OBJECTIVES

1. Health-Related Quality of Life in Adolescents with Neurofibromatosis-1: A Pattern of Similarity with Other Serious Chronic Illnesses Jessica M. Joseph1, Rebecca E. Shefsky1, W. Hobart Davies2, 3, Bonita P. Klein-Tasman1, & Molly M. Garwood3 1University of Wisconsin-Milwaukee, and 2Children’s Hospital of Wisconsin, 3Medical College of Wisconsin RESULTS RESULTS (cont) OBJECTIVES • HRQOL ratings from this sample were compared to previously established composites using one-sample t-tests (see Table 1). • Differences in HRQOL for children with and without a parent with NF-1 were also examined with independent samples t-tests (see Table 2). • Adolescent reports of HRQOL (M=77.30) were not significantly different than healthy controls (M=83.00) or the chronic illness composite (M=77.19). • There were also no significant differences based on fathers’ reports of total HRQOL (M=75.23) with either the chronic illness composite (M= 74.11) or the healthy controls (M = 87.61). • Mother’s reports of total HRQOL (M=69.04) did differ significantly from that of healthy controls (M=87.61) and was numerically less than but did not differ significantly from the chronic illness composite (M = 74.22). • No significant differences were found for youth-report HRQOL ratings between adolescents with a parent with NF-1 and adolescents without a parent with NF-1. • Neurofibromatosis (NF-1) is the most common single-gene autosomal dominant disorder (North et al., 1994) affecting approximately 1 in 3000 individuals of all races and ethnicities. • NF-1 is the result of a random or inherited genetic mutation with approximately 50% of individuals having a parent with NF-1. • Previous research has shown that adolescents with NF-1 report lower rates of health-related quality of life (HRQOL) than healthy children (Graf, et al., 2006; Wolkenstein, et a., 2006). • Preliminary research has also examined the impact of having a parent with NF-1 on HRQOL (Reiter-Purtill et al., 2008) which will be further examined in this study. • The goal of the current study is to compare HRQOL in adolescents with NF-1 with both healthy children and children with other chronic medical conditions using a measure well validated for a variety of pediatric populations. CONCLUSIONS • The use of the PedsQL allowed for the comparison of HRQOL in youth with NF-1 to other chronic illness populations and established control groups (Varni et al., 2001). • Child-, mother-, and father- HRQOL total score means were all closer to the scores of the chronic illness composite than the healthy controls although only mother-reported values were statistically different from healthy controls. • There were no significant differences between reported HRQOL and the chronic illness composites suggesting that adolescents with NF-1 and their parents are reporting rates of impaired quality of life similar to other chronic illness populations (Varni et al., 1999). • Adolescents’ self-report of their overall, physical and psychosocial functioning did not vary by parental NF-1 status. There was a large effect size found for youths’ reports of their ability to do physical activities, though, indicating that with a larger sample size a significant difference would be anticipated. • In summary, adolescents with NF-1 were described as having lower quality of life compared with adolescents without chronic illnesses at rates that were similar with other chronic illness populations. • Future work with multiple measures and larger samples would allow differences to be examined within the domains of quality of life and obtain a better description of the impact of having NF-1 on social, emotional, school and physical functioning. METHODOLOGY *T-test is significant at the 0.05 level (2-tailed), **T-test is significant at the 0.01 level (2-tailed) • Participants were recruited through a NF-1 clinic at a large Midwestern children’s hospital. Participants had a diagnosis of NF-1, were between the ages of 12 and 18 years, and lived within 120 miles of the hospital. Families were interviewed in their homes. • The final sample size was twenty-five adolescents ages 12 to 18 years (M = 13.96, SD = 2.03), with 56 percent of the sample being female. • Twenty-four mothers and 14 fathers participated in this study but demographic information was provided for all 50 parents. • Twelve of the adolescents (48%) had a parent with NF-1. • HRQOL was assessed with the Pediatric Quality of Life Inventory v 4.0 (PedsQL; Varni et al., 2001). Higher HRQOL scores indicate better functioning. • NF-1 HRQOL scores were compared with healthy control and chronic illness composites that were established by Varni (2001) from a sample of 963 children and 1677 parents who completed the PedsQL during their visits to hospital specialty clinics. CONTACT INFORMATION Corresponding Author: Jessica M. Joseph, B.A. University of Wisconsin-Milwaukee 2441 E. Hartford Ave. Milwaukee, WI 53211 Email address: josephjm@uwm.edu *T-test is significant at the 0.05 level (2-tailed), **T-test is significant at the 0.01 level (2-tailed) Poster presented at the 2009 Midwest Conference on Pediatric Psychology, Kansas City, Missouri This project is supported by a grant awarded to the last author from the Children’s Research Institute, Children’s Hospital of Wisconsin.