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.

Impossible Meetings With Doctors

On Monday we sat at a table in a conference room at the Children's Hospital opposite two extremely nice doctors, having a conversation about "end of life decisions" for our girl. About intubation, resuscitation, levels of intervention, and what it all means for quality of life; for being kind, for making sure she's not in pain or distress. This isn't because she's about to die, but because she could have died a week before this conversation, and a month before that, and a month earlier . . . and because it might happen next month, or maybe next year.

It looks rather bland when you write it down. As if it's straightforward.

It can even look cold, or uncaring, or too clinical.

Oh, hardly.

Two weeks ago today Miz M really wasn't well when she woke up, even though she'd been fine the night before. Laboured breathing; very tired; working hard. Luckily, our fabulous physio and speechy were coming over that morning for an appointment, which meant they took one look and said, hmmmmm, something is not right here. A respiratory rate of 60+ (about twice what it should be for a 3 year old). Meanwhile, the airconditioning had broken, so I was having conversations about pieces of machinery that were broken, while awaiting the young woman who comes to give us respite for 6 hours a week, so I could break the rules and ask her to mind Clancy while I took M into Emergency. Aaaaah, she had a gastro thing and couldn't make it.

So, off to Emergency with a sicker and sicker Morgaine, and a rambuctious 20 month old. Cue, temperature (39.5); cue, low SAT levels (82); cue, mucho oxygen. And thankfully, a rather nice young doctor who found a portable DVD player and the Wiggles for Mr Can't Sit Still.

And by now, the doctors all say -- but weren't you? But didn't I? And I say yep, we were in here a month ago, and two weeks before that, and a month before that.

Four major admissions since the beginning of the year.

And for some reason, we always end up in the same cubicle, right opposite the doctor's station, where once she's been whipped in to be seen to (no waiting around for us! Ever! nothing like a crisis response to let you avoid a drama filled waiting room) then I can watch them. The large, comforting doctor who always introduces himself and shakes your hand, and you'd follow him anywhere he seems so kind. The blond nurse with messy hair who just screams competence, is in five places at once, and acted so quickly the time before last when M had a seizure and suddenly lost oxygen in Emergency. She's also a paramedic, it turns out. The Baby Doctors, who veer between anxious and arrogant depending on (ahem) gender (who said that?).

I even read exactly the same magazine two admission in a row. There were weeks between admissions, but the same 6 year old magazine was there, boring me to sobs. Always always always have a novel, pen and paper, a spare pare of undies and a toothbrush. And a toddler, with plenty of snacks. Abe had a meeting he couldn't get out of a fortnight ago, but Clancy was pretty fabulous really.

Oh, and M also has hyponaetremia (spelling?) - low sodium, due to SIADH - so I now know to say, no, not just a chest X-Ray and NPA (snot test), please take venous blood asap. Really. And when you get the terminology right, and can remember the names of half the nurses, they tend to believe you. Although they have such a horrible time getting bloods, that I almost always watch 3, 4, 5 different doctors try before they get it done.

That night, after Abe and Clancy had gone home, and we were still in Emergency, M's breathing suddenly looked odd. Can you check this, I said to one of those eternally calm, unflappable, older nurses - who then told me she'd been worried and had just gone to get the head doctor to look at her. Respiratory Rate over 80 per minute. Not so good. So we end up on the ward, for two nights and a day.

It looks like aspiration pneumonia this time (as opposed to Human Meta Pneumo Virus and RSV and an unknown wacky virus the other times this year).

The first night, I sleep on the ward - after hearty greetings of "Oh, we know this child, and we know this mother!" Yep, been to this ward, before, thank you! Even been to this room. We get up there late, it's almost midnight, and there's one other woman and her child in there. Large and cheerful, introduces herself. The next day, slowly, we exchange stories. This one's a bit more Suburban Gothic than most: dreadful parents-in-law who've accused her of faking her child's illness (endless 5 minute long seizures, that'd take some coaching); break up with her husband; baptist-home-schooling stories; best friend whose husband was abusing the kids. Aaaaaaargh. Makes me feel shockingly middle class and happy. Mind you, she was quite nice; while I kept bumping into others and having bizarre conversations with people filed under P for Peculiar in Hospital Central Casting. Oi oi oi.

The next night, I go home. After so many weeks with our girl in hospital, poor Clancy is starting to notice. A mother who completely disappears? Not fair, really. And he seems fine, then gets a bit stroppy and overly emotional. So with a whole overcoat of guilt on, I head home. And was about to go back in the next morning, when the hospital calls to say, she's suddenly a lot worse. Oh, and they've put her up to 16 Litres of Oxygen (what? she was on 6 when I left) which is as much as they can give her on the ward. I go in, and the nurses won't leave her side. There's a flurry of doctors, of all varieties. Meanwhile, I had agreed to participate in a practice exam for one of the Registrars (I've done about four practice "long cases" this year, and any number of practice examinations - it kills time, and tends to be pretty interesting). So, we're going to transfer her to Intensive Care. Fine, I say, to the young doctor, I'll do the case with you as long as you don't mind that there's stuff going on at the same time. It's a way to slow my heartrate, but not, I suspect, his.

Six days in ICU. She was within a hair's breadth of intubation. They used BiPAP instead (where the machine breathes for you), then CPAP (a different type of pressure), before a transfer back to the ward for another - oh, I don't know - four days.

Thirteen days.

And last Friday, the neurologist came around, to say that he thought there was something else going on. Something besides the Hypotonic Cerebral Palsy, something that he can't quite get to the bottom of. I think he has a few ideas, but can't get him to really spell them out. It's because of the low sodium; it's because she's so very disabled. It's because she has no myelin at all in her brain. So he's ordered genetic karyotyping, and a "CGH Array", which is all about genome mapping and a possible chromosomal abnormality - of the rare and "interesting" variety. Demyelination. Leukodystrophies.

You know, he says, her prognosis is not very good.

Well, slowly, I had come to realise that. Maybe that's why I haven't been so good at writing here -- much as I really needed to.

Abe already knew. He knew before I did. And a few months ago we'd forced ourselves to confront that, and did it very badly at first. I began to work out how to ask the right questions, and found the research on Life Expectancy for children with CP when they have M's level of disability. Such limited mobility, being non verbal, peg feeding, little head control, having been born full term, tended to mean a 40 % chance of living to be ten, for example. But that's probably way outside what we can think of. Especially because there's something else gong on.

And poor Abe. My unthinking optimism made it very hard for him to say, look, her quality of life isn't very good. It silenced his despair. It didn't give him space to say, actually, this is pretty fucking heartbreaking. 

So I'd celebrate a smile - we both did - and a tiny tiny improvement, and he'd also be terribly sad about all the things she couldn't do. It took a really difficult conversation, months ago, to realise that our perspective on things needed to come closer together, or at least, needed to acknowledge each other better.

So I'd be worried about how vulnerable she'd be as a teenager or a young woman; and he'd tell me to live in the moment (which was fabulous), while really knowing that it wasn't something we were ever going to be dealing with.

And slowly, the question of how you deal with hospital and medical systems in a type of real crisis had come to the fore. A year ago, standing by a hospital bed while on holiday in Proserpine, a nurse asked us if we'd made decisions about resuscitation. It jolted me like the lightning that struck the character in Tracey Chevalier's latest book. We were completely unprepared then; not much better recently. What, exactly, does it mean, and in what circumstances?

So for the last month or two we'd started to talk about it. I thought Abe might know, as a medical person, but he wasn't sure how these decisions come about either, in light of a general duty of care and doctor's ethics, and how anybody can sensible respond to what you've decided. It's different if you're an adult, making your own Advanced Care Directive. It was on my agenda for an appointment with the paediatrician that was going to happen tomorrow. 

Then, after that conversation with the neurologist, I decided to contact the Social Work Dept at the hospital, and asked if they had any guidelines or reading or advice. What we really needed, was some terminology and a framework. Of course, it's bread and butter to them. It shows the ways in which these large institutions really are compassionate, and complex, and subtle. They deal with this type of thing all the time. A Family Conference, she said, that's what you do, with the doctors, and an advocate, and a social worker, and you go through in detail if this, then this; if this, then this; and it's in the Chart, and you can have a copy yourselves, and you can change your mind at any time.

Oh.

So suddenly, it happened. Just the two of us, and these two rather wonderful women doctors, having an impossible meeting.

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.

Paediatric Intensive Care Unit

I had hoped to blog an elegant travel blog, with three weeks on the road north up to Cooktown.

Instead, after less than a week on the road we unded up back home, with Morgaine in the Paediatric Intensive Care Unit with pneumonia, after sever breathing difficulties, and having had her first ever seizures (possibly caused by high temp, low sodium, high CO2 - or maybe connected to her underlying condition).

Middle of the night to a small local hospital; then ambulance to Mackay A&E. The scariest thing Abe and I have ever experienced. When he started to cry I knew it was very very serious. Then watched 6 or 7 different doctors work on her for 4-5 hours straight, and finally intubate her etc.

Late that night a med team came up from Bris to get her; and I was able to go in the plane with the Royal Flying Doctor Service. Two nights of no sleep and feeling almost hysterical from lack of sleep and from absorbing so much about medical procedures and terminology. Also watched so many doctors try to put iv lines into her and failing. In the end they opted for medication straight into the bone marrow via a sodding great needle and little drill thing.

Abe and Clancy had to drive back - 900 km or whatever it is.

Still in ICU. Getting a bit better. It's Wednesday now; it all started in the middle of the night on Friday. Maybe they'll take the breathign tube out today.

Much to think about and reflect on; many fears to face; stories to tell. No time.