Impact of Fragile X Syndrome on Their Families

• Autores: Concha Iriarte Redín, Manuel Soriano Ferrer, Beatriz Hidalgo Alés
• Localización: Psicología educativa, ISSN 1135-755X, Vol. 27, Nº. 1, 2021, págs. 93-99
• Idioma: inglés
• Títulos paralelos:
  • Impacto del Síndrome de X frágil en sus familias
• Enlaces
  • Texto completo (pdf)
• Resumen
  • español

    El objetivo del presente estudio es describir las alteraciones conductuales y emocionales en el síndrome de X frágil (SXF) y analizar las reperecusiones de tener un miembro con SXF en diferentes aspectos del funcionamiento familiar. Participaron 79 padres con un miembro con SXF y 80 padres con un miembro con desarrollo típico. Los resultados mostraron que entre el 17% y el 66% de los niños y adolescentes con SXF mostraron trastornos conductuales y emocionales significativos y menos comportamientos prosociales. Además, después de controlar las dificultades emocionales y de comportamiento, nuestros resultados mostraron que las familias afectadas por SXF experimentaron un impacto más negativo en los sentimientos relativos a la crianza de los hijos, las finanzas, los hermanos y el grado de dificultad de vivir con un niño o adolescente con SXF y más capacidad de adaptación familiar y cohesión que el grupo de comparación. Como conclusión, nuestros resultados destacan que en la práctica clínica es importante tener en cuenta estas diferencias al apoyar y asesorar a las familias afectadas por SXF

  • English

    The aim of the present study is to describe the behavioral and emotional disturbances in the Fragile X Syndrome (FXS) and to analize the impact of having a member with FXS on different aspects of family functioning. A sample of 79 parents with a member with FXS and 80 parents with a member with typical development participated in the study. Results showed that between 17% and 66% of children and adolescents with FXS displayed significant behavioral and emotional disturbances and fewer prosocial behaviors. In addition, after controlling for behavioral and emotional difficulties, our results showed that families affected by FXS experienced a more negative impact on feelings about parenting, finances, siblings, and the degree of difficulty of living with a child or adolescent with FXS, and greater family adaptability and cohesion than the comparison group. In conclusion, our results highlight that it is important to consider these differences in clinical practice when supporting and counselling families affected by FXS.

• Referencias bibliográficas
  • Allison, M., & Campbell, M. (2015). Mothers’ perceptions of the quality of childhood sibling relationships affected by disability. The...
  • Angriman, M., Caravale, B., Novelli, L., Ferri, R., & Bruni, O. (2015). Sleep in children with neurodevelopmental disabilities. Neuropediatrics,...
  • Bailey D. B., Raspa, M., Bishop, E., Mitra D., Martin, S., Wheeler, A., & Sacco, P. (2012). Health and economic consequences of fragile...
  • Baker, J. K., Seltzer, M. M., & Greenberg, J. S. (2012). Behaviour problems, maternal internalising symptoms and family relations in families...
  • Berglund, E., Lytsy, P., & Westerling, R. (2015). Health and wellbeing in informal caregivers and non-caregivers: a comparative cross-sectional...
  • Blancher, J., Begum, G. F., Marcoulides, A., & Baker, B. (2013). Longitudinal perspective of child positive impact on families: relationship...
  • Burke, M. & Heller, T. (2016). Individual, parent and social–environmental correlates of caregiving experiences among parents of adults...
  • Cantwell, J., Muldoon, O., & Gallagher, S. (2015). The influence of selfesteem and social support on the relationship between stigma and...
  • Chan, W., Smith, L. E., Greenberg, J. S, Hong, J., & Mailick, M. R. (2017). Executive functioning mediates the effect of behavioral problems...
  • Chevreul, K., Brigham, K. B., Brunn, M., Des Portes, V., & BURQOL-RD Research Netwrok (2015). Fragile X syndrome: Economic burden and...
  • Chevreul, K., Gandré, C., Brigham, K. B., López-Bastida, J., Linertová, R., Oliva-Moreno, J., & Iskrov, G. (2016). Social/economic costs...
  • Chromik, L. C., Quintin, E. M., Lepage, J. F., Hustyi, K. M., Lightbody, A. A., & Reiss, A. L. (2019). The influence of hyperactivity,...
  • Cordeiro, I., Ballinger, E., Hagerman, R., & Hessi, D. (2011). Clinical assessment of DSM-IV anxiety disorder in fragile X syndrome: Prevalence...
  • Donenberg, G., & Baker, B. L. (1993). The impact of young children with externalizing behaviors on their families. Journal of Abnormal...
  • Emerson, E. (2005). Use of Strengths and Difficulties Questionnaire to assess the mental health needs of children and adolescents with intellectual...
  • Frolli, A., Piscopo, S., & Conson, M. (2015). Developmental changes in cognitive and behavioural functioning of adolescents with fragile-X...
  • Gardiner, E., Miller, A. R., & Lach, L. M. (2018). Family impact of childhood neurodevelopmental disability: Considering adaptive and...
  • Goodman, R. (2001). Psychometric properties of the strengths and difficulties questionnaire. Journal of the American Academy of Child &...
  • Grein, K. A., & Glidden, L. M. (2015). Predicting well-being longitudinally for mothers rearing offpring with intellectual and developmental...
  • Haessler, F., Gaese, F., Huss, M., Kretschmar, C., Brinkman, M., Peters, H., Elstner, S., Colla, M., & Pittrow, D. (2016). Characterization,...
  • Hall, S. S., Barnett, R. P., & Hustyi, K. M. (2016). Problem behaviour in adolescent boys with fragile X syndrome: Relative prevalence,...
  • Hare, E., Lozano, R., Partington, L., & Hagerman, R. (2016). Fragile X Syndrome. En L. Rubin, J. Merrick, D. E. Grydanus, & D. Patel...
  • Hartley, S. L., Seltzer, M. M., Head, L., & Abbeduto, L. (2012). Psychological well-being in fathers of adolescents and young adults with...
  • Hastings, R. P., Beck, A., & Hill, C. (2005). Positive contributions made by children with an intellectual disability in the family. Journal...
  • Hayes S. A., & Watson S. L. (2013). The impact of parenting stress: a metaanalysis of studies comparing the experience of parenting stress...
  • Holsen, L. M., Dalton, K. M., Johnstone, T., & Davidson, R. J. (2008). Prefrontal social cognition network dysfunction underlying face...
  • Hunter, J., Rivero-Arias, O., Angelov, A., Kim, E., Fotheringham, I., & Leal, J. (2014). Epidemiology of fragile X syndrome: A systematic...
  • International Labour Organization (ILO, 2012). International standard classification of occupations. ISCO-08. International Labour Office....
  • International Test Commission. (2017). The ITC guidelines for translating and adapting tests (2nd edition). www.InTestCom.org
  • Iriarte, C., Lizasoáin, O., & Peralta, F. (2015). Relaciones intergeneracionales en las fratrías donde hay un hermano con discapacidad...
  • Jess, M., Totsika, V., & Hastings, R. R. (2018). Maternal stress and the functions of positivity in mothers of children with intellectual...
  • Johnston, C., Hessl, D., Blasey, C., Eliez, S., Erba, H., Dyer-Friedman, J., & Reiss, A. L. (2003). Factors associated with parenting...
  • Kerr, C., Breheny, K., Lloyd, A., Brazier, J., Bailey Jr, D. B., Berry-Kravis, E., & Petrillo, J. (2015). Developing a utility index for...
  • Klusek, J., Martin, G. E., & Losh, M. (2014). Consistency between research and clinical diagnoses of autism among boys and girls with...
  • Lewis, P., Abbeduto, L., Murphy, M., Richmond, E., Giles, N., Bruno, L., & Schroeder, S. (2006). Cognitive, language and social-cognitive...
  • Low Kapalu, C. M. & Gartstein, M. A. (2016). Boys with fragile X syndrome: Investigating temperament in early childhood. Journal of Intellectual...
  • Ly, A. R. & Goldberg, W. A. (2014). New measure for fathers of children with developmental challenges. Journal of Intellectual Disability...
  • Martínez-Pampliega, A., Iraurgi, I., Galíndez, E., & Sanz, M. (2006). Family Adaptability and Cohesion Evaluation Scale (FACES): desarrollo...
  • McHale, S. M., Updegraff, K. A., & Feinberg, M. E. (2016). Siblings of youth with autism spectrum disorders: Theoretical perspectives...
  • Migerode, F., Maes, B., Buysse, A., & Brondeel, R. (2012). Quality of life in adolescents with a disability and their parents: The mediating...
  • Minnes, P., Perry, A., & Weiss, J. A. (2015). Predictors of distress and well-being in parents of young children with developmental delays...
  • Miodrag, N., Burke, M., Tanner-Smith, E., & Hodapp, R. M. (2015). Adverse health in parents of children with disabilities and chronic...
  • Moskowitz, L. J., & Jones, E. A. (2015). Uncovering the evidence for behavioral interventions with individuals with fragile X syndrome:...
  • Muller, K., Brady, N., Warren, S., & Fleming, K. (2018). Mothers’ perspectives on challenging behaviours in their children with fragile...
  • Mulroy, S., Robertson, L., Aiberti, K., Leonard, H., & Bower, C. (2008). The impact of having a sibling with an intellectual disability:...
  • Newman I., Leader, G., Chen, J. L., & Mannion, A. (2015). An analysis of challenging behavior, comorbid psychopathology, and attention-deficit/hyperactivity...
  • Norling, D., & Broger, M. (2013). Parents of children with and without intellectual disability: Couple relationship and individual well-being....
  • O’Neill, L. P., & Murray, L. E. (2016). Perceived parenting styles fail to mediate between anxiety and attachment styles in adult siblings...
  • Olson, D. H., Portner, J., & Lavee, Y. (1985). Manual de la Escala de Cohesión y Adaptabilidad Familiar [FACES III Manual]. Life Innovation.
  • Ortuño-Sierra, J., Aritio-Solana, R., & Fonseca-Pedrero, E. (2017). Mental health difficulties in children and adolescents: The study...
  • Ouyang, L., Grosse, S. D., Riley, C., Bolen, J., Bishop, Raspa, M. E., & Bailey Jr, M. (2014). A comparison of family financial and employment...
  • Patton, K. A., Ware, R., McPherson, L., Emerson, E., & Lennox, N. (2018) Parent-related stress of male and female carers of adolescents...
  • Raspa, M., Sacco, P., Candrilli, S. D., Bishop, E., & Petrillo, J. (2016). Validity of a condition specific outcome measure for fragile...
  • Raspa, M., Wheeler, A. C., & Riley, C. (2017). Public health literature review of fragile X syndrome. Pediatrics, 139(3), 153-171. https://doi....
  • Richardson, J. T. E. (2011). Eta squared and partial eta squared as measurements of effect size in educational research. Educational Research...
  • Roberts, J. E., Tonnsen, B. L., Robinson, M., McQuillin, S. D., & Hatton, D. D. (2014). Temperament factor structure in fragile X syndrome:...
  • Rodas, N. V. Zeedyk, S. M., & Baker, B. L. (2016). Unsupportive parenting and internalising behaviour problems in children with or without...
  • Rossetti, Z. & Hall, S. (2015). Adult sibling relationships with brothers and sisters with severe disabilities. Research and Practice...
  • Schlebusch, L., Samuels, A. E., & Dada, S. (2016). South African families raising children with autism spectrum disorders: Relationship...
  • Summers, J.A., Poston, D.J., Turnbull, A.P., Marquis, J., Hoffman, L., Mannan, H., & Wang, M. (2005). Conceptualizing and measuring family...
  • Suzumura, S. (2015). Quality of life in mothers of preschoolers with high-functioning pervasive developmental disorders. Pediatrics International,...
  • Symons, F.J., Byiers, B.J., Raspa, M., Bishop, E., & Bailey, D.B. (2010). Selfinjurious behavior and fragile X syndrome: Findings from...
  • Thurman, A. J., McDuffie, A., Hagerman, R., & Abbeduto, L. (2014). Psychiatric symptoms in boys with fragile X syndrome: A comparison...
  • Usher L.V., DaWalt L. S., Greenberg J. S., & Mailick M. R. (2018). Unaffected siblings of adolescents and adults with fragile X syndrome:...
  • Wheeler, A. C., Raspa, M., Bann, C., Bishop, E., Hessl, D., & Bailey, D. B. (2014). Anxiety, attention problems, hyperactivity, and aberrant...
  • Wheeler, A. C., Raspa, M., Bishop, E., & Bailey Jr., D. B. (2016) Aggression in fragile X syndrome. Journal of Intellectual Disability...
  • Wheeler, A. C., Skinner, D. G., & Bailey, D. B. (2008). Perceived quality of life in mothers of children with fragile X syndrome. American...
  • Woodman, A. C. Mawdsley, H. P., & Hauser-Cram, P. (2015). Parenting stress and child behavior problems within families of children with...
  • World Medical Association. (2013). World medical association declaration of Helsinki: Ethical principles for medical research involving human...