Knowledge and Self-Esteem of Individuals with Neurofibromatosis Type 1 (NF1)
Neurofibromatosis Type 1 (NF1) is a progressive genetic disorder characterized by physical findings such as café-au-lait macules, Lisch nodules, and neurofibromas in addition to other medical complications. Learning and social problems are more prevalent among individuals affected with NF1. It has been reported that people with NF1 have lower self-esteem (SE) when compared to the general population. Additionally, a study published over 20 years ago found that overall knowledge of NF1 was lacking in individuals affected with the condition. The goals of our study were to evaluate NF1 knowledge in adolescents and adults with the condition, as well as to determine if there is a link between patient knowledge and SE. Furthermore, we explored the impact of other factors, such as attendance at a NF1 support group and having a family history of NF1, on knowledge and SE. A survey comprised of knowledge-based questions and the Rosenberg Self-Esteem Scale was distributed to individuals with NF1 through the Texas NF Foundation. Overall, the 49 respondents (13 to 73 years of age) had a mean knowledge score of 77.9 % correct answers. Consistent with previous studies, the SE of our study population was lower when compared to general population norms. Although no correlation between knowledge and SE was observed, SE scores were on average higher if a person reported the following: having friends with NF1 (p = 0.009); attending a NF1 support group (p = 0.006); receiving care at a NF clinic (p = 0.049); or having received genetic counseling (p = 0.008). Further research is needed to better understand the relationship between these factors and SE in the NF1 population.
KeywordsNeurofibromatosis Self-esteem Knowledge Education Coping
This study was completed in partial fulfillment of the requirements for the first author’s Master of Science in genetic counseling from University of Texas Health Science Center at Houston. The authors would like to thank the cooperation and support of the Texas NF Foundation during this study.
Compliance with Ethical Standards
Conflict of Interest
The authors declare that they have no conflict of interest.
Human Studies and Informed Consent
All procedures performed in studies involving human participants were in accordance with the ethical standards of the institutional and/or national research committee and with the 1964 Helsinki declaration and its later amendments or comparable ethical standards. Informed consent was obtained from all individual participants included in the study.
No animal studies were carried out by the authors for this article.
- Krab, L. C., Oostenbrink, R., de Goede-Bolder, A., Aarsen, F. K., Elgersma, Y., & Moll, H. A. (2009). Health-related quality of life in children with neurofibromatosis type 1: contribution of demographic factors, disease-related factors, and behavior. The Journal of Pediatrics, 154(3), 420-425–425 e421. doi: 10.1016/j.jpeds.2008.08.045.CrossRefGoogle Scholar
- Oates, E. C., Payne, J. M., Foster, S. L., Clarke, N. F., & North, K. N. (2013). Young Australian adults with NF1 have poor access to health care, high complication rates, and limited disease knowledge. American Journal of Medical Genetics Part A, 161A, 659–666. doi: 10.1002/ajmg.a.35840.CrossRefPubMedGoogle Scholar
- Plumridge, G., Metcalfe, A., Coad, J., & Gill, P. (2011). The role of support groups in facilitating families in coping with a genetic condition and in discussion of genetic risk information. Health Expectations, 15, 255–266. doi: 10.1111/j.1369-7625.2011.00663.x.CrossRefPubMedPubMedCentralGoogle Scholar
- Psychological & Social Implications. (2010). Understanding genetics: A New England guide for patients and health professionals. Needham, MA: Genetic Alliance; New England Public Health Genetics Education Collaborative.Google Scholar
- Resta, R., Biesecker, B., Bennett, R., Blum, S., Estabrooks Hahn, S., Strecker, M., et al. (2006). A new definition of genetic counseling: National Society of genetic counselors’ task force report. Journal of Genetic Counseling, 15(2), 77–83. doi: 10.1007/s10897-005-9014-3.CrossRefPubMedGoogle Scholar
- Rosenberg, M. (1989). Society and the adolescent self-image. Revised edition. Middletown, Connecticut: Wesleyan University Press.Google Scholar
- Sinclair, S., Blais, M., Gansler, D., Sandberg, E., Bistis, K., & Locicero, A. (2010). Psychometric properties of the Rosenberg self-esteem scale: overall and across demographic groups living within the United States. Evaluation & the Health Professions, 31, 56–80. doi: 10.1177/0163278709356187.CrossRefGoogle Scholar