Quality of Life Assessment in Patients with Neurofibromatosis Type II

Abstract

Objectives:

(1) Develop a multidimensional metric for assessing quality of life (QoL) in patients with NF2. (2) Compare NF2 results with reference values for the general population and patients with head and neck or brain cancer.

Methods:

Structured interviews with NF2 providers and patients identified relevant domains. Items in these domains were extracted from validated EORTC modules, combined with items unique to NF2 and pre-tested on NF2 providers and patients (N = 118). The questionnaire included 61 items assessing overall QoL and 10 additional domains including hearing, balance, facial function, vision, oral intake, future uncertainty, psychosocial, cognition, sexual activity, pain and vocal communication. Responses were compared to reference values for the general population and head and neck cancer and brain cancer patients.

Results:

QoL in NF2 patients was lower than that of the general population (P < .01) and similar to that of patients with cancer. NF2 patients whose treatment included radiation therapy had worse overall QoL than patients who did not undergo radiation. Patients with more facial weakness, hearing loss and imbalance reported significantly lower QoL. However, domains most predictive of overall QoL were psychosocial, future uncertainty and pain. When compared to patients with head and neck and brain cancer, NF2 patients demonstrated significantly higher levels of psychosocial stressors, including disease-related anxiety, personal and financial stress, and lack of social support (P < .01).

Conclusions:

Psychosocial stress and pain significantly impact QoL in NF2 indicating that mental health, pain management, and financial counseling could have an important impact on QoL in this population.