Breathing Easy

I think this post will be pretty short and sweet.

About 2 years ago, I remember walking along the corridor at the Alfred Hospital that separates the car park and the main building. I had my suite case rolling along behind me, on my way in for yet another admission without a long time between drinks. It was probably the standard 4 – 6 week intermission in my life at the time.

The corridor is probably 100m long and by the end of it I was buggered, short of breath, struggling to stand up and needing desperately to get up stairs and in to a bed to rest up.

Fast forward to Saturday 26th October 2013 and I’ve just completed a 42km Marawalk for TLC for Kids. A charity that I’ve been involved with for more than 15 years and one that I will have a close relationship with for the rest of my life.

42km’s and nearly $4k in funds raised!

I used to struggle for breath lying in bed; trying to go to sleep I’d wake myself up with a kind of quick groan that was uncontrollable at times, a way for my body to try and stop me coughing or to keep me in breath or something. That was a noise that scared me, I used to hear that from the REALLY sick people I went to hospital with but was a noise that was pretty unfamiliar to me otherwise. Walking short distances was hard and oxygen was a must at one point! Yet, a little over 14 months taking Kalydeco and my life has been transformed.

I’ve been able to measure how much with playing cricket and doing other daily things around the house, etc. but being able to do the Marawalk shows just how big a difference this medication has made. My lungs were the absolute least of my worries across the day. I was able to do the worrying for others in our group and it was up to me at one point, with 4km’s to go, to encourage one of the group to keep going and come with me to finish the course!

That’s me, Shane McCarter - the same bloke that, 18 months ago, needed a lung transplant. The same bloke that wasn’t sure if he’d see another Christmas after a family holiday down the beach across the 2011/12 Christmas – New Year period. The same bloke mentioned above that couldn’t walk 100m without needing a rest, let alone 42000m!

About 40 people started the Marawalk, I was among only about 20 that finished the entire course. And there were people behind me too! It was a 7 hours, 27 minutes experience that I’ll never forget and I’ll be able to look back on for years to come. To Anto, Dmac, Herb and Macca –Thanks for being part of the day boys. You can see below a graph of the walk with times, etc.

One blue pill, taken twice a day, what a difference!

For something different I think we’ll start this post with a few “On This Day” facts from history:

·      1859 – Petroleum is discovered in Titusville, Pennsylvania leading to the world’s first commercially successful oil well.

·      1916 – Romania declares war against Austria-Hungary, entering World War I as one of the Allied Nations.

·      1962 – The Mariner 2 unmanned space mission is launched to Venus by NASA.

·      2003 – Mars makes it’s closest approach to Earth in nearly 60,000 years, passing at 34,646,418 miles (55,758,005 km) distance.

·      2012 – Shane McCarter takes his first Kalydeco tablet.

In case you missed it, today marks 1 year since my life changed so dramatically forever. Just like 12 months without a hospital admission, it's hard to believe it's been 365 days! You can see me struggling with the packet and popping the first pill below, or by clicking here.

One tablet twice a day over the last year has meant that I can live a close to normal life with no hospital admissions (touch wood), no bad chest infections, minimal reliance on antibiotics, no more being under weight and malnourished.

I’ve mentioned countless times before how sick I was, how transplant was looking like my only option, if I even got that chance before getting too sick, who knows what the outcome could have been if that was the case.

So this entry will be more about the positives, what I’ve been able to do and what I plan on doing over the coming months and a thank you for everyone’s thoughts and support across the last year.

Since early July I’ve been training for the TLC for Kids Marawalk. The Marawalk is a 42km walk along the foreshore between Brighton and Port Melbourne and is something that I have always wanted to do, but because of my health, have never been able to attempt, let alone complete.

It’s only because of Kalydeco that I’ve got the chance to give back a little of what TLC have given me across the 15 years I have been involved with them. They have been a constant source of support, advice and good times through more than a few difficult periods and by doing the Marawalk and, hopefully, raising a big wad of cash, I can go a small to way to saying thank you for everything they have given to and done for me. Making my life easier has been only one aspect of what Kalydeco has done. Being fitter, healthier and stronger means I’m able to be help make life easier for others, it’s a seriously good feeling to be on the other side of the fence! As well as TLC for Kids, I’m doing the Marawalk for those that can’t. For sick kids and adults for friends and family and for each and every one of the friends I, and others, have lost to CF and other illnesses. For everyone that didn't get the second chance I did.

Before I started Kalydeco, I was barely able to walk around the house. Since the beginning of July I’ve covered close to 100km’s and every step I take is easier than the last. I’ve had great support from family and friends and a couple of them have put their hands up to do the walk with me, which is amazing, so I thank them for that.

On a less personal, but equally as important, level one of the problems we’ve encountered in the last few months has been getting Kalydeco on the PBS to make it affordable for people with CF that aren’t as sick as I was. I need to say that no body should have to get that sick to be able to have their life saved and changed for good. Being eligible for the Named Patient Program through Vertex is a luxury that healthier CF sufferers don’t have and it’s simply not fair. I can’t tell you how grateful I am to Vertex for giving me this chance, but I do hope they can come to an arrangement quickly with the PBAC to get things sorted out and to allow everyone eligible to experience the effect Kalydeco can have.

In the last week or so especially, my Facebook and Twitter feeds have been overflowing with support from family and friends, driven mostly by my wife and a few others in the Kalydeco for Australians Facebook group, and I have to say that it’s been a bit overwhelming.

There have been people using the #YesToKalydeco hash tag, people sharing links, people asking their friends and family to do the same and those friends and family asking their contacts to spread the word to speed up the process to get it on the PBS so each of the 200 CF sufferers with the G551D mutation can be given the chance that I’ve been given.

So, simply I’d like to say THANK YOU! With Kalydeco will come other drugs that will help more and more people with CF live longer and more fulfilling lives. So any little thing you can do will help make a difference.

Kalydeco aside Cystic Fibrosis has always been a disease that very few people had even heard of, let alone known about medications that could change the course of the future for its sufferers! Now, it’s in the papers, on the news, it’s being advertised in TV commercials, it’s not one of the least known, most common genetic diseases in the world anymore and that’s a huge step. Again, made possible by you guys by spreading the word.

Anyway, I just thought that I’d write this today to again show you all what a difference Kalydeco has made to my life. To thank you for the support you have given to the #YesToKalydeco campaign and to ask you to keep supporting it.

And to ask for your generosity and support once more with a donation, big or small to TLC for Kids as I give this Marawalk a red-hot go. If you can spare some loose change, click here and follow the prompts.

So, yesterday marked 12 months since I came home from hospital for the last time.

12 months, it’s incredible.

I could barely last 12 days before noticing the early stages of yet another chest infection, and you could bet your bottom dollar that at some stage around the 12 week mark I’d have either been in hospital already or would be screaming out for an admission, barely able to walk around the house without getting puffed out and/or needing a rest.

I really can’t believe how much one tablet, taken twice a day, has changed my life.

A year ago I needed a lung transplant. No questions asked.

I’ve thought mostly about the operation its self, the risk, then the recovery; the strict regiment of tablets, anti rejection drugs, etc. the rehab, getting your body working again. Then more risk: rejection, infection, etc.

But someone brought it to my attention last week – Before all of that could have happened, there was the waiting.

What if I didn’t get that chance? What if I decided to go down that path and I missed my chance, I’ve seen it happen before, it’s a seriously scary thought.

How lucky have I been?  It’s almost unbelievable.

I’ve said this before, but when you’re that sick, you actually don’t realise how sick you are. Your body learns to cope and function at a certain level that lets everything work. You subconsciously walk slower, avoid lifting heavy things, you sleep more and feel tired all the time no matter how much rest you have, but when things need to get done, you some how find the strength to do it.

It’s only when you’ve recovered a bit that you realise how bad things were. Then once you’ve recovered a bit more, you realise that even that initial recovery didn’t bring you back to an acceptable level.

I’ve had that realisation more and more over the last few months in particular.

How I feel now seems an absolute eternity away from how I felt 12 months ago, yet that’s all it’s been. 12 months. 365 days. And it’s all thanks to one small blue tablet!

Some people get a good 5 minutes of a great time from taking a small blue pill (if you know what I mean…!), I’ve had 12 months, a year and there are no signs of it slowing down.

I still get chest infections, but I think I could count on one hand how many I’ve had in the last year. And they’re no big deal anymore, all it takes is a bit of extra physio and some oral antis and I’m away. A week at the most and I’m back to normal.

No days in bed, no hospital, nothing serious. It’s almost normal!

My weight is now steady at around 10 kilos more than it has ever been steady at. I used to have to work seriously hard at getting to 60kg, using high fat supplements and eating to the point of feeling sick, now I haven’t dropped below 63kg in over 6 months. I’m not doing anything out of the ordinary, I skip meals and don’t eat well enough when I get busy, but the weight stays on. It’s something that wasn’t normal before taking Kalydeco.

I could drop a kilo a day if I wasn’t on top of my eating habits, pill taking, etc. before Kalydeco.

Again, that’s scary! No wonder I got so badly sick, so often.

It’s been a while since I posted here, but I find it hard to write about just being normal. I suppose it’s exciting for me, but for the sake of keeping this interesting (I hope), me writing about how little mucus I’m coughing up or how much weight I’ve put on will be kept to a minimum!

For anyone with any sort of power in the community, doctors, directors, politicians, you know who you all are; this next bit is for you.

This drug is a serious lifesaver. I’m proof of that. There are still people out there who can benefit from Kalydeco and other drugs like it that are being trialled for other CF mutations, you have the power to save people’s lives without the absolute last resort option of transplant. You have the power to prevent CF from ruining people’s lives. Please, use the power you have, use it wisely and use it soon. Like I said, there are people out there that need the second chance that Kalydeco has given me.

I want nothing more than for the people I know, and the people I don’t, that can have the opportunity that I’ve been given, to have it.

For anyone suffering at the moment, this next bit is for you.

Hang in there. I’ve been there. It’s tough, but you’ve got the strength to stick it out. The rewards are coming, new medications are just around the corner, all you need is for the people in power to do the right thing and you’ll have your second chance. Push them, ask the questions you need to ask, believe in them and yourselves and things will work out.

That’s some seriously deep stuff right there, but I thought I’d take the opportunity after reflecting on the last 12 months of my life, a life that if things didn’t fall my way could have been over.

It’s a sobering thought.

Now, wipe your tears and have a drink for Kalydeco this weekend and, more importantly, do me a favour and get behind the Aussies when the Ashes kicks off in just 7 days time! Now that Boof Lehmann is in charge, the sky is the limit!

It’s been 29 weeks since I started Kalydeco and, without doubt, it’s been an experience that has changed my life.

Before I started, as you can read below, I was barely able to walk around my own house. Walking up stairs was a struggle, sleep was always interrupted and as a result I was always tired, just couldn’t be bothered doing anything, it was a huge achievement for me to be out of hospital for any longer than 6 weeks.

To put it simply, I was crook. I was staring down the barrel of a lung transplant, something which I’ve never been super keen on, but when you’ve got no other option, you’ve got to what you’ve got to do. It was only going to be a matter of a few short months before I was under the knife, I'm sure of it.

But, as I said, Kalydeco has changed that. I haven’t been in to hospital since I had the big transplant work up I've written about previously. That was back in June 2012. That’s 8 and a half months ago! I haven’t been out of hospital for any longer than a couple of months in about 8 years and I don’t look like going back for some time yet.

Right now, though, I'm getting over a pretty bad cold. It started last weekend and got worse as the week went on, then almost overnight, everything moved to my chest and it's brought back a few memories and made me realise just how fragile Cystic Fibrosis can be.

It’s frightening to think that how I’ve felt for the last few days is how I felt every day for a long time, no wonder I couldn’t be bothered with anything! It’s even more frightening to think where I would be if I hadn’t been given the chance to start Kalydeco. A lung transplant would have been the next step, then who knows what. Even with the advances in technology over the years, it’s still not a 100% guarantee of survival. I've seen both sides of it, good and bad and I’m happy to be able to avoid it for as long as I can!

Already, though, I can feel myself getting better. Before Kalydeco, I’d be on my way to hospital by the end of this week, staring down a 2-3 week stint. I’ve got confidence in my body to bounce back now. It’s a really great feeling.

Day to day life has been much easier over the last 6 months – I haven’t had to rely on treatments everyday just to keep me functioning. I’ve been able to lessen the load of physio, antibiotics and the like, which in turn means my body has started to build it’s self back to some sort of normal. And it means I can fall back on it all and I'm able to rely on oral antis and physio, etc at home to get me better if I need it, rather than needing to go to hospital for IV antis because nothing else is strong enough!

I haven’t missed a game of cricket this season due to illness either, something that hasn’t happened since I started playing about 10 years ago. That’s been the true indicator for me as to how much better I have become recently – Even when I was ‘healthy’, I would still need a game off here and there, but not one was missed this season.

Another indicator that my body doesn’t have to work as hard as it has done my whole life is my weight. When I started Kalydeco, I was eating like a man possessed, whatever I could get in I was, it saw me tip 64 kilos. Not heavy, but the heaviest I've ever been and it's a gain of 13 kilos since January 2012.

While the weight gain alone is way out of character, it’s been the fact that since then, I have dropped back to 62 kilos and have maintained that on the same calorie intake that, previously, would only maintain a weight of between 55 kilos and 57 kilos.

Basically my body is working at a much more efficient rate – Where it needed X amount of food just to maintain the lesser weight before, I can now not push myself to eat so much that I feel sick, yet I can maintain a higher weight and if I needed to put on weight for any reason, I feel I'd be able to no worries. Just a little thing that makes a big difference!

Anyway, having this cold over the last week got me tinking about how ordinary my health was just 6 short months ago. I then read through my previous posts and decided to update my blog.

Not sure if there’s anything worth bottling in this one, but hey, I’m happy to make a big deal of what some/most people might take for granted.

Til next time…

"Where to start?" always seems to be the most difficult part of sitting down to write a new entry. There’s always plenty going on but not all of it is as interesting as some parts, yet it all needs to go in so the juicy stuff all makes sense, so here goes….

A few days after my last post I was off again to the Monash Medical Centre to do a trial for a drug called Bronchitol.

It’s basically a powder-dry version of hypertonic saline that comes in small capsules that get put in to a mouth piece that then punctures the capsules, one at a time. Once that’s done, you suck on the mouthpiece and breath the powder in, irritating your lungs to make you cough and cough until you’ve cleared whatever can be cleared. That’s all done 10 times, twice a day. It’s full on.

I’ve never breathed icing sugar in, but Bronchitol tastes and has the same consistency as icing sugar, so imagine doing that and you can get a pretty fair idea of what it’s like.

Anyway, the trial I sat consisted of me doing the procedure above, mixed in with 4 breathing tests at different stages through out the trial. The trial guidelines state that any person’s lung function can drop by as much as 20% before the trial is deemed unsafe and needs to be stopped, meaning that person is unfit for prescription of the drug.

Mine dropped 19% so I just scraped in! And even then, it was only because I had a better than normal recovery and ended up blowing a better lung function post the trial than before it. You could say I had instant results!

That same day I had my post Kalydeco sweat test, which went much the same way as the pre sweat test, med student watching and all. There was no great surprise or anything to report.

I also picked up my second lot of Kalydeco; that first three months went really quickly!

A few days later I got an email from the CF coordinator at the hospital with my results:

Hi Shane

Please see your results below.

The left column being your pre result and the right your post!

Cheers

Sarah

Sweat Weight:            458                             243     mg

Sweat Sodium:            138 H                         65 H    mmol/L   (0-60)

Sweat Chloride:           110 H                         46       mmol/L   (0-60)

Comments:

Clinical notes: Cystic fibrosis, sweat test collection three months postcommencing Kalydeco.

Comment: Chloride markedly reduced in response to treatment.

To say that I was amazed would be a huge understatement!

Those results are basically saying that my body is now processing and using salt the way any normal human body would, with the only exception being my very slightly elevated Sodium level of 65mmol/L.

That’s still a reduction of 73 points! Not to mention the 64-point reduction and the COMPLETELY normal level of 46mmol/L in sweat chloride!

Like I said, unbelievable! No wonder I feel so good!

To know that Kalydeco is actually doing what it’s supposed to do and having seen first hand the results it’s given me only makes me hungry to work hard to see how healthy I can get now.

I’m a little ashamed to say it, but at some stage over the last 18 months I lost that absolute want to work hard and get/stay healthy. Things got to point were they were just too hard. Coughing, breathing, the whole lot just became really hard work and it made me tired just thinking about it.

Doing the hard stuff had always been something I prided myself on and was complemented on by physios and doctors and it got me down a bit to know that when things did get a bit tough I lost that hunger to excel.

It’s back now though and I’m enjoying busting my gut and working as hard as I can to get my health as good as it can be going forward. I’m not sure if there have been studies done on the psychological benefits of Kalydeco along side the obvious physical ones, but I reckon it might be well worth it.

After a few days of the sweat test results sinking in and being on top of the world, I was ready to confront another dark cloud that has been hanging over my head for the last 6-8 months – Transplant.

Late last week I had an appointment at the Alfred Hospital with the transplant docs and was really positive about the whole thing from the beginning.

I walked in and immediately ran in to the ward round: doctors, nurses, physios, dieticians and numerous other hangers on.

These were all the people that saw a fast and dramatic decline in my health late last year and earlier this year and it was great to see not only the surprise on their faces, but also the anticipation and wonder in their voices of what was next in my life and with my health.

Keeping in mind these are the same people that are STILL fighting their hospital management and admin to be able to dispense Kalydeco and, to be honest, until they had seen me last week, I don’t think they even really believed that one drug, two tablets a day, could make such a difference!

It’s a bit funny, and a bit sad at the same time, that I was peddled that same line I had been over 5 months ago when it came to the question “Are you guys any closer to being able to dispense Kalydeco?” that response being “We’re so close, it’s not the medical people holding it up, it’s the hospital board/legal/ethics teams that won’t do it. But we’re close!”

I was told as I walked out the doors for the last time as an Alfred patient that it could be “as soon as the end of this week”. Like I said, that was over 5 months ago.

It’s just such a shame for the patients who would be eligible for the NPP, that can’t get it and, just as bad, the patients that don’t even know about it because the docs can’t tell them because they’re not allowed to dispense it!

Talk about unethical! But I digress…

The transplant appointment went nearly exactly as I had planned, the only stumbling block being I only got 4 months before I have to go back instead of the 6 months I had set myself up for!

The doc was pretty happy, he read over all the correspondence that had been sent over from the Monash detailing my treatment and progress over the last little while.

He did warn me not to get “sucked in to the story” though. And he has a point.

At the end of the day my lung function is still only approx. 35%. That’s about 2 and a half bad colds/flus, and not complete recovery, away from absolutely needing a transplant. So I do still need to be careful and work at it. Something I’m trying hard to do every day.

It was interesting again, though, when I tried to explain that mine was a bit of a unique situation given me taking Kalydeco. He looked at me blankly for a few seconds before I asked if he knew what that was. His reply: “No. What is it?”

I went through everything, how it works, what it does, results, etc. and I could see him start to wander off in his head thinking “who does this bloke think he is? Trying to tell me about medicine!?”

I just can’t help but think that some people who have others’ lives in their hands have their heads so far up their own behinds that they can’t see what they’re costing their patients as far as a chance at living a longer, more prosperous life.

That’s just the feeling I get and my opinion, now having seen a complete other side to the way some doctors go about treating their patients.

I’m probably lucky to have people in my corner with a keen eye for new research and drug treatments that keep themselves and me informed when it comes to new breakthroughs. Maybe if I thought I knew more about it and had some success with one way of doing things I’d shut myself off from anything new and exciting by way of treatments too.

I could be way off, but I reckon I’ve been around hospitals long enough and have dealt with enough doctors to be able to pick them pretty well. It’s just a feeling I get sometimes.

Anyway, once I finished trying to explain it all to him, walked away a happy man given the time period before he wanted to see me again. He was confident enough to send me away on the proviso that I kept looking after myself and to keep the team in the loop should there be a sudden decline between now and March 2013.

So now, life goes on.

I’m still improving but still have a long way to go before I’m happy with where my health is at. It’s heartening though to be able to see results for any and all work I put in, instead of busting a gut just to stay at a level that isn’t great to begin with.

Improvement is something that went by the wayside for a little while, but now that it’s back, as I said, I’m hungry for more and more! I mean, who doesn’t like to see reward for effort?

I’ll finish off by saying thanks, again, to everyone that has asked about and commented on my health.

In 3 weeks time I will have been out if hospital for 6 months, to think that I couldn’t go 6 weeks without an admission just a few short months ago is almost unbelievable!

So yes, thank you. Your support means the world and it makes that hard work I need to do just that little bit easier.

Til next time.

OK, I’m back!

I’ll apologise again for my lack of blogging over the last month, it’s been hard to find the time to sit down and get everything that’s going on down on paper or on the screen, as the case may be.

It’s hard to write about the attention and questions asked about the blog without pumping up your own tyres too much, but that’s been the case more and more as the time has passed since my last entry. So, thanks to everyone for boosting my motivation and getting my a*** in to gear. And what better timing to sit inside and type one out than a beautiful, sunny, 26 degree Melbourne day! I’d hash-tag sarcasm, but they tell me hash-tags don’t work outside of Twitter or Instagram.

So my last blog was basically a recap of the first month of being on Kalydeco and a trip to Bali that was way too short and way too long ago.

Since then, everything has sort of been cruising along nicely. I got over the trip and the flights there and back with no problems, I haven’t really been sick or well, but I’ve been at a happy medium where things are good. I feel great, but my numbers on paper, where my lungs are concerned, have been either stable or have dropped a touch due to a slight cold/flu about 3 weeks.

I had out patient’s clinic last week where my lung function fell below 30%, which was very disappointing and added to my lack of inspiration to write another update, and was strange given how well everything else has been tracking.

My exercise tolerance is through the roof compared to BK (before Kalydeco), I can run around and kick a footy, I’m getting through cricket training and games with no real issues and being able to day to day stuff is as close to normal as I can be. Like I said, I feel great.

Where the exercise is concerned, it’s almost like my body isn’t creating as much lactic acid – My legs don’t get sore or heavy as much as they used, it’s like I have more oxygen getting around my body, helping everything work as it should. An example of how far I’ve come was at training last week – We set out penalties for bowling poorly, 5 or 10 push-ups per bad ball. True to form, I bowled poorly and went home feeling fresh after doing somewhere between 50-70 push-ups over the course of the night. It’s not a huge number, but when you’ve got spaghetti arms like mine, and could barely blow a candle out after walking a lap of an oval 12 months ago, it’s a big deal.

I’ve put weight on too, I’m now a hefty 61kgs, the heaviest I have ever been and people are commenting on how well I look. That’s something that hasn’t happened for quite a few years, even when I was well previously, I was still under weight and have looked crook in one way or another for years. It’s great to get that sort of feedback from people. It’s refreshing to have shit hung on you because your chest and arms are growing, it’s just never happened before.

I get a real idea of how far I’ve come when I think back to last Christmas – I was 51kgs in January. That’s a BMI of 17.2 – The lowest of the normal range is 18. I was able to put on half the weight by myself, but I was stretching it every day. I had to take a weight gain supplement with every meal and just hated the thought of eating. It’s completely different now, no supplements, minimal junk, I won’t go as far as saying I’m on a lean and mean diet, but it’s nothing like it used to be.

My weight etc. is good on paper too – My BMI is up to 21, which is smack-bang in the middle of that normal range for my age and height. The last time I was anywhere north of the lower side of normal was back in the late 90’s as a kid. That was when my lung function was hovering around 70-80%. 

I feel like my body is stronger now too – It got to a point BK where I felt really weak all the time, my muscles would get fatigued seriously quickly and I’d start to shake like I’d done 100 push ups, just from bringing the shopping in from the car. That’s stopped. I feel stronger, like everything has been rebuilding its self, back to what it was before I got really sick.

All of that has come on the back of very little junk food too – I won’t lie, when I was sick with the flu, my cravings went up/motivation for cooking food went down and I was eating McDonald’s for breakfast every morning, and would push through a Popcorn Chicken here and there if I was desperate for some food. But I didn’t enjoy it; it actually made me feel sick. The only times I’ve really enjoyed take away like I used to was last weekend and the weekend before on the back of two big Saturday nights. It’s strange; I used to live on the stuff!

In the last month I’ve been trying a few different things, tinkering with the way I take Kalydeco, the foods I take it with, etc. all due to the fact I haven’t had the big improvement in lung function. Given that everything else was going OK, I thought it was time to try a few things.

I’ve worked out that I need a serious intake of fat more so than a high calorie meal, and as a result, need to take more Creon to get the full effect. Four Creon before I start, then eating half and taking another Creon with Kalydeco, then finishing off is the routine that seems to work best. Toasted ham and cheese sandwiches, hot dogs and banana flavoured Big M’s have been on high rotation.

Since I started doing this, there’s been a steady improvement, I’ve noticed my cough is more productive, but what’s coming up is lighter and thinner than anything I’ve coughed up in living memory. I have a before and after photo of some sputum, but I’ll save your eyes and stomach the trouble, you can just take my word for it.

My FEV1 is the highest it’s been a long time too – Before I started Kalydeco I posted it was 21%. Two days ago it had cracked 35% and I can still feel improvement coming.

I can’t tell you how exciting that is. It’s still low, but when you’re dealing with sub 20 numbers (the worst one I had done earlier this year was 18%), anything above 30 is amazing.

I’ve got a follow up sweat test booked in for next month sometime which is exciting, it’ll let me know exactly how well Kalydeco is working, looking forward to that. I’ll also be taking part in a 1-hour trial for a new inhaled drug that could help even further, but I’ll save that one for next time. Hopefully there’s something promising and/or entertaining to talk about there!

I think the biggest and most important thing to come out of all of this so far is the confidence I have back that going to hospital and being treated for chest infections isn’t what will keep me alive and, potentially, will let me keep my own lungs for who knows how long.

Hospital has been such a big part of my life forever and in particular over the last 12 months, but I’ve been out now for 18 weeks and don’t even look like going back in. “I ain’t goin’ back, I ain’t goin’ back!” for anyone that has seen Bad Boys 2!

Anyway, that’s about it for now – It feels good to write all this down, so thanks again to everyone that’s been on my back about it.

Give me a week or so and you can feel free to start again!

Good luck to anyone having a punt this weekend too, hope you can back a winner in the Cup.

So, first off I’ll apologize for my non-blogging over the last few weeks, things got a bit hectic before a trip to Bali and then there was the trip its self, of course. But I’m back on deck now and ready to jump in headfirst!

I wrote in my last post that I had a big appetite and had put on a couple of kegs, I’m happy to report that that trend has continued and I have tipped the scales at a whopping 60kgs for the first time in over 3 years! I feel and look a lot less bony than I did, that probably goes without saying, but it’s an improvement none the less.

The last time I was this heavy (if you can call it that) was when I was at my healthiest since I finished year 12 – I had a Lung Function of approximately 55% and I weighed 60kgs. Hopefully my lung function can get back to somewhere near that over the next few months and can keep moving upward from there.

One of the major changes has come in the form of a distinct lack of craving for junk food. I used to live on take away – KFC, McDonald’s, Red Rooster were all on high rotation. But since starting Kalydeco I haven’t craved any of these, or other fast foods, at all! It’s not quite at the point of feeling crook at the mention of it, but I would much prefer something cooked at home than anything cooked by a pimply-faced kid with more grease in his hair than in the deep fryer! I’m not much in to stereotypes, but that one just felt like it fits.

I’ve read up on it and the thinking behind it is that people on Kalydeco don’t crave salt as much as they did when their body wasn’t absorbing it. Meaning that because my body is retaining salt and doing what it should be doing, I don’t need or crave as much as before.

I can safely say that, not only could I have not gone to Bali in the first place, but there’s absolutely no way I could have done half of what I did over there without Kalydeco!

Waterbom Park is the perfect example: All the slides start at the top of a tower with about 10 flights of stairs between them and the ground. I wouldn’t have got through 2 before starting Kalydeco, yet I was able to not only get through all 10 flights each time (about 15 times through out the day), but was first up the tower ready to go on most occasions. It was only on the last couple of goes that I started to struggle and get tired, needing a rest before the slides. But even then, it was nothing compared to what I was like as little as month ago!

I’ve noticed a huge difference in the taste and feel of my sweat too, it’s hardly salty at all now where it was almost unbearable to taste when CF was in full flight. My theory was backed up when we were playing golf at Nirwana – I only had a few Pringles with my tablet and could feel my chest reverting back to it’s old ways and once we’d finished and the sweat dried, I felt pretty crusty and tasted like a minimum chips with extra salt!

The prescribing guidelines tell you to take Kalydeco with fatty or high calorie foods. It limits the significance of side effects and helps the tablet “take” and do its thing. It’s hard to say, because Bali Belly was going around the villa at the time, but I did have an upset stomach. It didn’t hang around long enough to be classified as Bali Belly, in my mind, so I’m going with it as being a side effect due to not having enough fat/calorie content when I took my tablet that morning.

All of the physical stuff we did, walking, swimming, pool volleyball, the water slides, golf to a certain extent, all came pretty easily compared to what they would have been like at home, let alone in a very hot and humid environment. The flight over was done with no worries and it was only because of a delay in taking my last dose over there that the flight home was any different. Usually I would be straight in to hospital for an admission after any plane trip, but I feel pretty good at the moment and don’t think any admission is on the radar for the foreseeable future.

I have read on other blogs that people taking Kalydeco have noticed their fingers and feet are less wrinkly when they’re swimming, etc. I can attest to this one too! It would only take a few minutes for my fingers to get wrinkly in a pool or even in the shower previously, last week though, I was in a pool all day for days on end and noticed hardly any wrinkling at all. Even Mandy’s hands were wrinklier at one point and I think I’d been swimming for longer than she had at the time!

The people I was with (Cal, Justin and Shannon – There’s your mention!) all said that they noticed my distinct lack of coughing throughout the whole holiday too, so it was good to get feed back from the outside that things are working.

And looking through photos from the trip, even I can see how sick I was and looked before starting Kalydeco compared to now – It’s a massive boost when you can see improvement like that!

The last dose of the Bali trip was taken about an hour late due to traffic jams like you wouldn’t believe and I coughed most of way home on the plane. It was a return of the really ‘CF sounding’ cough that had disappeared nearly all together. Up to that point I can’t remember having a real cough fit that were a more than once daily occurrence before starting Kalydeco.

I had an appointment booked in with the doc for yesterday, but had it changed due to a red eye flight from Bali, I wasn’t going to not enjoy a day in bed after a sleepless flight, so I’m booked in to go back in a few weeks for follow up testing on my lung function and I think they’ll want me to do a follow up sweat test to see where my levels are at after nearly a month taking Kalydeco. (It really does need a nickname! Maybe Special K or K will do the trick?)

So, Special K, has made a significant difference already and I’m sure there is still more to come – I haven’t yet had that massive clean out of old mucus that people have talked about and I haven’t really done much by way of fitness work other than cricket training. I’ve got plans to give it a good shake over the next few weeks, so hopefully I can have some big improvement once I try a few different things. Fingers crossed, hey? Rest assured though, things have changed are still changing. It's an exciting time! It's just a pity K couldn't help the Eagles get over the line against Collingwood a couple of weeks ago...!

Until next time, may your news be good news!

Well, well here we are: A week and a bit in and there have been a few noticeable differences…

The first thing I have noticed is how much I’m eating and how hungry I’m getting! Instead of looking at my food and thinking “Shit, how am I going to fit all of this in?” I’m now looking around saying “I’m bloody hungry, where’s my next meal coming from?!”

As a result, I’ve put on 2 and a bit kilos in a week! I wouldn’t have put on 2 kilos in a year recently so I’m stoked!

Certain things are tasting different too, not better or worse, just different.

Another change has been me sleeping much better. Sleep was never really an issue, I could sleep for hours before, but now I’m sleeping much deeper and, unfortunately for Mandy, she tells me I’m snoring like nothing else! Stiff Mandy.

I think it might be as a result of better sleep, but I’ve got more energy too.

My exercise tolerance has improved already, where I was really blowing up before I’m only slightly out of breath now – Can’t wait to do some fitness work and see how much better I can get!

I have a portable lung function meter at home and my numbers have been stable over the last week so I’m looking forward to some improvement there in the coming weeks.

The one thing I’m measuring my exercise tolerance on at the moment is cricket training on a Monday night.

Last cricket season it got to a point where if there was any sort of running or fielding drills as part of our warm ups I’d have to sit them out. I just couldn’t get through them and I’d be too tired to do much of the other stuff, so I was preserving my energy for batting and bowling.

Even the first few sessions of this year (we’ve been training for about a month now) were a bit of a struggle and I hadn’t really taken part in any warm ups at all.

Last night, however, I was able to run around and kick the footy before we started, I took part in three quarters of a fielding drill that involved a fair bit of running and then I was able to bowl most of the night, except for the last 15 minutes where it got a bit much.

I was very pleased to say the least!

Another thing has been how easy it is to bring up anything I need to – Before I’d have to really cough and strain myself to get anything up, now it’s coming up relatively easily. It’s almost like, even though it’s still thick and sticky, it’s a bit lighter. Like it has more air in it or something, it's hard to explain.

I had to stop hypertonic saline treatment a while ago but am going to start again to see what happens. It might be something that can kick start the really yucky old stuff coming up.

I'm extra motivated due to a holiday coming up next week. Need to be as healthy as possible before I go, I haven't really flown well in the past so it's going to be interesting to see how that goes too!

We’ve been doing a bit of research in to the time frames others have had in terms of getting massive improvement in their chests – We’ve seen anywhere from 2 hours to 6 weeks so I’m not too concerned I haven’t been coughing up any of the really old stuff or that I haven’t seen an improvement in my lung function, it’s only been a week after all.

I'm happy-ish so far - I haven't gone backwards by any means and there have been improvements in a heap of areas so it's all looking good. I just wish I could give some first hand accounts of some of the stuff I have read and heard from others.

Maybe next time!

I’ve been inundated with “get your next blog post up ASAP” messages and calls so I need to tell everyone that I haven’t been putting it off, it’s been more ‘a wait to have something awesome to tell everyone’ gap between my last post and this one…!

We found out last Friday, after speaking to another patient taking Kalydeco that a big shipment of it had arrived at the hospital and they were in the process of drawing up the scripts ready for them to dispensed.

Mandy got on the phone straight away and rang the doc – She was excited and we made an appointment for Monday morning.

As you would have seen from the video below, I took my first tablet at 10pm Monday night.

Unfortunately, the change hasn’t been as dramatic as I had hoped but there are definitely things happening that weren't before.

The main thing I’ve noticed is my chest not getting so dry over night and ‘that’ cough in the morning is nowhere near as severe as it has been!

I’m not getting as puffed out doing normal things like making the bed, walking up stairs, etc. and anything I’m coughing up is coming up much easier than it was and it tastes a bit different.

I’m not bringing up a huge amount of anything and certainly none of the really old stuff that others have spoken about. But it's nowhere near the struggle it was to get the same amount out.

I’m eating more, partly because you need to when you take the tablets and partly because I’m getting hungry when I wouldn’t have been before. This has resulted in putting on 1kg in a couple of days.

I’ve heard it takes anywhere between 2 hours and 5 days for things to really start heating up and everything to get moving. I’m looking like a 5-day prospect, I think.

One of the good things about having things change slowly has been a lack of side effects – No flu like symptoms, no headaches, rash or sore joints. This is something I was really worried about and that others have reported as a real downside to taking Kalydeco.

Half of me was expecting nothing to happen at all, but I was so hopeful I’d be one of the ones that noticed a massive difference within the first couple of hours after taking their first pill. I'm happy to be one of the ones it takes longer to work on, so long as it actually happens!

I just keep telling myself to take small steps and things will definitely happen. I’ve ramped up my physio and inhalations in a bid to fast track it all so fingers crossed for something in the next couple of days! And if nothing else, the docs and physios will be happy I've pulled my finger out and we'll see some improvement regardless!

It’ll happen and I seriously can’t wait for a big change, it’s just a matter of time and you guys will be among the first to know!

So it’s finally happened, here’s the proof!

Let’s hope this is the first tablet of many and the start of something completely off the charts!

I’ll post a bit more of a detailed entry tomorrow, but I just couldn’t wait to get this up!

It’s been a slow few days since Thursday when I had my appointment with the transplant team at the Alfred. I’m in a holding pattern, no transplant list but also no Kalydeco.

I had decided before I went in that my plan was to tell them I needed more time to think about what I was going to do – I had made my mind up that they would tell me I needed a transplant so there was no shock when the doctor, who I’d never met and who failed to introduce herself, told me that was her recommendation.

There was a bit of discussion back and forth but nothing too deep or intellectual. She told me she didn’t have many questions because she had received a letter from my new doctor at Monash a couple of days prior, explaining the situation RE my transferring across and hope for the results of taking Kalydeco.

The doc seemed pretty positive and was happy to hold off any decision making until at least November when I’d go back for a review after having been taking Kalydeco for a couple of months at least (hopefully).

When the words “Kalydeco has come too late for you” my heart skipped a beat. But she clarified what she meant by saying in an ideal world I would have been taking Kalydeco since birth in order to avoid any irreparable damage to my lungs.  Yes, and in an ideal world I’d be 6’5, 100kgs and be able to kick a footy 75m on my left foot. I didn’t need the mini heart attack, thanks love!

It’s interesting when I think back to when the transplant talks all started – It was January this year. I had been what, I thought, was a little sick for a about a month, but was putting off a hospital admission because of the Christmas/New Year break and then the Australia Day long weekend which included a very good mates Bucks’ Day. I figured I had a good window between his Bucks and wedding in which I’d get in to hospital and get out again, good as new.

Sometime between early Jan and Australia Day I fractured a rib from coughing and that sort of sent me in a downward spiral. It was painful to say the least and I needed to dose myself up on Nurofen and other pain killers to get by.

The pain came in waves, sometime I could go half a day without any actual ‘pain’, it was always uncomfortable, but it wasn’t too bad at times. One night it got to the point where I needed to go to hospital though. I had been at the cricket club on a Thursday night and stopped at the supermarket to get something on the way home. My chest was bit sore but nothing too bad, I took a phone call in the car that lasted about 10 minutes, then ran in to a mate in the car park on the way in. The pain came on pretty suddenly, and I didn’t get to the door of the shop before having to turn around and get back to the car to drive home ASAP.

I had a shower in the hope it’d calm down, but that was no good so I had to get Mandy to drive me to the emergency department at the Austin Hospital.

They gave me a couple of panadein forte and told me to wait half an hour.

The pain was no better so they took me in and gave me some stronger stuff. That worked, but the doctor was pretty concerned about my chest infection.  They took some blood and measured my oxygen levels, which were low. The blood tests came back and everything pointed to a pretty bad chest infection.  The doc wanted to either admit me or transfer me to the Alfred by ambulance. I told him I’d ring the Alfred the following Monday (this was a Thursday night).

The pain stayed away for the most part and I got through another two or so weeks before I got a bed at the Alfred. My oxygen levels on admission were a pretty low 85 and I needed oxygen for a few days.

I lost about 8 kilos in the lead up to that admission and hadn’t taken a proper, deep breath since I fractured my rib. I think it was about a 3 - 4 week period. Looking back, that was a scary time. I know I won’t make that mistake again.

That admission I didn’t do a lung function test on arrival and it only got back to about 30% by the time I went home.

During the two weeks I was there I had appointments with counsellors, doctors and specialists, all to do with transplant, that I’d never seen before.

It was also during this admission that Kalydeco was approved by the FDA in America so I was very hopeful that it wouldn’t be too far away from release or availability in Australia.

We had absolutely no idea though, so even though it was a long way off in my mind, transplant was a very real, very confronting reality.

My lowest recorded lung function this year was 18% - That was just before I started the steroids, which helped enormously.

They helped my lung function back to 35% in 2 months. It was the first time it had gone up without a hospital admission since I could remember. It was a massive relief as my doctor had told me that my CF was actually only mid-range and that there was another underlying problem. If the ‘roids didn’t help, he said he wasn’t sure whether any drug, Kalydeco included, would help. Transplant would have been the one and only option.

That’s enough doom and gloom for today I think!

Since Thursday I’ve had a couple of emails from my doctor who said I now have TGA approval and was in the system of the drug distributor and they were only waiting on delivery. I’ve heard it’s about 3 days after TGA approval that the hospital takes delivery. Today was day 3.

“Very soon” was the time frame I got, but I’m still waiting. Patiently, I might add! It’s very hard not to get on the phone every hour and ask where it is!

Hopefully it’s coming in the next day or so and my next update will include some form of early result or progress!

There’s nothing yet though, unfortunately.

I wrote this before cricket training tonight so this bit is a bit of a post script, more of an insight to how I’m feeling right now:

Two weeks ago I wrote of how I could only bowl ten balls at training and had to pack up and watch for the rest of the night. Then a week ago a thought I may be improving a touch as I was able to bowl all night with a rest in between each ball, basically doing half as much as everyone else.

But tonight was another night where I wasn’t quite up to scratch. I was going OK, but about half an hour – 45 minutes in I had to stop, I thought just for a drink of water and I’d be OK to go again, but I just didn’t quite have the energy to get going. It’s a shame; I was bowling really well!

I’m not sure which session to take the most away from, the ‘healthy’ session or the ‘not so healthy’ sessions.

Ah well, one more thing to look forward to with Kalydeco!

I was up early this morning to make the trek out to Clayton for a baseline sweat test, pre Kalydeco, at the Monash Medical Centre.

One thing I won’t miss about mornings, early or otherwise, is the first cough of the day!

I’ll explain it as best I can, both for you and for me to look back on, hopefully, and realise what I’m NOT missing.

The best way to describe it is: Think of an old car trying to kick over first thing on a cold morning.

It’s usually after I have a shower, drying myself makes me a bit out of breath then my chest gets a bit tight, I know it’s coming but more often than not, the actual coughing fit comes a bit from nowhere, but it goes like this:

*Cough, cough, cough, cough, cough, cough* Take a breath. *Cough, cough, cough, cough, cough* Try to take a deep breath but only get half way. *Cough, cough, cough, cough, cough* Take a breath.

By this stage your eyes are watering and your head feels like it’s about to explode, your stomach muscles are sore and feel like they could cramp at any second.

There’s a few more dry coughs before you get a bit of moisture in there (think: oil getting around an old engine ready for it to get going). By now I’ve usually got enough control to slow my breathing down a bit and stop the coughing fit. But there are always two or three more coughs and on some days there's some dry retching, then a bit of mucus will come up and it's like the engine has started, ready for the day ahead! It doesn’t take long to recover, but it’s a pretty full on experience.

This happens every morning, the only exception being for the first few days (used to be weeks) after a hospital admission.

Anyway, back to today’s sweat test – The last time I had one done I was about 6 months old, it was how I was diagnosed with CF. Obviously I don’t remember it so I wasn’t sure what to expect when I sat down and saw what looked like a chemistry set with a battery charger tacked on to one side.

First off they clean your arm with some super strong alcohol to get rid of any bugs, etc. that might be hanging around. They then put two square cotton patches in place on the inside of your arm, roughly 10cm apart, before soaking those with a special chemical that reacts when an electric current runs through it.

Next comes the fun bit: they attach two electrodes to your arm and turn the machine on, slowly increasing the amount of electricity being pumped in to your body until all your hair is standing on end, you start to shake and feel like you’re about to lift off the chair…. Not really.

It does feels like there are about 100 ants with sharp claws under each of the cotton patches, though. They need to keep these wet with the chemical (not sure of the name) and each time they drip a bit more in it gets a bit more uncomfortable.

That’s done for about 10 minutes, which is long enough to stimulate the sweat glands sufficiently for the test to begin.

The test is really simple and painless, all they do is put a ‘weighed and dried’ piece of gauze where one of the cotton patches was, just above the wrist, cover it with a thin, clear plastic and tape it down. Then bandage it up and leave it for 45 minutes.

They told me I could do whatever I liked for that 45 minutes, so I went to the car and tried to have a sleep! That was no good though so I read the free paper I got with my Bacon and Egg McMuffin breakfast on the way, just one of the many benefits of an hour and a half drive in peak hour!

Once the 45 minutes is up, the bandage, tape and plastic comes off and the gauze is placed in a sterile container and sent off to the lab for testing. The testing includes weighing the gauze and measuring the amount of sodium and chloride expressed throughout the test. I should know the results by Thursday or Friday.

When I was called back in to the room to finalise the test, I was introduced to the head of the ‘sweat test’ department who was pretty excited to meet me. He said something along the lines of “we’ve been doing these tests for 30 years and this is the first time we’ve seen the sorts of results Kalydeco is producing” and that “this is certainly a new frontier and a very exciting time”.

I may have covered this previously, but apparently I’m only the 4th or 5th person on Kalydeco through the Monash and possibly the first male patient. As the doc said, exciting times.

We had a slight hope that today could have been the day I actually received Kalydeco, but there has been no call yet. I tried calling the CF department before I left the hospital but no one answered the phone. (They have a pretty strict cross-infection policy so I didn’t actually go up there to see if I could speak to anyone, I figured they’d ring if they had any news)

So that’s one side of what’s happening this week. The other side is transplant talks.

I’ve got an appointment with the transplant team at the Alfred on Thursday afternoon. I haven’t made a decision yet on what I’ll do if they tell me I need a transplant, hopefully Kalydeco will be doing its thing while I’m thinking about it all and I’ll be able to put it off for a while. Who knows how long for!

Most people who will read this will know that I have Cystic Fibrosis and that the main/only reason for keeping this blog is to track my progress when I start a new gene-altering medication.

That medication is Kalydeco. It was made readily available for patients in the US with the correct genotype at the beginning of 2012 and May it was made available here via a Named Patient Program through Vertex, the drug company responsible for this amazing pill.  (For those that want to know more about it, go and have a look on old mate Google or his buddy Wikipedia).

So, after a decent 3-month battle with the Alfred hospital to access Kalydeco via the NPP, I made the decision to switch to the cross-town rivals at Monash Medical Centre to better my chances of getting Kalydeco ASAP. It was a tough decision and one that I’ve been throwing around in my head seriously for about 6 weeks, given my familiarity with the Alfred and the relationships I’ve built up over the last 8 years, but in the end I had to bite the bullet.

*I say cross-town rivals in jest, I had the full support of my treating doctor at the Alfred and he was happy to give me the referral I needed along with my recent medical history and all the other required info for a transfer*

My application for a place on the NPP was sent within 2 hours of walking through the front door at Monash! It all happened so quickly and without fuss that I didn’t actually know if it had happened at all...! I should have it or know if I'm not getting it within 10 days.

I’m not getting overly excited just yet though; due to so many false starts at the Alfred.

I keep picturing in my head the dude that gives the OK for me to participate in the NPP shaking his head and knocking me back. Maybe that’s so I won’t be as disappointed if that does actually happen! I’m smart enough to realise, though, that Monday was a very significant day in my life.

I spoke to a friend of mine who suggested I should try and keep a diary between now and when I start Kalydeco to give an insight in to what life is like at the moment and for me to look back on in a few months time to make sure I appreciate the difference it makes.

Here’s a brief medical report from Today – This will be as close to the sickest I will be before I start taking Kalydeco.

Height: 172cm

Weight: 56kg

BMI: 20

FEV1: 1.03 ltrs or 21% of the predicted ‘normal’ without any medication.

I’m not on the lung transplant list yet, but I have an appointment the docs there later this month and I fully expect the team to make a recommendation for a transplant given the results from the testing I had 6 weeks ago.

I’ve felt increasingly tired over the last few weeks, my mucus is thick, sticky, and difficult to cough up and green in colour. Coughing is a chore; it’s turning in to real hard work where it hasn’t been before. I’m a bit off my food and am struggling sometimes to find the motivation to eat.

My asthma has been playing up and I’ve needed to increase my use of  Symbicort to try and get that under control again.

Some or most of this could be explained away by the fact I’ve stopped taking the steroids I was on for the Bronchiolitis that was diagnosed around the same time Kalydeco was made available for Australians.

Monday night I had my first cricket pre-season training session and was able to bowl about 10 balls before I had to pack it in. I thought I felt a bit better than that, but apparently not. Last season I was only able to play 5 games and bowled about 60 overs across those games. The year before I played nearly every game and bowled upwards of 200 overs. That just goes to show the decline in my health over the last 18 months.

I’ve always had a pretty good exercise tolerance and it may get better as a matter of course as we get further in to the season. But given how I felt beforehand, I’m starting to think my body has just adapted to a lower level of “getting by” and I’m not in as good a shape as I thought I was.

Anyway, I suppose I had to start somewhere and this was it. 26 years stuck in phase one of my life and I’m about to kick off phase 2. Who knows what’s ahead!

One last thing for today is a big thank you to everyone who has worked with me and for me to get to this point. There are too many to name and I’ll no doubt forget one or two if I try to, but I will single out my wife, Mandy. She’s an amazing woman who has worked harder than anyone. Me included.

I’ll be back, all up in your grill soon.