Development of consensus-based guidelines for managing communication of individuals with Rett syndrome

Figures & data

Table 1. Potential search terms for conversion to database-specific keywords.

Participant terms	Communication terms		Intervention terms	Other terms
Rett, Rett syndrome, Rett disorder	Communication, language, speech, vocalization, gesture, understanding, comprehension, cognition, cognitive skills	Learning, literacy, reading, behavior, attention, eye gaze, eye tracking, augmentative and alternative communication, AAC	Assessment, intervention, management, therapy, outcome	Best practice, guidelines, state-of-the-art, comparison, review

Table 2. Delphi survey participants by role.

Role	n (%)^a
Speech-language pathologist^b	13 (29)
Parent	10 (22)
Other therapist (occupational, music)^b	6 (13)
Rett clinic director/coordinator	5 (11)
Education staff (teacher, educational psychologist, social education worker)^b	4 (9)
Researcher (University professor, lecturer)	4 (9)
Medical doctor	3 (7)

^aN = 36, but several participants reported more than one role.

^bThese categories included individuals who also identified as assistive technology and/or augmentative and alternative communication specialists.

Table 3. Delphi survey: question types and numbers.

Round	Statements and recommendations^a		All/most/some/no-one questions^d	Open-ended questions	Case scenarios
	Agreement^b	Importance^c
1	195	84	41	32	8
2	125	5	3	0	0

^a Five-point Likert rating scales were used to rate statements and recommendations for agreement and importance.

^b Agreement rating, 1 (strongly agree), 2 (agree), 3 (neither agree nor disagree), 4 (strongly disagree), 5 (disagree).

^cImportance rating, 1 (extremely important), 2 (very important), 3 (moderately important), 4 (slightly important), 5 (not at all important).

^dParticipants were asked to grade intervention goals according to whether they were suitable for all individuals, most individuals, some individuals, or no-one with Rett syndrome.

Table 4. Delphi survey results: levels of consensus.

Round	Overall level of consensus n/N (%)	Statements and recommendations reaching consensus
		Agreement^a					Importance^b
		1	2	3	4	5	1	2	3	4	5
1	267/279 (96)	124	47	6	8	1	48	32	1	0	0
2	124/130 (95)	74	38	9	0	0	1	2	0	0	0

^a 1 (strongly agree), 2 (agree), 3 (neither agree nor disagree), 4 (strongly disagree), 5 (disagree).

^b 1 (extremely important), 2 (very important), 3 (moderately important), 4 (slightly important), 5 (not at all important).

Table 5. Evidence table example: intervention techniques

Recommendation 8.75	Prompts or cues such as gestures, demonstrations, touch, and signals can be used to increase the likelihood that individuals will make correct responses.
Level of evidence	IIb
Consensus rating	Agree 27/31 (87.1%)
Supporting evidence
Published literature	Prompts have been demonstrated to increase the frequency of correct responses in individuals with RTT; however, in certain cases the prompts could not be faded (withdrawn) without a subsequent loss of skill. (Byiers, Dimian, & Symons, 2014; Lancioni et al., 2014; Simacek, Reichle, & McComas, 2016; Stasolla et al., 2015) (Level IIb)
Gray literature	- nil -
Focus group and professional survey	"Describe the most appropriate prompts." "Pause to prompt - give the girls LOADS of time to respond and use indirect prompts."
Caregiver survey	"When needed, offer prompt."
Delphi survey	"Describing the most appropriate prompts."
	"Reinforcement, stimulus prompting and fading, and discrimination teaching
	“I would use discrimination teaching and reinforcement (and prompting as needed) to chain these open-ended choices following the "yes/no" choice.”
References	Byiers, B. J., Dimian, A., & Symons, F. J. (2014). Functional communication training in Rett syndrome: A preliminary study. American Journal on Intellectual and Developmental Disability, 119, 340-350. doi:10.1352/1944-7558-119.4.340
	Lancioni, G. E., Singh, N. N., O'Reilly, M. F., Sigafoos, J., Boccasini, A., La Martire, M. L., . . . Sacco, V. (2014). Microswitch-aided programs for a woman with Rett syndrome and a boy with extensive neuro-motor and intellectual disabilities. Journal of Developmental and Physical Disabilities, 26, 135-143. doi:10.1007/s10882-013-9349-x
	Simacek, J., Reichle, J., & McComas, J. (2016). Communication intervention to teach requesting through aided AAC for two learners with Rett syndrome. Journal of Developmental and Physical Disabilities, 28, 59-81. doi:10.1007/s10882-015-9423-7
	Stasolla, F., Perilli, V., Di Leone, A., Damiani, R., Albano, V., Stella, A., & Damato, C. (2015). Technological aids to support choice strategies by three girls with Rett syndrome. Research in Developmental Disabilities, 36, 36-44. doi:10.1016/j.ridd.2014.09.017