Use of speech-generating devices (SGDs) improves the quality of life of children with spinal muscle atrophy (SMA) type 1 through better communication skills, greater independence, and personal relationships, according to the results of a survey of parents.
The study, “Communication skills among children with spinal muscular atrophy type 1: A parent survey,” was published in the journal Assistive Technology.
SMA type 1, the most severe and common form of the disease, increases the risk for communication issues, including reduced speech intelligibility, or ease of understanding, and speech-related fatigue. This limits the communication between these children and their parents, and may also lower cognitive performance, social function, and quality of life.
Although their use in SMA type 1 has been seldom described, augmentative and alternative communication (AAC) interventions may benefit these patients because they address motor, sensory and perceptual skills. AAC systems are composed of so-called communicative representations (such as pictures and symbols), messages and message management (such as peer interactions, language learning), training, and ongoing evaluation.
Researchers at Children’s National Health System and Oregon Health Science University surveyed parents of patients with SMA type 1 to better understand the association between functional communication and use of AAC. Language and motor skills were ranked from one (no difficulty) to five (unable, assisted only).
Specifically, the scientists wanted to find out whether the use of AAC was associated with differences in gender, age, race/ethnicity, socioeconomic status, motor, and cognitive function. They also explored which communication methods are used by children with SMA type 1, how they are selected, and if AAC improves quality of life.
A total of 32 respondents were included in the analysis, 17 of whom were parents of males and 15 of females with SMA type 1. The mean age of children was 7.75 months, ranging from 6 months to 30 years. Most participants (30) were located in the U.S.
Results revealed minimal to no disability in language comprehension and eye movement, but severe impairments in respiratory function, speech, and finger, lip, and tongue movement. Language expression and finger movement were also significantly more impaired than language comprehension and eye movement, respectively.
Assessments of natural speech revealed speech deficits related to clarity, independence, and intelligibility, while greater confidence in speaking was associated with greater willingness to talk. Reduced speech clarity correlated with greater assistance needed and less independence with more reluctance to speak, meaning the less independent they were, the more reluctant they were to talk). Greater communication-related frustration was also associated with needing greater assistance.
Among communication methods, three children used all four assessed approaches — speech, gesture, speech-generating devices, and no-tech picture or symbol board/book. Seven children with no functional natural speech used gestures only.
Speech-generating devices were used by 12 children to gain attention, make requests, show interest, make comments, protest and reject, and to provide messages.
A significant difference was identified regarding referral of speech-generating devices based on income, with these devices more commonly used by participants above the median income. However, no difference was found pertaining to age, race or ethnicity, and gender.
The period from referral to evaluation of speech-generating devices often exceeded five months, ranging between one and 27 months. “Most frequently, participants indicated that during the evaluation process, they were presented a limited variety or only a single SGD [speech-generating devices],” the researchers wrote.
As for speech-generating device access, three children used finger to touch-screen contact, four finger or other body part switch activation, 13 eye-tracking, and five used a combination of these methods. Eye-tracking was reported as the most effective for long-lasting communication.
Public health insurance (two patients), private health insurance (nine), a school system (two), and an agency all contributed to SGD funding, while two parents paid for these devices themselves.
Of 16 participants, 13 faced obstacles to acquiring an SGD, most often with funding (nine patients). In addition, while evaluating speech-generating devices, the patients had access to a limited variety of devices (up to four).
Eleven participants reported prolonged SGD use (median of 30 months), most of whom reported regular SGD use either daily (five participants) or four to six days per week (five participants). Limited movement and fatigue associated with SMA type 1 were relevant limitations to daily SGD implementation.
Importantly, the use of speech-generating devices was associated with improved ability to express emotions (six patients), express needs and be understood (eight), better personal relationships (five), greater independence (eight), improved relaxation and recreational interactions (five), broaden school interactions (six), and with building a support system (four). Most (nine) parents reported an improved quality of life with SGDs.
“Overall, families indicated that SGD increases quality of life and provides valued improvements through expanded functional communication,” the researchers wrote.