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

Abstract

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. Patients felt that educational resources were inadequate and inaccessible. Overall, patients had reasonable expectations for IPF-specific therapies, and were enthusiastic about trying promising new therapies. Numerous domains of health and functioning identified by patients as important were impaired because of IPF. Existing patient-reported outcome instruments (including assessments of health-related quality of life) do not capture many of these domains or are irrelevant to patients with IPF, highlighting the need for an IPF-specific instrument. Patients treated in centers of excellence expressed greater satisfaction with quality of care and treatments, and also valued the opportunity to interact with other IPF patients.

Keywords::

• health-related quality of life
• idiopathic pulmonary fibrosis
• insterstitial lung disease
• patient expectations
• patient experiences

Financial & competing interests disclosure

In the last 5 years, JJ Swigris has been a paid consultant for Actelion, Boehringer Ingelheim, Gilead, Genentech/Roche, InterMune and UCB Biosciences. The author received or is currently receiving grant funding from InterMune, the LAM Foundation, the NIH and PCORI. In each case, the money goes to National Jewish Health and not directly to JJ Swigris. A Belkin has no competing interests to declare.

Editorial assistance was provided by J Hirsch of BioScience Communications, New York, NY, and was funded by InterMune, Brisbane, CA.

Key issues

• Idiopathic pulmonary fibrosis (IPF) is an interstitial lung disease associated with extremely poor prognosis and for which no treatments are approved in the USA.

• Given the limited therapies currently available for IPF, it is particularly important for clinicians to educate patients about optimal management of the disease and to be aware of patients' experiences and perceptions of IPF from symptom onset, through diagnosis and, ultimately, the end of life.

• Five issues of particular relevance to IPF patients have been identified: timely diagnosis, expectations of treatment, access to educational resources, health-related quality of life and access to tertiary care centers/centers of excellence.

• There is considerable room for improving patients' experiences of being diagnosed and living with IPF, and most patients will benefit from referral to specialized treatment centers.