Prof R:New Zealand is crazy. They're dumbing down everything.
Prof R:Forgive my prejudice, but they've got a ton of Maoris and lesbians after funding over there since they changed their name from medical to the health research council. People doing real research can't get their foot in the door.
Opened up my inbox to day to see an email from my mother:
Hi melissa Here is the photo on my graduate day, in 1984, that time i was same age with you now. This year is 30th year since we went to the uni. Someone will organize for reunion, I don’t think I will come back for that. Uni life is beautiful.
I teared up when I saw it. It’s strange to know so much about what would happen in the subsequent years of her life - having a child, being hungry, being poor, being confused and frightened and wet and tired and working three jobs and I just wonder — is the hardest part still to come? For all of us?
Mum looked so young then, standing on the stairs of a building with her friends (all women) - just a blouse and a simple silk skirt and the brightest smile. I can see why dad married her.
“Who has never killed an hour? Not casually or without thought, but carefully: a premeditated murder of minutes. The violence comes from a combination of giving up, not caring, and a resignation that getting past it is all you can hope to accomplish. So you kill the hour. You do not work, you do not read, you do not daydream. If you sleep it is not because you need to sleep. And when at last it is over, there is no evidence: no weapon, no blood, and no body. The only clue might be on the shadows beneath your eyes or a terribly thin line near the corner of your mouth indicating something has been suffered, that in the privacy of you life you have lost something and the loss is too empty to share.”—
from ‘House Of Leaves’ - Mark Z. Danielewsk (via Kindelling)
What is it about total strangers that make you want to tell things to them?
1am in a taxi, after A and J had gone and the cab driver turned around to me and said, “So you didn’t drink?”
I guess it was pretty obvious that I was the only sober person in his car. I explained that, for various reasons, I’d decided not to drink at social functions for a while. Not that I’m against drinking - more that lately I’ve been having strangely decreased tolerance and so it wasn’t that great, drinking anything (one two and I was done).
He told me about his fiancee in the Bronx, and her plans to become a pharmacist, despite being in medical school over there. Her parents’ family had already bought a pharmacy. After she graduated, she was moving here with him, and he had plans to finish off his accounting degree.
"But you hate accounting," I said, getting involved in the story.
Could I be envious of a cab driver? Yeah I could. I envied his quiet assurance, of his life slotting in place one day at a time: his fiancee would study until she was done. She’d join him in Australia. They’d open a pharmacy together - he’d finish his accounting degree. They’d make a life for themselves together.
I told him about liking some person who didn’t like me back, and awkward it was wanting to talk to someone but never having anything to say. Then the logical thing to do becomes avoiding them - a kind of natural instinct to avoid awkwardness; uncool, really. It’s a perpetual cycle - you definitely rub off on the other person too if you keep doing that. Wanting to talk to someone is a mutual thing. It’s weird. Love is never spectacular or sensational - to like someone quietly, without much scandal or fuss — I think that’s when I know it’s not just a passing thing. That, and being able to picture a life together, invented or otherwise, years down the track. I’m supposed to be a fatalist - I imagine the end of things before they begin. To picture continuation is almost a miracle.
I’m confused as to the extent of acceptance of someone I hardly ever interact with. It frightens me in some ways.
Autumn came, by way of thunder. One day it got dark early and there was so much lightning that my eyes began watering walking from my house to the common room of MMC to type up a revised proposal. Strangely it didn’t rain that day - a dry kind of storm, where only the trees suffered, losing leaves (still green) everywhere. It was strange that day too, how thunder was muted - almost nothing. Was the lightning the kind that passed only from cloud to cloud, never hitting the ground? It was hard to say from the ground, huddled in sweat pants and Alex’s old sweater (a comfort sweater that smells of sesame oil and my bedsheets).
What can I tell you? Life’s been so unassuming. Brown leaves are starting to gather on the nature strips in the neighbourhood which has been tainted by murder (a person was stabbed near the train station next to my house). It was a Chinese man, they say. He’d just come to the country recently. His body lay there for hours before they could move it due to peak morning traffic. I remember catching the train later that day, seeing all the police tape and two officers standing around, looking troubled.
People keep living their lives as usual, although perhaps a little more afraid. I overhear people on street corners talking about it, and how ominous these days are becoming. Still, they’re out and about and shopping, or holding the hands of their pink-cheeked children (beautiful, by the way, always).
Since reading Norwegian Wood (Murakami) again, I have had this urge to write letters, like the Toru wrote constantly to Naoko, and sometimes to Midori (when she was angry). I’d write about little things - like how I saw a flock of birds flew in such a coordinated way from the branches of one tree to another (A mere 5 metre distance) one wet, miserable day while sheltering under Robert Blackwood Hall. Did birds mind rain? I wanted to ask. Also, who out of a flock decided when it was appropriate to leave the branch, and where they would alight next? Did birds speak? What do they think of when they sit in trees?
I’m trying to cheer myself up from this research thing. I think being absentminded and distracted has come across in major ways - academically I’ve been blanking out common things suddenly (things I do at least a few times a week, like reporting blood films or reading X-rays or, for instance, the name of common medical phenomena like clubbing — which I could at the time describe perfectly, list causes of but just could not for some reason come up with the actual word). Forgetting things in brief blank outs this is actually starting to terrify me. Usually when I blank out on a word, I can get away with it by describing everything else medically about it, and avoid the noun. I try and not get into situations when naming things might be important (although some jerk registrars still try make you do it).
Behaviourally I’ve just been curled up in warm places with Herodotus ‘The Histories’, choosing Greek and Persian history over most other things. Including work.
Ev says I have to choose.
Ev is completely right.
That, or I could make sticky date pudding on rainy afternoons, clean things, have dinner with Michelle, talk about being unhappy, and hating choosing. By the way, you can spot an indecisive person from a mile away. Big decision indecisiveness, I mean - not just little everyday choices about the colour of tie or what to eat on a lunch menu.
Leaves continued to brown down the street. I went on a date one night, but we both (I’m hoping) spontaneously decided that we’d let it turn into a friendly thing. Somewhere along the way, I turned 22 years of age.
Birthdays always remind me how fortunate I am to have so many amazing people around me, or having been a part of who I am even remotely. I’m so grateful for everyone who said happy birthday via various mediums. Even my old Psychiatry consultant phoned me to say hi (how he remembered what day it was was beyond baffling).
People were still sending belated birthday greetings 3 days after the fact. I felt like I was reliving the actual day over and over.
These are the people who matter.
Warm fuzzy feelings, enough to melt away the worst of the autumn chill.
Half past 7 in Prahan - the sky was pitch black, and the trees in Fawkner Park were indistinguishable in the background from everything around them. I’d come in for a meeting at 6pm, which hadn’t started until half past anyway.
Prof R. met me at the bridge between the old university quarter and the patient lounge on Level 3, having had to unlock the door. It was a spectacular kind of moment, just like a horror movie. The lights flickered on at the end of bridge; through the small window in the door, I saw a figure skulking toward me - it raised its hand, and I pawed the glass in return. On closer inspection, it was an old man, thin, with a stranger gait. In his hand was a manual drill (the ones that you used in carpentry, in the olden days). On even closer inspection, he was covered head to toe in fresh blood.
"Come in, he said, inviting me into the darkness.
I checked behind me. None of the patients had noticed, in the brief moment the light hit him, the gore. I slipped through, shutting the door tightly behind me.
He whirled the drill a few times, like a child excited about a toy. “Come and see what we’re doing.”
What they had been doing was extracting a greyhound heart. I entered a brightly lit room at the end of the dark hallway. The first thing to avoid was blood splatter (I was wearing white shoes). One of the research assistants held the heart over a bucket, emptying it of blood before they could weigh and assess it. A complicated apparatus with blood in all of its tubes was on the side as a simulated circuit for systemic pressures.
The metal table was empty, recently cleaned. I could smell it in the air - a distinctly doggish smell that is recognisable anywhere.
"Where’s the dog?"
"Oh, we put it in one of those bins," a lady said. "I know, it’s sad. But it was going to die anyway. We get them from the pound. It was a very good dog, this one. The poor thing."
I keep wondering what they mean when they say the dog was very good - I’ve heard it thrown around often at lab meeting. Was the dog compliant? Did it go calmly under anaesthetic? Was its arrest uncomplicated? It was chilling. A compliant, docile animal - not gittery - accepting of death. Or did they mean that its heart was healthy, easy to extract?
"Show Melissa the heart beating on an echo," Prof R came up to us. "See here, they’re the papillary muscles. They are huge on dogs." (They actually are. I learnt something new that day; also, that dogs don’t have blood groups).
I tried to rush the meeting over the project proposal, needing to be elsewhere fairly quickly. He wasn’t interested in reading it, despite messaging me late the night before with a research proposal - an ammendment that I didn’t agree with, but I wrote up anyway.
There was a lot of drawing diagrams on the board, speculating about the results - imagining of what the figures would look like.
I bit my lip. Something didn’t seem right. I told him so. I didn’t care about my thesis; I cared about study design; I wanted to do something worthwhile, something I could stand by.
We argued (in that polite way that professionals argue about things). He tried to confuse me with Sigma-stat and some statistical calculations (irrelevant). “Not important,” I shook my head.
To my surprise, I was winning.
"Maybe you’re right," he said. "Maybe it’s better to do things properly instead of doing things quickly."
A profound hush fell. I tried not to fidget, or tear my hair out.
"Don’t you have to go," he asked.
"Where do you need to be?"
"Glen Waverley by 8."
"You have the car?"
In the end, he saw me out, saying he’d call over the weekend. Food for thought, really.
It wasn’t until I was half leaving when he made a casual statement over my shoulder that sent me keeling: “Thank you for making an honest researcher out of me. I was going to do something quick and dirty.”
The words were echoing in my ear as I bustled out into the corridor from the rickety Centre block lifts, all the way through the corridors (full of visitors and dinner crowds) and out onto busy Commercial Road. I was incredulous. An honest researcher? An honest researcher? If I hadn’t stood up for myself, if I had let myself get coddled and confused with all his explanations…
It’s been 4 months of…nothing happening. This whole time I’ve felt like the world’s biggest whiner (I object to something at every step) and it was integrity I was arguing for after all?
I’ve been so unhappy and stressed. How many things could have been avoidable? Late nights, embarrassing mornings with clinics and more meetings and filling out forms about results that I didn’t feel I could stand by..? Embarrassing myself in class, while exhausted? Incredible bouts of nominal aphasia that lead me to feel like I had a brain tumour and actually go see a doctor when I’m pretty sure it was out of late nights and an overnight shift?
If I learn one thing from this year it will be to know exactly what I stand for and defend it like crazy. I’m too easily manipulated.
To be honest, I hate going out. I do it a fair bit, which is confusing.
Hein, when I sighed that I had antisocial tendencies once in his car, but he’d knocked me back.
"Don’t kid yourself," he said. "You’re very social!"
"Sometimes I feel rude if I don’t go to stuff people invite me to, even if it’s inconvient to go. Usually it’s because I like whoever the person it is a lot too," I protested.
"That’s why social people are social." (Catrina once said that once Hein gets an idea in his head, there’s no convincing him otherwise).
This conversation flashed back to me while standing on the street corner after midnight, waiting for a taxi. There was a German man in his 50s and a sturdy woman dressed in a track suit in her late 40s, parcel tucked under her arm in a plastic shopping bag.
"Oh look, he’s gone," she said when the German man went across the road to try call a taxi company. "Move up the line, move up the line." She nudged me roughly with her forearm and I caught a whiff of cigarette and alcohol.
I moved over. “That’s a bit mean. I’m sure he’ll be back. I suppose he can share my cab if one comes.”
“Well he’s not sharing mine. Who knows, he might have a gun in there? Just gonna rob you and all. No way. Nuh Uh. He’s not going in my cab.”
"Where are you from?” I asked, looking at her. She looked like Maori woman…and it turned out that she was from New Zealand - Wellington, to be precise. Her partner was from Auckland.
She gestured to a drunken, homeless looking man sitting cross leggedly in the parking lot, unable to get up. “Oi pig! This woman says she’s from New Zealand aye! Says she’s from Auckland.”
He clambered up with great difficulty. He was around my father’s age, stopped with tattoos down his arm, moving sluggishly, like those old floppy clowns. I looked at the German man with his bags. He’d moved across the road, feeling it was unsafe. I looked at the large, stumbling man. Didn’t exactly blame him. He looked like a murderer.
“I went to primary school in Hillsborough,” I said, when he got closer. “Roskill was right next door.”
His eyes lit up. “Roskilllllll!” He tried to hug me but it turned into a bit of a weird handshake/grapple (his wife interceded). We must have been an odd sight. A rough looking biker-guy, drunkenly leaning into a small Asian girl, shivering in the cold — his partner puffed on her cigarette beside us.
“Five years…soon. I came over for study.”
“You study ‘ere?”
“Yes, up at the Monash,” his partner interjected. “She’s at the Alfred too, sometimes. Like me! (The woman was a food services opperator at the hospital)” They slapped each other on the arm crossly - him for her cutting in and she because he’d slapped her, and it was annoying. I wanted to laugh. They’d been together a long time, it looked like.
“Came ‘ere in 1986, I did,” he said. “She came in 1988”
“I was born in ‘88!”
“Making me feel old, you are.” the woman huffed. She slurred a little too.
“They say everyone up at that uni Monash uni up there is the brains for the next century.”
This time I did laugh. The brains for the next century… if only they knew what really went on during O-week and Medcamp. “Who says that? That’s frightening.
The woman announced that she was leaving us to go find a payphone. They fought again, over who had change and whether or not to just use their cellphone.
“Sowheresyour’usband?” the man asked, turning back to me when she’d gone.
Startled, I laughed again. “Where’s my husband? Where’s my boyfriend?”
“Yeah where is he? Husband, boyfriend, whatever.”
“In a coma, maybe,” I smiled. “…in Abu Dhabi. Or sailing around the world on a rickety ship.”
“There’s not enough love in the world,” he nodded. “So much hate.” — it came out as s’not love enough s’not..not enough love aye! full of hate everywhere! s’not much love. — “Better to be picky than not choosy and like a beggar.”
I thought of his partner (were they married..? I’d just assumed so). They seemed to fit somehow, between swearing and cuffing each other and arguing about payphones and change.
“You’ve found a good one though,” I said, genuinely. Imagine in the world, finding one person who accomodated to your quirks and whims. Who would wait with you while you’re drunk in the street, somewhat drunk herself, in your 40s or 50s (to each their own, I suppose). — willing to be poor, willing to share. Someone who fed you, clothed you, who kept you alive and vice versa so far from home.
He turned serious. “She can be a c*nt aye. But she’s alright, that one. She’s alright.” (C-word aside, the sentiment was not lost)
A taxi came. He let me have it. The tattooed man and I shook hands. His name was Tangi, he said. He was overjoyed when I got it right. “God bless you” he said.
It made me miss home a little. New Zealanders look after their own.
People who give so abruptly just as easily take it away
I’ve come to realise that people who act inconsistently make me uncomfortable/frighten me.
For instance people who talk to you a lot and all of a sudden stop talking to you; or people who never really talked to you before suddenly messaging you on Facebook announcing that they’d like to get to know you better.
It makes me wonder on what basis they decide these things — one day to be your friend, the next day not to. At the very least, explain why.
“If I’d been a different sort of person, a braver sort, I’d have taken him by the shoulders and said, “Want whatever you want more fiercely. Be more difficult and demanding. Or you’ll never make a life that uses you.”—from ‘A Home At The End Of The World’ - Michael Cunningham
I cut myself scoring chestnuts in tonight. They were soft, just boiled from the large pot beside me, still steaming softly. I drifted off. The knife slipped. The sight of blood momentary stunned me.
It had been so cold downstairs - nobody home, a glass of red wine, barefoot on hardwood. I’d been wearing Alex’s sweater from when I lived out East, the one I’d demanded as a joke, which he eventually gave me before he left for overseas for good.
Thursday has never been my favourite day of any week, though for this particular one it signaled a strange (and early) end to a fairly good one. I had something on Monday, the same day as a presentation I had to make which was cancelled. I won a few [heated] academic debates with Prof R, which, for me, is a huge deal (I have a hard time pushing my own agenda). Misplacing a pair of jeans on Wednesday, I was forced to into very old ones I hadn’t worn since first year— and subsequently found $50 dollars in the back pocket.
This project is intensely interesting conceptually; at the same time, intensely frustrating. I discovered that Prof R’s knowledge about the patient population was limited; also, his knowledge of some of the compounds he was proposing to give, and their actions. I’m often telling people that I don’t want to talk about my project — it’s not true. When you are doing something/reading about something so much, you really, really want to talk about it. The difference is, you want to talk about it with someone who knows what you’re talking about, who can particpate in a discussion about it. Otherwise you’re just describing it from the beginning and it’s irritating after a while.
Academically isolated, I met another medical student on the tram as I was going home from the Alfred one day. When she asked what my research was about and I told her that I didn’t really want to talk about it… she became insistant and told me to try her.
"Seriously? Are you sure?" I asked.
"Yeah! I really want to know."
"It’ll be boring."
"No way! Try me."
So I told her about thalassemia, the principles of thalassemic cardiomyopathy, free radical molecule formation as a biproduct of the respiratory chain enhanced in the presence of iron, the role of antioxidants, oxidative stress and how cell membranes lose integrity as the result of it, etc, etc. She nodded her head. I was excited — maybe I could tell her a bit about being concerned about the Fenton/Haber-Weiss reactions in relation to these people having high free iron in their blood eventually..
"So how do you guys measure oxidative stress?" she asked. "Is it just using blood and looking at the pCO2 and pO2?"
I faltered. “No I meant oxidation as in…oxidation-reduction reactions. Not as in too much oxygen gas.”
"Like what they taught us in high school, when there’s a chemical reaction, and something loses an electron/oxygen or gains a hydrogen—- [she seemed confused] Nevermind. I like your shoes.."
We had a long conversation about shoes.
Sometimes I wish I had more academic friends. Moving to a different country, people you end up hanging with are often purely coincidental. I talk about clothes, silly things, movies; I’m pretty sure I have a frivolous, flakey, messy, clumsy persona. Not entirely unrepresentative of me (I’m generally a happy, if not accident prone person), but a little exaggerated too.
I get excited when someone talks about a paper he read or someone else read the same paper in fish oils the other day; or someone is interested in Haematology (only one person so far, and only because she was on rotation at the Alfred a few months ago…). Or even when people talk about respiratory physiology out of personal interest. I can see why consultants favour people who like their own specialty/show interest.
Meanwhile, Prof R does things like text message me at 10pm exclaiming that he’s impressed about some lipoic acid review articles… (we are having an argument about whether or not lipoic acid oral therapy is worthwhile).
Back home, Mum emails me photos of my brother on his nice, shiny new bike. It’s red, simple. I am almost sure it’s his first, although somehow I’d always thought we’d had one before. He tells me about a weird mixture of kitchen materials that he mixed into a drink the chucked out — something I used to do at his age (I went one step further - I would put everything into a bowl, add flour, then try and bake it: soy sauce, flour, milk, orange juice, tomato sauce, baking soda, etc.)
It made me realise that I like hearing things about people’s families. Stories about their siblings, what they used to do as a kid, their mother and father… road trips in the car, etc.
Sunset tonight, I was on the phone with a friend, legs propped against the wall, laughing at her ED stories.
You know, I’ve been so awkward and clumsy this week. Not pleasant to be around — I’m always so moody in April.
“You have never been hurt before, never had a relationship end before you were ready, never failed to have any man you have wanted, any job you have wanted. You do not know what it is just to shut down, to walk around dead inside your clothes. You will eventually convince yourself that this never happened, that I never happened. Then only I will know. But if only I know, did it still happen?”—from ‘The Reasons I Won’t Be Coming’ - Elliot Pearlman
The world’s so dull and grey today, lifeless. Days like these make, listless - a throwback from being an only child for a long time.. I’d sit up on the windowsill of my parents’ bedroom as a child, foot dangling over the edge, grazing the tips of flowers - a game, waiting for rain. If I were lucky, I’d go over to Oliver’s place and we’d sit in his living room, inventing games or playing with his dinosaurs, or wondering what we could be doing when it was sunny again.
Today, there’s no urgency. I wake up around noon, stretch, reach for the jug of water on the table and check the time. In the kitchen, I pour myself a glass of juice and wander to the glass doors leading to the yard. There’s nothing to see. The grass - a jungle of weeds - is unkempt, there’s no laundry on the line. The red roof tiles of a building two doors down, the corner of our neighbour’s townhouse with its black drain pipe.. tree in the yard, all of them unmoving. Such an average stillness -.ordinary. There’s only the lingering hint of rain (the smell of wet pavement, though outside the road is dry and the a dog barks incessantly three doors down; a child crying, unable to be quieted).
Should I do some work today? Maybe. Yeah ok. I’m convinced. I’ve done barely any, and it’s Friday.
Being called into the Alfred very very early on Wednesday morning (Curse surgeons and their ridiculous ward round hours), I was so sleepy! I’m never quite used to the main entrance of that place, where a bizarre mix of extremely sick and extremely healthy people hang out (the difference is really pronounced there).
In the five minutes before the meeting, I went to withdraw some money. A demented looking, disorientated old lady touched my arm suddenly and demanded, haughtily, that I look at the plaque next to me and tell her what number to call because the machine ate her bank card. “Then go tell that man,” she pointed to a random guy. “He’ll call them up for me.”
"Which bank are you with?" I asked. "It says to contact your personal bank if you lose your card to it."
"Oh ok. Um. I guess we can call Westpac."
She went on to tell me to call the cleaning department because she’d dropped 3 tissues on the ground. I told her it was ok, kindly, and that I’d pick them up — she told me I was useless and that she’d get someone else. When I’m old and stressed, I hope I’m not cross with people, I thought, looking at her with concern. Her hands had Z-deformities, and she drooled a little. When she wasn’t looking, I picked the tissues up after her.
A little girl came into clinic this week - 10 years old, house captain, sports captain…and transfusion dependent thalassemic. But God, she was lovely - happy, healthy, glowing. Even hooked up to a bag of blood she looked more alive than anyone in the room. Her mother, however, was the most anxious person you’d ever meet. She worried about everything; worse, she projected the worry onto her daughter. For example, her Exjade (an oral medication) — her mother said that the girl was worried about drinking it, reluctant to.. that she’d missed a few because she’d been flu-ey the last few days. One look at the child and you could tell they were her mother’s concerns, not hers, but she just took it when we reassured her and told her that the drink would be not a big deal.
"Lately she’s been having this huge craving for salt," her mother said, in the kind of way that anxious people do when they try not to sound anxious. "She just wants to eat salty food. Specifically, potato chips"
On further questioning the girl liked salt and vinegar chips and sweet chilli flavoured ones; thin chips instead of thick — definitely potato, not corn chips (we had a conspiratorial chat while I was examining her. It turned out that neither of us liked chicken flavoured chips and had once been ostracised by our peers for choosing the plain one instead. We tolerated honey soy chicken, only because there was honey soy in it).
I glanced back at her mother. Had she never been 10 years old? Who didn’t love potato chips back then?
I could hear the registrar in the background. “No, really Mrs X, it’s not a sign of kidney failure. Yes I know. No, no, it’s normal to want salty foods. No I don’t think three packets in a row is excessive in a medical sense.” Also, there was a bone jutting out in her back. Was it cancer..? Was it ok to be sports captain..? Could she play sport..?
In the end the little girl got fed up. “Mum, I have a runny nose too. Do you want them to look up my nose?” she said crossly and I thought, good on you — stand up for yourself. Nobody else will.
"You saw it too, huh," Shahla, the registrar, said afterwards. "It’s like that every time I see her. She’s a fiery thing but she’s slowly being crushed. I called her in today, and the first thing she did was call for her mother and father — other kids happily totter in by themselves. And her dad, you saw him. He doesn’t say anything. I wish he would do something, stand up to his wife! If only for the sake of this small little girl."
Suddenly I realised, last year on the Paediatrics ward, all the patients were acute. We saw them for their hospital stay, then they were gone. Nobody ever had to see this side of things - the chronic side. Imagine seeing a child become a victim to bad parenting right before your eyes, month after month when she came in for review, or slowly fading away from what she could have been. I thought of the girl, maybe 10 years from now - what kind of person would she be? Introverted? Nervous? There was no call for that - it made me angry that her mother couldn’t see. Even worse! Seeing a child parent her own parents, like the little 8 year old whose parents were deaf, who was brought in by her mother to translate when one of her siblings was sick. I think, for good Paediatricians, they become advocates. The child’s fights become their fights, whether it be against disease or grown ups or bad days and bad dreams. They become parents in lieu of good parents.
I think of N, suddenly. Who stood up for her when she was young? It makes me mad. Why didn’t anyone?
When I’m a mother, I’d let my child be a child for as long as possible. Never force them to grow up too fast, let them get dirty, take up whatever hobby they want… once in a while, we’ll have dessert before dinner.
My footsteps leave little imprints on the floorboards which disappear slowly. I pause at the stairs to watch this small trace of myself being removed from existence.
I find myself bargaining/making these promises a lot these days.
Haemoglobin Haemoglobin (Hb) is the major component of a red blood cell (RBC). It’s made up of little units called globin chains, each of which binds to a heme molecule, which binds to iron, which binds to oxygen. It is this binding that allows the oxygen to be carried by the red blood cell to tissues around the body.
There are 4 globin chains in each Hb molecule - 2 each of a different kind. Different combinations of globin chains type mean that there are different types of Hb possible. While foetuses and some genetically mutated individuals have rarer types, in normal adults the majority of our RBC contains HbA (written shorthand as α2β2 — denoting 2xα 2xβ chains), some HbA2 (a2δ2) and little or no HbF (α2γ2), which is the one that babies have Thalassemia Definition A genetic disorder where a person is unable to make β globin chains, or only able to make a decreased amount, resulting in dyserythropetic anaemia.
Classificaitons β0 thalassemia = patient makes no β chains at all β+ thalassemia = patient makes some β chains, but less than usual.
Thalassemia major= Severe homozygous disease (usually) which leaves the patient blood transfusion dependent for survival. Thalassemia intermedia = Patients get anaemia and splenomegaly, but are not transfusion dependent. Thalassemia minor = Patients who are symptomless carriers of the thalassemia trait (Heterozygous disease).
Epidemiology Commonest in Mediterranian, South east Asian people, but the thalassemia band actually extends through the Middle East, India and North West Africa too, as well as parts of Southern China, Yugoslavia and Romania.
Pathophysiology (What happens) There are two main problems these patients get: 1) Severe dyserythropoetic anaemia (and complications of what their body does to try and make more blood to compensate to the nonfunctional blood cells). 2) Iron overload due to the repeat blood transfusions to treat their severe anaemia.
1) The anaemia: Because there aren’t β chains produced, the body’s α chains have nothing to pair up with.
Confused, they buddy up with themselves and become α4 molecules in the red blood cells, which is unstable. Degraded α4 molecules precipitate in RBC and can be seen as inclusion bodies. The body kills these off before they leave the bone marrow (ineffective haemopoesis). This leaves the patient anaemic (dyserythropoetic anemia).
α-Chains also try desperately to bind with other types of globin that might be lying around to form a 4-globin unit. This usually means the δ and γ types lying around. Therefore, in thalassemic patients, the proportion of HbA2 and HbF also increases compared to normal people.
In fact, the body for some reason creates more HbF than usual in general by selectively allowing red blood cell progenitors that are destined to make this type of Hb live in β-thalassemic patients. This is problematic as HbF is more selfish with the oxygen than your usual Hb and hates giving it up to tissues (high oxygen affinity), so tissues still get less oxygen than if HbA could be formed, not helping the situation.
The lack of oxygen going to tissues (hypoxia) makes the body think it doesn’t have enough red blood cells being produced. It tries to make more by increasing erythropoetin production (a growth factor made in the kidneys which stimulates red blood cell production). This makes the bone marrow, red blood cell factory, work extra hard. The spleen and liver enlarges to try and help the bone marrow make blood, trapping red blood cells in them, destroying even more of them.
Unfortunately, because the bone marrow is being driven hard, it starts to expand. This causes skeletal deformmities and brittle bones (fractures!). High cell turnover creates strain on the body, and the person becomes hypermetabolic. This causes wasting and poor growth in children, most of the energy being used on making new blood. They also get folate deficiencies as folate is used up creating new marrow cells. Folate deficiency can make anaemia worse, as red blood cells also need folate.
Other things they get include gout, also, to their high rate of red blood cell breakdown and dilutional anaemia due to splenic pooling, also extravascular haemolysis due to enlargement.
We treat these problems by giving them blood transfusions, usually 3 weekly, to replenish their blood counts.
2) Iron loading Three things lead to the buildup of iron in thalassemic patients. The first is from the transfusions, which obviously contain lots of iron rich blood - more than the body’s usually able to handle over time. The second is from the patient’s own body desperately trying to absorb iron because it’s trying to make more red blood cells, triggered by oxygen starved tissues. For reasons still being explored, the gut, which absorbs this iron, does so in a way that is dysfunctional and ends up taking in more than it needs. Lastly, red blood cells themselves contain iron, so when they are broken down that iron is released. As these patients are breaking down a lot of their red blood cells, more iron is released into the blood.
Iron deposits in end organs, causing damage. In the liver, scarring (cirrhosis) may occur over time. In the heart it causes cardiomyopathy, arrhythmias and heart failure. In the pancreas, it causes diabetes. In the thyroid, it causes thyroid dysfunction. In testes and ovaries, it can cause hypogonadism, etc.
Iron overload is treated with chelation therapy. When the above listed complications occur, however, they are treated individually.
Other problems The problems that thalassemic people get include, for some reason, a increased clotting tendency. There is a degree of immune dysfunction present for some reason inherently, and iron overload people are also more susceptibe to Yersinia infections also, as this organism loves iron rich blood.
Clinical picture When infants are born, their blood is mainly composed of HbF. In the normal progression of life HbF production is decreased and HbA is increased gradually within the first few years of life. As β-globin is only needed in the production of HbA symptoms such as paleness and tired and jaundic (from haemolytic and dyserythropoetic anaemia), spleen enlargement, etc. usually come during/after the first year. In people with thalassemia major, symptoms start developing usually in the first year. Intermedia patients might be asymtpomatic for a couple of years, etc.
In a well transfused person and well chelated person, iron overload and its sequelae doesn’t really happen until around puberty and no boney deformity occurs. In a poorly transfused person person, dysfunction begins early on and may lead to bone abnoralities and short stature/poor growth. Iron buildup also occurs around puberty due to the dysfunctional absorption of iron from the gut.
Workup History, examination, FBE (full blood count), peripheral blood film, Hb eletrophoresis, analysis of HbF and HbA2 counts (should be raised in beta thalassemia).
Differential diagnosis Leukemia and other marrow infiltrative disease and other haemoglobinopathies.
Management 1. Transfuse 3 units 3-4 weekly + regular review of Hb and iron levels 2. Chelate iron when ferritin reaches 500 3. Consider splenectomy, marrow transplant, etc 4. Regular monitoring of end organs for signs of iron buildup/dysfunction
Transfusion begins early in life, to correct the anaemia before the side effects such as high marrow turnover has time to cause boney changes and hypermetabolism. Patients average 3 units of blood every 3-4 weeks.
Chelation starts when Ferritin reaches 500, using either subcutaneous injections of Desferal (desferoxamine) or once daily oral Exjade (deferasirox). Chelation is continued until Ferritin is below 500, and is ceased. If the level creeps up, the lowest possible dose is started over again. The effects of dosage changes can take up to 6 months to reflect in serum ferritin levels.
Some centres add low dose Vitamin C to enhance the chelation process as it frees up iron in the blood. Monitoring is needed as high dose vitamin C increase iron absorption, which is not good.
Important things consider, as indicated: Regular FBE and film, ferritin (+/- full iron studies), UEC, LFT (as a large amount of iron is stored in the liver), Vitamin C, E, D levels; HIV, HCV, HBV serology, Genetics, regular echocardiography and mangement of cardiac issues if indicated, consider thyroid function tests if indicated, management of diabetes if the pancreas is affected by iron, testosterone or the pill for hypogonadism if present… etc etc.