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Bioptic Low Vision Aids for the Visually Impaired

Albinism - Improving Vision for Individuals with Albinism

  Macular Degeneration Stargardt's Disease 

What is Albinism?


Individual with Oculo-cutaenous Albinism

Albinism occurs when one of several genetic defects makes the body unable to produce or distribute melanin, a natural substance that gives color to hair, skin, and the iris of the eye. About one in 17,000 people have Albinism.

The defects may be passed down through families.

There are two main types of albinism:

  • Type 1 albinism is caused by defects that affect production of the pigment, melanin.
  • Type 2 albinism is due to a defect in the "P" gene. People with this type do have a slight amount of skin coloring at birth.

The most severe form of albinism is called oculocutaneous albinism. People with this type of albinism have white or pink hair, skin, and iris color, as well as vision problems.

Individuals with albinism often have
pale blue eyes and white eyelashes

Courtesy University of Arizona, Department of Ophthalmology and Vision Science

Albino eyes will appear very
red when a light is shown into them.

Courtesy Scottish Sensory Centre, University of Edinburgh


Another type of albinism, called ocular albinism type 1 (OA1), affects only the eyes. The person's skin and eyes retain some color, however the retina will be affected.
 

Eye Symptoms

Albinism is associated with the following visual difficulties:

  • Reduced Visual Acuity
  • Light sensitivity (Photophobia)
  • Rapid eye movements (Nystagmus)
  • Misaligned eyes (Strabismus)

 

Reduced Visual Acuity: Individuals with albinism do not have clear vision due to an underdevelopment of the central part of the retina called the macula. The macula is responsible for sharp, detail vision which works most well in bright light.  The retina is very pale because of the lack of pigment.

 Fundus in type IA oculocutaneous albinism  Normal Retina

An albino retina is very pale
because of the lack of pigment

Courtesy University of Arizona, Department of Ophthalmology and Vision Science

This is a diagram of a normal retina

Image © Ocutech, Inc.


Photophobia: Since eyes with albinism have insufficient pigment (melanin) to effectively absorb light, sunlight and brightly-lit environments may be uncomfortable and even painful and cause even more difficulty seeing. Individuals are often more sensitive to light coming from the sides and overhead.  


Individuals with Albinism often have high light sensitivity called Photophobia

Courtesy DermAtlas, Johns Hopkins University

 

Nystagmus: In forms of vision loss that are present at birth (congenital) it is common for individuals to have an involuntary back and forth movement of the eyes called nystagmus. Individuals with nystagmus do not see the world moving, nor will their eyes appear to be moving when they look in a mirror.  The only way they will notice their own eye movement will be if they see themselves in a film or video. Studies have shown that stopping nystagmus does little to improve vision.  Individuals with nystagmus usually respond very well to telescopic vision aids.

The Null Point:  Individuals with nystagmus will have some position of their eyes where there is the least amount of movement, called the Null point.  When the eyes turn away from the null point nystagmus often increases. While the null point is often straight ahead, sometimes it can be off to one side or above or below, causing the individual to have a head or eye turn that may appear unnatural but that provides their best vision. Children especially should be encouraged and allowed to assume whatever posture they feel provides their best vision.

Strabismus (misalignment of the eyes): It is very common in albinism to have either an inward (Esotropia) our outward (Exotropia) turning of the eyes.  Individuals will use their preferred, dominant, eye.  As the eyes do not work together depth perception (stereopsis) is absent. 


Individual with an in-turning right eye called Esotropia

Courtesy David Garrick

Prognosis: Albinism is a stable condition and does not worsen with age. 

Low Vision Care: Patients with albinism are excellent candidates for low vision care and respond well to telescopic low vision aids.  

Eyeglasses: Individuals with albinism almost always have high degrees of astigmatism (meaning the eye is out of round).  They may also be farsighted or nearsighted.  Often, despite measurements showing that there is a high prescription, individuals don’t find that conventional eyeglasses significantly improve their vision and often choose not to wear them.  Not wearing glasses usually causes no worsening of the eyes.


Young boy with glasses for farsightedness and astigmatism.
Notice how the eyes look enlarged behind the eyeglass lenses.

Courtesy PBWorks


Contact Lenses: 
Since albinos eyes move back and forth and as a result do not look consistently through the center of an eyeglass lens, the individual may not benefit fully from their optical correction.  Since contact lenses rest on the eye and move with the eye, the individual is always benefiting from the best optical result. In individuals with high amounts of myopia,  the contact lens will magnify the image a small amount which can also be helpful.  There is some possibility that the lens itself may reduce the amount of nystagmus.  Since there are usually high amounts of astigmatism, albinos often require rigid gas permeable (RGP) or toric soft contact lenses.

Distance Vision:  As eyeglasses by themselves usually do not offer adequate vision improvement for the individual to engage in normal visual activities, they have two remaining choices -- either move close enough to see the object of regard, or bring it closer using miniature telescopes or binoculars. Albinos respond very well to telescopic magnification which can be provided as a handheld device (monocular) or a pair of eyeglasses with the telescope attached (bioptic telescope) which allows them to be hands-free.  Bioptic telescopes are useful in the classroom, in social and work settings and in appropriate situations can enable the individual to be able to drive.

Ocutech VES-Sport in pink

Ocutech VES-Sport Bioptic Telescope
(In HOT pink...a favorite of albino girls!)

 

Reading with Albinism

Remarkably, despite often having significantly reduced vision at distance, individuals with albinism can frequently read very well simply by holding the material close to their eyes.  While this may be disconcerting to family and teachers, there is nothing wrong nor can any damage result.  Short working distances can be fatiguing and efforts should be made to address ergonomic issues to support posture including inclined reading stands.  Strong power lenses either as just reading glasses or as a bifocal can make reading less strenuous, especially with smaller print and extended reading demands. Optical and electronic magnifiers (CCTVs) are also available that can assist reading activities. Software to enlarge the print on the computer screen can also be very helpful.


Helping Albino Children in the Classroom

Review our "10 ways to support children with low vision in school"
 

Additional Resources

 

  Macular Degeneration Stargardt's Disease 

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