Successful use of an eye gaze AAC communication board by a young adult with advanced Sanfilippo Syndrome (MPS IIIA): Case report.

ABSTRACT

Sanfilippo syndrome (Mucopolysaccharidosis Type III or MPS III) is a family of rare, lysosomal disorders characterized by progressive cognitive and motor deterioration. Even though individuals with MPS III present with complex communication needs, research regarding augmentative and alternative communication (AAC) in this population is scarce. While life expectancy for individuals with MPS IIIA typically does not exceed 20 years of age, this case report involves a 22-year-old adult with postregression MPS IIIA. Prior to this study, the participant could not communicate using speech and only responded to yes/no questions using eye blink responses. The participant was given a low-tech AAC system utilizing eye gaze so that she could respond to a variety of caregiver questions and take conversational turns. The following communication outcomes were measured during each session in which caregivers used the AAC system number of eye gaze responses, total number of responses (using any means), the percent of responses to questions asked, and the total count of expressive vocabulary words available to the participant with the AAC system. Increases were observed in the number of eye gaze responses per session and in the expressive vocabulary accessible via the eye gaze board. A higher percentage of responses given caregiver questions was noted for the intervention sessions (71%) compared to the baseline sessions (55%). There were also qualitative changes characterized by the types of questions the participant could respond to during conversational exchanges. Despite the progression of MPS IIIA, the results suggest that use of the eye gaze board resulted in quantitative and qualitative changes in functional communication. This case report provides preliminary evidence that AAC can improve communication in a young adult with postregression MPS IIIA.

Successful use of an eye gaze AAC communication board by a young adult with advanced Sanfilippo Syndrome (MPS IIIA) Case Report This article reports a case of a 22-year-old woman who was diagnosed with mucopolysaccharidosis type III (MPS III) or Sanfilippo Syndrome. MPS III is a rare lysosomal disorder characterized by progressive cognitive and motor deterioration. Children with MPS III experience regression in speech and communication skills and speech is typically lost by age eight years. AAC (Augmentative and Alternative Communication) are systems or devices that can be used by individuals with limited to no speech to aid or supplement communication. Even though individuals with MPS III have significant impairment in communication and could potentially benefit from AAC, research regarding the use of AAC (Augmentative and Alternative Communication) by this population is scarce. In this case, an eye-gaze AAC system was introduced to a young adult with postregression MPS IIIA who is well beyond average life expectancy for this disease. Despite the progression of MPS IIIA and complicating medical issues, there were quantitative and qualitative changes and improvement in this woman's functional communication after the eye gaze board was introduced. This case study provides preliminary evidence that AAC use can potentially improve communication in individuals with postregression MPS IIIA.