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!!
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
You've never done exercise before? Yikes, why not? Good luck with the bike riding, keep at it! Though you'll get tons walking that gorgeous gorgeous pup (should probably say 'running' not walking - I bet he's fast and doesn't tire easily!)
ReplyDeleteAnd omg those new lung function machines are awesome - I did my best blow ever too!! Though my chest was crap that clinic so I think they're a bit on the optimistic side...! Shhh let's not tell the docs :-P
Enjoy planning your 'bloody wedding' - you must be so excited. Hope you continue on this upward spiral!
Lastly, sorry if this is nosey, but what's your other lung function percentage? Is there one they say the Special K will affect more? You know, first second one and overall volume one...
Laura x
Great blog post! As far as lung function tests, if you feel great, don't get caught up too much in the numbers. Dean had clinic on 8/7/2012 and FEV1 was down slightly to 82% and he was a little diasapointed. But then his doctor said it was because he had grown an inch in height and that changed the expected number- otherwise he was great. And at 13 years old, boy's lungs lag about 8-10 months behind their height growth so there's all sorts of things going on there with PFT numbers. Keep riding that bike and blogging!
ReplyDeleteDon