28th May 2012
Kalydeco is a new ground breaking drug designed to treat the underlying cause of Cystic Fibrosis in patients who hold the genotype G551D mutation. For those who don't know, Cystic Fibrosis is Australia's most common life threatening recessive genetic disease. C.F affects a number of organs in the body (especially the lungs and pancreas) by clogging them with thick, sticky mucus. The average life expectancy for a person living with Cystic Fibrosis is currently 37 years old. Kalydeco is the first drug that has ever been developed to treat the underlying CAUSE and not a symptom of Cystic Fibrosis!! This drug has the potential to completely change a person’s life with C.F forever...
Currently Kalydeco has not been processed through the Therapeutic Goods Administration (TGA) nor has it been subsidized by the Australian Government to allow it to be affordable and accessible to Cystic Fibrosis sufferers in Australia. It is currently available in the U.S and the U.K for just under $300,000K US per year, per patient!!!
This may seem a bit odd to some people but I have decided to keep a diary of my progress while taking Kalydeco for my Cystic Fibrosis. I'm mainly doing this to help communicate to others with C.F but also to communicate with a wider audience to create awareness for this life-changing drug. I am in an extremely fortunate position that has allowed me to gain access to this life changing drugs for 3 months. I am unbelievably grateful for everything my family is doing to allow me to access this drug however the circumstances come at an enormous price. I hope that by blogging my progress (hopefully positive progress) I can help create awareness and lobby for this drug to be passed through the TGA and PBS as soon as possible!!
Here's a little bit about ME :-)
My name is Alexandrena Parker (long name I know!!), I’m 23 years old and I was diagnosed with C.F at 6 weeks old. I am the eldest of three children (I'm the only one with C.F) and grew up in country Victoria. I am currently a freelance commercial photographer as well as studying teaching at La Trobe Uni. I'm probably the biggest chatterbox you'll ever meet and I'm a self confessed Mc Donalds addict! I’m 5"8 and resemble the stature of a greyhound! haha not really... more like a stick insect!
I wrote a short post on my photography blog late last year and I have included a bit of it in this post.
It’s not a secret that I live with C.F, it’s not something I generally talk about a lot but it’s most definitely not something that I hide from people. My whole life I have been fairly fit and healthy and it has never stopped me from doing anything I wanted to do. I use to think that Cystic Fibrosis didn’t really affect me like it affected other suffers but over the last two years I have had quite a significant decline in my health.
Cystic Fibrosis is an extremely hard disease to explain and to this day I wonder if some of the closest people in my life really understand what goes on behind closed doors. I feel like people need something tangible to hold on to, to understand. I feel like people need to ‘see’ an open wound, a pale face or a bed-ridden body to even begin to empathize.
Cystic Fibrosis is silent; it’s slow and imperceptible. People see me run here and run there, and do this and do that and I even begin to fool my self that anything is holding me back, but then suddenly it all slowly creeps up and only I can feel it pull me back and the world keeps turning and people forget because nothing seemed wrong before. It’s like one day you feel fine and the next you realize that your not and people wonder why you hadn’t mentioned it before, like you’ve been hiding something from them. It’s not like I don’t communicate to people how I feel it’s just hard to explain that some weeks are good and others are bad but they all just seem to blend together because your trying not to let it get to you.
I’m going to write a little bit about my current medications and health in general (I’m so sorry if it grosses/FREAKS people out…but C.F is far from glamorous at the best of times!!)
Today 28th May 2012:
Lung function (PTF): Currently 65%.
This test measures the capacity of your lungs and how well they are working. From age 6-18 years I was around 90% and during the last couple of years I have dropped down to 65%. People who sit around the 20-40% mark usually require lung transplants.
I usually have 3-4 tune up’s (hospital admissions per year now) I elect mostly to do Hospital in the home where I administer all my own Intravenous antibiotics twice a day.
Currently exercise daily (running, cycling, swimming – mainly cardio puffed out) I have a personal trainer as well that I train with approx. 3 times a week.
1. Augmentin 875/125mg-amoxycillin- 1 per day. This is an antibiotic to help fight of lung infections.
2. Azithromycin 500mg – 1 per day. This is an anti-inflammatory drug to help fight lung infection
3. Pulmozyme 2500U (2.5mg) 1mg/mL- 1 nebulizer per day (this is a pump) Medication to help assist breaking down mucus in lungs
4. Pariet 20mg- 1 per day- Helps reduce reflux symptoms
5. Nexium 40mg-1 per day- Helps reduce reflux symptoms
6. Motilium 10mg-2-3 per day-Helps reduce reflux symptoms
7. Creon 40,000-approx 25-30 per day- Helps digest food. I take these with everything I eat
8. Symbicort 200/6- 2-4 puffs per day- assists with asthma related problems
9. Swisse Vitamin D (D3 100IU)-D- I am very deficient in Vitamin D due to malnutrition.
10. Cefepime 2mg IV antibiotics – twice a day
11. Tobi Nedulizer or Tobramycin 280mg IV- once per day.
Go to bed 11.30pm- doze off to sleep sitting up because I cant lay down to go to sleep because coughing is too bad. I often have huge coughing fits when I go to bed due to bad gastro reflux. Reflux is a common problem in people with C.F due to the acidic levels in the stomach.
Wake up around 3am coughing- have to sit up again to stop coughing.
Get up at 6.30am to get ready for Uni Prac- start the morning with coughing fit…cough so much that I vomit. This is a normal thing to happen to me- often vomit from coughing, as it is so much pressure on my whole body.
Shower with my arm out the door as I have an PICC IV line in and it cant get wet!
Take Meds and do my Pulmozyme nebulizer- this takes 15 mins.
Eat toast with HEAPS of butter and vegemite, cup of tea with full cream Milk; force-feed myself a chocolate bar (Yes strange I know but got to squeeze in some extra calories!) 4-5 pills with breakfast
Get to school- coughing quite a lot in the morning- trying to suppress cough so I don’t make a scene in front of people. Cough usually subsides around mid-day when my lungs have settled down.
Eat chocolate bar, bag of chips, big M, piece of fruit and some bakers delight bread twist for snack. I take 6-8 pills with snacks. Lunch I eat more B.D twist bread, chocolate bar, handful of nuts, peach ice tea drink, 2 Tim Tams- 6-8 pills for lunch
I often feel very bloated after eating and get bad pains in the stomach due to bad digestion.
Go for a run around the Tan (3km)- basically feel like I’ve rolled around in a sea of salt after. (C.F people sweat out a lot of salt!)
Go home to have a coughing fit- this is a good thing because I can get all the ‘yucky’ mucus up. Sometimes coughing fit happens during the run, which is absolutely lovely for strangers to witness as they stand by and wonder whether I’m about to hyperventilate!
Eat some more food- usually chips (as I’m craving salt) Eat Mc Donalds- 6 nugget meal with chips and coke 6 pills with this. Sometimes ill get a chocolate sundae too if I’m craving sugar. Usually if I go 3 hours or more without food I start getting the shakes!
So exhausted I need a ½ hour – 1 hour sleep to keep me going till dinnertime.
Eat dinner- Huge Chicken Parma with chips and salad and a coke. 6 pills with this.
Before Bed Snack- Jelly beans and a bag of chips! Yes I know not ideal before going to bed but I’ve got to get the calories in somewhere! 4 pills with this.
I didn’t really want to write this as it is not the greatest fact to share publically but seeing’s its for a greater cause… here I go. I spend a lot of time on the toilet… food basically likes to go straight through me even though I have pancreatic enzymes with everything I eat. I am really REALLY looking forward to the changes Kalydeco has on this element of my health!
Take Meds and attempt to go to sleep…. Sitting up... DREAMING of Kalydeco tomorrow!!!