IPF Care: A Support Program for Patients with Idiopathic Pulmonary Fibrosis Treated with Pirfenidone in Europe

Abstract

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.Electronic supplementary materialThe online version of this article (doi:10.1007/s12325-015-0183-7) contains supplementary material, which is available to authorized users.

An Open-Label Study of the Long-Term Safety of Pirfenidone in Patients with Idiopathic Pulmonary Fibrosis (RECAP)

“…These events occurred most frequently during the first year of treatment and in patients who were newly initiating pirfenidone (data not shown), suggesting that patients must be carefully monitored during this early treatment period to reduce the risk of discontinuation. Patient support programs have shown that early and careful management reduces the rate of discontinuations [18]. While the overall discontinuation rate is higher than in the shorter phase 3 trials, RECAP was a study conducted over several years, and IPF worsening did not appear to be the main driver for treatment discontinuations.…”

Liposomal hydrogel formulation for transdermal delivery of pirfenidone

“…The marketed product of PFD (Esbriet) is available as an oral solid dosage form against IPF (Duck et al, 2015). The frequency of administration is three times a day because of the lower half-life and high elimination rate (Lou et al, 2012).…”

“…PFD has been found to down regulate tumor necrosis factor-a (TNF-a) expression and transforming growth factor-b (TGF-b) (Jung et al, 2012). PFD has been approved for the treatment of IPF in European Union (2011), Canada (2012) and the United States (2014) (Duck et al, 2015).…”

Treatment of idiopathic pulmonary fibrosis in Australia and New Zealand: A position statement from the Thoracic Society of Australia and New Zealand and the Lung Foundation Australia

“…However, in qualitative interviews, patients reported benefits including reduced isolation, putting the disease in perspective and providing comfort. An alternative model is IPF Care, which provides support and education for patients receiving pirfenidone in Europe, funded by the supplier . Participants receive regular telephone calls and/or home visits from nurses, as well as individually tailored information booklets.…”

“…Although a major function of the programme is to support treatment adherence, 30% of topics discussed were not related to pirfenidone and included oxygen therapy, test results, home supports, exercise, transplantation and concerns for the future. Retention in the programme was 71% over 18 months and patient‐reported satisfaction was high …”