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THE SUNDAY TELEGRAPH MAGAZINE
(United Kingdom) 5 OCTOBER 2003
BEYOND THE PALE
By Rose George©
Photographs By Rick Giles©
One
day Bianca Knowlton decided to perform an
experiment. It was nothing spectacular or
scientific, nothing so different to what she
did every day, shopping in Bournemouth with
friends. But this time one friend walked
behind her to count the comments. 'I can't
remember how many there were,' says Bianca,
now a confident 21-year-old sitting in her
parents' living room in nearby Wimborne.
`But it was two out of three people.'
Bianca is an albino. The comments were
nothing she hadn't heard before: 'Ooh,
what's that?'; 'Ghost!'; 'What's wrong with
her?'; and, after Halle Berry's snowy-haired
character in the X-Men films, `Storm!' Once,
she says, still with disbelief, a man stood
still in front of her, looked her up and
down, then, as she left, shouted, 'What the
hell was that?
'They don’t realise,' says Bianca, dryly,
'that we're partially sighted, but we're not
deaf.' She laughs about it now. 'It used to
really upset me. Now it depends on my mood:
It helps that she can't see the stares,
because of her poor vision. But she can feel
perfectly well. 'People are always coming
up to me and stroking my hair. Usually, I
purr, and that stops them.'
I can see the temptation: her hair is long,
white, shiny and gorgeous. With her white
brows and pale skin, Bianca is like nothing
I've ever seen - but then there are only
about 3,000 people in Britain with albinism.
The rate of albinism in the general
population here is one in 17,000; though in
inbreeding tribes elsewhere in the world it
can jump to one in 125. Albinos are more
common on screen, says Bianca, whose
personal website lists dozens of albino
characters in Hollywood films - all
white-haired, white-skinned, ghostly and
bad.
‘I don’t blame people if they look at me
funny,' says Bianca, 'when all they see in
films are evil albinos.' Still, Storm -X-Men
mutant, but not a villain - is a welcome
change after years of being called Casper,
'granny', or, simply, `albino', like it's an
insult in itself. Nor is Bianca surprised
when people expect her eyes to be red, if
all they see are red-eyed film villains.
Her eyes are usually mauve. Sometimes they
look blue. Sometimes, when the light is just
so, they glow. Bianca's father Mike delights
in telling the story of when she was small,
and the spotlights in a shop turned her eyes
glowing red, spooking the shop assistants.
Mike's a cheery man, and Bianca is clearly
well-defended against society's shock by her
family. Forthright is best, in Mike's view.
`I'll always tell people I have two
daughters, and one's an albino. I wont
lecture, but I'll get the point across.'
This point is, there's a simple
physiological explanation why one daughter
is nicknamed Salt and the other (dark-haired
Tanya) is Pepper; gene mutation. There are
at least ten forms of albinism, caused by
various gene mutations, with varying
effects. Some look like Bianca, who has OCA1
(Oculocutaneous albinism). Mike calls this
`the worst case scenario': it provides all
the traits -white hair, violet eyes,
unpigmented skin. Then there are people with
albinism who have red, blonde or brown hair,
with all kinds of eye colour. People you
wouldn't look twice at, even though most
share albinisms key: a lack of pigment in
the fovea section of the retina, which means
that when a light shines on the eye, there
are only the blood vessels to reflect it
back. It also means that most people with
albinism can't see fine detail. Bianca can
make her way through a crowded room, but she
won't be able to see your face unless it's
close up. More mysterious than the glowing
fovea, though, is the way in which a lack of
pigment scrambles neurological pathways
between the eye and the brain. `The idea is
that nerves need to be guided,' explains
Richard Abadi, professor of clinical
psychophysics at the University of
Manchester Institute of Science and
Technology, 'and one of the guides is [the
pigment] melanin. But this is something
people have been working on for 20 years.
Science isn't like Hollywood. 'Nor is
albinism. Albinos have long been dismayed by
the film industry's tendency to portray them
as cartoonish evil-doers, most recently in
films such as The Time Machine, Star Wars
Episode 11: Attack of the Clones and Die
Another Day.
But not until this year, and Matrix
Reloaded, did the albinism community
finally - and politely - lose its temper.
That film's two white-haired, pale-skinned
characters known as the Twins prompted the
American National Organisation for Albinism
and Hypopigmentation (NOAH) to write to
Warner Bros, the studio behind the Matrix
films. There was no response, so they went
to the airwaves.
'The use of albinism as a literary device is
so cheap,' says Mike McGowan, the NOAH
president, 'yet they keep on doing it, again
and again.' When their objections reached
Jay Leno's Tonight Show, Warner Bros finally
reacted with denials. The Twins were
vampires, or ghosts, depending on the
interview. (A feeble defence: official
merchandise offers Albino Twins dreadlocks,
coats and sunglasses.) Many albinos believe
even fictional films have a drip-drip
effect. Mike McGowan says, 'we understand
that movies don't cause people to
discriminate, but this proliferation of
stereotypes can give rise to myths. Parents
of a child with albinism often find out that
doctors know very little about it. They look
at popular culture and think, "My kid's a
freak." Ignorance abounds in the unlikeliest
places.'
'I looked up albinism in a medical
textbook,' says Bianca. 'It said,
"Hereditary condition with a lack of
pigment"' Nothing about poor vision,
Nystagmus (where the eyes move
uncontrollably). No one in her family can
trace another case of albinism. When Bianca
was born (and before she was named after
next-door's cat), her parents spent three
days in hospital hearing the nurses call her
`Snowdrop', watching medical students troop
in to see the pale-skinned oddity. Only then
was a diagnosis of albinism given, without
explanation. 'I went to our local GP,'
remembers Mike. `He dusted off a file and
said, "I remember this from 26 years ago."'
The file belonged to another girl with
albinism, whose parents proved to be Mike
and his wife Carin's only source of
information.
Albinism has been recognised since Pliny,
who recorded seeing albinos on the west
coast of Africa. But 2,000 years of
intervening knowledge have yet to prevent
parents being told that their albino
children will be blind. 'Doctors look up
albinism; says Mike McGowan, 'and they see
"legally blind", and they'll decide the kid
can't see. People think we're haemophiliac,
that we're retarded, that we're blind and
deaf.'
Every week Bianca gets dozens of e-mails
through her website from people who have
just given birth to a child with albinism,
and don’t know what it means. The classic
screen villain probably has OCA1, like
Bianca. But there are all sorts. OCA2’s can
have straw-coloured hair and blue eyes.
Someone with ocular albinism probably has
normal pigment. 'People think albinism is
homogenous,' says Glen Jeffery, a researcher
and albinism expert at the Institute of
Ophthalmology. 'It's a very mixed bag.'
For these reasons, the Albinism Fellowship
was set up in 1979. Robin Spinks, its
president, thinks its primary mission is to
inform. He should `People think that we're
haemophiliac, that we're retarded, that
we're blind and deaf know: his parents knew
nothing about the condition when he was
born. Now the 33-year-old Scot could tell
them all they need to know: about an
autosomal recessive genetic condition which
means that two albinos can produce properly
pigmented children, about the fact that
albinism is a condition fairly easily lived
with, not a death sentence; and about how
not being able to drive, and taking care in
sunlight, are - name-calling aside - the
toughest things.
Robin is an optimistic person. 'My brother
and I are very similar, though he doesn't
have albinism. But I'm much more outgoing
than him. Maybe I had to prove myself more.'
He was the first person with albinism at his
university, and he was the one who got in
touch with the Albinism Fellowship, when he
was 19. `My parents said they wished they'd
known about it when I was young, because
they'd been gutted when I was born. When
you have a child you have this expectation
that this child is going to be a perfect
representation of you, and then you're
presented with this pale little bundle, and
you're not sure how well you'll do. It's
quite a lot to take in.'
Robin, president of the Fellowship for two
years, is used to the media spotlight. 'We
get 12 to 15 approaches every six months. I
like to know whether they have an agenda,
whether they want us to fit it.' He doesn’t
agree to all media approaches (and had to
put the request for this story to the board,
before he agreed to meet). And not the
ludicrous ones, like the James Bond
producers who phoned to ask him for an
albino to drive a car through an ice palace.
'I did say, "Do you know we cant drive? And
an ice palace? There's no contrast- it would
be impossible to see."
There's still no hard-and-fast test for the
condition. A hair bulb test - where a hair
is plucked and placed in a test-tube - can
detect an absence of tyrosinase (the enzyme
which produces melanin in skin and eyes),
but it doesn't work for all varieties of
albinism. Parents who've already had a child
with albinism can get subsequent foetuses
tested with amniocentesis, which tests cells
for the presence of albinism genes from both
parents. 'We need a battery of tests,' says
Richard Abadi, who specialised in Nystagmus
for ten years. 'Looking at whether the eyes
wobble, family history, brain signals -when
albinos look at a pattern, you can see that
the signals are scrambled.' But albinism
isn't a fashionable enough research topic
for anyone to be racing to find a definitive
test, never mind a cure. 'It's a benign
condition,' says Abadi, 'so only the crazies
do research on it.' The sane prefer to find
a cure for age-related macular degeneration,
the biggest cause of blindness in the
western world. Albinism research is trendy
now, again,' says Jeffery. 'But in the way
that owning a 1953 MG is trendy.' Jeffrey
receives e-mails from a parent who has read
that his work is linked with
'breakthroughs'. `But,' he says, `if I had a
child with albinism, I wouldn't get excited.
We can change the pigment in an embryo
mouse, but that's a long way from a cure.'
Sara Butler, who has Oculocutaneous
albinism, is 18, but has grown up fast. 'You
have to,' she says. `People think they've a
right to say things, like you're a public
oddity.' She dealt with it like Robin did:
by becoming an extrovert. 'My theory was if
I got in people's faces before they got in
mine, they'd leave me alone.'
I meet Sara in a London pub, where she's
having a drink with her close friend
Kristina Venning, 26. Sara and Kristina both
have OCA1 albinism with that white hair -
and both have Nokia 3510s, which Kristina
calls the `blindie phones' because they have
the best screens for poor vision. Kristina's
school days were hell. 'They weren't the
best times,' she says. 'But it's looking
back now as a happy, confident, sorted adult
- that's when I realised it was hideous.'
A few examples: a teacher, when she couldn't
read an overhead projection, telling her she
didn't belong there; another teacher who
made her stand up and read from Romeo and
Juliet in front of the class and, when she
held the book to her face, told her to get
better glasses (they don’t help with
Nystagmus or an undeveloped fovea). The
usual abuse: Mr Whippy, Father Christmas,
freak, monster. Every single day. 'It was
just my life. All the crap at school was
what I got. I expected it.' But it ground
her down. She turned monosyllabic, hunched,
unhappy. After an attempt to start
university, she dropped out and sat at home,
miserable.
Lara Forsyth didn't go through hell at
school, but she didn’t exactly have an easy
time of it. Now the PA to the assistant
director of the RNIB in Edinburgh, her
confidence is hard-won. 'I was in a maths
class. The teacher sat me at the back. I
couldn't see the board. I'd fail the yearly
test and drop to the bottom class, where I
sat at the front and I did really well. I
remember thinking, "Don't the teachers think
this is strange?"'
Even well-meaning teachers get it wrong.
Kristina tells of being in the science block
one day. Another class had been studying
genetic mutations or hereditary diseases.
'Everyone came up to me at the end and said,
"Oh, you can't have kids, then?" The teacher
had taught them that!' Sports was another
black hole. For Sara, rounders was the
worst. 'It was, "Well, somebody has to bat
for Sara, and the people on the posts call
her name when she's running round, and
everyone clap at the end because she got
around and isn’t she marvellous!" For a
15-year-old, that's extreme humiliation.
They were trying to normalise, but in a
really misguided way.' When Sara finally
told her sports teachers they were putting
her through `living hell' she was excused.
Lara dropped out of school with four
0-levels to become a professional showjumper.
She's about to go to university `to try
education again'. Kristina eventually went
back to university and had a fantastic time.
She's now a BBC researcher (and worked on
What Are you Staring at?, the recent
documentary on the maxillofacial surgeon Ian
Hutchinson). 'I got my confidence at
university and I built and built on it.'
Then she met the fashion photographer Rick
Guidotti, who used to photograph Cindy,
Naomi and Kate, until he got sick of being
told what beauty was. Today he runs Positive
Exposure, a group that raises awareness
about genetic conditions, principally
albinism. Guidotti's turning point came when
he spotted an African-American teenager with
albinism at a bus stop. 'She was laughing
with her friends, and she looked beautiful,'
he says from New York, where he's resting
after albinism-related trips to Fiji, New
Zealand and Polynesia. He'd never seen an
albino, or thought he'd seen one, and ran
off to look it up in `You have to grow up
fast. People think they've a right to say
things, like you're a public oddity' medical
textbooks. He was horrified. 'There were all
these pictures of people in their underwear,
standing up in surgeries. They looked like
they'd never had a slice of pizza, never
been kissed by their mother.' He wanted to
correct this the only way he knew how - by
taking pictures. 'I contacted NOAH, but they
wanted me to go away. They've had so much
exploitation and negative portrayals - I can
understand that:
Initially, it was hard to get portrait
subjects. 'Some people walk into the studio
with their heads down, monosyllabic,
shoulders hunched. When they see they're
beautiful, it's extraordinary. They grow 15
feet!' In 1998 Guidotti published portraits
of the unpigmented in Life magazine. The
next year, at the UK Albinism Fellowship's
biannual conference, he met Kristina. Her
self-esteem wasn't great- she didn’t want
her portrait taken. But Guidotti's charm
paid off, with Kristina and Sara. 'You think
I joke,' says Sara, `but that was the first
time anyone said, "You're beautiful."'
Besides a thick skin, those with albinism
must also develop a certain resourcefulness.
Lara didn't see why albinism should stop her
from being a professional show-jumper - she
had to calculate the distance to the jumps,
because she couldn't see them well. Or
there's Bianca, who was a talented gymnast
(until knee trouble), and who works
part-time in a pizzeria while she gets her
college qualification as a masseuse. She's
memorised the menu that she can't read
without magnification.
`There was a new manager who didn’t think I
could do the job. He said I wouldn't be able
to see when tables needed refilling. I got
him suspended. He's polite now.'
`The hate and abuse will never disappear
from my life,' writes Kristina on the
website. 'But what has disappeared is the
hate I felt for myself.' Sara concurs.
'The social problems have got easier. I deal
with it better.' (Often by giving the
curious a five-minute lecture on autosomal
recessive gene conditions.) But the past
year has been difficult - all her friends
were learning to drive, when she never will.
Being unable to study medicine - barred to
the visually impaired - can't have helped
either. Even so, she wouldn't change her
looks. 'I like-me! That sounds arrogant, but
I do.'
Sara can cope with the daily slathering of
SPF60 sunscreen, with the headaches and
nausea from sunburn, the way her hair goes
khaki from too much swimming-pool chlorine
('You smother it with ketchup.') And she
remains philosophical. She tells me about a
woman with albinism who's a 'life dodger'.
She worked on a checkout, but would drop
people's credit cards on the floor so she
could secretly read them with her
magnifier. 'Ridiculous!' says Sara,
laughing. 'It's easier to be upfront.
Because you know, even with everything,
albinism is an OK thing to have.'
By Rose George
© |
The word "albinism" refers
to a group of inherited conditions. People with albinism have little
or no pigment in their eyes, skin, or hair. They have inherited
genes that do not make the usual amounts of a pigment called
melanin. 
