Quality of life and exercise tolerance tools in children/adolescents with cystic fibrosis: Systematic review

Authors:
Blanco-Orive, P., Del Corral, T., Martín-Casas, P., Ceniza-Bordallo, G., and López-de-Uralde-Villanueva, I.
Abstract:
INTRODUCTION: This systematic review aims to analyze the validity and reliability of available tools to assess health-related quality of life (HRQoL) and exercise tolerance in children and adolescents with cystic fibrosis (CF).

MATERIALS AND METHODS: A review of observational studies studying the validity and reliability of the tools was conducted. The COSMIN (COnsesus-based Standards for the selection of health Measurements INstruments) guide was followed to analyze the quality of these tools.

RESULTS: A total of 18 studies were selected. Of the eight HRQoL tools, five generic and three specific, analyzed in 14 studies, the Cystic Fibrosis Questionnaire-Revised (CFQ-R) presents the best properties. Of the four tools analyzed to assess exercise tolerance, cycle ergometry and the modified shuttle test are the most valid and reliable.

CONCLUSIONS: The CFQ-R, cycloergometry and the modified shuttle test have the best properties for the assessment of children with CF, but more studies are needed.
DOI:
10.1016/j.medcli.2021.06.025
Journal:
Med Clin (Barc)
issn:
0025-7753
Publication year:
2021
pages:
Biological and physiological variables:
Biological and physiological variables
Functional status:
Physical functioning
General health perceptions / HRQoL:
Health-related quality of life
Age:
Children (0-18)
Disease:
Diseases of and symptoms related to the respiratory system
PRO / non-PRO:
Patient Reported Outcome
Type of measurement instrument:
1 - Questionnaires
4 - Performance-based tests
6 - Clinical rating scales
Instrument:
6MWT - 6 Minute Walk Test (versions: 6MWT app; 6MWT Lite app; M6MWT - Modified 6MWT; 6mWTPC - 6MWT on parquet and carpet)    |  | also see: database.cosmin.nl
CFQ - Cystic Fibrosis Questionnaire (versions: CFQ-R; 14+ for teens; Adolescents; Child; Parent/proxy)   
CFQoL - Cystic Fibrosis Quality of Life Questionnaire   
CPET - cardiopulmonary exercise testing
Cycle ergometry   
DISABKIDS (versions: DISABKIDS-37; condition-specific modules; DISABKIDS-CP)   
FLZ - Fragen zur Lebenszifriedenheit (Questions on Life Satisfaction) (versions: FLZM; FLZM-Cystic Fibrosis)
PLC - Quality of life profile for Chronic Diseases
SF-36 - 36-item Short Form Health Survey [alias: RAND-36][alias: MOS SF-36]] (versions: VSF-36 LoA - Visual Version of the SF-36 Limitations of Physical Activities domain; VR-36 - Veterans SF-36)    |  | also see: database.cosmin.nl
Shuttle Test (versions: Modified ST; Modified pro-ST; Modified long ST)

Tools