Title: a QUALITATIVE SURVEY of EUROPEAN PATIENTS with IDIOPATHIC PULMONARY FIBROSIS

Title: A QUALITATIVE SURVEY OF EUROPEAN PATIENTS WITH IDIOPATHIC PULMONARY FIBROSIS: PHYSICAL AND EMOTIONAL IMPACT OF THE DISEASE AND PERCEPTIONS OF CURRENT THERAPY.

Vancheri C. 1, Russell A.M. 2, Maronati M. 3, Giot C 3. 1University of Catania, Catania Italy 2Royal Brompton Hospital, London United Kingdom 3Intermune IAG, Muttenz Switzerland

Objectives: Idiopathic pulmonary fibrosis (IPF) is a rare, progressive, irreversible chronic lung disease, with a median survival time of 2–5 years1-4. This study aimed to explore the impact of IPF on patients at both a physical and emotional level and to evaluate the patients’ experience of pirfenidone, the first approved treatment for IPF.

Methods: Patients with a physician-confirmed diagnosis of IPF and currently on pirfenidone therapy were enrolled in a qualitative survey. One-to-one in-depth interviews of 1 hr duration were conducted by research specialists in three European countries (Germany, Italy, and the United Kingdom) between September 24th and October 19th 2012,

Results: Patients (N=45; 71% male) had a mean age of 68.5 years, with a mean time since diagnosis of 3.5 years. Most patients perceived that IPF caused substantial functional limitations in their lives (56%). Fatigue was reported as the main symptom that generated physical limitations (82%), with loss of appetite, coughing, difficulty lifting objects, and disturbed sleep also cited (Figure 1). At the emotional level, a strong majority of patients experienced fear about the future (72%), while patients also reported frustration and anger (36%), and social isolation (18%) (Figure 2). IPF also impacted family and caregivers. Information about IPF, including the use of pirfenidone in IPF, and updates on new research and treatment for IPF, was sought by 74% of patients. As the first treatment available for IPF, patient’s experiences of pirfenidone therapy were positive overall (average patient satisfaction rating of 7.4 out of 10). Patients experiencing side effects due to the therapy cited needing more reassurance regarding the efficacy profile of the drug.

Conclusions: For the majority of patients, a diagnosis of IPF had a major physical and emotional impact. Psychological support for both patients and their families should become an integral part of the management of the disease. The availability of the first treatment for IPF, pirfenidone, was perceived by patients to offer hope and reassurance for the future, although improved management of side effects is still needed. Clearer information on IPF, drug treatment benefits, and potential side effects should also be made available.

Word count abstract body text: 349 (limit of 350)

Acknowledgements:

The survey was sponsored by InterMune International AG. CV and AMR have received honoraria from InterMune. MM and CG are employees of InterMune. Reference List:

1. Collard HR, King TE, Jr., Bartelson BB et al. Changes in clinical and physiologic variables predict

survival in idiopathic pulmonary fibrosis. Am J Respir Crit Care Med 2003;168:538-542.

2. Kim DS, Collard HR, King TE, Jr. Classification and natural history of the idiopathic interstitial pneumonias. Proc Am Thorac Soc 2006;3:285-292.

3. Meltzer EB, Noble PW. Idiopathic pulmonary fibrosis. Orphanet J Rare Dis 2008;3:8-22.

4. Raghu G, Collard HR, Egan JJ et al. An official ATS/ERS/JRS/ALAT statement: Idiopathic

pulmonary fibrosis: Evidence-based guidelines for diagnosis and management. Am J Resp Crit

Care Med 2011;183:788-824.

Figures:

Figure 1: The main symptoms spontaneously reported by patients with IPF leading to physical limitations Figure 2: The emotional impact of living with IPF spontaneously reported by patients