Fragile X and literacy: What’s the role of phonological awareness?

Although individuals with Fragile X Syndrome (FXS) are known to have literacy impairments, we have little research to draw from for reading instruction with this population—problematic,  because literacy skills have a huge impact on quality of life, employment, and social opportunities! This paper described two experiments meant to help bridge that gap.

Participants in the first study were adolescents with FXS aged 16 to 23, all males with intellectual disabilities. Their phonological awareness skills predicted their oral word reading performance, which provides evidence of an association between phonological awareness skills and reading skills in this population. The authors state, “Instruction targeting phonological awareness and phonics should not be ruled out for individuals with FXS” and recognize that we need more causal data here—in other words, we need to know more about the effects of phonological awareness instruction on individuals’ reading skills so that we can improve our intervention.

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On that note, the second study looked at feasibility—can individuals with FXS complete a phonological reading intervention that was originally designed for mainstream students? A small group, 8 individuals with FXS aged 7 to 23, participated in the HeadSprout Early Reading Program (HERP). Since there were no control groups or conditions, this study doesn’t tell us about effectiveness of the intervention. Instead, the authors found that individuals with FXS were able to access the web-based instruction and identified factors that enabled their success. Even though we need more information to know how best to approach literacy instruction with individuals with FXS, we should keep in mind the important relationship between phonological awareness and reading skills within this population.

 

Adlof, S. M., Klusek, J., Hoffmann, A., Chitwood, K. L., Brazendale, A., Riley, K., Abbeduto, L. J., & Roberts, J. E. (2018). Reading in Children with Fragile X Syndrome: Phonological Awareness and Feasibility of Intervention. American Journal on Intellectual and developmental Disabilities, 123(3), 193–211. doi:10.1352/1944-7558-123.3.193

Self-advocacy during communication breakdowns

This article shows that some children and teens with neurodevelopmental disorders are at a particularly high risk for not speaking up (or gesturing, signing, using AAC) to indicate when they don’t understand what others trying to say. Self-advocacy during communication breakdowns is particularly challenging for boys with comorbid Fragile X and Autism, and also challenging for those with Down Syndrome. In addition to clinicians simply being aware of populations at particular risk for challenges, this article is also useful because their tasks for measuring non-comprehension are well-described. Thus, clinicians could reasonably use some of the tasks as inspiration for probing non-comprehension and resultant communicative behaviors in clinical settings.

Martin, G.E., Bartstein, J., Hornickel, J., Matherly, S., Durante, G., & Losh, M. (2017). Signaling of noncomprehension in communication breakdowns in fragile X syndrome, Down syndrome, and autism spectrum disorder. Journal of Communication Disorders, 65, 22–34.

And more...

  • Barton-Hulsey et al. present three case studies of dynamic assessment for an AAC device. Their results, per client, can’t be generalized to a broader population. However, the article presents clear and explicit methods for AAC evaluation and data collection, which may be worth clinical consideration.
  • Iarocci et al., in study of 174 children with and without autism, found that, “… exposure to a second language is not associated with an adverse impact on the communication and cognitive skills of children with ASD.” The authors acknowledge some of the common concerns of bilingualism in low-language children, and review research on the benefits of bilingualism in these children.
  • Morgan et al. show that cleft palate is a risk factor for language development, and that internationally-adopted children with cleft palate are at an even greater risk of low language skills (presumably because of the interruption in language as they switch from L1 to a new primary L2). We’ve talked about the impact of foreign adoption on language before.
  • Tenenbaum et al. examined visual conditions to support word learning in typically-developing children and those with autism spectrum disorder. They found that for children with autism (ages pre-K through early-elementary, with Preschool Language Scales (PLS) scores of at least 12 months, and producing at least single words), the children learned new object words better when the targeted object was held close to the speaker’s face while producing the word (but without covering the mouth), and that this supported word learning better than attention to the mouth alone or attention to the object alone.
  • Thurman et al. examine differences between the language skills of male children with fragile X and autism, and found that boys with fragile X have a relative strength in lexical skills compared to boys with autism.

 
Barton-Hulsey, A., Wegner, J., Brady, N.C., Bunce, B.H., & Sevcik, R.A. (2017) Comparing the effects of speech-generating device display organization on symbol comprehension and use by three children with developmental delays. American Journal of Speech­–Language Pathology. Advance online publication. doi:10.1044/2016_AJSLP-15-0166.
 
Iarocci, G., Hutchison, S.M. & O’Toole, G.J. (2017). Second language exposure, functional communication, and executive function in children with and without autism spectrum disorder (ASD). Journal of Autism and Developmental Disorders. Advance online publication. doi: 10.1007/s10803-017-3103-7
 
Morgan, A.R., Bellucci, C.C., Coppersmith, J., Linde, S.B., Curtis, A., Albert, M., O'Gara, M.M., & Kapp-Simon, K. (2017). Language development in children with cleft palate with or without cleft lip adopted from non–English-speaking countries. American Journal of Speech–Language Pathology. Advance online publication. doi:10.1044/2016_AJSLP-16-0030.

Tenenbaum, E.J., Amso, D., Righi, G., & Sheinkopf, S.S. (2017). Attempting to “Increase intake from the input”: attention and word learning in children with autism. Journal of Autism and Developmental Disorders. Advance online publication. doi:10.1007/s10803-017-3098-0.

Thurman, A.J., McDuffie, A., Hagerman, R.J., Josol, C.K., & Abbeduto, L. (2017). Language skills of males with fragile X syndrome or nonsyndromic autism spectrum disorder. Journal of Autism and Developmental Disorders, 47(3), 728–743.

And more...

  • Gick et al. show us that the tongue is often bracing against the upper molars during speech, that this is normal, and cite a few previous studies with similar results. This finding isn't generalizable yet (particularly to kids; needs performed with more people, and extended to younger participants). Nonetheless, it’s an important point for pediatric SLPs to consider as we proceed with treatment of articulatory placement (as in, if you ask your student where his tongue is in his mouth, and he reports that the sides are touching his upper molars, don't freak out or ask him to change it).

  • Komesidou et al. discuss how “…expressive syntax is a vulnerable domain for children with Fragile X…”. In a longitudinal study of 39 kids (ages 2–10) with Fragile X, they found that children with Fragile X were able to make gains in expressive syntax over time (slowed in later years), but expressive language scores were significantly lower for those with Fragile X and autism symptoms.

  • Stadskliev et al. describe a parent support group for families of young AAC users. This small study provides insight into parents’ perspectives on learning to support their child with AAC, and provides clinicians with some ideas for how to better support children and their families.


Gick, B., Allen, B., Roewer-Després, Stavness, I. (2017). Speaking tongues are actively braced. Journal of Speech, Language, and Hearing Research. Advance online publication. doi: 10.1044/2016_JSLHR-S-15-0141.

Komesidou, R., Brady, N.C., Fleming, K., Esplund, A., Warren, S.F. (2017). Growth of expressive syntax in children with Fragile X Syndrome. Journal of Speech, Language, and Hearing Research. Advance online publication. doi:10.1044/2016_JSLHR-L-15-0360.

Stadskleiv, K. (2017). Experiences from a support group for families of preschool children in the expressive AAC user group. Augmentative and Alternative Communication. Advance online publication. doi: 10.1080/07434618.2016.1276960.
 

And more...

  • Haebig & Sterling provide evidence of relatively lower receptive compared to expressive language in 36% of older children with autism and 14% of children with both autism and Fragile X. The clinical implication for SLPs is to carefully consider clients' receptive language skills and progress, not just expressive, particularly in clients with ASD.
  • Hebert et al.'s meta-analysis provides evidence that teaching students about expository text structures (description, sequence, cause-effect, compare/contrast, problem/solution) has generally positive outcomes on expository reading comprehension. The authors suggest that, “text structure instruction be included as one component of a comprehensive approach to expository reading instruction.”
  • Snow & Woodward provide an intervention study on an underrepresented group—incarcerated youths—and found these students to be quite receptive to speech–language therapy. The number of these children with language difficulties is quite high (Snow et al. 2015), yet many barriers prevent them from receiving adequate services (see article for summary). Though this paper may not apply to many SLPs’ current caseloads, it is relevant to consider for the purposes of awareness and advocacy.

Haebig, E., & Sterling, A. (2016). Investigating the Receptive-Expressive Vocabulary Profile in Children with Idiopathic ASD and Comorbid ASD and Fragile X Syndrome. Journal of Autism and Developmental Disorders. Advance online publication. doi:10.1007/s10803-016-2921-3.

Hebert, M., Bohaty, J.J., Nelson, J.R., Brown, J. (2016). The effects of text structure instruction on expository reading comprehension: A meta-analysis. Journal of Educational Psychology, 108(5), 609–629. 

Snow, P., & Woodward, M.N. (2016). Intervening to address communication difficulties in incarcerated youth: A Phase 1 clinical trial. International Journal of SpeechLanguage Pathology. Advance online publication. doi:10.1080/17549507.2016.1216600.