Family history does not influence stress or major coping styles in adults with neurofibromatosis type 1

Abstract

Neurofibromatosis type 1 (NF1) is a genetic condition that affects about 1 in 3000 individuals. Approximately 50% of individuals with NF1 have a family history of the condition. Individuals with NF1 experience variable symptoms that contribute to increased stress. This study investigated whether a family history of NF1 influences levels of stress and coping strategies in adults with NF1. Adults with NF1 who live in the United States and speak English were recruited through the Children's Tumor Foundation's (CTF) NF Registry, CTF's NF Clinic Network, and the Neurofibromatosis Network. Participants completed a survey about their personal and family history of NF1, the Perceived Stress Scale 10-Item Version (PSS-10), the Brief Coping Orientation to Problems Experienced Inventory (Brief-COPE), short response questions, and demographics. Overall, 547 of 646 responses met analysis criteria. Participants with affected parents were assigned to the inherited NF1 group (n = 222) and those with unaffected parents were assigned to the sporadic NF1 group (n = 325). No differences were found in mean PSS-10 scores between the two study groups (p = 0.568). Females had significantly higher PSS-10 scores than males (p < 0.001). After Bonferroni correction, no differences were found across Brief-COPE subscales or major coping styles between the two groups. A stagewise multivariable regression indicated that 42% of the variance in PSS-10 scores was accounted for by sex assigned at birth, age, problem-focused, and avoidant coping styles (R² = 0.42, p < 0.001). Family history did not predict PSS-10 scores alone or as an interaction variable with major coping styles. This study showed no significant differences in stress or major coping styles between adults with inherited versus sporadic NF1. However, other factors may influence the stress and coping experiences of adults with NF1. Fostering discussions about patients' stressors and coping strategies could help promote stress management.

Keywords: coping; family history; lived experience; neurofibromatosis type 1; psychosocial; stress.