Retinitis Pigmentosa
A Case Study
Teri deJong
SPE 516

Overview:

Pablo is a cheerful 14 year old boy living at home with his mother and 19 year old brother. He has limited verbal skills, medical concerns, and delays in all areas of development. Pablo has a medical diagnosis of Retinitis Pigmentosa. He was born in El Paso, Texas at 37 weeks gestation, weighing 7 pounds and 1 ounce. According to his mother, delivery was spontaneous and no problems were experienced during pregnancy. He had one kidney removed as a baby and has asthma. Based on past records and current evaluations, Pablo meets the criteria for the eligibility criteria of Autism, Visual Impairment, Other Health Impairment, Speech Impairment, and Multiple Disabilities.

He currently receives services on a separate campus for students with significant disabilities. He has attended this campus since the age of three years. He participates in a full day life skills program with an emphasis on personal care, motor, communication, social, and cognition skills. All areas of educational performance are affected byPablo's disabilities and his global cognitive delay. Pabloneeds supervision throughout his day, due to behaviors related to autism. He needs visual structure, assistance withpersonal care,and aspecialized academic program, presented through pre-requisite skills of the general education curriculum. Speech, Assistive Technologyand Visual Impairment services are provided for Pablo.

Pablo communicates using visual communication supports such as picture symbols and a voice activated output device. Pablo’s primary mode of communication is his verbal language, though his intelligibility is poor to the untrained listener unless it is within a context. He understands Spanish and English, but prefers English in school related tasks.

VISION:

Pablo’s mom started noticing that he bumped into things and had trouble finding dropped objects in 2004. In 2005 Pablo was diagnosed with Retinitis Pigmentosa (RP). The prognosis is permanent and progressive. No one else in the family has this condition, but both Pablo’s parents tested positive for the gene that produces RP. A Texas Eye Report was obtained dated 1/7/11,from Dr. Jeffery Hunter. Based on this evaluation Pablo continues to meet the eligibility criteria for the handicapping condition of Visual Impairment.

Texas Eye Report Results:

The eye exam report indicates that this student has a diagnosis of Retinitis Pigmentosa, (progressive retinal degeneration in both eyes causing poor night vision and narrowing field of vision beginning in childhood), Optic Neuropathy, (degeneration or abnormal optic nerve in both eyes) and Pathologic Myopia, (is an extremely high amount of nearsightedness that causes a major alteration of the shape or globe of the eye, which may lead to profound vision loss in both eyes). The prognosis is permanent for Retinitis Pigmentosa and optic Neuropathy with Pathologic Myopia being improved with glasses.

Based on the Functional Vision Evaluation and Learning Media Assessment conducted by Shelly Sumner on 1/9/12, Pablo is functionally blind (which means he requires tactile media as the primary tool in learning to communicate in both reading and writing at the same level of proficiency as other students of comparable ability).

Functional Vision Evaluation Results:

Pablo’s performance of tasks in a variety of environments requiring the use of near and distance vision are as follows:

A Precision Vision Distance Chart was used to test visual acuities at distance with glasses. His distance visual acuity was measured at 20/60 at 10 feet with both eyes while wearing glasses. His near visual acuity was not tested using a chart however he was able to see a 1cm candy of low contrast at 2 feet.

Visual Fields:

Visual field testing was done with Pablo by moving objects and lights into his visual field. He attended to lights in various visual fields however due to visual inattention and inconsistency it was difficult to determine the extent of peripheral vision. His strongest visual field appeared to be his central field and his weakest field appeared to be his lower field. His peripheral fields and upper fields were inconsistent.

Visual Abilities:

In order to get a better idea of the functional implications ofhis visual impairment, He was asked to locate a number of small objects at various points in space. He recognizes picture symbols but is not reading at this time. He was able to show visual discrimination by recognizing and matching 2 inch pictures. Size perception ability was not observed as visual sequencing skills were not present as he was not able to duplicate the order objects. A presence of visual skills needed for pre-academic print reading is evidenced through his ability to perform some of these skills. He was able to visually place objects in a container and to scribble while visually attending. This indicates the presence of some visual fine motor abilities needed for pre-academic writing skills. Further assessment of visual skills needed for academic performance was not administered due to Pablo’s present level of cognitive functioning.

Mid-Range to Distance:

Pablo can see projected images from overhead projectors, or on the white board. He is able to locate and identify a variety of targets in the school environment such as balls located across the gym at a distance of 15 ft. He was able to attend to 2 inch objects at 10 ft. in distance, to imitate gross body movement, to match and recognize 2 to 4 inch objects at distance of at least 5 feet. Tracking abilities at a distance of were demonstrated by Pablo in horizontal, vertical, and sometimes in the diagonal patterns. He was able to change focus from a very small pen light and toys within 6 inches to a ring and lights at 10 feet. The ability to track and recognize and match objects at a distance indicates the presence of visual skills necessary for board work activities in the classroom.

Outdoor:

Outdoor visual functioning was observed around on the playground. Lighting conditions included bright sunlight. Pablo had no difficulty in getting around independently. He could see swings at a distance of 15 feet; see various objects on the playground at 20 feet. He is able to adjust to lighting changes when moving from outdoors to indoors, and vice versa. He did not change gait, manifest a posture change, or shade his eyes to avoid the sun.

He wears glasses with transition lenses.

The student was not recommended for a clinical low vision or orientation and mobility evaluation.

Learning Media Assessment Results:

Visual learning:

Pablo, as a non-reader, uses oral instruction, visual stimuli, auditory prompts, pictures and picture symbols as his primary literacy media.

Auditory:

Pablo has no difficulty with the following audio visual equipment when it is used in the classroom. Based on the results of the learning media assessment, Pablo’s primary learning media include: verbal guidance, interactive smart board, computer,oral instruction and auditory prompts.

Tactual:

Pablo should use pictures, picture symbols, real objects, manipulatives and the interactive smart board with guidance and physical prompting as needed.

Pablo will not need braille.

Educational implications:

Pablo’s physical activities and mobility may be impaired when there is bad weather, or when lighting is low. He may need to move closer to instruction; high illumination with minimal glare on work area is helpful. Materials should be positioned in his central field. Pablo is easily distracted by sounds and movement around him. He does best when working in a quiet, not visually distracting area such a study carrel. He is at risk of retinal detachment.

Preferred Activities:

At home Pablo's favorite activity is playing on the iPad. His mother says this activity is very reinforcing and Pablo will comply with most anythingwhen rewarded with iPad time. On the computer, He enjoys using the touch screen for cause and effect activities as well as activities that require icon discrimination. Applications and programs that include music and high contrast graphics are preferred. Pablo is a sensory seeker in relation to movement, tactile, and visual input. He explores objects through touching, shaking, and moving them through space. Pablo enjoys bright, lighted toys and objects that spin or move back and forth. Pablo creates movement experiences for himself and enjoys rocking back and forth in a stationary or rocking chair, bouncing, and riding the adaptive trike.

Summary:

Currently, Pablo’s RP does not affect his daily living skills as much as his other health impairments, inability to take care of personal hygeine needs and attention deficits related to his Autism. According to his mother, she is most concerned with his chronic constipation and yearly asthma flare ups which have resulted in pneumonia and subsequent hospitalizations. His ability to communicate wants and needs, though limited, and use the iPad independently for enjoyment has sustained his quality of life. Since he will be under someone’s care for the rest of his life, his diminishing eye sight will be one more challenge for his caretakers to meet with the support of family and available community agencies.

References:

Ponce, Carla. Phone interviews. July 6 and 8, 2013

Full Initial Evaluation. February 7, 2013. Retrieved July 6, 2013 from ESPED. https://cambridge.esped.com.login/m.jsp