Comprehensive Quality of Life (QoL) in Neurofibromatosis Type II (NF2): Development, Refinement and Standardization of a Multi-Dimensional Metric
Abstract
Neurofibromatosis Type 2 (NF2), a genetic disorder with highly penetrant autosomal dominant transmission, is characterized by the predictable development of bilateral vestibular schwannomas (VS), and other benign intracranial and spinal tumors. Tumor progression, as well as therapeutic intervention, may lead to significant cranial, spinal and peripheral nerve dysfunction, resulting in global impairment across of variety of domains. Few NF2-specific, validated metrics are currently available. The objective of this research was to develop, refine and validate a multi-dimensional metric for assessment of QoL in patients with NF2. A reliable, externally valid NF2-specific 63-item QoL module was created and tested in an NF2 population (n=118.) Results were analyzed and compared with data from the normative population data as well as cancer. Overall, QoL in NF2 patients was lower than that of the general population (p < 0.01) and similar to that of patients with cancer. Patients whose treatment included radiation therapy had worse overall QoL than patients who did not undergo radiation. Domains related to facial function, hearing and balance were significantly correlated with QoL: patients with more facial weakness, hearing loss and imbalance reported lower QoL. Domains most predictive of overall QoL were psychosocial, future uncertainty and pain. When compared to cancer patients (including sub-groups with head and neck and brain cancer,) responses of NF2 patients demonstrated significantly higher levels of psychosocial stressors, including disease-related anxiety, personal and financial stress and lack of social support. These results demonstrate a significant role for psychosocial and pain domains in NF2 QoL and suggest that these areas may not be adequately addressed by current treatment protocols. Utilization of expertise in areas of mental health, pain management and financial counseling could make an important impact on QoL in this population.
Document Details
- Document Type
- Technical Report
- Publication Date
- Dec 01, 2012
- Accession Number
- ADA591083
Entities
People
- Maura Cosetti
Organizations
- New York University