Figures
- FIGURE 1
Flow of consensus meetings involving stakeholders. PF-ILD: progressive fibrosing interstitial lung disease; PRO: patient-reported outcome.
- FIGURE 2
Important symptoms and impacts reported by participants in the online poll for determining treatment benefit. Other symptoms: chest discomfort 0%, other chest symptoms 0%, runny nose 0%. Other impacts: fear of infection 0%, loss of freedom 0%, oxygen use impacting on activities of daily living (ADLs) 0%, concern for family 0%, anxiety 0%, depression 0%, thoughts of death 0%, implication of disease 0%. SoB: shortness of breath.
- FIGURE 3
Final conceptual model. Concepts prioritised in the consensus meeting are in red and those prioritised in the online survey are in green. All concepts of high prevalence (at least one literature publication reporting a prevalence of ≥50%) are in bold. Impacts where the relationship to signs and symptoms was indirect/unclear are categorised as distal. PF-ILD: progressive fibrosing interstitial lung disease; DR: disease-related; TR: treatment-related.
- FIGURE 4
Process of developing the conceptual model. Green: prioritisation at online survey; red: prioritisation at consensus meeting. PF-ILD: progressive fibrosing interstitial lung disease. #: muscle loss, stiffness in joints, joint pain and swollen and inflamed joints. ¶: lack of psychological support, lack of satisfaction with healthcare and positive feelings/experiences with healthcare, concerns with diagnosis and need for more disease awareness, being more vigilant towards avoiding antigens. +: symptoms from the online survey were adapted from the preliminary consensus model: three phlegm symptoms (difficulty bringing up phlegm, producing phlegm and bad-tasting phlegm) were grouped together; the shortness of breath (SoB) concept was split into SoB at rest and SoB on exertion; change in appetite added to differentiate from loss of appetite. §: muscular wasting was added as part of the final conceptual model.
- FIGURE 5
a) The most important characteristics of the prioritised concepts; b) the most appropriate recall period; c) the most appropriate response scale reported by participants in the meeting to measure prioritised concepts. The total number of votes was 14 for most concepts, except where participants selected two categories when voting. Data are presented as percentage of total votes, with the actual number of votes shown above each bar. SoB: shortness of breath; ADLs: activities of daily living; VAS: visual analogue scale; NRS: numeric rating scale.
- FIGURE 6
Priority concepts for defining treatment benefit in progressive fibrosing interstitial lung disease (PF-ILD). NRS: numeric rating scale.
Tables
- TABLE 1
Summary of patient-reported outcome measure (PROM) questionnaires and concepts captured
Content validity Psychometric properties Items Recall period Response scale SoB on exertion Cough Fatigue Physical functioning ADL Emotional wellbeing K-BILD [17, 18] Input from patients with ILD (including IPF) Cross-sectional and longitudinal measurement properties established in patients with IPF 15 2 weeks 7-point Likert scale F: 3 items INF: 1 item INF: 1 item SGRQ-I [18, 19] Target: IPF
Rasch analysis using HRQoL data from RCTCross-sectional and/or without longitudinal measurement properties established in patients with IPF 34 Current/3 months/6 months/12 months 3–5-point Likert scale S: 1 item; INC: 4 items S: 2 items; INC: 1 item INC: 1 item S: 8 items; INC: 1 item; INF: 2 items INC: 5 items;
INF: 3 itemsL-IPF [20] Input from patients with IPF Cross-sectional measurement properties established patients with IPF 35 24 h (symptoms)
1 week (impacts)0–4 NRS format S: 7 items; INC: 1 item;
INF: 3 itemsF: 3 items;
S: 2 items; INF: 1 itemS: 3 items; INF: 1 item S: 6 items;
INF: 1 itemS: 5 items;
INF: 2 itemsL-PF [16] Input from patients with PF-ILD Not available 44 24 h (symptoms)
1 week (impacts)0–4 NRS format S: 12 items;
F: 1 item; INF: 3 items;
INC: 1 itemF: 5 items;
S: 2 items;
INC: 1 itemS: 3 items;
INF: 1 itemS: 8 items
INF: 1 itemS: 7 items;
INF: 3 itemsCASA-Q [21, 22] Input from patients with chronic bronchitis Cross-sectional and longitudinal measurement properties established in patients with COPD and chronic bronchitis 20 1 week 5-point Likert scale F: 2 items S: 1 item;
F: 2 itemsF: 1 item INF: 2 items INF: 4 items LCQ [23] Input from patients with chronic cough Cross-sectional and longitudinal measurement properties established in patients with chronic cough or COPD 19 2 weeks 7-point
Likert scaleS: 1 item; F: 1 item F: 2 items INF: 2 items E-RS (COPD+IPF) [24, 25] Input from patients with COPD Cross-sectional and longitudinal measurement properties established in patients with COPD 11 24 h 5-point
Likert scaleS: 5 items F: 1 item S: 1 item INF: 1 item INF: 2 items Input from patients with IPF Cross-sectional and longitudinal measurement properties established in patients with IPF FACIT-Dyspnoea [26] Input from patients with dyspnoea Cross-sectional measurement properties established in patients with dyspnoea Item bank: 33; short form: 10 1 week 4-point Likert scale S: 34 items INF: 12 items INF: 18 items FACIT-Fatigue [27] Input from patients with anaemic cancer Cross-sectional measurement properties established in a mixed cancer patient population 40 1 week 5-point Likert scale S: 7 items
INF: 1 itemINF: 3 items
S: 2 itemsINF: 1 item SoB: shortness of breath; ADL: activity of daily living; K-BILD: King’s Brief Interstitial Lung Disease; SGRQ-I: idiopathic pulmonary fibrosis (IPF)-specific version of St George's Respiratory Questionnaire; L-IPF: Living with Idiopathic Pulmonary Fibrosis; L-PF: Living with Pulmonary Fibrosis; CASA-Q: Cough and Sputum Assessment Questionnaire; LCQ: Leicester Cough Questionnaire; E-RS: Evaluating Respiratory Symptoms; FACIT: Functional Assessment of Chronic Illness Therapy; ILD: interstitial lung disease; F: frequency; INF: interference; HRQoL: health-related quality of life; RCT: randomised controlled trial; PF-ILD: progressive fibrosing interstitial lung disease; S: severity; INC: incidence; NRS: numeric rating scale.
- TABLE 2
Review of the Living with Pulmonary Fibrosis (L-PF) questionnaire for measuring symptoms and impacts of progressive fibrosing interstitial lung disease (PF-ILD)
Consensus recommendation Relevant items from the L-PF Comments Interference on daily life of SoB (on exertion) in past 24 h Interference on daily life of SoB
Impacts module:
1) How much did shortness of breath prevent you from doing things you wanted to do?
2) How much did fear of becoming too short of breath limit your physical exertion?
17) How has shortness of breath affected your quality of life?The L-PF has 17 items measuring the incidence, severity, frequency and interference of SoB upon exertion. Further thought is needed as to whether the three impact items on interference should be used in isolation and how a score would be calculated.
The recall period for the three items on interference also assesses the past week, rather than 24 h. The other 14 items in the symptoms module assesses SoB over the past 24 h.
The L-PF response scale (0–4 numeric scale) is consistent with consensus recommendationsFrequency of cough in past 24 h Frequency of cough
Symptoms module:
13) Over the last 24 h, how often did you cough?
14) Over the last 24 h, how often did you cough when you took a deep breath?
15) Over the last 24 h, how often did you cough when you were breathing hard or fast?
16) Over the last 24 h, how often did you cough when you over-exerted yourself?
17) Over the last 24 h, how often did coughing make you short of breath?The L-PF has five items for measuring the frequency of cough over the past 24 h. Three other items assess the severity and incidence of cough. Interference on daily life of fatigue in past week Interference on daily life of fatigue
Impacts module:
19) How much has your energy level affected your quality of life?The L-PF questionnaire may lack specificity in the impact items for fatigue. There is only one fatigue-related item, which focuses on energy levels.
If a treatment benefit on fatigue was expected, a fatigue-specific questionnaire may be needed in addition to the L-PFSeverity of physical functioning limitations in past week Severity of physical functioning limitations
Symptoms module:
4) How short of breath did walking up a short, gradual incline make you?
6) How short of breath did walking outside on a level surface make you?
7) How short of breath did walking from room to room inside your home make you?
8) How short of breath did getting ready to leave your home (e.g. find keys, put on coat, lock doors) make you?
10) How short of breath did doing light cleaning around the house make you?
12) How short of breath did lifting and carrying a light load a short distance make you?
Impacts module:
14) How much did you have to pace yourself to make it through the day?
15) How much time did it take to get yourself ready to leave the house?The L-PF has six items in the symptoms module (24 h) and two items in the impacts module (past week) that assess the severity of physical functioning. The items in the symptoms module focus on SoB. Severity of ADL limitation in past week Severity of ADL limitation
Symptoms module:
1) How short of breath did getting dressed make you?
2) How short of breath did walking up one flight of stairs make you?
3) Over the last 24 h, how short of breath have you been while sitting down, relaxing, reading or watching TV?
5) How short of breath did grooming make you?
6) How short of breath did walking outside on a level surface make you?
9) How short of breath did bathing or showering make you?
Impacts module:
3) How was your stamina when you exerted physically?The L-PF has six items in the symptoms module (24 h) and one item in the impacts module (past week) that assess the severity of ADL limitation. The items in the symptoms module focus on SoB. Interference on daily life of emotional wellbeing in past week NA NA SoB: shortness of breath; ADL: activity of daily living; NA: not applicable.
Supplementary Material
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Supplementary material 00681-2021.SUPPLEMENT
