You Have Mail

Received a letter today from clinic. It was a summary of Ruby's annual review. It didn't have anything in there that I didn't already know about the disease.

So why did it upset me so much?

Reading things on paper, in reports, in black and white can be so confronting.

I know that CF affects lungs, so why does it hurt to read words like "increase in hilar markings and interstitial markings in both lung fields".

I know that Ruby's fingers have changed shape and its not necessarily indicative of the severity of her disease. But it cuts deep to see the clubbing field marked as yes + .
And she loses points for these things! Yes, that's right, she receives a score in 4 different health categories. I've always hated tests. Especially when the results mean so much.



We do all the things we do to slow the progression of an unstoppable disease. Although we have our ups and downs, on the whole she is happy and active. The CF part of her is often invisible, it is an extension of her. Not necessarily in a negative way. It just is what it is.
As Ruby says when we call her nicknames, "I'm not (insert cute nickname here), I'm just Ruby Monster Superhero".

So emotionally, reading the facts and figures of Ruby's health, covering topics from nutrition to gut health, was a real kick in the guts. I'm not a medical professional, I'm a mother. And as a mother, it is hard reading about the issues your child faces.

On the other hand, I am so grateful that they sent us this report. It gives me more to look over, more to study, more information to digest. And although some of the results were less than perfect, it would be wrong of me to not feel grateful for all of the positives. Her weight was great, we got good feedback about the wholesome diet we have. Her CF overall is classified as "mild". We are going a good job! So many others have it so much worse.

I may sound over sensitive, but what I really am is scared. It scares the living crap out of me that this is a progressive disease. It scares the hell out of me that at only 3 years old, she is already declining in the one thing that is practically irreversible - lung health. It scares me that our next annual report could be very different. And then the one after that, then the one after that.


But we will keep fighting. We will never let our guard down. We will never give up or slow down.

Operation Fatten Up

Maintaining a healthy weight can be a struggle for people with CF for a combination of reasons. Most people with CF are pancreatic insufficient, which means their body does not break down fats and protein. These people need to take digestive enzymes every time they eat. A high calorie diet is needed to counteract this mal-absorption. It's also needed as people with CF are working harder to breath than you or I. Simply living, burns more calories than average. And during periods of sickness, weight is easily lost., when it is needed the most.
There is a direct link between having a high BMI and being healthier overall. It wasn't until people with CF were told to have a high calorie diet, in conjunction with enzymes being used, that the a age life expectancy increased past teenage years.
All of this information puts stress on parents feeding their kids with CF. Suddenly "healthy" takes in a new meaning. In the early days, your baby is monitored, charted, weighed, measured so closely, you feel like you are raising a prize turkey.
During our visits with our dietician, I was interrogated with hundreds of questions. How much of this? How much salt? How much do you add to this? How much milk? How many poos? What colour/consistency? How long do you spend eating?"
If the dot on the graph drops below a certain point, then there are things to try. Polyjoule, pediasure, formula, appetite stimulants, Ensure, feeding tube. It's like a big black cloud hanging over you, waiting to rain at any time.

And then like everything else on my CF journey so far, I stop listening enough to do my own research and start thinking for myself. There is a whole world of good food out there. Why can't I use it? To be fair, our dieticians have always told us that a CF diet is not a free pass to junk food. But in my experience, healthy alternatives have not been widely suggested.

So the whole point of this entry is to share the following link with you. I started to post it on Team Ruby but waffled on do much, it passed "status length". The post is written by Jo at Quirky Cooking and is a wealth of knowledge about gaining weight in a healthy way. Because, to be brutally honest (as I always am), people with CF never used to have to worry about their health in old age. Getting to old age was beating the odds in itself. Now, people with CF are living past their 30's and overall health is so so important. That's hard to achieve if a high fat diet is a result of empty calories from McDs and KFC.
So if you want to get a bit if an idea on what "real" food is out there, read through this link:
Nourishing & Strengthening Foods . . . for those who are Underweight or Recovering from Illnesses

Oh, and did I mention I'm getting a Thermomix? It's my new weapon in my war against CF!

Annual Review 2012

Ruby's second annual review was today. Sort of like a birthday, but a celebration we would rather not be having. All 4 of us took the trip down to Westmead today. I was very organised and packed a bag full of food and entertainment for the kids.

We got there at 9.30 and started with the normal weigh-in and height measurement. Ruby was very compliant and did exactly as she was told. In fact, she was like that most of the day. It takes a lot to upset her.

Eliza lending some weight to Ruby

Her sweat test wasn't until 10.30 so we started off with the normal nurse checkup. Much to our amusement, Ruby and Eliza burst into squeals of excitement when the suction machine was wheeled in, as it has a huge tin of lollies on it. Ruby had her sputum suction and fir the first time ever didn't cry. It will take a few days to get the results back.  Both kids received a specimen jar full of jellybeans!

Physio checkup was all good too. We discussed different blowing exercises and will be starting off with blowing bubbles in the back with a piece of tubing.  We also discussed getting a vest again. Lot's to think about there especially as a long term study has been completed where it was shown that PEP therapy is more efficient than the vest. 

Then we were off to the sweat test. Electrodes were attached to Ruby's skin to stimulate the sweat glands, then some filter paper was placed onto her skin to collect the sweat for analysis. She left this on for half an hour, but unfortunately not enough sweat was produced and we will have to try again another day. All through the process, Ruby just watched intently what was going on and never got upset. Until it was time to pull the tape off, and then it really hurt her and she had a good cry. 

The electrodes that stimulates the sweat cells to test the level of chloride

Then a consultation with the gastro dr who felt her belly for any enlarged organs and hard parts of the bowel but all was good and squishy. We then chatted to the dietitian which is always painful as I can never actually remember all the things I feed Ruby and I get asked questions like "how much cream do you add" or "how much water does she drink" and my answers of 3 splashes, or showing an amount with my fingers aren't good enough. But dieto is happy with the amount she is eating and the amount of Creon that I am giving her, and no changes need to be made, or supplements added.

Finally, we see Ruby's main dr who checks her lungs (all clear) and we talk about new drugs coming and her general health. We decide to start her on Pulmozyme which is a drug that changes the DNA of mucous, making it shorter, therefore thinner and easier to cough up. This drug is used in many countries as a preventative and works really well. It is try expensive ($25,000 a year) and can only be covered by the PBS if you meet a certain criteria. Which Ruby does, based on her age and recent fight with pseudo. As the doctor said, "we are trying to preserve Ruby's lungs until there is a drug available that will manage all of CF" (referring to the new miracle dug being trialed with different gene types, Kalydeco).

So I feel very lucky that we don't have to push our clinic to try these things, and that we have a health care system that makes this affordable to us. I also appreciate that these things can seem inconsistent between clinics. As Ruby is too young to have the lung function tests, she does not have to show lung improvement to stay on this drug. 

New toys from clinic for being a good girl

So then we pick up a huge bag of meds from the CF pharmacy, pickup the bits and pieces that we have gained (including 2 cute dr teddy bears), make a date to come back in 2 months and treat ourselves to lunch at the hospital cafe.  Oh yeah, then walk to Parramatta Park where we had to park.

A busy day, but both kids did well. Eliza looked out for Ruby and Ruby was charming to the staff as always. 

CF never stops. We are always learning something new, can't rest for  second. 

Clinic Visit May 2012

All 4 of us made the trip to Westmead today. I swear the hospital shrinks every time we go there. It used to be a huge imposing city within a building, now it doesn't seem so big, and only slightly less daunting.

As soon as we get there, Ruby gets weighed and measured. Her weight had dropped ever so slightly but that is to be expected after the 3 months of fighting pseudo. Now that the pseudo is gone, Operation Fat is number one priority again. You would think that keeping your child a healthy weight is easy, but actually it is quite hard. It requires planning, calculating, coaxing and buying extra high fat food. And you can't relax about it, it is never ending. Then you have to factor on the enzymes. No point working hard to serve healthy, high fat meals if you are not giving enough Creon (pancreatic enzymes) to cover it. 

Her lungs sound good, her fingers show no signs of clubbing. Although they were embarrassingly dirty! Eliza took the opportunity to ask the dr if people with Cystic Fibrosis were allowed rabbits, and was very pleased when the answer was yes! With precautions of course with cleanliness. 

Dr Eliza!

Ruby had another sputum sample collected. She has finally worked out that a jar of jellybeans follow the procedure, so no fighting occurred. 

Our CF nurse suctioning for a sputum sample

We took in our new Aeroneb Go nebuliser, and our CF nurse and physiotherapist had lots of questions about it. They were all happy with how it worked, even though Ruby didn't want to wear it for them. They have been recommending to to patients but haven't had many personal reviews on it, so we were happy to provide one. They were amazed at how quick it nebulised 4 mls of hypertonic saline. 

Speaking of saline, the concentration that Ruby has been having was increased. So she was on 3% (which is the same as sea water) and now she is on 6%. Hopefully this will produce a more productive cough.

A while back, Ruby's Vitamin A & E levels were very low so we doubled the dosage of liquid vitamins that she was on. So today she had to have blood tests to check the levels again. And my little warrior didn't even flinch. She just watched the needle go in and then showed off her pretty bandaid. That's the first time we have had no tears xx

Showing off her pretty bandaid (and yet another specimen jar of jellybeans!)

We have booked in to go back for a sweat test. This is one of the ways to diagnose CF. It measures the amount of sodium in sweat. A high reading indicates CF. Although we don't need to confirm Ruby's diagnosis, it is good to have a reading to use as a baseline, especially if she is chosen for any clinical trials.

We also booked in for our next visit which will be her annual checkup. My baby will be 2 years old soon, sob!

Dare To Hope

Maybe you have noticed that I don't often post about a cure. Maybe not.
Some big discoveries have been made in the last 20 years, allowing our kids to make it to teenagers and beyond. We are always working towards a cure. It's something I hope for but something I am not relying on.

A few years ago, a company called Vertex started making real progress with actually changing the way the damaged cells work. It was the closest thing that anyone had ever gotten to a cure. With each bit of progress made, it was shared like wildfire throughout the CF community. Statuses updated, links shared, etc etc.

I was a bit of a party pooper. I never shared the news or let myself become excited about it. To me, it was still too far away. Still at "Today Tonight" level.

So it turned out that the drug wasn't suitable for the majority of the CF population. But from it came a different version of the drug which helped people with the G551D mutation.


I'm getting ahead of myself. Cystic Fibrosis occurs when a baby inherits 2 CF mutations. These mutations/genes have names. The most common mutation is DeltaF508. Ruby has one DeltaF508 and the other mutation she has is 621+1G>T. These mutations stop the process of salt being transferred throughout the cells. The drug that Vertex is producing is making that salt transfer happen, therefore stopping all the symptoms of CF. 


As Ruby doesn't have the mutation that the drug targets, I didn't really think much of it. And to be honest, I thought it would be years before anyone could see the benefits.
Then I started hearing things like this:


Since starting Kalydeco (the brand name of the drug)

"His exercise tolerance blows my mind. The increase in energy has been undeniable."


"A week ago I couldn't chase my dog around my back yard more then two maybe three times. I am now able to do it 5 or 6 times before I feel like I'm about to pass out"






 Sweat tests have lowered, people have been able to stop using Creon, lung function has increased. 




Pretty amazing stuff...life changing stuff! The more of these things I read (and there are HEAPS) of them, the more I allowed myself to think that yes, maybe there will be a cure in Ruby's future. Once they have  
a drug that targets her mutation.






So I'm on Facebook today as normal, and a post pops up on my newsfeed. And the post is written by an American mum who got Kalydeco for her daughter who has the mutations DF508 & 621+1G>Tand how she has been on it for a month with awesome improvements! So that means that there IS hope!


A long way to go with getting the drug to Australia and approved and paid for...but now I have something to focus on xx




The lady who posted about her daughter was posting because her insurance will now no longer cover Kalydeco as she doesn't have the G551D mutation. This means for her daughter to still have this drug, she needs to pay retail, which is $294,000 per year.  



.

How Is She?

If you ask me how Ruby is going, please excuse the strange look on my face while I figure out how to answer you. A simple question but sometimes not quite a simple answer.

You see, the answer will vary depending on who you are and where we are.

If you are a doctor at clinic day, you will get the unabridged version where the answer will cover everything from bowel movements to runny noses.

If you are a close friend or family, you will be told what bugs she is fighting and how she is coping with treatments.

If you are a casual acquaintance that I bump into at the shops, you will probably be told "not too bad thanks"

Because although I really appreciate you taking the time to ask, and for keeping Ruby in your thoughts, I find it hard to answer the question without either grossing you out or boring you to sleep. I guess the technical answer would be something like this;
"she is a bit congested at the moment, but her lungs are clear. Her vitamin D level is borderline and her Vitamin A was low so she will have a blood test at the end of the month to re-check. Her body is responding well to the Tobi although treatment time is sometimes very stressful. She is eating well but her weight is low. Thanks for asking!"

But I guess the most important information can summed up like this;
"she is really happy at the moment, being cheeky and always smiling. We are working really hard to keep her healthy, and although her body is having a bit of an internal battle at the moment, she isn't doing too bad. Thanks for asking!"

So there is the answer to the simple question! (I was always one to over think things)

Be a Good Friend

We get told some pretty scary things about CF from our kids doctors. Lots of it won't affect you. You don't have to worry about how to help our kids avoid diabetes or osteoporosis, you don't have to measure up the pros and cons of sending our kids to swimming lessons, you don't have to make sure that physio is done properly. And we don't expect you to worry about all of those things. That's our responsibility, our priveledge.


One of the scary things we are told about, is how a common cold, and other illnesses that are minor to most, can affect our child in the long term. What might be a day off school for your child, could very easily be a hospital admission for ours. A week of illness for your child could equal permanant lung damage for ours. Your child will feel better and be better. Our child may feel better, but all of these "little" illnesses shorten our childs life. That's just how it is.


Again, not your problem. Or is it? Being the mum of a CF child can be stressful and sometimes isolating. We don't want to lose friends, we need you more than ever. But we know more now, and our priorities may have changed slightly.


The good news is, it's easy for you to still be a good friend! There are just a few simple rules. These should help you out.

Accept that Cystic Fibrosis is serious.

One of the hardest things about CF is that our kids don't often look or act sick. And we don't really like reminding people. But the fact is, our kids have an incurable, life shortening, chronic illness that needs to be managed every single day. They are not the same as other children. We are not being cotton wool parents, we are keeping our children as healthy as we can for as long as we can.

Learn the basics.

We don't expect you to become an expert on the subject, but knowing the basics of the disease will help you understand why we are so anal about some things! In the smallest nutshell in the world: Cystic Fibrosis is a disease that causes the thickening of secretions in the bodies organs. This means that germs like to breed in the lungs, causing irreversible lung damage and eventually respiratory failure. If you would like to know more...ask! Your head won't get bitten off, I promise. Or you can check out the links at the bottom of the page. And no, they won't grow out of it.

Practice good hygeine.

Our kids don't need to be stuck in an antibacterial bubble. Practicing normal good hygeine will protect our kids. Wash your hands often, cover your mouth when you sneeze or cough. If you want to be a super good friend, let us know that you know that we want you to know! ie "oh give me a cuddle of your bubba! I can't wait to get my freshly washed hands on that gorgeous bundle of joy!" And of course...

Stay away if you are sick!

(or smell like smoke).

This is the most important point. Read the start of the page again. Your minor illness is our major fear. We won't get offended if you cancel a playdate, we won't hate you if you can't come to our dinner party. In fact, we will love you even more if you avoid us when you or your kids aren't well. This is the part where you can help us with managing our child's illness. Please please please let us know if your child has been sick. You don't have to decide if we should stay away or not, we will make the call. But we need to be able to make an informed choice. The smoking part is self explainatory and common sense.

Don't turn us into the bad one.

These rules will never change. Our child will always be sick, you will always need to practice good hygeine, we will always want to know if you or your kids are sick. Please don't make us bring it up all the time. We would really really really appreciate it if you remembered these points. It's no fun telling people over and over to wash their hands or cover their mouths. It's frustrating and sometimes embarrassing, but we will do it if we have to. We hope our friendship is worth the trouble.

Kiss and cuddle our kids!

I hope I haven't scared you off. The rules aren't too tricky, and our kids (like all kids) need all the love and good times that they can get. Big cuddles are awesome, tickle time is hilarious, holding hands is precious. If you are well and washed, please don't treat our kids differently.


That's pretty much it! Our lives changed dramatically when we got this diagnosis. We try to keep things as normal as possible, but it's not always possible. Our friends and family are so important to us, our childs health more so. We need you, please be there for us.

Cystic Fibrosis Australia

Oli & Nush A short cartoon about CF.

One More Step

This Saturday, a team of people are going to do an amazing thing. They are going to walk 65km in a day. That's a BIG walk, probably bigger than what a few of them realise!

To be honest, I don't really care if they do 65k or 65 steps. The fact that they have joined our team is enough for me. That in itself speaks volumes.

But for the ones who have a personal goal of going the whole distance and are looking for extra motivation, this is for you. When you are struggling for breath, when your legs are wobbly, when the last lap seems so far away, think of this.

Think of Ruby's start to life, a major operation at 3 days old - take one more step
Think of the little needles and tubes that she had in NICU - take one more step
Think of the scars that some of them have already left - take one more step
Think of the infections ruining her lungs - take one more step
Think of the invasive tests and prodding she has at every clinic visit - take one more step
Think of the many meds she has to have to survive - take one more step
Think of the physio she has to do every.single.day - take one more step
Think of the years that she will miss out on - take one more step
Think of the way that her breath will be laboured (like you on your 5th lap) - take one more step
Remember that this is how it will always be for her - take one more step

Think of the cure that is currently being worked on - take one more step
Think of the difference YOU are making - take one more step
Think of how much this means to us - take one more step.

www.mycause.com.au/teamruby

Thank-you.

Erin & Hudson

Believe it or not, I'm not the only mum with a CF story to tell! Everyone has a different experience, here is Erin's.

Its quite hard for a mum to write about the happenings of their child coming into the world at the best of time, but I'll do my utmost to put it down without too much sop sop (not sure who I am kidding here)
On the 3-3-2011 we welcomed our Hudson Trevor Kingsley into the world. Fourth time was another 'blissful' labour, no complications and just as smooth and kind to me as the other 3 were. Hudson was tired pretty much from the time he was born til days after he was diagnosed. His body temperature was also quite cool and getting him to feed seemed impossible and he was almost 12 hours old before having a feed from me. But of course at this stage it was just put down to how hard of a job it was for the lil guy to come into the world.

I can actually recall quite a few times during my pregnancy that I had some inner feelings that all would not be as well with baby number four and even that it wouldn't be something we would pick up on right away. But of course I had him and those thoughts were forgotten until my best friend reminded me that I had those intuitive thoughts. A few days after being home the midwife came to do the routine heel prick test. I remember how lovely she was, complementing me on my lovely children and the great family I had made and even took a family photo for us just before she left. Before leaving her famous words were 'no doubt you will hear nothing from me but as per the other children, you only will if there is something wrong' Again another lil pang in my tummy that I was not as confident in hearing nothing like I was with my other babies. In days that thought was again gone and didn't think of it again. 

Hudson was a wonderful baby, he slept like I could not believe, only waking for a lil feed and for a change and really was never awake for long at all. Days past and this started making me feel like it was not OK that he like this, not to mention the frequent poo's he was having. No kidding I could change 1 nappy, not even get him back to the lounge and he would have gone again (number two's I mean) I can remember my cousin Donna being over for a visit and I expressed to her that this happened often and I didn't feel like it was normal. After not hearing from the local maternal health nurse to see when they would be doing his home visits and setting him up appointments, I took it upon myself to go there, knock on the door and insist they weighed him and spoke of his routine and frequent bowel movement but was told "don't complain, you're lucky, he is just a really good baby". That gave me no mind relief what so ever. Then she weighed Hudson and just as I had known, the lil guy was shrinking, it wasn't in my overtired (how could I be he slept all the time) mind. From there is was recommended I would need to wake him every 3 hours and give him a good feed and do whatever I needed to get him to wake up properly. I could strip him down to his nappy and he wouldn't stir but continued to do as recommended for the next almost 2 weeks and just began to enjoy him and try and not worry about why he was like that.

One gorgeous sunny morning (I know it sounds corny but it was, that kind of weather always makes me feel at my best) I had dropped Lachy off at school, came home and was in the lounge with my 2 girls and Hudson just admiring them loving him and enjoying him. I was texting Steve back and forth, just things like how much I love him and thank you for giving me another perfect child, how lucky we are and how happy I am while snapping away with the camera and Hudson and the girls. Within minutes of this unreal day that all mum's love to have and live for, after all its the reason we have them isn't it? My mood, life world had turned from the happiest to well, the worst I have ever felt in my life. The telephone rang but as I was in the head space of the moments I was sharing with Steve. I didn't really take note of where the lady who was on the other end said she was calling from. I snapped out of it when she asked how Hudson was, I assumed it was the health center nurse calling to let me know when I could bring him back in. So I was in my la la land saying, 'Oh he is fabulous, actually he is the perfect baby, he sleeps well feeds well and seems very content (god only knows how the lady on the other end must have been feeling, something like 'this poor girl is going on and on about how fantastic things are with her baby and I have to tell her this terrible news). Eventually I stopped bragging about my perfect baby and she said. "Erin I'm calling about Hudson's birth screen test."  (My heart was over pretty much at this point) One of the things we test for is Cystic Fibrosis and its come back that Hudson has tested positive. I'm crying beyond control at this point. The house starts closing in around me and my head feels the pressure like its been put in a vice and someone keeps tightening it. The lady is still talking saying something like 'call who you need to call, I'll give you an hour before I call back to let you know what you need to do". I don't recall hanging up the phone, I can remember that I was trying to call Steve but I could not work the phone, I must have got a text through to him and my father in law. Poppy had called me back and I was crying to him letting him know I needed them (him and nanny, Steve's parents) to come down to be with the girls cos something is wrong with my baby. Before I knew it Steve was inside the house home from work, that is how I know I must have managed a text. he himself was very confused what could be so so wrong when only minutes ago he was receiving texts from me about how happy I was and all of those good things and I told him, Our baby has Cystic Fibrosis and we cried and we cried. It's only as I write this I am now wondering how our girls must have been feeling [:(] )

The hospital called back and we were told we needed to take Hudson in right away, I don't remember the trip to the hospital at all. First we met the genetic counsellor who explained how it came about how Hudson has CF and a little bit about what it actually is. I will say right now, we too were not at all familiar with what it was and I had actually mistaken it for CP (Cerebral Palsy) because when they called I remember taking him out of his bouncer and making him move his limbs around and looking at his head and screaming no he doesn't have it he is normal he is completely normal! I honestly thought we would get to the hospital and they would soon learn they had given us someone elses test results and mixed them up under Hudson's name. But it wasn't true. We were taken across to the hospital to meet what would and has now become, our CF team, our CF Monash family. we were taken to a room and introduced to more people than I can remember, I didn't really look at anyone, just their feet, there were so many different pairs of shoes. I held Hudson, he clung to me like a lil koala bear and he slept, as he always did. I can remember thinking. Who are all you people? You have no right to bring us in hear and turn my families world inside out and upside down with one phone call. T he sound of them all rusting through papers and taking notes was so so loud I wanted to scream at them, and for the lady that just constantly spoke and spoke and kept giving us all this information about how we would have to manage our lives I just wanted her to stop talking, I wanted her to shut up, in my mind I swore to god if another person walked in those doors and said, you will now meet so so and an they will be your such and such I was going to storm out of there with my baby and never go back. But I knew I couldn't, I was now at the mental point of knowing that the information all of these people had meant life and death for my son, these people would be the people who will forever help us keep him alive. These people were not to blame, they kept telling us 'its not your fault, there is no way you could have known' and that was just my problem. No one stuffed up majorly while I was pregnant, no midwife stuffed up while I was in labour, and we didn't stuff up after taking him home. There was no one available to point the finger at and yell and scream at to get my hurt and anger out on. No one. Because Hudson having CF, is no ones fault. Its a genetic disease that Steve and I had no idea we were carriers of to know that it was a possibility that one day we may have a child that had 1 in 4 chances of having CF. So our dear Team Hudson supporters, that is how Team Hudson started. After much sorrow and sadness (there still is and no doubt always will be) we knew our energy had to be used for the great of good and top make change for our dear lil Hudson and all lil CF sufferers out there. I told Steve, there is a reason Hudson has CF and as vain as this sounds, I believe with my whole heart its in his journey to have it, because he is so loved and instantly liked by all that cross his path that people wont be able to resist want to help fight the CF fight with and for him. because of how loved he is I know that he will make so much change for the greater good for this horrible disease that til this day, still takes way to many precious children from their loved ones. Because of Hudson I believe so much good will be done. Even if its just awareness of the disease, I cant ask for more than that, cos as I already admitted, until Hudson was diagnosed, we had no idea what CF was. It is the mission of Team Hudson to spread awareness of CF to anyone and everyone we can. Cos lets be honest, no one put money into something they are not educated on so I know that education will equal much more funds being raised and that will lead to our CFers having a longer easier managed life that what they do as it stands. I'm his Mummy, I'm his voice and that people, is not my job but my dream and my goal. Thank you all for helping us achieve it.

Bless you all [♥] Hudson's Mummy Erin. ~BREATHE~

Our June Hospital Admission

This blog entry has been a long time coming. So many details, so many emotions that it just seemed like too much to write down. But the specifics are starting to get blurry, so I guess it's time.


The day started like normal. Ruby was happy and eating well. But just before I gave her her afternoon bottle, she did one of those huge projectile vomits like you see on Australia's Funniest Home Video Show. She seemed ok, she didn't cry, but she wasn't interested in her bottle either. Same with dinner later, not very interested in that either but she did take some milk. Which was also projectile vomited shortly after. Nothing is simple with CF and you don't have the luxury of waiting it out, or saying "see how it goes". So we called the CF fellow who said we need to go to emergency at the local hospital for an x-ray to rule out a bowel blockage. (not Westmead)


Sounded easy enough. Hospital bag (always packed, ready to go) in the car, Pop over to watch Eliza and off we go. Well, as any parent whose taken their kid to emergency knows, we entered a timewarp. We got seen to quite quickly and were put into a separate cubicle thingy. We told them why what we were there for and that having CF meant that Ruby was susceptible to blockages in the bowel. Then we sat and waited. Ruby just slept most of the time, only waking when her obs were being taken.  A dr saw us a few hours later. We re-iterated again that we just needed an x-ray to see if there was a blockage or not. Not as easy as that. Ruby's tummy was squeezed by every second person who came to see us. I realised that it had then been 24 hours since her last poo and I expressed again that I was worried about a blockage.

We were told multiple times that kids pick things up, and gastro was going around.

Waiting waiting waining in emergency

It was 2.00 in the morning when we were taken for an x-ray. Craig was supposed to leave for his new work position at 4.30 in the morning. Needless to say, he took the day off.

The x-rays were inconclusive, her urine sample came back clear for UTI. So now the dr left it up to us. Go home and see how she goes or get admitted to further observation. Ruby chose that moment to projective vomit again which made that decision easy. Off to the ward we went.

Using a pillow so she didn't choke on her 

vomit in her sleep

And that's when I really felt like I was running under water. Intentionally or not, I was made to feel like I was over-reacting. I can sort of see why. She had vomited twice, was off her food, had a slight temp and hadn't done a poo for a day. BUT SHE HAS CF! Nobody seemed to understand that bit. Every question was fobbed off

"have you contacted her CF team at Westmead?", "oh, the dr will do that"

"she is not keeping anything down, should we start IV's? (CFers are very susceptible to dehydration)" "oh, the drs don't think it's necessary yet"


I called her CF team who said they were keeping tabs on her, but unless the hospital said to transfer her, then she should stay where she is.

Two nights we were there, sweet FA happened. I have no idea what they were waiting for, but I have never felt so frustrated or helpless before.


Then her vomit had bile in it. It was like a slap in the face to me. That was how our whole CF journey started. Bile at 12 hours old, bowel surgery not much later.

So I turned into mama bear. I demanded the nurse to call a dr for me. She said they were just about to do the rounds and will be here soon. I said it was urgent and we need help now. So she disappeared and came back and said I would be first to be seen. Dr came in 5 minutes later and said she vomit was probably that colour because her stomach was empty. I was a bit rude then. It pretty much went like this:

"That vomit is not normal. Last time she had that, she had surgery so excuse me for being one of "those" mums. She hasn't done a poo for 2 days, she is vomiting bile, she hasn't kept anything down, and she is not even on IVs. Do you know how HARD I work to keep weight on her? I need her CF team at Westmead to know. I think they may want to assess her. Has anyone even called them?"

She gave me a hard stare, said she would get IV's started and then call Ruby's CF team. Then turned and left. And I finally felt like something was happening, especially when the nurse got the IV started.

Finally got IV's started

Dr Glare came back in about 3 minutes later and said very bluntly "Westmead called, she's getting transferred". I was a bit shocked, "what now?". "yes, that ambulance is on it's way". And she just left.

I found out later that the staff though I had called Westmead, but in fact Westmead called for a progress report and organised the transfer as soon as they heard about the bile.


So a few hours later we were settled in our room at Westmead. Within half an hour of being there, we were seen by her whole team, had bloods taken and had a colonoscopy done.

The transfer to Westmead

NG tubes fitted, pulled out, re-inserted...X-rays, ultrasounds...

Needless to say, it was horrible. Ruby became withdrawn. She would cry whenever the door to her room opened. She whimpered in her sleep, she stared into space when she was awake.

The last option we had before using surgery was to feed her a solution through her NG tube which was potent enough to break through the blockage. But not without her vomiting it up the first round. I can't even imagine what it was like to bring that stuff up. But it worked, and finally, she pooed. Phew...Yay for shit! Observed for another day, and we were right to go. She even started smiling at a few of the med staff before we left which I never thought would happen!

After the blockage finally moved

What a heartbreaking time it was. Ruby was 9 months. Old enough to know that painful things were happening to her, but not old enough to understand why. As a mum of a child with a chronic illness, it's just going to be a part of life where I have to hold my child down while strangers are hurting her. A horrible part of life, but unavoidable. I don't want Ruby to get used to this type of thing. I wish that the next admission wouldn't be for a long long time.

Next time...because there will be a next time...we go straight to Westmead.

Finally able to eat again!

Too Aware?

Eliza became a bit more aware today.

She started to watch the NBN interview on my iPhone (she can work the thing like a 20 year old). The first line came through loud and clear. "if a cure isn't found, 9 month old Ruby will DIE from cystic fibrosis" That was as far as we got as I snatched the phone off her. She looked at me with her big blue eyes and said "I don't WANT Ruby to die"

What did I say? "Don't worry, she won't. Well she will, but not yet. When she's really old. Is that Daddy home? Want an easter egg?"  Didn't handle that one too well.

She didn't say anything more about it until about 10 minutes later. It was obviously on her mind. She stated how clever she was saying cystic fibrosis properly. She asked why Kai and Ollie didn't have CF. "but why does Ruby?'

I hope she understood what I meant when I said that everyone is different, on the inside and the outside.

She knows the names of Ruby's medicine, she checks to see if she needs her Creon. She helps(?) with physio. Eliza is a very switched on little girl. She makes sure Ruby doesn't touch other kids drink bottles and I've even heard her tell someone off for not covering their mouth when they coughed. She is always seeking answers and wanting to know more. No detail goes unnoticed with Eliza. So I have a feeling that Eliza will still be very young when the gravity of the situation hits home for her. When she starts asking the hard questions. When she can't be so easily distracted.

This of course hasn't saved Ruby from the normal sibling rivalry. Which I am grateful for!Nothing like a bit of a shove or a stolen toy to make you feel normal ! Thankfully balanced out with lots of cuddles and gigges together xx

Dear Blog

Dear Blog, 

Sorry I haven't written here for a while...the world has been finding things for me to do lately. I promise I will return and offload all of the things going on in my mind when the universe slows down just a tiny bit. I am at the end stages of the Team Ruby auction (still trying to buy my baby a cure), another side project that just seemed to snowball into something amazing. Once again I have under-estimated the compassion and generosity that people have shown us. It makes me wonder what we have done to deserve this wonderful support, instead of letting me wonder why we deserved to have CF in our lives at all.

Ruby has been waking with severe tummy pains throughout the night so I have been tired, even too tired to come and visit you. I haven't had a good entry in a while. I know I posted many times in May for 31 Days of May, the Cystic Fibrosis Way but they were more for awareness purposes, not lightening my emotional load.

So thanks for waiting for me...

From Ruby's Dad

These words are from Ruby's dad, Craig. We went through this together, and we were there for each other. During our time at Westmead, we took turns at being the strong one, and we held onto each other for comfort. Absolutely no way I would have be able to go through this without him.

I will never forget the night Ruby was born. Some say there is no such thing as love at first sight. Well they obviously don’t have children, because the moment I saw Ruby, I was totally in love with her. This was also the case with Eliza. To have such strong feelings for someone you have only just met and who has only been on earth a few seconds, is a feeling that has to be experienced to be understood.


I remember the birth and my first hold of Ruby. Looking into her innocent eyes, at that moment I made a contract with Ruby, it was a pretty simple one, it was to do all I could to protect and help her. I believe all parents mentally make similar contracts the moment the see or hold their newborn children.

When Ruby started to have her initial problems at hospital I was shocked as she had looked so well when she was born and seemed happy. She breastfed almost straight away and seemed to be the classic healthy baby. My mind was dealing with a hundred thoughts and as many emotions when they transferred her to the high dependency unit. As her condition worsened and the outlook became more serious, I went into auto pilot. You just do what needs to be done. I never thought I would need to start fulfilling my contract so early.

When Ruby was transferred by Newborn Emergency Transport (NETS) to Westmead, I was so overwhelmed with what to do. I had a wife who was devastated that her newborn daughter was being taken away, a 3 year old who just wanted her parents and a newborn daughter who was seriously ill. What do you do??? I had to leave Danni in tears and take Eliza home and then try and get some sleep. All after having very little sleep and with less to come, I set the alarm for 5am and set off with Eliza to Westmead, racked with guilt that I hadn’t been there for Ruby all night. I arranged for my parents to pick up Danni from Gosford Hospital and bring her down as soon as she could be discharged and to drop Eliza off at Danni’s aunties. I later found out Danni’s parents had called into Westmead on the way from Melbourne and made sure Ruby was ok. This was such a relief, as Ruby hadn’t been totally alone.

The following weeks were a mixture of worry, sleep deprivation, relief and sadness all mixed in with some wonderful joyful moments. I had never before experienced such a time of turbulent emotions. Watching Ruby going to have major surgery at 3 days old was the hardest thing I had ever done. This was the first time I had cried since Ruby’s birth. Hearing her diagnosed with Cystic Fibrosis after being told how well her surgery went just a few moments earlier, was like a kick in the guts. Rocking her to sleep for the first time was a fantastic feeling and wheeling her outside the first time was such a relief. Speaking to the CF team and getting all the relevant information and do’s and do not’s, as well as collecting all the medications just made me feel sadness for the future of Ruby. But when we left that hospital and arrived home I felt absolute relief and joy, as well as a determination to make Ruby’s life a beautiful, healthy and wonderful life.

Ruby looks well but she is sick. Her life has many medications and treatments already and without her enzymes she would waste away. Her future will hold many more challenges, it will also hold many wonderful moments and will be the best life we can possibly offer her. The support network of family and friends Ruby has are the best she could wish her. Her Mummy is the most wonderful mum in the world. The care she gives her, the research she has done, the diet she has worked out, as well as the love she provides are all Ruby needs and then some. This is whilst still providing the same caring and loving mothering to Eliza. Danni blows me away each and everyday.

As Ruby’s Daddy, I aim to fulfil my contract to her. I will be there for her at every part of this journey. I don’t know what the future will hold but I understand the realities of this disease and will ensure Ruby is never alone in this fight. I am not a doctor or scientist, so I can’t cure her but I can fight for her at every opportunity. I can help raise money for research and care and do my utmost to raise the awareness of CF. So when you think that maybe we are bombarding you with CF information and stories and if you think maybe we are a little too focussed on this disease, think about the ‘contract’ we all make with our children. Whether we make this contract consciously or unconsciously we all do it and I would hope, all fulfil it to the best of our abilities. Think how you would react if Ruby was your daughter.

Thanks to all our family and friends who have provided such wonderful support to us and Team Ruby. We love you all………

7th Day - SCHOOL

School school school.
This is a long way off but it actually plays on my mind quite a bit.

What if Ruby misses out on so much that she falls behind?
What if she is bullied because of her CF?
Will she want to tell people about it or keep it a secret?
Will she miss out on things like school camps?

I  believe that she will be healthy enough to not miss out on too much. I see her as being an assertive child who will not take crap from anyone. Confident enough not to be ashamed of having CF, intuitive enough to pick friends who will accept.  Plus, she will have a secret weapon there..her big sister! If all else fails, I will homeschool her. Simple as that.

But before school, there is childcare. Not planning on using that. I am lucky enough (at the moment) to be  a stay at home mum so there is no need. Now that Eliza is 3, she goes once a week to a small pre-school. Maybe Ruby will too, when she is 3, we will see. I'm not worried about Ruby not learning social skills before this age. Her health is more important. We have a circle of friends we see quite often, she is most certainly not kept in a bubble. I have a few great friends who I trust to mind her if I need to. I am blessed.

DIAGNOSIS

This is how we were told about Ruby's CF.

We (me, Hubby, my dad, my cuz and my aunt) were waiting for the surgeon in the tiny parents room at the NICU ward. Ruby had just come out of surgery for a suspected bowel blockage. She was three days old. The surgeon came in and assured us that Ruby was fine and in recovery. On a paper towel, he drew an explanation of what had happened. A part of her bowel was blocked up with her meconium (that first yucky baby poo). The blockage had killed off part of the bowel, and had to be cut out (20cm). The two ends were then rejoined which was a bit tricky as they were different circumferences. The technical term for the blockage is meconium ileus.

But the surgeon was really happy with the outcome of the surgery. She didn't require a stoma, colostomy bag or follow-up surgery. He estimated that she would be able to feed by mouth in about 10 days.

Then he said now, I also have some bad news. Whoops, suck in that sigh of relief! He explained that meconium ileus is normally a result of Cystic Fibrosis. In fact, there was a 90% chance that Ruby had CF.

This is a day after her surgery. The Drs were very impressed with the speed of her recovery.

Clever little bubba!

Now imagine that your child needed their tonsils out. You feel a bit scared, as any kind of surgery on your child would be. When it's over, you are just glad that it's over. That's the end of that. Or is it? Imagine that the surgeon then tells you that they also saw a lump on your child's throat, and that there was a 90% chance that it may be cancerous. It's not over anymore, it's just beginning...

To be honest, I didn't even know what CF was. I was getting confused with Spina Bifida. And to be really really honest, I was relieved when the surgeon corrected me and I realised there was no physical deformity/disability. In other words, I was glad the she will look normal. 

The diagnosis was further confirmed with the newborn screening (heel prick test) 5 days later, and then with a genetic blood test. She hasn't had a sweat test yet, she will after her 1st birthday.

The CF team worked with us the day after the surgery, they will be our team until she is 18.  We felt like parents for the first time again, so much to learn, so little time to do it in. Every time I thought I had the gist of it, another piece of information would throw me.  It was mentioned a few times about the shortened lifespan,  but it was a while before we were told the magic number of 37 years. And you know what? I was relieved! I was expecting 6, or 10 or 18. How horrible, to be relieved that you baby has a life expectancy of 37 years. Sounds like the kind of question you ask when purchasing a dog, or a washing machine. This shouldn't even have to be discussed when talking about a new life.

We were reminded many many times how much the quality of life for CFers has improved, how much research is helping this disease. This is no comfort to a newly diagnosed family. It means nothing. It doesn't change the diagnosis, doesn't help you leave hospital early, doesn't break the news to your friends for you. It's a bandaid on an amputation. It's something safe for people to say to you. Nowhere as near as comforting as a hug, a text, a hospital visit. I am lucky, I received many messages of support, an much help with Eliza who was not coping well at all.

We fell in love with Ruby the day she was born. She was our Ruby, our baby girl. When we were told of the possibility of her having CF, I kept reminding people, she is still our Ruby. Nothing will change that, our love won't diminish. 

I will not pity her, I will not cry for her. I will not sit around the hospital cafeteria and say "poor her". She is my Ruby, she is the baby that stole our hearts, just like her sister did 3 years earlier. CF is something she has, not something she is.