Abstract
Idiopathic pulmonary fibrosis (IPF) is a chronic, progressive, and fatal lung disease of unknown origin. Despite recent advancements, the diagnosis and management of IPF remains a distinct clinical challenge; as a result, patients often experience considerable delays in receiving an accurate diagnosis and confusion regarding prognosis and the availability of treatment options. In order to gain further insights regarding patients' perspectives on the diagnostic process, disease education, emotional well-being, and quality of life, we conducted a qualitative in-depth survey among IPF patients in Europe. Patients with a physician-confirmed IPF diagnosis were recruited to participate in an in-depth interview conducted by a trained facilitator who used a qualitative topic guide. A total of 45 patients from 5 European countries participated in the survey. The median reported time from initial presentation to confirmed diagnosis of IPF was 1.5 years (range <1 week to 12 years); in 58% of cases there was a delay of >1 year between initial presentation and a confirmed diagnosis of IPF. Additionally, 55% of patients reported consulting ≥3 physicians before receiving an IPF diagnosis. Patient satisfaction with medical care and disease education appeared to be higher among patients who were receiving care at a recognized center of excellence. Patients generally had reasonable expectations regarding the goals of therapy, with most recognizing the irreversibility of the disease and the limited prospects for achieving a cure. The most common unmet needs cited by participants were disease education resources, access to centers of excellence, and familial support programs. Our findings suggest that patients with IPF commonly experience protracted delays in receiving an accurate diagnosis and generally perceive the level of medical care as suboptimal, despite expressing reasonable expectations regarding the goals of therapy. These results support the need for further improvement in the areas of diagnosis, disease management, and patient education.
Introduction
Idiopathic pulmonary fibrosis (IPF) is a chronic, progressive, and universally fatal lung disorder that primarily impacts people in the later decades of life.1–3 It is characterized by reduced lung volumes, impaired gas exchange, and progressively worsening dyspnea that limits and eventually precludes routine physical activity. Due to the nonspecific nature of the typical presenting symptoms, the limited knowledge regarding pathogenesis, and the paucity of available treatment options, the diagnosis and management of IPF represents a distinct clinical challenge. As a result, patients often experience considerable delays in receiving an accurate diagnosis, as well as confusion regarding their prognosis and the availability of treatment options. 4
Despite the gravity of the disease and its devastating impact on patients and caregivers, relatively little is known about patients' attitudes and perceptions regarding the diagnostic process and the clinical management of their disease.
In a survey of patients with pulmonary fibrosis in the United States, two-thirds of the respondents reported a clear lack of information and educational resources at the time of diagnosis, and less than half of the respondents indicated that they felt well informed about important topics like treatment options, the role of supplemental oxygen, pulmonary rehabilitation, and transplantation. 4 Additionally, 55% of respondents reported a ≥1 year delay between initial presentation and the diagnosis of pulmonary fibrosis, and 38% reported consulting ≥3 physicians before the diagnosis was established. A subsequent study employing focus groups and in-depth patient interviews with 20 IPF patients in the United States identified several important IPF-related quality-of-life domains that are not covered by existing instruments, including specific symptoms, therapy, forethought (the need to plan and prepare for routine activities before undertaking them), quantity and quality of sleep, employment and finances, dependence, and mortality. 5 A single physician-based survey examined the presentation, diagnosis, and initial clinical management of IPF patients in Europe; however, patients' attitudes and perceptions were not specifically addressed. 6 Therefore, in order to gain further insights regarding the patient experience among European patients with IPF, we conducted qualitative in-depth interviews with a total of 45 IPF patients from 5 European countries, including Germany, France, Italy, Spain, and the United Kingdom. The main objectives were to generate in-depth insights regarding the patient journey, including symptoms, triggers to seeking medical care, referral patterns, initial diagnoses, follow-up, and current disease management. We also wished to explore the emotions encountered at each stage of this health care ‘journey’ and examine perceived relationships with health care practitioners and caregivers, with the overall aim of identifying unmet needs and opportunities for improving the perceived quality of care.
Doxa Pharma S.r.l. was commissioned to execute the research protocol, which was conducted in accordance with the The International Chamber of Commerce (ICC)/The European Society for Opinion and Market Research (ESCOMAR) International Code on Market and Social Research and other ESOMAR/European Pharmaceutical Market Research Association (EphMRA) professional standards. Data was processed in accordance with Legislative Decree No.196 (30 June 2003), as well as the ESOMAR Standards and the ‘Code of Ethics and Conduct’ issued by the Guarantor of Privacy (16 June 2004).
Methods
Patients with a physician-confirmed diagnosis of IPF were recruited to participate in an in-depth interview conducted by a trained facilitator. Whenever possible, the caregivers were encouraged to participate as well (caregivers participated in 18 [40%] of the 45 interviews). All interviews were performed in the respondents' homes and typically lasted for 1 hour in length or longer. The interviews were conducted using a qualitative discussion guide; participants were asked to recount their IPF-related health care experience in an open and free-ranging manner. The discussion guide was developed after a comprehensive review of the limited relevant medical literature and with input from health care practitioners with expertise in IPF.
In order to encourage open discussion, the interviews were structured to include two projective techniques: an associative technique, whereby respondents are asked to select images that express their feelings; and a constructive technique in which respondents are asked to recall what was said in a particular situation such as the moment that the diagnosis was communicated. 7 These techniques originated in clinical psychology and are commonly used in qualitative research to access responses that would otherwise be inaccessible through direct questioning; they enable respondents to express personal issues freely without feeling threatened by projecting them onto some other person or object and thereby depersonalizing their responses. 8 The choices of images and the output of the constructive task were not considered to have any interpretive value except in the context of what the respondents verbalized on completion of the task. 9
Informed consent was obtained from each subject and family member prior to the interview. Audio recordings were made during each of these interviews and used to assist with accurate and thorough data capture and subsequent analysis only. Audio recordings of the interviews were transcribed and all individual data were then collated in content analysis grids with data from other respondents and presented in aggregate with complete confidentiality maintained for each participant. Researchers from all five counties analyzed the raw data in a collaborative process aimed at identifying the primary themes emerging from the results of the survey.
At the onset of each interview, the moderator read a scripted introduction that described the purpose and nature of the discussion as follows: ‘The overall purpose of this discussion is to understand how your IPF is managed, and what your current needs are. The discussion will take the form of an interactive session, with exercises/tasks which are designed to be enjoyable as well as informative for us; they allow us to access information that we would not be able to access by asking a straight question. In particular, we are interested in understanding not just what you think but why you think it.’
Because of the exploratory nature of the research objectives, wherever possible, attempts were made to elicit spontaneous responses, with prompts being used only as necessary to advance the discussion and to glean the study elements of interest.
Results
Demographics
A total of 45 subjects with a physician-confirmed diagnosis of IPF participated in the survey. The median age of the participants was 67 years (Table 1). The majority of participants (60%) were diagnosed within the preceding 5 years; only 16% (7 of 45) were diagnosed prior to 2000, the year in which the current diagnostic criteria were published. 2 Most participants (76%) reported a history of smoking; however, none admitted to current tobacco usage. The most common comorbid conditions reported by participants were gastroesophageal reflux disease (44%), other pulmonary diseases (29%), cardiovascular disease (27%), and musculoskeletal conditions (11%). A confirmed familial history of IPF was reported by just 1 participant (2%); 5 participants (11%) reported a suspected family history, describing family members who died with IPF-like symptoms but whose death was attributed to other diseases.
Demographics
aChronic obstructive pulmonary disease, emphysema, and pulmonary hypertension.
bFractures, osteoporosis, and secondary osteoporosis due to corticosteroid use.
cArthritis, rheumatoid arthritis, and arthrosis.
The most commonly reported presenting symptoms were dyspnea/shortness of breath with exertion (68%), cough (59%), and fatigue (28%). Other symptoms at presentation included chest pain (6%), finger clubbing (4%), stiffness in joints (2%), and unexplained weight loss (2%).
The median time reported from initial presentation to confirmed diagnosis of IPF was 1.5 years (range, <1 week to 12 years); in 58% of cases there was a delay of >1 year between first consultation of a physician and a confirmed diagnosis of IPF (Table 2). Further, 55% of patients reported consulting ≥3 physicians before receiving an IPF diagnosis.
Although it is clear that each IPF patient’s medical journey and experiences are unique, two distinct routes toward diagnosis were evident. The more common ‘protracted route’ was characterized by an initial dismissal of the presenting symptoms, with repeated physician visits (most often to a general practitioner or internist and occasionally by a cardiologist) for further evaluation and testing. This course, which was reported by 58% (26 of 45) of patients, was commonly interrupted by an ‘acute event,’ which was often initially attributed to other causes, including transient ischemic attack, acute pneumonia, anorexia, and pulmonary embolism. These acute events frequently resulted in an emergency room visit and subsequent hospitalization, where a detailed evaluation by a pulmonologist would eventually result in a diagnosis of IPF. This process reportedly took as long as 2–12 years, despite repeated visits to health care practitioners during this period.
Patients who were subjected to this protracted route to diagnosis were often critical of the care they received, citing both a lack of empathy and emotional support and an apparent lack of competence among health care practitioners (Figure 1 ). There was a tendency among these patients to perceive the initial diagnosis not as a working hypothesis but rather as an erroneous or missed diagnosis. Commonly reported initial ‘misdiagnoses' included asthma, chronic obstructive pulmonary disease (COPD), pneumonia, pertussis, pleurisy, and pericarditis.
Representative responses from patients who experienced a protracted route to a confirmed diagnosis of idiopathic pulmonary fibrosis.
In a minority of cases (16%), the diagnosis was made within a month of the patient’s initial presentation. In such cases, early detection was attributed to a well-informed patient researching their symptoms online (including one patient who had a sibling who died of IPF), a well-informed physician detecting the distinctive ‘Velcro® rales' on chest auscultation, which prompted further evaluation for possible interstitial lung disease, or routine surveillance of known drug toxicities (e.g. amiodarone). Predictably, perceptions regarding the quality of care among patients who received a prompt diagnosis were generally better than those of patients who experienced a protracted route to diagnosis. Patients in both groups, however, expressed dissatisfaction with the manner in which the diagnosis was divulged, citing insensitivity on the part of the health care practitioner and insufficient time during the consultation to address the full range of patients' questions and concerns (Figure 2 ).
Representative responses from patients when asked to comment on the manner in which the diagnosis of idiopathic pulmonary fibrosis was divulged by their health care practitioner.
While patients' initial attitudes and perceptions were influenced by the path to diagnosis, post-diagnosis perceptions regarding the quality of care were dependent on the setting in which the care was received. Marked differences in patient attitudes and perceptions were observed between patients who were treated in a recognized center of excellence and those who were treated in a community practice setting; patients treated in a tertiary care center consistently reported greater satisfaction with the quality of care, the availability of treatment options (including enrollment in a clinical trial), the knowledge and expertise of health care practitioners, and the frequency of follow-up visits and routine monitoring. Additionally, patients treated in a tertiary care center commonly reported that the opportunity to interact with other IPF patients provided important benefits, including psychological support and practical disease management tips. Patients treated in the community practice setting consistently reported infrequent follow-up visits (typically once per year), short duration of visits (generally less than 10 minutes), and a lack of available treatment options. In general, these patients were less well informed about their disease and the available treatment options, including pulmonary rehabilitation, lung transplantation, and enrollment in a clinical trial.
Patients generally expressed realistic expectations of the therapies availed to them, with most recognizing that there was no prospect for disease reversal. Personal aims were typically focused on disease stability and efforts to slow progression if feasible. For only a small minority of those surveyed, the emphasis was either on lung transplantation as the ‘hope’ for future survival beyond IPF, or some belief that their particular condition was atypical and associated with a less dire prognosis.
There were a number of additional common unmet needs identified by the participants (Figure 3 ). These included improved diagnostic techniques, improved access to ‘centers of excellence,’ more effective treatment options, increased availability of patient education resources, and fewer bureaucratic barriers to scheduling specialist appointments and obtaining supplemental oxygen. Lastly, many participants referred to the need for the establishment of a patient-based advocacy group that could more efficiently and effectively represent the needs of IPF patients and serve as a resource for ongoing patient education and support.
Commonly reported unmet medical needs among patients with idiopathic pulmonary fibrosis.
Impact of IPF on Patients' Quality of Life and Emotional Well-Being
Patients reported a deep and wide-ranging negative impact of IPF on the quality of daily life in three broad areas: personal independence, relationships with others, and financial status. Patients described a gradual loss of personal independence that coincided with the deterioration in health and the increasing inability to perform routine daily tasks. Additionally, the requirement for supplemental oxygen was commonly identified as a milestone in the patients' loss of independence, as it is at this point that the disease becomes highly visible to others and excursions outside the home begin to require significant logistical planning. In many cases, this loss of independence has a notable impact on the patient’s emotional well-being, as they begin to perceive themselves as a burden to both their family and society (Figure 4 ).
Representative responses from patients when asked about the effect of idiopathic pulmonary fibrosis on social relationships and emotional well-being.
Patients also expressed considerable difficulty in continuing relationships with friends and acquaintances due to their worsening pulmonary status and immobility, as well as a general lack of awareness and understanding of the disease.
Finally, although health care in the European Union is generally free, approximately 20% of respondents reported financial difficulties as a result of their inability to work and the consequent reduction in income. This further served as a stressor, as well as the concern that they were now an increasing burden to their families and loved ones.
Discussion
A diagnosis of IPF carries a uniformly poor prognosis and has devastating consequences for patients and their caregivers. While several recent studies have examined health-related quality of life in patients with IPF, only one previous study has systematically examined patients' attitudes and perceptions regarding the quality of care and ongoing unmet needs. 4 In the present study, we conducted in-depth qualitative interviews of IPF patients from five European countries in order to gain further insights regarding patients' perspectives on the diagnostic process, disease management, educational resources, emotional well-being, and quality of life.
Our survey yielded several important observations. First, despite recent advancements in imaging and surgical techniques and the publication of consensus guidelines for the diagnosis of IPF, 2 the diagnostic process remains complex and fraught with challenges. Consistent with the findings of a recent survey of IPF patients in the United States, 4 58% of patients in our survey reported a delay of more than 1 year between initial presentation and a confirmed diagnosis of IPF, and more than half reported consulting ≥3 physicians before receiving an IPF diagnosis. Second, while patients' attitudes and perceptions regarding the quality of care were initially influenced by the duration of the diagnostic process, perceptions post-diagnosis were almost uniformly dependent on the setting in which the care was provided. Patients treated at a recognized center of excellence consistently reported greater satisfaction with the quality of care than those who were treated in a community practice. Among the factors influencing the higher satisfaction among patients in the tertiary care setting were more frequent follow-up visits, longer duration of visits, availability of treatment options, and the ability to interact with other IPF patients. Third, despite the perceived lack of adequate educational resources, patients generally had reasonable expectations regarding the goals of therapy, with most recognizing the irreversibility of the disease and the limited prospects for achieving a cure. Taken together, these findings have potentially important implications for the delivery of care to patients with IPF and suggest that patient experiences can be substantially improved by frank and empathetic discussions regarding the nature of the disease and the various therapeutic options, regular follow-up visits, and patient support groups that allow for interaction with others who share similar challenges.
The results of the survey also highlight several unmet needs among IPF patients in Europe. As noted previously, patients consistently reported a need for quality educational resources that are accessible to patients and caregivers. Current resources were generally perceived to be limited or inadequate, with a tendency to be overly alarmist and lacking in practical information related to disease management and coping skills. Additionally, several patients identified a need for patient-based advocacy groups to lobby pharmaceutical, medical, and government organizations and serve as a resource for ongoing education and support. Finally, participants consistently identified a need for increased general awareness and understanding of IPF among health care professionals, as limited awareness was frequently cited as a contributing factor to the protracted delays in receiving an accurate diagnosis.
There are several important limitations to our survey. First, while only patients with a physician-confirmed IPF diagnosis were included in the survey, we did not have access to patient records and therefore could not confirm the diagnosis on the basis of the current clinical, radiographic, and pathologic criteria 10 . Additionally, results based on the ability of respondents to accurately recall detailed historical information are subject to considerable error and therefore introduce the potential for bias. These limitations, coupled with the limited sample size, undoubtedly limit the generalizability of the reported findings. Despite these limitations, we believe the results of this survey provide important insights regarding the experience of European patients with IPF and highlight several important unmet needs, including better and more widely accessible educational materials, enhanced communication between health care practitioners and patients, and the creation of patient support and advocacy groups.
In conclusion, our findings suggest that patients with IPF commonly experience protracted delays in receiving an accurate diagnosis and generally perceive the level of medical care as suboptimal, despite expressing reasonable expectations regarding prognosis and the goals of therapy. While these results suggest a need for substantial improvements in the care of patients with IPF, considerable improvements in the patient journey can be made through the implementation of easier access to centers of excellence once a diagnosis of IPF is being considered, more substantial support of educational programs as well as more widely integrated advocacy groups throughout Europe.
Footnotes
Acknowledgements
We would like to thank all the IPF patients, along with their primary caregivers and families for their assistance and participation in this research effort. We would also like to thank Dr Roberto Cassandro of the Ospedale San Giuseppe in Milan, Professor Carlo Vancheri of the Università degli Studi di Catania, Dr Liberatore Giuseppe Monaco of the Ospedale San Giovanni in Rome, Dr Thierry Jeanfaivre of the Centre Hospitalier et Universitaire d’Anger, Dr Geneviève Letanche of the Portes du Sud clinic, Dr Gérard Durand of the Champeau Clinic, Dr J-Pierre Dussol of Le Rabelais Medical Centre, Dr Charles Alain Montantin as well as the following patient organizations: pulmonaryfibrosis.org.uk, Pulmonary Fibrosis UK-Yahoo Group, lungenfribose.de.
This research was supported by InterMune, Inc.