Take This Moment...

Ok, so, again, I am just soooo not good at keeping on top of a blog. I think I have to accept the fact that I will never be one of those people who write something here every day. We have had so much happening of late... the last month or so has just been utterly chaotic.. but I will talk more about that later... maybe.

Right now, I want to talk about a friend.

This friend probably isn't what a lot of people would consider a friend. I have never stood in the same room as her, nor have I heard the sound of her voice. I have never held her hand or hugged her, I don't know what sports she follows or what music she listens to, but we share a common ground that is so deeply entrenched in our hearts and souls that none of that seems to be very important.

We both have daughters that are HRAS positive.

Our precious daughters are almost the same age, they even share some of the same traits... the same gorgeous smile, the same big eyes and the same sunny outlook on life... but that's about where the similarities in our lives end.

Tonight, as I watch my daughter sleeping in her bed and reflect on how far we have come and how far we have to go, my friend is watching her daughter sleeping and reflecting on the fact that her life is nearing an end.

You see, my friend's beautiful, precious little girl has cancer that cannot be treated and they have made the heart wrenching decision to let nature take it's course.

I cannot even begin to fathom the immensity of this decision, or the emotions that they struggle with on a daily, or even hourly basis, yet she does it with poise and grace. I read her emails and I cannot help but cry because even now, in her deepest hour of need, she is not only a friend, but a teacher, using her own pain to help guide others on this journey.

And with such a good and gracious person as a teacher, how can I not be a student?

Indeed, from her words, I have learned a great many things, some of which I already knew that have been reinforced in my heart, and some which are new words of wisdom that I am holding fast to.

I have been reminded that every moment is precious. We never know that is just around the corner. It is time now to hug my children, play with them, make them smile, make them laugh. Revel in the joyousness that is the foundation of new life, spend time with my partner and just delight in the little things that make us both smile.

I have learned to appreciate therapy and medications and doctors visits and all of the other stuff that comes with this life that I have had thrust upon me, for even though I may curse these things for being the bane of my existance on a day to day basis, but each time I curse these things I am also reminded that I have her in my life. I may despise therapy, but every day we do it is another day that I still have my daughter.

I have learned that even in the face of adversity that may seem impossible to overcome, I will find a way to get through. I have the strength and courage of a family with wisdom that spans half a century and encompasses the face of the globe, all of whom will be standing with me when I need them.

Nicola and her medical complexity may have turned my life inside out and upside down, and it may have challenged everything I ever thought I knew in my life, but it has also brought me friends and family who have become an important part of my life.

I may not see them every day, or even every other month. I am lucky if I see some of them every other year. I may not know their faces or hear their voices, but they are still in my thoughts every day. I laugh with them in their moments of happiness and I cry with them in their moments of pain, but most of all, I am just thankful that I have them as friends.

Now... with that, I have three sleeping children, medication has been given and feeds are up to date... so I am going to go and enjoy a few moments of precious sanity, savour a nice warm shower in this faux winter and then curl up in bed with a good movie and enjoy some nice spacious sprawl time before all of my off spring end up in my bed, which will be in about two hours and thirty seven minutes, give or take a few seconds.

But I leave you with this...

Take this moment and own it. Cuddle your children, kiss your partner, eat that piece of chocolate, and revel in the small things... don't take a single moment forgranted, because you never know what tomorrow will bring.

<3

The Emotional Roller Coaster

When you have a child with special needs, there are a lot of emotions you feel. You feel grief at the child that you have 'lost' and you feel anger at the powers that be for this predicament in your life. You feel resentment toward those with 'normal' children, even if you have said 'normal' children yourself... you feel sadness, happiness, isolated, lonely, afraid, confused...

I guess the same can be said for having any child, whether they have special needs or not... but the big difference is that when you have a child with special needs, you usually feel all of these emotions at once... and they conflict and battle within, trapping you in a hair raising, spine tingling emotional roller coaster as each emotion battles for ultimate supremacy.

When you have a child with a rare condition that is considered life threatening, it feels like each one of those emotions is intensified a thousand times over.

We become hardened and battle weary, at least to the outside observer. We hide our scars along with our tears behind a false facade of determination, yet behind every smile and laugh, there is always that little bit of doubt, that little fear... that little voice whispering in the deepest, darkest recesses of your mind where only your worst nightmares dwell.

I remember when Nicola was 3 weeks old. We had done our journey in NeoNatal Intensive Care Unit and Special Care Nursery and we were being discharged to "Parentcraft" for 24 hours of observation. This strange ritual was allegedly to make sure we were capable of parenting a baby before being discharged, something I found somewhat ludicrous considering that I already had two other babies at home waiting for me.

We were sitting in Special Care Nursery and they were doing her discharge assessment and then Dr Az turned around and told me that he thought she had a heart murmur.

Well, that was it. I broke down and I just sobbed. My baby had a heart murmur, she had a defective heart... after 3 weeks of all tests coming back 'normal' or with nothing found that to be told there was actually something wrong just shattered me, and really, in the grand scheme of things, it wasn't even something overly serious!

Oh how we change...

Since then we have endured countless more diagnosis', not to mention surgeries, and procedures. I have held my daugher as she battled at the brink of death, I have fought doctors who didn't believe in her, and I have seen her defy odds and create miracles that no one can explain to me...

I have experienced a level of intensity of my own emotions that I never knew to be possible... an intensity so totally overwhelming that it is actually terrifying.

I have laughed when I wanted to cry, I have cried when I wanted to scream, I have held fast to hopes that seemed impossible... and I have lived with the mantra, "There but for the grace of God go I!"

If I could take everything I have learned in the past 18 months and turn it into one statement to give to any one who is about to embark on this journey, it would be simply 'do not be afraid of the darkness'.

No matter how dark moments of my life may be, they are only moments, and those moments shall pass. There is always a light to look toward...

"I will love the light for it shows me the way.

Yet I will endure the darkness for it shows me the stars..."

With Love...

Tall Poppy Syndrome

Since the days of the infamous Aristotle etched his thoughts on parchment, Tall Poppy Syndrome has been documented throughout history. Given that some of you may not be familiar with the concept, TPS is a term used to describe a social phenomenon in which people with genuine merit are resented, attacked, cut down or criticized because their achievements distinguish them from their general peers.

I've often heard about this phenomenon, and outside obvious celebrity bashing, I had never actually witnessed it in person until just recently.

My husband and I recently dragged all of our children through what can only be described as a voyage into the second circles of hell last week. We took a 5 day trip to Melbourne. Drama's and nightmares and unwitting entrances into what could potentially have been the setting for a new slasher flick, I got to witness my first actual real life act of TPS.

Standing around at the RCH in Melbourne on Sunday afternoon, I was having a discussion about our goals for Nicola, and one of the things I raised was the fact that I was soooo excited about the prospect of being able to go to the UK Conference this year.

I was absolutely flabbergasted when one of the participants of the conversation laughed almost bitterly and responded with "It's nothing more than a Mr X. publicity stunt."

Now, I can't even begin to tell you on how many levels that is incorrect! I mean, I know how much work Mr X. and his beautiful wife Mrs X. have put into this event. And yes, there are one or two aspects of the event that are publicity driven, but seriously, how the hell else are we supposed to raise awareness of a condition so rare that most people will never even hear about it let alone meet someone who suffers from it?

But now, 3 days on from the moment those absinthal words were spoken, I have come to realise that it is a classic example of TPS.

Mr X. is one of those people that you often read about in those heartwarming stories that go out of their way to help others. He was the driving force behind the International Costello Syndrome Support Group and he has been one of the leading forces behind the conferences. He was also the very first person I ever spoke to about Costello Syndrome.

The day I was given Nicola's diagnosis he phoned me from the UK and listened to me bawl down the phone and virtually held my hand while I walked through those tentative first weeks.

He is part of the heart and soul of this group... and his support is invaluable to almost every member.

I guess that I should be thankful that despite the crap, there are people like Mr X. who still put their own needs aside to be there to support others. It's just sad that there are people who seem to delight in attacking and belittling their efforts and really it just goes to show that they are little more than sad, bitter and lonely individuals.

So why would someone want to attack a person as virtuous as Mr X.?

I guess there are as many reasons as there are stars in the sky... but at the end of the day, it comes back down to TPS.

The social phenomenon in which people with genuine merit are resented, attacked, cut down or criticized because their achievements distinguish them from their general peers.

All that aside, it was wonderful to be able to have a few days break, even if it was pure chaos! The girls had a great time, we got to do a couple of the sights and spend some wonderful down time as a family.

The girls absolutely loved going to the Zoo and it was wonderful to finally get a chance to meet up with one of my dearest and closest friends... (Love you Lizzie and miss you heaps!!!) and the girls loved going to Wonderland...

It was also great, although somewhat confusing, to be able to talk to Sue and Bronwyn again.

Though this meeting has now filled me with a lot of questions and a lot more confusion about the state of my daughter's health.

It seems to be the pattern that our lives are falling into now. Every time we get some answers, something else somewhere changes and we become even more confused and frustrated with the lack of answers or information that seems to be forthcoming.

Now, after all of that... I want to end this entry with a totally heartwarming note. Tuesday morning my brother arrived from North Queensland to help us with moving. He is very much a manly man type of guy... and has always been what I think is really a little bit afraid of Nicola because she's always been so fragile...

This morning he was playing with her and watching her respond and interact with him was just incredible... but the heart melting moment???

When my brother turned around and signed 'Good Girl' in Makaton to her.

I <3 my family, and despite everything, today I just feel blessed.

A Moment of Sadness.

Ok... so good intentions aside... I really struggle to find time to sit down and write stuff here... and when I do finally get all the girls into bed and medications and feeds done and everything under control, I have hundreds of ideas of things that I want to write, but I struggle to find the words to write everything down.

Tonight I need to write something because I have so many thoughts rushing through my head that they're starting to jumble and crash... so it's time to put some out in the open.

So... anyway... here goes.

On Friday night Nicola got rushed into emergency theatre to have a shunt revision done. Nicola has a Programmable VP shunt inside her head. In laymans terms there is a catheter that goes into her brain and drains spinal fluid into a reservoir. The reservoir then drains fluid down through a valve into the cavity around her gut which is then reabsorbed by her body. The idea is that it is supposed to remove the excess fluid from around her brain and relieve the pressure around her brain.

So anyway, Thursday night we noticed that the site around her shunt on the back of her head was looking a bit squidgy... Friday morning we phoned the neurosurgeons to ask for advice... Friday afternoon she had a shunt series done to check the status of it and Friday night she was in theatre.

It was a bit overwhelming... but then again I am starting to get a bit used to this. My husband found it funny... every time anyone asked him about our plans for the weekend he responded with some quip about just popping up to the hospital for a spot of last minute neurosurgery... like it's no big deal or anything... you know, we may as well have just popped down to the local shops for how blase he was being about it all.

But this is our life, and we are adapting quickly. We are resilient, we have to be for our other children.

Anyway... this is where the real point of my post tonight starts.

Saturday night I get around to checking my email and find an email from one of the other Costello Syndrome Support Group Mothers, letting people know that her son had passed away.

He was 27 years old. He wasn't unwell, he wasn't in hospital, he hadn't had surgery... he just passed away. They think it was his heart.

It was a simple email, just one line... but it shattered my heart.

This hasn't been a good year for the Costello community. Willa has terminal cancer... she's only 2. Joanne passed away from complications from routine surgery... she was one of my dearest friends and she left behind two beautiful little children (one with very special needs) and a loving husband. Now with the death of Bret...

I feel like every other email that comes over the support group list leaves me in tears... the sad thing is, that no one in my real life understands why or how I can get upset over people who are virtually strangers.

To me, these people aren't strangers. Some of these people I have never met, some of these people I have met once... but still they are part of my inner circle... my nearest and dearest.

No matter where I look in my real life, there is no one that understands what we are going through on a daily basis. They don't understand the constant fear, the constant admsisions, the health problems, the medical complications... they don't understand the pain and the torment that becomes part of our daily lives...

We recently attended a support group meeting that was organised by one of the local disability services groups for families dealing with rare conditions. There were 4 families there... and out of all of them, no one understood what kind of life we were living. They haven't had the ongoing admissions or medical complications that seem to plague our every day lives.

And then there is my Costello Family.

While they don't necessarily deal with every single thing that we deal with, as a general rule, they understand. They get the babies that cry almost constantly for no reason... they get the feeding problems and the failure to thrive and the metabolic and endocrin problems and the gastro problems and the heart problems, the fear of cancer, and the combination of all of the above...

And they understand the isolation of dealing with a condition that no one else has heard of or understands.

We went to a cardio review last week and there was a new receptionist. While we were waiting to be checked in I glanced over the pamphlets sitting there and saw one for autism and one for downs syndrome, both talking about support groups and what help is available.

No one has heard of Costello Syndrome.

But my Costello Family understands, because they are living the same life that we are living. They are my light, they are my rock... they are my foundations. Without their support, I really don't know how I could get through every week.

That's why I get so upset when bad things happen to my other family. That's why I cry at the heartache of someone who is a virtual stranger... because even though they are virtual strangers, they are my family, and I love them all deeply... even the ones that I disagree with!

But beyond that, there is also the fact that it makes me question a lot of things that I try and push to the back of my mind... like the fact that there but for the grace of God go I.

And it sounds sooooo shallow and self absorbed to say that, especially in light of everything that has happened... but regardless, I feel it.

It could just as easily be my daughter, my family... and that thought tears me apart. I hate even thinking about it, but I can't help but think about it when the reminders are constantly there. One bad news email after another, one complication after another... every day there are reminders that our lives are just totally not 'normal' and that's ok... but every three months comes the slap in the face that reminds us just how bad it could be.

But at the end of the day, when I still have my daughter here, no matter how bad a day I might be having, what right do I have to complain when there are clearly others who are so much worse than I?

At least tonight I can still go and kiss my children goodnight...

A Little Lightheartedness

We all have to start somewhere, right?

So I figured I would start this out with a little bit of a light hearted post... so, here are a few random little facts for you.

Did you know that we have:

* 1:576,000 chance of being struck by lightening
* 1:7000 chance of being considered possessed by Satan
* 1:88,000 chance of dating a supermodel
* 1:117 chance of being on a plane with a drunken pilot
* 1:60,000 chance of striking it rich on Antiques Roadshow
* 1:20,000,000 chance of being named a Saint
* 1:3,000,000 chance of spotting a UFO
* 1:10,000,000 chance of becoming president of the USA
* 1:215 chance of dating a millionaire
* 1:220 chance of writing a New York Times Best Seller
* 1:3,800,000 chance of winning First Division Lotto
* 1:28,000,000 chance of having a baby diangosed with Costello Syndrome.

“Success is not measured by what you accomplish, but by the opposition you have encountered, and the courage with which you have maintained the struggle against overwhelming odds.”

<3

Another Surgery is Done!

Woohoo! Another surgery that has been straight forward! Nicola had her T's and A's removed on Monday morning. The surgery was simple, but the surgeons said it was necessary. They were stunned by the size of her adenoids and her tonsils! So hopefully this will make a huge improvement to her sleeping and in turn her general mood!

She went into theatre around 8.45am and was out by 10am and only spent a few hours in HDU for observation before being transferred to the ward. One night in hospital and then we were discharged to the Lodge for recovery.

It's nice not being in hospital and having the freedom to come and go as we please and do what we want, but it's a pain in the butt being stuck here for the next two weeks just sitting around twiddling our thumbs!

The downside is that before she was discharged we got to see the rehabilititation specialists who looked at her muscle contractures on the right hand side of her body and they believe she has a hemi-dystonia that is most likely neurological in origin.

I soooooo look forward to the day when I can get to actually cross ONE specialist off her list instead of adding more and more!

At least we can go shopping! We're planning a shopping trip on Friday... a chance to scope out some bargains and catch up with some old friends and some new friends... but mostly just a couple of hours of normalcy which I am REALLY looking forward to!

Random Ramblings

Going to the conference in the US was a god send for us, in so many ways. It gave us access to vital information that, now that we are back home and again lost in the depths of a thoroughly useless medical system, has proved to be a very very useful weapon to help get quality care for Nicola.

Unfortunately, the issues we deal with on a daily basis seem to be beyond the scope of anything our doctors have seen or dealt with… and because of this, we are often finding ourselves stuck in the ‘too hard to handle’ category and sent on our way, with a vague affirmation to return should the symptoms persist or should she deteriorate any further.

Today was yet another classic example of this.

For the past couple of weeks Nicola has slowly deteriorated into a pattern of excessive sleepiness. To the point that now, she will happily sleep for anything up to 20 hours a day. If she is forced to be awake, she will cry without consolation until finally she is back in her bed asleep again.

We spoke to our surgical consultant today who assured me that the problem is most definitely not a post surgical complication and in his expert opinion, it is another problem caused by the Chiari 1 Malformation that we know she has. He also advised that he felt it was best that we present to the local emergency department immediately for consultation with our paediatric team.

Before you read this next part, you need to understand how our medical system here works. We are at the state’s major children’s hospital, which is a teaching hospital. So the Consultant is the senior doctor. He is the one that all other paediatric doctors in the team defer to. Below him is the fellow. She is the one that has done all of her rotations and has an understanding of most of the workings of every department and has rotated back into general paediatrics to complete her specialty. Then below the Fellow is the Registrar. They are a semi qualified doctor able to make some basic day to day decisions, but still well and truly under qualified for anything even remotely unusual or different (and really, what part of ANY Costello Child is usual or mainstream?)… and then we have the Resident, fresh out of medical school, teaming with that whole megalomaniac ‘I am a doctor so I am elite’ sociopathy.

So dutifully I phone up our paediatricians. Our paediatric consultant is only in the hospital for 9 hours a week. Yes, that’s correct, it’s not a typo. 9. Nine. One more than eight, but one less than ten. Nine.

I end up with the Fellow who is rushing out the door on her way to an all important gathering (the Melbourne Cup is on today!) and so she just tells us to present to the emergency department for immediate assessment.

So we bundle the children into the car and traipse into the emergency department, and have to sit there and tell Nicola’s life story to the triage nurse. We finally get to see a doctor, and again, I have to tell Nicola’s entire life story, only to be told that they can’t find her medical records. They have somehow been lost between the outpatients clinic yesterday and the medical records department across the hall. But, never mind, they will just call our regular paediatric team in to assess her, after all, they know her best, right?

HAH!!

They bring in the registrar, who we have never met before today… So for the third time in as many hours, I find myself getting increasingly frustrated as I once again tell Nicola’s entire life story… only to be met with a blank stare… and then finally the resident, who looks like she is all of ten years old, pipes up and says “So what exactly are your concerns? Is it just that she’s sleeping and a bit cranky? Isn’t that what babies do?”

Seriously, this is about the point where one starts fantasizing about jumping up and just going completely postal. Visions of scenes from the movie John Q start flashing before one’s eyes…

So once again, I explain my concerns about her extreme irritability and excessive sleepiness… I explain to them that it is very much out of character for Nicola, and I am concerned about it. I have spoken to the senior surgical consultant and he has advised me that he believes that it’s neurological, stemming from her Chiari, and we need her to be assessed by the neurosurgeons that have been assigned to her.

Well, anyone would have thought I had asked them to go tap dancing on the moon given their reactions! The registrar tells me that that’s not how things are done and decides that she will have to do a complete assessment on Nicola.

So the first thing she does is get Nicola to lay down on the bed so she can have a look at her… then she turns to me and says “Are you sure she has Costello Syndrome?”

I explain that we’ve had the gene test and that it was confirmed and it was a conclusive test.

And she says “Are you sure? Only, she doesn’t have all the classic facial features?”

I felt like saying “No, I just feel like saying it, day after day, week after week, month after freakin month!”

She gets on with the assessment and asks a hundred questions and gets two hundred answers… and then she stands back and says “well, I think that’s it…”

“And the verdict is?”

“Oh, I don’t know, I’ll have to get the neurosurgeons to come and assess her… it’s most likely neurological.”

Well, you don’t’ say!!!

Long story short… 7 hours later, we finally leave DEM… we still haven’t seen the Neurosurgeons, and we’re still no closer to an answer. We have a vague assurance that some point in the future they will contact us to schedule another MRI to be done at some other point in the future, which will be reviewed by the neurosurgeons at… you guessed it… yet another point in the future!

Oh what I wouldn’t give for a crystal ball right about now so I could predict the future!

But all along, she kept questioning whether or not Nicola actually really has her diagnosis correct… after all, she doesn’t seem to fit neatly into the little box that someone somewhere has created just for people with Costello Syndrome.

Nicola doesn’t have an oral aversion. She loves foods, she loves tastes, she loves having things in her mouth and like most babies, everything that makes it into her hand goes straight into her mouth. Now that she has teeth she is getting very good at biting and chewing and can make a right mess of pieces of meat and biscuits and rusks etc, and she can devour a piece of chocolate like a monster in a frenzy… but she cannot swallow! She has the same issue when she tries to bottle feed. We know she has a weak and somewhat uncoordinated suck, but with a haberman feeder she used to do very very well and was on full oral feeds.

Apparently that is not normal for a Costello Child.

Nicola has Chiari 1 Malformation, which is allegedly supposed to be an asymptomatic adult condition… not a condition found in a baby of 14 months, and apparently she is ‘symptomatic’ of Chiari 2 instead… which, of course, as we all know, is totally impossible, because Chiari 1 never has the same symptoms as Chiari 2, especially not in the world of medical, tick the box and make it all fit, perfection. And of course, it’s entirely possible that Nicola doesn’t really have Chiari at all because Costello children “often” have enlarged ventricles and droopy brains just because! After all, how do they know that a “normal” Costello brain looks like?

So now, instead of having a medical team who ignore us and our needs… we have a consultant who is never available, and a registrar who thinks she knows everything because she’s read a few pages on the internet this morning.

But of course, none of this is really relevant anyway, because after all, it’s all in my head. We have an ideal medical system that never has any issues dealing with people, so I’m just a whinging, complaining, psychotic, neurotic mother.

Oh, and I’m also a bitch… but that’s a whole other story!

In love and madness!!!