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!!!
