Interstitial pneumonia with autoimmune features (IPAF) is a research classification proposed by the European Respiratory Society/American Thoracic Society Task Force on Undifferentiated Forms of ...Connective Tissue Disease-associated Interstitial Lung Disease as an initial step to uniformly define, identify, and study patients with interstitial lung disease (ILD) who have features of autoimmunity, yet fall short of a characterizable connective tissue disease. Since its publication in July 2015, there has been substantial interest in IPAF. Centers from around the world have published their findings of retrospectively identified cohorts of patients who fulfill IPAF criteria, suggestions for modification of the criteria have been offered, and patients who fulfill IPAF criteria are being included as a subset in the ongoing phase II multicenter unclassifiable ILD treatment trial with pirfenidone. The IPAF designation represents an important first step toward studying and furthering our understanding of the natural history of this cohort of patients with ILD using uniform nomenclature and a standardized set of criteria. Prospective evaluations and, ideally, interdisciplinary and multicenter collaborations will inform best practices for treatment and management and will guide future refinement to the IPAF criteria. This review focuses on the relevant background that led to the development of IPAF, summarizes the proposed criteria, discusses cohort studies of patients with IPAF published to date and what they have taught us about the IPAF phenotype, and offers insights into future directions in this arena. Clinical trial registered with www.clinicaltrials.gov (NCT03099187).
Evidence-based guidelines are needed for effective delivery of home oxygen therapy to appropriate patients with chronic obstructive pulmonary disease (COPD) and interstitial lung disease (ILD).
The ...multidisciplinary panel created six research questions using a modified Delphi approach. A systematic review of the literature was completed, and the Grading of Recommendations Assessment, Development and Evaluation approach was used to formulate clinical recommendations.
The panel found varying quality and availability of evidence and made the following judgments:
) strong recommendations for long-term oxygen use in patients with COPD (moderate-quality evidence) or ILD (low-quality evidence) with severe chronic resting hypoxemia,
) a conditional recommendation against long-term oxygen use in patients with COPD with moderate chronic resting hypoxemia,
) conditional recommendations for ambulatory oxygen use in patients with COPD (moderate-quality evidence) or ILD (low-quality evidence) with severe exertional hypoxemia,
) a conditional recommendation for ambulatory liquid-oxygen use in patients who are mobile outside the home and require >3 L/min of continuous-flow oxygen during exertion (very-low-quality evidence), and
) a recommendation that patients and their caregivers receive education on oxygen equipment and safety (best-practice statement).
These guidelines provide the basis for evidence-based use of home oxygen therapy in adults with COPD or ILD but also highlight the need for additional research to guide clinical practice.
Primary palliative care is needed to meet the complex needs of patients with serious illness and their families. However, patient perceptions of primary palliative care are not well understood and ...can inform subsequent primary palliative care interventions and implementation.
Elicit the patient perspective on a primary palliative care intervention, Collaborative Care to Alleviate Symptoms and Adjust to Illness (CASA), from patient perspectives.
Qualitative study using patient interviews and two methods of triangulation.
Between July 2014 and September 2015, we interviewed 45 patients who participated in the intervention in a Veterans Affairs (VA) (primary site), academic, and urban safety-net health system in the United States.
Participants were asked about what was most and least helpful, how the intervention affected participants' lives, and what should be changed about the intervention. Data were analyzed using a general inductive approach. To enhance validity of the results, we triangulated the findings from patient interviews, reviews of care coordinator documentation, and interprofessional palliative care providers.
The six themes identified that primary care intervention: (1) Cared for My Psychosocial Needs, (2) Encouraged Self-Management, (3) Medication Recommendations Worked, (4) Facilitated Goal Attainment, (5) Team was Beneficial, and (6) Good Visit Timing.
Participants experienced benefits from the primary palliative care intervention and attributed these benefits to individualized assessment and support, facilitation of skill building and self-management, and oversight from an interprofessional care team. Future primary palliative care interventions may benefit from targeting these specific patient-valued processes.
Patients with idiopathic pulmonary fibrosis, an incurable, progressive fibrotic interstitial lung disease, suffer an impaired quality of life due to symptoms, resultant functional limitations, and ...the constraints of supplemental oxygen. Two antifibrotic medications, nintedanib and pirfenidone, are approved for the treatment of idiopathic pulmonary fibrosis. Both medications slow the rate of decline of lung function, but their effect on patient-reported outcomes is not yet fully understood. Nintedanib may slow the decline in health-related quality of life for treated patients. Pirfenidone may slow the progression of dyspnea and improve cough. Patients and providers should participate in shared decision-making when starting antifibrotic therapy, taking into consideration the benefits of treatment in addition to drug-related side effects and dosing schedules. Although antifibrotic therapy may have an impact on health-related quality of life, providers should also focus on comprehensive care of the patient to improve health-related outcomes. This includes a multidisciplinary evaluation, diagnosis and treatment of comorbid medical conditions, and referral to and participation in a pulmonary rehabilitation program.
Right ventricular (RV) failure is a major cause of morbidity and mortality in patients with hypoplastic left heart syndrome (HLHS), but the longitudinal course of RV volumes through staged palliation ...(SP) has not been previously investigated. The aim of this study was to evaluate RV volume and function longitudinally through SP of HLHS using real-time three-dimensional echocardiography.
A total of 18 subjects with HLHS were prospectively studied at four time points from diagnosis through stage 2 (SP2). Real-time three-dimensional echocardiographic full-volume data sets were acquired in high-frame rate mode with electrocardiographic gating. Volumetric and functional analyses were performed using a semiautomatic contour detection algorithm. Eighteen age-matched and sex-matched normal infants (aged 0-6 months) were studied at comparable time points as controls.
Presurgical examinations (pre-stage 1 SP1; n = 18) were performed at a mean age of 4 days, post-SP1 examinations (n = 17) at a mean age of 20 days, pre-SP2 examinations (n = 14) at a mean age of 4.6 months, and post-SP2 examinations (n = 14) at a mean age of 5.5 months, constituting a total of 63 examinations. The mean values of RV end-diastolic volume indexed to body surface area (EDVi) at the four time points were 87 ± 30, 104 ± 39, 112 ± 34, and 102 ± 35 mL/m(2), respectively. There was an increase in EDVi (P = .024) from pre-SP1 to post-SP1 but no significant change between post-SP1 and pre-SP2. The decrease in EDVi after SP2 did not reach statistical significance. Mean RV ejection fractions (EFs) were 50 ± 5%, 45 ± 5%, 46 ± 5%, and 38 ± 4%, respectively. There was a trend toward decreasing EF throughout SP, with statistically significant decreases from pre-SP1 to post-SP1 (P = .003) and from pre-SP2 to post-SP2 (P < .001). In normal infants, the mean RV EDVi was 50 ± 10 mL/m(2) (approximately half that of patients with HLHS), and the mean EF was 51 ± 3%. There was good interobserver agreement for EDVi, end-systolic volume indexed to body surface area, and EF.
Real-time three-dimensional echocardiography is a reproducible means for evaluating RV volumes and EFs in patients with HLHS. Indexed RV diastolic volume remains stable to slightly increased, and RV EF deteriorates as the first two stages of surgical palliation are accomplished. The findings of this study highlight the adverse physiology of HLHS, which deteriorates even among early survivors despite SP.
Patients prescribed supplemental oxygen (O
) therapy face challenges as they adjust to being constantly "tethered" to an oxygen delivery device. Informal caregivers (ICs) of patients with pulmonary ...fibrosis (PF) face their own, often overlooked hardships when O
is brought into their home and added to their lives. Our aim was to understand the multiple effects of supplemental oxygen therapy on ICs of patients with PF.
We conducted single, semi-structured telephone interviews with twenty ICs of patients with PF who were using O
for at least 8 months. We performed a qualitative, content analysis based in grounded theory to examine data across subjects.
ICs initially reacted to O
with trepidation and sadness as they came to recognize the changes it would cause in the lives of their patient-loved one (PLO). ICs recognized both beneficial and negative effects of O
on their PLOs. ICs also realized that O
created significant changes in their own lives, including introducing new roles and responsibilities for them, altering their home environments and significantly impacting their relationships with their PLOs. Although O
was a tangible and constant reminder of disease progression, over time ICs were able to adapt and accept their new lives with O
.
ICs of patients with PF experience many life changes when their PLO is prescribed O
. Having O
prescribers anticipate and recognize these challenges provides an opportunity to give support and guidance to ICs of PF patients who require O
in the hopes of limiting the negative impact of O
on their lives.
Clinicaltrials.gov , registration number NCT01961362 . Registered 9 October 2013.
People living with chronic heart failure (CHF), chronic obstructive pulmonary disease (COPD), and interstitial lung disease (ILD) suffer impaired quality of life due to burdensome symptoms and ...depression. The Advancing Symptom Alleviation with Palliative Treatment (ADAPT) trial aims to determine the effect of a multidisciplinary, team-based intervention on quality of life in people with these common diseases.
The ADAPT trial is a two-site, patient-level randomized clinical trial that examines the effectiveness of the ADAPT intervention compared to usual care on patient-reported quality of life at 6 months in veterans with CHF, COPD or ILD with poor quality of life and increased risk for hospitalization or death. The ADAPT intervention involves a multidisciplinary team-a registered nurse, social worker, palliative care specialist, and primary care provider (with access to a pulmonologist and cardiologist)-who meet weekly to make recommendations and write orders for consideration by participants' individual primary care providers. The nurse and social worker interact with participants over six visits to identify and manage a primary bothersome symptom and complete a structured psychosocial intervention and advance care planning. The primary outcome is change in patient-reported quality of life at 6 months as measured by the Functional Assessment of Chronic Illness Therapy-General questionnaire. Secondary outcomes at 6 months include change in symptom distress, depression, anxiety, disease-specific quality of life hospitalizations, and advance care planning communication and documentation. Intervention implementation will be assessed using a mixed-methods approach including a qualitative assessment of participants' and intervention personnel experiences and a quantitative assessment of care delivery, resources, and cost.
The ADAPT trial studies an innovative intervention designed to improve quality of life for veterans with common, burdensome illnesses by targeting key underlying factors-symptoms and depression-that impair quality of life but persist despite disease-specific therapies. Leveraging the skills of affiliate health providers with physician supervision will extend the reach of palliative care and improve quality of life for those with advanced disease within routine outpatient care. The hybrid effectiveness/implementation design of the ADAPT trial will shorten the time to broader dissemination if effective and create avenues for future research.
ClinicalTrials.gov, NCT02713347 . Registered March 19, 2016.
Management of patients with interstitial lung disease (ILD) requires subspecialized, comprehensive, multidisciplinary care. The Pulmonary Fibrosis Foundation established the Care Center Network (CCN) ...in 2013 with identified criteria to become a designated CCN site. Despite these criteria, the essential components of an ILD clinic remain unknown.
How are ILD clinics within the CCN structured? What are the essential components of an ILD clinic according to ILD physician experts, patients, and caregivers?
This study had three components. First, all 68 CCN sites were surveyed to determine the characteristics of their current ILD clinics. Second, an online, three-round modified Delphi survey was conducted between October and December 2019 with 48 ILD experts participating in total. Items for round 1 were generated using expert interviews. During rounds 1 and 2, experts rated the importance of each item on a 5-point Likert scale. The a priori threshold for consensus was more than 75% of experts rating an item as important or very important. In round 3, experts graded items that met consensus and ranked items deemed essential for an ILD clinic. Third, ILD patient and caregiver focus groups were conducted and analyzed for content to determine their perspectives of an ideal ILD clinic.
Forty items across four categories (members, infrastructure, resources, and multidisciplinary conference) achieved consensus as essential to an ILD clinic. Patient and caregiver focus groups identified three major themes: comprehensive, patient-centered medical care; expanded access to care; and comprehensive support for living and coping with ILD.
The essential components of an ILD clinic are well-aligned between physician experts and patients. Future research can use these findings to evaluate the impact of these components on patient outcomes and to inform best practices for ILD clinics throughout the world.
Supplemental oxygen is prescribed to patients with pulmonary fibrosis to normalize oxygen saturations, decrease symptoms and improve quality of life. Along with potential benefits, patients face ...challenges as they incorporate oxygen into their lives.
Our aim was to better understand the perceptions and experiences of patients with pulmonary fibrosis as they confronted the possibility and realities of using supplemental oxygen.
We performed a mixed-methods study in which we conducted a series of four structured telephone interviews with five patients with pulmonary fibrosis enrolled in a longitudinal observational study. Questionnaires were administered at the time of the interviews, which were conducted at enrollment in the longitudinal study, immediately prior to starting supplemental oxygen, one month and then 9–12 months after starting oxygen. We used conventional content analysis to analyze interview data for themes within and across the four time points.
Prior to starting supplemental oxygen, participants uniformly expected it would improve their physical function and quality of life. They also expected practical and psychological limitations, which after starting oxygen, they found to be more pronounced than anticipated. Despite the challenges, participants attributed benefits in symptoms, confidence and mobility to oxygen and came to a reluctant acceptance of it. Their expectations for guidance and support were inadequately met.
For patients with pulmonary fibrosis, starting and using supplemental oxygen on an everyday basis confers benefits while also presenting a significant number of challenges. The process could be improved by providing them with clearer expectations and trustworthy educational resources. Oxygen case managers could help patients incorporate supplemental oxygen more seamlessly into their lives.
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