Development of ATAQ-LAM: a tool to assess quality of life in Lymphangioleiomyomatosis.

Background: Lymphangioleiomyomatosis (LAM) is a progressive lung disease that impairs health-related quality of life (HRQL).

Objective: To develop and conduct initial testing of ATAQ-LAM (A Tool to Assess Quality of Life in LAM).

Methods: A pilot version of the questionnaire was administered to respondents with LAM.

Physical functional capacity in idiopathic pulmonary fibrosis: performance characteristics of the continuous-scale physical function performance test.

Background: Physical functional capacity is impaired in idiopathic pulmonary fibrosis (IPF). There is no tool to measure this key clinical outcome. The continuous-scale physical function performance (CS-PFP) test is one that assesses activities of daily living, but it has never been used in IPF.

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.

A qualitative study of informal caregivers' perspectives on the effects of idiopathic pulmonary fibrosis.

Background: Idiopathic pulmonary fibrosis (IPF) is a life-shortening lung disease that leads to significant morbidity in patients. The devastation IPF imposes extends beyond patients: it affects their spouses, loved ones and any other person who might take on the role of informal caregiver (IC) to the patient.

Objective: The aim of this study was to capture ICs' perspectives on how they are affected by having a loved one with IPF.

Protocol for a mixed-methods study of supplemental oxygen in pulmonary fibrosis.

Background: Little is known about whether or how supplemental oxygen affects patients with pulmonary fibrosis.

Methods/design: A mixed-methods study is described. Patients with pulmonary fibrosis, informal caregivers of pulmonary fibrosis patients and practitioners who prescribe supplemental oxygen will be interviewed to gather data on perceptions of how supplemental oxygen impacts patients.

"Getting stuck with LAM": patients perspectives on living with lymphangioleiomyomatosis.

Background: Lymphangioleiomyomatosis (LAM) is a rare, progressive lung disease that affects almost exclusively women and is most often diagnosed before menopause. The main symptom of LAM is shortness of breath. LAM patients' perceptions of how the disease impacts their lives is largely unknown, but such information could be useful to generate patient reported outcome measures for use in drug trials (or other research studies) and to formulate interventions aimed at easing the burdens LAM imposes on patients.

Patient expectations and experiences in idiopathic pulmonary fibrosis: implications of patient surveys for improved care.

For patients with idiopathic pulmonary fibrosis (IPF), prognosis is extremely poor and treatment options are limited. An improved understanding of the experiences and expectations of IPF patients could lead to better clinical management and patient satisfaction. A review of the literature found that diagnosis of IPF typically involved a protracted course including multiple evaluations.

Assessing exertional dyspnea in patients with idiopathic pulmonary fibrosis.

Background: Dyspnea is a hallmark symptom of idiopathic pulmonary fibrosis (IPF), and dyspnea induced physical activity limitation is a prominent driver of quality of life impairment among IPF patients.

Methods: We examined response data for the 21 physical activity items (the first 21 of 24) from the University of California San Diego Shortness of Breath Questionnaire (UCSD) collected at baseline in a recently conducted IPF trial. We used Rasch analysis and hypothesis testing with conventional statistical methodology to achieve three objectives: 1) to examine the items to identify the one characteristic that distinguishes one from another; 2) to asses these items for their ability to measure dyspnea severity in IPF; 3) to use the items to develop a dyspnea ruler.

Health-related quality of life in idiopathic pulmonary fibrosis: where are we now?

Purpose Of Review: To summarize current knowledge of health-related quality of life (HRQL) assessment in idiopathic pulmonary fibrosis (IPF), with an emphasis on reviewing available data on HRQL instruments, operationalizing HRQL assessment in IPF research and interventions that show promise for improving HRQL in patients with IPF.

Recent Findings: Findings from several studies support the validity of the Medical Outcomes Study Short-Form 36-item Questionnaire and St George's Respiratory Questionnaire for assessing HRQL in IPF. IPF-specific HRQL instruments are in development, but additional research is needed before their broad implementation.