Table 2.
CF-specific PROMs
| PROM | Studies included | Year developed | Target population | Languages* | Number of items and domains | Psychometric properties |
| Cystic Fibrosis Questionnaire—Revised28 35 39–41 48 88 89 | 49 | 2003 | Teen/adult (14+ years) | English | Number of Items: | Reliability: α>0.7 except treatment burden and social functioning domains in some studies Test–retest reliability† >0.6 Validity: known groups validity with FEV1, age and body mass index Ceiling effects: eating disturbances (46.4%), body image (39.6%), digestion (37.2%) |
| Adolescent (12–13 years) | Polish | Adult: 50 | ||||
| Child (6–11 years) | German | Adolescent: 35 | ||||
| Parent (proxy for 6–13 years) | Hungarian | Child: 35 | ||||
| Dutch | Parent: 44 | |||||
| Hindi | Domains: physical, vitality, emotion, social, role/school, body image, treatment burden, health perceptions, weight, respiratory, digestion | |||||
| Portuguese | ||||||
| Spanish | ||||||
| Swedish | ||||||
| Turkish | ||||||
| Cystic Fibrosis Quality of Life Questionnaire3 29 32 50 53–56 71 79 90 | 14 | 2000 | Adult (14+ years) | English | Adult: 52 | Reliability: α: 0.72–0.95 |
| Polish | Domains: physical, social, treatment, emotional, relationships, career, future, chest symptoms, body image | Test–retest reliability >0.7 | ||||
| Greek | Validity: all domains correlated with FEV2, sensitive to change over time | |||||
| Portuguese | ||||||
| Cystic Fibrosis Questionnaire27 58 70 91–94 | 7 | 1997 | Teen/adult (14+ years) | English | Number of Items: | Reliability: α=0.62–0.93 for most domains in adult and child questionnaires |
| Child (6–13 years) | German | Adult: 48 | Validity: some domains correlated with FEV1 | |||
| Parent (proxy for 6–13 years) | Dutch | Adolescent: | ||||
| Portuguese | Child: 35 | |||||
| Parent: 44 | ||||||
| Domains: physical functioning, vitality, emotional state, social limitations, role/school, body image, treatment constraints, embarrassment, eating disturbances, health status, weight, respiratory, digestion | ||||||
| DISABKIDS-CF Module34 59 | 2 | 2013 | Child (8–17 years) | Portuguese | Number of items: 10 | Reliability: α: 0.71–0.76 |
| Parent (proxy for 8–17 years) | Domains: impact, treatment | Validity: good convergent and divergent validity assessed by MTMM | ||||
| Ceiling effects: 27.5% impact domain | ||||||
| CF Symptom Diary60 | 1 | 2009 | All ages | English | Number of items: 16 | Not reported |
| Domains: symptom, emotional impact, activity impact | ||||||
| Cystic Fibrosis Respiratory Symptom Diary26 | 1 | 2018 | CFRSD0–6 (proxy for 0–6 years) | English | Number of items: 17 | Validity: discriminates between sick and well patients with CF |
| CFRSD7–11 (proxy for 7–11 years) | Domains: respiratory signs, CF-related impacts | |||||
| Respiratory Symptoms in CF61 | 1 | 2017 | Adult (18+) | English | Number of items: 4 visual assessment scales | Test–retest reliability† >0.7 for 3/4 items |
| Validity: correlates with Cystic Fibrosis Questionnaire—Revised and responsive to changes in health | ||||||
| Cystic Fibrosis Symptom Progression Survey33 | 1 | 2015 | Child (0–15 years, self-report and proxy) | Arabic | Number of items: 10 | Reliability: α=0.76 |
| Validity: content validity demonstrated using factor analysis |
*Languages included in this review.
†Test–retest reliability measured by intraclass correlation coefficient.
CF, cystic fibrosis; CFRSD, Cystic Fibrosis Respiratory Symptom Diary; FEV1, forced expiratory volume in 1 s; MTMM, multitrait–multimethod matrix; PROM, patient-reported outcome measure.