Don't call me super mum!

I'm a mum to three, and one of my children has extra needs of a medical nature that makes me an official full time carer. Yes this statement is true but please don't call me super mum!

I've been called a super mum by a few people and even by some staff at the Royal Children's Hospital (RCH) including some of the social workers but I cringe every time I hear it. Yes I know I do a little bit more than the average parent for my child because he has Cystic Fibrosis but I don't do it because I want to I do it because I have to.

* If I don't do Cadel's enzymes he won't absorb his food and won't get the much needed nutrients from his food to grow big and strong and to fight off infection.

*If I don't give him his physio sessions he can't move the thick sticky mucous that clogs up his lungs and infections will set in.

*If I don't give him his daily nebulised medicine it won't help to break down the mucous.

* If I don't give him his liver medication his liver disease may progress sooner and need a liver transplant.

*If I don't give him his vitamins he may get brittle bones from not getting something as simple as enough vitamin D.

*If I don't give him his antibiotics a simple virus may attack his low immune system.

*If I don't give him his tube feeds during the day he won't get enough calories that his body needs just to be a normal 2 year old.

So as you can see I do it because I need to not for want.

I'm an organised person who loves lists and routine. I again need to be. With two older children at school and extra curricular activities I need to organised and have dinner made by ten in the morning so that I can get to dancing or taekwondo or even clinic for Cadel to have a check up. This is just so that there is some normalcy in our families life. Hubby and I strive to make life as normal as possible for the kids because it shouldn't be normal to spend over a 1/3 of a year staying in a hospital or visting your mum and brother in a hospital. Or helping by holding down your little brother's legs while Mum reinserts a misplaced nasogastric tube. ( a memory of Xavier I will never erase from my mind, he is 4 years old silently enters the room sees me struggling and just kneels down and helps, breaks my heart but also warms it that he is so caring and helpful). Normal should be going to school, doing dancing and taekwondo and playing in a park and not worrying about catching the mucous your little brother is coughing up because you are closer as mum dives for the cloth nappy and races over. ( Lorelei is notorious for this.) So to have some normalcy I need to make lots of lists and be prepared for it all. This is when people usually see my extreme organisation and comment "oh aren't you the super mum!"

I have to also give a massive shout out to my support system which has stepped up into overdrive in the last 8 months or so. Hubby has always been a great support and helps out with all of Cadel's and the older kids needs also but there have been a few friends who have really stepped up without us asking by looking after the kids, cooking meals, asking how we are all REALLY doing, put smiles on our faces and just being there to listen. So I have a lot of people behind the scenes helping to make me look better and look like I have it all together a bit more then I actually do.

So when someone pulls out the super mum tag I cringe because I feel it puts so much pressure on me. Yes you may have said it as a compliment and truly no malice whatsoever but I feel deep down inside there is a guttural scream of "NO" as I then think I can't fail now I have to always be on my game. I sometimes put on a brave face when I feel like my world is crumbling down around me. Those closest to me have learnt to see through the fake smiles, I do this as most people do because I don't want to look like a failure because sometimes you feel there are certain expectations on you. These expectations are probably put on by ourselves and society in general, we have to have it all together and be ready to take on more. Somedays this just isn't the case and I drop all the balls where as other days I'm amazed at my own juggling skills.

So just don't call me super mum, I'm just doing what any parent would do for their child to keep them well and somedays I have really bad days too but smile to hide the destruction of my day. Oh and don't get me started on the whole " oh I don't know how you do it, you are so strong! "

K

Its hard to be the patient sometimes.

So I am one day post gallbladder removal surgery and I am busting to get out of this hospital, why? I don't know the answer. Well maybe I do....It's an enforced rest period yet my mind is racing and I'm so bored because I'm not doing the usual morning routine of rushing around getting the kids ready for school.

I'm also usually the hand holder when I'm in a hospital. I'm very used to being in and around hospitals with Cadel having CF and having 20 admissions and over 10 general anaethestics I have seen my fair share of procedures and insides of theatres but I'm usually the one holding his hand and reassuring him everything is ok.

Being on the other side is kind of terrifying, maybe that is because I've been in and around hospitals so much in the last two years I am more aware of what happens, it may also be the fact that I'm an adult and I'm supposed to ok and tough and strong and not nervous. But who am I kidding as I got wheeled off to the theatre then left alone while they prepared the theatre the anxiety really set in. All I kept thinking is adult hospitals could learn a lot from children's hospitals. They need to still reassure us and make sure we are ok.

It may also be that I'm a massive control freak and the feeling of grogginess scares the bejesus out of me because its that complete feeling of no control. I'm still feeling yuck about all that when I think about it now. For the life of me though I cannot work out why when I woke up I was thinking " I'm stuck in Facebook and Pinterest, I need to get out of here" it was like I was trapped and then I was looking up at the blank recovery ward ceiling feeling ergh!

I would hate to think what nurses and Drs are like when they are patients when it feels this bad after being the hand holder. I know it will help out when Cadel next is in hospital as I have that extra understanding, I just hope that I don't have the Facebook and Pinterest flashback! Shhh don't tell hubby he would just tell me it's because I spend too much time on this sites!

K

Compassion, where did it go?

I am writing to vent and for therapeutic reasons as I feel that I was in a situation that required some compassion from someone and found that they had none, not a bit for me, my family or my situation. The situation itself is not important but the bigger question is when did it become okay to have no compassion for others. After having the biggest gift of compassion of all, being the gift of a pool in our yard how can the great feelings of warm and fuzziness be striped away with one swift move of inconsideration.

It got me thinking about what has happened to our world and what made people loose their ability to have consideration for others. Are we so caught up in our own little world that we stopped thinking of others. Do people still give up seats on the train or bus for the elderly or pregnant ladies. Are you the sort of person who will hold or open a door for a parent pushing a pram? Do you park in the parents park? I know that parents parks are a privilege and not a right but as a mum of three trust me when you have kids to get in the car it's so much easier to be able to open the car door up completely when you have to buckle the child into the middle seat. So why is it ok to park there if you aren't a parent or you don't have your children there. Are you not thinking about the parent that could of used that park for its intended purpose when you see a close park to the shops.

I have found this world to be a cruel one sometimes, don't even get me started on smokers! I just think it is common courtesy and politeness to not smoke around children, let alone a child that has an OXYGEN bottle clearly labelled as an oxygen bottle, hanging off the pram.

We need to stop being selfish all the time start to give and share and be kind. Let that car with the indicator in, in traffic, hold the door open for someone, give up your seat for the older gentleman on your train. I have a friend who was notorious for every time she went through the gateway toll she would pay for the person behind her. This deed went unnoticed by many, had no big fanfare about it all and I'm sure it put a smile on her face as well as the complete stranger that got a surprise when they went to pay. Sadly it's all electronic now but I'm sure that friend has found another way to give.

I think we need to look back at history and the way that people were more compassionate and learn and take note. I know I am. I will set myself a mission to do an act of kindness for a stranger that will put a smile on the receivers face and I hope that you can find some compassion too and do the same.

Lets make the world compassionate once again.

K

Military Precision Packing!

So we recently went away for the weekend and I have to say the packing with three kids doesn't get any easier let alone throw a child in the mix that needs medications and oxygen machines!

I remember the days of just throwing a few things in a bag last minute and taking off but that was what hubby and I refer to as BC, before children that is.

Even as babies going to the shops was a big drama taking extra outfits, bibs, nappies wipes dummies etc. you would never use them then one day you would think ok let's just take the nappy and wipes and then BAM you have the poo explosion of the century ( Mum's you know the ones I'm talking about) and here you are battling with poo and floppy arms and legs and wishing that you should have bought along that extra outfit and wrap to cover the crime scene left behind in the pram.

So back to the past weekend. I must say I've gotten better and more organised at packing and getting the kids involved helps a lot. I have found to pack the kids bags into outfits for each day and then put them in plastic zip locks for the kids to decide which outfit for each day seems to be working well. It's still a military precision operation to get organised and takes a lot of time to even prepare to pack. Cadel has extra things to pack! There are his countless medications his oxygen, the extra oxygen bottles, the hi flow oxygen machine, the folder full of his scripts just in case we forget some medications or they run out, the nebuliser and all its chargers and of course the all important letter explaining Cadel's CF complications and his treatments.

So I'm always doing a mental checklist even as we drive away going through everything in my head ticking it off even though I've ticked them all off physically on the list I wrote out at the start of the week in preparation for the two night stay that we were going on. My anxiety levels go through the roof and I don't think I relax until I unpack at our destination and am sure that we have everything.

I always love seeing the hotel staff and the shocked faces as we unload our massive amount of luggage. The car is always so loaded up for a two night stay, boy but you should see it when we go for a week we almost need a trailer!

K

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 

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 

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

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

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

I can't take credit for the idea of the A to Z is 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)

A

A for us right now is antibiotics. At the moment while Cadel is admitted he is on IV Peptaz to help him clear out the infections. At home he is on oral antibiotics. There has never been a time since Cadel's diagnosis he hasn't been on antibiotics to help keep him healthy. 

The problem with Cadel and antibiotics is that he has become allergic to the most effective ones that are used commonly for CF he has reactions to a list of about 5 and the Peptaz he is on right now has previously made him really unwell and given him a drug fever. So he is closely monitored. 

Also the bugs sometimes become resistant to the antibiotics which is also the case for Cadel and his mycobacterium which is now just maintained so it doesn't take over as it is colonised and set in.

It is like always a fine delicate balance between good and evil. 

B 

B is for Bacteria. Our biggest enemy in the CF community! 

I must confess before Cadel's diagnosis I was a germo phob but boy now I'm worse and Scott is just as bad. But we need to be! 

Bacteria gets trapped in the thick sticky mucous in the lungs and repeated infections cause scarring and ultimately lead to severe lung disease. 

Hand sanitizer is a must and we have it in the pram, the car, my hand bag, numerous spots around the house and back ups too! 

Hand washing is a big deal and were are obsessive about it as a family. ( The kids are notorious for asking people if they have washed their hands). I remember a Dr telling us the cold virus can live on a hard surface for 24 hours. That means the handrail/door knob/lift button you just touched someone who had a cold may have touched it, then you touch your face and bang in a couple of days you are swept up into the world of a cold! Sometimes I feel like I can almost see the germs and bacteria in a cartoon form sitting around waiting for the next in expecting victim. 

Cadel has had the following bacteria ( that I can remember) 

Adnovirus

Pseudomonas 

RSV

Achromobacter

Norovirus

Mycobacterium 

Staph

And plenty more in his short time. 

Some are the common cold and some are more serious and can colonise and be there just causing havoc and become resistant to treatment. Even the common cold can lead to severe damage to a CFer's lungs. So please think twice when you have the sniffles that it could be so much more than 'just the sniffles' to someone we love. 

C

C is for clinic. 

Cadel attends a CF clinic at the RCH at least every two weeks. When first diagnosed Cadel was visiting clinic weekly then fortnightly then we eventually got to 4 weekly. We were told that we would have to visit 3 monthly one day but because Cadel has been so unwell we haven't gone anymore then around 4 weeks between clinics. 

At clinic we see Cadel's 

Consultant, 

Physio, 

Dietician,

Social Worker,

Occupational Therapist and the Nurse Consultant. 

Luckily we are assigned a room and all the above come to us to avoid cross infection from other CF patients. 

They also weigh him and take his height. 

He also may have to have bloods or X-rays before we attend clinic also. We sometimes have to pick up some medications so waiting around for pharmacy can take some tome too. So days when we have clinic take a lot of planning for the whole family.

Sometimes clinic is a quick one and we are out in 40 mins but sometimes it can take hours. 

Eventually Cadel will have to a lung function test before clinic but for the moment he is too young. 

Lung function tests measure:

How much air you can take into your lungs. How much air you can blow out of your lungs and how fast you can do it.

How well your lungs deliver oxygen to your blood. The strength of your breathing muscles. These tests help the Drs to see how much all the infections and scarring has affected the ability to just breathe. 

D

D is for DF508. This is the gene mutation that Scott and I both inherited and that when combined cause CF. As we are both carriers of the CF gene their is a 1 in 4 chance of having a child with CF every pregnancy and a 2 in 3 chance of having a carrier. A carrier like Scott and I has only one copy of the gene error ( prefer error then mutation) and does not have any symptoms of CF. 

There are thousands of different gene errors and some have milder symptoms but DF508 is the most common and has the most side effects so Cadel got the double whammy! 

The main characteristics of DF508 are:

Pancreatic insufficiency. Cadel doesn't produce enzymes to break down his food to help absorb the nutrients, this is why he takes enzymes with all of his food. 

Thicker mucous that clogs up organs all through his body. 

He is also most likely infertile as the Vas Deferens don't form correctly because of the presence of the mucous at fetal development. 

CF is the most common inherited disease and like Scott and I most people don't know they are carriers. The biggest population of carriers is the European Caucasian population with 1 in 25 carriers and they don't even know it. We had no previous family history that we know of. This is why the heel prick test done on newborns is so important as it picks up CF ( most of the time) before anyone even knows that something is wrong. 

Lorelei and Xavier will most likely test their partners when they are ready to have children (when they are much much much older! ) to see if they are carriers. Even if so they can be aware of the chance of having a child with CF.

E 

E is for escape. Or should I say no escape. CF will always be a big part of Cadel's life and our family's life. There is no day off from CF. There will always be medications that need to be taken and physio that needs to be done. Sometimes just for a moment you can forget when you see Cadel playing with his brother and sister but then you are jolted back when you hear a cough. 

I'm guilty of saying I just want a day of from CF and really I can but Cadel never ever will. 

Like Scott and I say CF lives with us and make it fit into our lives not the other way round and that is something we will help Cadel to understand. We will teach him to reach for the stars and never let his CF hold him back. 

F

F is for fighter. Cadel is such a fighter. His personality does not allow him to give up and it is definitely something Scott and I encourage in all our kids. 

Most days Cadel's body fights to just breathe. It takes a lot of energy to breathe when he is unwell and this is why CFers need to eat extra calories and have a high fat and salt diet as they need reserves for when they are unwell just to function. 

Fighter is also the name of the song that Scott and I have choosen as Cadel's song as the words say :

"Give 'em hell, turn their heads

Gonna live life 'til we're dead

Give me scars, give me pain

Then they'll say to me, say to me, say to me

There goes a fighter, there goes a fighter

Here comes a fighter

That's what they'll say to me, say to me, say to me

This one's a fighter"

Give 'em hell Bubba Boo! 

G 

G is for Gastroenterology Team. 

Yes Cadel has CF and his team of Doctors are primarily from the Respiratory Team but he is also under the Gastro Team. 

This is the team that put in Cadel's feeding tubes, did swallow tests on him and help us look after Cadel's CF related liver disease. 

Cadel has had numerous Nasogastric feeding tubes and just over 12 months ago the Gastro team placed a Gastronomy button in his stomach for tube feeding. This is where Cadel gets 99% of his nutrition. He has three bolus feeds a day of 120 mls over 1/2 hour and then has overnight feeds of 700mls over 10 hours. The feed is mixed and formulated specifically for Cadel and is 1.25 calories per ml. He has mouse nibbles on food offered through the day but thanks to the Gastro team we are able to get much needed calories in. 

Now his liver. Cadel was diagnosed with CF related liver disease in around May 2012 after a biopsy was taken. His liver is enlarged and has some scarring. There is only a small percentage of CF patients that will get liver disease and Cadel is in that group. He is unusually young to be diagnosed with the disease and liver disease is very slow and unpredictable when developing. There is not much we can do at the moment only give him medicines that help maintain his liver twice a day. He recently had an ultrasound on his liver and there was nothing new developing so that was good. We were told when diagnosed that because he contracted it so young Cadel is at a higher risk of one day needing a liver transplant. The only problem with that is the fact that his lungs need to be in a good condition for that to happen. It is one big vicious circle. 

H 

H is for Hospital In The Home or HITH. Cadel is currently on the HITH program and technically still admitted while we are away staying on the Gold Coast this week. This great program allows patients like Cadel to spend less time in hospital. 

HITH is a program where in special circumstances IV antibiotics are put into infuser bottles that will give a dose of medication over a 20 - 24hr period. A nurse will come out daily and change the bottles over as well as for CF patients there will be a physio that will attend twice daily for treatments.

We are lucky here in Queensland that we have the HITH program, in South Australia they are losing the funding for the HITH program. ( I have included a link to sign a petition to help out SA, please sign if you haven't already). I cannot understand why with our struggling health system, the program allows many patients to stay at home freeing up hospital beds and I think Cadel gets better quicker in his own environment and not stuck in a hospital. 

I can't say enough fantastic words for this great valuable program that helps our family function a little more easily and still gives Cadel the treatment he needs. 

http://www.gopetition.com/petitions/save-the-wch-hospital-in-the-home-program.html

I 

I is for iPad. The hospital has provided us with an iPad and Cadel's Dr and the CF Nurse Consultant have a matching one so we can Skype if we are worried or just for a check up. 

This saves our family the sometimes 1 hour long drive to the hospital and the crazy expensive parking costs when we visit the RCH. Sometimes we can Skype the hospital 3 or more times a week and sometimes not at all but it is a valuable resource that we know is available. 

It is also handy for when the physio's are doing their home visit and the Dr can Skype getting a visual on Cadel and talk in clinical terms to them about anything that is happening. 

We can only hope that this is available to all patients in the future as this is the way technology is going but for right now we feel pretty special. 

I must admit the iPad has also kept at times the whole family occupied during those looong clinic appointments and during admissions. It has also come in handy during Cadel's 40 min liver ultrasounds. Keeping a two year old occupied for this long is a little easier when there is a game or two to play on the iPad. 

J

J is for JUST being a Mum and Dad to all our children. This is JUST what we do everyday. Even if sometimes that means hooking Cadel up to IV antibiotics, physio, doing all his meds, doing Lorelei's hair for dancing and running Xav around for grading of Taekwondo. Yes we sometimes do a little bit more than the so called typical parent but put anyone else in our shoes and I'm sure you would all JUST step up and do the same. 

We are constantly told by friends, acquaintances, family, hospital staff, Doctors, nurses and complete strangers how strong we are and inspiring we are but I would like to say that we are nothing without our support network of family and friends but especially our 'salty' family of other CF parents who it feels like we have known for a life time who share our journey even if on different paths. 

Two quotes I really like are :

"You never know are strong you are until being strong is the only choice you have. " author unknown 

"When something bad happens you have three choices. You can let it define you, let it destroy you, or you can let it strengthen you. "

P.S They are called our salty family because all our CF children taste salty when you kiss them because of the amount of salt lost through their skin due to the condition.

K

K is for Kindness. We have had so much kindness and compassion come our way especially in the last 6 months. 

We have the great support of our families that always help out when we need them. They give up their time to help us out sitting by Cadel's bedside to give us a break, looking after Lo and Xav and even organising family holidays for us. 

We have some fantastic friends who without even thinking or us asking, help us out looking after the kids or cook us some yummy meals during Cadel's admissions to take the burden off.

Then there was the time when we came home from holiday and our back and front yard had been blitzed and our freezer was full of food thanks to our salty family. 

The biggest kindness of all has come from friends and a big group of complete strangers. Scott the kids and I cannot put into words how grateful we are to all these people for their valuable time and kind heartness that will forever stay with us and I'm not quite sure how to thank them all. I know I'm being a bit secretive but trust me all will be revealed in a couple of weeks and you too will see how big and kind hearted people truly can be. 

All this kindness has given us some tears but plenty of smiles and made our hearts all warm and fuzzy as it makes us realise that there are still great wonderful giving and sharing people in this world. We have had to fight through the weeds but the people in our lives now have made our lives blossom with happiness. 

L

L is for the love of life. CF is a fatal disease and the average life expectancy is only 38 years old. Although many live beyond that age there are also many that don't make it. 

That is why we live life to the max and enjoy every moment that we have with all our children. I find myself locking away all the little moments in my brain so I'll never forget every moment we have with each child but especially Cadel. 

Cadel is an old soul who has been here before and is so wise beyond his years. Like most people with CF he is resilient, brave, strong, fun loving and packs so much into his day. 

We are always doing things to make special family memories. Even just small things like a water fight in the backyard or big things like visiting theme parks. We are surrounded by love and make sure that those important to us know they are loved. 

We will never put a limit on Cadel's life and encourage him to always chase his dreams and enjoy every moment and never ever give up hope that one day they will find a cure for CF. 

K

Jar of Giggles

I've become obsessed with jars at the moment and I blame Pinterest. Whenever I have a spare minute you will find me on Pinterest trolling through the most popular pins finding inspiration and new clever ideas or receipes. Well I stumbled upon some pretty jars filled with simple things and I fell in love. Then I happen to be browsing through a shop and saw a whole heap of different sized jars on sale! So I bought some of course then worked furiously to make a clear spot for them on my kitchen bench. Then it was ok so what do I fill them with, I wanted something bright and colourful so they are filled with cupcake cases, lollipops and sprinkles.

It wasn't until yesterday that I had an even better idea to fill up a jar with something that will brighten up my day not just my kitchen.  It all came about because Xavier gave me a big giggle with something he said. I had just been for a blood test and it being school holidays took all three kids along with me. The lady was lovely and we were the only ones in the centre so she paid real attention to all three of them. In the car on the way home, Xavier being the clever old soul he is piped up from the back seat. "Mum, that lady was really nice, even though she was old!" I had a little giggle to myself and said " yes Xav she was". These little quotes that come out of their mouths are the things I never want to forget. So I decided to write that quote down and date it. Then I thought where can I keep these treasured moments, brainwave, I need another jar! I will have to go out and buy a  big jar to fill up with these moments and giggles so that they are frozen in time. Then when I'm having a tough day or the kids are pushing me to the limits like we all know all kids do, I can open up the jar pull out a quote and have a giggle and remember what life is all about. The little moments that make you laugh and cherish the family you have.

Now I'm just waiting with my note pad and pen to write down those little things that fly out of their mouths without judgement and censorship. I'm sure Hubby and I will have some to add too!

K

Welcome

Welcome peeps,

After a gentle push from some good friends and support from the hubby I have decided to start a blog. This is a place where as the tittle suggests you will get an insight into my bubble of fun that is my world, my family, my life.

So I guess I should introduce myself. I'm Kristy, I'm married to Scott and a Mum to three wonderful children who all have their lovely quirks and great personalities that make them so unique. Lorelei is 6, Xavier is 5 and our youngest Cadel is 2. We live in Queensland, Australia.

The last two years and our families journey has inspired me to write this blog. Cadel was diagnosed with Cystic Fibrosis (CF) at 5 weeks of age.  Cystic Fibrosis is the most common genetic life threatening disease that affects the whole body but mainly the respiratory and digestive system. I will explain more with an entry of the A to Z of CF that kind of started off the whole blogging ordeal. I will try to blog about our day to day lives but keep it fun of course.

I hope that you enjoy the blog.

K