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

Kalydeco has arrived!!!

So this is my last blog before starting kalydeco tomorrow morning and hopefully changing my life for a while or even forever!!

My expectations are soooo high for kalydeco and as nearly everyone at this hospital thinks that's a bad way to think I don't!- lets face it if you really want something to work and you aim high then you get what you want and it will happen, this drug has had amazing results so why won't it happen to me? I also believe in power of the mind!
I control how my life is going to be and what happens!
If I want this drug to work to my expectation then it will! I'm on a mission to prove it to everyone!

Also something iv never really spoken much about before but it's something that's really important to me and i feel that honestly it works!, and if everyone in my position or with any deteriorating illness like mine was to think the same way as me, it would honestly help them be a stronger person and healthier in a way.

Some people brush stuff like this off, so you gotta be open to it to believe it.

A pure example- at the beginning of this year I was feeling pretty negative, not completely as I'm not a depressive person but in a sense I started to give up, come to terms with the fact that this was it, I was constantly getting infections and I was basically waiting for a transplant to change that! I hated where I was living and all I wanted was a nice bungalow with a garden and Get a dog....

From day one of going on the transplant list iv said to my mum, family and people close to me that whatever happens I really wanted to get a new place, then a dog then my transplant call..... And funnily enough that's the way it has happened minus the transplant call...

Strange, coincidence? Or is it because that's the way I wanted it to go?

And since getting the two things, I'm a much happier person I LOVE my life no1 can put me down and my health has been so much better recently too!

I want people to think about all of this and put it into there own life's, how they want there life to be weather there not well or just having a bad time, your in complete control of your life no1 else.

Anyways enough preaching haha

Sooo I'm being aloud to escape hospital aswell tomorrow which I'm
Very excited about! Plan to have a fatty breakfast and take my first dose of Kalydeco at 10am!....maybe with a giant glass of champers! Haha

The doctors want me to hang around a few hours to make sure I don't react... Not 100% sure this is true as andy pandy said it to me even though my consultant didn't mention anything of the sort in ward round today...I think he just likes to annoy me on my release day, so il nod and agree but I'm not hanging around too long! Lol

Also got my little machine to monitor my blood sugars today, interesting little thing! So il keep an eye on my sugars but they have calmed down quite a lot :-) So hoping those high numbers were a one off.

Don't really have much more to go on about all my head is thinking is kalydeco kalydeco KALYDECO and it's killing me the fact that it's sitting right in front of me and I cannot take a tablet until 2moz morning but hey Ho all good things come to those who wait ;)

Gna try see if I can get one of the staff to print off my x ray so I can do a before kalydeco and then maybe one in a few month into it hehe

Love love
13 hours till start kalydeco
4,878 hours, 6 months 20 days on transplant list

Count down to Kalydeco Friday!!

Day 7 in the brompton today...and everything seems to be going in the right direction!

Crp is down to 22 and lung function has shot up to 19% and weights up to 39.8kg :) so I'm over the moon about all of that!

My sugars went up a few nights after two hours into my feed, one night was 16 and another was 11. There ment to be between 4-9 so was a bit on the high side, have to monitor them closer now, but did them again last night and it was 6.2 so hoping it was a one off and they calmed down again now!
Not really feeling the idea of becoming diabetic yet! I know it will happen in the future it's standard for cfs they call it cf related diabetes.

Anyways keeping an eye on that one!

Not doing my feed tonight cause I'm sick to death of waking up in the morning, coughing too much and ending up being sick and throwing up my feed-so frustrating!
So on strike tonight haha

Also been made to try the NIV machine again :-( its a bit easier but seriously not feeling it at all, plus it's so big and chunky and ugly I don't want to bring it home to my beautiful bedroom!! So gna try get myself out of that one :-)

Count down over for my birthday, had such a lovely day and managed to escape thank god, I had a little surprise meal with my friends which was lovely thought it was just going to be lee and I but there were all there when I turned up, cried like a twat!

Cannot wait till Friday now I'm so excited its unreal! Also now in contact with a girl who's exactly same age, same genes and at same hospital who's also started kalydeco today, as far as we know we are the only two from our hospital who are having it so far and glad were in contact so can see the changes and symptoms etc

Also it's national transplant week until Sunday the 15th and feel like I should be doing something to help but I'm not very imaginative or any good at organising things esp as in this place and with Friday coming up so my contribution is to tweet as much and nag people on Facebook to sign up for organ donation!! After all I am on the active transplant list waiting for some new lungs myself!!

Lastly I saw my little boy mason on my birthday and he's sooo big now and less than two weeks till I get him home!! Very excited here's a piccy of him!! And one from birthday.

Love love

59 hours until start kalydeco
4,833 hours- (6months 18 days) on transplant list