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Stargardt's Disease - Inherited Vision Loss

Stargardts Disease What is Stargardt's disease?
Stargardt's disease causes progressive vision loss in children and young adults. It was first reported in the early 1900s by Karl Stargardt, a German ophthalmologist. Over 25,000 people in the United States have this condition, and as many as 1 in 10,000 children may be affected. Stargardt's disease is sometimes also called fundus flavimaculatus, though some researchers believe this to be a variant of the disease.

Stargardt's disease causes damage to the macula (macular dystrophy), which is the center area of the retina responsible for our finely-detailed, central field of vision. Some of the surrounding retina is also affected. The disease destroys central vision, but usually does not cause total blindness; peripheral vision is left intact.

Researchers believe that Stargardt's disease is caused by a mutation of a gene called ABCR. The condition is inherited with 90% of cases showing recessive inheritance (a copy of the defective gene must be received each parents), though occasionally dominant inheritance is seen (only one copy of the defective gene is needed to cause the disease).

Symptoms and progression of the disease
Stargardt's disease usually begins causing damage and vision loss between the ages of 6 and 20, and the condition is usually diagnosed by age 20. Occasionally, vision loss will not be noticeable until later in life, and the patient will not be diagnosed until their 30s or 40s.

Vision is often near normal in the beginning. One of the first signs of a problem may be mild blurry vision that is not fixable with glasses. Vision loss progresses slowly until the patient has 20/40 vision, but once that point is reached, vision will rapidly deteriorate until it is 20/200 (legally blind).

Even though the disease is genetically inherited, there can be variation in the progression of symptoms among family members. For example, one sibling may begin experiencing vision loss in early childhood, while another does not have problems until their 20s.

Symptoms include:

  • Spots in vision
  • Blurry vision
  • Blind spots, which get larger in size as the disease progresses
  • Light sensitivity
  • Difficulty adjusting from a bright light to a dimmer room
  • Difficulty recognizing faces
  • Decreased color vision, in the later stages of the disease
  • Phantom vision/hallucinations

Diagnosis
In the beginning stages of the disease, Stargardt's is sometimes difficult to diagnose, but over time, will become more evident during ophthalmoscopy. The doctor will be able to see signs of damage to the retina, as well as the accumulation of small yellow-white flecks which are a hallmark of the disease.

Other tests which may be performed to confirm Stargardt's disease are fluorescein angiography, electroretinography, and electro-oculography.

Living with Stargardt's disease
There is unfortunately no cure for Stargardt's disease, and very little that can be done to slow its progression. Studies in mice have shown that exposure to light may play a role, so sunglasses with 100% UV protection should be worn whenever possible. Some researchers believe that vitamin A supplements should be avoided, as this vitamin may also contribute to the condition.

Mainly, children with Stargardt's will need help dealing with everyday tasks (such as schooling) and coping with the emotional aspect of losing their vision. Large print books and magnification devices can allow children with vision loss to read more easily and keep up with their classmates. If glare or light-sensitivity is a problem, the child should be seated away from the windows, or curtains can be used. Children may need counseling to help them face their fears and understand their vision loss. Support groups and meeting other Stargardt's patients may also be helpful.

If the child wishes to participate in sports, special precautions will need to be taken. Sports like swimming or track, where contact is minimal and vision less critical, are ideal choices. Sports that involve fast-moving objects, such as baseball or hockey, can sometimes be played successfully, but a face shield should be worn to prevent injury.

Teachers and friends should be educated on the specifics of Stargardt's disease. Many times, the child will be unable to clearly see a large object in front of them, but will be able to see smaller objects well with their undamaged peripheral vision. This can lead to the misconception that the child is faking or exaggerating his or her condition.

Progression of the disease will vary somewhat from person to person. Your child's eye doctors, teachers, and counselors can advise you on how best to help your child.

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