Project EPIC (Empowering People to Independence in COPD): Study protocol for a hybrid effectiveness-implementation pilot randomized controlled trial of telephonic, geriatrics-palliative care nurse-coaching in older adults with COPD and their family caregivers.

BACKGROUND: EPIC (Empowering People to Independence in COPD) is a geriatric-palliative care telephonic, nurse coach intervention informed by Baltes' Theory of Successful Aging and adapted from the ENABLE (Educate, Nurture, Advise, Before Life Ends) intervention. EPIC, focused on improving independence, mobility, well-being, and COPD symptoms, has undergone formative and summative evaluation for adults with COPD. METHODS: The primary study aim is to assess the refined EPIC intervention's feasibility and acceptability via a pilot hybrid effectiveness-implementation randomized control trial in community-dwelling older adults with moderate to severe COPD and their family caregivers. The secondary aim is to explore the impact of EPIC on patient and caregiver outcomes. Older adults with COPD and their family caregivers (target N = 60 dyads) will be randomized to EPIC (intervention) or usual COPD care (control). EPIC includes six patient and four family caregiver weekly, telephone-based nurse coach sessions using a manualized curriculum (Charting Your Course), plus three monthly follow-up calls. Feasibility will be measured as completion of EPIC intervention and trial components (e.g., recruitment, retention, data collection). Acceptability will be evaluated using satisfaction surveys and post-study feedback interviews. A blinded data collector will assess exploratory outcomes (e.g., Life-Space mobility, quality of life, caregiver burden, emotional symptoms, loneliness, cognitive impairment, functional status, healthcare utilization) at baseline, 12, and 24 weeks. DISCUSSION: This intervention fills a gap in addressing the geriatrics and palliative care needs and equity for adults with COPD and their family caregivers. TRIAL REGISTRATION: ClinicalTrials.gov Identifier: NCT05040386.

Association of musculoskeletal involvement with lung function and mortality in patients with idiopathic pulmonary fibrosis.

Idiopathic pulmonary fibrosis (IPF) is a progressive disease associated with high mortality. Low muscle mass, frailty and sarcopenia lead to functional impairment that negatively impact quality of life and survival but are not used in clinical practice. We aimed to determine the association of Fat-free mass index (FFMI) and frailty with lung function, exercise tolerance and survival in patients with IPF. In this study, 70 patients with IPF underwent assessment of body composition, lung function, 6-min walk distance (6MWD) testing, hand grip strength, quality of life (QoL) assessment by St. George's Respiratory questionnaire (SGRQ) and frailty assessment using the SHARE-FI tool. FFMI was calculated using pectoralis muscle cross-sectional area (PM-CSA) on CT chest images and the lowest quartile defined reduced muscle mass. Sarcopenia was defined as low FFMI and handgrip strength. Regression analyses were conducted to determine predictive value of frailty, low FFMI and sarcopenia on clinical outcomes. The Cox proportional hazards model was used to analyze the impact of FFMI and frailty score on survival. The mean age was 70 years with moderate impairment in lung function (mean ppFVC 68.5%, ppDLCO 45.6%). Baseline forced vital capacity (p < 0.001), diffusion capacity of lung for carbon monoxide (p = < 0.01), 6WMD (p < 0.05) were significantly lower in frail patients compared to non-frail patients. BMI was found to closely correlate with FFMI (r = 0.79, p < 0.001), but not with frailty score (r = - 0.2, p = 0.07). Frailty was a significant predictor of FVC, DLCO, 6MWD, SGRQ scores when adjusted for age and gender. Muscle mass and sarcopenia were significant predictors of FVC, DLCO, but not 6MWD or QoL scores. Multivariate cox-proportional hazards ratio model adjusting for age and gender showed that frailty was significantly associated with increased mortality (HR = 2.6, 95% CI 1.1-6.1). Low FFMI (HR = 1.3, 95% CI 0.6-2.8), and sarcopenia (HR = 2.1, 95% CI 0.8-5.3), though associated with a trend to increased mortality, were not statistically significant. Frailty is associated with lower lung function and higher mortality in patients with IPF. Longitudinal evaluations are necessary to further determine the associations between low FFMI, sarcopenia and frailty with outcomes in IPF.

Project EPIC (Early Palliative Care In COPD): A Formative and Summative Evaluation of the EPIC Telehealth Intervention.

CONTEXT: Early, concurrent palliative care interventions in chronic obstructive pulmonary disease (COPD) are limited. Project EPIC (Early Palliative Care In COPD) is a multiphase mixed methods study working to fill this gap. OBJECTIVES: To conduct a formative and summative evaluation of EPIC, a telephonic nurse coach-led early palliative care intervention for COPD adapted from the ENABLE© intervention in cancer. METHODS: Phase I Formative Evaluation: Patients with moderate-to-very-severe COPD, family caregivers, and pulmonary and palliative care clinicians rated the acceptability and feasibility of EPIC (≥4 out of five on a Likert-scale survey). Phase II Summative Evaluation: Patients and family caregivers in Phase I participated in a pilot of the three month EPIC prototype to evaluate intervention and data collection feasibility (≥70% completion) and to seek qualitative feedback. RESULTS: Phase I Formative Evaluation: Patients (n=10), family caregivers (n=10), pulmonary clinicians (n=6), and palliative care clinicians (n=6) found EPIC acceptable and feasible to support adaptation, while priority early palliative care needs in COPD from our prior research mapped well to the EPIC prototype. Phase II Summative Evaluation: Patients (n=5; ages 49-72, 40% moderate COPD, 40% Black) and their family caregivers (n=5; ages 51-73, 40% Black) completed 100% of EPIC prototype components, including weekly telephone sessions, a one month follow-up call, Advance Directive, palliative care clinic attendance, and 95% of monthly phone data collection sessions. Feedback from participants about EPIC was all positive. CONCLUSION: EPIC was acceptable and feasible in patients with COPD and their family caregivers. Larger feasibility and effectiveness trials are warranted.

Provider Perspectives on and Access to Palliative Care for Patients With Interstitial Lung Disease.

BACKGROUND: Interstitial lung disease (ILD) results in profound symptom burden and carries high mortality. Palliative care (PC) is dedicated to improving quality of life in patients with serious illness. Early PC provision improves rates of advance care planning and symptom management in patients with ILD. RESEARCH QUESTION: What are the current perspectives on PC among ILD providers, and what are the barriers to PC in ILD specialty centers? STUDY DESIGN AND METHODS: A 24-question electronic survey was disseminated to providers at the 68 Pulmonary Fibrosis Foundation Care Centers across the United States from October 2020 to December 2020. RESULTS: The survey was completed by 128 participants representing all 68 Pulmonary Fibrosis Foundation Care Center Network sites. Most respondents were physicians. Most providers exhibit good knowledge of, feel comfortable assessing a patient's readiness for, and agree with the need for PC for patients with ILD. Providers are most likely to refer to PC at objective disease and/or symptomatic progression rather than at initial diagnosis. In comparison with providers who report referring their patients to PC, providers who report rare referral are more likely to cite lack local PC availability (P < .01) and less likely to feel comfortable discussing prognosis/disease trajectory (P = .03) or feel it is important to address advance directives in ILD clinic (P = .02). There is a lack of standardized measures used to assess specific symptoms, overall symptom burden, or health-related quality of life across institutions. Discordance exists between self-reported and actual access to local inpatient and outpatient PC services. INTERPRETATION: Most ILD providers use PC and are comfortable discussing PC. Barriers to PC identified in this survey include the following: perceived lack of local access to PC, lack of systematic tools to assess symptom burden, lack of established optimal timing of PC referral, and unclear need for specialized PC delivery.

Top Ten Tips Palliative Care Clinicians Should Know About Prognostication in Oncology, Dementia, Frailty, and Pulmonary Diseases.

Prognostication has been described as "Medicine's Lost Art." Taken with diagnosis and treatment, prognostication is the third leg on which medical care rests. As research leads to additional beneficial treatments for vexing conditions like cancer, dementia, and lung disease, prognostication becomes even more difficult. This article, written by a group of palliative care clinicians with backgrounds in geriatrics, pulmonology, and oncology, aims to offer a useful framework for consideration of prognosis in these conditions. This article will serve as the first in a three-part series on prognostication in adults and children.

Management of repository corticotrophin injection therapy for pulmonary sarcoidosis: a Delphi study.

In patients treated with repository corticotrophin injection (RCI) for pulmonary sarcoidosis, effective management of adverse events may improve adherence. However, management of adverse events may be challenging due to limitations in real-world clinical experience with RCI and available published guidelines.We surveyed 12 physicians with a modified Delphi process using three questionnaires. Questionnaire 1 consisted of open-ended questions. Panellists' answers were developed into a series of statements for Questionnaires 2 and 3. In these, physicians rated their agreement with the statements using a Likert scale.Key consensus recommendations included a starting dose of 40 units twice a week for patients with less severe disease, continued at a maintenance dose for patients who responded, particularly those with chronic refractory sarcoidosis. Panellists reached consensus that concomitant steroids should be quickly tapered in patients receiving RCI, but that concomitant use of immunosuppressive medications should be continued. Panellists developed consensus recommendations for adverse event management, and reached consensus that RCI should be down-titrated or discontinued if other interventions for the adverse effects fail or if the adverse effect is severe.In the absence of clinical evidence, our Delphi consensus opinions may provide practical guidance to physicians on the management of RCI to treat pulmonary sarcoidosis.

A Qualitative Study of Pulmonary and Palliative Care Clinician Perspectives on Early Palliative Care in Chronic Obstructive Pulmonary Disease.

Background: Guidelines recommend that pulmonary clinicians involve palliative care in chronic obstructive pulmonary disease (COPD); however, integration before advanced stage, that is, early palliative care, is rare. Objective: To explore and compare pulmonary and palliative care clinician perspectives on barriers, facilitators, and potential referral criteria for early palliative care in COPD. Design: Qualitative descriptive formative evaluation study. Setting/Subjects: Pulmonary and palliative care clinicians at a tertiary academic medical center. Measurements: Transcribed interviews were thematically analyzed by specialty to identify within- and across-specialty perspectives on barriers, facilitators, and referral criteria. Results: Twelve clinicians (n = 6 pulmonary, n = 6 palliative care) participated. Clinicians from both specialties agreed that early palliative care could add value to disease-focused COPD care. Perspectives on many barriers and facilitators were shared between specialties along broad educational, clinical, and operational categories. Pulmonary and palliative care clinicians shared concerns about the misconception that palliative care was synonymous to end-of-life care. Pulmonologists were particularly concerned about the potential risks of opioids and benzodiazepines in COPD. Both specialties stressed the need for clearly defined roles, consensus referral criteria, and novel delivery models. Although no single referral criterion was discussed by all, frequent hospitalizations and emotional symptoms were raised by most across disciplines. Multimorbidity and poor prognosis were discussed only by palliative care clinicians, whereas medication adherence was discussed only by pulmonary clinicians. Conclusions: Pulmonary and palliative care clinicians supported early palliative care in COPD. Continued needs include addressing pulmonologists' misconceptions of palliative care, establishing consensus referral criteria, and implementing novel early palliative care models.

Clinical features of sarcoidosis associated pulmonary hypertension: Results of a multi-national registry.

BACKGROUND: Pulmonary hypertension (PH) is a significant cause of morbidity and mortality in sarcoidosis. We established a multi-national registry of sarcoidosis associated PH (SAPH) patients. METHODS: Sarcoidosis patients with PH confirmed by right heart catheterization (RHC) were studied. Patients with pulmonary artery wedge pressure (PAWP) of 15 mmHg or less and a mean pulmonary artery pressure (mPAP) ≥ 25 Hg were subsequently analyzed. Data collected included hemodynamics, forced vital capacity (FVC), diffusion capacity of carbon monoxide (DLCO), chest x-ray, and 6-min walk distance (6MWD). RESULTS: A total of 176 patients were analyzed. This included 84 (48%) cases identified within a year of entry into the registry and 94 (53%) with moderate to severe PH. There was a significant correlation between DLCO percent predicted (% pred) andmPAP (Rho = -0.228, p = 0.0068) and pulmonary vascular resistance (PVR) (Rho = -0.362, p < 0.0001). PVR was significantly higher in stage 4 disease than in stage 0 or 1 disease (p < 0.05 for both comparisons). About two-thirds of the SAPH patients came from the United States (US). There was a significant difference in the rate of treatment between US (67.5%) versus non-US (86%) (Chi Square 11.26, p = 0.0008) sites. CONCLUSIONS: The clinical features of SAPH were similar across multiple centers in the US, Europe, and the Middle East. The severity of SAPH was related to reduced DLCO. There were treatment differences between the US and non-US centers.

A retrospective pilot study examining the use of Acthar gel in sarcoidosis patients.

INTRODUCTION: Acthar was reported as effective for the treatment of pulmonary sarcoidosis in the 1950s. Use of drug waned due to cost and toxicity compared to prednisone. Recent interest has reemerged as an alternative to high dose oral glucocorticoids. METHODS: Chart review was performed on all advanced sarcoidosis patients seen at two centers who received at least one dose of Acthar gel therapy with at least six months of posttreatment follow up. In all cases prior sarcoidosis therapy and indications for use along with clinical outcome were noted. All patients initially received 80 IU intramuscular or subcutaneous administration twice a week. RESULTS: A total of 47 patients were treated with Acthar gel therapy during the study period, and 18 (37%) discontinued drug within six months due to cost (four patients), death (two patients), or drug toxicity (eleven patients), or noncompliance (1 patient). Of the remaining 29 patients, eleven experienced objective improvement in one or more affected organs. All but two patients noted disease improvement or oral glucocorticoid reduction. Twenty-one patients were treated for more than six months (Median 274 days). Nineteen patients were on prednisone at time of starting Acthar gel: seventeen had their prednisone dosage reduced by more than fifty percent and one patient discontinued cyclophosphamide therapy. CONCLUSION: In this group of advanced sarcoidosis patients, Acthar gel treatment for at least three months was associated with objective improvement in a third of patients. A third of patients were unable to take at least a three months of treatment.