Tag Archives: Thalassaemia

The all too mortal child vampires of Phnom Penh.

Zombies vs Vampires.  Many people have a preference, nearly everyone has an opinion. I do not care for zombies and I shall speak of them no more. For me it’s always been vampires, or at least since I saw The Lost Boys as a young teenager. Corey Feldman (sigh.) And then Bram Stoker’s Dracula.  The horror, the love that transcends death, the secret desire that maybe the bad guy will win because he loved Mina so much (And let’s face it – Gary Oldman or Keanu Reeves?)…  Onto Buffy the Vampire Slayer.  Add some teen angst, a bit more sexy. And then True Blood.  Lots more sexy and set in The South.  Anne Rice’s marriage of the vampire myth with New Orleans should be lauded as genius.  Poppy Z Brite cemented their presence in that city with her insane novel “Lost Souls” so clearly to me that I was downright disappointed that I didn’t meet a chartreuse slurping, candy gobbling creature of the night lurking in Jackson Square when I visited a few years ago.  That city that was so familiar to me from reading those books countless times.  But there was another reason it felt familiar.  The balmy September nights, the silent, deadly strength of the chocolate brown Mississippi, the celebration of gastronomy, the French influence, the street chatter – nobody was a stranger and everyone told you to come on back soon now.  It reminded me of the faraway city I call home.  A little city with a big, brown, deadly river running round it.  Balmy evenings.  Life lived on the streets.  Where it’s just plain rude to not ask where you’re off to tonight?  Have you eaten rice yet?  But a city that has real vampires, not the kind you just find on the pages of a book.  And a real vampire is very different to the kind you find on paper.  The horror is very real, but not for the reasons the fiction would have you believe.

I know four little boys.  They are smaller than they should be because they haven’t grown how little boys should grow.  They are quieter than they should be because they haven’t got energy to spend on chatter.  When I met them, one of them had a very big scar across his belly that marked the place that his oversized spleen used to be.  When you peek under the lids of their little eyes, it is milky white instead of rosy red.  If you look closely you can see their little hearts beating through the skin on their little chests.  The rise and fall of their little chests is more laboured than it should be, as though they have to will their lungs to suck in air.  If you look closer still, into their eyes, some days you will see nothing looking back at you.  No spark, no recognition, no sadness, no joy.  Nothing at all in fact.  But take a step back and you will simply see a little boy who can only focus on the act of taking the next breath.  Maybe that is where the idea of soul-less comes from.  Those eyes.  They can be terrifying.  And they will keep you awake at night, afraid of what their emptiness might mean.

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Their deaths are documented on paper.  “The results of these blood tests are incompatible with life.”     Maybe there wasn’t meant to be a spark in those eyes, it said so on those pieces of paper.

And then there is the blood.  It is the key to their survival.  Any longer than three weeks and their organs start to crash, they become the husks I described above.  Two units of whole blood.  The fresher the better – the red cells last longer that way.    But a real vampire would never waste a commodity as difficult to come by as clean blood on their digestive systems.  Any method other than straight into the vein would render their life source useless.

The kryptonite of real vampires is a cruel joke.  It is iron.  Sunlight would be much easier to avoid.  According to the Kennedy method, the average iron content in the blood of a “normal” male is 52.5mg per 100ml.  One unit is 350ml therefore it is reasonable to conclude that it contains about  157.5mg of iron, a bit less if the donor was a woman.  This iron builds up around the vital organs, overloading them.  Particularly the heart.  SO the very thing that saves them, that nourishes them also destroys them.  In developed countries, an individual with Beta Thalassaemia major has a life expectancy of forty years or longer (depending on your information source) with the most common cause of death being heart complications caused by iron overload.  Mind you, this is in countries where iron chelation therapies are readily available.  Iron chelators flush iron out of the body.  Here in Cambodia, as in other developing countries, chelators are not readily available and relatively expensive.  Up to $1 per day for the cheapest variety.  Multiply that by four children and the grand total equals more than this family earns per day.  Faced with the expense, most parents have to accept that their children are temporary – something that no parent should have to face for what many consider to be small change.

I spent a lot of time trolling Thalassaemia forums from around the world, looking for a solution.  That was until a local medical professional informed me that was not where the answers lay.  Elsewhere, this is considered a manageable genetic disorder.  Here, it is a death sentence.

Are you horrified yet?  It gets worse.  So much worse.

A child with Beta Thalassaemia Major in Cambodia has a life expectancy of between 5 and 15 years.  Less than a child that is HIV+.

Of the studies that have been conducted, it is estimated that up to 48% of the population carry the gene that results in Thalassaemia.  Without the introduction of genetic screening and counselling, this problem is going to grow.

Very few doctors in the country are aware of the existence of this disorder and generally treat the anaemia that Thalassaemics present with iron supplements – increasing the overload on their organs, worsening their condition.

Thalassaemics are more susceptible to infection as their immune systems are compromised.  A thalassaemic child who contracts an infection or virus is far more likely to succumb to that illness than a non-thalassaemic child.  As this condition is rarely recognized, deaths that are actually due to the  underlying cause of thalassaemia are rarely recognized as such, undermining efforts to have research, medical training, genetic screening and public education conducted.

Donating blood is a culturally sensitive issue in Cambodia.  With the shortage of blood stocks, thalassaemics are mostly unable to access their requirements to maintain health.  Unless facing organ failure and close to death – thus being considered a medical emergency – they have to go without or find replacement donors.  And the replacement donor model is easily corrupted, in many instances giving a monetary value to blood resulting in a black market.

A good friend of mine told me I should write a horror story based on my outlandish vampire comparison.  Maybe I should.  Maybe I could make a tonne of cash and sort this mess out, fund the research and education programs required.  I feel a little guilty for this vampire bizzo.  Whilst in the forums I noticed a few people expressing their dislike for the comparison.  I mean them no disrespect.  Others didn’t mind, finding it a useful way to explain their conditions.  The last eight months have been a steep learning curve for me.  Trying to find medical experts that understand the local complications and hurdles; convincing all my friends to donate blood; convincing random tourists to donate blood; raising money for iron chelation; spending far too much time in a paediatric ward; becoming far too attached to four little boys;  getting involved with the National Blood Transfusion Centre and the Cambodian Thalassaemia Association.  So I hope Thalassameics will forgive my indiscretion.  I am simply trying to write a piece that people will read and share.  And hopefully some vampire fan out there has the answer for me, or a friend at the WHO that is looking for a new project to save thousands of lives.  Because we vampire fans should stick together.  Unless you like Twilight, in which case I have nothing positive to say to you.

Pre-warning:  This is only the beginning of this tale.  It’s actually barely an introduction.  But if I write too much, people miss the joy the funny and the ridiculous. SO:

The joy:  The people I have met who bust their ass for this disease are some of the most dedicated, professional, intelligent and compassionate people I have had the honor of meeting in my life.  It is humbling to count them as friends now.

The funny:  I have what is apparently known as shy veins.  Getting blood out of my is tricky and usually hurts a fair bit.  I kinda have a phobia about it.  So I shouldn’t take the piss out of people who get a bit dizzy after donating blood.  Because it is mean.  And I am awful.  And they are lovely.  But it is kinda funny.

The ridiculous:  Vampires.  Really?  Yes it is ridiculous.  You know it.  But still not as ridiculous as Twilight.  Sparkly diamond skin in the sunlight?  Really?  Hang your head in shame you panderer to the tween market…

And on an unrelated but related note:

Beta Thalassaemia Major.  Thalassemia if you are a fan of American spelling.  Personally, I like the aem in my blood words.  Heamoglobin.  Haemotology.  Anaemia.  I like the way that my Spellcheck underlines them in red.  I know it is trying to tell me to conform but I refuse.

***Photo credit : Conor Wall

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