Abstract
Few studies have examined the impact of children with genetic disorders and their unaffected siblings on family functioning. In this study, the reciprocal causal links between problem behaviors and maternal distress were investigated in 150 families containing a child with fragile X syndrome (FXS) and an unaffected sibling. Both children's behavior problems appeared to have strong, direct effects on maternal distress, but maternal distress did not appear to have any reciprocal causal effects on either child's behavior problems. Surprisingly, there were no significant differences in the effects of the two children's behavior problems on maternal distress. These data suggest that the problem behaviors of children with FXS, as well as their unaffected siblings, can have a substantial and additive impact on maternal depression and anxiety. Future research efforts should employ longitudinal research designs to confirm these findings.
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Notes
Some children with FXS also had siblings with FXS, although diagnosis was not always confirmed.
We also performed the analyses treating the lowest scores as missing data. The results of these analyses were very similar.
The correlation between the Conflict subscale of the FES and the CBC-EXT scale of the CBC was 0.23 for children with FXS and 0.46 for unaffected siblings.
This method can provide consistent parameter estimates in the presence of missing data, even when the data are not missing completely at random. Three alternative methods of estimating models with missing data include mean substitution, listwise deletion, and pairwise deletion. These methods are less efficient and provide consistent estimates only under the stronger assumption that any missing data are missing completely at random.
Some investigators prefer to call these terms “Other Causes,” since they contain all the unexplained variance of each observed variable, including unknown systematic causes as well as random measurement errors.
One cannot assume that this is actually a method factor resulting from maternal bias. It could also represent the fact that the two children's behaviors at home may be more highly correlated than their behaviors at school. This would not be surprising, since the home environment as defined here is identical, while the two school environments for the children will be significantly different. This “method factor” could just as easily be represented as a correlated error term. Statistically, this would be an alternative but equivalent representation of the same phenomenon.
In a tau-equivalent factor, the regression coefficient for all the indicators are equal, indicating that they all contain the same amount of factor variance. In a parallel factor, all the error variances are also equal, indicating they all have identical r-square values. In a super parallel factor, the regression coefficients, error variances, and intercepts for all the indicators are equal.
One could argue that the behavior factors might conceivably have causal effects on the IQ factors. In fact, some investigators have proposed such a causal linkage. We tested this possibility in a non-recursive model and found that it was grossly inconsistent with the data, and could be rejected.
This test was performed by subtracting the χ 2 and df of the measurement model from the χ 2 and df of the structural model.
Five females and 15 males with FXS scored in the “autism” range on the Autism Behavior Checklist (Krug, Arick, & Almond, 1993) and these children had significantly elevated scores on the CBC-EXT and TRF-EXT. Inclusion of autism status in the model, however, indicated that children who showed symptoms of autism did not directly influence mothers' level of anxiety or depressive symptoms.
We measured FMR Protein levels in these children, and plan to report those results separately.
References
Abidin, R. R. (1995). Parenting Stress Index -Third Edition: Professional Manual. Odessa, FL: Psychological Assessment Resources, Inc.
Achenbach, T. M. (1991a). Manual for the Child Behavior Checklist/4–18 and 1991 Profile. Burlington, VT: University of Vermont, Department of Psychiatry.
Achenbach, T. M. (1991b). Manual for the Teacher's Report Form and 1991 Profile. Burlington, VT: University of Vermont, Department of Psychiatry.
Arbuckle, J. L. (1996). Full information estimation in the presence of incomplete data. In G. A. Marcoulides & R. E. Schumacker (Eds.), Advanced Structural Equation Modeling. Mahwah, New Jersey: Lawrence Erlbaum Associates.
Arbuckle, J. L. (2005). Amos 6.0 User's Guide. Chicago, IL: SPSS Inc.
Baker, B. L., McIntyre, L. L., Blacher, J., Crnic, K., Edelbrock, C., & Low, C. (2003). Pre-school children with and without developmental delay: behaviour problems and parenting stress over time. Journal of Intellectual Disability Research, 47, 217–230.
Barry, T. D., Dunlap, S. T., Cotten, S. J., Lochman, J. E., & Wells, K. C. (2005). The influence of maternal stress and distress on disruptive behavior problems in boys. Journal of the American Academy of Child and Adolescent Psychiatry, 44(3), 265–272.
Baumgardner, T. L., Reiss, A. L., Freund, L. S., & Abrams, M. T. (1995). Specification of the neurobehavioral phenotype in males with fragile X syndrome. Pediatrics, 95(5), 744–752.
Beckman, P. J. (1991). Comparison of mothers' and fathers' perceptions of the effect of young children with and without disabilities. Am J Ment Retard, 95(5), 585–595.
Bentler, P. M., & Bonett, D. G. (1980). Significance tests and goodness of fit in the analysis of covariance structures. Psychological Bulletin, 88, 588–606.
Bollen, K. A. (1989). Structural equations with latent variables. New York: John Wiley & Sons.
Bristol, M. M., Gallagher, J. J., & Schloper, E. (1988). Mothers and fathers of young developmentally disabled and nondisabled boys: Adaptation and spousal support. Developmental Psychology, 24, 441–451.
Browne, M. W., & Cudeck, R. (1993). Alternative ways of assessing model fit. In K. A. Bollen & J. S. Long (Eds.), Testing Structural Equation Models (pp. 136–162). Newbury Park, CA: Sage.
Burns, D. D., & Nolen-Hoeksema, S. (1992). Therapeutic empathy and recovery from depression in cognitive-behavioral therapy: A structural equation model. Journal of Consulting and Clinical Psychology, 60, 441–449.
Cameron, S. J., & Orr, R. R. (1989). Stress in families of school-aged children with delayed mental development. Canadian Journal of Rehabilitation, 2, 137–144.
Carr, E. G., & Durand, V. M. (1985). The social-communicative basis of severe behavior disorders. In S. Reiss & R. Bootzin (Eds.), Theoretical Issues in Behavior Therapy. New York: Academic Press.
Cohen, H., Amerine-Dickens, M., & Smith, T. (2006). Early intensive behavioral treatment: replication of the UCLA model in a community setting. J Dev Behav Pediatr, 27(2 Suppl), S145–S155.
Cole, D. A., & Maxwell, S. E. (2003). Testing mediational models with longitudinal data: Questions and tips in the use of structural equation modeling. Journal of Abnormal Psychology, 112, 558–577.
Crawford, D. C., Meadows, K. L., Newman, J. L., Taft, L. F., Stanfeild, M. L., Holmgreen, P., et al. (1999). Prevalence and phenotype consequence of FRAXA and FRAXE alleles in a large, ethnically diverse, special education-needs population. American Journal of Human Genetics, 64, 495–507.
Derogatis, L. R. (1994). SCL-90-R Administration, Scoring and Procedures Manual (3rd ed.). Minneapolis, MN: National Computer Systems, Inc.
Donenberg, G., & Baker, B. (1993). The impact of young children with externalizing behaviors on their families. Journal of Abnormal Child Psychology, 21(2), 179–198.
Dyson, L. L. (1997). Fathers and mothers of school-age children with developmental disabilities: parental stress, family functioning, and social support. Am J Ment Retard, 102(3), 267–279.
Essex, E. L., Seltzer, M. M., & Krauss, M. W. (1999). Differences in coping effectiveness and well-being among aging mothers and fathers of adults with mental retardation. American Journal on Mental Retardation, 104, 545–563.
Finegan, J. A. (1998). Study of behavioral phenotypes: Goals and methodological considerations. American Journal of Medical Genetics, 81(2), 148–155.
Franke, P., Leboyer, M., Gansicke, M., Weiffenbach, O., Biancalana, V., Cornillet-Lefebre, P., et al. (1998). Genetype-phenotype relationship in female carriers of the premutation and full nutation of FMR-1. Psychiatry Research, 80, 113–127.
Gold, N. (1993). Depression and social adjustment in siblings of boys with autism. Journal of Autism and Developmental Disorders, 23(1), 147–163.
Hall, S., Oliver, C., & Murphy, G. (2001). Early development of self-injurious behavior: An empirical study. American Journal on Mental Retardation, 106, 189–199.
Hall, S. S., DeBernardis, G. M., & Reiss, A. L. (2006). Social escape behaviors in children with fragile X syndrome. Journal of Autism and Developmental Disorders, 36, 935–947.
Hanushek, E. A., & Jackson, J. E. (1977). Statistical methods for social scientists. Orlando, FL: Academic Press.
Hastings, R. P. (2003a). Brief report: Behavioral adjustment of siblings of children with autism. Journal of Autism and Developmental Disorders, 33, 99–103.
Hastings, R. P. (2003b). Child behaviour problems and partner mental health as correlates of stress in mothers and fathers of children with autism. Journal of Intellectual Disability Research, 47, 231–237.
Hodapp, R. M., Fidler, D. J., & Smith, A. C. M. (1998). Stress and coping in families of children with Smith-Magenis syndrome. Journal of Intellectual Disability Research, 42, 331–340.
Johnston, C., Eliez, S., Dyer-Friedman, J., Hessl, D., Glaser, B., Blasey, C., et al. (2001). Neurobehavioral phenotype in carriers of the fragile X premutation. American Journal of Medical Genetics, 103, 314–319.
Johnston, C., Hessl, D., Blasey, C., Eliez, S., Erba, H., Dyer-Friedman, J., et al. (2003). Factors associated with parenting stress in mothers of children with fragile X syndrome. Developmental and Behavioral Pediatrics, 24(4), 267–275.
Koegel, R. L., Schreibman, L., Loos, L. M., Dirlich-Wilhelm, H., Dunlap, G., Robbins, F. R., et al. (1992). Consistent stress profiles in mothers of children with autism. Journal of Autism and Developmental Disorders, 22, 205–216.
Konstantareas, M. M., & Homatidis, S. (1989). Assessing child symptom severity and stress in parents of autistic children. Journal of Child Psychology and Psychiatry, 30, 459–470.
Krug, D. A., Arick, J. R., & Almond, P. J. (1993). Autism Screening Instrument for Educational Planning: Examiner's Manual. Austin, Texas: Pro-Ed.
Lovaas, O. I. (1987). Behavioral treatment and normal educational and intellectual functioning in young autistic children. Journal of Consulting and Clinical Psychology, 55, 3–9.
Mash, E. J., & Johnston, C. (1983). Parental perceptions of child behavior problems, parenting self-esteem, and mothers' reported stress in younger and older hyperactive and normal children. Journal of Consulting and Clinical Psychology, 51, 86–99.
Moos, R. H., & Moos, B. S. (1994). Family Environment Scale Manual. Palo Alto, CA: Consulting Psychologists Press Inc.
Oliver, C., Murphy, G. H., Crayton, L., & Corbett, J. A. (1993). Self-injurious behavior in Rett syndrome: Interactions between features of Rett syndrome and operant conditioning. Journal of Autism and Developmental Disorders, 23, 91–109.
Orr, R. R., Cameron, S. J., Dobson, L. A., & Day, D. M. (1993). Age-related changes in stress experienced by families with a child who has developmental delays. Mental Retardation, 31(3), 171–176.
Patterson, G. R. (1982). Coercive Family Process. Eugene, OR: Castalia.
Patterson, G. R. (2002). The early development of coercive family processes. In J. B. Reid, G. R. Patterson, & J. Snyder (Eds.), Antisocial behavior in children and adolescents: A developmental analysis and model for intervention (pp. 25–44). Washington, DC: American Psychological Association.
Perry, A., Sarlo-McGarvey, N., & Factor, D. C. (1992). Stress and family functioning in parents of girls with Rett syndrome. Journal of Autism and Developmental Disorders, 22(2), 235–248.
Plomin, R., & De Fries, J. C. (1985). Origins of individuals differences in infancy: The Colorado Adoption Project. Orlando, Florida: Academic Press, Inc.
Quine, L., & Pahl, J. (1985). Examining the causes of stress in families with severely mentally handicapped children. British Journal of Social Work, 15, 501–517.
Rivers, J. W., & Stoneman, Z. (2003). Sibling relationships when a child has autism: Marital stress and support coping. Journal of Autism and Developmental Disorders, 33(4), 383–394.
Rossiter, L., & Sharpe, D. (2001). The siblings of individuals with mental retardation: A quantitative integration of the literature. Journal of Child and Family Studies, 10, 65–84.
Sarimski, K. (1997). Behavioural phenotypes and family stress in three mental retardation syndromes. Eur Child Adolesc Psychiatry, 6(1), 26–31.
Skinner, B. F. (1988). Beyond Freedom and Dignity. Peregrine Books.
Stores, R., Stores, G., Fellows, B., & Buckley, S. (1998). Daytime behaviour problems and maternal stress in children with Down's syndrome, their siblings, and non-intellectually disabled and other intellectually disabled peers. Journal of Intellectual Disability Research, 42, 228–237.
Thompson, N. M., Gulley, M. L., Rogeness, G. A., Clayton, R. J., Johnson, C., Hazelton, B., et al. (1994). Neurobehavioral characteristics of CGG amplification status in fragile X females. American Journal of Medical Genetics, 54, 378–383.
Thornton, L., & Dawson, K. P. (1990). Prader-Willi syndrome in New Zealand: A survey of 36 affected people. New Zealand Medical Journal, 103(97–98).
Tomarken, A. J., & Baker, T. B. (2003). Introduction to the special section on structural equation modeling. Journal of Abnormal Psychology, 112, 523–525.
Tomarken, A. J., & Waller, N. G. (2003). Potential problems with “well fitting” models. Journal of Abnormal Psychology, 112, 578–598.
Verkerk, A. J., Pieretti, M., Sutcliffe, J. S., Fu, Y. H., Kuhl, D. P., Pizzuti, A., et al. (1991). Identification of a gene (FMR-1) containing a CGG repeat coincident with a breakpoint cluster region exhibiting length variation in fragile X syndrome. Cell, 65, 905–914.
Wechsler, D. (1991). Wechsler Intelligence Scale for Children—Third Edition. Administration and Scoring Manual. San Antonio: The Psychological Corporation.
Wechsler, D. (1997). Wechsler Adult Intelligence Scale—Third Edition. Administration and Scoring Manual. San Antonio: The Psychological Corporation.
Willoughby, J. C., & Glidden, L. M. (1995). Fathers helping out: shared child care and marital satisfaction of parents of children with disabilities. American Journal on Mental Retardation, 99(4), 399–406.
Wolf, L. C., Noh, S., Fisman, S. N., & Speechley, M. (1989). Psychological effects of parenting stress on parents of autistic children. Journal of Autism and Developmental Disorders, 19(1), 157–166.
Acknowledgements
The authors would like to thank David Hessl, Jennifer Dyer-Friedman, Jacob Wisbeck, Bronwyn Glaser, Donna Mumme, and Cindy Johnston for their participation in this project. This research was supported by NIH grants MH50047 and MH01142.
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Hall, S.S., Burns, D.D. & Reiss, A.L. Modeling Family Dynamics in Children with Fragile X Syndrome. J Abnorm Child Psychol 35, 29–42 (2007). https://doi.org/10.1007/s10802-006-9081-4
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DOI: https://doi.org/10.1007/s10802-006-9081-4