Date of Graduation

5-2015

Document Type

Thesis (MS)

Program Affiliation

Genetic Counseling

Degree Name

Masters of Science (MS)

Advisor/Committee Chair

Myla Ashfaq, MS, CGC

Committee Member

Syed Hashmi, MD, MPH, PhD

Committee Member

Hope Northrup, MD

Committee Member

John Slopis, MD, MPH

Committee Member

Sarah Noblin, MS, CGC

Abstract

Neurofibromatosis Type 1 (NF1) is a progressive genetic disorder characterized mainly by café-au-lait macules, Lisch nodules, as well as cutaneous and subcutaneous neurofibromas among other traits. Due to the physical manifestations of the condition, it has been observed that individuals 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 poor in individuals affected with the condition. The goal of our study was to reassess knowledge in this population and investigate whether it is related to 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 old) had higher than expected knowledge of NF1 (mean score = 77.9% correct answers) across various aspects of the condition. Consistent with previous studies, the SE of our study population was lower compared to general population norms. Although there was no correlation detected between knowledge and SE, SE scores were on average higher if a person reported to have friends with NF1, attended an NF1 support group, attended a NF clinic, or received genetic counseling. Having friendships with people who have the same condition as well as attending support groups may help those affected by NF1 to feel less isolated. Additionally, genetic counseling provides an opportunity for these individuals to potentially identify ways to improve coping through positive coping strategies such as educating peers about NF1.

Keywords

neurofibromatosis, self-esteem, knowledge, education, stigma, cope

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