The associations between pediatric weight status and cystic fibrosis‐related diabetes status and health‐related quality of life among children and young adults with cystic fibrosis: A systematic review

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.

Cystic Fibrosis-Related Diabetes in Poland

“…Generally, it is estimated that 70% to 90% of people with CF will have been diagnosed with one type of diabetes by the age of 40 [ 20 ]. Evidently, patients with CFRD have a significantly reduced health-related quality of life, and their diagnosis and treatment require the coordinated work of specialists from various fields of medicine [ 21 , 22 ]. Moreover, they have difficulties with higher-level processes known as “executive function”, which demand greater cognitive load and recruit the prefrontal cortex [ 23 ].…”

Polish Cystic Fibrosis Patients’ Health-Related Quality of Life and Its Influencing Factors: A Cross-Sectional, Single-Centre Study

“…For the purpose of the study and statistical analysis, results were collected in the of calculated percent predicted values in accordance with patients’ height, weight, age, sex and racial background. Spirometry was performed in accordance with the recommendations of the European Respiratory Society and the American Thoracic Society [ 22 , 23 ].…”

“…Frequent exacerbations, hospitalisations, the need to spend time on often time-consuming therapies and limitations in everyday functioning are some of the factors that can negatively affect patients’ quality of life. According to studies and systematic reviews, the most important factors are FEV1% (percent predicted forced expiratory volume in 1 second), patients’ sex, body mass index (BMI), age and pulmonary exacerbations [ 19 , 20 , 21 , 22 ].…”

Evolution of nutritional management in children with cystic fibrosis – a narrative review

“…It is well documented and established that optimising nutritional status improves lung function, 1 survival 2 and quality of life 3 . Consequently, international CF organisations across four continents developed nutritional guidelines, aiming to optimise patient nutritional status as part of their long‐term management goals 4–8 .…”

“…It is well documented and established that optimising nutritional status improves lung function, 1 survival 2 and quality of life. 3 Consequently, international CF organisations across four continents developed nutritional guidelines, aiming to optimise patient nutritional status as part of their long-term management goals. [4][5][6][7][8] Evidence now suggests that the implementation of these guidelines, as early as possible in patient care, has been instrumental in improving patient nutritional status over time (reflected as body mass index [BMI] changes), [9][10][11] and thus survival.…”