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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.

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!

Clinic Visit April 2012

Our clinic have changed the way they operate. We get assigned a room to stay in and everyone comes to visit us. This means that there is no separate clinic for different ages and different bugs as they used to do for infection control.  We all go on the same day, but as we aren't sharing a common area (like a waiting room), the risk of cross infection is much lower.

So when we turned up, I couldn't stop looking at all of the other kids. It sounds strange, but I hadn't seen a CF "big kid" before. I couldn't help but think, that will be Ruby in 10 years time. Of course I know that Ruby will be going to clinic forever, but this was a little glimpse into the future. Instead of feeling sad about it, I was just a bit curious  and fascinated. These kids were confident and self assured. Some qualities I already see in Ruby. The nature of the disease?

First things first, the weight in. And we have a gain! In fact, it was quite a good gain and Ruby now weighs 10.44kg. She is in a good proportion to her height. This keeps the dietitian happy and we don't have to discuss supplements of any kind.

Next we see a respiratory doctor . It's not her normal doctor and I feel like we didn't get as much out of the consultation as we normally do. But he said Ruby's lungs sounded clear and he is going to get some info to me about the phthalate that is still used in Creon.

After the doctor comes the CF nurse. She brings in the suction machine to collect Ruby's sputum. This will be tested for any bugs that may be growing in her lungs, but we a re particularly looking out for how much pseudo is growing. If the growth is heavier than last time, despite being on very strong nebulised antibiotics, we will be admitted to hospital for IV antibiotics.      (please please let's hope the tobi has done its job)

Sputum collection always causes tears but is soon fixed with a specimen jar full of jellybeans!

Checking her sats

Next on the list is our super friendly physiotherapist, Alli. We go through our current physio regime, and practice our "vibrations" with Ruby. We also do a trial of hypertonic saline. This involves testing Ruby's oxygen levels before, during and after the treatments. She tolerates it well. Hypertonic saline is used through the nebuliser before or during physiotherapy to help the lungs move out the mucus. It also re-hydrates the lungs. When using the hypertonic saline, Ruby gave quite a few big chunky coughs. This is the result we want, it means it's doing it's job. But it was also quite confronting. More evidence that her lunged are full of goop. It's not nice to hear your 20 month old cough like a smoker.

So now our treatment regime will be:
hypertonic saline
chest physio therapy and vibrations
inhaled tobi
twice a day. It should take about an hour each time. We borrowed a new nebuliser from clinic as the previous one we borrowed seemed to take so long for each treatment. We are looking at buying our own nebuliser because let's face facts - she is always going to need it. They range from $450 to $1900.

What else? Oh, we picked up some meds.

That's about it. Ruby slept the whole way home. Our next visit is at the end of May. Now I will go online to my CF mumma friends and have a good debrief. xx

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!

Not so distant memories

Will my memories of NICU always be so acute? Will it seem like just last month forever? Does it ever fade?

9th Day - GREATEST MILESTONE OR BIGGEST CHALLENGE

I choose...biggest challenge! And I am going to be a bit self-focused and write about MY greatest challenge with Ruby. And it was breastfeeding.

Now I know breastfeeding can be a bit of a contentious issue and also a touchy subject. Just remember, these are my feelings about the way I wanted to nourish my baby. What other people choose to do is their business.  I am a huge believer in informed choice. Whatever you choose to do, I believe you should research your choices. So my choice was to breastfeed Ruby. Firstly because of the health benefits of breastmilk. Secondly because I wanted to do something for her. Once she was admitted to NICU, everything felt so artificial. Even when we brought her home, there was so much factory made, processed, branded, plastic, chemist bought CRAP. I didn't feel like she was getting anything REAL. I wanted to do more for her than just syringe fluro coloured drugs down her throat.

I also wanted to use breastfeeding as a way to bond. And I don't mean emotionally. I have felt connected to her from the day I felt her kick inside me. I mean physically. I had skin-to-skin contact with her once she was born and that was it. Yes, I could look at her all I wanted in that little plastic NICU bed, and I could hold her when I asked the nurses to help me with the cords, but I never snuggled with her, never rocked her to sleep, never held her naked against my bare chest. Even her first night at home was spent in seperate rooms due to a broken air conditioner and her inability to sleep restfully by my side. And who can blame her? She had been sleeping by her own since she was born.

When she was in NICU, I went every three hours to the cold expressing room, where the four chairs were facing each corner like you were in trouble and the only noise was the sound of the squeaky pumping machines. Then bag it tag it and store it. My last pump each night was about 11.00 when I used to walk from the Ronald McDonald house to the hospital in the dark and freezing cold. I then woke up with a thudding chest and had to go and "get Ruby's milk" no matter how much Eliza cried for me to stay.  I felt like a cow. Them my beautiful midwife came all the way from the central coast and loaned me a hospital grade pump that I coud use in R.McD room. That helped so much with Eliza.

When the surgeons gave the go ahead for Ruby to start feeding again after her bowel surgery (she went 10 days without eating) I wondered if she remember how to do what she did so well 10 minutes after birth. And she did! She latched on with ease as to say "what took you so long?". And it felt SO DIFFERENT to that stupid machine! Everyone was impressed, everyone was happy. The nurses were especially glad that there was one less mouth that they had to feed, and as I was never far away, they just called me if she was hungry and I wasn't there. I wasn't a cow anymore, I was an on demand breastfeeding mum.

Then we went home and things were different. Ruby was fussy, always coming off, always crying afterwards. I was scared she wasn't getting enough but didn't want to say it to the clinic staff. But her weight gain was so slow that they wanted to try something different. I tried to talk about it to them. I told them that Eliza was also very slow in gaining weight, even worse than Ruby. "but Eliza doesn't have CF". Would I be willing to try formula? No thank, I don't want to feed my child a bottle of crap. I can do this, I'm her mum, it's my job. So we tried PolyJoule, which I made up everyday to give after each feed. Still too slow. Try formula now? No, there is nothing wrong with my boobs! So I started expressing. I hired a hospital grade pump. My day consisted of making PJ, boiling water, steralising bottles, feeding, pumping, steralising more, feeding. As well as the "normal" med dispensing, physio giving, child raising duties. Pumping didn't work. I got hardly anything. So I finally had to admit to clinic that I couldn't feed my child on my own. So we talked about supplementing with formula. That's OK, it though. This happened with Eliza. We comped for about 4 months and then she was heavier and happier and fed much better until she was 18 months. Ruby had different ideas. Craig had to by that first can of crap. He bought the super dooper plus this plus that version. He apologised to me for buying Nestle! I just can't catch a break!  As mum I know that this marketed crap is not as good as my milk. As a mum, I also knew that my baby was full and satisfied after a bottle of formula. At the next fortnightly clinic weigh-in, she had put on enough to make the staff happy. I cried. I felt like a reject.

Soon the breastfeeding was supplementing the bottle, and eventually at 4 months, she didn't want the breast at all. Didn't even want to lie against me. I went around the house singing "Ruby doesnt want my booby!" So she still has her formula with an extra scoop plus added salt. It has only been in the last month that I have started to look at formula as another CF medication that she has to have. Still don't like it though. Still have giving her a bottle in public. Still hate buying the cans. One every four days. That's alotta cans.

This is why I am a bit obsessed with feeding her real food.

There is now an organisation called HM4HB which is a milk sharing network. If this came along a bit earlier, I would have seriously considered it..if I was strong enough to deal with her medical team about it.

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………

6th Day - CLINIC

  CLINIC DAYS!

I will always remember the first time we went to clinic. I hated it. It was like walking into a secret society. The staff were welcoming, they had all heard about Ruby and were excited to meet her. I hated that they were so nice. I wanted to tell them "don't be too friendly, we won't be coming back". I didn't want to be part of this club, I didn't want to meet any of the other parents, I didn't want to have a reason to be there. 

But it is a part of our lives now, and it always will be. I don't want clinic days to be a negative thing for Ruby, so I have changed my attitude. 

Clinic is pretty much an all round check-up. The last one was just two days ago, Ruby was 9 months. First she was weighed then measured. Then a chat with the physio who was impressed by how she can now blow. Then another chat with the nutritionist which is always informative. 

Then the sputum sample. This is where spit is sucked out from the back of Ruby's throat to see if she is culturing any bugs. Depending on the result of the sample, her antibiotics may be changed. The plan is to catch anything early before it causes any damage.

Here is what happens:

Clinic days are scheduled by age groups and what bugs your child has previously cultured. This is to avoid the spread of certain germs that can be very detremential to a CF childs health. Believe it or not, clinic kids look normal. If you walked in by accident, you would not even realise that there is anything wrong with these kids. There's that whole "invisible disease" thing again...

We go to the clinic at Westmead Children's which is over an hours drive away. It makes for a long day for a little bubba but she does well.There is a clinic closer to us at the local hospital, but I just don't feel comfortable changing care yet. Westmead have been there from the start, it's just how it is.

Links to our clinic updates:

http://danni-herewegoagain.blogspot.com/search/label/clinic

2nd May - THE FIRST YEAR, THE WORST YEAR

Well we are still in the first year, so I can't really say it is the worst year! It definitely has bad times, but there are so many wonderful moments as well. 


Compared to others experiences, I would say our first year so far has been quite uneventful. We've had a bit of everything but nothing too serious. It's been like an orientation period. A period of learning and unlearning. All the experience we thought we had as parents to a 3 year old? Out the window! Everything had changed. The way we fed Ruby, the physio, the medication...
She has never even spent the night in our room. She was an independent one alright. Didn't like lying in peoples arms, wouldn't go to sleep if you were holding her, total opposite of Eliza!

Eliza and Ruby meet for the first time

I found it hard learning about the limitations she will have. Being told things like, no indoor pools, no sandpits, no bath toys, no eating dirt, no jumping in puddles, use antibacterial products at all times, avoiding childcare. All said in the same breath as "but you can't stop her from being a kid".


The first year is hard because these are babies we are talking about. Babies who should be at home with their families in a safe environment. Not poked and prodded and detached from their family. 

Ruby's first breath of fresh air EVER! 15 days old.

The first year (so far) has also been so wonderful! Ruby is a great sleeper, that is all I ever wanted! We have seen her little cheeky character come out. She is a determined little thing with a beautiful nature. She wins people over by smiling at them, from the day she learned to smile. She loves watching her sister and I love watching them together. Just like a family without CF, the first year is a special year full of discoveries and special moments. Despite the start that we have had, I am loving this year so far!

 

Our first family photo! Ruby was 15 days old and finally cord free and able to leave the ward

Clinic Update Feb 2011

We left Eliza with nanny & pop today as she had a cough and I refuse to be one of those parents who take their kid to pre-school when they aren't well. Craig wasn't well either so he hung out at the cafeteria while Ruby and I went to clinic.

I always forget to prepare myself for the initial walk inside the hospital doors. It's like you enter another world. A world that exists purely on the fact that shitty things happen to kids. It sucks the breath out of me when I first walk in, but it only lasts for a second.

Because Ruby has had a bit of a runny nose, we were put in a room on our own, which was actually quite nice!

First stop, the weigh-in...and she weighed...6.595kg!  And she is 54.4cm long. Good work on both accounts.

Then the physio came to visit us. She is totally in love with Ruby and always insists on giving her big cuddles. Because Ruby is sitting up now, we can add some other aspects to her physio. Mainly bouncing her up and down and using an exercise ball. Physio for CF is fun for kids, as anything that pushes the air out quickly is good for their lungs. So bring on the trampolines and pogo sticks!


Next visit was from Ruby's respiratorist who looks after the overall care of Ruby. He gave her lungs the all clear and wrote of a script for more Creon. As I left, I noticed the script was made out to Ruby Creon so next time I'll be careful not to talk to him whilst he is writing!

Then we had a sputum sample taken. Tube down the throat, a bit of gagging, and it's all over. Hopefully nothing sneaky growing down there.

Last visit was from the dietitian, who came in with a huge smile on her face. She was very pleased with Ruby's weight gain and gave me the "gift" of letting me throw out the PolyJoule. (PolyJoule is powdered carbohydrate, when made up it is as sticky as superglue and I just hate it). We are still on a super dooper strength of formula though, until her weight catches up with her length a bit more. She asked me what I was doing with her solids, and she was very impressed with what I have been feeding Ruby.

And I felt really proud of myself. 

Choosing Ruby's food is the first time I have felt that I have total control over an aspect of her care. I can make my own decisions, my own informed choices. I chose to skip the goopy baby cereal and make a chia seed gel instead. I chose to add coconut oil to her foods instead of the normal lump of butter. I let Ruby decide that she was really for solids, and Ruby only. I may have failed at breastfeeding, but now I can choose what foods she is eating, and as I prepare it all myself, I know exactly what is going in her body. And, the best thing is, they were the right decisions, Ruby is doing really well.

For other CF mamas reading this, here are some questions we asked:

What is the "perfect" climate/environment for a CFer to live in?

"Your house". The doctors way of saying don't do anything drastic. He then said that coastal climates are best, due to the salty air and surf. Also, it is more important to have good health care close by, than the actual conditions of the environment.

Now that Ruby is 6 months, do we buy the next stage formula?

Dietitian informed us that there is no reason to change formulas, there really is no beneficial difference between them all. Discussed how formula companies are evil.

Time frame of giving Creon and continuing eating?

Creon 5000 lasts half an hour, so if still eating after half hour, give some more

Next visit in a months time! 

A Few Memories

I remember being in the special care nursery at Gosford hospital, thinking everything would be ok once Ruby did her first poo.

I remember noticing in NICU how long some of the babies had been there for. Months and months, and they weren't even premmies.

I remember the glass cupboard in the tea room full of books like: So Your Baby Has A Disability, Living With Multiple Sclerosis, Understanding Cystic Fibrosis. I remember being glad that I wouldn't have to worry about anything like that.

I remember wondering why the nurses hands weren't sore, dry and red raw from the antibacterial soap that had to be constantly used.

I remember Craig & I walking Ruby to the operating theatre. I was so scared but I didn't want Ruby to see me cry so I just smiled stupidly at her and told her how much she was loved. I remember wondering if it would be the last time I would see her alive.

I remember relief warming me when the surgeon told us how well the surgery went. I remember fear chilling me when he then told us there was a 90% chance that Ruby had Cystic Fibrosis. I remember confusing CF with Spina Bifida.

I remember the first time Ruby smiled. It was as we were leaving Grace Ward to go home and it was a proper smile, not gas! Good timing bubba! She hasn't stopped smiling since.

Wrong Answer

Ruby and I were 100 kilometers apart for what should have been our first full night together, all because I said Yes when I should have said No.

The day went from being calm and relaxed to rushed and urgent in what felt like a couple of minutes but was actually around 6 hours. The snowball started gradually, before cascading into a full-on avalanche. In a nutshell:
Me: Nurse, Ruby has brought up a bit of this weird green vomit.
Nurse: hmmm, that's odd. I'll tell the Dr to come around. Has she done a poo yet? (answer is no)
Registrar: hmmm, that's not entirely normal. I need to consult with my other important doctor person. Has she done a poo yet? (answer is no)
Pediatrician: hmmm, could be this, or that. Has she done a poo yet? No? Lets move her to special care, not feed her, shove a finger up her butt and give her her first x-ray.
I think special care was when our attitudes started to change a bit. We were no longer in a maternity ward full of families and their new babies, we were in a ward with sick babies and big scary cribs and no visitors allowed. I couldn't wait to get back on the ward when this was sorted.

After lots of idle time waiting around, and Ruby having more tests, the Dr came back to us and told us that Ruby would need to go to Westmead, and the nurses were arranging a chopper or ambulance now, whichever is available first. What??? Blah blah no poo blah blah maybe blockage blah blah might require operation...
My first question, can I go with her? Shouldn't be a problem.

I rushed back to my bed on the ward and started packing my stuff all up. As I was doing this, the NETS (newborn emergency transport service) arrived by ambulance. They were a lovely team of people but then all the red tape stuff began. I couldn't be transferred as a patient, I had to be discharged first. No problem, I had a really helpful nurse who rushed all the paperwork and gave me a crash course on expressing. She was doing her final check-up and asked me casually if my legs were sore. I said Yes.


She looked at me and said "really?". That should have been a huge sign for me to say "just kidding!" but no. I told her really. She suddenly got all concerned and started feeling my legs and explaining how I couldn't leave if I might possibly have DVT.

Umm, hello? I went on a bushwalk yesterday, I gave birth 16 hours ago and I have been on my feet in special care for the last 4 hours!

But no matter how much I downplayed it, she wouldn't budge. I wouldn't get the all clear until my legs were ultrasounded the next morning (this was about 8.30pm). Finally after much convincing, she chased up a doctor who was happy to discharge me after I was injected with something or other and promised to follow it up.
Oh the relief, my baby wouldn't be alone! But...while the dr was explaining this, Craig, Eliza and the NETS team were outside my room with Ruby all hooked up to a spaceship and listening to our conversation. I came out all ready to go only to be told by the NETS dr that I couldn't travel with them if I was a health risk.
My brain quickly tried to think of a solution but I couldn't get my thoughts straight. I kept looking at Ruby inside that thing but I didn't have time to really think of her. The NETS team were eager to get moving, with or without me. My options were:
a. Discharge myself and Craig drives us all down. Really not practical with a 3 year old to think of as well
b. Discharge myself, I stay home with Eliza, Craig drives to Westmead to be with Ruby and we head down in the morning
c. I stay in hospital alone, Craig and Eliza go home and we all go down in the morningl
Well we ended up choosing C. That way Eliza and Craig could get a good night sleep in preparation for a busy next day and I could get my legs checked out first thing in the morning. Craig promised that he would leave really early in the morning and I would meet him there once I got the all clear. So Ruby was whizzed away after I peered goodbye to her through the plastic window and the rest of my little family left also. I was all alone surrounded by other mums and their babies.

I grabbed my phone, walked to the visitors room, sat there in the dark, called my mum and finally let the tears run. I was sad for Ruby, not for myself. This is not how she should have been introduced to the world. This was not the cocoon I wanted her wrapped in.
I felt so bad that she had no family with her. But sometimes things do go your way. My parents were driving from Melbourne to Sydney and were about an hour away from the children's hospital. With no hesitation they drove straight there to be there for Ruby. They arrived just as they were settling her into NICU. Mum was able to give her a cuddle, and that is so important. I am so so grateful to my parents. Not only have they always been there for me, they were there for their youngest granddaughter when she needed them.

This is like what Ruby was transported in but hers had a metal casing over the top so you could only see her through the side window

Clinic

Clinic is where Ruby sees a team of specialists to help manage her Cystic Fibrosis. She normally sees  her respiratory specialist, CF nurse, nutritionist and physiotherapist. The team also includes a gastroenterologist and social worker. The clinic is at Westmead Children's Hospital. As a newborn, we were there at least every second week, now that she is a bit older, and more importantly, heavier, we only need to go down once a month. If she stays healthy, the visits will be spaced out to every three months.

Here is a photo journal of our clinic visit today:

 As soon as we arrive, Ruby is weighed and measured. She has put on weight and grown well! Infact she is growing longer so quickly that her weight is just keeping in proportion with her length.

  The staff at clinic always take the time to come and say hello. Ruby gets alot of attention as she always smiles at everyone.

This is the most unpleasant part of our visits. a sputum sample is taken to make sure there is no bacteria growing in her throat. The sample is taken by a suction tube being shoved down the back of her throat. Doesn't take very long to do. 

 This is the snack box in the nutritionists room where the kids are encouraged to help themselves! 

 There is always something going on at a childrens hospital! Today we got a visit from some fairies and superheroes. Eliza was very lucky and was given a bag full of treats! Textas, colouring books, stickers, jewellery, a magnetic "learn chinese" kit(?!)...

 Ruby with her respiritory doctor who is her primary medical carer. He is listening to her lungs to make sure they are all clear, which they are.

We saw the gastroenterologist today who checked out her surgery scar and asked lots of questions about poo!

We picked up some of Ruby's medication for the next few months. We always leave with more than what we came with.

A Common Cold?

Ruby in her adult sized hospital bed watching Play School

Ruby got her first cold the other day. Not a bad cold but ended up in a trip to the hospital regardless. Her snot is really sticky, not runny (thanks CF) so it didn't run out her nose. Babies can't blow noses or cough up phlegm purposely so everything ended up being swallowed. This resulted in her being constipated with a big sticky goopy poo which made it's way out with some help! Nothing is straightforward with a CF baby.

But we learn. Next time I won't be so freaked out by seeing green bile vomit and just give her some Glycerol. Next time I won't let Eliza watch Ruby get her temp checked rectally as a think we are in for a surprise when she next plays doctors...