Ramping It Up: Children w disabilities, opinions, books

Being a test case: Paediatricians, hospitals, and a Dysmorphic Appearance

Early last week, in an oddly calm moment, I went for a walk with Miz M to a nearby cafe, and counted up this year's hospital visits from my diary. Fifty one days over six admissions, including eleven days in Intensive Care. I didn't bother counting up the outpatient appointments, the visits to pick up equipment, the therapy appointments. Neither did I count the number of books I'd read at the bedside, the number of days of work I'd missed, the amount of guilt I'd dished up for myself, the number of nurses I'd spoken to, the posses of doctors I'd met, the emails I'd sent to friend. The number of times Clancy had wondered where I was, and where his sister had disappeared to. The litres of oxygen she'd been given.

The number of months that might be left in her little life.

Some things, even with a little red Moleskine diary, can't be easily quantified.

But I did the adding up the day after she last came out of hospital. Another twelve days in, including five days in Intensive Care. This, only two weeks after the last almost identical admission.

And then, being a sucker for punishment, five days later I was back there, voluntarily, spending a morning with Miz M being an examination subject for paediatric registrars who are on their way to being consultants. I knew the drill. For the last six months, whenever we've been on a ward, doctors pop in to ask if they can use her as a test case, for their practice. They scour admissions, looking for "interesting" cases. Oh, to be boring. What they want is the not easily categorised, the tricky, the multiple disabilities, the chronic and acute, the odd ones out. I've done many a "long case", which means I talk talk talk about the pregnancy the birth the early days; the signs something was "not quite right"; the tests, the lack of a diagnosis, the speculative diagnosis, the clinical presentations, the other tests, the waiting for results, the next tests, the referrals the therapy the eventual maybe that's a diagnosis. Then, after you're parched from talking and each and every one has said, "oh, do you have a medical background" (no, bugger off, I just pay attention in class), then they finally examine my girl. They then go off to write it up and present her to a panel. The good ones ask you new questions; the good ones give you something to think about. It was in this context I was first asked if we'd thought through our decisions on resuscitation. It's in this context they ask you about depression and your relationship and what next. 

Somewhere, I imagine a group of examiners rolling their eyes. Here's Morgaine again. Haven't we already heard her story 8, ten, twelve times already?

And then there are the "short cases", in which the parent or carer is invisible - no context here, no storytelling, no asking questions - and it's "only" about examining the child. She usually lifts up her hand in her languid, soft way, and rests it on their hand or their stethoscope. Gives them a little pat. Giggles when they try to get her to follow a toy with her eyes.

And I figure it's a pay-off. It's a public hospital, they treat us very well, why not do what you can?

And I figure it's strategic. This way, they know her. This way, we might learn something.

So when they asked if we'd do a "real" exam with her, we said yes. When I say we, Abe rolled his eyes at the idea of another morning in the hospital, so I said I'd go.

And there we all were. Yet another community you never thought you'd be a part of. A group of people who may very well look Peculiar with a capital P to outsiders. Children with rare and unusual conditions, looking "different", requiring all sorts of aids and equipment, sitting around on chairs that are too small on a Saturday morning. I meet yet another woman who's on her own, and has had to leave her job, and who's coping with a child who has rages that last for an hour and a half. Who has what they call "challenging" behaviour. I meet a very poised girl of about twelve or thirteen, who seems relaxed about being examined and poked and prodded and asked questions about herself. A small boy who looks partly melted - not from burns, but rather as if while growing something simply slipped. He's a wizz on the computer, and uses makaton signs to signal his pleasure to everyone. Miz M just smiles happily.

We're on for a short case and, after a month or two in which we'd had our meeting and made our decisions about resuscitation etc; in a week in which we'd met with the non-cancer Palliative Care team, and acknowledged that what she has is a "Life Limiting Condition" (a new phrase for me, and oddly precise - not terminal, not in decline, but definitely "Life Limiting" given we don't know when the ultimate crisis will hit); in a time like that you think nothing else can be confronting. What's to think about?

But of course, a group of new doctors will examine her. Clinically. Of course. And I will keep it zipped, apart from saying, "she can roll" when I put her on the bed (oy, so don't let her fall of, right!); while they will hold forth and give their pronouncements. 

Here is a three year old girl, they say, who cannot walk. Examine her legs, from a neurological perspective, and give your findings.

Gawd: those poor people were so very very very nervous. Bells rang. There were masses of junior doctors around the place to help (and I recognised about 90% of them from the Emergency Dept and various ward rounds), and we had a Group of Three examiners, who put these doctors through their paces. As well as the usual range of tests and reflexes (none!), they all talked about her Dysmorphic Appearance. And here we have the confronting bit. Our usual doctors had been a bit shy about this. The neurologist suggested it; our regular paediatrician poo-poohed the idea. But there were four new doctors, and three examiners, all commenting on her "dysmorphic appearance".

Am I being too technical here? It means Funny Looking.

Apparently, when children are first born, doctors and nurses used to sometimes write "FLK" on the charts. It meant, Funny Looking Kid - because some conditions, especially chromosomal abnormalities, really do have distinct looks about them. (The most well-known, of course, Down Syndrome.) But just in case, they have a quick look at the parents, and sometimes they'd write "FLK/ KLM": Funny Looking Mother. Look no further. I gather this practice is now frowned upon. Hence, the Dysmorphic Appearance.

So. Her large head; the shape of her large beautiful blue eyes; the position of her ears; the space between her eyes. Another also suggested that her face wasn't as mobile as it might be - certainly, she smiles sweetly, but it is true she doesn't express the same range of emotions as her little brother, who has the toddler's tendency to Emote like Laurence Olivier at every turn. So every doctor had something to say about her look - although they also had a range of suggestions for what her condition might be and how you'd test for it and what you'd do next. As well as the hypotonic CP, they all suggested genetic caryotyping, which is only just happening for us now (no results yet), so clearly there are a few chromosomal disorders they have in mind. And only one who I think was way off the mark.

Interestingly, the last woman was the most nervous - which is saying something. You could have run a small car off the energy being generated in the department that morning. She was also the most dithery in her manner and presentation. And this is where I don't know whether my PC-antenna had been too finely tuned, but I got a distinct sense of impatience from one of the examiners, as soon as this woman walked in the room. Immediately. Was it the headscarf, the muslim attire, the long robes? I hope I was being overly sensitive.

And so I took my beautiful little FLK back home.

A Flea in a Fit: Seizures and what the hell they might mean

OK, well my lovely girl is still in hospital. Almost 5 days in intensive care; moved to an overflow public bed in a private ward; and now finally in a public bed in a public ward - so I can relax now. Her "sats" fell last night, so they had to bump the oxygen back up again, and then this afternoon they were pulled back again. There's nothing like a hospital to insitutionalise a person. Sats and desats are of course oxygen saturation levels, and I can now ask about blood gas reads, remind them that the remaining lines will flush but they can't get bloods from them, ask about the sodium levels, see what's happening with CO2, ask about the pressure spot on the back of her head, wonder what the cultures found on the intra-osseous sites, and help with the suctioning. Although to be honest my stomach still turns over with the sound of the suctioning.

Walk the walk; talk the talk; and rubberneck my way into reading the full chart whenever they leave it "unguarded" for a moment. I know it's all about privacy and legality but bugger that, I want to go cross-eyed over all their acronyms.

So, it's Adenovirus, which has lead to pneumonia, which lead to her difficulty breathing, which lead to low sodium and elevated CO2 and high temps, all of which may or may not have lead to her seizures. The rest of us have mild versions of the same virus: I have a charmingly red left eye, and that's it; Abe looks as though he's been hit by a truck and is exhausted; and Clancy is just like usual only with very red eyes and an occasional cough. So given his high energy, we're talking energizer-bunny-with-myxomytosis. Charming, hey.

And it's easy enough to be lighthearted about it at 10.30 at night, after winding down and returning from a very long day by her bedside. Not so easy when she's not breathing properly; not so easy when you have to keep retelling her little story and spelling out her life challenges - to doctors, nurses, registrars, physios, speech pathologists and the rest.

Not so easy when I keep wondering and worrying about those two seizures I saw. What have they done? What do they mean? Have they damaged her brain - even more? Were they because of what was going on that morning, or because of her underlying condition? It also makes me realise that for all my supposed care with language, where I know that the old sticks and stones axiom is bullshit, and I've never ever said "that's so gay" (partly because I'm too old; but also because it's homophobic bullshit); and I've never said and will never say "that's retarded" because even before my girl came along I objected, your honour, to that phrase. And yet, I seem to say, "he's like a flea in a fit", to describe Clancy's way of bouncing cheerfully off the walls; will say I had a fit about something, when I don't mean that at all and KAPOW just like that, with a closely observed set of twitches in a plump little hand, that travelled up her arm and then over to the other side, just like that as I was so utterly and completely scared that even thinking about it now my stomach is in a knot and there are tears in my eyes, just like that these phrases glow now. They are surrounded by electric lights like those around the make-up mirror of a starlet in a backstage dressing room.

And just like that, KAPOW, I'm confronted yet again by another set of presumptions, by the way resilience and optimism and hope are all configured. Because since my girl was just a babe, and as soon as we knew "something was wrong", I've been loudly and often telling people that it's all ok, it's ok that she won't walk, that she'll be dependant upon us for ever, that she has such high level needs

because

at least

she can communicate, can smile, can engage with us.

And now I'm forced to realise that that's a bullshit reason to give. I love her anyway and always will. Because what if she CAN'T smile back at us, giggle with utter pleasure in such a winning way?

She hasn't smiled at us, or anyone, for a whole week. Even before we took her in to emergency in the middle of the night, she'd had a day where she was sick and not connecting, then she was sedated, then when she came around she was "awake" and not really seeing us. Not connecting. Certainly not smiling. So I've been forced to think about what it might mean if something has irrevocably shifted.

This afternoon, she was starting to engage even more. I got half a smile. She's probably just wiped out and sore and sick; she might be feeling odd from the anti-seizure medication, phenotoin; she probably wonders why she feels like a pincushion (I watched six doctors not manage to get a line in to her this week, including 3 who used ultrasound to try to find a vein); she might be wondering why the hell we didn't act sooner.

So, yes, I do hope she returns to being herself. And I"ll never think about those damn fleas in the same way again.

Periventricular Leukomalacia (PVL), or, The Trouble with Diagnoses

It started to rain last Tuesday. And when I say rain, I mean cats, dogs, dingoes, sabre-tooth tigers and various types of cat-shaped deities beloved of the pharaohs type of rain. Bucketing, pouring, torrenting, flooding. Or, in the Oz vernacular: It was faiiir-lee PISSing down. Gutters were gutted; creeks overflowed; roads were cut; football fields becamae lakes. And overnight, it sounded pretty wonderful on the roof, but seemed to freak young Clancy out a bit, so he had his first ever case of "night terrors", and woke every hour - or didn't wake, but cried and shrieked and thrashed about.

And so we get to Wednesday morning. "Eyes like piss-holes in the snow", as my little sister used to say. Abe had various high-level work meetings, of the crisis variety, so I was going off to the Children's Hospital on my own, Morgaine and Clancy in tow, to finally get the results of her MRI. It was raining to hard I could hardly see. Luckily, I've been to the hospital enough that I know which parking place to park in, so that although I have to walk through many buildings and labyrinthine corridoors, I can do it all while staying undercover. But did I say, fast? I hate being even a minute late for an appointment. Of course, I was on time and then had an hour to wait.

I met a woman, who was there with her mother - both in gorgeous saris with cardigans over the top, that classic Indian look that combines style and functionality - and her little girl in the zippiest zappiest pink manual wheelchair you've ever seen. The little girl had spina bifida, and it was a relief to meet such a positive little family. I've met a few other families with SB in the same waiting room, who have been unusually grim and depressing and a teeny bit peculiar. That was just them, obviously, nothing to do with the condition! And Clancy was remarkably patient.

So anyway, we finally got to see the neurologist. He had a young medical student with him, from Oxford, which meant he explained M's condition rather well and in great detail, and made it clear that yes, they have been a bit stumped, and yes it has been a bit of a mystery, and that's why we've done so many damn tests (merosin-deficient muscular dystrophy, Spinal Muscular Atrophy, etc etc all ruled out).

Then he took me through the MRI scans themselves, one by one. See there, he said, that's a dark spot on the brain stem. And yes, she does have enlarged ventricles. But the main thing, is the level of myelination. Myelin is the white stuff in the brain, which coats the nerves (I think that's how you'd explain it), and is about both making the central nervous system work, but is also about complex thinking and brain development. Her level of myelination is way way behind what it should be. (I think you call this hypomyelination.) It's at about the level of a 7-9 month old. There was also evidence of leukomalacia, he said.

My mind was starting to move very fast by about this point in the appointment. The medical terms were flying, which I don't really have a problem with, except interrupting occasionally to check how they're spelt, as I make notes in my diary.

And this is the point where, as an active and informed Medical Consumer, we're supposed to ask questions.

But as the neurologist looked at me expectantly, awaiting a question or two, it occurred to me that the real question I wanted to ask was: what form of speech is that? Is Leukomalacia a noun or a verb? Is it a process or a condition? And it's a little hard to frame that question. Because he really was speaking another language - and I don't just mean the latin that I didn't learn at school (Dapto High? I don't think so.)

So . . . it's all still a bit vague then?

No, he said, it's surprisingly clear I think. I didn't expect to see it at this MRI, but it seems clear that she does have Periventricular Leukomalacia.

Which means . . . ? And how . . .?

Which means brain damage that happened in utero, for reasons we don't know. (He suggested maybe from a faulty placenta. Or it could've been a virus or something. Mostly it turns out to occur with premie babies, which she wasn't.) And PVL (oh look, another acronym) falls broadly into the category of Cerebral Palsy - although hers is rare and of the hypotonic variety, and from what i've read it's in the severe end of that spectrum too - and the neurologist hinted that maybe that was something to think about rather quietly. A hint I didn't get because I'm an idiot. (In other words, think about it for a while and just shut up about it.)

Trying to absorb it all later (after the drama of driving home through heavy heavy rain, wrestling children out of the car and into the house, getting mobility device out of the boot, me getting soaked etc etc; discovering that not long after the roads all round the hospital were underwater and cut off and all that), I couldn't quite work out why I was so thrown and why I was so upset. Major disability? Nothing new there. Intellectual disability? On all rational levels already knew that. An easy label? Why does that feel so hard?

So in the spirit of transparency and blurting it all out, I immediately emailed all the relevant therapists at The Therapy Place, to say it was PVL. Then I saw the rather fabulous physio the next day, and that's when the regret started to kick in. Rather gently, she had to tell me, that this means M can't stay with the Therapy Place. Because it's CP, we'll have to move to a specialist CP organisation.

I'm devastated. Even though the rational part of me knows I shouldn't be. Even though Miz Phyz explained that at least this shows it's not degenerative; yes the neurologist said progress would be slow, but at least there'll be some progress; and with hypotonic CP, we should even meet some other kids who are more like her, rather than M being the one in the room always able to do the least, move the least, communicate the least. But the thought of having to start again, learn again, get the protocols and systems and advocacy and personalities and timings and resources and knowledge and politics again again again lead me to, yes, shed quite a few tears over poor Abe. Besides, I really like these people from the Therapy Place, I trust them.

So I let loose and gave myself a few hours on Thursday night to fall apart a bit; before resolutely heaping it all back in again.

But then I wondered, is there something else dodgy going on here?

While it's a given that there's something useful about a diagnosis, what does it mean that I've resisted it? Perhaps it's the idea of placing my girl in a box, a category, a carry-all: after all, CP doesn't hold any easy answers. Besides, there are many people with CP with no intellectual impairment at all, and comparing them to M won't help. I immediately remember a young woman I taught at uni years ago, who described the way her dad called her "twinkle toes", and it made her think affectionately of her twisted feet. Or maybe it's the thought of limiting M by seeing the category as too easy, boxing her in while ticking that box.

And Myff said, but maybe it's good to know that her developmental stage is at 7-9 months, rather than thinking that it's not, and she's trapped in there. That was a clever response, a thoughtful one, the horror of being too too aware of everything but unable to get those ideas or ambitions out. Like The Childhood Story of Christy Brown, which I read years ago for its verve and its energy and its sheer Irishness. His mother has sort of inspired me in this, her indomitable belief in keeping on trying, in believing in her kid, in trying no matter what. And I'm not about to stop, just because we've been able to look inside her brain to trace the white bits. (Although black humour has come back to bite me. The Childhood Story of Christy Brown was made into the film My Left Foot, where Daniel Day Lewis was renowned for his method acting. What my sister and I used to call the "strap 'em in a wheelchair and throw porridge at them" school of acting. Oh hah-de-fucking-ha-ha.)

Or is it all dodgier than that? Did I not want a diagnosis because something rare, unknown, mysterious, made us "special"? We watched Revolutionary Road on DVD the other night, a terrific and clever and deeply stressful film, about the horrors and mysogyny of 1950s suburbia, the "lives of quiet desparation" . . . and the delusion that they were really terribly special. Not like everyone else. Is that what we were doing, too?

Or rather, was that me?

Something unusual, something myserious, something that might be resolved like a Saturday afternoon movie matinee (throw off the lap rug, get to her feet, and say, Look, Ma, I can walk!); or go from observing, listening, being read to, participating in all of Clancy's lessons until the day she grabs his HB pencil and writes, "Hey, it's me, I'm in here"?

I hope not: that'd be me being vain and silly and unrealistic. But I think there's something in it. The mystery and un-knowing gave me space for extra hope and possibility. So I have to work out how to remake this label and category, to stretch it, to make sure that it also keeps that same space for optimism. I know I can do that.

Besides, it's stopped raining.

Feeling Annoyingly Tired

So, what's a girl to do? Try to write herself out of feeling as though she's been run over by a truck, that's what. And there's no excuse: Clancy's MCAD has now reached the stage where he can go 7 hours without being fed, and he charmingly woke up with a squeek with only 4 minutes to go on the alarm, so I slept for 7 hours. Other parents of 7 month-olds should be shrieking at me right about now.

And as this is my one and only chance of the week to do something productive, I really can't afford to piss about. I need to think myself into analytic and self-critical mode, with a touch of bravado, as I have to put together a proper "skills-based CV" in order to see what other options there might be for me in the organisation when I return to work. Perhaps I should call it The Organisation with capital letters and make it sound both ominous and Soviet at the same time? No, perhaps not. There are also still applications for funding to be completed, to deal with the endless round of Morgaine-shaped equipment she needs. Still, she does seem to enjoy being in her stander. (Although various people I know - especially those I'm related to - keep wanting to call it a Walker, and I have to shatter their illusions and say no, she won't be walking. Really. I'm not making it up or being a drama queen here.)

Abe and I went off to the Mt Coot-tha Botanic Gardens yesterday, with Clancy and Morgaine of course, and walked serpentine routes through various lovely landscapes. One minute in a rainforest section, then turn a corner and go up a steep bit, and there are mountainous cold-weather trees. Lush and green here; spiky and architectural there. And ducks, moor hens, ibises and sulphur-crested cockatoos everywhere. Clancy, for the first time, really saw the ducks. He didn't know whether to be thrilled or scared, but he did know he was both fascinated and ASTONISHED. At 7 months, the world is unfolding before his eyes - and it is, of course, his own personal set of origami paper (or does the unfolding really refer to one of those large scale maps, with pop-up bits and dragons off to the side?). This was funny and cute and endearing, as well as seeing the cognitive development right before your eyes.

And then I couldn't help but be a bit wistful, because M was oblivious to all of this. It's not that she's oblivious to the world. She sees and knows us, she's interested in things. But there's such a limit to what she engages with. I don't know how much this is merely physical, and how much developmental. In my darkest moments I worry that she might not even really know us, but that rather she's responding to people who are good to her, who are warm and affectionate and loving. Whoever they may be. And than and then and then. I don't want to underestimate her skills, her perception, her intelligence or her possibilities. Which is why I know I'll always fight for her; why I'll always do whatever I can; and why I'll feel guilty every day at not doing more.

But thinking of doing more, I heard an interview on Radio National breakfast last week, with a neurologist talking about the plasticity of the brain. Norman Doige, The Brain that Changed Itself. I'm often wary of writers like this, especially with the increasingly popular use of biologically determinant ways of thinking. And it's not just because of Morgaine that they irritate me: as a feminist, I've always thought that was a bit dodgy. (And, yes there are some serious implications for race in that critique too. ) But where I thought this guys was interesting, was that he talked about how the scientific understanding of the brain and its development has shifted fundamentally from when (eg) he was first trained; and he also allowed a role of culture and context (hallelujah!) and talked about a woman with disabilities who "trained" her brain to do things she couldn't previously do. So I reckon I should get that one, have a read, and see what I think. I've been selective in what I've read in this area, to minimise my frustration in not knowing what the hell is going on with M, except for the very general "some type of brain mis-development" angle. Although I do have a copy of neuroscientist Lise Eliot's What's Going on in There, which is interesting although not necessarily directly helpful.

That reminds me: we still haven't had any results from M's MRI scan, which was ages ago now. Add that to list: contact hospital, hustle, try to get appointment.

The hustle. Yep, that's it, am ready to hustle.