Retinitis Pigmentosa

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Retinitis Pigmentosa

Question:

My 5-year-old niece was just diagnosed with the eye disease called retinitis pigmentosa. What is it?

Answer:

Retinitis Pigmentosa (RP) is an eye disease that causes damage to the retina (back part of the eye). Unfortunately, the damage tends to become worse over time.

There are parts in the eye called rods that control night vision. When a person gets RP, these rods are involved. In addition, peripheral (side) vision could be lost over time if not treated. This may even lead to partial blindness and loss of central vision in advanced cases.

RP is a common genetic disorder that may run in the family. It should not be a huge surprise if there is not any family history though. Parents with normal vision may each carry this gene and then both pass it on to their offspring.

Make sure your niece has regular visits to an eye specialist (ophthalmologist). He or she can screen for pigment changes inside the eye, as well as cataracts (clouding of the lens) or glaucoma (pressure buildup inside the eye).

I do not know how or why this diagnosis was made for your niece.

Sometimes, RP is associated with other conditions. I'm sure the eye specialist treating your niece will check for them.

There is no one effective treatment that can completely get rid of RP. But, there are some things your niece may be advised to do. For example, wearing sunglasses can protect the retina from sunlight. Antioxidants like vitamin A may slow the disease.

In the meantime, other family members, especially those with children, should consider genetic counseling. It is always better for RP to be known early on.

Henry (Hank) Bernstein, D.O. is a Senior Lecturer in Pediatrics at Harvard Medical School and Professor of Pediatrics at Dartmouth Medical School. Formerly the Associate Chief of General Pediatrics and Director of Primary Care at Children's Hospital Boston, he currently is the Chief of General Academic Pediatrics at Children's Hospital at Dartmouth. He has extensive and varied experience as a primary care pediatrician, and is a spokesperson for the news media on a variety of pediatric health care topics, including vaccination, common childhood illnesses, and practical information for caregivers.

Last updated:	July 20, 2009