Funding Agency: the National Institute on Dental and Craniofacial Research

The purpose of the 2-year Phase I study which ended 1/31/02, was the development of craniofacial-specific quality of life (QoL) modules to augment the generic Youth Quality of Life Instrument (YQOL), developed by Patrick, Edwards, and Topolski. In the Phase I study, the following data were collected: (1) 33 semi-structured, in-depth interviews with adolescents aged 12-18 with a range of congenital and acquired craniofacial conditions (CFCs) on how their CFCs affected their lives; (2) a subset of 15 in-depth interviews with parents of these adolescents; (3) one focus group with young adults with CFCs; (4) one focus group with parents of adolescents with CFCs; (5) the YQOL was administered to 56 adolescents with CFCs; and (6) the contextual (verifiable) portion of the YQOL was administered to 52 parents of adolescents with CFCs. The YQOL contains two types of items: 'perceptual', known only to the adolescent, and 'contextual', which are potentially verifiable. In addition, an advisory meeting was held with 6 clinical and academic experts working in the field, in which the newly developed Youth Quality of Life Instrument - Facial Differences module (YQOL-FD) and Facial Surgery (YQOL-FS) module were evaluated. The adolescents who were interviewed were purposively selected to represent a broad range of craniofacial conditions: (1) acquired (burns, gun shot wound, etc.); (2)birth marks; (3)branchial arch disorders; (4)isolated or syndromic craniosynostoses; (5) cleft lip and/or palate; and (6) other diverse conditions. The interviewees were evenly divided by gender and age.

We generated an item pool from (1) qualitative interviews and focus groups with adolescents with CFCs and their caregivers, and (2) input from "experts," clinicians working in the craniofacial field. Normally we would have consulted existing measures for this purpose as well, but a review of the literature revealed that the instruments available in the field are focused primarily on functional limitations and anatomical variables, and to a lesser degree on psychosocial maladjustment. We did not find any instruments which suited our purposes in developing QoL measures. One-hundred twenty-five perceptual items were retained and presented to the expert panel and a group of 6 parents for assessment. Each panel member/parent selected the 30 items which they thought best captured the QoL issues for adolescents with CFCs. This process resulted in 75 perceptual items, across seven categories: (1) coping;(2) intimacy, trust; (3) negative emotions; (4) positive consequences; (5) self-image; (6) stigma, isolation; and (7) surgery. The items in the surgery category comprise the YQOL-FS module, while those in the remaining six categories comprise the YQOL-FD module. The core group of investigators selected the YQOL-FD perceptual items for which verifiable items could be written to comprise a contextual set of items. Investigators wrote 18 contextual items using this process. The perceptual items were cognitively debriefed with four adolescents who participated in the original interviews. These adolescents were also asked to select the most important 30 perceptual items in their judgment. A readability analysis was conducted on the items, and necessary wording changes were made to ensure that they were all readable at the 5th grade level.

The analyses of the YQOL data collected from the adolescents with CFCs and their parents are still in progress, but preliminary results show that the total perceptual YQOL score for adolescents with CFCs is significantly lower than that for adolescents without any clinical conditions, and is comparable to adolescents diagnosed with attention-deficit hyperactivity disorder and those with mobility limitations (from data collected in a prior study). Thus, it appears that compared to adolescents without clinical conditions, general quality of life is lower for adolescents with CFCs, and is comparable to adolescents with other clinical conditions and disabilities.

The goal of the Phase II study which began 5/1/02 is to describe the impact of congenital and acquired craniofacial conditions on the health and quality of life of adolescents, and to validate CFC-specific quality of life outcome measures for evaluating effectiveness of treatments. This project builds on the prior qualitative phase and includes CFCs present at birth or acquired up to age 18. To achieve this goal, specific aims are:

1. To describe and compare the quality of life associated with craniofacial conditions and facial differences in adolescents aged 12 to 18;
2. To validate the Youth Quality of Life Instrument - Facial Difference Module (YQOL-FD): scale structure, test-retest reliability, discriminant and convergent validity, and responsiveness to change;
3. To evaluate the ability of the YQOL - Facial Surgery Attitudes Module (YQOL-FS) to predict changes in quality of life (as assessed by the YQOL-R generic measure and YQOL-FD) associated with treatment; and
4. To measure preferences of adolescents and their professional and family caregivers for further treatment (surgery and therapies) of facial differences, and to compare their perspectives on the point of "diminishing returns."

This project is being undertaken by CDPR's Youth Quality of Life Group
For more information about this group, go to www.yqol.org