ABSTRACT
Although patients with cystic fibrosis (CF) are experiencing increased longevity, it is unclear that improved quality of life (QoL) accompanies the greater quantity of life. This is especially true of children with CF where the burdens of treatment are substantial. Assessing QoL in such children is difficult, and only one instrument--the Quality of Well-Being (QWB) scale--seems to have been used extensively to assess QoL in children with CF. After thoroughly reviewing the literature, we surveyed the 113 certified CF centers in the United States about QoL assessment. With 84% responding, it was found that only 7 centers are assessing QoL--4 have been using the QWB instrument. Concurrently, we pilot tested the QWB instrument in children being followed longitudinally in the Wisconsin CF Neonatal Screening Project. Our results indicated that QWB scores reflecting QoL decreased as the number of respiratory infections increased, as the number of concurrent medical conditions increased, and as the number of different medications increased. However, we recognized problems with the assessed domains and data interpretation. In fact, the QWB instrument has limited sensitivity and responds more to the extent of physician-determined treatment than to QoL. There is a need for more QoL assessment and better instruments, particularly in young children with CF.