Allison Gomes1, Rachael Patusco2, Mei Chung3, Margaret R Dreker4, Laura Byham-Gray5, Craig Lapin6, Jane Ziegler7. 1. Department of Nutrition, Cedar Crest College, Allentown, Pennsylvania, USA. 2. Medical Affairs-Wellness, GlaxoSmithKline Consumer Healthcare, USA. 3. Department of Public Health and Community Medicine, School of Medicine, Tufts University, Medford, Massachusetts, USA. 4. Interprofessional Health Sciences Library, Hackensack Meridian School of Medicine, Nutley, New Jersey, USA. 5. Department of Clinical and Preventive Nutrition Sciences, School of Health Professions, Patient-Centered Outcomes Research in Nutrition-PCORN Lab, Rutgers University, Newark, New Jersey, USA. 6. Pulmonary Division, Connecticut Children's Medical Center, Hartford, Connecticut, USA. 7. Department of Clinical and Preventive Nutrition Sciences, School of Health Professions at Rutgers University, Newark, New Jersey, USA.
Abstract
BACKGROUND: Cystic fibrosis (CF) is a life-shortening genetic disease, yet life expectancy has recently increased, shifting the focus to disease management and health-related quality of life (HRQoL). Identification of clinical factors, such as weight status and CF-related diabetes (CFRD), that are associated with HRQoL can inform clinicians about the patient's health perception. The goal of this systematic review was two prong: identify the association of pediatric weight status and HRQoL and determine how CFRD status impacts HRQoL. METHODS: A systematic review of published research was conducted following the methodology in the Cochrane Handbook on Systematic Reviews for Interventional Studies. Results were reported according to the Preferred Reporting Items for Systematic Review and Meta-Analyses. Risk of bias was assessed using the National Heart Lung & Blood Institute tool. A meta-analysis was not performed due to variability of the inclusion/exclusion criteria, differences in outcome reporting, and insufficient primary outcome data to pool. RESULTS: Nine studies met inclusion criteria (n = 6 explored weight status and n = 3 studied CFRD), for a total of 1585 subjects (CFRD cases = 87). Pediatric weight status was positively associated with HRQoL, most commonly the Body Image and Eating Disturbance domains. CFRD was negatively associated with HRQoL, specifically the Treatment Burden and Weight domains. CONCLUSIONS: Based on the limited data available, improved pediatric weight status appears to increase HRQoL while a CFRD diagnosis appears to decrease HRQoL. More research is needed to fully understand the role of these clinical factors on HRQoL, especially with life expectancy increasing among those with CF.
BACKGROUND:Cystic fibrosis (CF) is a life-shortening genetic disease, yet life expectancy has recently increased, shifting the focus to disease management and health-related quality of life (HRQoL). Identification of clinical factors, such as weight status and CF-related diabetes (CFRD), that are associated with HRQoL can inform clinicians about the patient's health perception. The goal of this systematic review was two prong: identify the association of pediatric weight status and HRQoL and determine how CFRD status impacts HRQoL. METHODS: A systematic review of published research was conducted following the methodology in the Cochrane Handbook on Systematic Reviews for Interventional Studies. Results were reported according to the Preferred Reporting Items for Systematic Review and Meta-Analyses. Risk of bias was assessed using the National Heart Lung & Blood Institute tool. A meta-analysis was not performed due to variability of the inclusion/exclusion criteria, differences in outcome reporting, and insufficient primary outcome data to pool. RESULTS: Nine studies met inclusion criteria (n = 6 explored weight status and n = 3 studied CFRD), for a total of 1585 subjects (CFRD cases = 87). Pediatric weight status was positively associated with HRQoL, most commonly the Body Image and Eating Disturbance domains. CFRD was negatively associated with HRQoL, specifically the Treatment Burden and Weight domains. CONCLUSIONS: Based on the limited data available, improved pediatric weight status appears to increase HRQoL while a CFRD diagnosis appears to decrease HRQoL. More research is needed to fully understand the role of these clinical factors on HRQoL, especially with life expectancy increasing among those with CF.