The onset of liver damage from cystic fibrosis liver disease (CFLD) occurs before adulthood. A person may experience no symptoms at all or only minor ones. Patient health may deteriorate with progressive liver injury even before liver disease becomes clinically apparent. To further understand how early CFLD affects both overall and disease-specific quality of life, researchers conducted a comprehensive analysis of Health-Related quality of life (HRQOL) data from children participating in a multi-center research study of CFLD. Ultrasound (US) patterns  of normal (NL), heterogeneous (HTG), homogeneous (HMG), or nodular (NOD) to indicate those at risk for progressive CFLD. They shared the outcomes with the parents.  They assessed parent/child-reported (age ≥5 years) HRQOL by PedsQL 4.0 Generic Core and CF Questionnaire-revised (CFQ-R) prior to the US and annually. 

HRQOL was compared by US pattern before, after, and 5 years after the intervention. If there are significant differences between the various US groups, a MANOVA with a Hotelling-Lawley trace will show that. There was no evidence that HTG or NOD US was linked with decreased PedsQL/CFQ-R at baseline among 515 participants and their parents before US. No improvement was noted in PedsQL/CFQ-R scores from baseline to 12 months later among parents of children with NOD. Many scales, including physical function, on the child-reported PedsQL/CFQ-R (95 NL, 20 NOD), improved between baseline and year 5. Parents of children with HMG said their children’s CFQ-R weight ratings improved.

HRQoL was impressively consistent in children with CF, independent of liver involvement, even in those with early, undiagnosed, or pre-symptomatic liver illness.