Palliative care in the older adult with advanced lung disease

Introduction

Advanced lung disease increases in incidence and prevalence as the population ages, causing escalating symptom burden and disability as individuals age and comorbidities increase. Many life limiting respiratory illnesses are predominantly diseases of older adults, especially chronic obstructive pulmonary disease (COPD), lung cancer, interstitial lung disease (ILD), and bronchiectasis. Related co-morbidities, particularly cardiac disease, can also cause significant respiratory symptoms. Advanced lung disease can be generally categorized into obstructive or restrictive lung diseases. In this chapter, we will focus on COPD as a representative obstructive lung disease and ILD as a representative restrictive lung disease, noting that there is significant overlap in the management of dyspnea and advanced lung disease that may be broadly applicable. We aim to provide a general overview of advanced lung disease for palliative care and primary care clinicians, with an updated perspective regarding aspects of palliative care of the older adult with advanced lung disease, including symptom management, advance care planning (ACP), and end-of-life (EOL) issues.

COPD

COPD is characterized by persistent airflow obstruction or limitation. Obstruction is defined as a decreased post-bronchodilator forced expiratory volume in 1 second (FEV₁) over forced vital capacity (FVC) ratio, thus spirometry is required to confirm the diagnosis. A decreased post-bronchodilator FEV₁/FVC ratio of less than 0.7 is a commonly used diagnostic cutoff, as endorsed by the Global Initiative for Chronic Obstructive Lung Disease (GOLD) (1). It is caused by an inflammatory response in the airways and lungs to noxious particles and gases, most commonly tobacco smoke but also cooking over an open flame, leading to progressive destruction of alveoli and loss of airway recoil. There is a genetic predisposition that is not yet well understood.

COPD has increased in prevalence in recent decades and both morbidity and mortality have risen. COPD is more prevalent in older adults and is the leading cause of respiratory-related mortality in older adults. According to the Centers for Disease Control and Prevention, 13.2% of United States adults over age 75 years had COPD in 2021, up from 11.8% in 2011 (2). It is the third leading cause of death globally (3) and sixth in the United States (2). COPD patients have a high rate of morbidity, and patients who survive hospitalizations from acute exacerbations of COPD (AECOPD) often experience further sequelae of COPD for the remainder of their lives, spending increasing amounts of time in the hospital as COPD progresses (4,5). Compared with patients with lung cancer, patients with COPD are more likely to die with poorly controlled dyspnea (6).

Disease trajectory and prognostication in severe COPD

The disease trajectory and rate of COPD progression are highly variable. Severity of disease is measured using FEV₁, with lower values indicating more severe disease. Additionally, degree of dyspnea, functional impairment, and frequency of exacerbations are taken into account when evaluating patients. Taken together, these factors are used to predict mortality risk, but accuracy remains a challenge, especially in the elderly population (7). The trajectory of COPD is characterized by gradual slow decline in lung function over the course of many years, with marked reduction in functional capacity during AECOPDs. A partial return to the prior level of functioning follows resolution of an individual exacerbation. Even very severe exacerbations may be followed by a slow but significant gradual improvement, thus patients and families may come to expect a similar improvement with future exacerbations. This makes it more difficult to predict a terminal phase and imminent death (6). The challenges in predicting the terminal phase of COPD also make it more difficult to time serious illness conversations and plan for EOL as well as to facilitate enrollment of patients into hospice.

ILD

ILD describes a heterogenous collection of conditions that affect the interstitium of the lungs, rather than the airspaces. The conditions are characterized by inflammation and scarring (fibrosis) in the interstitial space that typically leads to restrictive physiology on pulmonary function testing (PFT), also called restrictive lung disease. This is reflected by a decrease in FEV₁, FVC, and total lung capacity (TLC) as well as impaired diffusion capacity of lung for carbon monoxide (DLCO). Representative conditions include idiopathic pulmonary fibrosis (IPF), hypersensitivity pneumonitis (HP), drug-induced pneumonitis, autoimmune associated lung diseases, and sarcoidosis among others. Cancer treatments such as radiation, chemotherapy, and immunotherapy may also cause ILDs. While ILD is much less common than COPD, its prevalence is increasing, and is highest in those aged 80 to 84 years old (8,9). While standard staging criteria do not exist for these heterogenous conditions, monitoring of progression by PFT and computed tomography (CT) imaging is routine.

Disease trajectory and prognostication in ILD

Like COPD, the progression of disease in ILD is variable and difficult to predict, particularly given the heterogeneity of ILDs. Progression in disease is defined by worsening of respiratory symptoms, decline in FVC and DLCO, and worsening of CT findings. Chronic respiratory failure, both hypoxic and hypercapnic, as well as pulmonary hypertension, are common sequelae of ILD that suggest shorter survival (10). In IPF, progression of disease, as defined by a decrease in FVC, is slowed in the short to medium term, by newer antifibrotic treatments such as pirfenidone and nintedanib (11-13). Despite this, prognosis and survival remain poor in IPF with a median survival of less than 5 years after diagnosis (14-16). Some ILDs may have a more relapsing and remitting course with long periods of relative clinical stability, but fibrosis is permanent and progressive. When there is sustained decline over one year, this predicts a poorer prognosis similar to IPF (10). Acute exacerbations may occur in many of the ILDs with acute worsening in respiratory status. These are also heterogenous ranging from a mild increase in dyspnea and temporary need for supplemental oxygen to severe respiratory failure requiring prolonged intensive care unit stays and possibly mechanical ventilation. Treatment of acute exacerbations are not well defined and evidence is variable, but tend to include treatment for possible infection and pneumonia with antibiotics as well as anti-inflammatories such as steroids of varying strengths and durations. Some patients may experience significant response to treatment of exacerbations, others may not; there are no accurate models to predict patient response. Because of the heterogenous nature of progression and exacerbations, prognostication in ILD is also very difficult. Thus, ACP and palliative care are recommended early in the course of disease, but patients may find this difficult to accept when conditions stabilize for a long time, and patients may assume that they will recover from the next exacerbation as well as they did from the prior.

Palliative care in COPD and ILD

The European Respiratory Society defines palliative care for COPD and ILD as a holistic and multidisciplinary, person-centred approach aiming to control symptoms, and improve quality of life (QOL) of people with serious health-related suffering because of COPD or ILD, and to support their informal caregivers (17). There is a significant body of research that shows gaps in health care providers knowledge, skills and training on EOL care in COPD in important areas such as prognostication and informing patients and families about the death process (18). In a study by Rocker et al., patients with advanced COPD at high risk of 6-month mortality completed questionnaires on what aspects of EOL care were most important to them and to rate their level of satisfaction with these aspects of care. The main unmet needs were symptom relief, not wanting to be a burden to family, being adequately informed about the risks and benefits of treatment, the availability of care staff to discuss questions in an understandable way, and adequate health services following hospital discharge (19).

In spite of the need for palliative care in COPD and ILD, patients are much less likely to receive palliative care and are less likely to die at home than those with other chronic illnesses such as cancer. In 2023, the National Hospice and Palliative Care Organization reported that of patients who enrolled in the Medicare Hospice Benefit, only 4.3% were enrolled for a diagnosis of COPD with an additional 1.1% enrolled for a diagnosis of COPD with an acute exacerbation (20). Instead of applying the traditional approach of palliating the terminal phase of a chronic illness, a better paradigm would be to incorporate palliative care principles at all stages of the illness and increase them as the illness progresses (17,21,22). This would facilitate improved symptom management, serious illness conversations, and ACP and EOL planning.

Palliative care in ILD

Due to the irreversible and progressive nature of most ILDs, it is important that palliative care is begun at the time of diagnosis, as diagnosis is a particularly distressing time for patients (23). Psychological and social support, disease education, inclusion of caregivers and practical/legal advice for disease progression and EOL planning are recommended as aspects of palliative care that should be integrated. Primary palliative care should be provided by the pulmonary team with incorporation of an interprofessional palliative care team as needs escalate. The heavy symptom burden of cough, dyspnea, mental health challenges, and poor QOL are all important palliative aspects to consider (24). It is important to emphasize to both patients and clinicians that palliative care in ILD is not synonymous with EOL care but is provided alongside disease modifying measures to relieve symptoms and optimize QOL (25). After initial stabilization, it may be possible to space out palliative care interventions and conversations, which may increase again when symptoms worsen. This important area of growth needs further study, especially because prognosis and disease trajectory are so difficult to predict.

Considerations in elders

In older patients, it is important to acknowledge the importance of comorbidities, uncontrolled symptoms, caregiver burden, and concurrent mental health complications such as depression and anxiety. Elders who are already at heightened risk of loss of muscle mass, frailty, reduced exercise tolerance can be expected to experience greater loss from the non-pulmonary manifestations of COPD such as loss of body mass index (pulmonary cachexia) and mobility related dyspnea. Elderly patients may be more susceptible to side effects of medications, particularly in the setting of polypharmacy. For example, anticholinergic drugs can worsen cognitive decline. Guideline directed therapy may not be sufficient to relieve dyspnea with advancing illness. They are at risk of functional disability, osteoporosis, falls, and fractures, especially in those that are steroid dependent (26). The physical and cognitive changes that are common in the elderly, particularly those aged 75 years and older, may interfere with the proper administration of inhaled therapies, resulting in insufficient dosing, jeopardizing health outcomes, reducing QOL, and adding to the economic burden of COPD. Consideration should be given to using nebulizers in elders who have difficulty with inhalers (26), as difficulty with coordination, pain from arthritis, timing of inhalation, and adequate airflow may impede proper delivery of medication via inhaler. Poor eyesight or hearing loss may make it more difficult to adhere to treatment. In addition, of the increase in depression in older adults may make medication adherence more difficult, particularly those who reside in nursing homes (27).

Symptom management in advanced lung disease

Patients with advanced lung disease may experience a myriad of complex overlapping symptoms, due to the disease itself and as side effects of treatment. Dyspnea and associated functional limitation tend to be very distressing. Patients with COPD experience multiple symptoms including dyspnea, anorexia, constipation, dry mouth, cough, anxiety, depression, drowsiness, irritability, and wheezing (28).

Dyspnea

Dyspnea is defined by the American Thoracic Society as the subjective experience of breathing discomfort that consists of qualitatively distinct sensations that vary in intensity. The terms “breathlessness” or “shortness of breath” are often used interchangeably with the term “dyspnea”. Multiple distinct mechanisms and afferent pathways are involved in different sensory qualities and often do not occur in isolation. For example, the feeling of chest tightness may point toward bronchospasm as an etiology of dyspnea, whereas the feeling of air hunger may refer to an increased drive to breathe with limited tidal volume. Dyspnea involves interactions across multiple physiological, social and environmental factors that may result in secondary physiological and behavioral responses (29). Amy Abernethy, taking a cue from the concept of total pain as articulated by Dame Cicely Saunders during the early hospice movement, developed the term “total dyspnea” to emphasize its physical, psychological, interpersonal (social), and spiritual elements. To adequately address dyspnea, the approach to dyspnea should be multidimensional, evaluating and managing accompanying symptoms, stressors, and spiritual distress (30,31). Dyspnea for patients with COPD is a significant source of disability and loss of health-related QOL (HRQOL), particularly for those with a lower socioeconomic background (32). This can be expected to be especially difficult for elders, many of whom live on fixed incomes and are already frail or on the cusp of frailty due to comorbidities.

While dyspnea is by definition subjective, it is important to measure dyspnea in order to adequately assess and treat it. In measuring dyspnea, Parshall et al. (29) have proposed that measurement of dyspnea be classified into three domains: as pertaining to the sensory-perceptual experience, affective distress, or symptom/disease impact or burden. For example, the modified Borg Dyspnea Scale, commonly called “Borg”, measures severity of dyspnea in a range from 0 to 10, similar to common pain scales. A Visual Analogue Scale is also sometimes used to measure severity of breathlessness, generally ranging from 0 to 10 with associated visual or verbal cues (33). Measures affective distress can include severity of distress or unpleasantness, anxiety, or consequences (29). The modified Medical Research Council (mMRC) dyspnea scale measures functional status and disability and ranges from Grade 0 to Grade 4 (34), similar to grading of function and dyspnea in heart failure.

Nonpharmacological approaches to dyspnea

After careful management of the primary condition, a multidisciplinary and multimodal nonpharmacologic approach to dyspnea should be applied for the older adult with advanced lung disease. Nonpharmacological options to target dyspnea include fans, neuromuscular electrical stimulation, assist devices, pursed lip breathing, meditation, relaxation and behavioral techniques, and acupuncture. Neuromuscular stimulation of the calves and quadriceps has been associated with improvement in dyspnea, muscular strength, and activities of daily living. Chest percussion is effective for the relief of dyspnea (35). Hand-held fans, as well as blowing air across the nose and mouth have been shown to reduce breathlessness in a broad range of patient populations (36,37). A 2012 randomized controlled trial of acupuncture in patients with moderate to advanced stage COPD randomized patients to either acupuncture or sham therapy showed an improvement in dyspnea after a 6-minute walk test, as well as improvements in overall walking distance and QOL (38), and this has been corroborated in a systematic review and meta-analysis (39).

Pulmonary rehabilitation, which includes a monitored graduated exercise program over weeks to months, has been well studied for its benefits in functional status, exercise capacity, dyspnea, and QOL for people with advanced lung disease, notably COPD and ILD (40). Tai Chi has been studied as an alternative or adjunct to traditional pulmonary rehab (41). Overall, exercise and movement is important in maintaining function and decreasing dyspnea.

In contrast, the “dyspnea spiral” or “death spiral” refers to a progressive cycle whereby older adults with pulmonary disease and associated dyspnea begin to avoid physical activity, leading to further deconditioning and functional deterioration which then in turn exacerbates the dyspnea. This cycle may be associated with depression, cachexia, and sarcopenia. A multidisciplinary approach is thus needed to tackle this multifactorial cycle (29,42,43). To better target the myriad causes of dyspnea in the population of complex older adults with respiratory illness, multiprofessional breathlessness services have been developed to support these patients. Available in a number of countries including the UK and Germany, these holistic teams may include palliative care staff, psychological support, respiratory and physical therapists, with proven benefit in a randomized controlled trial (44). The services have been well received by patients and have minimal risk with data to support improvement in breathlessness, QOL, and psychological outcomes.

Pharmacologic management of dyspnea

Opioids have long been used in the pharmacologic management of refractory dyspnea and cough for patients with advanced lung disease. Many studies target patients with COPD who have been optimally medically managed. Several systematic reviews of randomized controlled trials in COPD showed that opioids when administered orally and parenterally were effective in reducing the sensation of dyspnea (45-47). While the older reviews did not demonstrate an improvement in exercise tolerance or QOL, the 2023 systematic review by Liu et al. (45) demonstrated significant benefits of opioids in increasing exercise endurance. While this review has been criticized in its methods (48) the authors also found that intermittent short-acting opioids had significant benefit without substantial risk or harm, but long-term use of sustained-release opioids did not improve dyspnea or exercise endurance and had more significant opioid side effects. Nebulized opioids have also not been shown to be effective for the alleviation of dyspnea (29) but may be occasionally used in practice. Overall, the literature offers conflicting recommendations regarding oral and parenteral opioids for the relief of dyspnea (29,49), with most recent guidelines recommending against their use (50).

The doses of opioids for the management of dyspnea are usually lower than for the management of chronic pain, often don’t require escalation of dose over months, and are not associated with an increased rate of respiratory depression (51), nor are they associated with increased rates of hospitalization or deaths in patients who have oxygen dependent COPD (52). However, clinicians must exercise caution when starting opioids in elders with advanced lung disease. They can be expected to have reduced clearance and metabolism of opioids due to reduced hepatic and renal functioning. They also have higher prevalence of other comorbidities that render them at higher risk of side effects from opioids such as sedation and delirium due to underlying mild cognitive impairment or dementia, and respiratory depression concerns due to congestive heart failure and obstructive sleep apnea. For elders it is common clinical practice to lower the starting dose and widen dosing intervals from what is used in younger adults, especially in elders with reduced body mass indexes (BMIs). A common starting dose of opioids for dyspnea is approximately 10–20 mg of oral morphine over 24 hours, either in a single extended release formulation or divided doses such as 2.5 to 5 mg every 6 hours (51,53,54). Clinicians should expect higher rates of constipation from opioids in elders especially when they have reduced mobility (55). Selection of opioid, route of administration, and dosing regimens must be individualized with close evaluation and reassessment of patients during this critical period (56).

In contrast, benzodiazepines and antidepressants have not consistently shown clinically significant benefit for the relief of dyspnea (57). A 2016 systematic review by Simon et al found no evidence of efficacy in advanced COPD for relief from dyspnea with the use of benzodiazepines (58). Antidepressants such as sertraline and mirtazapine showed promise for modulation of dyspnea (59), but the Better-B trial, published in 2024, showed no benefit on breathlessness from mirtazapine with increased side effects (60). Therefore, benzodiazepines and antidepressants are not recommended for primary treatment of dyspnea in advanced lung disease.

Depression and anxiety

Depression and anxiety are common in patients with advanced lung disease of any type, including COPD and ILD (61). For patients with ILD, ranges for anxiety are reported between 21% to 60%, and depression is reported between 14% to 49% (62,63). Depression and anxiety are significantly associated with worse dyspnea and worse healthcare related QOL, and attention should be paid to each area to try to improve overall functional status and QOL (64,65). Family members of patients who died with COPD report that low mood was present for 77% of patients and anxiety or panic attacks were present in 35% of patients (66). A 2011 meta-analysis of 39,587 patients with COPD and 39,431 controls found that one in four COPD patients had clinically significant depressive symptoms and anxiety occurs with a prevalence of 40% (67), and a 2023 meta-analysis found a pooled global prevalence of depression among COPD patients of 34.5% (68). Studies of COPD patients indicate that COPD patients are 10 times more likely to have panic disorder than the general population (69). The level of severity of COPD does not impact levels of depression or anxiety, rather it is associated with impaired HRQOL, dyspnea and low exercise tolerance. There is some evidence to suggest that selective serotonin reuptake inhibitors improve HRQOL and depression scores (70). Serotonin norepinephrine reuptake inhibitors such as mirtazapine have also been used for depression in this older population. Benzodiazepines are commonly used in the management of panic attacks and anxiety in patients with advanced lung disease, but may be associated with significant risk and morbidity (71-73). A 2013 meta-analysis of psychological and lifestyle interventions by Coventry et al. found that multicomponent pulmonary rehabilitation was associated with improved anxiety and depression, and cognitive behavioral approaches and meditation were associated with small but insignificant improvement in symptoms (74).

Improving communication and reducing delays in EOL decision making

ACP

ACP is important advanced lung disease such as COPD and ILD, given the gradual yet unpredictable decline, with acute exacerbations that can increase the risk of more rapid decline or death. Many studies have demonstrated that patients with advance lung disease welcome and desire earlier ACP discussions (66,75-79). These may include conversations about disease course, prognosis, and the dying process as well as eliciting goals and values around treatment plans including mechanical ventilation and EOL preferences. Patients may worry that their physicians don’t understand their EOL care preferences and are reluctant to discuss them, but would prefer that their physicians initiate these discussions.

While it has been difficult to quantify how frequently ACP is actually happening among patients with advanced lung disease (80), it is well established that health care providers could benefit from training and guidance to improve their ACP skills (77). In addition to standard ACP conversations, tools such as decision supports can help patients who are facing EOL treatment choices. Structured decision aids have been studied as adjuncts for education and counseling about intubation and mechanical ventilation and found to be feasible and beneficial but have not been widely adopted (78,81). Overall, in advanced chronic lung disease such as COPD and ILD, early ACP with detailed information about prognosis, symptoms, and EOL issues is desired by patients and their caregivers, but improvements are needed in providing these conversations.

Pulmonary rehabilitation programs have been thought to be an appropriate setting for delivery of ACP given the longitudinal and interprofessional nature of the programs (75,82,83). The group setting provides an appropriate venue for broader educational sessions and discussions which have been well received by patients (84), however further education and implementation is needed in this area.

Dying with chronic lung disease

A relatively small percentage of people dying from COPD, ILD, and other chronic lung disease die with the hospice benefit in place. In the United States, Medicare has defined guidelines for hospice eligibility with an anticipated prognosis of 6 months are less (85). Guidelines for terminal pulmonary disease that support hospice eligibility include the following criteria, with a final common pathway for end stage pulmonary disease of all etiologies assumed. Severe chronic lung disease and either hypoxemia or hypercarbia are required, with other data serving as support. Documentation of severe lung disease includes disabling dyspnea at rest (documentation FEV₁ after bronchodilator, less than 30% of predicted is objective evidence for disabling dyspnea, but is not necessary) and progression of end stage pulmonary disease as evidenced by increasing use of health care services or serial decrease of FEV₁ (by more than 40 mL/year). Supporting data include right heart failure, unintentional weight loss of more than 10% in 6 months, and resting tachycardia with a heart rate greater than 100 beats per minute.

Because of the challenges with prognostication, some patients with advanced lung disease who meet all the criteria may survive for much longer on hospice care (86), and others may utilize the hospice benefit for mere days. A large epidemiologic study published in 2020 showed that hospital and nursing facility deaths for people with COPD, ILD, and cystic fibrosis have decreased substantially and home and hospice facility deaths have increased in the period from 2003 to 2017. In 2017, 34.7% of these deaths were in the home and 9% were in a hospice facility, compared to 23.3% and 0.1% in 2003 (87). This study highlights the importance of focusing on quality home palliative and hospice care in people with advanced lung disease, and continuing to improve terminal care in this population.

Conclusions

Advanced lung disease, including COPD and various types of ILD, are common disabling respiratory conditions with increasing prevalence in older adults. While the diseases tend to be gradually progressive, the time course and prognostication is highly variable with long periods of stability and occasional severe exacerbations. The symptom burden in advanced lung disease is high, with significant dyspnea, both physiologic dyspnea from hypoxia and hypercarbia as well as total dyspnea encompassing various facets of life. Functional status can be significantly impaired, and co-morbid depression and anxiety are common. Palliative care is important for symptom management as well as ACP and EOL preparations. Patients with COPD and ILD desire early ACP and it is recommended that clinicians initiate palliative care relatively early in the disease course. Further research and education are needed to enhance palliative care efforts in this complex population.

Acknowledgments

Funding: None.

Footnote

Provenance and Peer Review: This article was commissioned by Guest Editors (Pragnesh Patel, Susan Nathan and Lara Skarf) for the series “Outpatient Palliative Care in Geriatric Clinics” published in Annals of Palliative Medicine. The article has undergone external peer review.

Peer Review File: Available at https://apm.amegroups.com/article/view/10.21037/apm-24-111/prf

Conflicts of Interest: Both authors have completed the ICMJE uniform disclosure form (available at https://apm.amegroups.com/article/view/10.21037/apm-24-111/coif). The series “Outpatient Palliative Care in Geriatric Clinics” was commissioned by the editorial office without any funding or sponsorship. The authors have no other conflicts of interest to declare.

Ethical Statement: The authors are accountable for all aspects of the work in ensuring that questions related to the accuracy or integrity of any part of the work are appropriately investigated and resolved.

Open Access Statement: This is an Open Access article distributed in accordance with the Creative Commons Attribution-NonCommercial-NoDerivs 4.0 International License (CC BY-NC-ND 4.0), which permits the non-commercial replication and distribution of the article with the strict proviso that no changes or edits are made and the original work is properly cited (including links to both the formal publication through the relevant DOI and the license). See: https://creativecommons.org/licenses/by-nc-nd/4.0/.

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