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Specialist Palliative Care is More Than Drugs: A Retrospective Study of ILD Patients
Abstract and Figures
This study aimed to assess the palliative care needs of progressive idiopathic fibrotic interstitial lung disease (PIF-ILD) populations in two London ILD centres.
Patients' records from Royal Brompton Hospital (RBH) and King's College Hospital (KCH) were extracted to assess palliative care needs, use of palliative treatments, and whether end-of-life preferences were documented and achieved.
Forty-five PIF-ILD patients were identified (26 RBH, 19 KCH). Patients at RBH were younger (37-81 years, median = 61 years) and predominantly white British (23/26) compared to KCH's older, more racially diverse population (70-99 years, median = 82 years, 6/19 nonwhite). Seventeen of 45 patients had specialist palliative care team involvement. Nearly all patients (42/45) experienced breathlessness in their last year of life. Additional symptoms included cough, fatigue, depression/anxiety, and chest pain. All patients given opioids (22/45) or benzodiazepines (8/45) had documented benefit. Nonpharmacological treatments were rarely used. Few patients had preferred place of care (8/45) or preferred place of death (6/45) documented.
Despite demographic variation, the patient populations at the two hospitals experienced similar symptoms. There was use of standard pharmacological treatments with symptom benefit. Nonpharmacological interventions were seldom used and documentation of preferred place of care and preferred place of death was poor.
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... The World Health Organization recommends early palliative care intervention to improve the quality of life of patients and their families facing problems associated with a life-threatening illness. Since the course of IPF is unpredictable, early palliative care interventions can be beneficial in a multiplicity of ways including symptom management, [20,84] emotional support and in the initiation of advance care planning conversations [59]. ...
... [47,61] Startlingly, despite these recommendations, patients with IPF do not receive optimal palliative care over the course of their disease, resulting in high symptom burden and decreased quality of life for patients. [59,84] There is increasing evidence that patients with IPF do not always have access to palliative care input and when it is introduced the timeline is rarely optimal. ...
... A critical unmet need for patients with IPF is timely referral to palliative care as part of their overall management plan and care package. Despite calls from several patient charters and international IPF position statements [20,35] highlighting the importance of access to palliative and supportive care services to manage symptoms, [47,61] there continues to be poor palliative care referral and access to palliative care specialists, directly impacting patients' quality of life [59,84]. ...
Aims
Patients diagnosed with idiopathic pulmonary fibrosis (IPF) have a high symptom burden and numerous needs that remain largely unaddressed despite advances in available treatment options. There is a need to comprehensively identify patients’ needs and create opportunities to address them. This scoping review aimed to synthesise the available evidence and identify gaps in the literature regarding the unmet needs of patients diagnosed with IPF.
Methods
The protocol for the review was registered with Open Science Framework (DOI 10.17605/OSF.IO/SY4KM ). A systematic search was performed in March 2022, in CINAHL, MEDLINE, Embase, PsychInfo, Web of Science Core Collection and ASSIA Applied Social Science Index. A comprehensive review of grey literature was also completed. Inclusion criteria included patients diagnosed with IPF and date range 2011–2022. A range of review types were included. Data was extracted using a data extraction form. Data was analysed using descriptive and thematic analysis. A total of 884 citations were reviewed. Ethical approval was not required.
Results
52 citations were selected for final inclusion. Five themes were identified: 1.) psychological impact of an IPF diagnosis. 2.) adequate information and education: at the right time and in the right way. 3.) high symptom burden support needs. 4.) referral to palliative care and advance care planning (ACP). 5.) health service provision-a systems approach.
Conclusion
This review highlights the myriad of needs patients with IPF have and highlights the urgent need for a systems approach to care, underpinned by an appropriately resourced multi-disciplinary team. The range of needs experienced by patients with IPF are broad and varied and require a holistic approach to care including targeted research, coupled with the continuing development of patient-focused services and establishment of clinical care programmes.
... Patients with chronic progressive pulmonary diseases, such as chronic obstructive pulmonary disease (COPD) or interstitial lung diseases (ILDs), have as high symptom burden in end of life as patients with lung cancer. [1][2][3][4] Already years before death, patients with nonmalignant pulmonary diseases suffer from breathlessness, cough, fatigue, anxiety, depression, and pain, and their quality of life is often markedly decreased. [5][6][7][8][9] Compared with patients with cancer, a few patients with chronic pulmonary diseases receive palliative care. ...
... In nonmalignant pulmonary diseases, receiving palliative care is associated with better symptom relief and quality of life, increased rate of advance care planning, death outside the hospital, and decreased burden from futile therapies and investigations in end of life. 3,10,18,19,[27][28][29][30] The symptom burden and the need of palliative care in different nonmalignant pulmonary diseases are similar but the disease trajectory varies. 31,32 Patients with COPD may live long with advanced disease and severe breathlessness, whereas survival time is typically shorter among patients with end-stage ILD. ...
Background: Few patients with chronic nonmalignant pulmonary diseases receive specialist palliative care consultation, despite their high symptom burden in end of life.
Objectives: To study palliative care decision making, survival, and hospital resource usage in patients with nonmalignant pulmonary diseases with or without a specialist palliative care consultation.
Methods: A retrospective chart review of all patients with a chronic nonmalignant pulmonary disease and a palliative care decision (palliative goal of therapy), who were treated in Tampere University Hospital, Finland, between January 1, 2018 and December 31, 2020.
Results: A total of 107 patients were included in the study, 62 (58%) had chronic obstructive pulmonary disease (COPD), and 43 (40%) interstitial lung disease (ILD). Median survival after palliative care decision was shorter in patients with ILD than in patients with COPD (59 vs. 213 days, p = 0.004). Involvement of a palliative care specialist in the decision making was not associated with the survival. Patients with COPD who received palliative care consultation visited less often emergency room (73% vs. 100%, p = 0.019) and spent fewer days in the hospital (7 vs. 18 days, p = 0.007) during the last year of life. When a palliative care specialist attended the decision making, the presence and opinions of the patients were recorded more often, and the patients were more frequently referred to a palliative care pathway.
Conclusions: Specialist palliative care consultation seems to enable better end-of-life care and supports shared decision making for patients with nonmalignant pulmonary diseases. Therefore, palliative care consultations should be utilized in nonmalignant pulmonary diseases preferably before the last days of life.
... Dyspnea has been associated with impaired health-related quality of life (HRQoL) [3], exercise tolerance [4,5] and physical activity [6], and it represents the main cause of suffering at the end stage of the disease [7]. A pooled data analysis of multinational phase 3 trials conducted in IPF showed that pirfenidone (antifibrotic agent) had no effect on dyspnea increase over time [8]. ...
Objective
To evaluate the short-, medium- and long-term benefits of home-based PR on the physical and affective components of dyspnea in people with fibrotic idiopathic interstitial pneumonias (f-IIPs). Anxiety and depressive symptoms, fatigue, health-related quality of life and exercise tolerance were also assessed.
Methods
Data on 166 individuals with f-IIPs who enrolled in an 8-week home-based PR programme (weekly supervised 90-minute session) was retrospectively analysed. Assessments included the Dyspnea-12 questionnaire, Hospital Anxiety and Depression Scale, Fatigue Assessment Scale, Visual Simplified Respiratory Questionnaire and Six-Minute Stepper Test and were performed at home at short-, medium- (6 months) and long- (12 months) term.
Results
Among the 166 individuals with f-IIPs who enrolled in PR, 75 (45%) and 91 (55%) participants had a diagnosis of idiopathic pulmonary fibrosis and fibrosing non-specific interstitial pneumonia, respectively and 87 (52%) participants concluded a full year of follow-up. In the total group, both physical and affective components of dyspnea were improved, at short-, medium- and long-term, after PR. Overall, half of the participants reached the minimally important difference of 3 points of the dyspnea-12 questionnaire at the end of PR, and at the 6- and 12-month follow-up. Anxiety and depressive symptoms, fatigue and health-related quality of life were also improved, while the short benefits in exercise tolerance were not maintained one-year after PR.
Conclusion
Individualised home-based PR programme resulted in short-, medium- and long-term improvements in both physical and affective components of dyspnea assessed by the D-12 questionnaire.
... Chronic, or persistent, breathlessness, defined as persistent disabling breathlessness despite optimal treatment of the underlying pathophysiology [1] , is frightening, worsens with disease progression, and is associated with poor quality of life, physical and psychosocial limitations, and high health service utilisation [2][3][4][5] . It is prevalent in chronic progressive illnesses, affecting nearly everyone with advanced lung cancer [6] , non-malignant chronic lung disease [7,8] , and heart failure [9] . Non-pharmacological interventions for breathlessness form the bedrock of management, but there is an emerging evidencebase for morphine [10][11][12][13] . ...
Chronic breathlessness, a persistent and disabling symptom despite optimal treatment of underlying causes, is a frightening symptom with serious and widespread impact on patients and their carers. Clinical guidelines support the use of morphine for the relief of chronic breathlessness in common long-term conditions, but questions remain around clinical effectiveness, safety and longer-term (>7 days) administration. This trial will evaluate the effectiveness of low-dose oral modified release morphine in chronic breathlessness.
This is a multicentre, parallel group, double-blind, randomised, placebo-controlled trial. Participants [n=158] will be opioid-naïve with chronic breathlessness due to heart or lung disease, cancer or post-COVID-19. Participants will be randomised 1:1 to 5 mg oral modified-release morphine/placebo twice daily and docusate/placebo 100 mg twice daily for 56 days. Non-responders at Day 7 will dose escalate to 10 mg morphine/placebo twice daily at Day 15.
The primary endpoint (Day 28) measure will be worst breathlessness severity (past 24 h). Secondary outcome measures include worst cough, distress, pain; functional status; physical activity; quality of life; and early identification and management of morphine-related side effects. At Day 56, participants may opt to take open-label, oral modified-release morphine as part of usual care and complete quarterly breathlessness and toxicity questionnaires.
The study is powered to be able to reject the null hypothesis and an embedded normalisation process theory-informed qualitative sub-study will explore the adoption of morphine as a first-line pharmacological treatment for chronic breathlessness in clinical practice if effective.
... In this regard, in a study of 27 patients with IPF treated with opioids for worsening dyspnoea, none showed any significant increase in PaCO 2 or reduction in PaO 2 and all patients experienced significant relief of dyspnoea and decreased respiratory rate. The authors concluded that opioids reduce work of breathing, hence decrease respiratory rate, but do not affect alveolar ventilation [34]. This is also supported by the results of other small retrospective studies and, therefore, opioids should be evaluated as an early palliative treatment in these patients [35,36]. ...
Idiopathic pulmonary fibrosis (IPF) is a chronic disease with an unknown etiology that causes deterioration of the structure of the lung parenchyma, resulting in a severe and progressive decline in respiratory function and early mortality. IPF is essentially an incurable disease, with a mean overall survival of 5 years in approximately 20% of patients without treatment. The combination of a poor prognosis, uncertainty about the disease's progression, and the severity of symptoms has a significant impact on the quality of life of patients and their families. New antifibrotic drugs have been shown to slow disease progression, but their impact on health-related quality of life (HRQoL) has to be proven yet. To date, studies have shown that palliative care can improve symptom management, HRQoL, and end-of-life care (EoL) in patients with IPF, reducing critical events, hospitalization, and health costs. As a result, it is essential for proper health planning and patient management to establish palliative care early and in conjunction with other therapies, beginning with the initial diagnosis of the disease.
Background: Palliative care (PC) interventions aim to relieve and prevent suffering in the physical, psychosocial, and spiritual domain. Therefore, palliative care is carried out by a multi-professional team with different occupations (e.g., physician, nurse, psychologist, social worker and chaplain). Remaining skepticism concerning the need for palliative care may be based on the scarcity of high-quality evaluations about the external evidence for palliative care. Therefore, the aim of a systematic review is to examine the effects of palliative care for adults with life threatening illness.
Methods/design: The comprehensive systematic literature search will include randomized controlled trials (RCTs) and cluster RCTs. We will search the databases MEDLINE, EMBASE, Cochrane Central Register of Controlled Trials (CENTRAL), and Pubmed. Patients must be adults suffering from life-limiting diseases. Proxy and caregiver outcomes will not be assessed in order to ensure a clear and well-defined research question for this review. Interventions may be in or outpatient setting, e.g., consulting service, palliative care ward, and palliative outpatient clinic. In line with the multi-dimensional scope of palliative care, the primary outcome is quality of life (QoL). Key secondary outcomes are patients’ symptom burden, place of death and survival, and health economic aspects. We used the Cochrane Collaboration risk of bias assessment tool. The quality of evidence judged according to the Grading of Recommendations Assessment, Development, and Evaluation (GRADE) approach.
Discussion: The available evidence summarized and discussed to provide a basis for decision-making among health care professionals and policy makers. For PC, we believe that multi-professional care is of utmost importance. Therefore, single-profession interventions such as physician consultations would not be included. Based on the multidimensional scope of palliative care, we chose QoL as the primary outcome, despite an expected heterogeneity among the QoL outcomes. The statement suggests that using one-dimensional endpoints such as "pain" to measure the scope of patient care (i.e., quality of life) may be inadequate, as per the World Health Organization's definition. This implies that a more comprehensive approach is needed to capture the full extent of patient well-being.
Background: Research on health-related quality of life (HRQoL) is crucial for developing comprehensive palliative care in idiopathic pulmonary fibrosis (IPF). Objectives: To study IPF patients' HRQoL compared with general population and its association with dyspnea in a longitudinal follow-up. Design: Assessment of IPF patients' HRQoL by a generic tool. Comparison of baseline data with the general population and a 30-month follow-up with 6 months intervals. Setting/Subjects: In total, 246 IPF patients were recruited from the Finnish nationwide real-life study, FinnishIPF. Measurements: Modified Medical Research Council (MMRC) dyspnea scale for dyspnea and the generic HRQoL tool 15D for the total and dimensional HRQoL were used. Results: At baseline, the mean 15D total score was lower (0.786, standard deviation [SD] 0.116) in IPF patients than in the general population (0.871, SD 0.043) (p < 0.001) and among the IPF patients with MMRC ≥2 compared with those with MMRC <2 (p < 0.001). In patients with MMRC ≥2, significant impairment compared with general population existed in 11 dimensions of HRQoL, such as breathing, usual activities, and sexual activity, whereas this was true in only 4 dimensions in MMRC <2 category. Mental function was not impaired in either group. During the follow-up, 15D total score decreased in both MMRC categories (p < 0.001) but stayed constantly worse in the MMRC ≥2 group. Seven and two dimensions of HRQoL significantly declined in the categories of MMRC <2 and MMRC ≥2, respectively. Conclusions: Patients with IPF, especially if dyspnea limits everyday life, suffer from widely impaired HRQoL, although self-assessed mental capability is preserved. Integrated palliative care is supported to face the multiple needs of IPF patients.
There is increased awareness of palliative care needs in people with chronic obstructive pulmonary disease (COPD) or interstitial lung disease (ILD). This European Respiratory Society (ERS) task force aimed to provide recommendations for initiation and integration of palliative care into the respiratory care of adult people with COPD or ILD.
The ERS task force consisted of 20 members, including representatives of people with COPD or ILD and informal caregivers. Eight questions were formulated, four in the ‘Population, Intervention, Comparison, Outcome’ (PICO) format. These were addressed with full systematic reviews and application of Grading of Recommendations Assessment, Development and Evaluation (GRADE) for assessing the evidence. Four additional questions were addressed narratively. An ’evidence-to-decision’ framework was used to formulate recommendations.
The following definition of palliative care for people with COPD or ILD was agreed: A holistic and multidisciplinary, person-centred approach aiming to control symptoms, and improve quality of life of people with serious health-related suffering because of COPD or ILD, and to support their informal caregivers. Recommendations were made regarding people with COPD or ILD and their informal caregivers to: consider palliative care when physical, psychological, social, or existential needs are identified through holistic needs assessment; offer palliative care interventions, including support for informal caregivers, in accordance with such needs; offer advance care planning in accordance with preferences; and integrate palliative care into routine COPD and ILD care. Recommendations should be reconsidered as new evidence becomes available.
Background and objective:
Interstitial lung disease (ILD) is progressive with high symptom burdens and poor prognosis. Patients with ILD need optimal palliative care to maintain their quality of life, however, few nationwide surveys have addressed palliative care for ILD.
Methods:
A nationwide, self-administered questionnaire was conducted. Questionnaires were sent by mail to pulmonary specialists certified by the Japanese Respiratory Society (n = 3423). The current practices of PC for ILD, end-of-life communication, referral to a PC team, barriers to PC for ILD, and comparison of PC between ILD and lung cancer (LC).
Results:
1332 (38.9%) participants completed the questionnaire, and the data of 1023 participants who had cared for ILD patients in the last year were analysed. Most participants reported that ILD patients often or always complained of dyspnoea and cough, but only 25% had referred them to a PC team. The timing of end-of-life communication tended to be later than the physician-perceived ideal timing. The participants experienced significantly greater difficulty in symptomatic relief and decision-making in PC for ILD compared to LC. Prescription of opioids for dyspnoea was less frequent for ILD than for LC. ILD-specific barriers in PC included an 'inability to predict prognosis', 'lack of established treatments for dyspnoea', 'shortage of psychological and social support', and 'difficulty for patients/families to accept the disease's poor prognosis'.
Conclusion:
Pulmonary specialists experienced more difficulty in providing PC for ILD compared to LC and reported considerable ILD-specific barriers in PC. Multifaceted clinical studies are needed to develop optimal PC for ILD.
Pulmonary fibrosis is the common end point of more than 150 individual diseases. Within this heterogenous spectrum of diseases, idiopathic interstitial pneumonias (IIPs) represent a unique and quantitatively relevant subgroup. Liebow's original classification of IIPs has been recently replaced by a new international multidisciplinary consensus classification which is based on histological patterns, but allows to link these different forms of IIPs to certain types of clinical manifestations, response to therapy and prognosis. Therefore, the use of the consensus classification to categorize patients with pulmonary fibrosis of unknown etiology will improve the management of these patients in clinical practice, and for the first time allows to perform clinical trials in relatively homogenous groups of patients.
Background: If symptoms are to be recognized and effectively addressed in clinical research, they must be collected using sensitive, specific, reliable, and clinically meaningful methods.
Objective: To perform a comparison of self-administered symptom survey data with data from conventional provider-reports.
Design/Methods: Secondary data analysis of AIDS Clinical Trials Group Study 081 (ACTG 081), a randomized trial taking place in 33 sites comparing three approaches to prophylaxis for Pneumocystis carinii-related pneumonia that found no difference among treatment arms. The study was performed on 842 subjects with advanced HIV infection. No intervention was undertaken as a result of this study. ACTG 081 included data on functional status, global quality of life and survival, and two methods of symptom measurement: an open-ended, provider-reported symptom assessment (provider-report) and a self-administered symptom survey (self-report). Agreement was measured using kappa scores. Sensitivity and specificity were calculated using self-report as the standard. Reliability was measured by intersite variation and test-retest reliability (8 weeks later). Clinical validity was evaluated by testing expected associations with functional status, global quality of life, and survival.
Results: Symptom data were available for 808 patients (96%). Patient and provider agreement was poor (mean [kappa], 0.14; range, 0.07-0.25). Compared with self-report, providers underreported the presence and severity of symptoms (mean symptom count, 5.2 versus 1.3; mean severity score, 1.3 versus 0.74). provider-report demonstrated greater variability by site (R2 associated with site, 0.02 versus 0.16) and poorer test-retest reliability (mean [kappa], 0.34 versus 0.25). Provider-report severity scores were less strongly associated than were self-report with functional status ([chi]2, 252 versus 80), global quality of life (R2 for model, 0.57 versus 0.15), and survival ([chi]2, 38 versus 24). Self-reported symptom severity was strongly correlated to patient-reported global quality of life (p, 0.75; p < .0001).
Conclusions: Provider-reported symptoms as currently collected within the ACTG are less sensitive and reproducible than a self-administered symptom survey. Provider-reported severity scores are also more weakly associated with functional status, global quality of life, and survival. A self-reported symptom survey may provide a better method of symptom measurement for HIV research.
(C) 1999 Lippincott Williams & Wilkins, Inc.
To determine the median survival of patients with cryptogenic fibrosing alveolitis, in comparison to that expected of individuals of the same age and sex from the general population. To estimate the effect of survival bias incurred by studying both incident and prevalent cases. To identify factors associated with survival.
Cohort study.
Nine hospitals from the Trent Region of England.
Two hundred forty-four cases of cryptogenic fibrosing alveolitis: 168 patients were alive on day 1 of the study (prevalent cases), and 76 patients had newly diagnosed conditions over a prospective 18-month period of patient recruitment (incident cases).
Age, sex, date of diagnosis, lung function at presentation, and details of treatment prescribed were extracted from hospital clinical records. Data on lifetime smoking habits were collected by postal questionnaire. Vital status was established from the general practitioner. The expected survival of each case was extracted from the English life tables.
Median survival for incident cases was 2.9 years and for prevalent cases 9 years, compared to expected values of 10 and 13 years, respectively. Incident cases had significantly worse survival than prevalent cases, even after adjustment for the effects of age, sex, smoking history, lung function at presentation, and treatment (hazard ratio, 4.53 [95% confidence interval, 2.71 to 7.56]; p<0.001). Lower FVC at presentation and the use of corticosteroid treatment were both associated with worse survival.
The inclusion of prevalent cases leads to significant overestimation of the median survival for patients with cryptogenic fibrosing alveolitis. In newly diagnosed cases of cryptogenic fibrosing alveolitis, median survival is only 2.9 years, and expected life span is reduced by approximately 7 years.
Decisions to forgo medical interventions are typically made by balancing their benefits and burdens. Often omitted from consideration is the environment in which the proposed treatment is to be administered. A case is presented of a 77-year-old man with end-stage pulmonary fibrosis who developed dependence on high flow oxygen, a technology unavailable in his community outside the hospital setting. Medical staff struggled with the appropriateness of discontinuing the oxygen because it was not the face mask that the patient found burdensome, rather the setting in which the treatment was provided. The case is discussed from the perspective of clinical ethics, organizational ethics, and the law.