Patient expectations andexperiences in idiopathicpulmonary fibrosis:implications of patientsurveys for improved careExpert Rev. Respir. Med. 8(2), 173178 (2014)

Amanda Belkin andJeffrey J Swigris*Autoimmune Lung Center and

Interstitial Lung Disease Program,

National Jewish Health, CO, USA

*Author for correspondence:

Tel.: +1 303 398 1621

swigrisj@njc.org

For patients with idiopathic pulmonary fibrosis (IPF), prognosis is extremely poor and treatmentoptions are limited. An improved understanding of the experiences and expectations of IPFpatients could lead to better clinical management and patient satisfaction. A review of theliterature found that diagnosis of IPF typically involved a protracted course including multipleevaluations. Patients felt that educational resources were inadequate and inaccessible. Overall,patients had reasonable expectations for IPF-specific therapies, and were enthusiastic abouttrying promising new therapies. Numerous domains of health and functioning identified bypatients as important were impaired because of IPF. Existing patient-reported outcomeinstruments (including assessments of health-related quality of life) do not capture many ofthese domains or are irrelevant to patients with IPF, highlighting the need for an IPF-specificinstrument. Patients treated in centers of excellence expressed greater satisfaction with qualityof 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 . patientexpectations . patient experiences

Idiopathic pulmonary fibrosis (IPF) is aninterstitial lung disease characterized by pro-gressive parenchymal fibrosis and ventilatoryrestriction and is associated with extremelypoor prognosis [13]. It affects approximately1 in 1000 Americans aged >65 years [2,4]. Inthe USA, there is no US FDA-approved ther-apy for IPF, and many of the pharmacologi-cal approaches used with regularity in thepast were associated with significant toxic-ities. Nearly, every patient with IPF willeventually require supplemental oxygen and,even with oxygen, patients experience relentlessprogression of dyspnea and severe limitationsin physical activities without the prospects ofany treatment to reverse the disease.To ensure whether patients with IPF receive

the disease education they need and are awareof suitable, acceptable therapeutic and psycho-social interventions, clinicians need to be awareof patients experiences and perceptions of IPF

from symptom onset, through diagnosis and,ultimately, the end of life. Our goal was tobetter understand these experiences and per-ceptions across the spectrum of IPF severity.To accomplish this goal, we conducted aPubMed literature search to identify studies inwhich investigators captured patients percep-tions of what it is like to live with IPF. Searchterms included the following: interstitial lungdisease, IPF, treatment, management,expectations of treatment benefit, patientsurveys, patient expectations, patient experi-ence, patient treatment satisfaction, patienteducation and quality of life. Althoughquality of life was a search term, this paper isnot a systematic review of quality of life stud-ies; rather, we focused on papers that alsoaddressed psychosocial components of patientcare. This search captured five key studies(TABLE 1) [59]. We identified five issues as beingof particular relevance and importance to the

informahealthcare.com 10.1586/17476348.2014.880056 2014 Informa UK Ltd ISSN 1747-6348 173

Review

IPF patient population: timely diagnosis, expectations of treat-ment, access to educational resources, health-related quality oflife (HRQoL) and access to tertiary care centers/centers ofexcellence. In the remainder of this review, we delve deeperinto these issues and suggest opportunities for improved man-agement of patients with IPF.

Timely diagnosisStudy findings

Diagnosis of IPF requires exclusion of other known causes offibrosing interstitial lung diseases and, given the demographicthat most commonly develops IPF (age: ~65 years), other morecommon causes of exertional dyspnea need to be consid-ered [5,6,10]. Patients typically report a very protracted timebetween initial presentation with IPF symptoms and receipt ofan IPF diagnosis, with the diagnosis frequently requiring inter-action with multiple physicians. In a qualitative study of in-depth interviews with 45 patients with IPF from five Europeancountries, Schoenheit and colleagues [5] reported that themedian time from initial presentation to confirmed diagnosiswas 1.5 years (range: 1 year in 58% of patients and 3 years in 29%. Overall, 55%of patients consulted 3 physicians before receiving a diagnosisof IPF [5].Two distinct routes to IPF diagnosis rapid and

protracted were observed [5]. The more frequent, protractedroute generally included the supposition that the presenting

symptoms were not markers of a life-threatening illness,followed by repeated physician visits (usually to a general prac-titioner, internist or cardiologist) for additional evaluation andtesting. This course was frequently interrupted by an acuteevent, often attributed to other causes (e.g., acute pneumonia,pulmonary embolism), which resulted in an emergency depart-ment visit, hospitalization and subsequent evaluation by a pul-monologist who considered the diagnosis of IPF. Commoninitial diagnoses included asthma, chronic obstructive pulmo-nary disease, pneumonia, pertussis, pleurisy and pericarditis.Patients with a protracted route to diagnosis were often criticalof the care they received, citing a perceived lack of empathy,emotional support and competence on the part of practitioners.Patients who received a relatively prompt correct IPF diagnosiswere generally more satisfied with their quality of care com-pared with those subjected to long diagnostic delays. Bothgroups, however, expressed general dissatisfaction with themanner in which the diagnosis was divulged, frequently citinginsensitivity on the part of physicians making the diagnosis anda lack of adequate time at clinic visits to address their questionsand concerns.In a survey of 1448 US patients with pulmonary fibrosis

reported by Collard and colleagues [6], 55% reported a 1-yeardelay between symptom onset and pulmonary fibrosis diagno-sis. The most frequently reported initial diagnoses includedbronchitis (19%), asthma (14%), chronic obstructive pulmo-nary disease (8%), emphysema (7%) and heart disease (5%).

Table 1. Five key surveys capturing idiopathic pulmonary fibrosis patients experiences/perspectives.

Study (year) Type Patients (n) Instruments Ref.

Schoenheit et al.(2011)

Qualitative in-depth survey of IPF

patients in five European countries,

designed to gain insight on patients

perspectives on diagnostic process,

disease education, emotional well-

being and HRQoL

45 In-depth interview conducted by trained

facilitator who used qualitative discussion

guide developed after comprehensive review

of relevant medical literature and with input

from healthcare practitioners with IPF

expertise

[5]

Collard et al.

(2007)

Survey of experiences of patients

diagnosed with pulmonary fibrosis,

focusing on patient education and

resources

1448 Survey of 52 defined choice and open-ended

questions developed in conjunction with

clinicians experienced in diagnosis and

management of pulmonary fibrosis

[6]

Swigris et al.(2005)

Focus groups and individual in-depth

interviews with IPF patients to collect

their perspectives on how IPF affected

their lives (focus on HRQoL)

20 Set of questions based loosely on Flanagans

Quality of Life Scale; domains compared with

WHOQoL-100, SF-36TM

Health Survey, CRQ,

and SGRQ

[7]

De Vries et al.

(2000)

Focus groups of IPF patients to identify

relevant aspects of HRQoL or health

status

10 CRQ, SQRQ, WHOQoL-100 [8]

De Vries et al.

(2001)

Study assessing HRQoL in IPF patients,

and relation between HRQoL,

depressive symptoms and

breathlessness in this population

41 IPF patients;

41 healthy

controls

WHOQoL-100; Beck Depression Inventory;

Bath Breathlessness Scale; social support

questionnaire; questionnaire concerning

perceived seriousness of illness

[9]

CRQ: Chronic respiratory questionnaire; HRQoL: Health-related quality of life; IPF: Idiopathic pulmonary fibrosis; SGRQ: St. Georges Respiratory Questionnaire;WHOQoL-100: WHO Quality of Life assessment instrument-100.Data taken from [59].

Review Belkin & Swigris

174 Expert Rev. Respir. Med. 8(2), (2014)

Overall, 38% of patients reported seeing three or more physi-cians before being given a diagnosis of IPF.

Clinical implications

Diagnosing IPF can be complex and challenging: it is a rela-tively rare disease; early in the disease course, commonly usedtests to evaluate respiratory health (e.g., chest radiography,spirometry and resting peripheral oxygen saturation) may benormal; and the demographic of patients ultimately diagnosedwith IPF either have or are at risk for other conditions thatmay present with similar features (e.g., emphysema, cardiacischemia). The protracted delays in accurate diagnosis [5,10] willbe avoided only if IPF is on the radar of potential diagnosesfor patients presenting with the insidious onset of exertionaldyspnea or nagging, dry cough and if clinicians, radiologistsand pathologists are given the opportunity to interact in mean-ingful ways around a cache of patient data [11].The studies we identified regarding patient experiences with

diagnosis were published prior to the revised 2011 guidelinesfor IPF diagnosis issued jointly by the American Thoracic Soci-ety, European Respiratory Society, Japanese Respiratory Societyand Latin-American Thoracic Association [12]. It is possible thatthese updated and simplified criteria will allow for more confi-dent and rapid IPF diagnosis in the future [10], providingpatients with greater opportunity to access adequate informa-tion on the disease, timely referral to a tertiary care center,avoidance of inappropriate drug treatments and better access toclinical trials and new treatments [13,14].

Patient expectations of therapyStudy findings

Treatments prescribed

At the time patients completed the survey by Collard and col-leagues [2], 75% of those with pulmonary fibrosis reported tak-ing pharmacological therapy, including prednisone in 50%(21% in the past), cyclophosphamide in 3% (6%), azathioprinein 10% (9%), colchicine in 6% (7%), interferon-g-1b in 21%(8%) and herbs or nutritional supplements in 24%. Supple-mental oxygen was being used by 61% of patients, and aminority had been referred to pulmonary rehabilitation (32%)or physical therapy (24%). Transplantation had been discussedwith 59% of patients. The most common reasons for notreceiving pharmacologic therapy were fear of side effects(26%), presumed ineffectiveness of therapy (23%), no treat-ment offered (23%) and early or stable disease (22%). Thisstudy was conducted at a time when combined therapy withprednisone and a cytotoxic agent was more commonplace [15].

Patients attitude toward treatment

Schoenheit and colleagues [5] found that patients with IPFgenerally had reasonable expectations regarding goals of ther-apy, with most recognizing the irreversibility of the diseaseand limited prospects for achieving cure. Patients were typi-cally focused on slowing disease progression or maintainingstability, if feasible. These expectations are generally in line

with international guidelines that, while not specifying thera-peutic goals for IPF treatment, emphasize the importance ofmonitoring disease progression including worsening dyspnea,progressive fibrosis, worsening oxygenation and acute exacer-bation [12]. For a minority of patients in the Schoenheit sur-vey, the emphasis was on lung transplantation as a hope forlonger survival or with the belief that in their specific case,the course would be atypical and associated with a lessdire prognosis.In a study of 20 US patients with IPF, Swigris and col-

leagues [7] observed that most perceived the then-conventionalIPF therapies as being difficult to tolerate and, in many ways,worse than the disease itself, given the low likelihood of abeneficial response and frequent adverse events. Those patientswho stopped taking certain conventional therapies were glad toget rid of medications; however, almost all patients were will-ing to be a medical guinea pig by taking novel experimentaltherapies.

Clinical implications

The study by Collard and colleagues [6] suggests that patientswith IPF and their physicians are increasingly opting to avoidtherapy with potentially toxic conventional therapies. Webelieve it is crucial that clinicians do not view IPF with a senseof therapeutic nihilism, and that they conduct thoughtful dis-cussions about pharmacological and nonpharmacological ther-apy at each encounter. Although IPF is a life-shortening illness,patients may benefit from any number of therapeutic interven-tions (e.g., supplemental oxygen, pulmonary rehabilitation) andthey will definitely benefit from the sense of partnership theyfeel with their clinicians. Most patients want to know the truthabout their illness and the expected response to therapeuticinterventions; however, clinicians caring for patients with IPFneed to strike a balance between truth telling and projecting anair of hopelessness [16].Finally, it is important to be aware that patients who receive

diagnoses of chronic illness may find their emotional reactionmore disabling than the illness itself [17]. Not surprisingly, dataindicate that depression symptoms are found in approximately25% of patients with IPF [9]. Care of patients with IPF shouldinclude mechanisms and resources for diagnosing and treatinganxiety and depressive symptoms.

Table 2. Time from initial presentation to confirmedidiopathic pulmonary fibrosis diagnosis.

Time Patients (n; %)

Patient education resourcesStudy findings

Patient education including an understanding of disease patho-physiology, prognosis and trajectory represents a key componentof care for patients with IPF [18]. The majority of these patients,however, either somewhat or strongly agreed that there was a lackof information/resources about IPF available at the time of theirdiagnosis [5,6]. The available resources were felt to be limited andinadequate, alarmist and lacking in information on disease man-agement and coping skills. Only half of patients reported beinggenerally or very well informed about IPF treatment options;fewer reported being generally or very well informed aboutthe benefits of pulmonary rehabilitation, managing supplementaloxygen, nutrition and the risks/benefits of lung transplantation.

Clinical implications

There is a clear need for better patient/caregiver IPF educationalmaterials and access to them. Potential educational approachescan include booklets, Web-based content and workshops tailoredtoward patient needs. There are a number of these approachesavailable, including those developed by patient advocacy groupslike the Pulmonary Fibrosis Foundation and Coalition for Pul-monary Fibrosis. We encourage clinicians who care for patientswith IPF to direct them to either organizations Website [19,20],where they can obtain trustworthy educational materials.

Health-related quality of lifeStudy findings

It is clear that IPF significantly affects HRQoL. Schoenheit andcolleagues [5] found that patients experienced a loss of personalindependence in conjunction with declining health status and areduced ability to perform activities of daily living. Patients fre-quently cited the need for supplemental oxygen as a milestonein their loss of independence since it both made the disease visi-ble to others and made leaving the house more difficult. Forsome patients, inability to work and reduction in income causedfinancial difficulties, a stressor exacerbated by the concern ofbecoming an increasing burden on families and loved ones.Similarly, Swigris and colleagues [7] found that symptoms(shortness of breath and cough) significantly impaired HRQoL,with breathlessness being associated with extreme distress andcurtailment of physical activity. Exhaustion and overwhelmingfatigue were prominent and reported to be as bad as breathless-ness by some patients. The prospect of disease progression madepatients sad and fearful, particularly of becoming more depen-dent on loved ones. Loss of privacy due to dependence onothers and frustration over the likelihood of becoming a physi-cal or financial burden were common concerns. The need forsupplemental oxygen and distress over the oxygen tanks as a vis-ible indicator of disability limited patients willingness and abil-ity to leave home, participate in social activities or travel.

Clinical implications

Although dyspnea and dyspnea-induced limitations in physi-cal activity, in particular is the primary driver of HRQoL

impairment among patients with IPF, cough or exhaustion canbe equally (or more) debilitating. Like other patients withchronic illness, those with IPF are at risk for developing anxietyand feelings of isolation or hopelessness. Clinicians caring forpatients with IPF should ask about these feelings at everyencounter and be quick to treat or refer them to otherclinicians with the necessary expertise.

Access to centers of excellenceStudy findings

Patients with IPF treated in a community setting appeared tohave different attitudes and perceptions from those treated in arecognized center of excellence [5]. Patients treated in a center ofexcellence reported higher levels of satisfaction with their treat-ment options (including enrolment in clinical trials), the knowl-edge and expertise of healthcare practitioners, quality of care andfrequency of follow-up visits and routine monitoring. Thesepatients also noted that interaction with other patients with IPFhelped to provide psychological support and allowed them toshare practical disease management tips. In contrast, patientstreated in the community setting said they felt poorly informedabout IPF and the treatment options available to them. Many ofthese patients also noted that follow-up visits did not occur fre-quently enough (usually once yearly), visits with clinicians weretoo short (typically

inadequately educated about many aspects of the disease. Mak-ing the diagnosis of IPF and managing IPF patientslongitudinally is probably best undertaken at a center withexpertise in these areas. Data suggest that doing so leads toimproved patient satisfaction. Ongoing efforts by patient advo-cacy groups are reliably informing patients and their caregiversabout what to expect as IPF progresses and alerting them tobeneficial resources. All patients with IPF should be encouragedto participate in a pulmonary rehabilitation program and untilan effective drug is approved in the US offered the opportu-nity to participate in research studies and/or drug trials.

Five-year viewThe span of the next 5 years will be an exciting time for theIPF community. Data from Phase III trials of pirfenidone,N-acetyl cysteine and nintedanib will add to our understanding

of the disease. Depending on the results, we may see the firstdrug approved for IPF by the FDA. We anticipate that trialsof antifibrotic drugs in combination will be planned and IPF-specific patient-reported outcome measures will be fully devel-oped to capture outcomes meaningful to patients.

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.

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c.17476348.2014.880056_20780.pdf17476348.2014.880056.pdfTimely diagnosisStudy findingsClinical implications

Patient expectations of therapyStudy findingsTreatments prescribedPatients' attitude toward treatment

Clinical implications

Patient education resourcesStudy findingsClinical implications

Health-related quality of lifeStudy findingsClinical implications

Access to centers of excellenceStudy findingsClinical implications

ConclusionExpert commentaryFive-year viewFinancial & competing interests disclosure