Slipping

I feel like I am starting to lose control of Ruby's health. Not in an end of the world kind of way, but little bit by little bit until it turns into an avalanche. My focus on her diet has always given me the self confidence boost that I am managing a part of her CF well. She has dropped from the 20th percentile to the 12th percentile. Her vitamin levels have dropped again, putting her generalhealth at risk. She is back on anti-biotics, fighting a bug plus a staph infection. Did I not use enough Glenn 20? Her excema has flared up and she is itchy. I can't seem to make her comfortable. I can't seem to make her happy. If she's not happy, I'm not happy. The harmony has gone. How do I fix this? Will I regain control? Will my family ever get a good night sleep again?

Three different people told me this yesterday:

And if three people say it, then it must be true, right?

Our June Hospital Admission

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


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


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

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

Waiting waiting waining in emergency

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

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

Using a pillow so she didn't choke on her 

vomit in her sleep

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

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

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


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

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


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

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

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

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

Finally got IV's started

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

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


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

The transfer to Westmead

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

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

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

After the blockage finally moved

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

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

Finally able to eat again!

Too Aware?

Eliza became a bit more aware today.

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

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

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

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

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

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

Photo Friday

OK, so I'm a day late with Photo Friday...

SOMETIMES IT'S

OK IF THE

ONLY THING

YOU DID

TODAY WAS 

BREATHE

Breathe-Emptiness by Must Valge

 

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?

support?

I don't even know where to start with this one...maybe at the beginning.

I love photography. Not just taking photos, but looking at them. My own, friends, artists. Ask any of my friends, I'm always the one with the camera. I managed a photography studio for many years. When I look at a picture, it takes me right back to the time it was taken, emotions and all.

Since Ruby was born, I sometimes choke up a bit looking at peoples professional newborn pictures. To me, they represent how the post-natal period should be. Warm, snuggled, safe, perfect. Things that our family missed out on. It's just a reminder that life turned out different for us. It's not a bad thing that I get like this, I don't throw things at the screen when I see a new bubba! But I do sometimes get that pang of "oh, I wish I had that".

There are many chat/support groups on Facebook for people affected by CF. One of them is called "Get it off your Chest" and it's supposed to be a place where you can say what you're feeling.

Apparently not.

I posted in it about how I was how sometimes looking at these portraits sometimes niggles at me and how it reminds me of our experience. Well somehow on the screen it must have come out as
Geeze, my life sux! I can't believe that CF stopped me having cute photos to hang on the wall. I wish I didn't have Ruby, as not having any photos is sooo tragic that surely nothing else could even compare!!
Or something like that. Because the reaction I got was not what I was expecting. I was told by other CF mums that I should be grateful for what I have, and how "they wouldn't change anything" (like I was implying I would). I was sworn at, and told I was whining about stupid things.

I tried to explain that I'm not so shallow as to have a cry about missing out on nice photos, but about what type of emotions it bring up to me to see others photos.

Made no difference. Got told I should stop writing and f#@k off. So I stopped. I couldn't believe that I could be so misunderstood. Total feeling of frustration.

I checked back a bit later on and saw that Maddy had left a comment. She is 20 years old and is at the end stage of CF. She wrote this
"Ey everyone- this is called 'Get it Off your Chest' for a reason. Just because someone is going through something 'lesser' than yourself- you shouldn't look down on them. Lay off Danielle, she was only saying these 'perfect' pictures are her symbol of what ruby's birth wasn't. It wasn't the "STANDARD" birth where there's a mum and a dad and a smiling nurse and a "HERE'S YOUR BEAUTIFUL BABY BOY/GIRL!" photo still where everyone's beaming and saying "He or she is so beautiful- they are perfect, a perfect HEALTHY BABY" well done sperm and egg! You have A HEALTHY BABY ALL IS WELL. The poor girl is saying for her it was a mad rush of get this kid into ICU- humidcrib, stuck with iv's whatever happened to her- it was the beginning of her CF journey- and who wants to start that? If choice be given, you wouldn't want your kid to start that journey- or anybody to start that journey- because I tell you there's a lot of SUCK in this journey. I'm sure like any of you good mothers- you wish the best for your children..and Danielle only wished the best for Ruby- and ruby was born with CF- and that isn't the BEST possible outcome. Don't apologise, I didn't realise that "Get it off your chest" came with a list of no go zones either. She never said she wasn't grateful for Ruby- and we can't all be a ray of freakin sunbeam ALL the time either! We all have our off days, hell when it gets really bad- we can have off weeks- maybe even off years..we should all SUPPORT each other? Even if one of us is whinging about a sore throat. You'll be a better person for it..maybe. I think everyone saying they weren't offended is a bit of a fib, going on the general consensus of "oh my god appreciate what you have" thing going on there. 'Taking the higher ground' doesn't mean pretending you weren't pissed by what people are saying.?

And I thought, thank God someone gets it. I have also had a few other message of support since. But still, really opened my eyes to another side of the internet. The post quickly disappeared after that. I have since been "defriended" from a few people and chucked out of a few CF groups (including that one).

I'm still a bit baffled by it all...on one hand I know I shouldn't care what a bunch of strangers think of me, but on the other hand, I don't want people to believe that I am a dark, negative person. If you have ever read my blog, so will see my positive attitude I have towards life. 


I've never really had to deal with anything like this before...

 

Dear Blog

Dear Blog, 

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

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

So thanks for waiting for me...

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.

16th Day - !@#!? UPS

 OK, so common theme here, I don't really have much to write about, but I'm not complaining! I do remember this though.

 When unexpected situations arrive, when you are not a seasoned hospital mumma, you tend to just trust that the medical staff are doing things for a reason. Before you have gathered your thoughts or gotten your bearings, you just stand in the middle of the storm, watching all these "important" things happen around you.

Then, the longer you are in that environment, the more you feel a part of it, whether you want to or not. You go from timidly asking the nurse if you can change your baby's nappy to pretty much doing the "cares" yourself. You understand more what each cord it, what alarms not to worry about and what fluids are going through the IV lines.

The second week at NICU, an orderly came u[ to Ruby's crib. He looked at his paperwork and said "Ruby?" Yep, I replied. 

"I'm just going to take her down for her ECG" he said. 

Now normally, I would have just presumed it was just another procedure that she had to have, but this time it just didn't seem right to me. I got him to check his paperwork again. It definately said Ruby, he confirmed. One of Ruby's nurses turned up (they were never far away) and checked the paperwork herself. Yes, it said Ruby.but not our Ruby. It was the Ruby on the other side of the room with congenital heart disease. 

Although this was not a life threatening mistake, it was a wake-up call for me. It showed me that although NICU was run like a tight ship, mistakes can and undoubtebly will happen. It is my job as Ruby's mum to protect her from these mistakes. Just as you would hold your child's hand when crossing the road, I will check my child's medication.

Another CF mum gave me some advice, she said that I am my childs advocate..her voice..and that...I should always go with my gut instinct and if no ones listening or taking me seriously...then keep on perservering!

 

 

 

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.

11th Day - P WORDS

My P word is physiotherapy

Ruby has been having physio since 2 weeks old 

She will have it for the rest of her life

She doesn't mind it, as you will see from the video. She doesn't really enjoy being on her side, but she doesn't put up too much of a fight...yet! 

The aim of physio is to knock the sticky mucous out of her lungs. Coughing is encouraged, to help get that sticky mucous up. As she gets older, other physio activires will be added such as trampolining and bubble blowing. Not everything is a drag with CF!

 

 

 

 . 

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………

10th Day - PRO'S &CON'S

  Pro's of CF? I'll let you know when I find one.


Here is a con for you:

Your mum calling you in tears to cancel her long awaited visit due to ill health.

 

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.

7th Day - SCHOOL

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

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

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

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

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

5th Day - DIET

FAT, CALORIES AND CREON

When we were getting CF explained to us, it was always mentioned how the CF diet required extra fat and calories and salt. I didn't worry about it too much as Ruby was still on intravenous TPN, let alone solids. I envisaged changes like switching from low fat yoghurt to full fat.

That's not quite what they meant.

avocado mixed with Greek yoghurt and grated cheese.

I could feed Ruby McDonalds for breakfast lunch and dinner and get a thumbs up from the nutritionist. I could dip her fruit in chocolate and call it a healthy meal. I could feed her a stick of butter for a snack... ewwwww.......

But I don't want to do that.

So I use other things instead. Here is a typical days meal for her at 9 months old

Breaky: 2 weetbix with chia seeds and milk, or bowl of Greek yoghurt with chia gel.  Vegemite on toast.

Snack: cheese (loves cheese!), avocado, tomato and sultanas

Lunch: meat fingers (made with beef mince, grated vegies, coconut oil)

Snack: baked beans and grated cheese

Dinner: fritata type thing I make in the bowl with egg and vegies and cheese and microwave. Washed down with a drink of Hydralyte

Desert: chocolate (plenty left from Easter still!)

Plus 3 - 4 bottles of fortified formula per day

The snackbox in the CF dietitian's room

When I was introducing solids, I would just cook up some vegies and add coconut oil. Then I started making things specific for her like red lentil casserole.

Before, she was on solids, I did my research. I finally felt like a had a heads up on something. Time to prepare, time to learn. Here was a way I could take control of my baby's health. No prescriptions needed. I could research and make my own decision as to what I felt was best for her. Some interesting thing I have found.

Broccoli helps clear damaged lungs as well as being a great immunity booster

Coconut oil is amazing! Google it! The parts that I like are: increased immunity, proper digestion, had Vitamin K & E, and is has the highest amount of fat. And it smells good!

Chia seeds are also high in fat and are known as a "superfood". They can be mixed with water to make a gel, and have no taste. 

raw tomato, tuna and cheese covered broccoli & carrots

 


I don't do the whole calorie counting thing, I just feed her as much as possible. I adjust the amount of enzymes when I notice her poo is oily, or if I am told to. At the moment, Ruby has 2 capsules/scoops of Creon 5000 for every meal unless the meal is very low fat or I think most of it will end up on the floor. 

Sometimes I feel like I'm trying to fatten up a prize goose to take to market! But I enjoy it, it is therapeutic, and Ruby certainly isn't complaining!

zucchini & mushrooms sauteed in lotsa butter

cheesy tuna & vegie rissoni with coconut oil

.

4th Day - ILLNESS & ADMISSIONS

I'll be totally honest, this entry is a total rush job. Ruby is restless in bed, she is not well. I am tired and still need to get ready for our clinic day tomorrow.

So in a nutshell

Five days after coming home from NICU, we took Ruby to the local hospital as she was lethargic and not feeding (it was Fathers Day)

She ended up staying there hooked to IV antibiotics for 5 nights for a high fever and unknown reasons. Not even really sure if it was to do with CF. 

I cried for the first time when she had 4 lumbar punctures (3 unsuccessful) to rule out menengacoccly. Spelt that wrong I know!


Dr casually pointed out that she has a systolic heart murmer and may need heart surgery. Westmead later told me she will probably grow out of it, which she has.


I realised during this time that CF really is a free pass to be paranoid of sickness and demanding of hospital staff.


The only other hospital visit was for what ended up to be constipation. Another CF lesson. HYDRATE HYDRATE HYDRATE!


You can read about it here:


A Common Cold?


I can hear Ruby coughing in her sleep, I hoope I won't have another admission to blog about...

3rd May - MEDICATIONS

Medication will always be a part of Ruby's life. Preventative medicine to stop her getting a bug, and aggressive medicine when she does get a bug, to stop it turning into a lung infection. These  repeated infections eventually lead to lung damage. Many people don't realise that with CF comes digestion issues. Most people with CF are pancreatic insufficient which means they are unable to absorb fat or fat soluble vitamins which lead to ill health and poor weight gain. 

Here is a photo of Ruby's daily medication (the suppositories aren't daily!)

 

1. Probiotics - actually these were for me when Ruby was breastfeeding. To try to stop any issues with constantly taking antibiotics
2. Antibiotic - Ruby is on a prophylactic dose for 18 months. This means it is a low dose to help her fight off any bugs while her lungs are still growing to give them a chance to get nice and strong. The dose is low enough to let her immune system still develop. She has this 3 times a day.
3. Pentavite - a multivitamin for general health and immunity, as well as a boost of the fat soluble vitamins
4. Micel E - vitamin E supplement (also fat soluble). For some reason, it is not in Pentavite
5. Plain old table salt. This is added to her milk and food. The cause of all the CF problems (sticky mucus) is due to salt not moving properly through the body. Extra salt is needed as CFers have much saltier sweat and loose it quickly, leading to dehydration and other problems. 
6. Suppositories for constipation. Constipation can occur for many reasons, including not enough salt in the diet
7. Creon (digestion enzymes) They are the most important! These do what the pancreas can't, they break down fat. Ruby has this before any food or drink that contains fat which is pretty much anything except for most fruits and vegies. The dosage depends on her weight and fat intake. At one stage she was on 3/4 of a capsule, that was fun...She has been having this for as long as she has been feeding ans she will do for the rest of her life. The older she gets, the more she has. Could be 10 capsules before a normal meal. If she doesn't have this, she will literally poo out fat and oil and have a gut ache
8. Super dooper formula with probiotics. I have listed this as a med as she has a need for it, it was not a feeding choice. She is on a high ratio of formula:water and goes through a can every 4 days
9. Nasal saline spray. Most mums have used this for their babies. It's basically salt water I squirt up her nose to keep the snot moving. I use it all the time
10. Creon again, but the granules are already in a jar so I don't have to keep splitting open capsules (as she can't swallow then yet)
11. Antibacterial soap - no she doesn't eat it, but it was in the med drawer
12. Apple gel - this is what I tip the Creon onto for Ruby to eat. The Creon needs to be coated in something acidic so it gets broken down in the right place in the gut. If I forget apple gel, I normally find tomato sauce somewhere

 And that is all at the moment! Trust me, this is not much. Not pictured is a bottle of Hyrdralyte which she drinks for the same reason as adding salt. We qualify for a health care card so we get the Creon and antibiotics cheaper. Everything else is full price. The formula is $28 a can (can normally find it on special) and the Hydralye $11. We go through one of each every 4-5 days.

So much for my natural parenting, toxin free ideals!

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.