A narrative literature review of palliative care regarding patients with idiopathic pulmonary fibrosis

Abstract Aim The aim of this study was to examine the reported characteristics of extant studies on palliative care for patients with idiopathic pulmonary fibrosis. Design Narrative review. Methods A comprehensive search of the following electronic databases in English and Japanese commenced from 2002 ‐ December 2017. Eligibility criteria was determined by the inclusion and exclusion criteria. Results Nineteen articles were eligible. The characteristics of palliative care for patients with idiopathic pulmonary fibrosis were symptoms relief, start time of palliative care and palliative care needs of patients and care partners. Also, patients' education of disease management including advanced care planning and developing a palliative care system by the healthcare provider including multidisciplinary professional teams was identified. The care provided was a “care conference” and integrated palliative care was carried out in the patient's home. The majority of the studies were qualitative and retrospective in design. The palliative care system and the development of palliative care were limited.


| INTRODUC TI ON
Idiopathic pulmonary fibrosis (IPF) is a refractory and progressively fatal disease. The course is variable and unpredictable; patients can have long periods of stability and then have sudden debilitating episodes (Raghu et al., 2011). Some patients experience the episodes of acute respiratory deterioration. There are also cases with a shorter life expectancy than with other malignant diseases and acute exacerbations of unknown cause may lead to a life-threatening crisis (Raghu et al., 2011). Evidence-based therapies and diagnostic procedures have not been fully established (Purokivi, Hodgson, Myllarniemi, Salomaa, & Kaarteenaho, 2017), but may be indicated for lung transplantation. In addition, antifibrotic drugs such as nintedanib and pirfenidone do not provide an improvement in life prognosis (Canestaro, Forrester, Raghu, Ho, & Devine, 2016).
Optimal medical treatment for IPF has yet to be found. The sensation of dyspnoea is one of the characteristic symptoms of IPF, which impedes the patient's independent living capacity.
Moreover, research found that those patients' experiences of coping with dyspnoea brought up questions and concerns about the meaning of their illness experiences (Igai, 2016). Therefore, researchers began urging that specific goals fall within the realm of palliative care and needed to include a focus on symptom control (Gilbert & Smith, 2009). The goal of palliative care is improving the quality of life (QOL). Health-related QOL of patients with IPF tends to be low, particularly in the domain of physical and psychological | 537 IGAI well-being (de Vries, Kessels, & Drent, 2001;Tomioka, Imanaka, Hashimoto, & Iwasaki, 2007). Dyspnoea was found to be the main factor that caused a decline in the quality of life for patients with IPF (Nishiyama et al., 2005).
The IPF statement (Raghu et al., 2011) noted that patients and families also sought symptomatic relief from phenomenon such as physical, emotional and psychological distress and needed spiritual support. Lee, McLaughlin, and Collard (2011)  Patients with cancer tended to receive palliative care for the full range of painful situations including spiritual pain when searching for meaning of life and purpose; unfortunately patients with IPF are still suffering from the burden of symptoms and emotions (Igai, 2016).
In the systematic review on nursing to improve the quality of life for IPF patients, much of the research was qualitative and retrospective research. For this reason, the author adopted the narrative literature review method and investigated palliative care aimed at improving the quality of life for patients with IPF.

| AIMS
The aim of this narrative literature review was to examine the characteristics of extant studies on palliative care for patients with IPF.

| Ethical considerations
Study participants from reviewed studies had been provided informed consent. The patients from case reports and retrospective studies had an informed consent waiver not an actual informed consent. Research included in this narrative review had received ethical approval.

| Methodological approach
This narrative review was based on the methods of Cipriani and Geddes (2003) and Pae (2015). Accordingly, the research method (Cipriani & Geddes, 2003;Pae, 2015) did not have a predefined protocol-basis and the hypothesis was a broad overview of the topicrelated research area. The search method necessarily involved subjective selection bias, with the search media mainly from PubMed or Medline database. The data extraction was a simple description of study findings and interpretation containing the authors' subjective intention.
The author conducted the comprehensive search of electronic databases in English and Japanese, which included the following: the

| Eligibility criteria
Eligibility criteria included research of mainly patients with IPF and in English or Japanese. Exclusion criteria included only drug or treatment interventions, studies of medical economics, reviews, letters to editor and commentaries, animal studies, basic science research and duplicates.

| Research screening method
For the retrieved documents, one reviewer provided the primary screening to satisfy the eligibility criteria based on the article title and abstract. Research was not included if it did not clearly meet the eligibility criteria. In the secondary screening, the same reviewer carefully read the full article and using a matrix documented the researcher's discipline, country, research design, aim, sample, age, gender, environment, the content of the intervention and outcomes.
No further articles were excluded during the phase of a full article review.

| Search results
By applying the described strategy, 133 articles were found, 67 of which were relevant hits. After carefully sorting through the relevant hits, the duplicates were removed. Applying the exclusion criteria, 19 articles were found appropriate and included ( Figure 1).

| Mixed methods study
There were two mixed methods study. They both included randomized controlled trials (Bajwah et al., 2015;Lindell et al., 2010).

Authors and Year Main Results
Mixed methods study Bajwah et al. (2015). (UK) Mixed methods study (N = 25; 67 SD 11 years) of the feasibility and acceptability on the impact of a case conference intervention delivered in the home showing that community case conferences improved the quality of life. Lindell et al. (2010). (US) Mixed methods study (N = 21; 66 SD 11 years) of the disease management programme to decrease symptom burden and stress and improve the HRQOL finding that intervention group rated their HRQOL less positively.
Quantitative study Ahmadi et al. (2016). ( and tended to report more anxiety (p = 0.077) compared with controls; however, the care partners rated their own stress at a lower level (p = 0.018) compared with controls. The themes from the analysis of qualitative data were "did not feel isolated when participating in a disease management program," "able to put individual disease into perspective," and "felt that it was important to participate in research to help other with the disease."

| Quantitative studies
Retrospective studies

| Case study
There were three case reports Periyakoil et al., 2005;Swetz et al., 2012).  reported on "care through collaboration" that was comprised of a registered respiratory therapist, nurse practitioner (NP), palliative care nurse, occupational therapist (OT) and physical therapist (PT) along with the ILD multidisciplinary physicians. The researchers suggested that the first care component of patients with IPF was ACP. Periyakoil et al. (2005) reported on three case studies involving panic episodes that accompanied dyspnoea. The panic episodes were quantified using the Acute Panic Inventory (API). In one case, the patient experienced less frequent and less intense panic attacks from effective medical treatment and was able to spend time with friends and family. Swetz et al. (2012) concluded that the use of systematic opioids was appropriate as a mainstay of therapy in the treatment of dyspnoea. Oxygen therapy should also be included although compared with opioids, it had less impact on the distress of dyspnoea.

| D ISCUSS I ON
The studies of palliative care for patients with IPF were limited.
Qualitative and retrospective research comprised most of the papers. Palliative care research for patients with IPF were thought to be appropriately situated at the early stage involved in describing the phenomenon of patients' experience. Mixed methods study designs were used to study interventions for palliative care. That approach was adopted to qualify and quantify the impact of the intervention on the patients and to deeply understand the patient's experience.

| Characteristics of existing studies in palliative care
Regardless of site where patients received care, palliative care was usually requested. Furthermore, three studies corroborated the recommendations to introduce palliative care at the time of or early after diagnoses (Bajwah et al., 2015;Liang et al., 2017;. In qualitative research, it was stated that the patients suffered from dyspnoea and cough, losing autonomy due to the constraints of using home oxygen therapy and losing their ability to enact their usual social roles (Bajwah et al., 2013;Duck et al., 2015;Overgaard et al., 2016). For this reason, patients with IPF were considered to have a unique experience that degraded the quality of their lives.
Therefore, health-care professionals must carefully consider how and when to support the introduction of palliative care that includes introducing how palliative care might improve the patients' quality of life.
In addition, the effectiveness of patients' education consisted of integrated care interventions, patient education by group sessions and respiratory rehabilitation (Bajwah et al., 2015;Kalluri et al., 2016Lindell et al., 2010;Rajala et al., 2016). Programmes to support patients with COPD included integrated care models combining drug therapy, oxygen therapy, nutritional advice, self-management education and psychological support (Nici & ZuWallack, 2012). Support by multiple experts was thought to have provided spiritual wellness through social support to IPF patients and caregivers who both experienced losses. Therefore, it is necessary to develop palliative care for psychological, social and spiritual distress that is informed by patients' experiences and not only to alleviate symptoms but also to attain the highest quality of life possible. Moreover, introducing multidisciplinary and collaborative care was reported to improve the patient's end-of-life care (Rajala et al., 2016) and reduced emergency room visits and hospitalization due to breathing difficulty (Sampson et al., 2015). Support by multidisciplinary teams was thought to bring some emotional stability to the patient because of the increased number of staff attending the patient.
The case study describing patients' panic attacks due to breathing difficulty brought up an important aspect of dyspnoea. Dyspnoea can be negatively influenced by emotional aspects particularly fear.
Therefore, the vicious cycle must be interrupted and is a necessary component of care as well as the management of physical symptoms (Kinzel, 1991). Support from multiple disciplines though integrated care including psychological and emotional care can be considered as support for patients and their families, but since it was a retrospective study, prospective studies are needed.

| Advanced care planning in palliative care of patients with IPF
Advanced care planning was an issue that was noted by several researchers. Lee et al. (2011)  Yet, some patient resistance to ACP has been reported  and Lee et al. (2014) also described the existence of stigma against hospice and palliative care. The stigma surrounding palliative care and hospice is evident from the Taiwan study where researchers found that a significant number of public and private facilities do not even use the terms palliative or hospice opting for more hopeful terms like heart, love, grace and peace (Dai, Chen & Lin, 2017). Curtis, Patrick, Caldwell, and Collier (2000) (Gilbert & Smith, 2009) and the ACP is suggested as helpful in thinking through preliminary instructions (Egan, 2011). This report substantiated that health-care providers need more training. In Japan, it is reported that patients with IPF have 40% more deaths due to acute exacerbations and was greater than that of respiratory failure (Natsuizaka et al., 2014). Researchers found that families made the decision for resuscitation at the time when the patient suddenly experienced an acute episode (Arita et al., 2010). Therefore, the process of ACP is important in reflecting the intention of the patient receiving medical care. The ACP is a process of thinking how patients | 543 IGAI want to live their lives. Although there is a report of resistance to ACP, the ACP is ultimately helping patients and medical staff to think about their future way of living and the thought is that this provides the patient with a process to control their own future.
For patients with severe COPD, advanced directives and good advanced care planning are thought to provide opportunities to improve the quality of palliative care (Curtis, 2008). Making a decision on end-of-life care influences the experience of the process of death (Gilbert & Smith, 2009) and the ACP is suggested to be helpful in thinking through preliminary instructions (Egan, 2011). Au et al. (2012) reported that an intervention using patientspecific feedback about preferences for end-of-life care and communication between patients with COPD and their clinicians improved the occurrence and quality of communication from patients' perspectives. implemented by nurses and social workers and were feasible based on administration time and acceptability by patients and providers. Using such a guideline for ACP is helpful not only for patients with IPF and their carers but also for healthcare providers as they deal with complex end-of-life issues and the stigma surrounding palliative or hospice care (Collins, et al., 2017).

| Limitations
There were several limitations that should be addressed. It was possible that despite efforts to locate all relevant extant studies, some were missed. The language restriction to English and Japanese was also a limitation. Regardless of these potential limitations, the findings were fairly consistent regarding the desire and need for early palliative care.

| RELE VAN CE TO CLINI C AL PR AC TI CE
The palliative care system for patients with IPF was weak and the development of palliative care for patients with IPF was limited.
Palliative care for patients with IPF needs to include interventions based on their illness specific experiences.

ACK N OWLED G EM ENTS
I would like to thank Professor Tomoko Kamei (PhD) of St. Luke's International University for her valuable comments.
Special thanks to Dr. Sarah E. Porter for editing this article, her guidance and support.

CO N FLI C T O F I NTE R E S T
The author declares that they have no conflict of interests.