Publications by category
Journal articles
Saketkoo LA, Frech TM, Gordon JK, Fligelstone K, Mawdsely A, Chaudhry H, Valenzuela A, Christensen A, Khalique SM, Jensen K, et al (In Press). Patient Experience of Systemic Sclerosis related Calcinosis: an International Study Informing Clinical Trials, Practice and the Development of the Mawdsley Calcinosis Questionnaire. Rheumatic Disease Clinics of North America
Saketkoo LA, Patterson KC, Russell A-M (2023). Pause for thought: navigating the complex scientific domains of fatigue and of mindfulness-based practices in sarcoidosis and beyond. The Lancet Respiratory Medicine, 11(3), 219-220.
Grillo L, Russell A-M, Shannon H, Lewis A (2023). The Physiotherapy Assessment of Breathing Pattern Disorder: a Qualitative evaluation.
BMJ Open,
10Abstract:
The Physiotherapy Assessment of Breathing Pattern Disorder: a Qualitative evaluation
What is already known on this topic:
Breathing Pattern Disorder (BPD) is an important condition. It is associated with significant morbidity and can be treated with physiotherapy. Limited evidence exists regarding how best to assess and recognise BPD, which may limit the opportunity for patients to be referred promptly to services to receive the care they need.
What this study adds:
The first qualitative clinician-focused investigation of Breathing Pattern Disorder (BPD) that includes in-depth evaluation of physiotherapists’ opinions of breathing pattern assessment and provides a practical summary of the important components of BPD assessment to be used in clinical practice
How this study might affect research, practice or policy
The study provides a clear description of the need for consistency around terms used and approaches to the assessment of Breathing Pattern Disorder (BPD). The themes identified in this study could help to direct future education, training and guidance for this condition and help underpin the development of future research into BPD.
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Saketkoo LA, Obi ON, Patterson KC, Russell A-M (2022). Ageing with Interstitial lung disease: preserving health and well being.
Curr Opin Pulm Med,
28(4), 321-336.
Abstract:
Ageing with Interstitial lung disease: preserving health and well being.
PURPOSE OF REVIEW: Ageing, the accrual of molecular and cellular damage over a lifetime confers progressive physiologic dysfunction of bodily systems, leaving the body in a heightened state of vulnerability to biophysical and psychosocial stressors. The inflection point is frailty which easily leads to disability and death. Interstitial lung disease (ILD) creates biophysical and psychosocial stresses difficult for even optimally fit patients to cope with. With evolving ILD treatment pathways, people with ILD are living longer. RECENT FINDINGS: ILD and ageing are bi-directionally influential: ILD, its treatments, complications, and collateral systemic extra-pulmonary damage (hypoxic and oxidative stress) wear on the ageing person and ageing impacts a person's tolerance of ILD. ILD extent may proportionally accelerate age-related vulnerabilities. ILD related to inflammatory systemic diseases, e.g. connective tissue diseases or sarcoidosis, exert an even more complex biophysical impact on the body. SUMMARY: the present review stresses goals of preventing frailty in ILD and preserving general health and well being of people living with ILD of any age, from time of diagnosis and as they age. The development of a prediction score is proposed to classify those at risk of frailty and guide interventions that preserve successful ageing for all levels of ILD severity. VIDEO ABSTRACT: http://links.lww.com/COPM/A32.
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Bonella F, Wuyts WA, Vancheri C, Russell AM, Lievens D, Stansen W, Wijsenbeek MS (2022). Effects of nintedanib in patients with idiopathic pulmonary fibrosis and varying severities of cough. Pneumologie, 76, s20-s21.
Antoniou KM, Vasarmidi E, Russell A-M, Andrejak C, Crestani B, Delcroix M, Dinh-Xuan AT, Poletti V, Sverzellati N, Vitacca M, et al (2022). European Respiratory Society statement on long COVID follow-up.
European Respiratory Journal,
60(2), 2102174-2102174.
Abstract:
European Respiratory Society statement on long COVID follow-up
Patients diagnosed with coronavirus disease 2019 (COVID-19) associated with severe acute respiratory syndrome coronavirus 2 (SARS-CoV-2) infection frequently experience symptom burden post-acute infection or post-hospitalisation. We aimed to identify optimal strategies for follow-up care that may positively impact the patient's quality of life (QoL). A European Respiratory Society (ERS) Task Force convened and prioritised eight clinical questions. A targeted search of the literature defined the timeline of “long COVID” as 1–6â
months post-infection and identified clinical evidence in the follow-up of patients. Studies meeting the inclusion criteria report an association of characteristics of acute infection with persistent symptoms, thromboembolic events in the follow-up period, and evaluations of pulmonary physiology and imaging. Importantly, this statement reviews QoL consequences, symptom burden, disability and home care follow-up. Overall, the evidence for follow-up care for patients with long COVID is limited.
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Whear R, Abbott RA, Bethel A, Richards DA, Garside R, Cockcroft E, Iles-Smith H, Logan PA, Rafferty AM, Shepherd M, et al (2022). Impact of COVID-19 and other infectious conditions requiring isolation on the provision of and adaptations to fundamental nursing care in hospital in terms of overall patient experience, care quality, functional ability, and treatment outcomes: systematic review.
Journal of Advanced Nursing,
78(1), 78-108.
Abstract:
Impact of COVID-19 and other infectious conditions requiring isolation on the provision of and adaptations to fundamental nursing care in hospital in terms of overall patient experience, care quality, functional ability, and treatment outcomes: systematic review
Aim: This systematic review identifies, appraises and synthesizes the evidence on the provision of fundamental nursing care to hospitalized patients with a highly infectious virus and the effectiveness of adaptations to overcome barriers to care. Design: Systematic review. Data Sources: in July 2020, we searched Medline, PsycINFO (OvidSP), CINAHL (EBSCOhost), BNI (ProQuest), WHO COVID-19 Database (https://search.bvsalud.org/) MedRxiv (https://www.medrxiv.org/), bioRxiv (https://www.biorxiv.org/) and also Google Scholar, TRIP database and NICE Evidence, forwards citation searching and reference checking of included papers, from 2016 onwards. Review Methods: We included quantitative and qualitative research reporting (i) the views, perceptions and experiences of patients who have received fundamental nursing care whilst in hospital with COVID-19, MERS, SARS, H1N1 or EVD or (ii) the views, perceptions and experiences of professional nurses and non-professionally registered care workers who have provided that care. We included review articles, commentaries, protocols and guidance documents. One reviewer performed data extraction and quality appraisal and was checked by another person. Results: of 3086 references, we included 64 articles; 19 empirical research and 45 review articles, commentaries, protocols and guidance documents spanning five pandemics. Four main themes (and 11 sub-themes) were identified. Barriers to delivering fundamental care were wearing personal protective equipment, adequate staffing, infection control procedures and emotional challenges of care. These barriers were addressed by multiple adaptations to communication, organization of care, staff support and leadership. Conclusion: to prepare for continuation of the COVID-19 pandemic and future pandemics, evaluative studies of adaptations to fundamental healthcare delivery must be prioritized to enable evidence-based care to be provided in future. Impact: Our review identifies the barriers nurses experience in providing fundamental care during a pandemic, highlights potential adaptations that address barriers and ensure positive healthcare experiences and draws attention to the need for evaluative research on fundamental care practices during pandemics.
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Sugg HVR, Richards DA, Russell A, Burnett S, Cockcroft EJ, Thompson Coon J, Cruickshank S, Doris FE, Hunt HA, IlesâSmith H, et al (2022). Nurses’ strategies for overcoming barriers to fundamental nursing care in patients with. <scp>COVID</scp>. â19 caused by infection with the. <scp>SARSâCOV</scp>. â2 virus: Results from the ‘. <scp>COVIDâNURSE</scp>. ’ survey. Journal of Advanced Nursing, 79(3), 1003-1017.
Saketkoo LA, Jensen K, Nikoletou D, Newton JJ, Rivera FJ, Howie M, Reese RK, Goodman M, Hart PB, Bembry W, et al (2022). Sarcoidosis Illuminations on Living During COVID-19: Patient Experiences of Diagnosis, Management, and Survival Before and During the Pandemic.
J Patient Exp,
9Abstract:
Sarcoidosis Illuminations on Living During COVID-19: Patient Experiences of Diagnosis, Management, and Survival Before and During the Pandemic.
Background: Inspired by intense challenges encountered by patients and clinicians, we examined the experiences of living with sarcoidosis in three of the hardest impacted English-speaking cities during the early COVID-19 pandemic: London, New Orleans, and New York. Methods: a multi-disciplinary, multi-national research team including 6 patient leaders conducted qualitative investigations with analyses rooted in grounded theory. Recruitment occurred by self-referral through patient advocacy groups. Results: a total of 28 people living with sarcoidosis participated. The majority of patients had multi-system and severe sarcoidosis. Dominant themes were consistent across groups with differences expressed in spirituality and government and health systems. Racial, gender, and able-bodied inequity were voiced regarding healthcare access and intervention, societal interactions, and COVID-19 exposure and contraction. Agreement regarding extreme disruption in care and communication created concern for disability and survival. Concerns of COVID-19 exposure triggering new sarcoidosis cases or exacerbating established sarcoidosis were expressed. Pre-COVID-19 impediments in sarcoidosis healthcare delivery, medical knowledge, and societal burdens were intensified during the pandemic. Conversely, living with sarcoidosis cultivated personal and operational preparedness for navigating the practicalities and uncertainties of the pandemic. Optimism prevailed that knowledge of sarcoidosis, respiratory, and multi-organ diseases could provide pathways for COVID-19-related therapy and support; however, remorse was expressed regarding pandemic circumstances to draw long-awaited attention to multi-organ system and respiratory conditions. Conclusion: Participants expressed concepts warranting infrastructural and scientific attention. This framework reflects pre- and intra-pandemic voiced needs in sarcoidosis and may be an agent of sensitization and strategy for other serious health conditions. A global query into sarcoidosis will be undertaken.
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Ndili O, Saketkoo LA, Russell A-M, Baughman RP (2022). Sarcoidosis: Updates on therapeutic drug trials and novel treatment approaches. Frontiers in Medicine, 9
Cahalan R, Russell AM, Meade C, Hayes G (2022). SingStrong – singing for better lung health in pulmonary fibrosis: a feasibility study.
Physiotherapy Practice and Research,
43(1), 17-25.
Abstract:
SingStrong â singing for better lung health in pulmonary fibrosis: a feasibility study
BACKGROUND & PURPOSE: Pulmonary fibrosis (PF) is a debilitating, incurable disease. Strategies to optimise health-related quality of life and minimise symptom impact are advocated. Available treatment options such as pulmonary rehabilitation have been severely disrupted due to COVID-19. This feasibility study explored the clinical efficacy and acceptability of an online singing and breathing retraining programme (SingStrong) for people with PF. METHODS: the weekly online programme conducted over 12 weeks was comprised of 45-minute classes of mindfulness, breathing retraining, vocal exercises and singing conducted by a trained vocal coach. People with PF were invited to participate and sessions were recorded for non-attenders. Demographic data were collected, and the St Georges Respiratory Questionnaire (SGRQ) and Idiopathic PF Patient Reported Outcome measure (IPF-PROM) were administered. The questionnaire also invited participants to provide feedback on the utility, enjoyability and main pros/cons of the intervention. Participation in the research element of the programme was not required to attend the weekly classes. RESULTS: of 24 participants recruited, data from 15 (mean (Standard Deviation) age of 66 (8.7); male: nâ=â8) who completed both pre and post-intervention questionnaires were analysed. Statistically significant improvements were recorded in the IPF-PROM (pâ=â0.019) and self-reported quality of life (pâ=â0.028). Class attendance by study participants and the broader PF group cumulatively, increased from 14 to 25 participants between weeks 1 and 12. Qualitatively, strong satisfaction with classes and improved efficacy in self-management of lung health, in particular breathlessness, were reported. CONCLUSIONS: Singing and breathing retraining interventions may endow biopsychosocial benefits for people with PF, in the presence of modest objective clinical gains. Singing programmes are popular and may provide helpful adjuncts to existing clinical strategies such as pulmonary rehabilitation.
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Saketkoo LA, Escorpizo R, Varga J, Keen KJ, Fligelstone K, Birring SS, Alexanderson H, Pettersson H, Chaudhry HA, Poole JL, et al (2022). World Health Organization (WHO) International Classification of Functioning, Disability and Health (ICF) Core Set Development for Interstitial Lung Disease. Frontiers in Pharmacology, 13
Saketkoo LA, Escorpizo R, Varga J, Keen KJ, Fligelstone K, Birring SS, Alexanderson H, Pettersson H, Chaudhry HA, Poole J, et al (2022). World Health Organization (WHO) International Classification of Functioning, Disability and Health (ICF) Core Set Development for Interstitial Lung Disease: a Collaboration with the Global Fellowship on Rehabilitation and Exercise in Systemic Sclerosis (G-FoRSS).
Journal Frontiers in PharmacologyAbstract:
World Health Organization (WHO) International Classification of Functioning, Disability and Health (ICF) Core Set Development for Interstitial Lung Disease: a Collaboration with the Global Fellowship on Rehabilitation and Exercise in Systemic Sclerosis (G-FoRSS)
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
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Saketkoo LA, Frech T, VarjĂș C, Domsic R, Farrell J, Gordon JK, Mihai C, Sandorfi N, Shapiro L, Poole J, et al (2021). A comprehensive framework for navigating patient care in systemic sclerosis: a global response to the need for improving the practice of diagnostic and preventive strategies in SSc. Best Practice & Research Clinical Rheumatology, 35(3), 101707-101707.
Richards DA, Sugg HVR, Cockcroft E, Cooper J, Cruickshank S, Doris F, Hulme C, Logan P, Iles-Smith H, Melendez-Torres GJ, et al (2021). COVID-NURSE: evaluation of a fundamental nursing care protocol compared with care as usual on experience of care for noninvasively ventilated patients in hospital with the SARS-CoV-2 virus—protocol for a cluster randomised controlled trial.
BMJ Open,
11(5), e046436-e046436.
Abstract:
COVID-NURSE: evaluation of a fundamental nursing care protocol compared with care as usual on experience of care for noninvasively ventilated patients in hospital with the SARS-CoV-2 virusâprotocol for a cluster randomised controlled trial
IntroductionPatient experience of nursing care is correlated with safety, clinical effectiveness, care quality, treatment outcomes and service use. Effective nursing care includes actions to develop nurse–patient relationships and deliver physical and psychosocial care to patients. The high risk of transmission of the SARS-CoV-2 virus compromises nursing care. No evidence-based nursing guidelines exist for patients infected with SARS-CoV-2, leading to potential variations in patient experience, outcomes, quality and costs.Methods and analysiswe aim to recruit 840 in-patient participants treated for infection with the SARS-CoV-2 virus from 14 UK hospitals, to a cluster randomised controlled trial, with embedded process and economic evaluations, of care as usual and a fundamental nursing care protocol addressing specific areas of physical, relational and psychosocial nursing care where potential variation may occur, compared with care as usual. Our coprimary outcomes are patient-reported experience (Quality from the Patients’ Perspective; Relational Aspects of Care Questionnaire); secondary outcomes include care quality (pressure injuries, falls, medication errors); functional ability (Barthell Index); treatment outcomes (WHO Clinical Progression Scale); depression Patient Health Questionnaire-2 (PHQ-2), anxiety General Anxiety Disorder-2 (GAD-2), health utility (EQ5D) and nurse-reported outcomes (Measure of Moral Distress for Health Care Professionals). For our primary analysis, we will use a standard generalised linear mixed-effect model adjusting for ethnicity of the patient sample and research intensity at cluster level. We will also undertake a planned subgroup analysis to compare the impact of patient-level ethnicity on our primary and secondary outcomes and will undertake process and economic evaluations.Ethics and disseminationResearch governance and ethical approvals are from the UK National Health Service Health Research Authority Research Ethics Service. Dissemination will be open access through peer-reviewed scientific journals, study website, press and online media, including free online training materials on the Open University’s FutureLearn web platform.Trial registration numberISRCTN13177364; Pre-results.
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Wijsenbeek MS, Bonella F, Orsatti L, Russell A-M, Valenzuela C, Wuyts WA, Baile WF (2021). Communicating with patients with idiopathic pulmonary fibrosis: can we do it better?.
ERJ Open Research,
8(1), 00422-2021.
Abstract:
Communicating with patients with idiopathic pulmonary fibrosis: can we do it better?
Communications between clinicians and patients with idiopathic pulmonary fibrosis (IPF) have the potential to be challenging. The variable course and poor prognosis of IPF complicate discussions around life expectancy but should not prevent clinicians from having meaningful conversations about patients’ fears and needs, while acknowledging uncertainties. Patients want information about the course of their disease and management options, but the provision of information needs to be individualised to the needs and preferences of the patient. Communication from clinicians should be empathetic and take account of the patient's perceptions and concerns. Models, tools and protocols are available that can help clinicians to improve their interactions with patients. In this article, we consider the difficulties inherent in discussions with patients with IPF and their loved ones, and how clinicians might communicate with patients more effectively, from breaking the news about the diagnosis to providing support throughout the course of the disease.
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Pettersson H, Alexanderson H, Poole JL, Varga J, Regardt M, Russell A-M, Salam Y, Jensen K, Mansour J, Frech T, et al (2021). Exercise as a multi-modal disease-modifying medicine in systemic sclerosis: an introduction by the Global Fellowship on Rehabilitation and Exercise in Systemic Sclerosis (G-FoRSS).
Best Pract Res Clin Rheumatol,
35(3).
Abstract:
Exercise as a multi-modal disease-modifying medicine in systemic sclerosis: an introduction by the Global Fellowship on Rehabilitation and Exercise in Systemic Sclerosis (G-FoRSS).
Systemic sclerosis (SSc) is a heterogeneous multisystem autoimmune disease whereby its main pathological drivers of disability and damage are vascular injury, inflammatory cell infiltration, and fibrosis. These mechanisms result in diffuse and diverse impairments arising from ischemic circulatory dysfunction leading to painful skin ulceration and calcinosis, neurovascular aberrations hindering gastrointestinal (GI) motility, progressive painful, incapacitating or immobilizing effects of inflammatory and fibrotic effects on the lungs, skin, articular and periarticular structures, and muscle. SSc-related impairments impede routine activities of daily living (ADLs) and disrupt three critical life areas: work, family, social/leisure, and also impact on psychological well-being. Physical activity and exercise are globally recommended; however, for connective tissue diseases, this guidance carries greater impact on inflammatory disease manifestations, recovery, and cardiovascular health. Exercise, through myogenic and vascular phenomena, naturally targets key pathogenic drivers by downregulating multiple inflammatory and fibrotic pathways in serum and tissue, while increasing circulation and vascular repair. G-FoRSS, the Global Fellowship on Rehabilitation and Exercise in Systemic Sclerosis recognizes the scientific basis of and advocates for education and research of exercise as a systemic and targeted SSc disease-modifying treatment. An overview of biophysiological mechanisms of physical activity and exercise are herein imparted for patients, clinicians, and researchers, and applied to SSc disease mechanisms, manifestations, and impairment. A preliminary guidance on exercise in SSc, a research agenda, and the current state of research and outcome measures are set forth.
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Cassidy N, Fox L, Love M, Byrne I, Doyle AM, Korn B, Shanagher D, Shone T, Cullen M, Cullen T, et al (2021). Fibrotic interstitial lung disease - palliative care needs: a World-Café qualitative study.
BMJ Supportive & Palliative CareAbstract:
Fibrotic interstitial lung disease - palliative care needs: a World-Café qualitative study
ObjectivesThe importance of palliative care in those with advanced fibrotic interstitial lung diseases (F-ILD) is recognised, but the palliative care requirements of patients and caregivers affected by F-ILD regardless of disease course are not established. We set out to explore this and identify optimal solutions in meeting the needs of a F-ILD population in Ireland.MethodsImplementing a World-Café qualitative research approach, we captured insights evolving, iteratively in interactive small group discussions in response to six predefined topics on palliative care and planning for the future. Thirty-nine stakeholders participated in the World-Café including 12 patients, 13 caregivers, 9 healthcare professionals, 4 industry representatives and 1 representative of the clergy.ResultsPalliative care emerged as fundamental to the care and treatment of F-ILDs, regardless of disease progression. Unmet palliative care needs were identified as psychological and social support, disease education, inclusion of caregivers and practical/legal advice for disease progression and end-of-life planning. Participants identified diagnosis as a particularly distressing time for patients and families. They called for the introduction of palliative care discussions at this early-stage alongside improvements in integrated care, specifically increasing the involvement of primary care practitioners in referrals to palliative services.ConclusionPatients and caregivers need discussions on palliative care associated with F-ILD to be included at the point of diagnosis. This approach may address persisting inadequacies in service provision previously identified over the course of the last decade in the UK, Ireland and European F-ILD patient charters.
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Sugg HVR, Russell A-M, Morgan L, Iles-Smith H, Richards DA, Morley N, Burnett S, Cockcroft E, Thompson Coon J, Cruickshank S, et al (2021). Fundamental nursing care in patients with the SARS-CoV-2 virus: results from the ‘COVID-NURSE’ mixed methods survey into nurses’ experiences of missed care and barriers to care. BMC Nursing, 20
Saketkoo LA, Russell A-M, Jensen K, Mandizha J, Tavee J, Newton J, Rivera F, Howie M, Reese R, Goodman M, et al (2021). Health-Related Quality of Life (HRQoL) in Sarcoidosis: Diagnosis, Management, and Health Outcomes.
Diagnostics,
11(6), 1089-1089.
Abstract:
Health-Related Quality of Life (HRQoL) in Sarcoidosis: Diagnosis, Management, and Health Outcomes
Health-related quality of life (HRQoL), though rarely considered as a primary endpoint in clinical trials, may be the single outcome reflective of patient priorities when living with a health condition. HRQoL is a multi-dimensional concept that reflects the degree to which a health condition interferes with participation in and fulfillment of important life areas. HRQoL is intended to capture the composite degree of physical, physiologic, psychological, and social impairment resulting from symptom burden, patient-perceived disease severity, and treatment side effects. Diminished HRQoL expectedly correlates to worsening disability and death; but interventions addressing HRQoL are linked to increased survival. Sarcoidosis, being a multi-organ system disease, is associated with a diffuse array of manifestations resulting in multiple symptoms, complications, and medication-related side effects that are linked to reduced HRQoL. Diminished HRQoL in sarcoidosis is related to decreased physical function, pain, significant loss of income, absence from work, and strain on personal relationships. Symptom distress can result clearly from a sarcoidosis manifestation (e.g. ocular pain, breathlessness, cough) but may also be non-specific, such as pain or fatigue. More complex, a single non-specific symptom, e.g. fatigue may be directly sarcoidosis-derived (e.g. inflammatory state, neurologic, hormonal, cardiopulmonary), medication-related (e.g. anemia, sleeplessness, weight gain, sub-clinical infection), or an indirect complication (e.g. sleep apnea, physical deconditioning, depression). Identifying and distinguishing underlying causes of impaired HRQoL provides opportunity for treatment strategies that can greatly impact a patient’s function, well-being, and disease outcomes. Herein, we present a reference manual that describes the current state of knowledge in sarcoidosis-related HRQoL and distinguish between diverse causes of symptom distress and other influences on sarcoidosis-related HRQoL. We provide tools to assess, investigate, and diagnose compromised HRQoL and its influencers. Strategies to address modifiable HRQoL factors through palliation of symptoms and methods to improve the sarcoidosis health profile are outlined; as well as a proposed research agenda in sarcoidosis-related HRQoL.
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Althobiani MA, Evans RA, Alqahtani JS, Aldhahir AM, Russell A-M, Hurst JR, Porter JC (2021). Home monitoring of physiology and symptoms to detect interstitial lung disease exacerbations and progression: a systematic review.
ERJ Open Research,
7(4), 00441-2021.
Abstract:
Home monitoring of physiology and symptoms to detect interstitial lung disease exacerbations and progression: a systematic review
BackgroundAcute exacerbations (AEs) and disease progression in interstitial lung disease (ILD) pose important challenges to clinicians and patients. AEs of ILD are variable in presentation but may result in rapid progression of ILD, respiratory failure and death. However, in many cases AEs of ILD may go unrecognised so that their true impact and response to therapy is unknown. The potential for home monitoring to facilitate early, and accurate, identification of AE and/or ILD progression has gained interest. With increasing evidence available, there is a need for a systematic review on home monitoring of patients with ILD to summarise the existing data. The aim of this review was to systematically evaluate the evidence for use of home monitoring for early detection of exacerbations and/or progression of ILD.MethodWe searched Ovid-EMBASE, MEDLINE and CINAHL using Medical Subject Headings (MeSH) terms in accordance with the PRISMA guidelines (PROSPERO registration number CRD42020215166).Results13 studies involving 968 patients have demonstrated that home monitoring is feasible and of potential benefit in patients with ILD. Nine studies reported that mean adherence to home monitoring was >75%, and where spirometry was performed there was a significant correlation (r=0.72–0.98, p<0.001) between home and hospital-based readings. Two studies suggested that home monitoring of forced vital capacity might facilitate detection of progression in idiopathic pulmonary fibrosis.ConclusionDespite the fact that individual studies in this systematic review provide supportive evidence suggesting the feasibility and utility of home monitoring in ILD, further studies are necessary to quantify the potential of home monitoring to detect disease progression and/or AEs.
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Noth I, Cottin V, Chaudhuri N, Corte TJ, Johannson KA, Wijsenbeek M, Jouneau S, Michael A, Quaresma M, Rohr KB, et al (2021). Home spirometry in patients with idiopathic pulmonary fibrosis: data from the INMARK trial.
European Respiratory Journal,
58(1), 2001518-2001518.
Abstract:
Home spirometry in patients with idiopathic pulmonary fibrosis: data from the INMARK trial
BackgroundData from the INMARK trial were used to investigate the feasibility and validity of home spirometry as a measure of lung function decline in patients with idiopathic pulmonary fibrosis (IPF).MethodsSubjects with IPF and preserved forced vital capacity (FVC) were randomised to receive nintedanib or placebo for 12â
weeks followed by open-label nintedanib for 40â
weeks. Clinic spirometry was conducted at baseline and weeks 4, 8, 12, 16, 20, 24, 36 and 52. Subjects were asked to perform home spirometry at least once a week and ideally daily. Correlations between home- and clinic-measured FVC and rates of change in FVC were assessed using Pearson correlation coefficients.ResultsIn total, 346 subjects were treated. Mean adherence to weekly home spirometry decreased over time but remained above 75% in every 4-week period. Over 52â
weeks, mean adherence was 86%. Variability in change from baseline in FVC was greater when measured by home rather than clinic spirometry. Strong correlations were observed between home- and clinic-measured FVC at all time-points (r=0.72–0.84), but correlations between home- and clinic-measured rates of change in FVC were weak (r=0.26 for rate of decline in FVC over 52â
weeks).ConclusionHome spirometry was a feasible and valid measure of lung function in patients with IPF and preserved FVC, but estimates of the rate of FVC decline obtained using home spirometry were poorly correlated with those based on clinic spirometry.
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Shuttleworth R, Nancarrow T, Wollteron R, White M, Lines S, Mandizha J, Duckworth A, Russell A, Gibbons M, Scotton C, et al (2021). P144 Red cell distribution width (RDW) and neutrophil lymphocyte ratio (NLR) as prognostic markers in idiopathic pulmonary fibrosis (IPF). Thorax, 76(Suppl 2), a145-a146.
Aronson KI, Danoff SK, Russell A-M, Ryerson CJ, Suzuki A, Wijsenbeek MS, Bajwah S, Bianchi P, Corte TJ, Lee JS, et al (2021). Patient-centered Outcomes Research in Interstitial Lung Disease: an Official American Thoracic Society Research Statement.
Am J Respir Crit Care Med,
204(2), e3-e23.
Abstract:
Patient-centered Outcomes Research in Interstitial Lung Disease: an Official American Thoracic Society Research Statement.
Background: in the past two decades, many advances have been made to our understanding of interstitial lung disease (ILD) and the way we approach its treatment. Despite this, many questions remain unanswered, particularly those related to how the disease and its therapies impact outcomes that are most important to patients. There is currently a lack of guidance on how to best define and incorporate these patient-centered outcomes in ILD research. Objectives: to summarize the current state of patient-centered outcomes research in ILD, identify gaps in knowledge and research, and highlight opportunities and methods for future patient-centered research agendas in ILD. Methods: an international interdisciplinary group of experts was assembled. The group identified top patient-centered outcomes in ILD, reviewed available literature for each outcome, highlighted important discoveries and knowledge gaps, and formulated research recommendations. Results: the committee identified seven themes around patient-centered outcomes as the focus of the statement. After a review of the literature and expert committee discussion, we developed 28 research recommendations. Conclusions: Patient-centered outcomes are key to ascertaining whether and how ILD and interventions used to treat it affect the way patients feel and function in their daily lives. Ample opportunities exist to conduct additional work dedicated to elevating and incorporating patient-centered outcomes in ILD research.
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Althobiani M, Alqahtani JS, Hurst JR, Russell A-M, Porter J (2021). Telehealth for patients with interstitial lung diseases (ILD): results of an international survey of clinicians.
BMJ Open Respiratory Research,
8(1), e001088-e001088.
Abstract:
Telehealth for patients with interstitial lung diseases (ILD): results of an international survey of clinicians
IntroductionClinicians and policymakers are promoting widespread use of home technology including spirometry to detect disease progression for patients with interstitial lung disease (ILD); the COVID-19 pandemic has accelerated this. Data collating clinicians’ views on the potential utility of telehealth in ILD are limited.AimThis survey investigated clinicians’ opinions about contemporary methods and practices used to monitor disease progression in patients with ILD using telehealth.MethodsClinicians were invited to participate in a cross-sectional survey (SurveyMonkey) of 13 questions designed by an expert panel. Telehealth was defined as home monitoring of symptoms and physiological parameters with regular automatic transmission of data from the patient’s home to the clinician. Data are presented as percentages of respondents.ResultsA total of 207 clinicians from 23 countries participated in the survey. A minority (81, 39%) reported using telehealth. 50% (n=41) of these respondents completed a further question about the effectiveness of telehealth. A majority of respondents (32, 70%) rated it to be quite or more effective than face-to-face visit. There were a greater number of respondents using telehealth from Europe (94, 45%) than Asia (51, 25%) and America (24%). Clinicians reported the most useful telehealth monitoring technologies as smartphone apps (59%) and wearable sensors (30%). Telehealth was most frequently used for monitoring disease progression (70%), quality of life (63%), medication use (63%) and reducing the need for in-person visits (63%). Clinicians most often monitored symptoms (93%), oxygen saturation (74%) and physical activity (72%). The equipment perceived to be most effective were spirometers (43%) and pulse oximeters (33%). The primary barriers to clinicians’ participation in telehealth were organisational structure (80%), technical challenges (63%) and lack of time and/or workload (63%). Clinicians considered patients’ barriers to participation might include lack of awareness (76%), lack of knowledge using smartphones (60%) and lack of confidence in telehealth (56%).ConclusionThe ILD clinicians completing this survey who used telehealth to monitor patients (n=81) supported its’ clinical utility. Our findings emphasise the need for robust research in telehealth as a mode for the delivery of cost-effective healthcare services in ILD and highlight the need to assess patients’ perspectives to improve telehealth utility in patients with ILD.
Abstract.
Burki TK (2021). The importance of patient-reported outcomes and measures. The Lancet Respiratory Medicine, 9(11), 1218-1220.
Castro-Sanchez E, Russell A-M, Dolman L, Wells M (2021). What place does nurseâled research have in the COVIDâ19 pandemic?. International Nursing Review, 1-5.
Seligman WH, Fialho L, Sillett N, Nielsen C, Baloch FM, Collis P, Demedts IKM, Fleck MP, Floriani MA, Gabriel LEK, et al (2021). Which outcomes are most important to measure in patients with COVID-19 and how and when should these be measured? Development of an international standard set of outcomes measures for clinical use in patients with COVID-19: a report of the International Consortium for Health Outcomes Measurement (ICHOM) COVID-19 Working Group.
BMJ Open,
11(11), e051065-e051065.
Abstract:
Which outcomes are most important to measure in patients with COVID-19 and how and when should these be measured? Development of an international standard set of outcomes measures for clinical use in patients with COVID-19: a report of the International Consortium for Health Outcomes Measurement (ICHOM) COVID-19 Working Group
ObjectivesThe COVID-19 pandemic has resulted in widespread morbidity and mortality with the consequences expected to be felt for many years. Significant variation exists in the care even of similar patients with COVID-19, including treatment practices within and between institutions. Outcome measures vary among clinical trials on the same therapies. Understanding which therapies are of most value is not possible unless consensus can be reached on which outcomes are most important to measure. Furthermore, consensus on the most important outcomes may enable patients to monitor and track their care, and may help providers to improve the care they offer through quality improvement. To develop a standardised minimum set of outcomes for clinical care, the International Consortium for Health Outcomes Measurement (ICHOM) assembled a working group (WG) of 28 volunteers, including health professionals, patients and patient representatives.DesignA list of outcomes important to patients and professionals was generated from a systematic review of the published literature using the MEDLINE database, from review of outcomes being measured in ongoing clinical trials, from a survey distributed to patients and patient networks, and from previously published ICHOM standard sets in other disease areas. Using an online-modified Delphi process, the WG selected outcomes of greatest importance.ResultsThe outcomes considered by the WG to be most important were selected and categorised into five domains: (1) functional status and quality of life, (2) mental functioning, (3) social functioning, (4) clinical outcomes and (5) symptoms. The WG identified demographic and clinical variables for use as case-mix risk adjusters. These included baseline demographics, clinical factors and treatment-related factors.ConclusionImplementation of these consensus recommendations could help institutions to monitor, compare and improve the quality and delivery of care to patients with COVID-19. Their consistent definition and collection could also broaden the implementation of more patient-centric clinical outcomes research.
Abstract.
Saketkoo LA, Alexanderson H, Lammi MR, LeSage D, Jensen K, Scholand MB, Volkmann ER, Russell AM (2020). An ode to the primal tonic of dance—congratulating the Life of Breath project. The Lancet Respiratory Medicine, 8(12), e90-e91.
Kim JW, Olive S, Jones S, Thillai M, Russell A-M, Johnson MJ, Wilson A (2020). Interstitial lung disease and specialist palliative care access: a healthcare professionals survey.
BMJ Supportive & Palliative Care,
12(e6), e748-e751.
Abstract:
Interstitial lung disease and specialist palliative care access: a healthcare professionals survey
BackgroundFibrotic interstitial lung disease is an incurable disease with poor prognosis. We aimed to understand factors affecting decisions regarding referrals to specialist palliative care services and to address barriers and facilitators to referrals from healthcare professionals’ perspectives.MethodsA survey study of healthcare professionals, including respiratory physicians, interstitial lung disease nurse specialists, respiratory nurse specialists and palliative care physicians, was conducted using a questionnaire, entailing 17 questions.ResultsThirty-six respondents, including 15 interstitial lung disease nurse specialists completed the questionnaire. Symptom control, psychological/spiritual support, general deterioration and end-of-life care were the most common reasons for referrals to specialist palliative care services. Most respondents felt confident in addressing palliative care needs and discussing palliative care with patients. A few participants emphasised that experienced respiratory nurse specialists are well placed to provide symptom management and to ensure continuity of patient care. Participants reported that access to palliative care could be improved by increasing collaborative work between respiratory and palliative care teams.ConclusionsMost respondents felt that enhancing access to specialist palliative care services would benefit patients. However, palliative care and respiratory care should not be considered as mutually exclusive and multidisciplinary approach is recommended.
Abstract.
Russell A (2020). Letter from the. <scp>UK</scp>. Respirology, 25(12), 1325-1327.
Drake TM, Docherty AB, Harrison EM, Quint JK, Adamali H, Agnew S, Babu S, Barber CM, Barratt S, Bendstrup E, et al (2020). Outcome of Hospitalization for COVID-19 in Patients with Interstitial Lung Disease. An International Multicenter Study. American Journal of Respiratory and Critical Care Medicine, 202(12), 1656-1665.
Saketkoo LA, Scholand MB, Lammi MR, Russell A-M (2020). Patient-reported outcome measures in systemic sclerosis–related interstitial lung disease for clinical practice and clinical trials.
Journal of Scleroderma and Related Disorders,
5(2_suppl), 48-60.
Abstract:
Patient-reported outcome measures in systemic sclerosisârelated interstitial lung disease for clinical practice and clinical trials
Systemic sclerosis (SSc) is a progressive vasculopathic, fibrosing autoimmune condition, portending significant mortality; wherein interstitial lung disease (ILD) is the leading cause of death. Although lacking a definitive cure, therapeutics for (SSc-ILD) that stave progression exist with further promising primary and adjuvant compounds in development, as well as interventions to reduce symptom burden and increase quality of life. To date, there has been a significant but varied history related to systemic sclerosis–related interstitial lung disease trial design and endpoint designation. This is especially true of endpoints measuring patient-reported perceptions of efficacy and tolerability. This article describes the underpinnings and complexity of the science, methodology, and current state of patient-reported outcome measures used in (SSc-ILD) systemic sclerosis–related interstitial lung disease in clinical practice and trials.
Abstract.
Edwards C, Costello E, Cassidy N, Vick B, Russell A-M (2020). The utility of an electronic health journal with home spirometry in a real world population of people with fibrotic lung conditions. Journal of Medical Internet Research, 8
Edwards C, Costello E, Cassidy N, Vick B, Russell A-M (2020). Use of the patientMpower App with Home-Based Spirometry to Monitor the Symptoms and Impact of Fibrotic Lung Conditions: Longitudinal Observational Study.
JMIR mHealth and uHealth,
8(11), e16158-e16158.
Abstract:
Use of the patientMpower App with Home-Based Spirometry to Monitor the Symptoms and Impact of Fibrotic Lung Conditions: Longitudinal Observational Study
. Background
. Daily home-based spirometry in idiopathic pulmonary fibrosis (IPF) has been shown to be feasible and clinically informative. The patientMpower app facilitates home-based spirometry along with home-based monitoring of IPF-related symptoms. The patientMpower app can be downloaded to the user’s mobile phone or tablet device, enabling the recording of objective and subjective data.
.
.
. Objective
. The aim of this paper is to report on the 1-year experience of using patientMpower with home-based spirometry by 36 participants with self-reported pulmonary fibrosis (PF) treated with usual care.
.
.
. Methods
. Self-selecting participants enrolled in this community-based participatory research program through a patient advocacy group in their country: Irish Lung Fibrosis Association in Ireland and PF Warriors in the United States. Disease severity was comparable with a baseline mean predicted forced vital capacity (FVC) of 64% and 62% in the Irish and US participants, respectively. Both groups of participants were allocated to identical, in-country, open-label, single-group observational studies and were provided with a Bluetooth-active Spirobank Smart spirometer integrated directly with patientMpower. Data collected via patientMpower included seated FVC (daily), breathlessness grade (modified Medical Research Council scale score), step count, medication adherence, and symptoms and impact of IPF on daily life, which were measured by a patient-reported outcome measure (PROM) scale that was specifically developed for IPF. Longitudinal patient-reported data on oximetry and oxygen consumption were also collected.
.
.
. Results
. A large majority of the 36 participants reported that their experience using patientMpower was positive, and they wanted to continue its use after the initial 6-week observation. Out of 36 participants, 21 (58%) recorded home-based spirometry without prompting for ≥180 days, and 9 (25%) participants continued with recording home-based spirometry for ≥360 days.
.
.
. Conclusions
. The patientMpower app with associated Bluetooth-connected devices (eg, spirometer and pulse oximeter) offers an acceptable and accessible approach to collecting patient-reported objective and subjective data in fibrotic lung conditions.
.
Abstract.
Moor CC, Wijsenbeek MS, Balestro E, Biondini D, Bondue B, Cottin V, Flewett R, Galvin L, Jones S, Molina-Molina M, et al (2019). Gaps in care of patients living with pulmonary fibrosis: a joint patient and expert statement on the results of a Europe-wide survey.
ERJ Open Research,
5(4), 00124-2019.
Abstract:
Gaps in care of patients living with pulmonary fibrosis: a joint patient and expert statement on the results of a Europe-wide survey
IntroductionPulmonary fibrosis (PF) and its most common form, idiopathic pulmonary fibrosis (IPF), are chronic, progressive diseases resulting in increasing loss of lung function and impaired quality of life and survival. The aim of this joint expert and patient statement was to highlight the most pressing common unmet needs of patients with PF/IPF, putting forward recommendations to improve the quality of life and health outcomes throughout the patient journey.MethodsTwo online surveys for patients and healthcare professionals (HCPs) were conducted by the European Idiopathic Pulmonary Fibrosis and Related Disorders Federation (EU-IPFF) in 14 European countries.ResultsThe surveys were answered by 286 patients and 69 HCPs, including physicians and nurses. Delays in diagnosis and timely access to interstitial lung disease specialists and pharmacological treatment have been identified as important gaps in care. Additionally, patients and HCPs reported that a greater focus on symptom-centred management, adequate information, trial information and increasing awareness of PF/IPF is required.ConclusionsThe surveys offer important insights into the current unmet needs of PF/IPF patients. Interventions at different points of the care pathway are needed to improve patient experience.
Abstract.
Lim RK, Humphreys C, Morisset J, Holland AE, Johannson KA (2019). Oxygen in patients with fibrotic interstitial lung disease: an international Delphi survey.
European Respiratory Journal,
54(2), 1900421-1900421.
Abstract:
Oxygen in patients with fibrotic interstitial lung disease: an international Delphi survey
RationalePatients with fibrotic interstitial lung disease (ILD) frequently develop resting or exertional hypoxaemia. There is heterogeneity in clinical practice and a paucity of evidence guiding supplemental oxygen use in this patient population. The objectives of this study were to build international expert-based consensus on the indications and goals of supplemental oxygen from the perspective of healthcare providers, and identify potential barriers to its access.MethodsSemistructured interviews and a comprehensive literature search informed items for the Delphi survey, with items not meeting consensus included in round 2. Round 3 contained survey questions regarding regional funding coverage for oxygen therapy.A prioridefinitions of consensus were median scores of 4 (agree) to 5 (strongly agree) for “agreement”, 1 (strongly disagree) to 2 (disagree) for “disagreement” or 3 (unsure) with an interquartile range of 0–1.Results42 out of 45 (93%) experts completed all three survey rounds, representing 17 countries. 20 out of 36 items met consensus for agreement or disagreement, 10 items met consensus for unsure and four items did not meet consensus. Experts agreed that oxygen should be recommended for patients with severe resting hypoxaemia and in cases of exertional desaturation to <85–89%, particularly with attributable symptoms or exercise limitation. There are regional differences in funding coverage for oxygen, based on desaturation thresholds, clinical symptoms and testing requirements.ConclusionsExperts achieved consensus on 20 items guiding supplemental oxygen use in fibrotic ILD. These findings may inform research, clinical recommendations and funding policy.
Abstract.
Russell AM, Olive S, Lines S, Murphy A, Hocking J, Newell K, Morris H, Harris E, Dixon C, Agnew S, et al (2018). Contemporary challenges for specialist nursing in interstitial lung disease.
Breathe,
14(1), 36-41.
Abstract:
Contemporary challenges for specialist nursing in interstitial lung disease
The role of clinical nurse specialists (CNSs) in interstitial lung disease (ILD) is evolving in response to clinical guidelines and the growth of clinical research. The role is well established in the UK, although more ILD posts are needed to ensure supply meets clinical demand. This phenomenon is also happening across Europe. An appreciation of the similarities and differences between CNS and advanced nurse practitioners is important given the challenges in defining, developing and supporting this nursing specialisation. Globally, different models exist. In some countries charitable organisations take a leading role in supporting patients. Many European centres look to the National Institute for Health and Care Excellence guidelines and quality standards as a template to develop and evaluate the role of the ILD CNS. We present a UK perspective in the context of a government subsidised healthcare system to promote professional discussion and debate regarding the future of nursing practice in the ILD specialty.
Abstract.
Visca D, Mori L, Tsipouri V, Fleming S, Firouzi A, Bonini M, Pavitt MJ, Alfieri V, Canu S, Bonifazi M, et al (2018). Effect of ambulatory oxygen on quality of life for patients with fibrotic lung disease (AmbOx): a prospective, open-label, mixed-method, crossover randomised controlled trial.
Lancet Respir Med,
6(10), 759-770.
Abstract:
Effect of ambulatory oxygen on quality of life for patients with fibrotic lung disease (AmbOx): a prospective, open-label, mixed-method, crossover randomised controlled trial.
BACKGROUND: in fibrotic interstitial lung diseases, exertional breathlessness is strongly linked to health-related quality of life (HRQOL). Breathlessness is often associated with oxygen desaturation, but few data about the use of ambulatory oxygen in patients with fibrotic interstitial lung disease are available. We aimed to assess the effects of ambulatory oxygen on HRQOL in patients with interstitial lung disease with isolated exertional hypoxia. METHODS: AmbOx was a prospective, open-label, mixed-method, crossover randomised controlled clinical trial done at three centres for interstitial lung disease in the UK. Eligible patients were aged 18 years or older, had fibrotic interstitial lung disease, were not hypoxic at rest but had a fall in transcutaneous arterial oxygen saturation to 88% or less on a screening visit 6-min walk test (6MWT), and had self-reported stable respiratory symptoms in the previous 2 weeks. Participants were randomly assigned (1:1) to either oxygen treatment or no oxygen treatment for 2 weeks, followed by crossover for another 2 weeks. Randomisation was by a computer-generated sequence of treatments randomly permuted in blocks of constant size (fixed size of ten). The primary outcome, which was assessed by intention to treat, was the change in total score on the King's Brief Interstitial Lung Disease questionnaire (K-BILD) after 2 weeks on oxygen compared with 2 weeks of no treatment. General linear models with treatment sequence as a fixed effect were used for analysis. Patient views were explored through semi-structured topic-guided interviews in a subgroup of participants. This study was registered with ClinicalTrials.gov, number NCT02286063, and is closed to new participants with all follow-up completed. FINDINGS: Between Sept 10, 2014, and Oct 5, 2016, 84 patients were randomly assigned, 41 randomised to ambulatory oxygen first and 43 to no oxygen. 76 participants completed the trial. Compared with no oxygen, ambulatory oxygen was associated with significant improvements in total K-BILD scores (mean 55·5 [SD 13·8] on oxygen vs 51·8 [13·6] on no oxygen, mean difference adjusted for order of treatment 3·7 [95% CI 1·8 to 5·6]; p
Abstract.
Author URL.
Saketkoo LA, Karpinski A, Young J, Adell R, Walker M, Hennebury T, Wickremasinghe M, Russell A-M (2018). Feasibility, utility and symptom impact of modified mindfulness training in sarcoidosis. ERJ Open Research, 4(2), 00085-2017.
Newell K, Smith K, Russell A-M (2018). Palliative and end of life care in idiopathic pulmonary fibrosis. Primary Health Care, 28(4), 27-35.
Maher TM, Oballa E, Simpson JK, Porte J, Habgood A, Fahy WA, Flynn A, Molyneaux PL, Braybrooke R, Divyateja H, et al (2017). An epithelial biomarker signature for idiopathic pulmonary fibrosis: an analysis from the multicentre PROFILE cohort study.
The Lancet Respiratory Medicine,
5(12), 946-955.
Abstract:
An epithelial biomarker signature for idiopathic pulmonary fibrosis: an analysis from the multicentre PROFILE cohort study
Background Idiopathic pulmonary fibrosis (IPF) is a progressive, fatal disorder with a variable disease trajectory. The aim of this study was to assess potential biomarkers to predict outcomes for people with IPF. Methods PROFILE is a large prospective longitudinal cohort of treatment-naive patients with IPF. We adopted a two-stage discovery and validation design using patients from the PROFILE cohort. For the discovery analysis, we examined 106 patients and 50 age and sex matched healthy controls from Nottingham University Hospitals NHS Trust and the Royal Brompton Hospital. We did an unbiased, multiplex immunoassay assessment of 123 biomarkers. We further investigated promising novel markers by immunohistochemical assessment of IPF lung tissue. In the validation analysis, we examined samples from 206 people with IPF from among the remaining 212 patients recruited to PROFILE Central England. We used the samples to attempt to replicate the biomarkers identified from the discovery analysis by use of independent immunoassays for each biomarker. We investigated the predictive power of the selected biomarkers to identify individuals with IPF who were at risk of progression or death. The PROFILE studies are registered on ClinicalTrials.gov, numbers NCT01134822 (PROFILE Central England) and NCT01110694 (PROFILE Royal Brompton Hospital). Findings in the discovery analysis, we identified four serum biomarkers (surfactant protein D, matrix metalloproteinase 7, CA19-9, and CA-125) that were suitable for replication. Histological assessment of CA19-9 and CA-125 suggested that these proteins were markers of epithelial damage. Replication analysis showed that baseline values of surfactant protein D (46·6 ng/mL vs 34·6 ng/mL, p=0·0018) and CA19-9 (53·7 U/mL vs 22·2 U/mL; p
Abstract.
Spruit MA, Burtin C, Langer D, Makonga J, Russell A-M, York J, Steenbruggen I (2017). Assembly 9: allied respiratory professionals. Breathe, 12(4), e113-e114.
van Manen MJG, Birring SS, Vancheri C, Vindigni V, Renzoni E, Russell A-M, Wapenaar M, Cottin V, Wijsenbeek MS (2017). Effect of pirfenidone on cough in patients with idiopathic pulmonary fibrosis.
EUROPEAN RESPIRATORY JOURNAL,
50(4).
Author URL.
Kreuter M, Bendstrup E, Russell A-M, Bajwah S, Lindell K, Adir Y, Brown CE, Calligaro G, Cassidy N, Corte TJ, et al (2017). Palliative care in interstitial lung disease: living well.
LANCET RESPIRATORY MEDICINE,
5(12), 968-980.
Author URL.
Maher TM, Molina-Molina M, Russell A-M, Bonella F, Jouneau S, Ripamonti E, Axmann J, Vancheri C (2017). Unmet needs in the treatment of idiopathic pulmonary fibrosis-insights from patient chart review in five European countries.
BMC PULMONARY MEDICINE,
17 Author URL.
van Manen MJG, Birring SS, Vancheri C, Cottin V, Renzoni EA, Russell A-M, Wijsenbeek MS (2016). Cough in idiopathic pulmonary fibrosis.
EUROPEAN RESPIRATORY REVIEW,
25(141), 278-286.
Author URL.
Russell A-M, Maher TM (2016). Daily Home Spirometry: a New Milestone in the Field of Pulmonary Fibrosis Reply.
AMERICAN JOURNAL OF RESPIRATORY AND CRITICAL CARE MEDICINE,
194(8), 1034-1035.
Author URL.
Russell A-M, Adamali H, Molyneaux PL, Lukey PT, Marshall RP, Renzoni EA, Wells AU, Maher TM (2016). Daily Home Spirometry: an Effective Tool for Detecting Progression in Idiopathic Pulmonary Fibrosis.
AMERICAN JOURNAL OF RESPIRATORY AND CRITICAL CARE MEDICINE,
194(8), 989-997.
Author URL.
Russell A-M, Ripamonti E, Vancheri C (2016). Qualitative European survey of patients with idiopathic pulmonary fibrosis: patients' perspectives of the disease and treatment.
BMC PULMONARY MEDICINE,
16 Author URL.
Lewis A, Cave P, Stern M, Welch L, Taylor K, Russell J, Doyle A-M, Russell A-M, McKee H, Clift S, et al (2016). Singing for Lung Health-a systematic review of the literature and consensus statement.
NPJ PRIMARY CARE RESPIRATORY MEDICINE,
26 Author URL.
Russell A-M, Swigris JJ (2016). What's it like to live with idiopathic pulmonary fibrosis? Ask the experts.
EUROPEAN RESPIRATORY JOURNAL,
47(5), 1324-1326.
Author URL.
Russell AM (2015). Care planning in idiopathic pulmonary fibrosis.
Nursing Times,
111(18), 20-22.
Abstract:
Care planning in idiopathic pulmonary fibrosis
Patients with idiopathic pulmonary fibrosis require accurate diagnosis and treatment to improve their quality of life and prognosis. This case study follows a patient's care from presentation to diagnosis and management over four years. It illustrates the recommended care pathway, the complexity of the condition and the importance of shared care between specialist and local services. See page 16 for the latest NICE standards for IPF care.
Abstract.
Russell A-M (2015). Idiopathic pulmonary fibrosis: Care Standards. Nursing Times, 111:18, 23-25.
Russell AM (2015). Interstitial pulmonary fibrosis: Care standards.
Nursing Times,
111(18), 16-17.
Abstract:
Interstitial pulmonary fibrosis: Care standards
Idiopathic pulmonary fibrosis (IPF), an interstitial idiopathic pneumonia, is the most common of the interstitial lung diseases. The emergence of new treatments for the condition has increased the need for efficient, high-quality care, preferably in specialist centres. This article reviews the latest NICE standards for IPF care; a case study (page 20) describes the management of a patient with IPF.
Abstract.
Jenkins RG, Simpson JK, Saini G, Bentley JH, Russell A-M, Braybrooke R, Molyneaux PL, McKeever TM, Wells AU, Flynn A, et al (2015). Longitudinal change in collagen degradation biomarkers in idiopathic pulmonary fibrosis: an analysis from the prospective, multicentre PROFILE study.
LANCET RESPIRATORY MEDICINE,
3(6), 462-472.
Author URL.
Lammi M, Baughman R, Birring S, Russell A-M, Ryu J, Scholand M, Distler O, LeSage D, Sarver C, Antoniou K, et al (2015). Outcome Measures for Clinical Trials in Interstitial Lung Diseases. Current Respiratory Medicine Reviews, 11(2), 163-174.
Russell A-M, Sprangers MAG, Wibberley S, Snell N, Rose DM, Swigris JJ (2015). The need for patient-centred clinical research in idiopathic pulmonary fibrosis.
BMC MEDICINE,
13 Author URL.
Russell AM, Maher TM (2014). Detecting anxiety and depression in patients diagnosed with an interstitial lung disease. Can we do better?.
RESPIROLOGY,
19(8), 1095-1096.
Author URL.
Wuyts WA, Peccatori FA, Russell A-M (2014). Patient-centred management in idiopathic pulmonary fibrosis: similar themes in three communication models. European Respiratory Review, 23(132), 231-238.
Molyneaux PL, Cox MJ, Willis-Owen SAG, Mallia P, Russell KE, Russell A-M, Murphy E, Johnston SL, Schwartz DA, Wells AU, et al (2014). The Role of Bacteria in the Pathogenesis and Progression of Idiopathic Pulmonary Fibrosis. American Journal of Respiratory and Critical Care Medicine, 190(8), 906-913.
Stock CJ, Sato H, Fonseca C, Banya WAS, Molyneaux PL, Adamali H, Russell A-M, Denton CP, Abraham DJ, Hansell DM, et al (2013). Mucin 5B promoter polymorphism is associated with idiopathic pulmonary fibrosis but not with development of lung fibrosis in systemic sclerosis or sarcoidosis.
THORAX,
68(5), 436-441.
Author URL.
Adamali HI, Anwar MS, Russell A-M, Egan JJ (2012). Non-pharmacological treatment of idiopathic pulmonary fibrosis. Current Respiratory Care Reports, 1(4), 208-215.
Yorke J, Swigris J, Russell A-M, Moosavi SH, Kwong GNM, Longshaw M, Jones PW (2011). Dyspnea-12 is a Valid and Reliable Measure of Breathlessness in Patients with Interstitial Lung Disease.
CHEST,
139(1), 159-164.
Author URL.
Yorke J, Russell AM (2008). Interpreting the language of breathlessness.
Nursing times,
104(23), 36-39.
Abstract:
Interpreting the language of breathlessness.
Janelle Yorke and Anne-Marie Russell discuss the sensation of breathlessness and the language used by patients and healthcare professionals to describe it.
Abstract.
Chapters
Russell AM, Saketkoo LA (2021). Patient-Centredness and Patient-Reported Measures (PRMs) in Palliation of Lung Disease. In (Ed) Palliative Care in Lung Disease, 43-75.
Russell A-M (2016). Assessing Anxiety and Depression. In Preston W (Ed)
Respiratory Nursing at a Glance, Chichester, Sussex: John Wiley & Sons, 46-47.
Abstract:
Assessing Anxiety and Depression
Abstract.
Russell A-M (2016). Interstitial Lung Disease. In Preston W, Kelly C (Eds.)
Respiratory Nursing at a Glance, Chichester, Sussex: John Wiley & Sons, 68-69.
Abstract:
Interstitial Lung Disease
Abstract.
Russell A-M (2016). Sarcoidosis. In Preston W, Kelly C (Eds.)
Respiratory Nursing at a Glance, Chichester Sussex: John Wiley & Sons, 70-71.
Abstract:
Sarcoidosis
Abstract.
Conferences
Adonis A, Russell A-M, Cassidy E, Asford S, Taylor G (2022). Defining research priorities for people living with HTLV-1 through patient/public involvement and engagement (PPIE) workshops. 2022 International Virtual Conference on Human Retrovirology: HTLV 2022. 8th - 11th May 2022.
Abstract:
Defining research priorities for people living with HTLV-1 through patient/public involvement and engagement (PPIE) workshops
Abstract.
Fabbri L, Wright L, Jones S, Cowan K, Russell A-M, Hart S, Gibbons M, Chaudhuri N, Lynch-Wilson J, Jenkins G, et al (2022). Defining stakeholders’ research priorities in progressive pulmonary fibrosis in the United Kingdom. European Respiratory Society Conference. 4th - 6th Sep 2022.
Abstract:
Defining stakeholdersâ research priorities in progressive pulmonary fibrosis in the United Kingdom
Abstract.
Shuttleworth R, Althobiani M, Duckworth A, Conway J, Dainton J, Mandizha J, Lines S, Jorge Da Ponte A, Scotton C, Gibbons M, et al (2022). Digital device usage for home-monitoring in interstitial lung disease [ILD]:mixed-method survey. European Respiratory Society Congress. 4th - 6th Sep 2022.
Crestani B, Wyuts WA, Vancheri C, Bonella F, Russell A-M, Lievens D, Stansen W, Wijsenbeek M (2022). Effets du nintédanib chez des patients atteints de fibrose pulmonaire idiopathique et présentant des sévérités variables de toux. 26e CONGRÈS DE PNEUMOLOGIE DE LANGUE FRANÇAISE. 21st - 23rd Jan 2022.
Abstract:
Effets du nintédanib chez des patients atteints de fibrose pulmonaire idiopathique et présentant des sévérités variables de toux
Abstract.
Mandizha J, Gibbons M, Lines S, Brown G, Ana Jorge Da Ponte A, Duckworth A, Shuttleworth R, Scotton C, Russell A, Crosby M, et al (2022). Experiences of home-monitoring in patients with Interstitial Lung Disease (ILD): an exploratory, qualitative study. European Respiratory Society Congress. 4th - 6th Sep 2022.
Mandizha J, Duckworth A, Almond H, Brown G, Lines S, Jorge Da Ponte, A, Shuttleworth R, Lanario J, Seward M, Sayers R, et al (2022). Modification of the Rheumatoid Arthritis (RA) Patient Reported Experience Measure (PREM) for patients with Interstitial Lung Disease (ILD). European Respiratory Society Congress 2022. 4th - 6th Sep 2022.
Abstract:
Modification of the Rheumatoid Arthritis (RA) Patient Reported Experience Measure (PREM) for patients with Interstitial Lung Disease (ILD)
Abstract.
Grillo L, Lewis A, Shannon H, Russell A-M (2022). PHYSIOTHERAPISTS’ OPINIONS OF THE PHYSIOTHERAPY
ASSESSMENT OF BREATHING PATTERN DYSFUNCTION: a QUALITATIVE STUDY. British Thoracic Society Winter Metting 2022. 23rd - 25th Nov 2022.
Abstract:
PHYSIOTHERAPISTSâ OPINIONS OF THE PHYSIOTHERAPY
ASSESSMENT OF BREATHING PATTERN DYSFUNCTION: a QUALITATIVE STUDY
Abstract.
Fabbri L, Cowan K, Adams W, Conway J, Jones S, Wright L, Chaudhuri N, Russell A-M, Gibbons M, Hart S, et al (2022). PROGRESSIVE PULMONARY FIBROSIS: TOP TEN
RESEARCH PRIORITIES. British Thoracic Society winter Meeting 2022. 23rd - 25th Nov 2022.
Abstract:
PROGRESSIVE PULMONARY FIBROSIS: TOP TEN
RESEARCH PRIORITIES
Abstract.
Wuyts WA, Vancheri C, Bonella F, Russell A, Lievens D, Stansen W, Wijsenbeek MS (2021). Effects of Nintedanib in Patients with Idiopathic Pulmonary Fibrosis and Varying Severities of Cough.
Author URL.
Althobiani M, Hurst J, Russell A, Porter J (2021). P6â
Telehealth for patients with interstitial lung diseases (ILD): results of an international survey of clinicians. British Thoracic Society Winter Meeting 2021 Online, Wednesday 24 to Friday 26 November 2021, Programme and Abstracts.
Fox L, McLeodl L, Russell A, Edwards C, O'Reilly KMA (2020). An Observational Study of Self-Monitoring of Spirometry and Symptoms via a Mobile Electronic Health Journal in Usual Care of Patients with Idiopathic Pulmonary Fibrosis.
Author URL.
Koschel D, Maher T, Cottin V, Russell A, Corte T, Hammerl P, Michael A, Rohr K, Quaresma M, Stowasser S, et al (2020). Correlation between home and clinic spirometry in subjects with IPF: results from the INMARK trial. 61. Kongress der Deutschen Gesellschaft für Pneumologie und Beatmungsmedizin e.V.
Russell A, Wickremasinghe M, Aul R, Datta A, Saketkoo L, Wells M (2020). Measuring Patient Activation Measurement in Patients Diagnosed with Interstitial Lung Disease (ILD) to Inform Enabling and Compassionate Care.
Author URL.
Russell A-M, Aul R, Datta A, Wickremasinghe M (2020). Measuring patient activation in an interstitial lung disease (ILD) population: an exploratory study. ERS International Congress 2020 abstracts.
Fontanilles E, Vancheri C, Wijsenbeek M, Antoniou K, Rivera P, Bonella F, Renzoni E, Anne-Marie A-M, Vicens-Zygmunt V, Suarez-Cuartin G, et al (2020). Monounsaturated Fatty Acid diet associates less gastrointestinal adverse events of pirfenidone: phase IV clinical trial. ERS International Congress 2020 abstracts.
Fox L, Mcleod L, Russell A-M, Edwards C, O'Reilly K (2020). Single-arm observational study of a mobile app + home spirometry in idiopathic pulmonary fibrosis. ERS International Congress 2020 abstracts.
Russell A, Shone A, Love M, Shanagher D, Byrne I, Fox L, Korn B, Doyle A, Cassidy N (2019). A World Cafe Approach to Palliative Care and Planning for the Future in Fibrotic Lung Disease.
Author URL.
Maher T, Cottin V, Russell A-M, Corte T, Hammerl P, Michael A, Rohr KB, Quaresma M, Stowasser S, Noth I, et al (2019). Correlation between home and clinic spirometry in subjects with IPF: results from the INMARK trial.
Author URL.
Russell A-M, Datta A, Newell K, Jones S, Conway J, Saktkoo LA, Wickremasinghe M (2019). Development of a patient reported experience measure (PREM) for idiopathic pulmonary fibrosis (IPF).
Author URL.
van Manen MJG, Birring SS, Vancheri C, Odink AE, Hussain B, Vindigni V, Renzoni E, Russell A-M, Wapenaar M, Cottin V, et al (2019). Predictors of objective cough in patients with idiopathic pulmonary fibrosis (IPF).
Author URL.
Cassidy N, O'Dowd G, McGowan M, Shone T, Russell A-M (2018). Evaluation of a World Cafe Forum on Palliative Care and Planning for the Future.
Author URL.
Fleming S, Firouzi A, Farquhar M, Tsipouri V, Visca D, Mori L, Canu S, Hogben CR, Kokosi M, Kouranos V, et al (2018). Experiences of Supplemental Ambulatory Oxygen for Patients with Fibrotic Lung Disease: Qualitative Evidence from the Mixed Method AmbOx Trial.
Author URL.
Walsh S, Cahill T, Edwards C, Costello E, Walsh J, Russell A, O'Regan AW (2018). Patient-Reported Monitoring of Symptoms and Spirometry Via the patientMpower Platform in Idiopathic Pulmonary Fibrosis.
Author URL.
Russell A, Wickremasinghe M, Saketkoo L, Borril Z, Fletcher S, Adamali HI, Wells AU, Maher TM, Renzoni EA, Fleming S, et al (2018). Preliminary Testing of the Idiopathic Pulmonary Fibrosis Patient Reported Outcome Measure (IPF PRoM).
Author URL.
Russell AM, Wickremasinghe M, Renzoni E, Adamali H, Borril Z, Fletcher S, Maher TM, Kwong GNM, Saketkoo LA, Fleming S, et al (2018). THE IDIOPATHIC PULMONARY FIBROSIS PATIENTS REPORTED OUTCOME MEASURE (IPF-PROM) IS RELIABLE AND VALID FOR USE IN POPULATIONS WITH IPF.
Author URL.
Edwards C, Cassidy N, Costello E, Russell A-M (2017). A longitudinal approach to supported self-management: sustainability of the idiopathic interstitial pneumonia (IIP) electronic health journal (patientMpower) with integrated home spirometry.
Author URL.
Edwards C, Cassidy N, Costello E, Russell A-M (2017). A longitudinal approach to supported self-management: sustainability of the idiopathic interstitial pneumonia (IIP) electronic health journal (patientMpower) with integrated home spirometry.
Author URL.
Visca D, Mori L, Tsipouri V, Canu S, Bonini M, Pavitt M, Fleming S, Firouzi A, Farquhar M, Leung E, et al (2017). AmbOx trial: does ambulatory oxygen improve quality of life in patients with fibrotic interstitial lung disease?.
Author URL.
Visca D, Mori L, Tsipouri V, Canu S, Bonini M, Pavitt M, Fleming S, Firouzi A, Farquhar M, Leung E, et al (2017). Ambox: a Randomised Controlled, Crossover Trial Evaluating the Effects of Ambulatory Oxygen on Health Status in Patients with Fibrotic Interstitial Lung Disease.
Author URL.
Russell A-M, Jones G, Saketkoo L, Doyle A-M, Sanderson T, D'Accord C, Adamali H, Borrill Z, Fletcher S, Wickremasinghe M, et al (2017). Development and Preliminary Testing of the Idiopathic Pulmonary Fibrosis Patient Reported Outcome Measure (ipf-Prom): Uk and Ireland Multi-Centre Study.
Author URL.
Mori L, Canu S, Visca D, Tsipouri V, Bonini M, Pavitt M, Fleming S, Firouzi A, Farquhar M, Leung E, et al (2017). PREDICTORS OF UPTAKE OF AMBULATORY OXYGEN ON COMPLETION OF THE AMBOX TRIAL, a STUDY TO ASSESS EFFECTS OF AMBULATORY OXYGEN ON QUALITY OF LIFE IN PATIENTS WITH FIBROTIC INTERSTITIAL LUNG DISEASE.
Author URL.
Russell A-M, Cave P, Taylor K, Lewis A, Hopkinson NS, Wells AU (2017). Rhythm and song: Breath management in Idiopathic Interstitial Pneumonias (IIP's). Pilot study.
Author URL.
Russell AM, Sonecha S, Datta A, Hewitt R, Howell I, Elliott A, Wickremasinghe M (2016). DEVELOPMENT OF PATIENT REPORTED EXPERIENCE MEASURE (PREM) FOR IDIOPATHIC PULMONARY FIBROSIS (IPF).
Author URL.
Hogben C, Renzoni E, Wells AU, Kokosi M, Chua F, Maher TM, Russell AM (2016). Development of pre-admission information for referrals with suspected interstitial lung disease to a specialist unit.
Author URL.
Russell A-M, Johnston KN, Yorke J, Williams MT (2016). Differences in Perceptual Experiences of Breathlessness and Impairment Between People with Moderate-Severe COPD in Australia and the United Kingdom.
Author URL.
Russell A-M, Johnston KN, Yorke J, Williams MT (2016). Dyspnoea-12 Scores Reported "these Days" Are Greater Than Those Reported "today": Differences in People with COPD, Asthma and Ild.
Author URL.
Russell A-M, Jones G, Sabella R, Cassidy N, Burge G, Lines S, Stanley L, Fletcher S, Weallans M, Bray M, et al (2016). Health status & impact of living with idiopathic pulmonary fibrosis (IPF): UK & Ireland Delphi survey.
Author URL.
Russell A-M, Scholand M, Snyder EA, Russell A, Burdett C, Doyle A-M, Lasky J, Renzoni E, Wells A, Saketkoo L, et al (2016). Impact, Survival, Symptoms and Management: Us and Uk Patient Perceptions of Living with Idiopathic Pulmonary Fibrosis.
Author URL.
Stock C, Bonini M, Kingston S, Lindahl GE, Russell A-M, Molyneaux PL, Saunders P, Kokosi MA, Sestini P, Wells AU, et al (2016). Muc5b and Tollip Variants: Association with Disease Progression and Survival in an Ipf Cohort.
Author URL.
Saketkoo L, Young J, Adell R, Karpinski AC, Walker M, Russell A-M (2016). Perceptions of a Modified Mindfulness Training (mt) Program in Sarcoidosis.
Author URL.
Visca D, Fleming S, Firouzi A, Farquhar M, Hopkinson N, Hogben C, Banya W, Cullinan P, De Lauretis A, Kokosi M, et al (2016). Randomised controlled, crossover trial to evaluate the effects of ambulatory oxygen on health status in patients with fibrotic lung disease.
Author URL.
Russell A-M, Scholand M, Snyder EA, Doyle A-M, Russell A, Burdett C, Lasky J, Saketkoo LA (2016). Trajectory of Symptom Burden, Impact and Survival in an Idiopathic Pulmonary Fibrosis Population.
Author URL.
Maher TM, Molina-Molina M, Russell A-M, Bonella F, Jouneau S, Ripamonti E, Axmann J, Vancheri C (2016). UNMET NEEDS IN THE TREATMENT OF IDIOPATHIC PULMONARY FIBROSIS (IPF) - INSIGHTS FROM PATIENT CHART REVIEW IN FIVE EUROPEAN COUNTRIES.
Author URL.
Russell A-M, Doyle A-M, Burdett C, Gane J, Aden Z, Fleming S, Cullinan P (2015). A patient centred approach to the development of a patient reported outcome measure (PRoM) in idiopathic pulmonary fibrosis (IPF).
Author URL.
Russell A-M, Doyle A, Fleming S, Burdett C, Ross D, Aden Z, Maher TM, Cullinan P (2015). Development of a Patient Reported Outcome Measure ( prom) in Idiopathic Pulmonary Fibrosis (ipf): Incorporating a Research Partnership with Patients.
Author URL.
Russell A-M, Sanderson T, Wells AU, Fleming S, Maher TM, Cullinan P (2015). Development of a Patient Reported Outcome Measure (prom) in Idiopathic Pulmonary Fibrosis (ipf): an Iterative Process.
Author URL.
Saketkoo LA, Escorpizo R, Keen KJ, Fligelstone K, Birring SS, Lammi MR, Lasky JA, LeSage DR, Renzoni E, Russell AM, et al (2015). INTERNATIONAL CLASSIFICATION OF FUNCTIONING, DISABILITY, AND HEALTH (ICF) CORE SETS FOR CONNECTIVE TISSUE DISEASE INTERSTITIAL LUNG DISEASE (CTD-ILD) AND IDIOPATHIC PULMONARY FIBROSIS (IPF) - a NECESSARY MAP TO HEALTH CARE PROVISION IN THE ERA OF ICD-11.
Author URL.
Russell A-M, Sanderson T, Fleming S, Wells A, Maher T, Cullinan P (2015). Item generation for a patient reported outcome measure (PRoM) in idiopathic pulmonary fibrosis (IPF): Application of consensus methods.
Author URL.
Russell AM, Doyle AM, Ross D, Burdett C, Gane J, Fleming S, Aden Z, Maher TM, Cullinan P (2015). PATIENT AND CARER CO-INVESTIGATORS: SHARED EXPERIENCES OF a RESEARCH STEERING GROUP FROM THE IDIOPATHIC PULMONARY FIBROSIS PATIENT REPORTED OUTCOME MEASURE (IPF-PROM) STUDY.
Author URL.
Cove J, Russell AM, Wright J, Hogben C, Kokosi M, Mak V, Chua F, Wells A, Doyle AM, Renzoni E, et al (2015). PILOT STUDY TO TEST THE FEASIBILITY OF a PSYCHOLOGICAL SUPPORT WORKSHOP FOR PATIENTS NEWLY DIAGNOSED WITH IDIOPATHIC PULMONARY FIBROSIS (IPF) AND THEIR FAMILIES.
Author URL.
Doyle A-M, Burdett C, Gane J, Aden Z, Russell A-M (2015). Psychological distress in UK patients with idiopathic pulmonary fibrosis; use of emotion thermometers interpreted within a biopsychosocial constructionist framework.
Author URL.
Saketkoo L, Escorpizo R, Keen KJ, Fligelstone K, Lammi MR, LeSage D, Russell A-M, Birring SS, Sarver C, Varga J, et al (2015). The World Health Organization (who) International Classification of Functioning, Disability, and Health (icf) Core Sets for Connective Tissue Disease Interstitial Lung Disease (ctd-Ild) and Idiopathic Pulmonary Fibrosis (ipf) - a Necessary Map to Health Care Provision in the Era of Icd-11.
Author URL.
Russell AM, Sanderson T, Fleming S, Wells AU, Maher TMM, Cullinan TP (2014). DEVELOPMENT OF AN IDIOPATHIC PULMONARY FIBROSIS (IPF) PATIENT REPORTED OUTCOME MEASURE (PROM): AN ITERATIVE APPROACH TO ITEM GENERATION.
Author URL.
Escorpizo R, Keen KJ, Fligelstone K, Lammi MR, LeSage D, Russell A-M, Birring S, Sarver C, Varga J, Distler O, et al (2014). International Classification of Functioning, Disability, and Health (ICF) Core Sets for Connective Tissue Disease Interstitial Lung Disease (CTD-ILD) and Idiopathic Pulmonary Fibrosis (IPF) - a Necessary Map to Health Care Provision in the Era of ICD-11.
Author URL.
Russell AM, Vancheri C, Maronati M, Giot C (2013). A QUALITATIVE EUROPEAN SURVEY OF PATIENTS PERCEPTIONS OF CURRENT MANAGEMENT OF IDIOPATHIC PULMONARY FIBROSIS.
Author URL.
Russell A-M, Fraser U, Molyneaux P, Wells A, Renzoni E, Lukey P, Maher T (2013). Quality of life measures in patients with idiopathic pulmonary fibrosis.
Author URL.
Russell A-M, Molyneaux PL, Wells AU, Renzoni E, Luley P, Maher TM (2013). Symptom Measures in Patients with Idiopathic Pulmonary Fibrosis.
Author URL.
Russell A-M, Molyneaux PL, Lukey PT, Fraser UH, Renzoni EA, Wells A, Maher TM (2012). Daily Hand-Held Spirometry for the Monitoring of Patients with Idiopathic Pulmonary Fibrosis. American Thoracic Society 2012 International Conference, May 18-23, 2012 • San Francisco, California.
Russell A-M, Molyneaux PL, Adamali HI, Fraser UH, Lukey PT, Wells A, Maher T (2012). Symptom Related Quality of Life Measures in Patients with Idiopathic Pulmonary Fibrosis. American Thoracic Society 2012 International Conference, May 18-23, 2012 • San Francisco, California.
Adamali HI, Delgado CM, Stock C, Lindhal GE, Molyneaux P, Russell A-M, Wells A, Renzoni EA, Maher T (2012). Telomere (TL) shortening is associated with disease severity in scleroderma (SSC) associated interstitial lung disease (ILD).
Author URL.
Adamali HI, Stock C, Lindahl GE, Leoni-Garcia P, Russell A-M, Molyneaux PL, Wells A, Renzoni E, Maher T (2012). The Effect of Telomere Length in Idiopathic Pulmonary Fibrosis. C109. EFFECTS OF SENESCENCE AND MANIFESTATIONS OF AGING IN THE LUNG.
Saini G, Jenkins G, Mckeever T, Simpson J, Hubbard R, Johnson S, Braybrooke R, Russell A-M, Meakin G, Sweeney L, et al (2012). The PROFILE Study: a Prospective Study of Fibrosis in Lung Endpoints to Discover and Qualify Biomarkers for Use in Clinical Trials. C103. PATHOGENESIS, BIOMARKERS, AND RISK FACTORS FOR INTERSTITIAL LUNG DISEASE: FROM BENCH TO BEDSIDE.
Molyneaux PL, Russell AM, Cox MJ, Moffatt MF, Cookson WO, Maher TM (2012). The Respiratory Microbiome in Idiopathic Pulmonary Fibrosis. C103. PATHOGENESIS, BIOMARKERS, AND RISK FACTORS FOR INTERSTITIAL LUNG DISEASE: FROM BENCH TO BEDSIDE.
Yorke J, Moosavi SH, Russell AM, Jones PW (2010). Psychological Distress is Associated with Greater Breathlessness Severity. C55. ADVANCES IN DYSPNEA MEASUREMENT.
Yorke J, Moosavi SH, Russell AM, Jones PW (2009). Does Slight Variation in Situational Wording on Breathlessness Questionnaires Make a Difference?.
Author URL.
Yorke J, Moosavi SH, Russell AM, Jones PW (2009). Validity and Reliability of the Dyspnoea-12.
Author URL.
Publications by year
In Press
Saketkoo LA, Frech TM, Gordon JK, Fligelstone K, Mawdsely A, Chaudhry H, Valenzuela A, Christensen A, Khalique SM, Jensen K, et al (In Press). Patient Experience of Systemic Sclerosis related Calcinosis: an International Study Informing Clinical Trials, Practice and the Development of the Mawdsley Calcinosis Questionnaire. Rheumatic Disease Clinics of North America
Duckworth A, Ruth KS, Prague JK, Russell A-M, Almond H, Conway J, Beaumont RN, Wood AR, Martin S, Lunnon K, et al (In Press). Study of the associations between short telomeres, sex hormones and pulmonary fibrosis.
Abstract:
Study of the associations between short telomeres, sex hormones and pulmonary fibrosis
AbstractBackgroundPulmonary fibrosis (PF) is an incurable fibrotic lung disease with limited treatment options and a high mortality. Evidence is growing that short telomeres cause both heritable and idiopathic pulmonary fibrosis (IPF). Based on survival data, we hypothesised that sex hormones are protective against premature telomere attrition and could influence PF disease onset and/or progression.MethodsAssociations between IPF, sex hormone concentrations and measured leukocyte telomere length (LTL) were examined for unrelated UK Biobank participants of European ancestry with a diagnosis of IPF (415 females, 718 males) against controls (204,321 females, 174,254 males). Polygenic risk scores were used to explore causality between sex hormone indices, LTL and disease.FindingsStrong associations were found between IPF and LTL. For females, higher odds of having IPF was associated with early menopause and premature ovarian failure. Menopause age correlated positively with both age of IPF diagnosis and age of death. For males, IPF prevalence and stages of disease were associated with serum bioavailable testosterone concentrations. For both sexes, evidence of lower concentrations of sex hormones was associated with shorter LTL. Genetic analysis also inferred bi-directional causal links between sex hormone binding globulin concentration, which impacts free testosterone concentration, and LTL in males.InterpretationOur findings suggest that higher sex hormone concentrations protect against IPF onset and progression, possibly by slowing telomere shortening. Hormonal supplementation may delay or prevent disease onset for those with telomere-associated PF risk and improve disease prognosis. This warrants further exploration in a randomised controlled trial.FundingMedical Research Council.
Abstract.
2023
Saketkoo LA, Patterson KC, Russell A-M (2023). Pause for thought: navigating the complex scientific domains of fatigue and of mindfulness-based practices in sarcoidosis and beyond. The Lancet Respiratory Medicine, 11(3), 219-220.
Grillo L, Russell A-M, Shannon H, Lewis A (2023). The Physiotherapy Assessment of Breathing Pattern Disorder: a Qualitative evaluation.
BMJ Open,
10Abstract:
The Physiotherapy Assessment of Breathing Pattern Disorder: a Qualitative evaluation
What is already known on this topic:
Breathing Pattern Disorder (BPD) is an important condition. It is associated with significant morbidity and can be treated with physiotherapy. Limited evidence exists regarding how best to assess and recognise BPD, which may limit the opportunity for patients to be referred promptly to services to receive the care they need.
What this study adds:
The first qualitative clinician-focused investigation of Breathing Pattern Disorder (BPD) that includes in-depth evaluation of physiotherapists’ opinions of breathing pattern assessment and provides a practical summary of the important components of BPD assessment to be used in clinical practice
How this study might affect research, practice or policy
The study provides a clear description of the need for consistency around terms used and approaches to the assessment of Breathing Pattern Disorder (BPD). The themes identified in this study could help to direct future education, training and guidance for this condition and help underpin the development of future research into BPD.
Abstract.
2022
Saketkoo LA, Obi ON, Patterson KC, Russell A-M (2022). Ageing with Interstitial lung disease: preserving health and well being.
Curr Opin Pulm Med,
28(4), 321-336.
Abstract:
Ageing with Interstitial lung disease: preserving health and well being.
PURPOSE OF REVIEW: Ageing, the accrual of molecular and cellular damage over a lifetime confers progressive physiologic dysfunction of bodily systems, leaving the body in a heightened state of vulnerability to biophysical and psychosocial stressors. The inflection point is frailty which easily leads to disability and death. Interstitial lung disease (ILD) creates biophysical and psychosocial stresses difficult for even optimally fit patients to cope with. With evolving ILD treatment pathways, people with ILD are living longer. RECENT FINDINGS: ILD and ageing are bi-directionally influential: ILD, its treatments, complications, and collateral systemic extra-pulmonary damage (hypoxic and oxidative stress) wear on the ageing person and ageing impacts a person's tolerance of ILD. ILD extent may proportionally accelerate age-related vulnerabilities. ILD related to inflammatory systemic diseases, e.g. connective tissue diseases or sarcoidosis, exert an even more complex biophysical impact on the body. SUMMARY: the present review stresses goals of preventing frailty in ILD and preserving general health and well being of people living with ILD of any age, from time of diagnosis and as they age. The development of a prediction score is proposed to classify those at risk of frailty and guide interventions that preserve successful ageing for all levels of ILD severity. VIDEO ABSTRACT: http://links.lww.com/COPM/A32.
Abstract.
Author URL.
Adonis A, Russell A-M, Cassidy E, Asford S, Taylor G (2022). Defining research priorities for people living with HTLV-1 through patient/public involvement and engagement (PPIE) workshops. 2022 International Virtual Conference on Human Retrovirology: HTLV 2022. 8th - 11th May 2022.
Abstract:
Defining research priorities for people living with HTLV-1 through patient/public involvement and engagement (PPIE) workshops
Abstract.
Fabbri L, Wright L, Jones S, Cowan K, Russell A-M, Hart S, Gibbons M, Chaudhuri N, Lynch-Wilson J, Jenkins G, et al (2022). Defining stakeholders’ research priorities in progressive pulmonary fibrosis in the United Kingdom. European Respiratory Society Conference. 4th - 6th Sep 2022.
Abstract:
Defining stakeholdersâ research priorities in progressive pulmonary fibrosis in the United Kingdom
Abstract.
Shuttleworth R, Althobiani M, Duckworth A, Conway J, Dainton J, Mandizha J, Lines S, Jorge Da Ponte A, Scotton C, Gibbons M, et al (2022). Digital device usage for home-monitoring in interstitial lung disease [ILD]:mixed-method survey. European Respiratory Society Congress. 4th - 6th Sep 2022.
Bonella F, Wuyts WA, Vancheri C, Russell AM, Lievens D, Stansen W, Wijsenbeek MS (2022). Effects of nintedanib in patients with idiopathic pulmonary fibrosis and varying severities of cough. Pneumologie, 76, s20-s21.
Crestani B, Wyuts WA, Vancheri C, Bonella F, Russell A-M, Lievens D, Stansen W, Wijsenbeek M (2022). Effets du nintédanib chez des patients atteints de fibrose pulmonaire idiopathique et présentant des sévérités variables de toux. 26e CONGRÈS DE PNEUMOLOGIE DE LANGUE FRANÇAISE. 21st - 23rd Jan 2022.
Abstract:
Effets du nintédanib chez des patients atteints de fibrose pulmonaire idiopathique et présentant des sévérités variables de toux
Abstract.
Antoniou KM, Vasarmidi E, Russell A-M, Andrejak C, Crestani B, Delcroix M, Dinh-Xuan AT, Poletti V, Sverzellati N, Vitacca M, et al (2022). European Respiratory Society statement on long COVID follow-up.
European Respiratory Journal,
60(2), 2102174-2102174.
Abstract:
European Respiratory Society statement on long COVID follow-up
Patients diagnosed with coronavirus disease 2019 (COVID-19) associated with severe acute respiratory syndrome coronavirus 2 (SARS-CoV-2) infection frequently experience symptom burden post-acute infection or post-hospitalisation. We aimed to identify optimal strategies for follow-up care that may positively impact the patient's quality of life (QoL). A European Respiratory Society (ERS) Task Force convened and prioritised eight clinical questions. A targeted search of the literature defined the timeline of “long COVID” as 1–6â
months post-infection and identified clinical evidence in the follow-up of patients. Studies meeting the inclusion criteria report an association of characteristics of acute infection with persistent symptoms, thromboembolic events in the follow-up period, and evaluations of pulmonary physiology and imaging. Importantly, this statement reviews QoL consequences, symptom burden, disability and home care follow-up. Overall, the evidence for follow-up care for patients with long COVID is limited.
Abstract.
Mandizha J, Gibbons M, Lines S, Brown G, Ana Jorge Da Ponte A, Duckworth A, Shuttleworth R, Scotton C, Russell A, Crosby M, et al (2022). Experiences of home-monitoring in patients with Interstitial Lung Disease (ILD): an exploratory, qualitative study. European Respiratory Society Congress. 4th - 6th Sep 2022.
Whear R, Abbott RA, Bethel A, Richards DA, Garside R, Cockcroft E, Iles-Smith H, Logan PA, Rafferty AM, Shepherd M, et al (2022). Impact of COVID-19 and other infectious conditions requiring isolation on the provision of and adaptations to fundamental nursing care in hospital in terms of overall patient experience, care quality, functional ability, and treatment outcomes: systematic review.
Journal of Advanced Nursing,
78(1), 78-108.
Abstract:
Impact of COVID-19 and other infectious conditions requiring isolation on the provision of and adaptations to fundamental nursing care in hospital in terms of overall patient experience, care quality, functional ability, and treatment outcomes: systematic review
Aim: This systematic review identifies, appraises and synthesizes the evidence on the provision of fundamental nursing care to hospitalized patients with a highly infectious virus and the effectiveness of adaptations to overcome barriers to care. Design: Systematic review. Data Sources: in July 2020, we searched Medline, PsycINFO (OvidSP), CINAHL (EBSCOhost), BNI (ProQuest), WHO COVID-19 Database (https://search.bvsalud.org/) MedRxiv (https://www.medrxiv.org/), bioRxiv (https://www.biorxiv.org/) and also Google Scholar, TRIP database and NICE Evidence, forwards citation searching and reference checking of included papers, from 2016 onwards. Review Methods: We included quantitative and qualitative research reporting (i) the views, perceptions and experiences of patients who have received fundamental nursing care whilst in hospital with COVID-19, MERS, SARS, H1N1 or EVD or (ii) the views, perceptions and experiences of professional nurses and non-professionally registered care workers who have provided that care. We included review articles, commentaries, protocols and guidance documents. One reviewer performed data extraction and quality appraisal and was checked by another person. Results: of 3086 references, we included 64 articles; 19 empirical research and 45 review articles, commentaries, protocols and guidance documents spanning five pandemics. Four main themes (and 11 sub-themes) were identified. Barriers to delivering fundamental care were wearing personal protective equipment, adequate staffing, infection control procedures and emotional challenges of care. These barriers were addressed by multiple adaptations to communication, organization of care, staff support and leadership. Conclusion: to prepare for continuation of the COVID-19 pandemic and future pandemics, evaluative studies of adaptations to fundamental healthcare delivery must be prioritized to enable evidence-based care to be provided in future. Impact: Our review identifies the barriers nurses experience in providing fundamental care during a pandemic, highlights potential adaptations that address barriers and ensure positive healthcare experiences and draws attention to the need for evaluative research on fundamental care practices during pandemics.
Abstract.
Mandizha J, Duckworth A, Almond H, Brown G, Lines S, Jorge Da Ponte, A, Shuttleworth R, Lanario J, Seward M, Sayers R, et al (2022). Modification of the Rheumatoid Arthritis (RA) Patient Reported Experience Measure (PREM) for patients with Interstitial Lung Disease (ILD). European Respiratory Society Congress 2022. 4th - 6th Sep 2022.
Abstract:
Modification of the Rheumatoid Arthritis (RA) Patient Reported Experience Measure (PREM) for patients with Interstitial Lung Disease (ILD)
Abstract.
Sugg HVR, Richards DA, Russell A, Burnett S, Cockcroft EJ, Thompson Coon J, Cruickshank S, Doris FE, Hunt HA, IlesâSmith H, et al (2022). Nurses’ strategies for overcoming barriers to fundamental nursing care in patients with. <scp>COVID</scp>. â19 caused by infection with the. <scp>SARSâCOV</scp>. â2 virus: Results from the ‘. <scp>COVIDâNURSE</scp>. ’ survey. Journal of Advanced Nursing, 79(3), 1003-1017.
Grillo L, Lewis A, Shannon H, Russell A-M (2022). PHYSIOTHERAPISTS’ OPINIONS OF THE PHYSIOTHERAPY
ASSESSMENT OF BREATHING PATTERN DYSFUNCTION: a QUALITATIVE STUDY. British Thoracic Society Winter Metting 2022. 23rd - 25th Nov 2022.
Abstract:
PHYSIOTHERAPISTSâ OPINIONS OF THE PHYSIOTHERAPY
ASSESSMENT OF BREATHING PATTERN DYSFUNCTION: a QUALITATIVE STUDY
Abstract.
Fabbri L, Cowan K, Adams W, Conway J, Jones S, Wright L, Chaudhuri N, Russell A-M, Gibbons M, Hart S, et al (2022). PROGRESSIVE PULMONARY FIBROSIS: TOP TEN
RESEARCH PRIORITIES. British Thoracic Society winter Meeting 2022. 23rd - 25th Nov 2022.
Abstract:
PROGRESSIVE PULMONARY FIBROSIS: TOP TEN
RESEARCH PRIORITIES
Abstract.
Saketkoo LA, Jensen K, Nikoletou D, Newton JJ, Rivera FJ, Howie M, Reese RK, Goodman M, Hart PB, Bembry W, et al (2022). Sarcoidosis Illuminations on Living During COVID-19: Patient Experiences of Diagnosis, Management, and Survival Before and During the Pandemic.
J Patient Exp,
9Abstract:
Sarcoidosis Illuminations on Living During COVID-19: Patient Experiences of Diagnosis, Management, and Survival Before and During the Pandemic.
Background: Inspired by intense challenges encountered by patients and clinicians, we examined the experiences of living with sarcoidosis in three of the hardest impacted English-speaking cities during the early COVID-19 pandemic: London, New Orleans, and New York. Methods: a multi-disciplinary, multi-national research team including 6 patient leaders conducted qualitative investigations with analyses rooted in grounded theory. Recruitment occurred by self-referral through patient advocacy groups. Results: a total of 28 people living with sarcoidosis participated. The majority of patients had multi-system and severe sarcoidosis. Dominant themes were consistent across groups with differences expressed in spirituality and government and health systems. Racial, gender, and able-bodied inequity were voiced regarding healthcare access and intervention, societal interactions, and COVID-19 exposure and contraction. Agreement regarding extreme disruption in care and communication created concern for disability and survival. Concerns of COVID-19 exposure triggering new sarcoidosis cases or exacerbating established sarcoidosis were expressed. Pre-COVID-19 impediments in sarcoidosis healthcare delivery, medical knowledge, and societal burdens were intensified during the pandemic. Conversely, living with sarcoidosis cultivated personal and operational preparedness for navigating the practicalities and uncertainties of the pandemic. Optimism prevailed that knowledge of sarcoidosis, respiratory, and multi-organ diseases could provide pathways for COVID-19-related therapy and support; however, remorse was expressed regarding pandemic circumstances to draw long-awaited attention to multi-organ system and respiratory conditions. Conclusion: Participants expressed concepts warranting infrastructural and scientific attention. This framework reflects pre- and intra-pandemic voiced needs in sarcoidosis and may be an agent of sensitization and strategy for other serious health conditions. A global query into sarcoidosis will be undertaken.
Abstract.
Author URL.
Ndili O, Saketkoo LA, Russell A-M, Baughman RP (2022). Sarcoidosis: Updates on therapeutic drug trials and novel treatment approaches. Frontiers in Medicine, 9
Cahalan R, Russell AM, Meade C, Hayes G (2022). SingStrong – singing for better lung health in pulmonary fibrosis: a feasibility study.
Physiotherapy Practice and Research,
43(1), 17-25.
Abstract:
SingStrong â singing for better lung health in pulmonary fibrosis: a feasibility study
BACKGROUND & PURPOSE: Pulmonary fibrosis (PF) is a debilitating, incurable disease. Strategies to optimise health-related quality of life and minimise symptom impact are advocated. Available treatment options such as pulmonary rehabilitation have been severely disrupted due to COVID-19. This feasibility study explored the clinical efficacy and acceptability of an online singing and breathing retraining programme (SingStrong) for people with PF. METHODS: the weekly online programme conducted over 12 weeks was comprised of 45-minute classes of mindfulness, breathing retraining, vocal exercises and singing conducted by a trained vocal coach. People with PF were invited to participate and sessions were recorded for non-attenders. Demographic data were collected, and the St Georges Respiratory Questionnaire (SGRQ) and Idiopathic PF Patient Reported Outcome measure (IPF-PROM) were administered. The questionnaire also invited participants to provide feedback on the utility, enjoyability and main pros/cons of the intervention. Participation in the research element of the programme was not required to attend the weekly classes. RESULTS: of 24 participants recruited, data from 15 (mean (Standard Deviation) age of 66 (8.7); male: nâ=â8) who completed both pre and post-intervention questionnaires were analysed. Statistically significant improvements were recorded in the IPF-PROM (pâ=â0.019) and self-reported quality of life (pâ=â0.028). Class attendance by study participants and the broader PF group cumulatively, increased from 14 to 25 participants between weeks 1 and 12. Qualitatively, strong satisfaction with classes and improved efficacy in self-management of lung health, in particular breathlessness, were reported. CONCLUSIONS: Singing and breathing retraining interventions may endow biopsychosocial benefits for people with PF, in the presence of modest objective clinical gains. Singing programmes are popular and may provide helpful adjuncts to existing clinical strategies such as pulmonary rehabilitation.
Abstract.
Saketkoo LA, Escorpizo R, Varga J, Keen KJ, Fligelstone K, Birring SS, Alexanderson H, Pettersson H, Chaudhry HA, Poole JL, et al (2022). World Health Organization (WHO) International Classification of Functioning, Disability and Health (ICF) Core Set Development for Interstitial Lung Disease. Frontiers in Pharmacology, 13
Saketkoo LA, Escorpizo R, Varga J, Keen KJ, Fligelstone K, Birring SS, Alexanderson H, Pettersson H, Chaudhry HA, Poole J, et al (2022). World Health Organization (WHO) International Classification of Functioning, Disability and Health (ICF) Core Set Development for Interstitial Lung Disease: a Collaboration with the Global Fellowship on Rehabilitation and Exercise in Systemic Sclerosis (G-FoRSS).
Journal Frontiers in PharmacologyAbstract:
World Health Organization (WHO) International Classification of Functioning, Disability and Health (ICF) Core Set Development for Interstitial Lung Disease: a Collaboration with the Global Fellowship on Rehabilitation and Exercise in Systemic Sclerosis (G-FoRSS)
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
Abstract.
2021
Saketkoo LA, Frech T, VarjĂș C, Domsic R, Farrell J, Gordon JK, Mihai C, Sandorfi N, Shapiro L, Poole J, et al (2021). A comprehensive framework for navigating patient care in systemic sclerosis: a global response to the need for improving the practice of diagnostic and preventive strategies in SSc. Best Practice & Research Clinical Rheumatology, 35(3), 101707-101707.
Richards DA, Sugg HVR, Cockcroft E, Cooper J, Cruickshank S, Doris F, Hulme C, Logan P, Iles-Smith H, Melendez-Torres GJ, et al (2021). COVID-NURSE: evaluation of a fundamental nursing care protocol compared with care as usual on experience of care for noninvasively ventilated patients in hospital with the SARS-CoV-2 virus—protocol for a cluster randomised controlled trial.
BMJ Open,
11(5), e046436-e046436.
Abstract:
COVID-NURSE: evaluation of a fundamental nursing care protocol compared with care as usual on experience of care for noninvasively ventilated patients in hospital with the SARS-CoV-2 virusâprotocol for a cluster randomised controlled trial
IntroductionPatient experience of nursing care is correlated with safety, clinical effectiveness, care quality, treatment outcomes and service use. Effective nursing care includes actions to develop nurse–patient relationships and deliver physical and psychosocial care to patients. The high risk of transmission of the SARS-CoV-2 virus compromises nursing care. No evidence-based nursing guidelines exist for patients infected with SARS-CoV-2, leading to potential variations in patient experience, outcomes, quality and costs.Methods and analysiswe aim to recruit 840 in-patient participants treated for infection with the SARS-CoV-2 virus from 14 UK hospitals, to a cluster randomised controlled trial, with embedded process and economic evaluations, of care as usual and a fundamental nursing care protocol addressing specific areas of physical, relational and psychosocial nursing care where potential variation may occur, compared with care as usual. Our coprimary outcomes are patient-reported experience (Quality from the Patients’ Perspective; Relational Aspects of Care Questionnaire); secondary outcomes include care quality (pressure injuries, falls, medication errors); functional ability (Barthell Index); treatment outcomes (WHO Clinical Progression Scale); depression Patient Health Questionnaire-2 (PHQ-2), anxiety General Anxiety Disorder-2 (GAD-2), health utility (EQ5D) and nurse-reported outcomes (Measure of Moral Distress for Health Care Professionals). For our primary analysis, we will use a standard generalised linear mixed-effect model adjusting for ethnicity of the patient sample and research intensity at cluster level. We will also undertake a planned subgroup analysis to compare the impact of patient-level ethnicity on our primary and secondary outcomes and will undertake process and economic evaluations.Ethics and disseminationResearch governance and ethical approvals are from the UK National Health Service Health Research Authority Research Ethics Service. Dissemination will be open access through peer-reviewed scientific journals, study website, press and online media, including free online training materials on the Open University’s FutureLearn web platform.Trial registration numberISRCTN13177364; Pre-results.
Abstract.
Wijsenbeek MS, Bonella F, Orsatti L, Russell A-M, Valenzuela C, Wuyts WA, Baile WF (2021). Communicating with patients with idiopathic pulmonary fibrosis: can we do it better?.
ERJ Open Research,
8(1), 00422-2021.
Abstract:
Communicating with patients with idiopathic pulmonary fibrosis: can we do it better?
Communications between clinicians and patients with idiopathic pulmonary fibrosis (IPF) have the potential to be challenging. The variable course and poor prognosis of IPF complicate discussions around life expectancy but should not prevent clinicians from having meaningful conversations about patients’ fears and needs, while acknowledging uncertainties. Patients want information about the course of their disease and management options, but the provision of information needs to be individualised to the needs and preferences of the patient. Communication from clinicians should be empathetic and take account of the patient's perceptions and concerns. Models, tools and protocols are available that can help clinicians to improve their interactions with patients. In this article, we consider the difficulties inherent in discussions with patients with IPF and their loved ones, and how clinicians might communicate with patients more effectively, from breaking the news about the diagnosis to providing support throughout the course of the disease.
Abstract.
Wuyts WA, Vancheri C, Bonella F, Russell A, Lievens D, Stansen W, Wijsenbeek MS (2021). Effects of Nintedanib in Patients with Idiopathic Pulmonary Fibrosis and Varying Severities of Cough.
Author URL.
Pettersson H, Alexanderson H, Poole JL, Varga J, Regardt M, Russell A-M, Salam Y, Jensen K, Mansour J, Frech T, et al (2021). Exercise as a multi-modal disease-modifying medicine in systemic sclerosis: an introduction by the Global Fellowship on Rehabilitation and Exercise in Systemic Sclerosis (G-FoRSS).
Best Pract Res Clin Rheumatol,
35(3).
Abstract:
Exercise as a multi-modal disease-modifying medicine in systemic sclerosis: an introduction by the Global Fellowship on Rehabilitation and Exercise in Systemic Sclerosis (G-FoRSS).
Systemic sclerosis (SSc) is a heterogeneous multisystem autoimmune disease whereby its main pathological drivers of disability and damage are vascular injury, inflammatory cell infiltration, and fibrosis. These mechanisms result in diffuse and diverse impairments arising from ischemic circulatory dysfunction leading to painful skin ulceration and calcinosis, neurovascular aberrations hindering gastrointestinal (GI) motility, progressive painful, incapacitating or immobilizing effects of inflammatory and fibrotic effects on the lungs, skin, articular and periarticular structures, and muscle. SSc-related impairments impede routine activities of daily living (ADLs) and disrupt three critical life areas: work, family, social/leisure, and also impact on psychological well-being. Physical activity and exercise are globally recommended; however, for connective tissue diseases, this guidance carries greater impact on inflammatory disease manifestations, recovery, and cardiovascular health. Exercise, through myogenic and vascular phenomena, naturally targets key pathogenic drivers by downregulating multiple inflammatory and fibrotic pathways in serum and tissue, while increasing circulation and vascular repair. G-FoRSS, the Global Fellowship on Rehabilitation and Exercise in Systemic Sclerosis recognizes the scientific basis of and advocates for education and research of exercise as a systemic and targeted SSc disease-modifying treatment. An overview of biophysiological mechanisms of physical activity and exercise are herein imparted for patients, clinicians, and researchers, and applied to SSc disease mechanisms, manifestations, and impairment. A preliminary guidance on exercise in SSc, a research agenda, and the current state of research and outcome measures are set forth.
Abstract.
Author URL.
Cassidy N, Fox L, Love M, Byrne I, Doyle AM, Korn B, Shanagher D, Shone T, Cullen M, Cullen T, et al (2021). Fibrotic interstitial lung disease - palliative care needs: a World-Café qualitative study.
BMJ Supportive & Palliative CareAbstract:
Fibrotic interstitial lung disease - palliative care needs: a World-Café qualitative study
ObjectivesThe importance of palliative care in those with advanced fibrotic interstitial lung diseases (F-ILD) is recognised, but the palliative care requirements of patients and caregivers affected by F-ILD regardless of disease course are not established. We set out to explore this and identify optimal solutions in meeting the needs of a F-ILD population in Ireland.MethodsImplementing a World-Café qualitative research approach, we captured insights evolving, iteratively in interactive small group discussions in response to six predefined topics on palliative care and planning for the future. Thirty-nine stakeholders participated in the World-Café including 12 patients, 13 caregivers, 9 healthcare professionals, 4 industry representatives and 1 representative of the clergy.ResultsPalliative care emerged as fundamental to the care and treatment of F-ILDs, regardless of disease progression. Unmet palliative care needs were identified as psychological and social support, disease education, inclusion of caregivers and practical/legal advice for disease progression and end-of-life planning. Participants identified diagnosis as a particularly distressing time for patients and families. They called for the introduction of palliative care discussions at this early-stage alongside improvements in integrated care, specifically increasing the involvement of primary care practitioners in referrals to palliative services.ConclusionPatients and caregivers need discussions on palliative care associated with F-ILD to be included at the point of diagnosis. This approach may address persisting inadequacies in service provision previously identified over the course of the last decade in the UK, Ireland and European F-ILD patient charters.
Abstract.
Sugg HVR, Russell A-M, Morgan L, Iles-Smith H, Richards DA, Morley N, Burnett S, Cockcroft E, Thompson Coon J, Cruickshank S, et al (2021). Fundamental nursing care in patients with the SARS-CoV-2 virus: results from the ‘COVID-NURSE’ mixed methods survey into nurses’ experiences of missed care and barriers to care. BMC Nursing, 20
Saketkoo LA, Russell A-M, Jensen K, Mandizha J, Tavee J, Newton J, Rivera F, Howie M, Reese R, Goodman M, et al (2021). Health-Related Quality of Life (HRQoL) in Sarcoidosis: Diagnosis, Management, and Health Outcomes.
Diagnostics,
11(6), 1089-1089.
Abstract:
Health-Related Quality of Life (HRQoL) in Sarcoidosis: Diagnosis, Management, and Health Outcomes
Health-related quality of life (HRQoL), though rarely considered as a primary endpoint in clinical trials, may be the single outcome reflective of patient priorities when living with a health condition. HRQoL is a multi-dimensional concept that reflects the degree to which a health condition interferes with participation in and fulfillment of important life areas. HRQoL is intended to capture the composite degree of physical, physiologic, psychological, and social impairment resulting from symptom burden, patient-perceived disease severity, and treatment side effects. Diminished HRQoL expectedly correlates to worsening disability and death; but interventions addressing HRQoL are linked to increased survival. Sarcoidosis, being a multi-organ system disease, is associated with a diffuse array of manifestations resulting in multiple symptoms, complications, and medication-related side effects that are linked to reduced HRQoL. Diminished HRQoL in sarcoidosis is related to decreased physical function, pain, significant loss of income, absence from work, and strain on personal relationships. Symptom distress can result clearly from a sarcoidosis manifestation (e.g. ocular pain, breathlessness, cough) but may also be non-specific, such as pain or fatigue. More complex, a single non-specific symptom, e.g. fatigue may be directly sarcoidosis-derived (e.g. inflammatory state, neurologic, hormonal, cardiopulmonary), medication-related (e.g. anemia, sleeplessness, weight gain, sub-clinical infection), or an indirect complication (e.g. sleep apnea, physical deconditioning, depression). Identifying and distinguishing underlying causes of impaired HRQoL provides opportunity for treatment strategies that can greatly impact a patient’s function, well-being, and disease outcomes. Herein, we present a reference manual that describes the current state of knowledge in sarcoidosis-related HRQoL and distinguish between diverse causes of symptom distress and other influences on sarcoidosis-related HRQoL. We provide tools to assess, investigate, and diagnose compromised HRQoL and its influencers. Strategies to address modifiable HRQoL factors through palliation of symptoms and methods to improve the sarcoidosis health profile are outlined; as well as a proposed research agenda in sarcoidosis-related HRQoL.
Abstract.
Althobiani MA, Evans RA, Alqahtani JS, Aldhahir AM, Russell A-M, Hurst JR, Porter JC (2021). Home monitoring of physiology and symptoms to detect interstitial lung disease exacerbations and progression: a systematic review.
ERJ Open Research,
7(4), 00441-2021.
Abstract:
Home monitoring of physiology and symptoms to detect interstitial lung disease exacerbations and progression: a systematic review
BackgroundAcute exacerbations (AEs) and disease progression in interstitial lung disease (ILD) pose important challenges to clinicians and patients. AEs of ILD are variable in presentation but may result in rapid progression of ILD, respiratory failure and death. However, in many cases AEs of ILD may go unrecognised so that their true impact and response to therapy is unknown. The potential for home monitoring to facilitate early, and accurate, identification of AE and/or ILD progression has gained interest. With increasing evidence available, there is a need for a systematic review on home monitoring of patients with ILD to summarise the existing data. The aim of this review was to systematically evaluate the evidence for use of home monitoring for early detection of exacerbations and/or progression of ILD.MethodWe searched Ovid-EMBASE, MEDLINE and CINAHL using Medical Subject Headings (MeSH) terms in accordance with the PRISMA guidelines (PROSPERO registration number CRD42020215166).Results13 studies involving 968 patients have demonstrated that home monitoring is feasible and of potential benefit in patients with ILD. Nine studies reported that mean adherence to home monitoring was >75%, and where spirometry was performed there was a significant correlation (r=0.72–0.98, p<0.001) between home and hospital-based readings. Two studies suggested that home monitoring of forced vital capacity might facilitate detection of progression in idiopathic pulmonary fibrosis.ConclusionDespite the fact that individual studies in this systematic review provide supportive evidence suggesting the feasibility and utility of home monitoring in ILD, further studies are necessary to quantify the potential of home monitoring to detect disease progression and/or AEs.
Abstract.
Noth I, Cottin V, Chaudhuri N, Corte TJ, Johannson KA, Wijsenbeek M, Jouneau S, Michael A, Quaresma M, Rohr KB, et al (2021). Home spirometry in patients with idiopathic pulmonary fibrosis: data from the INMARK trial.
European Respiratory Journal,
58(1), 2001518-2001518.
Abstract:
Home spirometry in patients with idiopathic pulmonary fibrosis: data from the INMARK trial
BackgroundData from the INMARK trial were used to investigate the feasibility and validity of home spirometry as a measure of lung function decline in patients with idiopathic pulmonary fibrosis (IPF).MethodsSubjects with IPF and preserved forced vital capacity (FVC) were randomised to receive nintedanib or placebo for 12â
weeks followed by open-label nintedanib for 40â
weeks. Clinic spirometry was conducted at baseline and weeks 4, 8, 12, 16, 20, 24, 36 and 52. Subjects were asked to perform home spirometry at least once a week and ideally daily. Correlations between home- and clinic-measured FVC and rates of change in FVC were assessed using Pearson correlation coefficients.ResultsIn total, 346 subjects were treated. Mean adherence to weekly home spirometry decreased over time but remained above 75% in every 4-week period. Over 52â
weeks, mean adherence was 86%. Variability in change from baseline in FVC was greater when measured by home rather than clinic spirometry. Strong correlations were observed between home- and clinic-measured FVC at all time-points (r=0.72–0.84), but correlations between home- and clinic-measured rates of change in FVC were weak (r=0.26 for rate of decline in FVC over 52â
weeks).ConclusionHome spirometry was a feasible and valid measure of lung function in patients with IPF and preserved FVC, but estimates of the rate of FVC decline obtained using home spirometry were poorly correlated with those based on clinic spirometry.
Abstract.
Shuttleworth R, Nancarrow T, Wollteron R, White M, Lines S, Mandizha J, Duckworth A, Russell A, Gibbons M, Scotton C, et al (2021). P144 Red cell distribution width (RDW) and neutrophil lymphocyte ratio (NLR) as prognostic markers in idiopathic pulmonary fibrosis (IPF). Thorax, 76(Suppl 2), a145-a146.
Althobiani M, Hurst J, Russell A, Porter J (2021). P6â
Telehealth for patients with interstitial lung diseases (ILD): results of an international survey of clinicians. British Thoracic Society Winter Meeting 2021 Online, Wednesday 24 to Friday 26 November 2021, Programme and Abstracts.
Russell AM, Saketkoo LA (2021). Patient-Centredness and Patient-Reported Measures (PRMs) in Palliation of Lung Disease. In (Ed) Palliative Care in Lung Disease, 43-75.
Aronson KI, Danoff SK, Russell A-M, Ryerson CJ, Suzuki A, Wijsenbeek MS, Bajwah S, Bianchi P, Corte TJ, Lee JS, et al (2021). Patient-centered Outcomes Research in Interstitial Lung Disease: an Official American Thoracic Society Research Statement.
Am J Respir Crit Care Med,
204(2), e3-e23.
Abstract:
Patient-centered Outcomes Research in Interstitial Lung Disease: an Official American Thoracic Society Research Statement.
Background: in the past two decades, many advances have been made to our understanding of interstitial lung disease (ILD) and the way we approach its treatment. Despite this, many questions remain unanswered, particularly those related to how the disease and its therapies impact outcomes that are most important to patients. There is currently a lack of guidance on how to best define and incorporate these patient-centered outcomes in ILD research. Objectives: to summarize the current state of patient-centered outcomes research in ILD, identify gaps in knowledge and research, and highlight opportunities and methods for future patient-centered research agendas in ILD. Methods: an international interdisciplinary group of experts was assembled. The group identified top patient-centered outcomes in ILD, reviewed available literature for each outcome, highlighted important discoveries and knowledge gaps, and formulated research recommendations. Results: the committee identified seven themes around patient-centered outcomes as the focus of the statement. After a review of the literature and expert committee discussion, we developed 28 research recommendations. Conclusions: Patient-centered outcomes are key to ascertaining whether and how ILD and interventions used to treat it affect the way patients feel and function in their daily lives. Ample opportunities exist to conduct additional work dedicated to elevating and incorporating patient-centered outcomes in ILD research.
Abstract.
Author URL.
Althobiani M, Alqahtani JS, Hurst JR, Russell A-M, Porter J (2021). Telehealth for patients with interstitial lung diseases (ILD): results of an international survey of clinicians.
BMJ Open Respiratory Research,
8(1), e001088-e001088.
Abstract:
Telehealth for patients with interstitial lung diseases (ILD): results of an international survey of clinicians
IntroductionClinicians and policymakers are promoting widespread use of home technology including spirometry to detect disease progression for patients with interstitial lung disease (ILD); the COVID-19 pandemic has accelerated this. Data collating clinicians’ views on the potential utility of telehealth in ILD are limited.AimThis survey investigated clinicians’ opinions about contemporary methods and practices used to monitor disease progression in patients with ILD using telehealth.MethodsClinicians were invited to participate in a cross-sectional survey (SurveyMonkey) of 13 questions designed by an expert panel. Telehealth was defined as home monitoring of symptoms and physiological parameters with regular automatic transmission of data from the patient’s home to the clinician. Data are presented as percentages of respondents.ResultsA total of 207 clinicians from 23 countries participated in the survey. A minority (81, 39%) reported using telehealth. 50% (n=41) of these respondents completed a further question about the effectiveness of telehealth. A majority of respondents (32, 70%) rated it to be quite or more effective than face-to-face visit. There were a greater number of respondents using telehealth from Europe (94, 45%) than Asia (51, 25%) and America (24%). Clinicians reported the most useful telehealth monitoring technologies as smartphone apps (59%) and wearable sensors (30%). Telehealth was most frequently used for monitoring disease progression (70%), quality of life (63%), medication use (63%) and reducing the need for in-person visits (63%). Clinicians most often monitored symptoms (93%), oxygen saturation (74%) and physical activity (72%). The equipment perceived to be most effective were spirometers (43%) and pulse oximeters (33%). The primary barriers to clinicians’ participation in telehealth were organisational structure (80%), technical challenges (63%) and lack of time and/or workload (63%). Clinicians considered patients’ barriers to participation might include lack of awareness (76%), lack of knowledge using smartphones (60%) and lack of confidence in telehealth (56%).ConclusionThe ILD clinicians completing this survey who used telehealth to monitor patients (n=81) supported its’ clinical utility. Our findings emphasise the need for robust research in telehealth as a mode for the delivery of cost-effective healthcare services in ILD and highlight the need to assess patients’ perspectives to improve telehealth utility in patients with ILD.
Abstract.
Burki TK (2021). The importance of patient-reported outcomes and measures. The Lancet Respiratory Medicine, 9(11), 1218-1220.
Castro-Sanchez E, Russell A-M, Dolman L, Wells M (2021). What place does nurseâled research have in the COVIDâ19 pandemic?. International Nursing Review, 1-5.
Seligman WH, Fialho L, Sillett N, Nielsen C, Baloch FM, Collis P, Demedts IKM, Fleck MP, Floriani MA, Gabriel LEK, et al (2021). Which outcomes are most important to measure in patients with COVID-19 and how and when should these be measured? Development of an international standard set of outcomes measures for clinical use in patients with COVID-19: a report of the International Consortium for Health Outcomes Measurement (ICHOM) COVID-19 Working Group.
BMJ Open,
11(11), e051065-e051065.
Abstract:
Which outcomes are most important to measure in patients with COVID-19 and how and when should these be measured? Development of an international standard set of outcomes measures for clinical use in patients with COVID-19: a report of the International Consortium for Health Outcomes Measurement (ICHOM) COVID-19 Working Group
ObjectivesThe COVID-19 pandemic has resulted in widespread morbidity and mortality with the consequences expected to be felt for many years. Significant variation exists in the care even of similar patients with COVID-19, including treatment practices within and between institutions. Outcome measures vary among clinical trials on the same therapies. Understanding which therapies are of most value is not possible unless consensus can be reached on which outcomes are most important to measure. Furthermore, consensus on the most important outcomes may enable patients to monitor and track their care, and may help providers to improve the care they offer through quality improvement. To develop a standardised minimum set of outcomes for clinical care, the International Consortium for Health Outcomes Measurement (ICHOM) assembled a working group (WG) of 28 volunteers, including health professionals, patients and patient representatives.DesignA list of outcomes important to patients and professionals was generated from a systematic review of the published literature using the MEDLINE database, from review of outcomes being measured in ongoing clinical trials, from a survey distributed to patients and patient networks, and from previously published ICHOM standard sets in other disease areas. Using an online-modified Delphi process, the WG selected outcomes of greatest importance.ResultsThe outcomes considered by the WG to be most important were selected and categorised into five domains: (1) functional status and quality of life, (2) mental functioning, (3) social functioning, (4) clinical outcomes and (5) symptoms. The WG identified demographic and clinical variables for use as case-mix risk adjusters. These included baseline demographics, clinical factors and treatment-related factors.ConclusionImplementation of these consensus recommendations could help institutions to monitor, compare and improve the quality and delivery of care to patients with COVID-19. Their consistent definition and collection could also broaden the implementation of more patient-centric clinical outcomes research.
Abstract.
2020
Fox L, McLeodl L, Russell A, Edwards C, O'Reilly KMA (2020). An Observational Study of Self-Monitoring of Spirometry and Symptoms via a Mobile Electronic Health Journal in Usual Care of Patients with Idiopathic Pulmonary Fibrosis.
Author URL.
Saketkoo LA, Alexanderson H, Lammi MR, LeSage D, Jensen K, Scholand MB, Volkmann ER, Russell AM (2020). An ode to the primal tonic of dance—congratulating the Life of Breath project. The Lancet Respiratory Medicine, 8(12), e90-e91.
Koschel D, Maher T, Cottin V, Russell A, Corte T, Hammerl P, Michael A, Rohr K, Quaresma M, Stowasser S, et al (2020). Correlation between home and clinic spirometry in subjects with IPF: results from the INMARK trial. 61. Kongress der Deutschen Gesellschaft für Pneumologie und Beatmungsmedizin e.V.
Kim JW, Olive S, Jones S, Thillai M, Russell A-M, Johnson MJ, Wilson A (2020). Interstitial lung disease and specialist palliative care access: a healthcare professionals survey.
BMJ Supportive & Palliative Care,
12(e6), e748-e751.
Abstract:
Interstitial lung disease and specialist palliative care access: a healthcare professionals survey
BackgroundFibrotic interstitial lung disease is an incurable disease with poor prognosis. We aimed to understand factors affecting decisions regarding referrals to specialist palliative care services and to address barriers and facilitators to referrals from healthcare professionals’ perspectives.MethodsA survey study of healthcare professionals, including respiratory physicians, interstitial lung disease nurse specialists, respiratory nurse specialists and palliative care physicians, was conducted using a questionnaire, entailing 17 questions.ResultsThirty-six respondents, including 15 interstitial lung disease nurse specialists completed the questionnaire. Symptom control, psychological/spiritual support, general deterioration and end-of-life care were the most common reasons for referrals to specialist palliative care services. Most respondents felt confident in addressing palliative care needs and discussing palliative care with patients. A few participants emphasised that experienced respiratory nurse specialists are well placed to provide symptom management and to ensure continuity of patient care. Participants reported that access to palliative care could be improved by increasing collaborative work between respiratory and palliative care teams.ConclusionsMost respondents felt that enhancing access to specialist palliative care services would benefit patients. However, palliative care and respiratory care should not be considered as mutually exclusive and multidisciplinary approach is recommended.
Abstract.
Russell A (2020). Letter from the. <scp>UK</scp>. Respirology, 25(12), 1325-1327.
Russell A, Wickremasinghe M, Aul R, Datta A, Saketkoo L, Wells M (2020). Measuring Patient Activation Measurement in Patients Diagnosed with Interstitial Lung Disease (ILD) to Inform Enabling and Compassionate Care.
Author URL.
Russell A-M, Aul R, Datta A, Wickremasinghe M (2020). Measuring patient activation in an interstitial lung disease (ILD) population: an exploratory study. ERS International Congress 2020 abstracts.
Fontanilles E, Vancheri C, Wijsenbeek M, Antoniou K, Rivera P, Bonella F, Renzoni E, Anne-Marie A-M, Vicens-Zygmunt V, Suarez-Cuartin G, et al (2020). Monounsaturated Fatty Acid diet associates less gastrointestinal adverse events of pirfenidone: phase IV clinical trial. ERS International Congress 2020 abstracts.
Drake TM, Docherty AB, Harrison EM, Quint JK, Adamali H, Agnew S, Babu S, Barber CM, Barratt S, Bendstrup E, et al (2020). Outcome of Hospitalization for COVID-19 in Patients with Interstitial Lung Disease. An International Multicenter Study. American Journal of Respiratory and Critical Care Medicine, 202(12), 1656-1665.
Saketkoo LA, Scholand MB, Lammi MR, Russell A-M (2020). Patient-reported outcome measures in systemic sclerosis–related interstitial lung disease for clinical practice and clinical trials.
Journal of Scleroderma and Related Disorders,
5(2_suppl), 48-60.
Abstract:
Patient-reported outcome measures in systemic sclerosisârelated interstitial lung disease for clinical practice and clinical trials
Systemic sclerosis (SSc) is a progressive vasculopathic, fibrosing autoimmune condition, portending significant mortality; wherein interstitial lung disease (ILD) is the leading cause of death. Although lacking a definitive cure, therapeutics for (SSc-ILD) that stave progression exist with further promising primary and adjuvant compounds in development, as well as interventions to reduce symptom burden and increase quality of life. To date, there has been a significant but varied history related to systemic sclerosis–related interstitial lung disease trial design and endpoint designation. This is especially true of endpoints measuring patient-reported perceptions of efficacy and tolerability. This article describes the underpinnings and complexity of the science, methodology, and current state of patient-reported outcome measures used in (SSc-ILD) systemic sclerosis–related interstitial lung disease in clinical practice and trials.
Abstract.
Fox L, Mcleod L, Russell A-M, Edwards C, O'Reilly K (2020). Single-arm observational study of a mobile app + home spirometry in idiopathic pulmonary fibrosis. ERS International Congress 2020 abstracts.
Edwards C, Costello E, Cassidy N, Vick B, Russell A-M (2020). The utility of an electronic health journal with home spirometry in a real world population of people with fibrotic lung conditions. Journal of Medical Internet Research, 8
Edwards C, Costello E, Cassidy N, Vick B, Russell A-M (2020). Use of the patientMpower App with Home-Based Spirometry to Monitor the Symptoms and Impact of Fibrotic Lung Conditions: Longitudinal Observational Study.
JMIR mHealth and uHealth,
8(11), e16158-e16158.
Abstract:
Use of the patientMpower App with Home-Based Spirometry to Monitor the Symptoms and Impact of Fibrotic Lung Conditions: Longitudinal Observational Study
. Background
. Daily home-based spirometry in idiopathic pulmonary fibrosis (IPF) has been shown to be feasible and clinically informative. The patientMpower app facilitates home-based spirometry along with home-based monitoring of IPF-related symptoms. The patientMpower app can be downloaded to the user’s mobile phone or tablet device, enabling the recording of objective and subjective data.
.
.
. Objective
. The aim of this paper is to report on the 1-year experience of using patientMpower with home-based spirometry by 36 participants with self-reported pulmonary fibrosis (PF) treated with usual care.
.
.
. Methods
. Self-selecting participants enrolled in this community-based participatory research program through a patient advocacy group in their country: Irish Lung Fibrosis Association in Ireland and PF Warriors in the United States. Disease severity was comparable with a baseline mean predicted forced vital capacity (FVC) of 64% and 62% in the Irish and US participants, respectively. Both groups of participants were allocated to identical, in-country, open-label, single-group observational studies and were provided with a Bluetooth-active Spirobank Smart spirometer integrated directly with patientMpower. Data collected via patientMpower included seated FVC (daily), breathlessness grade (modified Medical Research Council scale score), step count, medication adherence, and symptoms and impact of IPF on daily life, which were measured by a patient-reported outcome measure (PROM) scale that was specifically developed for IPF. Longitudinal patient-reported data on oximetry and oxygen consumption were also collected.
.
.
. Results
. A large majority of the 36 participants reported that their experience using patientMpower was positive, and they wanted to continue its use after the initial 6-week observation. Out of 36 participants, 21 (58%) recorded home-based spirometry without prompting for ≥180 days, and 9 (25%) participants continued with recording home-based spirometry for ≥360 days.
.
.
. Conclusions
. The patientMpower app with associated Bluetooth-connected devices (eg, spirometer and pulse oximeter) offers an acceptable and accessible approach to collecting patient-reported objective and subjective data in fibrotic lung conditions.
.
Abstract.
2019
Russell A, Shone A, Love M, Shanagher D, Byrne I, Fox L, Korn B, Doyle A, Cassidy N (2019). A World Cafe Approach to Palliative Care and Planning for the Future in Fibrotic Lung Disease.
Author URL.
Maher T, Cottin V, Russell A-M, Corte T, Hammerl P, Michael A, Rohr KB, Quaresma M, Stowasser S, Noth I, et al (2019). Correlation between home and clinic spirometry in subjects with IPF: results from the INMARK trial.
Author URL.
Russell A-M, Datta A, Newell K, Jones S, Conway J, Saktkoo LA, Wickremasinghe M (2019). Development of a patient reported experience measure (PREM) for idiopathic pulmonary fibrosis (IPF).
Author URL.
Moor CC, Wijsenbeek MS, Balestro E, Biondini D, Bondue B, Cottin V, Flewett R, Galvin L, Jones S, Molina-Molina M, et al (2019). Gaps in care of patients living with pulmonary fibrosis: a joint patient and expert statement on the results of a Europe-wide survey.
ERJ Open Research,
5(4), 00124-2019.
Abstract:
Gaps in care of patients living with pulmonary fibrosis: a joint patient and expert statement on the results of a Europe-wide survey
IntroductionPulmonary fibrosis (PF) and its most common form, idiopathic pulmonary fibrosis (IPF), are chronic, progressive diseases resulting in increasing loss of lung function and impaired quality of life and survival. The aim of this joint expert and patient statement was to highlight the most pressing common unmet needs of patients with PF/IPF, putting forward recommendations to improve the quality of life and health outcomes throughout the patient journey.MethodsTwo online surveys for patients and healthcare professionals (HCPs) were conducted by the European Idiopathic Pulmonary Fibrosis and Related Disorders Federation (EU-IPFF) in 14 European countries.ResultsThe surveys were answered by 286 patients and 69 HCPs, including physicians and nurses. Delays in diagnosis and timely access to interstitial lung disease specialists and pharmacological treatment have been identified as important gaps in care. Additionally, patients and HCPs reported that a greater focus on symptom-centred management, adequate information, trial information and increasing awareness of PF/IPF is required.ConclusionsThe surveys offer important insights into the current unmet needs of PF/IPF patients. Interventions at different points of the care pathway are needed to improve patient experience.
Abstract.
Lim RK, Humphreys C, Morisset J, Holland AE, Johannson KA (2019). Oxygen in patients with fibrotic interstitial lung disease: an international Delphi survey.
European Respiratory Journal,
54(2), 1900421-1900421.
Abstract:
Oxygen in patients with fibrotic interstitial lung disease: an international Delphi survey
RationalePatients with fibrotic interstitial lung disease (ILD) frequently develop resting or exertional hypoxaemia. There is heterogeneity in clinical practice and a paucity of evidence guiding supplemental oxygen use in this patient population. The objectives of this study were to build international expert-based consensus on the indications and goals of supplemental oxygen from the perspective of healthcare providers, and identify potential barriers to its access.MethodsSemistructured interviews and a comprehensive literature search informed items for the Delphi survey, with items not meeting consensus included in round 2. Round 3 contained survey questions regarding regional funding coverage for oxygen therapy.A prioridefinitions of consensus were median scores of 4 (agree) to 5 (strongly agree) for “agreement”, 1 (strongly disagree) to 2 (disagree) for “disagreement” or 3 (unsure) with an interquartile range of 0–1.Results42 out of 45 (93%) experts completed all three survey rounds, representing 17 countries. 20 out of 36 items met consensus for agreement or disagreement, 10 items met consensus for unsure and four items did not meet consensus. Experts agreed that oxygen should be recommended for patients with severe resting hypoxaemia and in cases of exertional desaturation to <85–89%, particularly with attributable symptoms or exercise limitation. There are regional differences in funding coverage for oxygen, based on desaturation thresholds, clinical symptoms and testing requirements.ConclusionsExperts achieved consensus on 20 items guiding supplemental oxygen use in fibrotic ILD. These findings may inform research, clinical recommendations and funding policy.
Abstract.
van Manen MJG, Birring SS, Vancheri C, Odink AE, Hussain B, Vindigni V, Renzoni E, Russell A-M, Wapenaar M, Cottin V, et al (2019). Predictors of objective cough in patients with idiopathic pulmonary fibrosis (IPF).
Author URL.
2018
Russell AM, Olive S, Lines S, Murphy A, Hocking J, Newell K, Morris H, Harris E, Dixon C, Agnew S, et al (2018). Contemporary challenges for specialist nursing in interstitial lung disease.
Breathe,
14(1), 36-41.
Abstract:
Contemporary challenges for specialist nursing in interstitial lung disease
The role of clinical nurse specialists (CNSs) in interstitial lung disease (ILD) is evolving in response to clinical guidelines and the growth of clinical research. The role is well established in the UK, although more ILD posts are needed to ensure supply meets clinical demand. This phenomenon is also happening across Europe. An appreciation of the similarities and differences between CNS and advanced nurse practitioners is important given the challenges in defining, developing and supporting this nursing specialisation. Globally, different models exist. In some countries charitable organisations take a leading role in supporting patients. Many European centres look to the National Institute for Health and Care Excellence guidelines and quality standards as a template to develop and evaluate the role of the ILD CNS. We present a UK perspective in the context of a government subsidised healthcare system to promote professional discussion and debate regarding the future of nursing practice in the ILD specialty.
Abstract.
Visca D, Mori L, Tsipouri V, Fleming S, Firouzi A, Bonini M, Pavitt MJ, Alfieri V, Canu S, Bonifazi M, et al (2018). Effect of ambulatory oxygen on quality of life for patients with fibrotic lung disease (AmbOx): a prospective, open-label, mixed-method, crossover randomised controlled trial.
Lancet Respir Med,
6(10), 759-770.
Abstract:
Effect of ambulatory oxygen on quality of life for patients with fibrotic lung disease (AmbOx): a prospective, open-label, mixed-method, crossover randomised controlled trial.
BACKGROUND: in fibrotic interstitial lung diseases, exertional breathlessness is strongly linked to health-related quality of life (HRQOL). Breathlessness is often associated with oxygen desaturation, but few data about the use of ambulatory oxygen in patients with fibrotic interstitial lung disease are available. We aimed to assess the effects of ambulatory oxygen on HRQOL in patients with interstitial lung disease with isolated exertional hypoxia. METHODS: AmbOx was a prospective, open-label, mixed-method, crossover randomised controlled clinical trial done at three centres for interstitial lung disease in the UK. Eligible patients were aged 18 years or older, had fibrotic interstitial lung disease, were not hypoxic at rest but had a fall in transcutaneous arterial oxygen saturation to 88% or less on a screening visit 6-min walk test (6MWT), and had self-reported stable respiratory symptoms in the previous 2 weeks. Participants were randomly assigned (1:1) to either oxygen treatment or no oxygen treatment for 2 weeks, followed by crossover for another 2 weeks. Randomisation was by a computer-generated sequence of treatments randomly permuted in blocks of constant size (fixed size of ten). The primary outcome, which was assessed by intention to treat, was the change in total score on the King's Brief Interstitial Lung Disease questionnaire (K-BILD) after 2 weeks on oxygen compared with 2 weeks of no treatment. General linear models with treatment sequence as a fixed effect were used for analysis. Patient views were explored through semi-structured topic-guided interviews in a subgroup of participants. This study was registered with ClinicalTrials.gov, number NCT02286063, and is closed to new participants with all follow-up completed. FINDINGS: Between Sept 10, 2014, and Oct 5, 2016, 84 patients were randomly assigned, 41 randomised to ambulatory oxygen first and 43 to no oxygen. 76 participants completed the trial. Compared with no oxygen, ambulatory oxygen was associated with significant improvements in total K-BILD scores (mean 55·5 [SD 13·8] on oxygen vs 51·8 [13·6] on no oxygen, mean difference adjusted for order of treatment 3·7 [95% CI 1·8 to 5·6]; p
Abstract.
Author URL.
Cassidy N, O'Dowd G, McGowan M, Shone T, Russell A-M (2018). Evaluation of a World Cafe Forum on Palliative Care and Planning for the Future.
Author URL.
Fleming S, Firouzi A, Farquhar M, Tsipouri V, Visca D, Mori L, Canu S, Hogben CR, Kokosi M, Kouranos V, et al (2018). Experiences of Supplemental Ambulatory Oxygen for Patients with Fibrotic Lung Disease: Qualitative Evidence from the Mixed Method AmbOx Trial.
Author URL.
Saketkoo LA, Karpinski A, Young J, Adell R, Walker M, Hennebury T, Wickremasinghe M, Russell A-M (2018). Feasibility, utility and symptom impact of modified mindfulness training in sarcoidosis. ERJ Open Research, 4(2), 00085-2017.
Newell K, Smith K, Russell A-M (2018). Palliative and end of life care in idiopathic pulmonary fibrosis. Primary Health Care, 28(4), 27-35.
Walsh S, Cahill T, Edwards C, Costello E, Walsh J, Russell A, O'Regan AW (2018). Patient-Reported Monitoring of Symptoms and Spirometry Via the patientMpower Platform in Idiopathic Pulmonary Fibrosis.
Author URL.
Russell A, Wickremasinghe M, Saketkoo L, Borril Z, Fletcher S, Adamali HI, Wells AU, Maher TM, Renzoni EA, Fleming S, et al (2018). Preliminary Testing of the Idiopathic Pulmonary Fibrosis Patient Reported Outcome Measure (IPF PRoM).
Author URL.
Russell AM, Wickremasinghe M, Renzoni E, Adamali H, Borril Z, Fletcher S, Maher TM, Kwong GNM, Saketkoo LA, Fleming S, et al (2018). THE IDIOPATHIC PULMONARY FIBROSIS PATIENTS REPORTED OUTCOME MEASURE (IPF-PROM) IS RELIABLE AND VALID FOR USE IN POPULATIONS WITH IPF.
Author URL.
2017
Edwards C, Cassidy N, Costello E, Russell A-M (2017). A longitudinal approach to supported self-management: sustainability of the idiopathic interstitial pneumonia (IIP) electronic health journal (patientMpower) with integrated home spirometry.
Author URL.
Edwards C, Cassidy N, Costello E, Russell A-M (2017). A longitudinal approach to supported self-management: sustainability of the idiopathic interstitial pneumonia (IIP) electronic health journal (patientMpower) with integrated home spirometry.
Author URL.
Visca D, Mori L, Tsipouri V, Canu S, Bonini M, Pavitt M, Fleming S, Firouzi A, Farquhar M, Leung E, et al (2017). AmbOx trial: does ambulatory oxygen improve quality of life in patients with fibrotic interstitial lung disease?.
Author URL.
Visca D, Mori L, Tsipouri V, Canu S, Bonini M, Pavitt M, Fleming S, Firouzi A, Farquhar M, Leung E, et al (2017). Ambox: a Randomised Controlled, Crossover Trial Evaluating the Effects of Ambulatory Oxygen on Health Status in Patients with Fibrotic Interstitial Lung Disease.
Author URL.
Maher TM, Oballa E, Simpson JK, Porte J, Habgood A, Fahy WA, Flynn A, Molyneaux PL, Braybrooke R, Divyateja H, et al (2017). An epithelial biomarker signature for idiopathic pulmonary fibrosis: an analysis from the multicentre PROFILE cohort study.
The Lancet Respiratory Medicine,
5(12), 946-955.
Abstract:
An epithelial biomarker signature for idiopathic pulmonary fibrosis: an analysis from the multicentre PROFILE cohort study
Background Idiopathic pulmonary fibrosis (IPF) is a progressive, fatal disorder with a variable disease trajectory. The aim of this study was to assess potential biomarkers to predict outcomes for people with IPF. Methods PROFILE is a large prospective longitudinal cohort of treatment-naive patients with IPF. We adopted a two-stage discovery and validation design using patients from the PROFILE cohort. For the discovery analysis, we examined 106 patients and 50 age and sex matched healthy controls from Nottingham University Hospitals NHS Trust and the Royal Brompton Hospital. We did an unbiased, multiplex immunoassay assessment of 123 biomarkers. We further investigated promising novel markers by immunohistochemical assessment of IPF lung tissue. In the validation analysis, we examined samples from 206 people with IPF from among the remaining 212 patients recruited to PROFILE Central England. We used the samples to attempt to replicate the biomarkers identified from the discovery analysis by use of independent immunoassays for each biomarker. We investigated the predictive power of the selected biomarkers to identify individuals with IPF who were at risk of progression or death. The PROFILE studies are registered on ClinicalTrials.gov, numbers NCT01134822 (PROFILE Central England) and NCT01110694 (PROFILE Royal Brompton Hospital). Findings in the discovery analysis, we identified four serum biomarkers (surfactant protein D, matrix metalloproteinase 7, CA19-9, and CA-125) that were suitable for replication. Histological assessment of CA19-9 and CA-125 suggested that these proteins were markers of epithelial damage. Replication analysis showed that baseline values of surfactant protein D (46·6 ng/mL vs 34·6 ng/mL, p=0·0018) and CA19-9 (53·7 U/mL vs 22·2 U/mL; p
Abstract.
Spruit MA, Burtin C, Langer D, Makonga J, Russell A-M, York J, Steenbruggen I (2017). Assembly 9: allied respiratory professionals. Breathe, 12(4), e113-e114.
Russell A-M, Jones G, Saketkoo L, Doyle A-M, Sanderson T, D'Accord C, Adamali H, Borrill Z, Fletcher S, Wickremasinghe M, et al (2017). Development and Preliminary Testing of the Idiopathic Pulmonary Fibrosis Patient Reported Outcome Measure (ipf-Prom): Uk and Ireland Multi-Centre Study.
Author URL.
van Manen MJG, Birring SS, Vancheri C, Vindigni V, Renzoni E, Russell A-M, Wapenaar M, Cottin V, Wijsenbeek MS (2017). Effect of pirfenidone on cough in patients with idiopathic pulmonary fibrosis.
EUROPEAN RESPIRATORY JOURNAL,
50(4).
Author URL.
Mori L, Canu S, Visca D, Tsipouri V, Bonini M, Pavitt M, Fleming S, Firouzi A, Farquhar M, Leung E, et al (2017). PREDICTORS OF UPTAKE OF AMBULATORY OXYGEN ON COMPLETION OF THE AMBOX TRIAL, a STUDY TO ASSESS EFFECTS OF AMBULATORY OXYGEN ON QUALITY OF LIFE IN PATIENTS WITH FIBROTIC INTERSTITIAL LUNG DISEASE.
Author URL.
Kreuter M, Bendstrup E, Russell A-M, Bajwah S, Lindell K, Adir Y, Brown CE, Calligaro G, Cassidy N, Corte TJ, et al (2017). Palliative care in interstitial lung disease: living well.
LANCET RESPIRATORY MEDICINE,
5(12), 968-980.
Author URL.
Edwards C, Cassidy N, Costello E, Russell A-M (2017). Pilot Study to Test the Utility and Acceptability of an Electronic Health Record (patientMpower) for Patients with Lung Fibrosis. Iproceedings, 3(1).
Russell A-M, Cave P, Taylor K, Lewis A, Hopkinson NS, Wells AU (2017). Rhythm and song: Breath management in Idiopathic Interstitial Pneumonias (IIP's). Pilot study.
Author URL.
Maher TM, Molina-Molina M, Russell A-M, Bonella F, Jouneau S, Ripamonti E, Axmann J, Vancheri C (2017). Unmet needs in the treatment of idiopathic pulmonary fibrosis-insights from patient chart review in five European countries.
BMC PULMONARY MEDICINE,
17 Author URL.
2016
Russell A-M (2016). Assessing Anxiety and Depression. In Preston W (Ed)
Respiratory Nursing at a Glance, Chichester, Sussex: John Wiley & Sons, 46-47.
Abstract:
Assessing Anxiety and Depression
Abstract.
van Manen MJG, Birring SS, Vancheri C, Cottin V, Renzoni EA, Russell A-M, Wijsenbeek MS (2016). Cough in idiopathic pulmonary fibrosis.
EUROPEAN RESPIRATORY REVIEW,
25(141), 278-286.
Author URL.
Russell AM, Sonecha S, Datta A, Hewitt R, Howell I, Elliott A, Wickremasinghe M (2016). DEVELOPMENT OF PATIENT REPORTED EXPERIENCE MEASURE (PREM) FOR IDIOPATHIC PULMONARY FIBROSIS (IPF).
Author URL.
Russell A-M, Maher TM (2016). Daily Home Spirometry: a New Milestone in the Field of Pulmonary Fibrosis Reply.
AMERICAN JOURNAL OF RESPIRATORY AND CRITICAL CARE MEDICINE,
194(8), 1034-1035.
Author URL.
Russell A-M, Adamali H, Molyneaux PL, Lukey PT, Marshall RP, Renzoni EA, Wells AU, Maher TM (2016). Daily Home Spirometry: an Effective Tool for Detecting Progression in Idiopathic Pulmonary Fibrosis.
AMERICAN JOURNAL OF RESPIRATORY AND CRITICAL CARE MEDICINE,
194(8), 989-997.
Author URL.
Hogben C, Renzoni E, Wells AU, Kokosi M, Chua F, Maher TM, Russell AM (2016). Development of pre-admission information for referrals with suspected interstitial lung disease to a specialist unit.
Author URL.
Russell A-M, Johnston KN, Yorke J, Williams MT (2016). Differences in Perceptual Experiences of Breathlessness and Impairment Between People with Moderate-Severe COPD in Australia and the United Kingdom.
Author URL.
Russell A-M, Johnston KN, Yorke J, Williams MT (2016). Dyspnoea-12 Scores Reported "these Days" Are Greater Than Those Reported "today": Differences in People with COPD, Asthma and Ild.
Author URL.
Russell A-M, Jones G, Sabella R, Cassidy N, Burge G, Lines S, Stanley L, Fletcher S, Weallans M, Bray M, et al (2016). Health status & impact of living with idiopathic pulmonary fibrosis (IPF): UK & Ireland Delphi survey.
Author URL.
Russell A-M, Scholand M, Snyder EA, Russell A, Burdett C, Doyle A-M, Lasky J, Renzoni E, Wells A, Saketkoo L, et al (2016). Impact, Survival, Symptoms and Management: Us and Uk Patient Perceptions of Living with Idiopathic Pulmonary Fibrosis.
Author URL.
Russell A-M (2016). Interstitial Lung Disease. In Preston W, Kelly C (Eds.)
Respiratory Nursing at a Glance, Chichester, Sussex: John Wiley & Sons, 68-69.
Abstract:
Interstitial Lung Disease
Abstract.
Stock C, Bonini M, Kingston S, Lindahl GE, Russell A-M, Molyneaux PL, Saunders P, Kokosi MA, Sestini P, Wells AU, et al (2016). Muc5b and Tollip Variants: Association with Disease Progression and Survival in an Ipf Cohort.
Author URL.
Saketkoo L, Young J, Adell R, Karpinski AC, Walker M, Russell A-M (2016). Perceptions of a Modified Mindfulness Training (mt) Program in Sarcoidosis.
Author URL.
Russell A-M, Ripamonti E, Vancheri C (2016). Qualitative European survey of patients with idiopathic pulmonary fibrosis: patients' perspectives of the disease and treatment.
BMC PULMONARY MEDICINE,
16 Author URL.
Visca D, Fleming S, Firouzi A, Farquhar M, Hopkinson N, Hogben C, Banya W, Cullinan P, De Lauretis A, Kokosi M, et al (2016). Randomised controlled, crossover trial to evaluate the effects of ambulatory oxygen on health status in patients with fibrotic lung disease.
Author URL.
Russell A-M (2016). Sarcoidosis. In Preston W, Kelly C (Eds.)
Respiratory Nursing at a Glance, Chichester Sussex: John Wiley & Sons, 70-71.
Abstract:
Sarcoidosis
Abstract.
Lewis A, Cave P, Stern M, Welch L, Taylor K, Russell J, Doyle A-M, Russell A-M, McKee H, Clift S, et al (2016). Singing for Lung Health-a systematic review of the literature and consensus statement.
NPJ PRIMARY CARE RESPIRATORY MEDICINE,
26 Author URL.
Russell A-M, Scholand M, Snyder EA, Doyle A-M, Russell A, Burdett C, Lasky J, Saketkoo LA (2016). Trajectory of Symptom Burden, Impact and Survival in an Idiopathic Pulmonary Fibrosis Population.
Author URL.
Maher TM, Molina-Molina M, Russell A-M, Bonella F, Jouneau S, Ripamonti E, Axmann J, Vancheri C (2016). UNMET NEEDS IN THE TREATMENT OF IDIOPATHIC PULMONARY FIBROSIS (IPF) - INSIGHTS FROM PATIENT CHART REVIEW IN FIVE EUROPEAN COUNTRIES.
Author URL.
Russell A-M, Swigris JJ (2016). What's it like to live with idiopathic pulmonary fibrosis? Ask the experts.
EUROPEAN RESPIRATORY JOURNAL,
47(5), 1324-1326.
Author URL.
2015
Russell A-M, Doyle A-M, Burdett C, Gane J, Aden Z, Fleming S, Cullinan P (2015). A patient centred approach to the development of a patient reported outcome measure (PRoM) in idiopathic pulmonary fibrosis (IPF).
Author URL.
Russell AM (2015). Care planning in idiopathic pulmonary fibrosis.
Nursing Times,
111(18), 20-22.
Abstract:
Care planning in idiopathic pulmonary fibrosis
Patients with idiopathic pulmonary fibrosis require accurate diagnosis and treatment to improve their quality of life and prognosis. This case study follows a patient's care from presentation to diagnosis and management over four years. It illustrates the recommended care pathway, the complexity of the condition and the importance of shared care between specialist and local services. See page 16 for the latest NICE standards for IPF care.
Abstract.
Russell A-M, Doyle A, Fleming S, Burdett C, Ross D, Aden Z, Maher TM, Cullinan P (2015). Development of a Patient Reported Outcome Measure ( prom) in Idiopathic Pulmonary Fibrosis (ipf): Incorporating a Research Partnership with Patients.
Author URL.
Russell A-M, Sanderson T, Wells AU, Fleming S, Maher TM, Cullinan P (2015). Development of a Patient Reported Outcome Measure (prom) in Idiopathic Pulmonary Fibrosis (ipf): an Iterative Process.
Author URL.
Saketkoo LA, Escorpizo R, Keen KJ, Fligelstone K, Birring SS, Lammi MR, Lasky JA, LeSage DR, Renzoni E, Russell AM, et al (2015). INTERNATIONAL CLASSIFICATION OF FUNCTIONING, DISABILITY, AND HEALTH (ICF) CORE SETS FOR CONNECTIVE TISSUE DISEASE INTERSTITIAL LUNG DISEASE (CTD-ILD) AND IDIOPATHIC PULMONARY FIBROSIS (IPF) - a NECESSARY MAP TO HEALTH CARE PROVISION IN THE ERA OF ICD-11.
Author URL.
Russell A-M (2015). Idiopathic pulmonary fibrosis: Care Standards. Nursing Times, 111:18, 23-25.
Russell AM (2015). Interstitial pulmonary fibrosis: Care standards.
Nursing Times,
111(18), 16-17.
Abstract:
Interstitial pulmonary fibrosis: Care standards
Idiopathic pulmonary fibrosis (IPF), an interstitial idiopathic pneumonia, is the most common of the interstitial lung diseases. The emergence of new treatments for the condition has increased the need for efficient, high-quality care, preferably in specialist centres. This article reviews the latest NICE standards for IPF care; a case study (page 20) describes the management of a patient with IPF.
Abstract.
Russell A-M, Sanderson T, Fleming S, Wells A, Maher T, Cullinan P (2015). Item generation for a patient reported outcome measure (PRoM) in idiopathic pulmonary fibrosis (IPF): Application of consensus methods.
Author URL.
Jenkins RG, Simpson JK, Saini G, Bentley JH, Russell A-M, Braybrooke R, Molyneaux PL, McKeever TM, Wells AU, Flynn A, et al (2015). Longitudinal change in collagen degradation biomarkers in idiopathic pulmonary fibrosis: an analysis from the prospective, multicentre PROFILE study.
LANCET RESPIRATORY MEDICINE,
3(6), 462-472.
Author URL.
Lammi M, Baughman R, Birring S, Russell A-M, Ryu J, Scholand M, Distler O, LeSage D, Sarver C, Antoniou K, et al (2015). Outcome Measures for Clinical Trials in Interstitial Lung Diseases. Current Respiratory Medicine Reviews, 11(2), 163-174.
Russell AM, Doyle AM, Ross D, Burdett C, Gane J, Fleming S, Aden Z, Maher TM, Cullinan P (2015). PATIENT AND CARER CO-INVESTIGATORS: SHARED EXPERIENCES OF a RESEARCH STEERING GROUP FROM THE IDIOPATHIC PULMONARY FIBROSIS PATIENT REPORTED OUTCOME MEASURE (IPF-PROM) STUDY.
Author URL.
Cove J, Russell AM, Wright J, Hogben C, Kokosi M, Mak V, Chua F, Wells A, Doyle AM, Renzoni E, et al (2015). PILOT STUDY TO TEST THE FEASIBILITY OF a PSYCHOLOGICAL SUPPORT WORKSHOP FOR PATIENTS NEWLY DIAGNOSED WITH IDIOPATHIC PULMONARY FIBROSIS (IPF) AND THEIR FAMILIES.
Author URL.
Doyle A-M, Burdett C, Gane J, Aden Z, Russell A-M (2015). Psychological distress in UK patients with idiopathic pulmonary fibrosis; use of emotion thermometers interpreted within a biopsychosocial constructionist framework.
Author URL.
Saketkoo L, Escorpizo R, Keen KJ, Fligelstone K, Lammi MR, LeSage D, Russell A-M, Birring SS, Sarver C, Varga J, et al (2015). The World Health Organization (who) International Classification of Functioning, Disability, and Health (icf) Core Sets for Connective Tissue Disease Interstitial Lung Disease (ctd-Ild) and Idiopathic Pulmonary Fibrosis (ipf) - a Necessary Map to Health Care Provision in the Era of Icd-11.
Author URL.
Russell A-M, Sprangers MAG, Wibberley S, Snell N, Rose DM, Swigris JJ (2015). The need for patient-centred clinical research in idiopathic pulmonary fibrosis.
BMC MEDICINE,
13 Author URL.
2014
Russell AM, Sanderson T, Fleming S, Wells AU, Maher TMM, Cullinan TP (2014). DEVELOPMENT OF AN IDIOPATHIC PULMONARY FIBROSIS (IPF) PATIENT REPORTED OUTCOME MEASURE (PROM): AN ITERATIVE APPROACH TO ITEM GENERATION.
Author URL.
Russell AM, Maher TM (2014). Detecting anxiety and depression in patients diagnosed with an interstitial lung disease. Can we do better?.
RESPIROLOGY,
19(8), 1095-1096.
Author URL.
Molyneaux PL, Cox MJ, Willis-Owen SAG, Russell KE, Mallia P, Russell AM, Johnston SL, Wells AU, Cookson WOC, Maher TM, et al (2014). High Bacterial Load Predicts Poor Outcomes in Patients with Idiopathic Pulmonary Fibrosis.
Escorpizo R, Keen KJ, Fligelstone K, Lammi MR, LeSage D, Russell A-M, Birring S, Sarver C, Varga J, Distler O, et al (2014). International Classification of Functioning, Disability, and Health (ICF) Core Sets for Connective Tissue Disease Interstitial Lung Disease (CTD-ILD) and Idiopathic Pulmonary Fibrosis (IPF) - a Necessary Map to Health Care Provision in the Era of ICD-11.
Author URL.
Wuyts WA, Peccatori FA, Russell A-M (2014). Patient-centred management in idiopathic pulmonary fibrosis: similar themes in three communication models. European Respiratory Review, 23(132), 231-238.
Molyneaux PL, Cox MJ, Willis-Owen SAG, Mallia P, Russell KE, Russell A-M, Murphy E, Johnston SL, Schwartz DA, Wells AU, et al (2014). The Role of Bacteria in the Pathogenesis and Progression of Idiopathic Pulmonary Fibrosis. American Journal of Respiratory and Critical Care Medicine, 190(8), 906-913.
2013
Russell AM, Vancheri C, Maronati M, Giot C (2013). A QUALITATIVE EUROPEAN SURVEY OF PATIENTS PERCEPTIONS OF CURRENT MANAGEMENT OF IDIOPATHIC PULMONARY FIBROSIS.
Author URL.
Stock CJ, Sato H, Fonseca C, Banya WAS, Molyneaux PL, Adamali H, Russell A-M, Denton CP, Abraham DJ, Hansell DM, et al (2013). Mucin 5B promoter polymorphism is associated with idiopathic pulmonary fibrosis but not with development of lung fibrosis in systemic sclerosis or sarcoidosis.
THORAX,
68(5), 436-441.
Author URL.
Russell A-M, Fraser U, Molyneaux P, Wells A, Renzoni E, Lukey P, Maher T (2013). Quality of life measures in patients with idiopathic pulmonary fibrosis.
Author URL.
Russell A-M, Molyneaux PL, Wells AU, Renzoni E, Luley P, Maher TM (2013). Symptom Measures in Patients with Idiopathic Pulmonary Fibrosis.
Author URL.
2012
Russell A-M, Molyneaux PL, Lukey PT, Fraser UH, Renzoni EA, Wells A, Maher TM (2012). Daily Hand-Held Spirometry for the Monitoring of Patients with Idiopathic Pulmonary Fibrosis. American Thoracic Society 2012 International Conference, May 18-23, 2012 • San Francisco, California.
Adamali HI, Anwar MS, Russell A-M, Egan JJ (2012). Non-pharmacological treatment of idiopathic pulmonary fibrosis. Current Respiratory Care Reports, 1(4), 208-215.
Russell A-M, Molyneaux PL, Adamali HI, Fraser UH, Lukey PT, Wells A, Maher T (2012). Symptom Related Quality of Life Measures in Patients with Idiopathic Pulmonary Fibrosis. American Thoracic Society 2012 International Conference, May 18-23, 2012 • San Francisco, California.
Adamali HI, Delgado CM, Stock C, Lindhal GE, Molyneaux P, Russell A-M, Wells A, Renzoni EA, Maher T (2012). Telomere (TL) shortening is associated with disease severity in scleroderma (SSC) associated interstitial lung disease (ILD).
Author URL.
Adamali HI, Stock C, Lindahl GE, Leoni-Garcia P, Russell A-M, Molyneaux PL, Wells A, Renzoni E, Maher T (2012). The Effect of Telomere Length in Idiopathic Pulmonary Fibrosis. C109. EFFECTS OF SENESCENCE AND MANIFESTATIONS OF AGING IN THE LUNG.
Saini G, Jenkins G, Mckeever T, Simpson J, Hubbard R, Johnson S, Braybrooke R, Russell A-M, Meakin G, Sweeney L, et al (2012). The PROFILE Study: a Prospective Study of Fibrosis in Lung Endpoints to Discover and Qualify Biomarkers for Use in Clinical Trials. C103. PATHOGENESIS, BIOMARKERS, AND RISK FACTORS FOR INTERSTITIAL LUNG DISEASE: FROM BENCH TO BEDSIDE.
Molyneaux PL, Russell AM, Cox MJ, Moffatt MF, Cookson WO, Maher TM (2012). The Respiratory Microbiome in Idiopathic Pulmonary Fibrosis. C103. PATHOGENESIS, BIOMARKERS, AND RISK FACTORS FOR INTERSTITIAL LUNG DISEASE: FROM BENCH TO BEDSIDE.
2011
Yorke J, Swigris J, Russell A-M, Moosavi SH, Kwong GNM, Longshaw M, Jones PW (2011). Dyspnea-12 is a Valid and Reliable Measure of Breathlessness in Patients with Interstitial Lung Disease.
CHEST,
139(1), 159-164.
Author URL.
2010
Yorke J, Moosavi SH, Russell AM, Jones PW (2010). Psychological Distress is Associated with Greater Breathlessness Severity. C55. ADVANCES IN DYSPNEA MEASUREMENT.
2009
Yorke J, Moosavi SH, Russell AM, Jones PW (2009). Does Slight Variation in Situational Wording on Breathlessness Questionnaires Make a Difference?.
Author URL.
Yorke J, Moosavi SH, Russell AM, Jones PW (2009). Validity and Reliability of the Dyspnoea-12.
Author URL.
2008
Yorke J, Russell AM (2008). Interpreting the language of breathlessness.
Nursing times,
104(23), 36-39.
Abstract:
Interpreting the language of breathlessness.
Janelle Yorke and Anne-Marie Russell discuss the sensation of breathlessness and the language used by patients and healthcare professionals to describe it.
Abstract.
