everything! It's been like a rollercoaster . It's crazy how you think life can be going one way, then the next it's completely changed!

One photo album that popped up whilst looking through was my "funeral pics" I'd gone through the Internet picking out flowers and all sorts I began to plan it perfectly....yet now I struggle to plan a bloody wedding!
Can't believe I even thought about a funeral and now I couldn't feel more away from that time in my life!!

It's been such a crazy emotional year, didn't even realise how bad it had got, been thinking about it loads lately, must of put such a strain on everyone around me as well!

It scares me to think I would/could ever get back to that again, I think mentally it would be a lot harder to deal with!

As far as I know from reading up on people on kalydeco the don't seem to get any worse, so hopefully that's how it will go for me and I will never have to rely on oxygen and wheelchair again, time will tell, the only disadvantage I have is that I have been that low so my lungs already have been damaged so once all the crap has stopped coming up should find out what the situation is really, weather my lungs were mainly blocked with mucus crap or weather it is mainly scarring from infections.
Even if I get a good 2 years with my lungs like this il be happy then I can try the transplant root again. Just think I would be sooo nervous going for transplant again now. I received my clinic appointment letter high lighting everything that was mentioned etc and it said on there about going back on the active transplant list once I'm married...but I'm too scared And don't think it's the right thing for me! I know summertime is always the best time of year for me anyways so maybe see how I get on at christmas and then see what and how I'm feeling in January if things are still good then transplant isn't right for me yet, if things aren't good then it's back on the list I go. By January i will have been on kalydeco 6 months so long enough to see what the crack is!

God life is a confusing mess sometimes! Can't just be simple in my little world! And no matter what I decided there's always someone who doesn't agree or has an opinion on it! In the end of the day, it's my life and I know what's best for me and I'm sooo stubborn and never listen to what anyone's says anyways ;)

Iv already proven the right people wrong ;p

Had a good little day today, cleaned my gorgeous little house done all my washing and put it out to dry on the washing line in the beautiful sun! Then went over to the famalams for dinner and took our dogs for a walk to the park! Love the fact I could walk there and around and back without struggling or getting out of breath! Sooo good il add some piccys at the bottom.

Also to prove my sats are so good just done them and took a pic! Pulse rate is sooooooo low for me like I said before its normally at rest over 100! And sats were good at 95%

Thank god for kalydeco! If I could personally meet the people who invented the drug I would give them the biggest kiss on the cheek EVER!! Words can't express how much I love them! I hope there nice people as I only like nice people lol

That's all that's in sprogs head!
Mucho loveo!
Xx

4 weeks on Kalydeco

Thought I'd best start updating this blog a bit more like I use to!

I think now I have settled into life on this new drug, think I'm over the hype of it now and it all being a bit surreal!

Since last week iv had some crap days, yesterday ESP, the night before I was non stop coughing during the night and could not catch my breathe or rest it was such a horrible feeling, felt like I was being choked! And cause of the amount of coughing I was doing after about 20-30 mins solid I started getting chest pains and all I wanted to do was relax and sleep! So decided to take some oramorph which iv not taken since before my hospital admission pre kalydeco. I didn't have a syringe to measure and was so frustrated and knackered so I just poured the liquid into the lid and took that so dunno how much that was but couldn't have been a lot but almost instantly my breathe relaxed and managed to stop coughing and lay down and rest and sift back to sleep! Which was great.....

Until I woke up in the morning!!

Omg I regretted taking the morphine almost instantly. I was overwhelmed with sickness and I couldn't handle it! I've not felt sick in such a long time it was completely alien to my body and I literally felt like the biggest drama queen but I couldn't function!
10am came and it was time to take my dose of kalydeco but I couldn't literally I was heaving and the thought of putting anything fatty near my mouth made me feel even worse, I wanted to force myself but I knew either way I'd be sick and didn't want to sick up the tablet

Didn't no what to do, I need to eat as the longer I left it anyway my body was feeling sick cause I was hungry.. I haven't had to deal with worrying bout sickness in so long I was a bit lost on how to handle the situation lol and I needed to take my tablet!

The only way round it was to try make myself sick but I was too scared haha
So decided to go do some DNase get myself coughing and bush bosh bash I was sick. Lol finally that's the last time I was taking morphine cant handle that feeling, I managed to take my tablet around 12 and eat some pizza with it but I felt so crap the whole day, was so drowsy, had no energy, lifeless George everything was such hard work, it was a flash back to a few months ago... Although breathing is bit easier now.

Thankfully I was back to normal today!

Something that mum and I have noticed, that I think may have something to do with the drug.

I haven't checked in such a long time as never really feel the need to nowadays but I have my own little sats machine at home which measures my heart rate and oxygen. And one thing that really did shock me was my resting pulse rate. For me normally my heart rate at rest is anything from 101-125
That's because my heart is working harder because my lungs are so crap....welllllll when I checked them yesterday my heart rate at rest was 70!! That's crazy for me! I was shocked! That's like a normal persons heart rate! :) happy Sprog!

Not much other to report on health although I'm not feeling as great as I was, keep feeling really tight at times, but then it goes, think I'm being greedy, let's face it I can walk about a lot more than I was and not confined to a wheelchair on oxygen! So I should be grateful but still clear lots and it is early days still :)

Wedding plans are coming along nicely, can't really take any credit for that and I have realised I'm not girly at all really, weddings are so over rated these days...I think people forget what the purpose of weddings are!and there so expensive! So many things i want but unrealistic! But I have found my dress and venue and booked a date so I'm leaving lee and his best man to do the rest!

Mainly Looking forward to my holiday the beginning of oct! Soooo needed and I'm soooo excited! Cannot wait to tan up eat lots of gambas pil pil! Take a trip to marbella and Gibraltar to see the monkeys and just chill! It's been far too long! :)

That's all to update

Love love

20 days since came off transplant list

First Clinic Since Starting Special K! (kalydeco)

this blog has taken me ages to write, keep getting side tracked been writing it for days now!!....

Since my last blog i been feeling a bit up and down. the day after i wrote my last entry i was feeling a bit rubbish, got a temperature for a couple days, had headaches and generally feeling a bit feverish. Been feeling much better now though aside from feeling the odd tightness here and there but that normally means something is coing up and is a bit stuck on the way. i have been using my isleep a bit recently to help things along which is like using the "bird machine" in hospital, because kalydeco helps it comeup easier and shifts alot more in my lungs using the isleep just gives it that extra boost of air and deep breathe! and lots of ventolin and dnase too!

So Tuesday 31st came, i was so nervous about going up there and yet so excited at the same time! the thought of having to tell my two consultants that i had taken it upon myself to come off the transplant list just made me feel sick! I knew i had/have done this for all the right reasons as i had spoken to harefield about it and they were totally fine, but explaining it to my team at the brompton was going to be a completely different story..... like i said i was going to do, i kept it all hush hush until i saw my doctors at the end!

so dietian came in and my weight was only 38.4kg....but to be fair thank god for kalydeco cause iv hardly eaten and not because i dont want to or feel sick as the sickness has COMPLETELY gone from my life, but i feel like iv just had no time! i eat normal meals but not really been concentrating on how much fat or calories are in each meal! the most important thing for me at the moment seems to be what fatty foods im taking with my kalydeco tablets at 10am and 10pm! so need to start focusing on my weight more or try drinking some of my fortisp drinks. i dont want to do my feeds at the moment purely because im being a stubborn bitch!! i hate them and i made myself do it before because i was on transplant list...im hoping soon i will just get a really good appetite again! iv already seen an improvement as normally when i have a meal infront of me i get half way through and cant finish cause its too much or i feel sick and thats not the case anymore!!

anyways back to clinic! so after dietian the physio came in, this was the one person i normally dread seeing yet this time i was too eager! lol
so she came in and she had a funky new machine to test lung function on which was also a sats machine!
sats were 96% at rest! then we tried the lung function....
well first of all i did a blow and it didnt regisiter because i blew at the wrong time or something wasnt pressed at right time so first one was a right off.....
now a few months ago getting me to do at least one lung function was a battle in itself as it normally completely wipes me out and i would end up having no energy, yet this time after it not registering it didnt bother me one bit havign to blow again-i wanted to blow more!!

so second time! then a third and fourth! haha with LOTS of coughing up in between and my lung function has gone up to 23% thats the highest it has been for over 18 months!!! so amazing!! its gone up 4% in two weeks which is a MASSIVE increase for someone like me esp as iv not had ivs or steriods!!

so that was it i was on a high....then she left, the next bit i was dreading!! which doctor would i see!
then my little irish cf nurse came in! can never remember her name but shes soooooo lovely! she was asking how i was getting on so mum and i told her how things had changed, so it had inspired her to go home that night and read up all about kalydeco! also spoke to her about being engaged and needing a fit to fly etc! and also asked her about wanting to do a sweat test to see what the difference is now after being on kalydeco. a sweat test is something they do to diagnose Cystic Fibrosis to be honest i dont really know too much about it but i know that being on kalydeco is ment to lower something haha so she said shes going to look into it and sort that out for me :)
as she was talking thats when my doctor came in...

the relieve on my face when i saw who it was haha (it wasnt any of my consultants)
so i knew breaking the news about being off the transplant list and wanting to travel was going to be alot easy to explain about!

he was asking how i am on kalydeco etc as he was quite involved in the clinical trials that are still going on, but me and another girl are like guinea pigs because all the trials have been done on people that have 40%+ lung function and who generally are alot healthier!

he totally understood why i have decided to come off the transplant list until after the wedding, he said if it was him he would have done the same, and i didnt really bring up how i felt about wanting to stay off it constantly i didnt think it was worth mentioning...one thing at a time! lol

then i asked if i would be ok to fly and go to spain to get married would it be safe, he said he couldnt see why not, people who are alot iller than me can fly so hes booked me for a fit to fly in september which i asume as iv had one before will just be wanting me to bring oxygen!

i had anoother liver function test whilst i was there so im assuming thats all good as i havent heard anything :)

so got another appointment booked for a months time.

achievement for me..i decided to borrow my sisters bike for a bit to build up this chest of mine and get some exercise going in my life...for the first time EVER! lol
so went over to my mum and dads with lee and dad fitted the bike seat for my size and then thought id take it out to see if i can still ride a bike and lee came with me to make sure i was ok, i started off a bit wobbly but i havent ridden a bike since i was at secondary school so i was bound to be a bit shakey but i got the hang of it and lee showed me how to use the gears and i was off! felt so good, wind in my hair, felt like someone from a film you no forest gump the bit when  hes running ahahaha CHEESEY!!! but yep that was me, but cycling my legs started to kill me within the first few mintues, prob a complete shock to them as i do NO exercise, then my hands went all tingerly haha...i managed to get around the block ish before i had to stop and go back as i started to struggle, i should have had oxygen with me and really felt it after,id hate to have seen what my sats had dropped too, so still not a new person yet but even just getting as far as i did was more than iv been able to do in years!

so for anyone that says Kalydeco doesnt work for people with "low" lung function is talking rubbish!! im living proof it does and its worth trying, i am soooo grateful to have been given this second chance again, iv still got a long way to go as it has only been 3 weeks and real changes take alot longer to show, but even if it doesnt and i stay like this i would happily live my life like this! after getting so low in health infection after infection, non stop ivs and hospital addmissions and hardly lasting 17 days out of hospital before i would be back in again for at least  2-3 weeks my life has turned around. not having to rely on oxygen to breathe, going out without having to use my wheelchair, being able to walk around my house without having to sit down for at least half hour to get my breathe back, having a bath and get ready straight away after getting out rather than having a bath with oxygen haning  off me and being wiped out and falling asleep on my bed after because i was exhausted, the list could go on..... i had started to give up and just resigned to the fact i was waiting for a transplant, i stopped wanting to go out much and just felt safe with lee and my mum! it wasnt much of a life to live looking back!

i hope and prey everyone that has my gene the G551D gets the chance i have been given and also kids now born with this gene with never have to experiance how ill i got and will never have to worry about waiting for new lungs or have to plan there funeral with there family and loved ones incase the call for a transplant never came!

anyways enough of the serious depressing stuff!! not like me to go that deep but just want it to be a bit of an eye opener to people who dont understand how important this drug is!

so i have blogged before about my friend mickey "the machine" as i like to call him after doingabout 4 marathons already this year rasing money for Cystic Fibrosis welllllll.....

hes only gone and done another thing!! Tour De Berkshire bike ride which was over 20 miles!!! not only did he do it though his amazing son reis also took part along with his dad and completed the 20 mile route!! he sonly 11 years old!! im so proud of both of them and truely grateful to them for doing it esp for CF heres some piccys!!

so massive thank you to Reis and Mickey Park!! :)

lastly heres couple pics ofmy little pooch! hes getting so big so fast!

thats all will try be a bit better at blogging next time got so much going on at moment planning this

bloody wedding! haha

love love

14 days off transplant list

24 days since started kalydeco

59 days until i get married ahhhh

Officially Off The Transplant List!

Sorry its been a while, life has been a bit full on at the moment- having a puppy isn't as easy as i thought it was going to be....not quite sure what i was expecting but it wasn't this! no1 tells you about the sleepless nights and the crying! lol haha so its taken me a week to adjust its like having a baby no time for myself and when the pup sleeps I SLEEP haha

So last time i blogged i was coughing up none stop and sleepless nights, this all seems to have calmed down, iv not had any more chest pains either! its such a weird crazy feeling. like i said before its only when i sit back and think about how i am i actually realise! i just seem to be getting on with life without struggling! although this past week iv not had much sleep due to the puppy keeping me up at night, i still have so much energy during the day! i know the tablets working because having to run around after my pup is such hard work...but its not tiring me out! i only get tired at the end of the day like a normal person would!

Im still coughing and can bring lots up and can become really tight with my breathing at times, i still have CF in the end of the day but i feel like the old george is back! :) i can do food shopping on my own and even carry the bags in form the car! been carrying the dog to and from the car etc taking him to the vets or around my mums. going to dentist on my own, running around the garden like a crazy lady entertaining Mason (pup) and not getting tired after, even bianca noticed after putting my head over the bath to wash my hair yesterday didn't get me out of breathe which it used to, and started walking a bit faster around shops lol. these things sound so minor to normal people but its such an achievement for me! oxygen hasn't been used for god nos how long now...and wheelchair..what wheelchair hahaha this drug really is AMAZING!

so i took the plunge on Thursday and decided to ring the Transplant coordinator at Harefield to tell them i wanted to temporally come off the list!!- i was absolutely shitting myself for some reason! last thing i wanted to do was upset harefield or affect my chances in the future!

this wasn't a spur of the moment decision, i put alot of thought into it and had been thinking about it for about a week. i had also spoken to lee, my mum and close family to see how they felt about my decision as it was a big choice to make! they all fully supported me and my reasons for coming off.

the reason i have temporally come off the list is because....firstly im planning on getting married in spain in october so realistically if i was to get a call now im not going to take it, so instead of wasting anyones time and ruining the chance of someone else getting new lungs i wanted to come off, times so precious when they get the all clear dr carby said if at any time i feel i would refuse a call to come off straight away so just doing what im told ;)
im feeling on top of the world at the moment and hoping that things could get even better than this as iv only been on the drug for 12 days, but also even if it doesn't im quite happy to spend the rest of my life like this, i have my independence back to a level of which im happy with, i know how bad things can get so im on a major high at the moment. also the transplant team said i can go back on the list at any time i just have to call!
the way i look at it is, im living a good quality of life right now compared to how it could have been and if i was to get a call and have a transplant and for it to not go to plan and for me to have a bad rejection and possibly even die, then how can i say that would be the right choice?? im not ill enough to take that risk now...

any ways some good stuff to back up the drug is working... been a little geek and brought a lung function machine and had a little play with it yesterday

so when i was last admitted into hospital my lung function numbers were
FEV1 thats is...

on admission - 0.40 liters (14%)
when i left - 0.50 liters (19%)

so this little machine i used yesterday said my FEV1 was 0.65 liters.... now im not 100% sure how good this machine is or how accurate it is and it doesn't tell you the % and also i cant hear the bleeps go off when your meant to blow cause im stupid and deaf!! lol but my mum googled it and put in my age height etc and numbers came back that im 24%

now 24% if thats right thats AMAZING for me!! it hasn't been that high since before my lung collapsed last year!!
also i hadn't done any of my treatment yet before doing lung function so potentially could be higher..

not getting hopes up but will see what happens at clinic on Tuesday! going to bring my little machine with me and do the lung function on both to compare!

oh also i haven't actually told my doctors at the brompton iv come off the list yet so wish me a MASSIVE good luck with that one...i cant imagine its going to go down all to well hahaha, my plan is to casually mention it at the end when they ask if there's anything else hahahaha bad enough that iv gotta persuade them to let me fly to spain in october!! haha tuesday is going to be a very interesting day. might take some morphine before i see them to keep me calm lol

anyways thats my life at the moment pretty good!

heres some piccys :)

love love

12 days since started Kalydeco (ivacaftor)
47 hours since came off the Transplant List!

5th full day of kalydeco

Thought I'd do a blog before I go to sleep as its been one of the most noticeable days today!

Tonight has been dose 10!

So last night I had a really restless night and quite frankly I was hating it, thought that it possibly could have been due to kalydeco but was pissed off as all I wanted to do was sleep! I was none stop coughing, but it wasn't a loose cough it was an annoying dry one and quite tight and I kept waking up on and off until about 2am then it calmed down....
Until about 5am when it came back with a vengeance lol but thankfully this time looser and manage to clear! LOADS! But was still fed up and just wanted to sleep.

Woke up around 9ish to get myself up in plenty of time to take my 10am
Dose with a nice curry lol after practically being up all night I was pretty tired so knew the day would be a right off and planned to chill on the sofa watching crap tv!
Caught up on big brother done a face mask and then started feeling more energetic and wanted to get out the house so jumped in my car and went to meet the family in Woking for a bit of shopping, well just planned to get my finger measured. (for engagement ring)

On the way I was doing some serious multi tasking whilst driving, mixture of coughing up, holding the steering wheel and driving and having a drink so my throat wasn't so dry...and they say girls can't multi task! Pfft Lol

So got into Woking and actually felt pretty good, I didn't bring oxygen and didn't want to use wheelchair and surprisingly enough I felt good.

I didn't get tight, or out of breathe thinking bout it...I don't even think I coughed? I certainly didn't get into any embarrassing coughing fits! :-)

Spent at least an hour walking from shops to shops with my little sister and felt fine, the only reason I couldn't quite keep up at times wasn't because of my chest but cause my legs ached a bit...I got to point where I barely walked anywhere so need to build up the muscle in my legs again!

Its weird I want to say I feel amazing and I think I generally do...it's not until I sit back and realise what Ive done and how Iv handle it I think wow I couldn't do that a few months ago that I really can say its amazing!

After shopping I came home, put away shopping, tried on clothes I'd brought, cooked myself some food, washed up, then decided to unwrap and sort out my new medicine basket draws thing, fill it up with my drugs, then put that on the shelve, then decided to clean the bathroom....

Last few months if I'd gone out shopping with my fam in my wheelchair and oxygen getting pushed around a shopping centre I'd be exhausted once coming home!

It's very surreal!

I just seem to have this energy and can do things...surely it's the drug? If its not whatever's going on its amazing!!

Funny cause each day so far has been different cause yesterday I had energy but I sounded awful! My mum said I sounded like I should have been heading back into hospital and I was literally coughing up throughout the day but even though I was coughing up it wasn't tiring me out it was easy!

This evening I got some weird chest pains like right down at the bottom
Of both of my lungs. I lent forward and did about 10 deep deep breathes to try shift whatever could have been stuck and got lee to tap where the pain was, it must have worked cause the pain went...made me wonder is there old crap starting to move!!
I really hope it is!!

Also my weights been really good! Even though I have hardly eaten my weights gone up from weekend! I was 38.4kg in the evening and this morning I was 39.3kg so hopefully that will go up some more! Not done my feeds I know it's naughty but I don't wanna throw up everywhere ESP on my new carpet! Lol
If weight carries on improving maybe won't have to bother with feeds fingers crossed!

I'm hoping il get some rest tonight as I'm picking up my pup finally 2moz now so that alone il need all the energy I can!

Will up date again and hopefully things will carry on improving! :-)

Love love

5,074 hours, 6 months 28 days on transplant list
Xx

Day 4, my 7th dose of Kalydeco!

sorry to anyone whos been wanting to find out whats going on, been a bit side tracked recently!

so iv just taken my 7th dose of ivacaftor (kalydeco) now. (with a nice greasy fatty curry)
after my last blog it hit me that night, i could not stop coughing and coughing and coughing!! my lungs were in full swing! cleared at least 3 pots of crap under 24 hours!

since then things have calmed down a bit but still very easy to cough up.
yesterday i began to feel like i had a temperature, i didnt check if i had one...dont think i did just felt a bit cold but i was clearly boiling and tiny bit shaky, i had a tiny headache and was sneezing on and off. really random! last night was the first night i woke up quite a bit during the night coughing, had a restless nights sleep. my chest feels quite wheezy and rattly at the moment which it shouldn't be considering ive just finished ivs so im guessing all of these symptoms are to do with kalydeco!

my weights staying stable, and since last night i feel my appetite getting better. Also some good news that must mean its working as well, my skin doesnt taste as salty anymore...the big tester will be once im on holiday but yeah thats cheered me up a bit, hopefully my dog will still remember me though but my mums dog has already chilled out a bit now and doesnt bite me as much haha

been struggling to figure out what to take my tablet with as it says we have to take with fatty foods?? but i wish they would actually say how much fatty you need at least and because im not taking it at normal meals times its differcult to know what to eat it with! i want this tablet to work to its best, and things that i think will be really good to take it with can have lots of calories but not much fat! hard work and i dont like chocolate or the usually fatty foods- im a pain in the arse really!! lol

and im MEGA paranoid about everything i eat or drink now im on this tablet because of interactions. bloody annoying! literally feel on edge with every drink i have now incase it has stuff init that will affect the drug or make me have a funny turn! im sure il chill out and get use to what i can or cant have!!

iv booked a doctors appointment for monday to have my liver function tests done make sure every things ok! and also waiting for doctor to ring me this afternoon to discuss what type of contraception to use, i want the implant ideally so i don't have to worry about forgetting anything and its just there, but obviously il have to see what is best! these are the conditions for having kalydeco, that i have to be careful! etc i don't want kids anyways jheeze they agg me so much! haha

picking up my pup 2moz VERY excited!

thats about it!

love love

5,037 hours, 6 months 26 days on transplant list

Second dose!

This is just a little blog so I'm not going quiet! Just taken my second tablet of the day now! With a nice angel slice, and a strong large ribena and a packet of quavers :)

Today I haven't coughed up as much as I thought I would but at first felt quite tight about mid day and then manage to get some crap up and then this evening iv really felt tight chested and been on my oxygen since about 8pm after having a bath it completely wiped me out...I'm hoping it's the little kalydeco working its magic!

Like I said not much to say hopefully will be a little bit more interesting 2moz!

That's all
Love love
4,977 hours on transplant list

The Future Mrs Compton! & ANOTHER set back :(

Sorry i havent blogged sooner the reason being is ive been pretty pissed off about the whole Kalydeco situation il explain whats happened...

so Friday the 13th came the big day to start the Special K! i was sooooooooooooo excited and done everything i could to keep myself occupied in the morning until 10am came! mum got up the hospital early so i could get myself all packed and ready as i was being discharged from hospital that day aswell!!

i was absolutely knackered as i had a really bad nights sleep, i had set up my feed and gone to sleep and woken up about 5am that morning and thrown up all my feed up! i was so fed up just made me even more keen to get that kalydeco down me!!

so had my morning ivs, had needle taken out and then watched that clock minute by minute!!

then it came 10am!! finally i could take my first dose of the wonder drug! and obviously i captured the special moment.....

it was weird taking it to think what could this drug be doing that precise few minutes like i could just imagine it dissolving in my stomach and making its way to all the right parts of my body and repairing that faulty gene of mine!! 

i took it with two slices of angel slice couldnt leave the hos had too much to pack up still and they wanted me to hang around incase i reacted! and to be fair i couldnt be bothered to sit in a cafe i was far too tired!

when we was aloud to leave i decided to bring a little pot in the car on the way home incase i decided to cough my guts up on the way home, i was being prepared for anything....i got a bit carried away pacing about the ward without oxygen and kinda knackered myself before i got into the car so spent most of the journey on oxygen haha il never learn ;)

got back to my mum n dads to chill and i dunno if the drug would work that quick or weather it was my mind thinking it was the drug but all of sudden i literally couldnt stop coughing up, i was knackered form coughing so much so part of me thought mayeb it was the dnase from the morning but its unusually for it to be like that mmm 

then went back to my house, mum unpacked from hospital etc and i sat catching up on katie price :) then she went home and i had to keep a pot beside me as yet again i couldnt stop coughing up but this time it was sooo much looser, again could ben reading too much into it but this is what happened!

i had a few missed calls from unknown number and listened to my voicemails and it was one of the shos from the ward had been trying to get hold of me...i didnt call back cause i thought well if its urgent or somethign was wrong he would have said in voicemail...typical me!! 

then lee came home and we was chatting for a while and cut along story short- were now engaged! :) happiest girl in the world and totally unexpected so tht afternoon was on a high because of that...then i get a text form my mate whos still in hospital saying the drs want me to call them urgently arghh couldnt ignore them this time so i ring....

basically i was told i wasnt to take that evenings dose of Kalydeco...i could feel my face going red with anger!! not again!! i had to wait 5 days to start it again as they had given me fluconazole whilst on ivs and i cant be on that drug and on Kalydeco, i was sooooooooooo mad! i said to the drs whilst i was in there that i didnt want to be given ANY drugs that would affect me starting kalydeco!! i was fucking fumming!!! 

set back after set back and after thinking that this drug had already started to work its magic!! luckily enough i had forgotten to take the fluconazole on thursday and friday, so have to stop having it for 5 days before i can take kalydeco again so that would make it tuesday dr said but im going to start it monday! its out of my system now and im sick to death of waiting....

im still on edge about what i should and shouldnt be on and feel like i cant trust anyone at the moment and as its been the weekend i couldnt speak to my consultants so this weekend i know its bad but iv not taken any of my tablets apart from creon so that i can speak to drs tomorrow and know exactly where i stand!!

so had some good and bad news but im REALLY hoping to have some more exciting and positive news for my next blog regaurding Kalydeco! 

and hopefully as soon as i can get this drug in me and working and get my lung function and sats right up and my body in tip top condition im planning to get married on a beach in spain!! :) 

:) 

also heres a touchy and beautiful Video made by kirstie please watch and share and SIGN UP for ORGAN DONATION!! 

http://www.youtube.com/watch?v=t5qLimCppbA&feature=player_embedded

also cant finish a blog without a little piccy of my pup mason at 7 weeks, we get him this friday!! very excited!!

thats all for now!!

love love

4,954 hours on transplant list