European IPF Patient Charter: unmet needs and a call to action for healthcare policymakers.

Patient advocacy groups play an important role in supporting patients with chronic diseases and promoting better care. The aim of this patient-physician initiative was to gather perceptions from European idiopathic pulmonary fibrosis (IPF) patient advocacy groups regarding inequalities and unmet needs in IPF care, in order to develop a Patient Charter to advocate for better care.In total, 11 European patient advocacy groups were interviewed regarding the care of patients with IPF in their countries. Interview feedback was presented to a Working Group including patient advocacy group representatives and IPF specialists; key areas of agreement were developed into the European IPF Patient Charter.The interviews identified five key themes that fed into the final Charter: the need for improved diagnosis, treatment access, holistic care, disease awareness and palliative care. The final Charter was endorsed by patient advocacy groups and presented to 26 Members of the European Parliament in September 2014. It has received >8900 signatures to date.This patient-physician initiative highlights the inequalities and unmet needs in IPF care across Europe, and demonstrates how this insight can inform the development of a Patient Charter, designed as a call to action for healthcare policymakers to drive improvement in European IPF care.

IPF Care: a support program for patients with idiopathic pulmonary fibrosis treated with pirfenidone in Europe.

Idiopathic pulmonary fibrosis (IPF) is a chronic, progressive, and irreversible fibrotic lung disease that requires long-term treatment. Given the importance of adherence to treatment and management of adverse events (AEs), patients with IPF need long-term, high-quality support in living with their condition, and adhering to therapy so they can derive maximum benefit. The IPF Care Patient Support Program (IPF Care) provides support, education, and empowerment to patients receiving pirfenidone for the treatment of IPF in Europe, through the provision of frequent, patient-managed discussions with specialist IPF nurses. In this review, we describe the structure of IPF Care in the United Kingdom (UK) and Austria, two of the longest-running IPF Care programs to date, and describe the benefits that these programs provide to patients with IPF. Analysis of results demonstrates a low rate of discontinuation from the program, and provides insight into the questions and concerns that patients express, not only with respect to pirfenidone (the only approved treatment for IPF at the time of analysis), but also in relation to other aspects of living with IPF. Pirfenidone dose modifications are common in patients in IPF Care and AEs most commonly occur early in treatment, with the majority of affected patients continuing on a stable maintenance dose. This highlights the value of the advice and support that patients receive in IPF Care regarding management of AEs and staying on treatment. Patient satisfaction was high in a survey of the UK program, with patients reporting high scores regarding 'feeling in control of their condition', 'knowing what to expect from treatment', and 'feeling confident about how their disease is managed'. IPF Care in Europe will continue to evolve over time, striving to provide individually tailored support and patient-friendly information to improve treatment outcomes and quality of life for patients living with IPF.

Perceptions, experiences and needs of patients with idiopathic pulmonary fibrosis.

AIMS: To understand the perceptions, needs and experiences of patients with Idiopathic Pulmonary Fibrosis. BACKGROUND: Idiopathic pulmonary fibrosis is a progressive interstitial lung disease, with a mean life expectancy similar to some forms of cancer of 2-4 years from diagnosis. Unlike the cancer literature, which is rich with studies exploring the needs of their disease group, few publications exist on patient needs with this severe fibrotic lung disease. DESIGN: A Qualitative study which took place between 2007-2012. METHODS: Seventeen patients with a multidisciplinary team confirmed diagnosis of Idiopathic Pulmonary Fibrosis, with moderate to advanced disease severity and six of their informal carers were interviewed. An interview topic guide was developed by the researchers and service user group. The interviews were audio-recorded, semi-structured and took place at a regional respiratory and lung transplant centre in North West England. Interviews were transcribed verbatim and data analysed using Framework Analysis. FINDINGS: Three main themes were identified: 'Struggling to get a diagnosis'; 'Loss of the life I previously had'; and 'Living with Idiopathic Pulmonary Fibrosis'. Patients reported struggling to get a diagnosis and coping with a life-limiting, rapidly progressive illness with no good treatment and few support structures. CONCLUSIONS: There is an urgent need for a better understanding of the difficulties faced by people with Idiopathic Pulmonary Fibrosis and their carers. This can be used to develop better supportive care in the United Kingdom and ultimately improve the quality of life of these patients.

Cross-Atlantic modification and validation of the A Tool to Assess Quality of Life in Idiopathic Pulmonary Fibrosis (ATAQ-IPF-cA).

RATIONALE: The A Tool to Assess Quality of Life in Idiopathic Pulmonary Fibrosis (ATAQ-IPF) was developed in the USA to assess health-related quality of life in patients with IPF. It is likely that some of the original ATAQ-IPF items perform differently when applied in different countries. This paper reports results of a study conducted to identify the need to refine the content of the ATAQ-IPF to minimise cross-country bias between the USA and the UK. METHODS: The ATAQ-IPF and other study measures were completed by patients attending specialist IPF clinics in the USA and UK. Rasch analysis was used to determine which items performed differently across countries (USA vs UK) and refine the original ATAQ-IPF to an instrument without cross-country bias (ATAQ-IPF-cA). Preliminary validation of the modified instrument was examined by assessing correlations between ATAQ-IPF-cA scores and scores from dyspnoea-specific patient-reported outcome (PRO) measures. RESULTS: 139 patients with IPF (USA=74; UK=65) participated in the study. A total of 41 items and 4 domains were removed from the original, 86-item instrument to yield the 43 items and 10 domains of the ATAQ-IPF-cA. Each domain had good fit to the Rasch model, internal consistency was comparable to the corresponding domains for the original ATAQ-IPF, and validity was supported by significant correlations between its scores and scores from dyspnoea-specific PROs. CONCLUSIONS: The reliability and validity of the substantially shortened ATAQ-IPF-cA are acceptable and comparable to the original instrument. We recommend use of the ATAQ-IPF-cA in IPF studies in which participants are enrolled from the USA and UK.

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.

Real world experiences: pirfenidone is well tolerated in patients with idiopathic pulmonary fibrosis.

Idiopathic pulmonary fibrosis (IPF) is a debilitating condition with life expectancy of two to five years from diagnosis. Treatment strategies for IPF are disappointingly limited and pirfenidone is currently the only licensed drug that has been shown to reduce the decline in forced vital capacity (FVC) at six months. We demonstrate our experience in prescribing pirfenidone in a single centre observational study of forty patients involved in a named patient programme (NPP) from September 2011 to January 2013. We demonstrate that improved adherence and compliance can be achieved by specialist nurse and clinician review, support and education of the patient. Twenty three of 40 (58%) patients experienced predominantly gastrointestinal adverse effects. Importantly we have enhanced patient adherence and compliance from an initial discontinuation rate of six patients (15%) at the beginning of the study to a zero discontinuation rate in the subsequent ten months. This study shows that in the real world pirfenidone is well tolerated and with expert regular specialist review adherence can be optimised and improved.

Effect of recording site on pulse oximetry readings.

BACKGROUND: Ambulatory oxygen requirements are routinely assessed and titrated using portable finger pulse oximetry. However, movement artefacts from hands moving or reduced circulation may adversely affect the accuracy of measurement. At Wythenshawe Hospital, we decided to use an ear oximeter routinely in addition to the finger oximeter. AIM: To evaluate and compare the use of portable oximetry measurements taken from the ear and finger during ambulatory assessments. METHOD: Before, during and after a six-minute walking test, 304 patients had ear and finger pulse oximetry measurements recorded. RESULTS: There was a significant difference between ear and finger measurements before and after exercise (p=<0.001 for both). The differences would have altered the clinical outcome in 27% of those assessed.

Principles to effectively manage people with interstitial lung disease in the community.

This article examines the problems of caring for people with interstitial lung disease in primary care. It describes simple strategies that can support people to remain at home during the end stage of their illness. The diagnosis and investigations of ILD have been covered in previous Nursing Times articles (Duck, 2007a; 2007b).

Nursing patients with interstitial lung disease.

Annette Duck considers the important factors for supporting patients with a diagnostic label of interstitial lung disease.

Interstitial lung diseases. 1: Diagnosis and investigation.

This is part 1 of a two-part unit on interstitial lung diseases (ILDs). The unit aims to unravel some misunderstandings surrounding ILDs and discusses how nurses can help to inform and support patients. It examines the background, diagnosis and investigation of ILDs.

Interstitial lung diseases 2: management strategies.

This is the second part of a two-part unit on interstitial lung diseases (ILDs). Part 1 examined the challenges of diagnosis and the range of investigations. This part explores management strategies for the three most common ILDs.

Cost-effectiveness and efficacy in long-term oxygen therapy.

Changes to the new home oxygen service have raised awareness among health professionals of the potential hazards and benefits of oxygen therapy. Annette Duck reviews the use of long-term oxygen therapy and highlights the importance of assessment in selecting both patients and suitable delivery systems.