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CF Wonder Drug

There have been some amazing developments in treatment of cystic fibrosis over the last few years, changing the lives of those living with the condition.

kalydecocollA couple of years ago, the world exploded with the release of Kalydeco. It was a drug aimed at certain CF gene types (there are many types out there, so this drug wasn’t suitable for everyone) 

It is a little pill that treats the underlying cause of cystic fibrosis rather than just the symptoms. The results of the drug were ground-breaking and life changing. People taking the drug were showing lung function improvement of up to 65% and had a new lease on life.

The downfall of such an amazing drug was the price. The cost of Kalydeco was around $300,000 a year! It simply wasn’t affordable.

After much lobbying and petitions, the Australian government approved Kalydeco on to the Pharmaceutical Benefits Scheme (PBS) which caused a sigh of relief around the country as the drug became much more affordable.

orkamabiFast forward to 2016, and another drug has hit the market. This one is called Orkambi, which is aimed at another gene type. Our family is excited about this one as it is aimed at Cameron’s gene type! This drug is expected to help almost half the Australian CF population. It has similar effects as Kalydeco

Again, the downfall is the price. Orkambi is currently about $260,000 a year!!

There was a recent petition to have it placed on the PBS list to make it affordable for everyone. However in the “wisdom” of the Australian government, they REJECTED the bid, claiming it was not “cost-effective”.

I don’t understand how they could think this? It is improving quality of life. It is improving health, which means less of other medications, less treatments, less time in hospital. Surely that all adds up in the end?

price

How much? Too much!!

So the petitions are starting again. Click here to sign and help show your support.

This drug is important and needs to be made more affordable! Lives depend on it!

Bella 🙂

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Our CF journey so far…

On the 16th May, it will be seven years since our family was completed. On the 12th June, it will be seven years since our family member’s lives were irreversibly changed by two little letters.

Cam funny

Cam- our little joker!

The last seven years has had its up’s and down’s. Cameron is a vivacious little boy who lives his life to the fullest. He doesn’t walk, he runs through life (literally and figuratively!) If there is a joke to be found…Cam will find it! His diagnosis has not dragged him down in the slightest.

But that doesn’t mean there haven’t been hard times.

In the last seven years, Cam has been admitted in to hospital four times. There was one when he was diagnosed, sick with double pneumonia and underweight due to malabsorption.

The second time was when he was two years old. A bronchoscopy at annual review that year showed he was growing the dreaded super-bug Pseudomonas in his lungs, which is an instant minimum two-week admission into hospital for intravenous antibiotics.

Cam sick

Hospital is never fun 😦

The third time was unrelated to his cystic fibrosis. He had severe tonsillitis and his throat closed over. It was a scary 24 hours! We were more worried about that than anything he had gone through with his CF by that point.

The fourth time, he was five years old, and another routine bronchoscopy showed Pseudomonas again, which resulted in a gruelling two weeks in hospital and another week of treatment at home. We were all exhausted by the end, and it was a rather traumatic experience for Cam which resulted in a slight fear of needles.

Over the years, he has also had a staph infection twice. The first time, we discovered he was allergic to one of the antibiotics they used to treat it.

Cam nebuliser

He hates nebulizer treatments

He currently has about 30-35 tablets a day to keep him healthy. This includes Creon tablets (which help him digest his food), salt tablets (as salt loss is massive with CF-ers), a multivitamin, Vitamin D tablets (to ward off osteoporosis, which is high risk in CF-ers) and antibiotics to prevent infections. This doesn’t include when he has a moist cough, where we treat him with two weeks of nebulizer antibiotics at home. If there is no improvement after that time, it requires a hospital admission for IV drugs.

Cam Pep

Cam using his PEP mask. He will try anything to get out of doing it

He has progressed from percussion physiotherapy to resistance breathing techniques using a PEP mask and “huffing” through a wide pipe to help move any mucous around in his lungs. He is now in charge of his own physiotherapy (of course, under close watch by us, as he is well-known for trying to worm his way out of it!)

His high fat, high salt diet is now easily maintained. It was a massive learning curve when he started solids, but now we just add the “goodies” to meals once separated from the rest of the family’s meal, or add a few extra high-calorie snacks to his lunch box, and extra helpings whenever he requests. Cam is a little on the shorter side, but in terms or weight and build…he is actually a little Buddha for a CF kid , who are generally notoriously lanky/skinny.

Cam eating

This kid LOVES his food!

He is pretty good about dealing with his condition. About two years ago, he had a little breakdown. He suddenly didn’t want to do his physio. He didn’t want to have his medication. He didn’t want needles. He didn’t want to see doctors. He didn’t want to have cystic fibrosis anymore. There was a bit of focus on him at school as he was in pre-primary, and the other kids were taking notice that he was a bit different. In the past, his outlook was “I am different, and that makes me AWESOME!”…suddenly, it was “I’m different, and I don’t know if I like it anymore…”

We decided to just pull back a bit and allow him to work through it on his own. We didn’t push him in to anything he didn’t want to do (with the exception of physio and medication…sorry buddy…no escaping that!) We let him talk things out. I spoke to his teacher, and they tried to make things as discreet as possible at school (ie. having medication away from the rest of class)

I have to admit, I was a bit surprised at how young he was when his “breakdown” came. I am sure that many more are expected over the coming years, but I’m sure we can work through it.

We don’t stop Cam from doing anything. We want him to experience everything he can. He recently started sword fighting classes, which he loves considering he loves pirates and ninjas! He has a great circle of friends who understand his condition, but don’t see him as his condition. He plays pranks on his family, much to our chagrin sometimes. He also gives the best cuddles (but not in front of anyone….of course!)

Silly Cam

Nothing is going to stop this kid!

Life certainly isn’t easy when you live with cystic fibrosis, but it certainly isn’t as bad as what I envisioned it would be when he was first diagnosed. I’m not making light of the condition….CF sucks…it truly, truly sucks. But we have been lucky with our experience so far….a lot of other families haven’t.

May is CF Awareness Month. 65 Roses Day is on the 27th May this year. All I ask is that you take a moment to familiarize yourself with the condition. I’m not going to push for donations (though it would be awesome if you could) but the whole focus is awareness.

I just hope that sharing our experiences, it sheds a little light.

 

Bella 🙂

 

Well HOWDY!

Im alive

 

Well HELLO there!!

I know…it has been a very long time since I posted anything…but I just wanted to shout out that I am still indeed alive and kicking. Life just got in the way!

I hope to be able to to update you all on some of that in the near future. Please feel free to kick my butt in the meantime in the comments or on my blog Facebook page.

I have a lot of stories and anecdotes in the works. Hopefully I will start pumping them out soon.

I hope you haven’t all forgotten me!

Love Bella 🙂

 

Good part, bad part, funny part

I want to share a little game/ritual we have in our household. I actually got this idea from another mother at school while waiting for our kids to exit class.
Our kids, particularly our eldest, Aaron, was having a hard time at school. He was being bullied relentlessly by another student, and was struggling with anxiety. He wouldn’t tell us much of what had happened at school, and we would usually find out details from his siblings, or even other students.

So we started sharing our days in a fun way around the dinner table.

Each night, we all take a turn of sharing what was our best part, bad part, and the funniest thing that we saw/heard or did that day.

The “good” part is usually about the meal (to compliment the chef!) and ranges from winning an award, getting to play a certain game enjoyed at school, or, in the parent’s case, it could be that we finished work early that day.

The “bad” part is where things get interesting. This is where we find out if they are having a hard time at school, or if they hurt themselves during the day, or if they have had a fight with one of their friends. In the parental case, it might be that we had a really yucky job that day, etc. We share the bad experiences, and we work together to figure out a way to solve the issue.

The “funny” part was an idea of the kids. We didn’t originally have this part, but they wanted to finish on a good note. Usually this is a joke they heard at school, or funny experience, like Dad singing “Let it go” really loudly, or someone farted at an inappropriate time, or the cat playing with a toy crazily.

It is one of the most anticipated parts of our day, and no one is exempt, even dinner guests! But we relish being able to share everything together. We encourage honesty and push to have faith/trust in each other, kids and adults alike. My husband and I sharing our days showed that we were approachable. I think our openness with each other makes us stronger. The school have now realised, that if you mess with one of our kids, you mess with all of them.  They look out for each other in the playground. Their bond is unbreakable.

We are family!

Do you have a tradition like this in your family? Share in the comments below!

Bella 🙂

It’s been AAAAGES!! (sorry!)

I know, I know….it’s been a while since I have written anything of substance! Forgive me…it’s been hectic here!!

So much has happened in the last few months that I barely know where to start!

Cameron has had a bit of a rough time. When he had his annual review bronchoscopy about four months ago, they found that he was growing staph in his lungs. He went on some heavy-duty oral and nebulizer antibiotics for a while. He was quite sick in this period. He went off his food and dropped a fair bit of weight, which are still struggling to get him to put back on….but at least he isn’t sickly looking any more.

Medication time in our house!

Medication time in our house!

When we returned to Perth for a checkup about a month ago, we came prepared for an admission for IV drugs (which is a minimum two-week admission) We managed to escape that bullet by the skin of our teeth! The doctor was fairly certain he no longer had a staph infection anymore, that he had picked up a different bug. We were to return home and do two weeks of nebulizer antibiotics again. If there was no improvement in that fortnight….absolutely, 100%, Cameron was going to be admitted into Princess Margaret Hospital for treatment. If there was improvement, but no better within a month, we had to return within 6 weeks for a review. If he was completely fine within two weeks, then we didn’t have to return for three months.

Eva has become a real little helper. Often assisting with meal prep and has earnt the trust to get Cameron's meds ready for him

Eva has become a real little helper. Often assisting with meal prep and has earnt the trust to get Cameron’s meds ready for hin we didn’t have to return for three months.

So many variables! I am happy to say that he was completely fine after one fortnight of drugs. His weight is still a kilogram lighter than he was three months ago, but in the meantime, he is throughly enjoying all the chips, chocolate and smoothies we are stuffing into him!

Eva is going…well…ok. We still don’t have any answers with any of her issues. The respiratory doctor put her on a new drug called Singulair, which is classed as an asthma preventer. While they are confident she doesn’t have asthma either, this drug is still in test phase on her. We all doubt it is actually going to do anything, but it’s worth a shot. She is still on three-monthly reviews at respiratory clinic.
Her food compulsion is peaking a little bit at the moment. While it is nowhere near as bad as it used to be, we have had a couple of incidents where she has stolen food, hovers around food, gorges while eating occasionally and once was caught eating the fat at the bottom of a baking tray I hadn’t yet cleaned. All we can really do is be vigilant until we find out what could be causing that.
We recently had an appointment with an endocrinologist (a doctor who deals mostly with hormones) Last time that we saw him, blood tests were ordered to check for a genetic condition called Prader Willi Syndrome, which she exhibits a lot of red flags for. They were also check her thyroid levels.
However, there is a problem…they seem to have lost test results 😦 So no answers as to her issues yet.

Ethan *LOVES* Spiderman!

Ethan *LOVES* Spiderman!

Ethan has been going really well with his constipation issues. The continence specialist we have been seeing the last six months has done some amazing things for him! Ethan is now in undies during the day, and almost ready to be out of nappies at night. As I write this, he does seem to be having a bit of constipation issues again, but a bit of tweaking to his medication (Movicol Half) will fix that up in no time. He is almost ready to be discharged from the specialist as we are so happy with his progress…thank god!!

Aaron has been plodding along with school life. He is still VERY attached to the Playstation….addictively so! I am still pushing to get him reassessed for autism, but out main focus right now is to get him treatment for his anxiety, which is slowly spiralling out of control. Having anxiety myself, I truly feel for him, and try to help him through his panic attacks the best I can. I feel that we are at a point where medication might be needed.

Aaron in his recent assembly costume. Cutest little alien or what?

Aaron in his recent assembly costume. Cutest little alien or what?

My husband, Mark,  is back at work after having nine months off with a bad shoulder injury. The injury is still there and still causing a great deal of pain, but we are hoping to have that dealt with soon.
In the meantime, we are very busy with work. Christmas and New Year is our busiest season of the year, with everyone wanting spring cleaning done before their holiday guests arrive. We havent even hit December yet, and I already have most of the week before Christmas booked out! Word of advice…if you ever want your house cleaned during the festive season, book a cleaner at least three weeks in advance!! It is guaranteed that I will have to turn away at least ten jobs a couple of days before Christmas because people have left it too late!

Hope you are all travelling well, my little readers…keep an eye out, as I have a few more blogs posts up my sleeve. Lots to catch up on!

Bella 🙂

 

Contact me any time!

Hi everyone!

I just wanted to write this quick note to you all. I have been receiving some lovely emails and comments lately.

Some have been words of encouragement, others have been letters seeking advice.
wine and computer
I hope I have been able to answer all of you so far. I always try to, usually with a glass of wine in hand…so please forgive me if they come out as gibberish sometimes!!

I really love getting these notes, so please do not hesitate to contact me if you ever want to….about ANYTHING

My email is bellasblog@hotmail.com

Go for it!!

CHAT SOON!
Bella 🙂

I’m BA-ACK!!!

Hi everyone!

I just wanted to write a quick note to say that I am still around….apologies for not writing much but life has been hectic with work, a couple of birthdays, and Cameron recently had his CF annual review, with a check up for Eva…both of which are some of future topics that will hopefully come out soon!

A big welcome to all the new followers that have signed up from the advertisement in the recent Great Southern Disability Services newsletter. It was awesome to see the link to my blog there (and I hope you enjoyed my witty little post “Expectations of the Modern Mum”…there are many more like that!)
A few of you also signed up from the recent rafflecopter. Hope you won something and keep an eye out for the upcoming one launching soon!

So while I am still getting my act together, have a look around at some of the older blog posts. Or if you are bored, or want some new recipes, check out my other blog (which I have unfortunately also been neglecting lately) www.athomewithbella.wordpress.com

Chat soon everyone!!
Bella 🙂