Measurement properties of scoring tools based on outcomes reported by people with cystic fibrosis (CF)
| Test or tool | Content validity | Convergent validity | Discriminant validity | Concurrent | Predictive | Intra or inter-rater and test–retest | Internal consistency | Measurement error | Responsiveness | Comments/MCID |
| Pulmonary exacerbations | ||||||||||
| AWESCORE | NR | NR | NR | Correlation of total AWESCORE and CFQ-R scores: r=0.632 (p=0.003) | NR | Pearson's correlation coefficient 0.854, p<0.0005 | NR | NR | For exacerbation, score 47.5 (sd 11.2) at start of treatment and 21.6 (sd 15.6) at end of treatment (100=highest symptom severity) | 11 points Mean change of −16.5 (95% CI −13.2 to −19.7 for exacerbation reported) No MCID for emotional score |
| CFRSD/CFRSD-CRISS | Involved people with CF in testing clarity of items | Step-rate significantly higher in those who did NOT experience difficulty breathing, cough, tightness or feeling tired (respiratory items) or feeling worried, cranky or frustrated (emotional items) | Respiratory scores distinguished between moderate/severe and mild/severe disease; emotional scores distinguished between mild/severe disease | Respiratory and emotional score established using daily step count (not FEV1) | NR | ICC 0.79 for respiratory scale using a 1-day interval | Cronbach's α for CFRSD-CRISS was 0.77 | Test–retest reliability after 7–10 days; 0.9 for the emotional and 0.93 for the chest score | Total score been demonstrated to improve over 2 weeks' i.v. treatment | No MCID suggested on the basis of statistical analysis, but MCID >1 after 2 weeks of i.v. ABX suggested based on experience with COPD patients |
| Symptom score | Patients not involved in construction | All 4 items correlated with each other r>0.38; p<0.001 | NR | Total score correlation with FEV1: r=−0.41, (p<0.0001) and respiratory score on CFQ-R: r=−0.62 (p<0.001) and CFQ-R: r=−0.47 (p<0.001) | NR | NR | NR | NR | NR | |
| Respiratory | ||||||||||
| Borg Dyspnoea Scale | NR | NR | NR | NR | NR | ICC=0.933 | NR | NR | Mean change in score −3.1 with mean effect size 1.3 from baseline to 4 weeks | Appears to be valid, reliable and responsive in CF For those reporting improvement, scores changed −2.9 overall, −3.5 for cough, −3.5 for congestion and −3.1 for sputum domains |
| ReS-CF | NR | NR | NR | Correlation between ReS-CF and CFQ-R; rs=−0.5 (p<0.001) | NR | ICCs for 4 scores >0.7 | NR | NR | NR | |
| SOBQ | NR | SOBQ scores correlated negatively with physiological measures of disease severity (FVC % pred: r=−0.36, p<0.05 and FEV1 % pred: r=−0.5, p<0.01) Scores correlated positively with Borg scale ratings of perceived breathlessness after 6MWT and QWB (r=−0.41, p<0.01) | NR | NR | NR | NR | α=0.96 | NR | NR | MCID: 5 unit change |
| Pain | ||||||||||
| BPI | NR | Correlation of BPI pain interference and airway clearance therapy (p=0.002), coughing and breathing (p<0.012), pain prevalence and CFQoL physical function (p=0.01), CFQoL treatment (p=0.03), CFQoL work/school (p=0.02), CFQoL social (p=0.013) and CFQoL emotional scale (p=0.017) Pain intensity also correlated with CFQoL physical function, CFQoL treatment and CFQoL school/work (p<0.01) | NR | Correlation of BPI pain prevalence and sleep quality (p=0.045), sleep disturbance (p<0.001), daytime dysfunction (p=0.001) and sleep interference and global BPI score rho-0.56, p<0.0001 OR 1.27 (p=0.012) of impaired sleep quality in those with pain | BPI pain severity correlated with risk of exacerbations (OR 1.65, p=0.04) for exacerbations with higher pain intensity and OR of 2.28 (p=0.008) of death with higher pain intensity | NR | NR | NR | NR | |
| DPAQ-CF | NR | CFQ-R social function (r=0.269, p<0.01), CFQ-R treatment burden (r=0.269, p<0.01), CFQ-R respiratory symptoms (r=0.241, p<0.05), HADS- depression scale (r=0.29, p<0.01) and HADS-anxiety (r=0.29, p<0.01) DPAQ-CF pain intensity correlated with CFQ- treatment burden and respiratory symptoms (p<0.01) DPAQ-CF pain duration correlated with CFQ-R treatment burden and respiratory symptoms (p<0.01) | NR | Correlation of pain and ppFEV1 R=0.239, P<0.05 | NR | NR | NR | NR | NR | |
| MPI | NR | Correlation of BPI pain severity and Shwachman scale history scale; r=0.24 (p=0.04) and BPI pain interference and total Shwachman score r=0.2 (p=0.09) | NR | NR | NR | NR | NR | NR | NR | |
| Abdominal | ||||||||||
| CF-Abd score | NR | NR | Differentiated patients with CF and healthy controls with large effect size (17.3+1.1 versus 8.0 +0.7 points; p<0.001; Cohen's d=0.85) | NR | NR | ICC 0.932 (95% CI 0.874–0.963) | Cronbach's α=0.7–0.92 | NR | NR | |
| Gastrointestinal symptom tracker | NR | Nutritional status is related to more stable lung function and fewer exacerbations | NR | NR | NR | Reliability established based on test–retest and internal consistency (unspecified) | NR | NR | NR | |
| Symptom and impact score | ||||||||||
| MSAS-CF | Developed in accordance with COSMIN recommendations; patients not consulted | MSAS-Resp: correlation with CFQ-R (r=−0.60, p<0.05) and CFQoL (r=−0.7, p<0.05) MSAS-Psych: emotional scale good correlation with CFQ-R (r=−0.69, p<0.05) Poor correlation with MSAS-GI: strongest with weight (CFQ-R r=−0.49, p<0.05) Subscales moderately correlated with symptoms on CFQ-R and CFQoL | Respiratory, gastrointestinal and psychiatric scores were higher in patients with low FEV1 <40% pred (p<0.05) | Correlation with CFQ-R respiratory score (r=−0.6) and CFQoL chest score (r=−0.7, p<0.05) and CFQ-R emotional functioning score (r=−0.69, p<0.05). Weak correlation with CFQ-R digestive score (r=−0.19, p<0.05) | NR | NR | α 0.74–0.86 High in all domains; MSAS-Physical α 0.92, MSAS-Psych α 0.95, MSAS-Global α 0.82 | NR | NR | General tool; not specific for exacerbations; originally developed for an oncology population |
MCID: minimal clinically important difference; AWESCORE: Alfred Wellness Score; CFRSD: CF respiratory symptom diary; CRISS: chronic respiratory infection symptom score; ReS-CF: respiratory symptoms in CF tool; SOBQ: Shortness of Breath Questionnaire; BPI: brief pain inventory; DPAQ-CF: Daily Pain Assessment Questionnaire in CF; MPI: multiple pain inventory; CF-Abd: CF abdominal; MSAS: Memorial Symptom Assessment Scale; NR: not reported; FEV1: forced expiratory volume in 1 s; FVC: forced vital capacity; ICC: internal consistency coefficient; ABX: antibiotics ;6MWT: 6-min walk test; QWB: Quality of Well-Being Questionnaire; HADS: Hospital Anxiety and Depression Scale; Resp: respiratory; GI: gastrointestinal; CFQ-R: CF Questionnaire-revised; COSMIN: Consensus-based Standards for the selection of health Measurement INstruments initiative.