Lived experiences of patients with idiopathic pulmonary fibrosis: navigating through the complex healthcare system

Background : Over the last decade, idiopathic pulmonary fibrosis (IPF) has been attracting health care professionals’ attention worldwide due to its impact on poor survival rate and quality of life, such as ongoing physical and emotional distress experienced by patients and their families. The diagnosis and management of IPF patients often remain as a significant challenge for clinicians. Therefore, it is imperative to gain an in-depth understanding on experiences of IPF patients during their disease journey in order to improve the care delivered to this vulnerable group. The aim of this study was to explore the lived experiences of the disease journey and care needs of patients with IPF. Methods : A semi-structured face-to-face one on one interview was conducted with a purposive sampling of 16 IPF patients admitted to a respiratory medicine department of a tertiary hospital in Beijing, China. The consolidated criteria for reporting qualitative research checklist was used. Results : Four themes emerged from the qualitative data included the long and confusing journal to reach diagnosis, living with the disease, understanding the disease and treatment, and desire for continuity of care. Conclusions : The findings provide an in-depth understanding of the lived experience of the disease journey and the care needs in patients with IPF. There is an urgent need to improve the care delivery to this vulnerable population. To meet the health demands of this group of patients, it is of paramount importance to develop effective education programs for health professionals and IPF patients and also to improve the care models of healthcare systems, especially in the remote area in order to enhance the principle of continuing care for IPF patients in the communities. diagnosis for many participants. Two

IPF). Interstitial Lung Disease Group of the Chinese Thoracic Society (2004) revealed that 56 ILD cases were admitted to 10 hospitals in Beijing in 1990, accounting for 1.98% of yearly hospitalized cases, while the case load reached 485 in 2003, accounting for 4.66% of yearly hospitalized cases [7]. A survey conducted in ten hospitals in Tianjin province in China showed that hospitalized ILD cases accounted for 4.5% of all hospitalized cases in 2003. This rate rose to 39.5 % in 2009 [8]. This indicates the increased prevalence of patients with ILD (including IPF) in Chinese population.
It is suggested that there is an increased financial cost of IPF treatment and poor prognosis with a median survival rate between 3-5 years from the time of diagnosis to the death [4,9,10]. The diagnosis and management of IPF patients often remain as a significant challenge for clinicians. The evidence in the literature has suggested that patients with IPF often experience ongoing symptoms of dyspnea, dry coughs, sleep disturbance and emotional distress [2,11]. Such symptoms often result in loss of independence and restriction of daily activities [11][12][13]. As such, this group of patients often has poorer quality of life compared to patients with other types of respiratory diseases [14]. Despite the increasing incidence rate and poor quality of life, IPF is often misdiagnosed and managed inappropriately [15]. It has been reported that, in European country, there is usually 2-5 years delay in reaching a correct diagnosis for IPF patients [12,16]. Undoubtedly, this delay causes distress and adds frustration to patients and their families. Insufficient information and support for patients with IPF were also reported in some studies [12,17]. Current literature recommends that management of IPF patients should focus on slowing down the progression of lung fibrosis, which in turn helps to extend patients' life expectancy and improve their quality of life [18].
There have been some studies describing the perceptions from patients' experiences of living with IPF in European countries [12,17,19]. Still, there has been a paucity of research exploring the lived experience of patients with IPF, especially in Chinese population. Given the complex nature of IPF disease journey, there is a need to gain an in-depth understanding of the lived experiences with regards to their disease journey and care needs on Chinese patients with IPF.

Aim
This study aimed to explore the lived experiences of the disease journey and the care needs of Chinese patients with IPF.

Study design
A phenomenological approach was chosen to guide this study [20]. Phenomenology is a qualitative research method that seeks the narratives of the lived experiences of a certain phenomenon. It is considered the most appropriate methodology to explore the way people experience and interpret the world in which they live [21]. Individual interview was used as data collection method.

Setting and participants
Hospitals are classified as a 3-tier system in China, including primary-level, secondary-level and tertiary-level facilities. The 3-tier system recognizes a hospital's bed capacity and ability to medical treatments, education and research. Patient acuity is higher at the tertiary-level facility as it offers care to patients with complex healthcare needs.
This study was conducted in a 44-bed department of respiratory medicine in a 1400-bed tertiary hospital in Beijing, China. Inclusion criteria were: 1) patients with confirmed IPF diagnosis; 2) no psychiatric history; and 3) able to speak and understand Mandarin. Purposive sampling approach was used. Potential participants were identified via respiratory physicians in the respiratory department.
The researcher then recruited eligible participants.

Data collection
Between August 2018 and February 2019, an in-depth one on one face-to-face semi-structured interview was conducted with 16 participants in a private and quiet room in the hospital. The interviews were audio-recorded and ranged from 50-60 minutes. The interviews were conducted by the first author (YL) who is a female registered nurse and an experienced qualitative researcher with a master's degree in nursing. YL was a research nurse in this respiratory department who did not provide direct care to the patients being interviewed at the time of study. Prior to study commencement, YL established a rapport with the team leader and patients by attending and observing daily routine nursing care on the ward. Participants were fully informed of the nature of this qualitative study (i.e. background, aim and the audio recording) prior to signing the consent forms.
Participants were asked to fill out a demographic data form, which included gender, age, education level, occupation, long-term residence (city/countryside), time period from initial symptom to confirmed diagnoses.
Interview questions were based on pervious published literature and the researchers' experiences.
The interview started with a broad question: "Could you tell me your experiences regarding how you were diagnosed with IPF?" Participants were encouraged to use their own words and tell their own story in an open and free manner. The topic covered but was not limited to their experiences of diagnosis, their perception of the disease, treatment options, their experiences in living with the disease, and their care needs. Prompt questions were used, such as "then, what happened?" "Could you please tell me how that affected you?" "What were your thoughts and feelings at the time?" The interviewer also recorded patients' meaningful facial expressions and body language. The patients were told that the interview could be stopped at any time if needed. If the patients felt breathless, anxious or tired during the interview, they could ask to stop the interview and or choose to continue the conversation at another time. No participants stopped the interviews or withdrew from the study.
The data collection was ongoing until there was no evidence of recurring themes that emerged form the data.

Data analysis
Thematic analysis was conducted for the qualitative data [22]. The interview audio recordings were transcribed verbatim into Chinese and translated to English by the researcher (YL) who is bilingual. Braun and Clarke's (2006) six-step thematic data analysis guided the process: 1) familiarizing with the data; 2) generating initial codes; 3) searching for themes; 4) reviewing themes; 5) defining and naming themes; and 6) producing the report [22]. Two researchers (YL and YRJ) independently read and reread all the transcripts for immersion in the data. Key quotes were highlighted, and initial codes were developed based on participants' verbatim statements. Codes were grouped according to similarity into subthemes and then themes based on common threads throughout the data. Any disagreement or contested theme/subtheme was discussed within the team with all researchers (YL, YRJ, FLG, YLH, FL) until consensus was reached. Interview transcripts were returned back to participants for comments and further clarifications.
The reporting of this research follows the Consolidated Criteria for Reporting Qualitative Research (COREQ) [23] (please see Supplementary Material S1).
Supplementary Material S1

Ethics
The Human Research and Ethics Committee of the hospital approved the study (ethics number: 2018-8-23-1). All participants were fully informed of the nature of this study, including the background, aims and benefit and harms prior to the commencement of this study. Written consents were obtained before the interviews.

Trustworthiness
Methodological trustworthiness of a qualitative study could be guaranteed by evaluating credibility, dependability, confirmability and transferability [24]. To strengthen credibility, regular discussions within the research team (YL, YRJ, FLG, YLH, FL) were held with regards to the emerging codes, subthemes and themes. All researchers (YL, YRJ, FLG, YLH, FL) have knowledge of conducting qualitative research (i.e. study design, data collection and analysis, skills of conducting and organizing in-depth interview). Three authors (YL, YRJ and YLH) have had extensive clinical experiences, especially in respiratory area. In terms of dependability, regular open discussions were held with all researchers (YL, YRJ, FLG, YLH, FL) to discuss the similarities or differences of the interview contents, which may change during the collection of interview data. Transferability was ensured by providing a detailed description of the healthcare context in China and characteristics of participants in this study. As for confirmability, a paper trail was kept recording significant ideas or incidents emerging from the conversations with the participants. Participants were also asked by the interviewer (YL) to provide their feedback on the interview transcripts for further clarifications.

Results
16 patients participated in this research, with 13 males (81.3%) and 3 females (18.7%). Participants' age ranged from 35 to 72 years old (mean age was 59 years). Diagnosis journey (from initial symptoms to confirmed IPF diagnosis) was between 1.5-5 years with an average of 2.9 years. Around one third of participants had high school education, followed by primary and secondary schools. Only 3 participants had university education. Table 1 shows the characteristics of participants with IPF.   Table 2 shows the themes and associated sub-themes. There was a 3 to 4 years delay of being confirmed with the IPF diagnosis for many participants. Two participants from the countryside had more than 5 years delay. They looked for further medical advice at several hospitals. Some participants underwent multiple and repeated diagnostic tests and procedures, such as blood, X-ray and spirometry tests before being appropriately diagnosed with IPF. Desire for continuity of care Participants in this study described their experience in care needs from health professionals as: 1) lacking continuity of care, and 2) wanting better quality of healthcare in community hospitals.
In the interviews, participants expressed their desire for the continuity of care post discharge from the specialized hospitals. They hoped that health professionals in the specialized hospital could provide more knowledge, especially on how to self-manage the disease at home. They wanted specific instructions or education about diet, oxygen use, medication, and daily activities. But there was no support once they were discharged from the hospitals. As participants stated,

Discussion
While health professionals of respiratory medicine have been making every effort to diagnose and manage IPF effectively, patients' real-world experiences and care needs from their own perspectives have not been paid much attention by the clinicians in China. To our knowledge, this study was the first study exploring the lived experience of the diagnosis journey and the care needs of the Chinese patients with IPF. The interview data depicted a vivid picture of "being disconnected with the healthcare system" where IPF patients struggled throughout the disease journey. Four main themes emerged from the data featured in IPF patients' narratives of lived experiences. These included the long and confusing journal to reach diagnosis, living with the disease, understanding the disease and treatment, and desire for continuity of care.
Firstly, the most overwhelming issue expressed by the participants in this study is their long journey from having the initial symptoms to confirming the diagnosis. The journey took an average of 2-3 years. Participants were often initially "misdiagnosed" as asthma, COPD, recurrent pneumonia or "undiagnosed". This is consistent with the findings of US study [16]. In Collard's study (2007), the protract time issue of patients with IPF was highlighted. In his study, 55% of patients reported delay a t least one year from initial symptoms to a final diagnosis [16]. In a 2018 nationwide survey in t h e US, more than half of participants had to consult with more than three physicians to confirm the diagnosis of IPF [25]. This could be explained by the fact that diagnosing IPF is a challenging issue as patients often present with the initial symptoms of breathlessness and cough, which are common symptoms of other respiratory diseases. Besides, it is common among older population. While some often regard breathlessness is related to the natural process of aging, others with heart issues often attribute the symptoms to the cardiac disease [25]. Furthermore, the lack of primary care in China is another contributing factor, that is patients have to decide which specialists they see by themselves at the onset of the disease, which could lead to the misdiagnosis, and also prolongs their journey to reach a correct diagnosis [26]. Therefore, the structure of primary care is needed to shorten the disease diagnosis journey.
In addition, Qian (2012) stated that the public literacy of respiratory diseases is lower than other chronic diseases such as diabetes and hypertension among the public in China [27]. It is suggested that it is important to enhance people's knowledge on IPF [27]. Another reasonable suggestion is to provide systematic education and training to the primary health professionals in community hospitals in order to raise the awareness of IPF. It was also highly advocated by guidelines that early detection, timely and accurate diagnosis help to avoid unnecessary tests and potential harmful treatment and to improve the outcomes [28].
The second theme emerged from the data reflects the lived experiences of patients living with the IPF disease. Participants expressed the struggles of living with the physical symptoms and emotional distress and coping with loss of independence. They also expressed their feelings of uncertainty with the prognosis and the future. Symptoms expressed by participants in this study are similar to what had been reported in literature [29].
Current literature suggests that symptom management and palliative care are regarded as the cornerstones to improve quality of life for IPF patients [30]. The National Institute for Health and Care Excellence (NICE) (2015) published a quality statement in recommending that people with IPF should have an ILD specialist nurse available from the diagnosis to the end of life [31]. ILD specialist nurse can conduct comprehensive assessment and make individual care plan throughout all stages of the disease, such as activity of daily livings, oxygen use, education on medication management, and emotional support [32,17]. In the UK, 90% of patients reported that ILD specialist nurse was their main clinical contact [32]. Since the systematic training program of specialist nurses implemented in the year 2000 in China, there has been an increased numbers of trained specialist nurses in the areas of intensive care unit, operation theatre, emergency nursing, diabetes, pressure injury, and intravenous therapy [33,34]. However, there is still no specialist nurse training available in the area of ILD and IPF in China. This gap needs to be addressed to provide effective and quality care for patients with IPF in China.
The third theme emerged from the data was the understanding of the disease and treatment. and anxiety [36]. There is a strong recommendation to establish a patient-centered care model, which focuses on collaboration and a shared decision-making with patients [37]. Such model can help to facilitate the decision-making process with regards to the treatments and education on disease and symptom managements, which, in turn, improve the treatment outcomes and the quality of life of patients [18].
Desire for continuity of care also emerged from the interview data. Participants experienced the lack of continuity of care post discharge from the specialized hospital. They also expressed their uncertainty of the quality of the care provided by the community hospital doctors and nurses with regards to IPF. Due to the reform of healthcare policy in China, public hospitals especially in bigger cities have been under pressure to shorten patients' hospital length of stay [38]. Consequently, patients are often discharged from the hospital after the acute phase of the disease and that they are not ready to self-manage their symptoms at homes. Indeed, most patients participated in this study were from northern regions of China, a remote area that is hundreds of kilometers away from specialized hospitals which are often located in bigger cities. Thus, access to good quality of care can be a challenge for these patients after being discharged from the tertiary hospitals. The findings of this study indicate that there is a lack of care coordination between specialized hospitals and community hospitals in China. In European countries, the local network for ILD or IPF specialist centers have been established working in collaboration and partnership with healthcare professionals, policy makers to improve quality of care for patients in the community [9]. Despite recommendation from the NICE, which states that the pulmonary rehabilitation can improve exercise capacity and quality of life [39], there is still unequal access to pulmonary rehabilitation in the primary care settings and community hospitals in China.
This study suggests that it is of paramount importance to increase public awareness of IPF, improve treatment outcomes and symptom managements via collaboration with multidisciplinary team, and provide timely and accurate diagnosis. A care model with specialized ILD or IPF nurses is essential to provide a continuous care and improve care quality for patients with IPF.

Limitations Of The Study
There are some limitations of this study. Firstly, all the participants came from northern regions of China. The findings should be interpreted with caution because patients' experiences in other parts of China may be different due to the unbalanced/inconsistent quality and level of healthcare across China. Besides, the participants self-reported their journey retrospectively, there may be inaccuracies in their responses. However, the patients' experiences illustrated in this study may shed some light on how future improvements can be made to improve the quality and continuity of care for this vulnerable population. This study also acknowledges that there was an evidence of no recurring themes from the interview data but does not claim that the data saturation has occurred