PURPOSE: To examine the relationship between adolescents' families' perception of the severity of neurofibromatosis (NF1) and the clinical severity of NF1, a genetic condition with variable manifestations. METHODS: The Perception of Severity of Chronic Illness (PSCI) questionnaire was administered to 56 parents of 47 adolescents with NF1. Each participant was asked one open-ended question regarding the challenges of living with NF1. Scores assessing the clinical severity of NF1 were assigned by health care providers in the NF Clinic. Correlation coefficients and paired t-test were used to evaluate the relationship between the clinical severity and the families' perceptions. Qualitative data were reviewed and grouped into themes. RESULTS: Parental perceptions were correlated with the degree of medical (r = 0.3116, p <.05), cognitive (r = 0.4911, p <.0001), and behavioral (r = 0.3341, p <.05) impairment of the adolescent. Adolescent perception was correlated with the degree of cognitive impairment (r = 0.5429, p <.0001). Parental and adolescent perceptions were correlated (r = 0.6724, p <.0001); however, adolescents viewed the condition's impact as being less than their parents (p <.001). The qualitative data provide additional insight into the concerns of these families. CONCLUSIONS: Families dealing with more medical, cognitive, and behavioral complications of NF1 perceive the impact of the condition on daily life as being greater than those families with fewer complications. The quantitative and qualitative results of this study have several implications for the clinical care of adolescents with NF1 and their families.
PURPOSE: To examine the relationship between adolescents' families' perception of the severity of neurofibromatosis (NF1) and the clinical severity of NF1, a genetic condition with variable manifestations. METHODS: The Perception of Severity of Chronic Illness (PSCI) questionnaire was administered to 56 parents of 47 adolescents with NF1. Each participant was asked one open-ended question regarding the challenges of living with NF1. Scores assessing the clinical severity of NF1 were assigned by health care providers in the NF Clinic. Correlation coefficients and paired t-test were used to evaluate the relationship between the clinical severity and the families' perceptions. Qualitative data were reviewed and grouped into themes. RESULTS: Parental perceptions were correlated with the degree of medical (r = 0.3116, p <.05), cognitive (r = 0.4911, p <.0001), and behavioral (r = 0.3341, p <.05) impairment of the adolescent. Adolescent perception was correlated with the degree of cognitive impairment (r = 0.5429, p <.0001). Parental and adolescent perceptions were correlated (r = 0.6724, p <.0001); however, adolescents viewed the condition's impact as being less than their parents (p <.001). The qualitative data provide additional insight into the concerns of these families. CONCLUSIONS: Families dealing with more medical, cognitive, and behavioral complications of NF1 perceive the impact of the condition on daily life as being greater than those families with fewer complications. The quantitative and qualitative results of this study have several implications for the clinical care of adolescents with NF1 and their families.