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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?

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

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.

Clinic Update April 2011

Today we had Craig's parents come with us as we had a genetics appointment after clinic, but more about that later.

Ruby is starting to become more aware of these visits and she seems to know what is about to happen. As normal, she was very happy on arrival, and we are always greeted with cries of "here's Ruby!" The staff there are very friendly and genuine and are always commenting on how well she looks. Of course, Ruby encourages this behaviour by beaming at them all.

She was weighed, and then measured, and that's when she started to get suspicious. She really fought hard about being laid down for a length check and had quite a loud sook about it.

First up was the dietitian, which is the main reason we go monthly. I really like out dietitian Christy. Even if we don't agree on some things, I always feel I can argue my points and we often come to compromises. Ruby got full marks for being a big eater and liking all types of food. Christy was happy about the weight gain, but still wants her on the strongest mix of formula until her weight catches up with her length. She know I'm not happy about this. We talked Creon and fat content, food ideas etc etc.I tentatively mentioned to her that I made a point of giving Ruby Hydralyte to drink everyday, wondering if I would get told off. But she was happy I was doing this and told me I don't have to be so strict on adding salt to her diet now that I am going this. Yay for me!

Then we had a sputum test, which I found out later is pointless when she is on Bactrim (grrr). Obviously, this is never fun, but it was over quickly. I couldn't help but admire how Ruby maintained direct eye contact with the nurse whilst crying very loudly at her...if looks could kill.   (I wanted to take a photo but thought I would look like a bad mum)

Ruby's respiratorist wasn't there today so we saw the fellow instead. She asked all the right questions and listened to her chest. All clear! I also asked her to check Ruby's heart as I had been told previously (when we were admitted to Gosford hospital) that she has a systolic heart murmur. But she said all sounded normal.

By the end of it all, Ruby was crying whenever anyone looked at her for longer than 2 seconds. She even cried when I changed her nappy, I'm sure she was waiting for something unpleasant to happen. This is why I want to try to space these visits out as much as possible. I don't want Ruby to feel unsafe and scared, even if only for a day. 

So we will be going back in 4 weeks. I actually pushed for 2 months and they compromised with 6 weeks, but there were no appointments available, so 4 weeks it is...

So that was Ruby's visit. Now about me! I have a circle of Facebook friends who are CF mums. I surprised myself by becoming close to people I have never met. Is this how Dungeons & Dragons players feel? Anyway, I got to meet one face to face today! Only very briefly as her and her beautiful daughter were in isolation, and it was a bit tricky with my in-laws there also, but it was still exciting!

Team Ruby

Well our day has been and gone. I'm sure everyone by now has heard me rave on about how touched and blown way I was with the amount of support and love that we received, so I won't write about the day. Instead, I have made a montage! Don't worry, it's not too emotional and not too long. But a picture tells a thousand words, so here you are...




Our fundraising so far is $14250 with more on it's way. Half the money goes to our CF clinic at Westmead hospital, the other half to CF NSW. It's not too late to donate if you haven't already!

DONATE TO TEAM RUBY


***if you have more pics, send them to me! There are team members I am missing***

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! 

Bee in my Bonnett

Yes, I officially have a bee in my bonnett.

As a family, we have entered our first CF fundraiser/awareness event. We have entered a team in the 65k 4 65 Roses Walkathon. We are Team Ruby.

On the 5th of March Craig and other team members will be walking 65k in one day to raise money for the CF Clinic at Westmead Hospital and for CFNSW 

I feel like I am doing something. I have a goal, a purpose. I'm raising money to save my daughters life. I know that sounds melodramatic, but lets look at the facts. Let's look past her happy personality and beautiful smile and see whats inside. You would see a set of lungs full of mucus, ready to hold onto and breed bacteria, turning it into an infection, resulting in scar tissue. You would see a pancreas that doesn't work as it is too clogged up of that same mucus. You would see a re-sectioned bowel. You would see that same mucus again in the ovaries, stopping the release of eggs, leading to infertility.

Sometimes it's hard to compredend what is going on inside, when outside she is just so perfect.

But I'm getting off track here. Back to my bee. I've been buzzing around like mad, promoting our team and organising fundraising. This is so important to me that I sometimes have to take a step back and remember that not everyone has a child with CF. Not everyone feels as strongly about this as me. I have this sense of urgency about me and I don't really understand it. All I know is that money will cure Ruby's disease. I wish it wasn't like that but it is. The CF gene has been identified and has been cloned and "cured", but not permanently. They are so close to finding a cure , money pays for results, as simple as that.

I have had a few tears leak out during Team Rubys existance. The faith that I had lost in the universe (without really realising) is coming back. I have amazing friends. Hell, I even have amazing aquaintances! The amount of people that have helped us is just heart warming, it really is. Here are some amazing things that are happening for us:

My yoga teacher is donating a whole mornings takings to Team Ruby
My friend Shirley is donating 10% of her Avon sales to Team Ruby
My Tupperware lady is donating 20% of sales to Team Ruby
Our family friend Mel is walking the walk, but is also helping in a million and one other other ways, even though she is a mum and works full time
My friend Laura is giving away 3 remedial massages for us
My mothers group let me use them as an outlet, they are my best friends and I love them all
My cousin Michelle donated a manicure for us to use as a prize
My friend Caz is donating a family photography sitting to one lucky donor
My friend Anita has asked that no presents be bought for her sons birthday, just donations to Team Ruby

So, please help us out if you can! You can donate here:
www.mycause.com.au/teamruby 

And join our Facebook page here:
www.facebook.com/teamruby 

This will be our one major fundraising effort for the year

Thankyou.

When I First Knew

I don't think of myself as a negative person. I always see the glass half full, and always try to keep an open mind about things.

But when the surgeon told us that there was a 90% chance that Ruby's meconium ileus (bowel blockage) was the result of Cystic Fibrosis, I just knew that we were part of the 90%. I wasn't thinking worse case scenario, for some reason this was the only scenario. We were told it would take about a week for a first stage diagnosis (heel prick test results).

The next day (or so) Craig went back up to the coast for a bit. I took Eliza to the playground in the hospital grounds. I remember the day really well. The sun was shining and I was actually feeling relaxed. Eliza was behaving and we were getting in some much needed quality time together. Eliza was playing on the spinny thing with an older girl, about 5 or 6 years. Her mum asked me if I had the time, and, as women do, we started chatting. She asked me if we has someone in the hospital and I told her my 4 day old baby was recovering from surgery. The rest of the conversation was sorta like this:

Her: "oh, up in Grace Ward"
Me: "yeah"
Her: "Sally was a NICU baby too and had an operation at 4 days old. What sort of operation?"
      Now, in my time in hospital, I was beginning to realise that if you pronounce NICU like nic-you and not by the letters, it means you have normally spent a bit of time there yourself. She said nic-you
 Me: "her bowel. She had a blockage"
Her: "did she have meconium ileus?"
Me: (looking at her strangely)"yes..."
Her: "oh, Sally had exactly the same thing. It was diagnosed at my local hospital and we were rushed here and she was operated on after that. We stayed here for 3 weeks and once they were happy with her bowel working again we went home"

She then called her daughter over and told her to show me her scar. The girl dutifully yanked up her top before running off to play again. You could barely see it, it was in the exact same position as Ruby's. 

Then there was a bit of an uncomfortable silence. I didn't want to ask if her child had CF and she obviously didn't want to ask if I had been told about the risk yet. I can't remember who said what, but yes, Sally did have CF. I told her we were waiting for the test results. We had a big chat after that.

It was after this event that I was absolutely certain that Ruby had CF. Any amount of doubt was eradicated. Why, out of all days, would I meet this lady and her daughter? Seeing that happy, healthy looking, energetic little girl do something so normal as play on a playground with other kids prepared me for the diagnosis. That is when I started coming to terms with what was in store for us, not a week later when we received the official diagnosis.

*The girls name wasn't Sally, I can't remember her name!
 
 

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

CF in a Nutshell

So, what is it? Here are some facts without getting too complicated and without having to read all the horror stories out there on the net:

• Cystic Fibrosis is the most common life threatening, recessive genetic condition affecting Australian children. Both parents must be a carrier, you can't "catch" CF.
• Around 10% of babies born with CF have "meconium ileus" - an obstruction in the intestines caused by thick, sticky baby poop. This requires surgery, which is what happened with Ruby.
• In people with CF, salt and water aren't transferred properly from the cells, causing the body to produce thick sticky mucus.
• It affects a number of organs in the body (especially the lungs and pancreas) by clogging them with the thick, sticky mucus. 
• The mucus is a breeding ground for bacteria. Repeated infections and blockages can cause irreversible lung damage and premature death.
• Mucus can also cause problems in the pancreas preventing the release of enzymes needed for the digestion of food, especially fats. Malnutrition can be a problem.  

So what do we do for Ruby?

• Daily physio where we try to "knock the snot" out of her, is how I describe it.
• She is on antibiotics until she is 18 months to give her a good head start of being healthy and strong, and hopefully not getting any chest infections in this time
• Enzymes are given before every feed so her body breaks down food (milk). Otherwise it would just pass straight through her, nutrients and all
• Salt is great for people with CF, so she is constantly breathing in the lovely salt air!
• Excercise is also a very important part of keeping healthy as it prevents deterioration of the lungs and improves physical bulk and strength.
• Regular visits to the CF clinic at Westmead

The life expectancy of a person with CF is 35 years. But don't let that scare you, this is double what it was 25 years ago, and is always increasing. There is no cure for CF, but the faulty gene has been identified and doctors and scientists are working to find ways of repairing or replacing it. With today’s improved treatment most people with CF are able to lead reasonably normal and productive lives. A great amount of time is being directed towards finding new and improved ways of treating CF and of finally finding a cure.

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.