Nutritional status is associated with health-related quality of life in children with cystic fibrosis aged 9-19 years.

BACKGROUND

The impact of improved nutritional status on health-related quality of life (HRQOL) is unknown for children with cystic fibrosis (CF).

METHODS

Associations between nutritional status and HRQOL were examined over 2 years in 95 children, aged 9-19 years, who were followed in the Wisconsin Newborn Screening Project. HRQOL was assessed using the Cystic Fibrosis Questionnaire (CFQ). Associations between height z-score (HtZ), BMI z-score (BMIZ) and seven CFQ dimensions were evaluated.

RESULTS

Mean values of at least 80 were observed for all CFQ dimensions except respiratory symptoms and treatment burden. Treatment burden was significantly worse in patients with meconium ileus (57) compared to pancreatic insufficient (65) and sufficient (78) subjects, p<0.0001. HtZ and BMIZ were positively associated with physical functioning and body image (p<0.05).

CONCLUSIONS

Better nutritional status was associated with increased HRQOL scores. Early diagnosis through newborn screening and improved nutrition provides an opportunity to enhance quality of life and body image perception.