Abstract
Emerging adulthood is an important period in the development of one’s identity and autonomy. The ways in which identity and autonomy are viewed by emerging adults and how they impact quality of life (QoL) in individuals with early-onset neuromuscular conditions is not yet known. This study focused on understanding and exploring relationships between self-perceptions of emerging adulthood, autonomy, and QoL. Five previously validated measures were incorporated into an online survey and distributed to young adults with early-onset neuromuscular conditions and unaffected controls. Topics explored included individuals’ views regarding their overall QoL, disease-specific QoL, components of emerging adulthood, and autonomy. We found that a sense of higher disease impact was associated with a lower Overall General QoL. Additionally, perceptions of key autonomy factors “negativity” and “instability” were uniquely associated with Overall General QoL in the case group as compared to controls, whereas “attitudinal autonomy” (attaining the ability to plan and follow through with goals) was important to this age group regardless of health status. The specific factors of emerging adulthood and autonomy that were significantly correlated with Overall General QoL can be used for developing targeted counseling and interventions to improve QoL for individuals and their families.
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References
Anderson, R. A., Worthington, L., Anderson, W. T., & Jennings, G. (1994). The development of an autonomy scale. Contemporary Family Therapy, 16(4), 329–345.
Arnett, J. J. (2000). Emerging adulthood: a theory of development from the late teens through the twenties. American Psychologist, 55(5), 469–480.
Arnett, J. J. (2003). Conceptions of the transition to adulthood among emerging adults in American ethnic groups. New Directions for Child and Adolescent Development, 100, 63–75.
Bailey, S., O’Connell, B., & Pearce, J. (2003). The transition from paediatric to adult health care services for young adults with a disability: an ethical perspective. Australian Health Review, 26(1), 64–69.
Blomquist, K. B. (2007). Health and independence of young adults with disabilities. Two years later. Orthopaedic Nursing, 26(5), 296–309.
Brondolo, E., Gallo, L. C., & Myers, H. F. (2009). Race, racism and health: disparities, mechanisms, and interventions. Journal of Behavioral Medicine, 32, 1–8.
Carter, G. T., Han, J. J., Abresch, R. T., & Jensen, M. P. (2007). The importance of assessing quality of life in patients with neuromuscular disease. American Journal of Hospice and Palliative Medicine, 23(6), 493–497.
Cohen, J. S., & Biesecker, B. B. (2010). Quality of life in rare genetic conditions: a systematic review of the literature. American Journal of Medical Genetics Part A, 152A, 1136–1156.
Editors. (1992). Editorial: health-related quality of life research. Quality of Life Research, 1(1), 3.
Galambos, N. L., Darrah, J., & Magill-Evans, J. (2007). Subjective age in the transition to adulthood for persons with and without motor disabilities. Journal of Youth and Adolescence, 36(6), 825–834.
Galambos, N. L., Magill-Evans, J., & Darrah, J. (2008). Psychosocial maturity in the transition to adulthood for people with and without motor disabilities. Rehabilitation Psychology, 53(4), 498–504.
Grootenhuis, M. A., de Boone, J., & van der Kooi, A. J. (2007). Living with muscular dystrophy: health related quality of life consequences for children and adults. Health and Quality of Life Outcomes, 5(31), 1–8.
Hamilton, R., Williams, J. K., Bowers, B. J., & Calzone, K. (2009). Life trajectories, genetic testing, and risk reduction decisions in 18–39 year old women at risk for hereditary breast and ovarian cancer. Journal of Genetic Counseling, 18(2), 147–159.
Köpke, S., Kasper, J., Mühlhauser, I., Nübling, M., & Heesen, C. (2009). Patient education program to enhance decision autonomy in multiple sclerosis relapse management: a randomized-controlled trial. Multiple Sclerosis, 15, 96–104.
National Society of Genetic Counselors, Inc. (2007). Genetic Counselors’ Scope of Practice. Accessed June 23, 2011 at http://www.nsgc.org/client_files/SOP_final_0607.pdf.
Navon, S. (1999). The non-illness intervention model: psychotherapy for physically ill patients and their families. The American Journal of Family Therapy, 27(3), 251–270.
Nelson, L. J., & Barry, C. M. (2005). Distinguishing features of emerging adulthood: the role of self-classification as an adult. Journal of Adolescent Research, 20(2), 242–262.
Noom, M. J., Dekovic, M., & Meeus, W. (2001). Conceptual analysis and measurement of adolescent autonomy. Journal of Youth and Adolescence, 30(5), 577–595.
Raphael, D., Renwick, R., & Brown, I. (1996). The quality of life profile—adolescent version: background, description, and initial validation. Journal of Adolescent Health, 19, 366–375.
Raphael, D., Renwick, R., & Brown, I. (1998). Quality of life profile: Adolescent version. Quality of Life Resources Adolescents Series, Item #6-1. Toronto: Centre for Health Promotion, University of Toronto.
Reifman, A., Arnett, J. J., & Colwell, M. J. (2007). Emerging adulthood: theory, assessment, and application. Journal of Youth Development, 1, 1–12.
Rolland, J. S. (1994). Families, illness, and disability. New York: Basic Books, A Division of HarperCollins Publishers, Inc.
Rolland, J. S., & Williams, J. K. (2005). Toward a biopsychosocial model for 21st-century genetics. Family Process, 44(1), 3–24.
Rose, M., Kohler, K., Kohler, F., Sawitzky, B., Fliege, H., & Klapp, B. F. (2005). Determinants of the quality of life of patients with congenital heart disease. Quality of Life Research, 14, 35–43.
Rose, M., Brooks, V., Walburn, J., Sanders, D., Pandya, S., Kissel, J. et al. (2006). Validation of a quality of life measure for myasthenia gravis. Neuromuscular Disorders, 16, supplement: S152 (Abstract).
Szyndler, J. E., Towns, S. J., van Asperen, P. P., & McKay, K. O. (2005). Psychological and family functioning and quality of life in adolescents with cystic fibrosis. Journal of Cystic Fibrosis, 4, 135–144.
Uhlmann, W. R., Schuette, J. L., & Yashar, B. M. (2009). A guide to genetic counseling. NY: Wiley.
Vincent, K. A., Carr, A. J., Walburn, J., Scott, D. L., & Rose, M. R. (2007). Construction and validation of a quality of life questionnaire for neuromuscular disease (INQoL). Neurology, 68(13), 1051–1057.
Westwood, A., Henley, L. D., & Willcox, P. (1999). Transition from paediatric to adult care for persons with cystic fibrosis: patient and parent perspectives. Journal of Paediatrics and Child Health, 35, 442–445.
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Huismann, D.J., Sheldon, J.P., Yashar, B.M. et al. Quality of Life and Autonomy in Emerging Adults with Early-Onset Neuromuscular Disorders. J Genet Counsel 21, 713–725 (2012). https://doi.org/10.1007/s10897-012-9492-z
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DOI: https://doi.org/10.1007/s10897-012-9492-z