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albinism

Written by: Prof. Kent Caslo, Professional Director of ELIA, Developmental Optometrist.
General background
Albinism is an inherited congenital disease, characterized by a complete or partial deficiency of a pigment (a colored substance called melanin) that appears on the skin, hair and eyes. There are two main types of albinism; For ocular albinism (lack of pigment only in the eye) and for general albinism (throughout the body). Side effects of albinism are hypersensitivity to light, nystagmus (shaking of the eyes) and sometimes a lazy eye. If albinism is general, there is a higher risk of skin cancer and sunburn. It should be noted that except for effects on vision and exposure to the sun, albino has no other effects and does not impair physical or cognitive development.
 
Visual impact
The development of the visual system in the fetal period is highly dependent on the melanin material essential for retinal and iris development. The retina is located in the inner part of the eye, consists of cells (receptors) and nerve fibers – their function is to receive light rays and transmit an electrical message to the brain. The iris regulates the amount of light entering and contributes to visual acuity. Melanin deficiency leads to impaired development of the retina and iris properly and therefore there is a decrease in the quality of vision.
Possible effects of melanin deficiency:

  • Hypersensitivity to light (photophobia) Decreased visual acuity due to the way light is scattered in the eye.
  • Decreased visual acuity (blurring) due to immaturity in the development of the clear visual center in the eye – the phobia.
  • Nystagmus (flicker of the eye) which also contributes to a decrease in visual acuity.
  • Decreased communication between the eye and the brain due to a change in the organization of the optic nerve fibers on its way from the eye to the brain and therefore a change in the message that the brain receives from the eyes.
  • Underdevelopment of the optic nerve head within the eye.

Genetics
Generalized albinism (OCA) is usually inherited recessively from both parents, meaning that the parents are not albinos themselves but carriers of the disease gene. Ocular albinism (OC) is also inherited, more common in men than women. There are other types of albino but they are rarer.
Coping and treatment

  • As much as possible, use sunscreen to protect the skin of the body and the poor.
  • It is important to use sunglasses / dark glasses to protect the eye and prevent glare.
  • Proper choice of seating in the garden to prevent glare and make optimal use of vision.
  • Encouraging eye contact with the environment as this contact is affected by the decrease in visual acuity and the ability to direct the gaze.
  • Use of contrast (contrast) in colors Since along with the decrease in visual acuity there is also a decrease in sensitivity to contrast.
  • Development and encouragement of vision skills while addressing the characteristics of nystagmus (jitter). Tracking objects in motion, finding the right position in favor of the “zero point” (the position of the head and the angle of view in which the tremor in the poor decreases), providing time for identification and decoding and more..

For more information and how to treat preschool children with albinism and ways to work with children with visual impairment or blindness, visit the professional articles and instruction bookletsבאתר ELI-Association for the Advancement of Children with Visually Impaired or Blindness .

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