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!!!

The Sweetest Sound.

Today my precious little girl is 13 months old and we are now 4 weeks into this hospital admission with no end in sight.

Any parent who has been in the position we find ourselves in every day knows the pain and frustration of having a child in hospital. The pain and frustration of being in such an alien environment… and I think that being a mother makes the frustration about a hundred fold worse.

From conception, as her mother, it is my job to care for her… to nurture and nourish her… to care for her and to love her… and when that role is taken away from you, there is such a sense of loss. All of a sudden, a role that is as natural to us as it is primal and instinctive becomes ambiguous.

Things that were once so natural, are now a charted and documented process and gone is the freedom of being able to make decisions that you feel best meet her needs… and the care of someone so innocent and precious is being entrusted into the hands of a stranger, who changes every 12th hour.

This admission has been even more of a rollercoaster for us than most. There have been so many things that have gone wrong in the past 4 weeks, with straight forward surgery going horribly wrong. In the past 4 weeks, she has been into theatre 6 times, had two collapsed lungs, spent 16 days ventilated, 1 day on BiPAP, 3 days on CPAP, 8 days on supplemental oxygen, 2 blood transfusions, staph, strep, entracoccus, and pneumonia.

With everything that Nicola has been through in her short little life, I have never in my life been more afraid that I was going to lose her as I have been through the early weeks of this admission. Seeing her wheeled back from theatre with her little body painted in orange and her eyes taped closed, ventilated and bagged, with so many machines connected is a sight that will haunt me for many years to come.

Even now, I still wake in the middle of the night with the alarm of the ventilator sounding with an apnoea alert or a frequency alarm and the sight of the x-rays showing her lungs collapsed and her little chest full of infectious gunk is still something that I can clearly visualise, and probably always will.

But, as with everything else, she has pulled through.

Every step of the way she has proved again and again that she is stronger than they realise. Every time I fight for her to be given a chance, she proves to the doctors that she is capable of miracles. She has smiled through immeasurable pain, and tolerated indignities that no little baby should ever have to endure. She has been suctioned and sutured and swabbed… poked and prodded and pricked… and generally just tormented to tears on a daily basis.

But today… today she has proved yet again just how truly amazing she really is.

This afternoon she suffered through the indignity of a sponge bath on her bed, when all she really wanted was a shower. I dressed her in her little jammies, when all she really wanted was to stay nuddie. I rubbed moisturiser into her arms and legs and gave her a bit of a massage, which, come to think of it, she really didn’t complain about much at all… then, after all of that, I leaned in and smothered her with kisses…

And that’s when it happened…

I heard it…

The sweetest sound ever to be heard by anyone anywhere…

Despite all her pain and suffering…

In the face of so much adversity and against all the odds…

My precious little girl laughed, for the very first time.

Anger Abound...

Nicola has spent the largest part of her life as an inpatient at the hospital. It has been something that my family has struggled with from the onset.

We moved to Brisbane while I was pregnant with the intention of my husband going back to his former employer, which was a good, family friendly work place... a must given that we were about to have our third child in under as many years.

He lost his job in February this year after having too much time off work to care for our older children while I've been in the hospital with Nicola's repeated admissions...

But trying to make the medical staff understand the impact that this has on our lives has been nigh on impossible, to the point that we've just given up and put all our energy into the fight for her health care, a battle that was constantly being waged anyway.

After we were given her diagnosis, at our first paediatric consultation several weeks later, we had an extended meeting with the new paediatric consultant, as our original consultant was on maternity leave.

This was shortly after I wrote an extremely lengthy email to several high up persons to express my concern and unhappiness at the quality of the care she has received.

When we started this consultation, my husband and I sat down and took our time to explain our concerns... how we felt that our last paediatrician didn't listen to us and that our concerns were never considered, and that Nicola's care had been so far substandard that we didn't consider it to rate on the scale at all... and also the fact that they had REALLY dropped the ball with her diagnosis... that we were so angry and hurt by the way we had been informed of her condition and the fact that we were left on our own with no information, no support and nothing...

Of course, then and there, she said all the right things and made all the right gestures and promises...

Two weeks later she refused to sign the paperwork for us to travel. She told us that the risk of 'contracting seasonal respiratory viruses' was too high so she would not sign our medical clearance.

We dealt with that, we sorted that out... then today we've got a copy of Nicola's medical file to take to the conference with us.

Right in the front is a letter that this 'woman' has written and cc'd to every other department involved in Nicola's care as well as the local GP that *I* see and in it, amongst other anger intensifying comments, she states that we are 'hostile' and that our anger at the botched care our daughter has received is simply a 'severe grief reaction misdirected at medical and nursing staff'.

How the hell can she say that our anger over their screw up's is misdirected grief?

We've been angry since before the diagnosis, this is just the first time we've actually been able to get someone to listen to our concerns!

Gah!

I've sent an email tonight to the director of the hospital asking to have a new paediatric consultant assigned to Nicola's care. I need a doctor that I can trust dealing with my daughter, not a self righteous megalomaniac with a holier than thou superiority complex!

But still... soooooo angry I could cry!