RESUMO
INTRODUCTION: Cough is common in interstitial lung disease (ILD) and is associated with disease progression, yet its mechanisms are understudied. We investigated cough hypersensitivity features and impact in ILD. METHODS: Participants with ILD and cough (n = 195) completed a multiple choice and free text questionnaire on cough sensations/triggers and impacts. RESULTS: The majority of participants were male (54%), aged > 65 (64%), with idiopathic pulmonary fibrosis (IPF, 75%). Common cough triggers were body position (74%), physical activity (72%), and talking (62%). Common laryngeal sensations were globus (43%), and itch/tickle (42%). Cough impacted everyday life in 55%, and all activities in 31%, causing exhaustion (59%), social embarrassment (70%), urinary incontinence (46% females), and syncope/pre-syncope (12%). The total number of cough-provoking sensations/triggers correlated with impacts; ρ = 0.73, p < 0.001. CONCLUSION: Cough hypersensitivity symptoms are prevalent in ILD and detrimentally affect quality of life. Further studies investigating mechanisms of cough hypersensitivity and targeted pharmacotherapy are warranted.
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Tosse , Doenças Pulmonares Intersticiais , Qualidade de Vida , Humanos , Tosse/psicologia , Tosse/fisiopatologia , Masculino , Feminino , Idoso , Doenças Pulmonares Intersticiais/psicologia , Doenças Pulmonares Intersticiais/complicações , Doenças Pulmonares Intersticiais/fisiopatologia , Pessoa de Meia-Idade , Inquéritos e Questionários , Percepção , Fibrose Pulmonar Idiopática/complicações , Fibrose Pulmonar Idiopática/fisiopatologia , Fibrose Pulmonar Idiopática/psicologia , Síncope/fisiopatologia , Síncope/etiologia , Atividades CotidianasRESUMO
INTRODUCTION: Idiopathic pulmonary fibrosis (IPF) is a progressive disease presenting with symptoms like dyspnoea, dry cough, and fatigue, which affect physical function and quality of life. No earlier qualitative studies have investigated physical activity in IPF. This study aims to explore experiences of living with IPF in relation to physical activity. MATERIALS AND METHODS: Qualitative interviews were conducted with 14 participants living with IPF. The participants were 77 years old (range: 56-86) and diagnosed with IPF between 2 and 9 years ago. The analysis was performed by qualitative content analysis according to Graneheim and Lundman. RESULTS: The results indicated that life and one's ability to be physically active is affected by IPF. Despite this, it seems possible to navigate past obstacles, which was illustrated by an overall theme: "My life is constrained, but I am hanging on". Two major categories cover topics of IPF being a life changing diagnosis with changes in self-image and changed future plans regarding physical activity, as well as life. Physical activity was perceived to be challenging, yet in many ways used as a strategy, developed to manage life. CONCLUSIONS: IPF affects physical activity as well as life, from onset onwards. By developing strategies for facilitating physical activity as well as identifying barriers, it seems possible to maintain an active life despite the disease. The healthcare system needs to create support systems that meet different needs during different phases of the disease. TRIAL REGISTRATION: "FoU in Sweden" Research and Development in Sweden (id: 227081).
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Exercício Físico , Fibrose Pulmonar Idiopática , Pesquisa Qualitativa , Qualidade de Vida , Humanos , Fibrose Pulmonar Idiopática/psicologia , Fibrose Pulmonar Idiopática/fisiopatologia , Idoso , Masculino , Feminino , Exercício Físico/psicologia , Pessoa de Meia-Idade , Idoso de 80 Anos ou mais , Entrevistas como Assunto , AutoimagemRESUMO
OBJECTIVES: Pulmonary Rehabilitation (PR) is a high-impact intervention for individuals with idiopathic pulmonary fibrosis (IPF) but access is limited in India. PR barriers include distance to travel, lack of service provision and lack of healthcare professionals to deliver PR, thus it is disproportionate to the immense burden of IPF in India. We explored the lived experiences of people living with IPF, family caregivers (CGs) and healthcare workers (HCWs) as well as their views towards home-based PR (HBPR) in Delhi, India. METHODS: A qualitative study using semi-structured interviews with individuals with IPF (n = 20), CGs (n = 10) and HCWs (n = 10) was conducted. Data were analysed using codebook thematic analysis. RESULTS: Three major themes were generated: (i) Health impact, which included pathophysiological changes, range of symptoms experienced, disease consequences and impact of comorbidities; (ii) Disease management, which described strategies to control the progression and overall management of IPF, such as medications and exercises; (iii) Mode of Pulmonary Rehabilitation, which described perceptions regarding HBPR, comparisons with centre-based programmes, and how HBPR may fit as part of a menu of PR delivery options. CONCLUSION: People living with IPF, family caregivers and healthcare workers were positive about the potential implementation of HBPR and suggested the development of a paper-based manual to facilitate HBPR over digital/online approaches. The content of HBPR should be sensitive to the additional impact of non-IPF health issues and challenges of reduced interactions with healthcare professionals.
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Cuidadores , Serviços de Assistência Domiciliar , Fibrose Pulmonar Idiopática , Pesquisa Qualitativa , Humanos , Índia , Masculino , Fibrose Pulmonar Idiopática/reabilitação , Fibrose Pulmonar Idiopática/psicologia , Feminino , Cuidadores/psicologia , Pessoa de Meia-Idade , Idoso , Pessoal de Saúde/psicologia , Atitude do Pessoal de Saúde , Adulto , Entrevistas como AssuntoRESUMO
AIMS: Patients diagnosed with idiopathic pulmonary fibrosis (IPF) have a high symptom burden and numerous needs that remain largely unaddressed despite advances in available treatment options. There is a need to comprehensively identify patients' needs and create opportunities to address them. This scoping review aimed to synthesise the available evidence and identify gaps in the literature regarding the unmet needs of patients diagnosed with IPF. METHODS: The protocol for the review was registered with Open Science Framework (DOI 10.17605/OSF.IO/SY4KM). A systematic search was performed in March 2022, in CINAHL, MEDLINE, Embase, PsychInfo, Web of Science Core Collection and ASSIA Applied Social Science Index. A comprehensive review of grey literature was also completed. Inclusion criteria included patients diagnosed with IPF and date range 2011-2022. A range of review types were included. Data was extracted using a data extraction form. Data was analysed using descriptive and thematic analysis. A total of 884 citations were reviewed. Ethical approval was not required. RESULTS: 52 citations were selected for final inclusion. Five themes were identified: 1.) psychological impact of an IPF diagnosis. 2.) adequate information and education: at the right time and in the right way. 3.) high symptom burden support needs. 4.) referral to palliative care and advance care planning (ACP). 5.) health service provision-a systems approach. CONCLUSION: This review highlights the myriad of needs patients with IPF have and highlights the urgent need for a systems approach to care, underpinned by an appropriately resourced multi-disciplinary team. The range of needs experienced by patients with IPF are broad and varied and require a holistic approach to care including targeted research, coupled with the continuing development of patient-focused services and establishment of clinical care programmes.
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Fibrose Pulmonar Idiopática , Fibrose Pulmonar Idiopática/terapia , Fibrose Pulmonar Idiopática/diagnóstico , Fibrose Pulmonar Idiopática/psicologia , Humanos , Necessidades e Demandas de Serviços de Saúde , Avaliação das NecessidadesRESUMO
AIM: To explore the experience of people with idiopathic pulmonary fibrosis living through the COVID-19 pandemic. DESIGN: A qualitative descriptive design using semi-structured interviews. METHOD: Purposive sampling was employed to recruit 13 participants with idiopathic pulmonary fibrosis attending the respiratory department of a large urban teaching hospital in Limerick, Ireland. Data were collected between January 2021 and February 2021 through semi-structured interviews, using an online platform. Reflective thematic analysis was used for data analysis. RESULTS: Four key themes were identified from participant's experience of living through the COVID-19 pandemic: (1) fear of contracting COVID-19 disease, (2) living with reduced social interaction, (3) the adjustment in the relationship with healthcare professionals (HCP) and (4) navigating an altered landscape. CONCLUSION: Healthcare professionals have a key role in protecting the physical and psychological health of the person with idiopathic pulmonary fibrosis during this time and into the future. Through being cognisant of the additional supportive care needs of people with idiopathic pulmonary fibrosis, HCP can focus on developing targeted interventions aimed to enhance care provision. IMPACT: This study considers people with idiopathic pulmonary fibrosis as a particularly vulnerable group whose experiences of living through the COVID-19 pandemic warrant specific attention. Participants felt compelled to self-isolate due to fear and anxiety of contracting COVID-19 disease. Participants reported increased social isolation with some experiencing anger and resentment at loss of precious time with loved ones. Participants felt an increased responsibility for self-monitoring their condition and had concerns about differentiating symptoms of COVID-19 disease from an exacerbation. A variety of strategies that helped them cope through the pandemic were identified and also the important role these played. The results from this study can be used to inform HCP' understanding of challenges experienced by people with idiopathic pulmonary fibrosis during enforced restrictions related to the COVID-19 pandemic.
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COVID-19 , Fibrose Pulmonar Idiopática , COVID-19/epidemiologia , Pessoal de Saúde/psicologia , Humanos , Fibrose Pulmonar Idiopática/psicologia , Saúde Mental , Pandemias , Pesquisa QualitativaRESUMO
BACKGROUND: The multidimensional and complex care needs of patients with idiopathic pulmonary fibrosis (IPF) call for appropriate care models. This systematic review aimed to identify care models or components thereof that have been developed for patients with IPF in the outpatient clinical care, to describe their characteristics from the perspective of chronic integrated care and to describe their outcomes. METHODS: A systematic review was conducted using state-of-the-art methodology with searches in PubMed/Medline, Embase, CINAHL and Web Of Science. Researchers independently selected studies and collected data, which were described according to the Chronic Care Model (CCM). RESULTS: Eighteen articles were included describing 13 new care models or components. The most commonly described CCM elements were 'delivery system design' (77%) and 'self-management support' (69%), with emphasis on team-based and multidisciplinary care provision and education. The most frequently described outcome was health-related quality of life. CONCLUSIONS: Given the high need for integrated care and the scarcity and heterogeneity of data, developing, evaluating and implementing new models of care for patients with IPF and the comprehensive reporting of these endeavours should be a priority for research and clinical care.
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Prestação Integrada de Cuidados de Saúde/métodos , Fibrose Pulmonar Idiopática/psicologia , Fibrose Pulmonar Idiopática/terapia , Qualidade de Vida , Prestação Integrada de Cuidados de Saúde/tendências , HumanosRESUMO
INTRODUCTION: There is limited data available on the use of CPET as a predictive tool for disease outcomes in the setting of IPF. We investigated the feasibility of undertaking CPET and the relationship between CPET and quality of life measurements in a well-defined population of mild and moderate IPF patients. METHODS: A prospective, single-centre observational study. RESULTS: Thirty-two IPF patients (mild n = 23, moderate n = 9) participated in the study, n = 13 mild patients attended for repeat CPET testing at 12 months. At baseline, total K-BILD scores and total IPF-PROM scores significantly correlated with 6MWT distance, but not with baseline FVC % predicted, TLco % predicted, baseline or minimum SpO2. VO2 peak/kg at AT positively correlated with total scores, breathlessness/activity and chest domains of the K-BILD questionnaire (p < 0.05). VO2 peak significantly correlated with total IPF PROM scores and wellbeing domains (p < 0.05), with a trend towards statistical significance for total IPF-PROM and VO2 peak/kg at anaerobic threshold (p = 0.06). There was a statistically significant reduction in FVC% predicted at 12 months follow up, although the mean absolute decline was < 10% (p < 0.05). During this period VO2 peak significantly reduced (21.6 ml/kg/min ± 2.9 vs 19.1 ± 2.8; p = 0.017), with corresponding reductions in total K-BILD and breathlessness/activity domains that exceeded the MCID for responsiveness. Lower baseline VO2 peak/kg at anaerobic threshold correlated with greater declines in total K-BILD scores (r = - 0.62, 0.024) at 12 months. Whilst baseline FVC% predicted or TLco % predicted did not predict change in health status, CONCLUSION: We have shown that it is feasible to undertake CPET in patients with mild to moderate IPF. CPET measures of VO2 peak correlated with both baseline and change in K-BILD measurements at 1 year, despite relatively stable standard lung function (declines of < 10% in FVC), suggesting its potential sensitivity to detect physiological changes underlying health status.
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Fibrose Pulmonar Idiopática/diagnóstico , Diferença Mínima Clinicamente Importante , Qualidade de Vida/psicologia , Idoso , Idoso de 80 Anos ou mais , Limiar Anaeróbio , Estudos de Viabilidade , Feminino , Nível de Saúde , Humanos , Fibrose Pulmonar Idiopática/fisiopatologia , Fibrose Pulmonar Idiopática/psicologia , Fibrose Pulmonar Idiopática/terapia , Masculino , Estudos Prospectivos , Testes de Função Respiratória , Inquéritos e Questionários , Teste de Caminhada/métodos , Teste de Caminhada/psicologiaRESUMO
BACKGROUND: Idiopathic pulmonary fibrosis (IPF) highly impacts patients on several life dimensions and challenges healthcare practices in providing high-quality care. Consequently, it is crucial to establish integrated care processes, maximizing patient value and patients' individual needs. The aim of the study was to shed light on the care trajectory based on the perspectives of patients and healthcare professionals. METHODS: The study was conducted at a tertiary Belgian IPF centre of excellence. We conducted individual interviews with patients and healthcare professionals, guided by the Chronic Care Model (CCM) as a framework for integrated care. Thematic analysis was used to underpin data analysis. RESULTS: Experiences were gathered of nine patients with IPF (aged 57-83 years, of which the informal caregivers were present at five interviews) and nine professionals involved in the IPF care trajectory. Our findings identified pitfalls and suggestions for improvement covering all elements of the CCM, primarily at the level of the individual patient and the care team. We covered suggestions to improve the team-based care and pro-active follow-up of patients' needs. Self-management support was highlighted as an important area and we identified possibilities, but also challenges regarding the use of patient-reported outcomes and eHealth-tools. Furthermore, the importance of continuous training for professionals and the implementation of guidelines in routine care was pointed out. Also, participants mentioned an opportunity to collaborate with community-based organizations and raised challenges regarding the overall health system. Lastly, the pertaining lack of IPF awareness and the disease burden on patients and their caregivers were covered. CONCLUSIONS: Our research team has initiated a project aiming to optimize the current care delivery practice for IPF patients at a Belgian centre of excellence. These results will inform the further optimisation of the care program and the development of feasible supportive interventions.
Assuntos
Atitude do Pessoal de Saúde , Necessidades e Demandas de Serviços de Saúde , Fibrose Pulmonar Idiopática/terapia , Idoso , Idoso de 80 Anos ou mais , Cuidadores , Gerenciamento Clínico , Feminino , Humanos , Fibrose Pulmonar Idiopática/psicologia , Masculino , Pessoa de Meia-Idade , Papel Profissional , Pesquisa QualitativaAssuntos
COVID-19/psicologia , Pneumonias Intersticiais Idiopáticas/psicologia , Quarentena/psicologia , Idoso , COVID-19/epidemiologia , COVID-19/prevenção & controle , Estudos Transversais , Exercício Físico , Feminino , França/epidemiologia , Humanos , Pneumonias Intersticiais Idiopáticas/epidemiologia , Pneumonias Intersticiais Idiopáticas/terapia , Fibrose Pulmonar Idiopática/epidemiologia , Fibrose Pulmonar Idiopática/psicologia , Fibrose Pulmonar Idiopática/terapia , Masculino , Oxigenoterapia/estatística & dados numéricos , Distanciamento Físico , Inquéritos e QuestionáriosRESUMO
Idiopathic pulmonary fibrosis (IPF) is characterized by progressive loss of pulmonary function and exercise capacity, leading to loss of quality of life and often social isolation. A new walking aid, the walk-bike, showed an improvement in exercise performance in COPD patients. Aims of this pilot study were to evaluate feasibility of a homebased walk-bike intervention study in IPF patients and to explore the effect of the walk-bike on quality of life (QoL) and exercise capacity. Twenty-three patients with IPF were included in a randomized multicenter crossover study with 8 weeks of standard care and 8 weeks of walk-bike use at home. Ten patients completed both study phases. Study barriers included reluctance to participate and external factors (e.g. weather and road conditions) that hampered adherence. Patients' satisfaction and experience with the walk-bike varied greatly. After training with the walk-bike, health-related QoL (St. George's Respiratory and King's Brief Interstitial Lung Disease questionnaires) demonstrated a tendency towards improvement, exercise capacity did not. A clinically important difference was found between 6-minute walk test with the walk-bike and the standard test; median (range) respectively 602 m (358-684) and 486 m (382-510). Conclusions: Due to practical barriers a larger study with the walk-bike in patients with IPF seems not feasible. Individual patients may benefit from the use of a walk-bike as it improved action radius and showed a tendency towards improvement in QoL. No effect on exercise capacity was observed. (Sarcoidosis Vasc Diffuse Lung Dis 2020; 37 (2): 192-202).
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Deambulação com Auxílio , Terapia por Exercício/instrumentação , Tolerância ao Exercício , Fibrose Pulmonar Idiopática/terapia , Limitação da Mobilidade , Qualidade de Vida , Caminhada , Idoso , Idoso de 80 Anos ou mais , Estudos Cross-Over , Desenho de Equipamento , Estudos de Viabilidade , Feminino , Humanos , Fibrose Pulmonar Idiopática/diagnóstico , Fibrose Pulmonar Idiopática/fisiopatologia , Fibrose Pulmonar Idiopática/psicologia , Masculino , Pessoa de Meia-Idade , Países Baixos , Satisfação do Paciente , Projetos Piloto , Recuperação de Função Fisiológica , Fatores de Tempo , Resultado do TratamentoRESUMO
Idiopathic pulmonary fibrosis is an unrelenting form of interstitial lung disease associated with a high symptom burden and reported low health-related quality of life. Clinicians have access to limited pharmacologic interventions to help slow the disease progression. Nonpharmacologic interventions are vital in managing dyspnea for these individuals, which is one of the most frequently reported factors that negatively impacts health-related quality of life. Common methods of symptom control include integration of pulmonary rehabilitation, supplemental oxygen, and interdisciplinary support, such as support groups, palliative care, and case conferences, into routine medical care. This literature review describes a multidisciplinary approach for managing dyspnea to improve health-related quality of life for those with idiopathic pulmonary fibrosis. Findings demonstrate that structured pulmonary rehabilitation programs, fast-track case conferences, and supplemental oxygen therapy are most effective. Further research is needed to demonstrate a clinically significant benefit of palliative care visits in the long term for these individuals.
Assuntos
Dispneia/terapia , Fibrose Pulmonar Idiopática/complicações , Gerenciamento Clínico , Dispneia/etiologia , Humanos , Fibrose Pulmonar Idiopática/fisiopatologia , Fibrose Pulmonar Idiopática/psicologia , Qualidade de Vida/psicologia , Inquéritos e QuestionáriosRESUMO
BACKGROUND: Two antifibrotic drugs, nintedanib and pirfenidone, are available for treatment of idiopathic pulmonary fibrosis (IPF). Although efficacy and adverse events have been well studied, little is known about patient experiences with these drugs. We aimed to systematically and quantitatively evaluate patient expectations, experiences, and satisfaction with nintedanib and pirfenidone. Furthermore, we assessed which factors were associated with overall patient satisfaction with medication. METHODS: Outpatients with IPF prospectively completed the Patient Experiences and Satisfaction with Medication (PESaM) questionnaire before start, and after three and 6 months of antifibrotic treatment, as part of a randomized eHealth trial (NCT03420235). The PESaM questionnaire consists of an expectation module, a validated generic module evaluating patient experiences and satisfaction concerning the effectiveness, side-effects, and ease of use of a medication, and a disease-specific module about IPF. Satisfaction was scored on a scale from - 5 (very dissatisfied) to + 5 (very satisfied). RESULTS: In total, 90 patients were included, of whom 43% used nintedanib and 57% pirfenidone. After 6 months, the mean overall score for satisfaction with medication was 2.1 (SD 1.9). No differences were found in experiences and satisfaction with medication, and the number and severity of side-effects between nintedanib and pirfenidone. Perceived effectiveness of medication was rated as significantly more important than side-effects and ease of use (p = 0.001). Expectations of patients regarding effectiveness were higher than experiences after 6 months. Self-reported experience with effectiveness was the main factor associated with overall medication satisfaction. CONCLUSIONS: Patient experiences and satisfaction with antifibrotic treatment were fairly positive, and similar for nintedanib and pirfenidone. Systematic evaluation of patient expectations, experiences, and satisfaction with medication could enhance shared-decision making and guide drug treatment decisions in the future. TRIAL REGISTRATION: NCT03420235 .
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Anti-Inflamatórios não Esteroides/uso terapêutico , Fibrose Pulmonar Idiopática/tratamento farmacológico , Indóis/uso terapêutico , Motivação , Satisfação do Paciente , Piridonas/uso terapêutico , Idoso , Feminino , Humanos , Fibrose Pulmonar Idiopática/psicologia , Masculino , Pessoa de Meia-Idade , Estudos Prospectivos , Autorrelato , Inquéritos e Questionários , Resultado do TratamentoRESUMO
PURPOSE OF REVIEW: To present an overview of the impact of idiopathic pulmonary fibrosis (IPF) on patients' emotional well being and quality of life (QoL). RECENT FINDINGS: IPF is an interstitial lung disease which causes irreversible, progressive lung scarring in a pathological pattern of usual interstitial pneumonia. The incidence of IPF is increasing at a global level, subjecting an increasing number of people to its high morbidity and risk of mortality. Diagnosis is based on a multidisciplinary team approach and the exclusion of other interstitial lung diseases. Two novel antifibrotic treatments, pirfenidone and nintedanib, were recently approved by regulatory agencies around the globe, thus providing many IPF patients with treatment options for the first time. Several other drugs have entered the investigational pipeline, including many in early-phase or late-phase clinical trials. Given the incurable and progressive nature of IPF, even with antifibrotic therapy, depression and anxiety are common among patients; these and burdensome symptoms of breathlessness, cough and fatigue are factors that impact patients' emotional well being and QoL. In addition to even more effective drugs, there is a need for psychosocial interventions and mental health support strategies focused on improving patients' QoL so they are better equipped to live with this devastating condition. SUMMARY: The current article highlights the effects of IPF on patients' emotional well being and QoL and offers suggestions for strategies to help patients with IPF live as well as possible in their daily lives.
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Tosse/fisiopatologia , Dispneia/fisiopatologia , Fadiga/fisiopatologia , Fibrose Pulmonar Idiopática/fisiopatologia , Fibrose Pulmonar Idiopática/psicologia , Saúde Mental , Qualidade de Vida , Anti-Inflamatórios não Esteroides/uso terapêutico , Humanos , Fibrose Pulmonar Idiopática/terapia , Indóis/uso terapêutico , Pulmão , Inibidores de Proteínas Quinases/uso terapêutico , Piridonas/uso terapêuticoRESUMO
Rationale: Several new drugs for idiopathic pulmonary fibrosis (IPF) are in development. Tools are needed to assess whether these drugs benefit patients on outcomes that matter most to them. Health-related quality of life (HRQL) is one such outcome. It is influenced by many factors, but symptoms and their impacts are two strong drivers.Objectives: To develop a questionnaire to assess symptoms, disease impacts, and HRQL specifically for patients with IPF.Methods: Working with the U.S. Food and Drug Administration through the Drug Development Tool Qualification process, focus groups, concept elicitation, and cognitive debriefing interviews were conducted to inform the development of a 44-item pilot questionnaire. The pilot paper-and-pen questionnaire was migrated to an equivalent electronic version and field-tested in a 14-day study. Response data were subjected to psychometric testing, including exploratory factor analysis, item calibration using item response theory models, test-retest reliability, and validity testing.Measurements and Main Results: A total of 125 patients with IPF (62.4% men) completed the longitudinal study. The mean ± SD age of the cohort was 69 ± 7.60 years, and the mean FVC% predicted was 71 ± 20.0. After factor and item analyses, 35 items were retained, and these comprise the two modules (symptoms and impacts) of the Living with IPF (L-IPF) questionnaire. The L-IPF yields five scales demonstrating good psychometric properties, including correlation with concurrently collected FVC% predicted and the ability to discriminate between patients with differing levels of IPF severity.Conclusions: The L-IPF is a new questionnaire that assesses symptoms, disease impacts, and HRQL in patients with IPF.
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Atividades Cotidianas/psicologia , Fibrose Pulmonar Idiopática/fisiopatologia , Fibrose Pulmonar Idiopática/psicologia , Psicometria/normas , Qualidade de Vida/psicologia , Inquéritos e Questionários/normas , Avaliação de Sintomas/normas , Idoso , Idoso de 80 Anos ou mais , Estudos de Coortes , Feminino , Humanos , Fibrose Pulmonar Idiopática/epidemiologia , Estudos Longitudinais , Masculino , Pessoa de Meia-Idade , Reprodutibilidade dos Testes , Avaliação de Sintomas/estatística & dados numéricos , Estados Unidos/epidemiologiaRESUMO
In idiopathic pulmonary fibrosis (IPF), several factors may have a negative impact on the nutritional status, including an increased respiratory muscles load, release of inflammation mediators, the coexistence of hypoxemia, and physical inactivity. Nutritional abnormalities also have an impact on IPF clinical outcomes. Given the relevance of nutritional status in IPF patients, we sought to focus on some critical issues, highlighting what is known and what should be further learned about these issues. We revised scientific literature published between 1995 and August 2019 by searching on Medline/PubMed and EMBASE databases including observational and interventional studies. We conducted a narrative review on nutritional assessment in IPF, underlining the importance of nutritional evaluation not only in the diagnostic process, but also during follow-up. We also highlighted the need to keep a high level of attention on cardiovascular comorbidities. We also focused on current clinical treatment in IPF with Nintedanib and Pirfenidone and management of gastrointestinal adverse events, such as diarrhea, induced by these antifibrotic drugs. Finally, we concentrated on the importance of pulmonary rehabilitation program, including nutritional assessment, education and behavioral change, and psychological support among its essential components. More attention should be devoted to the assessment of the undernutrition and overnutrition, as well as of muscle strength and physical performance in IPF patients, taking also into account that an adequate clinical management of gastrointestinal complications makes IPF drug treatments more feasible.
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Fibrose Pulmonar Idiopática/metabolismo , Avaliação Nutricional , Fenômenos Fisiológicos da Nutrição/fisiologia , Estado Nutricional , Comportamento , Doenças Cardiovasculares , Comorbidade , Humanos , Fibrose Pulmonar Idiopática/fisiopatologia , Fibrose Pulmonar Idiopática/psicologia , Indóis/efeitos adversos , Força Muscular , Distúrbios Nutricionais/diagnóstico , Educação de Pacientes como Assunto , Piridonas/efeitos adversosRESUMO
BACKGROUND: Idiopathic pulmonary fibrosis (IPF) specific version of St. George's Respiratory Questionnaire (SGRQ-I) and King's Brief Interstitial Lung Disease questionnaire (K-BILD) are validated health-related quality of life (HRQL) instruments, but no or limited data exist on their responsiveness and minimal clinically important difference (MCID). The objectives of this study were to assess responsiveness of SGRQ-I and K-BILD and determine MCID separately for deterioration and improvement in a large, prospective cohort of patients with IPF in a real-world setting. METHODS: Consecutive patients with IPF were recruited. SGRQ-I, K-BILD, SGRQ, Shortness of Breath Questionnaire, pulmonary function tests and 6-min walk test measurements were obtained at baseline and at six and 12 months; at six and 12 months, patients also completed Global Rating of Change Scales. Responsiveness was assessed using correlation coefficients and linear regression. Cox regression was used for mortality analyses. MCID was estimated using receiver operating characteristic curves with separate analyses for improvement and deterioration. RESULTS: A total of 150 IPF patients were included and 124 completed the 12-month follow-up. Based on all HRQL anchors and most physiological anchors, responsiveness analyses supported the evidence pointing towards SGRQ-I and K-BILD as responsive instruments. Multivariate analyses showed an association between SGRQ-I and mortality (HR: 1.18, 95% CI: 1.02 to 1.36, p = 0.03) and a trend was found for K-BILD (HR: 0.82, 95% CI: 0.64 to 1.05, p = 0.12). MCID was estimated for all domains of SGRQ-I and K-BILD. MCID for improvement differed from deterioration for both SGRQ-I Total (3.9 and 4.9) and K-BILD Total (4.7 and 2.7). CONCLUSIONS: SGRQ-I and K-BILD were responsive to change concerning both HRQL and most physiological anchors. MCID was determined separately for improvement and deterioration, resulting in different estimates; especially a smaller estimate for deterioration compared to improvement in K-BILD. TRIAL REGISTRATION: Clinicaltrials.gov, no. NCT02818712. Registered 30 June 2016.
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Fibrose Pulmonar Idiopática/diagnóstico , Fibrose Pulmonar Idiopática/psicologia , Doenças Pulmonares Intersticiais/diagnóstico , Doenças Pulmonares Intersticiais/psicologia , Diferença Mínima Clinicamente Importante , Qualidade de Vida/psicologia , Idoso , Estudos de Coortes , Feminino , Humanos , Fibrose Pulmonar Idiopática/terapia , Doenças Pulmonares Intersticiais/terapia , Masculino , Pessoa de Meia-Idade , Estudos Prospectivos , Resultado do Tratamento , Teste de Caminhada/métodos , Teste de Caminhada/psicologiaRESUMO
Rationale: Idiopathic pulmonary fibrosis (IPF) is a deadly disease with increasingly impaired health-related quality of life (HRQOL). eHealth technologies facilitate collection of physiological outcomes and patient-reported outcomes at home, but randomized controlled trials (RCTs) on the effects of eHealth are scarce.Objectives: To investigate whether a home monitoring program improves HRQOL and medication use for patients with IPF.Methods: We performed a multicenter RCT in newly treated patients with IPF. Patients were randomly assigned to standard care or a home monitoring program on top of standard care for 24 weeks. The home monitoring program included home spirometry, reporting of symptoms and side effects, patient-reported outcomes, information, a medication coach, and eConsultations. The primary endpoint was between-group difference in change in King's Brief Interstitial Lung Disease Questionnaire (K-BILD) score at 24 weeks.Measurements and Main Results: A total of 90 patients were randomized (46 assigned to the home monitoring group and 44 to the standard care group). After 24 weeks, no statistically significant difference was found in K-BILD total score, with a 2.70-point increase in the home monitoring group (SD = 9.5) and a 0.03-point increase in the standard care group (SD = 10.4); between-group difference was 2.67 points (95% confidence interval [CI], -1.85 to 7.17; P = 0.24). Between-group difference in psychological domain score was 5.6 points (95% CI, -1.13 to 12.3; P = 0.10), with an increase of 5.12 points in the home monitoring group (SD = 15.8) and a decline of 0.48 points in the standard care group (SD = 13.3). In the home monitoring group, medication was more often adjusted (1 vs. 0.3 adjustments per patient; 95% CI, 0.2 to 1.3; P = 0.027). Patient satisfaction with the home monitoring program was high. Home-based spirometry was highly correlated with hospital-based spirometry over time.Conclusions: The results of this first-ever eHealth RCT in IPF showed that a comprehensive home monitoring program did not improve overall HRQOL measured with K-BILD but tended to improve psychological well-being. Home monitoring was greatly appreciated by patients and allowed for individually tailored medication adjustments.Clinical trial registered with www.clinicaltrials.gov (NCT03420235).