Outrunning the Train

The following is written by Ruby's dad. There are times in our life where it becomes evident that Ruby's CF plays on his mind, putting it mildly. After reading this piece, you may be able to feel what it is like, loving someone with a progressive chronic illness.

Picture a railway tunnel. You know the old style brick/sandstone ones with soot on the walls and  moss around the entrance. It is seen entering through the side of a mountain, just a dark opening. If you stood on the outside of this tunnel, it would stretch far off into the distance, with no visible end, with no light at the end of it, just a never-ending darkness with numerous dangers and obstacles.  This is the tunnel we entered as a family when Ruby was diagnosed with Cystic Fibrosis. She was 3 days old and at the time I didn’t even realise this was the path we had embarked on.

Now picture a big old steam train, huffing and puffing, smoke pouring from its stack, wheels screeching and carriages rattling, steam whistle blasting. This train has entered the tunnel behind us and the only way out is to make it to the end before this train bears down on us. In my mind, that train represents Ruby’s Cystic Fibrosis. It is coming through that tunnel, I can hear it, I can feel it but I’m not sure how close it is. I still can’t see its ominous light, I can only feel the wind it is pushing through the tunnel.  I am trying to drag us through to safety, we are running blindly. Every now and then a small light is on the tunnel wall, this is where we learn some of the dangers in the tunnel, we begin to understand the realities of negotiating this tunnel but so many obstacle still remain hidden.

Each time we trip or stumble that train gets a little closer, the noise a little louder, the rumble of its approach can be felt that bit more. We hope that we will see the daylight shining that signals the end of this tunnel. We are confident that one day the tunnel will end and Ruby will be free from this race. That train will have run out of steam or, a medical breakthrough will derail it for good. For now we can savour the moments of light and hope they increase, we can escape to another place at times and enjoy the good moments and joy her life is bringing to us but each and everyday we must re-enter that tunnel and keep on running and carrying our Ruby as far from that train as possible. There is no day off from this fight, there no cure to stop this train, only the reality that some day it will bear down on her and take her away, just like it has to so many before her.  All we can do is keep her medications, treatments and physio happening each and every day, in the hope we slow the train down.

For now our job is to carry her as far as we can, then the inevitable will come and she will need to run and take up the journey herself. All the lessons we can learn about the tunnel and where all the lights are, need to be passed on and taught to her. What obstacles she will encounter, what happens if she stumbles and how we can try and stay ahead of that train will need to be imparted.

Soon she will be old enough to ask ‘why she is in this tunnel and why is she running’. I dread that day, the day she will hear the train and feel  its rumble, its wind pushing behind her, the understanding of why we do all we do each and every day for her. The realities of what the train means.

Thankfully we have a great support from family, friends and her medical team at Westmead Children’s Hospital. The journey would be so much harder without these shoulders to lean on, without the good advice, without knowing people who care so much.

We need that cure to throw this train off its tracks.

Salt Therapy

I'm not ashamed to admit, I've been struggling in the last week. I have felt like I haven't been the mum I should be or the mum I can be. But what's been even worse, I have felt like my kids feel the same way.

Late this afternoon, our day was taking the same path as it has been doing lately. Kids getting cranky at each other, me getting cranky at them, setting ourselves up for a night of tears from all parties involved. It was nearly time to start dinner but I decided to take an impromptu trip to the beach. I wanted to get out of the house. I decided I should take the kids with me. (joking, joking, I was always going to take them).

10 minutes later we were down at the beach. Eliza headed to the water straight away to jump waves. Ruby, in true little sister style, followed straight after her. But she wouldn't let the water touch her feet, she was too scared.

So I went in to where the waves were mid-calf and she ran after me. Facing out to sea, she stood behind me and wrapped her arms tight around my legs. Every time a wave rolled in, she clung tighter, pressed her head against me and squealed with laughter. For 15 minutes she clung there. Her grip never faltered the sand swallowed her feet and the small but powerful waves rushed past her. She made me feel strong and happy. I was her rock, she trusted me 100% to keep her safe. It was exactly what I needed to remind me of what I mean to my kids. And what I need to be for them.

Eliza's need was totally different. She spent her time running in the water, jumping waves and chasing imaginary fish. She was content to play on her own, without any interruptions, periodically looking over her shoulder at me to make sure I was watching out for her. Which I was. I will never stop watching out for her.

We came home happy and salty.

Amanda

Utterly emotionally drained after saying farewell to a beautiful soul today. I cried for my friend, who lost her sister. I cried for her mum whom I share a common bond with. I cried for myself. The sadness in the chapel was tangible. No-one wanted to leave. No-one wanted it to be final.
Amanda was well loved. Her friends grief and tributes left me wishing I knew her better. She was an amazing person and I look forward to hearing more stories about her life, when her sister is ready to share them with me. A life so full of love and living that it's hard to understand how such a progressive illness eventually takes over.
The drumming tribute calmed and grounded me. What a beautiful parting gift her friends gave her. Observed by a lone girl, dressed in colour, shrouded in sadness. A woman who has lost her best friend. What can you say to ease the pain?
Amanda did not let Cystic Fibrosis get her down. Anything I say about her will sound like a cliche. Her life was about kindness and nature and for standing up for what you believe in. In 28 years, she did more, felt more, helped more than others do in 80 years.
I know I am now going to live every day better, for having known Amanda.
Today was a sad day, my friends and I held each other and cried. Friendship is a beautiful thing.

Closer

My skin is getting thinner instead of thicker. My bubble is popping. Reality is setting in. I don't like it, it shakes my soul. I have started this journey backwards.

Most people who have had a baby diagnosed with a chronic illness start off on shaky ground. They are worried, fearful. Who can blame them for thinking the worst? Their whole world has been turned upside down, and not for the better. After time, they start to find normality. No sorry, normality is the wrong word. Nothing about this is normal. They learn to live, and see the positives through the shit. They find hope in medical advances, feel purpose in being proactive. Like most things in life, I seem to have gone about it the wrong way. I started off full of confidence and purpose. I hit the ground running. Don't get me wrong, it didn't take me long to find out what CF was about. I made sure I knew as much about it as early as possible. And to me, this was (and still is, always so much more to learn) a way of coping and preparing. Much like you explain to young children what is going to happen in a check-up so they know what to expect be less fearful, I have tried to prepare myself for whatever challenges may come. Osteoporosis, diabetes, IV antibiotics, more hospital stays, lung function decrease, nasty bug, transplant list. Can't shock me CF, I know it all. And I won't let you scare me enough to fear life itself.


But now, Cystic Fibrosis has taken on a form for me. It's dark and fluid, and just out of the corner of my eye. It watches me give treatments to Ruby, it follows me around. CF isn't scared of me anymore, it's just loitering around us. Nothing has changed with Ruby health wise. In fact, she is clinically doing quite well at the moment. But it's still there, waiting.


Cystic Fibrosis claims lives all the time. You just have to be part of any CF group online to see how often. It hurts when we lose a member of our community.

 Then someone you know personally dies of CF. Someone I knew as a real-life person, separate to the CF world where we are joined by the same disease. Someone I got to know before I had Ruby and even knew what CF was. And it rips my heart out. It's the pin that pops the bubble. Her death leaves me wide open to questions I usually I would not tolerate. To the fact that this journey will not end well. No matter what I do. But most of all, I feel sadness for my friend who has lost her sister. I hope with all my heart that Eliza and Ruby will have the same kind of relationship that J & A did.

What difference does time make? If you know you are going to watch your child die, why should that hurt any less now, compared to when it is actually happening? Is the knowledge just as bad as the event? I'm guessing not. There is always more room for pain.

There are older people with CF out there. They teach us that there is the chance that my daughter can live to past her 30s. I should take comfort in this. But instead I get a bit sad that these people have something special about them, and that special something is their age. An age that is midlife to the rest of the world. Greedy me, I want Ruby to grow old and wrinkly.

I don't really know where I am emotionally at the moment, but I am happy to just drift along and see where I land. What will be, will be.


Dum Spiro Spero

Too Aware?

Eliza became a bit more aware today.

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

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

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

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

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

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

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?

13th Day - THE FIRST TIME I HEARD THAT WORD

Technically I didn't hear it, I read it

  Burkholderia Cepacia

Often known as B. Cepacia, here is the medical explaination:
Burkholderia cepacia (B. cepacia) is a group of bacteria that is found naturally in wet soil and decaying plants, such as rotting onions.
Once B. cepacia enters the body, there are three possible effects and it is not possible to predict which will happen. Sometimes B. cepacia colonizes in the lungs, causes no symptoms, and has no long term effect. Other times B. cepacia colonizes in the lungs and causes damaging lung infections and inflammation that lead to a slow deterioration of lung function. In the worst case scenario, B. cepacia can spread throughout the body causing “cepacia syndrome”, which leads to a rapid deterioration of lung function. Cepacia syndrome is a serious condition that does not always respond well to treatment. Unfortunately, even with proper treatment, cepacia syndrome can lead to death within a few weeks.

I've read a lot of shitty things about CF, but when I read this I honestly got chills down my spine. Onions don't get bought in our house unless I am using them for that nights dinner.

I'm having a hard time writing this because I don't know how to describe why I feel this way. It is not a worry, it is a dead set fear. Yes, there are other bugs out there that are also harmful to CFers. Yes, B. Cepacia is not very common, but it is there, and my brain has decided that I am going to be afraid of it.

People who have cultured B. Cepacia aren't allowed to attend CF activities or functions. They are often also automatically taken off the transplant list.

A couple of days after I learned about this bacteria, I was talking to a friend whose sibling has CF. They are the only person I know with CF who I haven't met through the CF community. I asked about their health and was told that they had picked up a bug that the Dr's were worried about. When I asked what it was, my friend couldn't remember exactly except that it sounded like "spacer". My friend had tried to look it up on the internet but didn't know what to read. The internet can be a dangerous place! We weren't in the right place to have a proper conversation and the coward in me was glad. I guessed correctly that B. Cepacia was what we were talking about.

I cried that night about how unfair life is. Our friends know the sibling is sick, but they can't realise the magnitude of it. I do and I think about it often, and again I get chilled to the core.

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………

8th Day - NON-COMPLIANCE

     Well once again I am very lucky to not have much to write about. Has your child ever been on a round of medicine that they didn't like taking? Imagine having that struggle every day.



"eat your crusts or your hair won't grow curly"
"have your medication or you won't be able to live"





Sometimes being a good parent is having to be the bad parent. The parent who physically holds down their child while they are getting shaken by a vest. The parent who forces their child to breathe in salty irritating air through a mask. The parent who doesn't save their child from the prodding doctors...

...all because we love our kids.

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.

31 Days of May - The Cystic Fibrosis Way!

This is a great idea that a fellow CF mum had to raise real awareness as to what goes on in a month of a CF family. We have chosen the month of May as it is international awareness month.

Everyday I am going to post something about Cystic Fibrosis  

Everyday you will find out something new about CF and how it affects our family and my child's life

Some topics won't be relevant to us...yet

We are one of many families who go through this, I will also be sharing their experiences with you

love and kisses to you all!

I am sitting here surfing the net (instead of cleaning the saucepan cupboard) looking at CF stuff. The fundraising and awareness part of CF I mean. And I am getting angrier and angrier and ANGRIER.

It's pretty poor really, the CF website is out of date by about a year, the links in it are also out of date (ie Great Strides info) and really, there doesn't seem to be much going on.

CF families, are we living in a CF bubble where only our friend know about this disease? This is not some weird quirky thing that only some people get!!! It seems that if you don't know someone with CF, then it doesn't exist!

I have only been in this CF bubble for 8 months. I had no idea about it a day before that. Why not? I should have. Every one should!

Everyone should know that one in 25 people is a CARRIER for this DISEASE. Everyone should know that one baby every 4 DAYS will be BORN with this DISEASE. Everyone should know that TRANSPLANT and DEATH before turning 37 is not a possibility, but is practically a STATISTIC! 

Excuse me, but where are that ads on TV? Anyone ever remember seeing one? There is a beautiful one for MS out at the moment. It breaks my heart, I want to give them my money. The autism one also gets me every time. Kids cancer awareness...well we all know how successful they are.

CF kids look healthy, BUT THEY ARE NOT!

65k 4  65 roses raised a LOT of money. I thought it was a starting point but it looks like the high point.

So I am angry. And I will continue to be angry until there is more done for our kids. I will do it myself if I fucking have to.

Now I'm pressing publish before I calm down. 

 

5th April
OK, so I have calmed down now and thought I should clear some things up before I get myself booted out of the CF community!   And also to correct my spelling mistakes. I just get frustrated and although Ruby is healthy at them moment, I have a real sense of urgency to get more support. I get frustrated when things aren't up to date eg I wanted info on buying an Entertainment Book as I know that is a good fundraiser for CFA. The webpage has not been updated since 2009, even though they are selling books for 10/11. This is just one fussy example. There are lots of others. 
There are many successful fundraisers that are organised by the CFA, I will do a separate blog entry on what is coming up.
And then there are the amazing parents and community members who organise their own fundraisers. People organising events in their own time to raise thousands and thousands of dollars.  65k for 65 Roses is a great example, raising around $140,000. I honestly though that this was the norm, but it is not.

So I'm sorry if I offended anyone. I know for a fact that the CF community is a strong, loving, supporting community who want what's best for the people and families living with CF in Australia. I know that the people working for CF Australia work really hard and are 100% committed. I know I sound like a new comer who doesn't know what she is on about. I just want more.

I want to help. 

Super Women - Helen Macnair

I am very lucky to have a handful of amazing women feature in my life somewhere. Some are so important to me, that it will take me a long time to be able to write about them. Some deserve a mention straight away. Helen Macnair is one of them. She is my pre & post natal yoga teacher.



Now, when I say yoga teacher, I don't mean one of those gym instructors who have taken a 6 week course in yoga to teach to the fitness freaks. I mean one of those teachers who have yoga running through their veins, those who live their lives as though it is one big asana. Just like you can tell a professionally trained dancer from the way she holds herself, you can tell that Helen is someone who cares for herself, mind body and spirit. She is a super qualified yoga teacher, having qualifications with Birthlight, Yoga Bugs and the Radiant child teacher training, and is also a trained doula. But to be honest, that doesn't really mean much to me. I am a beginner when it comes to the world of yoga. What is more important to me is what her classes taught me, pre-natal yoga in particular.

First of all, there is always something nice about sharing time with other pregnant women and listening to each others experiences. There is no sense of urgency in the classes, no rushing or skipping people. Helen always asked everyone how they were going with their pregnancies and always listened carefully to the answers. She is a fountain of knowledge when it comes to pregnancy ailments and non-invasive solutions. The yoga classes were often tailored to suit whatever was bothering us at the time, for example, bubs feet up in the rib cage! So many things she taught us, so much proof she showed us to trust our bodies.

I find it hard to be still. I could never meditate or slow my thoughts. Yoga changed that for me. I learned to breathe. I learned to take big, deep, slow breaths in. I learned to exhale out not only air, but pain and worry and fear.  These breaths gave me the strength to experience a natural, quiet, calm birth without intervention, without any distractions to disrupt that all important post natal time.

The benefit didn't stop there. These deep breaths also gave me strength when I needed it most. They stopped me from becoming depressed when I was separated from my baby by 100kms during her first night (as well as a good ol' cry to my mum) and spent the night in a maternity ward alone. I breathed my way through walking Ruby to surgery, I still don't know how my legs worked that day. When we were told about CF being a real possibility, I kept my composure by just breathing. I know it sounds basic, but can you remember a situation in your life that just took your breath away? Did you have someone there to whisper in your ear "just breathe"? I felt like I did.

Then there is the future. I have a daughter that will eventually develop chronic lung disease. Suddenly the phrase "just breathe" has taken on a whole new meaning. I want Ruby to learn how to breathe. And I don't mean like the blonde with the headphones kind of breathing. I mean the life saving, lung strengthening, head clearing, deep, breathing. Helen is my secret weapon, even if she doesn't know it yet! She will teach my daughter how to be strong, she will help her beat cystic fibrosis. I have full confidence in this, and a touch of mothers intuition.

www.bellaroseyoga.com

PI

It scares me that Ruby isn't self-sufficient. Or that I can't provide for her without help.


It scares me that she would not survive without her medication.


What if we had a natural disaster and all of her meds were lost?


What if Ruby and I are kidnapped? She would cry and cry and starve. 


What if there was some kind of massive evacuation and they only took people with no medical problems?


What if we got lost in the bush? I can't even breastfeed her and deal with the consequences of not having her Creon. 

Very unreal circumstances I know, so why do I worry about it so much?


Pancreatic Insufficient is the technical term.

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!
 
 

Eliza's Birthday

Family dynamics change when a new sibling comes along. Most parents with more than one child can attest to that! Our daughter Eliza had to adjust to so much so quickly, and without the comforts familiar to her. Her home, her friends, her toys, were taken away from her when she probably needed them most.

That's why we decided to make sure she had a proper 3rd birthday party. Her birthday is 3 days after Ruby and we were originally going to forgo a party for her this time. But she needed some time away from the hospital, and this was the perfect excuse.

 Lucky for us, we have amazing family and friends who organised a party in about a day. Craig and
I didn't have to worry about anything. There was just enough people there to make it special without it being too overwhelming. They were all people important to Eliza. I wasn't able to be there, but I really, truly didn't mind one bit. I was just happy knowing that she was having a fun day with the focus totally on her.

 When Eliza first arrived at the park with her dad, she stopped him and asked if he could please hold her hand while they walked. It took her a while to get into party mode, but with the help of her family and friends, she ended up having a fantabulous time!

Photo Friday

Isn't this a powerful photo? I love everything about it. I love the pose, the colour, her expression. I love that it is a real photo, documenting someones real story. The girl in the picture is Miranda Hutson. She is 22 years old and received a double lung transplant due to her Cystic Fibrosis in Jan 2010.  The hospital tags are hers, the oxygen tank was a part of her life before the transplant. I don't see the picture as being morbid or negative. I don't know why, but I just really love this photo.   Photographer: Curtis Almeter, who was also in hospital with Miranda.

So What If I Have Cystic Fibrosis, I'm Unstoppable!  is a Facebook page that Miranda has created. I love how the majority of the members are teenagers, and I sometimes stalk the page, watching them interact, and just be typical teenagers! It gives me great hope for Ruby.

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.

On and on...

I am a mum, I do mum things for my kids. Cook, clean, nurture, teach, play, raise...the list goes on. We all know how much is involved with being a parent, I am not alone in this!

But I am not a scientist, a researcher, a doctor or a health professional. I don't know how to cure Cystic Fibrosis, I don't even really understand the biological, genetic or technical side of it. I do know that the medical community is confident that a cure is close (and not in a "miracle cure" found on A Current Affair type shows way) and I have faith in that.

So, what can I do to help? I can spread awareness. I can let people know what CF is and how it affects us. I can hound you with raffle tickets, research links and status updates. I can share photos, stories and experiences.

So sorry if my blog posts and Facebook page seem a bit CF obsessive, but it's the only way I know how to contribute towards the best possible outcome.


 


Here is an interview with Dr Michael Boyle about the basic defect in CF and how the two drugs, VX-770 and VX-809, aim to help fix the underlying problems, but in different ways. Good news for CFers, even though it's not very exciting viewing!