** Please Note**
I have put a lot of thought and consideration into my words for this blog.
I pour out my heart and soul for you to read, learn and share about the ongoings in my families life.
I love that you like to share my words and blog so all I ask in return is that if you use my words, parts of my blog or entire posts to share via social media or anywhere that you give me a shout out and acknowledge that you are using MY words and maybe even include a link to the blog.
Thank you all for taking the time to read, learn and share my blog I really appreciate the compliments too and love knowing that I am reaching people near and far. I hope that you continue to enjoy my blog.

K

Thursday 11 April 2013

The A to Z of Cystic Fibrosis ( letters M to Z)


A to Z of Cystic Fibrosis (letters M to Z)

I can't take credit for the idea of the A to Z it was lead off by another family touched by CF also. It was an emotional journey that I took very serious about raising  awareness for CF so I put a lot of heart and soul and thought into my words. It was these posts on Facebook that lead me to the blog as many people encouraged me to do so. So here are our version of what the A to Z of CF means to me and my family.  I have to also give a shout out to the hubby as he also did some entries for the posts. (O,Q and W)


M

M is for Mycobacterium abscessus. This is a rapidly growing mycobacterium that is a common water contaminant. Mycobacterium abscessus may cause chronic lung disease, post-traumatic wound infections, and skin infections in immunodeficient patients.

This is a nasty bug that Cadel has picked up and has colonised in his lungs. The Drs have no real idea how he picked it up but with his low immunity they think the floods of 2011 may have been the source. It was only picked up in tests done last June while admitted and finding no other viruses or infections to cause Cadel to be so unwell. It is not known how long it had been there undetected as it not something they usually test for.

After Cadel had allergenic reactions to the antibiotics that treat this infection he has ruled out some specific medications to target the mycobacterium. While it is set in Cadel's lungs it has also become antibiotic resistant to other medication so now the Dr's treat it to maintain it and keep it at bay and make sure it doesn't get stronger. 


M is also for moments lost. Cadel is having a bit of an off day so he and I missed out on going to Lo and Xav's Easter hat parade. Luckily Scott was working close by and could go. 

It makes me really sad though that sometimes the big kids miss out because of Cadel's disease. There have been many moments I have missed with Lo and Xav, too many to list but two that stick out is the the two Christmas mornings that I haven't been able to see their excitement about what was left under the tree as they see it! 

Tiny little moments that you can never get back lost to this terrible disease that affects the whole family. Thanks for helping us spread awareness about CF so that one day CF won't exist and other families won't miss out on special moments. 


N

N is for Nurses. Cadel has met his fair share of nurses during his twenty admissions. There are the favourites of course in the wards, the nursing students who's eyes are opened wide on the wards, the theatre nurses who he despises in their scrubs, the recent graduates who Cadel teaches a thing or too, the ICU nurses who still pop in for a chat and play when they see Cadel admitted and who could forget Liz the CF Clinical Nurse Consultant (CNC). Liz is the bearer of bad news and a messenger of good news and changes of medications and our first port of call at the RCH when we call. At the moment we are calling in daily on weekdays and skyping Liz too. 
Without Liz we would be lost, she always visits daily when Cadel is admitted too. 

Nurses are the hardest working people in the hospital. Yes the Dr makes the final call but nurses are there to clean up the mess ( literally) and administer the medications, make the bed, spend time with Cadel and wipe away tears, even mine sometimes. On many occasions the nurses are the ones who suggest a treatment to the Dr. Some of them have become friends and will bring in gifts or baked goods. They have got to know Cadel quite well and know that he likes to help give his meds and make the bed. They, like all, people seem to make a real connection with our wise old soul that is Cadel and when he was in ICU they told us that on his handover sheet under complexities it said "extreme cuteness". Would have to agree with that one! 

They have even inspired me. When I have time I would love to go back to uni and study to become a nurse. I can't decide between childrens ICU or theatre. 


O

O is for my offsider! Scott has been my support, my strength and courage always but more then ever during the last few years. 

He gives me a break on weekends from the physio and medications and lets me have a sleep in. 

I couldn't do any of this without him, Scott will always visit in the morning before work and after when Cadel is admitted and then go home to spend time with the older kids. 

He is great father to all three of our kids and my best friend. Thanks Scott for just being you!


O is also for Oh Crap! 
(written by Scott as part of our awareness posts)

Times like this morning, we wake up and say Oh Crap (normally worse), Cadel's feeding tube came out overnight, so while I held his legs and arms, Kristy got it back into his stomach and its all working fine. That was followed by a couple of hours of Cadel not letting anyone touch him and him looking weak and in pain and us watching and waiting. He's all good now though.
Other times he has ended up getting rushed to emergency and once being taken away in an ambulance. There have been loads of these moments in hospital too, but Cadel being the fighter that he is, always gets through it and beats any challenge that comes his way.
It's amazing how well the big kids deal with all of this, today they tried to play with him and he sent them packing, but they never give up on him and eventually they were all playing together in the front room.


P

P is for Paradise Kids. Just last week we were at the Paradise Kids Holiday House. They provided us a getaway for the family that we all needed desperately. 

Paradise Kids is a Gold Coast Children’s Charity founded in 1996 to help children learn life-skills to deal with grief through death, loss or illness. Whether the loss is the death or approaching death of a parent, grandparent or sibling, grief through the stress of separation or divorce, or the child’s own chronic life-limiting illness, Paradise Kids provides programs to ‘help heal the heartache.’ 

The Drs did a great job of getting Cadel out on HITH so that we could go to this great illness support holiday program. We were provided with a week stay in a holiday house on the Gold Coast. They provided all meals and even sent Scott and I out to dinner on our own while someone sat with the kids. They also sent us to Dreamworld for the day with a personal meet and greet with Shrek. There of course was also the serious side of therapy sessions for Scott and I and play therapy sessions for the kids to help us through our journey. It was all topped off with morning tea with the lovely ladies who helped us out and a sand and unity candle to take home with us. 

They are a great charity that provide a much needed break for families just like ours. 


Q is for Questions
(Written by Scott for our awareness posts)
We get asked a lot 'How's Cadel?'

This is a really hard question to answer, so we normally say 'He's going ok', when most of the time its too complicated to really say, and you may not want to know all the details. I watch some people shut down when I start talking about lungs and sputum etc.

When anyone asks Cadel anything at the moment he responds with a definite 'No', no matter the question. Unless there is a smell in the air, then its 'Dad'.

Specific questions are great, because we can answer them with a meaningful answer. Feel free to ask questions about Cadel or CF, we love it. I love it when the doctor and dietitian asks how his poos are. Poos are very important to them.

We were in a shop the other day and the manager asked 'what's
going on here' probably because he had his IV infusion bottle
over his shoulder, I told her he had CF and needs antibiotics and it was all good, better than her staring at him like a lot of people do.

When he is in hospital and Kristy is with him, I probably ask her 10-20 times a day if anything has changed or have the doctors said anything new, it really gets to her because I'm one of 10 people asking the same thing and normally nothing has changed. I know how annoying it is cause she does it to me when I stay with him. It's just cause we all care.


R

R is for Respiratory rate and effort. This is a daily thing for Scott and I to keep track of with Cadel. It is one of the first questions we will be asked by Dr's or nurses. 

Cadel when unwell doesn't always 'drop his SATS' or better known as the oxygen saturation levels in his blood which can be taken from a monitor. He usually sits around 95/96. Well people would be 100%. 

So when Cadel is unwell he will compensate by keeping his SAT's up but he will breathe harder and faster.

So we are always counting his resp rate. Cadel's normal resp rate is anywhere between 50 and 60 breaths a minute. When he is sleeping he is known to get down in the forties. To put that all in perspective a 3 year old's resp rate is normally between 20 to 30 breaths a minute. An adult would have 13 to 18 breaths a minute. Scott and I are pretty good at judging by a quick look if his resp rate is normal ( for Cadel) or higher and we need to do a count for a full minute. For medical staff that are unfamiliar with Cadel there is always a little freak out moment until we explain what Cadel's base line is. 

Respiratory effort is when Cadel works harder to breath. This means a number of things. He will burn up more energy as he breathes faster and harder and he will have recessions around his ribs back and neck and sometimes nasal flaring. Again these are things the Dr and nurses will ask about all the time and is a visual sign that Cadel is unwell. 

This is why Cadel is on Hi Flow machines while sleeping as it helps him but giving him a rest and takes the pressure of his lungs so the machines does all the hard work forcing the room air into his lungs to give deep breaths. We only connect oxygen to the Hi Flow machine when he is very unwell. The Hi Flow machine sucks in room air through a filter then heats it to 34 degrees and pushes it through at 20 litres per minute. 

Thank goodness for hospital organising this machine at home it has saved us many trips to the hospital and the physiotherapists sometimes use it to help with their sessions. 


S

S is for Salt! Anyone who has ever kissed a person with CF will notice that they taste salt. Before Cadel's diagnosis I remember the consultant asking if when I kiss Cadel does he taste any different to the other kids and I said no and he then asked again, does Cadel taste salty when you kiss him. I again said no. It wasn't until I went home and thought about it I realised he did taste salty and in those few days of waiting for the diagnosis and not really knowing much about CF I remember sneaking in little licks of my newborn and licking my lips after kissing him to check for the salt! 

Although CF wasn't officially diagnosed until years later, literature from Germany and Switzerland in the 18th century warned "Woe to the child who tastes salty from a kiss on the brow, for he is cursed and soon must die," recognizing the association between the salt loss in CF and illness. 

Basically people with CF don't have the same ability as those without to transfer salt and water on a cellular level so they lose large amounts of salt thus needing it to be replaced. We do this with Cadel by adding it to his tube feeds and his food. Cadel has two plus teaspoons of salt a day. Sometimes when Cadel is admitted and his salt levels are low they just make up a solution and put it directly into his blood steam via his port. 

I'll never forget the filthy stares from fellow parents at the food court when I used to sit there and pour salt into Cadel's pureed foods. 

We have to make sure Cadel gets enough salt during the day otherwise he may become dehydrated. 

Salt is so important and we try and make sure Cadel sucks in some salty air by the beach to help thin out the thick mucous in his lungs also. 

There are so many great health benefits from salt to CF sufferers and I could go on for years about salt lamps, salt therapy, saline inhalation therapy and much more but I'll leave it there until another time. 



T is for TV crew! For the past few weeks we have been doing a few things with a camera crew from ABC 7.30 QLD Stories.

They have filmed us at clinic at home and done some one in one interviews. They are doing a story on our family and CF and another family that is friends of ours who are also a CF family. The story is about raising awareness for CF and how it affects everyone differently and also something else big! The other family have shown our family a huge about of love, kindness and support and along with 20 plus other trades and Queensland Family Pools have donated our family a pool! 

This is what I have been talking about in code for weeks now and I couldn't wait any longer to tell you all. The pool is finally finished, apart from some final finishing touches around the pool so the camera crew will film the first swim next Friday morning and the story will air in the coming weeks. We will surely let you all know when it's happening. 

I've said it many many times but word can't be strung together to say the huge amount of love, support and gratitude that we feel for all involved to help us create great family memories. 


U

U is for understanding. So I would like to thank you all for taking the time and reading our posts to help understand more about CF. I'm still learning new things about CF and don't think it will ever stop. We have only been on this journey for over two years, somedays it feels a lot longer. 

I'm glad you have all stuck by us through the A - Z. Their aren't many letters left to go and I know sometimes it may be a lot with us posting every day. This is what we live with everyday, there is no day off from CF for Cadel. 

We hope that you have enjoyed us sharing and opening up our hearts and you have learned and understood CF and our journey a little bit more.


V

V is for Vertex Pharmaceuticals. 
This is a company that has produced the drug called Ivacaftor. 

Ivacaftor (trade name Kalydeco) is a drug approved for patients with the G551D mutation of cystic fibrosis. Ivacaftor was developed by Vertex Pharmaceuticals in conjunction with the Cystic Fibrosis Foundation.

Kalydeco helps unlock that gate and restore the function of the mutation allowing a proper flow of salt and fluids on the surface of the lungs. This helps to thin the thick, sticky mucus caused by CF that builds up in the lungs.

Kalydeco is currently being evaluated for other cystic fibrosis mutations including F508del, the most common defect occurring in approximately 70% of the cystic fibrosis population.

Cadel has the double F508del mutation so we are watching this drugs development closely. 

This just proves that research is so important and to never give up hope as there are brilliant scientists out there working hard to give kids like Cadel more time. 


W is for Why?
(Written by Scott for our awareness posts)

Why is something that we ask a lot, as you can imagine.
Why?
Why Cadel?
Why us?
Why CF?
Why isn't there a cure?
Why so harsh?
Why another infection?
Why is he getting admitted again?
Why does Cadel have to stay in hospital?
Why more meds?
Why does mum have to stay with Cadel?
Why do we have to hold him down for needles, buttons etc to be put in?
Why can't I buy Kristy flowers?
Why did they make that mistake?
Why wasn't that picked up earlier?
Why can't we visit great Pa?
Why can't we go to the Ekka?
Why can't we go to the markets?
Why do we have to wash our hands all the time?
Why does Cadel have a Mickey Button?
Why does Cadel have so many visible scares?
Why the f@#k?

CF has changed our lives forever, and we have asked a lot of why's. 

We have also met some great people along the way and sometimes ask ourselves why are we so lucky?


X

X is for yup you guessed it X-ray. Cadel has had so many X-rays I have lost count. He is so cute when we walk into the X-ray rooms now he knows exactly what to do. For a while he would start crying every time because he knew what was happening. While he is so little the best way for them to X-ray his chest is by sitting in a high chair and I stand behind him holding his arms up beside his head. He now just walks up to high chair, points to the lead jacket, then back at me. He knows that I have to wear this to protect myself and then straps himself in. 

When he was ICU it was procedure that every Monday X-rays were done. This was when the machine would come to Cadel. Again he picked up what they wanted him to do quickly. He would shimmy himself over to the side of the bed and wait for them to put the slide behind his back then just sit there ever so still while the technicians did their thing. 

Cadel has had a few different types of X-ray. His had the usual X-ray, CT scans and many fluroscopes. They use the fluroscopes when they have contrast to see if his port or feeding tubes were in the right place. The fluroscope is a series of quick real time X-ray so as they pump the contrast through they can see where it is going. Sometimes this process can take up to an hour and Cadel just lays there so calm while the machines and Drs buzz around and over him. 

Sometimes X-rays tell the real story of what is going on inside Cadel's little body as he can look so well on the outside but the inside tells the truth. Like the last admission. 


Y

Y is for You Tube. There are many CF videos on You Tube but this one was passed on to us from someone at the hospital and it has stuck with me. It's from a Mum of a child with CF and so many feelings of what she shares is just how I felt and still feel. It's a simple video with a strong message. Please watch, learn, and share. 

http://m.youtube.com/watch?v=8ovtUJBeaoA


Z

Z is for ZERO SURVIVAL RATE! 

It's a cold hard fact that we try not to think about but it is a reality with this terrible disease. This post may be a little confronting and upsetting but we need you all to understand. 

CF has an average life expectancy of only late 30's. I am 35 this year and can't fathom the thought of having that statistic hanging over your head everyday. With modern medicine this has increased dramatically from in the 1970's when it was only 12. Lets hope in years to come it will be even more. 

This disease affects everyone so differently so there is no one treatment to fit all, there are no periods of treatment, a time when a sufferer can say I beat CF or is in remission, or can be a survivor! It is a LIFETIME of treatments everyday all day right from birth and hospitalisations from sometimes the common cold viruses. 

These are the cold hard facts that our family and many like ours live with everyday. You are reading these words but we are living them. There has never been a time when it is forgotten. The statistics stay with you and sit in the back of your mind. We have to celebrate and enjoy every second, minute, hour, day, week, month, year and decade. 

Scott and I remain positive about CF and enjoy our moments as a family but as this is the last in our A to Z of CF we have to share the honest and sometimes hard to hear truth. This disease is terrible and it takes children, friends, brothers, sisters, nieces, nephews, Mums, Dads, aunts uncles and grandchildren away from their loving families. Some sufferers describe it like drowning from the inside. 

We need to raise much needed awareness for the most common inherited life threatening chronic disease that affects thousands nationwide. One baby every four days is diagnosed with CF. Imagine that, you have this beautiful newborn baby, you are enjoying your first few months of a new bundle of joy and then you get a life threatening diagnosis for them. We lived through that scenario we can't imagine how it felt we know and so do thousands of others. Many tears have been spilled and still are all the time. 

So what can you do? Spread awareness and help raise much needed funds for research. 

We will be doing another fundraiser sometime this year and once again will call on you all for your support and donations so stay tuned. This time we have decided to donate to research so one day instead of just saying it all the time, CF really will stand for Cure Found. It may not happen in Cadel's lifetime or mine but Lorelei and Xavier's children's generation may be saved. 











K

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