Management and support of patients with fibrosing interstitial lung diseases.

ABSTRACT: Fibrosing interstitial lung diseases have a variable clinical course. Regular monitoring is important to assess disease progression and inform patient care and counseling. NPs play a key role in helping patients understand their disease and its treatment and manage the adverse reactions of pharmacologic therapies.

Idiopathic pulmonary fibrosis: a more common condition than you may think.

Idiopathic pulmonary fibrosis (IPF) is a chronic, progressive incurable lung disease that affects a significant amount of people in the UK. Many health professionals have a limited understanding of IPF, which can result in a delayed diagnosis and inadequate care for individuals and their families. This article aims to provide an overview of IPF and help to enhance health professionals' understanding of the disease, thus contributing towards improving the care that IPF sufferers receive. This article provides a definition of IPF and explores its pathophysiology. It discusses the causes and risk factors for developing the condition, examines how IPF is diagnosed and details the treatment options available for IPF patients.

Idiopathic pulmonary fibrosis: What nurses need to know.

ABSTRACT: Idiopathic pulmonary fibrosis (IPF) is a restrictive lung disease in which the cause cannot be determined. This article discusses restrictive lung diseases that fall under the general category of interstitial lung disease with a focus on IPF-a fatal disease characterized by progressive fibrosis and interstitial pneumonia, dyspnea, and decreasing pulmonary function.

Assessing Patterns of Palliative Care Referral and Location of Death in Patients with Idiopathic Pulmonary Fibrosis: A Sixteen-Year Single-Center Retrospective Cohort Study.

Background: Idiopathic pulmonary fibrosis (IPF) is a progressive and fatal lung disease with an unpredictable course and a median survival of three to four years. This timeline challenges providers to approach diagnosis, oxygen therapy, rehabilitation, transplantation, and end-of-life discussions in limited encounters. There is currently no widely accepted guideline for determining when IPF patients should be referred to palliative care (PC). Objective: We sought to describe the patient and clinical factors associated with PC referral, as well as its impact on mortality and location of death. We also aimed to examine temporal trends in PC referral in this population. Materials and Methods: Patient data were retrospectively extracted from the health system repository of our specialty referral center for all new IPF patients evaluated between 2000 and 2016 (n = 828). Exclusion criteria included transplant recipients and patients who did not have IPF. Results: One hundred twelve (13.5%) IPF patients received formal PC referral. Recipients were older at diagnosis (72 years vs. 69 years, p < 0.001), had higher frequency of Charlson Comorbidity Index ≥1 (55% vs. 42%, p = 0.011), resided closer to our institution (16 miles vs. 54 miles, p < 0.001), and had a higher number of total outpatient visits (7 vs. 4, p < 0.001). PC was associated with less in-hospital death (44% vs. 60%, p = 0.006) and more in-home and hospice death (56% vs. 40%, p = 0.006). Conclusions: IPF patients referred to PC were older with more severe comorbidities, resided closer to our specialty referral center, and had more outpatient follow-up. This was associated with more in-home and hospice deaths. The patient-provider relationship and frequency of follow-up visits likely play important roles in the introduction of end-of-life discussions.

End-of-life trajectory of coping and self-care of patients with idiopathic pulmonary fibrosis: A meta-synthesis using meta-ethnography.

AIM: This study aimed to extract patient narratives regarding the end-of-life trajectory of their self-care and coping experiences as patients with idiopathic pulmonary fibrosis and to use meta-ethnography to discover common metaphors of their experience in order to inform the development of nursing interventions. METHODS: A comprehensive search of qualitative research using electronic databases, in English and Japanese, regarding patients with IPF, identified four articles. The meta-ethnography approach followed seven steps, which provided a synthesis of the phenomena from both comparative and cumulative analyses of the qualitative data. RESULTS: Seven key concepts and metaphors were synthesized: (i) an extended period before obtaining a diagnosis ("Waiting for the other shoe to drop"); (ii) suffering as the pathway of illness ("Gloom and doom"); (iii) the burden of the symptoms and loss of independence ("Staggering under the load"); (iv) acceptance and living with the restrictions of home oxygen therapy ("A double-edged sword"); (v) changes in the family role ("All in the same boat"); (vi) existential suffering ("Dead-end streets and rays of hope"); and (vii) challenges to maintain independent living ("Making the best of it"). Reciprocal, refutational, and line-of-argument modes of synthesis revealed the relationships of the metaphors. Meta-ethnography provided a mechanism for a cumulative knowledge base for necessary nursing interventions. CONCLUSIONS: The critical nursing interventions were: the relief of symptoms, particularly dyspnea and cough; coordinating lifestyle changes; providing psychological support for living with an illness beginning from the time of diagnosis; and carefully starting the end-of-life discussions with patients and families.

Barriers to specialist palliative care in interstitial lung disease: a systematic review.

BACKGROUND: Current guidelines recommend palliative care based on individual needs for patients with idiopathic pulmonary fibrosis. However, patients with interstitial lung disease (ILD) are less likely to receive specialist palliative care services compared with patients with malignant disease. The aim of this review is to summarise recent studies addressing barriers to referring patients to specialist palliative care services. METHODS: PubMed, Embase, Medline and Web of Science were reviewed to identify relevant publications. Studies were selected if they examined the frequency of specialist palliative care referral and/or addressed issues surrounding access to palliative care services for patients with ILD. RESULTS: Ten studies with a total of 4073 people with ILD, 27 caregivers and 18 healthcare professionals were selected and analysed. Frequency of palliative care referrals ranged from 0% to 38%. Delay in palliative care referrals and end-of-life decisions, patients' fear of talking about the future, prognostic uncertainty and confusion about the roles of palliative care were identified as barriers to accessing palliative care services. CONCLUSION: Further research should concentrate on the early identification of patients who need specialist palliative care possibly with establishment of criteria to trigger referral ensuring that referrals are also based on patient's needs.

Effectiveness of non-pharmacological nursing interventions to improve the quality of life of patients with idiopathic pulmonary fibrosis: A systematic review.

AIM: To evaluate whether non-pharmacological nursing interventions improve the quality of life, including both physical and psychosocial states, for patients with idiopathic pulmonary fibrosis, compared to patients receiving the usual care visit or not receiving routine nursing. METHODS: A comprehensive search was conducted of the following electronic databases in English and Japanese: PubMed, MEDLINE, EMBASE, CINAHL, The Cochrane Central Register Controlled Trials (CENTRAL), NPO Japan Medical Abstracts Society's Ichushi - Web database, National Institute of Informatics' Scholarly and Academic Information Navigator (CiNii) database, Grants-in-Aid for Scientific Research (KAKEN) database, and Database of Health Labor Sciences Research Grants. The keywords were as follows: [interstitial lung disease], [interstitial pulmonary fibrosis], [idiopathic pulmonary fibrosis] AND [nurse OR nurses' role OR nursing]. A search method was used based on the eligibility criteria without including words, such as "randomized controlled trial" (RCT). Three categories defined the search parameters: (i) patients with idiopathic pulmonary fibrosis; (ii) non-pharmacological nursing interventions; and (iii) RCT. RESULTS: Three-hundred-and-ninety-four articles were reviewed. Two articles met all the eligibility criteria. The nursing interventions were "disease management program" and "community case conference," both involving nurse specialists. The quality-of-life scores decreased after 6 weeks of intervention with the disease management program. The quality-of-life scores improved after 4 weeks of intervention with the community case conferences. The risk of bias for these two studies was high. CONCLUSION: There was limited evidence of non-pharmacological nursing interventions improving the quality of life of patients with idiopathic pulmonary fibrosis.

Atención enfermera a pacientes con fibrosis pulmonar idiopática en fase avanzada / Nursing care for patients with advanced idiopathic pulmonary fibrosis

OBJETIVOS: describir las características de pacientes con fibrosis pulmonar idiopática (FPI) en fase avanzada y analizar los principales diagnósticos de Enfermería desde la experiencia de un Equipo de Soporte Paliativo Domiciliario (ESAPD).MÉTODO: estudio descriptivo transversal de pacientes con FPI atendidos por un ESAPD de Madrid, de 2011 a 2015. Se analizó: edad, sexo, situación funcional (índice de Barthel y la escala Palliative Performance Scale), número de fármacos, oxigenoterapia, aspectos sociales, días de seguimiento, número de visitas y lugar de éxitus. Se analizaron los diagnósticos de Enfermería según taxonomía NANDA. RESULTADOS: se evaluaron un total de 1.920 pacientes. 18(0,93%) fueron incluidos por presentar FPI. La edad media era 75,5años, un 55,6% eran hombres. Tenían un índice de Barthel de 50, un Palliative Performance Scale de 50, consumían una media de10 fármacos y todos utilizaron opioides para la disnea. Un 94,4%utilizaba oxigenoterapia a alto flujo. En un 55,5% el cuidador principal eran los hijos. Los diagnósticos de Enfermería "Patrón respiratorio ineficaz", "Riesgo de infección" y "Déficit de autocuidado: baño" estaban presentes en todos los pacientes. La mediana de días de seguimiento fueron 31 días; un 55,6% fue derivado en el último mes de vida. Al finalizar el estudio todos los pacientes habían fallecido, siendo el domicilio el lugar de preferencia para el éxitus (61,1%).CONCLUSIONES: los pacientes con FPI avanzada presentan disnea, deterioro funcional moderado y necesidad de ayuda en los autocuidados. La derivación a ESAPD fue tardía. Más de la mitad de los pacientes fallecieron en el domicilio

OBJECTIVES: to describe the characteristics of patients with Idiopathic Pulmonary Fibrosis (IPF) in advanced stage, and to analyze the main Nursing diagnoses based on the experience of a Team for Palliative Care at Home (TPCH).METHOD: a transversal descriptive study on IPF patients managed by a TPCH in Madrid, from 2011 to 2015. The following were analyzed: age, gender, performance status (Barthel Index and the Palliative Performance Scale), number of drugs, oxygen therapy, social aspects, follow-up days, number of visits, and place of death. The Nursing diagnoses were analyzed according to the NANDA taxonomy. RESULTS: in total, 1,920 patients were assessed; 18 of them(0.93%) were included because they presented IPF. Their mean age was 75.5 years; 55.6% were male. Their Barthel Index score was 50, and their Palliative Performance Scale score was 50. They took a mean number of 10 drugs, and they all used opioids for their dyspnea. A 94.4% of these patients were using high flow oxygen therapy. The main caregivers were patients' children, in 55.5% of cases. The following Nursing diagnoses were present in all patients: "Inefficient respiratory pattern", "Risk of infection", and "Self-care deficit: bathing". The median of follow-up days was 31;55.6% of patients were referred in their last month of life. When the study was completed, all patients had died, and their home was the preferred place for exitus (61.1%). CONCLUSIONS: patients with advanced IFP present dyspnea, moderate functional deterioration, and they require help for self care. There was a late referral to TPCH. More than half of patients died at home

Qualitative European survey of patients with idiopathic pulmonary fibrosis: patients' perspectives of the disease and treatment.

BACKGROUND: 'Living with IPF and an exploration of Esbriet® - a new treatment' was an exploratory, qualitative, real-world survey of European patients with idiopathic pulmonary fibrosis (IPF) who were receiving treatment with pirfenidone prior to its commercial availability. The aim of the survey was to probe the impact of IPF on patients' quality of life; the role of healthcare professionals and caregivers; the information needs of both patients and their caregivers; and patients' perceptions of pirfenidone as a new treatment option for IPF. METHODS: Patients from the UK, Germany and Italy, with a diagnosis of IPF (duration >3 months), who were being treated with pirfenidone, were recruited from patient support groups, specialist centres and advocacy groups. Semi-structured, qualitative, in-depth patient interviews of 1-h duration were conducted by an independent researcher. Patients were initially asked about their experiences of living with IPF and then prompted to describe their experiences of taking pirfenidone. Techniques utilised included: the bubble-speech technique; the icon cards projective exercise; and the free association exercise. All interviews were transcribed and analysed by an independent researcher. RESULTS: Forty-five patients (71% male) were interviewed (mean age 68.5 years; mean time since diagnosis 3.5 years); 87% of patients reported that diagnosis took >1 year. Patients reported that IPF had a significant physical and emotional impact on their quality of life. The beneficial role played by caregivers and interstitial lung disease specialist nurses (where available) was specifically highlighted. Although most patients were keen for information on IPF, this was often of poor quality, out of date, or in English only. Patients' perceptions of pirfenidone were largely positive and associated with 'hope' but were also influenced by the level of side effects experienced. CONCLUSIONS: This survey highlights the impact of IPF on patients' lives, and the need to adequately support both patients and their caregivers. These findings demonstrate the value of seeking patients' perspectives of a chronic disease such as IPF and how this information can be used to guide improvements in care, to best support the needs of patients with this devastating condition.

Management of idiopathic pulmonary fibrosis.

Idiopathic pulmonary fibrosis is a progressive interstitial lung disease; patients have a mean life expectancy of 2-4 years from diagnosis. This review summarises National Institute for Health and Care Excellence (2013a) guidance and identifies key priorities for patient care.

Impact of a disease-management program on symptom burden and health-related quality of life in patients with idiopathic pulmonary fibrosis and their care partners.

BACKGROUND: Patients were recruited from the Dorothy P. and Richard P. Simmons Center for Interstitial Lung Disease, located within the University of Pittsburgh Medical Center. Idiopathic pulmonary fibrosis results in scarring of the lung and respiratory failure, and has a median survival of 3 to 5 years from the time of diagnosis. The purpose of this study was to determine whether patients with idiopathic pulmonary fibrosis and their care partners could be more optimally managed by a disease-management intervention entitled "Program to Reduce Idiopathic Pulmonary Fibrosis Symptoms and Improve Management," which nurses delivered using the format of a support group. We hypothesized that participation would improve perceptions of health-related quality of life (HRQoL) and decrease symptom burden. METHODS: Subjects were 42 participants randomized to an experimental (10 patient/care partner dyads) or control (11 patient/care partner dyads) group. Experimental group participants attended the 6-week program, and controls received usual care. Before and after the program, all participants completed questionnaires designed to assess symptom burden and HRQoL. Patients and care partners in the intervention group were also interviewed in their home to elicit information on their experience after participating in the Program to Reduce Idiopathic Pulmonary Fibrosis Symptoms and Improve Management. RESULTS: After the intervention, experimental group patients rated their HRQoL less positively (P = .038) and tended to report more anxiety (P = .077) compared with controls. Care partners rated their stress at a lower level (P = .018) compared with controls. Course evaluations were uniformly positive. Post-study qualitative interviews with experimental group participants suggested benefits not exemplified by these scores. Patient participants felt less isolated, were able to put their disease into perspective, and valued participating in research and helping others. CONCLUSION: Further exploration of the impact of disease-management interventions in patients with advanced lung disease and their care partners is needed using both qualitative and quantitative methodology. Disease-management interventions have the potential to positively affect patients with advanced lung disease and their care partners.