Communication skills among children with spinal muscular atrophy type 1: A parent survey

ABSTRACT

Spinal muscular atrophy is one of the most common fatal autosomal recessive disorders. Children diagnosed with SMA Type 1 (SMAT1) demonstrate severe oral motor weakness and flaccid dysarthria progressing to complete anarthria. A review of literature illustrates that little has been described regarding augmentative and alternative communication (AAC) use among these children, although communication has a critical impact on quality of life and participation in daily activities. Responses to an investigator-developed parent survey were obtained to appraise communication skills and opportunities among children diagnosed with SMA1. Results illustrate parent perception of greater receptive than expressive language ability and highlight the benefits of implementing speech-generating devices (SGD). Barriers to SGD acquisition and implementation, including access and funding, are reported and described. Overall, families indicated that SGD increases quality of life and provides valued improvements through expanded functional communication.

KEYWORDS:

• Access
• assistive technology
• augmentative and alternative communication
• funding
• speech generating device
• spinal muscular atrophy
• survey

Acknowledgments

The authors wish to thank the families with children diagnosed with SMA who contributed their time and experiences to this study. This study acknowledges the collaboration of the International Spinal Muscular Atrophy Patient Registry (Indiana University) which is supported by Families of SMA. The authors wish to thank the Hearing and Speech Center Board at Children’s National Health System for their generous support of this research.