Care programs and their components for patients with idiopathic pulmonary fibrosis: a systematic review

Abstract 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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Lived experiences of patients with idiopathic pulmonary fibrosis: navigating through the complex healthcare system

10.21203/rs.2.15938/v1 ◽

2019 ◽

Author(s):

Yang Lyu ◽

Yanrui Jia ◽

Fengli Gao ◽

Yaling Huang ◽

Frances Lin

Keyword(s):

Idiopathic Pulmonary Fibrosis ◽

Pulmonary Fibrosis ◽

Lived Experience ◽

Health Care Professionals ◽

Lived Experiences ◽

Care Delivery ◽

Tertiary Hospital ◽

Care Needs ◽

Care Models ◽

Health Demands

Abstract Background : Over the last decade, idiopathic pulmonary fibrosis (IPF) has been attracting health care professionals’ attention worldwide due to its impact on poor survival rate and quality of life, such as ongoing physical and emotional distress experienced by patients and their families. The diagnosis and management of IPF patients often remain as a significant challenge for clinicians. Therefore, it is imperative to gain an in-depth understanding on experiences of IPF patients during their disease journey in order to improve the care delivered to this vulnerable group. The aim of this study was to explore the lived experiences of the disease journey and care needs of patients with IPF. Methods : A semi-structured face-to-face one on one interview was conducted with a purposive sampling of 16 IPF patients admitted to a respiratory medicine department of a tertiary hospital in Beijing, China. The consolidated criteria for reporting qualitative research checklist was used. Results : Four themes emerged from the qualitative data included the long and confusing journal to reach diagnosis, living with the disease, understanding the disease and treatment, and desire for continuity of care. Conclusions : The findings provide an in-depth understanding of the lived experience of the disease journey and the care needs in patients with IPF. There is an urgent need to improve the care delivery to this vulnerable population. To meet the health demands of this group of patients, it is of paramount importance to develop effective education programs for health professionals and IPF patients and also to improve the care models of healthcare systems, especially in the remote area in order to enhance the principle of continuing care for IPF patients in the communities.

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Evaluating integrated care for people with complex needs

Journal of Health Services Research & Policy ◽

10.1177/1355819620931872 ◽

2020 ◽

Vol 26 (1) ◽

pp. 46-53

Author(s):

Panagiotis Kasteridis ◽

Anne Mason ◽

Andrew Street

Keyword(s):

Primary Care ◽

Integrated Care ◽

Models Of Care ◽

Care Team ◽

Future Research ◽

Complex Care ◽

Care Needs ◽

Complex Needs ◽

Care Models ◽

Integrated Care Models

Objectives As part of the Vanguard programme, two integrated care models were introduced in South Somerset for people with complex care needs: the Complex Care Team and Enhanced Primary Care. We assessed their impact on a range of utilization measures and mortality. Methods We used monthly individual-level linked primary and secondary care data from April 2014 to March 2018 to assess outcomes before and after the introduction of the care models. The analysis sample included 564 Complex Care Team and 841 Enhanced Primary Care cases that met specific criteria. We employed propensity score methods to identify out-of-area control patients and difference-in-differences analysis to isolate the care models’ impact. Results We found no evidence of significantly reduced utilization in any of the Complex Care Team or Enhanced Primary Care cohorts. The death rate was significantly lower only for those in the first Enhanced Primary Care cohort. Conclusions The integrated care models did not significantly reduce utilization nor consistently reduce mortality. Future research should test longer-term outcomes associated with the new models of care and quantify their contribution in the context of broader initiatives.

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Patients’ and healthcare professionals’ perspectives on the idiopathic pulmonary fibrosis care journey: a qualitative study

BMC Pulmonary Medicine ◽

10.1186/s12890-021-01431-8 ◽

2021 ◽

Vol 21 (1) ◽

Author(s):

Anouk Delameillieure ◽

Fabienne Dobbels ◽

Sarah Vandekerkhof ◽

Wim A. Wuyts

Keyword(s):

Idiopathic Pulmonary Fibrosis ◽

Pulmonary Fibrosis ◽

Integrated Care ◽

Healthcare Professionals ◽

Care Delivery ◽

Care Program ◽

Management Support ◽

Continuous Training ◽

Patient Reported ◽

Centre Of Excellence

Abstract 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.

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Lived experiences of patients with idiopathic pulmonary fibrosis: navigating through the complex healthcare system

10.21203/rs.3.rs-45620/v1 ◽

2020 ◽

Author(s):

Yang Lyu ◽

Yanrui Jia ◽

Fengli Gao ◽

Ya-ling Huang ◽

Frances Lin

Keyword(s):

Idiopathic Pulmonary Fibrosis ◽

Pulmonary Fibrosis ◽

Continuity Of Care ◽

Lived Experiences ◽

Care Delivery ◽

Tertiary Hospital ◽

Care Needs ◽

Face To Face ◽

Care Models

Abstract Background: Over the last decade, idiopathic pulmonary fibrosis (IPF) has been attracting healthcare professionals’ attention worldwide due to its impact on poor survival rate and quality of life, such as ongoing physical and emotional distress experienced by patients and their families. The diagnosis and management of IPF patients often remain a significant challenge for clinicians. Therefore, it is imperative to gain an in-depth understanding on experiences of IPF patients during their disease journey in order to improve the care delivered to this vulnerable group. We aimed to explore the lived experiences of disease journey and care needs of patients with IPF. Methods: Semi-structured face-to-face interview were conducted with a purposive sampling of sixteen IPF patients admitted to the department of respiratory medicine in a tertiary hospital in China. The consolidated criteria for reporting qualitative research was followed. Results: Four themes emerged from thematic analysis of the qualitative data included: the long and confusing journey to reach diagnosis, living with the disease, understanding the disease and treatment, and desire for continuity of care. Conclusions: There is an urgent need to improve the care delivery to this vulnerable population in China. To meet their health needs, it is of paramount importance to develop effective education programs for health professionals and IPF patients and to improve care models of healthcare systems, especially in the remote area in order to improve the continuity of care in the communities.

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Faculty Opinions recommendation of Lung transplantation in idiopathic pulmonary fibrosis: a systematic review of the literature.

Faculty Opinions – Post-Publication Peer Review of the Biomedical Literature ◽

10.3410/f.718534195.793539200 ◽

2017 ◽

Author(s):

Sergio Harari ◽

Antonella Caminati

Keyword(s):

Systematic Review ◽

Idiopathic Pulmonary Fibrosis ◽

Pulmonary Fibrosis ◽

Lung Transplantation ◽

Review Of The Literature

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Prognostic role of Krebs von den Lungen-6 (KL-6) measurement in idiopathic pulmonary fibrosis: a systematic review and meta-analysis

Clinical Chemistry and Laboratory Medicine (CCLM) ◽

10.1515/cclm-2021-0199 ◽

2021 ◽

Vol 0 (0) ◽

Author(s):

Elena Aloisio ◽

Federica Braga ◽

Chiara Puricelli ◽

Mauro Panteghini

Keyword(s):

Systematic Review ◽

Idiopathic Pulmonary Fibrosis ◽

Pulmonary Fibrosis ◽

Binary Data ◽

Meta Analysis ◽

Mortality Prediction ◽

Asian Populations ◽

Increased Risk ◽

Using Data ◽

Patient Prognosis

Abstract Objectives Idiopathic pulmonary fibrosis (IPF) is a progressive interstitial disease with limited therapeutic options. The measurement of Krebs von den Lungen-6 (KL-6) glycoprotein has been proposed for evaluating the risk of IPF progression and predicting patient prognosis, but the robustness of available evidence is unclear. Methods We searched Medline and Embase databases for peer-reviewed literature from inception to April 2020. Original articles investigating KL-6 as prognostic marker for IPF were retrieved. Considered outcomes were the risk of developing acute exacerbation (AE) and patient survival. Meta-analysis of selected studies was conducted, and quantitative data were uniformed as odds ratio (OR) or hazard ratio (HR) estimates, with corresponding 95% confidence intervals (CI). Results Twenty-six studies were included in the systematic review and 14 were finally meta-analysed. For AE development, the pooled OR (seven studies) for KL-6 was 2.72 (CI 1.22–6.06; p=0.015). However, a high degree of heterogeneity (I2=85.6%) was found among selected studies. Using data from three studies reporting binary data, a pooled sensitivity of 72% (CI 60–82%) and a specificity of 60% (CI 52–68%) were found for KL-6 measurement in detecting insurgence of AE in IPF patients. Pooled HR (seven studies) for mortality prediction was 1.009 (CI 0.983–1.036; p=0.505). Conclusions Although our meta-analysis suggested that IPF patients with increased KL-6 concentrations had a significant increased risk of developing AE, the detection power of the evaluated biomarker is limited. Furthermore, no relationship between biomarker concentrations and mortality was found. Caution is also needed when extending obtained results to non-Asian populations.

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Breath Biomarkers in Idiopathic Pulmonary Fibrosis: A Systematic Review

10.1183/13993003.congress-2018.pa2921 ◽

2018 ◽

Author(s):

Conal Hayton ◽

Dayle Terrington ◽

Andrew M. Wilson ◽

Nazia Chaudhuri ◽

Colm Leonard ◽

...

Keyword(s):

Systematic Review ◽

Idiopathic Pulmonary Fibrosis ◽

Pulmonary Fibrosis

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Efficacy and safety of pirfenidone in the treatment of idiopathic pulmonary fibrosis patients: a systematic review and meta-analysis of randomised controlled trials

BMJ Open ◽

10.1136/bmjopen-2021-050004 ◽

2021 ◽

Vol 11 (12) ◽

pp. e050004

Author(s):

Wenjuan Wu ◽

Lingxiao Qiu ◽

Jizhen Wu ◽

Xueya Liu ◽

Guojun Zhang

Keyword(s):

Systematic Review ◽

Idiopathic Pulmonary Fibrosis ◽

Pulmonary Fibrosis ◽

Adverse Events ◽

Acute Exacerbation ◽

Randomised Controlled Trials ◽

Meta Analysis ◽

Controlled Trials ◽

Efficacy And Safety ◽

Randomised Controlled

ObjectivesIdiopathic pulmonary fibrosis (IPF) has been defined as a distinctive type of chronic fibrotic disease, characterised by a progressive decline in lung function and a common histological pattern of interstitial pneumonia. To analyse the efficacy and safety of pirfenidone in the treatment of IPF, a systematic review and meta-analysis was performed.DesignThis is a meta-analysis study.ParticipantsPatients were diagnosed as IPF.InterventionsUse of pirfenidone.Primary and secondary outcomeProgression-free survival (PFS), acute exacerbation and worsening of IPF and Impact on adverse events.MeasuresThe inverse variance method for the random-effects model was used to summarise the dichotomous outcomes, risk ratios and 95% CIs.ResultsA total of 9 randomised controlled trials with 1011 participants receiving pirfenidone and 912 controls receiving placebo were summarised. The pooled result suggested a statistically significant difference inall-cause mortality after pirfenidone use, with a summarised relative ratio of 0.51 (p<0.01). Longer PFS was observed in patients receiving pirfenidone compared with those who were given placebo (p<0.01). The IPF groups presented a high incidence of adverse events with a pooled relative ratio of 3.89 (p<0.01).ConclusionsPirfenidone can provide survival benefit for patients with IPF. Pirfenidone treatment was also associated with a longer PFS, a lower incidence of acute exacerbation and worsening of IPF.

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Systematic Review and Meta-analysis of Pirfenidone, Nintedanib, and Pamrevlumab for the Treatment of Idiopathic Pulmonary Fibrosis

Annals of Pharmacotherapy ◽

10.1177/1060028020964451 ◽

2020 ◽

pp. 106002802096445

Author(s):

Enrica Di Martino ◽

Alessio Provenzani ◽

Patrizio Vitulo ◽

Piera Polidori

Keyword(s):

Systematic Review ◽

Idiopathic Pulmonary Fibrosis ◽

Pulmonary Fibrosis ◽

Meta Analysis ◽

Random Effect ◽

Random Effect Model ◽

World Health ◽

Effect Model ◽

All Cause Mortality ◽

Positive Effect

Background: The comparative efficacy of pirfenidone, nintedanib, and pamrevlumab in slowing the rate of forced vital capacity (FVC) decline and mortality in patients with idiopathic pulmonary fibrosis (IPF) is unknown. Objective: To perform a systematic review and meta-analysis (MA) of these drugs for IPF. Methods: We searched CENTRAL, PubMed, EMBASE, ClincalTrials.gov, and the World Health Organization’s registry databases up to March 2020. Phase II/III randomized controlled trials in adults with IPF were eligible. The random-effect model was implemented calculating the effect size and respective 95% CI as Cohen’s d for change from baseline FVC (in percentage predicted and liters) and odds ratio (OR) for 10% reduction in FVC and all-cause mortality (ACM). Results: Six studies were included in the MA. For change from baseline in percentage predicted FVC, the MA indicated that the 3 drugs were more effective than placebo (pirfenidone: d=3.30%, 95% CI=2.15-4.45; nintedanib: d=3.15%, 95% CI=2.35-3.95; pamrevlumab: d=4.30%, 95% CI=0.45-8.15). These results are superimposable to those relating to change from baseline FVC in liters (pirfenidone: d=0.09L, 95% CI=0.04-0.14; nintedanib: d=0.13L, 95% CI=0.10-0.16; pamrevlumab: d=0.20L, 95% CI=0.05-0.35). Each drug had a positive effect on 10% reduction in FVC (pirfenidone: OR=0.57, 95% CI=0.45-0.74; nintedanib: OR=0.66, 95% CI=0.51-0.85; pamrevlumab: OR=0.24, 95% CI=0.08-0.73), but only pirfenidone showed an effect on ACM (OR=0.50; 95% CI=0.31-0.83). Conclusion and Relevance: This MA provided encouraging results on pamrevlumab efficacy in slowing the decline in FVC compared with pirfenidone and nintedanib. Actually, in phase 3, it could become a potential IPF treatment.

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