Supporting self-management for patients with Interstitial Lung Diseases: Utility and acceptability of digital devices.

INTRODUCTION: Patients diagnosed with Interstitial Lung Diseases (ILD) use devices to self-monitor their health and well-being. Little is known about the range of devices, selection, frequency and terms of use and overall utility. We sought to quantify patients' usage and experiences with home digital devices, and further evaluate their perceived utility and barriers to adaptation. METHODS: A team of expert clinicians and patient partners interested in self-management approaches designed a 48-question cross-sectional electronic survey; specifically targeted at individuals diagnosed with ILD. The survey was critically appraised by the interdisciplinary self-management group at Royal Devon University Hospitals NHS Foundation Trust during a 6-month validation process. The survey was open for participation between September 2021 and December 2022, and responses were collected anonymously. Data were analysed descriptively for quantitative aspects and through thematic analysis for qualitative input. RESULTS: 104 patients accessed the survey and 89/104 (86%) reported a diagnosis of lung fibrosis, including 46/89 (52%) idiopathic pulmonary fibrosis (IPF) with 57/89 (64%) of participants diagnosed >3 years and 59/89 (66%) female. 52/65(80%) were in the UK; 33/65 (51%) reported severe breathlessness medical research council MRC grade 3-4 and 32/65 (49%) disclosed co-morbid arthritis or joint problems. Of these, 18/83 (22%) used a hand- held spirometer, with only 6/17 (35%) advised on how to interpret the readings. Pulse oximetry devices were the most frequently used device by 35/71 (49%) and 20/64 (31%) measured their saturations more than once daily. 29/63 (46%) of respondents reported home-monitoring brought reassurance; of these, for 25/63 (40%) a feeling of control. 10/57 (18%) felt it had a negative effect, citing fluctuating readings as causing stress and 'paranoia'. The most likely help-seeking triggers were worsening breathlessness 53/65 (82%) and low oxygen saturation 43/65 (66%). Nurse specialists were the most frequent source of help 24/63 (38%). Conclusion: Patients can learn appropriate technical skills, yet perceptions of home-monitoring are variable; targeted assessment and tailored support is likely to be beneficial.

Body reprogramming for fibromyalgia and central sensitivity syndrome: A preliminary evaluation.

Objectives: Central sensitivity syndrome disorders such as fibromyalgia, provoke continued debate, highlighting diagnostic and therapeutic uncertainty. The Hyland model provides a way of understanding and treating the medically unexplained symptoms of central sensitivity syndromes using complexity theory and principles of adaption in network systems. The body reprogramming is a multi-modal intervention based on the Hyland model designed for patients living with medically unexplained symptoms. This preliminary, naturalistic and single-arm service evaluation set out to evaluate outcome after attending a body reprogramming course in patients living with fibromyalgia or central sensitivity syndrome. Methods: Patients diagnosed with fibromyalgia or central sensitivity syndrome were recruited. The body reprogramming courses consisting of eight sessions, each 2.5 h in length, were run at two study sites in England. Data were collected at baseline, post course and 3-months post course using questionnaires assessing symptomatology (FIQR/SIQR), Depression (PHQ9), Anxiety (GAD7) and quality of life (GQoL). Repeated measures t-tests were used, and all comparisons were conducted on an intention to treat basis. Results: In total, 198 patients with a mean age of 47.73 years were enrolled on the body reprogramming courses. Statistically and clinically significant improvement were observed in the FIQR from baseline to post course (mean change: 11.28) and baseline to follow-up (mean change: 15.09). PHQ9 scores also improved significantly from baseline to post course (mean reduction 3.72) and baseline to follow-up (mean reduction 5.59). Conclusions: Our study provides first evidence that the body reprogramming intervention is an effective approach for patients living with fibromyalgia or central sensitivity syndromes on a variety of clinical measures. Besides these promising results, important limitations of the study are discussed, and larger randomized controlled trials are clearly warranted.

Patient perspectives on home-spirometry in interstitial lung disease: a qualitative co-designed study.

BACKGROUND: Opportunities for home-monitoring are increasing exponentially. Home- spirometry is reproducible and reliable in interstitial lung disease (ILD), yet patients' experiences are not reported. Given the morbidity and mortality associated with ILDs, maintaining health-related quality-of-life is vital. We report our findings from a codesigned, qualitative study capturing the perspectives and experiences of patients using home-spirometry in a UK regional ILD National Health Service England (NHSE) commissioned service. METHODS: Patients eligible for home-spirometry as routine clinical care, able to give consent and able to access a smart phone were invited to participate. In-depth, semistructured interviews were conducted at serial time points (baseline, 1, 3 and 6 months), recorded, transcribed and analysed thematically. RESULTS: We report on the experiences of 10 recruited patients (8 males; median age 66 years, range 50-82 years; 7 diagnosed with idiopathic pulmonary fibrosis, 3 other ILDs) who generally found spirometry convenient and easy to use, but their relationships with forced vital capacity results were complex. Main themes emerging were: (1) anticipated benefits-to identify change, trigger action and aid understanding of condition; (2) needs-clinical oversight and feedback, understanding of results, ownership, need for data and a need 'to know'; (3) emotional impact-worry, reassurance, ambivalence/conflicting feelings, reminder of health issues, indifference; (4) ease of home-spirometry-simplicity, convenience and (5) difficulties with home-spirometry-technical issues, technique, physical effort. CONCLUSION: Home-spirometry has many benefits, but in view of the potential risks to psychological well-being, must be considered on an individual basis. Informed consent and decision-making are essential and should be ongoing, acknowledging potential limitations as well as benefits. Healthcare support is vital.

"Life-changing": the experience of super-responders to biologics in severe asthma.

BACKGROUND: There is limited information on the patient's perspective of how biologic treatments impact their lives. We conducted a qualitative study to explore the patient's experience of being considered a super-responder from a quality of life perspective. METHODS: Patients with severe asthma identified as super-responders were invited to semi-structured interviews conducted online. Participants could bring a family member/friend to the interview. The interviews explored experiences of biologic treatment, were transcribed and underwent thematic analysis. RESULTS: Twenty-five participants took part in this study. Themes emerged on the impact of biologic treatment for participants and for their friends/family: (i) Words used to describe their often life-changing experiences and (ii) the positive changes noted. Biologic treatment stopped the disruption of family life and social life caused by exacerbations. Improvements in mental health were also noted. Marked individual variations in the way it affected their lives were noted. Most participants noticed improvements 2-3 months after starting their biologic, but some noticed improvement within a few days and others after 6 months. CONCLUSIONS: Super-responders reported profound but heterogeneous improvements following biologic treatment beyond asthma symptoms and exacerbations including important benefits to social and family life. Improvements may be underestimated as social and family benefits are not reliably measured in current studies with implications for health economic evaluations. Not all patients are super-responders, and excellent responses may be lost in group mean data in trials. Individual time course and response patterns need further elucidation to identify who will respond best to biologics.

World Health Organization (WHO) International Classification of Functioning, Disability and Health (ICF) Core Set Development for Interstitial Lung Disease.

Background: The World Health Organization (WHO) introduced the International Classification of Functioning, Disability, and Health (ICF) as a scientific method of disability data collection comprised of >1,200 categories describing the spectrum of impairment types (functional, symptoms-based and anatomical) under the bio-psycho-social model with consideration of environmental and personal factors (pf). ICF Core Sets and ICF Checklists are streamlined disease-specific resources for clinical use, service provision, and for use in health economics and health policy. ICF can disclose strengths and weaknesses across multiple patient-reported outcome measures (PROMs) and help consolidate best-fitting question-items from multiple PROMs. Interstitial lung diseases (ILDs), are generally progressive, with restrictive physiology sometimes occurring in the context of multi-organ autoimmunity/inflammatory conditions such as connective tissue diseases (CTDs). In spite of significant associated morbidity and potential disability, ILD has yet to be linked to the ICF. Methods: Each instrument and their question-items within the consensus-recommended core sets for clinical trials in ILD were deconstructed to single concept units, and then linked per updated ICF linkage rules. Inter-linker agreement was established. Three additional subsequently validated measures were also included. Results: One-hundred-eleven ICF categories were identified for ten PROMs and three traditional objective measures that were amenable to ICF linkage. The proportion of agreement ranged from 0.79 (95% CI: 0.62, 0.91) to 0.93 (0.76, 0.99) with the overall proportion of inter-linker agreement being very high 0.86 (0.82, 0.89) for the initial instruments, with 94-100% for the three additional PROMs. Thirty-four new 'Personal Factors' emerged to capture disease-specific qualities not elsewhere described in ICF, e.g. 'pf_embarrassed by cough' or 'pf_panic/afraid when can't get a breath'. Conclusion: This first known effort in ICF linkage of ILD has provided important revelations on the current utility of the ICF in lung disease. Results have indicated areas for meaningful assessment of ICF descriptors for lung impairment. The mapping across PROMs provides insight into possibilities of developing more streamline and precise instrumentation. Finally, familiarity with the ICF in ILD may enable clinicians to experience a smoother transition with the imminent harmonization of ICD and ICF, ICD-11.

Preliminary development of a questionnaire to measure the extra-pulmonary symptoms of severe asthma.

BACKGROUND: Research into the effects of asthma treatments on the extra-pulmonary symptoms of severe asthma is limited by the absence of a suitable questionnaire. The aim was to create a questionnaire suitable for intervention studies by selecting symptoms that are statistically associated with asthma pathology and therefore may improve when pathology is reduced. METHODS: Patients attending a specialist asthma clinic completed the 65-item General Symptom Questionnaire (GSQ-65), a questionnaire validated for assessing symptoms of people with multiple medically unexplained symptoms. Lung function (FEV1%) and cumulative oral corticosteroids (OCS) calculated from maintenance dose plus exacerbations were obtained from clinic records. Pathology was represented by the two components of a principal component analysis (PCA) of FEV1% and OCS. LASSO regression was used to select symptoms that had high coefficients with these two principal components and occurred frequently in severe asthma. RESULTS: 100 patients provided data. PCA revealed two components, one where FEV1% and OCS were inversely related and another where they were directly related. LASSO regression revealed 39 symptoms with non-zero coefficients on one or more of the two principal components from which 16 symptoms were selected for the GSQ-A on the basis of magnitude of coefficient and frequency. Asthma symptoms measured by asthma control questionnaires were excluded. The GSQ-A correlated 0.33 and - 0.34 (p = 0.001) with the two principal components. CONCLUSION: The GSQ-A assesses the frequency of 16 heterogenous non-respiratory symptoms that are associated with asthma severity using the statistical combination of FEV1% and OCS.

Comparison of the sensitivity of patient-reported outcomes for detecting the benefit of biologics in severe asthma.

BackgroundThe sensitivity of patient-reported outcomes (PROs) to detect the effects of treatment change depends on the match between the change in items of the PRO and the change that takes place in a sample of people. The aim of this study is to compare the sensitivity of different PROs in detecting changes following the initiation of biologic treatment in asthma. Methods: Patients starting a biologic treatment as part of clinical care completed the Asthma Control Questionnaire (ACQ-6), the Severe Asthma Questionnaire (SAQ and SAQ-global scores) and the EQ5D (EQ-5D-5L and EQ5D-VAS) at baseline. They completed the ACQ-6, SAQ, SAQ-global and a retrospective global rating of change (GRoC) scale at weeks 4, 8 and 16 and completed the EQ-5D-5L and EQ5D-VAS at week 16. The SAQ-global and EQ5D-VAS differ but both are single item 100-point questions. Sensitivity was measured by Cohen's D effect size at each of the three time points. Results: 110 patients were recruited. Depending on the time of assessment, effect size varied between 0.45 and 0.64 for the SAQ, between 0.50 and 0.77 for the SAQ-global; between 0.45 and 0.69 for ACQ-6; between 0.91 and 1.22 for GRoC; 0.32 for EQ-5D-5L and 0.49 for EQ5D-VAS. Conclusion: The sensitivity to change of a questionnaire varies with the time of measurement. The three asthma-specific prospective measures (SAQ, SAQ-global and ACQ-6) have similar sensitivity to change. The single-item EQ5D-VAS was less sensitive than the asthma specific measures and less sensitive than the single-item SAQ-global. The EQ-5D-5L was least sensitive.

Use of Health Related Quality of Life in Clinical Trials for Severe Asthma: A Systematic Review.

BACKGROUND: Asthma Health Related Quality of Life (HRQoL) is an outcome important to patients with severe asthma and can provide clinicians with additional insight into the benefits of treatment. The aim of this systematic review is to examine the use and reporting of HRQoL questionnaires within randomised controlled trials (RCTs) of biologics, fevipiprant and bronchial thermoplasty. METHODS: We followed the guidelines on the Preferred Reporting Items for Systematic reviews and Meta-Analysis (PRISMA) statement. Of the 2380 retrieved articles, 52 studies were identified for inclusion. RESULTS: Sixty-three percent included an asthma HRQoL questionnaire. It was a secondary outcome in the majority of cases (73%). The proportion of studies including an asthma HRQoL questionnaire did not change significantly over a 20-year period. While the Asthma Quality of Life Questionnaire (AQLQ) was used in 45% of studies, 55% used a variety of 4 questionnaires. Most (70%) of the studies that included a HRQoL questionnaire did not report its subscale scores. Approximately half (52%) of studies that used HRQoL reported this in the abstract of the paper. A higher proportion of studies used an asthma control questionnaire compared to a HRQoL questionnaire (71% vs 63%). CONCLUSION: In order to increase the use of asthma HRQoL questionnaires in RCTs of severe asthma treatments, the drivers and barriers to their use must first be understood. At present, the patients' perspective is underrepresented in RCTs of biologics, fevipiprant and bronchial thermoplasty for severe asthma.

Validation of subscales of the Severe Asthma Questionnaire (SAQ) using exploratory factor analysis (EFA).

BACKGROUND: The Severe Asthma Questionnaire (SAQ) is a health related quality of life (HRQoL) questionnaire validated for use in severe asthma. It is scored using the mean value of 16 items (SAQ score) in addition to a single item global rating of HRQoL (SAQ-global). The aim was to validate clinically relevant subscales using exploratory factor analysis (EFA). METHODS: The SAQ was completed, along with measures of asthma control and EQ5D-5L by patients attending six UK severe asthma centres. Clinical data were included in the analysis. EFA using principal axis factoring and oblimin rotation was used to achieve simple structure of data. RESULTS: 460 patients with severe asthma participated, 65% women, mean age 51 (16-83) years. A three factor solution achieved best fit and showed that the SAQ items formed three distinct but inter-correlated groups of items where items were grouped in a way that was consistent with item content. The three subscales were differentially associated with clinically relevant variables (lung function and mood). Males and females interpreted the question of night disturbance in different ways. CONCLUSIONS: This paper provides a template for best practice in the use of EFA when validating HRQoL subscales. The SAQ can be scored as three subscales with content reflecting three different constructs people with severe asthma use when making judgements about their lives. The subscale 'My Life' assesses the impact of severe asthma on different life activities, 'My Mind' assesses the perceived emotional impact and 'My Body' the impact of extra-pulmonary symptoms and side effects.

Comparison of the effects of pulmonary and extra-pulmonary symptoms on health-related quality of life in patients with severe asthma.

OBJECTIVES: To survey the frequency of extra-pulmonary symptoms reported by a sample of patients with severe asthma, their contribution to quality of life and relationship to treatment pathways. METHODS: Consenting patients (N = 100) attending a severe asthma clinic completed questionnaire measures of extra-pulmonary symptoms (the General symptom Questionnaire, GSQ), pulmonary symptoms (Asthma Control Test, ACT), quality of life (the Severe Asthma Questionnaire, SAQ) and health status (EQ-5D-5L). RESULTS: A median of 21 extra-pulmonary symptoms were reported per week. GSQ correlated -0.65 with the ACT and 0.69 with the SAQ. Linear regression showed that both the ACT and GSQ were significant predictors of SAQ mean score, p < 0.001. In patients not receiving biologics, those with high cumulative OCS exposure (≥1120 mg per year) had significantly worse scores (p < 0.05) on all questionnaires except the ACT and GSQ compared to those with low cumulative OCS exposure. DISCUSSION: Extra-pulmonary symptoms were common in this sample of people with severe asthma. Extra-pulmonary and pulmonary symptoms contribute equal variance to the score of HRQoL, showing that they are equally important contributors to patients' experience of severe asthma. Extra-pulmonary symptoms are often overlooked in clinical medicine and in measures of quality of life. Participants receiving biologic treatments had lower extra-pulmonary symptoms possibly indicating that biologics reduce systemic symptoms more effectively than other treatments.

Symptom frequency and development of a generic functional disorder symptom scale suitable for use in studies of patients with irritable bowel syndrome, fibromyalgia syndrome or chronic fatigue syndrome.

OBJECTIVES: To describe the extent to which irritable bowel syndrome (IBS), fibromyalgia syndrome (FMS), and chronic fatigue syndrome (CFS) exhibit symptom overlap, and to validate a patient-derived, generic symptom questionnaire. METHODS: A patient-derived 61-item symptom-frequency questionnaire was completed by participants recruited through IBS, FMS and CFS self-help websites. Principal axis factor analysis with oblimin rotation was performed separately for those reporting an IBS, FMS or CFS diagnosis. RESULTS: Questionnaires were completed by 1751 participants of whom 851 reported more than one of the three diagnoses. Stomach pain on at least a weekly basis was reported by 79% of IBS, 52% of FMS, and 43% of CFS single diagnosis participants. Pain increasing the day after activity was reported by 32% of IBS, 94% of FMS, and 85% of CFS single diagnosis participants. Waking still tired at least once weekly was reported by 75% of IBS, 97% of FMS, and 95% of CFS single diagnosis participants. Exploratory factor analysis produced consistent results across all three diagnostic groups, the 61 items loading on 12 correlated factors with a single higher order factor on which all items loaded. Frequency analysis led to the rejection of one item (cold sores on or near lips), and freeform reporting by participants of additional symptoms identified an additional five, namely, restless legs, hair loss/brittle hair/thinning, dizziness/balance problems, blurred vision and urination problems. CONCLUSIONS: IBS, FMS and CFS are polysymptomatic spectrum disorders with a wide range of overlapping symptoms, many of which are unrelated to diagnostic criteria. Frequency analysis and factor analysis confirm the validity of using the same questionnaire across different diagnostic categories. The 65-item general symptom questionnaire (GSQ-65) is a valid generic symptom scale suitable for assessing the many different symptoms of people with IBS, FMS and CFS.

Evidence for similarity in symptoms and mechanism: The extra-pulmonary symptoms of severe asthma and the polysymptomatic presentation of fibromyalgia.

BACKGROUND: Asthma is a disease of the lung and a systemic disease. Functional disorders are associated with multiple systemic abnormalities that have been explained by complexity models. The aim was to test the similarity in type and aetiology between the extra-pulmonary symptoms of severe asthma and the symptoms of fibromyalgia. METHODS: One Hundred patients recruited from a specialist severe asthma clinic and 1751 people reporting different functional disorder diagnoses recruited via the internet completed the same 60-item questionnaire. Symptom patterns were compared between groups using a new measure, the symptom pattern similarity index where 0 = no relationship, 1 = identical patterns between groups. RESULTS: Severe asthma patients report numerous extra-pulmonary symptoms. The similarity index between the symptom pattern of the asthma patients with other groups was irritable bowel syndrome = 0.54, chronic fatigue syndrome = 0.69, and fibromyalgia = 0.75. The index between fibromyalgia and asthma patients with the most and least frequent extra-pulmonary symptoms was 0.81 and 0.55 respectively. CONCLUSIONS: Patients with severe asthma have numerous extra-pulmonary symptoms similar in type and pattern to the symptoms of fibromyalgia. The similarity of the symptom pattern between asthma and fibromyalgia increases as the number of extra-pulmonary symptoms increases as predicted by network theory and previously shown to be the case with other functional disorders. These findings support the hypothesis that functional disorders and extra-pulmonary asthma symptoms have a common complexity or network aetiology. Evidence based behavioural interventions for fibromyalgia may be helpful for patients with severe asthma reporting extra-pulmonary symptoms.

The construction and validation of the Severe Asthma Questionnaire.

The US Food and Drug Administration's procedure for scale validation requires a documented stepwise process of qualitative and quantitative data. The aim of this paper is to provide final quantitative validating data for the Severe Asthma Questionnaire (SAQ).The SAQ, Asthma Control Test, Mini Asthma Quality of Life Questionnaire and EQ-5D-5L were completed by 160 patients attending a severe asthma clinic; 51 patients completed the SAQ on two occasions for test-retest reliability analysis. The SAQ produces two scores, a SAQ score based on the average of 16 items and a SAQ-global score from a single 100-point global quality of life scale.Construct validity was demonstrated by factor analysis of the 16 items, convergent validity by correlations of >0.6 between the SAQ, SAQ-global and other questionnaires, and discriminant validity by the ability of the SAQ and SAQ-global to distinguish between different treatment levels. Test-retest reliability (intra-class correlation) was 0.93 for the SAQ and 0.93 for the SAQ-global, and the alpha coefficient for the SAQ was 0.93.The SAQ was developed using recommended qualitative and quantitative procedures for scale development, and can be used to gain insight into patients' perceptions of how severe asthma and its treatment affects their lives.

How patient participation was used to develop a questionnaire that is fit for purpose for assessing quality of life in severe asthma.

BACKGROUND: Previous research shows that existing asthma quality of life questionnaires fail to measure the burden of oral corticosteroids that can be used to treat severe asthma, and are therefore not fit for purpose for severe asthma according to the USA's Federal Drug Authority's (FDA) criteria for content validity. Patient input and documentation of that input is key to achieving content validity according to FDA guidelines. This paper describes the process of constructing a new questionnaire to measure the burden of asthma symptoms and burden of treatment in severe asthma, using criteria specified by the FDA. METHODS: A draft severe asthma questionnaire (SAQ) was constructed using qualitative input from severe asthma patients who took part in an earlier study. The aim of this study was to improve that draft questionnaire using a further group of patients. In four iterative focus groups, 16 people with severe asthma completed the draft questionnaire, discussed the wording and structure and suggested changes that were incorporated into the final version. RESULTS: The original intention to ask patients to identify whether problems were caused by asthma symptoms or side effects of medication was abandoned as the attribution of cause was found to be difficult and inconsistent. The recall period of 2 weeks was acceptable but fails to reflect the patients' desire to express the variability of severe asthma. Patients suggested improvements to the wording of the draft questionnaire, including splitting some items in two, combining two items in one, and changes to some of the words in individual items and the response scale. CONCLUSIONS: The final version of the questionnaire was substantially different from one constructed using only qualitative reports from patients about the quality of life deficits of severe asthma. Patients make a valuable contribution to the questionnaire if they are asked to comment and improve an initial draft and where patients are treated as partners in the process of questionnaire construction, rather than only as a source of information to experts who construct the questionnaire.