Skip to main content

Advertisement

Log in

Change in the Behavioral Phenotype of Adolescents and Adults with FXS: Role of the Family Environment

  • Original Paper
  • Published:
Journal of Autism and Developmental Disorders Aims and scope Submit manuscript

Abstract

The present study examined trajectories of adaptive behavior, behavior problems, psychological symptoms, and autism symptoms in adolescents and adults with fragile X syndrome (n = 147) over a three-year period. Adaptive behavior significantly increased over time, particularly for adolescents, and the severity of behavior problems decreased over time. Family environmental factors predicted phenotypic variables net of gender, intellectual disability status, and medication use. Maternal warmth was associated with higher levels of adaptive behavior, lower levels of autism symptoms, and decreases in behavior problems over time. Maternal depressive symptoms and criticism were associated with higher levels of psychological symptoms. Implications for interventions are discussed.

This is a preview of subscription content, log in via an institution to check access.

Access this article

Price excludes VAT (USA)
Tax calculation will be finalised during checkout.

Instant access to the full article PDF.

Fig. 1

Similar content being viewed by others

References

  • Abbeduto, L., Brady, N., & Kover, S. (2007). Language development and fragile X syndrome: Profiles, syndrome-specificity, and within-syndrome differences. Mental Retardation and Developmental Disabilities Research Reviews, 13(1), 36–47.

    Article  PubMed  Google Scholar 

  • Achenbach, T., & Rescorla, L. A. (2001). Manual for the ASEBA school-age forms & profile: An integrated system of multi-informant assessments. Burlington, VT: University of Vermont, Research Center for Children, Youth, & Families.

    Google Scholar 

  • Bailey, D., Hatton, D., & Skinner, M. (1998). Early developmental trajectories of males with fragile X syndrome. American Journal on Mental Retardation, 103(1), 29–39.

    Article  PubMed  Google Scholar 

  • Bailey, D., Raspa, M., Bishop, E., Mitra, D., Martin, S., Wheeler, A., & Sacco, P. (2012). Health and economic consequences of fragile X syndrome for caregivers. Journal of Developmental and Behaviorial Pediatrics, 33(9), 705–712. doi:10.1097/DBP.0b013e318272dcbc.

    Article  Google Scholar 

  • Bailey, D. B., Raspa, M., Holiday, D., Bishop, E., & Olmstead, M. (2009). Functional skills of individuals with fragile x syndrome: A lifespan cross-sectional analysis. American Journal on Intellectual and Developmental Disabilities, 114, 29–303.

    Article  Google Scholar 

  • Baker, J., Seltzer, M., & Greenberg, J. (2012). Behaviour problems, maternal interalising symptoms and family relations in families of adolescents and adults with fragile X syndrome. Journal of Intellectual Disability Research, 56(10), 984–995.

    Article  PubMed  PubMed Central  Google Scholar 

  • Beck, A., Daley, D., Hastings, R., & Stevenson, J. (2004). Mothers’ expressed emotion towards children with and without intellectual disabilities. Journal of Intellectual Disability Research, 48(7), 628–638. doi:10.1111/j.1365-2788.2003.00564.x.

    Article  PubMed  Google Scholar 

  • Borkowski, J., Ramey, S., & Bristol-Power, M. (2002). Parenting and the child’s world: Influences on academic, intellectual, and social-emotional development. Mahwah, NJ: L. Erlbaum.

    Google Scholar 

  • Brady, N., Warren, S. F., Fleming, K., Keller, J., & Sterling, A. (2014). Effect of sustained maternal responsivity on later vocabulary development in children with fragile X syndrome. Journal of Speech, Language, and Hearing Research, 57, 212–226.

    Article  PubMed  PubMed Central  Google Scholar 

  • Brown, W. (2002). The molecular biology of the fragile X mutation. In R. Hagerman & P. Hagerman (Eds.), Fragile X syndrome: Diagnosis, treatment, and research (pp. 110–135). Baltimore, MD: The Johns Hopkins University Press.

    Google Scholar 

  • Bruininks, R., Woodcock, R., Weatherman, R., & Hill, B. (1996). Scales of independent behavior-revised comprehensive manual. Itasca, IL: Riverside Publishing.

    Google Scholar 

  • Crawford, D., Acuna, J., & Sherman, S. (2001). FMR1 and the fragile X syndrome: Human genome epidemiology review. Genetics in Medicine, 3(5), 359–371.

    Article  PubMed  PubMed Central  Google Scholar 

  • Dyer-Friedman, J., Glaser, B., Hessl, D., Johnston, C., Huffman, L., Taylor, A., et al. (2002). Genetic and environmental influences on the cognitive outcomes of children with fragile X syndrome. Journal of the American Academy of Child and Adolescent Psychiatry, 41, 237–244.

    Article  PubMed  Google Scholar 

  • Dykens, E., Leckman, J., Paul, R., & Watson, M. (1988). Cognitive, behavioral, and adaptive functioning in fragile X and non-fragile X retarded men. Journal of Autism and Developmental Disorders, 18(1), 41–52.

    Article  PubMed  Google Scholar 

  • Einfeld, S., Tonge, B., & Turner, G. (1999). Longitudinal course of behavioral and emotional problems in fragile X syndrome. American Journal of Medical Genetics, 87(5), 436–439.

    Article  PubMed  Google Scholar 

  • Esbensen, A. J., Greenberg, J. S., Seltzer, M. M., & Aman, M. G. (2009). A longitudinal investigation of psychotropic and non-psychotropic medication use among adolescents and adults with autism spectrum disorders. Journal of Autism and Developmental Disorders, 39(3), 1339–1349.

    Article  PubMed  PubMed Central  Google Scholar 

  • Esbensen, A., Mailick, M., & Silverman, W. (2013). Long-term impact of parental well-being on adult outcomes and dementia status in individuals with Down syndrome. American Journal on Intellectual and Developmental Disabilities, 118(4), 294–309.

    Article  PubMed  PubMed Central  Google Scholar 

  • Esbensen, A., Seltzer, M., & Krauss, M. (2008). Stability and change in health, functional abilities, and behavior problems among adults with and without Down syndrome. American Journal on Mental Retardation, 113(4), 263–277.

    Article  PubMed  PubMed Central  Google Scholar 

  • Fisch, G., Simensen, R., & Schroer, R. (2002). Longitudinal changes in cognitive and adaptive behavior scores in children and adolescents with the fragile X mutation or autism. Journal of Autism and Developmental Disorders, 32(2), 107–114.

    Article  PubMed  Google Scholar 

  • Glaser, B., Hessl, D., Dyer-Friedman, J., Johnston, C., Wisbeck, J., Taylor, A., & Reiss, A. (2003). Biological and environmental contributions to adaptive behavior in fragile X syndrome. American Journal of Medical Genetics, 117A, 21–29.

    Article  PubMed  Google Scholar 

  • Greenberg, J., Seltzer, M., Baker, J., Smith, L., Hong, J., Warren, S., & Brady, N. (2012). Family environment and behavior problems in children, adolescents, and adults with fragile X syndrome. American Journal on Intellectual and Developmental Disabilities, 117, 331–346.

    Article  PubMed  PubMed Central  Google Scholar 

  • Hartley, S., Seltzer, M. M., Head, L., & Abbeduto, L. (2012). Psychological well-being in fathers of adolescents/young adults with Down syndrome, fragile X syndrome, and autism. Family Relations, 61(2), 327–342.

    Article  PubMed  PubMed Central  Google Scholar 

  • Hastings, R., Daley, D., Burns, C., & Beck, A. (2006). Maternal distress and expressed emotion: Cross sectional and logitudinal relationships with behavior problems of children with intellectual disabilities. American Journal of Mental Retardation, 111, 48–61.

    Article  PubMed  Google Scholar 

  • Hatton, C., & Emerson, E. (2003). Families with a person with intellectual disabilities: Stress and impact. Current Opinion in Psychiatry, 16, 497–501.

    Article  Google Scholar 

  • Hessl, D., Dyer-Fridman, J., Glaser, B., Wisbeck, J. B., Taylor, A., & Reiss, A. (2001). The influence of environmental and genetic factors on behavior problems and autistic symptoms in boys and girls with fragile X syndrome. Pediatrics, 108(5), E88.

    Article  PubMed  Google Scholar 

  • Hessl, D., Tassone, F., Cordeiro, L., Koldewyn, K., McCormick, C., Green, C., & Hagerman, R. (2008). Brief report: aggression and stereotypic behavior in males with fragile X syndrome–moderating secondary genes in a ‘single gene’ disorder. Journal of Autism and Developmental Disorders, 38(1), 184–189.

    Article  PubMed  Google Scholar 

  • Hustyi, K., Hall, S., Jo, B., Lightbody, A., & Reiss, A. (2014). Longitudinal trajectories of aberrant behavior in fragile X syndrome. Research in Developmental Disabilities, 35(11), 2691–2701.

    Article  PubMed  PubMed Central  Google Scholar 

  • Lord, C., Rutter, M., & Le Couteur, A. (1994). Autism Diagnostic Interview—Revised: A revised version of a diagnostic interview for caregivers of individuals with possible pervasive developmental disorders. Journal of Autism and Developmental Disorders, 24, 659–685.

    Article  PubMed  Google Scholar 

  • Maenner, M., Smith, L., Hong, J., Makuch, R., Greenberg, J., & Mailick, M. (2013). An evaluation of an activities of daily living scale for adolescents and adults with developmental disabilities. Disability and Health Journal, 6, 8–17.

    Article  PubMed  PubMed Central  Google Scholar 

  • Magaña, A., Goldstein, M., Karno, M., Miklowitz, D., Jenkins, J., & Falloon, I. (1986). A brief method for assessing expressed emotion in relatives of psychiatric patients. Psychiatry Research, 17, 203–212.

    Article  PubMed  Google Scholar 

  • Mailick, M. R., Greenberg, J. S., Smith, L., Sterling, A., Brady, N., Warren, S. F., & Hong, J. (2014). Fragile X-associated disorders: How the family environment and genotype interact. In J. Burack & L. Schmidt (Eds.), Cultural and contextual perspectives on development at risk. Cambridge: Cambridge University Press.

    Google Scholar 

  • Mazzocco, M. (2000). Advances in research on the fragile X syndrome. Mental Retardation and Developmental Disabilities Research Reviews, 6, 96–106.

    Article  PubMed  Google Scholar 

  • Natsuaki, M., Shaw, D., Neiderhiser, J., Ganiban, J., Harold, G., Reiss, D., & Leve, L. (2014). Raised by depressed parents: Is it an environmental risk? Clinical Child and Family Psychology Review, 17(4), 357–367.

    Article  PubMed  PubMed Central  Google Scholar 

  • Radloff, L. (1977). The CES-D scale: A self-report depression scale for research in the general population. Applied Psychological Measurement, 1(3), 385–401.

    Article  Google Scholar 

  • Raudenbush, S., & Bryk, A. (2002). Hierarchical Linear Models (2nd ed.). Thousand Oaks: Sage Publications.

    Google Scholar 

  • Roberts, J., Miranda, M., Boccia, M., Janes, H., Tonnsen, B., & Hatton, D. (2011). Treatment effects of stimulant medication in young boys with fragile X syndrome. Journal of Neurodevelopmental Disorders, 3(3), 175–184.

    Article  PubMed  PubMed Central  Google Scholar 

  • Rutter, M., Bailey, A., & Lord, C. (2001). Social communication questionnaire (SCQ). Los Angeles: Western Psychological Services.

    Google Scholar 

  • Sabaratnam, M., Murphy, N., Wijeratne, A., Buckingham, A., & Payne, S. (2003). Autistic-like behaviour profile and psychiatric morbidity in fragile X syndrome: A prospective ten-year follow-up study. European Child and Adolescent Psychiatry, 12(4), 172–177.

    Article  PubMed  Google Scholar 

  • Schneider, A., Ligsay, A., & Hagerman, R. (2013). Fragile X syndrome: An aging perspective. Developmental Disabilities Research Reviews, 18(1), 68–74.

    Article  PubMed  Google Scholar 

  • Seltzer, M. M., Greenberg, J. S., Taylor, J. L., Smith, L. E., Orsmond, G. I., Esbensen, A., & Hong, J. (2011). Adolescents and adults with autism spectrum disorder. In D. G. Amaral, G. Dawson, & D. Geschwind (Eds.), Autism spectrum disorders (pp. 241–252). New York: Oxford University Press.

    Chapter  Google Scholar 

  • Singer, J., & Willett, J. (2003). Applied longitudinal data analysis: modeling change and event occurrence. New York: Oxford University Press.

    Book  Google Scholar 

  • Smith, L., Barker, E., Seltzer, M., Abbeduto, L., & Greenberg, J. (2012a). Behavioral phenotype of fragile X syndrome in adolescence and adulthood. American Journal on Intellectual and Developmental Disabilities, 117, 1–17.

    Article  PubMed  PubMed Central  Google Scholar 

  • Smith, L., Greenberg, J., & Mailick, M. (2014). The family context of autism spectrum disorders: influence on the behavioral phenotype and quality of life. Child and Adolescent Psychiatric Clinics of North America, 23(1), 143–155.

    Article  PubMed  PubMed Central  Google Scholar 

  • Smith, L., Greenberg, J., & Seltzer, M. (2012b). Social support and well-being at mid-life among mothers of adolescents and adults with autism spectrum disorders. Journal of Autism and Developmental Disorders, 42(9), 1818–1826.

    Article  PubMed  PubMed Central  Google Scholar 

  • Smith, L., Greenberg, J., Seltzer, M., & Hong, J. (2008). Symptoms and behavior and problems of adolescents and adults with autism: Effects of mother-child relationship quality, warmth, and praise. American Journal on Mental Retardation, 113, 387–402.

    Article  PubMed  PubMed Central  Google Scholar 

  • StataCorp. (2013). Stata statistical software: Release 13. College Station, TX: StataCorp LP.

    Google Scholar 

  • Sterling, A., & Warren, S. (2008). Communication and language development in infants and toddlers with Down syndrome and fragile X syndrome. In J. Roberts, C. Chapman, & S. Warren (Eds.), Speech and language development and intervention in down syndrome and fragile X syndrome (pp. 53–76). Baltimore: Brookes Publishing.

    Google Scholar 

  • Totsika, V., & Sylva, K. (2004). The home observation for measurement of the environment revisited. Child and Adolescent Mental Health, 9(1), 25–35.

    Article  Google Scholar 

  • Vaugh, C., & Leff, J. (1976). The measurement of expressed emotion in the families of psychiatric patients. British Journal of Social and Clinical Psychology, 15, 157–165.

    Article  Google Scholar 

  • Warren, S., & Brady, N. (2007). The role of maternal responsivity in the development of children with intellectual disabilities. Mental Retardation and Developmental Disabilities Research Reviews, 13, 330–338.

    Article  PubMed  Google Scholar 

  • Warren, S., Brady, N., Sterling, A., Fleming, K., & Marquis, J. (2010). Maternal responsivity predicts language development in young children with fragile X syndrome. American Journal on Intellectual and Developmental Disabilities, 115(1), 54–75.

    Article  PubMed  PubMed Central  Google Scholar 

  • Wheeler, A. C., Bailey, D. B., Jr., Berry-Kravis, E., Greenberg, J., Losh, M., Mailick, M., et al. (2014). Associated features in females with an FMR1 premutation. Journal of Neurodevelopmental Disorders, 6(1), 30.

    Article  Google Scholar 

  • Wheeler, A., Hatton, D., Holloway, V., Sideris, J., Neebe, E., Roberts, J., & Reznick, J. (2010). Maternal responses to child frustration and requests for help in dyads with fragile X syndrome. Jounral of Intellectual Disability Research, 54, 501–515.

    Article  Google Scholar 

  • Woodman, A., Smith, L., Greenberg, J., & Mailick, M. (2015). Change in autism symptoms and maladaptive behaviors in adolescence and adulthood: The role of positive family processes. Journal of Autism and Developmental Disorders, 45(1), 111–126.

    Article  PubMed  PubMed Central  Google Scholar 

Download references

Acknowledgments

This study was supported by a grant from the National Institute of Child Health and Human Development to the IDDRC at the University of North Carolina (P30 HD003100-S1) to support a Fragile X Research Center. The Fragile X Research Center has three additional sites (Research Triangle Institute International, the University of Wisconsin-Madison, and University of Kansas). The present analysis was based on data collected at the UW-Madison Waisman Center site (M. Mailick, PI). We are extremely grateful to the families who participated in this study; without their generous support and commitment, our research would not be possible. We are also grateful for the support we received from the Waisman Center (P30 HD03352, A. Messing, PI).

Author Contributions

LS participated in analysis, led the interpretation of the data and drafting of the manuscript. JH led statistical analysis and participated in data interpretation and drafting the manuscript. JS and MM conceived and designed the study, participated in analysis and interpretation of the data and drafting of the manuscript. All authors read and approved the final manuscript.

Author information

Authors and Affiliations

Authors

Corresponding author

Correspondence to Leann E. Smith.

Rights and permissions

Reprints and permissions

About this article

Check for updates. Verify currency and authenticity via CrossMark

Cite this article

Smith, L.E., Hong, J., Greenberg, J.S. et al. Change in the Behavioral Phenotype of Adolescents and Adults with FXS: Role of the Family Environment. J Autism Dev Disord 46, 1824–1833 (2016). https://doi.org/10.1007/s10803-016-2714-8

Download citation

  • Published:

  • Issue Date:

  • DOI: https://doi.org/10.1007/s10803-016-2714-8

Keywords

Navigation