Symptom prevalence of patients with fibrotic interstitial lung disease: A systematic literature review

© 2018 The Author(s). Background: Those affected by advanced fibrotic interstitial lung diseases have limited treatment options and in the terminal stages, the focus of care is on symptom management. However, quantitatively, little is known about symptom prevalence. We aimed to determine the prevalence of symptoms in Progressive Idiopathic Fibrotic Interstitial Lung Disease (PIF-ILD). Methods: Searches on eight electronic databases including MEDLINE for clinical studies between 1966 and 2015 where the target population was adults with PIF-ILD and for whom the prevalence of symptoms had been calculated. Results: A total of 4086 titles were screened for eligibility criteria; 23 studies were included for analysis. The highest prevalence was that for breathlessness (54-98%) and cough (59-100%) followed by heartburn (25-65%) and depression (10-49%). The heterogeneity of studies limited their comparability, but many of the symptoms present in patients with other end-stage disease were also seen in PIF-ILD. Conclusions: This is the first quantitative review of symptoms in people with Progressive Idiopathic Fibrotic Interstitial Lung Diseases. Symptoms are common, often multiple and have a comparable prevalence to those experienced in other advanced diseases. Quantification of these data provides valuable information to inform the allocation of resources

Researching minoritised communities in palliative care: An agenda for change

Background: Palliative care access, experiences and outcomes of care disadvantage those from ethnically diverse, Indigenous, First nation and First people communities. Research into this field of inquiry raises unique theoretical, methodological, and moral issues. Without the critical reflection of methods of study and reporting of findings, researchers may inadvertently compromise their contribution to reducing injustices and perpetuating racism. Aim: To examine key evidence of the place of minoritised communities in palliative care research to devise recommendations that improve the precision and rigour of research and reporting of findings. Methods: Narrative review of articles identified from PubMed, CINAHL and Google Scholar for 10 years augmented with supplementary searches. Results: We identified and appraised 109 relevant articles. Four main themes were identified (i) Lack of precision when working with a difference; (ii) ‘black box epidemiology’ and its presence in palliative care research; (iii) the inclusion of minoritised communities in palliative care research; and (iv) the potential to cause harm. All stymie opportunities to ‘level up’ health experiences and outcomes across the palliative care spectrum. Conclusions: Based on the findings of this review palliative care research must reflect on and justify the classification of minoritised communities, explore and understand intersectionality, optimise data quality, decolonise research teams and methods, and focus on reducing inequities to level up end-of-life care experiences and outcomes. Palliative care research must be forthright in explicitly indentifying instances of structural and systemic racism in palliative care research and engaging in non-judgemental debate on changes required

One evidence base; three stories: do opioids relieve chronic breathlessness?

Published by the BMJ Publishing Group Limited. For permission to use (where not already granted under a licence) please go to http://www.bmj.com/company/products-services/rights-and-licensing/. The efficacy of low-dose systemic opioids for chronic breathlessness was questioned by the recent Cochrane review by Barnes et al We examined the reasons for this conflicting finding and re-evaluated the efficacy of systemic opioids. Compared with previous meta-analyses, Barnes et al reported a smaller effect and lower precision, but did not account for matched data of crossover trials (11/12 included trials) and added a risk-of-bias criterion (sample size). When re-analysed to account for crossover data, opioids decreased breathlessness (standardised mean differences -0.32; -0.18 to -0.47; I2=44.8%) representing a clinically meaningful reduction of 0.8 points (0-10 numerical rating scale), consistent across meta-analyses

To What Extent Do the NRS and CRQ Capture Change in Patients' Experience of Breathlessness in Advanced Disease? Findings From a Mixed-Methods Double-Blind Randomized Feasibility Trial.

CONTEXT: Chronic or refractory breathlessness is common and distressing. To evaluate new treatments, outcome measures that capture change in patients' experience are needed. OBJECTIVES: To explore the extent to which the numerical rating scale (NRS) worst and average, and the Chronic Respiratory Questionnaire capture change in patients' experience during a trial of mirtazapine for refractory breathlessness. METHODS: Convergent mixed-methods design embedded within a randomized trial comprising 1) semi-structured qualitative interviews (considered to be the gold standard) and 2) outcome measure data collected pre- and post-intervention. Data were integrated, exploring examples where findings agreed and disagreed. Adults with advanced cancer, chronic obstructive pulmonary disease, interstitial lung disease, or chronic heart failure, with a modified Medical Research Council dyspnea scale grade 3 or 4 were recruited from three U.K. sites. RESULTS: Data were collected for 22 participants. Eleven had a diagnosis of chronic obstructive pulmonary disease, eight interstitial lung disease, two chronic heart failure, and one cancer. Median age was 71 (56-84) years. Sixteen participants were men. Changes in the qualitative data were commonly captured in the NRS (worst and average) and the Chronic Respiratory Questionnaire. The NRS worst captured change most frequently. Improvement in the emotional domain was associated with physical changes, improved confidence, and control. CONCLUSION: This study found that the NRS using the question "How bad has your breathlessness felt at its worst over the past 24 hours?" captured change across multiple domains, and therefore may be an appropriate primary outcome measure in trials in this population. Future work should confirm the construct validity of this question

Holistic services for people with advanced disease and chronic breathlessness: a systematic review and meta-analysis

Background: Breathlessness is a common, distressing symptom in people with advanced disease and a marker of deterioration. Holistic services that draw on integrated palliative care have been developed for this group. This systematic review aimed to examine the outcomes, experiences and therapeutic components of these services. Methods: Systematic review searching nine databases to June 2017 for experimental, qualitative and observational studies. Eligibility and quality were independently assessed by two authors. Data on service models, health and cost outcomes were synthesised, using meta-analyses as indicated. Data on recipient experiences were synthesised thematically and integrated at the level of interpretation and reporting. Results: From 3239 records identified, 37 articles were included representing 18 different services. Most services enrolled people with thoracic cancer, involved palliative care staff and comprised 4-6 contacts over 4-6 weeks. Commonly used interventions included breathing techniques, psychological support and relaxation techniques. Meta-analyses demonstrated reductions in Numeric Rating Scale distress due to breathlessness (n=324; mean difference (MD) '2.30, 95% CI '4.43 to '0.16, p=0.03) and Hospital Anxiety and Depression Scale (HADS) depression scores (n=408, MD '1.67, 95% CI '2.52 to '0.81, p<0.001) favouring the intervention. Statistically non-significant effects were observed for Chronic Respiratory Questionnaire (CRQ) mastery (n=259, MD 0.23, 95% CI '0.10 to 0.55, p=0.17) and HADS anxiety scores (n=552, MD '1.59, 95% CI '3.22 to 0.05, p=0.06). Patients and carers valued tailored education, self-management interventions and expert staff providing person-centred, dignified care. However, there was no observable effect on health status or quality of life, and mixed evidence around physical function. Conclusion: Holistic services for chronic breathlessness can reduce distress in patients with advanced disease and may improve psychological outcomes of anxiety and depression. Therapeutic components of these services should be shared and integrated into clinical practice. Registration number: CRD42017057508

The effectiveness and cost-effectiveness of hospital-based specialist palliative care for adults with advanced illness and their caregivers

BackgroundSerious illness is often characterised by physical/psychological problems, family support needs, and high healthcare resource use. Hospital‐based specialist palliative care (HSPC) has developed to assist in better meeting the needs of patients and their families and potentially reducing hospital care expenditure. There is a need for clarity on the effectiveness and optimal models of HSPC, given that most people still die in hospital and also to allocate scarce resources judiciously.ObjectivesTo assess the effectiveness and cost‐effectiveness of HSPC compared to usual care for adults with advanced illness (hereafter patients) and their unpaid caregivers/families.Search methodsWe searched CENTRAL, CDSR, DARE and HTA database via the Cochrane Library; MEDLINE; Embase; CINAHL; PsycINFO; CareSearch; National Health Service Economic Evaluation Database (NHS EED) and two trial registers to August 2019, together with checking of reference lists and relevant systematic reviews, citation searching and contact with experts to identify additional studies.Selection criteriaWe included randomised controlled trials (RCTs) evaluating the impact of HSPC on outcomes for patients or their unpaid caregivers/families, or both. HSPC was defined as specialist palliative care delivered by a palliative care team that is based in a hospital providing holistic care, co‐ordination by a multidisciplinary team, and collaboration between HSPC providers and generalists. HSPC was provided to patients while they were admitted as inpatients to acute care hospitals, outpatients or patients receiving care from hospital outreach teams at home. The comparator was usual care, defined as inpatient or outpatient hospital care without specialist palliative care input at the point of entry into the study, community care or hospice care provided outside of the hospital setting.Data collection and analysisWe used standard methodological procedures expected by Cochrane. We assessed risk of bias and extracted data. To account for use of different scales across studies, we calculated standardised mean differences (SMDs) with 95% confidence intervals (CIs) for continuous data. We used an inverse variance random‐effects model. For binary data, we calculated odds ratio (ORs) with 95% CIs. We assessed the evidence using GRADE and created a 'Summary of findings' table.Our primary outcomes were patient health‐related quality of life (HRQoL) and symptom burden (a collection of two or more symptoms). Key secondary outcomes were pain, depression, satisfaction with care, achieving preferred place of death, mortality/survival, unpaid caregiver burden, and cost‐effectiveness. Qualitative data was analysed where available.Main resultsWe identified 42 RCTs involving 7779 participants (6678 patients and 1101 caregivers/family members). Twenty‐one studies were with cancer populations, 14 were with non‐cancer populations (of which six were with heart failure patients), and seven with mixed cancer and non‐cancer populations (mixed diagnoses).HSPC was offered in different ways and included the following models: ward‐based, inpatient consult, outpatient, hospital‐at‐home or hospital outreach, and service provision across multiple settings which included hospital. For our main analyses, we pooled data from studies reporting adjusted endpoint values. Forty studies had a high risk of bias in at least one domain.Compared with usual care, HSPC improved patient HRQoL with a small effect size of 0.26 SMD over usual care (95% CI 0.15 to 0.37; I2 = 3%, 10 studies, 1344 participants, low‐quality evidence, higher scores indicate better patient HRQoL). HSPC also improved other person‐centred outcomes. It reduced patient symptom burden with a small effect size of ‐0.26 SMD over usual care (95% CI ‐0.41 to ‐0.12; I2 = 0%, 6 studies, 761 participants, very low‐quality evidence, lower scores indicate lower symptom burden). HSPC improved patient satisfaction with care with a small effect size of 0.36 SMD over usual care (95% CI 0.41 to 0.57; I2 = 0%, 2 studies, 337 participants, low‐quality evidence, higher scores indicate better patient satisfaction with care). Using home death as a proxy measure for achieving patient's preferred place of death, patients were more likely to die at home with HSPC compared to usual care (OR 1.63, 95% CI 1.23 to 2.16; I2 = 0%, 7 studies, 861 participants, low‐quality evidence). Data on pain (4 studies, 525 participants) showed no evidence of a difference between HSPC and usual care (SMD ‐0.16, 95% CI ‐0.33 to 0.01; I2 = 0%, very low‐quality evidence). Eight studies (N = 1252 participants) reported on adverse events and very low‐quality evidence did not demonstrate an effect of HSPC on serious harms. Two studies (170 participants) presented data on caregiver burden and both found no evidence of effect of HSPC (very low‐quality evidence). We included 13 economic studies (2103 participants). Overall, the evidence on cost‐effectiveness of HSPC compared to usual care was inconsistent among the four full economic studies. Other studies that used only partial economic analysis and those that presented more limited resource use and cost information also had inconsistent results (very low‐quality evidence).Quality of the evidenceThe quality of the evidence assessed using GRADE was very low to low, downgraded due to a high risk of bias, inconsistency and imprecision.Authors' conclusionsVery low‐ to low‐quality evidence suggests that when compared to usual care, HSPC may offer small benefits for several person‐centred outcomes including patient HRQoL, symptom burden and patient satisfaction with care, while also increasing the chances of patients dying in their preferred place (measured by home death). While we found no evidence that HSPC causes serious harms, the evidence was insufficient to draw strong conclusions. Although these are only small effect sizes, they may be clinically relevant at an advanced stage of disease with limited prognosis, and are person‐centred outcomes important to many patients and families. More well conducted studies are needed to study populations with non‐malignant diseases and mixed diagnoses, ward‐based models of HSPC, 24 hours access (out‐of‐hours care) as part of HSPC, pain, achieving patient preferred place of care, patient satisfaction with care, caregiver outcomes (satisfaction with care, burden, depression, anxiety, grief, quality of life), and cost‐effectiveness of HSPC. In addition, research is needed to provide validated person‐centred outcomes to be used across studies and populations

Holistic services for people with advanced disease and chronic or refractory breathlessness: a mixed-methods evidence synthesis

Background : Breathlessness is a common and distressing symptom of many advanced diseases, affecting around 2 million people in the UK. Breathlessness increases with disease progression and often becomes chronic or refractory. Breathlessness-triggered services that integrate holistic assessment and specialist palliative care input as part of a multiprofessional approach have been developed for this group, offering tailored interventions to support self-management and reduce distress. Objectives : The aim was to synthesise evidence on holistic breathlessness services for people with advanced disease and chronic or refractory breathlessness. The objectives were to describe the structure, organisation and delivery of services, determine clinical effectiveness, cost-effectiveness and acceptability, identify predictors of treatment response, and elicit stakeholders’ evidence-based priorities for clinical practice, policy and research. Design : The mixed-methods evidence synthesis comprised three components: (1) a systematic review to determine the clinical effectiveness, cost-effectiveness and acceptability of holistic breathlessness services; (2) a secondary analysis of pooled individual data from three trials to determine predictors of clinical response; and (3) a transparent expert consultation (TEC), comprising a stakeholder workshop and an online consensus survey, to identify stakeholders’ priorities. Results : Thirty-seven papers reporting on 18 holistic breathlessness services were included in the systematic review. Most studies enrolled people with thoracic cancer, were delivered over 4–6 weeks, and included breathing training, relaxation techniques and psychological support. Meta-analysis demonstrated significant reductions in the Numeric Rating Scale (NRS) distress due to breathlessness, significant reductions in the Hospital Anxiety and Depressions Scale (HADS) depression scores, and non-significant reductions in the Chronic Respiratory Disease Questionnaire (CRQ) mastery and HADS anxiety, favouring the intervention. Recipients valued education, self-management interventions, and expertise of the staff in breathlessness and person-centred care. Evidence for cost-effectiveness was limited and inconclusive. The responder analysis (n = 259) revealed baseline CRQ mastery and NRS distress to be strong predictors of the response to breathlessness services assessed by these same measures, and no significant influence from baseline breathlessness intensity, patient diagnosis, lung function, health status, anxiety or depression. The TEC elicited 34 priorities from stakeholders. Seven priorities received high agreement and consensus, reflecting stakeholders’ (n = 74) views that services should be person-centred and multiprofessional, share their breathlessness management skills with others, and recognise the roles and support needs of informal carers.  Limitations : The evidence synthesis draws predominantly from UK services and may not be generalisable to other settings. Some meta-analyses were restricted by reporting biases and statistical heterogeneity.  Conclusions : Despite heterogeneity in composition and delivery, holistic breathlessness services are highly valued by recipients and can lead to significant improvements in the distress caused by breathlessness and depression. Outcomes of improved mastery and reduced distress caused by breathlessness are not influenced by patient diagnosis, lung function or health status. Stakeholders highlighted the need for improved access to person-centred, multi professional breathlessness services and support for informal carers.  Future work : Our research suggests that key therapeutic components of holistic breathlessness services be considered in clinical practice and models of delivery and educational strategies to address stakeholders’ priorities tested

‘Necessity is the mother of invention’: Specialist palliative care service innovation and practice change in response to COVID-19. Results from a multinational survey (CovPall)

Background:Specialist palliative care services have a key role in a whole system response to COVID-19, a disease caused by the SARS-CoV-2 virus. There is a need to understand service response to share good practice and prepare for future care.Aim:To map and understand specialist palliative care services innovations and practice changes in response to COVID-19.Design:Online survey of specialist palliative care providers (CovPall), disseminated via key stakeholders. Data collected on service characteristics, innovations and changes in response to COVID-19. Statistical analysis included frequencies, proportions and means, and free-text comments were analysed using a qualitative framework approach.Setting/participants:Inpatient palliative care units, home nursing services, hospital and home palliative care teams from any country.Results:Four hundred and fifty-eight respondents: 277 UK, 85 Europe (except UK), 95 World (except UK and Europe), 1 missing country. 54.8% provided care across 2+ settings; 47.4% hospital palliative care teams, 57% in-patient palliative care units and 57% home palliative care teams. The crisis context meant services implemented rapid changes. Changes involved streamlining, extending and increasing outreach of services, using technology to facilitate communication, and implementing staff wellbeing innovations. Barriers included; fear and anxiety, duplication of effort, information overload and funding. Enablers included; collaborative teamwork, staff flexibility, a pre-existing IT infrastructure and strong leadership.Conclusions:Specialist palliative care services have been flexible, highly adaptive and have adopted low-cost solutions, also called ‘frugal innovations’, in response to COVID-19. In addition to financial support, greater collaboration is essential to minimise duplication of effort and optimise resource use

Randomised, double-blind, multicentre, mixed-methods, dose-escalation feasibility trial of mirtazapine for better treatment of severe breathlessness in advanced lung disease (BETTER-B feasibility)

© Author(s) (or their employer(s)) 2020. Re-use permitted under CC BY. Published by BMJ. New treatments are required for severe breathlessness in advanced disease. We conducted a randomised feasibility trial of mirtazapine over 28 days in adults with a modified medical research council breathlessness scale score ≥3. Sixty-four patients were randomised (409 screened), achieving our primary feasibility endpoint of recruitment. Most patients had COPD or interstitial lung disease; 52 (81%) completed the trial. There were no differences between placebo and mirtazapine in tolerability or safety, and blinding was maintained. Worst breathlessness ratings at day 28 (primary clinical activity endpoint) were, 7.1 (SD 2.3, placebo) and 6.3 (SD 1.8, mirtazapine). A phase III trial of mirtazapine is indicated. Trial registration: ISRCTN 32236160; European Clinical Trials Database (EudraCT no: 2015-004064-11)

Psychometric validation of the needs assessment tool : progressive disease in interstitial lung disease

ABSTRACT The inter-rater/test–retest reliability and construct validity of a palliative care needs assessment tool in interstitial lung disease (NAT:PD-ILD) were tested using NAT:PDILD- guided video-recorded consultations, and NAT:PD-ILD-guided consultations, and patient and carer-report outcomes (St George’s Respiratory Questionnaire (SGRQ)-ILD, Carer Strain Index (CSI)/Carer Support Needs Assessment Tool (CSNAT)). 11/16 items reached at least fair inter-rater agreement; 5 items reached at least moderate test–retest agreement. 4/6 patient constructs demonstrated agreement with SGRQ-I scores (Kendall’s tau-b, 0.24–20.36; P<0.05). 4/7 carer constructs agreed with the CSI/CSNAT items (kappa, 0.23–20.53). The NAT:PD-ILD is reliable and valid. Clinical effectiveness and implementation are to be evaluated