Meena Kalluri

Extract In fibrotic interstitial lung disease (F-ILD), characterized by progressive scarring, breathlessness is a defining symptom contributing to hospitalizations, mortality, and poor quality of life (QoL). [1, 2] Patients with F-ILD experience distress from breathlessness, while caregivers report feeling overwhelmed, anxious and depressed due to lack of support [3–5]. Without preparation to manage breathlessness at home, patients often seek emergency care, leading to respiratory hospitalizations and escalating costs [6]. Median survival post hospitalizations are 2.8 months, compared to 27.7 months for non-respiratory hospitalizations [7], highlighting need for dedicated dyspnoea care. Despite guidelines that recommend symptom management and quality of life measures with disease-modifying therapy [8, 9], breathlessness remains an unmet need. Shareable abstract Themes of invisibility and visibility; complexity of assessment; and prioritizing breathlessness in fibrotic interstitial lung disease revealed knowledge gaps, need for activity-based assessment, and experiential learning for clinicians.

In this perspective, we argue that healthcare systems around the world are in a state of crisis, partly due to the loss of humanity. We trace this in part to the new public management systems that were implemented to increase efficiency and have had a counterproductive effect. We argue that these processes have contributed to the rising trend of burnout and moral injury that threaten healthcare workforces across the world. A multifaceted approach is required to address this complex issue. We believe that one of the key solutions is the transformation of the practice of medicine to facilitate a meaningful, valuable, person, and relationship-centered approach, caring for "this patient" instead of "patients like this." Establishing trusting relationships is important not only for care and decision-making but also for mutual fulfilment in a doctor-patient relationship. The sense of meaning and fulfilment builds resilience and may prevent moral injury that often leads to burnout. This cannot be accomplished without the practice of compassion in care. Therefore, creation of a compassionate physician workforce should be a top priority to address this issue. We advocate to include compassion as a required competence and as the "first physician role" in the medical education framework. We discuss the evidence to support these ideas and provide examples of such teaching aids. The transformation of medical education and care will require tectonic shifts in thinking, attitudes, and acceptance at both the personal and organizational levels. Through this perspective, we hope to share our vision, seek valuable feedback, and possibly inspire action on an issue that affects us all.

Rationale: The epidemiology of adult interstitial lung disease (ILD) is uncertain, given heterogeneous estimates from prior studies. Objective: We sought to define the incidence, prevalence, and mortality of ILD over a 10-year period using population-based data. Methods: We created an administrative ILD cohort in Alberta, Canada between 2010 and 2019 using population-based administrative data (inpatient, ambulatory, and outpatient physician billing databases) for a repeat cross-sectional study. Case definitions were developed from an established ILD cohort and applied to the general population, with performance characteristics tested using a nested case-control design. Age- and sex-standardized annual incidence and point prevalence rates were estimated for ILD overall and within diagnostic subgroups, with trends over time, per 100,000 at-risk adults and to permit comparisons with other studies, per 100,000 total population. Cox models estimated risk of death or lung transplantation. Results: Between 2010 and 2019, 31,492 incident and 42,549 prevalent adult ILD cases were identified. The case definition for ILD performed well with 96.8% sensitivity, 98.5% specificity, and a positive predictive value of 94.3% in the population cohort. Mean age-standardized ILD incidence was 107.9/100,000 at-risk adults, 90.9/100,000 for females and 129.1/100,000 for males. Age-standardized ILD point prevalence increased from 416.5/100,000 at-risk adults in 2010 to 789.7/100,000 in 2019, higher in males versus females, and in rural versus urban areas. Age-standardized mean idiopathic pulmonary fibrosis (IPF) incidence was 36.9/100,000 at-risk adults, and point prevalence was 205.3/100,000 at-risk adults in 2019. Mean age-standardized ILD incidence and prevalence was 84 and 516.9/100,000 total population, respectively. One-year all-cause mortality for ILD patients decreased from 14.5% in 2011 to 11.7% in 2018 (for 2018 vs. 2011, adjusted rate ratio = 0.76; 95% confidence interval = 0.67–0.86). One-year all-cause mortality for IPF similarly decreased from 20.9% in 2011 to 14.7% in 2018 (for 2018 vs. 2011, adjusted rate ratio = 0.71; 95% confidence interval = 0.59–0.85), representing improved survival. Conclusions: In this population-based cohort, claims-based case definitions derived from an established ILD cohort performed well to develop an administrative cohort. Incidence remained stable over time, whereas prevalence increased and mortality decreased, for ILD overall and within the IPF subgroup. These estimates are higher than most prior reports, suggesting an overall underestimate of ILD burden.

Background: Breathlessness is a distressing and prevalent symptom in fibrotic interstitial lung disease. Dyspnea management requires systematic assessment including patients’ lived experiences; however, most dyspnea tools are point-in-time numerical severity scales. The Edmonton Dyspnea Inventory was developed to assess severity at rest, during activities of daily living and self-reported activities. It enables documentation of crisis dyspnea episodes and triggers clinicians to guide action plans and dyspnea management. This study is part of a larger project to validate the tool. The purpose was to describe patient perceptions of assessment of breathlessness of patient use of the tool. Methods: Patients with fibrotic interstitial lung disease were invited to share their perceptions and experiences of breathlessness and the tool. Focus groups were led on Zoom©, with patient-participants in their homes. Data were analysed with inductive content analysis for development of themes. Results: Thirteen patients participated in 2 focus groups. There were 4 major themes, each with minor themes: physicians need to explicitly ask about breathlessness; the tool conveys breathlessness and disease progression; the tool increases self-awareness of breathlessness and complexity; and the tool helps prevent crises and manage breathlessness. Patient-participants perceived the tool provided the needed language and means to focus and relay their breathlessness to others. Conclusion: Patient-participants reported the tool was easy to understand and integrate in daily living. They recommended its use for general and specialized practitioners. Developed to assess breathlessness, the tool may provide a framework to promote patient self-awareness, describe individual progression, and tailor breathlessness self-management.

Background: Dyspnea is a prevalent and distressing symptom in interstitial lung diseases with significant effects on patients’ quality of life and associated with poorer prognosis. Guidelines recommend a multidimensional dyspnea assessment tool. We developed a validated 9-item scale, the Edmonton Dyspnea Inventory (EDI), in which dyspnea severity is rated across different settings including at rest, during activities of daily living, and self-reported exercise and crises. The standardized, multidimensional tool captures dyspnea intensity for specific contexts, which clinicians can use to manage dyspnea more individually and effectively. Early studies support the feasibility to use the EDI in outpatient settings. The purpose of this study was to explore perceptions of the EDI by community health care professionals. Methods: We conducted a qualitative study using an inductive approach and open coding for content analysis. Email invitations were sent to community health care professionals and informed consent obtained from the twelve participants. Two focus groups and one key informant interview were conducted. Themes were extracted from transcripts and field note analyses. Results: Four main themes described their dyspnea assessment with the EDI: the EDI is a meaningful clinical assessment tool; they explicitly engage and educate patients to effectively use the EDI; they use the EDI to personalize and evaluate dyspnea management; and the EDI is valuable for communication and interprofessional collaboration. Conclusion: Community health care professionals perceived the EDI as valuable to assess dyspnea and personalize management. They recommended it be used in clinical practice and healthcare education for interprofessional dyspnea management for ILD patients.

Background Interstitial lung disease (ILD) is comprised of a heterogeneous group of pulmonary diseases. Oxygen therapy is used in patients with advanced lung disease; however, there are challenges associated with initiation of oxygen therapy specific to individuals with ILD. The key objectives of this study were to create a common understanding of the facilitators and barriers to oxygen therapy for patients with ILD, and healthcare professionals (HCP) caring for patients with ILD. Methods This qualitative study included 1 hour semistructured focus groups/interviews. An iterative and concurrent process was used for data collection and analysis to allow for supplementary development of themes and concepts generated. Data analysis used a three-phase approach: coding, categorising and development of themes. Results A total of 20 patients and/or caregivers and 31 HCP took part in 34 focus groups/interviews held over 3 months (November 2022–January 2023). Facilitators to oxygen therapy were identified including support from HCP and support groups, the perseverance and self-advocacy of patients, a straightforward administrative process and vendors/private industry that expedite access to oxygen therapy. There were also several barriers to accessing oxygen therapy for patients with ILD. The themes identified include rural disparity, testing requirements and qualifying for funding and the need for ILD-specific evidence base for oxygen therapy. Conclusion Further research is needed to facilitate development of specific exertional oxygen criteria for patients with ILD, to create supports for oxygen use and monitoring and to enable providers to tailor therapy to patients. Oxygen therapy education for ILD should address the benefits and risks of oxygen therapy.

Shareable abstract Clinical dyspnoea trials often recruit patients based on MRC or modified MRC grades without recognising the inherent heterogeneity in dyspnoea severity perception among patients within the same dyspnoea grade. This has implications for trial design.https://bit.ly/3PADBc3

Purpose of review: Updated clinical practice guidelines for pulmonary fibrosis recommend early integrated palliative care using a primary palliative approach. Clinicians need to be aware of the various palliative interventions in order to implement guidelines’ recommendations. This review provides an update on evidence-based palliative therapies. Recent findings: Literature review indicates early integration of palliative care in pulmonary fibrosis is feasible and meets patient needs. Key components of a primary palliative approach include comprehensive symptoms and needs screening, systematic symptom management using nonpharmacologic interventions, supplemental oxygen and opioids for dyspnea and cough. Patient-centered communication is essential for successful integration of palliative care. Early, iterative advance care planning in clinic to understand patient goals, values and preferences for current and future care, improves patient care and satisfaction. Prioritizing caregiver inclusion in clinics can address their needs. Collaborating with a multidisciplinary allied team facilitates integration of palliative care and supports patients throughout the disease course. Different models of palliative care delivery exist and can be adapted for local use. The use of artificial intelligence algorithms and tools may improve care and continuity. Summary: Clinicians must develop competency in palliative care. Organizational and policy support is essential to enable seamless integration of palliative care throughout the care continuum.

Chronic breathlessness (CB) or dyspnea is prevalent in fibrotic interstitial lung diseases (F-ILD). It is the main driver of a poor health-related quality of life (HRQOL). Timely and accurate assessment and management of CB are paramount in F-ILD care. This is reflected in latest American and European guidelines that recommend early integration of symptom-targeted therapies. Despite calls for improved CB care, evidence indicates that it remains under recognized and under treated. This narrative review focuses on the current evidence for CB assessment and management in F-ILD and proposes an algorithm for patient-centered management of CB in an outpatient setting. An overview of CB assessment tools is provided along with recommendations from guidelines and experts. The limited evidence base for CB interventions in ILD is reviewed; existing dyspnea guidelines recommend a hierarchical approach to therapies starting with the implementation of nonpharmacologic interventions (NPI). Pulmonary rehabilitation is the most common NPI in F-ILD, that improves function, dyspnea, and HRQOL. Oxygen can be prescribed to treat CB associated with exertional hypoxemia early in the course of F-ILD, with evidence suggesting short-term improvements in CB and HRQOL. For patients with severe, persistent CB despite optimization of NPI and oxygen, opioids can be prescribed, initially as short-acting, low-dose oral morphine with prophylactic doses for exertion and as needed for crises. Self-management education and written action plans may help improve patient confidence and control. Development of competency in symptom management and fostering a professional and institutional culture prioritizing CB will advance patient care and should be a priority for F-ILD patients.