Journal of Genetic Counseling

, Volume 21, Issue 1, pp 59–71

Influence of Genetic Risk Information on Parental Role Identity in Adolescent Girls and Young Women from Families with Fragile X Syndrome

  • Allyn McConkie-Rosell
  • Elizabeth Melvin Heise
  • Gail A. Spiridigliozzi
Original Research

Abstract

Using a multi-group cross-sectional design, we explored self-concept related to parental role salience and enactment in 53 young women (14 to 24 years) with knowledge they were either carriers, non-carriers, or could be a carrier of fragile X syndrome (FXS). Parental role salience included the participants’ desire “to be a mother” and the importance they placed on this role. Enactment focused on the participants’ views regarding ways to become a mother (reproductive options), parenting a child affected by FXS, and the development of partner relationships (marriage). Participants completed the FXS Adolescent Interview and the FX-Visual Analog Scale. Participants’ knowledge of their genetic risk status appears to have influenced both salience and enactment of the parental role, and the effect varied based on carrier status. For many, knowledge of genetic risk appears to have led to reappraisal, redefinition, and re-engagement with the goal of becoming a parent. This process was prominent in those who were carriers and less so in those who were at-risk, and it did not typically occur in those who were non-carriers. Findings offer valuable insight into the impact of genetic risk information on developing perceptions of the parental role and offer new directions for genetic counseling with adolescents and young women with a family history of FXS.

Keywords

Fragile X syndrome Self-concept Parental role Adolescent Carrier testing Genetic counseling 

References

  1. Areskog, B., Uddenberg, N., & Kjessler, B. (1984). Postnatal emotional balance in women with and without antenatal fear of childbirth. Journal of Psychosomatic Research, 28, 213–220.PubMedCrossRefGoogle Scholar
  2. Axworthy, D., Brock, D. J. H., Bobrow, M., & Marteau, T. M. (1996). Psychological impact of populations-based carrier testing for cystic fibrosis: 3-year follow up. Lancet, 347(9013), 1443–1446.PubMedCrossRefGoogle Scholar
  3. Balk, D. E. (1994). An overview of adolescence. In Adolescent Development: Early through late adolescence (pp. 3–40). Pacific Grove: Brooks/Cole Publishing Company.Google Scholar
  4. Baumeister, R. F. (1998). The Self. In D. Gilbert, S. Fiske, & G. Lindzey (Eds.), The Handbook of Social Psychology (pp. 680–740). New York: Oxford University Press.Google Scholar
  5. Bond, A., Shine, P., & Bruce, M. (1995). Validation of visual analogue scales in anxiety. International Journal of Methods in Psychiatric Research, 5, 1–9.Google Scholar
  6. Brandtstadter, J., & Rothermund, K. (2002). The life-course dynamics of goal pursuit and goal adjustment: a two-process framework. Developmental Review, 22, 117–150.CrossRefGoogle Scholar
  7. Crawford-Little, J., & McPhail, N. I. (1973). Measures of depressive mood at monthly intervals. Biritish Journal of Psychiatry, 122, 447–452.CrossRefGoogle Scholar
  8. DeVellis, R. F. (1991). Scale development: Theory and applications (Vol. 26). Newbury Park: Sage.Google Scholar
  9. Erikson, E. H. (1963). Childhood and Society (2nd ed.). New York: Norton.Google Scholar
  10. Fitts, W., & Warren, W. (1996). Tennessee self-concept scale (2nd ed.). Los Angles: Western Psychological Services.Google Scholar
  11. Hagerman, P. J., & Hagerman, R. J. (2004). The fragile-X premutation: a maturing perspective. American Journal of Human Genetics, 74(5), 805–816.PubMedCrossRefGoogle Scholar
  12. Hattie, J. (1992). A Theory of Self-Concept. In Self-Concept (pp. 36–57). Hillsdale: Lawrence Erlbaum Associates.Google Scholar
  13. Heller, T., & Arnold, C. (2010). Siblings of adults with developmental disabilities: psychosocial outcomes, relationships, and future planning. Journal of Policy and Practice in Intellectual Disabilities, 7(1), 16–25.CrossRefGoogle Scholar
  14. Howard, J. A. (1991). Introduction: The self-society dynamic. In J. A. Howard & P. L. Callero (Eds.), The self-society dynamic: Cognition, emotion, and action (pp. 1–17). New York: Cambridge University Press.CrossRefGoogle Scholar
  15. Kay, E., & Kingston, H. (2002). Decision making in women known to be carriers of X-linked conditions: feelings associated with being a carrier and characteristics of reproductive. Journal of Health Psychology, 7(2), 169–181.PubMedCrossRefGoogle Scholar
  16. Lee, C., & Gramotnev, H. (2006). Motherhood plans among young Australian women: who wants children these days? Journal of Health Psychology, 11(1), 5–20.PubMedCrossRefGoogle Scholar
  17. Markus, H., & Nurius, P. (1987). Possible selves: The interface between motivation and the self-concept. In K. Yardley & T. Honess (Eds.), Self and identity: Psychosocial perspectives (pp. 157–172). Chichester: Wiley.Google Scholar
  18. McConkie-Rosell, A., Del Giorno, J., & Heise, E. M. (2011). Communication of genetic risk information to daughters in families with fragile X syndrome: the parent’s perspective. Journal of Genetic Counseling, 20(1), 58–69.PubMedCrossRefGoogle Scholar
  19. McConkie-Rosell, A., & DeVellis, B. M. (2000). Threat to parental role: a possible mechanism of altered self-concept related to carrier knowledge. Journal of Genetic Counseling, 9(4), 285–302.CrossRefGoogle Scholar
  20. McConkie-Rosell, A., Finucane, B., Cronister, A., Abrams, L., Bennett, R. L., & Pettersen, B. J. (2005). Genetic counseling for fragile X syndrome: updated recommendations of the national society of genetic counselors. Journal of Genetic Counseling, 14(4), 249–269.PubMedCrossRefGoogle Scholar
  21. McConkie-Rosell, A., Heise, E., & Spiridigliozzi, G. A. (2009). Genetic risk communication: perceptions of adolescent girls and young women from families with fragile X syndrome. Journal of Genetic Counseling, 18(4), 313–325.PubMedCrossRefGoogle Scholar
  22. McConkie-Rosell, A., Spiridigliozzi, G., Dawson, D., Sullivan, J., & Lachiewicz, A. M. (2002). Carrier testing in fragile X syndrome: when to tell and test. American Journal of Medical Genetics, 110, 36–44.PubMedCrossRefGoogle Scholar
  23. McConkie-Rosell, A., Spiridigliozzi, G., Melvin, E., Dawson, D., & Lachiewicz, A. (2008). Living with genetic risk: effect on adolescent self-concept. American Journal of Medical Genetics, 148C, 56–69.PubMedCrossRefGoogle Scholar
  24. McConkie-Rosell, A., Spiridigliozzi, G. A., Dawson, D., Sullivan, J., & Lachiewicz, A. M. (2000). Carrier testing in fragile X syndrome: effect on self-concept. American Journal of Medical Genetics, 92(5), 336–342.PubMedCrossRefGoogle Scholar
  25. McConkie-Rosell, A., Spiridigliozzi, G. A., Dawson, D., Sullivan, J., & Lachiewicz, A. M. (2001). Longitudinal study of the carrier testing process for fragile X syndrome: perceptions and coping. American Journal of Medical Genetics, 98(1), 37–45.PubMedCrossRefGoogle Scholar
  26. Meeus, W. (2011). The study of adolescent identity formation 2000–2010: a review of longitudinal research. Journal of Research on Adolescence, 21(1), 75–94.CrossRefGoogle Scholar
  27. Nolin, S., Brown, W. T., Glicksman, A., Houch, G., Gargano, A., Sullivan, A., et al. (2003). Expansion of the fragile X CGG repeat in females with premutation or intermediate alleles. American Journal of Human Genetics, 72, 454–464.PubMedCrossRefGoogle Scholar
  28. Potter, W. J., & Levine-Donnerstein, D. (1999). Rethinking validity and reliability in content analysis. Journal of Applied Communication Research, 27, 258–284.CrossRefGoogle Scholar
  29. Rothermund, K. (2011). Counter-regulation and control-dependency: affective processing biases in the service of action regulation. Social Psychology, 42(1), 56–66.CrossRefGoogle Scholar
  30. Stein, K. F. (1995). Schema model of the self-concept. Image: Journal of Nursing Scholarship, 27(3), 187–193.CrossRefGoogle Scholar
  31. Stryker, S. (1991). Exploring the relevance of social cognition for the relationship of self and society: Linking the cognitive perspective and identity theory. In J. A. Howard & P. L. Callero (Eds.), The self-society dynamic: Cognition, emotion, and action (pp. 19–41). New York: Cambridge University Press.CrossRefGoogle Scholar
  32. Thoits, P. A. (1991). On merging identity theory and stress research. Social Psychology Quarterly, 54(2), 101–112.CrossRefGoogle Scholar
  33. Wrosch, C., Scheier, M. F., Carver, C. S., & Schulz, R. (2003). Adaptive self-regulation of unattainable goals: goal disengagement, goal reengagement, and subjective well-being. Self and Identity, 2, 1–20.CrossRefGoogle Scholar

Copyright information

© National Society of Genetic Counselors, Inc. 2011

Authors and Affiliations

  • Allyn McConkie-Rosell
    • 1
    • 3
  • Elizabeth Melvin Heise
    • 1
  • Gail A. Spiridigliozzi
    • 2
  1. 1.Division of Medical Genetics, Department of PediatricsDuke University Health SystemDurhamUSA
  2. 2.Department of Pediatrics, Department of PsychiatryDuke University Health SystemDurhamUSA
  3. 3.Division of Medical GeneticsDuke University Health SystemDurhamUSA

Personalised recommendations