Patient and caregiver shared experiences of pulmonary fibrosis (PF): A systematic literature review.

Pulmonary Fibrosis (PF) describes a group of lung diseases characterised by progressive scarring (fibrosis). Symptoms worsen over time and include breathlessness, tiredness, and cough, giving rise to psychological distress. Significant morbidity accompanies PF, so ensuring patients' care needs are well defined and provided for, represents an important treatment strategy. The purpose of this systematic review was to synthesise what is currently known about the psychosocial morbidity, illness experience and needs of people with pulmonary fibrosis and their informal caregivers. Eight databases (MEDLINE, EMBASE, PUBMED, Cochrane database of Systematic reviews (CDSR), Web of Science Social Sciences Citation Index, PsycINFO, PsycARTICLES and CINAHL) were used to identify studies exploring the supportive needs of adults with PF and/or their caregivers. Methodological quality was assessed using the Mixed Methods Appraisal Tool. 53 studies were included, the majority using qualitative methodology (79 %, 42/53), 6 as part of mixed methodological studies. Supportive care needs were mapped to eight domains using an a priori framework analysis. Findings highlight a lack of psychological support throughout the course of the illness, misconceptions about and barriers to, the provision of palliative care despite its potential positive impacts. Patients and caregivers express a desire for greater disease specific education and information provision throughout the illness. Trials of complex interventions are needed to address the unique set of challenges for patients and carers living with PF.

Psychological impact of genetic and clinical screening for pulmonary fibrosis on asymptomatic first-degree relatives of affected individuals.

Screening for pulmonary fibrosis may help to identify early stages of the disease. We assessed the psychological impact of screening undiagnosed first-degree relatives of patients with pulmonary fibrosis by administering two validated measures after participants received their results: the Decisional Regret Scale and the Feelings About genomiC Testing Results Questionnaire. More than 90% of relatives reported either no or mild decisional regret. Increased measures of decisional regret and negative feelings were present in those found to have a low diffusion capacity of carbon monoxide or interstitial lung abnormalities. Results of telomere length and genetic testing did not significantly impact regret.

Minimum important difference of the EQ-5D-5L and EQ-VAS in fibrotic interstitial lung disease.

RATIONALE: The European Quality of Life 5-Dimensions 5-Levels questionnaire (EQ-5D-5L) is a multidimensional patient-reported questionnaire that supports calculation of quality-adjusted life-years. Our objectives were to demonstrate feasibility of use and to calculate the minimum important difference (MID) of the EQ-5D-5L and its associated visual analogue scale (EQ-VAS) in patients with fibrotic interstitial lung disease (ILD). METHODS: Patients who completed the EQ-5D-5L were identified from the prospective multicentre CAnadian REgistry for Pulmonary Fibrosis. Validity, internal consistency and responsiveness of the EQ-5D-5L were assessed, followed by calculation of the MID for the EQ-5D-5L and EQ-VAS. Anchor-based methods used an unadjusted linear regression against pulmonary function tests (PFTs) and dyspnoea and other quality of life questionnaires. Distribution-based method used one-half SD and SE measurement (SEM) calculations. RESULTS: 1816 patients were analysed, including 472 (26%) with idiopathic pulmonary fibrosis. EQ-5D-5L scores were strongly correlated with the dyspnoea and other quality of life questionnaires and weakly associated with PFTs. The estimated MID for EQ-5D-5L ranged from 0.0050 to 0.054 and from 0.078 to 0.095 for the anchor-based and distribution-based methods, respectively. The MID for EQ-VAS ranged from 0.5 to 5.0 and from 8.0 to 9.7 for the anchor-based and distribution-based methods. Findings were similar across ILD subtypes, sex and age. CONCLUSION: We used a large and diverse cohort of patients with a variety of fibrotic ILD subtypes to suggest validity and MID of both the EQ-5D-5L and EQ-VAS. These findings will assist in designing future clinical trials and supporting cost-effectiveness analyses of potential treatments for patients with fibrotic ILD.

The supportive care needs of people living with pulmonary fibrosis and their caregivers: a systematic review.

BACKGROUND: People with pulmonary fibrosis often experience a protracted time to diagnosis, high symptom burden and limited disease information. This review aimed to identify the supportive care needs reported by people with pulmonary fibrosis and their caregivers. METHODS: A systematic review was conducted according to PRISMA guidelines. Studies that investigated the supportive care needs of people with pulmonary fibrosis or their caregivers were included. Supportive care needs were extracted and mapped to eight pre-specified domains using a framework synthesis method. RESULTS: A total of 35 studies were included. The most frequently reported needs were in the domain of information/education, including information on supplemental oxygen, disease progression and prognosis, pharmacological treatments and end-of-life planning. Psychosocial/emotional needs were also frequently reported, including management of anxiety, anger, sadness and fear. An additional domain of "access to care" was identified that had not been specified a priori; this included access to peer support, psychological support, specialist centres and support for families of people with pulmonary fibrosis. CONCLUSION: People with pulmonary fibrosis report many unmet needs for supportive care, particularly related to insufficient information and lack of psychosocial support. These data can inform the development of comprehensive care models for people with pulmonary fibrosis and their loved ones.

Peer Connect Service for people with pulmonary fibrosis in Australia: Participants' experiences and process evaluation.

BACKGROUND AND OBJECTIVE: People living with pulmonary fibrosis (PF) report unmet needs for information and support. Lung Foundation Australia (LFA) have developed the Peer Connect Service to facilitate telephone support for people with PF across Australia. This project documented the experiences of participants and the resources required to support the service. METHODS: Consenting participants took part in semi-structured interviews by telephone. Primary peers (peers who agreed to initiate contact) and secondary peers (eligible patients who sought a peer match) were interviewed. Thematic analysis was undertaken by two independent researchers. Data were collected on the number of matches and contacts required to establish each match. RESULTS: Interviews were conducted with 32 participants (16 primary peers, 15 secondary peers and 1 who was both), aged from 53 to 89 years with 56% being male. Major themes included the value of shared experiences, providing mutual support and the importance of shared personal characteristics (e.g. gender and hobbies) in allowing information and emotional support needs to be met. Participants saw face-to-face contact with peers as highly desirable whilst acknowledging the practical difficulties. Primary peers were cognizant that their role was not to provide medical advice but to listen and share experiences. In the 12-month period, 60 peer matches were made, each match requiring a minimum of seven staff contacts. CONCLUSION: The Peer Connect Service provides a unique opportunity for people with PF to share experiences and offer mutual support. This telephone matching model may be useful in providing peer support for individuals with rare diseases who are geographically dispersed.

Patients' perceptions and patient-reported outcomes in progressive-fibrosing interstitial lung diseases.

The effects of interstitial lung disease (ILD) create a significant burden on patients, unsettling almost every domain of their lives, disrupting their physical and emotional well-being and impairing their quality of life (QoL). Because many ILDs are incurable, and there are limited reliably-effective, life-prolonging treatment options available, the focus of many therapeutic interventions has been on improving or maintaining how patients with ILD feel and function, and by extension, their QoL. Such patient-centred outcomes are best assessed by patients themselves through tools that capture their perceptions, which inherently incorporate their values and judgements. These patient-reported outcome measures (PROs) can be used to assess an array of constructs affected by a disease or the interventions implemented to treat it. Here, we review the impact of ILD that may present with a progressive-fibrosing phenotype on patients' lives and examine how PROs have been used to measure that impact and the effectiveness of therapeutic interventions.

Effect of ambulatory oxygen on quality of life for patients with fibrotic lung disease (AmbOx): a prospective, open-label, mixed-method, crossover randomised controlled trial.

BACKGROUND: In fibrotic interstitial lung diseases, exertional breathlessness is strongly linked to health-related quality of life (HRQOL). Breathlessness is often associated with oxygen desaturation, but few data about the use of ambulatory oxygen in patients with fibrotic interstitial lung disease are available. We aimed to assess the effects of ambulatory oxygen on HRQOL in patients with interstitial lung disease with isolated exertional hypoxia. METHODS: AmbOx was a prospective, open-label, mixed-method, crossover randomised controlled clinical trial done at three centres for interstitial lung disease in the UK. Eligible patients were aged 18 years or older, had fibrotic interstitial lung disease, were not hypoxic at rest but had a fall in transcutaneous arterial oxygen saturation to 88% or less on a screening visit 6-min walk test (6MWT), and had self-reported stable respiratory symptoms in the previous 2 weeks. Participants were randomly assigned (1:1) to either oxygen treatment or no oxygen treatment for 2 weeks, followed by crossover for another 2 weeks. Randomisation was by a computer-generated sequence of treatments randomly permuted in blocks of constant size (fixed size of ten). The primary outcome, which was assessed by intention to treat, was the change in total score on the King's Brief Interstitial Lung Disease questionnaire (K-BILD) after 2 weeks on oxygen compared with 2 weeks of no treatment. General linear models with treatment sequence as a fixed effect were used for analysis. Patient views were explored through semi-structured topic-guided interviews in a subgroup of participants. This study was registered with ClinicalTrials.gov, number NCT02286063, and is closed to new participants with all follow-up completed. FINDINGS: Between Sept 10, 2014, and Oct 5, 2016, 84 patients were randomly assigned, 41 randomised to ambulatory oxygen first and 43 to no oxygen. 76 participants completed the trial. Compared with no oxygen, ambulatory oxygen was associated with significant improvements in total K-BILD scores (mean 55·5 [SD 13·8] on oxygen vs 51·8 [13·6] on no oxygen, mean difference adjusted for order of treatment 3·7 [95% CI 1·8 to 5·6]; p<0·0001), and scores in breathlessness and activity (mean difference 8·6 [95% CI 4·7 to 12·5]; p<0·0001) and chest symptoms (7·6 [1·9 to 13·2]; p=0·009) subdomains. However, the effect on the psychological subdomain was not significant (2·4 [-0·6 to 5·5]; p=0·12). The most common adverse events were upper respiratory tract infections (three in the oxygen group and one in the no-treatment group). Five serious adverse events, including two deaths (one in each group) occurred, but none were considered to be related to treatment. INTERPRETATION: Ambulatory oxygen seemed to be associated with improved HRQOL in patients with interstitial lung disease with isolated exertional hypoxia and could be an effective intervention in this patient group, who have few therapeutic options. However, further studies are needed to confirm this finding. FUNDING: UK National Institute for Health Research.

Informal caregivers experience of supplemental oxygen in pulmonary fibrosis.

BACKGROUND: Patients prescribed supplemental oxygen (O2) therapy face challenges as they adjust to being constantly "tethered" to an oxygen delivery device. Informal caregivers (ICs) of patients with pulmonary fibrosis (PF) face their own, often overlooked hardships when O2 is brought into their home and added to their lives. Our aim was to understand the multiple effects of supplemental oxygen therapy on ICs of patients with PF. METHODS: We conducted single, semi-structured telephone interviews with twenty ICs of patients with PF who were using O2 for at least 8 months. We performed a qualitative, content analysis based in grounded theory to examine data across subjects. RESULTS: ICs initially reacted to O2 with trepidation and sadness as they came to recognize the changes it would cause in the lives of their patient-loved one (PLO). ICs recognized both beneficial and negative effects of O2 on their PLOs. ICs also realized that O2 created significant changes in their own lives, including introducing new roles and responsibilities for them, altering their home environments and significantly impacting their relationships with their PLOs. Although O2 was a tangible and constant reminder of disease progression, over time ICs were able to adapt and accept their new lives with O2. CONCLUSION: ICs of patients with PF experience many life changes when their PLO is prescribed O2. Having O2 prescribers anticipate and recognize these challenges provides an opportunity to give support and guidance to ICs of PF patients who require O2 in the hopes of limiting the negative impact of O2 on their lives. TRIAL REGISTRATION: Clinicaltrials.gov , registration number NCT01961362 . Registered 9 October 2013.

Psychiatric disorders and psychological distress in patients undergoing evaluation for lung transplantation: a national cohort study.

OBJECTIVE: We sought to investigate type and prevalence of psychiatric disorders and psychological distress in patients being evaluated for lung transplantation. METHODS: One hundred eighteen patients were assessed [74% with chronic obstructive pulmonary disease (COPD)] with the MINI Neuropsychiatric Interview, the General Health Questionnaire (GHQ), and Hospital Anxiety Depression Scale (HADS). Spirometry and the 6-min walk test (6MWT) assessed lung function with data subject to multivariate regression analyses. RESULTS: Current and lifetime prevalence for mental disorders were 41.5% and 61.0% respectively, with anxiety (39.8% of patients), mood disorders (11.8%), and subsyndromal disorders (8.7%) identified. 15% of patients reported feelings of panic during the last week, 9% reported hopelessness, and 3% felt that life was not worth living. Statistically significant correlates were derived for HADS-depression with lung function (P=.0012) and 6MWT (P=.030) for the entire group (P=.012), and with lung function (P=.030) for COPD patients (P=.045), for whom higher chronic GHQ-scores correlated with poorer lung function (P=.009). In multivariate regression analysis, history of mental disorder was strongest predictor of current distress. CONCLUSIONS: Our findings underline the importance of assessing past, current, and sub-syndromal psychiatric disorders in addition to levels of distress in transplant candidates, with prospective studies needed to investigate impact on long-term outcome after transplantation.

Validity and Reliability of the Chronic Respiratory Disease Questionnaire in Elderly Individuals with Mild to Moderate Non-Cystic Fibrosis Bronchiectasis.

BACKGROUND: The chronic respiratory disease questionnaire (CRDQ) is designed to assess health-related quality of life (HRQOL) in chronic respiratory conditions, but its reliability, validity and responsiveness in individuals with mild to moderate non-cystic fibrosis (CF) bronchiectasis are unclear. OBJECTIVES: This study aimed to determine measurement properties of the CRDQ in non-CF bronchiectasis. METHODS: Participants with non-CF bronchiectasis involved in a randomised controlled trial of exercise training were recruited. Internal consistency was assessed using Cronbach's α. Over 8 weeks, reliability was evaluated using intra-class correlation coefficients and Bland-Altman analysis for measures of agreement. Convergent and divergent validity was assessed by correlations with the other HRQOL questionnaires and the Hospital Anxiety and Depression Scale (HADS). The responsiveness to exercise training was assessed using effect sizes and standardised response means. RESULTS: Eighty-five participants were included (mean age ± SD, 64 ± 13 years). Internal consistency was adequate (>0.7) for all CRDQ domains and the total score. Test-retest reliability ranged from 0.69 to 0.85 for each CRDQ domain and was 0.82 for the total score. Dyspnoea (CRDQ) was related to St George's respiratory questionnaire (SGRQ) symptoms only (r = 0.38), with no relationship to the Leicester cough questionnaire (LCQ) or HADS. Moderate correlations were found between the total score of the CRDQ, the SGRQ (rs = -0.49) and the LCQ score (rs = 0.51). Lower CRDQ scores were associated with higher anxiety and depression (rs = -0.46 to -0.56). The responsiveness of the CRDQ was small (effect size 0.1-0.24). CONCLUSIONS: The CRDQ is a valid and reliable measure of HRQOL in mild to moderate non-CF bronchiectasis, but responsiveness was limited.

Dyspnea and quality of life in patients referred for lung transplantation.

BACKGROUND: In the years 2007-2010 in the Department of Lung Diseases and Tuberculosis, Medical University of Silesia, 86 patients fulfilling ISHLT criteria qualified for lung transplantation. OBJECTIVES: The aim of the study was to assess the correlation between dyspnea and quality of life, and how it is related to clinical data in the examined group. MATERIAL AND METHODS: MRC, OCD, BDI and Borg scale were used for dyspnea evaluation, whereas quality of life was evaluated with SF-36 and SGRQ. A reference group consisted of 18 females and 68 males of the mean age 52 ±10 years and BMI 24 ±6. Thirty patients were diagnosed with IPF, 22 with COPD, and 34 with IIP. RESULTS: In the reference group, there was a significant correlation between dyspnea and quality of life: between MRC and Pf (SF-36 domain) r = -0.53; OCD and activity (SGRQ) r = 0.56; OCD and Pf r = -0.55; BDI and impact (SGRQ) r = 0.51; Borg scale and impact r = 0.47. In patients after lung transplantation, correlation between MRC and SF was r = -0.92; OCD and Pf, Bp, MH, PCS r = -0.97; OCD and RE r = -0.89; BDI and Pf r = -0.89; BDI and activity r=0.9; BDI and PCS r = -0.84. Depending on the diagnosis, the strongest correlation in IIP patients was found between OCD and activity (r = 0.62), in COPD patients - between BDI and impact (r = 0.79), and in IPF patients r = - 0.62 for OCD and Pf. Summing up the results, we can state that there is a significant correlation between dyspnea and quality of life. This correlation seems the strongest in patients after lung transplantation. CONCLUSIONS: The correlation found between the level of dyspnea and quality of life domains in lung transplant patients suggests that it would be worthwhile to add questions regarding dyspnea to assess the severity of the disease, clinical symptoms, and functional impairment during referring the patients for lung transplantation.

Written disclosure therapy for patients with chronic lung disease undergoing pulmonary rehabilitation.

PURPOSE: Chronic lung diseases are typically associated with impaired quality of life, stress, and anxiety. Written disclosure therapy (WDT) reduces stress in patients with a variety of chronic illnesses. We sought to determine whether WDT benefits patients with chronic lung disease. METHODS: A prospective, randomized, controlled trial was performed to evaluate the effect of using WDT in patients (N = 66) participating in a pulmonary rehabilitation program. Patients were randomly assigned to write about a particularly traumatic life event (WDT group) or to write about an emotionally neutral subject (control group). Exercise capacity, dyspnea and quality of life, and values of spirometry were recorded at baseline, at the end of the program, and at 6 months. RESULTS: The 6-minute walk distance (6MWD) significantly improved in both groups at 2 months, from 278 to 327 m in WDT and from 269 to 314 m in control groups (P < .01 in both groups). There was no difference in improvement in 6MWD between groups (P = .88). At 6 months, the gains made in 6MWD were no longer present. Dyspnea severity, as well as most of the other domains of the Chronic Respiratory Disease Questionnaire and the St. George's Respiratory Questionnaire, showed improvement within each group, but not between WDT and control groups. CONCLUSION: WDT did not add any additional benefit in patients with chronic obstructive pulmonary disease or idiopathic pulmonary fibrosis when included as a component of pulmonary rehabilitation. These results are in contrast to previously seen benefits in patients with asthma.

Rehabilitation and palliative care in lung fibrosis.

The idiopathic interstitial pneumonias are a heterogeneous group of diffuse parenchymal lung diseases with varying degrees of inflammation and fibrosis, like interstitial pulmonary fibrosis. Functional exercise tolerance and quality of life have been shown to be significantly affected in patients with lung fibrosis. Moreover, interstitial pulmonary fibrosis is a progressive disease with poor prognosis and limited response to conventional pharmacological treatment like immunosuppressive agents. So, in patients with lung fibrosis there seems a clear indication to refer them for comprehensive pulmonary rehabilitation programmes and to initiate palliative care in an early phase of the disease. In the current review we will present a rationale for pulmonary rehabilitation in patients with lung fibrosis and the effects of this type of non-pharmacological intervention on exercise capacity and quality of life. In addition, we will discuss possibilities for palliative care in these patients.

Sleep quality and health-related quality of life in idiopathic pulmonary fibrosis.

BACKGROUND: Idiopathic pulmonary fibrosis (IPF) is a progressive disorder resulting in irreversible scarring of the lung parenchyma. Although fatigue is a prominent symptom for patients with IPF, little is known about sleep quality in patients with IPF. METHODS: In this cross-sectional study of 41 patients with IPF from a prospectively designed cohort, we ascertained sleep quality by means of the Pittsburgh sleep quality index (PSQI) and the Epworth sleepiness scale. Health status, baseline demographics, and physiologic parameters were also assessed. RESULTS: Patients with IPF reported extremely poor sleep quality and high frequency of daytime sleepiness, which differs significantly from normal control populations. Further, poor sleep quality was not associated with body mass index, age, gender, or lung function. This population also demonstrated extremely poor health status in a number of domains, including physical function and vitality. Poor sleep quality (by the global PSQI) was significantly associated with decreased quality of life (QOL) in several domains, including role of physical function (r = - 0.58, p = 0.001), vitality (r = - 0.43, p = 0.015), and role of emotions (r = - 0.40, p = 0.023). CONCLUSIONS: Poor sleep quality is extremely common in patients with IPF and is not predicted by variables traditionally associated with sleep-disordered breathing. Further, poor sleep quality is associated with poor QOL. These findings suggest that systematic evaluation of the cause of poor sleep quality in IPF is merited.

Health-related quality of life in patients with idiopathic pulmonary fibrosis--cross-sectional and longitudinal study.

OBJECT: To validate the cross-sectional and longitudinal use of the Medical Outcome Study Short Form 36 (SF-36) for measuring health-related quality of life (HRQL) in patients with idiopathic pulmonary fibrosis (IPF). METHODS: Patients were administered the SF-36 and concomitantly underwent laboratory and physiologic tests and high-resolution computed tomography (HRCT). Forty-six patients participated in the initial cross-sectional study, and 32 patients who were available more than one year later again underwent these studies under the same conditions. RESULTS: Patients with IPF had significantly lower scores across all 8 domains of the SF-36 when compared with the general population. Significant decline of HRQL was observed in 2 physical domains. There were significant differences in within-subject changes in a few domains according to worsening of the physiologic parameters. Vital capacity as percent of predicted was significantly correlated with the results of 6 subscales and its changes were significantly correlated with those of 4 subscales. The 6-min-walk distance was correlated significantly with 3 subscales and its changes were significantly correlated with those of 4 subscales. Changes in the HRCT ground-glass score were significantly correlated with those of 3 subscales. No significant correlations between changes in 3 domains and those of any clinical parameters were observed. CONCLUSION: Patients with IPF had significantly impaired HRQL in both physical and psychological functions. This disease clearly decreased the physical aspects of HRQL over time. HRQL instruments should be incorporated into routine evaluations of IPF patients, since they measure dimensions not fully estimated by clinical assessment.

Comparison of two questionnaires which measure the health-related quality of life of idiopathic pulmonary fibrosis patients.

The objective of the present study was to determine if there is a health-related quality of life (HRQL) instrument, generic or specific, that better represents functional capacity dysfunction in idiopathic pulmonary fibrosis (IPF) patients. HRQL was evaluated in 20 IPF patients using generic and specific questionnaires (Medical Outcomes Short Form 36 (SF-36) and Saint George's Respiratory Questionnaire (SGRQ), respectively). Functional status was evaluated by pulmonary function tests, 6-min walking distance test (6MWDT) and dyspnea indexes (baseline dyspnea index) at rest and after exercise (modified Borg scale). There was a restrictive pattern with impairment of diffusion capacity (total lung capacity, TLC = 71.5 +/- 15.6%, forced vital capacity = 70.4 +/- 19.4%, and carbon monoxide diffusing capacity = 41.5 +/- 16.2% of predicted value), a reduction in exercise capacity (6MWDT = 435.6 +/- 95.5 m) and an increase of perceived dyspnea score at rest and during exercise (6 +/- 2.5 and 7.1 +/- 1.3, respectively). Both questionnaires presented correlation with some functional parameters (TLC, forced expiratory volume in 1 s and carbon monoxide diffusing capacity) and the best correlation was with TLC. Almost all of the SGRQ domains presented a strong correlation with functional status, while in SF-36 only physical function and vitality presented a good correlation with functional status. Dyspnea index at rest and 6MWDT also presented a good correlation with HRQL. Our results suggest that a specific instead of a generic questionnaire is a more appropriate instrument for HRQL evaluation in IPF patients and that TLC is the functional parameter showing best correlation with HRQL.