Albinism: An Introduction
Overview of the Genetic Condition's Scientific and Cultural Roots
What is Albinism?
Albinism is set of inherited conditions that prevent processes essential for the production of color in organisms such as chlorophyll in plants and the dark pigment melanin that, in people, gives color to the skin, hair, and eyes and provides protection from ultraviolet light.
Recessive genes (present in about 1 in 70 people) carried by both parents cause albinism, which occurs approximately once in every 17,000 births. Albinism impedes the enzyme tyrosinase from converting tyrosine, an amino acid, into Dihydroxyphenylalanine (DOPA), a chemical essential for producing melanin.
Facts About Albinism, available on the International Albinism Center’s website, provides a succinct introduction to the condition’s medical and genetic aspects.
Appearance
Most persons with albinism have white or light hair, blue or gray eyes, and light skin. Most parents of children with albinism have normal hair and eye color and no family history of the condition. Albinism affects people of every nation, ethnicity, and religion.
An enduring myth is that people with albinism have red eyes. Lacking melanin, the highly vascular pupil can have a reddish tinge in the right light, and photographic “red-eye” is common, but iris color ranges from dull gray to brown.
Visual Defects
Visual defects associated with albinism classify it as a disability. Most with the condition are legally blind, with best-corrected acuity below 20/200. Albinism causes abnormal nerve pattern development between the eye and the brain. Lack of pigment in the retina (the part of the eye that perceives light and transmits signals to the brain) is believed to hinder development of the fovea, the portion of the retina that provides sharp, clear vision. Visual defects associated with albinism also include Nystagmus (involuntary eye movements) and photophobia (light sensitivity).
Living with low vision is albinism’s primary challenge, affecting education (especially reading), socialization (including sports participation and peer acceptance), and mobility. Most obstacles can be overcome with technology and adaptive aids, such as recorded texts and large print books, and bioptic lenses (small glasses-mounted telescopes) that enhance visual acuity, enabling many with albinism to obtain drivers’ licenses.
One of the largest advocacy organizations for persons with albinism and their families is the National Organization of Albinism and Hypopigmentation (NOAH), which offers support and information on all aspects of the condition through its regional chapters, conferences, and its website.
Albinism in Culture
Albinism is deeply etched in the imagination and has been mythologized or vilified in culture from ancient times (Zeus incarnating as a white bull and depictions of Jesus in Revelation) to the cloaked killer Silas in Dan Brown’s 2003 novel The Da Vinci Code.
Until the 1920s, it was common for persons with albinism to be exhibited as curiosities and sideshow freaks.
A catalyst for albinism’s ongoing use as a symbol of evil is Herman Melville’s 1851 novel Moby Dick, in which the narrator, Ishmael, details the dread felt when facing unrelenting whiteness, calling it the “crowning attribute of the terrible” and “the colorless all-color of atheism.”
Albinism is later used to infuse evil in many famous novels such as H. G. Wells Invisible Man, Jamaica Inn by Daphne du Maurier, and James Dickey’s Deliverance.
The condition has long been used widely in film to create villains that invoke our deepest fears—stalkers, soulless assassins, characters who are hideous, relentless, and impervious to pain. Famous examples include You’re a Big Boy Now (1966), The Omega Man (1971), The Eiger Sanction (1975), Foul Play (1978), Stick (1985), and Disturbing Behavior (1996).
On television, the word “albino” has long been a one-word punchline.
The word “albino” (considered derogatory by persons with albinism) warrants a spell-check entry in the Associated Press Style Guide, yet those with the condition are so few that writers can, with impunity, exploit its characteristics to amuse or appall, despite a decades-old disability movement and society’s current climate of hair-trigger humanism.
Albinism predates language and society and is still used to tap our deepest fears, making it the first prejudice, and the last.
