We describe evidence and intervention strategies for parents, educators, and researchers who seek to enhance communication and literacy in children with Rett syndrome (RS). Four girls with RS and their mothers videotaped their storybook interactions at home for 4 months. Parent-child storybook interactions were coded for child behaviors (e.g., use of augmentative communication devices, attention to book, or vocalizations) and parent behaviors (e.g., pointing in the book or asking predictive questions). Correlation and multiple regression analyses of these variables revealed that girls with RS can learn to communicate in meaningful ways through storybooks; however, mothers may need to engage in different strategies to facilitate better use of augmentative strategies. Successful strategies are discussed for the mother-daughter dyads.
American Association on Mental Retardation. (2002). Mental retardation: Definition, classification, and systems of support (10th ed.). Washington, DC: Author.
3.
American Psychiatric Association. (2000). Quick reference to the diagnostic criteria from DSM-IV-TR. Washington, DC: Author.
4.
AmirR. E.Van den VeyverI. B.WanM.TranC. Q.FranckeU.ZoghbiH. Y. (1999). Rett syndrome is caused by mutations in X-linked MECP2, encoding methyl-CpG-binding protein 2. Nature Genetics, 23, 185–188.
5.
BarlowD. H.HersenM. (1984). Single case experimental designs: Strategies for studying behaviour change (2nd ed.). New York: Pergamon.
6.
BayleyN. (1993). Bayley scales of infant development (2nd ed.). San Antonio, TX: Psychological Corporation.
7.
BedrosianJ.RobertsB.NeynaberM.RaapD. (1995, December). Facilitating participatory storybook reading in young children using AAC systems. Paper presented at the meeting of the American Speech Language Hearing Association, Orlando, FL.
8.
BellonM. L.OgletreeB. T.HarnW. E. (2000). Repeated storybook reading as a language intervention for children with autism. Focus on Autism and Other Developmental Disabilities, 15 (1), 52–59.
9.
CarleE. (1990). The very quiet cricket. New York: Philomel.
10.
Charlop-ChristyM. H.CarpenterM.LeL.LeBlancL. A.KelletK. (2002). Using the picture exchange communication system (PECS) with children with autism: Assessment of PECS acquisition, speech, social-communicative behavior, and problem behavior. Journal of Applied Behavior Analysis, 35, 213–231.
11.
Clayton-SmithJ.WatsonP.RamsdenS.BlackG. C. M. (2000). Somatic mutation in MECP2 as a non-fatal neurodevelopmental disorder in males. Lancet, 356, 830–832.
12.
ColemanM.BrubakerJ.HunterK.SmithG. (1988). Rett syndrome: A survey of North American patients. Journal of Mental Deficiency Research, 32, 117–124.
13.
ColemanP. P. (1991). Literacy lost. Unpublished doctoral dissertation, University of North Carolina, Chapel Hill, NC.
14.
CroweL. K.NorrisJ. A.HoffmanP. R. (2000). Facilitating storybook interactions between mothers and their preschoolers with language impairments. Communication Disorders Quarterly, 21 (3), 131–146.
15.
DexterM. (1998). The effects of aided language stimulation upon verbal output and augmentative communication during storybook reading for children with pervasive developmental disabilities. Unpublished doctoral dissertation, Johns Hopkins University, Baltimore, MD.
16.
Enabling Devices/Toys for Special Children, 385 Warburton Ave., Hastings-on-Hudson, NY 10706. Tel. 1-800-832-8697.
17.
HagbergB.AicardiJ.DiasK.RamosO. (1983). A progressive syndrome of autism dementia, ataxia, and loss of purposeful hand use in girls: Rett's syndrome: Report of 35 cases. Annals of Neurology, 14, 471–479.
18.
HetzroniO.RubinC.KonkolO. (2002). The use of assistive technology for symbol identification by children with Rett syndrome. Journal of Intellectual and Developmental Disability, 27, 57–71.
19.
HillE. (1980). Where's Spot?New York: Putnam.
20.
KoppenhaverD. A.EricksonK. A.HarrisB.McLellanJ.SkotkoB. G.NewtonR. A. (2001). Storybook-based communication intervention for girls with Rett syndrome and their mothers. Disability and Rehabilitation, 23 (3/4), 149–159.
21.
KoppenhaverD. A.EricksonK. A.SkotkoB. G. (2001). Supporting communication of girls with Rett syndrome and their mothers in storybook readings. International Journal of Disability, Development, and Education, 48 (4), 395–410.
22.
KoppenhaverD. A.EvansD. A.YoderD. E. (1991). Childhood reading and writing experiences of literate adults with severe speech and motor impairments. Augmentative and Alternative Communication, 7 (1), 20–33.
23.
LightJ.BingerC.SmithA. Kelford (1994). The story reading interactions of preschoolers who use AAC and their mothers. Augmentative and Alternative Communication, 10, 255–268.
24.
LightJ.RobertsB.DiMarcoR.GreinerN. (1998). Augmentative and alternative communication to support receptive and expressive communication for people with autism. Journal of Communication Disorders, 31, 153–180.
25.
MartinB.Jr. (1992). Brown bear, brown bear, what do you see?New York: Henry Holt.
26.
Mayer-Johnson Co., P.O. Box 1579, Solana Beach, CA 92075. Tel. 1-800-588-4548.
27.
NeumanS. B. (1996). Children engaging in storybook reading: The influence of access to print resources, opportunity, and parental interaction. Early Childhood Research Quarterly, 11 (4), 495–513.
28.
Raffi. (1998). The wheels on the bus. New York: Crown.
29.
RettA. (1966). On an unusual brain atropic syndrome with hyperammonemia in childhood. Wien Med Wochenschr, 116, 723–726.
30.
SchwartzmanJ. S.ZatzM.VasquezL. R.GomesR. R.KoiffmannC. P.FridmanC.OttoP. G. (1999). Rett syndrome in a boy with 47,XXY karyotype. American Journal of Human Genetics, 64, 1781–1785.
31.
SigafoosJ.LaurieS.PennellD. (1995). Preliminary assessment of choice making among children with Rett syndrome. Journal of the Association for Persons with Severe Handicaps, 20 (3), 175–184.
32.
SigafoosJ.LaurieS.PennellD. (1996). Teaching children with Rett syndrome to request preferred objects using aided communication: Two preliminary studies. Augmentative and Alternative Communication, 12, 88–96.
33.
SirianniN.NaiduS.PereiraJ.PillottoR. F.HoffmanE. P. (1998). Rett syndrome: Confirmation of X-linked dominant inheritance, and localization of the gene to Xq28. American Journal of Human Genetics, 63, 1552–1558.
34.
SparrowS. S.BallaD. A.CicchettiD. V. (1984). Vineland adaptive behavior scale. Circle Pines, MN: American Guidance Service.
35.
StricklandD. S.MorrowL. M. (1989). Emerging literacy: Young children learn to read and write. Newark, NJ: International Reading Association.
TjusT.HeimannM.NelsonK. E. (2001). Interaction patterns between children and their teachers when using a specific multimedia and communication strategy: Observations from children with autism and mixed intellectual disabilities. Autism, 5, 175–187.
38.
TrevathanE.NaiduS. (1988). The clinical recognition and differential diagnosis of Rett syndrome. Journal of Child Neurology, 3 (Suppl.), 6–16.
39.
Van AckerR.GrantS. H. (1995). An effective computer-based requesting system for persons with Rett syndrome. Journal of Childhood Communication Disorders, 16 (2), 31–38.
40.
VillardL.LevyN.XiangF.KpebeA.LabelleV.ChevillardC.ZhangZ.SchwartzC. E.TardieuM.ChellyJ.AnvretM.FontesM. (2001). Segregation of a totally skewed pattern of X chromosome inactivation in four familial cases of Rett syndrome without MECP2 mutation: Implications for the disease. Journal of Medical Genetics, 38, 435–442.
41.
WeissM. (1996, May). Assessment and intervention of hand behaviors. Paper presented at the meeting of the International Rett Syndrome Association, Boston, MA.
42.
WoodyattG. C.OzanneA. E. (1992). Communication abilities and Rett syndrome. Journal of Autism and Developmental Disorders, 22 (2), 155–173.
43.
WoodyattG. C.OzanneA. E. (1993). A longitudinal study of cognitive skills and communication behaviors in children with Rett syndrome. Journal of Intellectual Disability Research, 37, 419–435.
44.
WoodyattG. C.OzanneA. E. (1994). Intentionality and communication in four children with Rett syndrome. Australia and New Zealand Journal of Developmental Disabilities, 19 (3), 173–184.
45.
ZappellaM.MeloniI.LongoI.HayekG.RenieriA. (2001). Preserved speech variants of Rett syndrome: Molecular and clinical analysis. American Journal of Medical Genetics, 104, 14–22.
