Albinism is a set of inherited disorders that result from the body’s inability to produce melanin pigment. Melanin is dark the pigment that protects our tissues from ultraviolet radiation. The formation melanin in the body is a multi-step process that may be affected by genes on six different chromosomes. Due to the many genetic variations causing this condition many different forms of albinism may occur. The most severe form of albinism presents with little or no pigmentation of the skin, hair and eyes.
It presents itself in individuals with white / platinum hair, pink skin and often a pinkish eye color. Other forms of albinism may affect only the eyes. These individuals will present with many of the eye and vision problems related to albinism, but have normal skin and hair pigmentation.
Photophobia: In albinism, failure of the eye to develop pigmentation results in extreme light sensitivity. Thus the patient may not only have too much light entering the eye, but also have no way to handle the excess stray light once it enters.
Reduced Vision & Macular/ Foveal Hypoplasia:Patients with albinism have reduced visual acuity primarily from an underdevelopment of the center of the retina
Nystagmus: It is often the first sign that parents recognize when an infant has vision loss It is crucial that family and teachers allow a child with albinism to tilt or turn their head or eyes to the position that lets them see the best. When bifocals are prescribed, the low vision specialist must select the type and position that works with the patient’s null position.
Sunburn: Patients with albinism are facing the risk of severe sunburn. Children must be taught to protect their skin. Sunscreens and adequate clothing are essential. Sun may pass through clothing, especially wet clothing, and burn the skin. Increased risk of sunburn occurs at higher altitudes, and may occur at lower latitudes around reflective surroundings such as sand and water.
Stable Condition: Albinism is a stable condition. In fact, some patients show slight improvement in visual acuity by the time, they are young adults. This may be related to increased pigmentation or a better control of the nystagmus.
Emotional Aspects: Patients with albinism must deal with the emotional trauma of appearing different. Pink skin and eyes, white or platinum hair, unusual head or eye null positions, thick eyewear and the need to hold reading material very close unfortunately cause these patients to stand out more.
Fluctuating Vision: A patient’s nystagmus often fluctuates when under stress or when tired. As the nystagmus increases, the vision may blur further. Children with nystagmus may not do well with timed tests as they may be under stress. Even covering one eye may cause a change in the vision of the viewing eye due to an increase in Strabismus .The loss of binocular vision is common in albinism and this result in reduced depth of perception.
Low Vision Care for Albinism
Patients with albinism are excellent candidates for low vision care. Albinism is a mild to moderate, stable central vision loss. These patients have excellent residual side vision. Simple magnification, correction of refractive errors, light and glare control and use of adaptive aids may all benefit albinism patients.
Refractive Error: Albinism usually results in significant amounts of farsightedness or near sightedness combined with astigmatism. Glasses cannot fix low vision, but they do ensure that the person’s eyes see the clearest image possible and can easily focus at close distances. Refracting the nystagmus patient requires special techniques to reduce stress and avoid increasing the nystagmus. Children with albinism should be carefully refracted by the low vision specialist at an early age.
Contact Lenses: Contact lenses can be used to correct the refractive error (myopia, farsightedness and astigmatism) of an albinism patient and in some cases an improvement in visual acuity is obtained. One theory is that the tactile feedback of feeling the contact lenses on the eyes may lead to better control of the movement and allow the patient better vision. Another benefit of contact lenses is that they move with the eyes and provide better image quality.
Close Reading Distance: A loss of visual acuity mild to moderate, an albino child may need to hold the materials closer. Parents and teachers often worry about the close distance, but the child should be allowed to read at the most comfortable viewing distance where they see the best. Most albinism patients benefit from bifocals to reduce the strain of focusing at such a close distance. Magnifiers, strong reading glasses and closed circuit television systems may be helpful.
Filters & Sun Protection: Tints, selective filters and hats may aid the albinism patient. The low vision specialist must consider the need for adequate filters both inside and out. Sunlight may be 100 times brighter than inside light. Filters for general wear are usually not adequate for outside wear. Adequate education on the use of sunscreens, clothing is necessary for all albinism patients
Low Vision Devices
There are different low vision devices to help improve the lives of people with albinism. To get the most from a low vision device, it’s important to see and understand what the device can and cannot do. Since different devices help with different tasks, the person with low vision will likely choose a mix of different devices to accomplish all of his or her goals.
.Distance vision devices
Telescopes can help people with low vision improve their distance vision. Distance vision includes seeing a chalkboard in a classroom, a sporting event. Telescopes are differentiated from one another by their magnification power and the field of view. Typical magnification powers for low vision telescopes range from 2x to 4x. When a person with 20/100 vision uses a 4x telescope, that person theoretically sees an image 4 times bigger than normal. However, as magnifying power increases, the field of view, or the size of the area enlarged by the telescope, generally decreases.
Hand-held telescope: This is one of the most common low vision devices, also called a monocular. Hand-held telescopes come in a wide variety of sizes, magnification powers and prices. Hand-held telescopes work best to quickly view a distant object, or to perform tasks such as reading a sign or locating an object. Clip on telescopes allow the user to slip the telescope over his or her glasses for hands-free use. The clip on telescope works well for the person who needs to use the telescope for extended time periods, such as for watching TV, a movie, or a live stage performance.
Near vision devices
Reading glasses and hand-held and stand magnifiers are near vision devices that help people with albinism read, look at pictures, diagrams, and maps, and accomplish other tasks that require seeing small details up close. Reading glasses help the user focus on text or other objects while holding the object close to the user’s eyes. Reading glasses allow the widest field of view for reading compared to other devices. Hand-held and stand magnifiers enlarge close up images, allowing the user to see small print and images at greater distances from the user’s eyes.
Electronic devices: When traditional optical low vision devices don’t help a person accomplish a task, electronic aids might help. Closed Circuit Television (CCTV) systems help people who need greater magnification than reading glasses and magnifiers. CCTV systems also allow the user to adjust the size, brightness, and contrast of the magnified image to best match the user’s vision. The user can even read white letters on a black background to decrease glare. CCTV systems are expensive and far less portable than other near vision aids. However, CCTV is now smaller, more versatile, more portable, and less expensive.
Contrast enhancement devices: For people with albinism, bigger isn’t always better. Increasing the contrast of text is often more effective than increasing the size of text. Black felt-tipped pens and dark lined paper can make writing easier for some people with low vision. Writing guides, which are templates with open areas where one writes, can help people write in straight lines. Colored filters can make it easier to see certain colors. For example, a yellow filter can make light blue letters appear darker and easier to read. Lighting plays a major role in how well a person with low vision sees. Experimenting with different types and brightness of light, as well as the location of the light, can make tasks a lot easier. One needs to keep in mind that too much light and too little light can both cause problems for low vision person with albinism