Transcript Retinitis Pigmentosa SPE 516 Group Project Micha Munro

Presented by
Chino Anyanwu
Lynn Baudoin
Mona L. Bell
Emily Diaz
Wendy Lewis
Kathy Sanders
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Understanding Retinitis Pigmentosa
Common Problems of Retinitis Pigmentosa
The Effects of Retinitis Pigmentosa on the Visual System
Treatment of Retinitis Pigmentosa
Medications used when treating Retinitis Pigmentosa
Surgical Care when treating Retinitis Pigmentosa
Functional Implications of Retinitis Pigmentosa
Low Vision Aids for People with Retinitis Pigmentosa
Americans with Disabilities Act
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Retinitis Pigmentosa (RP) is also known as
Rod/Cone Dystrophy.
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RP affects 1 in 3,500 people in the United States.
Worldwide, RP is thought to affect roughly 1
in 5,000 people.
RP is defined as a hereditary and progressive
group of disorders that gradually leads to visual
field loss and retinal degeneration.
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The name “retinitis pigmentosa” refers to what the retina looks
like in individuals diagnosed with RP.
When viewed with a specialized tool, the RP retina often shows
clusters of pigment that are not seen in a normal retina.
The word “pigmentosa” refers to the pigment deposits seen
during the eye exam.
The term “retinitis pigmentosa” refers to a group of eye
conditions that affect the retina, or the layer of nerve cells at the
back of the eye.
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Tunnel Visual Field -
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Night Blindness -
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Sensitivity to Light & Glare-
Progressive loss of visual fields is a
characteristic of RP. The visual field loss often begins as a donut-like ring in
mid-periphery. As it progresses both centrally and peripherally, the resultant
tunnel vision begins to affect the patient’s activities, driving and mobility. In
severe tunnel vision cases, the patient may report seeing “two separate
world.”
Another characteristic of RP is night blindness.
It may be the first presenting sign.
When RP patients go into bright
sunlight, they are often overwhelmed by “white-out” debilitating glare. They
are aided by the use of dark plum and amber filters used often with side
shields. RP patient also frequently report glare problems inside. Many find a
light amber filter in their general wear eyeglasses improves their tolerance. A
variety of filters are available.
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Double Vision -
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Cataracts -
Tunnel vision interferes with the
brain’s ability to accurately control alignment of the eyes. To
treat the resultant double vision, prisms and orthoptic therapy
may be used.
RP patients frequently develop a form of cataract.
It may reduce visual acuity and increase glare.
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Decreased Visual Acuity -
A portion of RP
patients, will experience a loss of central vision. The macular
area may deteriorate making it difficult for the patient to see
detail as in seeing a faces or reading. Simple magnification and
increased task lighting may be helpful.
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A person with RP may experience:
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Lighting will be a major factor in visual
functioning.
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Low vision aids may be necessary as vision
reduces.
a reduction in depth perception
difficulty with discerning fine details
difficulty entering or leaving different
environments due to slow adjustment to light
changes
a reduction in peripheral vision
Normal Vision
Tunnel Vision
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Presently, there is no therapy available for the vast majority
of individuals with RP.
Attention is directed towards low vision aids, and proper
social and governmental programs for the visuallyimpaired.
Because much remains unknown about RP conditions,
research is very active in this area.
While therapies are limited, physicians should emphasize
the therapies that are available and as new therapies
emerge, routine evaluation can keep patients informed of
clinical trials and new treatments.
Some research indicates that daily use of microcurrent
stimulation may help preserve vision.
Medication
Medical Benefit
Acetazolamide
Useful in reducing macular
edema, which can reduce vision
in the later stages of RP
Corticosteroids
May be useful for macular
edema but has not been well
studied
Lutein and Zeaxanthin
Useful in protecting the macula
from oxidative damage
Vitamins A&E, Omega-3
Fatty Acids, other
antioxidants, and COQ10
May help slow down RP and
preserve or even improve vision
Cataract extraction
can often be
beneficial in the later
stages of RP.
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Most cases of retinitis pigmentosa are
inherited. However, some people develop
the disease even if they have no family
history. Others may develop the
condition as part of another disorder.
Signs of RP can usually be detected
during a routine eye exam when the
patient is around 10 years old. However,
symptoms usually do not develop until
adolescence.
Although the disease worsens over time,
most patients retain at least partial
vision, and complete blindness is rare.
It is perfectly natural for someone with
vision loss to feel anxious, fearful, angry,
or unhappy. It is also natural for them to
have feelings of uncertainty, anxiety, and
fear over what the future may hold.
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Most adults describe living a full and
interesting life despite the practical and
social problems that can arise because
of RP.
Although many individuals with RP are
diagnosed in childhood, some may not
be diagnosed until they are adults.
Others might not have a need for low
vision adaptation until they are adults
with established careers. When this
happens, they may feel as though they
can no longer perform their job
functions. However, individuals with RP
can and do have a wide variety of jobs,
from computer programmer to
musician, and often find it unnecessary
to change careers because of RP.
Some people may benefit from using special lighting to
complete their daily tasks. There are also devices and
programs that make it easier to use computers. Some
computer-based programs can act as magnifiers, or even
convert text to audio formats. Patients with very late-stage
disease are often helped by learning how to use a cane to
find objects or curbs in their path when walking. They may
also find a guide dog helpful. Some patients ask if it is
necessary to learn Braille. Only a small number of patients in
very late stages of RP need Braille for reading.
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The Americans with Disabilities Act gives
civil rights protections to individuals with
disabilities, such as low vision due to RP.
Its policies prevent employment
discrimination and allow equal access to
education, healthcare, and public
transportation.
For more information, you can call 800514-0301 or visit www.ada.gov.
Resources:
Texas School For the Blind: tsbvi.edu
American printing House for the blind: aph.org
The Nova Clinic of Houston: opt.uh.edu/uei/services/pediatrics.com
References:
Wikipedia. (2010, April 3).Cone Dystrophy. Retrieved July 5, 2010, from
http://en.wikipedia.org/wiki.conedystrophy
Eye Conditions and Other Retinal Diseases. (2004-2010). Cone-Rod Dysrophy.
Retrieved July 5, 2010, from
http://www.blindness.org/index.php?view=article&catid=38%3Aother-retnaldiseases&id=...
Selected Anomalies and Diseases of the Eye. (1990). Cone-Rod Dystrophy.
Retrieved July 5, 2010, from http://www.tsbvi.edu/education/anomalies/nystagmus.
htm
University of Edinburg. (n.d.). SSC Scottish Sensory CentreRod-Cone Dystrophy.
Retrieved July 5, 2010, from
http://www.ssc.education.ed.ac.uk/resources/vi&multi/eyeconds/RcDyst/html
American Academy of Ophthalmology: Berkley. (2006). High Resolution Retinal
Imaging of Cone –Rod Dystophy. Retrieved July 5, 2010, from
http://www.berkely.edu/imagingofroordalab/pubs/wolfing.pdf