The term interstitial lung disease encompasses a wide range of parenchymal lung conditions that affect the pulmonary interstitium characterized by inflammation and pulmonary fibrosis, or lung scarring. It is widely recognized that treatment for specific types of interstitial lung disease depends on the extent of lung damage, as it is irreversible and progressive.1 In cases such as idiopathic pulmonary fibrosis, the most common and arguably the most aggressive form of pulmonary fibrosis, individuals are faced with a survival rate of a mere 2 to 3 years after diagnosis, high symptom burden, and reported poor quality of life.2,3 Treatment methodologies often rely on slowing disease progression, relieving symptom burden, and improving quality of life.1 Specialized health care networks and providers to assist individuals with idiopathic pulmonary fibrosis with proper or timely treatment are scarce.4 Individuals are often misdiagnosed for an average of 1.5 years, worsening unmet needs regarding psychological support, treatment plans, and symptom management.4 These concerns, unfortunately, are often prevalent even after an accurate diagnosis is made.
A lack of treatment options remains a concern when addressing how to improve health-related quality of life for these individuals. Individuals with idiopathic pulmonary fibrosis frequently report an unrelenting dry cough and progressive dyspnea with exertion, which severely encumbers their ability to be active participants in their daily lives, thus reducing health-related quality of life.5 Beneficial components of treatment reported by individuals with idiopathic pulmonary fibrosis include high-quality education on disease progression, emotional support, timely and accurate diagnosis, supportive end-of-life care planning, collaboration with a knowledgeable health care team, and both pharmacologic and nonpharmacologic methods of symptom management.6 The purpose of this literature review is to describe the multidimensional management of dyspnea in individuals with idiopathic pulmonary fibrosis.
SEARCH STRATEGY
The databases CINAHL, Cochrane Library Databases, PubMed, Scopus, and Google Scholar were queried between January 15 and January 21, 2020. The following search terms were used alone or in combination: "dyspnea," "breathlessness," "interstitial lung disease," "idiopathic pulmonary fibrosis," "quality of life," and "pulmonary fibrosis." In addition, manual searches of relevant research were carried out to review pertinent studies and provide a comprehensive search. Only peer-reviewed journal articles in English published within the last 5 years in academic journals that focused on idiopathic pulmonary fibrosis populations were included. Of the initial search, articles were omitted for the following reasons: individual involvement in lung transplantation, no standard measurement for health-related quality of life, and use of inpatient settings. Thirteen articles are included in this literature review, including 7 randomized controlled trials and 6 observational studies of various designs.
SYNTHESIS OF LITERATURE
The 13 articles were sorted based on the intervention used to alleviate dyspnea and impact self-reported health-related quality of life as a primary or secondary outcome in individuals with idiopathic pulmonary fibrosis. The operational definition of quality of life for all studies used reliable and valid health-related quality of life assessments including (1) Saint George's Respiratory Questionnaire, (2) Saint George's Respiratory Questionnaire IPF-specific version, (3) King's Brief Interstitial Lung Disease Questionnaire, (4) Short Form 36 Health Survey, (5) University of California San Diego Shortness of Breath Questionnaire, (6) Visual Simplified Respiratory Questionnaire, (7) Psychological General Well-Being Index, and (8) Chronic Respiratory Disease Questionnaire.
Five articles assessed the impact of outpatient facility-based pulmonary rehabilitation or exercise programs on health-related quality of life assessments.7-11 Two articles assessed the impact of outpatient home-based pulmonary rehabilitation or exercise programs on health-related quality of life assessments.12,13 Unstructured exercise, or daily activity level, was evaluated by 2 groups.14,15 The impact of supplemental oxygen was assessed in 1 article.16 Three studies analyzed the effect of various methods of multidisciplinary support on pulmonary rehabilitation, including palliative care, fast-track case conferences, and support groups.17-19 For organizational purposes of this review, the studies are grouped into 3 categories based on the overarching intervention used to impact health-related quality of life: (1) exercise to improve dyspnea, (2) intensive interdisciplinary support to improve dyspnea, and (3) supplemental oxygen to improve dyspnea (Table).
Managing Dyspnea With Exercise
Recommended supportive measures to help individuals manage dyspnea, and subsequently improve health-related quality of life, include pulmonary rehabilitation.5 Pulmonary rehabilitation has been shown to assist individuals in gaining more control over their symptom burden of dyspnea and associated depression, anxiety, and even feelings of loss of control over one's life.6 However, as it is viewed as a treatment option primarily for chronic obstructive pulmonary disease (COPD), there is often a disparity in pulmonary fibrosis individuals' ability to access pulmonary rehabilitation programs in their area.20 The most updated American Thoracic Society guidelines for the treatment and management of idiopathic pulmonary fibrosis advise that pulmonary rehabilitation programs are not appropriate for all idiopathic pulmonary fibrosis patients.3 Pulmonary rehabilitation programs may need to be explicitly tailored to the individual's idiopathic pulmonary fibrosis needs and take into consideration the degree of baseline functional status.3 There are inconsistencies as to what length, regimen, or educational component of pulmonary rehabilitation programs is most beneficial when tailored for individuals with interstitial lung disease.
Severe exertional dyspnea in individuals with idiopathic pulmonary fibrosis can inhibit their desire to complete simple daily tasks, often noted as a key determinant of their health-related quality of life.21 An improvement in idiopathic pulmonary fibrosis individually reported health-related quality of life has been found to have a robust link with improvement in clinical outcomes, including rates of lung function decline, hospitalization, and acute exacerbations of disease.6
Outpatient Facility-Based Pulmonary Rehabilitation
Outpatient facility-based pulmonary rehabilitation programs with short-term durations ranging from 8 to 12 weeks were the most commonly evaluated intervention to reduce dyspnea and improve health-related quality of life.7-9,11 Arizono et al,7 Dowman et al,8 and Naz et al9 assessed short-term effects of pulmonary rehabilitation using a validated questionnaire for self-reported quality of life, the Saint George's Respiratory Questionnaire, with Dowman et al8 further specifying the questionnaire to be the idiopathic pulmonary fibrosis-specific version. Findings from these 3 studies demonstrated that short-term pulmonary rehabilitation programs produced significant improvement in health-related quality of life evidenced by improved parameters on the Saint George's Respiratory Questionnaire. Naz et al9 provided evidence that short-term pulmonary rehabilitation can improve all subparameters of Saint George's Respiratory Questionnaire, whereas Dowman et al8 demonstrated improvement in all subparameters except for Saint George's Respiratory Questionnaire-impact score. Arizono and colleagues7 showed evidence of significantly improved Saint George's Respiratory Questionnaire total scores for individuals with idiopathic pulmonary fibrosis when compared with a control group of COPD patients. Interestingly, Naz and colleagues9 further demonstrated that the significant improvement in Saint George's Respiratory Questionnaire scores was maintained and even more profound at 12 weeks than 8 weeks, demonstrating a continued positive effect.
Two randomized controlled trials evaluated the long-term effects of pulmonary rehabilitation on health-related quality of life using the Saint George's Respiratory Questionnaire.10,11 Vainshelboim and colleagues11 demonstrated that a 12-week supervised pulmonary rehabilitation program had a statistically significant improvement for both Saint George's Respiratory Questionnaire-total and Saint George's Respiratory Questionnaire-impact scores after 11 months. Perez-Bogerd and colleagues10 used a 6-month pulmonary rehabilitation program design, resulting in clinically significant improvement in Saint George's Respiratory Questionnaire activity scores, as well as Chronic Respiratory Disease Questionnaire dyspnea, emotion, and mastery scores. This clinically meaningful difference was present even at the 1-year follow-up assessment.10 Thus, both studies, assessing a shorter-duration pulmonary rehabilitation program of 12 weeks and a longer-duration pulmonary rehabilitation program of 6 months, demonstrated maintenance of health-related quality of life outcomes for individuals with idiopathic pulmonary fibrosis in the long term. Interestingly, changes in 6-minute walk distance, a commonly monitored indicator of lung function in individuals with idiopathic pulmonary fibrosis, were associated with changes in reported health-related quality of life.11 Although the studies reviewed supported facility-based pulmonary rehabilitation, home-based pulmonary rehabilitation is also recognized as an intervention for symptom management of dyspnea.
Outpatient Home-Based Pulmonary Rehabilitation
Two studies analyzed the effects of outpatient home-based pulmonary rehabilitation programs and its subsequent impact on health-related quality of life for individuals with idiopathic pulmonary fibrosis.12,13 In an observational study with retrospective analysis, Wallaert and colleagues12 demonstrated that a structured 2-month pulmonary rehabilitation program with once-weekly visits for education and psychosocial support had clinical improvement in self-reported health-related quality of life at 2-, 6-, and 12-month follow-up intervals using the Visual Simplified Respiratory Questionnaire. Of note, disease severity did not impact Visual Simplified Respiratory Questionnaire scores between groups.12 In contrast, Yuen and colleagues13 used a less structured exercise format of using a home-based exercise game involving full-body movements 3 times weekly. After 12 weeks, this regimen failed to yield any clinically significant improvement in health-related quality of life, as demonstrated by self-reported Saint George's Respiratory Questionnaire scores.13 In addition to structured facilitated and home-based pulmonary rehabilitation, unstructured activity has also been evaluated in relation to the symptom management of dyspnea in individuals with idiopathic pulmonary fibrosis.
Unstructured Activity
Nishiyama et al14 used a prospective controlled observational study to evaluate the impact of physical activity on individuals with idiopathic pulmonary fibrosis in an outpatient setting; idiopathic pulmonary fibrosis patients used an activity monitor for 7 days, with assessments of health-related quality of life through Saint George's Respiratory Questionnaire reporting at baseline and completion. There was no clinically significant correlation between physical activity variables, including steps walked and activity energy expenditure on Saint George's Respiratory Questionnaire scores.14 Similarly, in a prospective cohort study, Root et al15 evaluated the association between physical activity and health-related quality of life in individuals with pulmonary fibrosis of varying etiology who did not use supplemental oxygen by tracking activity for 7 consecutive days. No causation was established between physical activity, measured in steps per day, and improved health-related quality of life scores, as assessed with a variety of health-related quality of life tools, namely, University of California San Diego Shortness of Breath Questionnaire and Short Form 36 Health Survey.15 Both studies provide support that physical activity alone, such as exercise that is not performed in a formal pulmonary rehabilitation setting, is not enough to have clinically meaningful impacts on self-reported health-related quality of life for individuals with idiopathic pulmonary fibrosis.14,15 However, it adds to the discussion of what aspects of pulmonary rehabilitation programs may influence clinically significant improvements in quality of life that general physical activity does not. Whereas pulmonary rehabilitation is an important part of managing idiopathic pulmonary fibrosis, interdisciplinary care teams are another strategy for supporting symptom management of dyspnea in individuals with idiopathic pulmonary fibrosis.
Improving Dyspnea Symptom Management With Interdisciplinary Support
The most effective interventions to help manage symptom burden, particularly that of dyspnea, are multidisciplinary in nature and encompass both physical and psychosocial needs.5 These approaches are accomplished most effectively through multidisciplinary coordination of care to provide individuals with treatment options.22 Specialized interstitial lung disease registered nurses are crucial resources for individuals with idiopathic pulmonary fibrosis during discussions of palliative care, symptom burden, oxygen use, and methods of increasing abilities to participate in activities of daily living.20 Early palliative care referrals are noted as a recommended course of action to help individuals with idiopathic pulmonary fibrosis understand the disease process and associated symptom burden.23-25
The use of various methods of providing more intensive direct multidisciplinary support to help individuals better understand and cope with breathlessness was assessed by 3 studies.17-19 Janssen and colleagues18 assessed the impact of structured palliative care visits at 3 and 6 months integrated into standard of care clinic visits in a randomized controlled trial. Magnani and colleagues19 analyzed the impact of attending an idiopathic pulmonary fibrosis-specific support group for 6 months in a quasi-experimental pretest-posttest study. Bajwah and colleagues17 evaluated the use of implementing a fast-track case conference, Hospital2Home, in a randomized controlled trial.
There was no clinically beneficial impact of structured palliative care visits on health-related quality of life for individuals with mild idiopathic pulmonary fibrosis. However, the data do offer novel insights; palliative care may be helpful in enhancing health-related quality of life for those with more severe disease progression who are aware of their long-term outcome.18 After 6 months of attendance at an idiopathic pulmonary fibrosis-specific support group, there was a null association between participation and psychosocial well-being and health-related quality of life, as demonstrated by the Psychological General Well-Being Index scores.19 This study provided data that showed interesting increases in psychosocial well-being parameters that deserve further investigation in the use of support groups for enhancing health-related quality of life in larger sample sizes.19 Hospital2Home implementation improved health-related quality of life after 4 and 8 weeks as evidenced by Saint George's Respiratory Questionnaire-impact and Saint George's Respiratory Questionnaire-total scores.17 Similar improvements were seen when the control group received the Hosptial2Home addition to their routine medical care.17 This demonstrated that early integration of home-based case conferences has a more valuable impact on health-related quality of life for idiopathic pulmonary fibrosis patients than either participation in idiopathic pulmonary fibrosis-specific support groups or early incorporation of palliative care visits.17 Although interdisciplinary approaches show some promise for managing dyspnea, less is known about the use of oxygen.
Controlling Dyspnea With Supplemental Oxygen
Limited data exist to support the use of oxygen for palliative benefit, although it is recommended for severe breathlessness.26,27 Visca et al16 evaluated the short-term effects of using supplemental oxygen on health-related quality of life for individuals with fibrotic interstitial lung disease with exertional hypoxia through a prospective open-laboratory, mixed-method, crossover randomized controlled trial. Saint George's Respiratory Questionnaire and King's Brief Interstitial Lung Disease Questionnaire scores were evaluated at baseline and at 2- and 4-week follow-up points.16 Supplemental oxygen was associated with clinically significant short-term improvement in the following subdomains: King's Brief Interstitial Lung Disease Questionnaire total, King's Brief Interstitial Lung Disease Questionnaire-breathlessness, King's Brief Interstitial Lung Disease Questionnaire-chest symptoms, Saint George's Respiratory Questionnaire total, and Saint George's Respiratory Questionnaire-activity.16 Notably, there was no clinically significant improvement associated between supplemental oxygen use and King's Brief Interstitial Lung Disease Questionnaire-psychological symptom scores.16 Three centers were used to recruit for this study, allowing for a diversity of study population. However, no other study used supplemental oxygen as an intervention. Despite negative perspectives on oxygen use, as evidenced by qualitative interviews and a low adherence rate of oxygen use during the study, early obtainment of supplemental oxygen for individuals' idiopathic pulmonary fibrosis with exertional hypoxia is an effective method of improving patient-reported health-related quality of life.16
GAPS IN LITERATURE AND LIMITATIONS
Limitations of this literature review include the use of studies with small sample sizes, some mixed interstitial lung disease populations, and subjective measures of reporting health-related quality of life. Larger randomized controlled trials should be used in future investigations to improve representation. Moreover, participants had varying use of antifibrotics, steroid medications, and supplemental oxygen. These were not always controlled for in all studies, limiting the applicability of these literature review findings to all individuals with idiopathic pulmonary fibrosis, such as those with mild, moderate, and severe diseases. Moreover, not all questionnaires used are validated specifically for idiopathic pulmonary fibrosis. For example, the Visual Simplified Respiratory Questionnaire used by Wallaert and colleagues,12 is validated for use with persons with COPD but was used with an idiopathic pulmonary fibrosis sample because of ease of use and success in trials assessing home-based pulmonary rehabilitation in COPD patients. The studies reviewed provided interventions in the context of team-based care and did not focus on registered nurse-specific care interventions.
DISCUSSION
Emerging clinical research trials are looking at more innovative and effective methods of combatting and, hopefully, in the future, curing idiopathic pulmonary fibrosis. The most updated American Thoracic Society guidelines for treatment and management of idiopathic pulmonary fibrosis advise on the use of both pharmacologic and nonpharmacologic strategies.3 There is no cure and only limited pharmacologic recommendations for treatment options. Therefore, nonpharmacologic interventions are key in providing comprehensive care for symptom management of idiopathic pulmonary fibrosis patients, particularly when managing dyspnea. The findings of this literature review support the American Thoracic Society recommendations but also help discern which nonpharmacologic interventions for dyspnea are more effective in improving health-related quality of life for individuals with idiopathic pulmonary fibrosis. Registered nurses can play an essential role in providing symptom management, palliative care, and behavioral health for these individuals.
Pulmonary rehabilitation programs are not recommended for all idiopathic pulmonary fibrosis patients.3 Furthermore, evidence from this literature review supports that all pulmonary rehabilitation programs are not adequately designed to elicit effective responses for all individuals with idiopathic pulmonary fibrosis; outpatient facility-based and structured home-based pulmonary rehabilitation programs of any length were the most effective in improving health-related quality of life, with both clinically significant short- and long-term effects.7-12 Activity alone in an unstructured and informal setting, such as at home, is not enough to provide clinically significant impacts on health-related quality of life.13-15 Additional nonpharmacologic interventions to help improve dyspnea and, subsequently, improve health-related quality of life for individuals with idiopathic pulmonary fibrosis may include earlier use of supplemental oxygen for exertional dyspnea and fast-track case conferences in conjunction with their routine medical care.16,17 Registered nurses should ensure that these nonpharmacologic interventions to alleviate dyspnea are included in routine management for persons with idiopathic pulmonary fibrosis to enhance health-related quality of life and improve clinical outcomes.
RECOMMENDATIONS FOR FUTURE RESEARCH
Future research opportunities exist in exploring both pulmonary rehabilitation program components and the impact of palliative care visits in the long term for individuals with idiopathic pulmonary fibrosis to improve health-related quality of life. To determine how to tailor pulmonary rehabilitation programs for idiopathic pulmonary fibrosis, researchers should explore the noted differences in pulmonary rehabilitation programs across facilities. This includes the length of sessions, frequency of sessions, monthly duration of programs, included educational components, and degree of pulmonary impairment before individuals are enrolled; this would aid in explaining why pulmonary rehabilitation programs, not physical activity alone, are beneficial in improving health-related quality of life. Studies with palliative care interventions were limited in both idiopathic pulmonary fibrosis disease progression (only focusing on individuals with mild disease) and time length (only short-term). Positive benefits have been shown in studies looking at the impact of early palliative care conversations focused on prognosis, symptom management, and advance care planning in the diagnosis of serious chronic illnesses in other populations.28-30
References