Patient experiences with pulmonary fibrosis

Collard, Harold R.; Tino, Gregory; Noble, Paul W.; Shreve, Mark Anthony; Michaels, Maureen; Carlson, Bruce W.; Schwarz, Marvin I.

doi:10.1016/j.rmed.2006.10.002

Cited by 110 publications

(129 citation statements)

References 16 publications

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“…In a prospective study in the US of 129 patients with IPF, the median delay between the onset of dyspnea and referral to a tertiary care center was 2.2 years [74]. In a survey of 1448 patients with IPF and caregivers of patients with IPF, 55% reported a delay of >1 year between the onset of symptoms and diagnosis, and 38% saw >3 physicians before a diagnosis of IPF was established [75]. Moreover, delayed access to a tertiary care centre was associated with a higher risk of death in patients with IPF independent of disease severity [74] (Figure 3).…”

Section: Importance Of Prompt Diagnosis and Referralmentioning

confidence: 99%

Natural history of idiopathic pulmonary fibrosis

Kim

Perlman

Tomic

2015

Respiratory Medicine

149

109

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Idiopathic pulmonary fibrosis (IPF) is a parenchymal lung disease characterized by progressive interstitial fibrosis. IPF has a poor prognosis, with a median survival time of 2-3 years from diagnosis, but varying from a few months to a decade. The natural history of IPF is highly variable and the course of disease in an individual patient is difficult to predict. Some patients with IPF experience rapid decline, others progress much more slowly, and some patients show periods of relative stability interspersed with acute deteriorations in respiratory function. Many clinical, radiographic, serologic, and histopathologic variables have been shown to predict mortality in IPF. However, the accuracy of these predictors varies due to the retrospective nature of some of the studies and variations in study design. The ability to identify clinical characteristics that predict disease progression and survival would be useful for counseling patients, treatment decision-making, and prompt consideration for lung transplantation. A number of indices for predicting mortality in patients with IPF are available, but they require further validation. As high-resolution computed tomography scans become more widely available and patients with IPF are diagnosed earlier, survival times following diagnosis will improve. Early referral to interstitial lung disease specialty centers is important for accurate diagnosis and may be associated with improved outcomes. The goal of this review is to examine the natural history of IPF, discuss predictors of mortality, and highlight the importance of prompt diagnosis and referral for patients with IPF.

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Section: Importance Of Prompt Diagnosis and Referralmentioning

confidence: 99%

Natural history of idiopathic pulmonary fibrosis

Kim

Perlman

Tomic

2015

Respiratory Medicine

149

109

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“…Results of a survey of 1251 IPF patients and 197 caregivers conducted in the USA found that 55% reported at least a 1-year delay between initial presentation and diagnosis, with some 38% having been seen by three or more physicians. 35 Incorrect diagnoses included bronchitis, asthma, chronic obstructive pulmonary disease (COPD), emphysema and heart disease. The study also found that 64% of responders agreed that there was a lack of information and/or resources on IPF when they were diagnosed.…”

Section: Current Service Provisionmentioning

confidence: 99%

The clinical effectiveness and cost-effectiveness of treatments for idiopathic pulmonary fibrosis: a systematic review and economic evaluation

Loveman

Copley

Colquitt

et al. 2015

Health Technology Assessment

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BackgroundIdiopathic pulmonary fibrosis (IPF) is a life-limiting lung disease that generally affects people over 60 years old. The main symptoms are shortness of breath and cough, and as the disease progresses there is a considerable impact on day-to-day life. Few treatments are currently available.ObjectivesTo conduct a systematic review of clinical effectiveness and an analysis of cost-effectiveness of treatments for IPF based on an economic model informed by systematic reviews of cost-effectiveness and quality of life.Data sourcesEleven electronic bibliographic databases, including MEDLINE, EMBASE, Web of Science, and The Cochrane Library and the Centre for Reviews and Dissemination databases, were searched from database inception to July 2013. Reference lists of relevant publications were also checked and experts consulted.MethodsTwo reviewers independently screened references for the systematic reviews, extracted and checked data from the included studies and appraised their risk of bias. An advisory group was consulted about the choice of interventions until consensus was reached about eligibility. A narrative review with meta-analysis was undertaken, and a network meta-analysis (NMA) was performed. A decision-analytic Markov model was developed to estimate cost-effectiveness of pharmacological treatments for IPF. Parameter values were obtained from NMA and systematic reviews. Univariate and probabilistic sensitivity analyses were undertaken. The model perspective is NHS and Personal Social Services, and discount rate is 3.5% for costs and health benefits.ResultsFourteen studies were included in the review of clinical effectiveness, of which one evaluated azathioprine, threeN-acetylcysteine (NAC) (alone or in combination), four pirfenidone, one BIBF 1120, one sildenafil, one thalidomide, two pulmonary rehabilitation, and one a disease management programme. Study quality was generally good, with a low risk of bias. The current evidence suggests that some treatments appear to be clinically effective. The model base-case results show increased survival for five pharmacological treatments, compared with best supportive care, at increased cost. General recommendations cannot be made of their cost-effectiveness owing to limitations in the evidence base.LimitationsFew direct comparisons of treatments were identified. An indirect comparison through a NMA was performed; however, caution is recommended in the interpretation of these results. In relation to the economic model, there is an assumption that pharmacological treatments have a constant effect on the relative rate of per cent predicted forced vital capacity decline.ConclusionsFew interventions have any statistically significant effect on IPF and a lack of studies on palliative care approaches was identified. Research is required into the effects of symptom control interventions, in particular pulmonary rehabilitation and thalidomide. Other research priorities include a well-conducted randomised controlled trial on inhaled NAC therapy and an updated evidence synthesis once the results of ongoing studies are reported.Study registrationThis study is registered as PROSPERO CRD42012002116.FundingThe National Institute for Health Research Health Technology Assessment programme.

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“…The loss of independence may also impact on the patients' relationships as they may perceive themselves to be a burden to their family and society. There may also be financial implications if the patient is no longer able to work [12,13,22]. A major challenge faced by patients at the diagnostic stage is to balance the initial relief that they ''do not have cancer'' with the latter realisation that the prognosis is, in fact, not significantly different.…”

Section: Problems Faced By Ipf Patients During the Course Of Their DImentioning

confidence: 99%

“…In a recent survey of 1448 IPF patients, respondents reported a clear lack of information and resources on pulmonary fibrosis at the time of diagnosis. Information regarding treatment options, the role of supplemental oxygen, pulmonary rehabilitation and transplantation was also limited [12,13]. …”

Section: Problems Faced By Ipf Patients During the Course Of Their DImentioning

confidence: 99%

Patient-centred management in idiopathic pulmonary fibrosis: similar themes in three communication models

Wuyts¹,

Peccatori²,

Russell³

2014

Eur Respir Rev

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The progressive and highly variable course of idiopathic pulmonary fibrosis (IPF) can present patients and their families with various challenges at different points of the disease. Structured communication between the healthcare professional and the patient is vital to ensure the best possible support and treatment for the patient. While research in this area has been limited, an increasing number of studies are emerging that support the role of communication in patients with debilitating and fatal lung diseases.Communication models used in other conditions that share many challenges with IPF, such as cancer, provide important insights for developing specifically designed patient support and communications models in IPF. Three communication models will be described: 1) the patient-centred care model (for oncology); 2) the three pillars of care model (for IPF); and 3) the Brompton model of care (for interstitial lung disease). Themes common to all three models include comprehensive patient education, encouraged patient participation and an accessible healthcare system, all supported by a collaborative provider-patient relationship. The development of effective communication skills is an on-going process and it is recommended to examine communication models used in other chronic diseases. @ERSpublications Communication models in other conditions provide important insights for support and communications models in IPF

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Patient experiences with pulmonary fibrosis

Cited by 110 publications

References 16 publications

Natural history of idiopathic pulmonary fibrosis

Natural history of idiopathic pulmonary fibrosis

The clinical effectiveness and cost-effectiveness of treatments for idiopathic pulmonary fibrosis: a systematic review and economic evaluation

Patient-centred management in idiopathic pulmonary fibrosis: similar themes in three communication models

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