TABLE 1

Characteristics of quality of life (QoL) tools developed for use in people with cystic fibrosis (CF)

Test or tool	Description	Constructs(s)	Target population	Administration	Recall period	Range of scores	Feasibility/cultural validity
CFIQ	40 items; 5–6 min to complete	Activity limitation (physical, social, leisure), school/work limitations, vulnerability/lack of control, emotional impact, treatment burden and future outlook	Children and adults with CF and their carers; interview templates for children aged 6–11 years, adolescents, adults >12 years and carers of children aged 0–18 years	Paper	36 items 7 day recall, the remainder “current status”	5- or 7-point verbal rating scale	Largely developed in Caucasian population; further validation required
CFQoL	QoL and symptom scoring tool; 52 items over nine domains; 15–20 min to complete	Two symptom scales (chest and emotional) and seven QoL domains: physical functioning (10), social functioning (4), treatment issues (3), future concerns (6), interpersonal relationships (10), body image (3), career issues (4)	Adults and adolescents	Paper	14 days	Each response 6-point Likert scale; total score 0–100	Time consuming
CFQoL scale (single item)	VAS: how has CF affected your QoL in the last 2 weeks? Couple of mins to complete	Single QoL question	Adults	Paper	14 days	0–10	Quick Validated in population who had not had pulmonary exacerbation for past 6 weeks
CFQ-R	QoL and symptom scoring tool; four scales: 1) CFQ-R >14 years (44 items over 9 domains); 2) CFQ-R child (8 domains, 35 items), interviewer-administered 6–13 years and self-report 12–13 years; 3) CFQ-R parent: 44 items, 10 min; 4) preschool 3–6 years, 28 items, 15 min to complete	Activity limitation (physical, social, leisure), school/work limitations, vulnerability, lack of control, emotional impact, treatment burden and future outlook	CFQ-R >14 years; CFQ-R child: interviewer- administered 6–13 years and self-report 12–13 years CFQ-R parent: proxy report for children 6–13 years Separate are not proxy report for children 4–60 months	Paper/electronic	14 days	4-point Likert scale; total score 0–100	Most widely used HRQoL questionnaire in CF; translated into 34 languages; EMA/FDA supports use in clinical trials
eCF-QUEST	Electronic, 3 domains, 4 items	Global measure (40 items), gastrointestinal (5 items) and general health (2 items)	Adults	Paper/electronic	NR	NR	NR
DISABKIDS-CF	6 items, 2 min to complete	Impact and treatment dimensions	Exclusively for use in children and adolescents 8–17 years; self-report and proxy version (carer)	Paper	Each dimension 0–100	5-point Likert scale; scores 0–100% (higher score=higher QoL)	English and Spanish versions
FLZ-CF	Healthy and general patient population; 18 items over 8 domains, 9-item weighted scale, 5 min to complete	9 items: cough/dyspnoea, abdominal, sleep, eating, therapy routine, adherence, understanding by others, being needed by others, disadvantage	Adolescents >15 years and adults	Paper	28 days	0–100 (high score=high satisfaction)	Screening test
CQOLCF	35 item carer QoL instrument, <10 minutes	Physical, emotional, family and social functioning	Carers of people with CF	Paper	NR	Each response 5-point Likert scale	NR

CFIQ: CF impact questionnaire; CFQoL: CF QoL; CFQ-R: revised CF questionnaire; eCF-QUEST: electronic version of the CFQoL evaluative self-administered test; FLZ-CF: Questions of Life Satisfaction; CQOLCF: caregiver QoL CF scale; VAS: visual analogue scale; HRQoL: health-related QoL; EMA: European Medicines Association; FDA: US Food and Drug Administration; NR: not reported.