A reflection...

Imagine you just woke up with a cold. It’s not the worst cold you’ve ever had but it’s enough to knock the edge off you. You feel congested, tight in the lungs, your nose is blocked and you feel really lethargic. Imagine you’ve also got a stomachache, you feel bloated, irritated and uncomfortable. You lay in bed feeling sorry for your self, hoping that it will pass in a few days and you can go back to doing all the things you want to do. Now imagine someone told you that your cold and stomachache were permanent, that it was never going way. Imagine that they told you that it actually was just the beginning and it would most likely only get worse. Imagine this is now your ‘normal’ state of being from here on in.

It’s not unbearable and you’re not in a huge amount of pain but you’re now burden by your body no matter what you do everyday. You wake up every morning struggling to breathe because you have so much mucus in your body and your tired cause you haven’t slept much because your coughing kept you up over night. You’re not hungry but you know you have to eat because you’re already underweight from the stress on your body. You feel nauseas after you eat and you’re stomach is even more upset than it was before. You force your self to exercise in hope that it might just move the mucus from your lungs and make you feel better. Slowly your cold and stomachache gets a little worse each month. You realize that you can’t keep up with your friend’s busy social schedule and work is becoming overwhelming. Imagine your cold and stomach ache now requires IV antibiotics on a regular basis because the oral medications just don’t cut it now. You’re falling behind at work because your constantly disrupted by the hospital admissions and you begin to loose touch with some friends who can’t cope with your ill health. Your dreams seem to be getting further away as your health begins to consume your every movement and every thought.

Imagine one day someone told you they could give you 2 little blue pills a day and all your suffering would almost instantly stop. Imagine they told you that you could go back to waking up in the morning with energy, that you could stop vomiting from coughing and you could simply breathe easily. The pills would relieve you from your stomachache and your lungs would no longer feel congested. They would make you feel like a new person and you could finally say you felt healthy again. Your dreams start to become a reality and you go back to living a normal, energetic and fun filled life. Your family and friends could stop worrying about your declining health and you could finally start living life the way you were meant to.

This is what happened to me. This is what happened to me the moment I started on Kalydeco. It’s not until now when I sit and reflect that I actually realize how fast my life was sliding on a downward spiral. I can honestly say without a single hesitation that this drug has changed my life. I’m not angry or resentful I was born with Cystic Fibrosis because without it I wouldn’t be the person I am today. I’ve always had big plans and big dreams and I have always been determined to never let Cystic Fibrosis stand in the way of anything I set out to do in life. Whilst I was determined and I never lost sight of my dreams the reality was that Cystic Fibrosis was pushing them further and further away.

After moving out of home at age 18 to study and pursue a career in photography I found my self-questioning my future at the beginning of 2011. I had dreams of travelling the world and becoming a renowned and respected photographer but after a rapid decline in health these dreams seemed more like a fantasy rather than reality. At age 22 Id worry on a daily basis about whether Id actually get to do all the things Id wanted to do before I died. I’d worry that I wouldn’t get to see all the places I wanted to see because travel was too straining on my health, Id worry that I wouldn’t get to live life to my full potential, I’d worry that I wouldn’t get to raise a family or grow old to see my children grow up. Since starting on Kalydeco it’s like all of these worries have lifted and I feel as though anything is possible. I’m not even really sure how to put into words the gratitude I have for all the people that have helped develop such an amazing phenomenon. 

Kalydeco is more than just a drug that allows me to ‘breathe easily’, it gives me the opportunity to live my life to it’s full potential, it gives me back my happiness and it gives me back my future.

GREAT NEWS!!!! SWEAT TEST RESULTS ARE IN!!

29/5/12 -PRE KALYDECO

Sodium: 132
Chloride: 114

2/10/12- POST KALYDECO
Sodium: 44
Chloride: 33

VERY HAPPPY!!!!!! So amazing to actually see the numbers!!

What this basically means is that my body is now basically working like a normal ‘non’ C.F body all thanks to KALYDECO!!!! A normal persons body chloride levels are normally around 30 and mine have come down from 114 to 33!!!!!! PRETTTY AMAZING for two small blue tablets a day!!

Before starting Kalydeco whenever I would run or do any physical exercise I would always end up with a huge amount of salt all over my face and body. Since starting Kalydeco it is allowing the channels in my body to work correctly and because the channels are working properly my body isn't producing lots of thick sticky mucus, which causes all my problems (mainly lung infections, reflux, digestion problems etc etc.) in my body. So a long story cut short it basically means I'll hopefully grow really really old and get wrinkly and grey hair!!!!!!!

:-D

p.s cant wipe the smile off my face!!! 

Here is me and my beautiful friend Em celebrating the great news!! 

Up and Down

It’s been 2 months since my last blog post and there have been a lot of up’s and downs with my health in this time. 

As per usual I have been running around like a mad lady. I’d been frantically finishing school assignments, teaching year 10 and 11 photography during my secondary placement, running my business and trying to keep up with my busy social life.

Mid August came around and I could tell that something wasn’t right. I was feeling fine in myself; still plenty of energy but my cough began to get worse. I noticed that I had began coughing up small steaks of blood but I ignored it for a few days as this has happened to me before and it just passed by its self. I woke up on the Saturday and I noticed I was coughing up quite a lot of blood (this was NOT normal for me) so I decided to take myself off to emergency at Monash Medical Centre. I waited for 5 hours in the waiting room and oh what a joy that was!! I contemplated going home about 3 hours in because I had stopped coughing up blood but my intuition told me to stay.  At around 6pm a doctor finally saw me and decided to admit me. As a C.F patient admitted on a weekend I found that none of doctors wanted to make any decisions without consulting my usual C.F team, so I spent the entire night in emergency! During most of the weekend I was stable and I was moved to MediHotel within the hospital but on late Sunday night I started coughing up quite a lot of blood. I was moved to the ward to keep a closer on eye on the hemoptysis. It was frightening as this had not really happened to me and I was very worried. The doctors predicted that I must have had an underlying lung infection causing the bleeding however it couldn’t be sure as my lung x-rays looked unchanged. The doctors took me off Kalydeco for 2 weeks as a precaution and to allow the mucus in my body to thicken up again to allow the bleeding to stop. My doctor suspected that Kalydeco was thinning my mucus to the extent that the bleeding part of my lung could not heal due to the fact that mucus was constantly moving around easily within my body. I stayed on IV antibiotics for a week but due to the blood thickening medication they were giving me my PICC line became blocked and I elected to go home on oral antibiotics once the bleeding had stopped. 

I definitely felt the difference being off Kalydeco within the first 2 days! I went back to coughing significantly in the night and especially in the mornings. My lung function dropped 15% in 1 week and I generally felt crappy!!

I’m back on Kalydeco now though and finally getting back to my energetic self. One thing I have noticed is that I have began to actually sweat!!! Before Kalydeco I would sweat but it would pretty much be dry salt on my skin straight away but NOW I have started sweating like a normal person!! Gross but interesting fact!

I am booked into have my POST Kalydeco sweat test for the 10^th of October so I will post the results ASAP. 

This is my lung function graph from Feb 09 until Present :-)

The Lung Function Scale...

80%!!!!!!!!

I can’t believe it! I’ve gone from 67% to 80% in 5 weeks and I’ve also gained 3KG!!!!! AMAZING!

As I mentioned in my previous post I have had a cold and I went to the hospital to do blood tests, get antibiotics and do a sputum culture earlier in the week. I called the CF coordinator at the hospital yesterday to get the results from the tests and mentioned that I have still had a cold. I decided to go into the hospital yesterday to see my doctor to see if I could prevent my cold from getting worse. I was so nervous to do the lung function- I think I’ve secretly been avoiding doing it because I was so worried that it wouldn’t be what I wanted it to be. Mum bought me a little home lung function monitor a couple of days ago and I refused to try it out of fear!! I bit the bullet and decided I needed to face reality whether I liked it or not. I did my first test and it was 78%! I was amazed but suddenly 80% was within reach and I was determined to make it. On my second try and I made it and I’d compare the feeing to winning Tattslotto!! I had not been at 80% since August 2009. After, my doctor and I looked at a graph that showed all my lung functions from 09 to present- I wish I had of got a copy to put on my blog. If I can get one I'll post it on here because it’s a very good visual representation of my decline in health over the past 2-3 years and my rapid increase in the past 5 weeks on KALYDECO!!! :-D

I know it’s just a number but I feel like my FEV 1 lung function numbers have been ruling my moods for my entire life. Mum and Dad would drive me to Melbourne from Shepparton (2 hour drive) when I was younger to go to my doctor’s appointments at the Royal Children’s Hospital every 3 months. I remember the drive home ever so distinctly- it was either a celebration and we would stop on the way to have a nice dinner and talk and laugh if my lung function was good and if it was bad, I would cry (mum often cried too!) driving down Flemington Road because mum and dad would be so worried! Looking back now I remember I use to hope that my lung function was good so that we got to go out for dinner and mum would be happy and I never really associated my lung function with my quality of life, future or health in general. Whilst I did grow old enough to realize that going out for dinner was not the epitome of a good lung function result I have continued to be ruled by a number as I grew to understand the importance and impact that these numbers could have on my health and my life. 


When I moved from the Children’s hospital to the Alfred in 2008 I saw a decrease in my lung function and this mortified me. I had always been in the 90% range at the children’s and suddenly I would see myself in the 80-70’s!! After a few changes in my health care I moved to Monash Medical Centre in 2009 and my health began to improve with the help of my amazing doctor (present doctor now) I maintained 80% for a while but complications and lung infections got the better of me and ever so slowly over the last 2 years I’ve got down to the 60%’s.  If you look at just numbers you would think there isn’t a great difference between 80% and 60% however when we are talking lung function every number counts so much. I can’t explain how hard it is to get a number back once it’s gone- its actually almost impossible.  It’s like each number is a day of your life and every time that number gets smaller you feel like your closer to the end. To a certain extent I guess I see my life on a scale of 100 to 0, the lung function scale. I’d said goodbye to 80% in late 2009 and I honestly, honestly thought I’d never see it again! I had accepted that the 60%’s was the norm and this was how it was.  I had said to myself if I could always keep above 40% I’d be okay, it was like 40% was the dark zone and I never wanted to see that! BUT NOW I have KALYDECO and it’s a miracle and I’m looking 80% right in the face! I’d love to say goodbye to 80% and say hello to 90% soon, but I’m taking one step at a time J Whilst my lung function is on the UP these numbers still continue to affect me and all the other G551D C.F patients eligible for Kalydeco, as we are constantly plotted on grids to determine whether or not we should be granted this amazing drug. I hope that very soon we can all stop plotting our lives on lung function scales and all have access to KALYDECO!!

xxxxx AP

Three steps forward and two steps backwards

I’ve hit a bit of a wall. I’ve got a cold. I feel yuck again… Not as bad as I usually would feel without Kalydeco but I certainly don’t feel 100%. I’m coughing up an obscene amount of mucus, my lungs are tight and I’m back to vomiting in the morning… GREAT!

I know it’s just a temporary glitch but it’s a bit of a reality check. I’ve been at uni lectures for the past 14 days every day and more than half the people have had some sort of cold/flu! I guess it was inevitable for me to catch a cold when so many people around me were sick and after all Kalydeco isn’t going to protect me from every living bug know to man!

I spent most of the morning coughing and vomiting into the toilet or bathroom sink because it was so much pressure on my body. I went for a run today and my lungs ached… they feel tight and irritated. I coughed badly most of today and it made me realize how exhausting it is! About 3pm I had to have a nanna nap to let my lungs rest for an hour. I had to sit up because the coughing got worse when I lay down.

I went to the hospital today to get some antibiotics to clear up my chest and did a few blood tests just to see how Kalydeco is affecting my liver function etc. I have my proper appointment with the C.F team and my doctor on July 27.

I’m worrying a lot that it’s going to be a long time until Kalydeco is approved and subsidized by the Australian government...

WOW!

I must have a guardian angle because it’s like someone’s been looking over me.

I’m not religious at all nor am I particularly superstitious however I’m starting to wonder whether there really is a higher source out there somewhere!? Over the last couple of weeks its like a switch has been flicked and all the stars have aligned in my life. Not only has my health improved but I have also been given some life changing opportunities in my career as a photographer.

To say the least I feel absolutely AMAZING! A M A Z I N G! It’s almost been a month now since I started Kalydeco and the changes in my health have been absolutely astounding!  I’m like a ball of energy. I feel electric. I feel like someone new! I wake up in the morning and I spring out of bed, I’m well rested and I have been sleeping laying down almost EVERY night. Occasionally I’ll have a bad night and need to sit up to go to sleep due to coughing but it is becoming less and less frequent. When I sit and reflect on how I actually felt prior to Kalydeco it actually scares me to think how sick I felt before! I guess my health had been gradually declining ever so slowly and it just became the norm without realizing the extent that it had on how I felt everyday. During the last 18 months I did find myself constantly struggling to get through the days without having enormous coughing fits, becoming out of breath just to get from one place to another, having sleeps during the day for hours and generally feeling nauseous and lousy all the time.

My life has completely changed!!! I feel like I can finally focus on the future without worrying that my health is going to hold me back. I’ve always been one to worry about dying. I’ve always worried that I’ll die before I get to do all the things I want to do. Whilst this all definitely makes me sound like a drama queen I think it gives me great awareness and appreciation for life. I think this way of thinking will continue to follow me throughout the rest of my life but I feel like I finally have a newfound optimism about my future! My life was on a downward spiral before Kalydeco and now its like someone’s pushing me back up the stairs. Towards the middle of last year I actually found myself quite depressed. I often couldn’t go out with my friends because I was in hospital, and I also lost friends and people in my life that simply couldn’t deal with my declining health. My business suffered because I simply couldn’t invest enough time towards it and I simply felt sick all the time. 

It blows my mind to think that 2 blue pills a day can change my life so significantly. Sometimes I look at the tablets and wonder what on earth is inside them, I wonder about all the money that has been invested into researching the formula, I wonder what kind of people did the research and I wonder if they know how much this drug can change a person’s life? I feel guilty sometimes that I have the drug and others don’t.  I feel guilty that other people are suffering more than me and probably need it more than I do. I feel guilty that my family is spending so much money on me. I worry that the drug wont get approved and I’ll have to go back to the way i was before… I don’t know if I could handle going back to that.  

Sorry that its been so long since my last post I’ve literally been running around like a crazy lady ( and I secretly LOVE it!!) I will continue to blog my progress a bit more frequently… STAY TUNED :-D

A new lease on life...

I have been so amazed and humbled at the amount of interest people are taking in my Kalydeco diary blog. I honestly didn’t think people would really care too much for the gory details of my life and in particular my health.

It’s funny, people ask me daily “how is your new drug going?” and I find this question extremely hard to answer. I cant tell people that I’m coughing less because I’m coughing more and I cant say I only have to take 2 tablets a day because I still take 40+ a day and I cant say I’ve gone from a size 6 to a size 10 cause I haven’t put on any weight at all… BUT I feel AMAZING! This does sound quite contradictory but I honestly can say I have never had this much energy and stamina in my life!

It’s hard to explain to other people how Cystic Fibrosis affects a person. Obviously you can explain that it clogs the lungs and pancreas with mucus but the problems lay so much deeper than this.  I suppose the best way I could explain my health is that it’s like playing dominos. When my lungs are infected I loose weight, I loose weight because my body is taking so much energy to fight infection. When I loose weight I feel sick and I feel sick because my liver isn’t working properly and when my liver isn’t working properly I’m tired.  When I’m tired all I want to do is sleep, but I can’t sleep because I can’t stop coughing and when I can’t go to sleep I can’t recover and the cycle just keeps going around and my health just keeps getting worse. It’s like Kalydeco has finally broken the cycle. Yes I am coughing more but my lungs are not infected. It’s like before my lungs were coated in thick sticky honey and it was hard to move the mucus from the sides of my lungs but now it’s like custard and it moves around a lot easier. Suddenly my body doesn’t have to work so hard to keep everything going and all the other problems don’t seem so prevalent and it has given me a new lease on life!!

Firstly, I can FINALLY sleep laying down, do you know how exciting that is!? I am able to get full nights sleep and I wake up well rested and ready to tackle the day. I do still have massive coughing fits in the morning and I wouldn’t say I wake up feeling like a can breath easily but the coughing fits move on much quicker than they previously had before. I am starting to wonder when on earth I’m going to stop coughing up so much disgusting mucus though??

For those who know me (especially my mother) will tell you that I ALWAYS like to over do things! I’m always over committed, over ambitious and over loaded with work, Uni assignments and social life! This week in particular has been a huge week to say the least in terms of primary placement, uni assignments and work. I have currently been completing my primary placement 5 days a week (8am-4pm) and running my photography business after school. I found out this week that I got booked on some really big and exciting photographic campaigns and this has really started to put the pressure on me. Normally I would be completely and utterly exhausted with these huge commitments but I’m better than ever. Normally I couldn’t go through an entire day without sleeping in the day for at least 3 hours, normally I would feel nauseas and loose my appetite and normally I’d be in hospital on IV antibiotics…but I’m not, I feel great!  

Its another big week ahead but I'm feeling really excited that for once I can finally keep up with my busy life without my health holding me back. 

A.P :-) xx